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

ustification: 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 whenrecommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and mayhave co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National ConsensusMeeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of MedicalSciences, 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 heartdiseases; (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 congenitalheart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductusarteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heartdiseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebsteinanomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Transposition of great arteries with aortopulmonary window: an unusual cause of prepared left ventricle at 11 months.

In patients with transposition of great arteries, presence of aortopulmonary window is very uncommon and associated with high morbidity and mortality. This report describes the case of an 11-month-old female patient in which aortopulmonary window was restrictive, and protected the patient from developing pulmonary vascular disease. The patient underwent successful arterial switch and repair of aortopulmonary window.

Total anomalous pulmonary venous connection: helical computed tomography as an alternative to angiography.

BACKGROUND: Echocardiographic evaluation of the pulmonary veins is inadequate at times. Cardiac catheterization, especially in sick neonates, may be a high-risk procedure. Helical computed tomography with three-dimensional reconstruction is noninvasive but remains an underutilized modality. METHODS AND RESULTS: Between January 2002 and February 2003, 7 computed tomography scans of children 3 weeks to 5 years of age were performed to evaluate the drainage of the pulmonary veins in suspected total anomalous venous drainage. Helical computed tomography (GE High speed Advantage) was performed using 2 mm sections, and rapid bolus hand injections of 2 ml/kg body-weight of nonionic intravenous contrast. Sagittal and coronal reformats, and three-dimensional reconstructions were performed, and reviewed by the radiologist. The findings were discussed with the pediatric cardiologist and surgeon involved in the case. The diagnoses included complex congenital heart disease (n = 5), isolated infradiaphragmatic total anomalous pulmonary venous connections (n = 1), and transposition of the great arteries with total anomalous pulmonary venous connections (n = 1). Cardiac computed tomography accurately demonstrated infradiaphragmatic total anomalous pulmonary venous connections in 4, and supracardiac drainage in 3 patients, in addition to the other cardiac findings. The findings on computed tomography scan correlated with surgical (n = 5) and/or angiographic findings (n = 2) in 7 patients. CONCLUSIONS: In sick, high-risk patients, cardiac computed tomography can be considered as an alternative to cardiac catheterization for the evaluation of pulmonary venous drainage.