BACKGROUND AND OBJECTIVES: With the increasing survival of preterm infants, pulmonary hypertension (PH) related to bronchopulmonary dysplasia (BPD) has become an important complication. The aim of this study was to investigate the characteristics and outcome of PH in preterm infants with BPD and to identify the risk factors for PH. SUBJECTS AND METHODS: We reviewed the records of 116 preterm infants with BPD cared for at a single tertiary center between 2004 and 2008. RESULTS: Twenty-nine (25%) infants had PH >2 months after birth. PH occurred initially at a median age of 65 days (range, 7-232 days). Severe BPD, a birth weight <800 g, long-term ventilator care and oxygen supplementation, a high ventilator setting, infection, and a patent ductus arteriosus (PDA) were related to PH based on univariate analysis (p<0.05). The infants who had longer oxygen supplementation were significantly more likely to have PH (odds ratio, 18.5; 95% confidence interval, 4.1-84.6; p<0.001). PH was improved in 76% of infants after a median of 85 days (range, 20-765 days). Four infants (14%) died. The death of 3 infants was attributed to PH. CONCLUSION: BPD was frequently complicated by PH. Although PH resolved in the majority of infants, PH in preterm infants with BPD can be fatal. Regular screening for PH and adequate management are required.
Birth Weight ; Bronchopulmonary Dysplasia ; Ductus Arteriosus, Patent ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Infant, Premature ; Mass Screening ; Oxygen ; Parturition ; Risk Factors ; Ventilators, Mechanical

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.
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