Characteristics, Long-term Survival, and Risk Assessment of Pediatric Pulmonary Arterial Hypertension in China

Abstract

BackgroundRegistry-based studies of pediatric pulmonary arterial hypertension (PPAH) are scarce in developing countries, including China. The PPAH risk assessment tool needs further evaluation and improvement.Research QuestionWhat are the characteristics and long-term survival of PPAH in China and what is the performance of the PPAH risk model in Chinese patients?Study Design and MethodsPatients with PAH were enrolled in the national prospective multicenter registry from August 2009 through December 2019. Children 3 months to 18 years of age at the time of PAH diagnosis were analyzed.ResultsA total of 247 children with PAH were enrolled. The median patient age was 14.8 years, and 58.3% of patients were female. Most patients had a diagnosis of PAH associated with congenital heart disease (CHD; 61.5%) and idiopathic or heritable PAH (37.7%). The median time from symptom onset to PAH diagnosis was 24 months. The mean pulmonary artery pressure and pulmonary vascular resistance index were 70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood Units·m2, respectively. Patients with CHD-associated PAH experienced a longer diagnostic delay and demonstrated higher pulmonary artery pressure, but better cardiac performance, than those with idiopathic or heritable PAH. An increased number of patients received targeted therapy at the last follow-up compared with baseline. The 5- and 10-year survival rates of the entire cohort were 74.9% and 55.7%, respectively, with better survival in patients with CHD-associated PAH than in those with idiopathic or heritable PAH. Patients with low risk had better survival than those with high risk according to the simplified noninvasive risk score model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up.InterpretationPatients with PPAH in China showed severely compromised hemodynamics with marked diagnostic delay. The long-term survival of PPAH is poor despite the increased usefulness of targeted drugs. The simplified noninvasive risk model demonstrated good performance for predicting survival in Chinese children with PAH.Trial RegistryClinicalTrials.gov; No.: NCT01417338; URL: www.clinicaltrials.gov Registry-based studies of pediatric pulmonary arterial hypertension (PPAH) are scarce in developing countries, including China. The PPAH risk assessment tool needs further evaluation and improvement. What are the characteristics and long-term survival of PPAH in China and what is the performance of the PPAH risk model in Chinese patients? Patients with PAH were enrolled in the national prospective multicenter registry from August 2009 through December 2019. Children 3 months to 18 years of age at the time of PAH diagnosis were analyzed. A total of 247 children with PAH were enrolled. The median patient age was 14.8 years, and 58.3% of patients were female. Most patients had a diagnosis of PAH associated with congenital heart disease (CHD; 61.5%) and idiopathic or heritable PAH (37.7%). The median time from symptom onset to PAH diagnosis was 24 months. The mean pulmonary artery pressure and pulmonary vascular resistance index were 70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood Units·m2, respectively. Patients with CHD-associated PAH experienced a longer diagnostic delay and demonstrated higher pulmonary artery pressure, but better cardiac performance, than those with idiopathic or heritable PAH. An increased number of patients received targeted therapy at the last follow-up compared with baseline. The 5- and 10-year survival rates of the entire cohort were 74.9% and 55.7%, respectively, with better survival in patients with CHD-associated PAH than in those with idiopathic or heritable PAH. Patients with low risk had better survival than those with high risk according to the simplified noninvasive risk score model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up. Patients with PPAH in China showed severely compromised hemodynamics with marked diagnostic delay. The long-term survival of PPAH is poor despite the increased usefulness of targeted drugs. The simplified noninvasive risk model demonstrated good performance for predicting survival in Chinese children with PAH. ClinicalTrials.gov; No.: NCT01417338; URL: www.clinicaltrials.gov FOR EDITORIAL COMMENT, SEE PAGE 1353Take-home PointsStudy Question: What are the characteristics and long-term survival of pediatric pulmonary arterial hypertension (PPAH) in China and what is the performance of the PPAH risk model in Chinese patients?Results: A total of 247 children with PAH were enrolled (median age, 14.8 years; 58.3% female) who experienced a long diagnostic delay (median, 24 months), showed marked elevated mean pulmonary artery pressure and pulmonary vascular resistance index (70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood Units·m2, respectively), and showed a 10-year survival of 55.7%. Patients with low risk demonstrated better survival than those with high risk according to the simplified noninvasive risk model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up.Interpretation: Patients with PPAH in China showed severely compromised hemodynamics and poor long-term survival with marked diagnostic delay, and the simplified noninvasive risk score model demonstrated good performance for predicting survival in Chinese children with PAH. FOR EDITORIAL COMMENT, SEE PAGE 1353 Study Question: What are the characteristics and long-term survival of pediatric pulmonary arterial hypertension (PPAH) in China and what is the performance of the PPAH risk model in Chinese patients? Results: A total of 247 children with PAH were enrolled (median age, 14.8 years; 58.3% female) who experienced a long diagnostic delay (median, 24 months), showed marked elevated mean pulmonary artery pressure and pulmonary vascular resistance index (70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood Units·m2, respectively), and showed a 10-year survival of 55.7%. Patients with low risk demonstrated better survival than those with high risk according to the simplified noninvasive risk model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up. Interpretation: Patients with PPAH in China showed severely compromised hemodynamics and poor long-term survival with marked diagnostic delay, and the simplified noninvasive risk score model demonstrated good performance for predicting survival in Chinese children with PAH. Pulmonary arterial hypertension (PAH) is a devastating disease that can affect all age groups. In contrast to adult PAH, registry data on pediatric PAH (PPAH) are relatively limited, especially in developing countries. The pulmonary hypertension (PH) registries from developing countries include only a small number of pediatric patients with PAH, which is far from representative.1Echazarreta D.F. Perna E.R. Coronel M.L. et al.Collaborative registry of pulmonary hypertension in Argentina (RECOPILAR). Final analysis.Medicina. 2021; 81: 180-190PubMed Google Scholar,2Thienemann F. Dzudie A. Mocumbi A.O. et al.The causes, treatment, and outcome of pulmonary hypertension in Africa: insights from the Pan African Pulmonary Hypertension Cohort (PAPUCO) Registry.Int J Cardiol. 2016; 221: 205-211Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar Some PPAH registries from developed countries provided valuable data on various aspects of the disease.3Fasnacht M.S. Tolsa J.F. Beghetti M. The Swiss registry for pulmonary arterial hypertension: the paediatric experience.Swiss Medical Weekly. 2007; 137: 510-513PubMed Google Scholar, 4van Loon R.L. Roofthooft M.T. Delhaas T. et al.Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies.Am J Cardiol. 2010; 106: 117-124Abstract Full Text Full Text PDF PubMed Scopus (113) Google Scholar, 5Fraisse A. Jais X. Schleich J.M. et al.Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France.Arch Cardiovasc Dis. 2010; 103: 66-74Crossref PubMed Scopus (109) Google Scholar, 6Haworth S.G. Hislop A.A. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.Heart (British Cardiac Society). 2009; 95: 312-317Crossref PubMed Scopus (242) Google Scholar, 7Moledina S. Hislop A.A. Foster H. Schulze-Neick I. Haworth S.G. Childhood idiopathic pulmonary arterial hypertension: a national cohort study.Heart (British Cardiac Society). 2010; 96: 1401-1406Crossref PubMed Scopus (186) Google Scholar, 8Barst R.J. McGoon M.D. Elliott C.G. Foreman A.J. Miller D.P. Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google Scholar However, most of the existing registries are limited primarily to relatively small sample sizes, retrospective designs, single centers, or short follow-up duration.3Fasnacht M.S. Tolsa J.F. Beghetti M. The Swiss registry for pulmonary arterial hypertension: the paediatric experience.Swiss Medical Weekly. 2007; 137: 510-513PubMed Google Scholar, 4van Loon R.L. Roofthooft M.T. Delhaas T. et al.Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies.Am J Cardiol. 2010; 106: 117-124Abstract Full Text Full Text PDF PubMed Scopus (113) Google Scholar, 5Fraisse A. Jais X. Schleich J.M. et al.Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France.Arch Cardiovasc Dis. 2010; 103: 66-74Crossref PubMed Scopus (109) Google Scholar, 6Haworth S.G. Hislop A.A. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.Heart (British Cardiac Society). 2009; 95: 312-317Crossref PubMed Scopus (242) Google Scholar, 7Moledina S. Hislop A.A. Foster H. Schulze-Neick I. Haworth S.G. Childhood idiopathic pulmonary arterial hypertension: a national cohort study.Heart (British Cardiac Society). 2010; 96: 1401-1406Crossref PubMed Scopus (186) Google Scholar, 8Barst R.J. McGoon M.D. Elliott C.G. Foreman A.J. Miller D.P. Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google Scholar Pediatric PH registries enroll larger numbers of patients with PAH, but detailed characteristics and survival of PAH subgroups are not demonstrated fully.9Berger R.M. Beghetti M. Humpl T. et al.Clinical features of paediatric pulmonary hypertension: a registry study.Lancet. 2012; 379: 537-546Abstract Full Text Full Text PDF PubMed Scopus (328) Google Scholar,10Abman S.H. Mullen M.P. Sleeper L.A. et al.Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry.Eur Respir J. 2022; 59: PubMed Scopus Google Scholar In the characteristics and survival of PPAH in countries and can a of of medical and diagnostic and et and survival of pulmonary arterial hypertension in insights from a national prospective 2022; PubMed Scopus Google M.S. 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Jais X. Schleich J.M. et al.Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France.Arch Cardiovasc Dis. 2010; 103: 66-74Crossref PubMed Scopus (109) Google S.G. Hislop A.A. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.Heart (British Cardiac Society). 2009; 95: 312-317Crossref PubMed Scopus (242) Google R.J. McGoon M.D. Elliott C.G. Foreman A.J. Miller D.P. Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google R.M. Beghetti M. Humpl T. et al.Clinical features of paediatric pulmonary hypertension: a registry study.Lancet. 2012; 379: 537-546Abstract Full Text Full Text PDF PubMed Scopus (328) Google Scholar,10Abman S.H. Mullen M.P. Sleeper L.A. et al.Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry.Eur Respir J. 2022; 59: PubMed Scopus Google Loon R.L. Roofthooft M.T. et pulmonary hypertension in the and the to PubMed Scopus Google A. A. et pulmonary hypertensive vascular disease in from the J Respir PubMed Scopus Google Scholar The patients received a diagnosis at age with a longer diagnostic delay, the in the diagnosis of PPAH in China. 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Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google R.M. Beghetti M. Humpl T. et al.Clinical features of paediatric pulmonary hypertension: a registry study.Lancet. 2012; 379: 537-546Abstract Full Text Full Text PDF PubMed Scopus (328) Google Loon R.L. Roofthooft M.T. et pulmonary hypertension in the and the to PubMed Scopus Google Scholar pulmonary vascular disease at age and longer diagnostic delay the in hemodynamics can associated with the high of patients with CHD-associated PAH, who showed higher and at diagnosis than patients in The long-term of to the of pulmonary vascular Patients with most those with CHD-associated PAH, with the pulmonary arterial pressure and subgroups of patients with CHD-associated PAH. 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Schleich J.M. et al.Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France.Arch Cardiovasc Dis. 2010; 103: 66-74Crossref PubMed Scopus (109) Google R.J. McGoon M.D. Elliott C.G. Foreman A.J. Miller D.P. Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google Loon R.L. Roofthooft M.T. et pulmonary hypertension in the and the to PubMed Scopus Google Scholar However, the 10-year survival of patients with PPAH in China was only 55.7%, than that of adult et and survival of pulmonary arterial hypertension in insights from a national prospective 2022; PubMed Scopus Google Scholar in the early and diagnosis of PAH in and better to the of patients with PPAH in China. In a and increased in children with PAH that patients with of Loon R.L. Roofthooft M.T. Delhaas T. et al.Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies.Am J Cardiol. 2010; 106: 117-124Abstract Full Text Full Text PDF PubMed Scopus (113) Google S. Hislop A.A. Foster H. Schulze-Neick I. Haworth S.G. Childhood idiopathic pulmonary arterial hypertension: a national cohort study.Heart (British Cardiac Society). 2010; 96: 1401-1406Crossref PubMed Scopus (186) Google R.J. McGoon M.D. Elliott C.G. Foreman A.J. Miller D.P. Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management.Circulation. 2012; 125: 113-122Crossref PubMed Scopus (287) Google Scholar and to affect the disease and of patients PH with in adult A. of and long-term survival in patients with pulmonary J Cardiol. Full Text Full Text PDF PubMed Scopus Google M. et and in idiopathic pulmonary arterial hypertension: and Cardiol. 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