Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Epidemiological data from national registries are growing worldwide, but are still unavailable in Eastern Europe. METHODS: A PAH registry was initiated in January 2007 using a nationwide network of echocardiographic centers and four diagnostic centers that specialize in PAH. All patients aged above 18 years, diagnosed with PAH and monitored between January 2000 and December 2007 were included. Patients diagnosed with PAH between January and December 2007 were classified as incident. The survival analyses were performed up to the end of 2010. Prognostic factors at the time of diagnosis were identified using uni- and multivariable Cox proportional hazard models. RESULTS: Overall, 191 patients were included (100 prevalent cases, 91 incident cases). Patients were predominantly female (n = 125) and had a mean age of 51.9 ± 16.9 years. Incident patients were significantly older at the time of diagnosis than prevalent patients (p < 0.001). Most patients (60.7%) had idiopathic PAH; 20.4% had PAH associated with congenital heart disease and 11.4% had PAH associated with connective tissue disease. Estimates of prevalence and incidence of PAH in adults were 22.4 cases per million and 10.7 cases per million per year, respectively. The 1-, 2- and 3-year survival rates in the incident PAH cohort were 89% (95% confidence intervals [CI] 83-95%), 78% (95% CI 70-87%) and 74% (95% CI 65-83%), respectively. Lower survival rates were significantly associated with higher age (hazard ratio [HR] 6.6 95% CI 1.4-30.9) and lower creatinine clearance (HR 3.3 95% CI 1.1-9.7). CONCLUSION: This is the first study in Eastern Europe to describe the prevalence, incidence and survival of patients with PAH from a national representative registry. This registry from the Czech Republic highlights that diagnosis of PAH is frequently made late in the disease continuum when patients have significant functional impairment.

Carotid intima-media thickness in young survivors of acute myocardial infarction.

BACKGROUND: Carotid intima-media thickness (CIMT) is considered to be a useful surrogate marker of coronary atherosclerosis. However, it is unclear whether this applies to young patients with acute myocardial infarction (AMI), in whom most cases are attributable to the destabilization of focal atheroma. OBJECTIVE: To assess CIMT in patients experiencing AMI at a young age. METHODS: CIMT was investigated in young survivors of AMI (78 male and 20 female) occurring before 45 years of age in men and before 50 years of age in women. CIMT values were compared with those of a sex-, age- and smoking status-matched sample selected from participants of a large epidemiological survey (115 men and 144 women). CIMT was measured on the anterior and posterior walls of the distal common carotid artery. RESULTS: In post-AMI male patients, the mean average CIMT, comprised of measurements of both the near and far walls on both sides, was significantly increased compared with controls (0.67±0.10 mm versus 0.60±0.09 mm; P<0.001), while it did not differ in post-AMI female patients (0.60±0.07 mm versus 0.60±0.10 mm). Mean maximum CIMT was greater in both male and female post-AMI patients (0.94±0.15 mm versus 0.81±0.13 mm; P<0.001 in men and 0.89±0.14 mm versus 0.80±0.11 mm; P=0.001 in women). CONCLUSIONS: In young AMI survivors, CIMT appeared to be significantly increased to a greater extent in men than in women. Although most patients had single- or double-vessel coronary disease, the overall increase in CIMT suggests that their coronary events were not due to destabilization of a single focal atheroma but may have reflected a generalized atherosclerotic process.