Krishna Subramony Iyer

Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals

Fifty-eight consecutive patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries with arborization defects, and major aortopulmonary collaterals were entered into a program for staged surgical repair between January 1979 and July 1989. Prerepair preparatory procedures were designed to (1) encourage native pulmonary artery growth by increasing blood flow and (2) unifocalize pulmonary blood supply by transplanting or ligating major collaterals. A total of 121 staging procedures were performed with an overall mortality of 10.3%. One hundred thirty-four major collaterals were either ligated or transplanted. Thirty patients eventually underwent hemodynamic repair with an early mortality of 3.3% and late mortality of 10.0%. Twenty-six current survivors of repair remain clinically well after a mean follow-up of 3.6 years. Ten patients are currently in various stages of preparation. Twelve patients (20.7%) failed to achieve minimum requirements for repair after staging and await further palliation or heart-lung transplantation. The principles of management have evolved over the years and are discussed.

Infant ECMO cannulation technique allowing preservation of carotid and jugular vessels

A technique is presented for cannulation of the cervical vessels for extracorporeal membrane oxygenation. Reconstruction of the vessels can be accomplished after decannulation. Doppler ultrasound confirms patency after decannulation in most cases.

Use of azygos vein as interposition graft for surgical unifocalization of pulmonary blood supply

Hemodynamic repair in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries with arborization defects, and major aortopulmonary collaterals necessitates prior unifocalization of pulmonary artery blood supply. When direct anastomosis between these collateral vessels and the central pulmonary arteries is not technically feasible, an interposition graft is required. Dacron, polytetrafluoroethylene, and pericardium have been used for this purpose. We describe our experience with the use of azygos vein as an interposition graft in 3 patients who underwent unifocalization of the pulmonary blood supply to the right lung. Our experience suggests that the azygos vein is a useful graft for use in this specific form of unifocalization procedure.

Rosai-Dorfman Disease of Right Atrium Mimicking Myxoma

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare multisystemic disorder that was first reported by Rosai and Dorfman in 1969. It is a distinct histioproliferative disorder due to overproduction of histiocytes, which accumulate in lymph nodes. The cardiac involvement of this disease is extremely rare, and until now, only 18 cases have been reported. We report the case of a 53-year-old woman with right atrial mass mimicking myxoma, which the histopathologic evaluation revealed to be Rosai-Dorfman disease of the right atrium.

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.

Double-orifice tricuspid valve in an infant with partial atrioventricular septal defect: a case report

A 1-year-old female child, weighing 7.7 kg was admitted to the hospital with history of low birth weight, failure to thrive and recurrent lower respiratory tract infections. On physical examination, there was alopecia, large pointed ears, dysmorphism, ectodermal dyplasia, and motor developmental delay. On auscultation, grade 3/6 early systolic murmur was audible at left upper sternal border. Transthoracic echocardiography showed situs solitus levocardia, concordant atrioventricular-arterial system, partial atrioventricular septal defect (AVSD) characterized by a large ostium primum atrial septal defect (ASD) with left-to-right shunt, cleft anterior mitral leaflet, and trivial mitral regurgitation. There was a tiny left ventricular to right atrium (LV–RA) shunt, pulmonary valvular stenosis, no tricuspid regurgitation, and normal ventricular function. Under general anaesthesia and standard monitoring, aortic and bicaval cannulations were done after systemic heparinisation and cardiopulmonary bypass (CPB) was established. Under moderate hypothermic circulatory arrest, the right atrium was opened. Surgical findings were a large ostium primum ASD, cleft anterior mitral leaflet, and a LV–RA shunt on saline testing. The tricuspid valve had two distinct orifices, the smaller of the two lying posteriorly close to the coronary sinus (Fig. 1). Both components of the tricuspid valve were competent on saline testing. Attempted closure of the mitral cleft resulted in valve stenosis so the cleft was left as such since there was no regurgitation. The ASD was closed with an untreated autologous pericardial patch. Right ventricular outflow tract took Hegar’s dilator size 11, which was appropriate for the age and body surface area, and so pulmonary valvotomy was not required. The accessory valve was left untreated as there was no regurgitation on testing with saline. Patient was weaned off CPB smoothly. Post-CPB epicardial 2D echocardiography showed ASD patch in situ with no residual shunt, no mitral stenosis/regurgitation, no tricuspid regurgitation, and normal ventricular function (Fig. 2)

Dual Right Coronary Arteries in Transposition of Great Arteries

A 37-day-old full-term male neonate weighing 3.5 kg presented with mild tachypnea and cyanosis observed since birth. The boy was observed to have transposition of great arteries (TGA) with malpositioned great vessels. He had a nonrestricted perimembranous ventricular septal defect (VSD) and an additional small mid-muscular VSD. The boy had a 2.3-mm PDA that shunted left to right. His coronary profile showed a large branch arising from the left facing sinus, giving rise to the left anterior descending artery and the right coronary artery (RCA) [1, 4, 5]. This RCA supplied the anterior aspect of the right ventricular free wall (Figs. 1, 2). An additional coronary artery, which traveled in the right atrioventricular groove (smaller than the RCA from the left facing sinus), supplied the remainder of the right ventricular aspect (Fig. 3). The left circumflex artery originated from the RCA arising from right facing sinus (Fig. 4). The boy underwent successful coronary artery translocation, VSD closure, and the Lecomptes maneuver with an arterial switch operation. The coronary artery anatomy was confirmed during surgery with successful translocation of the coronary buttons.

Arterial Switch Operation With Neoaortic Valve Replacement in a 13-Year Old Patient With Transposition of Great Arteries With Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction—A Case Report

The surgical management of d-transposition of great arteries (d-TGAs) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is ever evolving and still remains a challenge because of wide anatomic variability, age of presentation, surgical options available, and their variable long-term results in different series. We describe a patient with d-TGA, VSD, and LVOTO who presented to us at 13 years of age and underwent an arterial switch operation along with neoaortic valve replacement with a mechanical prosthesis. The postoperative course was uneventful, and at hospital discharge, the echocardiogram was satisfactory. We present the pros and cons of this hitherto undescribed treatment option.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot—Four Cases of a Rare Association

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.