Vamsi Yarlagadda

All Publications

• Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study. ASAIO journal (American Society for Artificial Internal Organs : 1992) Beshish, A. G., Amedi, A., Harriott, A., Patel, S., Evans, S., Scheel, A., Xiang, Y., Keesari, R., Harding, A., Davis, J., Shashidharan, S., Yarlagadda, V., Aljiffry, A. 2024; 70 (4): 328-335

  Abstract

  Patients requiring extracorporeal life support (ECLS) post-Norwood operation constitute an extremely high-risk group. We retrospectively described short-term outcomes, functional status, and assessed risk factors for requiring ECLS post-Norwood operation between January 2010 and December 2020 in a high-volume center. During the study period, 269 patients underwent a Norwood procedure of which 65 (24%) required ECLS. Of the 65 patients, 27 (41.5%) survived to hospital discharge. Mean functional status scale (FSS) score at discharge increased from 6.0 on admission to 8.48 (p < 0.0001). This change was primary in feeding (p < 0.0001) and respiratory domains (p = 0.017). Seven survivors (26%) developed new morbidity, and two (7%) developed unfavorable functional outcomes. In the regression analysis, we showed that patients with moderate-severe univentricular dysfunction on pre-Norwood transthoracic echocardiogram (odds ratio [OR] = 6.97), modified Blalock Taussig Thomas (m-BTT) shunt as source of pulmonary blood flow (OR = 2.65), moderate-severe atrioventricular valve regurgitation on transesophageal echocardiogram (OR = 8.50), longer cardiopulmonary bypass time (OR = 1.16), longer circulatory arrest time (OR = 1.20), and delayed sternal closure (OR = 3.86), had higher odds of requiring ECLS (p < 0.05). Careful identification of these risk factors is imperative to improve the care of this high-risk cohort and improve overall outcomes.

  View details for DOI 10.1097/MAT.0000000000002109

  View details for PubMedID 38557688

• Clinical Predictive Tool for Pediatric Cardiac Patients on Extracorporeal Membrane Oxygenation Therapy and Ultrafiltration. ASAIO journal (American Society for Artificial Internal Organs : 1992) Sedler, J., Sutherland, S. M., Uber, A. M., Jahadi, O., Ryan, K. R., Yarlagadda, V. V., Kwiatkowski, D. M. 2023

  Abstract

  Fluid overload is common among pediatric cardiac patients receiving extracorporeal membrane oxygenation (ECMO) and is often treated with in-line ultrafiltration (UF) or continuous renal replacement therapy (CRRT). We assessed whether CRRT was associated with poor outcomes versus UF alone. Additionally, we identified characteristics associated with progression from UF to CRRT. Retrospective chart review of 131 patients age ≤18 years treated with ECMO at a single quaternary center. Data were collected to compare patient demographics, characteristics, and outcomes. A receiver operator curve (ROC) was used to create a tool predictive of the need for CRRT at the time of UF initiation. Patients who required CRRT had a higher creatinine and blood urea nitrogen at time of UF initiation (p = 0.03 and p < 0.01), longer total ECMO duration (p < 0.01), lower renal recovery incidence (p = 0.02), and higher mortality (p ≤ 0.01). Using ROC analysis, presence of ≤3 of 7 risk variables had a positive predictive value of 87.5% and negative predictive value of 50.0% for use of UF alone (area under the curve 0.801; 95% CI: 0.638-0.965, p = 0.002). Pediatric cardiac patients treated with ECMO and UF who require CRRT demonstrate worse outcomes versus UF alone. A novel clinical tool may assist in stratifying patients at UF initiation.

  View details for DOI 10.1097/MAT.0000000000001924

  View details for PubMedID 36947828

• Multidisciplinary Stroke Pathway for Children Supported with Ventricular Assist Devices. ASAIO journal (American Society for Artificial Internal Organs : 1992) Lee, S., Ryan, K. R., Murray, J., Chen, S., Grant, G. A., Wilkins, S., Yarlagadda, V. V., Wintermark, M., Dodd, R., Rosenthal, D., Teuteburg, J., Navaratnam, M., Lee, J., Jordan, L. C., Almond, C. S. 2023

  Abstract

  Mechanical circulatory support (MCS), including ventricular assist device (VAD) support, is a leading cause of stroke in children; however, existing pediatric stroke recommendations do not apply to many pediatric VAD patients. We sought to develop a multidisciplinary pathway to improve timely and effective acute stroke care and examine the early performance of the pathway in expediting stroke care. Stakeholders from pediatric heart failure, cardiac intensive care, neurology, interventional radiology, neuroradiology, neurosurgery, pharmacy, and adult VAD care convened at Stanford University in August 2017 to discuss the challenges of providing high-quality acute stroke care to children on VAD support, and to develop multidisciplinary acute stroke pathways. Stakeholders identified multiple barriers to providing timely acute stroke care to pediatric VAD patients. These include delayed recognition of stroke, and lack of clarity related to the optimal imaging technique, when to emergently reverse antithrombotic therapy (AT), pediatric indications for thrombectomy and cranial decompression, and strategies to avoid unnecessary serial CTS. Four stroke pathways were created including evaluation and management of the pediatric patient with (1) an acute neurologic change before an imaging diagnosis; (2) an arterial ischemic stroke (AIS); (3) an intracerebral hemorrhage (ICH); and (4) a subdural hematoma (SDH). With the implementation of the stroke pathway, the median time-to-first-CT image decreased by 43 minutes from 66 to 23 minutes (P < 0.001) while the proportion with a CT within 30 minutes increased from 0% to 67% (P < 0.001). Despite a variety of challenges, multidisciplinary consensus can be achieved on a rapid stroke management pathway for children on VAD support that addresses important barriers to timely stroke care. Although too few stoke events occurred to differentiate clinical outcomes, the time-to-first-CT image was significantly shorter after pathway implementation.

  View details for DOI 10.1097/MAT.0000000000001822

  View details for PubMedID 36917842

• Respiratory Care for Neonates With Congenital Heart Disease. Pediatrics Bronicki, R. A., Benitz, W. E., Buckley, J. R., Yarlagadda, V. V., Porta, N. F., Agana, D. O., Kim, M., Costello, J. M. 2022; 150 (Suppl 2)

  View details for DOI 10.1542/peds.2022-056415H

  View details for PubMedID 36317970

• Functional Status Change Among Infants, Children, and Adolescents Following Extracorporeal Life Support: a Multicenter Report. ASAIO journal (American Society for Artificial Internal Organs : 1992) Beshish, A. G., Rodriguez, Z., Hani Farhat, M., Newman, J. W., Jahadi, O., Baginski, M., Bradley, J., Rao, N., Figueroa, J., Viamonte, H., Chanani, N. K., Owens, G. E., Barbaro, R., Yarlagadda, V., Ryan, K. R. 2022

  Abstract

  In our retrospective multicenter study of patients 0 to 18 years of age who survived extracorporeal life support (ECLS) between January 2010 and December 2018, we sought to characterize the functional status scale (FSS) of ECLS survivors, determine the change in FSS from admission to discharge, and examine risk factors associated with development of new morbidity and unfavorable outcome. During the study period, there were 1,325 ECLS runs, 746 (56%) survived to hospital discharge. Pediatric patients accounted for 56%. Most common ECLS indication was respiratory failure (47%). ECLS support was nearly evenly split between veno-arterial and veno-venous (51% vs. 49%). Median duration of ECLS in survivors was 5.5 days. Forty percent of survivors had new morbidity, and 16% had an unfavorable outcome. In a logistic regression, African American patients (OR 1.68, p = 0.01), longer duration of ECLS (OR 1.002, p = 0.004), mechanical (OR 1.79, p = 0.002), and renal (OR 1.64, p = 0.015) complications had higher odds of new morbidity. Other races (Pacific Islanders, and Native Americans) (OR 2.89, p = 0.013), longer duration of ECLS (OR 1.002, p = 0.002), and mechanical complications (OR 1.67, p = 0.026) had higher odds of unfavorable outcomes. In conclusion, in our multi-center 9-year ECLS experience, 56% survived, 40% developed new morbidity, and 84% had favorable outcome. Future studies with larger populations could help identify modifiable risk factors that could help guide clinicians in this fragile patient population.

  View details for DOI 10.1097/MAT.0000000000001711

  View details for PubMedID 35435861

• Variable clinical severity in TANGO2 deficiency: Case series and literature review. American journal of medical genetics. Part A Schymick, J., Leahy, P., Cowan, T., Ruzhnikov, M. R., Gates, R., Fernandez, L., Pramanik, G., Undiagnosed Diseases Network, Yarlagadda, V., Wheeler, M., Bernstein, J. A., Enns, G. M., Lee, C. 2021

  Abstract

  Biallelic pathogenic variants in the TANGO2 (transport and Golgi organization 2 homolog) gene have been identified as causing a rare metabolic disorder characterized by susceptibility to recurrent rhabdomyolysis, lactic acidosis, encephalopathy, and life-threatening tachyarrhythmias. Recently published reports suggest variable clinical severity and phenotypes. This study details five new patients from two families with biallelic pathogenic variants in the TANGO2 gene identified by whole exome sequencing and includes the largest number of affected individuals from a single family reported to date. We document significant intrafamilial variability and highlight that milder phenotypes may be underrecognized. We present biochemical and clinical data to help highlight the features that aid in consideration of this condition in the differential with disorders of fatty acid oxidation. We also present a comprehensive literature review summarizing the molecular, clinical, and biochemical findings for 92 individuals across 13 publications. Of the 27 pathogenic variants reported to date, the recurrent exons 3-9 deletion represents the most common variant seen in 42% of individuals with TANGO2 deficiency. Common clinical features seen in >70% of all individuals include acute metabolic crisis, rhabdomyolysis, neurologic abnormalities, developmental delay, and intellectual disability. Findings such as elevated creatine kinase, hypothyroidism, ketotic hypoglycemia, QT prolongation, or abnormalities of long-chain acylcarnitines and urine dicarboxylic acids should raise clinical suspicion for this life-threatening condition.

  View details for DOI 10.1002/ajmg.a.62543

  View details for PubMedID 34668327

• Durability of Pulmonary Valve Replacement with Large Diameter Stented Porcine Bioprostheses. Seminars in thoracic and cardiovascular surgery Maeda, K., Lui, G. K., Zhang, Y., Maskatia, S. A., Romfh, A., Yarlagadda, V. V., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention. During the study period, 203 patients underwent PVR using a porcine BPV ≥27 mm, 94% of whom received a Mosaic valve (Medtronic Inc., Minneapolis, MN). Twenty patients underwent reintervention from 3.4-12.0 years after PVR: 5 surgical and 15 transcatheter PVR procedures. The indication for reintervention was regurgitation in 13 patients, stenosis in 2, mixed disease in 4, and endocarditis in 1. Estimate freedom from reintervention was 97±1% at 5 years and 82±4% at 10 years, and freedom from prosthesis dysfunction (moderate or severe regurgitation and/or a maximum Doppler gradient ≥50 mmHg) over time was 91±2% at 5 years and 74±4% at 10 years. Younger age and smaller true valve diameter were associated with shorter freedom from reintervention, but valve oversizing was not. The durability of large stented porcine bioprostheses in the pulmonary position is generally excellent, particularly in adolescents and adults, similar to various other types of BPV. In the current study, relative valve size was not associated with valve longevity, although the low event-rate in this population was a limiting factor.

  View details for DOI 10.1053/j.semtcvs.2021.03.044

  View details for PubMedID 33971298

• Hyperoxia During Cardiopulmonary Bypass Is Associated With Mortality in Infants Undergoing Cardiac Surgery. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Beshish, A. G., Jahadi, O., Mello, A., Yarlagadda, V. V., Shin, A. Y., Kwiatkowski, D. M. 2021

  Abstract

  OBJECTIVES: Patients undergoing cardiac surgery using cardiopulmonary bypass have variable degrees of blood oxygen tension during surgery. Hyperoxia has been associated with adverse outcomes in critical illness. Data are not available regarding the association of hyperoxia and outcomes in infants undergoing cardiopulmonary bypass. We hypothesize that among infants undergoing cardiac surgery, hyperoxia during cardiopulmonary bypass is associated with greater odds of morbidity and mortality.DESIGN: Retrospective study.SETTING: Single center at an academic tertiary children's hospital.PATIENTS: All infants (< 1 yr) undergoing cardiopulmonary bypass between January 1, 2015, and December 31, 2017, excluding two patients who were initiated on extracorporeal membrane oxygenation in the operating room.INTERVENTIONS: None.MEASUREMENTS AND MAIN RESULTS: The study included 469 infants with a median age of 97 days (interquartile range, 14-179 d), weight 4.9 kg (interquartile range, 3.4-6.4 kg), and cardiopulmonary bypass time 128 minutes (interquartile range, 91-185 min). A PaO2 of 313 mm Hg (hyperoxia) on cardiopulmonary bypass had highest sensitivity with specificity greater than 50% for association with operative mortality. Approximately, half of the population (237/469) had hyperoxia on cardiopulmonary bypass. Infants with hyperoxia were more likely to have acute kidney injury, prolonged postoperative length of stay, and mortality. They were younger, weighed less, had longer cardiopulmonary bypass times, and had higher Society of Thoracic Surgeons and the European Association for Cardio-Thoracic Surgery mortality scores. There was no difference in sex, race, preoperative creatinine, single ventricle physiology, or presence of genetic syndrome. On multivariable analysis, hyperoxia was associated with greater odds of mortality (odds ratio, 4.3; 95% CI, 1.4-13.2) but failed to identify an association with acute kidney injury or prolonged postoperative length of stay. Hyperoxia was associated with greater odds of mortality in subgroup analysis of neonatal patients.CONCLUSIONS: Hyperoxia occurred in a substantial portion of infants undergoing cardiopulmonary bypass for cardiac surgery. Hyperoxia during cardiopulmonary bypass was an independent risk factor for mortality and may be a modifiable risk factor. Furthermore, hyperoxia during cardiopulmonary bypass was associated with four-fold greater odds of mortality within 30 days of surgery. Hyperoxia failed to identify an association with development of acute kidney injury or prolonged postoperative length of stay when controlling for covariables. Validation of our data among other populations is necessary to better understand and elucidate potential mechanisms underlying the association between excess oxygen delivery during cardiopulmonary bypass and outcome.

  View details for DOI 10.1097/PCC.0000000000002661

  View details for PubMedID 33443979

• Adverse Events Associated with Cardiac Catheterization in Children Supported with Ventricular Assist Devices. ASAIO journal (American Society for Artificial Internal Organs : 1992) Power, A., Navaratnam, M., Murray, J. M., Peng, L. F., Rosenthal, D. N., Dykes, J. C., Yarlagadda, V. V., Maeda, K., Almond, C. S., Chen, S. 2021

  Abstract

  Children on ventricular assist device (VAD) support can present several unique challenges, including small patient size, univentricular or biventricular congenital heart disease (1V- or 2V-CHD) and need for biventricular VAD (BiVAD) support. While cardiac catheterization can provide valuable information, it is an invasive procedure with inherent risks. We sought to evaluate the safety of catheterization in pediatric patients on VAD support. We performed a retrospective review of patients on VAD support who underwent catheterization at Lucile Packard Children's Hospital between January 1, 2014 and September 1, 2019. Using definitions adapted from Pedimacs, adverse events (AEs) after catheterization were identified, including arrhythmia; major bleeding or acute kidney injury within 24 hours; respiratory failure persisting at 24 hours; and stroke, pericardial effusion, device malfunction, bacteremia or death within 7 days. AEs were categorized as related or unrelated to catheterization. Sixty procedures were performed on 39 patients. Underlying diagnoses were dilated cardiomyopathy (48%), 1V-CHD (35%), 2V-CHD (8%), and other (8%). Devices were implantable continuous flow (72%), paracorporeal pulsatile (18%) and paracorporeal continuous flow (10%). Catheterizations were performed on patients in the ICU (60%), on inotropic support (42%), with deteriorating clinical status (37%) and on BiVAD support (12%). There were 9 AEs possibly related to catheterization including 6 episodes of respiratory failure, 2 major bleeding events, and 1 procedural arrhythmia. AE occurrence was associated with ICU status (P = 0.01), BiVAD support (P = 0.04) and procedural indication to evaluate worsening clinical status (P = 0.04). Despite high medical acuity, catheterization can be performed with an acceptable AE profile in children on VAD support.

  View details for DOI 10.1097/MAT.0000000000001627

  View details for PubMedID 34967779

• 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

  Abstract

  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

• RAPID AORTIC HOMOGRAFT DEGENERATION AND VAD SUPPORT IN A NEONATE WITH SINGLE VENTRICLE HEART DISEASE Ahmed, H., Chen, S., Yarlagadda, V., Almond, C., Murray, J., Rosenthal, D. N., Ma, M., Dykes, J., Maeda, K. ELSEVIER SCIENCE INC. 2020: 2865

  View details for Web of Science ID 000522979102649

• Evolution of Single Ventricular Assist Device Support for the Failing Bi-directional Glenn Patient. The Annals of thoracic surgery Maeda, K., Nasirov, T., Yarlagadda, V., Hollander, S. A., Navaratnam, M., Rosenthal, D. N., Dykes, J. C., Kaufman, B. D., Almond, C. S., Reinhartz, O., Murray, J., Chen, S. 2020

  Abstract

  BACKGROUND: Given poor outcomes, strategies to improve ventricular assist device (VAD) for single ventricle (SV) patients with bi-directional Glenn (BDG) palliation is needed.METHODS: Retrospective review of our institutional experience with VAD support for BDG patients from April 2011 to January 2019. Surgical strategies, complications and causes of death are described. Survival to transplant for various strategies are compared.RESULTS: Seven patients with BDG (weights 5.6-28.8 kg, ages 7 months - 11 years) underwent VAD implantation. Three patients received Berlin Heart EXCOR, 2 received Heartware HVADs and 2 patients received paracorporeal continuous flow devices. Four patients underwent ventricular inflow cannulation, and 3 underwent atrial inflow cannulation. At the time of VAD implant, the BDG was left intact in 3 patients, taken down in 3 patients, and created de novo in 1 patient. Over a total of 420 VAD support days, 2 patients survived to heart transplant; one with Heartware ventricular cannulation and intact BDG (after 174 days), and another with Berlin Heart atrial cannulation and BDG take-down (after 72 days). There were 3 deaths within 2 weeks of VAD implant (2 due to respiratory failure, 1 due to infection) and 2 deaths after 30 days due to strokes.CONCLUSIONS: The surgical strategy and postoperative management of VAD with BDG are still evolving. Successful support can be achieved with 1) both pulsatile and continuous flow pumps, 2) atrial or ventricular cannulation, and 3) with or without BDG take-down. Surgical strategy should be determined by individual patient anatomy, physiology and condition.

  View details for DOI 10.1016/j.athoracsur.2019.12.088

  View details for PubMedID 32151575

• Bridge to Transplant with Ventricular Assist Device Support in Pediatric Patients with Single Ventricle Heart Disease ASAIO JOURNAL Chen, S., Rosenthal, D. N., Murray, J., Dykes, J. C., Almond, C. S., Yarlagadda, V. V., Wright, G., Navaratnam, M., Reinhartz, O., Maeda, K. 2020; 66 (2): 205–11

  View details for DOI 10.1097/MAT.0000000000000983

  View details for Web of Science ID 000530106400023

• Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease. The Journal of pediatrics Luong, R. n., Feinstein, J. A., Ma, M. n., Ebel, N. H., Wise-Faberowski, L. n., Zhang, Y. n., Peng, L. F., Yarlagadda, V. V., Shek, J. n., Hanley, F. L., McElhinney, D. B. 2020

  Abstract

  To assess outcomes in a large cohort of patients with Alagille Syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex PA disease.Patients with ALGS who underwent PA reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch PA stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs RESULTS: Fifty-one patients with ALGS underwent PA surgery at our center: 22 with severe branch PA stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) had a complete repair at the first surgery, compared with 8 (89%) and 19 (86%) with TOF without MAPCAs and isolated branch PA stenosis, respectively. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median PA:aortic systolic pressure ratio of 0.38. Nine patients (18%), 8 with isolated branch PA stenosis, underwent liver transplantation.Most patients with ALGS and complex PA disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcome, with higher mortality and more frequent PA interventions compared with patients with TOF without MAPCAs or isolated branch PA stenosis. Complex PA disease is not a contraindication to liver transplantation in patients with ALGS.

  View details for DOI 10.1016/j.jpeds.2020.09.053

  View details for PubMedID 32980376

• A Quality Bundle to Support High-Risk Pediatric Ventricular Assist Device Implantation. Pediatric cardiology Knoll, C., Chen, S., Murray, J. M., Dykes, J. C., Yarlagadda, V. V., Rosenthal, D. N., Almond, C. S., Maeda, K., Shin, A. Y. 2019

  Abstract

  Pediatric ventricular assist device (VAD) implantation outcomes are increasingly promising for children with dilated cardiomyopathy and advanced decompensated heart failure (ADHF). VAD placement in patients with clinical features such as complex congenital cardiac anatomy, small body size, or major comorbidities remains problematic. These comorbidities have been traditionally prohibitive for VAD consideration leaving these children as a treatment-orphaned population. Here we describe the quality bundle surrounding these patients with ADHF considered high risk for VAD implantation at our institution. Over a 7-year period, a quality bundle aimed at the peri-operative care for children with high-risk features undergoing VAD implantation was incrementally implemented at a tertiary children's hospital. Patients were considered high risk if they were neonates (<30days), had single-ventricle physiology, non-dilated cardiomyopathy, biventricular dysfunction, or significant comorbidities. The quality improvement bundle evolved to include (1) structured team-based peri-operative evaluation, (2) weekly VAD rounds addressing post-operative device performance, (3) standardized anticoagulation strategies, and (4) a multidisciplinary system for management challenges. These measures aimed to improve communication, standardize management, allow for ongoing process improvement, and incorporate principles of a high-reliability organization. Between January 2010 and December 2017, 98 patients underwent VAD implantation, 48 (49%) of which had high-risk comorbidities and a resultant cohort survival-to-transplant rate of 65%. We report on the evolution of a quality improvement program to expand the scope of VAD implantation to patients with high-risk clinical profiles. This quality bundle can serve as a template for future large-scale collaborations to improve outcomes in these treatment-orphaned subgroups.

  View details for DOI 10.1007/s00246-019-02123-1

  View details for PubMedID 31087144

• Bridge to Transplant with Ventricular Assist Device Support in Pediatric Patients with Single Ventricle Heart Disease. ASAIO journal (American Society for Artificial Internal Organs : 1992) Chen, S., Rosenthal, D. N., Murray, J., Dykes, J. C., Almond, C. S., Yarlagadda, V. V., Wright, G., Navaratnam, M., Reinhartz, O., Maeda, K. 2019

  Abstract

  Ventricular assist device (VAD) support for children with single ventricle (SV) heart disease remains challenging. We performed a single-center retrospective review of SV patients on VAD support and examined survival to transplant using the Kaplan-Meier method. Patients transplanted were compared with those who died on support. Between 2009 and 2017, there were 14 SV patients with 1,112 patient-days of VAD support. Stages of palliation included pre-Glenn (n = 5), Glenn (n = 5), and Fontan (n = 4). Eight patients (57%) were successfully bridged to transplant at a median 107 days. Deaths occurred early (n = 6, median 16 days) and in smaller patients (10.1 vs. 28.3 kg, P = 0.04). All Fontan patients survived to transplant, whereas only 20% of Glenn patients survived to transplant. Adverse events occurred in 79% (n = 11). Five patients met hospital discharge criteria, with two patients (one pre-Glenn, one Glenn) discharged and transplanted after 219 and 174 days of VAD support. All transplanted patients were discharged at a median 21 days posttransplant. SV patients in various stages of palliation can be successfully bridged to transplant with VAD support. With use of intracorporeal continuous-flow devices, longer-term support and hospital discharge are possible.

  View details for PubMedID 30864969

• Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Feinstein, J. A., Yarlagadda, V. V., Lin, A. n., Wise-Faberowski, L. n., McElhinney, D. B. 2019

  Abstract

  To evaluate the incidence, severity, and outcomes of pulmonary hemorrhage in children with Alagille syndrome (AGS) undergoing cardiac catheterization, and to find variables associated with hemorrhage in this population.Children with AGS have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic.This was a retrospective single institution study of children with AGS undergoing catheterization from 2010 to 2018. Pulmonary hemorrhage was defined as angiographic or fluoroscopic evidence of extravasated blood in the lung parenchyma, or blood suctioned from the endotracheal tube with documentation of pulmonary hemorrhage by the anesthesiologist or intensivist. Univariate comparisons were made between catheterizations that did and did not have pulmonary hemorrhage.Thirty children with AGS underwent 87 catheterizations, 32 (37%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24 hr of mechanical ventilation. Moderate and severe hemorrhage occurred only in children with tetralogy of Fallot (TOF; 5 of 14, 36%). A higher right ventricle to aorta systolic pressure ratio (1.0 [0.85-1.1] vs. 0.88 [0.59-1.0], p = .029) and interventions on the branch PAs (14 of 26, 54% vs. 18 of 61, 30%, p = .032) were associated with hemorrhage.Pulmonary hemorrhage was common in children with AGS undergoing both intervention and diagnostic cardiac catheterization, and was associated with TOF, higher RV to aorta pressure ratio, and interventions on the branch PAs.

  View details for DOI 10.1002/ccd.28508

  View details for PubMedID 31584246

• Outcomes of Infants Supported With Extracorporeal Membrane Oxygenation Using Centrifugal Versus Roller Pumps: An Analysis From the Extracorporeal Life Support Organization Registry. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies O'Halloran, C. P., Thiagarajan, R. R., Yarlagadda, V. V., Barbaro, R. P., Nasr, V. G., Rycus, P. n., Anders, M. n., Alexander, P. M. 2019

  Abstract

  To determine whether mortality differs between roller and centrifugal pumps used during extracorporeal membrane oxygenation in infants weighing less than 10 kg.Retrospective propensity-matched cohort study.All extracorporeal membrane oxygenation centers reporting to the Extracorporeal Life Support Organization.All patients less than 10 kg supported on extracorporeal membrane oxygenation during 2011-2016 within Extracorporeal Life Support Organization Registry.Centrifugal and roller pump recipients were propensity matched (1:1) based on predicted probability of receiving a centrifugal pump using demographic variables, indication for extracorporeal membrane oxygenation, central versus peripheral cannulation, and pre-extracorporeal membrane oxygenation patient management.A total of 12,890 patients less than 10 kg were supported with extracorporeal membrane oxygenation within the Extracorporeal Life Support Organization registry during 2011-2016. Patients were propensity matched into a cohort of 8,366. Venoarterial and venovenous extracorporeal membrane oxygenation runs were propensity matched separately. The propensity-matched cohorts were similar except earlier year of extracorporeal membrane oxygenation (standardized mean difference, 0.49) in the roller pump group. Within the propensity-matched cohort, survival to discharge was lower in the centrifugal pump group (57% vs 59%; odds ratio, 0.91; 95% CI, 0.83-0.99; p = 0.04). Hemolytic, infectious, limb injury, mechanical, metabolic, neurologic, pulmonary, and renal complications were more frequent in the centrifugal pump group. Hemorrhagic complications were similar between groups. Hemolysis mediated the relationship between centrifugal pumps and mortality (indirect effect, 0.023; p < 0.001).In this propensity score-matched cohort study of 8,366 extracorporeal membrane oxygenation recipients weighing less than 10 kg, those supported with centrifugal pumps had increased mortality and extracorporeal membrane oxygenation complications. Hemolysis was evaluated as a potential mediator of the relationship between centrifugal pump use and mortality and met criteria for full mediation.

  View details for DOI 10.1097/PCC.0000000000002103

  View details for PubMedID 31567621

• 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

  Abstract

  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

• Successful use of a ventricular assist device in a neonate with hypoplastic left heart syndrome with right ventricular dysfunction JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maeda, K., Yarlagadda, V. V., Rosenthal, D. N., Almond, C. S. 2018; 156 (4): E171–E173

  View details for DOI 10.1016/j.jtcvs.2018.05.043

  View details for Web of Science ID 000445221200009

• Impact of Phrenic Nerve Palsy and Need for Diaphragm Plication Following Surgery for Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals SEMINARS IN THORACIC AND CARDIOVASCULAR SURGERY Greene, C. L., Mainwaring, R. D., Sidell, D., Yarlagadda, V. V., Patrick, W. L., Hanley, F. L. 2018; 30 (3): 318–24

  View details for DOI 10.1053/j.semtcvs.2018.03.004

  View details for Web of Science ID 000450371100013

• An alternative cannulation approach for venovenous extracorporeal membrane oxygenation in children for long-term ambulatory support JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maeda, K., Ryan, K., Conrad, C. K., Yarlagadda, V. V. 2018; 156 (1): E13–E14

  View details for DOI 10.1016/j.jtcvs.2018.03.099

  View details for Web of Science ID 000436592600005

• Successful use of a ventricular assist device in a neonate with hypoplastic left heart syndrome with right ventricular dysfunction. The Journal of thoracic and cardiovascular surgery Maeda, K., Yarlagadda, V. V., Rosenthal, D. N., Almond, C. S. 2018

  View details for PubMedID 29970231

• An alternative cannulation approach for venovenous extracorporeal membrane oxygenation in children for long-term ambulatory support. The Journal of thoracic and cardiovascular surgery Maeda, K., Ryan, K., Conrad, C. K., Yarlagadda, V. V. 2018

  View details for PubMedID 29685584

• Impact of Phrenic Nerve Palsy and Need for Diaphragm Plication Following Surgery for Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals. Seminars in thoracic and cardiovascular surgery Greene, C. L., Mainwaring, R. D., Sidell, D., Yarlagadda, V. V., Patrick, W. L., Hanley, F. L. 2018

  Abstract

  Injury to the phrenic nerves may occur during surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD and MAPCAs). These patients may develop respiratory failure and require diaphragm plication. The purpose of this study was to evaluate the impact of phrenic nerve palsy on recovery following surgery for PA-VSD and MAPCAs. Between 2007 and 2016, approximately 500 patients underwent surgery for PA-VSD and MAPCAs at our institution. Twenty-four patients (4.8%) subsequently had evidence of new phrenic nerve palsy. Sixteen patients were undergoing their first surgical procedure, whereas 8 were undergoing reoperations. All 24 patients underwent diaphragm plication. A cohort of matched controls was identified based on identical diagnosis and procedures but did not sustain a phrenic nerve palsy. Eighteen of the 24 patients (75%) had clinical improvement following diaphragm plication as evidenced by the ability to undergo successful extubation (5±2 days), transition out of the intensive care unit (32±16 days), and discharge from the hospital (42±19 days). In contrast, there were 6 patients (25%) who did not demonstrate a temporal improvement following diaphragm plication, as evidenced by intervals of 61±38, 106±45, and 108±46 days, respectively (P<0.05 for all 3 comparisons). The 6 patients who failed to improve following diaphragm plication had a significantly greater number of comorbidities compared to the 18 patients who demonstrated improvement (2.2 vs 0.6 per patient, P<0.05). When compared with the control group, patients who improved following diaphragm plication spent an additional 22 days and patients who failed to improve an additional 90 days in the hospital. The data demonstrate a bifurcation of clinical outcome in patients undergoing diaphragm plication following surgery for PA-VSD and MAPCAs. This bifurcation appears to be linked to the presence or absence of other comorbidities.

  View details for PubMedID 29545034

• INSURANCE TYPE IS ASSOCIATED WITH LONGER HOSPITAL DURATION BUT SIMILAR MORTALITY AMONG CHILDREN UNDERGOING CONGENITAL HEART SURGERY Ryan, K., Algaze, C., Sakarovitch, C., Do, T., Yarlagadda, V., Fernandes, S., McElhinney, D., Roth, S., Krawczeski, C., Shin, A. ELSEVIER SCIENCE INC. 2017: 569

  View details for Web of Science ID 000397342301091

• A novel inflow cannulation strategy for pediatric mechanical circulatory support in small left ventricles. journal of thoracic and cardiovascular surgery Ma, M., Yarlagadda, V. V., Rosenthal, D. N., Maeda, K. 2017

  View details for DOI 10.1016/j.jtcvs.2017.03.034

  View details for PubMedID 28416331

• Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries. World journal for pediatric & congenital heart surgery Ibrahimiye, A. N., Mainwaring, R. D., Patrick, W. L., Downey, L., Yarlagadda, V., Hanley, F. L. 2017; 8 (2): 203-209

  Abstract

  Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure.This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining.Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions.The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

  View details for DOI 10.1177/2150135116683939

  View details for PubMedID 28329464

• Temporary Circulatory Support in U.S. Children Awaiting Heart Transplantation. Journal of the American College of Cardiology Yarlagadda, V. V., Maeda, K. n., Zhang, Y. n., Chen, S. n., Dykes, J. C., Gowen, M. A., Shuttleworth, P. n., Murray, J. M., Shin, A. Y., Reinhartz, O. n., Rosenthal, D. N., McElhinney, D. B., Almond, C. S. 2017; 70 (18): 2250–60

  Abstract

  Extracorporeal membrane oxygenation (ECMO) has long served as the standard of care for short-term mechanical circulatory support in pediatrics. It is unknown whether newer-generation temporary circulatory support (TCS) devices afford children a meaningful survival advantage over ECMO.This study sought to determine whether bridge-to-heart transplant survival with a TCS device is superior to ECMO after adjusting for patient differences.All children ≤21 years of age listed for heart transplant from 2011 to 2015 who received a TCS device or ECMO as a bridge to transplant were identified using Organ Procurement and Transplantation Network data. Children supported with a TCS device were compared with a propensity score (PS)-matched cohort of children supported with ECMO as a bridge to transplant. The primary endpoint was Kaplan-Meier survival to transplant.The number of TCS devices implanted in children increased from ≤3 per year before 2011 to 50 in 2015. Overall, 93 patients implanted with TCS devices were included for analysis (59% left ventricular assist devices, 23% right ventricular assist devices, 18% biventricular assist devices). The most commonly used device was the CentriMag-PediMag system (65%), followed by TandemHeart (18%), Rotaflow (6%), and Impella (5%). Among 164 PS-matched patients, support duration was longer for the TCS cohort (median 19 days vs. 6 days; p < 0.001), and was longest for the CentriMag-PediMag (24 days vs. 6 days; p < 0.001) with 27% supported for >60 days. Compared with the ECMO cohort, the PS-matched TCS cohort had longer survival to transplant (hazard ratio: 0.49; 95% confidence interval: 0.30 to 0.79) and longer overall survival (hazard ratio: 0.61; 95% confidence interval: 0.39 to 0.96), with 90-day mortality before transplant that was modestly reduced (from 45% with ECMO to 39% with TCS).The use of TCS devices in children as a bridge to transplant has risen rapidly in recent years, led by the growth of magnetically levitated centrifugal flow pumps. Compared with conventional ECMO, TCS durations are longer, and more importantly, patient survival is superior.

  View details for PubMedID 29073953

• Alternative Strategy for Biventricular Assist Device in an Infant With Hypertrophic Cardiomyopathy. The Annals of thoracic surgery Dykes, J. C., Reinhartz, O. n., Almond, C. S., Yarlagadda, V. n., Murray, J. n., Rosenthal, D. N., Maeda, K. n. 2017; 104 (2): e185–e186

  Abstract

  We report an infant with hypertrophic cardiomyopathy who underwent biventricular assist device placement with two 15-mL Berlin Heart EXCOR pediatric ventricular assist devices using an alternative atrial cannulation strategy. The systemic circulation was supported by left atrium (LA) to aorta cannulation. The LA was accessed through the right atrium by extending a 6-mm EXCOR cannula with a Gore-Tex graft connected to an atrial septal defect. The pulmonary circulation was supported with cannulation of the right atrium to pulmonary artery. This alternative cannulation strategy facilitated effective biventricular support and may be applicable to other patients with hypertrophic or restrictive physiology.

  View details for PubMedID 28734448

• Anesthesia for Placement of a Paracorporeal Lung Assist Device and Subsequent Heart-Lung Transplantation in a Child with Suprasystemic Pulmonary Hypertension and End-Stage Respiratory Failure. A & A case reports Char, D. S., Yarlagadda, V., Maeda, K., Williams, G. 2016; 6 (10): 308-310

  Abstract

  Pediatric patients with end-stage respiratory failure and pulmonary hypertension traditionally have poor outcomes when bridged with extracorporeal membrane oxygenation to lung or heart-lung transplantation. Therefore, several institutions have attempted paracorporeal lung assist devices as a bridge. However, given the small number of patients, little is known about approaches to anesthetic induction in these hemodynamically unstable patients either before placement of a device or anesthetic induction once a device is in situ. In this case report, we describe our anesthetic experience managing a 13-year-old boy for both paracorporeal lung assist device placement and subsequent heart-lung transplantation.

  View details for DOI 10.1213/XAA.0000000000000300

  View details for PubMedID 27002753

• Major Causes of Stroke in Children With Congenital and Acquired Heart Disease. Elbers, J., Lee, E., Yarlagadda, V. V., Lo, C., Hanisch, D., Lin, A., Almond, C. S., Shin, A. Y. LIPPINCOTT WILLIAMS & WILKINS. 2016

  View details for Web of Science ID 000399956102226