A 62-year-old man arrives at the emergency room complaining of chest pain and difficulty breathing. He reports that the dyspnea started 2 months ago after he had the flu. At first, the difficulty breathing occurred whenever he went up and down 1 flight of stairs, but the dyspnea progressively worsened since then. This morning, he developed chest pain and difficulty breathing while sitting at the kitchen table. The patient’s medical history is significant for hypertension, type 2 diabetes mellitus, and rheumatoid arthritis. His medications include aspirin, lisinopril, metformin, and sulfasalazine. His temperature is 97°F (36.1°C), blood pressure is 130/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 97% O2 on room air. On physical exam, jugular venous distension during both inspiration and expiration, mild abdominal distention, and 2+ bilateral lower extremity edema is noted. A chest radiograph is obtained and the result is shown in Figure A. Which of the following is the most likely cause of this patient's symptoms?
Restrictive cardiomyopathy
Cirrhosis
Constrictive pericarditis
Cardiac tamponade
Constrictive pericarditis
This patient is presenting with peripheral edema, ascites, elevated jugular venous distension, and pericardial calcifications on chest radiograph, which are all consistent with a diagnosis of constrictive pericarditis.

Constrictive pericarditis is the result of scarring and loss of elasticity of the pericardial sac. Common etiologies include idiopathic, post-viral, post-cardiac surgery, post-radiation, tuberculosis-related, or connective tissue disorder-related. It presents as right heart failure including symptoms such as dyspnea, chest pain relieved by leaning forward, progressive peripheral edema, and ascites. A physical exam will identify an early diastolic pericardial knock, pericardial calcifications on chest radiograph, and jugular venous distension (JVD). Kussmaul sign can also occur where the JVD paradoxically rises during inspiration instead of falling. The diagnosis is supported with echocardiography. Management may require pericardiectomy for severe cases.

Welch and Oh review the evidence regarding the diagnosis and treatment of constrictive pericarditis. They discuss how this disease can be evaluated using a cardiac MRI. They recommend surgical pericardiectomy in many cases.

Figure/Illustration A is a frontal chest radiograph with pericardial calcifications (red circle). These findings are consistent with constrictive pericarditis.

Incorrect Answers:
Answer A: Cardiac tamponade can present similarly to constrictive pericarditis. Hypotension and pulsus paradoxus (a greater than 10 mmHg decrease in the systolic blood pressure during inspiration), will commonly present in patients with cardiac tamponade. A chest radiograph will show an enlarged cardiac silhouette rather than calcifications in some cases. Treatment is with emergent pericardial decompression.

Answer B: Cirrhosis can present with ascites and peripheral edema, but will not have a marked elevation in jugular venous pressure. Cirrhosis is the end-stage result of many hepatic pathologies. Treatment is with the control of sequelae such as hepatic encephalopathy and venous congestion.

Answer D: Pulmonary arterial hypertension, like constrictive pericarditis, can present with exertional dyspnea, chest pain, and peripheral edema if it progresses to right ventricular failure. A chest radiograph may show enlarged central pulmonary arteries and peripheral pulmonary vessels. Treatment is with reduction of pulmonary pressure such as with endothelin inhibitors.

Answer E: Restrictive cardiomyopathy can also present as right heart failure with similar symptoms to constrictive pericarditis. A history of an infiltrative disease (e.g., amyloidosis and sarcoidosis) often favors restrictive cardiomyopathy, as does an audible S3 on a physical exam. Calcification of the pericardium on the chest radiograph is more consistent with constrictive pericarditis.

Bullet Summary:
Constrictive pericarditis presents with symptoms of right heart failure, progressive peripheral edema, ascites, jugular venous distension, pericardial knock, and pericardial calcifications on a chest radiograph.