Related Experiment Video
Updated: Jun 4, 2026

Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure
Published on: February 8, 2022
Atypical Shone's complex diagnosed by echocardiography
Xiao-jing Ma1, Guo-ying Huang, Xue-cun Liang
1Children's Hospital of Fudan University, Shanghai, China, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China.
Insights
Echocardiography effectively diagnoses atypical Shone's complex, a severe congenital heart defect involving multiple left-sided obstructions. This study highlights echocardiography's crucial role in identifying the number, position, and severity of these cardiovascular lesions.
Area of Science:
- Cardiovascular Medicine
- Pediatric Cardiology
- Diagnostic Imaging
Background:
- Shone's complex is a rare, severe congenital condition characterized by multiple left-sided cardiovascular obstructive lesions.
- Accurate diagnosis is crucial for timely intervention and management of this complex pediatric cardiac anomaly.
Purpose of the Study:
- To present the experience and diagnostic utility of echocardiography in identifying atypical Shone's complex.
- To detail the spectrum of left-sided obstructive lesions and secondary cardiovascular changes associated with atypical Shone's complex.
Main Methods:
- Retrospective analysis of 38 consecutive pediatric patients diagnosed with atypical Shone's complex using echocardiography.
- Echocardiographic findings were correlated with cardioangiography and/or surgical data for confirmation.
- Detailed assessment of the number, location, morphology, and severity of obstructive lesions.
Main Results:
- Echocardiography successfully diagnosed atypical Shone's complex in all 38 patients.
- Commonly observed lesions included coarctation of the aorta (26 cases), aortic stenosis (25 cases), and mitral stenosis (24 cases).
- Secondary changes such as left atrial dilation, left ventricular hypertrophy, and pulmonary hypertension were frequently noted.
Conclusions:
- Echocardiography is a vital tool for the comprehensive diagnosis of atypical Shone's complex.
- The modality allows for detailed evaluation of multiple obstructive lesions and associated cardiac remodeling, guiding clinical management.
Abstract:
Shone's complex is a rare and severe type of congenital left-sided obstructive lesion of multiple cardiovascular levels. This report aims to present the authors' experience diagnosing atypical Shone's complex using echocardiography. Atypical Shone's complex was diagnosed for 38 consecutive patients (18 boys and 20 girls, ages 1 day to 15 years) using echocardiography. All the diagnoses were confirmed by cardioangiography, surgery, or both. Among the congenital left-sided obstructive lesions, four levels of obstruction coexisted in 3 cases (7.9%), three levels in 10 cases (26.3%), and two levels in 25 cases (65.8%). Supra-annular mitral stenosis caused by a supravalvular mitral ring was diagnosed in 1 case. Mitral valvular stenosis was diagnosed in 24 cases including a parachute mitral valve (4 cases), a double-orifice mitral valve (1 case), and partly fused thickened mitral valvular leaflets (19 cases). Subaortic stenosis was diagnosed in 10 cases including circumferential membranous subaortic stenosis (1 case), tunnel-type subaortic narrowing (2 cases), and asymmetric obstructive fibrous ridge below the aortic valve (7 cases). Aortic stenosis was diagnosed in 25 cases including the bicuspid aortic valve (12 cases), the hypoplasia aortic annulus (3 cases), and partly fused or thickened tri-leaflet aortic valves (10 cases). Supra-aortic stenosis was diagnosed in 6 cases including the localized type (4 cases) and the diffused type (2 cases). Coarctation of the aorta was diagnosed in 26 cases. Of these, focal coarctation was described in 15 cases, long segment coarctation in 7 cases, and dysplastic isthmus and/or descending aorta in 4 cases. Secondary changes in Shone's complex manifested as left atrial dilation (26 cases), left ventricular hypertrophy (26 cases), and pulmonary hypertension (19 cases). Echocardiography plays an important role in the diagnosis of atypical Shone's complex, in comprehensive evaluation of the number, position, morphology, and severity of left-sided obstructions.
Related Concept Videos
Imaging Studies for Cardiovascular System II:Types of Echocardiography
Types of Echocardiography
Transthoracic Echocardiography (TTE)
TTE is the most common type of echocardiogram which involves placing a transducer on the patient's chest, emitting sound waves to create heart images. TTE is invaluable for evaluating the heart's size, structure, and motion, making it particularly useful for diagnosing...
Mitral Stenosis II: Clinical features and Diagnostic Tests
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Acute Coronary Syndrome III: Diagnostic Studies
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
Heart Failure IV: Classification and Diagnostic Evaluation

