Dhananjay Malankar

Midterm outcome of primary arterial switch operation beyond six weeks of life in children with transposition of great arteries and intact ventricular septum.

Background: We have previously reported our experience in primary arterial switch operation (ASO) in children more than six weeks with transposition of great arteries and intact ventricular septum (TGA/IVS). The upper age limit for performing an ASO in these children is not yet settled and reports regarding outcome of ASO in these children are few. In this prospective observational study, we report the midterm results of children with TGA-IVS older than six weeks undergoing primary ASO. Methods: A total of 109 children aged more than 6 weeks with median age of 60 days (range 42-3,000 days), with regressed left ventricle underwent primary ASO. Extracorporeal membrane oxygenation was used in 20% (22 of 109) of them; 90.8% (99 of 109) of children who survived were prospectively followed, with a mean follow-up of 28 months (range 18-84 months). Results: Two late deaths occurred, and survival in the remainder was estimated to be 98% at seven years. The incidence of aortic regurgitation (AR) was found to have a decreasing trend with freedom from AR approaching 100% by 34 months. The left ventricular shape and function returned to normal within one to three months following surgery. None of these children had any rhythm disturbances or evidence of myocardial ischemia. Conclusions: Primary ASO can be safely performed in children with regressed ventricle, irrespective of age with encouraging results. The midterm results of these children are comparable in terms of survival and freedom from complications associated with preserved ventricle.

An electron microscopic study of left ventricular regression in children with transposition of great arteries

Over the years the age limit for the arterial switch operation (ASO) is being redefined with increasing expertise and adoption of extra-corporeal membrane oxygenator (ECMO) in the surgical program. We conducted a study to see the differences in ultrastructural features in eight children with transposition of the great arteries, four with prepared and the remaining four with regressed left ventricle (LV) during the ASO. Children with prepared LV had prominent Z bands with uniform and round mitochondria, few fat vacuoles and minimal collagen in the background, whereas children with regressed LV had Z band disruption with non-uniform elliptical mitochondria and myofibrillary disarray and an abundance of fat vacuoles and collagen in the background. Children with regressed LV and abundance of collagen had a prolonged postoperative course. Collagen deposition in the LV may point to the situation where the postoperative course following ASO may be prolonged due to the increased time required for the regressed LV to increase its mass and to sustain the systemic circulation.

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.

Unusual Left Ventricular Pseudoaneurysm in a Child After Disseminated Bacterial Infection

Left ventricular pseudoaneurysms are rare in children. A six-year-old boy developed left ventricular pseudoaneurysm despite successful control of sepsis. The pseudoaneurysm was diagnosed by two-dimensional echocardiography and detailed by computed tomography. The child underwent successful surgical correction with partial excision and plication of the aneurysmal sac.

Aortic valve replacement with biological substitutes in children.

Background: this study was performed to assess the results of aortic valve replacement in children with biological substitutes including homografts, pulmonary autografts (Ross procedure), and aortic valve reconstruction with autologous pericardium (Duran technique). Methods: between March 1992 and July 2009, 73 children with aortic valve disease (mean age, 11.8 ± 2.7 years) underwent aortic valve replacement with biological substitutes including homografts, pulmonary autografts, and aortic valve reconstruction with autologous pericardium. Associated procedures were mitral valve repair in 32 and subaortic membrane resection in 3. Results: early mortality was 1.4% (1 patient). Median follow-up was 94 months. Sixty (83.3%) survivors had insignificant aortic regurgitation. Reoperation was required in 7 (9.6%) patients: for autograft dysfunction alone in 2, autograft failure and failed mitral valve repair in 2, autograft dysfunction with severe pulmonary homograft regurgitation in 1, severe homograft aortic valve regurgitation in 1, and right ventricular outflow tract obstruction in 1. There were 4 (5.4%) late deaths. Actuarial reoperation-free, event-free, and aortic valve dysfunction-free survival were 92.5% ± 4%, 93.4% ± 3.3 %, 94% ± 2.9%, 86.2% ± 4.3%, respectively, at 94 months. Conclusions: aortic valve replacement with biological substitutes is associated with acceptable hemodynamics and midterm results.

An Alternative Technique for the Atrial Switch Operation for Transposition of the Great Arteries in an Unoperated Adult Patient

Abstract  We report a 26‐year‐old patient with d‐transposition of great arteries, intact ventricular septum, and an atrial septal defect. The relevant literature on late natural survivors with this condition is reviewed and the technical aspects of an alternative technique for accomplishing a successful atrial switch in this situation are discussed. (J Card Surg 2010;25:406‐409)

Surgical Management of Univentricular Heart with Total Anomalous Pulmonary Venous Drainage and Intrapulmonary Vertical Vein

Abstract  We report a patient with univentricular heart, heterotaxy syndrome with total anomalous pulmonary venous connection, and an intrapulmonary vertical vein draining into the left superior vena cava who underwent a successful bidirectional Glenn shunt without cardiopulmonary bypass. A polytetrafluoroethylene tube interposition graft was used between the superior vena cava and the pulmonary artery. This condition is unusual and physiologic palliation, rather than anatomic correction as described in this report, may be appropriate for managing this condition. (J Card Surg 2010;25:84‐86)

How to Do It: Repair of Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA) with Coronary Button Transfer and Lecompte Maneuver

Abstract  An alternative technique of coronary button transfer and Lecompte maneuver for anomalous left coronary artery (ALCAPA) arising from left lateral pulmonary sinus is described. This technique was used by us successfully in four patients aged 6 months to 3.5 years, weighing from 4.7 to 16 kg. The importance of trapdoor technique and Lecompte maneuver is discussed. (J Card Surg 2010;25:225‐227)

Temporary Sternotomy Wound Closure With Blood Bag

Many situations are encountered in cardiac surgery, especially paediatric cardiac surgery, which are associated with significant myocardial oedema. In such cases it is inadvisable or impossible to close the sternum primarily. Here we describe a simple and inexpensive technique of temporary sternotomy wound closure with a sterile blood bag.

Biventricular Repair in Interrupted Aortic Arch Type C With Aortic Atresia

We report a case of interrupted aortic arch type C with aortic atresia and a ventricular septal defect with two well-developed ventricles, who underwent a successful single-stage biventricular repair with the modified Yasui procedure and arch reconstruction. Angiography done during conduit revision showed bilateral brachiocephalic trunks with high branching. The child is doing well six years after the initial operation.