A.M. Cilliers

The Drakensberg Declaration on the Control of Rheumatic Fever and Rheumatic Heart Disease in Africa

Bongani Mayosi, Kate Robertson, Jimmy Volmink, Wole Adebo, Kingsley Akinyore, Albert Amoah, Charles Bannerman, Shan Biesman-Simons, Jonathan Carapetis, Antoinette Cilliers, Patrick Commerford, Anne Croasdale, Albertino Damasceno, Jenny Dean, Michael Dean, Robert de Souza, Antonio Filipe, Chris Hugo-Hamman, Sally-Ann JurgensClur, Pierre Kombila-Koumba, Christelle Kotzenberg, John Lawrenson, Pravin Manga, Jonathan Matenga, Tshimbi Mathivha, Phindile Mntla, Ana Mocumbi, Tiny Mokone, Elijah Ogola, Samuel Omokhodion, Chapman Palweni, Adrian Pearce, Avril Salo, Baby Thomas, Kathie Walker, Charles Wiysonge, Salah Zaher

Paediatric cardiac services in South Africa

South Africa has failed to treat its children well, as demonstrated by our failure to achieve adequate progress towards the Millennium Development Goal of reducing the under-5 mortality rate, doing far worse than many poorer countries.

Early presentation of endomyocardial fibrosis in a 22-month-old child: a case report.

We present an unusual, biopsy-proven case of endomyocardial fibrosis in a 22-month-old male child, which progressed rapidly resulting in death. The patient was born to a father originating from Mozambique, where the disease is endemic but who had not himself travelled there, suggesting a genetic link. Other remarkable features were the presence of a right ventricular diverticulum, and a positive Mycoplasma pneumoniae immunoglobulin M enzyme-linked immunosorbent assay test.

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.

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Background: Absent pulmonary valve syndrome (APVS) is most commonly associated with Tetralogy of Fallot (TOF). TOF with APVS is characterised by features of TOF with either rudimentary ridges or complete absence of pulmonary valve tissue. This is often associated with varying degrees of pulmonary stenosis and severe pulmonary regurgitation with massive dilatation of the proximal branch pulmonary arteries, causing compression of the tracheobronchial tree. Hence, respiratory symptoms are a common presenting feature. Methods: Cases of TOF with APVS were extracted from the paediatric cardiology database at Chris Hani Baragwanath Academic Hospital (CHBAH) for January 1981 - April 2016. Results: A total of 15 patients with TOF and APVS were seen at CHBAH over the 34-year study period. TOF with APVS comprised 3% of all TOF patients. Ten (67%) patients presented before 1 year of age. The majority of patients (67%) had respiratory symptoms as their cause of presentation, of which 6 (40%) were infants. Fourteen (93%) patients were described to have the characteristic to-and-fro murmur at presentation. Five patients (33%) were suspected of having 22q11.2 deletion syndrome, but only 4 were tested and 2 were found to be positive. A third of patients underwent surgical intervention and 4 of the 15 patients (27%) were known to be alive at 10 years of age. Conclusions: TOF with APVS compromised 3% of all TOF patients, correlating with the literature. It is associated with the 22q11.2 deletion syndrome and was suspected in 33% of patients. TOF with APVS should be strongly considered in a child less than 1 year presenting with respiratory symptoms and a characteristic to-andfro murmur.

Guidelines for Paediatric Echocardiography in South Africa

This document provides guidelines for standards of practice in paediatric transthoracic echocardiography in South Africa. Paediatric echocardiography is not a substitute for a proper clinical opinion and such studies are best performed as part of an assessment by a paediatric cardiologist.

Optimal paediatric cardiac services in South Africa – what do we need?

Most children with congenital heart disease have a good outcome if treated appropriately, however the majority of children with heart disease in South Africa do not receive appropriate care. This is related to serious deficiencies in the mechanisms and training for early detection as well as a major shortage of skilled personnel to care for these children at all levels. Most public sector hospitals are unable to cope with the number of patients requiring surgery, mainly due to inadequate theatre time allocation and intensive care facilities. Key interventions to address these deficiencies include: 1. Strategies to improve both the training and the retention of all professionals involved in the care of congenital heart disease. 2. Programmes to increase awareness of both congenital and acquired heart disease in children among health care personnel. 3. Ensuring appropriate infrastructure and equipment designed for children with congenital heart disease are available. 4. Development of congenital heart surgery as an independent subspecialty with dedicated resources and personnel. 5. Dedicated intensive care facilities for paediatric heart surgery. In addition, development of appropriate patterns of referral, stimulation of research and positive private-public partnerships are all necessary to ensure that appropriate care is delivered.

Coronary artery fi stulas in children: Experience from a Southern African tertiary care centre complex

Coronary artery fi stulas (CAF) are unusual coronary artery connections with low pressure cardiac chambers or vessels. The majority are congenital, but can also be acquired. Complications include heart failure, myocardial infarction and arrhythmias. Symptomatic and large CAF require treatment and options include surgical ligation or percutaneous device embolisation of the fi stula which has emerged as a less invasive and equally effi cacious management modality. Careful interrogation of the CAF is required prior to occlusion in order not to compromise normal coronary artery vasculature that may arise from the fi stula which can lead to myocardial ischaemia and infarction. Several reported cases highlight thrombus formation within large CAF after surgical ligation with propagation of the thrombus into coronary vessels arising proximally, resulting in myocardial compromise. We present a series of 6 children with CAF, 2 were treated by percutaneous embolisation (one developed a myocardial infarction post procedure) and 3 were treated surgically.

Guidelines for the Management of Grown Up Congenital Heart Disease (GUCH)

Commentary by the Paediatric Cardiac Society of South Africa (PCSSA) on the European Society of Cardiology Guidelines for the “Management of Grown Up Congenital Heart Disease. The Task Force on the Management of Grown Up Congenital Heart Disease of the European Society of Cardiology.