An unusual allergic reaction to an implanted defibrillator led a team of surgeons to pioneer a new approach. Discover how a gold-plated device and Gore-Tex leads provided a lifesaving solution for a young child with a rare heart condition.

Published: October 2, 2025

Estimated reading time: 4 minutes

The patient

A 4-year-old child with complex form of long QT syndrome needed surgical revision of her implantable cardioverter-defibrillator (ICD) due to device malfunction.

Long QT syndrome is a congenital heart condition that predisposes affected patients to suddenly develop serious heart rhythm disturbances (for example, ventricular tachycardia/fibrillation) that can be life-threatening. In addition to taking medications every day, some of these patients require implantable pacemakers or defibrillators to help support their underlying heart rhythms and potentially provide a lifesaving shock should that be necessary.

Pacemakers and ICDs have several components, including highly engineered “leads” (wires) to electrically connect the heart to a central generator, a hermetically sealed “can” holding electrical parts for “sensing” and pacing capabilities, a battery, programmable computer chips, and “clocks” to help regulate how the device controls the patient’s heart rhythms. A pacemaker helps ensure the heart rhythm isn’t too slow, and the ICD provides a shock to interrupt abnormally fast heartbeats. When very young children require a pacemaker or defibrillator, the system is implanted on the outer surface of the heart through an open-chest approach, a so-called “epicardial” system, and the generator is implanted in the abdomen. This is quite different than in adults with structurally normal hearts — they usually receive a “transvenous” system, with the leads implanted inside the heart through large veins draining in from above, and connected to a generator, usually implanted under the left collarbone.

The challenge

When it became clear that components of this child’s ICD were malfunctioning, we scheduled surgical replacement through an open chest. In the operating room, once the chest was opened, significant pockets of fluid that appeared to be pus were found around the prior ICD system. Concerned that this might be from a serious infection, the entire ICD system was removed, and all areas involved were thoroughly cleaned. Because the patient required a pacemaker so the heart rates wouldn’t get too slow, a temporary pacemaker was implanted before the chest was closed. The patient was started on IV antibiotics and followed closely in the Jennifer Lawrence Cardiac Intensive Care Unit. After extensive testing found no evidence of significant infection, we suspected this “sterile abscess” was due to the body’s reaction to implanted materials (for example, an internal allergy to the ICD generator itself). The challenge was how to solve this problem for this child who still required an ICD with pacemaker function.

Allergic reactions to implanted pacemakers or ICDs are extremely rare, so very few patients have faced this problem.

The solution

In very rare cases of adult patients, a gold-plated generator has been implanted with good outcomes, since that coating does not cause the same allergic reaction. In addition, we were concerned that the body’s reaction may have been triggered by the ICD leads. There are rare reports in adults of covering the outer surface of those leads with an inert material (Gore-Tex) before implantation, successfully preventing another allergic reaction after implantation.

Approximately one week after the surgery that found the abscess and prompted removal of the first ICD system, repeat open-chest surgery led to uneventful implantation of the new gold-plated ICD generator and Gore-Tex-covered ICD leads.

The result

The patient recovered quickly and was soon discharged home in good condition. When seen in outpatient clinic the following week, surgical incisions were healing well, and interrogation of the ICD system showed excellent performance.

The care team