SESSION TITLE: Cardiovascular Cases
SESSION TYPE: Case Reports
PRESENTED ON: Saturday, March 22, 2014 at 04:15 PM - 05:15 PM
INTRODUCTION: Dilatation of the main pulmonary artery in the absence of pulmonary hypertension is a rare condition with numerous identified etiologies. Idiopathic pulmonary artery dilatation (IPAD) is a diagnosis of exclusion and is an extremely rare cause of pulmonary artery dilatation. Here we discuss a case of IPAD presenting as unexplained dyspnea.
CASE PRESENTATION: 48 year old obese Caucasian male presented to clinic for evaluation of episodic dyspnea. Patient noted mild exertional dyspnea, orthopnea, occasional non-exertional chest discomfort and postural dizziness. Physical examination was unremarkable. A CTPA revealed a dilated pulmonary artery measuring 6.5 cm and a 5 mm right middle lobe nodule with no other parenchymal abnormalities. Subsequent echocardiogram showed normal ejection fraction and mildly elevated right ventricular systolic pressure of 35.12 mmHg. Pulmonary function tests were normal. BNP, ANA, RF, ANCA, RPR were normal. He was noted to have mild obstructive sleep apnea (OSA) on polysomnogram. A right heart catheterization demonstrated mild pulmonary hypertension with PCWP of 14 mmHg, mPAP of 37 mmHg, no intracardiac shunt and pulmonary artery trunk measuring 5.5 cm. The patient was diagnosed with IPAD as secondary etiologies were excluded. The patient currently follows in clinic with annual CTPA to monitor pulmonary artery size which has been stable. In addition, the patient is on BPAP therapy for OSA.
DISCUSSION: IPAD is an extremely rare entity with sporadic case reports in literature. It is often diagnosed incidentally but can present with nonspecific complaints. Original criteria for diagnosis of IPAD were established by Greene et al in 1949. Deshmukh et al in 19931 suggested including normal pressure in the right ventricle and pulmonary artery as an additional criteria. Although our patient does have mild pulmonary hypertension he did meet the original diagnostic criteria established by Greene et al. Moreover, the magnitude of pulmonary artery dilatation is out of proportion to the mild pulmonary hypertension which is likely explained by previously untreated OSA. The natural history of pulmonary artery aneurysm remains largely unknown. However, aneurysms do carry an inherent risk of rupture and sudden death. There are no specific guidelines regarding management of these patients.
CONCLUSIONS: IPAD is considered to be a benign condition. However, given the inherent risk of pulmonary artery rupture, it is prudent to observe these patients closely for a prolonged period of time.
Reference #1: Idiopathic Dilatation of the Pulmonary Artery. Circulation 1960;21:710-716
DISCLOSURE: The following authors have nothing to disclose: Syed Mudassar Naqshbandi, Naveed Sheikh, Kashif Aslam, Asra Afzal, Tina Dudney
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