Lethal Fungal Aortitis In Surgically Corrected Supravalvular Aortic Stenosis In A Child With Williams Syndrome.
J Ayub Med Coll Abbottabad
; 35(2): 320-323, 2023.
Article
in En
| MEDLINE
| ID: mdl-37422830
ABSTRACT
Williams syndrome (WS), is a multisystem disorder occurring in 1 in 10,000 live births with supravalvular aortic stenosis (SVAS) being the most common cardiovascular manifestation. We present the case of a 2.5 years old male, a known case of WS who presented with cognitive delay, a history of right-sided stroke and left hemiplegia. Echocardiography revealed severe SVAS with a gradient of 105 mmHg. The diameter of the Sino tubular junction was 4 mm. Computerized tomography angiogram showed diffuse stenosis of ascending aorta with intraluminal thrombus. At surgery, the ascending aorta was augmented with autologous pericardial patches and end-to-end anastomosis of the proximal and distal aorta completed the reconstruction. The patient was discharged in a stable condition. He presented 6 weeks post-op with a pulsating pseudoaneurysm through the sternal wound. Emergency surgery with the removal of fungal vegetation and reconstruction of the ascending aorta was performed. He expired due to fungal sepsis a week later.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Aortitis
/
Williams Syndrome
/
Aortic Stenosis, Supravalvular
Limits:
Child
/
Child, preschool
/
Humans
/
Male
Language:
En
Journal:
J Ayub Med Coll Abbottabad
Journal subject:
MEDICINA
Year:
2023
Document type:
Article
Affiliation country:
Estados Unidos