CONGENITAL HEART DISEASE
The 44Adultgv Form of the Scimitar
Syndrome
Claude Dupuis, MD, Louis A. C. Charaf, MD, Georges-Marie Brevi&re, MD, Pierre Abou, MD, Martine R6my-Jardin, MD, and Gunnar Helmius, MD
One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiiogic centers. The clinical, radiigraphic 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 lO years in the last 50 patients. Age, sex, heredity, clinical status, electrocardiography, plain chest films, cardiac catheterization and angiography were studied in all cases. Other examinations such as echccardiography, computerized tomography and spirometry with radioactive isotopes were performed in some centers. In a few cases, bronchoscopy and bronchography were also performed. As a matter of routine, medical and social history was obtained from all patients and the results of surgical treatment were studied. AUGUST
15, 1992
RESULTS Seventy-seven patients were women and 45 were men. The ages at diagnosis are listed in Table I. The parents of 2 of the patients were first cousins. These parents had 4 children of whom 2 were healthy and 2 had the scimitar syndrome. In another family, the father and his son both had the scimitar syndrome. Five children were born prematurely. One child was a twin. The other twin was normal. The patients were referred to hospitals for respiratory or cardiac problems probaby related to the scimitar syndrome: bouts of pneumonia (38 cases), slight dyspnea on exertion (23 cases), precordial systolic murmur (18 cases), paroxysmal tachycardia (1 case), deformation of the thorax with retraction of the right hemithorax (8 cases), and hemoptysis (7 cases). The remaining 27 patients were referred to centers for other symptoms unrelated to the scimitar syndrome. The
twins were both mentally retarded. One child had trisomia 21 and another had Turner’s syndrome. Other malformations were also present, both caidiac (23 cases) and noncardiac (30 cases). The cardiac malformations were an atria1 septal defect secundum type TABLE
I Age at Diagnosis
and at Surgery
Age at Diagnosis
122 Cases
Age at Surgery (37 cases)
< 1 year 1-12 years 13-20 years 21-58 years
23 56 17 26
0 24* 5 8
*The youngest patient to undergo operation was 5 years old.
than standard radiography of the chest. In the youngest patients (50% sometimes had the shunt decrease spontaneously with age. When the shunt is large, an associated malformation (patent ductus arteriosus, atria1 septal defect or abnormal systemic arteries arising from the abdominal aorta) should be researched and treated.
angiography are useful to confirm the diagnosis and make the therapeutic indications clear. Today, the use of more sophisticated noninvasive techniques such as magnetic resonance imaging and APPENDIX computerized tomography could confirm the diagnoThe different centers that contributed the files and sis. Echocardiography and Doppler can also assessthe follow-up of their patients are listed with the number of cases in brackets: France: Lille, Dupuis C. (34); prognosis. Computerized tomography has at least 3 advan- Lyons: Bozio A. (5), Verney RN (6); Paris-Trousseau: tages. (1) It clearly shows bronchiectasies when present. Sarda A. (9); Nancy Worms AM (5); Paris-Broussais: (2) It affirms the diagnosis of scimitar syndrome in Ourbak (6); Paris-Centre Chirurgical Marie Lanne Vemant F. (2); cases where opacification of the right pulmonary artery longue: Losay J. (2); Paris-La&xc: and of the right pulmonary veins is absent or very weak Amiens: Maingourd Y. (4); Rennes: Almange C. (4); (3 of our cases). (3) It may help in the diagnosis of Montpellier: Voisin M. (4); Tours: Chantepie A. (3); horseshoe lung, a malformation that seems to be an un- Bordeaux: Choussat A. (3); Reims: Mauran P. (2); common and often unrecognized part of the spectrum of Marseilles: Werner G. (2); Nantes: Lefi%re M. (2); pulmonary malformations associated with the scimitar Grenoble: Rossignol AM (2); Strasbourg: De Geeter B. syndrome.1q-21 (1); Dijo~ Petit A (1). Canada: Montreal: Hapital Ste. Vascularization of the left lung is increased by the Justine: Davignon A. (9). Sweden: Stockholm: Karoleft to right shunt. The vascular tree of the right lung is linska: Lundell B., Abon P. (3); Lund: Hanseus C. (3); often hypoplastic. The degree of hypoplasia varies and Uppsala: Charaf L. (1). Switzerland: Geneve: Friedli B. may be considered as an associated malformation. In (2); Luusanne: Payot M. (2). Belgium: Bruxellesthe isolated type of scimitar syndrome, the left to right U.C.L.: Vliers A. (2). The Netherlands: Rotterdam shunt is often
The 44Adultgv Form of the Scimitar
Syndrome
Claude Dupuis, MD, Louis A. C. Charaf, MD, Georges-Marie Brevi&re, MD, Pierre Abou, MD, Martine R6my-Jardin, MD, and Gunnar Helmius, MD
One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiiogic centers. The clinical, radiigraphic 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 lO years in the last 50 patients. Age, sex, heredity, clinical status, electrocardiography, plain chest films, cardiac catheterization and angiography were studied in all cases. Other examinations such as echccardiography, computerized tomography and spirometry with radioactive isotopes were performed in some centers. In a few cases, bronchoscopy and bronchography were also performed. As a matter of routine, medical and social history was obtained from all patients and the results of surgical treatment were studied. AUGUST
15, 1992
RESULTS Seventy-seven patients were women and 45 were men. The ages at diagnosis are listed in Table I. The parents of 2 of the patients were first cousins. These parents had 4 children of whom 2 were healthy and 2 had the scimitar syndrome. In another family, the father and his son both had the scimitar syndrome. Five children were born prematurely. One child was a twin. The other twin was normal. The patients were referred to hospitals for respiratory or cardiac problems probaby related to the scimitar syndrome: bouts of pneumonia (38 cases), slight dyspnea on exertion (23 cases), precordial systolic murmur (18 cases), paroxysmal tachycardia (1 case), deformation of the thorax with retraction of the right hemithorax (8 cases), and hemoptysis (7 cases). The remaining 27 patients were referred to centers for other symptoms unrelated to the scimitar syndrome. The
twins were both mentally retarded. One child had trisomia 21 and another had Turner’s syndrome. Other malformations were also present, both caidiac (23 cases) and noncardiac (30 cases). The cardiac malformations were an atria1 septal defect secundum type TABLE
I Age at Diagnosis
and at Surgery
Age at Diagnosis
122 Cases
Age at Surgery (37 cases)
< 1 year 1-12 years 13-20 years 21-58 years
23 56 17 26
0 24* 5 8
*The youngest patient to undergo operation was 5 years old.
than standard radiography of the chest. In the youngest patients (50% sometimes had the shunt decrease spontaneously with age. When the shunt is large, an associated malformation (patent ductus arteriosus, atria1 septal defect or abnormal systemic arteries arising from the abdominal aorta) should be researched and treated.
angiography are useful to confirm the diagnosis and make the therapeutic indications clear. Today, the use of more sophisticated noninvasive techniques such as magnetic resonance imaging and APPENDIX computerized tomography could confirm the diagnoThe different centers that contributed the files and sis. Echocardiography and Doppler can also assessthe follow-up of their patients are listed with the number of cases in brackets: France: Lille, Dupuis C. (34); prognosis. Computerized tomography has at least 3 advan- Lyons: Bozio A. (5), Verney RN (6); Paris-Trousseau: tages. (1) It clearly shows bronchiectasies when present. Sarda A. (9); Nancy Worms AM (5); Paris-Broussais: (2) It affirms the diagnosis of scimitar syndrome in Ourbak (6); Paris-Centre Chirurgical Marie Lanne Vemant F. (2); cases where opacification of the right pulmonary artery longue: Losay J. (2); Paris-La&xc: and of the right pulmonary veins is absent or very weak Amiens: Maingourd Y. (4); Rennes: Almange C. (4); (3 of our cases). (3) It may help in the diagnosis of Montpellier: Voisin M. (4); Tours: Chantepie A. (3); horseshoe lung, a malformation that seems to be an un- Bordeaux: Choussat A. (3); Reims: Mauran P. (2); common and often unrecognized part of the spectrum of Marseilles: Werner G. (2); Nantes: Lefi%re M. (2); pulmonary malformations associated with the scimitar Grenoble: Rossignol AM (2); Strasbourg: De Geeter B. syndrome.1q-21 (1); Dijo~ Petit A (1). Canada: Montreal: Hapital Ste. Vascularization of the left lung is increased by the Justine: Davignon A. (9). Sweden: Stockholm: Karoleft to right shunt. The vascular tree of the right lung is linska: Lundell B., Abon P. (3); Lund: Hanseus C. (3); often hypoplastic. The degree of hypoplasia varies and Uppsala: Charaf L. (1). Switzerland: Geneve: Friedli B. may be considered as an associated malformation. In (2); Luusanne: Payot M. (2). Belgium: Bruxellesthe isolated type of scimitar syndrome, the left to right U.C.L.: Vliers A. (2). The Netherlands: Rotterdam shunt is often
