Abstract:
Introduction:Pulmonary hypertension (PH) is adevastating, progressive disease with increasinglydebilitating symptoms and usually shortened overall lifeexpectancy due to a narrowing of the pulmonaryvasculature and consecutive right heart failure. Little isknown about PH in Africa, but limited reports suggestthat PH is more prevalent in Africa compared withdeveloped countries due to the high prevalence of riskfactors in the region.Methods and analysis:A multinational multicentreregistry-type cohort study was established and tailoredto resource-constraint settings to describe diseasepresentation, disease severity and aetiologies of PH,comorbidities, diagnostic and therapeutic management,and the natural course of PH in Africa. PH will bediagnosed by specialist cardiologists usingechocardiography (right ventricular systolic pressure>35 mm Hg, absence of pulmonary stenosis and acuteright heart failure), usually accompanied by shortnessof breath, fatigue, peripheral oedema and othercardiovascular symptoms, ECG and chest X-raychanges in keeping with PH as per guidelines(European Society of Cardiology and EuropeanRespiratory Society (ESC/ERS) guidelines). Additionalinvestigations such as a CT scan, a ventilation/perfusion scan or right heart catheterisation will beperformed at the discretion of the treating physician.Functional tests include a 6 min walk test and theKarnofsky Performance Score. The WHO classificationsystem for PH will be applied to describe the differentaetiologies of PH. Several substudies have beenimplemented within the registry to investigate specifictypes of PH and their outcome at up to 24 months.Data will be analysed by an independent institutionfollowing a data analyse plan.Ethics and dissemination:All local ethicscommittees of the participating centres approved theprotocol. The data will be disseminated through peer-reviewed journals at national and internationalconferences and public events at local care providers.
