Specific treatment of pulmonary artery hypertension in Eisenmenger patients: About 4 cases

Abstract

Eisenmenger's syndrome is defined by the appearance of cyanosis during the evolution of left-right shunts, due to a progressive alteration of the pulmonary arteriolar bed, leading to an increase in pulmonary vascular resistance. This evolution of the shunt towards a reaction of Eisenmenger, makes that its simple surgical closure becomes useless. The only surgical treatment is pulmonary transplantation, or cardiopulmonary transplantation. The interest of specific medical treatment of pulmonary arterial hypertension in Eisenmenger Syndrome remains controversial. Evaluate the impact of the specific treatment of PAH on Eisenmenger patients. We report the evolution over 1 year of 4 patients with congenital heart diseases in Eisenmenger. For each patient we evaluated NYHA functional class, 6 MWT, echocardiographic and hemodynamic parameters. Two of our patients had atrioventricular canal defect: – one complete; – the other partial; 2 patients with patent arterial duct. These patients were put under specific treatment of PAH: sildenafil + macitentan. Clinically, there was a slight improvement in the dyspnoea stage from stage III to II, with an average of 6MWT at 287 m. On the echocardiographic plan Peak tricuspid regurgitation velocity which averaged 4.59 m/s decreased to 3.5 m/s after one year of treatment with PASP by tricuspid regurgitation on average at 93 mmHg at the beginning of the treatment then at 70 mmHg at 1 year. Humodynamically, the PAPm (mmHg) were at 38 before specific treatment and decreased to 32 at the end of treatment, with a cardiac output that went from 2.3 to 2.5 l/min/m 2 , and an RVP of 7 to 5 wood units. We did not notice any complications and no deaths were reported. We noted that despite improved dyspnoea and quality of life in these patients, the prognosis of patients undergoing Eisenmenger treatment under PAH specific therapy remains reserved.