Key result
Cardiogenic stroke reveals rare overlap of ADPKD and LVNC cardiomyopathy in a 65-year-old man.
Why the study?
The association between non-compaction of ventricular myocardium and autosomal dominant polycystic kidney disease with cardiogenic cerebral embolism is rare and lacks standard treatment guidelines.
Case Report (n=1)
This case report highlights a rare association between autosomal dominant polycystic kidney disease and left ventricular non-compaction cardiomyopathy presenting with cardiogenic cerebral embolism.
Answers are grounded in this paper and cite their sources.
This case of LV non-compaction with ADPKD is hypothesis-generating; larger studies needed to confirm any association.
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.
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Rani et al. (2020) conducted a case report in Autosomal dominant polycystic kidney disease and left ventricular non-compaction cardiomyopathy (n=1). Guideline-directed pharmacotherapy for heart failure and anticoagulation was evaluated. A 65-year-old man presenting with cardiogenic cerebral embolism was diagnosed with the rare combination of autosomal dominant polycystic kidney disease and left ventricular non-compaction cardiomyopathy.
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