Isolated agenesis of the left pulmonary artery: A case report.

We present a case of a middle-aged patient followed up at the medical outpatient's department routinely over two years for hypertensive heart disease and tobacco-induced chronic obstructive pulmonary disease. The patient was found to have an additional problem of congenital absence of the left main pulmonary artery.

Bullous lung disease in association with an isolated giant plexiform neuroma: A case report and brief review of pulmonary involvement in neurofibromatosis type 1.

Neurofibromatosis is common. It can be accompanied by abnormalities related to the thorax. These vary in severity and can be life-threatening. We present a case of plexiform neuroma with associated extensive bullous lung disease.

2020 Heart Failure Society of South Africa perspective on the 2016 European Society of Cardiology Chronic Heart Failure Guidelines.

Heart failure with a reduced ejection fraction (HFrEF) is a condition frequently encountered by healthcare professionals and, in order to achieve the best outcomes for patients, needs to be managed optimally. This guideline document is based on the European Society of Cardiology Guidelines for the treatment of acute and chronic heart failure published in 2016, and summarises what is considered the best current management of patients with the condition. It provides information on the definition, diagnosis and epidemiology of HFrEF in the African context. The best evidence-based treatments for HFrEF are discussed, including established therapies (beta-blockers, ACE-i/ARBs, mineralocorticoid receptor antagonists (MRAs), diuretics) that form the cornerstone of heart failure management as well as therapies that have only recently entered clinical use (angiotensin receptor-neprilysin inhibitor (ARNI), sodium/glucose cotransporter-2 (SGLT2) inhibitors). Guidance is offered in terms of more invasive therapies (revascularisation, implantable cardioverter defibrillators (ICDs) and cardiac resynchronisation therapy (CRT) by implantation of a biventricular pacemaker with (CRT-D) or without (CRT-P) an ICD, left ventricular assist device (LVAD) use and heart transplantation) in order to ensure efficient use of these expensive treatment modalities in a resource-limited environment. Furthermore, additional therapies (digoxin, hydralazine and nitrates, ivabradine, iron supplementation) are discussed and advice is provided on general preventive strategies (vaccinations). Sections to discuss conditions that are particularly prevalent in sub-Saharan Africa (HIV-associated cardiomyopathy (CMO), peripartum CMO, rheumatic heart disease, atrial fibrillation) have been added to further improve clinical care for these commonly encountered disease processes. You are encouraged to read the complete 2016 ESC Heart Failure guideline: Ponikowski P, Voors AA, Anker SD, et al.; on behalf of the European Society of Cardiology. 2016 ESC guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2016,37:2129-2200.

Registration of amiloride in South Africa: Cutting the Gordian knot.

Amiloride is an antagonist of the renal tubular epithelial sodium channel (ENaC). As such, it is a diuretic that is both potassium and magnesium sparing. It is used for the treatment of potassium depletion and hypertension, and is the specific therapy for hypertension due to overactivity of the ENaC (Liddle syndrome and several additional genetic causes of the Liddle phenotype - low renin and low aldosterone). It is listed as a World Health Organization essential drug, but has never been registered in South Africa (SA) and can therefore only be prescribed under a Section 21 application to the SA Health Products Regulatory Authority (SAHPRA) on a case-by-case basis. In SA, >50% of patients treated for hypertension are not controlled. In the USA, the Reasons for Geographic and Racial Differences in Stroke (REGARDS) study reported that African Americans are more likely to be diagnosed with hypertension, more likely to be treated, more likely to be treated intensively, and less likely to achieve blood pressure (BP) control. Although the reasons are complex, studies show that 10 - 20% of blacks may carry the Liddle phenotype. Observational data and a controlled clinical trial done in three African countries have shown that these patients respond to amiloride and not to conventional guideline-based antihypertensive treatment. The former is likely to result in a significant reduction in cardiovascular, stroke and kidney morbidity and mortality, because of improved BP control. Amiloride is very unlikely to ever be registered in SA, as it was first developed >50 years ago, and SAHPRA regulations prevent widespread prescription of this essential drug. This is a classic Gordian knot that requires a novel approach from authorities to sever the knot and improve the health of many South Africans.

Heart Failure Society of South Africa (HeFSSA) perspective on the European Society of Cardiology (ESC) 2012 chronic heart failure guideline.

BACKGROUND: The South African Heart Association (SA Heart) is an affiliate of the European Society of Cardiology (ESC). SA Heart endorses ESC treatment guidelines with modification to suit local circumstances. The Heart Failure Society of South Africa (HeFSSA) is a special interest group of SA Heart. This guideline has been compiled on behalf of the HeFSSA and is based on the ESC guidelines for the diagnosis and treatment of acute and chronic heart failure 2012. The focus is on heart failure with reduced ejection fraction (HF-REF) (i.e. ejection fraction <50%). We have recommended interventions in symptomatic patients with HF-REF in general to clarify the 'grey area' between the ESC guidelines definition of REF (<50%) and the predefined ejection fraction used in randomised heart failure trials (<35%). OBJECTIVE: To highlight new changes in the diagnosis and treatment of chronic heart failure with particular emphasis on areas that are relevant to SA. CONCLUSIONS: Randomised clinical trials are a crucial, but not the only, guide in treating HF-REF patients. There always remain questions that are unanswered and groups of patients not studied, so prudent clinical decisions are required.

Falciparum malaria in a South African tertiary care hospital.

INTRODUCTION: This study was a retrospective case series over one year. OBJECTIVES: The purpose was to review the clinical presentation, travel history, laboratory findings and outcome of Plasmodium falciparum malaria. PATIENTS AND METHODS: The study was conducted in the medical wards of Dr. George Mukhari Hospital, a teaching hospital in South Africa that serves mainly black patients. Fifty-nine patients were evaluated. The mean age was 34 years. Twenty-three patients (39%) had strictly defined severe malaria. Ninety-eight percent acquired Plasmodium falciparum in Sub-Saharan Africa. The death rate was 1.7%. Virtually all patients had a travel history obtained in the emergency department and the diagnosis was confirmed in all cases within 24 hours of admission. RESULTS: In our study population, the differences in the percent parasitemia, platelet count, haemoglobin and bilirubin were not statistically significant between the cases with severe and those with less severe malaria. CONCLUSIONS: Plasmodium falciparum malaria should not carry a high mortality in adequately equipped centers, when the diagnosis is made early and therapy is instituted promptly.

In-hospital outcome of patients discharged from the ICU with tracheostomies.

OBJECTIVE: To document the outcome of patients discharged from the intensive care unit (ICU) with tracheostomies. DESIGN AND SETTING: This was a retrospective study conducted in the ICU of Dr George Mukhari Hospital, Pretoria. PATIENTS: All patients discharged from the ICU with tracheostomies over a period of 1 year from 1 January to 31 December 2003. INTERVENTIONS: None. MEASUREMENTS: The main variables studied were post-ICU mortality and length of hospital stay, the Glasgow Coma Scale (GCS) at discharge from ICU and the multiple organ dysfunction score on the day of discharge from the ICU. MAIN RESULTS: Forty-seven patients were discharged with tracheostomies during the study period. The post-ICU mortality was 57%. The mortality of patients discharged with a GCS below 8 was statistically higher than that of patients discharged with a GCS above 8 (79% v. 22%, p = 0.0002). Survivors had significantly longer duration of hospitalisation (26.95 +/- 21.47 days v. 13.48 +/- 14.24 days, p = 0.021) than non-survivors. The mortality rate was higher if the tracheostomy was performed for a low GCS than when it was performed for reasons other than a low GCS (p = 0.0001). The 20 surviving patients were decannulated before discharge from hospital. CONCLUSION: The outcome of patients discharged from the ICU with tracheostomies is, on the whole, unfavourable compared with predicted mortality. A GCS of less than 8 is a good predictor of poor outcome.

An uncommon cause of pulmonary hypertension.

A 32-year-old woman presented with severe pulmonary hypertension and cor pulmonale in congestive cardiac failure. The diagnosis was established by means of positive anti-schistosomal immunoglobulin G (IgG) antibodies and ultrasonographic evidence of hepatosplenic schistosomiasis with portal hypertension. Extensive investigations revealed no other cause for the pulmonary hypertension, providing proof for the diagnosis of pulmonary schistosomiasis. The literature reviewed indicates very few cases of this rare complication. The pathology in general is reviewed in this report as well as some 'pathogenic' mechanisms of this case.