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A clinical perspective on surgical and diagnostic strategies for neonatal partial shone complex: Insights from a case
Ayham Qatza1, Moumina Baroudi2, Abdullah Dukhan1
1Faculty of Medicine, Hama University, Hama, Syria.
Insights
Shone complex, a rare congenital heart defect, requires early diagnosis and staged surgical intervention for optimal outcomes. This case highlights successful management of partial Shone complex in a neonate.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Shone complex (SC) is a rare, complex congenital heart disease (CHD) involving multiple left-sided obstructive lesions.
- It accounts for a small percentage of all CHD cases, presenting significant clinical challenges.
Observation:
- A neonate presented with severe respiratory distress, tachycardia, and hypoxia.
- Echocardiography revealed critical left-sided heart obstructions including supravalvular mitral stenosis and coarctation of the aorta (CoA).
Findings:
- The neonate underwent successful surgical repair of severe coarctation of the aorta (CoA) at five months.
- One-year follow-up showed clinical stability with no significant pressure gradient changes, indicating effective staged management.
Implications:
- Early diagnosis and timely, staged surgical intervention are crucial for managing Shone complex.
- Point-of-care ultrasound plays a vital role in the diagnosis and management of complex CHD like SC.
Introduction And Clinical Importance:
Shone complex (SC) is a rare multilevel congenital heart disease (CHD) characterized by four left-sided heart obstructive lesions: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta (CoA), accounting for 0.6-0.7 % of CHD cases.
Case Presentation:
A 4-week-old male neonate presented with severe respiratory distress, tachycardia (150 beats/min), tachypnea (40/min), and hypoxia (80 % saturation). Blood pressure was 90/55 mmHg in the upper arms; lower extremity measurements were challenging. ECG showed a heart rate of 150 beats/min, normal sinus rhythm, left axis deviation, and left ventricular (LV) hypertrophy. Transthoracic echocardiography revealed mild concentric LV hypertrophy and reduced ejection fraction (45 %). A supramitral ring led to severe supravalvular mitral stenosis, and a bicuspid aortic valve caused moderate aortic stenosis. Suprasternal views confirmed severe CoA distal to the left subclavian artery. The patient underwent successful CoA repair at five months, with ongoing surveillance for other defects. One year later, he remained stable with no significant pressure gradient changes.
Clinical Discussion:
SC presents significant clinical challenge due to its associated congenital anomalies. Early echocardiographic diagnosis and timely surgical intervention are essential for optimizing patient outcomes, given the variability in severity and the potential for complications. Multidisciplinary management is crucial for addressing the complexities of this condition.
Conclusion:
This case illustrates effective staged surgical management of partial SC, emphasizing early diagnosis and the utility of point-of-care ultrasound.
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