Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.

COVID-19 associated renal artery stenosis in infancy - A report of two cases.

Organ-specific vasculitis is an uncommon, delayed complication of COVID-19 infection. It is usually seen in mildly symptomatic or asymptomatic patients. Underlying endothelitis is the most likely pathophysiological mechanism for such a manifestation. We report two infants with renal artery stenosis, most likely consequent to COVID-19 infection.

Successful balloon dilatation of an infolded polytetrafluoroethylene graft: An unusual cause of early Fontan failure.

Despite optimal medical management, an 8-year-old boy had persistent pleural drainage following total cavopulmonary connection. Detailed evaluation, including computed tomography angiography, confirmed obstruction at the lower end of the circuit due to infolding of the polytetrafluoroethylene graft. Balloon dilation of the obstruction resulted in prompt resolution of pleural effusion with sustained relief at 1-year follow-up. The case demonstrates the importance of careful assessment in the diagnosis and successful nonsurgical management of an unusual cause of obstruction in the Fontan circuit.

Neurodevelopmental outcomes in children with cyanotic congenital heart disease following open heart surgery.

Background: Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim: We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods: In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental "delay" and "no delay" groups. Results: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental "delay" and "no delay" groups (P = 0.04). Conclusion: Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Efficacy and safety of propranolol in infants with heart failure due to moderate-to-large ventricular septal defect (VSD-PHF study) - A prospective randomized trial.

AIMS: The utility of beta-blocker therapy in infants with heart failure (HF) due to significant left-to-right shunt lesions is not known. The study aimed to assess the efficacy and safety of propranolol in infants with HF due to moderate-to-large ventricular septal defect (VSD). METHODS: The prospective randomized trial included 80 infants with HF and moderate-to-large VSD, randomly allocated to receive either conventional therapy alone (n = 40) or propranolol plus conventional therapy (n = 40). The primary endpoint was a composite of all-cause mortality, hospitalization for HF and/or chest infection, and referral for surgery. The secondary clinical outcomes were the individual components of the composite endpoint. In addition, the patients were followed up to detect safety outcomes, for example, bronchospasm, bradyarrhythmia, and worsening HF symptoms. RESULTS: The addition of propranolol therapy to the conventional medications did not result in significant improvement in the primary composite endpoint (32.50% vs. 52.50%; P = 0.07). There was a trend toward improvement, but the study is underpowered for this important question. However, propranolol therapy significantly decreased the risk of hospitalization (12.50% vs. 32.50%; P = 0.03) and worsening of Ross HF class (5.41% vs. 28.21%; P = 0.01) as compared to conventional therapy (estimated number needed to treat = 5). Propranolol did not result in any significant safety concerns in these infants except bronchospasm in an infant. CONCLUSIONS: Propranolol therapy in infants with significant left-to-right shunt may prevent worsening in HF symptoms and hospitalization and is well tolerated. However, it does not reduce mortality or need for surgery.

Dextroversion due to giant left atrium in a child.

Giant left atrium is extremely rare in pediatric population. We hereby report a case of 4-year-old child with giant left atrium (LA) due to "non-rheumatic" mitral regurgitation (MR). The giant LA caused dextro-rotation of the heart, which immediately reverted to normal cardiac position after surgical repair. The case is reported for the unusual manifestation of giant LA as dextroversion.

A unique arrangement of "kissing" atrial appendages.

We report a case of a 2-month-old boy with tricuspid and pulmonary atresia with a unique configuration of "kissing" atrial appendages. The case highlights the importance of computed tomography angiography in identifying such anomalies associated with complex congenital heart defects.

"Dual Drainage" in the mixed variety of totally anomalous pulmonary venous connection.

We report a case of 6-week-old boy with a mixed variety of totally anomalous pulmonary venous connection where the common channel had a dual drainage into the coronary sinus and left brachiocephalic vein respectively. The case also highlights the role of preoperative computed tomography angiography in demonstrating the exact pulmonary venous anatomy, course and identifying obstruction, when present.

Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.

AIM: To evaluate the cardiovascular, tracheobronchial and pulmonary abnormalities associated with tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) on multidetector computed tomograpgy (MDCT) angiography. MATERIALS AND METHODS: We conducted a retrospective review of our electronic database from January, 2014 to December, 2020 to identify pediatric patients diagnosed with TOF-APVS on MDCT angiography. Pulmonary anatomy, associated tracheobronchial and coronary compression, compression of intrapulmonary bronchi, lung parenchymal changes and other associated cardiovascular abnormalities were evaluated. RESULTS: Forty-seven patients with TOF-APVS were included in the study. Compression of the airways was observed at multiple levels; 4 (8.5%) patients had carinal compression while right main bronchus and left main bronchus compression was seen in 12 (25.5%) and 16 (34.0%) patients, respectively. Forty-three (91.5%) patients showed abnormal pulmonary arterial branching at the segmental level and compression at the level of intrapulmonary bronchi was seen in 24 (51.1%) patients. Some degree of air trapping was seen in 35 (74.5%) patients whereas atelectasis was observed in 15 (31.9%) patients. Mild coronary arterial compression was seen in 3 (6.4%) patients. CONCLUSION: TOF-APVS is characterized by aneurysmal dilatation of pulmonary arteries leading to a varying degree of airway compression. However, even in the absence of compression of the trachea and main bronchi, there can be distal bronchial compression secondary to abnormal pulmonary arterial branching resulting in lung abnormalities in the majority of these patients. This study highlights the importance of dedicated airway assessment in addition to cardiovascular morphological assessment by MDCT angiography, before planning surgery.

Anomalies of coronary arteries in tetralogy of Fallot: Evaluation on multidetector CT angiography using dual-source scanner.

OBJECTIVES: The present study sought to determine the prevalence and evaluate the patterns of various anomalies of coronary arteries in patients with tetralogy of Fallot (TOF) on multidetector CT angiography using a dual-source CT scanner. METHODS: We retrospectively reviewed CT angiographies of 955 pediatric patients, diagnosed with TOF between 1st January 2015 and 31st December 2019 and having an optimal evaluation of coronary arteries, with respect to the origin, course, and termination of the coronary arteries and associated cardiovascular anomalies. RESULTS: Anomalies of coronary arteries were observed in 8.27% (79/955) patients with TOF. Origin of accessory/proper left anterior descending (LAD) artery from right coronary artery (RCA) or right coronary sinus was the most common anomalous pattern, seen in 3.14% (30/955) patients, followed by the presence of hypertrophied conal artery seen in 2.62% (25/955) patients. The anomalous coronary artery was crossing the right ventricular outflow tract (RVOT) in all cases where the LAD artery was arising from RCA or right coronary sinus or where RCA was arising from the LAD artery. Coronary arterial anomalies were seen in a significantly higher proportion of patients with an atrial septal defect compared to those without (7/25 [28%] vs. 72/930 [7.74%]; p = .0003). CONCLUSION: The prevalence of anomalies of coronary arteries increases significantly in the presence of TOF as compared to the general population. Majority of these anomalous coronary arteries course anterior to the RVOT which can potentially pose technical difficulties with a risk of adverse surgical outcomes and increased morbidity and mortality, rendering their diagnosis crucial before surgery.

Thrombus in transit after balloon atrial septostomy.

Deterioration following a successful balloon atrial septostomy (BAS) is not common. We report an unusual cause for the same. A thrombus in transit has not been previously reported following BAS.

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography.

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.

Isolated systemic arterial supply to normal lung - an unusual cause of extracardiac left-to-right shunt.

Isolated systemic arterial supply to a normal lung, a type of bronchopulmonary vascular malformation, is a rare cause of extracardiac left-to-right shunt. We describe such a case that was successfully managed by transcatheter closure of the anomalous arterial supply to otherwise normal lung.

Ruptured sinus of valsalva presenting late following repair of tetralogy of fallot.

Rupture of sinus of valsalva following repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient.

Thoracoscopic Left Cardiac Sympathetic Denervation in a Child with Refractory Long QT Syndrome.

Long QT syndrome is a cardiac disorder which presents with recurrent syncopal attacks and has risk of sudden cardiac death. A 5-year-old boy presented to us with this syndrome. The child was symptomatic despite medical management and was successfully managed with cardiac denervation. The current report highlights the efficacy and safety of the use of video-assisted thoracoscopic surgery for this procedure.