Eisenmenger Syndrome in Adults

Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era

Hyo Soon An, Gi Beom Kim, Mi Kyoung Song, Ji Seok Bang, Sangyun Lee, Eunjung Bae, Chung Il Noh

Research output: Contribution to journalArticleResearchpeer-review

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 SpO 2 < 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 × 10 9 /L) or low SpO 2 (< 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 SpO 2 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.

Original languageEnglish
Pages (from-to)23-28
Number of pages6
JournalPediatric Cardiology
Volume40
Issue number1
DOIs
StatePublished - 15 Jan 2019

Fingerprint

Eisenmenger Complex
Vasodilator Agents
Heart Diseases
Lung
Confidence Intervals
Therapeutics
Platelet Count
Mortality
Patent Ductus Arteriosus
Survivors
Multivariate Analysis

Keywords

  • Adult
  • Congenital heart disease
  • Eisenmenger syndrome
  • Platelet counts

Cite this

@article{52b43c8b934a4df89e7a9d0c84802add,
title = "Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era",
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 SpO 2 < 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 × 10 9 /L) or low SpO 2 (< 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 SpO 2 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.",
keywords = "Adult, Congenital heart disease, Eisenmenger syndrome, Platelet counts",
author = "An, {Hyo Soon} and Kim, {Gi Beom} and Song, {Mi Kyoung} and Bang, {Ji Seok} and Sangyun Lee and Eunjung Bae and Noh, {Chung Il}",
year = "2019",
month = "1",
day = "15",
doi = "10.1007/s00246-018-1956-y",
language = "English",
volume = "40",
pages = "23--28",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",
number = "1",

}

Eisenmenger Syndrome in Adults : Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era. / An, Hyo Soon; Kim, Gi Beom; Song, Mi Kyoung; Bang, Ji Seok; Lee, Sangyun; Bae, Eunjung; Noh, Chung Il.

In: Pediatric Cardiology, Vol. 40, No. 1, 15.01.2019, p. 23-28.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Eisenmenger Syndrome in Adults

T2 - Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era

AU - An, Hyo Soon

AU - Kim, Gi Beom

AU - Song, Mi Kyoung

AU - Bang, Ji Seok

AU - Lee, Sangyun

AU - Bae, Eunjung

AU - Noh, Chung Il

PY - 2019/1/15

Y1 - 2019/1/15

N2 - 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 SpO 2 < 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 × 10 9 /L) or low SpO 2 (< 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 SpO 2 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.

AB - 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 SpO 2 < 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 × 10 9 /L) or low SpO 2 (< 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 SpO 2 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.

KW - Adult

KW - Congenital heart disease

KW - Eisenmenger syndrome

KW - Platelet counts

UR - http://www.scopus.com/inward/record.url?scp=85051793623&partnerID=8YFLogxK

U2 - 10.1007/s00246-018-1956-y

DO - 10.1007/s00246-018-1956-y

M3 - Article

VL - 40

SP - 23

EP - 28

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

IS - 1

ER -