AJR Teaching File: High-Output Cardiac Failure in a Patient with a History of Hereditary Hemorrhagic Telangiectasia

Abstract

Case History A 62-year-old woman with a history of hereditary hemorrhagic telangiectasia (HHT) presents with high-output cardiac failure. Radiologic Description An anteroposterior chest radiograph (Fig. 1A) reveals cardio-megaly and enlargement of the central pulmonary vasculature, cephalization of pulmonary blood flow, and left pleural effusion. A single axial contrast-enhanced CT image of the chest obtained 2 years previously (Fig. 1B) shows an opacity in the right upper lobe that measures 10×9×15 mm. Pulmonary vessels are seen entering and exiting the opacity, which is suggestive of a pulmonary arteriovenous malformation. Transthoracic echocardiography, not shown, reveals a mildly to moderately dilated right ventricle, mild tricuspid valvular regurgitation, and a moderately to severely dilated right atrium. The left ventricle ejection fraction is estimated to be 65-75%. Axial images from the early arterial phase (Fig. 1C) and late arterial phase (Fig. ID) of contrast-enhanced CT of the abdomen show hepatomegaly, an enlarged inferior vena cava, and early opacification of the hepatic veins. Subcentimeter nodules that are too numerous to count fill the liver and show rapid enhancement, which is consistent with telangiectasia. No findings of cirrhosis or portal hypertension are evident. Differential Diagnosis The differential diagnosis for high-output cardiac failure is hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), arteriovenous fistula, chronic anemia, Paget's disease, hyperthyroidism, thiamine deficiency (beriberi), and poly-cythemia vera. Diagnosis The diagnosis is hereditary hemorrhagic telangiectasia. Commentary The differential diagnosis for high-output cardiac failure is broad. In addition to radiographic findings and clinical history, one can use laboratory data to help narrow the diagnosis. The patient's normal laboratory values, including hemoglobin and hematocrit, iron studies, thiamine, and thyroid function tests, help eliminate chronic anemia, hyperthyroidism, thiamine deficiency, and polycythemia vera from the differential diagnosis. Without a history of solid organ biopsy, penetrating trauma, or catheterization, an arteriovenous fistula would be much less likely. Paget's disease would manifest with diffuse osseous findings of blastic, lyric, or mixed lesions. A review of images showed that chest, abdomen, and pelvic CT images obtained at bone window settings were negative for such findings [1].