A 69-year-old man comes to the office due to 2 days of increasing shortness of breath, cough, and lower-extremity edema. He drinks a half of a bottle of vodka daily and has a 45-pack-year smoking history. There is no past history of hypertension or ischemic heart disease. The patient has a sedentary lifestyle due to disability from a work injury at age 20. He takes no medications and has not seen a physician in 20 years. His blood pressure is 130/90 mm Hg, pulse is 90/min, and oxygen saturation is 90% on room air. Cardiac examination shows faint heart sounds. Jugular venous pressure is 11 cm H₂O. Lung auscultation shows decreased breath sounds. The liver span is 18 cm and ascites is present. There is bilateral 3+ lower-extremity pitting edema up to the knees. Chest x-ray reveals enlarged central pulmonary arteries without evidence of vascular congestion. Which of the following is the most likely diagnosis?

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11ec25c8_d4b0_8dc7_bc81_1d82f8fee335_MD0003_00 This patient's presentation suggests cor pulmonale, defined as right-sided heart failure (RHF) from pulmonary hypertension (PH). PH may be idiopathic or due to chronic obstructive pulmonary disease (COPD), interstitial lung disease (eg, idiopathic pulmonary fibrosis), obstructive sleep apnea, pulmonary vascular disease (eg, thromboembolic), or chest wall disorders (eg, kyphoscoliosis). RHF due to left-sided or congenital heart disease is not considered cor pulmonale. COPD is the most common cause of cor pulmonale in the United States, with nearly 25% of COPD patients developing this disorder. Cor pulmonale typically has a gradual onset but can present acutely due to a sudden increase in pulmonary artery pressures (eg, pulmonary embolism). Patients often develop exertional symptoms (eg, dyspnea, angina, syncope). Physical examination may show loud P2 (pulmonic component of the 2nd heart sound), tricuspid regurgitation murmur (holosystolic at the left lower sternal border), elevated jugular venous pressure (JVP), peripheral edema, hepatomegaly due to hepatic congestion, and possible ascites. COPD patients usually have distant heart sounds due to hyperinflated lungs. End-stage cor pulmonale may present with hypotension, tachycardia, and other signs of cardiogenic shock due to decreased stroke volume. Chest x-ray may show enlarged central pulmonary arteries and loss of retrosternal air space due to right ventricular hypertrophy. ECG usually shows right axis deviation, right bundle branch block, right ventricular hypertrophy, and right atrial enlargement. Right heart catheterization is the gold standard for diagnosis and typically shows elevated central venous pressure, right ventricular end-diastolic pressure, and mean pulmonary artery pressure (≥25 mm Hg at rest) without left heart disease. Treatment involves optimizing right ventricular dynamics (preload, afterload, and contractility) with supplemental oxygen, diuretics, treatment of underlying etiology, and intravenous inotropes for severe decompensation. (Choice A) The cirrhotic liver is characteristically shrunken and fibrotic, but may occasionally be normal-sized or enlarged, and is often accompanied by splenomegaly. Stigmata of chronic liver disease and other symptoms of hepatic decompensation are typically present. This patient's smoking history, cough, lung findings, and elevated JVP make cor pulmonale due to COPD more likely. (Choice B) Cardiac tamponade is a life-threatening condition caused by a rapidly developing pericardial effusion that impairs cardiac filling. Classic findings include jugular venous distension, hypotension, distant heart sounds, and pulsus paradoxus (>10 mm Hg decrease in systemic blood pressure with inspiration). (Choice D) Coronary artery disease may cause left-sided or biventricular cardiac failure with evidence of pulmonary vascular congestion (eg, pulmonary edema on chest x-ray). Inferior wall myocardial infarction may cause isolated right-sided heart failure; however, these patients typically present with chest pain, nausea, and diaphoresis along with hypotension, elevated jugular venous distension, and clear lungs. (Choice E) Metastatic carcinoma of the liver can cause hepatomegaly (usually irregular hepatomegaly) and ascites but is not typically associated with dyspnea or elevated JVP, as seen in this patient. Educational objective: Cor pulmonale refers to isolated right-sided heart failure (RHF) from pulmonary hypertension, most commonly due to chronic obstructive pulmonary disease. Signs of RHF include jugular venous distension, increased intensity of P2 (pulmonic component of the 2nd heart sound), right ventricular heave, hepatomegaly, dependent pitting edema, and possible ascites. References: Cor pulmonale: an overview (http://www.ncbi.nlm.nih.gov/pubmed/16088545)

A 69-Year-Old Man Comes to the Office Due to 2