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.

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.

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.

Intra-Atrial Y-Graft Fontan for Univentricular Heart With Discontinuous Pulmonary Arteries.

The Y-graft Fontan as described today suffers from the disadvantage of being hostage to restrictions imposed upon the design of the limbs of the Y by existent cardiac anatomy. We describe a patient with discontinuous pulmonary arteries following a prior Glenn shunt, who underwent Fontan completion using an intra-atrial Y-limb placement for recruitment of the discontinuous pulmonary artery. Intracardiac placement of the limb(s) of the Y-graft could potentially increase the applicability of this Fontan modification without being constrained by external cardiac anatomy.

Anatomical repair of congenitally corrected transposition in the fifth decade of life.

Successful repair of congenitally corrected transposition with ventricular septal defect and pulmonary stenosis presenting with heart failure in the fifth decade of life is described. This is the oldest patient to undergo this surgery, as per existing literature.

Hepatoazygos venous shunt for Fontan completion after Kawashima operation.

Fontan completion after prior Kawashima repair for single ventricle with interruption of the inferior vena cava can be accomplished by various methods. We describe a patient who underwent the connection of hepatic to hemiazygos vein that we believe would be superior to the conventional cavopulmonary connection in our patient.

Banding of Nonrestrictive Large Hypertensive Aortopulmonary Collaterals in Patients With Ventricular Septal Defect and Pulmonary Atresia Presenting in Suboptimal Condition.

Infants with pulmonary atresia and nonrestrictive ventricular septal defect with large hypertensive aortopulmonary collaterals demand early surgical intervention. This presentation in the extremely low-weight child or in the moribund septic child may preclude single-stage repair even if anatomically suited. We propose that such infants may be temporized by means of banding of individual aortopulmonary collaterals as a means of bridging to a second-stage complete repair. Two such cases are presented.

Arterial Switch for Transposition of the Great Arteries With Large Ventricular Septal Defect and for Taussig Bing Anomaly: Experience From a Tertiary Care Center in the Developing World.

BACKGROUND: Results of the arterial switch operation (ASO) for transposition of the great arteries (TGA) with large ventricular septal defect (VSD) and for Taussig Bing anomaly (TBA) in a tertiary care center of a developing country were retrospectively analyzed. METHODS: From January 2007 through June 2013, a total of 30 patients with TBA and 54 patients having TGA with large VSD underwent ASO with VSD closure. Age at surgery for TBA was 27 days to 7 years (median age 3 months) and for TGA with VSD it was 1 day to 6 years (median age 2 months). In all, 46.7% of patients with TBA and 30% of patients with TGA/VSD presented with sepsis secondary to pneumonitis and were taken for surgery when the infection was under control. In all, 13% of patients with TBA and 5.5% of patients with TGA/VSD were on intermittent positive pressure ventilation (IPPV) prior to and continuing up to the time of surgery. RESULTS: On multivariate analysis, factors associated with mortality were the presence of preoperative IPPV for pneumonia (P < .006) and the need for peritoneal dialysis following surgery (P < .028). Neither diagnosis of TBA or TGA/VSD nor any associated anatomical feature (including aortic arch obstruction, unusual coronary anatomy, great artery relationship, or mitral valve anomaly) was found to be related to high early mortality or late complications. Follow-up was 92% complete, with a mean duration of follow-up of 4.1 years. Freedom from reoperation was 98% at five years, and actuarial survival was 83.3% at five years. CONCLUSIONS: Our experience at a tertiary center in a developing country suggests that preoperative need for IPPV for pneumonia is a major determining factor for mortality and morbidity in patients undergoing ASO for TBA and TGA/VSD. This appears to be of greater consequence than details of the underlying disease per se.

Biventricular repair in heterotaxy patients.

BACKGROUND: Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. METHODS: From 2007 until 2012, a total of 20 patients (11 male) with heterotaxy syndrome underwent biventricular repair (left atrial isomerism: 10 and right atrial isomerism: 10) in our center. Their median age at surgery was 40 (range: 3-108) months. Ten patients had dextrocardia. Eleven patients presented with bilateral superior vena cava, three with inferior vena cava (IVC) draining into left atrium, and six with IVC interruption with azygos or hemiazygos continuation. Anomalous pulmonary venous drainage was present in eight patients. One had a common atrium. Atrioventricular septal defect (AVSD) occurred in nine (complete AVSD in seven) patients. Eight patients had double outlet right ventricle (DORV), one had d-transposition of great arteries (d-TGA), and two had congenitally corrected transposition of the great arteries (CC-TGA). Prior palliative procedures included pulmonary artery banding in three patients and left modified Blalock-Taussig shunt in one patient. Complex intra-atrial baffle constructions were required in all patients to direct pulmonary and systemic venous inflow to the appropriate ventricle. Complete AVSDs were corrected using two-patch technique. Intraventricular tunnel repair was done for DORV. Combined atrial and arterial switch was required to rectify abnormal connections in a child with congenitally corrected transposition with normal pulmonary valve, while a Rastelli + Senning was needed in two children with CC-TGA with pulmonary atresia (n = 1) and double outlet of the right ventricle (n = 1). RESULTS: Major early postoperative complications included intestinal gangrene in four patients for which they underwent bowel resection. Two of these patients could not be salvaged. One patient required coiling of aortopulmonary collateral for early postoperative pulmonary hemorrhage. Two patients needed a tracheostomy for prolonged mechanical ventilatory support. Five patients had a pacemaker implanted for complete heart block. There were no instances of atrial baffle stenosis. Median follow-up was 27 (range: 2-46) months. There was one late death secondary to pneumonia. CONCLUSIONS: Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.

Unusual systemic venous Collateral channels to left atrium causing desaturation after Fontan operation closed percutaneously.

We present an unusual cause of progressive cyanosis in a child appearing 2 years after successful Fontan surgery for tetralogy of Fallot with hypoplastic right ventricle. The cause of cyanosis was identified as one large venous channel draining into the left atrium. The channel was closed by Amplatzer vascular plug resulting in improvement of oxygen saturation.

Anomalous origin of right coronary artery from pulmonary artery.

Anomalous origin of coronary artery from the pulmonary artery is a rare anomaly that most frequently involves the left coronary artery and very rarely the right coronary artery. These lesions can be missed on echocardiography unless carefully looked for. We describe a case of isolated anomalous origin of right coronary artery from pulmonary artery diagnosed on echocardiography and confirmed by computed tomography (CT) angiography.

Palliative arterial switch operation in the context of multiple ventricular septal defects, potentially biventricular and univentricular hearts with malposed great arteries: a review of 15 cases.

BACKGROUND: This study is an examination of our unit's experience with palliative arterial switch in univentricular and potentially biventricular hearts with transposition of the great arteries (TGA). METHODS: These patients were divided into three groups based on their physiology. (a) Single ventricle physiology (n = 8), in which all the patients had univentricular hearts, TGA, and subaortic stenosis (SAS). (b) Borderline biventricular physiology (n = 4), in which the patients had TGA, ventricular septal defect (VSD), and hypoplastic right ventricle (RV). (c) Biventricular physiology (n = 3), in which the patients had TGA and multiple VSDs. RESULTS: In all, 12 (80%) patients survived. Seven of these have undergone second stage surgery (cavopulmonary shunt, n = 5; biventricular repair, n = 2). CONCLUSION: Palliative arterial switch is an alternative to Norwood procedure and modifications thereof for managing SAS in single ventricle with malposed great arteries. Palliative switch with adjunctive pulmonary artery band may be a temporizing measure in TGA with multiple VSDs, where the VSDs are judged to be inaccessible through the tricuspid valve or through either of the great arteries. It may also be utilized for TGA and hypoplastic RV instead of committing them to univentricular pathway and keeping the option of biventricular repair.

Anatomic biventricular repair in right isomerism with noncommitted ventricular septal defect.

Biventricular repair in right atrial isomerism is rarely feasible due to associated anomalies of venous connection, ventricular imbalance, nonroutabilty of the interventricular communication, a common atrioventricular junction, and inadequate pulmonary arterial branches. These patients are also often not ideal for univentricular repair due to some of the above associations. We describe a novel surgical technique that was utilized in such a patient for biventricular repair of a child with right atrial isomerism with total anomalous pulmonary venous connection, regurgitant common atrioventricular valve, hypoplastic left ventricle, nonroutable ventricular septal defect, and pulmonary stenosis.

Occult anomalous origin of the left coronary artery from the pulmonary artery with ventricular septal defect.

Manifestations of anomalous left coronary artery from the pulmonary trunk may be masked in the presence of an associated shunt lesion that prevents fall of pulmonary artery pressures and allows perfusion of the anomalous coronary artery. We present such a patient with a large ventricular septal defect associated with the anomalous coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery with patent ductus arteriosus: a must to recognize entity.

Anomalous left coronary artery from the pulmonary trunk (ALCAPA) presents in early infancy with a clinical picture of congestive heart failure with left ventricular (LV) dysfunction and mitral insufficiency. These manifestations of myocardial ischaemia may be masked in the presence of an associated patent ductus arteriosus (PDA) or ventricular septal defect (VSD) which prevents the fall of pulmonary artery pressures and allows perfusion of the anomalous coronary artery. We present a case of a patient with large PDA-associated ALCAPA and preserved LV function. The importance of such a finding lies in the fact that VSD closure or PDA ligation in such cases would unmask the ALCAPA.

Challenges in delayed repair of atrioventricular septal defects.

Delayed diagnosis and surgery for atrioventricular septal defects are not uncommon in the developing world. This review details the challenges faced in managing this difficult subset of patients.

Anatomic repair for congenitally corrected transposition of the great arteries.

OBJECTIVE: Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. We present our 13-year experience with this approach. METHODS: Between May 1994 and September 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Thirty-one patients (group 1, mean age of 94.8 +/- 42.3 months) underwent a combined Rastelli and atrial switch operation. Thirty-seven patients (group 2, mean age of 36.1 +/- 46.9 months) underwent an arterial switch operation and atrial rerouting. Eight patients in group 2 had an intact ventricular septum. RESULTS: Group 1 had 5 early deaths (17%) but no late deaths. Three patients underwent conduit revision at a mean follow-up of 62 months. Group 2 had 5 early deaths (13.5%). There were 4 late reoperations (2 pulmonary baffle revisions, 1 mitral valve replacement, and 1 permanent pacemaker implantation) and 4 late deaths (1 secondary to progressive left ventricular dysfunction, 2 secondary to uncontrolled atrial tachyarrhythmia, and 1 secondary to pulmonary hypertension and right ventricular failure). In group 2, 4 patients have a left ventricular ejection fraction less than 40%, 5 patients have moderate aortic incompetence, 5 patients have symptomatic tricuspid incompetence, 1 patient has tricuspid stenosis, 1 patient has superior cava obstruction, and 3 patients are receiving antiarrhythmic therapy. CONCLUSION: The occurrence of left ventricular dysfunction indicate that anatomic repair in the arterial switch group is still fraught with imperfections. The Rastelli group required conduit revisions but has otherwise performed well.

Isolated atrioventricular discordance: surgical experience.

BACKGROUND: Isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance) is an extremely rare presentation of cyanotic congenital heart disease. The mode of presentation is akin to D-transposition of great arteries as systemic venous drainage and systemic arterial output connect to the same side of the cardiac septae, and pulmonary venous drainage and pulmonary arterial outflow to the opposite. Systemic oxygenation relies on intracardiac or extracardiac modes of mixing, as does survival, similar to transposition of the great arteries. Published literature is scant, mainly because of the rarity of this cardiac anomaly. We review our surgical experience with this lesion. METHODS: Five children with isolated ventricular inversion presented to us between the ages of 6 days and 22 months (mean, 12 months). Preoperative echocardiogram diagnosed large interventricular communication in 4, a patent ductus arteriosus in 4, and total anomalous pulmonary venous drainage with supracardiac connection in 1. One had associated narrowing of the left pulmonary artery origin. Four patients had atrial situs solitus, whereas 1 had right atrial isomerism. Three hearts had normally related great arteries whereas in 2, the aorta was to the right and anterior to the main pulmonary artery and arising in parallel fashion from the cardiac mass. Four children underwent trans-right atrial patch closure of the interventricular communications, with ligation of the patent ductus arteriosus. All 4 underwent a concomitant modified Senning's repair. The fifth patient underwent repair of total anomalous pulmonary venous drainage with a Mustard-type repair. One needed concomitant repair of the mitral valve for injury to a free edge chorda sustained during closure of the ventricular defect. RESULTS: There was 1 early death. Complete heart block developed in 2 children, of which 1 needed permanent pacemaker insertion, whereas the other converted to sinus rhythm with intermittent atrial tachycardia. All survivors are doing well on follow-up, at a follow-up duration ranging from 6 to 48 months (mean, 18). CONCLUSIONS: Repair of isolated atrioventricular discordance can be successfully achieved in the majority of patients presenting with this complex anomaly.