In the era of outpatient ventricular assist devices, is it time to reconsider the practice of bridging older children to transplant on outpatient inotropes?

OBJECTIVE: Ventricular assist devices (VADs) and inotropes are feasible modalities to bridge children to heart transplant (HT) in outpatient settings. However, it is unclear which modality yields superior clinical status at HT and posttransplant survival. METHODS: The United Network for Organ Sharing was used to identify patients aged 18 years or younger, weighing >25 kg, from 2012 to 2022 who were outpatients at HT (n = 835). Patients were grouped by bridging modality at HT: VAD (n = 235 [28%]), inotropes (n = 176 [21%]), or neither (no support) (n = 424 [50%]). RESULTS: VAD patients were of similar age (P = .260) but heavier (P = .007) and more likely to have dilated cardiomyopathy (P < .001) than their inotrope counterparts. VAD patients had similar clinical status at HT but superior functional status (performance scale >70%) (59% vs 31%) (P < .001). Overall posttransplant survival in VAD patients (1-year and 5-year survival, 97% and 88%, respectively) was comparable to patients with no support (93% and 87%, respectively) (P = .090) and those on inotropes (98% and 83%, respectively) (P = .089). One-year conditional survival was superior for VAD vs inotrope (2-year and 6-year survival, 96% and 91%, respectively vs 97% and 79%, respectively) (P = .030) and 5-year conditional survival for VAD patients was superior to inotrope (7-year and 10-year survival, 100% and 100%, respectively vs 100% and 88%, respectively) (P = .022) and no support (100% and 83%, respectively) (P = .011). CONCLUSIONS: Consistent with prior studies, short-term outcomes for pediatric patients bridged to HT in the outpatient setting with VAD or inotropes is excellent. However, compared with outpatients bridged to HT on inotropes, outpatient VAD support allowed for better functional status at HT and superior late posttransplant survival.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia.

BACKGROUND: We sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. METHODS: This retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. RESULTS: Of 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. CONCLUSIONS: For infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.

Changes in circumferential strain can differentiate pediatric heart transplant recipients with and without graft rejection.

BACKGROUND: Routine surveillance protocols rely heavily on endomyocardial biopsy (EMB) for detection of rejection in pediatric heart transplant recipients. More sensitive echocardiographic tools to assess rejection may help limit the number of EMBs. This study compared changes in left ventricular (LV) strain in patients who had rejection versus those who did not. METHODS: A single center retrospective review was conducted between 2013 and 2020. Patients were categorized based on rejection history. Echocardiograms were evaluated at the time of 2 consecutive EMBs; in the rejection group, the second echocardiogram was collected at the time of a rejection episode. Conventional measures of LV function and speckle-tracking echocardiography-derived longitudinal (LS) and circumferential strain (CS) were measured. RESULTS: 17 patients were in the non-rejection group and 17 were in the rejection group (30 total rejection episodes). The rejection group was older at the time of transplant (12.5 vs. 1.3 years, p = .01). A decline in CS was seen in the rejection group at the second echocardiogram [-18.5 (IQR -21.5, -14.6) to -15.7 (IQR -19.8, -13.2)] while CS improved in the non-rejection group [-20.8 (IQR -23.9, -17.8) to -23.9 (IQR -24.9, -20.1)]. This difference in change reached significance (p = .02). A similar pattern was seen in LS that neared significance (p = .06). There was no significant difference in ejection fraction change (p = .24). CONCLUSIONS: Patients in the non-rejection group displayed improvement in CS between echocardiograms while patients in the rejection group showed subsequent decline. Worsening of LV CS may help identify acute rejection in the early post-transplant period.

Two Brothers With Dextro-Transposition of the Great Arteries.

Dextro-transposition of the great arteries (d-TGA) is a common cause of cyanotic heart disease in neonates. Current thought is d-TGA is a sporadic occurrence in families with an unclear etiology. We describe a case of brothers with d-TGA. Genetic testing revealed that both are heterozygous for two gene variations that are associated with congenital heart disease.