ABSTRACT
<p><b>OBJECTIVE</b>To explore the demographic characteristics and clinical features of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.</p><p><b>METHODS</b>Between March 2007 and September 2010, IPAH diagnosis was confirmed by right heart catheterization in 150 adult patients from 31 clinical centers in China. Clinical and hemodynamic data were analyzed and patients were divided into WHO functional class I/II and WHO functional class III/IV group.</p><p><b>RESULTS</b>The mean age of 150 patients were 36 ± 13 years with female patient/male patient ratio of 2:1, and mean BMI was (21.3 ± 3.5) kg/m(2). Fatigue (n = 123, 82.0%) and dyspnea (n = 112, 74.7%) are the most common symptoms. Accentuated pulmonic second sound (P(2)) was detected in 92.0% (n = 138) of patients during physical examination, which was also the most common sign. About 49.0% (n = 73) patients were WHO functional class I/II patients and 46.0% (n = 68) patients were WHO functional class III/IV patients. Six minutes walking distance (6MWD) and Borg dyspnea score was (337 ± 101) m and 2.0 (2.0, 4.0), respectively. Right ventricular hypertrophy was suggested by ECG in 93.1% (n = 140) patients. Right atrial pressure was (10 ± 6) mm Hg, mean pulmonary artery pressure was (61 ± 16) mm Hg, cardiac index was (2.3 ± 0.8) L×min(-1)×m(-2) and pulmonary vascular resistance (1484 ± 699) dyn×s(-1)×cm(-5) in this cohort. 6 MWD (305 m ± 89 m vs. 377 m ± 88 m) was significantly shorter while Borg dyspnea score [3.0 (3.0, 5.0) vs. 2.0 (2.0, 3.0)] was significantly higher in WHO functional class III/IV patients than in WHO functional class I/II patients. Similarly hemodynamic parameters were also worse in WHO functional class III/IV patients than in WHO functional class I/II patients (all P < 0.05).</p><p><b>CONCLUSION</b>Idiopathic pulmonary arterial hypertension patients in this cohort affect mostly young adults, dominated by female gender and lower body mass index. Fatigue and dyspnea are the most common symptoms and accentuated pulmonic second sound (P(2)) is the most common sign. IPAH patients are often displaying severe functional and hemodynamic disturbance at first visit to hospitals. Dyspnea and hemodynamic impairment are related to 6MWD and WHO functional class.</p>
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Familial Primary Pulmonary Hypertension , Hemodynamics , Hypertension, Pulmonary , Diagnosis , Ventricular FunctionABSTRACT
<p><b>BACKGROUND</b>An echocardiographic right/left ventricular end-diastolic diameter ratio (RV/LV ratio) ≥ 0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. Right ventricular dilation is a common characteristic of both acute pulmonary embolism and idiopathic pulmonary arterial hypertension (IPAH). However, the prognostic value of the RV/LV ratio in patients with IPAH is unknown.</p><p><b>METHODS</b>Ninety-five consecutive patients with newly diagnosed IPAH were included, 17 were re-evaluated by echocardiography after 3 - 12 months of targeted therapy. Follow-up data were obtained by telephone interviews and review of the patients' records.</p><p><b>RESULTS</b>Higher RV/LV ratios were associated with greater functional impairment. The RV/LV ratio was positively correlated with pulmonary vascular resistance (r = 0.549, P < 0.001) and plasma N-terminal pro-brain natriuretic peptide level (r = 0.575, P < 0.001), but negatively correlated with cardiac output (r = -0.517, P < 0.001) and mixed venous oxygen saturation (r = -0.599, P < 0.001). Twenty-seven patients died during follow-up period. Sensitivity and specificity of an RV/LV ratio ≥ 0.84 for predicting death were 85.2% and 51.5%, respectively. The RV/LV ratio and body mass index were independent predictors of death by multivariate Cox analysis (P < 0.01). A baseline RV/LV ratio ≥ 0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis (P < 0.01).</p><p><b>CONCLUSION</b>The RV/LV ratio helps to assess the severity of IPAH and may serve as an independent predictor of prognosis in patients with IPAH.</p>