Dr. Kipps grew up in Santa Cruz, California and completed her medical degree at Harvard Medical School in 2003. After general pediatrics residency at Stanford, she completed pediatric cardiology fellowship at Boston Children’s Hospital from 2006 to 2009. Having concentrated her final year of fellowship on echocardiography, she came west to practice at University of California, San Francisco until 2012. She was recruited back to Stanford in 2012 to become the medical director for acute care cardiology and she remained active in the echocardiography laboratory until 2016. In 2014 she co-founded the PAC³ network with Nicolas Madsen and co-directs this today. Her academic focus is in clinical effectiveness and quality improvement science, and she completed her Masters of Science in Epidemiology at Harvard School of Public Health in 2016. Her other significant interest is in teaching; she co-directs the pediatric residency cardiology rotation at Stanford.

Clinical Focus

  • Pediatric Cardiology
  • Quality Improvement

Academic Appointments

Administrative Appointments

  • Medical Director for Quality, LPCH Cardiology Acute Care Unit (2013 - Present)
  • Medical Director, LPCH Cardiology Acute Care Unit (2013 - 2021)

Boards, Advisory Committees, Professional Organizations

  • Co-Director, Pediatric Acute Care Cardiology Collaborative (PAC3) (2014 - Present)

Professional Education

  • MS, Harvard School of Public Health, Epidemiology (2016)
  • Board Certification: American Board of Pediatrics, Pediatric Cardiology (2010)
  • Fellowship: Children's Hospital Boston (2009) MA
  • Board Certification: American Board of Pediatrics, Pediatrics (2006)
  • Residency: Stanford Hospital and Clinics (2006) CA
  • MD, Harvard Medical School, Medicine (2003)

Graduate and Fellowship Programs

  • Pediatric Cardiology (Fellowship Program)

All Publications

  • Feeding Variation Among Infants in Acute Care Cardiology Units. Pediatric cardiology Ware, A. L., Jones, C., Kipps, A. K., Khadr, L., Marcuccio, E., Patel, S. S., Plummer, S., Ravishankar, C., Figueroa, M. 2024


    Infants with heart disease are at high risk of feeding difficulties and complications. Feeding practices amongst acute care cardiology units are not standardized. This study aims to describe feeding practices for infants at the time of discharge from a Pediatric Acute Care Cardiology Collaborative (PAC3) center and practice variation between centers. Discharge encounters for infants in the PAC3 registry between February 2019 and October 2021 were included. Nutrition type and feeding route at discharge were summarized with descriptive statistics and a modified bump plot. Center variation was assessed using funnel plots with control limits set at the 99.9% confidence interval from the group mean. A total of 15,414 encounters across 24 PAC3 centers were recorded from 8313 unique patients (median encounters 1, range 1-25). Nutrition at discharge consisted of standard formula in 8368 (54%), human milk in 6300 (41%), and elemental formula in 3230 (21%), either alone or in combination. Feeds were fortified to ≥ 24 kcal/oz in 12,359 (80%). Discharge supplemental tube feeding was present in 7353 (48%) encounters with 4643 (63%) receiving continuous feeds, 2144 (29%) bolus feeds, and 566 (8%) a combination. Funnel plots demonstrated variability in nutrition type and feeding route at discharge. Infants with heart disease commonly require high calorie nutrition and supplemental tube feedings at discharge. Feeding strategies at discharge vary widely between PAC3 centers. Collaborative approaches to identify best practices in feeding strategies are needed.

    View details for DOI 10.1007/s00246-024-03541-6

    View details for PubMedID 38907873

    View details for PubMedCentralID 6820300

  • Mind the Gap! Working Toward Gender Equity in Pediatric and Congenital Heart Disease: Present and Future. Journal of the American Heart Association Chowdhury, D., Bansal, N., Rasheed, M. M., Saidi, A., Stiver, C., Laraja, K., Ronai, C. M., Baker Smith, C., Johnson, J. N., Co-Vu, J., Jain, S. S., Kipps, A. K., Sachdeva, R., Bauser-Heaton, H., Jone, P., Choueiter, N., Ansong, A., Sutton, N., Lopez, K. N., Karamlou, T., Fuller, S., Snyder, C., Taggart, N. W., Elliott, P. B., Shaffer, K., Williams, R. 2024: e032837


    Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender-associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.

    View details for DOI 10.1161/JAHA.123.032837

    View details for PubMedID 38639355

  • Immunisation deferral practices surrounding congenital heart surgery. Cardiology in the young Gal, D. B., Cleveland, J. D., Vergales, J. E., Kipps, A. K. 2024: 1-4


    Perioperative immunisation administration surrounding congenital heart surgery is controversial. Delayed immunisation administration results in children being at risk of vaccine-preventable illnesses and is associated with failure to complete immunisation schedules. Among children with CHD, many of whom are medically fragile, vaccine-preventable illnesses can be devastating. Limited research shows perioperative immunisation may be safe and effective.We surveyed Pediatric Acute Care Cardiology Collaborative member centres and explored perioperative immunisation practices. We analysed responses using descriptive statistics.Complete responses were submitted by 35/46 (76%) centres. Immunisations were deferred for any period prior to surgery by 23 (66%) centres and after surgery by 31 (89%) centres. Among those who deferred post-operative immunisation, 20 (65%) required deferral only for patients whose operations required cardiopulmonary bypass. Duration of deferral in the pre- and post-operative periods was variable. Many centres included exceptions to their policy for specific vaccine-preventable illnesses. Almost all (34, 97%) centres administer routine childhood immunisation to patients who remain admitted for prolonged periods.Most centres defer routine childhood immunisation for some period before and after congenital heart surgery. Centre specific practices vary. Immunisation deferral confers risk to patients and may not be warranted in this population. Further research would be necessary to understand the immunologic impact of these practices.

    View details for DOI 10.1017/S1047951124000507

    View details for PubMedID 38557603

  • Early Wound and Sternal Management Following Pediatric Cardiac Surgery. World journal for pediatric & congenital heart surgery Gal, D. B., Cleveland, J. D., Kipps, A. K. 2024: 21501351231216448


    Early postoperative wound management following congenital heart surgery remains an area without equipoise. Precautionary restrictions can impact quality of life, development, and delay access to other needed care. The influence of different practices on wound healing and complications is unknown. We surveyed Pediatric Acute Care Cardiology Collaborative member centers regarding postoperative wound closure, wound vacuum-assisted closure (VAC) use, sternal precautions, and restrictions in the early postoperative period. We analyzed responses using descriptive statistics. Responses were submitted by 35/46 (76%) centers. Most centers perform primary skin closure with subcutaneous sutures. Wound covers are removed after 48 h at 43% (15/35) of centers and after ≥72 h at 34% (12/35) of centers. For delayed sternal closure, 16 centers close skin with interrupted, externalized sutures, 5 utilize wound VAC-assisted closure, and 12 use variable practices. Generally, 33 centers use wound VACs for wound care. Patient selection for VAC use and length of therapy varies. We found great variability in duration of sternal precautions and in activity, bathing, and submersion restrictions. Finally, 29 centers require a waiting period between cardiothoracic surgery and other surgeries such as tracheostomy or gastrostomy tube placement. Postoperative wound and sternal management lack consistency across North American pediatric heart institutes. Some restrictive practices may prolong length of stay and/or negatively impact quality of life and neurodevelopment. Practices may also impact wound infection rates. Research linking practices with clinical outcomes is needed to better define standards of care and reduce potential negative consequences of overly conservative or aggressive practices.

    View details for DOI 10.1177/21501351231216448

    View details for PubMedID 38263797

  • Maternal Diabetes and Cardiovascular Health in the Offspring. Pediatric cardiology Jodah, R., Arunamata, A., Kipps, A. K., Chen, S., Stauffer, K. J., Selamet Tierney, E. S. 2023


    Pulse wave velocity (PWV) has been explored to predict cardiovascular health in adults. Less is known about neonatal PWV. We evaluated the association between arterial stiffness of neonates of mothers (NoM) with diabetes and childhood health.Neonatal brachial-femoral PWV (bfPWV) was measured after birth and neonates followed for a median of 5.2 years [1 month-6.6 years]. 36 pregnant women with pregestational diabetes mellitus PGDM (n= 12), gestational diabetes mellitus (GDM) (n = 13), and controls (n= 11) were enrolled. Neonates were similar in weight, gestational age, and delivery mode. 26 neonates had follow-up data including weight, height and blood pressure.More mothers with PGDM had poor glycemic control compared to mothers with GDM (83% vs. 8%; p =0.0002). PWV was higher in NoM with PGDM than controls (3.4± 0.5 vs. 2.6±0.8 m/s; p= 0.04). At follow-up, children of mothers with diabetes (n= 16) had higher weight percentile (78.5 ± 27.9 vs 49.5± 34.6%; p= 0.02) and diastolic blood pressure (DBP) (68± 13.6 vs 57.3± 4.3mmHg; p=0.01) than controls (n = 10). No correlation emerged between neonatal PWV and childhood body mass index (BMI) or maternal HbA1c. Results suggest maternal diabetes affect neonatal arterial stiffness and childhood blood pressure; however, the mechanism is unclear. The long-term implications of these findings warrant further investigation.

    View details for DOI 10.1007/s00246-023-03333-4

    View details for PubMedID 37930377

  • Caregiver and provider attitudes toward family-centred rounding in paediatric acute care cardiology. Cardiology in the young Gal, D. B., Rodts, M., Hills, B. K., Kipps, A. K., Char, D. S., Pater, C., Madsen, N. L. 2023: 1-6


    Family-centered rounding has emerged as the gold standard for inpatient paediatrics rounds due to its association with improved family and staff satisfaction and reduction of harmful errors. Little is known about family-centered rounding in subspecialty paediatric settings, including paediatric acute care cardiology.In this qualitative, single centre study, we conducted semi-structured interviews with providers and caregivers eliciting their attitudes toward family-centered rounding. An a priori recruitment approach was used to optimise diversity in reflected opinions. A brief demographic survey was completed by participants. We completed thematic analysis of transcribed interviews using grounded theory.In total, 38 interviews representing the views of 48 individuals (11 providers, 37 caregivers) were completed. Three themes emerged: rounds as a moment of mutual accountability, caregivers' empathy for providers, and providers' objections to family-centered rounding. Providers' objections were further categorised into themes of assumptions about caregivers, caregiver choices during rounds, and risk for exacerbation of bias and inequity.Caregivers and providers in the paediatric acute care cardiology setting echoed some previously described attitudes toward family-centered rounding. Many of the challenges surrounding family-centered rounding might be addressed through access to training for caregivers and providers alike. Hospitals should invest in systems to facilitate family-centered rounding if they choose to implement this model of care as the current state risks erosion of provider-caregiver relationship.

    View details for DOI 10.1017/S104795112300118X

    View details for PubMedID 37198962

  • Direct Discharge to Home From the Pediatric Cardiovascular ICU. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Gal, D. B., Kwiatkowski, D. M., Cribb Fabersunne, C., Kipps, A. K. 1800


    OBJECTIVES: To describe direct discharge to home from the cardiovascular ICU.DESIGN: Mixed-methods including retrospective Pediatric Cardiac Critical Care Consortium and Pediatric Acute Care Cardiology Collaborative data and survey.SETTING: Tertiary pediatric heart center.PATIENTS: Patients less than 25 years old, with a cardiovascular ICU stay of greater than 24 hours and direct discharge to home from January 1, 2016, to December 8, 2020, were included. Select data describing patients discharged from acute care internally and nationally from Pediatric Acute Care Cardiology Collaborative sites were compared with the direct discharge to home cohort.INTERVENTIONS: None.MEASUREMENTS AND MAIN RESULTS: Encounter- and patient-specific characteristics. Seven-day and 30-day readmission and 30-day mortality served as surrogate safety markers. A survey of cardiovascular ICU frontline providers assessed comfort and skills related to direct discharge to home. There were 364 direct discharge to home encounters that met inclusion criteria. The majority of direct discharge to home encounters were associated with a surgery or procedure (305; 84%). There were 27 encounters (7.4%) for medical technology-dependent patients requiring direct discharge to home. Unplanned 7-day readmissions among direct discharge to home patients was 1.9% compared with 4.6% (p = 0.04) of patients discharged from acute care internally. Readmission among those discharged from acute care internally did not differ from those at Pediatric Acute Care Cardiology Collaborative sites nationally. Frontline cardiovascular ICU providers had mixed levels of confidence in technical aspects and low levels of confidence in logistics of direct discharge to home.CONCLUSIONS: Cardiovascular ICU direct discharge to home was not associated with increased unplanned readmissions compared with patients discharged from acute care and may be safe in select patients. Frontline cardiovascular ICU providers feel time constraints challenge direct discharge to home. Further research is needed to identify patient characteristics associated with safe direct discharge to home and systems needed to support this practice.Summary statistics are described using proportions or medians with interquartile ranges (IQRs) and were performed using Microsoft Excel (Microsoft, Redmond, WA). Two-sample tests of proportions were used to compare readmission frequency of the DDH cohort compared with internal and national PAC3 data using STATA Version 15 (StataCorp, College Station, TX).

    View details for DOI 10.1097/PCC.0000000000002883

    View details for PubMedID 35044343

  • Successful Reduction of Postoperative Chest Tube Duration and Length of Stay After Congenital Heart Surgery: A Multicenter Collaborative Improvement Project JOURNAL OF THE AMERICAN HEART ASSOCIATION Bates, K. E., Connelly, C., Khadr, L., Graupe, M., Hlavacek, A. M., Morell, E., Pasquali, S. K., Russell, J. L., Schachtner, S. K., Strohacker, C., Tanel, R. E., Ware, A. L., Wooton, S., Madsen, N. L., Kipps, A. K. 2021; 10 (21): e020730


    Background Congenital heart disease practices and outcomes vary significantly across centers, including postoperative chest tube (CT) management, which may impact postoperative length of stay (LOS). We used collaborative learning methods to determine whether centers could adapt and safely implement best practices for CT management, resulting in reduced postoperative CT duration and LOS. Methods and Results Nine pediatric heart centers partnered together through 2 learning networks. Patients undergoing 1 of 9 benchmark congenital heart operations were included. Baseline data were collected from June 2017 to June 2018, and intervention-phase data were collected from July 2018 to December 2019. Collaborative learning methods included review of best practices from a model center, regular data feedback, and quality improvement coaching. Center teams adapted CT removal practices (eg, timing, volume criteria) from the model center to their local resources, practices, and setting. Postoperative CT duration in hours and LOS in days were analyzed using statistical process control methodology. Overall, 2309 patients were included. Patient characteristics did not differ between the study and intervention phases. Statistical process control analysis showed an aggregate 15.6% decrease in geometric mean CT duration (72.6 hours at baseline to 61.3 hours during intervention) and a 9.8% reduction in geometric mean LOS (9.2 days at baseline to 8.3 days during intervention). Adverse events did not increase when comparing the baseline and intervention phases: CT replacement (1.8% versus 2.0%, P=0.56) and readmission for pleural effusion (0.4% versus 0.5%, P=0.29). Conclusions We successfully lowered postoperative CT duration and observed an associated reduction in LOS across 9 centers using collaborative learning methodology.

    View details for DOI 10.1161/JAHA.121.020730

    View details for Web of Science ID 000713661400026

    View details for PubMedID 34713712

  • Early Functional Status After Surgery for Congenital Heart Disease: A Single-Center Retrospective Study. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Han, B., Yang, J. K., Ling, A. Y., Ma, M., Kipps, A. K., Shin, A. Y., Beshish, A. G. 2021


    OBJECTIVES: The objective of this study is to investigate the change in functional status in infants, children, and adolescents undergoing congenital heart surgery using the Functional Status Scale.DESIGN: A single-center retrospective study.SETTING: A 26-bed cardiac ICU in a free-standing university-affiliated tertiary children's hospital.PATIENTS: All patients 0-18 years who underwent congenital heart surgery from January 1, 2014, to December 31, 2017.INTERVENTIONS: None.MEASUREMENTS AND MIN RESULTS: The primary outcome variable was change in Functional Status Scale scores from admission to discharge. Additionally, two binary outcomes were derived from the primary outcome: new morbidity (change in Functional Status Scale ≥ 3) and unfavorable functional outcome (change in Functional Status Scale ≥ 5); their association with risk factors was assessed using modified Poisson regression. Out of 1,398 eligible surgical encounters, 65 (4.6%) and 15 (1.0%) had evidence of new morbidity and unfavorable functional outcomes, respectively. Higher Surgeons Society of Thoracic and the European Association for Cardio-Thoracic Surgery score, single-ventricle physiology, and longer cardiopulmonary bypass time were associated with new morbidity. Longer hospital length of stay was associated with both new morbidity and unfavorable outcome.CONCLUSIONS: This study demonstrates the novel application of the Functional Status Scale on patients undergoing congenital heart surgery. New morbidity was noted in 4.6%, whereas unfavorable outcome in 1%. There was a small change in the total Functional Status Scale score that was largely attributed to changes in the feeding domain. Higher Society of Thoracic and the European Association for Cardio-Thoracic Surgery score, single-ventricle physiology, and longer cardiopulmonary bypass times were associated with new morbidity, whereas longer hospital length of stay was associated with both new morbidity and unfavorable outcome. Further studies with larger sample size will need to be done to confirm our findings and to better ascertain the utility of Functional Status Scale on this patient population.

    View details for DOI 10.1097/PCC.0000000000002838

    View details for PubMedID 34593740

  • High Acuity Therapy Variation Across Pediatric Acute Care Cardiology Units: Results from the Pediatric Acute Care Cardiology Collaborative Hospital Surveys. Pediatric cardiology Harahsheh, A. S., Kipps, A. K., Hart, S. A., Cassidy, S. C., Clabby, M. L., Hlavacek, A. M., Hoerst, A. K., Graupe, M. A., Madsen, N. L., Bakar, A. M., Del Grippo, E. L., Patel, S. S., Bost, J. E., Tanel, R. E., Pediatric Acute Care Cardiology Collaborative (PAC3) 2021


    We utilized the multicenter Pediatric Acute Care Cardiology Collaborative (PAC3) 2017 and 2019 surveys to describe practice variation in therapy availability and changes over a 2-year period. A high acuity therapies (ATs) score was derived (1 point per positive response) from 44 survey questions and scores were compared to center surgical volume. Of 31 centers that completed the 2017 survey, 26 also completed the 2019 survey. Scores ranged from 11 to 34 in 2017 and 11 to 35 in 2019. AT scores in 2019 were not statistically different from 2017 scores (29/44, IQR 27-32.5 vs. 29.5/44, IQR 27-31, p=0.9). In 2019, more centers reported initiation of continuous positive airway pressure (CPAP) and Bi-level positive airway pressure (BiPAP) in Acute Care Cardiology Unit (ACCU) (19/26 vs. 4/26, p<0.001) and permitting continuous CPAP/BiPAP (22/26 vs. 14/26, p=0.034) compared to 2017. Scores in both survey years were significantly higher in the highest surgical volume group compared to the lowest, 33±1.5 versus 25±8.5, p=0.046 and 32±1.7 versus 23±5.5, p=0.009, respectively. Variation in therapy within the ACCUs participating in PAC3 presents an opportunity for shared learning across the collaborative. Experience with PAC3 was associated with increasing available respiratory therapies from 2017 to 2019. Whether AT scores impact the quality and outcomes of pediatric acute cardiac care will be the subject of further investigation using a comprehensive registry launched in early 2019.

    View details for DOI 10.1007/s00246-021-02584-3

    View details for PubMedID 33813599

  • Target Based Care: An Intervention to Reduce Variation in Postoperative Length of Stay. The Journal of pediatrics Shin, A. Y., Rao, I. J., Bassett, H. K., Chadwick, W., Kim, J., Kipps, A. K., Komra, K., Loh, L., Maeda, K., Mafla, M., Presnell, L., Sharek, P. J., Steffen, K. M., Scheinker, D., Algaze, C. A. 2020


    OBJECTIVES: To derive care targets and evaluate the impact of displaying them at the point of care on postoperative length of stay (LOS).STUDY DESIGN: A prospective cohort study using 2 years of historical controls within a freestanding, academic children's hospital. Patients undergoing benchmark cardiac surgery between May 4, 2014 and August 15, 2016 (preintervention) and September 6, 2016 to September 30, 2018 (postintervention) were included. The intervention consisted of displaying at the point of care targets for the timing of extubation, transfer from the intensive care unit (ICU), and hospital discharge. Family satisfaction, reintubation, and readmission rates were tracked.RESULTS: The postintervention cohort consisted of 219 consecutive patients. There was a reduction in variation for ICU (difference in SD -2.56, p < 0.01), and total LOS (difference in SD -2.84, P < .001). Patients stayed on average 0.97 fewer days (p<0.001) in the ICU (median -1.01 [IQR -2.15,-0.39], 0.7 fewer days (p<0.001) on mechanical ventilation (median -0.54 [IQR -0.77,-0.50], and 1.18 fewer days (p<0.001) for the total LOS (median -2.25 [IQR -3.69,-0.15]. Log transformed multivariable linear regression demonstrated the intervention to be associated with shorter ICU LOS (beta coefficient -0.19, SE 0.059, p<0.001), total postoperative LOS (beta coefficient -0.12, SE 0.052, p=0.02), and ventilator duration (beta coefficient -0.21, SE 0.048, p<0.001). Balancing metrics did not differ after the intervention.CONCLUSIONS: Target based care is a simple, novel intervention associated with reduced variation in LOS and absolute LOS across a diverse spectrum of complex cardiac surgeries.

    View details for DOI 10.1016/j.jpeds.2020.09.017

    View details for PubMedID 32920104

  • The Stanford acute heart failure symptom score for patients hospitalized with heart failure. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Almond, C. S., Chen, S., Dykes, J. C., Kwong, J., Burstein, D. S., Rosenthal, D. N., Kipps, A. K., Teuteberg, J., Murray, J. M., Kaufman, B. D., Hollander, S. A., Profita, E., Yarlagadda, V. Y., Sacks, L. D., Chen, C. 2020


    BACKGROUND: Currently, there are no simple tools to evaluate the acute heart failure (HF) symptom severity in children hospitalized with acute decompensated HF (ADHF). We sought to develop an inpatient HF score (HFS) that could be used as a clinical tool and for clinical trials.METHODS: Pediatric HF clinicians at Stanford reviewed the limitations of existing HFSs, which include lack of calibration to the inpatient setting, omission of gastrointestinal symptoms, need for multiple age-based tools, and scores that prioritize treatment intensity over patient symptoms. To address these, we developed an acute HFS corresponding to the 3 cardinal symptoms of HF: difficulty with breathing, feeding, and activity. The score was iteratively improved over a 3-year pilot phase until no further changes were made. The inter-rater reliability (IRR) across a range of providers was assessed using the final version. Peak HFSs were analyzed against mortality and length of stay (LOS) for all pediatric HF discharges between July and October 2019.RESULTS: The final HFS was a 4-point ordinal severity score for each of the 3 symptom domains (total score 0-12). Among clinicians who scored 12 inpatients with ADHF simultaneously, the intraclass correlation (ICC) was 0.94 (respiratory ICC = 0.89, feeding ICC = 0.85, and activity ICC = 0.80). Score trajectory reflected our clinical impression of patient response to HF therapies across a range of HF syndromes including 1- and 2-ventricle heart disease and reduced or preserved ejection fraction. Among the 28 patients hospitalized during a 3-months period (N = 28), quartiles of peak score were associated with LOS (p < 0.01) and in-hospital mortality (p < 0.01): HFS 0 to 3 (median LOS of 5 days and mortality of 0%), HFS 4 to 6 (median LOS of 18 days and mortality of 0%), HFS 5 to 9 (median LOS of 29 days and mortality of 23%), and HFS 10 to 12 (median LOS of 121 days and mortality of 50%).CONCLUSION: This simple acute HFS may be a useful tool to quantify and monitor day-to-day HF symptoms in children hospitalized with ADHF regardless of etiology or age group. The score has excellent IRR across provider levels and is associated with major hospital outcomes supporting its clinical validity. Validation in a multicenter cohort is warranted.

    View details for DOI 10.1016/j.healun.2020.08.002

    View details for PubMedID 33032871

  • Schedule-based Family-centered Rounds: A Novel Approach to Achieve High Nursing Attendance and Participation. Pediatric quality & safety Kipps, A. K., Albert, M. S., Bomher, S., Cheung, S., Feehan, S., Kim, J. 2020; 5 (2): e265


    Introduction: Bedside nurse (RN) presence during family-centered rounds (FCR) enhances communication and collaboration for safer, higher-quality care.1-3 At our institution, RN participation in FCR was variable and lower than desired. The content discussed at each bedside during rounds was inconsistent, contributing to the irregular achievement of established FCR checklist items.Methods: Using a scheduling tool with a prioritization algorithm and set time allotment/patient, we implemented schedule-based family-centered rounds (SBFCR) on a pediatric acute care unit. Primary outcome metrics included RN attendance and participation. We tracked rounding checklist compliance, parent presence on rounds, and adherence to the schedule. Surveys provided information on provider and family satisfaction. Perceived impact on teaching was the balancing measure because the structure discouraged spending extra time at the cost of team tardiness for the next patient.Results: We created a schedule for 95% workweek days, with the rounding order kept for 93%. Mean RN attendance increased from 69% to 87% and participation increased from 48% to 80% with SBFCR (P < 0.001 for each). FCR checklist compliance increased from 60% to 94% (P < 0.001). Families felt more informed and able to attend; their presence at rounds rose from 66% to 85% (P < 0.001). Most faculty and trainees felt SBFCR was efficient and observed increased teaching with SBFCR.Conclusions: SBFCR provides an organizational framework for increased RN attendance and participation as well as greater family presence during rounds. The system elevated provider satisfaction with rounding without degrading the perceived educational experience.

    View details for DOI 10.1097/pq9.0000000000000265

    View details for PubMedID 32426631

  • Intensive care unit and acute care unit length of stay following congenital heart surgery. The Annals of thoracic surgery Hart, S. A., Tanel, R. E., Kipps, A. K., Hoerst, A. K., Graupe, M. A., Cassidy, S. C., Hlavacek, A. M., Clabby, M. L., Bush, L. B., Zhang, W., Banerjee, M. M., Pasquali, S. K., Gaies, M., Madsen, N. 2020


    BACKGROUND: Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals following congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on post-operative LOS.METHODS: Congenital heart surgery hospitalizations within the Pediatric Cardiac Critical Care Consortium (PC4) registry (8/2014-2/2018) were included. Models were developed for ICU, ACU, and post-operative LOS adjusting for differences in case-mix across hospitals. PC4 hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC3) were also surveyed on ACU organizational factors and practice patterns.RESULTS: Overall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Post-operative LOS appeared to be most closely related to ICU LOS; 75% (6/8) of hospitals with shorter-than-expected post-operative LOS also had shorter-than-expected ICU LOS. A clear relationship between post-operative and ACU LOS was not observed. Hospitals with an ACU able to provide higher acuity care as indexed according to the PAC3 survey were more likely to have shorter post-operative LOS (p <0.01).CONCLUSIONS: For hospitals that achieve shorter-than-expected post-operative LOS following congenital heart surgery, ICU LOS appears to be the primary driver. Higher acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing post-operative LOS.

    View details for DOI 10.1016/j.athoracsur.2020.01.033

    View details for PubMedID 32114048

  • ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Sachdeva, R., Valente, A., Armstrong, A. K., Cook, S. C., Han, B., Lopez, L., Lui, G. K., Pickard, S. S., Powell, A. J., Bhave, N. M., Pickard, S. S., Baffa, J. M., Banka, P., Cohen, S. B., Glickstein, J. S., Kanter, J. P., Kanter, R. J., Kim, Y. Y., Kipps, A. K., Latson, L. A., Lin, J. P., Parra, D. A., Rodriguez, F. H., Saarel, E. V., Srivastava, S., Stephenson, E. A., Stout, K. K., Zaidi, A. N., Gluckman, T. J., Aggarwal, N. R., Bhave, N. M., Dehmer, G. J., Gilbert, O. N., Kumbhani, D. J., Price, A. L., Winchester, D. E., Gulati, M., Dehmer, G. J., Doherty, J. U., Bhave, N. M., Daugherty, S. L., Dean, L. S., Desai, M. Y., Gillam, L. D., Mehrotra, P. 2020; 75 (6): 657–703

    View details for DOI 10.1016/j.jacc.2019.10.002

    View details for Web of Science ID 000512903000013

    View details for PubMedID 31918898

  • The added value of the advanced practice provider in paediatric acute care cardiology. Cardiology in the young Willis, A. J., Hoerst, A. n., Hart, S. A., Holbein, D. n., Lowery, K. n., Harahsheh, A. S., Kipps, A. K., Madsen, N. n., Patel, S. S., Tanel, R. E. 2020: 1–4


    Advanced practice providers (APPs) are being employed at increasing rates in order to meet new in-hospital care demands. Utilising the Paediatric Acute Care Cardiology Collaborative (PAC3) hospital survey, we evaluated variations in staffing models regarding first-line providers and assessed associations with programme volume, acuity of care, and post-operative length of stay (LOS).The PAC3 hospital survey defined staffing models and resource availability across member institutions. A resource acuity score was derived for each participating acute care cardiology unit. Surgical volume was obtained from The Society of Thoracic Surgeons database. Pearson's correlation coefficients were used to evaluate the relationship between staffing models and centre volume as well as unit acuity. A previously developed case-mix adjustment model for total post-operative LOS was utilised in a multinomial regression model to evaluate the association of APP patient coverage with observed-to-expected post-operative LOS.Surveys were completed by 31 (91%) PAC3 centres in 2017. Nearly all centres (94%) employ APPs, with a mean of 1.7 (range 0-5) APPs present on weekday rounds. The number of APPs present has a positive correlation with surgical volume (r = 0.49, p < 0.01) and increased acuity (r = 0.39, p = 0.03). In the multivariate model, as coverage by APPs increased from low to moderate or high, there was greater likelihood of having a shorter-than-expected post-operative LOS (p < 0.001).The incorporation of paediatric acute care cardiology APPs is associated with reduced post-operative LOS. Future studies are necessary to understand how APPs impact these patient-specific outcomes.

    View details for DOI 10.1017/S1047951120003789

    View details for PubMedID 33143784

  • Center Variation in Chest Tube Duration and Length of Stay After Congenital Heart Surgery. The Annals of thoracic surgery Bates, K. E., Madsen, N. L., Khadr, L., Gao, Z., Crawford, K., Gaies, M., Graupe, M., Hanke, S. P., Hlavacek, A. M., Morell, E., Pasquali, S. K., Russell, J. L., Schachtner, S. K., Tanel, R. E., Ware, A. L., Kipps, A. K. 2019


    BACKGROUND: Nearly every child undergoing congenital heart surgery has chest tubes (CT) placed intraoperatively. Center variation in removal practices and impact on outcomes has not been studied previously. We evaluated variation in CT management practices and outcomes across centers.METHODS: We included patients undergoing any of 10 benchmark operations from June 2017-May 2018 at participating Pediatric Acute Care Cardiology Collaborative (PAC3)- Pediatric Cardiac Critical Care Consortium (PC4) centers. Clinical data from PC4 were merged with CT data from PAC3. Practices and outcomes were compared across centers in univariate and multivariable analysis.RESULTS: The cohort included 1029 patients (N=9 centers). Median CT duration varied significantly across centers for 9/10 benchmark operations (all p≤ 0.03) with a "model" center noted to have the shortest duration for 9/10 operations (range of 27.9-87.4% shorter duration vs. other centers across operations). This effect persisted in multivariable analysis (p<0.0001). The model center had higher volumes of CT output prior to removal [median 8.5 (model) vs 2.2 (other centers) cc/kg/24 hours, p <0.001], but did not have higher rates of CT reinsertion (model center 1.3% vs. 2.1%, p = 0.59) or readmission for pleural effusion (model center 4.4% vs. 3.0%, p = 0.31), and had the shortest LOS for 7/10 operations.CONCLUSIONS: This study suggests significant center variation in CT removal practices and associated outcomes after congenital heart surgery. Best practices utilized at the model center have informed the design of an ongoing collaborative learning project aimed at reducing CT duration and LOS.

    View details for DOI 10.1016/j.athoracsur.2019.09.078

    View details for PubMedID 31760054

  • "Echo pause" for postoperative transthoracic echocardiographic surveillance. Echocardiography (Mount Kisco, N.Y.) Cox, K., Arunamata, A., Krawczeski, C. D., Reddy, C., Kipps, A. K., Long, J., Roth, S. J., Axelrod, D. M., Hanley, F., Shin, A., Selamet Tierney, E. S. 2019


    BACKGROUND: No guidelines exist for inpatient postoperative transthoracic echocardiographic (TTE) surveillance in congenital heart disease. We prospectively evaluated indications for postoperative TTEs in patients with congenital heart disease to identify areas to improve upon (Phase 1) and then assessed the impact of a simple pilot intervention (Phase 2).METHODS: We included patients with RACHS-1 (Risk Adjustment for Congenital Heart Surgery) scores of 2 and 3 to keep the cohort homogenous. During Phase 1, we collected data prospectively to identify postoperative TTEs for which there were no new findings and no associated clinical management decisions ("potentially redundant" TTEs). During Phase 2, prior to placement of a TTE order, an "Echo Pause" was performed during rounds to prompt review of prior TTE results and indication for the current order. The number of "potentially redundant" TTEs during Phase 1 vs. Phase 2 was compared.RESULTS: During Phase 1, 98 postoperative TTEs were performed on 51 patients. Potentially "redundant" TTEs were identified in two main areas: (a) TTEs ordered to evaluate pericardial effusion and (b) TTEs ordered with the indication of "postoperative," "follow-up," or "discharge" in the setting of a prior complete postoperative TTE and no apparent change in clinical status. During Phase 2, 101 TTEs were performed on 63 patients. The number of "potentially redundant" TTEs decreased from 14/98 (14%) to 5/101 (5%) (P=.026).CONCLUSION: Our results suggest that the number of "potentially redundant" TTEs during inpatient postoperative surveillance of patients with congenital heart disease can be decreased by a simple intervention during rounds such as an "Echo Pause."

    View details for DOI 10.1111/echo.14505

    View details for PubMedID 31628768

  • Variation in care practices across pediatric acute care cardiology units: Results of the Pediatric Acute Care Cardiology Collaborative (PAC(3)) hospital survey CONGENITAL HEART DISEASE Hoerst, A., Bakar, A., Cassidy, S. C., Clabby, M., Del Grippo, E., Graupe, M., Harahsheh, A. S., Hlavacek, A. M., Hart, S. A., Kipps, A. K., Madsen, N. L., O'Neil, D. D., Patel, S. S., Strohacker, C. M., Tanel, R. E., Pediat Acute Care Cardiology Col 2019; 14 (3): 419–26

    View details for DOI 10.1111/chd.12739

    View details for Web of Science ID 000471070900014

  • Parental Acquisition of Echocardiographic Images in Pediatric Heart Transplant Patients Using a Handheld Device: A Pilot Telehealth Study JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Dykes, J. C., Kipps, A. K., Chen, A., Nourse, S., Rosenthal, D. N., Tierney, E. 2019; 32 (3): 404–11
  • Cardiac Networks United: an integrated paediatric and congenital cardiovascular research and improvement network. Cardiology in the young Gaies, M., Anderson, J., Kipps, A., Lorts, A., Madsen, N., Marino, B., Costello, J. M., Brown, D., Jacobs, J. P., Kasnic, D., Lihn, S., Lannon, C., Margolis, P., Pearson, G. D., Kaltman, J., Charpie, J. R., Redington, A. N., Pasquali, S. K., Cardiac Networks United Executive Committee and Advisory Board 2018: 1–8


    Optimising short- and long-term outcomes for children and patients with CHD depends on continued scientific discovery and translation to clinical improvements in a coordinated effort by multiple stakeholders. Several challenges remain for clinicians, researchers, administrators, patients, and families seeking continuous scientific and clinical advancements in the field. We describe a new integrated research and improvement network - Cardiac Networks United - that seeks to build upon the experience and success achieved to-date to create a new infrastructure for research and quality improvement that will serve the needs of the paediatric and congenital heart community in the future. Existing gaps in data integration and barriers to improvement are described, along with the mission and vision, organisational structure, and early objectives of Cardiac Networks United. Finally, representatives of key stakeholder groups - heart centre executives, research leaders, learning health system experts, and parent advocates - offer their perspectives on the need for this new collaborative effort.

    View details for DOI 10.1017/S1047951118001683

    View details for PubMedID 30567622

  • Variability in paediatric cardiac postoperative chest tube management CARDIOLOGY IN THE YOUNG Taylor, A. C., Bates, K. E., Kipps, A. K. 2018; 28 (12): 1471–74
  • Impact of Intensive Care Unit and Acute Care Unit Length of Stay on Total Length of Stay Following Congenital Heart Surgery: A Joint PC4 and PAC3 Multicenter Analysis Hart, S. A., Tanel, R. E., Kipps, A. K., Hoerst, A., Graupe, M., Cassidy, S. C., Hlavacek, A. M., Clabby, M. L., Bush, L., Zhang Wenying, Banerjee, M., Pasquali, S. K., Gaies, M. G., Madsen, N. LIPPINCOTT WILLIAMS & WILKINS. 2018
  • Applying Lessons from an Inaugural Clinical Pathway to Establish a Clinical Effectiveness Program. Pediatric quality & safety Algaze, C. A., Shin, A. Y., Nather, C., Elgin, K. H., Ramamoorthy, C., Kamra, K., Kipps, A. K., Yarlagadda, V. V., Mafla, M. M., Vashist, T., Krawczeski, C. D., Sharek, P. J. 2018; 3 (6): e115


    Introduction: Clinical effectiveness (CE) programs promote standardization to reduce unnecessary variation and improve healthcare value. Best practices for successful and sustainable CE programs remain in question. We developed and implemented our inaugural clinical pathway with the aim of incorporating lessons learned in the build of a CE program at our academic children's hospital.Methods: The Lucile Packard Children's Hospital Stanford Heart Center and Center for Quality and Clinical Effectiveness partnered to develop and implement an inaugural clinical pathway. Project phases included team assembly, pathway development, implementation, monitoring and evaluation, and improvement. We ascertained Critical CE program elements by focus group discussion among a multidisciplinary panel of experts and key affected groups. Pre and postintervention compared outcomes included mechanical ventilation duration, cardiovascular intensive care unit, and total postoperative length of stay.Results: Twenty-seven of the 30 enrolled patients (90%) completed the pathway. There was a reduction in ventilator days (mean 1.0+0.5 versus 1.9+1.3 days; P < 0.001), cardiovascular intensive care unit (mean 2.3+1.1 versus 4.6+2.1 days; P < 0.001) and postoperative length of stay (mean 5.9+1.6 versus 7.9+2.7 days; P < 0.001) compared with the preintervention period. Elements deemed critical included (1) project prioritization for maximal return on investment; (2) multidisciplinary involvement; (3) pathway focus on best practices, critical outcomes, and rate-limiting steps; (4) active and flexible implementation; and (5) continuous data-driven and transparent pathway iteration.Conclusions: We identified multiple elements of successful pathway implementation, that we believe to be critical foundational elements of our CE program.

    View details for DOI 10.1097/pq9.0000000000000115

    View details for PubMedID 31334447

  • Utility of screening echocardiogram after endomyocardial biopsy for identification of cardiac perforation or tricuspid valve injury. Pediatric transplantation Peng, D. M., Kipps, A. K., Palmon, M., Tacy, T. A., Peng, L. F., Hollander, S. A., McElhinney, D. B. 2018; 22 (7): e13275


    Per protocol, our institution obtains echocardiograms immediately after each EMB to rule out procedural complication. We sought to determine the incidence of echocardiogram-detected cardiac perforation and TV injury and to evaluate the utility of routine screening echocardiogram after each EMB in the current era. At a single center, 99% (1917/1942) EMB performed in 162 patients were immediately followed by an echocardiogram per protocol. There were five newly diagnosed pericardial effusions, and only one required pericardiocentesis. In the three echocardiograms demonstrating new flail TV, only one patient underwent surgical repair 2months later. This study demonstrates the very low incidence of significant hemopericardium and TV injury after EMB in pediatric heart transplant recipients and argues against the utility of post-EMB echocardiograms to screen solely for procedural complications.

    View details for PubMedID 30076684

  • Collective quality improvement in the paediatric cardiology acute care unit: establishment of the Pediatric Acute Care Cardiology Collaborative (PAC(3)) CARDIOLOGY IN THE YOUNG Kipps, A. K., Cassidy, S. C., Strohacker, C. M., Graupe, M., Bates, K. E., McLellan, M. C., Harahsheh, A. S., Hanke, S. P., Tanel, R. E., Schachtner, S. K., Gaies, M., Madsen, N. 2018; 28 (8): 1019–23
  • Inpatient-Derived Vital Sign Parameters Implementation: An Initiative to Decrease Alarm Burden PEDIATRICS Kipps, A. K., Poole, S. F., Slaney, C., Feehan, S., Longhurst, C. A., Sharek, P. J., Goel, V. V. 2017; 140 (2)
  • Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience JOURNAL OF PEDIATRICS Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Tierney, E. S. 2017; 180: 87-?


    To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

    View details for DOI 10.1016/j.jpeds.2016.09.061

    View details for Web of Science ID 000390028100018

  • Pediatric Echocardiography by Work Relative Value Units: Is Study Complexity Adequately Captured? Journal of the American Society of Echocardiography Balasubramanian, S., Kipps, A. K., Smith, S. N., Tacy, T. A., Selamet Tierney, E. S. 2016; 29 (11): 1084-1091


    Present resource-based relative value unit (RVU) assignment for echocardiography is based on Current Procedural Terminology (CPT) codes, which do not incorporate complexity of diagnosis, time spent for image acquisition, or interpretation of echocardiograms. The objective of this study was to determine whether CPT-based RVU assignment accurately reflects physician effort in performing and interpreting pediatric echocardiographic examinations.Cardiac complexity category (CCC) and physician time for study interpretation of 123 echocardiograms (June to September 2013) were prospectively assigned. Categories included (1) focused effusion/function evaluation, (2) normal anatomy/focused preterm infant studies, (3) acquired heart disease, (4) congenital heart disease excluding single ventricles, (5) single ventricles including heterotaxy syndrome, and (6) hearts on mechanical support. Subsequently, a random sample of echocardiograms (March to August 2013) were retrospectively analyzed, and each study was assigned a CCC and an extrapolated median interpretation time (MIT) on the basis of prospective data collection. Assigned work RVUs based on CPT codes were recorded. Comparisons were made between CCC and time for study interpretation, work RVUs, number of images acquired, and total scan time.A total of 933 echocardiograms were analyzed: 198 (21%), 174 (19%), 98 (11%), 359 (35%), 84 (9%), and 20 (2%) studies in CCCs 1 to 6, respectively. Total scan time, MIT, number of images, and work RVUs were different among CCCs (P < .0001). However, among the more complex studies (CCCs 2-5), work RVUs were similar, while number of images obtained and MIT were different (P < .001). Correlation analysis showed no association between work RVUs and CCC, total scan, or number of images per study. Compared with older patients, work RVUs of studies in children <2 years of age were lower, while all other markers of study complexity were higher (P < .05).Current CPT-based assignment of work RVUs does not discriminate study complexity and physician effort. The results of this study highlight the need for a refined system that accurately assesses physician effort in pediatric echocardiography.

    View details for DOI 10.1016/j.echo.2016.05.015

    View details for PubMedID 27405593

  • Decompressing vein and bilateral superior venae cavae in a patient with hypoplastic left heart syndrome. Echocardiography (Mount Kisco, N.Y.) Stauffer, K. J., Arunamata, A., Vasanawala, S. S., Behera, S. K., Kipps, A. K., Silverman, N. H. 2016; 33 (9): 1428-1431


    The levoatrial cardinal vein (LACV), first described in 1926, acts as a decompressing vessel for pulmonary venous return in cases of severe left-sided obstruction with an intact or significantly restrictive atrial septum. The LACV and the persistent left superior vena cava (LSVC) are thought to share similar embryologic origins. To challenge this notion, we present a unique case of a neonate with hypoplastic left heart syndrome, cor triatriatum, and a decompressing LACV in the presence of bilateral superior venae cavae.

    View details for DOI 10.1111/echo.13292

    View details for PubMedID 27641733

  • Utility of the Routine Screening Echocardiogram After Endomyocardial Biopsy for Identification of Hemopericardium or Tricuspid Valve Injury Peng, D. M., Kipps, A. K., Palmon, M., Khalapyan, T. Z., Almond, C. S., Chen, S., Hollander, S. A., Tacy, T. A., Rosenthal, D. N., McElhinney, D. ELSEVIER SCIENCE INC. 2016: S403
  • Changes in Efficiency and Safety Culture After Integration of an I-PASS-Supported Handoff Process. Pediatrics Sheth, S., McCarthy, E., Kipps, A. K., Wood, M., Roth, S. J., Sharek, P. J., Shin, A. Y. 2016; 137 (2): 1-9


    Recent publications have shown improved outcomes associated with resident-to-resident handoff processes. However, the implementation of similar handoff processes for patients moving between units and teams with expansive responsibilities presents unique challenges. We sought to determine the impact of a multidisciplinary standardized handoff process on efficiency, safety culture, and satisfaction.A prospective improvement initiative to standardize handoffs during patient transitions from the cardiovascular ICU to the acute care unit was implemented in a university-affiliated children's hospital.Time between verbal handoff and patient transfer decreased from baseline (397 ± 167 minutes) to the postintervention period (24 ± 21 minutes) (P < .01). Percentage positive scores for the handoff/transitions domain of a national culture of safety survey improved (39.8% vs 15.2% and 38.8% vs 19.6%; P = .005 and 0.03, respectively). Provider satisfaction improved related to the information conveyed (34% to 41%; P = .03), time to transfer (5% to 34%; P < .01), and overall experience (3% to 24%; P < .01). Family satisfaction improved for several questions, including: "satisfaction with the information conveyed" (42% to 70%; P = .02), "opportunities to ask questions" (46% to 74%; P < .01), and "Acute Care team's knowledgeabout my child's issues" (50% to 73%; P = .04). No differences in rates of readmission, rapid response team calls, or mortality were observed.Implementation of a multidisciplinary I-PASS-supported handoff process for patients transferring from the cardiovascular ICU to the acute care unit resulted in improved transfer efficiency, safety culture scores, and satisfaction of providers and families.

    View details for DOI 10.1542/peds.2015-0166

    View details for PubMedID 26743818

  • Isolation of the right subclavian artery in a patient with d-transposition of the great arteries. Annals of pediatric cardiology Arunamata, A., Perry, S. B., Kipps, A. K., Vasanawala, S. S., Axelrod, D. M. 2015; 8 (2): 161-163


    Isolation of the right subclavian artery (RSCA) is rare, and this finding in association with d-transposition of the great arteries (d-TGA) is extremely unusual. We present a case of an isolated RSCA in a newborn with d-TGA in whom the clinical presentation was diagnostic. We discuss the imaging modalities used to confirm the diagnosis, the embryological basis of the finding, and the surgical repair.

    View details for DOI 10.4103/0974-2069.154154

    View details for PubMedID 26085773

  • Implementation of Data Drive Heart Rate and Respiratory Rate parameters on a Pediatric Acute Care Unit. Studies in health technology and informatics Goel, V., Poole, S., Kipps, A., Palma, J., Platchek, T., Pageler, N., Longhurst, C., Sharek, P. 2015; 216: 918-?


    The majority of hospital physiologic monitor alarms are not clinically actionable and contribute to alarm fatigue. In 2014, The Joint Commission declared alarm safety as a National Patient Safety Goal and urged prompt action by hospitals to mitigate the issue [1]. It has been demonstrated that vital signs in hospitalized children are quite different from currently accepted reference ranges [2]. Implementation of data-driven, age stratified vital sign parameters (Table 1) for alarms in this patient population could reduce alarm frequency.

    View details for PubMedID 26262220

  • Prenatal Tricuspid Valve Size as a Predictor of Postnatal Outcome in Patients with Severe Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum FETAL DIAGNOSIS AND THERAPY Lowenthal, A., Lemley, B., Kipps, A. K., Brook, M. M., Moon-Grady, A. J. 2014; 35 (2): 101-107


    Tricuspid valve (TV) size at birth correlates with intervention strategy in patients with severe pulmonary stenosis (SPS) or pulmonary atresia/intact ventricular septum (PA/IVS). Prenatal features that might predict postnatal TV size have not been well studied. We hypothesized that prenatal echocardiographic measurements predict the postnatal TV Z-score in fetuses with SPS and PA/IVS.We identified 16 neonates (gestational age 28 ± 4.8 weeks) with a fetal diagnosis of SPS or PA/IVS from 2001 to 2010. Measurements were performed offline. ROC (receiver operating characteristic) analysis was used to generate AUC (areas under the curve) for each of the variables.AUC was 0.94 for tricuspid to mitral valve (TV/MV) ratio, 0.88 for TV Z-score, and 0.85 for TV inflow duration. A cut-off value of >0.63 for TV/MV yielded a sensitivity of 78%, specificity of 100% for predicting postnatal TV Z-score >-3. Neonates with TV Z-score ≥-3 and all fetuses with antegrade flow across the pulmonary valve or more than moderate tricuspid regurgitation had biventricular circulation in follow-up.Fetal TV/MV >0.63 predicts favorable TV Z-score at birth in patients with SPS and PA/IVS. Antegrade pulmonary valve flow and more than moderate tricuspid regurgitation also conferred a favorable outcome.

    View details for DOI 10.1159/000357429

    View details for Web of Science ID 000332498600005

    View details for PubMedID 24457468

  • Course, Predictors of Diaphragm Recovery After Phrenic Nerve Injury During Pediatric Cardiac Surgery ANNALS OF THORACIC SURGERY Smith, B. M., Ezeokoli, N. J., Kipps, A. K., Azakie, A., Meadows, J. J. 2013; 96 (3): 938-942


    Hemidiaphragm paralysis from phrenic nerve injury is a known complication of congenital cardiac surgery. Return of diaphragm function has been reported; however, prior studies on this subject have been limited by small numbers, static assessment methods, or observation of plicated or non-plicated patients alone. To describe return of function, we reviewed fluoroscopy and ultrasonography in all diagnosed cases of diaphragmatic paralysis.Surgical cases at our institution between 1991 and 2010 were identified for patients with postoperative hemidiaphragm paralysis diagnosed by chest X-ray, ultrasound, or fluoroscopy. Follow-up ultrasound and fluoroscopic studies were reviewed for return of diaphragm function.Seventy-two cases of postoperative hemidiaphragm paralysis were identified. Forty cases were plicated prior to discharge. Plicated patients were younger at time of diagnosis (median 46 days average 3.6 months; p=0.025) and had a larger proportion of single ventricle diagnoses (48% vs 16%) compared with non-plicated patients. Twenty-six patients with paralysis were excluded in follow-up due to lack of studies documenting diaphragm function after the diagnostic study. Of the remaining 46 cases, median follow-up was 353 days (range: 6 days to 17 years). Plicated and non-plicated patients regained function at similar frequency (60% and 54.8%, respectively). Plication status, Risk Adjustment for Congenital Heart Surgery (RACHS) 1 score, age at diagnosis, and side of paralysis did not predict failure of recovery.In the current era, return of diaphragm function after phrenic nerve injury sustained during congenital cardiac surgery is a known occurrence; however, predicting recovery continues to be difficult.

    View details for DOI 10.1016/j.athoracsur.2013.05.057

    View details for Web of Science ID 000323940200038

    View details for PubMedID 23932321

  • Outcomes of a modified approach to transcatheter closure of perimembranous ventricular septal defects CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Landman, G., Kipps, A., Moore, P., Teitel, D., Meadows, J. 2013; 82 (1): 143-149


    To describe the immediate and midterm results of a modified method for transcatheter closure of perimembranous ventricular septal defects (pmVSDs).Transcatheter closure of pmVSDs has been associated with development of heart block due to impingement on the ventricular conduction system. Ventricular septal aneurysms (VSAs) are common; the VSA tissue can serve as a target for the device without necessitating direct contact with the conduction system.Between 2004 and 2011, 15 patients underwent transcatheter closure of a pmVSD utilizing a device implanted into a VSA. Catheterization reports were examined in addition to pre-closure, post-closure, and current clinical, ECG, and echocardiographic data.The median age was 20 years (4-61 years), and the most common indication for closure was increasing LV dilation. Four different Amplatzer devices were utilized. Following device implantation there was a decrease in Qp:Qs (1.7-1.1) and in RV:LV pressure ratio (0.36-0.31). There were no deaths, no device embolizations, and no new heart block or PR interval prolongation. Three patients developed a new right bundle branch block (RBBB). The median follow-up time was 1.5 years (4 months to 7.1 years). Two patients required further procedures for important residual shunting. Six continued to have a "trivial/small" residual leak, but only one had any degree (mild) of residual LV dilation. None of the complications were significantly associated with age or weight at the time of procedure, original size of the VSD, or size or type of the device used.Transcatheter closure of pmVSD with placement of the device into the VSA is safe and effective, and may result in fewer instances of atrioventricular block and valve abnormalities than have been reported with alternative methods of pmVSD device closure. Persistent VSDs and new RBBBs remain an important issue.

    View details for DOI 10.1002/ccd.24774

    View details for Web of Science ID 000320787100030

    View details for PubMedID 23225758

  • Tissue Doppler is more sensitive and reproducible than spectral pulsed-wave Doppler for fetal right ventricle myocardial performance index determination in normal and diabetic pregnancies. Journal of the American Society of Echocardiography Bui, Y. K., Kipps, A. K., Brook, M. M., Moon-Grady, A. J. 2013; 26 (5): 507-514


    The aim of this study was to compare the reproducibility, agreement, and sensitivity of pulsed-wave Doppler tissue imaging (DTI) versus spectral Doppler assessment of right ventricular (RV) myocardial performance index (MPI) in midgestation fetuses in both a normal and a disease state.RV MPI was calculated using pulsed-wave DTI and spectral Doppler in normal pregnancies (n = 69) and in women with pregestational diabetes (n = 51). Intraobserver and interobserver variability and agreement were evaluated using Bland-Altman analysis. Student's t tests were used for comparisons of differences.In normal fetuses, RV MPI derived by the two methods showed no statistical difference, were interchangeable (DTI, 0.51 ± 0.10; spectral Doppler, 0.50 ± 0.12; P = .686), and were in agreement by Bland-Altman analysis. However, in fetuses of mothers with diabetes, the two methods produced different RV MPI measurements (DTI, 0.56 ± 0.10; spectral Doppler, 0.51 ± 0.12; P < .001). Intraobserver and interobserver bias was lower for DTI.The DTI method of measuring fetal RV MPI is more sensitive, has less variability and more precision, and is better able to demonstrate subtle abnormalities in cardiac function than the spectral Doppler method in diabetic versus normal pregnancies.

    View details for DOI 10.1016/j.echo.2013.02.006

    View details for PubMedID 23498900

  • Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2-year survival PRENATAL DIAGNOSIS Lowenthal, A., Kipps, A. K., Brook, M. M., Meadows, J., Azakie, A., Moon-Grady, A. J. 2012; 32 (5): 485-490


    To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction.Retrospective, cohort.Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atrial hypertension on pulmonary venous Doppler and/or an intact interatrial septum.Of 73 total fetal patients identified, 49 were live-born. Survival at 2 years was 29/35 [83% confidence interval (CI): 59.5%-88.9%] for the nonrestrictive group and 6/14 (43% CI: 17.7%-6.0%) for the restrictive group (p<0.0001). Of those who underwent stage 1 palliation (35 with nonrestrictive and 10 with restrictive atrial septa), both groups had a similar incidence of preoperative acidosis and need for ventilation and inotropic support. Postoperatively, there was no difference between groups in ventilator days, length of stay, or survival to discharge. There was decreased survival at 2 years in the restrictive group, 60% (CI: 26.2%-87.8%) versus 83% (CI: 66.4%-93.4%) in the nonrestrictive group. Furthermore, a disproportionate number of interstage deaths was evident in the restrictive group.Prenatal presence of any degree of atrial septal restriction in the setting of HLHS confers a significant survival disadvantage, with increases in both early and late mortality.

    View details for DOI 10.1002/pd.3850

    View details for Web of Science ID 000303797700015

    View details for PubMedID 22511219

    View details for PubMedCentralID PMC3348961

  • Natural history of exercise function in patients with Ebstein anomaly: A serial study AMERICAN HEART JOURNAL Kipps, A. K., Graham, D. A., Lewis, E., Marx, G. R., Banka, P., Rhodes, J. 2012; 163 (3): 486-491


    The clinical manifestations of Ebstein anomaly (EA) vary greatly; criteria for surgical intervention remain undefined. Decisions regarding surgical intervention in asymptomatic/mildly symptomatic patients would be helpfully informed by a detailed, quantitative understanding of the natural history of exercise intolerance in these patients. However, past studies of exercise function in EA have been of a cross-sectional, rather than a serial, nature. We, therefore, analyzed serial cardiopulmonary exercise (CPX) tests from patients with unrepaired EA to better appreciate the natural history of their exercise function.All patients with EA who had had at least 2 CPX tests, separated by at least 6 months, between November 2002 and October 2010 were identified. Patients with prior tricuspid valve surgery were excluded from the study.Cardiopulmonary exercise data from 23 patients (64 CPX tests; 2.8 ± 1.0 tests/patient) were analyzed. The median time interval between the first and last CPX tests was 3.3 (range, 0.6-7.3) years. The percentage of predicted peak oxygen consumption declined slowly (1.87 ± 8.04 percentage points/y) during the follow-up period. The decline was more pronounced (3.04 ± 6.78 percentage points/y) in patients <18 years old. On multivariate modeling, only the change in oxygen pulse at peak exercise (a surrogate for forward stroke volume) and the change in peak heart rate over time emerged as statistically significant correlates of the change in percentage of predicted peak oxygen consumption.The exercise function of patients with EA tends to deteriorate over time. This deterioration appears to be related to a progressive decline in their ability to augment their forward stroke volume and heart rate during exercise.

    View details for DOI 10.1016/j.ahj.2011.12.006

    View details for Web of Science ID 000301597200026

    View details for PubMedID 22424021

  • Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum CONGENITAL HEART DISEASE Kipps, A. K., Powell, A. J., Levine, J. C. 2011; 6 (5): 444-450


    To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum.Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome.Tertiary-care pediatric hospital.Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z-scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs.In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.

    View details for DOI 10.1111/j.1747-0803.2011.00541.x

    View details for Web of Science ID 000294919100006

    View details for PubMedID 21718454

  • Prenatal Diagnosis of Hypoplastic Left Heart Syndrome in Current Era AMERICAN JOURNAL OF CARDIOLOGY Kipps, A. K., Feuille, C., Azakie, A., Hoffman, J. I., Tabbutt, S., Brook, M. M., Moon-Grady, A. J. 2011; 108 (3): 421-427


    We sought to evaluate the relation of a prenatal diagnosis (preDx) with morbidity and mortality during the initial hospitalization in a contemporary cohort of patients with hypoplastic left heart syndrome (HLHS). A retrospective study of patients with HLHS presenting from 1999 to 2010 was performed. Patients with genetic disorders or a gestational age <34 weeks or who had intentionally received comfort care only were excluded. Of the 81 patients meeting the study criteria, 49 had a preDx and 32 were diagnosed postnatally (postDx). Birth weight (median 3.0 vs 3.4 kg; p = 0.007) and gestational age (median 38 vs 39 weeks; p <0.001) were lower in the preDx than in the postDx patients. Preoperatively, the postDx patients were intubated more frequently (97% vs 71%, p = 0.004) and ventilated longer (median 96 vs 24 hours, p = 0.005) than the preDx patients. They also had more preoperative acidosis, multiorgan failure, tricuspid valve regurgitation, and right ventricular dysfunction. Of the 73 patients undergoing surgery, no difference in survival was seen between the preDx and postDx groups (91% vs 89%). The median duration of postoperative ventilation was 7 days and the median length of stay was 36 days for the 66 survivors, with no difference between the 2 groups. Postoperative morbidities, including chylothorax and infection, were also similar in the preDx and postDx patients. No studied preoperative factor was associated with death, duration of postoperative ventilation, or length of stay. In conclusion, our recent experience has shown that preDx of HLHS was not associated with a survival advantage, fewer postoperative complications, or shorter length of stay. Improved preoperative status was observed in the preDx patients; however, they were born earlier with a lower birthweight. What effect these factors might have on longer term morbidity remains unknown.

    View details for DOI 10.1016/j.amjcard.2011.03.065

    View details for Web of Science ID 000293767400015

    View details for PubMedID 21624547

  • Longitudinal Exercise Capacity of Patients With Repaired Tetralogy of Fallot AMERICAN JOURNAL OF CARDIOLOGY Kipps, A. K., Graham, D. A., Harrild, D. M., Lewis, E., Powell, A. J., Rhodes, J. 2011; 108 (1): 99-105


    Patients with repaired tetralogy of Fallot have a reduced percentage of predicted peak oxygen consumption (VO(2)) and percentage of oxygen pulse (O(2)P%) compared to healthy controls. Because data regarding the progression of exercise intolerance in these patients is limited, we sought to analyze the serial exercise data from patients with Tetralogy of Fallot to quantify the changes in their exercise capacity over time and to identify associations with clinical and cardiac magnetic resonance imaging variables. The data from cardiopulmonary exercise tests (CPXs) from 2002 to 2010 for patients with repaired tetralogy of Fallot with ≥2 CPXs separated by ≥12 months were analyzed. Tests occurring after interventional catheterization or surgery were excluded. A total of 70 patients had 179 CPXs. They had a median age at the initial study of 23.6 years and an interval between the first and last CPX of 2.8 years. At the initial CPX, the peak VO(2) was 27.6 ± 8.8 ml/kg/min (78 ± 19% of predicted), and the peak O(2)P% was 89 ± 22% of predicted. At the most recent study, the peak VO(2) averaged 25.0 ± 7.4 ml/kg/min (73 ± 16% of predicted), and the peak O(2)P% averaged 83 ± 20% (p <0.01) for each versus the initial CPX. The decrease in the peak VO(2) was strongly associated with a decrease in O(2)P% and an increase (worsening) in the slope of the minute ventilation-versus-carbon dioxide production relation. Changes in the peak VO(2) did not correlate with concomitant changes in any other CPX variable. The rate of decrease was not related to a history of shunt palliation, age at CPX, or any other baseline clinical parameter, including cardiac magnetic resonance measurements. In conclusion, the exercise capacity of patients with repaired tetralogy of Fallot tends to decrease over time. This deterioration is variable and unpredictable and is primarily related to a decrease in the forward stroke volume at peak exercise.

    View details for DOI 10.1016/j.amjcard.2011.02.349

    View details for Web of Science ID 000292785600017

    View details for PubMedID 21529748

  • Blood transfusion is associated with prolonged duration of mechanical ventilation in infants undergoing reparative cardiac surgery PEDIATRIC CRITICAL CARE MEDICINE Kipps, A. K., Wypij, D., Thiagarajan, R. R., Bacha, E. A., Newburger, J. W. 2011; 12 (1): 52-56


    Perioperative transfusion has adverse effects in adults undergoing cardiac surgery. We sought to investigate whether greater use of blood and blood products might be an independent predictor of prolonged postoperative recovery, indicated by duration of mechanical ventilation (DMV), after reparative infant heart surgery.Secondary analysis of prospectively collected data from two randomized trials of hematocrit strategy during cardiopulmonary bypass in infant heart surgery to explore the association of DMV with perioperative transfusion and other variables.Tertiary pediatric hospital.Two hundred seventy infants undergoing two ventricle corrective cardiac surgery without aortic arch reconstruction.In univariable analyses, longer DMV was associated with younger age and lower weight at surgery, diagnostic group, and higher intraoperative and postoperative blood product transfusion (each p < .001). In multivariable proportional hazard regression, longer total support time and greater intraoperative and early postoperative blood products per kg were the strongest predictors of longer DMV. Patients in the highest tertile of intraoperative blood products per kg had an instantaneous risk of being extubated approximately half that of patients in the lowest tertile (hazard ratio, 0.51; 95% confidence interval, 0.35, 0.73). Patients who received any blood products on postoperative day 1, compared with those who did not, had a hazard ratio for extubation of 0.65 (95% confidence interval, 0.50, 0.85).In this exploratory secondary analysis of infants undergoing two ventricular repair of congenital heart disease without aortic arch obstruction, greater intraoperative and early postoperative blood transfusion emerged as potential important risk factors for longer DMV. Future prospective clinical trials are needed to determine whether reduction in blood product administration hastens postoperative recovery after infant heart surgery.

    View details for DOI 10.1097/PCC.0b013e3181e30d43

    View details for Web of Science ID 000285964500019

    View details for PubMedID 20453699

    View details for PubMedCentralID PMC3697008

  • Echocardiographic Risk Stratification of Fetuses with Sacrococcygeal Teratoma and Twin-Reversed Arterial Perfusion FETAL DIAGNOSIS AND THERAPY Byrne, F. A., Lee, H., Kipps, A. K., Brook, M. M., Moon-Grady, A. J. 2011; 30 (4): 280-288


    To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP).Clinical, echocardiographic and sonographic data of fetuses with SCT or TRAP during 1999-2009 were reviewed retrospectively. Measurements of cardiothoracic ratio (CTR), cardiac dimension Z-scores, combined ventricular output (CVO), valvular regurgitation, and cardiovascular profile scores (CVPS) were obtained.In total, 19 fetuses (11 SCT, 8 TRAP) met the inclusion criteria and 26 detailed fetal echocardiographic studies were reviewed. Outcome was poor in 7 pregnancies (group A) and good in 12 (group B). Group A had worse CVPS (8.5 vs. 10, p < 0.01) and higher CTR (0.37 vs. 0.30, p = 0.04). At least one of the following was present in each group A fetus: CTR >0.5, CVO >550 ml/min/kg, tricuspid or mitral regurgitation, or mitral valve Z-score >2. No group B fetus had any of these abnormalities. No fetus in either group had abnormal venous Doppler waveforms at presentation.Fetal echocardiography can identify abnormalities of cardiac size and systolic, but not diastolic, function in all fetuses who subsequently died or developed hydrops.

    View details for DOI 10.1159/000330762

    View details for Web of Science ID 000298661500005

    View details for PubMedID 22086180

  • Exercise Function of Children with Congenital Aortic Stenosis Following Aortic Valvuloplasty during Early Infancy CONGENITAL HEART DISEASE Kipps, A. K., McElhinney, D. B., Kane, J., Rhodes, J. 2009; 4 (4): 258-264


    The objectives of this study were to characterize the exercise function of patients treated with balloon aortic valvuloplasty at 6 years old with a history of neonatal AS to undergo exercise testing.We enrolled 30 patients (median age 13.1 years) who underwent balloon aortic valvuloplasty at a median age of 12 days. At time of exercise testing, the median maximum Doppler AS gradient was 34 mm Hg (0-70 mm Hg); 11 patients had moderate or severe aortic regurgitation. All patients were asymptomatic. Overall, peak oxygen consumption (VO(2)) was below normal (87 +/- 18% predicted; P < .001), and was severely depressed (

    View details for DOI 10.1111/j.1747-0803.2009.00304.x

    View details for Web of Science ID 000207893700007

    View details for PubMedID 19664028

    View details for PubMedCentralID PMC4269337

  • The Longitudinal Course of Cardiomyopathy in Friedreich's Ataxia During Childhood PEDIATRIC CARDIOLOGY Kipps, A., Alexander, M., Colan, S. D., Gauvreau, K., Smoot, L., Crawford, L., Darras, B. T., Blume, E. D. 2009; 30 (3): 306-310


    Clinical heart disease was recognized in the first descriptions of Friedreich's ataxia (FA). Cardiac manifestations reported for this progressive neurologic disease include hypertrophic cardiomyopathy, dilated cardiomyopathy, and electrophysiologic disturbances. Longitudinal data for childhood cases are limited. This study aimed to define the longitudinal course of the cardiac abnormalities with FA diagnosed during childhood and to correlate the presence of cardiomyopathy with clinical and genetic factors.A retrospective chart review was conducted, with prospective, blinded interpretation of echocardiograms and electrocardiograms. All the patients with a diagnosis of FA referred to the cardiology department of a single institution from 1974 to 2004 were included in the study.This study investigated a total of 113 echocardiograms for 28 patients. Overall, the group had left ventricular hypertrophy and normal systolic function, with a median mass z-score of 2.48 (range, -3.8 to 35.6) and a median ejection fraction (EF) of 61% (range, 23-81%). Of the 28 patients, 23 (82%) had two or more echocardiograms. The median follow-up time to the most recent echocardiogram was 5.1 years (range, 0.4-16.5 years). Many in this longitudinal follow-up cohort (57%) showed hypertrophic cardiomyopathy on at least one echocardiogram, with the last follow-up assessment showing systolic dysfunction for 38% of these patients. There was a slow nonlinear decline in systolic function over time, with the mean EF decreasing more rapidly as age increased (p = 0.02) and maintenance of EF in the normal range until the age of 22 years. Of the 12 patients with systolic dysfunction and follow-up echocardiograms, 10 showed improvement to the normal EF range on at least one echocardiogram, and 5 remained normal through the last study. None of the trends in cardiac function and morphology correlated with frataxin GAA repeat length (the primary genetic defect in FA) or ambulatory status. One patient required an implantable defibrillator. There were no deaths or heart transplantations.Overall, patients with FA have preserved cardiac function with increased mass throughout childhood. Because many patients who experience depressed systolic function show improvement in subsequent studies, evaluation for potentially reversible causes of heart failure should be conducted. Relative clinical stability during childhood and maintenance of normal systolic function into the second decade may be helpful for parent and patient education.

    View details for DOI 10.1007/s00246-008-9305-1

    View details for Web of Science ID 000264884500015

    View details for PubMedID 18716706

  • Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia PEDIATRIC ANESTHESIA Kipps, A. K., Ramamoorthy, C., Rosenthal, D. N., Williams, G. D. 2007; 17 (8): 775-781


    Children with cardiomyopathy (CM) often undergo procedures that require general anesthesia (GA) but little is known about anesthesia-related adverse events or postprocedural outcomes.After approval, all children with CM who underwent nonopen heart surgical procedures and/or diagnostic imaging under GA at a tertiary children's hospital during January 2002 to May 2005 were identified from a clinical database. Based on their preprocedure fractional shortening (FS) on echocardiogram, systemic ventricular dysfunction was categorized as mild (FS 23-28%), moderate (FS 16-22%), or severe (FS < 16%) and those with normal (FS > 28%) were excluded from review.Twenty-six patients underwent 34 procedures under GA, of whom 13 (38%) had mild or moderate ventricular dysfunction and 21 (62%) had severe dysfunction. Common procedures included pacer/defibrillator placement (43%) and imaging studies (18%). Eighteen complications were noted in 12 patients. Fifteen (83%) complications occurred in patients with severe ventricular dysfunction. One patient with severe ventricular dysfunction died (3% mortality). Hypotension requiring inotropic support was the most frequent complication (61%). Children with severe ventricular dysfunction often required hospital support pre- and postprocedure with 67% requiring intensive care. Hospital stay was longer for patients with severe ventricular dysfunction compared with children with mild or moderate ventricular dysfunction (P = 0.006).The 30-day mortality rate was low but complications were common, especially in patients with severe ventricular dysfunction. For these patients, we recommend early consideration of perioperative intensive care support to optimize cardiovascular therapy and monitoring.

    View details for DOI 10.1111/j.1460-9592.2007.02245.x

    View details for Web of Science ID 000247582600007

    View details for PubMedID 17596222