Prevalence of pulmonary arterial hypertension in adult congenital heart disease

Annelieke C.M.J. van Riel; Mark Schuuring; Irene D. van Hessen; Aeilko Zwinderman; Luc Cozijnsen; Stan C.L.A. Reichert; Jan Hoorntje; Lodewijk Wagenaar; M.C. Post; Arie van Dijk; Elke Hoendermis; Barbara Mulder; Berto Bouma

doi:10.1016/S0735-1097(14)60504-0

Prevalence of pulmonary arterial hypertension in adult congenital heart disease

Annelieke C.M.J. van Riel, Mark Schuuring, Irene D. van Hessen, Aeilko Zwinderman, Luc Cozijnsen, Stan C.L.A. Reichert, Jan Hoorntje, Lodewijk Wagenaar, M.C. Post, Arie van Dijk, Elke Hoendermis, Barbara Mulder, Berto Bouma

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Background: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence reports have a high degree of variability, with an overrepresentation of tertiary centers. We aimed to report robust prevalence estimates of PAH in adult CHD patients.

Methods and Results: We performed a cross-sectional analysis to determine prevalence of PAH in adults with CHD using the population-based Dutch Congenital Corvitia (CONCOR) registry. Of 12 624 adults with CHD alive in 2011, 5 487 (44%) had an initial diagnosis of systemic-to-pulmonary shunt and were thereby at risk for developing PAH. From these patients, a random sample of 1 814 subjects was obtained, mean age 40±15 years, and 135 PAH cases were observed. Total PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. A remarkable high number of patients had PAH after corrective cardiac surgery, with a prevalence of 5.7%. PAH prevalence increases with age, from 2.5% under 30 years until 35% in the oldest patients of the study cohort. In the entire CHD population the PAH prevalence was 3.2%. Based on an overall prevalence of CHD in the adult population of 3000 per million, we can assume a prevalence of PAH-CHD of 100 per million inhabitants.

Conclusion: Prevalence of PAH in CHD patients is 3.2%, and 100 per million in the general adult population. Especially in patients after corrective cardiac surgery and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutical and clinical care.

Original language	English
Journal	Journal of the American College of Cardiology
Volume	63
Issue number	12, Suppl.
DOIs	https://doi.org/10.1016/S0735-1097(14)60504-0
Publication status	Published - 2014
Externally published	Yes

Keywords

n/a OA procedure

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1016/S0735-1097(14)60504-0

Cite this

van Riel, A. C. M. J., Schuuring, M., van Hessen, I. D., Zwinderman, A., Cozijnsen, L., Reichert, S. C. L. A., Hoorntje, J., Wagenaar, L., Post, M. C., van Dijk, A., Hoendermis, E., Mulder, B., & Bouma, B. (2014). Prevalence of pulmonary arterial hypertension in adult congenital heart disease. Journal of the American College of Cardiology, 63(12, Suppl.). https://doi.org/10.1016/S0735-1097(14)60504-0

@article{f6d2d87de1bb44a3a3c4d3dff402b45b,

title = "Prevalence of pulmonary arterial hypertension in adult congenital heart disease",

abstract = "Background: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence reports have a high degree of variability, with an overrepresentation of tertiary centers. We aimed to report robust prevalence estimates of PAH in adult CHD patients.Methods and Results: We performed a cross-sectional analysis to determine prevalence of PAH in adults with CHD using the population-based Dutch Congenital Corvitia (CONCOR) registry. Of 12 624 adults with CHD alive in 2011, 5 487 (44%) had an initial diagnosis of systemic-to-pulmonary shunt and were thereby at risk for developing PAH. From these patients, a random sample of 1 814 subjects was obtained, mean age 40±15 years, and 135 PAH cases were observed. Total PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. A remarkable high number of patients had PAH after corrective cardiac surgery, with a prevalence of 5.7%. PAH prevalence increases with age, from 2.5% under 30 years until 35% in the oldest patients of the study cohort. In the entire CHD population the PAH prevalence was 3.2%. Based on an overall prevalence of CHD in the adult population of 3000 per million, we can assume a prevalence of PAH-CHD of 100 per million inhabitants.Conclusion: Prevalence of PAH in CHD patients is 3.2%, and 100 per million in the general adult population. Especially in patients after corrective cardiac surgery and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutical and clinical care.",

keywords = "n/a OA procedure",

author = "{van Riel}, {Annelieke C.M.J.} and Mark Schuuring and {van Hessen}, {Irene D.} and Aeilko Zwinderman and Luc Cozijnsen and Reichert, {Stan C.L.A.} and Jan Hoorntje and Lodewijk Wagenaar and M.C. Post and {van Dijk}, Arie and Elke Hoendermis and Barbara Mulder and Berto Bouma",

year = "2014",

doi = "10.1016/S0735-1097(14)60504-0",

language = "English",

volume = "63",

journal = "Journal of the American College of Cardiology",

issn = "0735-1097",

publisher = "Elsevier Inc.",

number = "12, Suppl.",

}

van Riel, ACMJ, Schuuring, M, van Hessen, ID, Zwinderman, A, Cozijnsen, L, Reichert, SCLA, Hoorntje, J, Wagenaar, L, Post, MC, van Dijk, A, Hoendermis, E, Mulder, B & Bouma, B 2014, 'Prevalence of pulmonary arterial hypertension in adult congenital heart disease', Journal of the American College of Cardiology, vol. 63, no. 12, Suppl. https://doi.org/10.1016/S0735-1097(14)60504-0

TY - JOUR

T1 - Prevalence of pulmonary arterial hypertension in adult congenital heart disease

AU - van Riel, Annelieke C.M.J.

AU - Schuuring, Mark

AU - van Hessen, Irene D.

AU - Zwinderman, Aeilko

AU - Cozijnsen, Luc

AU - Reichert, Stan C.L.A.

AU - Hoorntje, Jan

AU - Wagenaar, Lodewijk

AU - Post, M.C.

AU - van Dijk, Arie

AU - Hoendermis, Elke

AU - Mulder, Barbara

AU - Bouma, Berto

PY - 2014

Y1 - 2014

N2 - Background: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence reports have a high degree of variability, with an overrepresentation of tertiary centers. We aimed to report robust prevalence estimates of PAH in adult CHD patients.Methods and Results: We performed a cross-sectional analysis to determine prevalence of PAH in adults with CHD using the population-based Dutch Congenital Corvitia (CONCOR) registry. Of 12 624 adults with CHD alive in 2011, 5 487 (44%) had an initial diagnosis of systemic-to-pulmonary shunt and were thereby at risk for developing PAH. From these patients, a random sample of 1 814 subjects was obtained, mean age 40±15 years, and 135 PAH cases were observed. Total PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. A remarkable high number of patients had PAH after corrective cardiac surgery, with a prevalence of 5.7%. PAH prevalence increases with age, from 2.5% under 30 years until 35% in the oldest patients of the study cohort. In the entire CHD population the PAH prevalence was 3.2%. Based on an overall prevalence of CHD in the adult population of 3000 per million, we can assume a prevalence of PAH-CHD of 100 per million inhabitants.Conclusion: Prevalence of PAH in CHD patients is 3.2%, and 100 per million in the general adult population. Especially in patients after corrective cardiac surgery and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutical and clinical care.

AB - Background: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence reports have a high degree of variability, with an overrepresentation of tertiary centers. We aimed to report robust prevalence estimates of PAH in adult CHD patients.Methods and Results: We performed a cross-sectional analysis to determine prevalence of PAH in adults with CHD using the population-based Dutch Congenital Corvitia (CONCOR) registry. Of 12 624 adults with CHD alive in 2011, 5 487 (44%) had an initial diagnosis of systemic-to-pulmonary shunt and were thereby at risk for developing PAH. From these patients, a random sample of 1 814 subjects was obtained, mean age 40±15 years, and 135 PAH cases were observed. Total PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. A remarkable high number of patients had PAH after corrective cardiac surgery, with a prevalence of 5.7%. PAH prevalence increases with age, from 2.5% under 30 years until 35% in the oldest patients of the study cohort. In the entire CHD population the PAH prevalence was 3.2%. Based on an overall prevalence of CHD in the adult population of 3000 per million, we can assume a prevalence of PAH-CHD of 100 per million inhabitants.Conclusion: Prevalence of PAH in CHD patients is 3.2%, and 100 per million in the general adult population. Especially in patients after corrective cardiac surgery and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutical and clinical care.

KW - n/a OA procedure

U2 - 10.1016/S0735-1097(14)60504-0

DO - 10.1016/S0735-1097(14)60504-0

M3 - Article

SN - 0735-1097

VL - 63

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

IS - 12, Suppl.

ER -

Prevalence of pulmonary arterial hypertension in adult congenital heart disease

Abstract

Keywords

UN SDGs

Access to Document

Fingerprint

Cite this