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Autogenous atrial tunnel for direct cavopulmonary connection in infants and small children
Autogenous Atrial Tunnel for Direct Cavopulmonary Connection in Infants and Small Children Richard A. Perryman, MD Division of Thoracic and Cardiovascular Surgery, University of Miami School of Medicine, Miami, Florida
A technique is described for construction of an autogenous right atrial tunnel for direct cavopulmonary connection in infants and small children requiring Fontan operation. Advantages, in this subset of patients, of this method over others previously described using prosthetic or growth-limited materials are suggested. (Ann Thoruc Surg 2991;51:508-20)
T
heoretical and practical advantages have been proposed for creation of the Fontan circulation using direct cavopulmonary connection [141. Intraatrial channeling of inferior vena caval return has been described using pericardial baffles, Dacron or Gore-Tex tube grafts, or baffles cut from tube grafts [ 1 4 ] . To avoid the use of prosthetic or growth-restricted material in the infant or small child requiring direct cavopulmonary connection we have recently used a modification of the Senning technique in which a flap of right atrial free wall is used to create a circumferential atrial tunnel from inferior vena cava to superior vena cava. Described are 2 cases in which we have used this technique.
Technique Individual caval cannulation was carried out using angled metal cannulas. A right atrial free wall flap based on the sulcus terminalis was cut to size from eustachian valve inferiorly to just caudal of the superior vena cava superiorly. Where necessary, the atrial septal defect was enlarged anteriorly and superiorly. The right atrial tunnel was then constructed by suturing the atrial flap around the entrance of the inferior vena cava, using eustachian valve where present, and similarly around the origin of the superior vena cava extending somewhat medially to allow an enlarging incision (if necessary) into the right atrial wall for superior vena caval connection. The flap was then sewn along the atrial septum, posterior to atrial septal defect, to complete the tunnel (Fig 1). Coronary sinus was left in the low-pressure pulmonary venous chamber. Superior caval connections were then carried out as dictated by the pulmonary artery anatomy. The anterior wall of the right atrium was brought down and Accepted for publication Dec 24, 1990. Address reprint requests to Dr Perryman, University of Miami School of Medicine, PO Box 016960, Miami, FL 33101.
0 1991 by The Society of Thoracic Surgeons
anastomosed to the outer wall of the atrial tunnel to reconstruct the pulmonary venous chamber or the atrial wall defect was filled with a suitable pericardial patch. Case Reports
Patient 1 An 11-month-old, 4.9-kginfant had single ventricle, aorta arising from rudimentary left-sided chamber, severely restrictive ventricular septal defect (100-mm Hg gradient) and severe subpulmonic stenosis. At operation the main pulmonary artery was transected, subpulmonic obstruction resected, and a double outlet created (Damus-KayeStansel). The inferior vena caval blood was redirected to the transected, enlarged superior vena cava using the described intraatrial tunnel, and the caudal superior vena cava was anastamosed to the transected main pulmonary artery. The cephalad transected superior vena cava was connected end-to-end to the transected distal right pulmonary artery. Because of prolonged periods of complete heart block preoperatively, permanent ventricular pacing electrodes were left epicardially and a generator was placed postoperatively. Postoperative catheterization showed no obstruction of the inferior vena cava to left pulmonary artery blood flow (Fig 2). Twenty-four months postoperatively the atrial tunnel remains widely patent (Fig 3A).
Patient 2 A 20-month-old, 8-kg female infant with double-inlet single ventricle and L-malposition of the great vessels with aorta arising from a rudimentary left-sided right ventricle, a nonrestrictive ventricular septal defect, and moderate pulmonic stenosis was seen with failure to thrive and poorly controlled heart failure. At cardiac catheterization the pulmonary to systemic shunt ratio was 4:1, mean pulmonary artery pressure was 20 mm Hg, and the pulmonary to systemic resistance ratio was less than 0.1. An intraatrial tunnel for inferior vena caval return was constructed as described. Direct cavopulmonary connection was established by anastomosis of caudal transected superior vena cava to the right pulmonary artery at the junction with the main pulmonary artery and cephalad superior vena cava to the superior aspect of the right pulmonary artery. The child was discharged home, but returned 2 weeks later with ascites and pleural effusions. Repeated echocar0003-4975/91/$3.50
HOW TO DO IT PERRYMAN DIRECT CAVOPULMONARY CONNECTION
Ann Thorac Surg 1991;51:50%10
Fig 1. Right atrial free wall flap channels inferior vena caval blood to superior vena cava. Atrial septa1 defect provides inflow to right atrioventricular valve.
509
A
diographic evaluation revealed no right-sided obstruction (Fig 3B). She died 2 months postoperatively of Candida sepsis. She remained in normal sinus rhythm throughout her postoperative course.
Comment Advantages of total cavopulmonary connection for the Fontan procedure have been described. Reduced loss of
B Fig 3. (A) Echocardiogram of patient I, 24 months postoperatively. ( B ) Echocardiogram of patient 2 , 1 month postoperatively. (AT = atrial tunnel; IVC = inferior vena cava; LA = left atrium; LV = left ventricle; PA = pulmonary artery; PV = pulmonary veins; RA = right atrium; RV = right ventricle; SVC = superior vena cava.)
Fig 2. Postoperative inferior venacavogram of patient I .
energy by direct channeling of blood to pulmonary arteries has been suggested [l]. Advantages of maintaining low pressure in the coronary sinus have been reported [ 5 ] . There are cases of complex congenital cardiac anatomy in which intraatrial baffling or channeling of inferior vena caval blood to the superior vena cava is certainly advantageous for creation of the Fontan circulation. These include cases, predominantly of the single-ventricle complex type, where the right atrioventricular valve provides ventricular inflow (eg, mitral atresia) or where indeterminate nodal or conduction abnormalities make uncomplicated patch closure of the right atrioventricular valve difficult. Distortion of pulmonary anatomy by previous
510
HOWTODOIT PERRYMAN DIRECT CAVOPULMONARY CONNECTION
shunts or abnormal growth patterns also may be best managed by cavopulmonary connection, as may multiple venous-atrial connections (eg, atrial isomerism) [2, 41. Many techniques of construction of intraatrial tunneling of inferior vena caval blood to the superior vena cava have been described [14]. My colleagues and I believe that in the infant or small child a complete atrial tunnel has advantages over other methods. A similar technique used in the Senning procedure for transposition of the great arteries resulted in less obstruction when compared with methods using pericardium or Dacron baffles [ 6 ] .The use of complete intraatrial tube grafts is limited in infants by conduit size. Partial prosthetic tunnels are thought to grow through an increase in posterior wall size. That growth over time, however, remains to be evaluated, and recent experience has shown an increased rate of pulmonary pathway obstruction when prosthetic material has been employed in other techniques for Fontan connection [7].Long-term follow-up on growth of the autogenous atrial channel has not been done but, using the Senning procedure experience, growth of the tunnel can be expected. The technique presented here provides a method of direct cavopulmonary connection in infants and small children that avoids the use of prosthetic or growthlimited materials.
Ann Thorac Surg 1991;51:50610
References 1. De Leva1 MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations: experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988; 96~682-95. 2. JOMS RA, Castaneda AR. Modified Fontan Procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques. J Cardiac Surg 1988;3:91-6. 3. Matsuda H, Kawashima Y, Kishimoto H, et al. Problems in the modified Fontan operation for univentricular heart of the right ventricular type. Circulation 1987;76(Suppl3):45-52. 4. Puga FJ, Chiavarelli M, Hagler DJ. Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation 1987; 76(Suppl 3):53. 5. Ilbawi MN, ldriss FS, Muster AJ, et al. Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation. J Thorac Cardiovasc Surg 1986;92:231-7. 6. Bender HW Jr, Stewart JR, Merrill WH, Hammon JW Jr, Graham TP Jr. Ten years' experience with the Senning operation for transposition of the great arteries: physiological results and late follow-up. Ann Thorac Surg 1989;47218-23. 7. Fernandez G, Costa F, Fontan F, Naftel DC, Blackstone EH, Kirklin JW. Prevalence of reoperation for pathway obstruction after Fontan operation. Ann Thorac Surg 1989;48:654-9.
A technique is described for construction of an autogenous right atrial tunnel for direct cavopulmonary connection in infants and small children requiring Fontan operation. Advantages, in this subset of patients, of this method over others previously described using prosthetic or growth-limited materials are suggested. (Ann Thoruc Surg 2991;51:508-20)
T
heoretical and practical advantages have been proposed for creation of the Fontan circulation using direct cavopulmonary connection [141. Intraatrial channeling of inferior vena caval return has been described using pericardial baffles, Dacron or Gore-Tex tube grafts, or baffles cut from tube grafts [ 1 4 ] . To avoid the use of prosthetic or growth-restricted material in the infant or small child requiring direct cavopulmonary connection we have recently used a modification of the Senning technique in which a flap of right atrial free wall is used to create a circumferential atrial tunnel from inferior vena cava to superior vena cava. Described are 2 cases in which we have used this technique.
Technique Individual caval cannulation was carried out using angled metal cannulas. A right atrial free wall flap based on the sulcus terminalis was cut to size from eustachian valve inferiorly to just caudal of the superior vena cava superiorly. Where necessary, the atrial septal defect was enlarged anteriorly and superiorly. The right atrial tunnel was then constructed by suturing the atrial flap around the entrance of the inferior vena cava, using eustachian valve where present, and similarly around the origin of the superior vena cava extending somewhat medially to allow an enlarging incision (if necessary) into the right atrial wall for superior vena caval connection. The flap was then sewn along the atrial septum, posterior to atrial septal defect, to complete the tunnel (Fig 1). Coronary sinus was left in the low-pressure pulmonary venous chamber. Superior caval connections were then carried out as dictated by the pulmonary artery anatomy. The anterior wall of the right atrium was brought down and Accepted for publication Dec 24, 1990. Address reprint requests to Dr Perryman, University of Miami School of Medicine, PO Box 016960, Miami, FL 33101.
0 1991 by The Society of Thoracic Surgeons
anastomosed to the outer wall of the atrial tunnel to reconstruct the pulmonary venous chamber or the atrial wall defect was filled with a suitable pericardial patch. Case Reports
Patient 1 An 11-month-old, 4.9-kginfant had single ventricle, aorta arising from rudimentary left-sided chamber, severely restrictive ventricular septal defect (100-mm Hg gradient) and severe subpulmonic stenosis. At operation the main pulmonary artery was transected, subpulmonic obstruction resected, and a double outlet created (Damus-KayeStansel). The inferior vena caval blood was redirected to the transected, enlarged superior vena cava using the described intraatrial tunnel, and the caudal superior vena cava was anastamosed to the transected main pulmonary artery. The cephalad transected superior vena cava was connected end-to-end to the transected distal right pulmonary artery. Because of prolonged periods of complete heart block preoperatively, permanent ventricular pacing electrodes were left epicardially and a generator was placed postoperatively. Postoperative catheterization showed no obstruction of the inferior vena cava to left pulmonary artery blood flow (Fig 2). Twenty-four months postoperatively the atrial tunnel remains widely patent (Fig 3A).
Patient 2 A 20-month-old, 8-kg female infant with double-inlet single ventricle and L-malposition of the great vessels with aorta arising from a rudimentary left-sided right ventricle, a nonrestrictive ventricular septal defect, and moderate pulmonic stenosis was seen with failure to thrive and poorly controlled heart failure. At cardiac catheterization the pulmonary to systemic shunt ratio was 4:1, mean pulmonary artery pressure was 20 mm Hg, and the pulmonary to systemic resistance ratio was less than 0.1. An intraatrial tunnel for inferior vena caval return was constructed as described. Direct cavopulmonary connection was established by anastomosis of caudal transected superior vena cava to the right pulmonary artery at the junction with the main pulmonary artery and cephalad superior vena cava to the superior aspect of the right pulmonary artery. The child was discharged home, but returned 2 weeks later with ascites and pleural effusions. Repeated echocar0003-4975/91/$3.50
HOW TO DO IT PERRYMAN DIRECT CAVOPULMONARY CONNECTION
Ann Thorac Surg 1991;51:50%10
Fig 1. Right atrial free wall flap channels inferior vena caval blood to superior vena cava. Atrial septa1 defect provides inflow to right atrioventricular valve.
509
A
diographic evaluation revealed no right-sided obstruction (Fig 3B). She died 2 months postoperatively of Candida sepsis. She remained in normal sinus rhythm throughout her postoperative course.
Comment Advantages of total cavopulmonary connection for the Fontan procedure have been described. Reduced loss of
B Fig 3. (A) Echocardiogram of patient I, 24 months postoperatively. ( B ) Echocardiogram of patient 2 , 1 month postoperatively. (AT = atrial tunnel; IVC = inferior vena cava; LA = left atrium; LV = left ventricle; PA = pulmonary artery; PV = pulmonary veins; RA = right atrium; RV = right ventricle; SVC = superior vena cava.)
Fig 2. Postoperative inferior venacavogram of patient I .
energy by direct channeling of blood to pulmonary arteries has been suggested [l]. Advantages of maintaining low pressure in the coronary sinus have been reported [ 5 ] . There are cases of complex congenital cardiac anatomy in which intraatrial baffling or channeling of inferior vena caval blood to the superior vena cava is certainly advantageous for creation of the Fontan circulation. These include cases, predominantly of the single-ventricle complex type, where the right atrioventricular valve provides ventricular inflow (eg, mitral atresia) or where indeterminate nodal or conduction abnormalities make uncomplicated patch closure of the right atrioventricular valve difficult. Distortion of pulmonary anatomy by previous
510
HOWTODOIT PERRYMAN DIRECT CAVOPULMONARY CONNECTION
shunts or abnormal growth patterns also may be best managed by cavopulmonary connection, as may multiple venous-atrial connections (eg, atrial isomerism) [2, 41. Many techniques of construction of intraatrial tunneling of inferior vena caval blood to the superior vena cava have been described [14]. My colleagues and I believe that in the infant or small child a complete atrial tunnel has advantages over other methods. A similar technique used in the Senning procedure for transposition of the great arteries resulted in less obstruction when compared with methods using pericardium or Dacron baffles [ 6 ] .The use of complete intraatrial tube grafts is limited in infants by conduit size. Partial prosthetic tunnels are thought to grow through an increase in posterior wall size. That growth over time, however, remains to be evaluated, and recent experience has shown an increased rate of pulmonary pathway obstruction when prosthetic material has been employed in other techniques for Fontan connection [7].Long-term follow-up on growth of the autogenous atrial channel has not been done but, using the Senning procedure experience, growth of the tunnel can be expected. The technique presented here provides a method of direct cavopulmonary connection in infants and small children that avoids the use of prosthetic or growthlimited materials.
Ann Thorac Surg 1991;51:50610
References 1. De Leva1 MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations: experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988; 96~682-95. 2. JOMS RA, Castaneda AR. Modified Fontan Procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques. J Cardiac Surg 1988;3:91-6. 3. Matsuda H, Kawashima Y, Kishimoto H, et al. Problems in the modified Fontan operation for univentricular heart of the right ventricular type. Circulation 1987;76(Suppl3):45-52. 4. Puga FJ, Chiavarelli M, Hagler DJ. Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation 1987; 76(Suppl 3):53. 5. Ilbawi MN, ldriss FS, Muster AJ, et al. Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation. J Thorac Cardiovasc Surg 1986;92:231-7. 6. Bender HW Jr, Stewart JR, Merrill WH, Hammon JW Jr, Graham TP Jr. Ten years' experience with the Senning operation for transposition of the great arteries: physiological results and late follow-up. Ann Thorac Surg 1989;47218-23. 7. Fernandez G, Costa F, Fontan F, Naftel DC, Blackstone EH, Kirklin JW. Prevalence of reoperation for pathway obstruction after Fontan operation. Ann Thorac Surg 1989;48:654-9.