Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018

Abstract

In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article.

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

“…Pulmonary hypertension (PH) is one of the most severe complications of congenital anomalies of the heart and/or the great vessels (CHD) [1]. The presence of PH aggravates the natural, post-operative or post-interventional course of the underlying anomaly and impacts disease burden and outcome.…”

“…The clinical classification of PH comprises five major groups: pulmonary arterial hypertension (PAH, Group I); PH due to left heart disease (LHD, Group II); PH due to lung disease (Group III); chronic thromboembolic PH (Group IV); and unclear or multifactorial PH (Group V) [4]. According to guideline recommendations, PH associated with CHD is assigned to either Group I (PAH), Group II (due to LHD), or Group V (unclear/multifactorial mechanism) [1,2,[5][6][7].…”

“…In the future, this complication is likely to increase, largely determining morbidity and mortality of affected patients [6,[12][13][14][15][16]. Beyond that, PH is also an evolving phenomenon in aging patients with congenital anomalies of the left heart, e.g., in aortic coarctation, congenital aortic valve disease, or congenital mitral valve disease [1].…”

Medical therapy for heart failure in adult congenital heart disease: does it work?

“…Systolic dysfunction of the subpulmonic RV can occur in a variety of right-sided congenital cardiac lesions. Pulmonary hypertension represents the most common pressure-loading lesion that may result in RV systolic failure and PDE-5 inhibitors, endothelin-receptor antagonist or prostacyclin analogues are the drugs specifically recommended for the treatment of advanced pulmonary arterial hypertension 31. Among patients with volume loading, chronic pulmonary regurgitation (PR) in rToF is the most common cause of subpulmonic RV systolic failure.…”

A New <i>ISL1</i> Loss-of-Function Mutation Predisposes to Congenital Double Outlet Right Ventricle

“…2) Based on final anatomic and physiological phenotypes, CHDs have been classified into at least 25 distinctive types, such as ventricular septal defect (VSD), double-orifice mitral valve, atrial septal defect, coronary artery anomaly, patent ductus arteriosus, Ebstein's anomaly, tetralogy of Fallot, double outlet right ventricle (DORV), aortic stenosis, transposition of the great arteries, truncus arteriosus, interrupted aortic arch, coarctation of the aorta, pulmonary stenosis, endocardial cushion defect, hypoplastic left ventricle, and anomalous pulmonary venous connection. 1,[2][3][4][5][6][7][8] Although minor CHDs may resolve spontaneously, 2) severe CHDs require timely intervention in the first year of life and otherwise may give rise to reduced exercise capacity and poor health-related quality of life, [9][10][11] neurodevelopmental anomaly or brain injury, [12][13][14] pulmonary arterial hypertension, [15][16][17][18][19] hemorrhagic or thromboembolic stroke, [20][21][22] dementia, 23) infective endocarditis, [18][19][20][21][22][23][24][25][26][27][28][29][30] myocardial infarction, 31,32) myocardial fibrosis, [33][34]…”