Total Thoracic Aortic Aneurysm Replacement In a Child. A Simple Strategy.

P0354 

Case Video Submission 

NESTOR SANDOVAL (1), Pablo Sandoval (2), TOMAS Chalela (3), Jaime Camacho (4), Carlos Villa (5), Juan Umaña (6), Carlos Obando (7)

(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

NESTOR SANDOVAL    -  Contact Me
FUNDACION CARDIOINFANTIL

Pablo Sandoval    -  Contact Me
El Bosque University

TOMAS Chalela    -  Contact Me
N/A

Jaime Camacho    -  Contact Me
Fundación CardioInfantil

Carlos Villa    -  Contact Me
Fundacion Cardioinfantil

*Juan P. Umaña    -  Contact Me
La Cardio

Carlos Obando    -  Contact Me
N/A

Pablo Sandoval    -  Contact Me
El Bosque University

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.

Aortic Arch