Surgical Debulking of Large Ventricular Fibromas in Children
Presented During:
Sunday, April 28, 2024: 10:19AM - 10:26AM
Metro Toronto Convention Center
Posted Room Name:
Room 716
Abstract No:
137
Submission Type:
Abstract Submission
Authors:
Ajami Gikandi (1), Peter Chiu (2), Meena Nathan (3), Edward O'Leary (3), Edward Walsh (1), Rebecca Beroukhim (3), Pedro del Nido (3)
Institutions:
(1) N/A, N/A, (2) N/A, United States, (3) Boston Children's Hospital, Boston, MA
Submitting Author:
Ajami Gikandi
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Co-Author(s):
Peter Chiu
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*Meena Nathan
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Boston Children's Hospital
Boston Children's Hospital
Edward O'Leary
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Boston Children's Hospital
Boston Children's Hospital
Edward Walsh
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N/A
Rebecca Beroukhim
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Boston Children's Hospital
Boston Children's Hospital
*Pedro del Nido
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Boston Children's Hospital
Boston Children's Hospital
Presenting Author:
Ajami Gikandi
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N/A
Abstract:
Objective:
The optimal surgical resection strategy for ventricular fibromas is uncertain. We describe the outcomes of tumor debulking, ventricular cavity reconstruction, and atrioventricular valve repair if needed.
Methods:
Single-center, retrospective cohort study of patients undergoing ventricular fibroma resection between 2000 and 2023. Over the course of the study period, the approach has evolved from complete resection to a strategy of tumor debulking.
Results
Among 52 patients, median age at surgery was 2.0 years (IQR 9.2 months-4.6 years) and median weight was 11.5 (IQR 8.5-18.9) kilogram. Median tumor volume index was 69 (IQR 49-169) milliliters/m2. Tumor distorted the AV valve/subvalvar apparatus in 30 (58%) patients, caused outflow tract obstruction in 3 (6%) patients, and 5 (10%) patients had ≥ moderate AV valve regurgitation. Surgery was indicated for arrythmia (n=45, 86%), symptoms (n=14, 27%), and/or hemodynamic compromise (n=11, 21%). Tumor was primarily debulked in 34 (65%) patients, including the last 21 patients. The ventricular cavity was entered in 15 (29%) patients. Concomitant AV valvuloplasty was performed in 18 patients (15 mitral and 3 tricuspid), while 3 patients received intraoperative cryoablation. Residual rim of tumor was left around coronary arteries in patients who had signs of extrinsic coronary compression (n=7, 13%), myocardial bridging (n=6, 12%), and/or intratumor coronary artery course (n=4, 8%). Two patients required early reoperation for residual AV valve insufficiency and one patient late reoperation for LVOT obstruction. Median duration of follow-up was 2.5 (IQR 0.8 -6.5) years. There was no mortality or heart transplantation. During the follow-up period, sustained VT occurred in 1 (2%) patient. No other patients had evidence of ventricular arrythmia on Holter monitoring or EKG. Among 38 patients with post-op v-stim studies, 3 (8%) experienced inducible VT / VF requiring cardioversion / defibrillation. Two of these patients received an ICD. Pre and post-debulking LV EF did not significantly differ (P=.069). No patients had signs of outflow tract obstruction or > moderate AV valve regurgitation on latest imaging.
Conclusions:
Large ventricular fibromas can be resected safely with appropriate surgical planning and an emphasis on debulking rather than complete resection. Most children are cured of life-threatening arrythmias and maintain LV function.
The optimal surgical resection strategy for ventricular fibromas is uncertain. We describe the outcomes of tumor debulking, ventricular cavity reconstruction, and atrioventricular valve repair if needed.
Methods:
Single-center, retrospective cohort study of patients undergoing ventricular fibroma resection between 2000 and 2023. Over the course of the study period, the approach has evolved from complete resection to a strategy of tumor debulking.
Results
Among 52 patients, median age at surgery was 2.0 years (IQR 9.2 months-4.6 years) and median weight was 11.5 (IQR 8.5-18.9) kilogram. Median tumor volume index was 69 (IQR 49-169) milliliters/m2. Tumor distorted the AV valve/subvalvar apparatus in 30 (58%) patients, caused outflow tract obstruction in 3 (6%) patients, and 5 (10%) patients had ≥ moderate AV valve regurgitation. Surgery was indicated for arrythmia (n=45, 86%), symptoms (n=14, 27%), and/or hemodynamic compromise (n=11, 21%). Tumor was primarily debulked in 34 (65%) patients, including the last 21 patients. The ventricular cavity was entered in 15 (29%) patients. Concomitant AV valvuloplasty was performed in 18 patients (15 mitral and 3 tricuspid), while 3 patients received intraoperative cryoablation. Residual rim of tumor was left around coronary arteries in patients who had signs of extrinsic coronary compression (n=7, 13%), myocardial bridging (n=6, 12%), and/or intratumor coronary artery course (n=4, 8%). Two patients required early reoperation for residual AV valve insufficiency and one patient late reoperation for LVOT obstruction. Median duration of follow-up was 2.5 (IQR 0.8 -6.5) years. There was no mortality or heart transplantation. During the follow-up period, sustained VT occurred in 1 (2%) patient. No other patients had evidence of ventricular arrythmia on Holter monitoring or EKG. Among 38 patients with post-op v-stim studies, 3 (8%) experienced inducible VT / VF requiring cardioversion / defibrillation. Two of these patients received an ICD. Pre and post-debulking LV EF did not significantly differ (P=.069). No patients had signs of outflow tract obstruction or > moderate AV valve regurgitation on latest imaging.
Conclusions:
Large ventricular fibromas can be resected safely with appropriate surgical planning and an emphasis on debulking rather than complete resection. Most children are cured of life-threatening arrythmias and maintain LV function.
CONGENTIAL:
Neonatal and Pediatric Cardiac Surgery
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