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.

Percutaneous Transluminal Coronary Angioplasty Following Kawasaki Disease.

Kawasaki disease (KD) can result in coronary artery disease in the form of ectasia, aneurysm and stenosis. The final complication can be myocardial infarction. We report a child who presented with severe left ventricular dysfunction following KD and was detected on angiography to have total left anterior descending artery occlusion. Angioplasty was done which resulted in improvement in the flow. Follow up angiography a year later showed recurrence of total occlusion.

GastrointentinaI bleed with clopidogrel and aspirin.

Anti-platelet drugs have been used to prevent thrombosis of systemic to pulmonary artery shunts. Aspirin has traditionally been used. Clopidogrel is being studied as an alternative and in combination with aspirin for shunt patients. We report a near fatal gastro-intestinal bleed in a patient with shunt and on aspirin and clopidogrel. This combination has been known to produce similar bleeds. The authors recommend caution in combining them. Prospective studies currently underway should evaluate this aspect of the antiplatelet drugs.

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.

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.

Voriconazole in newborns.

Voriconazole is a newer systemic antifungal agent effective against Candida and Aspergillus. There are few reports of its safe use in newborns. We report the first case series of safe Voriconazole use in critically ill newborns with cardiac disease along with several other cardiac drugs without any significant drug interaction or side-effect.

Innominate artery compression of trachea.

Innominate artery may cross the trachea and cause airway obstruction is a rare cause of vascular obstruction of airway. We describe a child with stridor, inability to extubate in whom the diagnosis was suspected on fluoroscopy and confirmed by angiography. Reimplantation of the innominate artery resulted in excellent results with longterm follow up. Variantions of this condition and modalities for confirmation of diagnosis are discussed.

Central line retrieval in a neonate.

Peripherally inserted central lines are important for management of newborns in intensive care. 1% of PICC lines can migrate. Here we describe a preterm baby with a migrated PICC line enmeshed on the pulmonary valve. This was retrieved by snare from the pulmonary valve. The technique is described as also the challenges related to doing this in a small baby.

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.

Balloon dilatation of pulmonary valve in Tetralogy of Fallot's.

In some children of Tetralogy of Fallot's (TOF) presenting with progressive cyanosis, are palliative Blalock-Taussing (BT) shunt may be required. There are no reports of this modality of management in India, though this has been practiced in the other countries. The author reports an infant with Tetralogy of Fallot's who successfully underwent ballon dilatation of the pulmonary valve. Review of literature shows 332 patients with TOF undergoing pulmonary valve balloon dilatation as an alternative to BT shunt in 12 studies with significant increase in pulmonary artery 'Z' score and low incidence of conversion to shunt. This modality of management should be considered in selected patients to change a palliative surgery to an intervention.

Infective endocarditis.

Infective Endocarditis (IE) is a known and a well-described complication of congenital and acquired heart disease. Its onset may be related to a procedure (which is commonly dental) or to poor dental hygiene. Use of antibiotics prior to the procedure prevents IE. General awareness of dental hygiene and use of prophylactic antibiotic is as much a responsibility of the Pediatrician as it is of the Pediatric Cardiologist. This article reviews the changing epidemiology, pathophysiology, clinical manifestations, and the microbiology of IE. Duke criteria for diagnosis of IE and the role of echocardiography in diagnosis of IE are discussed. There is special emphasis on the prevention of IE.