Greg Adamson

All Publications

• Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension. Pulmonary circulation Kochanski, J. J., Feinstein, J. A., Ogawa, M., Ritter, V., Hopper, R. K., Adamson, G. T. 2024; 14 (1): e12328

  Abstract

  Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single-center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow-up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days-18 years treated with subcutaneous TRE, younger age (cut-off of 6-years of age, AUC 0.824) at TRE initiation was associated with superior 5-year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow-up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow-up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies.

  View details for DOI 10.1002/pul2.12328

  View details for PubMedID 38348195

  View details for PubMedCentralID PMC10860541

• Current practices for refractory chylothorax following congenital heart surgery. Cardiology in the young Adamson, G. T., Winder, M. M., Catton, K. G., Dewitt, A. G., Kozyak, B. W., Glenn, E. T., Bailly, D. K. 2023: 1-5

  Abstract

  Chylothorax following paediatric cardiac surgery is associated with significant morbidity, particularly those that are refractory to conservative therapy. It is our impression that there is important variability in the medical, surgical, and interventional therapies used to manage refractory chylothorax between congenital heart programmes. We therefore conducted a survey study of current practices for managing refractory chylothorax.The Chylothorax Work Group, formed with the support of the Pediatric Cardiac Critical Care Consortium, designed this multi-centre survey study with a focus on the timing and indication for utilising known therapies for refractory chylothorax. The survey was sent to one chylothorax expert from each Work Group centre, and results were summarised and reported as the frequency of given responses.Of the 20 centres invited to participate, 17 (85%) submitted complete responses. Octreotide (13/17, 76%) and sildenafil (8/17, 47%) were the most utilised medications. Presently, 9 (53%) centres perform pleurodesis, 15 (88%) perform surgical thoracic duct ligation, 8 (47%) perform percutaneous lymphatic interventions, 6 (35%) utilise thoracic duct decompression procedures, and 3 (18%) perform pleuroperitoneal shunts. Diagnostic lymphatic imaging is performed prior to surgical thoracic duct ligation in only 7 of the 15 (47%) centres that perform the procedure. Respondents identified barriers to referring and transporting patients to centres with expertise in lymphatic interventions.There is variability in the treatment of refractory post-operative chylothorax across a large group of academic heart centres. Few surveyed heart centres have replaced surgical thoracic duct ligation or pleurodesis with image-guided selective lymphatic interventions.

  View details for DOI 10.1017/S1047951123003918

  View details for PubMedID 38073584

• Acute Hemodynamics in the Fontan Circulation: Open-Label Study of Vasopressin. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Adamson, G. T., Yu, J., Ramamoorthy, C., Peng, L. F., Taylor, A., Lennig, M., Schmidt, A. R., Feinstein, J. A., Navaratnam, M. 2023

  Abstract

  OBJECTIVE: To describe the acute hemodynamic effect of vasopressin on the Fontan circulation, including systemic and pulmonary pressures and resistances, left atrial pressure, and cardiac index.DESIGN: Prospective, open-label, nonrandomized study (NCT04463394).SETTING: Cardiac catheterization laboratory at Lucile Packard Children's Hospital, Stanford.PATIENTS: Patients 3-50 years old with a Fontan circulation who were referred to the cardiac catheterization laboratory for hemodynamic assessment and/or intervention.INTERVENTIONS: A 0.03 U/kg IV (maximum dose 1 unit) bolus of vasopressin was administered over 5 minutes, followed by a maintenance infusion of 0.3 mU/kg/min (maximum dose 0.03 U/min).MEASUREMENTS AND MAIN RESULTS: Comprehensive cardiac catheterization measurements before and after vasopressin administration. Measurements included pulmonary artery, atrial, and systemic arterial pressures, oxygen saturations, and systemic and pulmonary flows and resistances. There were 28 patients studied. Median age was 13.5 (9.1, 17) years, and 16 (57%) patients had a single or dominant right ventricle. Following vasopressin administration, systolic blood pressure and systemic vascular resistance (SVR) increased by 17.5 (13.0, 22.8) mm Hg (Z value -4.6, p < 0.001) and 3.8 (1.8, 7.5) Wood Units (Z value -4.6, p < 0.001), respectively. The pulmonary vascular resistance (PVR) decreased by 0.4±0.4 WU (t statistic 6.2, p < 0.001), and the left atrial pressure increased by 1.0 (0.0, 2.0) mm Hg (Z value -3.5, p < 0.001). The PVR:SVR decreased by 0.04±0.03 (t statistic 8.1, p < 0.001). Neither the pulmonary artery pressure (median difference 0.0 [-1.0, 1.0], Z value -0.4, p = 0.69) nor cardiac index (0.1±0.3, t statistic -1.4, p = 0.18) changed significantly. There were no adverse events.CONCLUSIONS: In Fontan patients undergoing cardiac catheterization, vasopressin administration resulted in a significant increase in systolic blood pressure, SVR, and left atrial pressure, decrease in PVR, and no change in cardiac index or pulmonary artery pressure. These findings suggest that in Fontan patients vasopressin may be an option for treating systemic hypotension during sedation or general anesthesia.

  View details for DOI 10.1097/PCC.0000000000003326

  View details for PubMedID 37462430

• Prediction Model with External Validation for Early Detection of Postoperative Pediatric Chylothorax. Pediatric cardiology Moza, R., Winder, M., Adamson, G. T., Ou, Z., Presson, A. P., Vijayarajah, S., Goldstein, S. A., Bailly, D. K. 2023

  Abstract

  Earlier diagnosis of chylothorax following pediatric cardiac surgery is associated with decreased duration of chylothorax. Pleural fluid testing is used to diagnosis chylothorax which may delay detection in patients who are not enterally fed at time of chylothorax onset. Our aim was to develop and externally validate a prediction model to detect chylothorax earlier than pleural fluid testing in pediatric patients following cardiac surgery. A multivariable logistic regression model was developed to detect chylothorax using a stepwise approach. The model was developed using data from patients < 18 years following cardiac surgery from Primary Children's Hospital, a tertiary-care academic center, between 2017 and 2020. External validation used a contemporary cohort (n = 171) from Lucille Packard Children's Hospital. A total of 763 encounters (735 patients) were analyzed, of which 72 had chylothorax. The final variables selected were chest tube output (CTO) the day after sternal closure (dichotomized at 15.6 mL/kg/day, and as a continuous variable) and delayed sternal closure. The highest odds of chylothorax were associated with CTO on post-sternal closure day 1 > 15.6 mL/kg/day (odds ratio 11.3, 95% CI 6,3, 21.3). The c-statistic for the internal and external validation datasets using the dichotomized CTO variable were 0.78 (95% CI 0.73, 0.82) and 0.84 (95% CI, 0.78, 0.9) and performance improved when using CTO as a continuous variable (OR 0.84, CI: 95% CI 0.80, 0.87). Using the models described, chylothorax after pediatric cardiac surgery may be detected earlier and without reliance on enteral feeds.

  View details for DOI 10.1007/s00246-022-03034-4

  View details for PubMedID 36754886

• Role of left atrial hypertension in pulmonary hypertension associated with bronchopulmonary dysplasia Frontiers in Pediatrics Sullivan, R. T., Tandel, M. D., Bhombal, S., Adamson, G. T., Boothroyd, D. B., Tracy, M., Moy, A., Hopper, R. K. 2022: 1012136

  Abstract

  Left atrial hypertension (LAH) may contribute to pulmonary hypertension (PH) in premature infants with bronchopulmonary dysplasia (BPD). Primary causes of LAH in infants with BPD include left ventricular diastolic dysfunction or hemodynamically significant left to right shunt. The incidence of LAH, which is definitively diagnosed by cardiac catheterization, and its contribution to PH is unknown in patients with BPD-PH. We report the prevalence of LAH in an institutional cohort with BPD-PH with careful examination of hemodynamic contributors and impact on patient outcomes. This single-center, retrospective cohort study examined children <2 years of age with BPD-PH who underwent cardiac catheterization at Lucile Packard Children's Hospital Stanford. Patients with unrepaired simple shunt congenital heart disease (CHD) and pulmonary vein stenosis (only 1 or 2 vessel disease) were included. Patients with complex CHD were excluded. From April 2010 to December 2021, 34 patients with BPD-PH underwent cardiac catheterization. We define LAH as pulmonary capillary wedge pressure (PCWP) or left atrial pressure (LAP) of at least 10 mmHg. In this cohort, median PCWP was 8 mmHg, with LAH present in 32% (n = 11) of the total cohort. A majority (88%, n = 30) of the cohort had severe BPD. Most patients had some form of underlying CHD and/or pulmonary vein stenosis: 62% (n = 21) with an atrial septal defect or patent foramen ovale, 62% (n = 21) with patent ductus arteriosus, 12% (n = 4) with ventricular septal defect, and 12% (n = 4) with pulmonary vein stenosis. Using an unadjusted logistic regression model, baseline requirement for positive pressure ventilation at time of cardiac catheterization was associated with increased risk for LAH (odds ratio 8.44, 95% CI 1.46-48.85, p = 0.02). Small for gestational age birthweight, sildenafil use, and CHD were not associated with increased risk for LAH. LAH was associated with increased risk for the composite outcome of tracheostomy and/or death, with a hazard ratio of 6.32 (95% CI 1.72, 22.96; p = 0.005). While the etiology of BPD-PH is multifactorial, LAH is associated with PH in some cases and may play a role in clinical management and patient outcomes.

  View details for DOI 10.3389/fped.2022.1012136

  View details for PubMedCentralID PMC9615143

• Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD. Cardiology in the young Lion, R. P., Winder, M. M., Amirnovin, R., Fogg, K., Bertrandt, R., Bhaskar, P., Kasmai, C., Holmes, K. W., Moza, R., Vichayavilas, P., Gordon, E. E., Trauth, A., Horsley, M., Frank, D. U., Stock, A., Adamson, G., Lyman, A., Raymond, T., Diaz, I., DeMarco, A., Prodhan, P., Fundora, M., Aljiffry, A., Dewitt, A. G., Kozyak, B. W., Greiten, L., Scahill, C., Buckley, J., Bailly, D. K. 2022: 1-8

  Abstract

  A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap.Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. "Consensus" was defined as ≥ 80% of responses as "agree" or "strongly agree" to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not.The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for "low" and "high" volume patients, and timing and duration of fat-modified diet. All recommendations achieved "consensus" (agreement >80%) by the workgroup (range 81-100%). Ex vivo simulations demonstrated good understanding by developers (range 94-100%) and non-developers (73%-100%).The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

  View details for DOI 10.1017/S1047951122001871

  View details for PubMedID 35792060

• Risk Factors and Outcomes of Sudden Cardiac Arrest in Pediatric Heart Transplant Recipients. American heart journal Hollander, S. A., Barkoff, L., Giacone, H., Adamson, G. T., Kaufman, B. D., Motonaga, K. S., Dubin, A. M., Chubb, H. 2022

  Abstract

  Sudden cardiac arrest (SCA) is a prevailing cause of mortality after pediatric heart transplant (HT) but remains understudied. We analyzed the incidence, outcomes, and risk factors for SCA at our center.Retrospective review of all pediatric HT patients at our center from 1/1/2009-9/1/2021. SCA was defined as an abrupt loss of cardiac function requiring cardiopulmonary resuscitation and/or mechanical circulatory support (MCS). Events that occurred in the setting of limited resuscitative wishes, or while on MCS were excluded. Patient characteristics and risk factors were analyzed.Fourteen of 254 (6%) experienced SCA at a median of 3 (1, 4) years post-HT. Seven (50%) events occurred out-of-hospital. Eleven (79%) died from their initial event, 2 (18%) after failure to separate from extracorporeal membrane (ECMO). In univariate analysis, black race, younger donor age, prior acute cellular rejection (ACR) episode, pacemaker and/or ICD in place, and pre-mortem diagnosis of allograft vasculopathy were associated with SCA (P=0.003-0.02). In multivariable analysis, history of ACR, younger donor age, and black race retained significance. [OR=6.3, 95% CI: 1.6-25.4, P=0.01], [OR=0.9, 95% CI: 0.8-1, P=0.04], and [OR=7.3, 95% CI: 1.1-49.9, P=0.04], respectively. SCA occurred in 3 patients with a functioning ICD or pacemaker, which failed to restore a perfusing rhythm.SCA occurs relatively early after pediatric HT and is usually fatal. Half of events happen at home. Those who received younger donors, have a history of ACR, or are of black race are at increased risk. ICDs/pacemakers may offer limited protection.

  View details for DOI 10.1016/j.ahj.2022.06.003

  View details for PubMedID 35705134

• Virtual Transcatheter Interventions for Peripheral Pulmonary Artery Stenosis in Williams and Alagille Syndromes. Journal of the American Heart Association Lan, I. S., Yang, W., Feinstein, J. A., Kreutzer, J., Collins, R. T., Ma, M., Adamson, G. T., Marsden, A. L. 2022: e023532

  Abstract

  Background Despite favorable outcomes of surgical pulmonary artery (PA) reconstruction, isolated proximal stenting of the central PAs is common clinical practice for patients with peripheral PA stenosis in association with Williams and Alagille syndromes. Given the technical challenges of PA reconstruction and the morbidities associated with transcatheter interventions, the hemodynamic consequences of all treatment strategies must be rigorously assessed. Our study aims to model, assess, and predict hemodynamic outcomes of transcatheter interventions in these patients. Methods and Results Isolated proximal and "extensive" interventions (stenting and/or balloon angioplasty of proximal and lobar vessels) were performed in silico on 6 patient-specific PA models. Autoregulatory adaptation of the cardiac output and downstream arterial resistance was modeled in response to intervention-induced hemodynamic perturbations. Postintervention computational fluid dynamics predictions were validated in 2 stented patients and quantitatively assessed in 4 surgical patients. Our computational methods accurately predicted postinterventional PA pressures, the primary indicators of success for treatment of peripheral PA stenosis. Proximal and extensive treatment achieved median reductions of 14% and 40% in main PA systolic pressure, 27% and 56% in pulmonary vascular resistance, and 10% and 45% in right ventricular stroke work, respectively. Conclusions In patients with Williams and Alagille syndromes, extensive transcatheter intervention is required to sufficiently reduce PA pressures and right ventricular stroke work. Transcatheter therapy was shown to be ineffective for long-segment stenosis and pales hemodynamically in comparison with published outcomes of surgical reconstruction. Regardless of the chosen strategy, a virtual treatment planning platform could identify lesions most critical for optimizing right ventricular afterload.

  View details for DOI 10.1161/JAHA.121.023532

  View details for PubMedID 35253446

• Postoperative Acute Kidney Injury in Williams Syndrome Compared With Matched Controls. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Yokota, R., Kwiatkowski, D. M., Journel, C., Adamson, G. T., Zucker, E., Suarez, G., Lechich, K. M., Chaudhuri, A., Collins, R. T. 2022

  Abstract

  Cardiovascular manifestations occur in over 80% of Williams syndrome (WS) patients and are the leading cause of morbidity and mortality. One-third of patients require cardiovascular surgery. Renal artery stenosis (RAS) is common in WS. No studies have assessed postoperative cardiac surgery-related acute kidney injury (CS-AKI) in WS. Our objectives were to assess if WS patients have higher risk of CS-AKI postoperatively than matched controls and if RAS could contribute to CS-AKI.This was a retrospective study of all patients with WS who underwent cardiac surgery at our center from 2010 to 2020. The WS study cohort was compared with a group of controls matched for age, sex, weight, and surgical procedure.Patients underwent cardiac surgery and postoperative care at Lucile Packard Children's Hospital Stanford.There were 27 WS patients and 43 controls (31% vs 42% female; p = 0.36). Median age was 1.8 years (interquartile range [IQR], 0.7-3.8 yr) for WS and 1.7 years (IQR, 0.8-3.1 yr) for controls.None.Postoperative hemodynamics, vasopressor, total volume input, diuretic administration, and urine output were collected in the first 72 hours. Laboratory studies were collected at 8-hour intervals. Multivariable analysis identified predictors of CS-AKI.Controlled for renal perfusion pressure (RPP) and vasoactive inotrope score (VIS), compared with controls, the odds ratio (OR) of CS-AKI in WS was 4.2 (95% CI, 1.1-16; p = 0.034). Higher RPP at postoperative hours 9-16 was associated with decreased OR of CS-AKI (0.88 [0.8-0.96]; p = 0.004). Increased VIS at hour 6 was associated with an increased OR of CS-AKI (1.47 [1.14-1.9]; p = 0.003). Younger age was associated with an increased OR of CS-AKI (1.9 [1.13-3.17]; p = 0.015).The OR of CS-AKI is increased in pediatric patients with WS compared with controls. CS-AKI was associated with VIS at the sixth postoperative hour. Increases in RPP and mean arterial pressure were associated with decreased odds of CS-AKI.

  View details for DOI 10.1097/PCC.0000000000002872

  View details for PubMedID 34982759

• Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals JOURNAL OF INVASIVE CARDIOLOGY Long, Z. B., Adamson, G. T., Peng, L. F., Perry, S. B., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021; 33 (5): E378-E386

  View details for Web of Science ID 000648791300008

• To unifocalize or not to unifocalize?: A comparison of retro-esophageal versus anterior collaterals. The Annals of thoracic surgery Mainwaring, R. D., Adamson, G., Hanley, F. L. 2021

  Abstract

  BACKGROUND: The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retro-esophageal) and bronchus before entering the lung parenchyma. The purpose of this study was to compare the physiologic and surgical characteristics of retro-esophageal versus anterior located MAPCAs.METHODS: This was a retrospective review of 42 patients who had one (n=36) or two (n=6) retro-esophageal MAPCAs. These MAPCAs were then characterized as: 1) single supply, meaning no connection to the pulmonary arteries, 2) dual supply, but inadequate connection to the distal pulmonary vascular bed, and 3) dual supply with adequate connection.RESULTS: For the 42 patients there were a total of 187 MAPCAs, or 4.5 MAPCAs per patient. Forty-eight MAPCAs were retro-esophageal, including 40 that were single supply, 6 were dual supply with inadequate connection, and 2 had dual supply with adequate connection. Based on this anatomy and physiology, 96% of retro-esophageal MAPCAs were unifocalized. For the 139 anterior MAPCAs, 89 were single supply, 15 were dual supply with inadequate connection, and 35 were dual supply with adequate connection. Based on this anatomy, 75% of anterior MAPCAs were unifocalized (p < 0.01 compared to retro-esophageal MAPCAs).CONCLUSIONS: The data demonstrate that retro-esophageal MAPCAs had very different anatomy and physiology compared to anterior MAPCAs. These results suggest that nearly every retro-esophageal MAPCA should be unifocalized to incorporate the lung segments supplied.

  View details for DOI 10.1016/j.athoracsur.2021.02.017

  View details for PubMedID 33631151

• Alternative to heart-lung transplantation for end-stage tetralogy of Fallot with major aortopulmonary collaterals: Simultaneous heart transplantation and pulmonary artery reconstruction. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Adamson, G. T., Profita, E. L., Quinonez, Z. A., McElhinney, D. B., Rosenthal, D. N., Ma, M. n. 2021

  View details for DOI 10.1016/j.healun.2021.02.003

  View details for PubMedID 33674153

• The COAST Is Not Clear: Late Aneurysms and Fractures. Circulation. Cardiovascular interventions Adamson, G. T., Lee, K. J. 2021: CIRCINTERVENTIONS121010886

  View details for DOI 10.1161/CIRCINTERVENTIONS.121.010886

  View details for PubMedID 34039014

• Comprehensive diagnostic catheterization in children with major aortopulmonary collateral arteries: A review of catheterization technique and anatomic nomenclature. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Perry, S. B., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  Achieving an optimal surgical result in patients with major aortopulmonary collateral arteries (MAPCAs) requires a thorough preoperative evaluation of the anatomy and physiology of the pulmonary circulation. This review provides a detailed description of diagnostic catheterization in patients with MAPCAs, including a summary of catheterization techniques, an overview of commonly used terms, and a review of MAPCA and pulmonary artery angiographic anatomy.

  View details for DOI 10.1002/ccd.30013

  View details for PubMedID 34800077

• Unilateral branch pulmonary artery origin from a solitary arterial trunk with major aortopulmonary collaterals to the contralateral lung: anatomic and developmental considerations. Seminars in thoracic and cardiovascular surgery Adamson, G. T., Houyel, L. n., McElhinney, D. B., Maskatia, S. A., Ma, M. n., Hanley, F. L., Lopez, L. n. 2020

  Abstract

  In both truncus arteriosus communis (TAC) and tetralogy of Fallot (TOF), there is a rare phenotype that includes a single branch pulmonary artery (PA) arising from a solitary great artery and major aortopulmonary collaterals (MAPCAs) supplying the contralateral lung. We describe the intracardiac and great vessel anatomy of infants with this phenotype, consider rationale for classifying patients as TOF vs. TAC, and describe surgical outcomes. Our institution's surgical database was reviewed for patients with a single branch PA from a solitary arterial trunk and contralateral MAPCAs from 2007 to 2019. Demographic, imaging, and surgical data were collected and described. All 11 patients underwent complete repair with a median right ventricular to aortic pressure ratio of 0.36 (range 0.26-0.50). At 0.1-9.1 years after repair (median 0.8 years) there was approximately balanced left-right lung perfusion (median 52% to the right lung, range 34-74%). The MAPCA lungs exemplified the full spectrum of PA and MAPCA anatomy, from absent intrapericardial PAs with all single supply MAPCAs to a normally arborizing PA with all dual supply MAPCAs and present pulmonary valve leaflet tissue. All patients had a systemic semilunar valve with 3 thin and similarly sized leaflets and fibrous continuity with the tricuspid valve, and all had coronary origins and outflow tract morphology more consistent with TOF. It is appropriate to classify all patients with a single anomalous PA from a solitary arterial trunk and MAPCAs to the contralateral lung as TOF rather than TAC Type A3. All variants were amenable to surgical repair.

  View details for DOI 10.1053/j.semtcvs.2020.11.009

  View details for PubMedID 33181302

• Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot. Journal of the American Heart Association Adamson, G. T., McElhinney, D. B., Zhang, Y. n., Feinstein, J. A., Peng, L. F., Ma, M. n., Algaze, C. A., Hanley, F. L., Perry, S. B. 2020: e017981

  Abstract

  Background Due in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs. Methods and Results All infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross-sectional area were less likely to undergo complete repair. Conclusions MAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross-sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.

  View details for DOI 10.1161/JAHA.120.017981

  View details for PubMedID 33283588

• Postoperative Recovery of Left Ventricular Function following Repair of Large Ventricular Septal Defects in Infants. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Adamson, G. T., Arunamata, A., Tacy, T. A., Silverman, N. H., Ma, M., Maskatia, S. A., Punn, R. 2019

  Abstract

  INTRODUCTION: Early postoperative left ventricular (LV) dysfunction is observed following repair of large ventricular septal defects (VSDs), but the frequency and rate of recovery of LV function are unknown. This study aims to characterize the incidence and rate of recovery of postoperative LV dysfunction following repair of large VSDs and to improve understanding of LV mechanics before and after VSD repair.METHODS: Infants who underwent surgical repair of an isolated large VSD were included. Pre- and postoperative echocardiographic images were reviewed, and LV function was assessed by both conventional echocardiography and speckle-tracking strain analyses. Postoperative LV dysfunction was defined as an LV ejection fraction (LVEF)<50% using the 5/6 area-length method. Echocardiograms were reviewed to assess LVEF and LV volume through 1-year follow-up.RESULTS: Of 104 infants evaluated (median age, 0.31 [0.22, 0.56] years), all had normal preoperative LVEF and 39 (38%) had postoperative LV dysfunction. Follow-up echocardiograms were available in 31 (80%) patients, all of whom had LVEF>50% within 9months of surgery. Lower preoperative apical four-chamber longitudinal strain (A4LS) and greater LV end-diastolic volume indexed to body surface area1.38 were independently associated with postoperative LV dysfunction. An absolute preoperative A4LS < 16.7% (area under the curve=0.87; 95% CI, 0.78-0.95; P<.001) was most strongly associated with postoperative dysfunction.CONCLUSIONS: Infants with LV dysfunction following repair of large VSDs recover function within 9months. Preoperative A4LS can be helpful to detect subclinical LV dysfunction in the setting of a large hemodynamically significant VSD and guide postoperative expectations for providers and families.

  View details for DOI 10.1016/j.echo.2019.10.003

  View details for PubMedID 31866322

• Secondary repair of incompetent pulmonary valves after previous surgery or intervention: Patient selection and outcomes. The Journal of thoracic and cardiovascular surgery Adamson, G. T., McElhinney, D. B., Lui, G., Meadows, A. K., Rigdon, J., Hanley, F. L., Maskatia, S. A. 2019

  Abstract

  OBJECTIVES: Pulmonary valve (PV) regurgitation (PR) is common after intervention for a hypoplastic right ventricular outflow tract. Secondary PV repair is an alternative to replacement (PVR), but selection criteria are not established. We sought to elucidate preoperative variables associated with successful PV repair and to compare outcomes between repair and PVR.METHODS: Patients who underwent surgery for secondary PR from 2010 to 2017 by a single surgeon were studied. The PV annulus and leaflets were measured on the preoperative echocardiogram and magnetic resonance images, and the primary predictor variable was leaflet area indexed to ideal PV annulus area (iPLA) by magnetic resonance imaging. PV repair and PVR groups were compared using multivariable logistic regression, and with a conditional inference tree. Freedom from PV dysfunction and from reintervention were assessed with Kaplan-Meier survival analyses.RESULTS: Of 85 patients, 31 (36%) underwent PV repair. By multivariable analysis, longer PV total leaflet length (cm/m2) (beta=3.00, standard error [SE]=0.82, P<.001), larger PV z score (beta=1.34, SE=0.39, P=.001), and larger iPLA (beta=8.13, SE=2.62, P=.002) were associated with repair. iPLA of 0.90 or greater was 91% sensitive and 83% specific for achieving PV repair. At a median of 4.1years follow-up, there was greater freedom from significant PR in the PV repair group (log rank P=.008).CONCLUSIONS: Patients with an iPLA >0.9, and those with an iPLA between 0.7 and 0.9 with a PV annulus z score >0 should be considered for a native PV repair. At midterm follow-up, patients with a PV repair were not more likely to developPR or to require reintervention when compared with patients undergoing PVR.

  View details for DOI 10.1016/j.jtcvs.2019.06.110

  View details for PubMedID 31585750

• Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019; 93 (4): 660–63

  View details for DOI 10.1002/ccd.28073

  View details for Web of Science ID 000461016400030

• 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

• Subcutaneous and Intravenous Treprostinil Pharmacokinetics in Children With Pulmonary Vascular Disease. Journal of cardiovascular pharmacology Hall, K. n., Ogawa, M. n., Sakarovitch, C. n., Hopper, R. K., Adamson, G. T., Hanna, B. n., Ivy, D. D., Miller-Reed, K. n., Yung, D. n., McCarthy, E. n., Siehr-Handler, S. L., Feinstein, J. A. 2019; 73 (6): 383–93

  Abstract

  This study evaluated the pharmacokinetics of intravenous (IV) and subcutaneous (SC) treprostinil in pediatric patients with pulmonary vascular disease, and compared them with existing adult data from a similar cohort. Blood samples were collected from pediatric patients receiving steady-state IV or SC treprostinil and were assessed for plasma treprostinil concentration using liquid chromatography and tandem mass spectrometry. Forty participants, 15 receiving IV and 25 receiving SC treprostinil, were included in the analysis. Age ranged from 0.1 to 15.6 years. The median dose of treprostinil was 45.5 ng·kg·min with a range of 8-146 ng·kg·min. There was a linear relationship between treprostinil dose and plasma concentration with an R of 0.57. On average, there were higher blood concentrations per given dose of IV treprostinil compared with those per given dose of SC, but the difference was not significant. Compared with adult data, the slope of the pediatric data was similar, but the y-intercept was significantly lower. Additionally, the concentration per dose ratio was significantly higher in adults compared with children. Pediatric patients have significantly lower average blood concentrations of treprostinil per given dose compared with adults, and higher, but not significantly so, blood concentrations when treprostinil is administered IV as compared with SC administration.

  View details for DOI 10.1097/FJC.0000000000000674

  View details for PubMedID 31162247

• Texting preferences in a Paediatric residency CLINICAL TEACHER Draper, L., Kuklinski, C., Ladley, A., Adamson, G., Broom, M. 2017; 14 (6): 401–6

  Abstract

  Text messaging is ubiquitous among residents, but remains an underused educational tool. Though feasibility has been demonstrated, evidence of its ability to improve standardised test scores and provide insight on resident texting preferences is lacking. The authors set out to evaluate: (1) satisfaction with a hybrid question-and-answer (Q&A) texting format; and (2) pre-/post-paediatric in-training exam (ITE) performance.A prospective study with paediatrics and internal medicine-paediatrics residents. Residents were divided into subgroups: adolescent medicine (AM) and developmental medicine (DM). Messages were derived from ITE questions and sent Monday-Friday with a 20 per cent variance in messages specific to the sub-group. Residents completed surveys gauging perceptions of the programme, and pre- and post-programme ITE scores were analysed.Forty-one residents enrolled and 32 (78%) completed a post-programme survey. Of those, 21 (66%) preferred a Q&A format with an immediate text response versus information-only texts. The percentage change in ITE scores between 2013 and 2014 was significant. Comparing subgroups, there was no significant difference between the percentage change in ITE scores. Neither group performed significantly better on either the adolescent or developmental sections of the ITE. Text messaging… remains an underused educational tool CONCLUSIONS: Overall, participants improved their ITE scores, but no improvement was seen in the targeted subgroups on the exam. Although Q&A texts are preferred by residents, further assessment is required to assess the effect on educational outcomes.

  View details for DOI 10.1111/tct.12590

  View details for Web of Science ID 000414616600005

  View details for PubMedID 28078778

• Coarctation Index Predicts Recurrent Aortic Arch Obstruction Following Surgical Repair of Coarctation of the Aorta in Infants. Pediatric cardiology Adamson, G., Karamlou, T., Moore, P., Natal-Hernandez, L., Tabbutt, S., Peyvandi, S. 2017; 38 (6): 1241-1246

  Abstract

  Recurrent aortic arch obstruction (RAAO) remains a major cause of morbidity following surgical neonatal repair of coarctation of the aorta (CoA). Elucidating predictors of RAAO can identify high-risk patients and guide postoperative management. The Coarctation index (CoA-I), defined as the ratio of the diameter of the narrowest aortic arch segment to the diameter of the descending aorta, has been used to help diagnose RAAO in neonates following the Norwood Procedure. We sought to assess the predictive value of the CoA-I on RAAO after CoA repair in infants with biventricular circulation. Clinical, surgical, and echocardiographic data of infants with biventricular circulation following neonatal CoA repair between 2010 and 2014 were evaluated. RAAO was defined using a composite quantitative outcome variable: a blood pressure gradient >20, a peak aortic arch velocity >3.5 m/s by echocardiogram, or a catheter-measured peak-to-peak gradient >20 within 2 years of surgery. Univariate and multivariate logistic regression analyses were used. Of the 68 subjects included in the analysis, 15 (22%) met criteria for RAAO. In the multivariate model, only CoA-I (OR 35.89, 95% CI 6.08-211.7, p < 0.0001) and use of patch material (OR 9.26, 95% CI 1.57-54.66, p = 0.014) were associated with increased risk of RAAO. The odds of developing RAAO was higher in patients with a CoA-I less than 0.7 (OR 33.8, 95% CI 5.7-199.5, p < 0.001). Postoperative CoA-I may be used to predict RAAO in patients with biventricular circulation after repair of CoA. Patients with a CoA-I less than 0.7 or patch aortoplasty warrant close follow-up.

  View details for DOI 10.1007/s00246-017-1651-4

  View details for PubMedID 28608147

• Text4Peds: Feasibility of an Educational Text-Messaging Program for Pediatrics Residents. Journal of graduate medical education Adamson, G. T., Draper, L. R., Broom, M. A. 2014; 6 (4): 746-749

  Abstract

  There is an ongoing effort to maximize educational material provided to residents who are in a time-constrained work environment. Mobile technology, principally smartphone applications and online modules, has shown educational promise.We developed a text-messaging program, Text4Peds, to assist residents with preparation for their pediatric board examinations. Goals were to assess (1) the feasibility of texting educational messages to residents, and (2) resident satisfaction and perceived usefulness of a texting program.We conducted a prospective study of pediatrics and combined internal medicine-pediatrics residents. Messages derived from the most missed pediatric in-training examination questions were sent daily to residents. After 3 months, residents completed surveys that gauged their perception on the educational value of the text messages and the effect on their pediatric board preparation. Feasibility of the system was assessed as a total percentage of messages successfully received by residents.Of 55 residents, 35 (64%) participated in the program. Of 2534 messages sent out to participants, 2437 (96.2%) were delivered successfully. Positive comments cited the texting of board facts as a quick, helpful, daily study tool. Residents liked that messages were sent at 2:00 pm, and most felt that 1 to 5 messages per week was appropriate. Drawbacks included character restrictions of messages, content limitations, and the lack of a question-answer format.An educational text message-based program was successfully implemented in our residency program. Messages were delivered with a high success rate, and residents found educational value in the messages.

  View details for DOI 10.4300/JGME-D-13-00469.1

  View details for PubMedID 26140130

  View details for PubMedCentralID PMC4477575

• Text Messaging in Medical Education PEDIATRICS Broom, M. A., Adamson, G. T., Draper, L. R. 2014; 133 (3): E491-E493

  View details for DOI 10.1542/peds.2013-1529

  View details for Web of Science ID 000335330700018

  View details for PubMedID 24534409