More data are required to support the use of risk equations and risk scores to assess subsequent risk. Similarly, apart from the REVEAL score, which includes age >60 years and kidney dysfunction, the actual risk assessment instruments do not take into account age or comorbidity as prognostic markers.
Interestingly, and in contrast to what is seen at many PAH clinics, most randomised controlled drug trials exclude elderly patients with multiple comorbidities. Improved evaluation of older patients with dyspnoea might also contribute to this demographic shift. These patients may share features of both IPAH and PH secondary to diastolic heart failure, as shown by O pitz et al. Late-onset IPAH among older patients may include some degree of left ventricular diastolic dysfunction leading to a particular phenotype of “mixed” pre- and postcapillary PH. Prevalence among the elderly is increasing, with a mean age of 50–65 years reported at diagnosis. However, contemporary reports from worldwide pulmonary hypertension (PH) research registries show a new demographic picture for the IPAH population. Now, new treatments are available that target the disease-related vasoactive pathways and influence symptoms, quality of life and survival. The first consensus guidelines for the diagnosis and treatment of IPAH were published in 1993. Without effective treatments, the median survival time was estimated to be 2.8 years. This registry included 194 patients, with a mean age of 36 years, of whom 68% were women. The National Institutes of Health in the United States initiated the first large registry enrolling patients with idiopathic PAH (IPAH) 40 years ago. The increased right ventricular afterload results in right ventricular failure and ultimately death. Pulmonary arterial hypertension (PAH) is a deleterious, incurable disease that affects the small pulmonary arteries, with vasoconstriction and vascular proliferation leading to severe remodelling and an increased pulmonary vascular resistance. These findings highlight the importance of age and specific comorbidities as prognostic markers of outcome in addition to established risk assessment algorithms. Ischaemic heart disease and kidney dysfunction independently predicted survival. 5-year survival was highest in patients aged 18–45 years (88%), while the survival rates were 63%, 56% and 36% for patients in the groups 46–64, 65–74 and ≥75 years, respectively (p<0.001).
In the two youngest age groups, a significant number of patients improved (18–45 years, Z= −4.613, p<0.001 46–64 years, Z= −2.125, p=0.034), but no significant improvement was found in the older patient groups. The change in risk group from baseline to follow-up (median 5 months) and survival were compared across age groups. Individual risk profiles were determined according to a risk assessment instrument, based on the European Society of Cardiology and the European Respiratory Society guidelines. The study population (n=264) was categorised into four age groups: 18–45, 46–64, 65–74 and ≥75 years. We aimed to investigate the effects of age and comorbidity on risk stratification and outcome of patients with incident IPAH. Recent reports from worldwide pulmonary hypertension registries show a new demographic picture for patients with idiopathic pulmonary arterial hypertension (IPAH), with an increasing prevalence among the elderly.
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