Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease

Abstract

Background Adults with pulmonary hypertension associated with congenital heart disease (PH-CHD) often have residual shunts. Invasive interventions aim to optimise pulmonary flow and prevent right ventricular failure. However, eligibility for procedures strongly depends on the adaptation potential of the pulmonary vasculature and right ventricle to resultant circulatory changes. Current guidelines are not sufficiently applicable to individual patients, who exhibit great diversity and complexity in cardiac anomalies. Methods and Results We present four complex adult PHCHD patients with impaired pulmonary flow, including detailed graphics of the cardiopulmonary circulation. All these patients had an ambiguous indication for shunt intervention. Our local multidisciplinary Grown-Ups with Congenital Heart Disease team reached consensus regarding a patient-tailored invasive treatment strategy, adjacent to relevant guidelines. Interventions improved pulmonary haemodynamics and short-term clinical functioning in all cases. Conclusions Individual evaluation of disease characteristics is mandatory for tailored interventional treatment in PHCHD patients, adjacent to relevant guidelines. Both strict registration of cases and multidisciplinary and multicentre collaboration are essential in the quest for optimal therapy in this patient population.