SESSION TITLE: Pulmonary Vascular Disease 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: Patent ductus arteriosus (PDA) is a vascular structure that connects the proximal descending aorta to the root of the main pulmonary artery. The lack of spontaneous closure of a PDA after birth can lead to a congenital heart defect in which there is persistent communication between the descending thoracic aorta and the pulmonary artery. Surgical closure of a PDA in infancy usually prevents left-to-right shunting of blood flow and subsequent development of pulmonary hypertension. We present a case of adult-onset severe pulmonary hypertension despite surgical closure of PDA during childhood.
CASE PRESENTATION: A 29 year old man, with past medical history of PDA with corrective surgery in 1992, presented with dyspnea on exertion for a few weeks. On admission, he had stable vitals with occasional desaturations to 93% on RA. Physical exam was notable for a significant systolic murmur on the left parasternal border and mild bilateral lower extremity edema. Significant labs were elevated BNP, polycythemia and thrombocytopenia. Echocardiogram showed an ejection fraction between 45-50%, marked dilation of right atrium and markedly enlarged right ventricle with free wall hypertrophy. Patient experienced significant bradycardia and hypotension requiring intravenous atropine, dopamine and phenylephrine. EKG showed right atrial enlargement and right ventricular hypertrophy, a lower extremity Doppler was negative for deep vein thrombosis. A CT-PE showed no evidence of PE. Right heart catheterization revealed pulmonary artery pressure of 130/90, which nearly approached his systemic blood pressure. This did not respond to nitric oxide administration up to 80ppm. He was started on ambrisentan and sildenafil PO with treprostinil drip. He was discharged on the above medications with follow-up arranged in the pulmonary hypertension clinic.
DISCUSSION: This case presents a patient with severe pulmonary hypertension as an adult despite surgical closure of PDA in childhood. This is likely secondary to ongoing endothelin-based remodeling of the pulmonary vasculature and smooth muscle. The patient was classified as WHO Group 1, post congenital heart disease (s/p PDA repair), with Class III functional status. This delayed presentation of pulmonary hypertension post-surgical closure warrants reevaluation of the long term approach in managing patients with PDA.
CONCLUSIONS: Endothelin based remodeling of pulmonary vasculature is implicated in the development of pulmonary hypertension post-PDA repair. Appropriate follow up and early intervention is necessary to prevent the development of pulmonary hypertension post-PDA repair
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Reference #2: Haworth SG. Pulmonary hypertension in childhood. Eur Respir J 1993; 6:1037.
Reference #3: Rich S (ed). Executive summary from the World Symposium on Primary Pulmonary Hypertension, Evian, France, September 6-10, 1998, co-sponsored by The World Health Organization.
DISCLOSURE: The following authors have nothing to disclose: Tariq Yousuf, Lima Lawrence, Jason Kramer, Armand Krikorian
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