Korean Circ J.  2008 Dec;38(12):644-650. 10.4070/kcj.2008.38.12.644.

Pulmonary Arterial Hypertension in Children: A Single Center Experience

• Kim HW
• Kim GB
• Je HG
• Beak JS
• Bae EJ
• Noh CI
• Choi JY
• Yun YS

• KMID: 2225697
• DOI: http://doi.org/10.4070/kcj.2008.38.12.644

Abstract

BACKGROUND AND OBJECTIVES
Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. The aim of this study was to characterize PAH in pediatric patients by evaluating the patients demographics, clinical and hemodynamic variables, treatments, and outcomes. SUBJECTS AND METHODS: Sixty-five patients who were diagnosed with PAH at Seoul National University Children's Hospital between January 1985 and August 2007 were retrospectively reviewed. RESULTS: There was no difference in gender distribution (males, 33; females, 32). The mean age at the time of diagnosis was 5.7+/-5.2 years and the mean follow-up period was 6.3+/-5.5 years. The major causes of PAH were congenital heart disease (CHD) in 32 patients (49.2%) and idiopathic PAH in 11 patients (16.9%). The most common presenting symptom was dyspnea in 44 patients (67.7%). The mean cardiothoracic ratio was 58.9+/-8.3%, which decreased to 55.9+/-8.3% after vasodilator therapy (p=0.011). The mean pulmonary arterial pressure at the time of cardiac catheterization was 59.7+/-18.7 mmHg and the mean pulmonary vascular resistance was 14.9+/-9.7 wood units . m2. Forty-three of 65 patients (66.2%) had vasodilator therapy (prostacycline, sildenafil, and bosentan). A statistically significant decrease in tricuspid valve regurgitation velocity (4.8+/-0.8 m/sec vs. 3.6+/-1.0 m/sec, p=0.001), and an increase in diastolic dimension of the left ventricle (28.3+/-12.1 mm vs. 33.2+/-10.2 mm, p=0.021) on echocardiography before and after vasodilator therapy was demonstrated. The 5-, 10-, and 15-year survival rates were 96%, 92% and 65%, respectively. Six of 65 patients (9.2%) with PAH died. There was no significant correlation between outcome and the immediate response to the vasodilators. CONCLUSION: PAH is a devastating disease which is rare in children. PAH in children has a variable pattern cause and progression, the confirmation of which requires analysis of detailed registries from nationwide hospitals.

Keyword

Hypertension, pulmonary; Child; Epidemiology; Heart defects, congenital

MeSH Terms

Arterial Pressure
Cardiac Catheterization
Cardiac Catheters
Child
Demography
Dyspnea
Echocardiography
Female
Follow-Up Studies
Heart Defects, Congenital
Heart Diseases
Heart Ventricles
Hemodynamics
Humans
Hypertension
Hypertension, Pulmonary
Piperazines
Prognosis
Purines
Rare Diseases
Registries
Retrospective Studies
Sulfones
Survival Rate
Tricuspid Valve Insufficiency
Vascular Resistance
Vasodilator Agents
Wood
Sildenafil Citrate
Hypertension, Pulmonary
Piperazines
Purines
Sulfones
Vasodilator Agents

Figure

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  Fig. 1 Comparison of mean pulmonary arterial pressure (PAP) and pulmonary vascular resistance (Rp) change during the vasodilator test. After the vasodilator test, (A) the mean PAP and (B) Rp decreased significantly (p<0.05).

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  Fig. 2 Kaplan-Meier survival curve in patients with pulmonary arterial hypertension. The 5-, 10-, and 15-year survival rates were 96%, 92%, and 65%, respectively.

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