Abstract

MOROS¹, Claudio Gabriel et al. Pulmonary arterial hypertension in adult congenital heart disease: Impact and outcome Data from the GUTI-GUCH RegistryHipertensão arterial pulmonar em cardiopatias congênitas no adulto: Impacto e resultados Dados do Registro GUTI-GUCH. Insuf. card. [online]. 2016, vol.11, n.3, pp.115-121. ISSN 1852-3862.

Introduction. Pulmonary hypertension (PH) is a serious complication of adult congenital heart disease (ACHD), its incidence is associated with a marked increase in morbidity and mortality of these patients. Objective. We analyze the epidemiological characteristics clinical and pharmacological treatment in patients with pulmonary arterial hypertension (PAH) and their impact on survival. Materials and methods. Retrospective observational study, global data were analyzed using the GUTI-GUCH Registry. We selected patients with diagnosis of PAH related to congenital heart disease (PAH-CHD) and its subgroups. SG1: Eisenmenger syndrome, SG2: shunts left to right, SG3: PAH-CHD, SG4: postoperative PAH. Statistical analysis: SPSS 20, Chi square test, Kaplan Meier curve contrasted by Log Rank. Results. We included 1604 patients with a mean age of 32.5 years, 55.4% (889) were women. Patients with heart disease risk for PAH (HDRPAH): 70.5% (1131); PH: 4.2% (67) overall and 5.8% (67) of HDRPAH. And 71.6% (48) patients with PH were women, with age 41.7 ± 14.4 years and follow up of 33 ± 30.9 months. The 91% (61) belonged to PAH-CHD group representing 3.8% of registry. PAH-CHD patients were subdivided into: SG1: 47.5% (29); SG2: 26.2% (16); SG3: 5% (3) and SG4: 21.3% (13). The PAH-CHD group received pharmacological treatment, specific: 78.7% (48), monotherapy: 57.4% (35) and combination therapy: 21.3% (13). The follow-up mortality for patients with PH was 17.9% (12) and 16.4% (10) for PAH-CHD. Statistically significant differences were observed for PH: p=0.000; PAH-CHD: p=0.000; PAH-CHD subgroups: p=0.000. No differences were found with SG1: p=0.184. Conclusions. The HP and PAH-CHD negatively affect the survival of patients with ACHD. Half of them are carriers of Eisenmenger syndrome by the SG3 are better evolution. A high prevalence of women was observed.

Keywords : Adult congenital heart disease; GUCH; Pulmonary hypertension; Pulmonary arterial hypertension.

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