OBJECTIVETo study the epidemiology, clinical characteristics and prognosis of children with pulmonary arterial hypertension (PAH) secondary to connective tissue disease (CTD).

METHODSThe clinical record files of the pediatric inpatient with PAH in the population of CTD in the hospital treated between January 2000 and January 2007 were analyzed retrospectively.

RESULTS(1) In 299 patients with CTD and complete Doppler echocardiography files, 31 (31/299, 10.4%) patients (28 females and 3 males), aged from 7 to 18 years (average: 12.5 years), were found to have PAH, in whom, 5(62.5%)in 8 patients with overlapped CTD (OCTD), 2 (50.0%) in 4 patients with antiphospholipid syndrome (APS), 4(28.6%) in 14 patients with systematic vasculitis (SV), 3 (10.7%) in 28 patients with dermatomyositis (DM), 17(7.6%) in 223 systemic lupus erythematosus (SLE) had PAH. The CTD-associated PAH occurred in the 3rd week to 5th year after initial CTD manifestations (median onset: 1.5 years). (2) The onset of CTD-associated PAH was obscure and children with severe CTD-associated PAH presented with dyspnea (18/31, 58.1%) and heart failure (9/31, 29.0%). The children with Raynaud's phenomenon or positive anticardiophospholipid antibody (ACL) or positive lupus anticoagulant (LA) were prone to have more severe PAH. (3) Doppler echocardiography and pulmonary function test, especially the test of pulmonary diffusion function of CO (D(LCO)) were necessary to detect PAH early. (4) After treatment, the pulmonary arterial pressure in mild and moderate PAH cases could be normalized and in severe PAH cases could be decreased to mild or moderate levels. There was a lower PaO(2) level (P < 0.01), a higher pulmonary arterial systolic pressure (PASP) level (P < 0.05) in the cases of CTD-PAH who died as compared with the live patients.

CONCLUSIONSPAH is a common complication of CTDs, which occurs often 1.5 years after initial CTD manifestations. The early associated symptom is obscure and the severe cases manifest with dyspnea and heart failure. Those with Raynaud's phenomenon and positive ACL and LA are prone to develop more severe PAH. Early and regular Doppler echocardiography and pulmonary function test are necessary to detect PAH early. Severe CTD associated PAH in children could lead to poor outcome. PASP classification and PaO(2) level are important factors affecting prognosis.