Resilient Hearts: Measuring Resiliency in Young People With Congenital Heart Disease.

Background Congenital heart disease (CHD) is a life-long disease with long-term consequences on physical and mental health. Patients with CHD face multifaceted physical and psychosocial challenges. Resilience is an important factor that can be protective and positively impact mental health. We studied resiliency and its associated factors in teenagers and young adults with and without CHD using a social media-delivered survey. Resilience was measured using the 25-item Connor-Davidson Resilience Scale, a validated metric with a historical mean of 80.4/100 in the general adult population. Methods and Results Individuals with and without CHD, aged 10 to 25 years, were prospectively recruited on social media to complete an online survey. The survey was completed from January to February 2022. Respondents provided information on their demographics and CHD details (where applicable) and completed the Connor-Davidson Resilience Scale. As a group, participants with CHD had higher resilience scores compared with same-aged healthy individuals (65.3±16.1 versus 55.4±13.8; P<0.001). For both cohorts, sex, race, and age were not associated with differences in resilience score. For individuals with CHD, lower resilience was associated with more hospital admissions, lack of exercise, presence of a mental health diagnosis, and no participation in support groups or disease-specific camps. Conclusions Young people with CHD had higher resilience than individuals without CHD in our sample. We identified several factors, both modifiable and nonmodifiable, that are associated with higher resilience. Awareness of resiliency and its contributors in the population with CHD may assist medical teams in improving patient physical and psychological well-being.

Isolated Absent Aortic Valves: A Unique Fetal Case With Echocardiographic, Pathologic, and Genetic Correlation.

We present a 22-week fetus with isolated absent aortic valve and inverse circular shunt. The pregnancy was interrupted. Here, echocardiography and pathology images demonstrate this rare entity. Whole genome sequencing revealed a potentially disease-causing variant in the APC gene. Whole genome sequencing should be considered in severe and rare fetal diseases. (Level of Difficulty: Advanced.).

Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants.

BACKGROUND: The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied. METHODS: The 400 enrolled participants were randomized 1:1 to udenafil or placebo. Protocol echocardiograms were obtained at baseline and 26 weeks after initiation of udenafil/placebo. Linear regression compared change from baseline indices of single ventricle systolic, diastolic and global function, atrioventricular valve regurgitation, and mean Fontan fenestration gradient in the udenafil cohort versus placebo, controlling for ventricular morphology (left ventricle versus right ventricle/other) and baseline value. RESULTS: The udenafil participants (n=191) had significantly improved between baseline and 26 weeks visits compared to placebo participants (n=195) in myocardial performance index (P=0.03, adjusted mean difference [SE] of changes between groups -0.03[0.01]), atrioventricular valve inflow peak E (P=0.009, 3.95 [1.50]), and A velocities (P=0.034, 3.46 [1.62]), and annular Doppler tissue imaging-derived peak e' velocity (P=0.008, 0.60[0.23]). There were no significant differences in change in single ventricle size, systolic function, atrioventricular valve regurgitation severity, or mean fenestration gradient. Participants with a dominant left ventricle had significantly more favorable baseline values of indices of single ventricle size and function (lower volumes and areas, E/e' ratio, systolic:diastolic time and atrioventricular valve regurgitation, and higher annular s' and e' velocity). CONCLUSIONS: FUEL participants who received udenafil demonstrated a statistically significant improvement in some global and diastolic echo indices. Although small, the changes in diastolic function suggest improvement in pulmonary venous return and/or augmented ventricular compliance, which may help explain improved exercise performance in that cohort. REGISTRATION: URL: https://clinicaltrials.gov; Unique Identifier: NCT02741115.

Amniotic Fluid microRNA in Severe Twin-Twin Transfusion Syndrome Cardiomyopathy-Identification of Differences and Predicting Demise.

Twin-twin transfusion syndrome (TTTS) is a rare but serious cause of fetal cardiomyopathy with poorly understood pathophysiology and challenging prognostication. This study sought a nonbiased, comprehensive assessment of amniotic fluid (AF) microRNAs from TTTS pregnancies and associations of these miRNAs with clinical characteristics. For the discovery cohort, AF from ten fetuses with severe TTTS cardiomyopathy were selected and compared to ten normal singleton AF. Array panels assessing 384 microRNAs were performed on the discovery cohort and controls. Using a stringent q < 0.0025, arrays identified 32 miRNAs with differential expression. Top three microRNAs were miR-99b, miR-370 and miR-375. Forty distinct TTTS subjects were selected for a validation cohort. RT-PCR targeted six differentially-expressed microRNAs in the discovery and validation cohorts. Expression differences by array were confirmed by RT-PCR with high fidelity. The ability of these miRNAs to predict clinical differences, such as cardiac findings and later demise, was evaluated on TTTS subjects. Down-regulation of miRNA-127-3p, miRNA-375-3p and miRNA-886 were associated with demise. Our results indicate AF microRNAs have potential as a diagnostic and prognostic biomarker in TTTS. The top microRNAs have previously demonstrated roles in angiogenesis, cardiomyocyte stress response and hypertrophy. Further studies of the mechanism of actions and potential targets is warranted.

Repair of Anomalous Single Coronary Artery From the Pulmonary Artery (ASCAPA).

We describe the management of an infant presenting with severe heart failure at 6 weeks of age found to have an anomalous single coronary artery originating from the main pulmonary artery (MPA). This patient was transferred to our hospital and ultimately had their coronary artery translocated to the ascending aorta successfully. Preoperative severe left ventricular (LV) dysfunction and moderate/severe mitral regurgitation (MR) improved to normal function and mild-to-moderate MR 6 weeks postrepair. Three-dimensional CT reconstructions proved valuable and allowed for accurate preoperative planning leading to successful coronary transfer.

Amniotic fluid microRNA profiles in twin-twin transfusion syndrome with and without severe recipient cardiomyopathy.

BACKGROUND: Twin-twin transfusion syndrome presents many challenges for clinicians, and the optimal means of identifying pregnancies that will benefit most from intervention is controversial. There is currently no clinically available biomarker to detect twin-twin transfusion syndrome or to stratify cases based on the risk factors. microRNAs are small RNAs that regulate gene expression and are biomarkers for various disease processes, including adult and pediatric heart failure. To date, no studies have investigated amniotic fluid microRNAs as biomarkers for disease severity, specifically for severe recipient cardiomyopathy in twin-twin transfusion syndrome cases. OBJECTIVE: This study aimed to assess whether amniotic fluid microRNAs could be useful as biomarkers to identify pregnancies at greatest risk for severe recipient cardiomyopathy associated with twin-twin transfusion syndrome. STUDY DESIGN: Amniotic fluid was collected at the time of amnioreduction or selective fetoscopic laser photocoagulation from monochorionic diamniotic twin pregnancies with twin-twin transfusion syndrome at any stage. Fetal echocardiography was performed on all twins before the procedure, and severe cardiomyopathy was defined as a right ventricular myocardial performance index of the recipient fetus of >4 Z-scores. microRNA was extracted from the amniotic fluid samples and analyzed using an array panel assessing 379 microRNAs (TaqMan Open Array, ThermoFisher). Student t tests were performed to determine significant differences in microRNA expression between pregnancies with severe recipient cardiomyopathy and those with preserved cardiac function. A stringent q value of <.0025 was used to determine differential microRNA expression. Random forest plots identified the top 3 microRNAs that separated the 2 groups, and hierarchical cluster analysis was used to determine if these microRNAs properly segregated the samples according to their clinical groups. RESULTS: A total of 14 amniotic fluid samples from pregnancies with twin-twin transfusion syndrome with severe cardiomyopathy were compared with samples from 12 twin-twin transfusion syndrome control cases with preserved cardiac function. A total of 110 microRNAs were identified in the amniotic fluid samples. Twenty microRNAs were differentially expressed, and the top 3 differentiating microRNAs were hsa-miR-200c-3p, hsa-miR-17-5p, and hsa-miR-539-5p. Hierarchical cluster analysis based on these top 3 microRNAs showed a strong ability to differentiate severe cardiomyopathy cases from controls. The top 3 microRNAs were used to investigate the sensitivity and specificity of these microRNAs to differentiate between the 2 groups with a receiver operating characteristic curve demonstrating sensitivity and specificity of 80.8%. All 20 differentially expressed microRNAs were down-regulated in the group with severe cardiomyopathy. CONCLUSION: Amniotic fluid microRNAs demonstrated differential expression between twin-twin transfusion syndrome recipient fetuses with severe cardiomyopathy and those without and have the potential to be important biomarkers of disease severity in this population.

Suspected necrotising enterocolitis after surgery for CHD: an opportunity to improve practice and outcomes.

Infants with CHD are at increased risk of necrotising enterocolitis, which can interfere with the achievement of adequate nutrition and, ultimately, growth and development. Necrotising enterocolitis is classified by severity as suspected, confirmed, and advanced. We sought to quantify the incidence of all types of necrotising enterocolitis among infants who underwent surgery, with a particular focus on suspected necrotising enterocolitis. This is a retrospective review of all infants <6 months of age who underwent cardiac surgery during 2012 and 2013 at Children's Hospital Colorado. We examined the hospital course of 265 hospitalisations (n=251 patients) and found 18 patients (19 hospitalisations) with suspected necrotising enterocolitis and 16 patients (16 hospitalisations) with confirmed or advanced necrotising enterocolitis. Single-ventricle physiology, lower weight, and younger age were associated with necrotising enterocolitis. Patients with all types of necrotising enterocolitis experienced prolonged length of hospital stay. We found suspected necrotising enterocolitis to be as common as confirmed necrotising enterocolitis, and it frequently occurred early in the post-operative course. We speculate that suspected necrotising enterocolitis may often be overlooked in research owing to a reliance on billing codes. Nevertheless, suspected necrotising enterocolitis poses a substantial barrier to post-operative progression of the CHD patient, as does confirmed necrotising enterocolitis. Following the diagnosis of all types of necrotising enterocolitis, there was wide variability in practice patterns. In response to this variability, we developed care guidelines for the diagnosis and treatment of necrotising enterocolitis in this population.

Tachyarrhythmia following Norwood operation: a single-center experience.

BACKGROUND: The purpose of this study was to characterize tachyarrhythmias in children following the Norwood procedure. METHODS: This is a single-center retrospective study including all children who underwent stage I Norwood procedure (n = 98; January 2003-September 2011). The primary outcome measure is the development of tachyarrhythmia during hospitalization after the Norwood procedure. Secondary aims include quantification of mortality in patients with tachyarrhythmias and evaluation of potential risk factors for the development of tachyarrhythmia. RESULTS: Tachyarrhythmia occurred in 33 (34%) of 98 patients. The median time to onset of tachyarrhythmia was ten days (0-47 days). Tachyarrhythmia conferred no increase in overall mortality (P = .45), including operative mortality (P = .37) or interstage mortality (P = 1.00). There was no significant difference in the incidence of arrhythmia based on demographic, anatomic, or surgical variables, including shunt type (P = .23) except that patients with tachyarrhythmias were slightly larger (median weight 3.2 kg) at the time of stage I than those without tachyarrhythmia (median weight 2.93 kg; P = .02]. The odds of arrhythmia in males were 8.7 times higher than that in females (95% confidence interval 2.9-31.3; P < .0001). CONCLUSIONS: Postoperative tachyarrhythmia is common, occurring in 34% of patients after the Norwood operation. Onset of tachyarrhythmia occurred later after the Norwood operation than reported previously, and male gender is a risk factor. Further studies to elucidate the etiology and the timing of tachyarrhythmias after the Norwood procedure are necessary.

Pulmonary artery interventions after Norwood procedure: does type or position of shunt predict need for intervention?

OBJECTIVES: Pulmonary artery stenosis is a potential complication after Norwood palliation for hypoplastic left heart syndrome. It is unclear whether the shunt type or position in the Norwood procedure is associated with the risk of the development of pulmonary artery stenosis. We examined the risk of pulmonary artery stenosis and the need for pulmonary artery intervention in children undergoing the Norwood procedure with either the right ventricle to pulmonary artery conduit or modified Blalock-Taussig shunt. METHODS: A retrospective review was performed of all patients who underwent the Norwood procedure from January 1, 2003, to September 1, 2011. The data from 100 patients were reviewed, including catheterization and echocardiographic data, right ventricle to pulmonary artery conduit (n = 67, right shunt position in 17 and left in 50), and right ventricle to pulmonary artery (n = 33). The primary outcome measure was the need for operative or catheter-based pulmonary artery intervention. RESULTS: No patients in the right ventricle to pulmonary artery group required catheterization-based pulmonary artery interventions. Surgical pulmonary arterioplasty was performed frequently and equally in both the right ventricle to pulmonary artery and right ventricle to pulmonary artery groups at the bidirectional Glenn procedure. Catheter-based pulmonary arterioplasty was performed more frequently in the right ventricle to pulmonary artery conduit group, especially when the conduit was positioned to the right side of the neoaorta. These patients had a 12.73 increased odds of a pulmonary artery intervention compared with the left to right ventricle to pulmonary artery conduit (P = .04). CONCLUSIONS: Consistent with a previous multicenter randomized trial, patients who received a right ventricle to pulmonary artery conduit versus a right ventricle to pulmonary artery have a greater risk of requiring pulmonary artery interventions. Patients with right ventricle to pulmonary artery conduit placement to the right underwent a greater number of pulmonary artery interventions but demonstrated overall improved growth of the branch pulmonary arteries compared with the patients receiving a left-sided right ventricle to pulmonary artery conduit.