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Shone᾽s complex in pediatric age group: clinical characteristics, structural components, interventions, and outcomes
Shaimaa Rakha1, Marwa Said Ali2, Ahmad Abd El Aleem El Derie3
1Pediatric Cardiology Unit, Department of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, Egypt. drshimaarhaka@mans.edu.eg.
Insights
Shone's complex in children often presents as an incomplete subtype, commonly involving parachute mitral valve and aortic coarctation. Factors like initial myocardial contractility impact outcomes, necessitating long-term follow-up.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Shone's complex (SC) involves multiple left-sided heart obstructions.
- Limited data exists on pediatric SC, particularly factors influencing outcomes.
Purpose of the Study:
- To investigate clinical, structural, interventional, and outcome aspects of pediatric Shone's complex.
- To identify factors impacting morbidity and mortality in children with SC.
Main Methods:
- Retrospective analysis of 25 pediatric SC cases from a single tertiary center.
- Data collection included demographics, clinical presentation, echocardiography, MSCT, and interventions.
- Outcomes focused on morbidity and mortality, with statistical analysis of influencing factors.
Main Results:
- Incomplete SC was diagnosed in all patients, with parachute mitral valve (86%) and aortic coarctation (72%) being most common.
- Surgery was performed in 76% of cases; mortality rate was 12%.
- Lower age, weight, and weight Z-score at follow-up, and initial suppressed myocardial contractility were associated with mortality.
Conclusions:
- Pediatric SC predominantly presents as the incomplete subtype, with parachute mitral valve and aortic coarctation as key lesions.
- Echocardiography and MSCT enable comprehensive non-invasive diagnosis and intervention guidance.
- Families must be informed about potential recurrence, progression, and the need for prolonged follow-up.
Background:
Shone's complex (SC) comprises multi-level left-sided obstructive cardiac lesions. Limited research has addressed SC in the pediatric age, especially the factors impacting outcomes. Therefore, the current work aimed to study clinical, structural, interventional aspects, and outcomes of SC cohort in childhood.
Methods:
A retrospective analysis of SC cases from a single tertiary center was conducted. Demographics and clinical data were collected. Echocardiographic data, other imaging modalities, and required interventions (surgical or transcatheter) were determined. Outcomes regarding morbidity and mortality were collected.
Results:
Twenty five cases were included;17 (68%) were males with a median age of one month (0.2-4.8) at presentation and 33 (5-60) months at the last follow-up. All patients were diagnosed with incomplete SC, with the commonest lesions: Parachute mitral in 86% and aortic coarctation (COA) in 72%. Cardiac multi-slice CT (MSCT) was indicated in 16 (64%), and transesophageal echocardiography in 2 (8%). No intervention was performed in 5 (20%) cases, while interventional catheterization was performed in 4 (16%) and surgery was performed in 19 (76%). The mortality rate in the cohort was 3 (12%), with age of the patient, weight, and weight Z-score at the last follow-up significantly lower in deceased cases. The proportion of cases with suppressed myocardial contractility at the initial presentation was statistically significant in the deceased group (p = 0.03). However, anthropometrics and demographics at presentation, SC components, and interventions did not differ significantly between the two groups.
Conclusion:
Shone's complex is primarily presented in the atypical incomplete subtype. In pediatric age, parachute mitral and COA are the most common lesions. Echocardiography with MSCT could achieve a non-invasive, comprehensive diagnosis and guide the required intervention. The family should be informed of potential recurrence, progression, or reintervention for SC lesions, emphasizing the need for prolonged follow-up.
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