Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

Annelieke C M J van Riel; Mark J Schuuring; Irene D van Hessen; Aielko H Zwinderman; Luc Cozijnsen; Constant L A Reichert; Jan C A Hoorntje; Lodewijk J Wagenaar; Marco C Post; Arie P J van Dijk; Elke S Hoendermis; Barbara J M Mulder; Berto J Bouma

doi:10.1016/j.ijcard.2014.04.072

Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

Annelieke C M J van Riel, Mark J Schuuring, Irene D van Hessen, Aielko H Zwinderman, Luc Cozijnsen, Constant L A Reichert, Jan C A Hoorntje, Lodewijk J Wagenaar, Marco C Post, Arie P J van Dijk, Elke S Hoendermis, Barbara J M Mulder, Berto J Bouma^*

^*Corresponding author for this work

Cardiologie

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 estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population.

METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed.

RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million.

CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.

Original language	English
Pages (from-to)	299-305
Number of pages	7
Journal	International Journal of Cardiology
Volume	174
Issue number	2
DOIs	https://doi.org/10.1016/j.ijcard.2014.04.072
Publication status	Published - 15 Jun 2014

Keywords

Adult
Cross-Sectional Studies
Female
Heart Defects, Congenital
Humans
Hypertension, Pulmonary
Male
Middle Aged
Prevalence

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10.1016/j.ijcard.2014.04.072

Cite this

van Riel, A. C. M. J., Schuuring, M. J., van Hessen, I. D., Zwinderman, A. H., Cozijnsen, L., Reichert, C. L. A., Hoorntje, J. C. A., Wagenaar, L. J., Post, M. C., van Dijk, A. P. J., Hoendermis, E. S., Mulder, B. J. M., & Bouma, B. J. (2014). Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. International Journal of Cardiology, 174(2), 299-305. https://doi.org/10.1016/j.ijcard.2014.04.072

@article{a21329d87d414448a7c84adc174f4ee2,

title = "Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification",

abstract = "BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population.METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed.RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million.CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.",

keywords = "Adult, Cross-Sectional Studies, Female, Heart Defects, Congenital, Humans, Hypertension, Pulmonary, Male, Middle Aged, Prevalence",

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

year = "2014",

month = jun,

day = "15",

doi = "10.1016/j.ijcard.2014.04.072",

language = "English",

volume = "174",

pages = "299--305",

journal = "International Journal of Cardiology",

issn = "0167-5273",

publisher = "Elsevier Ireland Ltd",

number = "2",

}

van Riel, ACMJ, Schuuring, MJ, van Hessen, ID, Zwinderman, AH, Cozijnsen, L, Reichert, CLA, Hoorntje, JCA, Wagenaar, LJ, Post, MC, van Dijk, APJ, Hoendermis, ES, Mulder, BJM & Bouma, BJ 2014, 'Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification', International Journal of Cardiology, vol. 174, no. 2, pp. 299-305. https://doi.org/10.1016/j.ijcard.2014.04.072

Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. / van Riel, Annelieke C M J; Schuuring, Mark J; van Hessen, Irene D et al.
In: International Journal of Cardiology, Vol. 174, No. 2, 15.06.2014, p. 299-305.

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

TY - JOUR

T1 - Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

AU - van Riel, Annelieke C M J

AU - Schuuring, Mark J

AU - van Hessen, Irene D

AU - Zwinderman, Aielko H

AU - Cozijnsen, Luc

AU - Reichert, Constant L A

AU - Hoorntje, Jan C A

AU - Wagenaar, Lodewijk J

AU - Post, Marco C

AU - van Dijk, Arie P J

AU - Hoendermis, Elke S

AU - Mulder, Barbara J M

AU - Bouma, Berto J

PY - 2014/6/15

Y1 - 2014/6/15

N2 - BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population.METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed.RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million.CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic 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 estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population.METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed.RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million.CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.

KW - Adult

KW - Cross-Sectional Studies

KW - Female

KW - Heart Defects, Congenital

KW - Humans

KW - Hypertension, Pulmonary

KW - Male

KW - Middle Aged

KW - Prevalence

U2 - 10.1016/j.ijcard.2014.04.072

DO - 10.1016/j.ijcard.2014.04.072

M3 - Article

C2 - 24794056

SN - 0167-5273

VL - 174

SP - 299

EP - 305

JO - International Journal of Cardiology

JF - International Journal of Cardiology

IS - 2

ER -