Key result
Preoperative echocardiography misses an annulo-leaflet mitral ring in ~47% of Shone's complex cases.
Why the study?
Shone's complex is a rare syndrome with variable congenital left heart defects, and the causes of missed diagnosis and misdiagnosis by echocardiography need analysis.
Does preoperative echocardiography accurately diagnose the components of Shone's complex compared to intraoperative findings in pediatric patients?
Observational (n=66)
Does preoperative echocardiography accurately diagnose the components of Shone's complex compared to intraoperative findings in pediatric patients?
Echocardiography is effective for diagnosing Shone's complex, though it frequently misses the Annulo-Leaflet mitral ring, highlighting the need for improved understanding of the disease's complexity.
Preoperative echocardiography may miss annulo-leaflet mitral ring in Shone's complex; leaves open whether advanced imaging refines pediatric surgical planning.
BACKGROUND: Shone's complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients. AIM: To use echocardiography in the diagnosis of Shone's complex and analyze the causes of missed diagnosis and misdiagnosis. METHODS: This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14, 2008, to November 22, 2019. The patients were followed once a year at the outpatient clinic after surgery. RESULTS: Sixty-six patients were included. The patients were 2.7 (0.8-5.6) years of age, and 54.5% were male. Ten (15.2%) had a history of heart surgery. The most common heart defect was the Annulo-Leaflet mitral ring (ALMR) (50/66, 75.8%), followed by coarctation of the aorta (CoA) (43/66, 65.2%). The patients had a variety of combinations of defects. Only two (3.0%) patients had all four defects. None of the patients had a family history of congenital heart disease. The preoperative echocardiographic findings were examined against the intraoperative findings. Echocardiography missed an ALMR in 31 patients (47.0%), a parachute mitral valve (PMV) in one patient (1.5%), subaortic stenosis in one patient (1.5%), and CoA in two patients (3.0%). CONCLUSION: Echocardiography is an effective method to diagnose the Shone's complex. Due to this disease's complexity and interindividual variability, Improving the understanding of the disease can reduce misdiagnosis and missed diagnosis.
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Li et al. (2022) conducted an observational in Shone's complex (n=66). Echocardiography vs. Intraoperative findings was evaluated on Missed diagnosis of Annulo-Leaflet mitral ring (ALMR). Preoperative echocardiography missed an Annulo-Leaflet mitral ring in 47.0% of patients with Shone's complex when compared to intraoperative findings.
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