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Correction of Anomalous Subdiaphragmatic Inferior Vena Caval Drainage of the Right Pulmonary Veins (Scimitar Syndrome)
Correction of Anomalous Subdiaphragmatic Inferior Vena Caval Drainage of the Right Pulmonary Veins (Scimitar Syndrome) Gordon R. Tobin, M.D., Robert M. Anderson, M.D., and Arnold J. Arem, M.D.
ABSTRACT Correction of anomalous infradiaphragmatic inferior vena caval drainage of the right pulmonary veins can be repaired under conventional cardiopulmonary bypass by placing a circumferential tape below the diaphragm between the hepatic veins and the scimitar vein entrance. During repair, creation of an atrial septal defect in the distorted septalleft atrial junction of scimitar syndrome is facilitated by first incising the anteromedial aspect of the fossa ovalis to better delineate the optimal posterolateral margin. A case history illustrates the technique.
Since the advent of cardiopulmonary bypass, the left-to-right shunt of scimitar syndrome has been correctable in all cases. Two problems have remained, however, that have made repair somewhat complicated. The first is the inaccessibility of the anomalous vein entrance into the inferior vena cava while the patient is on conventional cardiopulmonary bypass with the inferior vena caval catheter and circumferential tape in place. The second problem is that there is distortion of the landmarks for atrial septectomy due to the absence of pulmonary venous attachments to the left atrium. The two problems were minimized by two technical maneuvers during correction of anomalous subdiaphragmatic inferior vena caval drainage of the right pulmonary veins in a 9-year-old girl. The difficulty of exposing the anomalous vein below the diaphragm during cardiopulmonary bypass was solved by splitting the diaphragm from the anterior midline to the vena caval hiatus, which gave access to the inferior vena cava below the entrance of the "scimFrom the Department of Surgery, University of Arizona College of Medicine, Tucson, AZ. Accepted for publication July 22, 1976. Address reprint requests to Dr. Anderson, Department of Surgery, University of Arizona College of Medicine, Tucson, A 2 85724.
267
itar" vein. Distortion of the atrial septum did not compromise accurate resection of the fossa ovalis in creating an atrial septal defect when the anteromedial aspect of the fossa ovalis was incised as the first step. Case Report A 9-year-old, 28 kg American Papago Indian girl had been known to have a heart murmur since age 5. She was becoming more easily fatigued and experienced dyspnea and wheezing on exertion. The right hemithorax was smaller than the left. The sternum was concave to the right. Right heart lift was marked. Rales and wheezes were present throughout both lung fields. A grade 3/6 systolic murmur and an early systolic ejection click were maximal in the second left interspace in the midclavicular line. A grade 1 diastolic murmur was loudest at the cardiac apex. The second cardiac sound was widely split and fixed. A roentgenogram (Fig 1) revealed a shadow near the right heart border pathognomonic of scimitar syndrome [41. A moderately hypoplastic right lung was associated with a mediastinal shift to the right. Pulmonary vascular markings were accentuated throughout. An electrocardiogram disclosed normal sinus rhythm with a + 100-degree axis and a QRS duration of 0.07 second. There was an oxygen saturation increase from 68% in the lower inferior vena cava to 84% at the junction of the inferior vena cava and right atrium. Cineangiography confirmed total anomalous right pulmonary venous drainage to the inferior vena cava below the diaphragm. No atrial septal defect could be demonstrated.
Operative Procedure Operation was performed through a median sternotomy. The diaphragm was split from its anterior attachment down to the inferior vena
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The Annals of Thoracic Surgery Vol 23 No 3 March 1977
Fig 1 . Small right lung, cardiac displacement to the right, and evidence of a n anomalous venous shadow at the right cardiac border are all characteristics of the scimitar syndrome.
caval hiatus (Fig 2). The inferior vena cava below the diaphragm was separated from the surrounding tissue. A tape around the inferior vena cava was placed between the hepatic veins and the scimitar vein (Fig 3). Another tape was placed around the superior vena cava. Vena caval drainage catheters were inserted through the right atrial appendage. Arterial perfusion was into the ascending aorta. After cardiopulmonary bypass was started, the heart was electrically fibrillated. A right atriotomy was extended from the right atrial appendage caudad into the anterior aspect of the inferior vena cava to within 5 mm of the inferior vena caval circumferential tape (see Fig 3). This atrial incision allowed adequate exposure of the anomalous pulmonary vein orifice in the inferior vena cava with the cannula in place and without interruption of cardiopulmonary bypass. The fossa ovalis was then excised from the intact atrial septum. Excision of the fossa ovalis was begun at its anteromedial border. Accurate delineation of the distorted posterolateral aspect, where absence of pulmonary venous juncture created an
acute angle between the left atrium and the atrial septum, was then possible. A pericardial patch was used to construct a tunnel from the orifice of the anomalous pulmonary vein to the surgically created atrial septal defect (Fig 4). The pericardial patch was made somewhat bulbous to avoid undue resistance to the flow of pulmonary blood. The pulmonary venous tunnel occupied approximately two-fifths of the cross-sectional area of the inferior vena cava. The right atriotomy was then closed with a continuous 4-0 suture. The heart was defibrillated, and bypass was discontinued. The diaphragm was repaired with interrupted sutures. The median sternotomy was closed in layers. The patient’s postoperative course was uneventful. She was discharged on the tenth postoperative day. A 31h-year follow-up examination disclosed a normally active 12-year-old girl who had no cardiorespiratory complaints and had normal cardiac sounds.
Comment Before the development of cardiopulmonary bypass, complete correction of anomalous inferior vena caval drainage of the right pulmonary veins was possible in only a few patients in whom peculiarities of the anatomy allowed reimplantation of the anomalous vein into the left atrium or in whom double insertion of the pulmonary venous drainage to both the left atrium and the inferior vena cava could be corrected by simple ligation [ l , 5,8]. Cardiopulmonary bypass allowed repair with a patch tunnel graft in the right atrium to convey the pulmonary venous blood from the scimitar vein orifice through an atrial septal defect into the left atrium [2, 3, 6, 7, 9-111. The circumferential vena caval tape placed within the pericardium in conventional cardiopulmonary bypass blocks access to the scimitar vein orifice. Murphy and associates [61 used hypothermia and circulatory arrest with removal of the inferior vena caval catheter to gain exposure and a dry field during suture of the tunnel patch over the scimitar vein orifice in the inferior vena cava. Shumacker and coworkers [9] and Yonehiro and associates [lo] circumvented the problem of exposure within
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How to Do It: Tobin, Anderson, and Arem: Correction of Scimitar Syndrome
Fig2. The diaphragm has been incised, exposing the infradiaphragmatic inferior vena cava.
Fig3. A circumferential tape placed below the scimitar vein entrance allows exposure without removal of the inferiorvena cava cannula. The fossa ovalis excision is begun at its anterornedical aspect.
270 The Annals of Thoracic Surgery Vol 23 No 3
March 1977
Fig4. A pericardial tunnel patch diverts pulmonary venous blood from the anomalous pulmonary vein through the fossa ovalis into the left atrium.
the inferior vena caval lumen by transecting the anomalous vein and reimplanting it into the right atrium. They then placed the tunnel patch over the new right atrial vein orifice and an atrial septal defect. In patients in whom the scimitar vein enters the inferior vena cava above the diaphragm, a circumferential tape within the pericardial sac allows repair without interruption of bypass or need for reimplantation [9,11]. The technique presented allows tunneling of scimitar vein flow to the left atrium in all cases without the need of hypothermia and circulatory arrest or reimplantation. The creation of an atrial Septa1 defect in the distorted septal left atrial junction of scimitar syndrome is facilitated by first incising the anteromedial aspect of the fossa ovalis to better delineate the optimal posterolateral margin.
References 1. Gazzaniga AB, Matloff JM, Harken DE: Anomalous right pulmonary drainage into the inferior vena cava and right atrium. J Thorac Cardiovasc Surg 57:251, 1969 2. Gilman RA, Skowron CAR, Musser GB, et al: Partial anomalous venous drainage. Am J Surg 94:688, 1957
3. Kirklin JW, Ellis FH, Wood EH: Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 39:389, 1956 4 Koch W, Silva A: Anomalous drainage of pulmonary veins into the inferior vena cava. Radiology 75:592, 1960 5 Moniuddin SM, Levin HR, Runco V, et al: Anomalous pulmonary venous drainage: a common trunk emptying into the left atrium and inferior vena cava. Circulation 24:46, 1966 6. Murphy JW, Kerr AR, Kirklin JW: Intracardiac repair for anomalous pulmonary venous connection of right lung to inferior vena cava. Ann Thorac Surg 11:38, 1971 7. Risch F, Hahn C: The technique of surgical correction of anomalies of the pulmonary veins in a series of twenty-five cases. Thorax 13:251, 1958 8. Sanger PW, Taylor FH, Robicsek F: The "scimitar syndrome." Arch Surg 86:580, 1963 9. Shumacker HB Jr, Judd D: Partial anomalous pulmonary venous return with reference to drainage and to the inferior vena cava and to an intact atrial septum. J Cardiovasc Surg (Torino) 5:271, 1964 10. Yonehiro EG, Hallman GL, Cooley DA: Anomalous pulmonary venous return from a hypoplastic right lung to the inferior vena cava (scimitar syndrome): report of successful correction and a review of surgical treatment. Baylor U Coll Med Cardiovasc Res Cent Bull 4:106, 1966 11. Zubiate P, Kay JH: Surgical correction of anomalous pulmonary venous connection. Ann Surg 156:234, 1962
ABSTRACT Correction of anomalous infradiaphragmatic inferior vena caval drainage of the right pulmonary veins can be repaired under conventional cardiopulmonary bypass by placing a circumferential tape below the diaphragm between the hepatic veins and the scimitar vein entrance. During repair, creation of an atrial septal defect in the distorted septalleft atrial junction of scimitar syndrome is facilitated by first incising the anteromedial aspect of the fossa ovalis to better delineate the optimal posterolateral margin. A case history illustrates the technique.
Since the advent of cardiopulmonary bypass, the left-to-right shunt of scimitar syndrome has been correctable in all cases. Two problems have remained, however, that have made repair somewhat complicated. The first is the inaccessibility of the anomalous vein entrance into the inferior vena cava while the patient is on conventional cardiopulmonary bypass with the inferior vena caval catheter and circumferential tape in place. The second problem is that there is distortion of the landmarks for atrial septectomy due to the absence of pulmonary venous attachments to the left atrium. The two problems were minimized by two technical maneuvers during correction of anomalous subdiaphragmatic inferior vena caval drainage of the right pulmonary veins in a 9-year-old girl. The difficulty of exposing the anomalous vein below the diaphragm during cardiopulmonary bypass was solved by splitting the diaphragm from the anterior midline to the vena caval hiatus, which gave access to the inferior vena cava below the entrance of the "scimFrom the Department of Surgery, University of Arizona College of Medicine, Tucson, AZ. Accepted for publication July 22, 1976. Address reprint requests to Dr. Anderson, Department of Surgery, University of Arizona College of Medicine, Tucson, A 2 85724.
267
itar" vein. Distortion of the atrial septum did not compromise accurate resection of the fossa ovalis in creating an atrial septal defect when the anteromedial aspect of the fossa ovalis was incised as the first step. Case Report A 9-year-old, 28 kg American Papago Indian girl had been known to have a heart murmur since age 5. She was becoming more easily fatigued and experienced dyspnea and wheezing on exertion. The right hemithorax was smaller than the left. The sternum was concave to the right. Right heart lift was marked. Rales and wheezes were present throughout both lung fields. A grade 3/6 systolic murmur and an early systolic ejection click were maximal in the second left interspace in the midclavicular line. A grade 1 diastolic murmur was loudest at the cardiac apex. The second cardiac sound was widely split and fixed. A roentgenogram (Fig 1) revealed a shadow near the right heart border pathognomonic of scimitar syndrome [41. A moderately hypoplastic right lung was associated with a mediastinal shift to the right. Pulmonary vascular markings were accentuated throughout. An electrocardiogram disclosed normal sinus rhythm with a + 100-degree axis and a QRS duration of 0.07 second. There was an oxygen saturation increase from 68% in the lower inferior vena cava to 84% at the junction of the inferior vena cava and right atrium. Cineangiography confirmed total anomalous right pulmonary venous drainage to the inferior vena cava below the diaphragm. No atrial septal defect could be demonstrated.
Operative Procedure Operation was performed through a median sternotomy. The diaphragm was split from its anterior attachment down to the inferior vena
268
The Annals of Thoracic Surgery Vol 23 No 3 March 1977
Fig 1 . Small right lung, cardiac displacement to the right, and evidence of a n anomalous venous shadow at the right cardiac border are all characteristics of the scimitar syndrome.
caval hiatus (Fig 2). The inferior vena cava below the diaphragm was separated from the surrounding tissue. A tape around the inferior vena cava was placed between the hepatic veins and the scimitar vein (Fig 3). Another tape was placed around the superior vena cava. Vena caval drainage catheters were inserted through the right atrial appendage. Arterial perfusion was into the ascending aorta. After cardiopulmonary bypass was started, the heart was electrically fibrillated. A right atriotomy was extended from the right atrial appendage caudad into the anterior aspect of the inferior vena cava to within 5 mm of the inferior vena caval circumferential tape (see Fig 3). This atrial incision allowed adequate exposure of the anomalous pulmonary vein orifice in the inferior vena cava with the cannula in place and without interruption of cardiopulmonary bypass. The fossa ovalis was then excised from the intact atrial septum. Excision of the fossa ovalis was begun at its anteromedial border. Accurate delineation of the distorted posterolateral aspect, where absence of pulmonary venous juncture created an
acute angle between the left atrium and the atrial septum, was then possible. A pericardial patch was used to construct a tunnel from the orifice of the anomalous pulmonary vein to the surgically created atrial septal defect (Fig 4). The pericardial patch was made somewhat bulbous to avoid undue resistance to the flow of pulmonary blood. The pulmonary venous tunnel occupied approximately two-fifths of the cross-sectional area of the inferior vena cava. The right atriotomy was then closed with a continuous 4-0 suture. The heart was defibrillated, and bypass was discontinued. The diaphragm was repaired with interrupted sutures. The median sternotomy was closed in layers. The patient’s postoperative course was uneventful. She was discharged on the tenth postoperative day. A 31h-year follow-up examination disclosed a normally active 12-year-old girl who had no cardiorespiratory complaints and had normal cardiac sounds.
Comment Before the development of cardiopulmonary bypass, complete correction of anomalous inferior vena caval drainage of the right pulmonary veins was possible in only a few patients in whom peculiarities of the anatomy allowed reimplantation of the anomalous vein into the left atrium or in whom double insertion of the pulmonary venous drainage to both the left atrium and the inferior vena cava could be corrected by simple ligation [ l , 5,8]. Cardiopulmonary bypass allowed repair with a patch tunnel graft in the right atrium to convey the pulmonary venous blood from the scimitar vein orifice through an atrial septal defect into the left atrium [2, 3, 6, 7, 9-111. The circumferential vena caval tape placed within the pericardium in conventional cardiopulmonary bypass blocks access to the scimitar vein orifice. Murphy and associates [61 used hypothermia and circulatory arrest with removal of the inferior vena caval catheter to gain exposure and a dry field during suture of the tunnel patch over the scimitar vein orifice in the inferior vena cava. Shumacker and coworkers [9] and Yonehiro and associates [lo] circumvented the problem of exposure within
269
How to Do It: Tobin, Anderson, and Arem: Correction of Scimitar Syndrome
Fig2. The diaphragm has been incised, exposing the infradiaphragmatic inferior vena cava.
Fig3. A circumferential tape placed below the scimitar vein entrance allows exposure without removal of the inferiorvena cava cannula. The fossa ovalis excision is begun at its anterornedical aspect.
270 The Annals of Thoracic Surgery Vol 23 No 3
March 1977
Fig4. A pericardial tunnel patch diverts pulmonary venous blood from the anomalous pulmonary vein through the fossa ovalis into the left atrium.
the inferior vena caval lumen by transecting the anomalous vein and reimplanting it into the right atrium. They then placed the tunnel patch over the new right atrial vein orifice and an atrial septal defect. In patients in whom the scimitar vein enters the inferior vena cava above the diaphragm, a circumferential tape within the pericardial sac allows repair without interruption of bypass or need for reimplantation [9,11]. The technique presented allows tunneling of scimitar vein flow to the left atrium in all cases without the need of hypothermia and circulatory arrest or reimplantation. The creation of an atrial Septa1 defect in the distorted septal left atrial junction of scimitar syndrome is facilitated by first incising the anteromedial aspect of the fossa ovalis to better delineate the optimal posterolateral margin.
References 1. Gazzaniga AB, Matloff JM, Harken DE: Anomalous right pulmonary drainage into the inferior vena cava and right atrium. J Thorac Cardiovasc Surg 57:251, 1969 2. Gilman RA, Skowron CAR, Musser GB, et al: Partial anomalous venous drainage. Am J Surg 94:688, 1957
3. Kirklin JW, Ellis FH, Wood EH: Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 39:389, 1956 4 Koch W, Silva A: Anomalous drainage of pulmonary veins into the inferior vena cava. Radiology 75:592, 1960 5 Moniuddin SM, Levin HR, Runco V, et al: Anomalous pulmonary venous drainage: a common trunk emptying into the left atrium and inferior vena cava. Circulation 24:46, 1966 6. Murphy JW, Kerr AR, Kirklin JW: Intracardiac repair for anomalous pulmonary venous connection of right lung to inferior vena cava. Ann Thorac Surg 11:38, 1971 7. Risch F, Hahn C: The technique of surgical correction of anomalies of the pulmonary veins in a series of twenty-five cases. Thorax 13:251, 1958 8. Sanger PW, Taylor FH, Robicsek F: The "scimitar syndrome." Arch Surg 86:580, 1963 9. Shumacker HB Jr, Judd D: Partial anomalous pulmonary venous return with reference to drainage and to the inferior vena cava and to an intact atrial septum. J Cardiovasc Surg (Torino) 5:271, 1964 10. Yonehiro EG, Hallman GL, Cooley DA: Anomalous pulmonary venous return from a hypoplastic right lung to the inferior vena cava (scimitar syndrome): report of successful correction and a review of surgical treatment. Baylor U Coll Med Cardiovasc Res Cent Bull 4:106, 1966 11. Zubiate P, Kay JH: Surgical correction of anomalous pulmonary venous connection. Ann Surg 156:234, 1962