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Cardiology

New guidelines: Identifying the source of heart muscle damage in children is critical to effective treatment.

A new scientific statement from the American Heart Association, which was published today in the journal Circulation, states that the treatment of children who have cardiomyopathy ought to be tailored to each child based on the underlying cause, symptoms, and stage of the condition.

Cardiomyopathy is a condition of the muscle of the heart that can make it hard for the heart to pump blood to the rest of the body, which could result in heart failure or death. Pediatric cardiomyopathies come in a variety of forms and are all uncommon but potentially fatal conditions. They influence 1 of every 100,000 youngsters, as per population-based examinations in the US, Finland, and Australia.

The new scientific statement, the first to focus on children’s cardiomyopathy treatment, emphasizes significant differences between adult and child cardiomyopathy and heart failure treatments. The statement is a companion to the 2019 scientific statement on pediatric cardiomyopathy diagnosis from the Association.

“Kids’ cardiomyopathies might have comparative names to cardiomyopathies in grown-ups; however, they frequently have very different causes, risk factors, progression paths, and outcomes,” stated the statement-writing group chair Steven E. Lipshultz, M.D., FAHA. Children generally do not smoke, so they do not have the same behavioral or environmental factors that contribute to cardiomyopathy. They don’t drink liquor. They do not have long-standing conditions like Type 2 diabetes or obesity. Therefore, children’s treatments, which may include replacing enzymes or a heart transplant, must be tailored to the underlying causes of their cardiomyopathy.”

“Children’s cardiomyopathies may have similar names to adult cardiomyopathies; however, they frequently have very different causes, risk factors, progression paths, and outcomes.”

Steven E. Lipshultz, M.D., FAHA, chair of the statement writing group. 

Instead of Type 2 diabetes, alcoholism, long-term high blood pressure, or a family history of the condition, as is the case in adults, many of the causes of cardiomyopathy in children are related to genetic abnormalities. Children with cardiomyopathy may experience breathing difficulties, heart palpitations, falling asleep while exercising, congestion of the neck veins, swelling in the ankles, feet, legs, or abdomen, or signs and symptoms of heart failure, such as difficulty breathing, inadequate nutrition or growth, excessive sweating, low blood pressure, or fatigue.

The treatment of the most prevalent cardiomyopathies in children was the primary focus of the statement-writing committee.

  • Dilated cardiomyopathy occurs when the heart is too big and the chambers that pump blood don’t work as hard.
  • The heart muscle in hypertrophic cardiomyopathy is thickened and stiff, making it hard for the heart to relax and for blood to fill the chambers.
  • Prohibitive cardiomyopathy is an uncommon type of heart muscle illness in which the hardened heart muscle doesn’t permit the ventricular chambers to load up with blood, typically maneuvering blood into the atria (the top offices of the heart), lungs, and body, causing the side effects and indications of cardiovascular breakdown.

According to the statement-writing committee, pediatric cardiomyopathy can be diagnosed at a variety of stages. Some patients may have the condition but not show any symptoms, while others may have severe symptoms or end-stage disease. To develop a treatment strategy, pediatricians are urged to conduct a thorough examination and evaluation of their patients.

Lipshultz, who is also a professor in the department of pediatrics and the former chair of pediatrics at the University at Buffalo’s Jacobs School of Medicine and Biomedical Sciences in Buffalo, New York, stated, “Genetic testing of all children with cardiomyopathy is prudent and is likely to have clinical value.” Savings in both financial and emotional costs may result from this genetic screening.”

Other important points from the committee that wrote the statement:

  • Treatment might be started in a few pediatric patients who are in danger for cardiomyopathy before they foster side effects to lessen the movement of the sickness.
  • Although adult heart failure treatments have limited efficacy in treating children with dilated cardiomyopathy, there is evidence that their use has reduced the number of children who die from the condition.
  • When compared to treatments and devices for adults, therapies for children with hypertrophic cardiomyopathies that prevent sudden death have age- and size-specific parameters. Examples of these therapies include exercise restrictions or an internal defibrillator.
  • End-stage pediatric cardiomyopathy can be treated with a heart transplant. While ventricular help gadgets are regularly utilized as a transitory measure while anticipating a heart relocation, the gadgets are associated with a low pace of ventricular recuperation, like the outcomes among adults.

“What’s to come is promising, as constraints in example size and financing in pediatric clinical preliminaries are being tended to with the utilization of learning networks, including different pediatric cardiomyopathy places,” Lipshultz said. “Future cause-specific therapy options for pediatric cardiomyopathy could be improved and refined by innovative use of multi-center registries and adaptive trial design.

More information: Treatment Strategies for Cardiomyopathy in Children: A Scientific Statement From the American Heart Association, Circulation (2023). DOI: 10.1161/CIR.0000000000001151

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