<p><b>OBJECTIVE</b>To analyze the clinical characteristics of
WHO Class I
pulmonary hypertension (PAH) in central
China.</p><p><b>
METHODS</b>Data was collected as a part of prospective
registry of PAH through Jan. 2009 to Oct. 2013 in Wuhan
Asia Heart Hospital. A total of 195
patients were recruited including 144 cases with
congenital heart disease with
pulmonary hypertension (CHD-PAH) and 51 cases with
idiopathic pulmonary hypertension (IPAH).</p><p><b>RESULTS</b>The age of all
patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years), 129 cases were
female (66.2%).
WHO Class I PAH accounted for 91.1%, CHD-PAH 67.3%, IPAH 23.8%, and other 8.9%.
WHO function class III/IV in newly diagnosed PAH accounted for 32.3%, the mean 6MWD was (397 ± 74) m. For
patients with IPAH, the median
time period between onset of symptoms and
diagnosis by right
heart catheterization was 38 months. The mean pulmonary
pressure,
pulmonary vascular resistance index, cardiac index of
patients with IPAH and CHD-PAH were measured by the right
heart catheterization and there was no difference between the two groups. Acute pulmonary
vasodilator testing was negative in all
patients in this cohort. Cardiac function was improved in the 121 cases
who received the targeted
drug treatment and 1
patient died out of these 121
patients while 5 cases died out of
patients receiving conventional
therapy.</p><p><b>CONCLUSION</b>In Central
China, Class I
pulmonary hypertension is the most predominant type of PAH, the cardiac function and
hemodynamic indexes of these
patients were significantly impaired at the
time of first PAH
diagnosis. Most of the
patients accepted targeted
drug treatment of
pulmonary hypertension, but the
drug dose used for the targeted
drug treatment is not effective enough in these
patients.</p>