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Rheumatic heart disease is a sorely neglected condition in the developing world, with almost half of affected patients not receiving penicillin prophylaxis, a study from the Global Rheumatic Heart Disease Registry found.

Among 3,343 patients enrolled in the registry between 2010 and 2012, only 54.8% were given antibiotic prophylaxis, which most often was intramuscular penicillin, according to Bongani M. Mayosi, MBChB, DPhil, of the University of Cape Town in South Africa, and colleagues.

And while 69.5% of patients in whom oral anticoagulants were indicated had prescriptions for these drugs, the international normalized ratio (INR) was in the therapeutic range in only 28.3%, the researchers reported online in the

"Our findings reflect the utter neglect of rheumatic heart disease in affected countries," Mayosi said in a press release.

"Most of the interventions that are not applied, such as a lack of penicillin use ... are available and cheap in the countries concerned, and their application will provide the greatest benefit," he added.

Approximately 4.1 million people die each year from rheumatic heart disease, and most of the deaths could be prevented.

suggested that antibiotic prophylaxis and oral anticoagulation were being underused in low- and middle-income countries, but whether that persists has not been documented.

"The WHO recommends the lifelong use of antibiotic prophylaxis to prevent rheumatic fever in patients with moderate-to-severe rheumatic heart disease, such as those enrolled in this study," the researchers noted.

To provide current data on this, the researchers established the registry, which includes patients from 12 countries in Africa as well as Yemen and India. The countries were classified as low income, such as Ethiopia, lower-middle income, such as Egypt, and upper-middle income, such as South Africa.

All patients had symptomatic rheumatic heart disease, and 21.8% had atrial fibrillation. Their median age was 28, two-thirds were women, and three-quarters were unemployed.

In low-income countries, 86.5% of women were of childbearing age.

Events such as stroke, peripheral embolism, and cardiovascular complications in general occurred more often among patients in the upper-middle income countries, but children in those countries less often had decreased left ventricular ejection fraction.

Procedures such as valve replacement were undertaken with greater frequency in countries with increased income levels.

A total of 72.9% of cases of mitral stenosis were moderate to severe, as were 60.4% of cases of mitral regurgitation, 59.4% of pulmonary stenoses, 54.2% of tricuspid stenoses, and 61.9% of aortic stenoses.

Among patients with native valve disease, the left ventricle was dilated in 16.4% of children and 23% of adults.

Adherence to antibiotics was higher among children than adults, both for intramuscular (81.8% versus 76.9%) and oral agents (83.1% versus 75%). But adherence was low, at 31.1%, during postsurgical periods.

Indications for the use of oral anticoagulation were present in 40.7% of patients. Among these, 91.6% of patients with mechanical heart valves were on anticoagulants, but only 20.3% of those with mitral stenosis in sinus rhythm who were at risk for cardiac embolism were receiving anticoagulant drugs.

The INR was in the subtherapeutic range in 32.7% and higher than the therapeutic range in 17.7%, while INR monitoring was not done in 21.4%.

Other medication use was common, with two-thirds taking diuretics, one-third being on digoxin, and almost 40% using beta-blockers.

For women of childbearing age, only 3.6% used contraceptives.

"The extremely low rate of the use of contraception in this cohort is alarming, and reflects the poor provision of family planning and pre-pregnancy advice for women with heart disease in many regions of the world," Mayosi and colleagues observed.

Of additional concern was that one in five pregnant women were taking warfarin, which is teratogenic. "This calls for safer alternatives in these women," the authors wrote.

Limitations of the study included its cross-sectional design and the researchers' inability to verify adherence through pill counts.

"There are gaps in the implementation of medical and surgical interventions of proven effectiveness for rheumatic heart disease in low- and middle-income countries," they concluded.

From the American Heart Association:

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