Covered Stent Correction for Sinus Venosus Atrial Septal Defects, an Emerging Alternative to Surgical Repair: Results of an International Registry.

Background: Covered stent correction for a sinus venosus atrial septal defect (SVASD) was first performed in 2009. This innovative approach was initially viewed as experimental and was reserved for highly selected patients with unusual anatomic variants. In 2016, increasing numbers of procedures began to be performed, and in several centers, it is now offered as a standard of care option alongside surgical repair.

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.

Early multicenter experience of a new balloon expandable MyVal transcatheter heart valve in dysfunctional stenosed right ventricular outflow tract conduits.

Background: Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI.

Methods: Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal.

Explanting the migrated hepatic stent in the right atrium by modified vena caval inflow occlusion.

Stenting of hepatic veins can be a long lasting solution for Budd-Chiari syndrome. These stents could very rarely migrate into the right atrium. During surgical retrieval, cardiopulmonary bypass (CPB) can be avoided if vena caval inflow occlusion (VCIO) is used.

Anomalous left coronary artery from nonfacing pulmonary sinus: direct aortic reimplantation.

We report a new technique of establishing a direct coronary transfer for anomalous left coronary artery arising from the nonfacing sinus of the pulmonary artery. This easily reproducible technique was successfully used in 2 patients. It achieves a dual coronary repair without the use of complex aortic or pulmonary arterial flaps and without causing any distortion to the great vessels.

Off-pump atrial septostomy with thoracoscopic scissors under transesophageal echocardiography guidance.

Selected children with congenital heart defects undergoing palliative closed heart procedures require a cardiopulmonary bypass (CPB) run only for the purpose of creating an inter-atrial communication. We report a simple technique of atrial septostomy using thoracoscopy scissors under transesophageal echocardiography guidance without the need for CPB.

Two-ventricle repair for complex congenital heart defects palliated towards single-ventricle repair.

Objectives: Complex congenital heart defects that present earlier in life are sometimes channelled towards single-ventricle repair, because of anatomical or logistic challenges involved in two-ventricle correction. Given the long-term functional and survival advantage, we have been consciously exploring the feasibility of a biventricular repair in these patients when they present later for Fontan completion.

Methods: Since June 2009, 71 patients were referred for staged completion of the Fontan procedure.

Tricuspid valve mycetoma in an infant successfully treated by excision and complex tricuspid valve repair followed by fluconazole therapy.

Background: Fungal valve endocarditis in children is an uncommon and lethal disease. The risk increases with use of central venous catheters (CVC), total parenteral nutrition (TPN), and use of broad-spectrum antibiotics during the neonatal period. Due to high mortality, a combination of surgery and antifungal therapy is usually recommended for treatment.

Palliative stenting of patent ductus arteriosus in older children and young adults with congenital cyanotic heart disease.

Objective: To describe the indications, technique and results (early and short-term follow-up) of palliative patent ductus arteriosus (PDA) stenting in selected patients (2 years and older) with congenital cyanotic heart disease with reduced pulmonary blood flow who were not candidates for definitive surgery in the immediate future.

Background: Stenting of PDA as a palliation has been advocated as safe and effective procedure in neonates, but this modality is underutilized for children and adults.

Methods: Hospital records of patients (≥2 years) undergoing PDA stenting between January 2007 and September 2009 were reviewed.

Anomalous systemic arterial supply to normal basal segment of the left lung.

Background: Anomalous systemic arterial supply to normal segments of the lung is an unusual anomaly. It represents part of a spectrum of bronchovascular abnormalities which have various anatomical and clinical manifestations.

Methods: We retrospectively analysed cases from January 2007 to April 2010 from two institutions diagnosed with an anomalous systemic arterial supply to a normal lung segment.

Decline in arterial partial pressure of oxygen after exercise: a surrogate marker of pulmonary vascular obstructive disease in patients with atrial septal defect and severe pulmonary hypertension.

Objectives: To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension.

Methods: Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise.

Isolated absence of right pulmonary artery.

Background: Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants

Objective: The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature.

Methods: The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectively

Results: Five patients were identified with this anomaly.

Transcatheter occlusion of patent ductus arteriosus in pre-term infants.

Objectives: The aim of this study was to describe our institutional experience with transcatheter coil occlusion of patent ductus arteriosus (PDA) in symptomatic low birth weight pre-term infants.

Background: Transcatheter treatment of PDA in very small infants (<2 kg) is technically challenging and therefore often not considered as an alternative to traditionally accepted modalities (surgical or medical treatment).

Methods: Coil occlusion was offered as an option to selected infants with symptomatic PDA.

HECT domain-containing E3 ubiquitin ligase Nedd4 interacts with and ubiquitinates Sprouty2.

Sprouty (Spry) proteins are important regulators of receptor tyrosine kinase signaling in development and disease. Alterations in cellular Spry content have been associated with certain forms of cancers and also in cardiovascular diseases. Thus, understanding the mechanisms that regulate cellular Spry levels are important.

Intermolecular interactions of Sprouty proteins and their implications in development and disease.

Receptor tyrosine kinase (RTK) signaling is spatially and temporally regulated by a number of positive and negative regulatory mechanisms. These regulatory mechanisms control the amplitude and duration of the signals initiated at the cell surface to have a normal or aberrant biological outcome in development and disease, respectively. In the past decade, the Sprouty (Spry) family of proteins has been identified as modulators of RTK signaling in normal development and disease.

Emergency balloon dilation or stenting of critical coarctation of aorta in newborns and infants: An effective interim palliation.

Background: Management of native uncomplicated coarctation in neonates remains controversial with current evidence favoring surgery. The logistics of organizing surgical repair at short notice in sick infants with critical coarctation can be challenging.

Methods And Results: We reviewed data of 10 infants (mean age of 2.

Vein of galen arteriovenous malformation: diagnostic clues from echocardiography.

We report a rare association of vein of Galen malformation in a young infant with associated sinus venosus atrial septal defect and aortic arch hypoplasia. This unique association of lesions provides insights into the embryological basis as well as offers potential echocardiographic clues for the diagnosis of the cerebral arterio-venous malformation.

Catheter closure of atrial septal defects with deficient inferior vena cava rim under transesophageal echo guidance.

Objectives: To describe the case selection, imaging considerations, technique, and results of catheter closure of atrial septal defects (ASD) with deficient inferior vena cava (IVC) rim.

Background: Transcatheter closure with Amplatzer septal occluder (ASO) has become standard treatment for most secundum ASDs. Defects with deficient IVC rim continue to be challenging to image and close in the catheterization laboratory.

Ruptured submitral aneurysm in an eight-year-old girl.

We present illustrative images of submitral aneurysm in a young girl. The diagnosis was established on echocardiography and the extent of the problem was defined through multi-detector computerized tomography.

Transcatheter closure of aortico right atrial tunnel--a rare cardiac anomaly.

Aortico right atrial tunnel is a rare anomaly with intriguing embryological basis characterized by a tubular communication between the ascending aorta and the right atrium. Few isolated cases reported in literature were often surgically managed on cardiopulmonary bypass. We report a successful transcatheter closure of a large aortico right atrial tunnel in 1 patient with anatomical and technical details.

Transcatheter closure of distal aortopulmonary window using Amplatzer device.

Aortopulmonary window is a rare congenital heart defect, often requiring open surgical closure. Distal windows with good margins from the semilunar valves and coronary artery origin are amenable for transcatheter closure. Experience in nonsurgical closure of such defects is limited.

Safety and feasibility of transcatheter closure of large patent ductus arteriosus measuring >or=4 mm in patients weighing

Objectives: The study aims to find safety of transcatheter closure of large patent ductus arteriosus (PDA) >or=4 mm in patients weighing
Background: Large PDA >or=4 mm in patients View Article and Find Full Text PDF

Analysis of protein folding and oxidation in the endoplasmic reticulum.

Proteins that travel through the secretory pathway undergo post-translational folding and oxidation steps that lead to correct conformation of the final protein. This unit focuses on methods for the analysis of folding and oxidation events and the factors responsible for their proper execution. Alkylation and nonreducing SDS-PAGE is use to analyze disulfide bond formation in ER-derived microsomes.

Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot.

Objectives: To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF).

Background: Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers.