The PRKAG2 syndrome is a rare autosomal dominant phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM), characterized by ventricular pre-excitation, progressive conduction system disease and left ventricular hypertrophy. This study describes the phenotype, genotype and clinical outcomes of a South-Asian PRKAG2 cardiomyopathy cohort over a 7-year period. Clinical, electrocardiographic, echocardiographic, and cardiac MRI data from 22 individuals with PRKAG2 variants (68% men; mean age 39.5 ± 18.1 years), identified at our HCM centre were studied prospectively. At initial evaluation, all of the patients were in NYHA functional class I or II. The maximum left ventricular wall thickness was 22.9 ± 8.7 mm and left ventricular ejection fraction was 53.4 ± 6.6%. Left ventricular hypertrophy was present in 19 individuals (86%) at baseline. 17 patients had an WPW pattern (77%). After a mean follow-up period of 7 years, 2 patients had undergone accessory pathway ablation, 8 patients (36%) underwent permanent pacemaker implantation (atrio-ventricular blocks—5; sinus node disease—2), 3 patients developed atrial fibrillation, 11 patients (50%) developed progressive worsening in NYHA functional class, and 6 patients (27%) experienced sudden cardiac death or equivalent. PRKAG2 cardiomyopathy must be considered in patients with HCM and progressive conduction system disease.

This article examines the transition ritual (nirvanam) in a specific community of thirunangais, a regional transfeminine community characterized by ritual practices of worship and labor, to inquire into forms of religious worlding and subject- formation that take place at the margins of dominant systems of religion, citizenship, and gender. Unlike those who identify exclusively with the category of transgender, thirunangais’ formations of self and subjecthood draw not only from modern and secular discourses such as those of human rights and identity politics but also from religious discourses and practices. These involve embodied experiences of sacrifice and pain that are considered “premodern” and abject even within hegemonic norms of religion in India. Drawing from how thirunangai narratives of self construct the nirvanam as an encompassing assemblage of both ritual observances and more medicalized practices of sex reassignment, the article looks at how thirunangais consistently queer modern prescriptions of the relationship among political, private, and religious spheres. What can thirunangais tell us about those bodies, practices, and discourses that are seen as inimical to the constitution of the modern religious subject in postcolonial contexts?

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