<abstract xmlns="http://www.w3.org/1999/xhtml"><bold>Introduction</bold>: Pulmonary arterial hypertension (PAH) is a rare disease associated with significant morbidity and mortality. Pediatric patients often present with mixted aetiologies.<bold>Objectives</bold>: To characterize the epidemiology, management and outcome of pediatric PAH.<bold>Methods</bold>: Children with PAH were included and followed prospectively for six months. WHO functional class, 6-minute walk test, biomarkers, electrocardiogram, spirometers and echocardiographic parameters were evaluated in progressive PAH group.<bold>Results</bold>: Two hundred and four children were included in the study from July 2012 until July 2013, with a mean age of 6.13 years. Transient PAH patients (n=170, 83.33%) included newborns with persistent pulmonary hypertension (n=8, 3.92%) and children with congenital heart defects with systemic-to-pulmonary shuntflow PAH (n=162, 79.41%) in whom PAH resolved after successful surgery correction. Progressive PAH (n=34, 16.66%) included patients with idiopathic PAH (n=5, 2.45%), Eisenmenger syndrome (n=17, 8.33%) and post-operative PAH (n= 6, 2.94%). Patients with progressive PAH remained stable in regards to clinical status, WHO functional class, 6-minute walk distance, biomarkers, spirometers parameters and echocardiographic parameters with prognostic value.<bold>Conclusions</bold>: Pediatric PAH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Pediatric PAH associated with congenital heart defects represents a heterogeneous group with highly variable clinical courses. PAH specific therapies may have contributed to disease stability and favorable outcomes.</abstract>

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease associated with significant morbidity and mortality. Pediatric patients often present with mixted aetiologies.Objectives: To characterize the epidemiology, management and outcome of pediatric PAH.Methods: Children with PAH were included and followed prospectively for six months. WHO functional class, 6-minute walk test, biomarkers, electrocardiogram, spirometers and echocardiographic parameters were evaluated in progressive PAH group.Results: Two hundred and four children were included in the study from July 2012 until July 2013, with a mean age of 6.13 years. Transient PAH patients (n=170, 83.33%) included newborns with persistent pulmonary hypertension (n=8, 3.92%) and children with congenital heart defects with systemic-to-pulmonary shuntflow PAH (n=162, 79.41%) in whom PAH resolved after successful surgery correction. Progressive PAH (n=34, 16.66%) included patients with idiopathic PAH (n=5, 2.45%), Eisenmenger syndrome (n=17, 8.33%) and post-operative PAH (n= 6, 2.94%). Patients with progressive PAH remained stable in regards to clinical status, WHO functional class, 6-minute walk distance, biomarkers, spirometers parameters and echocardiographic parameters with prognostic value.Conclusions: Pediatric PAH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Pediatric PAH associated with congenital heart defects represents a heterogeneous group with highly variable clinical courses. PAH specific therapies may have contributed to disease stability and favorable outcomes.

Epidemiological Characteristics and Prospective 6-Months Follow-up of Children with Pulmonary Arterial Hypertension

University of Medicine and Pharmacy Tîrgu Mureş, Department M4, IIIrd Pediatric Discipline

University of Medicine and Pharmacy Tîrgu Mureş, Department M3, Clinic of Cardiology

Acta Marisiensis - Seria Medica

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease associated with significant morbidity and mortality. Pediatric patients often present...

Epidemiological Characteristics and Prospective 6-Months Follow-up of Children with Pulmonary Arterial Hypertension

Published Online: Aug 06, 2015

Page range: 75 - 82

Received: Dec 09, 2014

Accepted: May 01, 2015

DOI: https://doi.org/10.1515/amma-2015-0026

Keywords
pulmonary arterial hypertension, children

© Şuteu Carmen et al.

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Epidemiological Characteristics and Prospective 6-Months Follow-up of Children with Pulmonary Arterial Hypertension

Published Online: Aug 06, 2015

Page range: 75 - 82

Received: Dec 09, 2014

Accepted: May 01, 2015

DOI: https://doi.org/10.1515/amma-2015-0026

Keywordspulmonary arterial hypertension, children

© Şuteu Carmen et al.

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
pulmonary arterial hypertension, children