Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

Emergency balloon dilation or stenting of critical coarctation of aorta in newborns and infants: An effective interim palliation.

BACKGROUND: Management of native uncomplicated coarctation in neonates remains controversial with current evidence favoring surgery. The logistics of organizing surgical repair at short notice in sick infants with critical coarctation can be challenging. METHODS AND RESULTS: We reviewed data of 10 infants (mean age of 2.9 +/-1.6 weeks) who underwent catheter intervention for severe coarctation and left ventricular (LV) dysfunction between July 2003 and August 2007. Additional cardiac lesions were present in 7. Mean systolic gradient declined from 51+/-12 mm Hg to 8.7+/-6.7 mm Hg after dilation. The coarctation segment was stented in five patients. Procedural success was achieved in all patients with no mortality. Complications included brief cardiopulmonary arrest (n =1), sepsis (n = 1) and temporary pulse loss (n = 2). LV dysfunction improved in all patients. Average ICU stay was 5+/-3.4 days and hospital stay was 6.5+/-3.4 days. On follow-up (14.1+/-10.5 months), all developed restenosis after median period of 12 weeks (range four to 28 weeks). Three (two with stents) underwent elective coarctation repair, two underwent ventricular septal defect (VSD) closure and coarctation repair and one underwent pulmonary artery (PA) banding. Two patients who developed restenosis on follow-up were advised surgery, but did not report. Two (one with stent) underwent redilatation and are being followed with no significant residual gradients. CONCLUSION: Balloon dilation +/- stenting is an effective interim palliation for infants and newborns with critical coarctation and LV dysfunction. Restenosis is inevitable and requires to be addressed.

Congenital coronary artery anomalies: diagnosis with 64 slice multidetector row computed tomography coronary angiography: a single-centre study.

Retrospective review of the congenital coronary artery (CA) anomalies detected by a 64-slice multidetector row computed tomographic (MDCT) angiography. The type of the anomaly, imaging characteristics, clinical relevance and the superiority of the MDCT over conventional coronary angiography are discussed. Multidetector row computed tomographic coronary angiography was carried out by the usual technique with 70 cc of non-ionic contrast agent and retrospective electrocardiogram gating. The volume data obtained were reconstructed in axial plane, along with volume-rendered three-dimensional reconstruction and virtual angioscopy in selected patients. The images were analysed by a radiologist, experienced in cardiac CT, and an experienced cardiologist. A retrospective review of the records was carried out, and subjects with congenital coronary anomalies were included in the study. Between 15 November 2005 and 27 February 2007, 1495 MDCT coronary angiograms were carried out. Eleven of them were found to have coronary anomalies. Five had absent left main CA. Two had interarterial course of the left main CA artery passing in between the right ventricular outflow tract and the root of aorta. In one patient, there was aberrant origin of right CA from the left aortic sinus with subsequent interarterial course and another one had aberrant origin of circumflex artery from the right aortic sinus. One patient each of congenitally absent circumflex artery and atresia of the right CA were found. Sixty-four slice MDCT coronary angiography provided accurate depiction of anomalous vessel origin and course along with the complex anatomical relation with the adjacent structures. CT can be considered as a first-line imaging method for delineating coronary arterial anomalies.

Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot.

OBJECTIVES: To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF). BACKGROUND: Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers. BT shunt carries the risk of branch pulmonary artery distortion and shunt occlusion. METHODS: Infants less than three months with a significant valvar pulmonary stenosis (with or without associated infundibular and annular component) and oxygen saturation

Device closure of residual ventricular septal defect after repair of tetralogy of fallot using the amplatzer duct occluder.

Residual ventricular septal defect after surgical repair for tetralogy of Fallot can occasionally be hemodynamically important requiring re-intervention. Transcatheter closure using ventricular septal defect devices is an attractive option for such defects. We describe two such cases where the Amplatzer duct occluder was used as an innovative, less costly alternative for closure of residual membranous ventricular septal defects. Complete occlusion of the residual ventricular septal defect with significant symptomatic improvement could be accomplished in both patients.

Successful management of isolated ventricular inversion in an adult.

We report a case of isolated ventricular inversion in a 42-year-old woman. This rare congenital cardiac anomaly was corrected by an intraatrial baffle procedure. She also underwent left-sided double-chamber endocardial pacemaker implantation for postoperative tachycardia bradycardia syndrome.