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

Congenital pulmonary vein stenosis at an African tertiary care centre over a 25-year period

Background: Congenital pulmonary vein stenosis (PVS) is a rare condition, which results from abnormal embryological incorporation of the common pulmonary vein into the left atrium.Methods: A retrospective descriptive case series study was conducted with the aim of describing the characteristics and outcome of children with congenital PVS at an African tertiary care centre over a 25-year period. A computerised paediatric cardiology database initiated in the early 1990s was sourced to identify patients, following which clinical records were retrieved and reviewed.Results: Five cases of congenital PVS were identified between January 1990 and January 2016 and accounted for 0.0007% of all congenital heart defects seen at the centre during the study period. The age at diagnosis ranged from 22 months - 13 years. Most patients presented with respiratory symptoms, with 2 patients presenting with recurrent haemoptysis. The diagnosis of PVS was confirmed by cardiac catheterisation and pulmonary angiography in all patients. All cases were right-sided unilateral PVS and all were associated with one or more congenital heart defects. Mild pulmonary hypertension and elevated capillary wedge pressures were found in all patients. Only one patient underwent specific surgery to relieve the PVS, which subsequently recurred. Another patient underwent a lobectomy following recurrent haemoptysis, but subsequently died of sepsis.Conclusion: Congenital PVS is a rare condition often associated with other congenital cardiac defects. Respiratory symptoms are common at presentation, with haemoptysis forming part of the presenting clinical spectrum, which is in keeping with the published literature. Elevated pulmonary artery pressures and increased ipsilateral pulmonary capillary wedge pressures are a common finding at catheterisation

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

An audit of surgical repair of Tetralogy of Fallot in an African tertiary care centre

Background: For patients undergoing Tetralogy of Fallot (TOF) repair at institutions in sub-Saharan Africa, data on type of surgical repair, operative mortality and important determinants of outcome such as age at operation and development of pulmonary regurgitation (PR) post-repair is scanty. Objective: To describe the outcomes of children diagnosed with TOF who underwent surgical repair at our center with emphasis on post-operative PR.Methods: This was a retrospective cohort study undertaken from 1 January 1994 to 31 December 2003 at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH). The descriptive analysis of the clinical audit was done in 2010. Results: Fifty four (75%) patients were operated upon: 50 (92.5%) had complete repair and 4 (7.4%) had a palliative procedure in the form of a Blalock-Taussig (B-T) shunt. The median age for corrective surgery was 39.5 months. Twenty out of 50 (40%) patients had simple repair, 10 (20%)had repair which included pulmonary valvotomy, 15 (30%) had a transannular patch (TAP) repair with or without monocusp and 5 (10%) had conduit insertion. An evaluation of severe PR among all the repair groups in the immediate post-operative period (<1 year) revealed that 5 out of 15 (33.3%) patients who had TAP repair developed severe PR, whilst no severe PR was noted in the simple, pulmonary valvotomy and conduit repair groups. During the intermediate post-operative period (1 - 5 years), severe PR was documented in 4 (27%) patients with TAP repair, 2 (10%) with simple repair and 2 (20%) in the pulmonary valvotomy group only. In the long term period (>5 years), severe PR was documented in 3 (30%) patients with pulmonary valvotomy, 8 (53%) who had TAP repair, 2 (10%) patients with simple repair including 1 (20%) patient with a xenograft conduit. One out of 50 (2%) surgically corrected patients who had a complex anatomy died in the immediate post-operative period. Twenty eight out of 50 (56%) patients who had complete correction were lost to follow-up. Conclusion: Surgical correction occurs much later in infancy and childhood compared to developed countries. Severe PR is a serious complication strongly associated with all types of surgical repair of TOF and these patients require lifelong follow-up. Despite the small sample size, the operative mortality compares favourably to fi rst world centers

Direct and indirect effects of the COVID-19 pandemic on children with cardiovascular disease

Coronavirus 2 (SARS-CoV-2) the causative agent of what we now know as “Coronavirus Disease 2019” (COVID-19), is the most serious global health crisis of our generation, with a significant and far-reaching impact upon health, economy, social cohesion and emotional and mental wellbeing. Although children do not bear the brunt of direct mortality, they are significantly affected in terms of morbidity and interruption to access, continuity and complexity of care, as well as the indirect social and financial effects impacting on their health outcomes. In this review we present some of the most recent data relevant to children with congenital and acquired heart disease, and consider some of the cardiac presentations noted. We discuss the necessary protections to staff in the echo and cardiac catherisation laboratories and present some general recommendations to general paediatricians and communities to ensure the continued health of our patients. Finally, we encourage ongoing registries and biorepositories and support clinical trials to ensure that children also receive new technologies and therapeutics as these become available

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

This paper reviews some research studies on tillage methods influencing soil and moisture conservation in the eastern African countries of Kenya, Tanzania, Malawi and Ethiopia during the past four decades. Most of these studies were conducted in marginal rainfall (semi arid ) areas and on shallow soils of various textures (sandy clay loam, sandy clay, clay and loam). The studies were meant to establish the effects of tillage and residue management practices on physico-chemical soil properties (i.e. structure, bulk density, soil moisture and organic matter contents), runoff and infiltration. This review emphasizes the importance of appropriate tillage and residue management methods (contour bunds and terraces, minimum tillage, tied ridging, mulching and conventional tillage) in providing soil conditions favourable for soil moisture conservation and subsequent crop performance and yield on smallholder farm

Rheumatic fever and rheumatic heart disease in Africa

The eradication of acute rheumatic fever/rheumatic heart disease (ARF/RHD) is a complex process that needs to be addressed at various levels. These include education of vulnerable communities about the disease, provision of easy access to medical care, and increasing the availability of free penicillin to treat group A streptococcal pharyngitis and for secondary prophylaxis against further attacks of ARF in patients with established RHD. Just as important is to address poverty and overcrowding, which are associated with high levels of ARF and RHD, as improvement in socioeconomic conditions has also been shown to promote the control of ARF. Improvement of economic circumstances in disadvantaged communities is therefore also an important component of the management of ARF/RHD. Until such communities are economically empowered, ARF/RHD will continue to be a problem despite interventions to control the disease. All these interventions may prove to be a challenge in some parts of SA and in countries in the rest of Africa.