"Infantile" form of the scimitar syndrome with pulmonary hypertension.

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.

[Surgical treatment of the scimitar syndrome in children, adolescents and adults. A cooperative study of 37 cases]. / Le traitement chirurgical du syndrome du cimeterre chez l'enfant, l'adolescent et l'adulte. Etude coopérative de 37 cas.

The authors report the results of a cooperative study of 37 operated cases of the scimitar syndrome. The diagnostic procedures, the operative indications, the surgical indications, the postoperative complications and the long-term outcome were reviewed. The results were disappointing irrespective of the surgical technique that was used (reimplantation of the right pulmonary veins in the left atrium, lobectomy, pneumonectomy). Only 12 patients had a satisfactory postoperative outcome and good long-term results; 21 patients had long-term chronic respiratory failure with a reduced exercise capacity and 4 patients died after surgery. Of the 21 patients with long-term sequellae, 17 had a thrombosis of the anastomosis between the right pulmonary veins and the left atrium; this occurred immediately after surgery.

The "adult" form of the scimitar syndrome.

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.