Clinic, therapy and prognosis of pulmonary arterial hypertension in congenital heart defects

anchelito /

Despite the increasingly comprehensive availability of cardiac surgery and interventional treatment methods for children with congenital heart defects (CHD), pulmonary arterial hypertension (PAH) remains a feared complication in the long-term progress for CHD patients. It is estimated that 5 – 10 % of all patients with CHD will develop PAH at some point. These patients, in comparison to other CHD patients, suffer from increased rates of morbidity and mortality. This is why the National Register for Congenital Heart Defects has been examining the current national health care situation and prognosis for patients with PAH-CHD under the auspices of PD Dr. Gerhard-Paul Diller, at the University Hospital in Münster.

PAH is a much feared long-term complication

For this, the register’s database was searched systematically for PAH-CHD patients. Patients aged one year and over that had developed persistent PAH were considered, including both post shunt-closure surgery patients and palliative or unoperated patients (such as patients with Eisenmenger’s syndrome). Patients with isolated post-capillary pulmonary hypertension, patients who display normalization of pulmonary arterial pressure after timely operative therapy, patients with idiopathic PAH or patients with persistent pulmonary hypertension of the newborn were excluded. PAH was defined in accordance with current guidelines based on invasive criteria; in the absence of current heart catheterization findings, an echocardiographic systolic pulmonary arterial blood pressure safe value (sPAP) over 50 mmHg was acceptable. Data about symptoms, exercise capacity, drug treatment and prognosis were compiled.

Survival rate for patients with Eisenmenger’s is low

In total, 184 patients were included. The average age was 24.6 ± 14.9; 59 % of the patients were female. A majority of the patients were distinctly symptomatic (62 % NYHA class III). 108 patients had Eisenmenger’s syndrome. These patients with Eisenmenger’s syndrome showed the same gender distribution as the rest of the cohort, but were on average 10 years older. In the six-minute walk test, the average distance covered was 382 ± 122 m (Eisenmenger’s 368 ± 118 m). Overall, 44 % of patients (Eisenmenger’s 51 %) received treatment with PAH specific drugs (70 % Bosentan, 25 % Sildenafil); 14 % received a dual drug therapy. Moreover, 51 % of the patients (Eisenmenger’s 52 %) took a cardiac insufficiency drug. Only 16 % received an oral anticoagulation drug (Eisenmenger’s 15 %); 22 % (Eisenmenger’s 25 %) however were treated with Aspirin. The average survival rate after 1, 3, 5, and 10 years for the entire cohort was 94, 83, 77 and 64 % respectively; Eisenmenger’s patients showed a particularly poor prognosis (survival rate of 93, 76, 66 and 49 % after 1, 3, 5 and 10 years).

PAH patients are symptomatic and have limited exercise capacity

The present study shows that there are a sizeable number of patients with PAH-CHD and particularly Eisenmenger’s syndrome, despite increasingly comprehensive and earlier surgical correction. Overall, these patients are distinctly symptomatic and have clearly limited objective exercise capacity on average. What also stands out in the present study is the large number of patients treated with cardiac insufficiency drugs and aspirin along with the relatively poor prognosis, despite the use of PAH-specific drugs, in fully 44 % of patients. The data in this study will be presented during the German Cardiac Society’s 80th spring convention in Mannheim.

Gerhard-Paul Diller, Marc Körten, Ulrike Bauer, Oliver Miera, Helmut Baumgartner: Clinic, Therapy and Prognosis of Pulmonary Arterial Hypertension in Congenital Heart Defects

Presentation at the 80th annual convention of the German Society for Thoracic and Cardiovascular Surgery
, April 23, 2014, 3:30 – 5:00 p.m.



Competence Network for Congenital Heart Defects and the National Register for Congenital Heart Defects

The register database is the core project of the Competence Network for Congenital Heart Defects, a non-profit scientific association led by the three main scientific cardiology societies – the German Society for Paediatric Cardiology (DGPK), the German Cardiac Society (DGK) and the German Society for Thoracic and Cardiovascular Surgery (DGTHG). The Competence Network has received funding from the German Federal Ministry for Education and Research (BMBF) since 2003 and is also a registered scientific association. Both organisations cooperate nationwide with registered doctors who treat patients with congenital heart defects in clinics, heart centres and rehabilitation centres as well as working closely with relevant patient and parent organizations.

This study is supported by Actelion Pharmaceuticals Deutschland GmbH.