Outcomes of Eisenmenger Syndrome in Germany

Study Questions:

What are the contemporary outcomes of Eisenmenger syndrome (ES) and the impact of pulmonary vasodilator therapy using nationwide registry data?

Methods:

Patients with ES were identified from the German National Register for Congenital Heart Defects. Basic demographic data, underlying diagnosis, medical therapy, and survival were collected. The impact of disease targeting therapies (DTT) was assessed.

Results:

A total of 153 patients with ES were included (mean age 34 ± 13.3 years). Simple defects (atrial or ventricular septal defects and patent ductus arteriosus) were present in 54% of patients, while complex defects were present in the remainder. Down syndrome was present in 32.7% of patients. A majority of patients (57.5%) were treated with DTT (76% bosentan, 20.5% sildenafil), while 17.6% were on dual DTT. Additional medical therapy included digoxin in 24.8%, angiotensin-converting enzyme inhibitors/angiotensin-receptor blockers in 10.5%, and beta-blockers in 17.6%. In terms on anticoagulants, 17.6% of patients were treated with oral anticoagulants, while 23.5% received aspirin. The overall survival rate was 92% at 1 year, 75% at 5 years, and 57% at 10 years. Survival was lower in treatment-naïve patients. Five-year survival was 60% for treatment-naïve patients and 83% for those receiving DTT.

Conclusions:

The authors concluded that ES is associated with significant mortality and that treatment with DTT is associated with better survival.

Perspective:

This registry-based trial from Germany demonstrates significant intermediate- and long-term mortality in patients with ES. It does confirm previous retrospective single-center studies suggesting survival benefit for patients receiving pulmonary vasodilator therapy. Although ES has historically been believed to be associated with better outcomes as compared with primary pulmonary hypertension, significant mortality risk remains, even in the setting of pulmonary vasodilator therapy. This study included a representative population of patients, with a significant number with Down syndrome, and a blend of patients with simple and complex underlying congenital heart disease. The study supports existing recommendations that pulmonary vasodilator therapy be discussed with all patients with ES.

Clinical Topics: Anticoagulation Management, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease, CHD & Pediatrics and Prevention

Keywords: Adrenergic beta-Antagonists, Angiotensin-Converting Enzyme Inhibitors, Anticoagulants, Aspirin, Digoxin, Down Syndrome, Ductus Arteriosus, Patent, Eisenmenger Complex, Heart Defects, Congenital, Heart Septal Defects, Atrial, Heart Septal Defects, Ventricular, Survival Rate, Vasodilator Agents


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