Rationale and design of the African Cardiomyopathy and Myocarditis Registry Program: The IMHOTEP study.

BACKGROUND: Heart failure (HF), the dominant form of cardiovascular disease in Africans, is mainly due to hypertension, rheumatic heart disease and cardiomyopathy. Cardiomyopathies pose a great challenge because of poor prognosis and high prevalence in low- and middle-income countries (LMICs). Little is known about the etiology and outcome of cardiomyopathy in Africa. Specifically, the role of myocarditis and the genetic causes of cardiomyopathy are largely unidentified in Africans. METHOD: The African Cardiomyopathy and Myocarditis Registry Program (the IMHOTEP study) is a pan-African multi-centre, hospital-based cohort study, designed with the primary aim of describing the clinical characteristics, genetic causes, prevalence, management and outcome of cardiomyopathy and myocarditis in children and adults. The secondary aim is to identify barriers to the implementation of evidence-based care and provide a platform for trials and other intervention studies to reduce morbidity and mortality in cardiomyopathy. The registry consists of a prospective cohort of newly diagnosed (i.e., incident) cases and a retrospective (i.e., prevalent) cohort of existing cases from participating centres. Patients with cardiomyopathy and myocarditis will be subjected to a standardized 3-stage diagnostic process. To date, 750 patients have been recruited into the multi-centre pilot phase of the study. CONCLUSION: The IMHOTEP study will provide comprehensive and novel data on clinical features, genetic causes, prevalence and outcome of African children and adults with all forms of cardiomyopathy and myocarditis in Africa. Based on these findings, appropriate strategies for management and prevention of the cardiomyopathies in LMICs are likely to emerge.

Ductal closure in infants under 6 kg including premature infants using AmplatzerTM duct occluder type two additional sizes: a single-centre experience in South Africa.

BACKGROUND: This is a report on percutaneous closure of patent ductus arteriosus (PDA) using Amplatzer Duct Occluder type two additional sizes (ADO II AS) in patients under 6 kg. METHODS: Prospective data were collected and a review of Patients' records was conducted. Demographics, and angiographic and clinical outcomes are reported in this article. RESULTS: During the period June 2011 to June 2017, of the 92 patients who underwent closure of the PDA using the ADO II AS device, 59 were under 6 kg. The median weight of the cohort at closure was 3.6 kg (range: 900 g - 5.8 kg). The median ductal diameter was 1.9 mm (range: 1.0-3.4 mm). Three embolisations in the cohort were all retrieved percutaneously. Two PDAs were closed percutaneously and one surgically. Four premature infants required blood transfusions. The closure rate was 96.6% before discharge. CONCLUSIONS: PDA closure using ADO II AS in small infants is feasible, effective and has few complications.

Delays in fast track antiretroviral therapy initiation and reasons for not starting treatment among eligible children in Eastern Cape, South Africa.

: We report data from an observational cohort of South African children living with HIV less than 12 years of age eligible for fast track antiretroviral therapy (rapid) initiation. We found that less than half of children eligible for rapid antiretroviral therapy initiation based on immunologic and disease status started treatment within 1 week.

HIV viral suppression and longevity among a cohort of children initiating antiretroviral therapy in Eastern Cape, South Africa.

INTRODUCTION: There are limited data on viral suppression (VS) in children with HIV receiving antiretroviral therapy (ART) in routine care in low-resource settings. We examined VS in a cohort of children initiating ART in routine HIV care in Eastern Cape Province, South Africa. METHODS: The Pediatric Enhanced Surveillance Study enrolled HIV-infected ART eligibility children zero to twelve years at five health facilities from 2012 to 2014. All children received routine HIV care and treatment services and attended quarterly study visits for up to 24 months. Time to VS among those starting treatment was measured from ART start date to first viral load (VL) result <1000 and VL <50 copies/mL using competing risk estimators (death as competing risk). Multivariable sub-distributional hazards models examined characteristics associated with VS and VL rebound following suppression among those with a VL >30 days after the VS date. RESULTS: Of 397 children enrolled, 349 (87.9%) started ART: 118 (33.8%) children age <12 months, 122 (35.0%) one to five years and 109 (31.2%) six to twelve years. At study enrolment, median weight-for-age z-score (WAZ) was -1.7 (interquartile range (IQR):-3.1 to -0.4) and median log VL was 5.6 (IQR: 5.0 to 6.2). Cumulative incidence of VS <1000 copies/mL at six, twelve and twenty-four months was 57.6% (95% CI 52.1 to 62.7), 78.7% (95% CI 73.7 to 82.9) and 84.0% (95% CI 78.9 to 87.9); for VS <50 copies/mL: 40.3% (95% CI 35.0 to 45.5), 63.9% (95% CI 58.2 to 69.0) and 72.9% (95% CI 66.9 to 78.0). At 12 months only 46.6% (95% CI 36.6 to 56.0) of children <12 months had achieved VS <50 copies/mL compared to 76.9% (95% CI 67.9 to 83.7) of children six to twelve years (p < 0.001). In multivariable models, children with VL >1 million copies/mL at ART initiation were half as likely to achieve VS <50 copies/mL (adjusted sub-distributional hazards 0.50; 95% CI 0.36 to 0.71). Among children achieving VS <50 copies/mL, 37 (19.7%) had VL 50 to 1000 copies/mL and 31 (16.5%) had a VL >1000 copies/mL. Children <12 months had twofold increased risk of VL rebound to VL >1000 copies/mL (adjusted relative risk 2.03, 95% CI: 1.10 to 3.74) compared with six to twelve year olds. CONCLUSIONS: We found suboptimal VS among South African children initiating treatment and high proportions experiencing VL rebound, particularly among younger children. Greater efforts are needed to ensure that all children achieve optimal outcomes.

High risk of loss to follow-up among South African children on ART during transfer, a retrospective cohort analysis with community tracing.

INTRODUCTION: Decentralization of HIV care for children has been recommended to improve paediatric outcomes by making antiretroviral treatment (ART) more accessible. We documented outcomes of children transferred after initiating ART at a large tertiary hospital in the Eastern Cape of South Africa. METHODS: Electronic medical records for all children 0-15 years initiating ART at Dora Nginza Hospital (DNH) in Port Elizabeth, South Africa January 2004 to September 2015 were examined. Records for children transferred to primary and community clinics were searched at 16 health facilities to identify children with successful (at least one recorded visit) and unsuccessful transfer (no visits). We identified all children lost to follow-up (LTF) after ART initiation: those LTF at DNH (no visit >6 months), children with unsuccessful transfer, and children LTF after successful transfer (no visit >6 months). Community tracing was conducted to locate caregivers of children LTF and electronic laboratory data were searched to measure reengagement in care, including silent transfers. RESULTS: 1,582 children initiated ART at median age of 4 years [interquartile range (IQR): 1-8] and median CD4+ of 278 cells/mm3 [IQR: 119-526]. A total of 901 (57.0%) children were transferred, 644 (71.5%) to study facilities; 433 (67.2%) children had successful transfer and 211 (32.8%) had unsuccessful transfer. In total, 399 children were LTF: 105 (26.3%) from DNH, 211 (52.9%) through unsuccessful transfer and 83 (20.8%) following successful transfer. Community tracing was conducted for 120 (30.1%) of 399 children LTF and 66 (55.0%) caregivers were located and interviewed. Four children had died. Among 62 children still alive, 8 (12.9%) were reported to not be in care or taking ART and 18 (29.0%) were also not taking ART. Overall, 65 (16.3%) of 399 children LTF had a laboratory result within 18 months of their last visit indicating silent transfer and 112 (28.1%) had lab results from 2015 to 2016 indicating current care. CONCLUSION: We found that only two-thirds of children on ART transferred to primary and community health clinics had successful transfer. These findings suggest that transfer is a particularly vulnerable step in the paediatric HIV care cascade.

Patent ductus arteriosus closure using Occlutech® Duct Occluder, experience in Port Elizabeth, South Africa.

BACKGROUND: Percutaneous closure of patent ductus arteriosus (PDA) has become standard therapy. Experience with the Occlutech® Duct Occluder is limited. METHODS: Data regarding ductal closure using Occlutech® Duct Occluder were reviewed and prospectively collected. Demographics, hemodynamic and angiographic characteristics, complications, and outcomes were documented. RESULTS: From March 2013 to June 2016, 65 patients (43 females and 22 males) underwent percutaneous closure of the PDA using Occlutech® Duct Occluder. The median age of the patients was 11 months (range, 1-454 months) and the median weight was 8.5 kg (range 2.5-78 kg). The mean pulmonary artery median pressure was 27 mmHg (range, 12-100 mmHg) and the QP: Qs ratio median was 1.8 (range, 1-7.5), with a pulmonary vascular resistance mean of 2.7 WU (standard deviation [SD] ±2.1). Thirty-two patients had Krichenko Type A duct (49%); 7, Type C (11%); 4, Type D (6%); and 22, Type E (34%). The ductal size (narrowest diameter at the pulmonic end) mean was 3.5 mm (SD ± 1.9 mm). The screening time mean was 17.3 min (SD ± 11.6). Out of 63 patients with successful closure of the PDA using Occlutech® Duct Occluder, there were 15 patients with small PDAs; 25 with moderate PDAs, and 23 with large PDAs. In one patient, the device dislodged to the descending aorta, and in two patients, to the right pulmonary artery immediately following deployment, with successful percutaneous (two) and surgical (one) retrieval. Complete ductal occlusion was achieved in all 63 patients on day one. CONCLUSION: The Occlutech® Duct Occluder is a safe and effective device for closure of ducts in appropriately selected patients.

Clinical presentation and outcomes of patients with acute rheumatic fever and rheumatic heart disease seen at a tertiary hospital setting in Port Elizabeth, South Africa.

BACKGROUND: The incidence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) has waned in Western countries, however that is not the situation in developing nations. METHODS: Records were reviewed of patients from the Eastern Cape municipal districts who presented to the Paediatric Cardiology Unit with ARF and RHD from January 2008 to August 2015. RESULTS: Total of 56 patients with ARF/RHD was reviewed. The majority of patients (n = 52) presented for the first time with RHD. Four patients presented with ARF and two had recurrent ARF. Six patients presented with a combination of RHD and congenital heart disease. Twenty-three patients were operated on for chronic rheumatic valve disease, with good outcomes. CONCLUSION: The true burden of ARF/RHD is unknown in the Eastern Cape. Prospective studies are needed to accurately determine the prevalence of RHD in this province.

Ebstein's anomaly and Down's syndrome.

We report on two cases presenting with a rare combination of Ebstein's anomaly and Down's syndrome. The first patient presented with respiratory distress, mild cyanosis and right heart failure immediately after delivery. The symptoms improved with heart failure medication. The patient remained asymptomatic on follow up. The second patient was diagnosed antenatally with marked apical displacement of the tricuspid valve and a very small functional right ventricle compared to the left ventricle. At birth, the patient presented with an extreme form of Ebstein's anomaly with severe cyanosis, marked right heart failure and ductal-dependent pulmonary blood flow. The patient died within days of birth.

Ductal closure using the Amplatzer duct occluder type two: experience in Port Elizabeth hospital complex, South Africa: cardiovascular topic.

OBJECTIVE: To report outcomes in percutaneous ductal closure using the Amplatzer duct occluder type two (ADO II). METHODS: Records of patients admitted for percutaneous closure of patent ductus arteriosus (PDA) were reviewed. RESULTS: From May 2009 to July 2012, 36 patients were assigned to closure using the ADO II. There were 21 females and 15 males. The median age was 16.5 (2-233) months; median weight, 8 (3.94-39.2) kg; and median height, 75 (55-166) cm. The mean pulmonary artery pressure was 24.4 (± 10.4) mmHg, the pulmonary blood flow:systemic blood flow (Qp:Qs) ratio was 2.25 (± 1.97), and mean pulmonary resistance (Rp) was 1.87 (± 1.28) Wood units. The mean ductal size was 2.74 (± 1.3) mm. In 30 patients the device was delivered through the pulmonary artery. Thirty-three patients achieved complete closure by discharge (day one). CONCLUSION: The ADO II is capable of closing a wide range of ducts in carefully selected patients. Our findings are comparable with other studies regarding ductal closure rates.

Impact of Highly Active Antiretroviral Therapy on paediatric Human Immunodeficiency Virus-associated left ventricular dysfunction within the Johannesburg teaching hospital complex.

OBJECTIVE: To analyse the outcome of children with left ventricular dysfunction placed on Highly Active Antiretroviral Therapy. METHOD: This study is a retrospective review of records of Human Immunodeficiency Virus-positive children with left ventricular dysfunction. Demographic data were collected. Left ventricular fractional shortening, CD4 percentage, viral load, and nutritional status were compared before and during antiretroviral therapy. RESULTS: We reviewed the records of 34 Human Immunodeficiency Virus-positive children with left ventricular dysfunction. In all, 18 patients received antiretroviral therapy (group one) and 16 were antiretroviral therapy naive (group two). The median age of group one at initial visit was 94 months, with a male-to-female ratio of 1:1. Of those, 17 children showed improved left ventricular function on treatment, with an increase in fractional shortening (median: 17-33.5%; p less than 0.0001). There was no significant statistical difference between the groups regarding initial fractional shortening. In group one, the CD4 percentage improved (median: 12% to 30.5%; p less than 0.0001), with viral load suppression (median: 24,900 copies per millilitre to less than 25 copies per millilitre; p less than 0.0001). There was weight gain in group one (median z-score: -1.70 to -1.32; p equal to 0.0083). Proper statistical analysis in group two was not possible because of poor follow-up of patients. CONCLUSION: The findings are in keeping with other reports that have shown improvement in left ventricular function in patients with Human Immunodeficiency Virus-associated cardiomyopathy treated with Highly Active Antiretroviral Therapy. Recovery of myocardial function is associated with improvement in immunological and nutritional statuses.