A multiple combined treatment in an adult patient with Eisenmenger's syndrome

Eisenmenger's syndrome is a left-to-right shunt caused by a congenital cardiac defect, leading to pulmonary arterial hypertension (PAH) and, in the late stage, to a reversal of the shunt right-into-left. Nevertheless, it has a better prognosis when compared to idiopathic PAH.We describe a case of woman carrying Eisenmenger's syndrome successfully treated with increasing doses of sildenafil associated to prostanoids. Themedical therapy improved her clinical condition and led to an optimal disease control till the heart transplantation. Eisenmenger's syndrome, the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart disease, has shown a reduced survival rate when compared to general population, but appears to have a better prognosis than idiopathic PAH in comparable functional classes [1,2]. Lung transplantation with repair of the cardiac defects or combined heart–lung transplantation represents the therapeutical options for the treatment of Eisenmenger's syndrome in patients with poor prognosis. We describe the case of a young (36 years old) woman with a diagnosis of great arteries transposition, interventricular defect, hypoplastic right ventricle and persistent left superior venous cava, undergone to pulmonary artery banding on 1977. The patient arrived to our center on December 2009 presenting signs and symptoms suggestive of pulmonary hypertension: e.g. severe dyspnoea, fatigue, significant cyanosis and digital clubbing. The cardiac catheterization showed normal pulmonary venous return, persistent left vena cava draining collateral blood flow as consequence of coronary sinus subtotally occluded, intact inter-atrial septum (with no significant gradients between left and right ventricle and between ventricles and the aorta), right pulmonary artery dilatation (diameter 20 mm), 78% saturation in the aorta and 82% in the pulmonary artery, QP/QS 1.55, pulmonary arterial pressure 48/15/29 mm Hg and transpulmonary gradient 21 mm Hg (Figs. 1 and 2). The pulmonary artery banding was well placed. The mean pulmonary artery pressure dropped to 24 mm Hg after inhalation of 100% oxygen. The examination was complicated by an initial precordial pain during the catheter's insertion into the left superior vena cava, followed by an irreversible complete AV block requiring a surgical epicardial dual-chamber pacemaker insertion. Then the patient underwent atrioseptostomy. The patient was discharged on December 2009 in fairly good conditions, with a medical treatment based on sildenafil 0.5 mg/kg t.i. d., and was followed up as outpatient every 40–60 days by echocardiographic test, 6-minute walking test (6MWT), blood tests, BNP, NGAL and pacemaker control. The sildenafil dose was progressively increased to 0.5/kg q.i.d. during the following 30 days after the hospital discharge. Over few months, the patient showed a significant clinical improvement, with reduction in dyspnea and an improvement of the 6MWT from 110 m (March 2010) to 360 m (December 2010). On June 2010, the patient underwent new cardiac catheterization showing pulmonary artery pressure 33/10/22 mm Hg, transpulmonary gradient 13 mm Hg, 73% oxygen saturation in aorta and 79% in the lungs, 59% in superior vena cava, QP/QS of 1.42, intraventricular pressure 85/70/10 mm Hg and no gradient between the atria. The patient was considered a suitable candidate for heart transplantation and she was initiated into a programwith the aim to increase dosages of the vasodilator therapy by associating sildenafil to intravenous prostanoids, followed by catheterization after 5–6 months. A treatment algorithm of the Eisenmenger's syndrome, based on the case of a patient with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts, is proposed. The efficacy of sildenafil therapy in patient with PAH has been described [3,4]. In our case, the combination of surgical and medical therapy has shown to be a reliable approach for the treatment of adult patients with PAH, thus limiting the heart transplant only in selected cases. We thank Natascia Crispino (Medical Dpt Pfizer Italy) for the scientific assistance. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology (Shewan and Coats 2010;144:1–2).