Pulmonary Venous Aneurysms in Hereditary Hemorrhagic Telangiectasia Detected by 3-Dimensional Magnetic Resonance Angiography
A 38-year-old woman with a history of presumed multiple pulmonary arteriovenous malformations (PAVMs), epistaxis, and hereditary hemorrhagic telangiectasia (HHT) presented with a 2-year history of progressive dyspnea on exertion. She had no orthopnea, paroxysmal nocturnal dyspnea, edema, or exertional chest discomfort. She denied hemoptysis and seizure activity but complained of severe migraine headaches. Her medical history was otherwise unremarkable. Her family history was notable in that her mother died at age 44 from respiratory failure of unknown etiology; a sister has multiple PAVMs, and her daughter has recurrent epistaxis. In both the sister and daughter, the diagnosis of HHT has been confirmed.
Physical examination was normal except for 3 telangiectasias—one on the palmar surface of the left fifth digit, one on the lower lip, and one on her left eyelid. Her oxygen saturation was 96% on room air. ECG was normal. Chest x-ray and a contrast-enhanced computed tomographic scan of the chest suggested multiple PAVMs. A time-resolved, ultrafast 3D gadolinium-enhanced magnetic resonance angiogram (MRA) was obtained to delineate the cardiopulmonary anatomy before referral for transcatheter embolotherapy. Coronal plane images demonstrated no abnormalities during the late arterial phase (Figure 1A) but during the venous phase identified large abnormal serpiginous pulmonary veins with multiple venous aneurysms (Figure 1B). The patient underwent pulmonary angiography, which confirmed the MRA findings (Figure 2).
Because these vascular anomalies were confined to the venous side of the pulmonary circulation, they were not amenable to transcatheter embolotherapy treatment. To our knowledge, this is the first description of multiple aneurysmal malformations confined to the pulmonary venous circulation in HHT. The patient was managed conservatively, and her symptoms have not progressed.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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