The heterotaxy syndrome: associated congenital heart defects and management.

Heterotaxy syndrome (HS) constitutes a spectrum of anomalies arising from embryological errors that result in abnormalities of lateralization involving thoraco-abdominal viscera and culminate in loss of normal asymmetric arrangement of these organs. Besides the unique challenges involved in planning and execution of surgical procedures aimed at correction or palliation of these anomalies, they have the potential to cause profound physiological and immunological consequences in the individual patient due to their cardiac and extra-cardiac manifestations. This article aims to review the literature on this rare and extraordinary subset of developmental anomalies with the intention of familiarizing the reader on the modes of presentation, manifestations, and the variations thereof while dealing with this anomaly. In our institutional experience with HS, 75 consecutive patients were seen between January 2011 and September 2018. Of these, 48 (64%) were confirmed to have isomerism of right atrial appendages (IRAA) and the rest had isomerism of left atrial appendages (ILAA). The cardiac and extra-cardiac manifestations of these patients were listed out. Fifty-four patients (34 with IRAA and 20 with ILAA) underwent 83 surgical procedures. While 49 patients were palliated on the univentricular pathway, 5 underwent biventricular repair. The in-hospital mortality was 7 (13%) in both groups combined (5 for patients with IRAA and 2 for ILAA). In conclusion, the surgical management of HS is associated with satisfactory outcomes in current era.

Tetralogy of Fallot with subarterial ventricular septal defect: Surgical outcome in the current era.

BACKGROUND: Tetralogy of Fallot (TOF) with subarterial ventricular septal defect (VSD) is more common among Asians than Caucasians. Compared with the regular subaortic VSD postoperative right ventricular outflow obstruction is more common because of the sub-pulmonary extension of the defect. The objective of this study is to analyze the surgical implications and outcomes of patients with TOF - subarterial VSD in the current era. PATIENTS AND METHODS: In all, 539 consecutive operated patients with TOF from May 2005 to September 2012 were retrospectively reviewed. Eighty-five patients had subarterial VSD. Seventy-nine of these underwent intracardiac repair. Preoperative clinical, echocardiographic features, operative and postoperative variables were assessed. RESULTS: The median age at surgery was 6 years and the median weight was 14 kilograms. The male to female ratio was 1.7:1. TOF with subarterial VSD was associated with frequent use of transannular patch (74.6%). The early mortality was 2.5%. Follow up was 92% complete with a mean duration of 20 months with actuarial survival of 97.3% at 5 years. Two patients required reoperation for significant right ventricular outflow tract obstruction (RVOTO) at one year and three years, respectively. CONCLUSIONS: Intra cardiac repair for TOF with subarterial VSD has low perioperative mortality and morbidity. Transannular patch augmentation of the right ventricular outflow tract (RVOT) is required in a significant proportion of these patients. Precise suturing of the VSD patch, adequate infundibular resection and lower threshold for a transannular patch placement ensures a smooth early postoperative recovery.

Transcatheter closure of multiple perimembranous ventricular septal defects with septal aneurysm using two overlapping Amplatzer Duct Occluders II.

Ventricular septal defect (VSD) is a common congenital heart defect in children. Perimembranous VSD (Pm VSD), a defect involving the membranous septum and the adjacent portion of the muscular septum, accounts for about 70 % of cases. Transcatheter closure of Pm VSDs using the Amplatzer Pm VSD device, although successful in many patients, has been associated with major adverse events. Complete heart block, thromboembolism, and new-onset valvular regurgitations in patients undergoing VSD closure using the Pm VSD occluder have been reported [3]. Recently, a new retrograde approach for transcatheter closure of Pm VSDs using the Amplatzer Duct Occluder II (ADO II) has been described with a 90 % success rate [1]. This report describes a child who had multiple Pm VSDs with septal aneurysm undergoing successful transcatheter closure using two ADO II occluders with relative ease.

Percutaneous closure of a large unligated vertical vein using the Amplatzer Vascular Plug II after supracardiac total anomalous pulmonary venous connection (TAPVC) repair.

It is well known that the vertical vein (VV) may have to be left open after repair of a total anomalous pulmonary venous connection (TAPVC) in children with preoperative obstruction, whose left heart chambers are small (Cope et al. in Ann Thorac Surg 64:23-29, 3). An unligated VV has been found to reduce pulmonary arterial pressure, decrease perioperative pulmonary hypertensive crisis, and provide better hemodynamics postoperatively (Chowdhry et al. in J Thorac Cardiovasc Surg 133:1286-1294, 2). Although these VVs are expected to close later, they may remain patent in about half of these children (Cheung et al. J Paediatr Child Health 41:361-364, 1).The patent VVs may be a cause for significant left-to-right shunting, and the children may be symptomatic. The case report describes a child who had a large patent VV after repair of supracardiac TAPVC and its closure using the Amplatzer Vascular Plug II device.

Left aortic sinus to right atrial fistula--percutaneous closure in a septuagenarian female.

A 71 year-old female presented with a large left aortic sinus to right atrial fistula causing severe congestive heart failure and atrial fibrillation. Successful percutaneous closure of the defect was achieved using a 20/18 duct occluder with dramatic improvement in symptoms.

Isolation of the left subclavian artery-origin from the left pulmonary artery by way of ductus arteriosus: multidetector row computed tomographic angiographic imaging.

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. It has been reported to occur with conotruncal anomalies and may be associated with 22q11 deletion. Multidetector-row computed tomographic angiogram images of a 15-year-old African boy with Tetralogy of Fallot who had right aortic arch and isolated left subclavian artery arising from the left pulmonary artery by way of ductus arteriosus are presented.