Over 39 million people worldwide suffer from rheumatic heart disease (RHD), a disease in which the heart’s valves are permanently damaged as a result of rheumatic fever, which can occur as a result of untreated or inadequately treated strep throat infection or scarlet fever.

The majority of RHD cases occur in low- and middle-income nations, where RHD is frequently diagnosed after serious valvular heart disease or other cardiovascular problems have occurred, resulting in increased death rates and decreased life expectancy.

RHD is often treated with an intramuscular injection of benzathine penicillin G (BPG) administered every three to four weeks for an extended period of time (e.g., 10 years, up to the age of 40 years or lifelong).

BPG treatment for RHD has been limited in part due to patient and physician concerns about a severe allergic reaction called anaphylaxis, despite the fact that the risk of anaphylaxis following BPG injection is low.

”Until recently, deaths within the minutes and hours after BPG injection have been assumed to be due to anaphylaxis,” says Chair of the presidential advisory writing group Amy E. Sanyahumbi, M.D., pediatric cardiologist at Texas Children’s Hospital and Assistant Professor of Pediatrics at Baylor College of Medicine.

“However, a growing number of reports of BPG-related deaths did not have the features of classic anaphylaxis, and, instead, point to cardiovascular reactions. This distinction is important, as it indicates the need for different strategies to prevent or stop these reactions to BPG.”

Cardiovascular signs and symptoms often appear shortly after BPG delivery, and sometimes even during injection.

They include low blood pressure, which can be fixed by moving your body in a different way, slow heart rate, and fainting, all of which can cause low blood flow to the heart, irregular heart rhythm, and sudden death.

Anaphylaxis symptoms, on the other hand, typically manifest themselves slightly later, up to an hour after the injection, and include coughing, respiratory distress, rapid heart rate, low blood pressure that does not react to position change, fainting, itching, and redness at the injection site.

Individuals with severe mitral stenosis, aortic stenosis, aortic insufficiency, or impaired left ventricular systolic function (ejection fraction 50%), as well as those with active symptoms of RHD, are at the highest risk of a cardiovascular reaction to BPG.

People with a low risk of cardiovascular reactivity and no history of penicillin allergy or anaphylaxis should be recommended BPG for the treatment and prevention of RHD, according to the advice.

BPG has been shown to be the most effective medication for preventing recurring rheumatic fever.

Treatment with oral penicillin should be actively considered for those with increased cardiovascular risks, such as severe valvular heart disease or heart failure.

The following standard practices are recommended for all BPG patients:

• Reducing injection pain and patient anxiety, both of which are known risk factors for injection-related fainting. Methods for pain reduction include applying firm pressure to the site for 10 seconds or application of an ice pack or the use of analgesics (such as acetaminophen, ibuprofen or other non-steroidal anti-inflammatory medications (NSAIDs).
• Patients should be well-hydrated prior to injection. Drinking at least 500 ml of water before injection has been found to prevent reflexive fainting.
• Eating a small amount of solid food within the hour before injection.
• Receiving the injection while laying down, which may reduce the risk of blood pooling in the extremities.
• Providers who administer BPG should be taught how to recognize and quickly treat symptoms such as low blood pressure, low heart rate or fainting.

“This advisory is urgently needed to raise awareness, provide risk stratification and guide health care professionals on easily implementable protocols to reduce risk and overcome reluctance to administer and receive BPG treatment for rheumatic heart disease,” adds Andrea Z. Beaton, M.D., vice-chair of the advisory writing group, a pediatric cardiologist at Cincinnati Children’s Hospital and associate professor of pediatrics at the University of Cincinnati School of Medicine.

Source: 10.1161/JAHA.121.024517

Image Credit: Getty

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