Neeraj Awasthy

Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated

We present three unusual cases of total anomalous systemic venous drainage (TASVD) where all the systemic veins were abnormally connected to the atrium that drained into the left ventricle (LV). All three patients had features consistent with left atrial isomerism. Based on the available evidence and review of literature we propose that TASVD be included as a part of the syndrome of left atrial isomerism. A classification for TASVD is also proposed with surgical implications.

Nonsurgical Management of a Congenital Aortocaval Fistula from Right Subclavian Artery to Superior Vena Cava Along with SVC Obstruction

Congenital arteriovenous malformations between the subclavian artery and the superior vena cava (SVC) is a very rare anomaly scantily described in the literature. We describe this anomaly in a 4-month-old infant who presented with congestive heart failure. He was diagnosed to have a right subclavian artery to SVC fistula associated with SVC stenosis .The child successfully underwent device (vascular plug) closure of the fistula and SVC stenting. Six months postprocedure, child is doing well, with complete resolution of symptoms.

Submitral aneurysm: An antenatal diagnosis

We describe the echocardiographic features of submitral aneurysm in a 26 week old fetus. The diagnosis was confirmed on neonatal echocardiography.

Occult anomalous origin of the left coronary artery from the pulmonary artery with ventricular septal defect

Manifestations of anomalous left coronary artery from the pulmonary trunk may be masked in the presence of an associated shunt lesion that prevents fall of pulmonary artery pressures and allows perfusion of the anomalous coronary artery. We present such a patient with a large ventricular septal defect associated with the anomalous coronary artery from the pulmonary artery.

Long-term results of percutaneous balloon valvuloplasty of congenital aortic stenosis in adolescents and young adults

Balloon aortic valvuloplasty (BAV) is a well accepted modality of treatment in congenital aortic stenosis in all age groups. Although in infants and children it is the modality of choice, in adolescents and young adults, it is of debatable efficacy. Aim To evaluate long-term results of aortic valvuloplasty particularly in adolescent and adults (>12 years) and compare the outcome in other age groups that are <1 year and between 1 are 11 years. Setting Tertiary referral center. Patients 165 consecutive patients treated at the median age of 9 years (1 day to 64 years). The follow-up was up to 14 years (median 3 years). The whole cohort was divided into 3 age-based subgroups: Group A (<1 year) n = 45, Group B (1 year–11 years) n = 52, and Group C (>12 years) n = 68. The characteristics of each subgroup were mutually compared. Intervention Percutaneous balloon valvuloplasty with mean (SD) balloon to annulus ratio of 0.93. Main outcome measures were repeat BAV, significant aortic regurgitation (AR), and aortic valve replacement/repair. Results The incidence of significant AR from the whole cohort was 9.9% (8% moderate, 1.9% severe); n = 16. Group A = significant AR 9.5% (7.1% moderate, 2.4% severe), Group B = significant AR 11.3% (9.4% moderate, 1.9% severe), and Group C = significant AR 9% (7.5% moderate, 1.5% severe); p value = 0.99 (Group C vs Group A) and 0.92 (Group C vs Group B). Repeat BAV rate was 13.3% (n = 22 out of 165 patients). Group A – n = 5 (11.9%), Group B – n = 10 (18.2%), and Group C – n = 7 (10.3%). p Value = 0.78 (C vs A) and 0.19 (C vs B). Surgery in follow-up was needed in n = 4 (2.4%), none in Group A, 2 patients in Group B (3.6%), and 2 patients in group C (2.9%). Patients were followed up for a period of 14 years; Group A = up to 8 years, Group B = up to 13 years, and Group C = up to 14 years. Mean survival probability after the procedure was 8 years (Group A = 6.5 years, Group B = 8.1 years, and Group C = 9.9 years), and p value = 0.49 (A vs B), 0.23 (B vs C), and 0.4 (A vs C). Conclusion There is no statistical difference in the long-term outcome in the adults and adolescents as compared to the children; thus BAV remains an obvious treatment of choice with good long-term outcome.

Lutembacher Syndrome With Unroofed Left Superior Vena Cava: A Diagnostic Dilemma

Lutembacher syndrome involving the association of congenital atrial septal defect (ASD), usually of the ostium secundum variety, and mitral valve disease is a well-known entity. Its association with a coronary sinus, ASD, and a persistent left superior vena cava (LSVC) draining into the left atrium (LA) (Raghib syndrome) is rarely described in the literature. This association in a 15-year-old boy erroneously deemed to be inoperable before referral to the authors’ hospital due to cyanosis in the presence of atrial septal defect (ASD) and mitral stenosis is described in this report. Evaluation by echocardiography followed by cine angiography confirmed the cause of cyanosis to be drainage of the LSVC into the LA together with an ASD and rheumatic mitral stenosis, a combination of Raghib and Lutembacher syndromes. The boy underwent successful surgical correction. The authors believe this is the second such case to be reported in the English literature and the first of its kind to be managed by surgical intervention.

Off-pump midline repair of coarctation of aorta when associated with intracardiac defects: an alternate approach for single-stage correction.

OBJECTIVES Strategies for the optimal surgical management of coarctation of aorta with associated intracardiac defects continue to be debated upon. We describe a previously unreported surgical technique for the management of this combination of defects and present our early results. METHODS Thirty-one patients (median age: 73 days and median weight: 3.9 kg) underwent repair of coarctation of aorta with associated cardiac defects, in a single-stage, through a midline sternotomy. Twenty-eight patients had associated distal arch hypoplasia and 1 had a type I interruption. The coarctation and when necessary, the distal arch, was repaired prior to the institution of cardiopulmonary bypass. Cardiopulmonary bypass was subsequently instituted for the repair of the associated intracardiac defects. RESULTS All patients had successful repair of coarctation and distal arch hypoplasia without the need for rescue cardiopulmonary bypass. In addition, 29 patients had closure of one or more ventricular septal defects, 3 patients had an arterial switch procedure and 1 had repair of double outlet right ventricle. Repair of supramitral ring and subaortic membrane was performed in another 3 patients. One patient underwent concomitant mitral valve repair. There was one in-hospital mortality (3.22%) due to fulminant sepsis. The median follow-up was 29.5 months (range: 1-58 months). There was no late mortality. Two patients required balloon dilatation for recurrent coarctation. Overall event-free survival rate was 88.5% at both 2 and 3 years of follow-up after surgery. However, intervention-free survival at 2 and 3 years of follow-up was 91.7% among the survivors. CONCLUSION Repair of coarctation and distal arch hypoplasia or type I arch interruption is feasible prior to the institution of cardiopulmonary bypass without the use of hypothermic circulatory arrest or regional cerebral perfusion, with acceptable and reproducible results.

Endotracheal tube as tracheal stent in vascular ring.

Management of vascular ring is always a matter of great concern. There is limited literature on management of carbon dioxide retention in mechanically ventilated patients with vascular rings due to double aortic arch, while awaiting corrective cardiac surgery. A 29-day-old girl with complete duplicated double aortic arch, left descending aorta, and a left patent ductus arteriosus, had severe upper respiratory tract obstruction with carbon dioxide retention. She was managed successfully by endotracheal tube manipulation.

Double orifice tricuspid valve in an infant with partial atrioventricular septal defect

Double orifice Tricuspid Valve is an extremely rare malformation with few case reports. We report such a case in an infant associated with atrioventricular septal defect.

Giant coronary cameral fistula with coarctation of aorta in a neonate.

A coronary cameral fistula (CCF) involves a sizable communication between a coronary artery and a cardiac chamber. We present a case of giant coronary cameral fistula associated with coarctation of aorta in a 13 days old neonate.