UNILATERAL ABSENCE OF PULMONARY ARTERY COMPLICATED BY SEVERE PULMONARY HYPERTENSION: FAVORABLE RESPONSE TO PARENTERAL THERAPY

TOPIC: Pulmonary Vascular Disease TYPE: Fellow Case Reports INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) is a rare disorder that can present as an isolated lesion or in association with other congenital heart defects. An important complication UAPA the development hypertension (PH). Herein, we discuss patient severe PH setting and highlight hemodynamic response to combination vasodilator therapy included parenteral treprostinil. CASE PRESENTATION: A 58-year-old female history left UAPA, lung hypoplasia presented for evaluation repeated syncopal episodes on minimal exertion. Two years prior presentation, RHC showed mPAP 56 mmHg, PAWP 11 CO 3.63 L/min, CI 1.9 L/min/m2, PVR 12.4 WU. The was receiving riociguat 2.5 mg po TID, ambrisentan 10 daily, selexipag 1600 mcg BID. She NYHA FC IV required oxygen at 6 L/min via oxymizer. Upon she severely hypoxic requiring HFNC 50 fraction inspired 0.9. Evaluation revealed Pro-BNP 6170 pg/ml cardiac echo EF 45%, RV dilation hypokinesis RVSP 100 mmHg. Chest CT contrast artery, enlargement main right arteries without embolism, hyperinflation lung, lung. PFTs TLC 64%, FEV1/FVC 86, FEV1 43%, FVC 39%, DLco 23% predicted. VQ scan marked decreased perfusion ventilation anterior some patchy posterior aspect normal perfusion. Repeat significant worsening hemodynamics 76 mmHg 28.6 transitioned from treprostinil rapid titration 40 ng/kg/min over few days. There improvement her dyspnea oxygenation weaning supplemental trended down 332 had no recurrence episodes. SQ further continued home 64 ng/kg/min. After 2 months, repeat 67%, moderate mild 60 51 10.5 DISCUSSION: Patients who develop pose management dilemma treatment consensus exists. Therapeutic approaches should be based severity symptoms, anatomy, associated cardiovascular anomalies, collateral circulation parameters. CONCLUSIONS: Select patients may benefit cautious trial under close observation. REFERENCE #1: Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G. artery: pathophysiology, diagnosis current treatment. Arch Cardiovasc Dis. 2013;106(8-9):448-454. doi:10.1016/j.acvd.2013.05.004 DISCLOSURES: Speaker/Speaker's Bureau relationship United therapeutics Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Honoraria Removed 04/21/2021 Response Advisory Committee Member $1001 $5000 value=Consulting fee Actelion Therapeutics Current Added 04/23/2021 2019 2020 04/29/2021 Consultant Acceleron Pharma No relevant relationships Meliha Hrustanovic-Kadic,