Pulmonary Hypertension in Apparently Healthy Children in Southern Nigeria.

OBJECTIVE: To determine the severity and prevalence of pulmonary hypertension and its relationship with age and body mass index (BMI) in healthy children. STUDY DESIGN: Observational study. PLACE AND DURATION OF STUDY: University of Nigeria Teaching Hospital Ituku-Ozalla Enugu, Lagos State University Teaching Hospital, Niger Delta University Teaching Hospital, Bayelsa and Blessed Children Hospital Enugu from January 2010 to December 2019. METHODOLOGY: Four hundred and seventy (470) apparently healthy children aged 1 to 17 years underwent Doppler echocardiographic studies. Their tricuspid regurgitation velocity (TRV) was measured with continuous wave Doppler. Pulmonary artery systolic pressure (PASP) was estimated using the Bernoulli equation. Elevated PASP were determined at PASP ≥35/mmHg. Values were compared against age and BMI. RESULTS: The mean age was 7.9 ± 3.3 years. Four hundred and fifty-nine subjects (97.7%) had normal PASP while 11 (2.3%) had elevated PASP. Those with elevated PASP had a significantly higher mean TRV of 2.7 ± 0.22 cm/s (95% CI; 2.55-2.85) vs TRV of 1.56 ± 0.43cm/s (95% CI; 1.52-1.60) and higher mean PASP of 39.27±4.89 mmHg (95% CI; 35.99-42.56) vs 20.45 ± 5.34 mHg (95% CI; 19.96-20.94) (p=0.001). Though majority of the children had appropriate weight for ages, those with elevated PASP had a significantly greater weight than those with normal PASP (p<0.001). There was a weak positive correlation of PASP with age (r=0.16) and BMI in normal weight (r=0.08). Obese children had a negative correlation value(r=-0.13). A weak negative correlation of PASP with BMI was seen in underweight (r=-0.17 and overweight (r=-0.73) children (p>0.05). CONCLUSION: The mean pulmonary artery systolic pressure in the studied apparently healthy Nigerian children was 20.45± 5.34/ mmHg. The frequency of elevated PASP was 2.3%, commoner in children with higher BMI. Age and body mass index are not optimal predictors of PASP. Key Words: Pulmonary artery systolic pressure, Children, Pulmonary hypertension, Echocardiography.

The causes, treatment, and outcome of pulmonary hypertension in Africa: Insights from the Pan African Pulmonary Hypertension Cohort (PAPUCO) Registry.

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. TRIAL REGISTRATION: ClinicalTrials.gov (NCT02265887).

Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces.

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Rationale and design of the Pan African Pulmonary hypertension Cohort (PAPUCO) study: implementing a contemporary registry on pulmonary hypertension in Africa.

INTRODUCTION: Pulmonary hypertension (PH) is a devastating, progressive disease with increasingly debilitating symptoms and usually shortened overall life expectancy due to a narrowing of the pulmonary vasculature and consecutive right heart failure. Little is known about PH in Africa, but limited reports suggest that PH is more prevalent in Africa compared with developed countries due to the high prevalence of risk factors in the region. METHODS AND ANALYSIS: A multinational multicentre registry-type cohort study was established and tailored to resource-constraint settings to describe disease presentation, disease severity and aetiologies of PH, comorbidities, diagnostic and therapeutic management, and the natural course of PH in Africa. PH will be diagnosed by specialist cardiologists using echocardiography (right ventricular systolic pressure >35 mm Hg, absence of pulmonary stenosis and acute right heart failure), usually accompanied by shortness of breath, fatigue, peripheral oedema and other cardiovascular symptoms, ECG and chest X-ray changes in keeping with PH as per guidelines (European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines). Additional investigations such as a CT scan, a ventilation/perfusion scan or right heart catheterisation will be performed at the discretion of the treating physician. Functional tests include a 6 min walk test and the Karnofsky Performance Score. The WHO classification system for PH will be applied to describe the different aetiologies of PH. Several substudies have been implemented within the registry to investigate specific types of PH and their outcome at up to 24 months. Data will be analysed by an independent institution following a data analyse plan. ETHICS AND DISSEMINATION: All local ethics committees of the participating centres approved the protocol. The data will be disseminated through peer-reviewed journals at national and international conferences and public events at local care providers.