P207. Management Of Interrupted Aortic Arch: 9 Years Of Experience at a Single Pediatric Center
Presented During: POD Abstracts (Available for viewing in the exhibit hall for the duration of the meeting)
Hospital de Pediatria SAMIC Juan P Garrahan
Ciudad autónoma de Buenos Aires, caba
Argentina - Contact Me
María Laura Zenobi, 32 years old, is a medical professional from Buenos Aires, Argentina. She graduated in Medicine at the University of Buenos Aires and completed her residency in Paediatric Cardiovascular Surgery and Heart Transplantation at the National Children's Hospital "Prof. Dr. Juan P. Garrahan" in Buenos Aires. In 2022/2023 she served as Chief of Residents, demonstrating her leadership qualities in the field of medicine. She is currently working as a Junior staff physician, contributing her skills in healthcare and expanding her expertise in the surgical field.
Thursday, April 25, 2024: 5:38 PM - 7:00 PM
Sheraton Times Square
Room: Central Park
Description
Objective:Summarize our center's 9-year surgical experience managing neonatal patients with Interrupted Aortic Arch (IAA)
Methods: We conducted a descriptive cross-sectional observational retrospective study, analyzing our tertiary care center's database of patients operated on for IAA between 2014 and 2023.Those with major congenital heart defects (CHD) other than the usual association with patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), Ventricular Septal Defect (VSD) and aberrant right subclavian artery, were excluded.
Initial imaging used Doppler echocardiography for detailed assessment.
Results: Medical charts of 48 patients diagnosed with IAA were reviewed, 8 were excluded due to other associated major defects, leaving a total of 40 patients.
Most patients required surgery within the first weeks of life. The overall average age at the time of surgery was 20.3 days, with a median of 14 days [6 - 88]. There was a slight female predominance, being 52.6% of the sample (22/40). The average weight at the time of surgery was 3.15 kg [2 - 4.13 kg].
A strong association between IAA and 22q11 microdeletion, was confirmed in 62.3% of the sample (25/40).
Regarding interruption types, 79% were type B IAA and 21% type A. No type C cases were found.
Biventricular Repair was achieved in a single stage by end-to-end anastomosis with homograft patch enlargement in 85% of cases. In only one patient, due to prematurity and low weight, an hybrid procedure of pulmonary artery branch banding and ductal stenting was chosen, Delaying correction.
ninety-eight percent of patients had an associated VSD (n: 39), and it was successfully closed during the same surgical procedure in 89% of them.
Damus-Kaye-Stansel (DKS) surgery was the initial approach in 12.5% (5/40), and 60% of them later achieved biventricular correction in a second stage. Among those with complete repair (n: 34), 11% required a second surgery for LVOTO relief in the following 3 years
The mean aortic annulus size in the re-intervened group was 4.68 mm , with an average Z score of -3.6. The Sinotubular junction (STJ) was measured on average at 4.85 mm, with a mean Z score of -3.31. Measurements in the group of corrected patients without reintervention requirements showed an average aortic annulus of 5.05 mm with a Z score of -3.12. The mean STJ measurement was 5.38 mm with a -2.5 mean Z score.
There were no significant differences in the length of hospitalization between both groups after the first surgery [57.4 days for reintervention patients vs.56.4 days for non-reintervention].
The overall mortality was 14% among those who underwent corrective surgery in neonatal period (5/40), with 3 of these deaths associated with infectious complications during hospitalization.
Within the initial DKS-type palliation group, there was one late death related to complications during the Yasui procedure later on.
Conclusions: Interrupted aortic arch is a severe and low-incidence CHD whose management has significantly improved over the past 40 years.
In the last 9 years at our center, 85% of the patients achieved successful biventricular repair in a single stage and among those who underwent initial DKS-type palliation or Pulmonary artery banding (remaining 15%), 66% achieved biventricular repair in a second stage.
However, close post-surgical follow-up remains crucial considering the risk of progressive LVOTO development in the medium- and long-term evolution
Authors
Maria Zenobi (1), Julia Blando (2), Agustina San Pedro (3), Gustavo Sivori (4), Ignacio Berra (5), Pablo Garcia Delucis (6)
Institutions
(1) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, ciudad autonoma de buenos aires, caba, (2) Hospital Nacional de Pediatría J.P. Garrahan, Ciudad Autónoma de Buenos, NA, (3) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Ciudad autónoma de Buenos Aires, Buenos Aires, (4) N/A, N/A, (5) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Morón, Buenos Aires, (6) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Buenos Aires
Methods: We conducted a descriptive cross-sectional observational retrospective study, analyzing our tertiary care center's database of patients operated on for IAA between 2014 and 2023.Those with major congenital heart defects (CHD) other than the usual association with patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), Ventricular Septal Defect (VSD) and aberrant right subclavian artery, were excluded.
Initial imaging used Doppler echocardiography for detailed assessment.
Results: Medical charts of 48 patients diagnosed with IAA were reviewed, 8 were excluded due to other associated major defects, leaving a total of 40 patients.
Most patients required surgery within the first weeks of life. The overall average age at the time of surgery was 20.3 days, with a median of 14 days [6 - 88]. There was a slight female predominance, being 52.6% of the sample (22/40). The average weight at the time of surgery was 3.15 kg [2 - 4.13 kg].
A strong association between IAA and 22q11 microdeletion, was confirmed in 62.3% of the sample (25/40).
Regarding interruption types, 79% were type B IAA and 21% type A. No type C cases were found.
Biventricular Repair was achieved in a single stage by end-to-end anastomosis with homograft patch enlargement in 85% of cases. In only one patient, due to prematurity and low weight, an hybrid procedure of pulmonary artery branch banding and ductal stenting was chosen, Delaying correction.
ninety-eight percent of patients had an associated VSD (n: 39), and it was successfully closed during the same surgical procedure in 89% of them.
Damus-Kaye-Stansel (DKS) surgery was the initial approach in 12.5% (5/40), and 60% of them later achieved biventricular correction in a second stage. Among those with complete repair (n: 34), 11% required a second surgery for LVOTO relief in the following 3 years
The mean aortic annulus size in the re-intervened group was 4.68 mm , with an average Z score of -3.6. The Sinotubular junction (STJ) was measured on average at 4.85 mm, with a mean Z score of -3.31. Measurements in the group of corrected patients without reintervention requirements showed an average aortic annulus of 5.05 mm with a Z score of -3.12. The mean STJ measurement was 5.38 mm with a -2.5 mean Z score.
There were no significant differences in the length of hospitalization between both groups after the first surgery [57.4 days for reintervention patients vs.56.4 days for non-reintervention].
The overall mortality was 14% among those who underwent corrective surgery in neonatal period (5/40), with 3 of these deaths associated with infectious complications during hospitalization.
Within the initial DKS-type palliation group, there was one late death related to complications during the Yasui procedure later on.
Conclusions: Interrupted aortic arch is a severe and low-incidence CHD whose management has significantly improved over the past 40 years.
In the last 9 years at our center, 85% of the patients achieved successful biventricular repair in a single stage and among those who underwent initial DKS-type palliation or Pulmonary artery banding (remaining 15%), 66% achieved biventricular repair in a second stage.
However, close post-surgical follow-up remains crucial considering the risk of progressive LVOTO development in the medium- and long-term evolution
Authors
Maria Zenobi (1), Julia Blando (2), Agustina San Pedro (3), Gustavo Sivori (4), Ignacio Berra (5), Pablo Garcia Delucis (6)
Institutions
(1) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, ciudad autonoma de buenos aires, caba, (2) Hospital Nacional de Pediatría J.P. Garrahan, Ciudad Autónoma de Buenos, NA, (3) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Ciudad autónoma de Buenos Aires, Buenos Aires, (4) N/A, N/A, (5) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Morón, Buenos Aires, (6) Hospital nacional de pediatria J.P.Garrahan, Buenos Aires, Argentina, Buenos Aires
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