TY - JOUR
T1 - Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry
AU - Duffels, M. G. J.
AU - Engelfriet, P. M.
AU - Berger, R. M. F.
AU - van Loon, R. L. E.
AU - Hoendermis, E.
AU - Vriend, J. W. J.
AU - van der Velde, E. T.
AU - Bresser, P.
AU - Mulder, B. J. M.
PY - 2007
Y1 - 2007
N2 - Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease is usually the result of a large systemic-to-pulmonary shunt, and often leads to right ventricular failure and early death. The purpose of this study was to determine the prevalence of PAH among adult patients included in a national registry of congenital heart disease and to assess the relation between patient characteristics and PAH. Methods: Patients with PAH associated with a septal defect were identified from the registry. Gender, age, underlying diagnosis, previous closure, age at repair and NYHA classification were recorded. PAH was defined as a systolic pulmonary arterial pressure (sPAP) greater than 40 mm Hg, estimated by means of echocardiographical evaluation. Results: The prevalence of PAH among all 5970 registered adult patients with congenital heart disease was 4.2%. Of 1824 patients with a septal defect in the registry, 112 patients (6.1%) had PAH. Median age of these patients was 38 years (range 18-81 years) and 40% were male. Of these patients, 58% had the Eisenmenger syndrome. Among the patients with a previously closed septal defect, 30 had PAH (3%). Ventricular septal defect (VSD) was the most frequent underlying defect (42%) among patients with PAH and a septal defect. Female sex (Odds ratio= 1.5, p=0.001) and sPAP (Odds ratio=0.04, p <0.001) were independently associated with a decreased functional class. Conclusion: PAH is common in adult patients with congenital heart disease. In our registry the prevalence of PAH in septal defects is around 6%. More than half of these patients have the Eisenmenger syndrome, which accounts for 1% of the total population in the CONCOR registry. Whether the prevalence of PAH will decrease in the future as a result of early detection and intervention remains to be awaited. (C) 2006 Elsevier Ireland Ltd. All rights reserved
AB - Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease is usually the result of a large systemic-to-pulmonary shunt, and often leads to right ventricular failure and early death. The purpose of this study was to determine the prevalence of PAH among adult patients included in a national registry of congenital heart disease and to assess the relation between patient characteristics and PAH. Methods: Patients with PAH associated with a septal defect were identified from the registry. Gender, age, underlying diagnosis, previous closure, age at repair and NYHA classification were recorded. PAH was defined as a systolic pulmonary arterial pressure (sPAP) greater than 40 mm Hg, estimated by means of echocardiographical evaluation. Results: The prevalence of PAH among all 5970 registered adult patients with congenital heart disease was 4.2%. Of 1824 patients with a septal defect in the registry, 112 patients (6.1%) had PAH. Median age of these patients was 38 years (range 18-81 years) and 40% were male. Of these patients, 58% had the Eisenmenger syndrome. Among the patients with a previously closed septal defect, 30 had PAH (3%). Ventricular septal defect (VSD) was the most frequent underlying defect (42%) among patients with PAH and a septal defect. Female sex (Odds ratio= 1.5, p=0.001) and sPAP (Odds ratio=0.04, p <0.001) were independently associated with a decreased functional class. Conclusion: PAH is common in adult patients with congenital heart disease. In our registry the prevalence of PAH in septal defects is around 6%. More than half of these patients have the Eisenmenger syndrome, which accounts for 1% of the total population in the CONCOR registry. Whether the prevalence of PAH will decrease in the future as a result of early detection and intervention remains to be awaited. (C) 2006 Elsevier Ireland Ltd. All rights reserved
U2 - https://doi.org/10.1016/j.ijcard.2006.09.017
DO - https://doi.org/10.1016/j.ijcard.2006.09.017
M3 - Article
C2 - 17182132
SN - 0167-5273
VL - 120
SP - 198
EP - 204
JO - International journal of cardiology
JF - International journal of cardiology
IS - 2
ER -