Long-term outcome of permanent epicardial pacemaker implantation in neonates: Experience from an Indian center.

Introduction: Permanent pacemaker implantation (PPI) in neonates is challenging with respect to indications, device selection, implantation technique, and long-term outcomes. Complex anatomy, the need for long-term pacing with high rates, and a problematic postoperative period are the major problems. Methods: We prospectively followed up 22 newborns who underwent PPI below 28 days of life at our institute. Results: The median age at implantation was 2 days (interquartile range 1-9 days), and 9% were born preterm. The average heart rate before implantation was 46.4 ± 7.2 bpm. Maternal lupus antibodies were positive in 8 (36.4%) neonates, whereas 11 (50.0%) had associated congenital heart disease. Nineteen neonates underwent single chamber (VVI) and three underwent dual chamber (DDD) pacemaker implantation. Over a median follow-up of 46 months (range 2-123 months), the average ventricular pacing percentage was 87.5 ± 24.9%, with a stable pacing threshold. Seven children underwent pulse generator replacement due to battery depletion at a median age of 47 months. Pacing-induced ventricular dysfunction was seen in five children at a median age of 23.6 months, and two underwent upgradation to cardiac resynchronization therapy. Overall mortality was 13.6%, all due to tissue hypoperfusion and lactic acidosis in the postimplantation period. Conclusions: PPI in neonates has a favorable outcome with excellent lead survival. Overall mortality is 13.6%, which is predominantly in the postimplantation period and related to myocardial dysfunction.

Atrial septal defect occluder device embolization: Experience of a tertiary care cardiac center.

Background: Percutaneous device closure of atrial septal defect (ASD) has become an increasingly popular procedure as it offers several advantages. However, it is associated with infrequent, but life-threatening complications such as device embolization. Objective: To analyze the risk factors, common sites of embolization, associated complications, timing of embolization, and the treatment executed. Settings and Design: A retrospective study was performed at a tertiary referral center for cardiac services. Material and Methods: Pre-procedure, intra-procedure, and post-procedure data of patients whose ASD device embolized was collected retrospectively and analyzed for risk factors, common sites of embolization, associated complications, timing of embolization, and the treatment executed. Results: Thirty devices were embolized, out of which 13 were retrieved percutaneously in the Catheter laboratory, whereas 17 patients underwent surgery. Fourteen patients had an unfavorable septal morphology for device closure. Ten devices were embolized in the catheter laboratory, five in the intensive care unit, and two in the ward. The devices were embolized to almost all chambers of the heart and great vessels. One patient had an inferior vena cava rim tear while attempting percutaneous retrieval. One patient required a short period of total circulatory arrest (TCA) for retrieval of the device from ascending aorta, while another required a lateral position for retrieval from descending aorta. One patient required re-exploration for bleeding, while another had an air embolism and succumbed. Conclusions: Once embolization occurs, the risks associated increase manifold. Most of the surgical extractions are uneventful; however, there could be certain complications that may need repair of valvular apparatus, the institution of TCA, or the need for the lateral position. Air embolization though very rare can occur which could be fatal.

Left ventricular noncompaction in primary systemic carnitine deficiency: A rare association.

Left ventricular noncompaction (LVNC) is a rare phenotype of dilated cardiomyopathy. We report a child with primary systemic carnitine deficiency having associated LVNC.

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.

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.

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.

Anomalous systemic and pulmonary veins-An unusual coexistence.

A 12-year-old child presented with recurrent respiratory infections and was diagnosed with Scimitar syndrome. Drainage of IVC and course of aberrant arterial supply from aorta were not clear by echocardiogram, and hence, additional imaging was planned. CT unraveled the presence of a rare combination of anomalous pulmonary venous connection (APVC) to IVC, with near-atresia/severe stenosis of IVC, superior to the drainage of scimitar vein. There was a prominent azygos vein with preferential contrast opacification on lower limb injection.

Usefulness of Doppler derived end diastolic flow gradient across the patent ductus arteriosus in selecting coils for ductal occlusion.

Transcatheter closure of patent ductus arteriosus (PDA) with coils is accepted as an alternative to surgical ligation. We evaluated whether flow gradient across PDA, obtained by Doppler echocardiography, can aid in selecting coils for percutaneous ductal occlusion. 79 consecutive patients with PDA, who underwent successful percutaneous coil occlusion were retrospectively reviewed. Patients with other structural heart disease and pulmonary hypertension with right-to-left shunt were excluded. Echocardiogram and cardiac catheterization were done in all patients. Gianturco (Occluding Spring Emboli; Cook, Bloomington, IN) non-detachable coils of 0.038 and 0.052-inch core sizes were used for ductal occlusion. Trough diastolic gradient was correlated with the size and the number of coils used. Mean age was 8.6 years (range 1.3 to 27 years); 24 males and 55 females; PDA diameter ranged from 1.3 to 4.5 mm. Number of coils used varied from 1 to 4. Echocardiography measured PDA size was 2.5 ± 0.6 mm and significantly differed from angiographically measured size 2.9 ± 0.6 mm (P = 0.05). End diastolic gradient below 38 mmHg predicted use of multiple coils or coils with larger surface area. End diastolic gradient correlated inversely with total surface area of the coils, which indirectly predicted size and number of coils. Thus, the prediction of the size and the number of coils for PDA occlusion can be assisted by the trough diastolic gradients of PDA.

Intra-atrial extension of Wilms' tumor.

Echocardiography in a 4-year-old boy, with nephroblastoma of the left kidney, revealed a large homogenous mass in right atrium extending from inferior vena cava, and protruding through tricuspid valve into right ventricle during diastole. Ultrasonography revealed the contiguous spread of the tumor through renal vein with near total caval occlusion. The patient had a fatal outcome before definite treatment could be started. Intracardiac extension of infradiaphragmatic tumors through caval route, although infrequent, can be seen with renal cell carcinoma, Wilms' tumor, hepatoma, lymphoma, and uterine and adrenal tumors. Detection of a mass in right atrium in a child should alert the echocardiographer about the possibility of caval spread from a renal neoplasm.

Type A aortic dissection with partial ostial occlusion of left main coronary artery.

A 48-year-old hypertensive male presented with acute retrosternal pain and aortic regurgitation. The electrocardiogram showed ST-segment depression with T-wave inversion in anterolateral leads. Transesophageal echocardiography in long axis view of aorta revealed a spiral intimal flap in ascending aorta extending to the arch, diagnostic of Type A aortic dissection. The short axis view of the aorta showed partial obstruction of the left main coronary artery (LMCA) by the intimal flap with turbulent flow at its ostium. An emergency repair of aortic dissection with reconstruction of aortic wall was done. Postoperative period and ECG were normal. At 12-months of follow up, patient was doing well.