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Unexpected sudden death in pregnancy - arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report.

ABSTRACT: Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries. Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive disease, undiagnosed pulmonary hypertension and cardiomyopathies being recognized as major health problems in these settings. This article reported a 27-year-old female who was normal on clinical examination and basic investigations, and on an antenatal visit was found collapsed in the toilet of her house and was pronounced dead on admission to hospital. She was found to be in the 11th week of pregnancy and had no history of significant illness in the past. Autopsy did not reveal any obvious macroscopic pathology except for a significant amount of epicardial fat infiltrating into myocardium of right ventricle. Detailed histopathological examination of the heart demonstrated fibro-fatty replacement of the heart muscle. The cause of death was arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). ARVC/D can cause unexpected sudden death during pregnancy. Therefore, it is recommended that an ECG and echocardiogram be included as screening tests during antenatal follow-up to minimize preventable cardiac deaths like ARVC/D.

SUBMITTER: Vadysinghe AN

PROVIDER: S-EPMC6197088 | biostudies-other | 2017

REPOSITORIES: biostudies-other

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Unexpected sudden death in pregnancy - arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report.

Vadysinghe Amal Nishantha AN Jayasooriya Rankothge Pemasiri RP Gunatilake G Keerthi Kumara GKK Sivasubramanium Murugupillai M

Forensic sciences research 20170523 3

Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries. Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive disease, undiagnosed pulmonary hypertension and cardiomyopathies being recognized as major health problems in these settings. This article reported a 27-year-old female who was normal on clinical examination and basic investigations, and on an antenatal visit was found collapsed in ...[more]

PMID: 30483636

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Project description:BackgroundArrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD.MethodsWe assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (?30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping.ResultsA total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77-10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69-0.80) and calibration slope of 0.95 (95% CI, 0.94-0.98) indicating minimal over-optimism.ConclusionsLTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

Dataset Information

Unexpected sudden death in pregnancy - arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report.

Publications

Unexpected sudden death in pregnancy - arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report.

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