title: The causes, treatment, and outcome of pulmonary hypertension in Africa: Insights from the Pan African Pulmonary Hypertension Cohort (PAPUCO) Registry.
abstract: BACKGROUND
  Epidemiology, aetiology, management and outcome data for various forms of pulmonary hypertension (PH) in Africa are scarce.
  
  
  METHODS
  A prospective, multinational cohort registry of 220 consecutive patients (97% of African descent) from 9 specialist centres in 4 African countries. The antecedents, characteristics and management of newly diagnosed PH plus 6-month survival were studied.
  
  
  RESULTS
  There were 209 adults (median age 48years [IQR 35, 64]) and 11 children (age range 1 to 17years). Most adults had advanced disease - 66% WHO Functional Class III-IV, median 6-minute walk test distance of 252m (IQR 120, 350) and median right ventricular systolic pressure 58mmHg (IQR 49, 74). Adults comprised 16% pulmonary arterial hypertension, 69% PH due to left heart disease, 11% PH due to lung disease and/or hypoxia, 2% chronic thromboembolic pulmonary hypertension, and 2% PH with unclear multifactorial mechanism. At 6-months, 21% of adults with follow-up data had died. On an adjusted basis (independent of sub-groups) mortality was associated with increasing functional impairment (p=0.021 overall - WHO Class IV versus I, OR 1.68 [95% CI 0.13, 4.36]) and presence of combined right atrial and ventricular hypertrophy (46% - OR 2.88, 95% CI 1.45, 5.72). Children commonly presented with dyspnoea, fatigue, cough, and palpitations with six and three children, respectively diagnosed with concurrent PH associated congenital heart disease and left heart disease.
  
  
  CONCLUSIONS
  These data provide new insights into PH from an African perspective, with clear opportunities to improve its prevention, treatment and outcomes.
  
  
  BACKGROUND
  ClinicalTrials.gov (NCT02265887).
authors.affiliation: Institute of Infectious Diseases and Molecular Medicine and Department of Medicine, Faculty of Health Science, University of Cape Town, Cape Town, South Africa.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Thienemann
authors.affiliation: Department of Internal Medicine, Douala General Hospital and Buea Faculty of Health Sciences, Douala, Cameroon.
authors.forename: Anastase
authors.initials: A
authors.lastname: Dzudie
authors.affiliation: Instituto Nacional de Saúde, and Universidade Eduardo Mondlane, Maputo, Mozambique.
authors.forename: Ana O
authors.initials: AO
authors.lastname: Mocumbi
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55902, USA.
authors.forename: Lori
authors.initials: L
authors.lastname: Blauwet
authors.affiliation: Department of Medicine, Bayero University Kano & Aminu Kano Teaching Hospital, PMB 3452 Kano, Nigeria.
authors.forename: Mahmoud U
authors.initials: MU
authors.lastname: Sani
authors.affiliation: Department of Medicine, Bayero University & Aminu Kano Teaching Hospital, Kano, Nigeria.
authors.forename: Kamilu M
authors.initials: KM
authors.lastname: Karaye
authors.affiliation: Division of Cardiology, Department of Medicine, University College Hospital Ibadan, Oyo State, Nigeria.
authors.forename: Okechukwu S
authors.initials: OS
authors.lastname: Ogah
authors.affiliation: Faculty of Medicine, Eduardo Mondlane University, Maputo, Mozambique.
authors.forename: Irina
authors.initials: I
authors.lastname: Mbanze
authors.affiliation: Department of Medicine, College of Medicine, University of Lagos, Lagos, Nigeria.
authors.forename: Amam
authors.initials: A
authors.lastname: Mbakwem
authors.affiliation: Department of Paediatrics, University of Uyo Teaching Hospital, Uyo, Nigeria.
authors.forename: Patience
authors.initials: P
authors.lastname: Udo
authors.affiliation: Department of Cardiovascular Medicine, Muhimbili National Hospital, Dar es Salaam, Tanzania; Hatter Institute for Cardiovascular Research in Africa and Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.
authors.forename: Kemi
authors.initials: K
authors.lastname: Tibazarwa
authors.affiliation: Faculty of Medicine, Eduardo Mondlane University, Maputo, Mozambique.
authors.forename: Albertino
authors.initials: A
authors.lastname: Damasceno
authors.affiliation: Mary MacKillop Institute for Health Research and NHMRC Centre for Research Excellence (CRE) to Reduce Inequality in Heart Disease, Australian Catholic University, Melbourne, Australia.
authors.forename: Ashley K
authors.initials: AK
authors.lastname: Keates
authors.affiliation: Hatter Institute for Cardiovascular Research in Africa and Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa; Mary MacKillop Institute for Health Research and NHMRC Centre for Research Excellence (CRE) to Reduce Inequality in Heart Disease, Australian Catholic University, Melbourne, Australia; Soweto Cardiovascular Research Unit, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Simon
authors.initials: S
authors.lastname: Stewart
authors.affiliation: Hatter Institute for Cardiovascular Research in Africa and Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa; Soweto Cardiovascular Research Unit, University of the Witwatersrand, Johannesburg, South Africa. Electronic address: Karen.Sliwa-Hahnle@uct.ac.za.
authors.forename: Karen
authors.initials: K
authors.lastname: Sliwa
mesh_terms: D000293:Adolescent; D000349:Africa / Q000453:epidemiology; D002648:Child; D065627:Familial Primary Pulmonary Hypertension / Q000175:diagnosis / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000150:complications / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy; D007223:Infant; D053208:Kaplan-Meier Estimate; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D016278:Ventricular Function, Right / Q000502:physiology
keywords: Africa; Congenital heart disease; HIV; Pulmonary hypertension; Registries