SESSION TITLE: Pulmonary Vascular Disease Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 27, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Pulmonary artery dilatation is mostly associated with both primary and secondary pulmonary hypertension, connective tissue diseases, pulmonary valve stenosis and congenital heart disease. Idiopathic pulmonary artery dilatation (IPAD) is a rare entity in the literature.
CASE PRESENTATION: An 83 year old African American female with Crohn's disease presented for evaluation of dyspnea and syncope. Presenting chest radiograph showed centrally enlarged pulmonary arteries. CTPA obtained in 2011 demonstrated a main pulmonary artery diameter of 4.3 cm at the main trunk compared to 3.95 cm in 2005. 2-Dimensional echocardiogram revealed normal left ventricular ejection fraction, normal right heart chambers and severely dilated pulmonary artery. Doppler interrogation demonstrated grade I left ventricular diastolic dysfunction, and an estimated right ventricular (RV) systolic pressure of 40 mmHg. BNP was less than 50 pg/ml. ANA and RF were negative. Pulmonary function testing showed normal spirometry with moderate diffusion impairment. Ventilation-perfusion lung scan was normal. Subsequent right heart catheterization demonstrated (in mmHg) a resting mean right atrial pressure 3, RV 36/3, mean pulmonary artery pressure (mPAP) 19, mean pulmonary capillary wedge pressure (PCWP) of 3, Fick Cardiac Output (CO) of 2.7 L/min. Administration of 1L of 0.9% normal saline, incremented her mPAP to 23, PCWP to 9 and CO to 2.2 L/min. Furthermore, symptom-limited exercise challenge using a recumbent bicycle showed a mPAP of 24, and PCWP 4, CO 2.27 L/min indicating the absence of pulmonary arterial hypertension in any of the tested conditions.
DISCUSSION: IPAD is a rare entity with an incidence of 0.0073% based on an autopsy study by Green et al (1). Little is known about the natural history of the disease or treatment options and its occurrence in an elderly patient has been reported in only one other case report (2). No clear guidelines exist regarding ideal method to monitor these patients for progression of disease or possible surgical management for symptomatic elderly patients.
CONCLUSIONS: IPAD is rare diagnosis, particularly in the elderly with no clear guidelines regarding ideal management.
Reference #1: Greene DG, Baldwin ED, Baldwin JS, et al: Pure congenital pulmonary stenosis and idiopathic congenital dilatation of the pulmonary artery. Am J Med 1949;6:24-40.
Reference #2: Naqshbandi S, Sheikh N, Aslam K, Afzal A, Dudney T. Idiopathic Pulmonary Artery Dilatation As An Unusual Cause Of Dyspnea. Chest. 2014;145(3_MeetingAbstracts), 1826433.
DISCLOSURE: The following authors have nothing to disclose: Sara Hegab, Hector Cajigas
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