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

Abstract

BackgroundPulmonary 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.MethodsA 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.ResultsOverall, 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).ConclusionThis 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.

The changing landscape of pulmonary arterial hypertension and implications for patient care

“…Depending on the registry, the proportions range from 39% to 61% for IPAH, 11-28% for CTD-PAH, 10-20% for CHD-PAH and 5-10% for portopulmonary hypertension-PAH [4,7,9,12]. These findings do not appear to have changed over time.…”

“…The PH registry of the UK and Ireland found a significant increase in the age of patients over the period from 2001 to 2009 [6]. Similarly, data from the Czech registry in [2000][2001][2002][2003][2004][2005][2006][2007] showed that patients diagnosed during 2007 were 10 years older than the population diagnosed prior to 2007 [7].…”

“…The most commonly reported subtype of PAH in adult patients is IPAH, followed by PAH associated with concomitant diseases such as CTD, CHD or portal hypertension (so-called portopulmonary hypertension) [4,7,9,12]. Depending on the registry, the proportions range from 39% to 61% for IPAH, 11-28% for CTD-PAH, 10-20% for CHD-PAH and 5-10% for portopulmonary hypertension-PAH [4,7,9,12].…”

“…In recent years, diagnostic algorithms have been updated [32,33] that aid physicians in the diagnosis of PAH. Additionally, screening programmes [7] have increased the number of PAH referrals and increased use of diagnostic techniques, such as Doppler echocardiography, may influence rates of diagnosis [18].…”

“…PAH is a rare disease, with an estimated prevalence ranging from 10 to 52 cases per million [2][3][4][5][6][7].…”