Radiological Category Thoracic Cardiovascular Principal Modality 1 General

Radiological Category: Thoracic Cardiovascular Principal Modality (1): General Radiography; Nuclear Medicine Principal Modality (2): Cardiac-gated CT Angiography Case Report 711 Submitted by: Ravinder Legha, MD Faculty reviewer: Eduardo Matta, MD The University of Texas Medical school at Houston Date accepted: 14 April 2010

Case History 41 year-old male with congestive heart failure (CHF) presents with increasing shortness of breath. -PMHx: alcohol use, hypertension. -CXR, pharmacologic stress test, and cardiac-gated CTA performed.

Radiological Presentations Cardiomegaly with blunting of right costophrenic sulcus.

Radiological Presentations Mild reversible septal perfusion defect. Scar of mid and basal segments of the inferior wall. No reversible ischemia. Enlarged, dilated left ventricle. Global left ventricular wall hypokinesis. EF (ejection fraction) 14%.

Radiological Presentations Right ventricle Tricuspid valve Right atrium Left ventricle Mitral valve Left atrium Global cardiomegaly with enlargement of all 4 chambers without evidence of coronary artery disease, wall-thinning, valvular abnormalities, or asymmetry of the interventricular septum.

Radiological Presentations Global cardiomegaly with enlargement of all 4 chambers KEY Orange: Left ventricle Purple: Right ventricle Yellow/Green: Left atrium Teal: Right atrium Red: Ascending aorta

Radiological Presentations Aortic valve Mitral valve Normal aortic and mitral valves.

Radiological Presentations 1 3 1 2 4 2 Systole Views 1: 2 chamber 2: 3 chamber (LVOT) 3: Short axis 4: 4 chamber 3 4 Diastole Minimal change in the volume of the left ventricle between systole and diastole represents global hypokinesis with EF 20%.

Radiological Presentations Minimal change in the volume of the left ventricle between systole and diastole represents global hypokinesis with EF 20%

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? • Dilated cardiomyopathy. • Hypertrophic cardiomyopathy. • Valvular heart disease.

Findings and Differentials Findings: Cardiomegaly and right pleural effusion on chest X-ray. Pharmacologic stress test demonstrates enlarged, dilated left ventricle with global left ventricular wall hypokinesis and EF 14%; Mild reversible septal perfusion defect and scarring of mid and basal segments of the inferior wall. Cardiac-gated CTA demonstrates global cardiomegaly and global hypokinesis without evidence of coronary artery disease and LVEF 20%. Differentials: • Dilated cardiomyopathy. • Hypertrophic cardiomyopathy. • Restrictive cardiomyopathy. • Valvular heart disease.

Discussion -Restrictive cardiomyopathy (CM) occurs when there is increased resistance to ventricular filling due to abnormal myocardial stiffness. There is biatrial enlargement with normal ventricular size, diastolic dysfunction, and CXR findings of CHF without cardiomegaly. -Hypertrophic CM occurs with ventricular hypertrophy with increased left ventricular mass without an obvious etiology. Hypertrophy tends to be asymmetric involving basal interventricular septum. -Valvular heart disease: -chronic severe MR, AR: result in increased ventricular volume and decreased systolic function. -end-state AS: results in left ventricular pressure overload, a dilated left ventricle, and decreased systolic function. -Dilated CM demonstrates ventricular dilatation and decreased systolic function when no atherosclerotic disease is present.

Diagnosis Dilated cardiomyopathy: -Most patients age 20 -60, more common in men and African Americans. -Presents with biventricular failure with progressive and gradual dyspnea with exertion, impaired exercise capacity, paroxysymal nocturnal dyspnea, orthopnea, and peripheral edema. -Multiple etiologies: idiopathic, familial, myocarditis, ischemic heart disease, infiltrative disease (amyloid, carcoid), peripartum, HTN, infection, connective tissue disease, toxic (alcohol, cocaine), chemotherapy (doxorubicin), metabolic (hypophosphatatemia, hypocalcemia, uremia), endocrinopathies. -Prognosis related to severity of LV dysfunction. -Tx: -Treat reversible causes. -Medication: ACE inhibitors, beta-blockers, diuretics, digoxin, anticoagulation. -AICD (if EF < 40%) -Cardiac transplantation.

References Abbara, et al. “Pericardial and Myocardial Disease. ” Cardiac Imaging: The Requisites. 2 nd Ed. Mosby. 270 -2, 2005. Cooke, et al. “Idiopathic Dilated Cardiomyopathy. ” The New England Journal of Medicine. 332: 1384 -6. 1995. Holden, et al. “Dilated Cardiomyopathy. ” Stat-Dx: Diagnosting Imaging for Radiology. Amirsys. 2010. Webb, et al. Fundamental of Body CT, 2006.