Tetralogy of Fallot associated with supramitral ring: “Paying for a near miss”

AbstractA 13-year-old girl developed the features of congestive heart failure on day three of intracardiac repair of tetralogy of Fallot. A routine transthoracic echocardiography identified the missed supramitral ring (SMR), closely placed just above the mitral valve, the very reason for severe mitral stenosis and its implication in the present clinical deterioration. A rare association of SMR with tetralogy of Fallot may be missed narrowly because closely placed SMR can sometimes mask its hemodynamic effect when present with significant right ventricular outflow tract obstruction as in this case.<Learning objective: This case report highlights when a case of complex congenital heart disease is referred for intervention or surgery, it is better to be verified by a pediatric cardiologist. Tetralogy of Fallot (TOF) associated with supramitral ring (SMR) is reported as an anecdotal case report. Although it is a mistake, it may be possible to miss the SMR when it is associated with TOF. The reasons are threefold: (1) The rare association of SMR with TOF. (2) The close proximity of SMR to mitral valve. (3) Right ventricular outflow obstruction in TOF reduces flow across mitral valve i.e. a low cardiac output state.

A Cross-sectional study to look at the determinants of poor adherence to secondary penicillin prophylaxis for rheumatic heart disease at a tertiary care center in South India

Background: Rheumatic heart disease (RHD) continues to create havoc in the developing countries even decades after its discovery. It is entirely preventable through primordial, primary, and secondary level intervention. Secondary prevention is a reasonable treatment option in patients in India, but it suffers due to poor adherence which remains the main impediment to its implementation. The aim is to study the compliance with benzathine penicillin as secondary prophylaxis in RHD patients and to establish the patient-related factors for adherence and reasons for missing of doses. Materials and Methods: This is a cross-sectional study of RHD patients presenting to our institute. The demographic data, clinical history, and details of penicillin prophylaxis were noted. The patient was labeled as compliant or noncompliant depending on frequency and duration of prophylaxis as prescribed. Potential factors between the two groups have been analyzed by univariate and binary logistic regression. Results: The study cohort of 500 patients consisted of 261 compliant and 239 noncompliant patients. Average age of presentation was 29 ± 13 years with females outnumbering the males. Noncompliance with secondary prophylaxis was more prevalent among male (P = 0.003), low socioeconomic class (P = 0.0009), uneducated (P = 0.000018), and the rural population (P = 0.025) while those with previous history of rheumatic fever (RF) were found to be more compliant (P = 0.04). Recurrences of RF were more common in those not on regular prophylaxis (P = 0.011). The most common reason cited for noncompliance was the absence of proper counseling followed by a sense of well-being, injection site pain and financial constraints. Conclusion: Compliance with secondary penicillin prophylaxis is essential to ensure eradication of RHD. Education about the importance and necessity of prophylaxis would improve compliance. A close patient and health personnel relationship is important in improving adherence to secondary prophylaxis

Infective endocarditis caused by Stenotrophomonas maltophilia: A report of two cases and review of literature

Stenotrophomonas maltophilia is known for nosocomial habitat. Infective endocarditis due to this organism is rare and challenging because of resistance to multiple broad-spectrum antibiotic regimens. Early detection and appropriate antibiotic based on culture sensitivity reports are the key to its management. We report the diagnosis, treatment, and outcome of two cases of infective endocarditis caused by S. maltophilia

Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement

Surgical exclusion of postsurgical pseudoaneurysm of the ascending aorta

Pseudoaneurysm of ascending aorta after cardiac surgery is rare in children. We report a case of successful surgical exclusion of ascending aortic pseudoaneurysm in a 15-year-old boy. The neck of the aneurysm was in close proximity to the right coronary artery (RCA)