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.

A number of guidelines are available for the management of congenital heart diseases (CHD) 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 CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, 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 CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Key technical steps in dual-pathway repair of congenital pulmonary vein stenosis.

We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted.

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

OBJECTIVES: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS: The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS: Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results.

Transposition of the Great Arteries With Total Anomalous Pulmonary Venous Connection.

Transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is a rare association. Very few such cases have been reported. Among them 1 patient underwent anatomic repair. The rest received repair at the atrial level with either a modified Senning technique or a Mustard technique. We report a rare combination of TGA, TAPVC draining to the coronary sinus, and left juxtaposition of the atrial appendages with a diminutive right atrium. The anomalies were successfully repaired by anatomic correction.

Right Posterior Thoracotomy for Open-Heart Surgery in a Rare Morphology.

The closure of atrial septal defects through right-sided limited posterior thoracotomy has been well established in selected subsets. We present a case of large ostium secundum atrial septal defect, pulmonary valvar stenosis, absent right superior vena cava, and isolated left superior vena cava draining to right atrium via coronary sinus. The child successfully underwent total correction through limited posterior thoracotomy with necessary modifications of intraoperative steps.

An unusual fenestration in single-stage Fontan operation.

Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation. We created an unusual fenestration between left pulmonary artery and juxtaposed right atrial appendage on the left side, due to anatomic complexity. Short-term results are encouraging.

Complex aortopulmonary window in a single ventricle setting: Technical considerations for staged palliation.

We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.

Closure of muscular ventricular septal defects guided by en face reconstruction and pictorial representation.

BACKGROUND: A surface reconstruction of the location and dimensions of muscular ventricular septal defects (VSDs) on right ventricular (RV) septal surface could serve as a better guide to surgical closure amid different classifications and confusing terminologies. METHODS: We reconstructed muscular VSD requiring surgery on an en-face view of the RV septal surface from echocardiographic orthogonal views in 34 consecutive patients. The location, dimensions of the defects, and relation to various RV septal landmarks are illustrated as a diagram. Recommendations are presented regarding surgical approach to the defects, along with predictions on the possibility of residual defects and heart block. RESULTS: Surgical findings were as predicted by the diagram in the 27 patients who underwent VSD closure. Seven infants (2.5 to 4.9 kg) underwent pulmonary artery (PA) banding based on predictions of heart block or major residual defects. Two patients with predicted risk of heart block underwent VSD closure with heart block ensuing in one of them. Based on the diagram limited ventriculotomy (n = 2) or detachment of tricuspid leaflets (n = 6) aided access to the VSD. Among patients undergoing VSD closure only 1 patient had a major residual defect that required PA banding. There were clinically insignificant residual defects in 8 patients. Four patients (12%) were anticipated preoperatively because of surgical inaccessibility and intentionally left alone. CONCLUSIONS: En-face reconstruction of single or multiple muscular VSDs is feasible from orthogonal echocardiographic views. It helps plan the surgical approach and predict the likelihood of heart block and residual defects after surgery.