Vikas Kohli

Pentalogy of Cantrell with left ventricular diverticulum: a case report and review of literature.

Pentalogy of Cantrell is a rare congenital anomaly involving deficiency of the following structures: anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and associated congenital intracardiac abnormality. We describe a 3-month-old child with this syndrome having left ventricular diverticulum along with omphalocele who presented to us with a pulsating mass in the epigastrium. The defect was evaluated and defined by computed tomography scan. A team of pediatric, cardiac, and plastic surgeons successfully repaired the defects. This case report discusses the review of literature along with management options and concludes that there must be an emphasis on early repair of left ventricular diverticulum to prevent complications. Antenatal ultrasound can also detect the anomaly, and early postnatal diagnosis of the syndrome, followed by immediate surgical repair, can prevent lethal complications.

Surgical reconstruction for absence of sternum and pericardium in a newborn

We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.

Posttransplant Complex Inferior Venacava Balloon Dilatation After Hepatic Vein Stenting

Orthotopic and living related liver transplantation is an established mode of treatment of end-stage liver disease. One of the major causes of postoperative complications is vascular anastomotic stenosis. One such set of such complications relates to hepatic vein, inferior vena cava (IVC), or portal vein stenosis, with a reported incidence of 1–3%. The incidence of vascular complications is reported to be higher in living donor versus cadaveric liver transplants. We encountered a patient with hepatic venous outflow tract obstruction, where the hepatic vein had been previously stented, but the patient continued to have symptoms due to additional IVC obstruction. The patient required double-balloon dilatation of the IVC simultaneously from the internal jugular vein and IVC.

Neonatal cardiac interventions: an Indian perspective.

Palliative interventions in newborn have been performed ever since balloon atrial septostomy was started. The scope and potential of these interventions have been extended to therapeutic interventions and also to replace palliative surgeries. The advancements have happened over the last two decades mainly due to the advancements in cardiac anesthesia of the newborn and primarily due to available hardware improving. The outcomes of these interventions have also improved significantly. The advantage of neonatal interventions is especially relevant in developing countries where limited resources, cost of procedure (lower with reused consumables), lower morbidity due to shorter ICU stay work in favor of the patient. Not only have newborn interventions become well established in centers specializing in pediatric cardiac care, but also premature babies requiring interventions have become successful. This article focuses on the spectrum of neonatal interventions in a contemporary pediatric cardiac centre in India.

Post-op Systemic Hypertensive Crises in an Infant with Total Anomalous Pulmonary Venous Connection

Systemic hypertension is not usually a complication following repair of total anomalous pulmonary venous connection (TAPVC). We report an infant with supracardiac TAPVC with hypertensive crises post-operatively resulting in pulmonary edema. We feel this might have been related to the pre-operative hemodynamics as described. Beta-blockers improved the hypertensive crises.

Homozygous familial hypercholesterolaemia in two boys aged 5 and 10 years.

Familial hypercholesterolaemia (FH) is an autosomal dominant lipid disorder. Homozygous FH (HFH), though rare, presents in early childhood. Two different presentations of HFH are reported. The first child presented at 5 years of age with xanthomas on the knees, elbows and buttocks and failure to thrive since the second year of life. He was found to be hypertensive with moderate aortic regurgitation. He is now stable on statins and antihypertensives. The second child presented at 10 years of age with multiple xanthomas and severe aortic stenosis. He died of refractory cardiac failure despite emergency aortic balloon valvoplasty due to diffuse coronary artery disease. Strong clinical suspicion can aid early diagnosis and delay cardiovascular complications.

Tulip Deformity with Cera Atrial Septal Defect Devices: A Report of 3 Cases

Device closure of secundum atrial septal defect (ASD) is the treatment of choice when anatomy is favourable. Amplatzer device has remained the gold standard for closure of ASD. Cobra deformity is a well-reported problem with devices. Recently, Tulip deformity has been reported in a single case. We report a series of cases where we noted Tulip deformity along with inability to retract the device in the sheath in Cera Lifetech devices. This resulted in prolongation of procedure, excessive fluoroscopic exposure and additional interventional procedures not usually anticipated in ASD device closure. We believe that the problem is due to the stiffness of the device resulting in its inability to be retracted into the sheath. We also report a unique way of retrieving the device.