Related Experiment Videos
Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision
Insights
A rare calcified right ventricular fibroma caused pulmonic stenosis in a child. Surgical removal successfully treated the obstruction, with significant hemodynamic improvement post-operation.
Area of Science:
- Cardiology
- Pediatric Oncology
- Cardiac Surgery
Background:
- Fibromas are rare benign tumors that can occur in the heart.
- Right ventricular fibromas can cause significant outflow tract obstruction and hemodynamic compromise.
- Calcification within cardiac tumors is uncommon, particularly in pediatric fibromas.
Observation:
- A 5-year-old boy presented with symptoms of pulmonic stenosis.
- Imaging revealed a large calcified mass in the right ventricular outflow tract, causing a 120 mm Hg gradient.
- Surgical exploration identified an oval-shaped tumor obstructing the right ventricular outflow tract, attached to the interventricular septum.
Findings:
- The obstructing tumor was surgically removed.
- Histological examination confirmed the mass as a fibroma.
- Post-operative hemodynamic studies showed near-complete resolution of the pressure gradient.
Implications:
- This case represents the fifth reported instance of a calcified right ventricular fibroma with successful surgical intervention.
- Intracardiac calcifications causing obstruction in children strongly suggest the possibility of a fibroma.
- Early diagnosis and surgical management are crucial for favorable outcomes in pediatric cardiac tumors causing obstruction.
Abstract:
A 5-year-old boy with clinical findings of pulmonic stenosis was found to have a large calcified mass in the right ventricular outflow region and a gradient of 120 mm Hg between the right ventricle and the pulmonary artery. At surgery, an ovalshaped tumor attached to the interventricular septum and obstructing the right ventricular outflow tract was removed. The child survived and is doing well. Histologically, the tumor had the characteristics of fibroma. A hemodynamic study three months after surgery showed almost complete abolishment of the gradient. To our knowledge this is the fifth reported case of calcified right ventricular fibroma with successful operation. In childhood intracardiac calcifications, together with obstruction, are highly suggestive of this lesion.