Non-compaction of ventricular myocardium with polycystic kidney disease with cardiogenic cerebral embolism

Abstract

Non-compaction of ventricular myocardium is a rare cardiomyopathy involving an early arrest of normal compaction of myocardium during fetal ontogenesis. Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary nephropathy characterised by multiple renal cysts replacing the renal parenchyma and extrarenal manifestations. Here, we report a case of 65-year-old man, chronic smoker, presented with sudden onset right brachial monoparesis, exertional dyspnoea, orthopnoea, bipedal swelling and diagnosed as a case of ADPKD with left ventricular non-compaction cardiomyopathy with acute left ventricular failure and cardiogenic cerebral embolism (no evidence of atrial fibrillation); based on characteristic appearance on two-dimensional echocardiography and cardiac magnetic resonance. The patient was managed with guideline-directed pharmacotherapy for heart failure and anticoagulation as a secondary stroke prevention measure. Through this case report, we try to discuss the association between two rare entities and individualisation of treatment options available as a case-based approach, as no standard treatment guidelines are available.