A 20-year-old male patient collapses and dies suddenly while jogging. Autopsy reveals significant left ventricular hyperophy predominantly affecting the septum. There are no valvular abnormalities. The patient’s family history is significant for an uncle who died suddenly at an age of 20 years. The gross examination of hea demonstrates the following finding. Assuming this patient died of an inherited condition, the most likely finding in would be?
Correct Answer: <img style="max-width: 100%" src=" />
Description: Sudden death in a previously asymptomatic, young, exercising individual with significant family history should raise strong suspicions of hyperophic cardiomyopathy. The autopsy finding of asymmetric septal hyperophy confirms the diagnosis. Hyperophic cardiomyopathy is due to autosomal dominant mutation involving the gene coding for beta myosin heavy chain. Histopathological findings are: Massive myocyte hyperophy Myocyte disarray Interstitial fibrosis Option A: Amyloidosis: (hemato xylin and eosin stain) shows glassy, grey-pink amorphous material infiltrating between cardiac myocytes. Option B: Fabry's disease: Transmission electron micrograph (myocardial biopsy specimen) showing the characteristic concentric lamellar inclusions of glycosphingolipids (accumulating due to deficient lysosomal enzyme alpha-galactosidase A). Option C: Sarcoidosis: Microscopic image (endomyocardial biopsy) showing a non-caseating granuloma and interstitial fibrosis suggestive of sarcoidosis. Option D: Hyperophic cardiomyopathy: Microscopic image of HCM showing the characteristic disarrayed myocyte architecture with swirling and branching. Myocyte nuclei are of variable size and interstitial fibrosis is present.
Category:
Unknown
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now