Eisenmenger Syndrome

Abstract

Eisenmenger syndrome (ES), the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart disease, is a devastating condition that has a considerable impact on patients' lives. Patients who develop ES typically exhibit 1 or more of a range of cardiac defects, including ventricular septal defects, atrial septal defects, and patent ductus arteriosus. The nature of the congenital defect underlying ES is important because it has prognostic implications. Although ES shares similar morphological findings with idiopathic PAH, clinical differences exist between the 2 etiologies. Adults with ES exhibit increased survival and more favorable hemodynamics than those with idiopathic PAH. Treatment options for patients with ES have historically been limited; however, recent successes have been achieved with the use of therapies targeted against the pathophysiological pathways that underlie PAH. The dual endothelin receptor antagonist bosentan was demonstrated to improve hemodynamics and exercise capacity without compromising oxygen saturation, both in the short and long term. Improvements in hemodynamics also have been observed with the single endothelin receptor antagonist sitaxsentan. The phosphodiesterase type V inhibitor sildenafil may improve functional class, oxygen saturation, and hemodynamics in patients with ES, and beneficial effects of prostacyclin and prostacyclin analogs in patients with ES have been reported. The treatment of patients with PAH with the use of combinations of targeted therapies is becoming increasingly commonplace and may offer an alternative option for treatment of patients with ES. The authors of future studies may seek to investigate whether the pulmonary vascular remodeling in ES can be targeted and reversed.

Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

“…4,5 The underlying pathology in EIS patients is typically a large, unrestrictive ventricular septal defect that exposes the pulmonary vasculature to excessive blood flow and pressures, eventually leading to irreversible pulmonary hypertension. 6,7 Although no pediatric data are available for comparison, our overall survival of EIS patients undergoing lung transplantation is similar to that for adults, with our longest survivor being 13 years post-transplant. 8,9 While encouraging, our results should be contrasted with the natural history studies on Eisenmenger syndrome that have shown a 3-year survival from time of diagnosis of 77% and a 25-year survival from time of diagnosis of 42%.…”

Pulmonary Embolism or Eisenmenger Syndrome?

“…The treatment regime to be adopted for pulmonary hypertension in Eisenmenger syndrome is mainly based on clinical experts consensus [4]. Long term oxygen therapy is able to reduce dyspnoea and, although there is a lack of literature support, specific drugs such as endothelin receptor antagonists, phosphodiesterase type-5 inhibitors and prostacyclin analogues, may be indicated for pulmonary hypertension [5,6,7,8]. Management of concomitant bleeding and clotting increased risk is controversial.…”

The Effects of Perioperative Inhaled Iloprost on Pulmonary Hypertension with Congenital Heart Disease

“…Approximately 50% of all patients with large unrepaired VSD, and approximately 10% of patients with large unrepaired ASD, are at risk for Eisenmenger's syndrome. A left-to-right shunt lesion increases the pulmonary blood flow, changing the vascular structure and leading to a rise in PAP [11]. Nevertheless, despite having established long-standing pulmonary vascular disease with evidence of significant vascular remodeling/obstruction, Eisenmenger patients often respond favorably to advanced therapy [12].…”