A 60-year-old man has experienced progressive shortness of breath over the last 6 months that is occasionally accompanied by chest pain. He was diagnosed with hypertension 10 years ago. Twenty years ago, he suffered from advanced-stage Hodgkin lymphoma that was treated with combination chemotherapy and radiation therapy. He is a lifetime nonsmoker. Blood pressure is 138/90 mm Hg and pulse is 78/min and regular. Physical examination shows a 2/4 early diastolic murmur at the left sternal border. Echocardiogram shows an enlarged left atrium, a normal-size left ventricle with an ejection fraction of 60%, and moderate diastolic dysfunction. Both the mitral and aortic valves appear sclerotic and calcified. The aortic root is normal-size but is echo bright. There is moderate aortic regurgitation. Cardiac catheterization shows ostial narrowing of the right and left main coronary arteries. Which of the following is the most likely diagnosis?

Question

A 60-year-old man has experienced progressive shortness of breath over the last 6 months that is occasionally accompanied by chest pain.  He was diagnosed with hypertension 10 years ago.  Twenty years ago, he suffered from advanced-stage Hodgkin lymphoma that was treated with combination chemotherapy and radiation therapy.  He is a lifetime nonsmoker.  Blood pressure is 138/90 mm Hg and pulse is 78/min and regular.  Physical examination shows a 2/4 early diastolic murmur at the left sternal border.  Echocardiogram shows an enlarged left atrium, a normal-size left ventricle with an ejection fraction of 60%, and moderate diastolic dysfunction.  Both the mitral and aortic valves appear sclerotic and calcified.  The aortic root is normal-size but is echo bright.  There is moderate aortic regurgitation.  Cardiac catheterization shows ostial narrowing of the right and left main coronary arteries.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Explanation: This patient most likely has radiation-induced cardiotoxicity. Long-term survivors of Hodgkin lymphoma are at risk of developing cardiovascular complications due to chemotherapy and/or radiation therapy. Radiation therapy causes diffuse fibrosis in the interstitium of the myocardium, along with progressive fibrosis of the pericardial layers, cells in the conduction system, and the cusps and/or leaflets of the valves. It also causes injury to the intimal layer, with arterial narrowing typically involving the ostial parts of coronary vessels. These effects lead to: Myocardial ischemia and/or infarction Restrictive cardiomyopathy with diastolic dysfunction Constrictive pericarditis Valvular abnormalities (mitral or aortic stenosis/regurgitation) Conduction defects (sick sinus syndrome or variable degrees of heart block) (Choice A) The anthracycline class of drugs causes a dose-dependent decline in the ejection fraction, leading to dilated cardiomyopathy. It is not associated with valvular disease or coronary artery disease. (Choice B) Chronic severe hypertension can lead to left ventricular hypertrophy with diastolic dysfunction; however, it is not associated with sclerosis/calcification of valves or ostial narrowing of the coronary arteries. (Choice D) Rheumatic heart disease causes progressive sclerosis of the mitral and/or aortic valve, causing mitral stenosis/regurgitation or aortic stenosis/regurgitation. The additional associated findings - diastolic dysfunction, echogenic aortic root, and ostial narrowing of the coronary arteries - are more consistent with radiation-induced cardiotoxicity. (Choice E) Secondary malignancy (eg, breast, lung or gastrointestinal cancer; acute leukemia, non-Hodgkin lymphoma) is the leading cause of death in survivors of Hodgkin lymphoma. There are no clinical findings suggesting a secondary malignancy in this patient. Educational objective: Clinical manifestations of radiation-induced cardiotoxicity include coronary disease with myocardial ischemia and/or infarction, restrictive cardiomyopathy with diastolic dysfunction, pericardial disease, valvular abnormalities, and/or conduction defects. References: Cardiac complications of oncologic therapy. (http://www.ncbi.nlm.nih.gov/pubmed/24108563) Clinically significant cardiac disease in patients with Hodgkin lymphoma treated with mediastinal irradiation. (http://www.ncbi.nlm.nih.gov/pubmed/20858859)

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