Invasive cardiac procedures in patients with Ehlers-Danlos syndrome (EDS) should be performed with extreme caution and only after a thorough risk/benefit analysis, according to a new case study.
The report, “Arrhythmias and myocardial fragility in Ehlers-Danlos syndrome: Complications after routine ICD placement,” was published in the journal HeartRhythm Case Reports.
EDS patients often have cardiovascular involvement, but a link between EDS and cardiac arrhythmias (abnormal heart rhythm) has never been assessed.
Now, researchers from the VA Greater Los Angeles Healthcare System describe the first reported case of sustained symptomatic ventricular arrhythmias (arrhythmias that originate in the bottom chambers of the heart, called the ventricles) in an EDS patient.
A 59-year-old man was hospitalized after several episodes of syncope (temporary loss of consciousness) and presyncope (state of lightheadedness and feeling faint). The man had a history of high blood pressure, high cholesterol, depression, and EDS of an unknown subtype.
A physical exam showed the patient had classic EDS symptoms — mild hyperextensible joints and extremely elastic skin. A two-week ambulatory monitor measured his heart rhythm and revealed significant abnormalities indicative of ventricular arrhythmias.
Imaging of the heart showed no cardiac irregularities, and the patient appeared to have normal cardiac function. Other tests were conducted to exclude coronary artery disease or coronary anomalies.
Because the presyncope and syncope events were thought to be the result of ventricular arrhythmias, physicians decided on an invasive procedure.
After considering their choices, the physicians inserted an implantable cardioverter-defibrillator (ICD), a small, battery-powered pulse generator, under the skin. Wires or leads run from the pulse generator to positions on the heart.
If the ICD detects an abnormal heart rhythm, it delivers an electric shock to restore a normal heartbeat.
The procedure was completed without complications, and the patient went home the next day.
But three days later, the patient returned to the emergency room with progressive worsening chest pain, atrial flutter (a common abnormal heart rhythm), and a high heart rate.
He was found to have experienced lead perforation, a complication that sometimes results from insertion of an ICD. Lead perforation is defined as the migration and perforation of an implanted lead. The patient had multiple perforations, so the device had to be removed.
Because of the fragility of the patient’s vascular tissue, physicians decided not to replace the ICD. The patient experienced other complications, but was discharged in stable condition 12 days after the initial operation and was prescribed antiarrhythmic medications.
After discussions with other electrophysiologists, physicians decided to continue medically managing the arrhythmias rather than risk more perforations.
Antiarrhythmic therapy using amiodarone and electrical cardioversion (where the heart is shocked to convert it from an irregular rhythm back into a normal one) improved the patient’s symptoms without the need for additional related hospitalizations.
“This case illustrates the hazards of cardiovascular percutaneous [made through the skin] and surgical intervention in EDS patients, highlighting the diffuse nature of the connective tissue disorder,” the researchers wrote.
“Patients with EDS pose a unique management challenge in cardiac electrophysiology. Ablations and device implantations should be undertaken with extreme caution, thorough risk/benefit analysis, and comprehensive discussion,” the team concluded.
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