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objective: to evaluate paediatric cardiac services in south Africa with respect to referral base; services provided and human resources. study design: A descriptive study design was used. An audit of the referral base; personnel and activity of paediatric cardiac units throughout south Africa was conducted by means of a questionnaire. A specialist from each centre was asked to provide the relevant data. Where accurate data was not available; estimates were provided by practitioners within each centre. results: All identified units participated in the audit. three were private sector units while the other five were primarily public sector units. twenty four paediatric cardiologists; equally distributed between public and private sector units; were practicing in the country as at end 2008; with a further eight paediatricians undergoing training in paediatric cardiology. this is significantly less than the 88 paediatric cardiologists required for the population of south Africa. Eight paediatric cardiac surgeons were operating predominantly on children in public hospitals and five in private institutions. An estimated 1370 operations for congenital heart disease were performed over a one year period; with 800 of these in the public sector. Extrapolating from accepted estimates of congenital heart disease incidence; this represents conservatively; less that 40of operations required for the population. Additionally; only 26of the estimated 114 simple transposition of great arteries born annually were operated on; indicating serious deficiencies in the ability to adequately detect and intervene in serious congenital heart disease presenting in the neonatal period. conclusion: the infrastructure and resources to detect and manage heart disease in children in south Africa; particularly within the public sector; are grossly inadequate

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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 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; timulation of research and positive private-public partnerships are all necessary to ensure that appropriate care is delivered

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OBJECTIVE: Atrial septal defects and patent foramen ovale can now be closed percutaneously. This report describes our initial and follow-up experience, especially in relation to efficacy and complications. METHODS: During a 2-year period, seven atrial septal defects and one patent foramen ovale were closed. The Amplatzer artrial septal defect occluder was used in five, the Cardioseal device in two and the Amplatzer patent foramen ovale occluder in one. A compliant sizing balloon was used to assess the stretched diameter of the defects. RESULTS: Percutaneous closure was successful in all patients. The median age was 5.4 years (range: 3.7-16.6 years) and median weight was 18 kg (range: 14-65 kg). The mean duration of the procedure was 2.3 hours. There were no acute complications. Patients were followed up for a mean of 1.1 years. One child experienced severe headaches and another developed an atrial flutter and a small leak 7 months after implantation, both of which resolved. CONCLUSIONS: Percutaneous closure of atrial septal defects and patent foramen ovale is effective and safe. In selected children, this may be offered as an alternative to surgery.

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We report on one affected twin who presented with cough, dyspnoea and severe failure to thrive. He was found to have gross cardiomegaly on chest X-ray. This proved to be due to an intrapericardial teratoma with an associated pericardial effusion. The operation on this rare tumour was successful.

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A successfully treated case of a spontaneous aortic thrombosis in a neonate is described as an illustration of the advantages of using low molecular weight heparin (LMWH) over unfractionated heparin (UFH) for anticoagulation therapy. A 5-day-old neonate presented with an abdominal aortic thrombosis detected by echocardiography. Intravenous UFH was commenced following thrombectomy. Poor venous access made monitoring the anticoagulation therapy problematic. Subcutaneous LMWH was substituted for UFH. It requires substantially less monitoring and no intravenous access, has fewer side effects, and allows for much earlier discharge from the hospital. Extensive investigation for a hypercoagulable state revealed no definite cause for the thrombus. The findings of homozygosity for a methylene tetrahydrofolate reductase (MTHFR) mutation and a mildly elevated homocysteine level are interesting but unlikely to account for the thrombotic event in this case. Anticoagulation with LMWH proved effective and more convenient than using UFH.

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OBJECTIVE: To document the development of aortic regurgitation following occlusion of a patent arterial duct. DESIGN: Case series involving nine children referred for surgical ligation of an isolated patent arterial duct. SETTING: Academic referral centre. METHODS: A preoperative transthoracic and transoesophageal echocardiogram was performed in theatre to look for aortic regurgitation. Thereafter, aortic flow was monitored throughout the operation by colour flow mapping with the transoesophageal probe in situ. Onset of aortic regurgitation was documented. An immediate postoperative transthoracic echocardiogram was performed on all patients and then daily until discharge on day 5. A follow up clinical and echocardiographic assessment was performed six weeks postoperatively. RESULTS: With ligation of the patient arterial duct, transoesophageal echocardiography showed immediate regurgitation in seven of the nine patients, seen as a small central jet on colour flow mapping. Six of the seven patients continued to have aortic regurgitation on transthoracic echocardiography before leaving theatre. In none was aortic regurgitation audible clinically. At discharge, five patients still had evidence of aortic regurgitation; of four seen at follow up six weeks later, only one had residual regurgitation. CONCLUSIONS: Ligation of the patient arterial duct results in the acute termination of the "run off" in a volume overloaded situation. This, together with a rise in the peripheral vascular resistance and the persistence of increased proximal vascular capacitance, is considered to be the underlying aetiology of the acquired aortic regurgitation.

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Two cases of cantharidin poisoning are described in which the patients developed the well-known complications of acute renal failure and pseudopolycythaemia. In addition both developed a Guillain-Barré type of flaccid paralysis but spontaneous reversal occurred. Cantharidin poisoning may be responsible for some of the unexplained neurological presentations in Black South Africans.

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