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Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry

Published online by Cambridge University Press:  20 December 2016

Tilman Humpl ,
Rolf M. F. Berger ,
Eric D. Austin ,
Margrit S. Fasnacht Boillat ,
Damien Bonnet ,
Dunbar D. Ivy ,
Malgorzata Zuk ,
Maurice Beghetti  and
Ingram Schulze-Neick
Tilman Humpl*
Affiliation:
Department of Critical Care Medicine, Division of Cardiology, Hospital for Sick Children, Toronto, Canada
Rolf M. F. Berger
Affiliation:
Department of Pediatrics, University Medical Center Groningen, Groningen, The Netherlands
Eric D. Austin
Affiliation:
Division of Pulmonary Medicine, Vanderbilt Children’s Hospital, Nashville, United States of America
Margrit S. Fasnacht Boillat
Affiliation:
Klinik für Kinder- und Jugendmedizin, Kantonsspital Winterthur, Winterthur, Switzerland
Damien Bonnet
Affiliation:
Paediatric Cardiology, Université Paris Descartes, Necker Enfants Malades, Paris, France
Dunbar D. Ivy
Affiliation:
Department of Pediatrics, Section of Cardiology, University of Colorado, Aurora, United States of America
Malgorzata Zuk
Affiliation:
Department of Cardiology, Children’s Memorial Health Institute, Warsaw, Poland
Maurice Beghetti
Affiliation:
Children’s University Hospital, Geneva, Switzerland
Ingram Schulze-Neick
Affiliation:
Great Ormond Street Hospital for Sick Children, London, United Kingdom
*
Correspondence to Dr T. Humpl, Department of Critical Care Medicine, Division of Cardiology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. Tel: +1 416 813 4918; E-mail: tilman.humpl@sickkids.ca.
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Abstract

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry.

Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg).

At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy.

There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.

Keywords

Pulmonary hypertensionchildrentreatment
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 6 , August 2017 , pp. 1123 - 1132
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Copyright
© Cambridge University Press 2016 

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Footnotes

*

TOPP registry investigators (not listed in the author’s list) by country alphabetical order: R. Weintraub, Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia. R. Geiger, Pediatric Cardiology, Innsbruck Medical University, Innsbruck, Austria. S. Mattos, Maternal-Fetal Cardiac Unit, Royal Portuguese Hospital, Recife, Brazil. Z. C. Jing and Z. Y. Han, Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. L. Sondergaard and T. Jensen, Rigshospitalet, Heart Center, University of Copenhagen, Copenhagen, Denmark. M. Levy, Paediatric Cardiology, Université Paris Descartes, Necker Enfants Malades, Paris, France. S. Mebus, German Heart Centre, Munich, Germany. C. Apitz, Universitätsklinikum Giessen, Giessen, Germany. A. Szatmari and L. Ablonczy, Pediatric Cardiac Center, Hungarian Institute of Cardiology, Budapest, Hungary. O. Milanesi and V. Favero, Dipartimento di Pediatria, Universita di Padova, Padova, Italy. B. T. Saji, Department of 1st Pediatrics, Division of Pediatric Cardiology, Toho University Medical Center Omori Hospital, Tokyo, Japan. T. Pulido and P. De La Garza, National Heart Institute, Mexico City, Mexico. J. M. Douwes, Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, The Netherlands. H. Brun, Rikshospitalet, Section for Pediatric Cardiology, Oslo, Norway. L. Moll and K. Michalak, Polish Mothers Hospital Research Institute, Lodz, Poland. W. Kawalec, Department of Cardiology, Children´s Memorial Health Institute, Warsaw, Poland. R. Olgunturk and S. Serdar Kula, Gazi Universitesi Tip Fakultesi, Pediyatrik Kardiyoloji ABD, Ankara, Turkey. A. Dursun, Hacettepe University Ihsan Dogramaci, Tip Fak. Pediyatrik Kardiyoloji, Ankara, Turkey. R. W. Day, Division of Pediatric Cardiology, University of Utah, University Health Care, Salt Lake City, United States of America. G. B. Mallory, Baylor College of Medicine, Texas Children’s Hospital, Houston, United States of America. D. Yung, Children’s Hospital and Regional Medical Center, Seattle, United States of America. D. J. Moore, Division of Cardiology, Vanderbilt Children’s Hospital, Nashville, United States of America. A. M. Atz, Medical University of South Carolina, Charleston, United States of America. J. A. Feinstein, Stanford University Medical Center Packard Children’s Hospital, Palo Alto, United States of America.

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