PURPOSE: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) is included in group 1 of the pulmonary hypertension clinical classification. However, patients with Eisenmenger syndrome may rarely have pulmonary wedge pressure (PWP) >15 mmHg, that theoretically exclude the diagnosis of PAH and the use of specific therapy. Aim of this study was to evaluate safety, tolerability, clinical and haemodynamic impact of oral bosentan in patients with Eisenmenger syndrome and PWP>15 mmHg.
METHODS: Clinical status, liver enzymes, WHO functional class, resting oxygen saturations, 6-min walk test (6MWT) and haemodynamics were assessed at baseline and after six month of oral bosentan therapy in patients with Eisenmenger syndrome and PWP>15 mmHg.
RESULTS: At baseline right heart catheterization, 9/62 (14%) patients (3 male, age 48±13 y) with CHD-related PAH and Eisenmenger syndrome showed PWP>15 mmHg (18.0±1.7 mmHg, range 16-20 mmHg). Transpulmonary pressure gradient (TPG) was 46±22 mmHg. All 9 patients were in WHO functional class 3. Two patients had complete AV canal, 3 ventricular septal defect, 2 atrial septal defect and 2 single ventricle. All patients well tolerated oral bosentan at common doses. No major side effects were observed during the follow-up. After 6 months therapy was observed a significant improvement in WHO functional class (2.5±0.4 vs 3.0±0.0; p<0.005), distance travelled in the 6MWT (365±74 vs 304±109 m; p<0.05), pulmonary vascular resistances index (15±4 vs 21±9 WU.m2; p<0.01) and pulmonary cardiac index (3.9±1.7 vs 2.9±1.0 l/min/m2; p<0.005). In contrast, PWP (17.2±2.3 vs 18.0±1.7 mmHg; p=ns) and TPG (41±18 vs 46±22 mmHg; p=ns) did not significantly change. Finally, bosentan did not worsen oxygen saturation (83±6 vs 82±7%; p=ns).
CONCLUSIONS: Bosentan was safe and well tolerated in adults with Eisenmenger syndrome and PWP>15 mmHg during 6 months of treatment. Clinical status, exercise tolerance and pulmonary haemodynamics significantly improved without compromising peripheral oxygen saturation.
CLINICAL IMPLICATIONS: Patients with Eisenmenger syndrome may rarely have pulmonary wedge pressure (PWP) >15 mmHg, that theoretically exclude the diagnosis of PAH and the use of specific therapy. Nevertheless, oral bosentan can be safely used in Eisenmenger patients having PWP >15 and <20 mmHg.
DISCLOSURE: The following authors have nothing to disclose: Michele D'Alto, Emanuele Romeo, Paola Argiento, Anna Correra, Berardo Sarubbi, Antonietta Caronna, Giancarlo Scognamiglio, Maria Russo, Raffaele Calabrò
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