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Published on: June 9, 2023
Unusual Outcome of a Right Ventricular Rhabdomyoma in an Infant
Bruno Lefort1, Julie Lothion2, Jean-Marc El Arid2
1Children Hospital Gatien de Clocheville, Tours, France University François Rabelais, Tours, France INSERM UMR 1069 «nutrition, croissance et cancer», Tours, France bruno.lefort@univ-tours.fr.
Insights
Cardiac rhabdomyomas, common benign heart tumors, can obstruct blood flow. This case highlights the need for close monitoring of infants with large rhabdomyomas, even if initially nonobstructive.
Area of Science:
- Cardiology
- Pediatric Oncology
- Tumor Biology
Background:
- Rhabdomyomas are the most frequent benign primary cardiac tumors, often linked to tuberous sclerosis.
- Spontaneous regression is a common outcome for cardiac rhabdomyomas.
Observation:
- An infant presented with a large, nonobstructive right ventricular rhabdomyoma at birth and four months.
- The rhabdomyoma progressed to cause severe right ventricle outflow tract obstruction by six months of age.
Findings:
- This case demonstrates a rare progression of a cardiac rhabdomyoma from nonobstructive to obstructive.
- Surgical intervention was required to remove the tumor causing significant outflow tract obstruction.
Implications:
- Close surveillance is crucial for infants diagnosed with large cardiac rhabdomyomas, irrespective of initial obstruction.
- Understanding the dynamic growth patterns of cardiac rhabdomyomas is essential for timely clinical management.
Abstract:
Rhabdomyomas are the most common benign cardiac tumors. They may often be associated with tuberous sclerosis. In many cases, cardiac rhabdomyomas undergo spontaneous regression. Here, we report the unusual case of an infant with a large nonobstructive right ventricular rhabdomyoma at birth and at four months of age, which subsequently caused severe right ventricle outflow tract obstruction at six months of age, prompting surgery to remove the tumor. Close monitoring should be done in infants with large nonobstructive cardiac rhabdomyomas.
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