Quick Case Study

Potentially Fatal Genetic Cardiac Condition Requires Specialized Surgery

Duke experts help patients manage arrhythmia syndromes

Child being hooked up to a halter monitor

After his other family members were diagnosed with a genetic cardiac condition, a school-aged boy was evaluated and also found to have catecholaminergic polymorphic ventricular tachycardia (CPVT), which causes sudden arrhythmias in otherwise healthy children. The arrhythmias may not be detected until a patient suffers syncope or cardiac arrest, leading to a high rate of mortality for the condition.

In CPVT, the heart responds in a disorganized manner to catecholaminergic stimuli including adrenaline. In times of emotional or physical distress, adrenaline may cause the heartbeat to accelerate into ventricular tachycardia. If the arrhythmia continues, it may develop into ventricular fibrillation, potentially causing cardiac arrest and sudden death.

The family came to Duke electrophysiologist and pediatric cardiologist Andrew P. Landstrom, MD, PhD, to manage the condition early on. “Because the family was aware of their history, we were following the patient as soon as we met. He never had a symptomatic day.”

The standard course of treatment for CPVT indicates medications first. However, even on beta blockers and other anti-arrhythmic medications, the patient was suffering from arrhythmias, detected only via his heart monitor during a stressful event.

How did Landstrom approach this patient’s case to better manage his condition?

“CPVT is one of the hardest syndromes we deal with,” Landstrom says. “Children will often have worsening of their arrhythmias, despite medication. After that, the only option is surgical intervention at a specialty center like Duke.”

During his training at the Mayo Clinic, Landstrom worked alongside Ryan M. Antiel, MD, now a Duke pediatric surgeon. While at the Mayo Clinic, the pair studied a pioneering surgical treatment for CPVT: sympathetic denervation.

Landstrom recommended the surgery to the family: “The patient’s mother asked, ‘If this were your child, what would you do?’ I said, ‘I would see Dr. Antiel.’”

In a minimally invasive, thorascopic surgery, Antiel removed a section of the boy’s sympathetic nerve, carefully dividing it at the stellate ganglion. Without this portion of the sympathetic nerve, the heart becomes less responsive to catecholaminergic stimuli, decreasing the pro-arrhythmia state to reduce future arrhythmias. Patients with other arrhythmia disorders, such as Long QT Syndrome (LQTS), where the heart repolarizes slowly after a heartbeat, creating a long interval between the Q and T waves of an ECG, can also benefit from the procedure.

Side effects from the surgery often include dryness of the skin of the arm and the face and lack of facial flushing on one side of the face. These subtle signs are expected and indicate the denervation was successful.

“The pain and discomfort are very minimal,” Antiel says. “Typically, the child is up and walking the afternoon following the procedure and home the next day.”

Almost a year after the procedure, the patient is managing his condition with medication with no further signs of life-threatening arrhythmias.

“If cardiologists have patients with arrhythmia syndromes, especially CPVT and LQTS, and are having a hard time managing those arrhythmias, this surgery is a very viable option to consider,” Landstrom says.

“Because these are rare procedures and not performed many places, it’s essential that patients receive care at centers of excellence like Duke, with a multidisciplinary approach and experience in this surgery,” says Antiel. “I couldn’t do this procedure without Dr. Landstrom and our support teams.”

Refer a Patient

To refer a patient, call Duke Heart Center at 919-681-5816 or log into Duke MedLink.