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The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT 02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO2), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO2, work rate at the ventilatory anaerobic threshold (VAT), VO2 at VAT, and ventilatory efficiency (VE/VCO2) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO2, baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation. Trial Registration NCT0274115. [ABSTRACT FROM AUTHOR]

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005–2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < − 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63–1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93–4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36–3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI. [ABSTRACT FROM AUTHOR]

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock–Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p < 0.001). [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) can lead to progressive heart failure with high morbidity and mortality. Cardiac catheterization (CC) is the gold standard for diagnosis and response to vasodilatory medications. The invasive nature of CC and associated anesthesia predispose this patient population to adverse events including death. Catheterization records were queried from 1/1/2011 to 10/31/2016. Patients with PH, defined as pulmonary vascular resistance (PVR) greater than 3 WU m2, pulmonary artery pressure above 20 mmHg, and pulmonary wedge pressure less than or equal to 15 mmHg, who underwent hemodynamic CC were included in this retrospective study. Both patients with and without congenital heart disease were included. There were 198 CC in 191 patients. Adverse events (n = 28, 14.1%) included cardiac arrest, increased respiratory support requiring ICU care, PH crisis, bradycardia/hypotension requiring intervention, and arrhythmias. Odds of an adverse event increased by 22% for every 15-min increase in procedure times (OR 1.22, CI 1.01–1.39, p = 0.002) and were significantly increased for procedures longer than 80 min (OR 3.75, CI 1.56–9.00, p = 0.007) (Fig. 1). Patients with an adverse event had higher mean pulmonary artery pressures while breathing oxygen (43 [35–58] versus 34 [27–44] mmHg, p = 0.017) and oxygen with inhaled nitric oxide (37 [32–56] versus 32 [25–40] mmHg, p = 0.026). Females carried more risk than males (OR 3.88, CI 1.44–10.40, p = 0.007). Younger age, medication regimens, prematurity, and genetic disease did not carry an increased risk. Adverse events are common in pediatric patients with PH undergoing CC. The risk of adverse events correlates with greater procedure times and higher mean pulmonary artery pressure. Minimizing procedure time may improve patient outcomes. [ABSTRACT FROM AUTHOR]

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (− 0.74 [− 1.75, − 0.26] vs. − 1.95 [− 2.91, − 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible. [ABSTRACT FROM AUTHOR]

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included. Using expert consensus, 40 unique procedure types were defined by diagnostic characteristics or the intervention(s) performed, and dose area product (DAP) per kilogram of body weight (µGy × m2/kg) was summarized. Using empiric and consensus methods, three radiation risk categories were created. A total of 11,735 cases were included for analysis. Thirteen (n = 7918) procedure types with median DAP/kg < 100 were categorized in the low radiation exposure category (median DAP/kg 39). The medium exposure category (n = 1807) consisted of 16 procedure types with median DAP/kg values ranging 100 to < 200 (overall median DAP/kg 131). Finally, the high radiation exposure category (n = 1073) consisted of 11 procedure types with median DAP/kg ≥ 200 (overall median DAP/kg of 231). The radiation exposure risk categories created in this multi-center dataset are a critical step towards the development of a robust risk adjustment methodology for radiation exposure in catheterization for congenital heart disease. [ABSTRACT FROM AUTHOR]

In isolated unilateral ductal origin of a pulmonary artery (DOPA), intervention to establish reperfusion of the affected lung without direct re-anastomosis may lead to pulmonary hypertension (PH) in the contralateral lung. Multicenter retrospective review of patients with unilateral DOPA, who underwent palliation with a ductal stent (DS) or Blalock-Taussig (BT) shunt with subsequent development of PH in the contralateral lung, was conducted. Ten patients (4 females; median weight 3.2 kg, range 2.1-5.2) who underwent DS or BT shunt and developed contralateral PH were identified. Eight infants had right-sided DOPA. Stents/shunt used had a median diameter of 3.5 mm (range 2.5-5.2). After DS in 9 patients, 7 patients were exclusively treated with medical therapies, whereas 2 patients received intermediate procedures: one received an additional contralateral DS and other underwent surgical banding of the DS prior to PA reimplantation. Seven patients who underwent DS and one patient with BT shunt underwent PA reimplantation at median of 3.3 (0.6-18) months. PA pressure was documented to be normal in 5 patients immediately following PA reimplantation, 1 year later in 2 patients, and 1 patient is on Tadalafil with elevated PVR of 5.5 indexed Wood units. One patient died and one patient is awaiting surgery with normal PA pressure. We describe the development of severe contralateral PH following DS or BT shunt as the initial intervention for unilateral DOPA. Pulmonary hypertension resolved in 7/8 patients who underwent surgical PA reimplantation. The cause of PH in the normally connected lung in these cases remains unclear. [ABSTRACT FROM AUTHOR]

To evaluate the association between initial management strategy of neonatal symptomatic Tetralogy of Fallot (sTOF) and later health-related quality of life (HRQOL) outcomes. We performed a multicenter, cross-sectional evaluation of a previously assembled cohort of infants with sTOF who underwent initial intervention at ≤ 30 days of age, between 2005 and 2017. Eligible patients’ parents/guardians completed an age-appropriate Pediatric Quality of Life Inventory, a Pediatric Quality of Life Inventory Cardiac Module Heart Disease Symptoms Scale, and a parental survey. The association between treatment strategy and HRQOL was evaluated, and the entire sTOF cohort was compared to published values for the healthy pediatric population and to children with complex congenital heart disease and other chronic illness. The study cohort included 143 sTOF subjects, of which 59 underwent a primary repair, and 84 had a staged repair approach. There was no association between initial management strategy and lower HRQOL. For the entire cohort, in general, individual domain scores decreased as age sequentially increased. Across domain measurements, mean scores for the sTOF cohort were significantly lower than the healthy pediatric population and comparable to those with other forms of complex CHD and other chronic health conditions. The presence of a genetic syndrome was significantly associated with a poor HRQOL (p = 0.003). Initial treatment strategy for sTOF was not associated with differences in late HRQOL outcomes, though the overall HRQOL in this sTOF cohort was significantly lower than the general population, and comparable to others with chronic illness. [ABSTRACT FROM AUTHOR]