Paroxysmal Hypertension: A Grave Cause.

A 62-year-old lady was referred with the diagnosis of hypertensive encephalopathy. She had episodes of paroxysms of hypertension while on the ventilator with normal saturations. She underwent a battery of tests to identify the cause of the paroxysms.

Severe pulmonary arterial hypertension in congenital sideroblastic anemia from PUS1 mutation - a case report.

Background: Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate.

Lack of "ventricular interdependence" in constrictive pericarditis and atrial septal defect.

Ventricular interdependence, i.e., reciprocal variations in the left and right ventricle pressures with respiration, is a hallmark of the hemodynamic diagnosis of constrictive pericarditis (CP).

Failure to wean off after a routine total anomalous pulmonary venous connection operation: An uncommon association.

A 6-month-old infant was operated on for supracardiac total anomalous pulmonary venous connection (TAPVC) with usual anatomy. The vertical vein was ligated. Weaning from bypass was attempted twice but was unsuccessful.

Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta.

Arterial thoracic outlet syndrome in an adolescent.

Arterial thoracic outlet syndrome (aTOS) is rarely diagnosed in children. A 15-year-old boy with recent-onset claudication was found to have aTOS on investigation. Bony abnormality other than the cervical rib might also cause aTOS.

COVID-19 associated renal artery stenosis in infancy - A report of two cases.

Organ-specific vasculitis is an uncommon, delayed complication of COVID-19 infection. It is usually seen in mildly symptomatic or asymptomatic patients. Underlying endothelitis is the most likely pathophysiological mechanism for such a manifestation.

Successful balloon dilatation of an infolded polytetrafluoroethylene graft: An unusual cause of early Fontan failure.

Despite optimal medical management, an 8-year-old boy had persistent pleural drainage following total cavopulmonary connection. Detailed evaluation, including computed tomography angiography, confirmed obstruction at the lower end of the circuit due to infolding of the polytetrafluoroethylene graft. Balloon dilation of the obstruction resulted in prompt resolution of pleural effusion with sustained relief at 1-year follow-up.

Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

Introduction: Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making.

Materials And Methods: In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year.

Neurodevelopmental outcomes in children with cyanotic congenital heart disease following open heart surgery.

Background: Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes.

Aim: We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD.

Infective endocarditis-induced complete closure of a ventricular septal defect and complete heart block in a child.

We hereby report rare occurrence of irreversible complete heart block in a child with tricuspid valve infective endocarditis. The tricuspid valve vegetation also caused complete closure of perimembranous ventricular septal defect, which was later discovered during surgery.

Heart Failure in a Child: Multimodality Approach Leading to an Unusual Cause.

Heart failure secondary to isolated pulmonary artery vasculitis is rarely described in children. We describe a 10-year-old child who presented with right heart failure symptoms, severe pulmonary hypertension, and bilateral branch pulmonary artery stenosis secondary to isolated pulmonary artery vasculitis. ().

Efficacy and safety of propranolol in infants with heart failure due to moderate-to-large ventricular septal defect (VSD-PHF study) - A prospective randomized trial.

Aims: The utility of beta-blocker therapy in infants with heart failure (HF) due to significant left-to-right shunt lesions is not known. The study aimed to assess the efficacy and safety of propranolol in infants with HF due to moderate-to-large ventricular septal defect (VSD).

Methods: The prospective randomized trial included 80 infants with HF and moderate-to-large VSD, randomly allocated to receive either conventional therapy alone ( = 40) or propranolol plus conventional therapy ( = 40).

Impact of COVID-19 pandemic on pediatric cardiac services in India.

Background: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India.