A 57-year-old woman suffering from recurrent pneumonia and considered to be high risk for surgical correction of an atrial septal defect (ASD) associated with a right lung sequestration vascularized by arteries coming from the abdominal aorta and an abnormal venous drainage into the inferior vena cava (Scimitar syndrome). Therefore stepwise transcatheter approach was offered as an alternative treatment. We performed ASD closure and embolization of the anomalous systemic arterial supply using Amplatzer Vascular Plugs II. The abnormal venous drainage was conservatively treated (Q(p) /Q(s) : 1.1/1 after ASD closure and anomalous arterial supply embolization). At 24-month follow-up, no recurrent pneumonia was reported, functional class improved and right cardiac cavities normalized.
[Show abstract][Hide abstract] ABSTRACT: This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Of 8,771 patients, 5 (0.057%) with scimitar syndrome were identified and constituted the study population. Follow-up ranged from 1 to 16 years (median: 10 years). Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Two patients presented with respiratory distress soon after birth and required early pneumonectomy in one case and coil embolization of the abnormal feeding arteries to the right lower lung followed by surgical rerouting of the abnormal pulmonary vein and repair of the atrial septal defect in the other case. The former was supported by ventilator therapy for 3 years after pneumonectomy, but was finally weaned from the ventilator. Among the other three, two had repeated pneumonia that resolved after rerouting of the abnormal right pulmonary vein and cardiac repair. The asymptomatic child did not receive any intervention. In spite of the abnormal orientation of the airways, none of the four patients with detailed computed tomography imaging showed any significant compression of the airways. All five patients were doing well as of the last follow-up. In conclusion, scimitar syndrome is a very rare disease in this Asian country and the varied symptoms, such as tachypnea and repeated infection, could be improved after interventions.
European Journal of Pediatrics 03/2008; 167(2):155-60. DOI:10.1007/s00431-007-0441-z · 1.89 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiologic centers. The clinical, radiographic and hemodynamic findings are described. The scimitar syndrome is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. Additional characteristics of this syndrome such as hypoplasia and abnormalities of the vascular supply to the right lung, dextrocardia and abnormalities of the bronchial segmentation are common; bronchiectases are rare. The left to right shunt was less than 50% in 100 of the 122 patients. The pulmonary arterial pressures were normal in 94 patients and slightly elevated in 28. A follow-up study of these patients showed that, without surgical correction, they lead a normal life. An awareness of this syndrome may avoid unnecessary invasive diagnostic procedures and surgical treatment for most patients.
The American Journal of Cardiology 09/1992; 70(4):502-7. DOI:10.1016/0002-9149(92)91198-D · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We describe a novel percutaneous approach for correction of partially anomalous pulmonary venous connection. We describe two cases of dual pulmonary venous drainage where embolization of the anomalous pulmonary venous connection was successful.
Catheterization and Cardiovascular Diagnosis 08/1998; 44(3):330-5. DOI:10.1002/(SICI)1097-0304(199807)44:33.0.CO;2-O
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