139. Left Ventricular Outflow Tract Obstruction as Modifiable Risk Factor for Recurrence of Endocardial Fibroelastosis
Presented During: Congenital Rapid Fire Orals
Boston Children's Hospital
Linz
Austria - Contact Me
Research Fellow in Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, MA, USA
Resident in Cardiac Surgery, Pediatric and Congenital Heart Surgery Linz, Austria
Sunday, April 28, 2024: 10:33 AM - 10:40 AM
7 Minutes
Metro Toronto Convention Center
Room: Room 716
Description
Objectives: Congenital heart defects with left ventricular outflow tract obstruction are often accompanied by subendocardial accumulation of collagen and elastic fibers referred to as endocardial fibroelastosis (EFE). Resection of EFE is performed to improve left ventricular functional recovery, but EFE recurrence is often observed, occasionally hampering LV recruitment endeavors. We previously identified longstanding left ventricular outflow tract obstruction (LVOTO) as risk factor for faster EFE recurrence after primary resection. The goal of this study was to determine whether early surgical elimination of LVOTO at the time of primary EFE resection affects the risk of EFE recurrence.
Methods: A retrospective chart review included all patients with congenital aortic valve (AoV) stenosis/LVOTO (n=93) who underwent primary resection of EFE at two centers between 01/2010 and 12/2021. Patients with LV long axis z-score >-2, mitral valve area z-score >-2, less than severe mitral stenosis and at least moderate LV function were included (58/93). Children with surgical treatment of LVOTO by a Ross (+/-Konno) procedure (22/58) were compared to patients with resembling anatomy who underwent other kinds of LVOT surgeries, mostly aortic valvuloplasty (36/58). EFE recurrence was defined as increased thickness or progressive appearance in previously resected or new areas determined by echocardiography and/or MRI during follow up. The primary outcome measure was time to EFE recurrence.
Results: Freedom from EFE recurrence was significantly higher in Ross (+/- Konno) patients ( P=0.003), with a median time to recurrence in the non-Ross patients of 2.67 years (Figure 1). Freedom from reintervention on the LVOT/AoV was higher in the Ross (+/-Konno) group, 1/22 (4.5%) vs 15/36 (41.7%) (log-rank test = 8.46, P = 0.004). 18/22 (81.5%) of patients after Ross were alive with a biventricular circulation at most recent follow up.
Conclusions: Our data indicate that LVOTO is a modifiable risk factor for EFE recurrence, and early surgical relief by enlargement of the LVOT has the potential to reduce the risk of LV EFE recurrence. This patient cohort also showed a reduced rate of reoperations on the LVOT and AoV. The correct patient selection and timing for surgical LVOTO treatment in the larger context of LV recruitment efforts has yet to be identified through prospective trials.
Authors
Gregor Gierlinger (1), Daniel Diaz-Gil (1), Andreas Tulzer (2), Roland Mair (3), Eva Sames-Dolzer (3), Steven Staffa (4), David Zurakowski (4), Michaela Kreuzer (3), Sitaram Emani (1), Pedro del Nido (1), Rudolf Mair (3), Ingeborg Friehs (1)
Institutions
(1) Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, (2) Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, JKU, Linz, Austria, (3) Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU, Linz, Austria, (4) Department of Surgery, Boston Children's Hospital, Boston, MA, USA
Methods: A retrospective chart review included all patients with congenital aortic valve (AoV) stenosis/LVOTO (n=93) who underwent primary resection of EFE at two centers between 01/2010 and 12/2021. Patients with LV long axis z-score >-2, mitral valve area z-score >-2, less than severe mitral stenosis and at least moderate LV function were included (58/93). Children with surgical treatment of LVOTO by a Ross (+/-Konno) procedure (22/58) were compared to patients with resembling anatomy who underwent other kinds of LVOT surgeries, mostly aortic valvuloplasty (36/58). EFE recurrence was defined as increased thickness or progressive appearance in previously resected or new areas determined by echocardiography and/or MRI during follow up. The primary outcome measure was time to EFE recurrence.
Results: Freedom from EFE recurrence was significantly higher in Ross (+/- Konno) patients ( P=0.003), with a median time to recurrence in the non-Ross patients of 2.67 years (Figure 1). Freedom from reintervention on the LVOT/AoV was higher in the Ross (+/-Konno) group, 1/22 (4.5%) vs 15/36 (41.7%) (log-rank test = 8.46, P = 0.004). 18/22 (81.5%) of patients after Ross were alive with a biventricular circulation at most recent follow up.
Conclusions: Our data indicate that LVOTO is a modifiable risk factor for EFE recurrence, and early surgical relief by enlargement of the LVOT has the potential to reduce the risk of LV EFE recurrence. This patient cohort also showed a reduced rate of reoperations on the LVOT and AoV. The correct patient selection and timing for surgical LVOTO treatment in the larger context of LV recruitment efforts has yet to be identified through prospective trials.
Authors
Gregor Gierlinger (1), Daniel Diaz-Gil (1), Andreas Tulzer (2), Roland Mair (3), Eva Sames-Dolzer (3), Steven Staffa (4), David Zurakowski (4), Michaela Kreuzer (3), Sitaram Emani (1), Pedro del Nido (1), Rudolf Mair (3), Ingeborg Friehs (1)
Institutions
(1) Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, (2) Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, JKU, Linz, Austria, (3) Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU, Linz, Austria, (4) Department of Surgery, Boston Children's Hospital, Boston, MA, USA
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.