Faculty Bibliography

CONTEXT/UNASSIGNED:Cardiac injury has been described in both acute COVID-19 and the multisystem inflammatory syndrome in children (MIS-C). Echocardiographic strain has been shown to be a sensitive measure of systolic function. AIMS/UNASSIGNED:We sought to describe strain findings in both the groups on initial presentation and follow-up. SETTINGS AND DESIGN/UNASSIGNED:A retrospective study analyzing echocardiograms of all patients presenting with acute COVID-19 infection and MIS-C at our institution between March 2020 and December 2020 was performed. SUBJECTS AND METHODS/UNASSIGNED:TOMTEC software was used for strain analysis in both the study groups (COVID-19 and MIS-C) and age-matched healthy controls. Strain was correlated with LV ejection fraction (EF) and serum troponin levels. RESULTS/UNASSIGNED:= 0.002) and troponin in patients with MIS-C. Abnormal strain persisted in one-third of patients in the MIS-C and acute COVID-19 groups on outpatient follow-up. CONCLUSIONS/UNASSIGNED:Patients with MIS-C and acute COVID-19 can develop myocardial dysfunction as seen by abnormal strain. LV longitudinal strain correlates with cardiac injury as measured by serum troponin in patients with MIS-C. Strain may provide an additional tool in detecting subtle myocardial dysfunction. It can be routinely employed at diagnosis and at follow-up evaluation of these patients.

A 33-week gestation, 1.75-kg female infant with mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome and severe ventriculo-coronary connections underwent surgical septectomy and bilateral pulmonary artery banding at five weeks of age (2.10 kg). After separation from bypass, she developed hemodynamic instability requiring venoarterial extracorporeal membrane oxygenation support. She was listed for heart transplantation and transplanted after three days of support with an oversized heart (4.7:1 donor-recipient weight ratio).

BACKGROUND:Coronary allograft vasculopathy (CAV) is a leading cause of mortality after heart transplantation (HT) in children. Variation in CAV screening practices may impact detection rates and patient outcomes. METHODS:Among 50 Pediatric Heart Transplant Society (PHTS) sites from 2001 to 2016, coronary evaluations were classified as angiography or non-invasive testing, and angiograms were designated as routine or symptom based. CAV detection rates stratified by routine vs symptom-based angiograms were calculated. Freedom from CAV and mortality after CAV diagnosis, stratified by study indication, were calculated. RESULTS:A total of 3,442 children had 13,768 coronary evaluations; of these, 97% (n = 13,012) were for routine surveillance, and only 3% (n = 333) were for cause. Over the study period, CAV was detected in 472 patients (14%). Whereas 58% (n = 29) of PHTS sites evaluate by angiography alone, 42% reported supplementing with a non-invasive test, although only 423 non-invasive studies were reported. Angiographic detection of CAV was higher for symptom-based testing than for routine testing (29% vs 4%, p < 0.0001), although routine testing identified a majority of cases (88%; n = 414). The 10-year freedom from CAV was 77% overall. Once CAV is detected, 5-year graft survival was 58%, with lower survival for patients diagnosed after symptoms angiogram than after routine angiogram (30% vs 62%; p < 0.0001). CONCLUSIONS:Development of a robust model for CAV risk should allow low-risk patients to undergo less frequent invasive angiography without adverse impact on CAV detection rates or outcomes.

BACKGROUND:The overall severity of cardiac disease secondary to acute SARS-CoV-2 (severe acute respiratory syndrome coronavirus-2) infection in children appears to be much lower when compared with adults. However, the newly described multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) has been associated with cardiac complications. METHODS:We reviewed the clinical course and cardiac testing results in pediatric patients hospitalized with MIS-C at 2 large hospital systems in the New York City metropolitan area over a 3-month period. RESULTS:Of the 33 patients (median age 2.8 years) in the study cohort, 24 (73%) had at least one abnormality in cardiac testing: abnormal electrocardiogram (48%), elevated brain natriuretic peptide (43%), abnormal echocardiogram (30%), and/or elevated troponin (21%). Electrocardiogram and echocardiogram abnormalities all resolved by the 2-week outpatient follow-up cardiology visit. CONCLUSION/CONCLUSIONS:While 73% of pediatric patients with MIS-C had evidence of abnormal cardiac testing on hospital admission in our study, all cardiac testing was normal by outpatient hospital discharge follow-up. Cardiac screening tests should be performed in all patients diagnosed with MIS-C given the high rate of abnormal cardiac findings in our study cohort.

A 15-year-old presented in cardiogenic shock secondary to viral myocarditis requiring venoarterial extracorporeal membrane oxygenation (ECMO) support. He developed large thrombi of the left ventricle and aortic root. Anticoagulation was increased, and medications were initiated to decrease the likelihood of aortic valve opening. He underwent balloon atrial septostomy followed by placement of a left atrial vent. A pigtail catheter was placed in the ascending aorta for direct heparin infusion. Serial echocardiograms showed progressive resolution of the thrombi. He was successfully weaned from ECMO and discharged home without neurological deficits.

BACKGROUND:Rejection with severe hemodynamic compromise (RSHC) carries a mortality risk approaching 50%. We aimed to identify current risk factors for RSHC and predictors of graft failure after RSHC. METHODS:Data from 3,259 heart transplant (HT) recipients between January 2005 and December 2015 in the Pediatric Heart Transplant Study (PHTS) were analyzed. Predictors for RSHC and outcome after RSHC were sought. Time to RSHC was analyzed using the Cox proportional hazards regression model. Cardiac allograft vasculopathy (CAV) after HT and CAV after RSHC were analyzed as time-dependent covariates. Timing of RSHC was analyzed as occurring before and after 4 years after RSHC. RESULTS:There were 309 patients (9.5%) with ≥ 1 RSHC episodes. In 143 patients with RSHC, the first episode was within 1 year after HT. Independent risk factors for RSHC were age 1 to 5 years at HT (hazard ratio [HR], 1.51; 95% confidence interval [CI], 1.04-2.18), age > 10 years at HT (HR, 1.83; 95% CI, 1.29-2.60), black race (HR, 1.64; 95% CI, 1.25-2.15), prior cardiac surgery (HR, 1.55; 95% CI, 1.03-2.31), ventricular assist device support at HT (HR, 1.65; 95% CI, 1.18-2.29), maintenance steroids (HR, 1.39; 95% CI, 1.06-1.82), and recipient on inotropes, pressors, or thyroid hormones (HR, 1.45; 95% CI, 1.09-1.94). Graft survival at 5 years after RSHC was 45.7%. RSHC was a greater risk factor for earlier CAV (HR, 7.78; 95% CI, 5.82-10.40) than other rejection types (HR, 2.31; 95% CI, 1.79-3.00). Patients with late RSHC, after 1 year after RSHC had increased risk of graft loss 4 years after RSHC (HR, 7.12; 95% CI, 2.18-23.22). The 5-year graft survival after RSHC was 50.5% for early RSHC and 39.0% for late RSHC. CONCLUSIONS:Mortality after RSHC is high in the current treatment era. Many patient risk factors for RSHC cannot be modified, including age, race, prior cardiac surgery, and ventricular assist device support. After RSHC, CAV is the only predictor of graft failure. Patients who have late RSHC fare worse than those who have RSHC within the first year after HT.

BACKGROUND:Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. OBJECTIVES/OBJECTIVE:This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. METHODS:Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. RESULTS:Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. CONCLUSIONS:Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391).

BACKGROUND:Historically, patients with a prior Fontan procedure for complex congenital heart disease (CHD) have been considered at higher risk for death after heart transplant (HT) compared with other HT transplant candidates. With the overall trend of improved survival of pediatric HT recipients, it is unclear of Fontan patient post-HT survival has also improved in the current era. METHODS:Data from the Pediatric Heart Transplant Study database for Fontan patients who underwent HT was compared between the early era (1993 to 2006, n = 150) and late era (2007 to 2014, n = 252). Post-HT survival and pre-HT characteristics were compared among eras and also with non-Fontan CHD patients. RESULTS:At time of HT, Fontan patients in the late era were more likely to require inotropic support, have protein-losing enteropathy, have failure to thrive, and be further from time of Fontan, although less likely to be on ventilator support. Only ventilator support and earlier year of HT were significant risk factors for death in the multivariate analysis. Post-HT Fontan patient survival significantly improved from the early to late era (p = 0.02), particularly in the early phase, with 1-year survival of 77% in the early era and 89% in the late era. Late era non-Fontan CHD patient 1-year post-HT survival was similar to Fontan patients at 92%. CONCLUSIONS:Survival of Fontan patients after HT has significantly improved in the current era. Currently, expected post-HT survival for Fontan patients is on par with other CHD patients. Fontan patients should not be excluded from consideration for HT solely on a history of Fontan.

BACKGROUND:Previous studies in adults have suggested that donor dopamine treatment may improve recipient outcomes in organ transplantation; in this analysis, we aimed to determine if donor dopamine reduces the incidence of postoperative right heart failure (RHF) in pediatric heart transplant recipients. METHODS:Data for recipients aged 18 years or younger transplanted at our institution between January 1, 2000, and June 15, 2011, and their respective donors were obtained. The presence of postoperative RHF was assessed for in all subjects. Donor dopamine dose was stratified into 3 groups: none, low-dose (≤5 μg/kg per minute), and high-dose (>5 μg/kg per minute). Logistic regression was used to assess the relationship between donor dopamine dose and recipient RHF. RESULTS:Of 192 recipients, 34 (18%) experienced postoperative RHF. There was no difference in baseline demographics between recipients with and without RHF. When controlling for pulmonary vascular resistance index, graft ischemic time, and cardiopulmonary bypass time, donor low-dose dopamine was independently associated with a decreased risk of RHF (odds ratio, 0.16; 95% confidence interval, 0.04-0.70; P = 0.02); however high-dose dopamine was neither associated with, nor protective of, RHF (odds ratio, 0.31; 95% confidence interval, 0.06-1.6; P = 0.16). CONCLUSIONS:Despite advances in perioperative care of the recipient, RHF persists as a complication of pediatric heart transplantation. In this study, donor pretreatment with low-dose dopamine is associated with a decreased risk of postoperative RHF in pediatric heart recipients. Further studies into this association may be useful in determining the utility of empiric donor pretreatment with low-dose dopamine.