Left Ventricular Outflow Tract Obstruction as Modifiable Risk Factor for Recurrence of Endocardial Fibroelastosis
Presented During:
Sunday, April 28, 2024: 10:33AM - 10:40AM
Metro Toronto Convention Center
Posted Room Name:
Room 716
Abstract No:
139
Submission Type:
Abstract Submission
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
Submitting Author:
Gregor Gierlinger
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Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Co-Author(s):
Daniel Diaz-Gil
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Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Andreas Tulzer
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Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, JKU
Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, JKU
Roland Mair
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Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Eva Sames-Dolzer
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Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Steven Staffa
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Department of Surgery, Boston Children's Hospital
Department of Surgery, Boston Children's Hospital
Dr David Zurakowski
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Department of Surgery, Boston Children's Hospital
Department of Surgery, Boston Children's Hospital
Michaela Kreuzer
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Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
*Sitaram Emani
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Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
*Pedro del Nido
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Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Rudolf Mair
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Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU
Ingeborg Friehs
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Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School
Presenting Author:
Gregor Gierlinger
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Abstract:
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.
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.
CONGENTIAL:
Left ventricular outflow tract obstruction
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