9: Hypertrophic Cardiomyopathy: Pathology USMLE Step 1

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Hypertrophic Cardiomyopathy (HCM), Dilated Cardiomyopathy (DCM), and Restrictive Cardiomyopathy (RCM) for USMLE Step 1
Hypertrophic Cardiomyopathy (HCM):
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Pathophysiology: HCM is characterized by asymmetric hypertrophy of the myocardium, often affecting the interventricular septum. Mutations in sarcomere proteins lead to abnormal myocardial contractility and impaired relaxation.
Clinical Features: Patients may present with exertional dyspnea, chest pain, syncope, or sudden cardiac death, particularly in young athletes. Physical examination may reveal a harsh systolic murmur, known as a crescendo-decrescendo murmur, heard best at the left sternal border.
Diagnosis: Echocardiography demonstrates left ventricular hypertrophy and dynamic outflow tract obstruction. Genetic testing may identify mutations in sarcomere genes. ECG may show left ventricular hypertrophy and T-wave abnormalities.
Treatment: Management includes beta-blockers or calcium channel blockers for symptom relief, with consideration for surgical myectomy or alcohol septal ablation in refractory cases. Avoidance of strenuous exercise is recommended.
Dilated Cardiomyopathy (DCM):
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Pathophysiology: DCM is characterized by dilatation of the left or both ventricles, leading to impaired systolic function and heart failure. Etiologies include genetic mutations, viral infections, autoimmune disorders, and toxic exposures.
Clinical Features: Patients may present with symptoms of heart failure such as dyspnea, fatigue, and peripheral edema. Physical examination may reveal signs of volume overload such as displaced apical impulse and S3 gallop.
Diagnosis: Echocardiography demonstrates dilated ventricles with reduced ejection fraction. Cardiac MRI may provide additional information about myocardial tissue characteristics. Endomyocardial biopsy may be performed to identify underlying etiologies.
Treatment: Management focuses on optimizing heart failure therapy with ACE inhibitors, beta-blockers, diuretics, and aldosterone antagonists. Device therapy such as implantable cardioverter-defibrillator (ICD) or cardiac resynchronization therapy (CRT) may be indicated in select cases. Heart transplantation may be considered for end-stage disease.
Restrictive Cardiomyopathy (RCM):
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Pathophysiology: RCM is characterized by impaired ventricular filling due to stiff and non-compliant myocardium. Etiologies include infiltrative diseases (e.g., amyloidosis, sarcoidosis), storage disorders (e.g., hemochromatosis), and idiopathic fibrosis.
Clinical Features: Patients may present with symptoms of heart failure such as dyspnea on exertion, peripheral edema, and ascites. Physical examination may reveal elevated jugular venous pressure, Kussmaul's sign (lack of inspiratory decline in jugular venous pressure), and hepatomegaly.
Diagnosis: Echocardiography demonstrates bi-atrial enlargement, normal or near-normal ventricular size, and preserved ejection fraction. Cardiac MRI or CT may provide additional information about myocardial tissue characteristics. Endomyocardial biopsy is often required to establish the underlying etiology.
Treatment: Management aims to alleviate symptoms with diuretics and optimize management of underlying disorders. Prognosis varies depending on the underlying etiology, with poor outcomes in infiltrative diseases such as amyloidosis.
Understanding the pathophysiology, clinical features, diagnostic criteria, and management principles of HCM, DCM, and RCM is essential for medical students preparing for USMLE Step 1 and future clinical practice. 📚
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[@SuryaPrakash-vb3lx]

Excellent video,keep it up

[@doctorbhanuprakash]

Thanks a lot

[@mohamedabdinasir9733]

Thnks Dr i understand this lecture, but i went verry small your writting please you write the clearly.