Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ KEY FEATURES +++ ESSENTIALS OF DIAGNOSIS ++ Sclerotic skin, esophageal dysfunction, Raynaud’s phenomenon Diastolic heart failure Functional and structural small-vessel coronary artery disease (CAD) Multisegmental myocardial perfusion abnormalities in the absence of epicardial CAD Pulmonary hypertension and cor pulmonale +++ GENERAL CONSIDERATIONS ++ Scleroderma or progressive systemic sclerosis is characterized by excessive accumulation of connective tissue and fibrosis of the skin, skeletal muscles, joints, blood vessels, kidneys, lung, gastrointestinal tract, and heart Scleroderma occurs in 10–20 per million people per year, is more common in women (3:1), and usually occurs in people between ages 30 and 50 years Ninety percent of patients with scleroderma have Raynaud’s phenomenon, esophageal dysfunction, and sclerotic skin changes in a focal (80%) or a diffuse pattern (20%) – The more common focal type is associated with the CREST syndrome (calcinosis, Raynaud’s, esophageal dysfunction, sclerodactyly, and telangiectasia) – Skin changes are often limited to the face and fingers – The diffuse type more frequently involves the abdominal and thoracic organs The diffuse type has a worse prognosis than the focal type The major causes of mortality are pulmonary hypertension, renal dysfunction, and heart disease The cardiac diseases most commonly associated with scleroderma include coronary artery disease, myocarditis, and pulmonary hypertension +++ CLINICAL PRESENTATION +++ SYMPTOMS AND SIGNS ++ Chest pain is common but is more often due to pericarditis or esophageal reflux than to myocardial ischemia Dyspnea, orthopnea, and edema +++ PHYSICAL EXAM FINDINGS ++ Typical skin findings of scleroderma, telangiectasia, subcutaneous calcified nodules Signs of heart failure, such as cardiomegaly, S3, jugular venous distention, pulmonary rales, and edema Pericardial friction rub, pulsus paradoxus, fever Right ventricular lift, loud pulmonic component of S2 with pulmonary hypertension +++ DIFFERENTIAL DIAGNOSIS ++ Typical coronary artery disease Other causes of myopericarditis and heart failure Other causes of pulmonary hypertension and cor pulmonale +++ DIAGNOSTIC EVALUATION +++ LABORATORY TESTS ++ Elevated cardiac biomarkers in myocarditis or infarction Exudative pericardial effusion +++ ELECTROCARDIOGRAPHY ++ Septal Q waves usually due to fibrosis rather than infarction Signs of acute pericarditis Conduction abnormalities Nonspecific ST-T–wave changes Right ventricular hypertrophy +++ IMAGING STUDIES ++ Chest x-ray: enlarged cardiac silhouette Echocardiography may show the following common findings: – Diffuse left ventricular dysfunction or segmental wall motion abnormalities – Left ventricular hypertrophy – Left atrial enlargement – Doppler evidence of diastolic dysfunction – Nonspecific valve thickening and regurgitation – Also seen are pericardial effusion, signs of tamponade, and right ventricular and atrial hypertrophy Rest or exercise radionuclide myocardial perfusion imaging may show multisegmental perfusion defects CT and cardiac MRI are useful for evaluating pericardial thickness +++ DIAGNOSTIC PROCEDURES +... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free a profile for additional features.