Lungen- und Atemwegsmedizin

Pulmonary Arterial Hypertension Associated with Adult Congenital Heart Disease, when Inoperable becomes Operable: A Case Report

Abstract

Andris Skride, Sabine Upmale, Kristaps Sablinskis, Ainars Rudzitis and Aivars Lejnieks

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is one of the most common forms of pulmonary arterial hypertension (PAH). Unlike the other forms, PAH-CHD can be treated surgically closing the extra- or intracardiac shunt if the shunt is left-to-right and the pulmonary vascular resistance (PVR) is low enough (<2,3-operable, 2,3-4,6 WU-borderline operable). If the PAH postoperatively persists the prognosis is even worse than for non-operated patients. Due to the previously mentioned fact the criteria for operability in such cases are often discussed. We report a case of a 54-year-old man who suffers from coronary artery disease (SYNTAX score-23,5) and PAH-CHD. As the patient required coronary artery bypass grafting (CABG) surgery, the atrial septal defect (ASD) was closed (surgically with a patch followed by transcatheter closure with an occluder) as well despite the fact that according to the current guidelines his pulmonary vascular resistance (4,7 WU) was considered too high for the defect to be operable. The patient was given PAH-specific treatment preoperatively in order to lower his PVR. This article describes the case and discusses the disease, its history, the new classification, diagnostics and treatment options.