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.

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.

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.

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.

"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.

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.

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.

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.

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.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.

Pericardiectomy in children <15 years of age.

Between January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.

Retrospective study of results of Kawashima procedure.

BACKGROUND: Patients with single ventricle physiology and an interrupted inferior caval vein undergo the Kawashima procedure. With increasing follow-up, pulmonary arteriovenous malformations may develop requiring hepatocardiac venous redirection. METHODS: Between January 2005 and December 2012, 15 patients underwent the Kawashima procedure. Preoperative and postoperative characteristics along with functional class, intra-operative details, post-operative course, saturations and haematocrit were recorded. RESULTS: Median age at operation was five years (range 1- 20 years). Five patients had antegrade pulmonary blood flow interrupted and 10 patients had open antegrade flow. There were no deaths. Mean saturation in postoperative period was 90.8 ±1.36%. Median duration of mechanical ventilation and inotropic support was six and 16hours respectively. Median duration of pleural drainage was six days. Median duration of pleural drainage in antegrade flow open and interrupted group was six days and four days respectively. The mean hospital stay was 10.5 days ±7.1 days. Mean follow up was 40.2 ±31 months (median-30 months). Nine patients (60%) were in New York Heart Association (NYHA) functional class 1 and five patients (33.3%) were in NYHA functional class 2 at last follow-up. Only one patient was in class 3 and needs completion Fontan. CONCLUSIONS: The Kawashima procedure can be safely performed with acceptable early results. Although studies have shown the risk of pulmonary arteriovenous malformations after Kawashima procedure in the mid and long-term, our findings do not support this. Kawashima procedure with open antegrade pulmonary blood flow as a definite intervention in such patients is debatable.

Chronic Thromboembolic Pulmonary Artery Hypertension or Fibrosing Mediastinitis?

We report a case of pulmonary hypertension in association with right heart thrombus and mediastinal lymphadenopathy leading to the diagnostic dilemma of chronic thromboembolic pulmonary hypertension vs fibrosing mediastinitis despite of extensive noninvasive workup, considering different treatment strategies and therapeutic implications. Surgical findings provided a conclusive diagnosis and excellent prognosis.

Role of systemic to pulmonary artery shunt after cavopulmonary anastomosis.

Superior cavopulmonary anastomosis and total cavopulmonary anastomosis are the procedures of choice for the management of patients with a functionally univentricular heart. We review the various indications, sites, advantages, and complications of a systemic to pulmonary artery shunt after the creation of superior cavopulmonary anastomosis. Systemic pulmonary artery shunt may be useful as a palliative strategy in patients who have hypoxemia and completion of total cavopulmonary anastomosis is not feasible.

Heart disease in pregnancy: cardiac and obstetric outcomes.

BACKGROUND: A retrospective analysis of the records of all the patients of heart disease with pregnancy at AIIMS, New Delhi, India, to find out the maternal and fetal outcome. MATERIALS AND METHODS: A retrospective analysis was carried out of 100 pregnancies in women with heart disease who delivered at ≥28 weeks of gestation from July 2009 through August 2012. RESULTS: Cardiac disease was found to complicate 3.8 % of pregnancies. Rheumatic heart disease (n = 64, 64 %) was the predominant cardiac disease. Congenital heart disease was found to complicate 36 pregnancies (n = 36, 36 %).Cardiac complications were seen in 32 (32 %) and fetal complications in 18 (18 %) pregnancies. Fewer cardiac and postpartum complications were present in NYHA class I/II patients compared to NYHA III/IV patients (P < 0.05). Pregnancy outcome was better in rheumatic heart disease patients who had undergone cardiac intervention prior to pregnancy (n = 29, 45.2 %) compared to those whose heart disease remained uncorrected (n = 35, 54.8 %) but the difference was not statistically significant. There was one maternal mortality in a patient with Eisenmenger syndrome. Two of the newborns of the 17 women who had received anticoagulants had features of warfarin embryopathy. CONCLUSION: Pregnancy in women in NYHA class III/IV is associated with significantly higher maternal morbidity and cardiac interventions before pregnancy, when indicated may improve pregnancy outcome.

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.

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.

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.