740 TREATMENT STRATEGY IN CASE OF REPAIRED TRUNCUS ARTERIOSUS AND AORTIC COARCTATION

Abstract

Abstract Background Common Truncus arteriosus (TA) is a rare congenital, cyanotic heart disease with a single vessel coming out from both ventricles and related low interventricular defect, right-left shunt, low pulmonary flow and right ventricle (RV) hypertension. TA is associated with aortic coarctation (CoA) in 10-20% of cases. Case A 15-year-old male affected by type 2 TA and CoA undergoing first post-natal cardiac surgery with pulmonary valved conduit (PVC) implantation and CoA repair, subsequently multiple percutaneous (PC) interventions due to right pulmonary artery (RPA) stenosis and re-CoA. He was referred to our Centre for dyspnoea on mild efforts. Echocardiography revealed RV hypertension (75% of systemic pressure) and mild disfunction. Angio-CT showed RPA re-stenosis due to peeling of previous stents, a degenerative PVC and mild residual CoA. Results Cardiac catheterization confirmed imaging findings, revealing the following systolic/diastolic blood pressures [mmHg]: 100/15 in RV, 33/10 in left pulmonary artery (LPA), 13/7 in RPA, 132/65 in ascending and 125/60 in descending aorta. First, extensive balloon-interrogation of stented RPA and RV outflow tract (RVOT) with semi-compliant balloon was performed followed by PC intra-stenting transluminal angioplasty of RPA with modified undersized non-compliant balloon. As RPA was very closed to CoA with risk of aortic rupture, and mostly the main pulmonary OT accounted for increased pressure gradient, RPA enlargement with re-stenting was avoided. Then, a long semi-opened-cell stent was arranged on the prosthetic RVOT and Melody valve was implanted as Valve-in-Valve (ViV), both with balloon post-dilatation. Dilated LPA with only moderate focal stenosis and mild residual CoA were excluded from intervention. Post-interventional right heart pressures were reduced: 45/4 in RVOT and 42/13 mmHg in the main PA. At 1 month dyspnoea disappeared with good effort tolerance, echocardiographic ViV mean gradient was 12 mmHg with improved RV systolic function and pressure (50% of systemic). Conclusions Repaired complex TA of type described may develop multiple re-stenosis of RPA. RPA optimization was not the goal of the procedure because at high risk of vessels’ injury due to the very closed anatomy with otherwise prosthetic CoA. RV pressures and dysfunction could depend on degenerative obstructive PVC. Thus, interventional approach mostly focused on the main PA aimed at working PVC could be the best effective treatment.