A 64-year-old man comes to the physician due to shortness of breath and abdominal distension. He was treated for Hodgkin lymphoma with radiation and chemotherapy 18 years ago and was told that he was cured. The patient drinks alcohol on a regular basis. His temperature is 36.7 C (98 F), blood pressure is 120/76 mm Hg, pulse is 92/min, and respirations are 20/min. Neck examination shows jugular venous pulsations 9 cm above the sternal angle. The abdomen is distended with a positive fluid wave. The liver edge is palpated 5 cm below the right costal edge. There is bilateral lower-extremity pitting edema. Initial laboratory results are as follows:
Serum creatinine 0.8 mg/dL Albumin 4.0 g/dL Total bilirubin 1.0 mg/dL Prothrombin time 11 sec
Which of the following is the most likely cause of this patient's condition?
Features of constrictive pericarditis | |
Etiology |
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Clinical presentation |
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Diagnostic findings |
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*Increase in JVD with inspiration. JVD = jugular venous distension. | |
This patient's presentation is suggestive of right heart failure, most likely due to constrictive pericarditis, a potential late complication of radiation therapy. Survivors of Hodgkin lymphoma are at increased risk for cardiac disease, which can present as much as 10-20 years or more after mediastinal irradiation and/or anthracycline therapy. Potential cardiac complications of Hodgkin lymphoma include acute or delayed pericardial disease, myocardial ischemia/infarction, restrictive cardiomyopathy, congestive heart failure, valvular abnormalities, and conduction defects.
Constrictive pericarditis occurs as a result of scarring and subsequent loss of normal elasticity of the pericardial sac. The inelastic pericardium prevents venous return to the right heart during inspiration and leads to right heart failure. Patients typically present with peripheral edema, ascites, and hepatic congestion with hepatomegaly, which can progress to cirrhosis (cardiac cirrhosis). Physical examination shows elevated jugular venous pressure (JVP) with prominent x and y descents and hepatojugular reflux, Kussmaul's sign (lack of decrease or increase in JVP on inspiration), or pericardial knock (mid-diastolic sound).
Patients may have pericardial calcifications on chest radiograph. Echocardiography confirms the diagnosis and typically shows increased pericardial thickness, abnormal septal motion, and biatrial enlargement. Diuretics can provide temporary relief, and pericardiectomy is the definitive treatment for patients with refractory symptoms.
(Choices B and C) Patients with portal vein thrombosis/compression are usually asymptomatic or develop signs and symptoms related to portal hypertension. Predisposing conditions for portal vein thrombosis include decompensated cirrhosis or hypercoagulable states. JVP is typically not elevated.
(Choice D) Obstruction of the thoracic duct (or its tributaries) leads to leakage of lymphatic fluid (chyle) into the pleural space, resulting in unilateral or bilateral chylothorax. Ascites and hepatomegaly are not associated with thoracic duct obstruction.
(Choice E) Heavy proteinuria and hypoalbuminemia can cause peripheral and/or generalized edema (ie, anasarca). Patients with severe hypoalbuminemia can develop intravascular volume depletion, and JVP is often reduced or normal. This patient's serum albumin is within normal limits.
(Choice F) Lower-extremity venous valve incompetence can lead to dependent edema, skin pigmentation, dermatitis/eczema, and venous ulcerations. Jugular venous distension, ascites, and hepatomegaly are typically not present.
Educational objective:
Constrictive pericarditis is a complication of mediastinal irradiation and an important cause of right heart failure. It should be suspected in patients with progressive peripheral edema, elevated jugular venous pressure, hepatomegaly, and ascites. Other findings include the presence of hepatojugular reflux, Kussmaul's sign (lack of decrease or increase in jugular venous pressure on inspiration), and a pericardial knock (mid-diastolic sound).