HIGH OUTPUT CARDIAC FAILURE DUE TO HEREDITARY HEMORRHAGIC TELANGIECTASIA

Bilal Saeed; Raj Dangol; Tolulope Agunbiade

doi:10.1016/S0735-1097(22)04370-4

Background: High output cardiac failure (HOCF) is a rare complication of Hereditary Hemorrhagic Telangiectasia (HHT). Hepatic arteriovenous fistulae (AVFs) occur in up to two-thirds of patients with HHT and are usually clinically silent. However, patients with large hepatic AVFs can present with recurrent heart failure exacerbations. Case: A 75-year-old man with a history of iron deficiency anemia and two prior hospital admissions for heart failure exacerbations presented to our hospital with worsening dyspnea on exertion and bilateral lower extremity edema for 3 weeks duration. Transthoracic echocardiogram showed left ventricular ejection fraction of 70-75%, increased velocity across the aortic valve, and left ventricular outflow tract suggestive of hyperdynamic circulation. He was diuresed with subsequent improvement in his symptoms. Given repeated heart failure exacerbations, he underwent right heart catheterization that showed elevated intra-cardiac pressures. A shunt run was also performed which showed a significant step up of oxygen saturations from the IVC to right atrium raising the question of intra-hepatic shunting. Cardiac MRI was negative for intra-cardiac shunt. Liver ultrasound was highly suggestive of intra-hepatic AVFs, and he was referred for interventional radiology guided hepatic angiography that showed multiple intra-hepatic AVFs. Embolization of the middle hepatic artery was performed. The patient later verbalized that he and his daughter have a history of recurrent epistaxis. He underwent nasal endoscopy that revealed multiple telangiectasias. He was then referred to an HHT center at a tertiary hospital, where he underwent genetic tests that confirmed the diagnosis of HHT. Decision-making: In our patient, dyspnea and hyperdynamic circulation were initially attributed to iron deficiency anemia with diastolic heart failure. But given repeated heart failure exacerbations, he underwent further testing, which eventually led to a diagnosis of HHT, resulting in HOCF. Conclusion: Recurrent heart failure exacerbations might be a rare manifestation of HHT. High index clinical suspicion and appropriate testing can lead to early detection and proper management.

HIGH OUTPUT CARDIAC FAILURE DUE TO HEREDITARY HEMORRHAGIC TELANGIECTASIA

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