Clinical, hemodynamic, echocardiographic, angiographic profiles and post-operative outcomes among DCRV patients from a tertiary care referral center in India

Background: This retrospective study of data from 2006 to 2018 at a tertiary care referral center in India aims to document the contemporary clinical and hemodynamic profile of patients who were diagnosed with double chamber right ventricle (DCRV) based on echocardiography and cardiac  catheterization. Patients were followed up and their outcomes were studied to document their short and long term outcomes.Methods: It is a retrospective observational study of patients diagnosed with DCRV in a tertiary care hospital in India. The diagnosis of DCRV was based on the following criteria: an echocardiographic diagnosis of DCRV with doppler evidence of a mid ventricular gradient; cardiac catheterization revealing a systolic pressure gradient between right ventricular inflow and outflow tracts; a right ventricular angiogram demonstrating an anomalous muscle bundle causing obstruction well below the infundibulum. All the patients were followed up for their long term outcomes.Results: All the patients underwent echocardiography and cardiac catheterization for confirmation of the diagnosis. Sixty percent of our patients presented during adulthood, which is very unusual presentation of this disease entity. Median age of our patient cohort was 23.5 years. Patients presenting during adulthood have atypical symptoms. Dyspnea was the most common presenting symptom in this study. Right ventricular hypertrophy (73.3%)and right bundle branch pattern (26.6%) were the common electrocardiographic findings in our patients. DCRV is commonly associated with other anomalies. Ventricular septal defect (VSD) was the commonest associated anomaly, which was seen in 80% of our patients. Mean gradient cross the anomalous muscle bundle was 67.5 mmHg. Three of our patients (20%) had no associated anomaly, which is very rare in DCRV. Eight patients underwent surgical correction with significant reduction in gradients in all and no perioperative mortality. Median follow up of 8 years showed no adverse outcomes and no progression of gradients.Conclusions: This study describes in detail the clinical profile, echocardiographic and angiographic identification of anomalous muscle bundles in DCRV patients, which will help the young readers in identifying this often missed diagnosis. It highlights the unusual presentation during adulthood with atypical symptoms in DCRV patients with excellent long-term outcomes on follow up.

Contemporary outcomes of percutaneous closure of patent ductus arteriosus in adolescents and adults

Background Catheter based treatment has gained wide acceptance for management of patent ductus arteriosus (PDA) ever since its introduction. Percutaneous closure in adults can be challenging because of anatomical factors including large sizes, associated pulmonary arterial hypertension (PAH) and co-morbidities. This study aimed to provide comprehensive contemporary data on the safety and efficacy of percutaneous device closure of PDA in adult and adolescent population at a large referral center. Methods This single-center retrospective analysis included 70 patients (33 adolescents and 37 adults) who underwent successful percutaneous device closure of PDA between January 2011 and February 2017.Baseline patient demographics, clinical characteristics, procedural and device related variables, and immediate outcomes during hospital stay were recorded. Patients were followed up for residual shunt and complications. Results Of 70 PDA device closure cases, 71.4% were females; the mean age was 23 years (range:10-58years). Devices used were 4-Cook’s detachable coils, 64-occluders (ADO-I and II, Lifetech, Cardi-O-Fix), 1-vascular plug and 1-ventricular septal occluder device. Device success was achieved in all including those with very large PDAs. At 24-h post-procedure, the success rate of transcatheter intervention was 95.7%. At 6-months follow up, complete closure was observed in all (mean follow up duration-531 days). In patients with severe PAH, significant immediate and sustained reduction of the mean pulmonary pressure was observed(77 mmHg to 33 mmHg;P = 0.014). No procedure-related complications including death, device embolization and stenosis of aorta or pulmonary artery occurred. Conclusions In contemporary practice, percutaneous device closure is an effective and safe treatment option for adolescent and adult PDA patients.

Nonresponders to clopidogrel therapy among Indian patients undergoing elective/adhoc angioplasty.

BACKGROUND: Clopidogrel has become the standard antiplatelet drug along with aspirin in patients undergoing coronary angioplasty; however, data regarding the nonresponse rate to clopidogrel therapy in Indian patients are limited. METHODS AND RESULTS: Platelet aggregation was measured at baseline and 2 and 24 hours post administration of bolus dose of 300 mg clopidogrel, followed by 75 mg once daily in patients undergoing elective or adhoc coronary angioplasty. Baseline platelet aggregation with 2.5 and 10 micromol/L ADP was 27.91 +/- 20.9% and 53.45 +/- 22.44%. Platelet aggregation at 2 hours and 24 hours with 2.5 micromol/L of ADP was 19.65 +/- 16.9% and 10.44 +/- 11.9%. The corresponding values with 10 micromol of ADP were 48.81 +/- 25.3% and 27.04 +/- 22.4%. Platelet aggregation was maximally inhibited at 24 hours with both 2.5 and 10 micromol/L of ADP. Marked interpatient variability in platelet aggregation in response to clopidogrel administration was observed and varied from -43 to 65%, -32 to 85% with 2.5 micromol/L at 2 hours and 24 hours and -65 to 53%, -35 to 97% with 10 micromol/L ADP at 2 hours and 24 hours. Nonresponse rate 2 hours after clopidogrel administration was 47.7%, and decreased to 29.2% at 24 hours post drug administration. CONCLUSION: Clopidogrel nonresponse is prevalent among Indian patients, and there is wide interpatient variability in platelet inhibition among individual patients. However, the clinical implications of these findings need to be substantiated in larger studies with clinical end points.

Incessant atrial flutter after device closure of atrial septal defect: successful radio frequency ablation.

A four-month-old baby presented with failure to thrive and congestive cardiac failure precipitated by a lower respiratory tract infection. He was found to have a large ostium secundum atrial septal defect measuring 18 mm. This was successfully closed percutaneously by a device (Blockaid). A month after the device deployment the child developed typical atrial flutter. Despite rate control drugs the ventricular rate remained l40/min over the next several months. In view of the incessant atrial flutter with fast ventricular response, the child underwent radiofrequency ablation at the age of 2 years. An isthmus block was created which successfully terminated the tachycardia.

Torsade de pointes in a case of pheochromocytoma--an unusual presentation of an uncommon disease.

We report the case of a middle aged lady with dilated cardiomyopathy, presenting with recurrent syncope due to torsade de pointes. Further evaluation revealed that she had a pheochromocytoma which caused the arrhythmia.

Prevalence of coronary artery disease in patients with rheumatic heart disease in the current era.

BACKGROUND: This study was undertaken to determine the prevalence of coronary artery disease in patients with rheumatic heart disease undergoing valve surgery. METHODS AND RESULTS: Consecutive patients with rheumatic heart disease (n=376) who were above the age of 40 years, and scheduled for valve surgery underwent diagnostic coronary angiogram to delineate coronary arteries. The patients were divided into three groups based on valve involvement (mitral valve, aortic valve, and combined aortic and mitral valve). Significant coronary artery disease was considered to be present if one or more coronaries showed 50% or more luminal stenosis. There were 287 (76.3%) males and 89 (23.7%) females. The mean age of the study population was 51.2+/-8.2 years. Eighty-nine (23.8%) patients had typical chest pain, 116 (30.6%) patients had atypical chest pain and 171 (45.5%) patients had no chest pain. Hypertension was noted in 88 (23.4%) patients, 65 (17.3%) patients had diabetes, 98 (26.1%) patients were smoker, and 66 (17.6%) patients had dyslipidemia, and 15 (4.0%) patients gave past history of myocardial infarction. Of the total 376 patients, 46 (12.2%) patients were found to have significant coronary artery disease. In patients with mitral vale disease the prevalence was 13.5% (13/96), while it was 15.3% (19/124) in patients with aortic valve disease and 9% (14/156) in those with combined mitral and aortic valve disease. CONCLUSIONS: Our results suggest that the overall prevalence of coronary artery disease in a group of patients with rheumatic heart disease undergoing valve surgery in the current era is 12.2%. This prevalence is much lower than the figures reported earlier in the Western literature.