Coronary-Sparing Resection of a Pediatric Left Ventricular Fibroma Involving the Left Circumflex Artery: Six-Year
Manuela Silva Lopes1, Maria Emanuel Amaral1, Andreia Francisco1
1Department of Pediatric Cardiology and Referral Center for Congenital Cardiac Defects, Unidade Local de Saúde de Coimbra, Coimbra, Portugal.
Insights
Pediatric left ventricular fibromas involving coronary arteries present surgical challenges. A conservative, coronary-sparing approach for this rare cardiac tumor in children demonstrated durable and safe outcomes.
Area of Science:
- Pediatric Cardiology
- Cardiac Surgery
- Cardiovascular Imaging
Background:
- Left ventricular (LV) fibromas are rare pediatric cardiac tumors.
- Tumor involvement of epicardial coronary arteries complicates surgical management.
- LV fibromas can cause significant symptoms like outflow tract obstruction or valve compression.
Purpose of the Study:
- To report a case of a pediatric LV fibroma involving the left circumflex coronary artery.
- To illustrate the utility of multimodality imaging in surgical planning.
- To evaluate the long-term outcomes of a conservative, coronary-sparing surgical strategy.
Main Methods:
- Case report of a 3-month-old female with an LV fibroma.
- Multimodality imaging including cardiac MRI and coronary angiography for surgical planning.
- Partial tumor resection with mitral valvuloplasty and coronary artery preservation.
Main Results:
- The patient underwent successful partial tumor resection and mitral valvuloplasty at 23 months of age.
- The intratumoral left circumflex coronary artery was preserved during surgery.
- At 6-year follow-up, the patient is asymptomatic with stable residual tumor, preserved LV function, and no arrhythmias.
Conclusions:
- A conservative, coronary-sparing surgical strategy is feasible and safe for selected pediatric LV fibromas involving major coronary arteries.
- Multimodality imaging is crucial for precise surgical planning in these complex cases.
- Long-term follow-up demonstrates durable and favorable outcomes with this approach.
Abstract:
Left ventricular (LV) fibromas in children can pose major surgical challenges when epicardial coronary arteries are involved. We report the case of a 3-month-old female diagnosed with a large intramyocardial LV fibroma causing mitral valve compression and traversed by the left circumflex coronary artery. Multimodality imaging, including cardiac magnetic resonance imaging and coronary angiography, was essential for surgical planning. Progressive tumor growth, causing compression of the left lung and worsening mitral regurgitation, prompted partial tumor resection with mitral valvuloplasty at 23 months of age at Boston Children's Hospital, preserving the intratumoral left circumflex artery. Six years later, the patient remains asymptomatic, with stable residual tumor size, preserved LV function, and no clinically significant arrhythmias. This case illustrates the durability and safety of a conservative, coronary-sparing surgical strategy in selected pediatric patients with ventricular fibromas involving major coronary arteries.
