SESSION TITLE: Imaging Student/Resident Case Report Posters
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Alveolar capillaries drain oxygenated blood to the pulmonary arteries. Bronchi and respiratory bronchioles are supplied by bronchial arteries. Anastomoses between these systems can occur at the capillary level, but radiologically apparent anomalies are extremely rare and sparsely documented. We report the late presentation of a systemic to pulmonary vascular malformation (S-PAVM) in a woman with subacute shortness of breath.
CASE PRESENTATION: A 63 year-old obese female with history of hypothyroidism presented with unexplained mild dyspnea on exertion for 3 years with subacute worsening over 2 weeks. Her family was concerned about the progressive decline in her ability to perform activities of daily living, intermittent paroxysmal nocturnal dyspnea, and mild orthopnea. She had no chest pain, recent illnesses, cough, or B symptoms. Oxygen saturation was 95% at rest. Exam, labs, and EKG were unrevealing. There were no telangiectasias. Echocardiogram showed EF 60-65%, normal pulmonary artery pressures, and no evidence of right-to-left shunt. CT angiogram revealed a single 1.2 cm right hilar bronchial artery aneursm (BAA) with otherwise normal lungs. Bronchial artery angiography demonstrated a BA-PA fistula, BAA, and no reversal of flow. PFTs were limited by shortness of breath, but FEV1 was 74% with FVC 67% of predicted suggesting possible restrictive defect.
DISCUSSION: S-PAVMs are exceedingly rare and incompletely understood. Bronchial artery to pulmonary artery (BA-PA) fistulas may develop after chronic inflammatory lung diseases. Congenital fistulas are more often described in young males; right lung involvement is more common than left; and lesions are usually solitary. Discovery may be accidental as many patients are initially asymptomatic. These are important distinguishments from non-systemic pulmonary arteriovenous malformations such as in hereditary hemorrhagic telangiectasia which may present with cyanosis, clubbing and/or polycythemia secondary to right-to-left shunt physiology. In S-PAVMs, angiography aids confirmation of left-to-left shunt physiology. The high systemic pressure to low pulmonary artery pressure gradient may contribute to gradual aneurysm formation. A high risk of rupture has prompted prophylactic embolization in some reports. At least one case report also noted relief of dyspnea post-embolization , although the mechanism of relief was not described.
CONCLUSIONS: In the absence of evidence for other pulmonary disease, the presenation was thought to be consistent with a congenital malformation. She ultimately underwent successful embolization. At 2 month follow-up, her dyspnea persisted; however at 6 months, the patient noted gradual improvement.
Reference #1: Yon JR and JG Ravenel. Congenital Bronchial Artery-Pulmonary Artery Fistula in an Adult. J Comput Assist Tomogr. 2010;34:418-420
Reference #2: Pouwels HMM, Janevski BK, Penn OCKM, Sie HT and GPM ten Velde. Systemic to pulmonary vascular malformation. Eur Respir J. 1992;5:1288-1291
DISCLOSURE: The following authors have nothing to disclose: Caleb Hsieh, Christina Lee, Keren Fogelfeld, Nader Kamangar
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