(975) A Case of Diffuse Distal Congenital Pulmonary Artery Hypoplasia Successfully Treated with Pulmonary Artery Stenting

IntroductionWe present a case of diffuse distal congenital pulmonary artery hypoplasia with elevated right ventricular systolic pressures, successfully treated with pulmonary artery stenting.Case ReportA 35-year-old female presented with increasing exertional dyspnoea, on a background of diffuse distal congenital pulmonary artery hypoplasia due to an elastin gene mutation and morbid obesity.There was evidence of mild right heart failure on examination and oxygen saturations were normal. Her transthoracic echocardiogram showed right ventricular (RV) hypertrophy with moderately impaired systolic function, moderate tricuspid regurgitation and an estimated RV systolic pressure (RVSP) of 120 mmHg. Her right atrial pressure was 8 mmHg on right heart catheterisation, RVSP was 138 mmHg, mean pulmonary artery pressure (mPAP) was 84 mmHg, with a normal cardiac output and pulmonary capillary wedge pressure. A decision was made for an experienced interventional radiologist to proceed initially with balloon pulmonary angioplasty (BPA). Her obesity made her an unsuitable candidate for lung transplantation. BPA to the left and right lower lobe pulmonary artery basal segments was performed, without clinical improvement. One week later, cutting BPA was performed to the left lower lobe pulmonary artery and its A7/8 and A9/10 trunks, without improvement. Finally, PA stenting was undertaken to the left lower lobe main branch and the proximal A7/8 and A9/10 trunks, right lower lobe PA and the right middle lobe A4/5 trunk. Completion angiography showed improved flow to all stented segments, except A4. The mPAP was 36 mmHg and there were no complications.The patient experienced a marked improvement in exercise tolerance at six months follow-up with normal RV contractility.SummaryWe present a rare case of congenital pulmonary artery hypoplasia treated with BPA and subsequent successful PA stenting. Treatments for this condition not well characterised, highlighting the importance of this case.