Pulmonary arterial hypertension after operation for congenital heart disease: analysis of baseline clinical characteristics of 122 Chinese patients / 中华心血管病杂志

ABSTRACT

<p><b>OBJECTIVE</b>To explore the baseline clinical characteristics of patients with pulmonary arterial hypertension after operation for congenital heart disease (AO-CHD-PAH) in China.</p><p><b>METHODS</b>A total of 122 consecutive patients diagnosed as AO-CHD-PAH in Shanghai Pulmonary Hospital from September 1, 2006 to August 31, 2011 were retrospective analyzed.</p><p><b>RESULTS</b>Sixty-seven percent patients were female. The median age at procedure and diagnosis were 14.8 and 24.8 years old, respectively. The median duration from procedure of CHD to diagnosis of PAH was 7.3 years. The most frequent initial symptom (109/122, 89.3%) attributed to PAH was dyspnea on exertion. The frequent sign was accentuation of second heart sound on pulmonary valve area (113/122, 92.6%).Incidence of WHO functional PAH classes I/II was 53.3% (65/122) and mean 6 minutes walk distance was limited to (408.4 ± 103.3) m. Borg dyspnea score was 2.0 (1.0, 3.0).Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure [(71.6 ± 24.2) mmHg, 1 mmHg = 0.133 kPa] and pulmonary vascular resistance index [(29.1 ± 16.0) Wood U·m(2)] in this patient cohort. Cardiac index was (2.7 ± 0.9) L·min(-1)·m(-2), 90.2% (110/122) patients received PAH-specific therapy. The majority of PAH-specific therapy was phosphodiesterase type 5 inhibitor, which is somehow different from PAH-specific therapy regimen of American-European developed countries.</p><p><b>CONCLUSIONS</b>PAH-specific therapy rate is satisfactory for pulmonary arterial hypertension after operation for congenital heart disease in China and phosphodiesterase type 5 inhibitors are predominant PAH-specific medication in China.</p>