Related Experiment Video
Updated: Apr 8, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience
Małgorzata Żuk1, Katarzyna Mazurkiewicz-Antoń, Anna Migdał
1Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland. m.zuk@czd.pl.
Insights
Pediatric pulmonary arterial hypertension (PAH) survival rates are comparable to published data. Disease severity at diagnosis, including right atrial pressure and pulmonary resistance, significantly impacts mortality risk in children with PAH.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Clinical Outcomes Analysis
Background:
- Pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting pulmonary arterioles, often with a poor prognosis.
- Understanding survival and prognostic factors is crucial for managing pediatric PAH.
Purpose of the Study:
- To evaluate survival rates and identify prognostic factors in children diagnosed with PAH between 2004 and 2013.
- To assess the impact of disease severity and treatment on outcomes in a pediatric PAH cohort.
Main Methods:
- Retrospective analysis of 55 children (average age 6.2 years) diagnosed with idiopathic PAH, PAH with shunts, or post-cardiac surgery PAH.
- Assessment of survival data, baseline clinical parameters, N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, World Health Organization functional class (WHO-FC), and treatment modalities.
Main Results:
- Overall survival rates at one, three, five, and ten years were 83.1%, 77.1%, 70.7%, and 65.2%, respectively.
- Lower baseline NT-proBNP (< 605 pg/mL), lower WHO functional class (I/II), and absence of syncope were associated with better prognosis.
- Higher right atrial pressure and pulmonary resistance were significant risk factors for mortality.
Conclusions:
- Survival in this pediatric PAH cohort is comparable to current international registry data.
- Disease severity at diagnosis, particularly elevated right atrial pressure and pulmonary resistance, is a key determinant of mortality risk in pediatric PAH.
Background:
Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis.
Aim:
To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004–2013.
Methods:
The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH — n = 23 (42%), PAH associated with systemic-to-pulmonary shunts — n = 17 (31%), and PAH after corrective cardiac surgery — n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy.
Results:
During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03).
Conclusions:
Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.
More Related Videos
11:20A Rodent Model of The Ross Operation: Syngeneic Pulmonary Artery Graft Implantation in A Systemic Position
Published on: April 1, 2022
09:23Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension
Published on: February 11, 2017
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:16Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
01:20COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...