Cardiovascular Disease: Student/Resident Case Report Poster - Cardiovascular Disease II |

Platypnea-Orthodeoxia Syndrome From a Giant Pulmonary Arteriovenous Malformation FREE TO VIEW

Anita Mehta, MD; Nayan Desai, MD; Janah Aji, MD
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Cooper University Hospital, Cherry Hill, NJ

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):108A. doi:10.1016/j.chest.2016.08.116
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SESSION TITLE: Student/Resident Case Report Poster - Cardiovascular Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Platypnea-orthodeoxia is defined as a syndrome of postural hypoxaemia and shortness of breath.Patients should be screened for the presence of intra-cardiac or extracardiac shunts. We present a case of a 34 year old male who presents with platypnea-orthodeoxia due to a caverous pulomary arteriovenous malformation (AVM).

CASE PRESENTATION: A 34 year old Asian male presents with complaint of worsening dyspnea since past 1 year. Dyspnea is worse on standing. He has noticed limitation in his daily activities from dyspnea. He recently migrated from Burma to United States. Reports a history of murmur. He denied any prior history of rheumatic fever, smoking or illicit drug use. Afebrile, pulse of 70 bpm and BP of 112/64 mm Hg. Oxygen saturation in supine positon on room air was 96% and on standing he desaturated to 86%. No cyanosis was present in sitting position. There was no digital clubbing. A 3/6 ejection systolic murmur was noted in the right second intercostal space. Hemoglobin of 19.4 gm% remainder of labs were normal. He underwent cardiac catheterization and CT scan of the chest with contrast (image 1 and 2) .Clinical presentation and imaging are consistent with a large left side pulmonary AVM. Echocardiography showed bicuspid aortic valve with severe valvular aortic stenosis. Right and left heart catheterization showed a right to left shunt with flow of 1.6 L/min, severe aortic stenosis and normal coronaries arteries. Patient underwent resection of the lower lobe of the left lung and mechanical aortic valve replacement.

DISCUSSION: Pulmonary arteriovenous malformations are direct communications between the pulmonary arterial and venous circulation with the absence of capillaries. Majority of pulmonary AVMs are congenital and associated with hereditary hemorrhagic telangiectasia (Rendu-Weber- Osler syndrome). Acquired causes include hepato-pulmonary syndrome.1,2 Patients are usually asymptomatic but if symptomatic symptoms can include dyspnea, neurological deficits due to paradoxical emboli, and rarely, hemoptysis. AVMs are classified as simple if a single pulmonary artery feeder is present, or complex if multiple pulmonary feeding arteries exist. Selective embolization is considered as a treatment of choice for pulmonary AVMs if anatomy is favorable.3

CONCLUSIONS: Patients presenting with the clinical feature of playtpnea and orthodexia should be screened for pulmonary AVM. Cirrhosis of liver and hepato-pulmonary syndrome needs to be excluded. In patient with symptoms percutaneous embolization or surgical lobectomy may be indicated.

Reference #1: Gossage J R, Kanj G. Pulmonary arteriovenous malformations: a state of the art review. Am J Respir Crit Care Med. 1998; 158 643-661.

Reference #2: Lv Y, Fan D. Hepatopulmonary Syndrome. Dig Dis Sci. 2015 Jul;60(7):1914-23.

Reference #3: White Jr R I, Pollak J S, Wirth J A. Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol. 1996; 7 787-804.

DISCLOSURE: The following authors have nothing to disclose: Anita Mehta, Nayan Desai, Janah Aji

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