Background: Despite advances, infants with single ventricle heart disease continue to have high morbidity and mortality in the first year of life. Home monitoring programs (HMPs) have reduced mortality and have grown to use integrative digital platforms. The objective was to evaluate how implementation of a digital HMP platform affects nutritional outcomes in infants undergoing staged single ventricle palliation. Methods: We conducted a retrospective, multicenter, observational study of all infants who required a neonatal operation as part of staged single ventricle palliation between 2013 and 2018. Patients were excluded if less than 35 weeks’ gestation or underwent biventricular repair in the first year of life. Implementation of a digital monitoring platform that allows for secure monitoring of nasogastric feed advancement and oxygen saturation occurred in 2016, creating the two groups in a similar surgical era. Results: There were 38 patients who fell under a standard HMP compared to 31 utilizing the digital platform. There was no difference in baseline demographics, anatomy, or preoperative factors between the groups. Use of a digital platform was associated with reduced postoperative length of stay (30.1 vs 33.1 days, P = .04). More children in the digital platform monitoring group were able to achieve oral feeding at one year of age (90% vs 68%, P = .03). A total of 25% of infants went home with a nasogastric tube, all but one transitioning to full oral feeds. Conclusions: Use of a digital, fully electronic medical record (EMR)-integrated, comprehensive HMP was associated with shorter postoperative length of stay in neonates undergoing staged single ventricle palliation and allowed for higher rates of full oral feeding.

Objectives Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction. Methods Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], orgothic [angular]), indexed neoaortic dimensions, and distensibility. Comparisons were made with 50 single-ventricle controls without prior arch reconstruction. Factors associated with ascending neoaortic dilation, reduced distensibility, and decreased ventricular function on the 14-month echocardiogram were evaluated using univariate and multivariable logistic regression. Results Interpretable angiograms were available for 326 of 389 subjects (84%). Compared with controls, study subjects more often demonstrated abnormal arch geometry (67% vs 22%, P < .01) and had increased ascending neoaortic dilation (Z score 3.8 ± 2.2 vs 2.6 ± 2.0, P < .01) and reduced distensibility index (2.2 ±1.9 vs 8.0 ± 3.8, P < .01). Adjusted odds of neoaortic dilation were increased in subjects with gothic arch geometry (odds ratio [OR], 3.2 vs crenel geometry, P < .01) and a right ventricle-pulmonary artery shunt (OR, 3.4 vs Blalock–Taussig shunt, P < .01) but were decreased in subjects with aortic atresia (OR, 0.7 vs stenosis, P < .01) and those with recoarctation (OR, 0.3 vs no recoarctation, P = .04). No demographic, anatomic, or surgical factors predicted reduced distensibility. Neither dilation nor distensibility predicted reduced right ventricular function. Conclusions After Norwood surgery, the reconstructed neoaorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these features.

Pediatric single ventricle patients have seen dramatic improvements in overall outcomes over the past several decades. This is attributed to the development of home monitoring programs for interstage patients. In today’s current COVID-19 pandemic, the use of telemedicine has allowed providers to care for these patients and support their families effectively while minimizing the risk of COVID-19 exposure. Our single-center study reviewed the charts of nine patients followed by our single ventricle team through the COVID-19 pandemic. Patients discharged from the hospital and enrolled in our digital home monitoring program were included. Records were retrospectively reviewed for total number of outpatient visits, adverse events, unplanned hospital readmissions, and unplanned procedures. These results were then compared to outcomes from 2018 to 2019. In-person visits averaged every 6 weeks compared to every 2–3-week pre-pandemic. Zero adverse events reported with the use of telemedicine compared to one adverse event pre-pandemic. There was a 50% decrease in unplanned readmissions and 60% decrease in unplanned procedures during our study period. One patient was diagnosed with acute COVID-19 infection and managed conservatively via telemedicine with full recovery. To our knowledge, this is the only case–control study reporting the use of telemedicine during the COVID-19 pandemic in the interstage population. Although not statistically significant, we report a decrease in total adverse events, unplanned procedures, and unplanned admissions. Telemedicine visits allowed for identification of issues requiring hospital readmission as well as conservative management of one patient with COVID-19.

Background Gerbode defect is a congenital or acquired communication between the left ventricle and right atrium. While the defect is becoming a more well-recognized complication of cardiac surgery, it presents a diagnostic and therapeutic challenge for providers. This case highlights the predisposing factors and imaging features that may assist in the diagnosis of Gerbode defect, as well as potential approaches to treatment. Case summary We report a patient with severe mitral stenosis as a result of remote mediastinal radiation who underwent extensive decalcification during surgical mitral valve replacement and tricuspid valve repair. Following the procedure, he developed progressive heart failure refractory to medical management. Extensive workup ultimately led to the diagnosis of iatrogenic acquired Gerbode defect. Close collaboration between adult cardiology, cardiothoracic surgery, and the congenital cardiology services led to an optimal treatment plan involving percutaneous closure of the defect. Discussion Gerbode defect is a rare complication of invasive procedures involving the interventricular septum or its nearby structures. An understanding of the key echocardiographic features will aid providers in timely diagnosis. Percutaneous repair should be strongly considered for patients who may be poor surgical candidates.

Objectives Abnormal early angiogenesis appears to impact both placental disorders and fetal congenital heart defects (CHD). We sought to assess the association of placental perfusion defects (PPD) and fetal (CHD). Methods Singleton pregnancies with isolated severe fetal CHD were compared to controls without congenital anomalies or maternal malperfusion (MVM) risk factors. CHD was categorized into group 1: single left ventricle morphology and transposition of the great vessels (TGA) and group 2: single right ventricle and two ventricle morphology. Malperfusion was defined as fetal vascular malperfusion (FVM), MVM, and both FVM and MVM. Results PPD was increased for all CHD (n = 47), CHD with or without risk factors, and CHD groups compared to controls (n = 92). Overall CHD cases and CHD with risk factors had an increased risk of FVM (30% and 80% vs 14%), and MVM (43% and 50% vs 21%), respectively. MVM rates were similar in CHD with and without maternal risk factors. FVM (38% vs 14%) and MVM (44% vs 21%) were increased in Group 1. MVM (42% vs 21%) and both FVM and MVM (16% vs 3%) were increased in Group 2. Conclusions PPD risk is increased in severe isolated fetal CHD. The highest risk is seen in fetal CHD with maternal risk factors.

encountered at earlier gestational ages than in the third trimester or at term, although a substantial proportion occurred after 32 weeks gestation (Figure 1). CONCLUSION: While it is known that women who have had previous stillbirths are at increased risk of subsequent stillbirths, we identified no specific modifiable risk factors for recurrent stillbirth in our cohort. Almost 20% of the recurrent SB occurred after 37 weeks, reflecting an opportunity for improvement.

Objectives: Aortic arch reconstruction in patients with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe potentially maladaptive aortic anatomy and physiology, risk factors, and impact on right ventricle (RV) function in patients with single RV lesions after Norwood. Methods: Pre-stage II angiograms for subjects from the Single Ventricle Reconstruction (SVR) Trial were compared to 50 single left ventricle controls with no prior arch reconstruction. Arch geometry was defined as normal, crenel (elongated), or gothic (angular). Aortic index (ascending /descending aorta diameter) and distensibility index (systolic aortic area - diastolic area) / (diastolic area x pulse pressure) were calculated. Univariable tests were used for comparisons, and Spearman’s rho was used for correlation. Results: Interpretable angiograms were available for 326/389 (84%) SVR subjects. Table 1 summarizes findings. Age at catheterization was similar for SVR subjects and controls. SVR subjects more often demonstrated abnormally elongated (crenel) arch geometry with dilated ascending and transverse aortae tapering to a small isthmus. Distensibility was reduced in both the ascending and descending aorta. Risk factors for ascending aortic dilation (aortic index ≥ 2.5) included native aortic stenosis (OR = 2.2 [95% CI: 1.2, 3.8] vs. aortic atresia) and receipt of a RV-pulmonary artery shunt (OR = 2.6 [1.47, 4.5] vs. Blalock-Taussig shunt). There was no association between reduced distensibility and any demographic, anatomic, or reported surgical factors. Aortic dilation and reduced distensibility did not correlate with RV function at 14-month echocardiogram (p=0.5). Conclusions: After Norwood single RV surgery, the reconstructed aorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these findings.

Introduction: Disparities in healthcare outcomes are well described among patients of different races and ethnicities including pediatric cardiology. Multicenter studies examining these outcomes are lacking in pediatric acute care cardiology. We hypothesize that Black and Hispanic patients admitted to pediatric acute care cardiology units have increased hospital and acute care encounter length of stay (LOS) and complication rates compared to their White and non-Hispanic peers. Methods: Utilizing the Pediatric Acute Care Cardiology Collaborative registry, we examined all acute care cardiology unit encounters from 2/1/2019 to 7/30/2021 ending in discharge to home or death. Hospitalizations were categorized by race and ethnicity. In-hospital complications included health-care acquired infections, iatrogenic incidents, pneumonia, sepsis, seizures and stroke. Data were analyzed for differences in LOS and complication rates using chi-square and ANOVA testing. We used Bonferroni correction to establish a significance threshold of 0.007. Results: Analysis included 30,404 hospitalizations from 29 centers. There were 16,233 White (70%), 4,533 Black (19%), 919 Asian (4%) and 1,629 other races (7%) encounters. There were 23,592 (78%) non-Hispanic and 4,583 (15%) Hispanic encounters. Black patients had higher rates of premature birth (21.4%) and low birth weight (10.7%), compared to White patients (15.6% and 5.9% respectively, p<0.0001). Both non-Hispanic Black and Hispanic patients had longer total hospital and acute care LOS than non-Hispanic White patients. Complication rates analyzed by race trended towards significance between Black and White patients, and Hispanic patients had a higher complication rate than non-Hispanics. ( Table 1 ) Conclusions: Despite improved outcomes for patients with congenital and acquired heart disease, significant racial and ethnic disparities continue to exist. Directed efforts are needed to achieve equitable results.