Naslov
Obscure nature of subaortic membrane development in children

Autori
Knez, Nora ; Belina, Dražen

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Skup
Croatian Paediatric Surgery Conference 2022

Mjesto i datum
Pula, Hrvatska, 21-24.09.2022

Vrsta sudjelovanja
Poster

Vrsta recenzije
Podatak o recenziji nije dostupan

Ključne riječi
Subaortic membrane ; Congenital Cardiac surgery

Sažetak
Pediatric subvalvular aortic stenosis (SAS) is a rare congenital heart defect caused by membrane below the aortic valve, resulting in an obstruction of the left ventricular outflow tract (LVOT). The asymptomatic course contributes to serous sequelae devel- opment and unrecognized SAS can cause sudden death during fight or flight stress response. We present a case of an 11-year-old boy with SAS who presented with dyspnea on ex- ertion. His past medical history revealed systolic murmur present at birth. Ultrasound follow-ups showed progressive membrane growth since 8 years of age. LVOT velocity was 2.5m/s at rest and 4m/s during the stress test. The pressure gradient in LVOT was 60 mmHg at the age of 11. Transaortic surgical membrane resection along with the ven- tricular septal myectomy was indicated. Intraoperatively, a circular 6 mm membrane was found 12 mm below the normal aortic valve in LVOT and was taken to the pathology department for analysis. Pathohistological evaluation of the membrane showed fibro- muscular tissue. Postoperative LVOT velocity was 1.9m/s and the LVOT region was free from obstruction. SAS is a rare congenital disorder that remains a clinical challenge due to its insidious nature and risk for significant long-term morbidity before the onset of symptoms. It usually presents in the first decade of life, although persons of all ages are affected. The etiopathogenesis remains unclear. Genetic factors, hemodynamic abnormalities, and underlying LVOT morphology promote turbulence at the outflow tract increasing the fluid shear stress. Cronic flow disturbances might result in abnormal endothelial and muscle proliferation and fibromuscular membrane formation. Clinical follow-ups have an important role to prevent fatal complications. Mild systolic murmur since early childhood should raise suspicion for SAS. Surgical resection is the only effective treat- ment but postoperative recurrence is common, although membrane resection along with septal myectomy decreases the risk of recurrence

Izvorni jezik
Engleski

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