P354. Total Thoracic Aortic Aneurysm Replacement In a Child. A Simple Strategy.
Presented During: POD Abstracts (Available for viewing in the exhibit hall for the duration of the meeting)
El Bosque University
Bogotá D.C., NA
Colombia - Contact Me
Im a 4th year General Surgery Resident from El Bosque University in Bogota, Colombia.
Im really interested in cardiac and congenital heart surgery and access to adequate cardiac surgery in undeveloped regions.
Thursday, April 25, 2024: 5:38 PM - 7:00 PM
Sheraton Times Square
Room: Central Park
Description
Objective:
To demonstrate a surgical technique employed in a child with a very unusual severe ascending, transverse arch and descending aortic aneurysm with a rare genetic disease.
Case Video Summary:
A 19-month child with a family history of aortic dilatation was referred to our center due to a severe ascending, transverse arch and descending aortic aneurysm. The dimensions showed a dilatation up to 45 mm in the aortic arch and preoperative echocardiogram showed aortic insufficiency secondary to the dilatation of the sinotubular junction.
A median sternotomy, cardiopulmonary bypass with arterial cannulation in the innominate artery, the descending aorta, and venous cannulation in the right atrium was initiated. Then, moderate hypothermia and selective antegrade cerebral perfusion at a rate of 50 cc/kg/minute was established. Near infrared spectroscopy (NIRS), and proximal and distal pressure monitoring was set.
The ascending aorta, transverse arch, and descending aorta was replaced up to where the dilatation was evidenced with a Dacron graft (22 mm) with independent reimplantation of the neck vessels.
The patient was weaned from cardiopulmonary bypass without complications.
Aortic cross clamp time was 77 minutes, cardiopulmonary bypass time was 130 minutes and selective antegrade cerebral perfusion was 24 minutes.
The patient was extubated 12 hours after the operation but due to vocal cord disfunction a traqueostomy was performed. The patient had an adequate evolution and was discharged home at day 23 with an angiotensin receptor antagonist. Genetic evaluation was made and the gene EFEMP2 was identified related to Autosomal Recessive Cutis Laxa Type 1B, a rare genetic disease that has various clinical manifestations including aortic aneurysms.
1 year follow up showed an adequate evolution, with no repercussions after surgery.
Conclusions:
Simple techniques with neck vessel reimplantation can be the solution for a very unusual case of thoracic aneurysm in a child. A thorough genetic evaluation should be done in these patients with infrequent presentations to identify further possible complications and establish a medical management for the future.
Authors
NESTOR SANDOVAL (1), Pablo Sandoval (2), TOMAS Chalela (3), Jaime Camacho (4), Carlos Villa (5), Juan Umaña (6), Carlos Obando (7)
Institutions
(1) FUNDACION CARDIOINFANTIL, BOGOTA, DC, (2) El Bosque University, Bogota, NA, (3) N/A, bogota, Colombia, (4) Fundación CardioInfantil, Bogota, NA, (5) Fundacion Cardioinfantil, Bogota, Colombia, (6) La Cardio, Bogota, (7) N/A, Bogota, Colombia
To demonstrate a surgical technique employed in a child with a very unusual severe ascending, transverse arch and descending aortic aneurysm with a rare genetic disease.
Case Video Summary:
A 19-month child with a family history of aortic dilatation was referred to our center due to a severe ascending, transverse arch and descending aortic aneurysm. The dimensions showed a dilatation up to 45 mm in the aortic arch and preoperative echocardiogram showed aortic insufficiency secondary to the dilatation of the sinotubular junction.
A median sternotomy, cardiopulmonary bypass with arterial cannulation in the innominate artery, the descending aorta, and venous cannulation in the right atrium was initiated. Then, moderate hypothermia and selective antegrade cerebral perfusion at a rate of 50 cc/kg/minute was established. Near infrared spectroscopy (NIRS), and proximal and distal pressure monitoring was set.
The ascending aorta, transverse arch, and descending aorta was replaced up to where the dilatation was evidenced with a Dacron graft (22 mm) with independent reimplantation of the neck vessels.
The patient was weaned from cardiopulmonary bypass without complications.
Aortic cross clamp time was 77 minutes, cardiopulmonary bypass time was 130 minutes and selective antegrade cerebral perfusion was 24 minutes.
The patient was extubated 12 hours after the operation but due to vocal cord disfunction a traqueostomy was performed. The patient had an adequate evolution and was discharged home at day 23 with an angiotensin receptor antagonist. Genetic evaluation was made and the gene EFEMP2 was identified related to Autosomal Recessive Cutis Laxa Type 1B, a rare genetic disease that has various clinical manifestations including aortic aneurysms.
1 year follow up showed an adequate evolution, with no repercussions after surgery.
Conclusions:
Simple techniques with neck vessel reimplantation can be the solution for a very unusual case of thoracic aneurysm in a child. A thorough genetic evaluation should be done in these patients with infrequent presentations to identify further possible complications and establish a medical management for the future.
Authors
NESTOR SANDOVAL (1), Pablo Sandoval (2), TOMAS Chalela (3), Jaime Camacho (4), Carlos Villa (5), Juan Umaña (6), Carlos Obando (7)
Institutions
(1) FUNDACION CARDIOINFANTIL, BOGOTA, DC, (2) El Bosque University, Bogota, NA, (3) N/A, bogota, Colombia, (4) Fundación CardioInfantil, Bogota, NA, (5) Fundacion Cardioinfantil, Bogota, Colombia, (6) La Cardio, Bogota, (7) N/A, Bogota, Colombia
Presentation Duration
PODS will be on display in the exhibit hall for the duration of the meeting during exhibit hall hours. PODS will also be available for viewing on the meeting website. There is no formal presentation associated with your POD, but we encourage you to visit the PODS area during breaks to connect with those viewing.