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.
Highlights
Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, leading to right heart failure and death
Patient population Between January 2000 and December 2007, 191 patients were diagnosed with PAH and included in the registry (79.6% at the 2nd Department of Internal Medicine, General University Hospital and 1st Medical Faculty of Charles University, Prague and 20.4% at the Cardiology Center, Institute for Clinical and Experimental Medicine, Prague)
APAH-CHD, pulmonary arterial hypertension associated with congenital heart disease; APAH-CTD, pulmonary arterial hypertension associated with connective tissue disease. †Other PAH etiologies included: portal hypertension and PAH associated with hemolytic anemia. *Difference between prevalent and incident cases maximal likelihood chi-square test
Summary
Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, leading to right heart failure and death. Pulmonary arterial hypertension (PAH) is a chronic, progressive and potentially fatal disease of the pulmonary vasculature that leads to heart failure. PAH is a clinical condition characterized by the presence of pre-capillary pulmonary hypertension. It is defined as an increase in mean pulmonary arterial pressure (PAP) ≥25 mmHg at rest in the absence of significantly elevated pulmonary capillary wedge pressure (PCWP ≤15 mmHg) as assessed by right heart catheterization (RHC) [1]. PAH is classified into a number of categories according to etiology [1]. At the recent World Symposium on Pulmonary Hypertension (PH [Nice 2013]) there were some suggestions to update the classification of PH, for example, to classify patients with hemolytic anemia into Group 5 [2]; in this paper we have classified patients according to the current guidelines
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