Related Experiment Videos
"Infantile" form of the scimitar syndrome with pulmonary hypertension
C Dupuis1, L A Charaf, G M Brevière
1Service de Cardiologie Infantile, Hôpital Cardiologique, Lille, France.
Insights
Scimitar syndrome in infants often causes pulmonary hypertension and cardiac failure. Ligation of abnormal arterial vessels supplying the right lung is the most effective treatment, improving survival rates.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Neonatology
Background:
- Scimitar syndrome is a rare congenital anomaly associated with significant morbidity and mortality in infants.
- Pulmonary hypertension and cardiac failure are common, life-threatening complications in affected newborns and infants.
- Vascular sequestration and pulmonary venous stenosis are key underlying pathologies contributing to pulmonary hypertension in this condition.
Purpose of the Study:
- To investigate the clinical presentation and outcomes of infants with scimitar syndrome and pulmonary hypertension.
- To evaluate the efficacy of different treatment modalities, including medical management and surgical interventions.
- To identify the optimal surgical approach for improving survival and clinical status in this patient population.
Main Methods:
- Retrospective analysis of 25 newborns and infants (<1 year) diagnosed with scimitar syndrome and pulmonary hypertension across 12 European pediatric centers.
- Review of patient data including clinical presentation, diagnostic findings, treatment strategies (medical and surgical), and outcomes.
- Categorization of pulmonary hypertension causes into vascular sequestration (23 cases) and pulmonary venous stenosis (2 cases).
Main Results:
- All 25 patients presented with cardiac failure and severe respiratory insufficiency.
- Medical treatment for 10 patients resulted in a 30% survival rate, with 1 patient experiencing residual pulmonary hypertension.
- Surgical intervention in 15 patients yielded a 40% survival rate; ligation of abnormal arterial vessels showed the best outcome with 5 of 6 patients surviving in good condition.
Conclusions:
- Scimitar syndrome in infants frequently leads to severe pulmonary hypertension and cardiac compromise.
- Ligation of abnormal arterial vessels supplying the sequestered lung segment is the most effective treatment, offering the best survival and clinical outcomes.
- Early diagnosis and appropriate surgical management, particularly ligation of abnormal arteries, are crucial for improving the prognosis of infants with scimitar syndrome and pulmonary hypertension.
Abstract:
Twenty-five newborns and infants aged < 1 year with the scimitar syndrome and pulmonary hypertension from 12 European pediatric centers were examined. Cardiac failure and severe respiratory insufficiency were always present. In 23 cases, pulmonary hypertension was due to a large shunt between abnormal arteries originating from the abdominal aorta and supplying the lower part of the right lung (vascular sequestration). In the last 2 cases, pulmonary hypertension was secondary to stenosis of the common trunk of the right pulmonary veins. Three of 10 patients who received only medical treatment survived; 2 are doing well, with pulmonary arterial pressures that have returned to normal, and the other had severe residual pulmonary hypertension. Six of 15 patients who underwent surgery survived. There were 5 ligations of a patent ductus arteriosus with 5 deaths, 3 pulmonary resections with 2 deaths, 1 dilation of a tight stenosis of the common trunk of the right pulmonary veins with 1 death, and 6 ligations of the abnormal arterial vessels with 5 surviving patients who are in good condition. Ligation of the abnormal arterial vessels appears to be the best type of treatment.