137. Surgical Debulking of Large Ventricular Fibromas in Children
Presented During: Congenital Rapid Fire Orals
Massachusetts General Hospital
Boston, MA
United States - Contact Me
I am a cardiothoracic surgery fellow at Massachusetts General Hospital. My clinical interest is congenital heart surgery. My academic interests are translation science, surgical eductation, and leadership. I am originally from Ohio.
Sunday, April 28, 2024: 10:19 AM - 10:26 AM
7 Minutes
Metro Toronto Convention Center
Room: Room 716
Description
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.
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
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.
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
Presentation Duration
You will have a 4 minute presentation followed by 3 minutes of discussion from the audience. All presenters must adhere to the presentation and discussion times provided. The AATS will begin to play music once your speaking time is exceeded.