Two Cases of High Output Heart Failure Caused by Hereditary Hemorrhagic Telangiectasia
A 68-year-old woman came to the hospital because of dyspnea {New York Heart Association (NYHA) class 3}. On physical examination, the patient was puffy and anemic. Multiple telangiectases were found on the lips, tongue and finger tips (). Her blood pressure was 100/70 mm Hg, she had a heart rate of 94/min, and a respiratory rate of 22/min. Cardiac examination revealed a midsystolic murmur along the sternal border and a bruit was audible over the epigastrium. Her medical history was positive for recurrent epistaxis and multiple episodes of gastrointestinal bleeding. She stated that her family members (her mother, one sister and one daughter) also had suffered from recurrent epistaxis.\nThe laboratory work-up revealed hypochromic microcytic anemia (hemoglobin 8.8 g/dL), normal liver function tests, and normal prothrombin time. Chest X-ray showed generalized cardiomegaly (CT ratio 65%) with increased pulmonary vascular markings and pulmonary edema (). Transthoracic echocardiogram showed a dilated hypercontractile left ventricle and a restrictive mitral inflow pattern (mitral E to A ratio=1.3, E/e=16.6). CT angiography of the abdomen showed a tortuous and dilated hepatic artery and near total replacement of liver parenchyma by engorged vascular structures with arteriovenous shunt formation (). Cardiac output by right heart catheterization was 12.47 L/min, capillary wedge pressure was 22 mm Hg and pulmonary artery pressure was 45/18 mm Hg. MRI of the brain with contrast showed an aneurysm (7 mm) in the right paraclinoid intracranial artery (ICA) and was managed with Guglielmi detachable coils (MicroVention, Aliso Viejo, CA, USA). After correction of anemia by iron supplements and treatment with angiotensin receptor blocker and diuretics, heart failure symptoms were significantly improved.