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Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era

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Abstract

Patients with Eisenmenger syndrome (ES) have a higher mortality rate than patients with simple congenital heart disease (CHD). To determine factors associated with death in the era of advanced pulmonary vasodilator treatment, we analyzed the characteristics of adult ES patients depending on underlying CHD. Simple septal defects and patent ductus arteriosus were classified as simple CHD, and other conditions were classified as complex CHD. Sixty-seven adult ES patients (50.7% women) were reviewed retrospectively. CHD was diagnosed at a median of 10.0 years of age and ES was diagnosed at 18.6 years. Thirteen patients (19.4%) died; the median age was 38.6 years (IQR 32.2–47.8). In a multivariate analysis, patients with SpO2 < 85% had a higher mortality rate than others [hazard ratio (HR) 9.7; 95% confidence interval (CI) 1.002–95.2, p = 0.05]. In simple CHD patients, those with a low platelet count (< 100 × 109/L) or low SpO2 (< 85%) were at a higher risk of death than those without (HR 16.32, 95% CI 1.25–2266.31, p = 0.032; and HR 38.91, 95% CI 3.44–5219.41, p = 0.001, respectively). Advanced pulmonary vasodilators were used more in survivors than in non-survivors (48.1% vs. 15.4%, p = 0.032). Low SpO2 and platelet count were related to mortality in adult ES, especially in those with simple CHD. Therefore, careful attention should be paid to the care of adult ES patients with this tendency; active pulmonary vasodilator treatment should be considered.

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Acknowledgements

We are grateful to Prof. So-hee Oh, Department of Statistics at the Seoul Metropolitan Government - Seoul National University, Boramae Medical Center.

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Authors and Affiliations

  1. Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea

    Hyo Soon An, Gi Beom Kim, Mi Kyoung Song, Sang Yun Lee & Eun Jung Bae

  2. Department of Pediatrics, Seoul Metropolitan Government - Seoul National University Boramae Medical Center, Seoul, South Korea

    Hyo Soon An

  3. Department of Pediatrics, Sejong General Hospital, Bucheon, South Korea

    Ji Seok Bang & Chung Il Noh

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  1. Hyo Soon An
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Correspondence to Gi Beom Kim.

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An, H.S., Kim, G.B., Song, M.K. et al. Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era. Pediatr Cardiol 40, 23–28 (2019). https://doi.org/10.1007/s00246-018-1956-y

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  • DOI: https://doi.org/10.1007/s00246-018-1956-y

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