Heart Failure and Cardiomyopathy in Children

What is “heart failure”?

Heart failure is a clinical diagnosis in which the heart isn’t pushing enough blood forward, due to impaired filling or impaired emptying, to meet the demands of all of the organs. This results in poor organ function, with blood also backing up in other organs, such as the lungs or the liver.

Why would someone have heart failure?

In pediatrics, heart failure is often found in patients with underlying structural problems in their heart (congenital heart disease) or of the muscle of the heart itself (cardiomyopathy) which cause the heart muscle to not squeeze or relax normally. Some cardiomyopathies can be inherited, some may be a symptom of a larger disorder affecting other organs, and some may never have a clear cause. Cardiac dysfunction can also happen after an infection causes the heart to be inflamed (myocarditis) or after exposure to some forms of chemotherapy.

How common are cardiomyopathies?

Not very. The Children’s Cardiomyopathy Foundation estimates that there are 1,000-5,000 new pediatric cases diagnosed yearly. American and international studies estimate that perhaps 12 in one million children may have a form of cardiomyopathy. The vast majority of cardiomyopathies with heart failure present in infancy.

Does everyone with a cardiomyopathy have heart failure?

No. Many children with cardiac dysfunction are without symptoms for a long time, while some children can become symptomatic quickly.

Can you treat heart failure?

Yes! When possible, a team of physicians, surgeons and nurses can help prevent patients from developing symptoms of heart failure. Sometimes that is accomplished through the use of oral medications. In some cases, however, intravenous medications need to be used. Less commonly, more advanced therapies, such as mechanical devices to assist the heart’s function (commonly called, Ventricular Assist Devices or VADs) or heart transplantation are needed.

In some patients, heart failure may be associated with electrical issues causing heart rhythm changes that have the potential to be life-threatening. In these cases, an Electrophysiologist may be consulted to consider other rhythm medications and possibly a device called an Implantable Cardioverter-Defibrillator (ICD).

Should patients be screened for heart failure? Who should get screened? What does screening entail?

It is recommended that first degree family members like parents and siblings of a patient with a cardiomyopathy be screened by a cardiologist. Usually, screening consists of an exam by a cardiologist with review of an electrocardiogram (ECG) which picks up the electrical signals of the heart and an echocardiogram, an ultrasound that looks at the size and function of the heart.

The age of screening matters. Cardiomyopathy and heart failure can occur at different ages, so it is important to screen at-risk infants. Since one-time screening might miss later disease, repeat screenings should be done every few years, before spacing out to every few years in young adulthood.

Does genetics play a role?

It certainly can. There is better understanding today of specific mutations that may cause cardiomyopathy than there was a decade ago. However, we still don’t fully understand all of the genetics involved. In a patient with disease, it may be possible to identify a mutation associated with that disease. This may make it easier to determine which family members are at an increased genetic risk by testing those family members for the specific mutation.

For more information on the Heart Failure and Heart Transplant Program at Nationwide Children’s Hospital, click here.

Deipanjan Nandi, MD MSc
Deipanjan "Deip" Nandi, MD, is a cardiologist with the Heart Transplant & Heart Failure Program at Nationwide Children’s Hospital. Originally from Fort Worth, Texas, he went on to receive an undergraduate degree in Biochemistry from Harvard University, and his medical degree from Duke University School of Medicine. He also obtained a Master’s of Science in Health Policy as a US-UK Fulbright Scholar from the London School of Economics. Dr. Nandi completed his internship and residency at CS Mott Children’s Hospital at the University of Michigan. He then moved to the Children's Hospital of Philadelphia, where he completed his general cardiology fellowship as well as an advanced fellowship in Heart Transplant & Heart Failure. His clinical interests include pediatric heart failure, cardiomyopathies, transplantation and mechanical circulatory support. His research interests include comparative-effectiveness research focusing on the management of pediatric heart disease, as well as outcomes research in pediatric heart transplantation.

Leave a Reply

Your email address will not be published. Required fields are marked *