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Multiple myocardial hamartomas causing ventricular tachycardia in young children: combined surgical modification and
F Gharagozloo1, C J Porter, H D Tazelaar
1Division of Cardiovascular and Thoracic Surgery, Mayo Clinic Scottsdale, Arizona.
Insights
Incessant ventricular tachycardia in infants can be caused by myocardial hamartomas. Surgical intervention combined with medical therapy effectively controlled the condition in two pediatric cases.
Area of Science:
- Pediatric Cardiology
- Cardiac Electrophysiology
- Cardiovascular Pathology
Background:
- Incessant ventricular tachycardia (IVT) in infants presents a diagnostic challenge, often lacking clear structural abnormalities on standard imaging.
- Early diagnosis and intervention are crucial due to potential hemodynamic instability.
Observation:
- Two infants presented with recurrent, hemodynamically compromising IVT.
- Echocardiography revealed no structural heart defects.
- Electrophysiologic studies localized the arrhythmogenic focus in one patient.
Findings:
- Diffuse myocardial hamartomas were identified as the cause of IVT in both patients.
- Combined surgical resection, cryoablation, and medical therapy led to sustained rhythm control.
- Patients achieved normal sinus rhythm postoperatively with ongoing medication.
Implications:
- Myocardial tumors, specifically hamartomas, should be suspected in pediatric IVT cases with negative initial imaging.
- Electrophysiologic studies are vital for localization when medical therapy fails.
- A multidisciplinary approach involving surgery and electrophysiology offers a viable treatment strategy for this rare condition.
Objective:
To describe two cases of incessant ventricular tachycardia caused by multiple myocardial hamartomas.
Material And Methods:
Two infants, 13 and 14 months old, who had had multiple episodes of symptomatic tachycardia were referred to our institution. Incessant ventricular tachycardia was diagnosed. Initially, the patients received pharmacologic therapy. The recurrent tachycardia resulted in notable hemodynamic instability. No structural abnormalities were detected on the echocardiograms. In one patient, an electrophysiologic study revealed that the site of ventricular ectopic beats was in the anterolateral wall of the left ventricle, midway between the apex and the base. In the other patient, a preoperative electrophysiologic study was not undertaken because of the inability to obtain central venous access.
Results:
Diffuse hamartomas were found throughout the ventricular myocardium in both patients. Surgical resection and cryoablation of the lesions in combination with medical therapy helped control the tachyarrhythmia. At 7 and 17 months postoperatively, the patients were in normal sinus rhythm and were receiving medication.
Conclusion:
In young children who have incessant ventricular tachycardia but no lesion evident on echocardiography, angiography, or other imaging modalities, a myocardial tumor should be suspected, and pharmacologic therapy should be instituted. If the medical regimen fails, surgical intervention should be undertaken, directed at areas localized by inspection and by preoperative and intraoperative electrophysiologic studies.