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Background: Patients with rheumatic mitral stenosis (MS) and atrial fibrillation (AF) are at risk for thromboembolism and restoration of sinus rhythm (SR) may be the preferred strategy. Percutaneous balloon mitral valvotomy (PBMV) improves hemodynamics, but may not be enough to restore SR. Methods: Prospective randomized study aimed at evaluating efficacy of early direct current cardioversion (DCCV) following successful PBMV in patients with long-standing AF. Group 1 (n = 20) had patients of rheumatic MS with AF who underwent successful PBMV. Group 2 (n = 15) patients were DC cardioverted and administered oral Amiodarone for 6 weeks. Primary endpoint was maintenance of SR after 6 months. Secondary endpoints were functional capacity, number of embolic episodes, adverse drug effects, and all-cause mortality. Results: In Group 2, all patients underwent successful cardioversion. At a mean follow-up of 7.6 months, 95% in Group 1 were in AF. In Group 2, 87% patients were in SR and 13% had reverted to AF. Difference in rate of SR was 0.82 (95% CI 0.2, 1.01) (p = 0.001), with relative risk of 7.1 (1.95, 25.9, 95% CI, p = 0.001) for patients to be in AF who underwent only successful PBMV, i.e. Group 1. There was significant improvement in quality of life (SF36) score in Group 2 (p = 0.001), with no deaths, stroke, or adverse drug effects in either group. Conclusion: In patients with rheumatic MS and AF, early DCCV and a short-duration oral Amiodarone, following successful PBMV, may be a reasonable strategy to attain long-term SR.

Objectives: This study aims to analyze the results of comprehensive genetic testing in patients presenting to a dedicated multidisciplinary inherited heart disease clinic in India. Methods: All patients presenting to our clinic from August 2017 to October 2023 with a suspected inherited heart disease and consenting for genetic testing were included. The probands were grouped into familial cardiomyopathies namely hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic cardiomyopathy (ACM) and peripartum cardiomyopathy (PPCM), channelopathies namely congenital long QT syndrome (LQTS) and Brugada syndrome (BrS), and heritable connective tissue disorder namely Marfan Syndrome (MFS). Next generation sequencing (NGS) was used, and pre-test and post-test counseling were provided to probands and cascade screening offered to relatives. Results: Mean age of the subjects (n = 77; 48 probands, 29 relatives) was 43 ± 18 years, 68 % male and 44 % symptomatic, with 36 HCM, 3 DCM, 3 ACM, 1 PPCM, 3 LQTS, 1 BrS and 1 MFS probands. The diagnostic yield of NGS-based genetic testing was 31 %; variants of uncertain significance (VUS) were identified in 54 %; and 15 % were genotype-negative. Twenty-nine relatives from 18 families with HCM (n = 12), DCM (n = 3), ACM (n = 2) and MFS (n = 1) underwent genetic testing. The genotype positive probands/relatives and VUS carriers with strong disease phenotype and/or high risk variant were advised periodic follow-up; the remaining probands/relatives were discharged from further clinical surveillance. Conclusions: Genetic testing guides treatment and follow-up of patients with inherited heart diseases and should be carried out in dedicated multidisciplinary clinics with expertise for counseling and cascade screening of family members.