The aetiology, clinical presentation and treatment of patients with pulmonary hypertension in Cape Town: A preliminary report from the Groote Schuur Hospital Pulmonary Hypertension Registry

Abstract

Background There is a paucity of knowledge about pulmonary hypertension (PH) in sub-Saharan Africa and an urgent need for its investigation in this context. The impact of HIV infection in PH is also unknown. Objectives To determine the aetiology, clinical presentation, severity and current management of PH at a tertiary-level hospital in Cape Town, South Africa (SA). Methods Demographic and clinical data, including from special investigations, were captured retrospectively for all patients referred to the Groote Schuur Hospital Pulmonary Hypertension Clinic between October 2015 and November 2017 (n=58) and entered into an online registry. Descriptive statistics were used to present the baseline data at enrolment. Results Patients were mainly young and female and almost half (48.3%) had severe symptoms according to World Health Organization classification. The main aetiologies were pulmonary arterial hypertension (PAH) and chronic thromboembolic PH. More than a fifth of the patients were HIV-positive, with nine patients presenting with HIV-associated PAH. The median time from initial presentation to referral to a specialist centre was 227 days (interquartile range: 72 - 625 days). Only a small number of patients were on PH-specific treatment at enrolment and a notable number never underwent right-heart catheterisation. Conclusion PH diagnosis is often delayed and even at a tertiary institution with a dedicated clinic and access to special investigations, PH is suboptimally investigated and managed. Expansion of this registry to better understand the phenotype of this disease in SA can improve outcomes for these patients through awareness, early identification and effective management.

Pulmonary hypertension: Spectrum of disease, clinical presentation and treatment outcomes at the main respiratory pulmonary hypertension clinic in KwaZulu‑Natal Province, South Africa

“…The present study highlights that HIV is probably the most common cause of disease in KZN Province and SA, as found in the Groote Schuur registry. [2] However, it contrasts with studies from Europe, [7,8] where the prevalence of HIV-associated PH is ~0.5%. There have not been many studies done in high-HIV prevalent areas to determine the true prevalence of HIV-associated PH.…”

“…The majority of patients were female, which is consistent with international trends [5] and the findings of the Groote Schuur registry study. [2] The racial balance did not follow provincial demographics, with ethnic Indians having the second highest prevalence although they are the minority in the province. This may suggest a genetic predisposition to the disease or a possible referral bias.…”

“…Pulmonary arterial hypertension (PAH) is an uncommon and lifethreatening disease with limited literature regarding its prevalence and aetiology in South Africa (SA). [1 , 2] Pulmonary hypertension (PH) was initially classified as a mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest; however, this was changed at the 6th World Symposium on Pulmonary Hypertension in 2018 to be mPAP >20 mmHg at rest. [3] It is broadly classified into 5 groups by the World Health Organization (WHO) based on similar clinical and pathophysiological characteristics.…”

“…[4] There is paucity of literature regarding the epidemiology of PH in sub-Saharan Africa. A recent study by Davies van Es et al [2] analysed the PH registry at Groote Schuur Hospital in Cape Town. The Pan African Pulmonary Hypertension Cohort (PAPUCO) is the largest registry in Africa, comprising four countries and 209 adults in total to analyse PH in Africa.…”

Pulmonary hypertension in South Africa – at last some original research!

“…Very little information is available in South Africa (SA) on pulmonary vascular disease. The article on pulmonary hypertension (PH) by Davies-van Es et al [1] in this issue of the AJTCCM is one of the first attempts to rectify this, and is highly welcome. The Groote Schuur Hospital Pulmonary Hypertension Clinic is one of very few specialised units in SA in the management of pulmonary vascular disease.…”