A case of large aneurysms of the coronary arteries and abdominal aorta in an infant.

Aneurysms of the coronary arteries and abdominal aorta are extremely rare in infancy. Due to the rarity of the anomaly, there are no existing guidelines on management of these aneurysms. We describe the challenges in diagnosis, evaluation and management of an infantile Marfan with this rare presentation.

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.

Normal pulmonary venous drainage in the setting of total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection (TAPVC) and anomalous pulmonary venous drainage are not synonymous. This has been described in the setting of right isomerism (bilateral right sidedness) where the pulmonary veins are connected anomalously but drain normally to the left-sided morphological right atrium. We describe another situation in right isomerism where normal pulmonary venous drainage is present in the setting of TAPVC.

Markedly dilated right atrium in an infant: what is the diagnosis?

An 18-month-old boy presented with recurrent episodes of irritability and documented fast heart rate suggestive of supraventricular tachycardia. Cardiovascular examination revealed significant cardiomegaly, normal heart sounds and no murmurs. The differential diagnosis of marked right atrial dilatation and management principles of idiopathic dilatation of the right atrium are described.

Early and Long-Term Clinical Outcomes of Ductal Stenting Versus Surgical Aortopulmonary Shunt Among Young Infants with Duct-Dependent Pulmonary Circulation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years.

Tricuspid Annular Plane Systolic Excursion (TAPSE) for the Assessment of Right Ventricular Function in Adult and Pediatric Cardiac Surgery: Modified Two-dimensional and M-mode TAPSE by Transesophageal Echocardiography Compared to M-mode TAPSE by Transthoracic Echocardiography.

Objectives: To compare transesophageal echocardiography-guided 2-dimensional and M-mode tricuspid annular plane systolic excursion (TAPSE) to transthoracic echocardiography (TTE)-guided M-mode TAPSE in terms of accuracy, interobserver, and intra-observer variability.

Design: A prospective, observational study.

Setting: Adult and pediatric operating rooms.

Clinical profile and outcomes of pediatric hypertrophic cardiomyopathy in a South Indian tertiary care cardiac center: a three decade experience.

Introduction: Although much research has been done on adult hypertrophic cardiomyopathy, data on pediatric hypertrophic cardiomyopathy is still limited.

Methods And Results: The study enrolled all patients with cardiomyopathy who presented to us between 1990 to 2020 and were younger than 18 yrs. During the thirty-year study period, we identified 233 cases of pediatric cardiomyopathy.

Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India.

Objectives: Congenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala to understand presentation of CHD, proportion of newborns with critical defects who receive timely intervention, outcomes at 1 month, predictors of mortality and barriers to timely management.

Methods: The congenital heart disease registry for newborns (≤28 days) in Kerala (CHRONIK) was a prospective hospital-based registry involving 47 hospitals from 1 June 2018 to 31 May 2019.

Tetralogy of Fallot with aortic stenosis and common arterial trunk: Is there a morphological overlap?

Tetralogy of Fallot with aortic stenosis is an extremely rare entity which bears some morphological similarities with the common arterial trunk. Through two illustrative cases of TOF with aortic stenosis we describe the shared anatomical peculiarities of the two anomalies with a review of the possible genetic and developmental factors responsible for the association.

Recurrent pericardial effusion in a boy: A clue to underlying pericardial and pulmonary lymphangiectasia.

Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.

Dual left anterior descending artery with anomalous left anterior descending artery from pulmonary artery: double trouble.

Anomalous origin of left anterior descending artery (LAD) from pulmonary artery (ALADCAPA) with dual LAD from left sinus is an extremely rare coronary anomaly. The diagnostic challenge lies in differentiating this from a coronary cameral fistula. Surgical reimplantation of the anomalous LAD is recommended to prevent the risk of myocardial ischaemia and ventricular arrhythmias.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

Objectives: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up.

Three-dimensional electroanatomical mapping guided right bundle branch pacing in congenitally corrected transposition of great arteries.

Aims: The ideal pacing strategy has been the Achilles' heel for patients with congenitally corrected transposition of great arteries (ccTGA) with bradycardia. Various pacing modalities were documented in the literature. This article describes a novel pacing strategy and its feasibility in ccTGA with an intact ventricular septum.

Heart failure in a newborn with tetralogy of Fallot: uncommon association of a common anomaly.

Heart failure in tetralogy of Fallot in the newborn period is rare and is usually due to either large aortopulmonary collaterals or absent pulmonary valve syndrome with severe pulmonary regurgitation. Pink tetralogy of Fallot and tetralogy of Fallot with disconnected pulmonary arteries from the aorta may present beyond the newborn period with heart failure when Pulmonary vascular resistance (PVR) falls. We describe the diagnostic and therapeutic pathway in a rare case of heart failure in newborn with tetralogy of Fallot.

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

A 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.

Pulmonary artery aneurysm: Harbinger of an ominous disease.

An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51.

Repercussions of a giant major aortopulmonary collateral aneurysm adjacent to vertebral artery origin.

An aneurysm of major aortopulmonary collateral in an adult with congenital cyanotic heart disease was detected incidentally following a hemoptysis episode. The location and size of the aneurysm needed special concern during treatment to avoid aneurysm-related complications and thromboembolism secondary to intervention.

Major Aortopulmonary Collateral Arteries.

Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmonary blood flow. MAPCAs are commonly associated with several congenital heart diseases that have compromised pulmonary circulation due to severe stenosis involving pulmonary valves or arteries or due to pulmonary atresia. Embryologically, MAPCAs are presumed to be persistent segmental arteries.

Ventricular septal defect with pulmonary arterial hypertension in an infant: Is there something more than what meets the eye?

An 11-month-old girl was detected to have muscular ventricular septal defect with hyperkinetic pulmonary hypertension (PH) was urgently operated upon. On follow-up, her PH worsened, resulting in right ventricular dysfunction and was later detected to have absent portal vein.

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.