The NCCPA™ PANCE Cardiology System Content Blueprint includes 4 different types of cardiomyopathy
PANCE Blueprint Roadmap
Cardiomyopathy translates to “heart muscle disease,” so cardiomyopathy is a broad term used to describe various issues resulting from disease of the myocardium or heart muscle. | |
Types of cardiomyopathies
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Impaired Systolic Function | |
Dilated Cardiomyopathy | Patient will present as → a 68-year-old patient who comes to the office because of increased shortness of breath for four months. His symptoms are particularly bad at night. Medical history includes long-standing hypertension and alcoholism. Examination shows a displaced apex beat and normal breath sounds. Cardiac auscultation shows an S3 gallop and a pan-systolic murmur radiating to the axilla. The chest X-ray shows an enlarged left ventricular shadow. Dilated cardiomyopathy is the most common cardiomyopathy (~90%), with left ventricular (LV) dilation and systolic dysfunction (EF <40%)
"An S3 gallop signifies the end of rapid ventricular filling in the setting of fluid overload and is often associated with dilated cardiomyopathy." DX: Echocardiography is the most definitive diagnosis - demonstrates left ventricular dilation and systolic dysfunction, and low cardiac output with poor EF (<40%)
TX: BAMD (Beta-blockers, ARNI (or ACEi/ARB), MRAs, Diuretics/SGLT2i
Start sequentially (e.g., ARNI + beta-blocker first, then MRA, SGLT2i)
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Impaired Diastolic Function | |
Hypertrophic Obstructive Cardiomyopathy (HOCM) | Patient will present as → a 25-year-old man is brought to the ED because he collapsed while playing tennis 20 minutes ago. Medical history includes unexplained chest pain and shortness of breath while exercising for three years. Family history includes an uncle who died of an unknown cardiac pathology at the age of 23. Cardiac auscultation shows a 2/6 systolic murmur is heard at the left of the sternum between the first two ribs. The murmur becomes louder when the patient performs a Valsalva maneuver and decreases with squatting. The hypertrophic portion of the septum - LV outflow tract is narrowed - during systole, and obstruction worsened with increased contractility Presentation: A young athlete with a positive family history has a sudden death or syncopal episode
Physical Exam:
"The murmur due to HCM will increase in intensity with any maneuver that decreases the volume of blood in the left ventricle (such as standing abruptly or the strain phase of a Valsalva maneuver ). " DX: Diagnosis is by echocardiography or MRI
TX: β-blockers (metoprolol) and/or rate-limiting Ca channel blockers (usually verapamil) to decrease myocardial contractility and slow the heart rate and thus prolong diastolic filling and decrease outflow obstruction
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Restrictive Cardiomyopathy | Patient will present as → a 58-year-old man complaining of several months of worsening shortness of breath and ankle swelling. He denies palpitations, lightheadedness, syncope, or chest pain. He has a past medical history significant for hereditary hemochromatosis. On physical exam, his temperature is 37 C (98.6 F), pulse is 78, blood pressure is 130/72 mm Hg, and respiratory rate is 16. He has elevated jugular venous pressure, diminished breath sounds at the lung bases, tender hepatomegaly, and bilateral pitting ankle edema. There are no murmurs, rubs, or gallops. EKG shows low-voltage QRS complexes without any signs of ischemia. His chest x-ray shows a normal-sized heart and bilateral pleural effusions. Echocardiography shows symmetrical thickening of the left ventricle, normal left ventricular volume, and mildly reduced systolic function. Right heart failure with a history of an infiltrative process
DX: Echocardiography shows a normal left ventricular ejection fraction. Common findings include dilated atria and myocardial hypertrophy
TX: often unsatisfactory unless the cause can be addressed
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Stress cardiomyopathy | Patient will present as → a 62-year-old female arrives with sudden chest pain and shortness of breath after receiving distressing news. She has hypertension, is postmenopausal, and appears anxious but hemodynamically stable. ECG shows anterior ST elevations, and troponin is mildly elevated but out of proportion to the ECG findings. Coronary angiography reveals no significant stenosis. An echocardiogram demonstrates apical ballooning with preserved basal function, confirming stress cardiomyopathy, a transient left ventricular dysfunction due to intense emotional stress. She is started on beta-blockers and an ACE inhibitor to support myocardial recovery. Anticoagulation is considered if an apical thrombus is detected. The patient is counseled that the prognosis is excellent with appropriate treatment, and a repeat echocardiogram is planned in 4-6 weeks to confirm resolution. Stress Cardiomyopathy (Takotsubo Syndrome) is a transient, stress-induced cardiomyopathy characterized by sudden, reversible left ventricular dysfunction, often mimicking acute coronary syndrome (ACS)
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