Rheumatic Heart Disease: Diagnosis and Management

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Until a vaccine for acute rheumatic fever is available, antibiotics and surgery remain the current standard of care for RHD.
Until a vaccine for acute rheumatic fever is available, antibiotics and surgery remain the current standard of care for RHD.

Among the top 10 infectious causes of death globally, the group A Streptococcus (GAS) bacterium can result in a range of diseases, including pharyngitis, scarlet fever, and post-streptococcal toxic shock syndrome. Acute rheumatic fever (ARF), another potential consequence of GAS infection, can lead to rheumatic heart disease (RHD), which is associated with high mortality, primarily in children, adolescents, and young adults in low- and middle-income countries.1  

RHD has been nearly eliminated in the United States, with a current incidence of approximately 0.04–0.06 cases per 1000 children annually, although rates are higher in some US territories and regions with large populations of immigrants from certain countries.2

Stanford Shoor, MD, clinical professor of medicine and rheumatology at Stanford University, Palo Alto, California, estimates that he sees 2 or 3 cases per year. “Because of widespread use of antibiotics in the United States, the incidence of rheumatic carditis is relatively low. In addition, unless the disease involves significant arthritis, we [rheumatologists] may not see them,” he told Cardiology Advisor.

When patients do present to a rheumatologist with symptoms characteristic of ARF, it must be considered in the diagnostic process, and the physician may be involved in the patient's ongoing care. “As patients with ARF can present with fever, rashes, and polyarthritis, we have to consider ARF in the differential diagnosis, as patients with such symptoms can appear to have rheumatoid arthritis, systemic lupus erythematosus, or vasculitis,” said Dr Shoor. “Therapeutically, if patients are suffering significantly from arthritis, we may work with cardiologists and use nonsteroidal anti-inflammatory drugs or even corticosteroids in low doses [for abbreviated] treatment [periods] until these symptoms resolve.”

Dr Shoor gives an example of a patient with ARF that he followed for 5 years who, after initial treatment with low-dose steroids for arthritis, has remained steroid-free for several years with no rash or arthritic disease flares, continues to take  long-term antibiotics, as is the case with all ARF patients.

While echocardiographic screening algorithms have improved diagnostic accuracy in RHD, there is an urgent need for an effective preventive vaccine.3 Researchers have explored several approaches toward the development of a vaccine for ARF, but until one is available, antibiotics and often surgery remain the current standard of care for RHD.1

Cardiology Advisor interviewed Jack Boyd, MD, clinical assistant professor of cardiothoracic surgery at Stanford University School of Medicine, to gain insight into the comprehensive management of RHD.

Cardiology Advisor (CA) : What is your specialty's role in treating patients with RHD, and how might you work with other specialists on such cases?

Dr Boyd: A team-based approach to the treatment of RHD provides the best outcomes. Appropriate triage, detailed echocardiographic evaluation, timely intervention, nursing input for patient and family education, procedural expertise, dependable follow-up, and regular audits of results necessitate a multidisciplinary group. Members of the team include nursing, a cardiology unit—cardiologists, imaging specialists, and interventional cardiologists—and cardiac surgery. This level of comprehensive care consistently gets the right patient the right treatment at the right time.

Surgical decision-making in patients with RHD is complex, and surgical repair of diseased valves requires experience and expertise. Rheumatic valves are among the most difficult valves to repair as the tissue [tends to be] fibrotic and scarred. Valvular repair, however, can avoid the need for long-term anticoagulation and mitigate the risks of thromboembolism [associated] with prosthetic valves. [In addition, surgical repair] makes pregnancy safe, allows for continued participation in sports, and avoids the sudden deterioration that can occur with prosthetic valves in the mitral position.

Symptoms, age, severity of stenosis, anatomy of the valve, [comorbid] cardiac conditions, reliable access to medications after surgery, and surgical risk are discussed by the team and factored into the final recommendation for intervention. 

CA: Have there been any recent developments in diagnosis, prevention, or treatment of RHD?

Dr Boyd: RHD is one of the world's most neglected and easily prevented diseases in children. Timely access to appropriate health care with the ability to diagnose GAS throat infections and treat it with a short course of antibiotics would prevent and control the vast majority of RHD cases. Investments in healthcare infrastructure, education, and resources in the developing world are increasing.

For those in which [infection] prevention is too late, valvular repair techniques continue to be refined.

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