Double outlet left atrium: successful management of a rare entity.

Double-outlet left atrium is an extremely rare congenital ventriculo-atrial mal-alignment anomaly, wherein, the left atrium drains into both ventricles, through either a common atrioventricular valve or two separate atrioventricular valves. The only egress from the right atrium is through an inter-atrial communication. We present a 16-month-old male, diagnosed to have double outlet left atrium and describe its surgical management.

Experience with surgical correction of double outlet both ventricles.

Double outlet both ventricles is a rare abnormal ventriculo-arterial malformation in which both great arterial trunks are committed to both the ventricles, albeit now being recognized with increasing frequency. Patients with the lesion present with a spectrum of clinical manifestations. The size and location of the interventricular communication dictate the feasibility of biventricular repair. Literature on the malformation, however, is sparse. We report our experience with five patients, all of whom underwent successful surgical biventricular repair.

Total anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.

BACKGROUND: The association of total anomalous pulmonary venous connection (TAPVC) with major congenital cardiac anomalies is rare. This coexistence has an impact on the diagnosis and management strategies. The objective of this single centre study was to analyse the outcomes of such association in bi-ventricular hearts. METHODS: This is a single-centre retrospective review of all patients who underwent repair for TAPVC associated with major congenital cardiac anomalies in bi-ventricular hearts from February 2015 to March 2020. Data were obtained from the hospital database. The primary aim was to assess the surgical outcomes. In addition, we described the incidence, pathophysiology, morphology and surgical strategies in this cohort of patients. RESULTS: Out of a total of 274 patients who underwent repair for TAPVC during the study period, 16 (5.8%) patients had one or more major associated cardiac defect anomalies. The major associated lesions were ventricular septal defects (9), tetralogy of Fallot (2), transposition of the great arteries (1), aorto-pulmonary window (1), partial atrioventricular canal defect (1), pulmonary stenosis (1) and severe tricuspid valve regurgitation due to flail chord (1). The mean follow-up period is 32 ± 13 months. There were two deaths in the post-operative period and no deaths in the follow-up period. CONCLUSION: The incidence of major associated cardiovascular defects with TAPVC in bi-ventricular morphology is 5.8%. The presence of a major associated lesion with TAPVC significantly alters the pathophysiology and clinical presentation with an impact on diagnosis and treatment.

Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot.

The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.

Circumflex aortic arch: presentation across various age groups: a case series.

Circumflex aortic arch (CAA) is a rare congenital anomaly where the aortic arch crosses the midline, posterior to the esophagus and trachea, and descends on the contralateral side. If patent ductus arteriosus (PDA) is present, this forms a true vascular ring. CAA can compress the trachea and esophagus leading to a myriad of symptoms which can present at any age. We describe our experience with three patients of the CAA, presenting across different age groups.

Transposition of the great arteries with total anomalous pulmonary venous connection in a 1½year-old child: Pulmonary arterial hypertension - An advantage.

Transposition of the great arteries with total anomalous pulmonary venous connection is a rare anomaly with varied management approach depending on the time of presentation; the management of cases which present early is usually by anatomical surgical approach while late presentation is by physiologic approach. This is due to early left ventricular mass regression as a result of the absence of volume and pressure load to the left ventricle (LV). We report a late presentation (at 1½ years of age) that had a successful anatomic surgical correction because the LV was "prepared" by both pressure and volume load from pulmonary arterial hypertension and large ostium secundum atrial septal defect.

Total Anomalous Pulmonary Venous Connection and Aortopulmonary Window: Successful Management of a Rare Association.

The association of total anomalous pulmonary venous connection (TAPVC) with aortopulmonary window (APW) is extremely rare. The coexistence of these two anomalies will have implications on clinical presentation, diagnosis, and pathophysiology. The lungs are exposed to pulmonary venous hypertension due to obstructed TAPVC as well as pulmonary arterial hypertension due to unrestrictive APW. We describe successful management of this unusual association in a three-month-old child.

Repair of Taussig-Bing anomaly with unusual coronary pattern using autologous pericardial tube extension.

The incidence of unusual coronary patterns including single coronary artery is high in Taussig-Bing anomaly (TBA). The relocation of a single coronary artery from a nonfacing sinus can be technically challenging with implications on early and late outcomes. Many innovative techniques for coronary transfer have been described and no coronary pattern precludes arterial switch operation in the current era. We describe a technique of coronary transfer using autologous pericardial tube extension with good early outcome.

Repair of tetralogy of Fallot associated with anomalous origin of right pulmonary artery and accessory left anterior descending coronary artery.

We present an unusual combination of lesions in an 8-month-old child diagnosed with tetralogy of Fallot, anomalous origin of right pulmonary artery, and anomalous coronary artery crossing the pulmonary annulus. The association of anomalous origin of branch pulmonary artery from aorta and tetralogy of Fallot (TOF) is extremely rare with an incidence of 0.4%. The incidence of anomalous coronary artery in TOF is 10.3%. However, a combination of all three lesions poses challenges to surgical repair and has not been previously reported.

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.

One-Stage Repair of Tetralogy of Fallot and Pseudoaneurysm of the Ascending Aorta After Central Shunt.

Pseudoaneurysm of the ascending aorta following a central aortopulmonary shunt is a rare and potentially fatal complication. Loss of pulmonary artery continuity is another complication following an aortopulmonary shunt. We report an unusual combination of these two complications following an aortopulmonary shunt performed in a child with tetralogy of Fallot. This was managed successfully along with intracardiac repair of tetralogy of Fallot incorporating only the left pulmonary artery.

Triple Outlet Right Ventricle, With Duplication of the Aortic Root and Intrapericardial Ascending Aorta.

We present a case of a highly unusual congenital cardiac malformation, namely, triple outlet right ventricle with duplication of the aortic root and the intrapericardial component of the ascending aorta. A girl, aged five, presented with complaints of cyanosis and effort intolerance and was diagnosed with double-outlet right ventricle and subpulmonary infundibular stenosis. Intraoperatively, we noted that the aortic root was guarded by two separate aortic valves, oriented anteroposteriorly relative to each other, and separated within the right ventricle by a muscle bar. Postoperative interrogation by both echocardiography and computed tomography confirmed the surgical findings. To the best of our knowledge, our case is the first example of duplication of the aortic root to produce triple outlet right ventricle.

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.

Aortic Root Translocation for Repair of Transposition of Great Arteries With Absent Pulmonary Valve.

Absent pulmonary valve is a rare congenital heart defect where the pulmonary valve is either absent or rudimentary. It is usually associated with tetralogy of Fallot although it has been reported with other lesions. We present a rare case of absent pulmonary valve associated with transposition of great arteries and non-routable ventricular septal defect that was surgically managed by aortic root translocation.

Transesophageal Echocardiography in a Distal Aortopulmonary Window.

Aortopulmonary window (APW) is a relatively rare cardiac lesion representing approximately 0.2%-0.4% of all cardiac malformation. It is a cardiac abnormality that results from abnormal communication between the proximal aorta and the main pulmonary artery in the presence of two normally separated aortic and pulmonary valves. In the past, the diagnostic and surgical approach to APW was almost always preceded by cardiac catheterization. With recent advances in noninvasive approach and techniques of two-dimensional echocardiography diagnosis of the defect and associated anomalies are facilitated without a cath study. We report a 4-month-old infant with a distal APW who was referred to our center for surgical repair. We emphasize the usage of transesophageal echocardiography as a valuable intraoperative tool which not only confirms the preoperative diagnosis but also helps in assessing the surgical repair of an APW.

Modified radial v/s biatrial maze for atrial fibrillation in rheumatic valvular heart surgery.

BACKGROUND: Atrial fibrillation (AF) is commonest sustained atrial arrhythmia producing high morbidity. Although Cox's Maze III procedure cures AF in majority, reduced atrial transport function (ATF) is a concern. Radial approach with ablation lines radial from sinus node towards atrioventricular annulii and parallel to atrial coronary arteries, has shown better ATF. METHODS: Single blind open randomized prospective study of 80 patients was undertaken in two groups (40 each) of modified Cox's maze III and modified radial approach, to evaluate conversion to normal sinus rhythm (NSR) and ATF. Patients undergoing surgery for rheumatic valvular heart disease with continuous AF were prospectively randomized. Ablation lines were created with radiofrequency (RF) bipolar coagulation with cryoablation for the isthmal lesions and coronary sinus. Results were compared at 6 months and ATF was evaluated by atrial filling fraction (AFF) and A/E ratio on echocardiography. RESULTS: The rate of conversion to NSR in both groups was statistically insignificant by Fisher's exact test (p > 0.05). ATF was better in modified radial approach compared to modified Cox's Maze III (A/E compared by unpaired t test:0.52 ± 0.08 v/s 0.36 ± 0.10; p < 0.05. AFF compared using Mann Whitney U test: median AFF for radial group was 23 v/s 20 for biatrial group; p < 0.05). DISCUSSION: In patients with AF undergoing rheumatic valvular surgery, radiofrequency radial approach is as effective as modified Cox's maze III for conversion to NSR with better atrial transport function.

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.