Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries.

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.

Audit of Paediatric Cardiac Services in South Africa

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

Optimal Paediatric Cardiac Services in South Africa - What do we Need? Statement of the Paediatric Cardiac Society of South Africa

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

Anti-inflammatory treatment for carditis in acute rheumatic fever.

BACKGROUND: Rheumatic heart disease remains the most important cause of acquired heart disease in developing countries. Although the prevention of rheumatic fever and the management of recurrences is well established the optimal management of active rheumatic carditis is still unclear. OBJECTIVES: To assess the effects of anti-inflammatory agents such as aspirin, corticosteroids and immunoglobulin for preventing or reducing further heart valve damage in patients with acute rheumatic fever. SEARCH STRATEGY: We searched the Cochrane Controlled Trials Register (Issue 4, 2000), MEDLINE (1966 to April 2002), EMBASE (1998 to November 2002), LILACS (1998 to November 2002), Index Medicus (1950 to December 2000) and references lists of identified studies. SELECTION CRITERIA: Randomised controlled trials comparing anti-inflammatory agents (e.g. aspirin, steroids, immunoglobulins) with placebo or controls, or comparing any of the anti-inflammatory agents with one another, in patients with acute rheumatic fever diagnosed according to the Jones, or modified Jones criteria. The presence of cardiac disease one year after treatment was the major outcome criteria selected. DATA COLLECTION AND ANALYSIS: Two reviewers independently extracted data and assessed trial quality. MAIN RESULTS: Eight randomised controlled trials involving 996 people were included. Several steroidal agents viz. ACTH, cortisone, hydrocortisone, dexamethasone and prednisone, and intravenous immunoglobulin were compared to aspirin, placebo or no treatment in the various studies. Six of the trials were conducted between 1950 and 1965, whilst the remaining two were done in the last 10 years. Overall there was no significant difference in the risk of cardiac disease at one year between the corticosteroid-treated and aspirin-treated groups (relative risk 0.87, 95% confidence interval 0.66 to 1.15). Similarly use of prednisone (relative risk 1.78, 95% confidence interval 0.98 to 3.34) or intravenous immunoglobulins (relative risk 0.87, 95%confidence interval 0.55 to 1.39) when compared to placebo did not reduce the risk of developing heart valve lesions at one year. REVIEWER'S CONCLUSIONS: There is no benefit in using corticosteroids or intravenous immunoglobulins to reduce the risk of heart valve lesions in patients with acute rheumatic fever. The antiquity of most of the trials restricted adequate statistical analysis of the data and acceptable assessment of clinical outcomes by current standards. New randomised controlled trials in patients with acute rheumatic fever to assess the effects of corticosteroids such as oral prednisone and intravenous methylprednisone, and other new anti-inflammatory agents are warranted. Advances in echocardiography will allow for more objective and precise assessment of cardiac outcomes.

Rheology of discrete subaortic stenosis.

The discrete form of subaortic stenosis is thought to be an acquired lesion, the aetiology of which may be a combination of factors which include an underlying genetic predisposition, turbulence in the left ventricular outflow tract, and various geometric and anatomical variations of the left ventricular outflow tract. A review of hypotheses relating to its aetiology is provided

Massive biatrial myxomas in a child.

Cardiac myxomas are rarely diagnosed in children, and biatrial tumors are an unusual presentation in any age group. An 8-year-old boy with massive biatrial cardiac myxomas, who presented in acute cardiogenic shock with no preceding cardiac symptomatology, is reported.

Sterile vegetations in children with severe tetralogy of Fallot.

The finding of bland, sterile vegetations in children with severe tetralogy of Fallot is unexpected, and to our knowledge, has not been reported previously. Eight patients diagnosed with tetralogy between January 1993 and July 1997 had sterile vegetations proven by histological and microbiological evaluation, in their right ventricular outflow tracts. Four of these patients were experiencing severe hypercyanotic spells, and four had severely reduced effort tolerance at presentation. They all underwent cardiac catheterization and were submitted for surgical repair. At surgery, the vegetations were thought to be causing further narrowing of the already tight fibrotic infundibular stenosis. Two of these patients had evidence of damaged valves, without evidence of active endocarditis. Although initially sterile, these vegetations, may in some instances, become infected.

Junctional ectopic tachycardia in six paediatric patients.

The presenting features and treatment responses of six children with junctional ectopic tachycardia are evaluated. Two of the patients were siblings and both presented in early childhood with cardiopulmonary failure. The elder sibling died, the surviving sibling was controlled on a combination of amiodarone, digoxin, and sotalol. The remaining four patients presented in later childhood with tachycardia induced cardiomyopathy. Two of the patients were diagnosed incidentally and have normalised their myocardial function on sotalol therapy. The other two presented in congestive cardiac failure. Radiofrequency His bundle ablation and insertion of a permanent pacemaker to control the arrhythmia was undertaken in the elder of the two patients. The remaining patient has had marginal recovery of myocardial function on a combination of amiodarone and sotalol treatment. Improvement in myocardial function may take several months and is dependent on control of the tachycardia in some patients. Sotalol, when used as single or combination treatment, was partially successful in four cases in reducing heart rate. None of the patients reverted to sinus rhythm.