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Scimitar syndrome in infancy
Y A Gao1, P E Burrows, L N Benson
1Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Infants with scimitar syndrome often have severe symptoms due to pulmonary hypertension, anomalous systemic arteries, and heart defects. Early treatment of these anomalies is crucial for improving outcomes in affected infants.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Medical Imaging and Diagnostics
Background:
- Scimitar syndrome diagnosis in infants typically presents with severe symptoms and poor prognosis, unlike incidental findings in older individuals.
- Infantile scimitar syndrome is characterized by significant clinical challenges and adverse outcomes.
Purpose of the Study:
- To identify the key anatomic and physiological factors contributing to severe symptoms and poor prognosis in infants with scimitar syndrome.
- To elucidate the underlying causes of severe presentation in infantile scimitar syndrome.
Main Methods:
- Review of clinical, catheterization, and imaging data from 13 infants with scimitar syndrome within the first six months of life.
- Emphasis on pulmonary artery pressure, cardiovascular anatomy, therapeutic interventions, and patient outcomes.
Main Results:
- Pulmonary hypertension was present in 12 of 13 infants; 6 patients died despite treatment.
- Associated cardiac malformations (11/13) and systemic arterial collateral channels (9/13) were common. Left-sided heart anomalies were associated with higher mortality.
- Interventions included ligation/embolization of systemic arteries (7 patients), angioplasty/stenting for pulmonary vein stenosis (2 patients), and surgical repair of associated anomalies.
Conclusions:
- Severe symptoms and pulmonary hypertension in infantile scimitar syndrome stem from multiple factors including anomalous systemic arterial supply, pulmonary vein stenosis, and associated cardiovascular anomalies.
- The feasibility of early treatment for these complex anomalies dictates the ultimate prognosis for affected infants.
Objectives:
The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome.
Background:
Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis.
Methods:
The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome.
Results:
Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions.
Conclusions:
The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.