Some children with severe neurological impairment (SNI) may have an abnormal heart rhythm, called an arrhythmia. This risk can be genetic, occur with congenital heart disease, occur with some medications, and be occasionally related to how the nervous system controls the heart rate. Arrhythmias can range from mild and harmless to serious and life-threatening. More severe rhythm problems may cause dizziness, fainting, or, in rare cases, sudden stopping of the heart (cardiac arrest) When there is risk, screening for arrhythmias is important and may include an electrocardiogram (ECG), evaluation in a cardiology clinic, and occasionally a wearable heart monitor worn for days. Treatment depends on the type of arrhythmia and may include medications to help control the heart rhythm or support the heart rate. If the cardiologist identifies an unsafe rhythm, a pacemaker may be recommended to help maintain a safe heart rhythm.

Children with SNI who have epilepsy have risk for SUDEP (sudden unexpected death in epilepsy). This uncommon event is thought to occur when a seizure disrupts the brain’s control over heart function. Your child’s neurologist can review this with you in detail. 

Some children are born with cardiac conditions such as holes in the heart, abnormal heart anatomic connections, or problems with the heart valves. These can be benign conditions with no long-term consequence. In some circumstances they can be serious and require medical treatment or even surgery. Your cardiologist will ​explain the possible effects of these conditions and suggest treatment if necessary. ​​​​

Cardiomyopathy refers to a problem with the heart muscle, causing it to not function properly. Cardiomyopathies can sometimes occur in children with SNI who have a genetic or metabolic condition. They can cause the heart muscle to become dilated (enlarged), hypertrophied (too thick), or restrictive (too stiff). While there is no cure for cardiomyopathy, medical therapies can help provide treatment and reduce symptoms arising from this condition.

The above problems can cause heart failure in some. Heart failure happens when the heart cannot pump enough blood to meet the body’s needs, or when fluid builds up in the lungs or other tissues because the heart is not working well. This can be caused by problems with the heart muscle (called cardiomyopathy), structural heart differences present at birth (congenital heart disease), or long-standing abnormal heart rhythms. Some genetic conditions affect both the brain and the heart, which can increase the risk of heart failure in children with SNI. 

Children with heart failure may have symptoms such as fast or labored breathing, swelling, poor feeding, low energy, or frequent respiratory illnesses. To evaluate heart failure, doctors may use heart imaging tests like an echocardiogram, along with blood tests to assess heart function and check how other organs, such as the kidneys and liver, are affected. Treatment focuses on reducing symptoms and supporting the heart. This may include medications to remove extra fluid, improve how strongly the heart pumps, or control heart rhythm.