Congenital heart defects (CHD) are the most common type of birth defect and the leading cause of infant deaths in the developed world. This implies a defect in the structure of the heart or blood vessels leading to or from the heart. The heart should have 4 chambers and 2 major vessels leading into the heart and two major vessels leading out of the heart. This includes mild defects such as Ventricular Septal Defects( VSD) while excluding defects associated with prematurity such as Patent Foramen Ovale(PFO) and patent ductusarteriosus (PDA)s. Critical congenital heart disease refers to a potentially life-threatening cardiac abnormality where either the systemic( blood to the body) or pulmonary( blood to the lungs) circulation is dependent on a PDA. These defects are present at birth but some are only recognised much later- weeks, months or even years later. The longer it takes to make the diagnosis , the more difficult the prognosis and in some cases, the more likely it is for surgery no longer to be an option.

Critical congenital heart disease manifests in the first few hours/days of life. They can either present with cyanosis or blueness. All babies have blue hands and feet at birth but this should improve and never be associated with a blue tongue or blue lips. This is an important danger sign and should never be ignored. If the blood is obstructed going to the body, the baby may first present with a fast heart beat, fast breathing, clod hands and feet and poor pulses to feel. An abnormal murmur may be not always be present.

Later on, babies who have intermittent blueness of the lips and tongue , again fast breathing and heart beats are concerning as well as those who tire or sweat with feeding. Although many children can have short murmurs, murmurs that are loud or associated with an enlarged heart or any symptoms must be referred to a cardiologist.

One suggestion has been to increase the surveillance immediately after birth using pulse-oximetry screening. Pulse oximetry has been used within clinical practice for some time and is a widely accepted method of assessing oxygen saturation. Critical congenital heart disease refers to a potentially life-threatening cardiac abnormality where either the systemic or pulmonary circulation is dependent on a patent ductusarteriosus. In both of these situations, abnormally low saturations on the foot and hand or a differential of >3% between the two readings should alert the clinician to possible cardiac anomalies. Pulse oximetry is a safe, feasible, easy test which is also cost-effective, has been shown to be acceptable to parents and medical staff alike and is unprecedented as a screening test to detect CCHD. However implementation necessitates integration into existing newborn screening programmes with commitment to training, sustainability and strengthening of collaborating healthcare infrastructure. The optimal programme for early detection, follow-up and data management in existing programs must be developed while CCHD screening must be integrated with current birthing services, ideally with centralized data management and quality control.

Acute rheumatic fever (ARF) is a serious illness which occurs mainly in children between the ages of 5 and 15 and results in Rheumatic Heart Disease (RHD) in up to 3% of untreated cases. In SA, RHD is the leading cause of acquired heart disease in children and young adults. Together, RF & RHD affect about 15.6 million people worldwide, 2.4 million of whom are children between five and fourteen years old in developing countries. ARF is an illness which thrives in poverty-stricken and developing countries especially in overcrowded, low-income areas, where housing conditions, nutrition, sanitation and health services are inadequate.

ARF is caused by an untreated sore ‘strep’ throat, which may lead to repeated attacks affecting the joints (arthritis), skin (rash) and heart (carditis). After attacks of untreated ARF, chronic heart valve damage (RHD) may develop. In the instance of RHD, open-heart surgery is necessary to repair or replace heart valves.

In developing countries, The World Health Organisation has recognised ARF as being a major problem, where approximately 100-200 per 100,000 school-aged children are affected. 2 Sadly, ARF and RHD are the most common cause of acquired heart disease affecting children and young adults worldwide.

It is not possible to accurately estimate the prevalence of Rheumatic Fever (RF) and Rheumatic Heart Diseases (RHD) in South Africa. A study showed that there is significant under-reporting of RF cases by health care professionals at hospital, municipal and provincial level. Lack of incidence reporting itself, is a direct result of a widespread lack of awareness among healthcare workers of the national guiding principles on RF/RHD which were released by the National Department of Health in 1997.

However, like any other developing country the general consensus among South African cardiologists is that the incidence of RF remains quite high in South Africa. This is supported by research showing that Rheumatic Heart Disease (RHD) is still the leading cause of acquired heart disease in children and young adults in South Africa.

1. To raise awareness among thousands of South African health workers, of the importance of the notification of Rheumatic Fever requirement and correct diagnosis and subsequent treatment of Rheumatic Fever
2. To raise awareness of the disease among millions of South Africans – the majority of whom live in poor economic conditions in rural areas and informal settlements in areas of the country.

Rheumatic fever is not an infection in and of itself. Rather, it is the consequence of an earlier infection of the throat by a specific organism, streptococcus which has been left untreated. RF generallymanifests around 3-6 weeks following the strep infection. Current estimates show that approximately 18% of all throat infections are caused by strep. This infection is infectious of course, and is especially prevalent in areas of poverty given the overcrowding, etc. Given the lack of access to care, many patients are thus not given penicillin and it is in a percentage of these persons that RF occurs.

Without intervention, patients who progress to RHD will require surgical intervention including heart valve repair, or replacement. The rate of success of heart valve replacement is currently 50%. In other words, 50% of people who undergo heart valves replacement surgery do not succeed. Furthermore, young patients will often need repeated valve surgery as they grow older and the body changes.

Given that prophylactic penicillin is the key element to prevent progression of the disease, teaching people about the importance of adhering to secondary prophylaxis will go a long way in ensuring:

• Children will not be denied proper schooling due to debilitating symptoms the disease is currently affecting young people (including their death) in the prime productive years.
• Reduction in the loss of income amongst caregivers having to attend clinics/hospitals
• State savings in terms of expensive heart surgery