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Tetralogy of Fallot with anomalous coronary artery: double outflow technique
Tetralogy of Fallot With Anomalous Coronary Artery: Double Outflow Technique B. Reddy Dandolu, MD, H. Scott Baldwin, MD, William I. Norwood, Jr, MD, PhD, and Marshall L. Jacobs, MD Division of Cardiothoracic Surgery, Deborah Heart and Lung Center, Browns Mills, New Jersey, and Divisions of Pediatric Cardiology and Cardiovascular Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
A new technique to repair tetralogy of Fallot with an anomalous coronary artery crossing the right ventricular outflow tract is described, together with intermediate term follow-up. Using a pedicled flap of the anterior pulmonary artery wall as the floor, and a vascular or prosthetic patch as the roof, a composite conduit with the potential for growth is constructed. Together with the native outflow tract, this provides unobstructed egress
from the right ventricle to the branch pulmonary arteries. Since 1990, 4 infants aged 2-weeks to 6-months have undergone primary repair using this technique. Intermediate term follow-up shows adequate durability of the repair.
A
Technique
bnormal coronary artery anatomy, with a major coronary vessel crossing the outflow tract of the right ventricle, poses special problems during repair of tetralogy of Fallot. The most common of such anatomical variants is right coronary origin of the anterior descending artery, such that the anterior descending coronary artery crosses the outflow tract of the right ventricle [1]. Less commonly, there is a single coronary artery, or right coronary origin of the circumflex artery, or dual blood supply to the anterior septum with the portion nearer the base of the heart supplied by a vessel originating from the right coronary artery, and that portion nearer the apex supplied by a branch of the left coronary artery. Most often, symptomatic infants with these anatomical variants are managed initially by creation of a systemic to pulmonary artery shunt, and subsequently by a reparative procedure using an extra-cardiac conduit [2]. Alternatively, a tailored right ventricular incision and outflow patch reconstruction [3], or a primarily transatrial approach to relief of right ventricular outflow tract obstruction have been used. Consistent with our philosophy of accomplishing primary reparative surgery in symptomatic infants with tetralogy of Fallot, we have devised a technique that uses a viable flap of autologous vascular tissue as part of the right ventricular outflow tract reconstruction, and maintains antegrade flow through the native outflow tract (when patent) as well. We hypothesized that this double outflow technique would be associated with durable relief of right ventricular outflow tract obstruction, as there is the potential for growth of the surgically created outflow pathway.
Accepted for publication Sep 16 1998. Address reprint requests to Dr Jacobs, Section of Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134.
© 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
(Ann Thorac Surg 1999;67:1178 – 80) © 1999 by The Society of Thoracic Surgeons
Coronary anatomy is carefully inspected upon opening the pericardium, and is correlated with preoperative angiographic findings. Cardiopulmonary bypass with moderate hypothermia or with deep hypothermia and circulatory arrest is used. After cardioplegic arrest, a vertical infundibulotomy incision is made beginning a few millimeters below the level of the coronary artery that crosses the right ventricular outflow tract. Hypertrophied infundibular muscle bundles are divided and resected through this incision. The malalignment type ventricular septal defect is closed with a fabric patch and continuous monofilament suture. Parallel incisions are made in the anterior portion of the main pulmonary artery and may be carried on to the proximal left pulmonary artery if needed to provide adequate length to the autologous flap. The incisions are joined distally on the main pulmonary artery, or the proximal left pulmonary artery, creating a proximally based flap of pulmonary artery tissue (Fig 1). The length of this flap should be slightly greater than the distance between the base of the flap and the upper margin of the infundibular incision in the right ventricle. When appropriate, pulmonary valvotomy is performed through the opening in the main pulmonary artery. The flap is turned down onto the anterior surface of the infundibulum of the right ventricle such that it lies over the course of the anomalous coronary artery. The end of the flap is sutured to the superior margin of the ventriculotomy using continuous monofilament suture (Fig 2). Care should be taken to ascertain that the length of the flap is sufficient that it will not be stretched over the surface of the coronary and potentially compromise coronary flow. Cryopreserved pulmonary artery tissue is thawed and washed. A portion is excised to create a generous elliptically shaped patch. Alternatively the patch can be 0003-4975/99/$20.00 PII S0003-4975(99)00115-0
Ann Thorac Surg 1999;67:1178 – 80
Fig 1. The ventriculotomy incision is begun below the coronary artery, which crosses the right ventricular outflow tract. The ventricular septal defect is closed through this incision. The broken line shows the sites of incision in the anterior wall of the main pulmonary artery.
fashioned from autologous or xenograft pericardium, or from prosthetic material. Using monofilament suture, the patch is sutured to the edges of the opened main pulmonary artery (and left pulmonary artery), then to the edges of the autologous pulmonary artery flap, and finally to the remaining edges of the infundibular ventriculotomy. This can be carried out with a Hagar dilator or other calibrating device placed beneath the patch, to calibrate the size of the surgically created outflow pathway. At the end of this procedure, the right ventricle has two outflow tracts: a presumably restrictive pathway through the infundibulum and native pulmonary valve; and the new pathway created using the flap of the pulmonary artery wall and the roof of homograft tissue. Our first use of this technique was in a 3-day-old neonate in 1990. Since then, 3 additional infants aged 2 weeks, 3 weeks, and 7 months have undergone primary repair using this technique. All patients underwent echocardiography in the immediate postoperative period, and during the subsequent follow-up period at regular intervals. One patient who had undergone repair at age 2 weeks and weighed 2.6 kg, had evidence at one year of age of recurrent right ventricular outflow tract obstruction. This proved to be muscular obstruction, proximal to the surgically created outflow tract, and resulted in nearly systemic level pressure in the body of the right
HOW TO DO IT REDDY DANDOLU ET AL ANOMALOUS CORONARY IN TETRALOGY
1179
ventricle. This was managed by more proximal extension of the right ventricular incision, excision of the obstructing muscle bundle, and proximal augmentation of the outflow tract patch. The remainder of the previously surgically created outflow pathway was left undisturbed, and this revision resulted in a postoperative RV: LV pressure ratio of 0.4 to 1. An additional patient who had undergone repair at 3 weeks of age underwent elective cardiac catheterization at 2 years of age. Findings included right ventricular systolic pressure of 41 mm Hg, with a RV: LV pressure ratio of 0.39 to 1. The peak-topeak gradient between the body of the right ventricle and the branch pulmonary arteries was 19 mm Hg. Angiography demonstrated the double outflow from the right ventricle to the branch pulmonary arteries (Fig 3). The same patient’s most recent echocardiographic evaluation at 5 years of age revealed appropriate growth of the branch pulmonary arteries, a widely patent surgically created outflow tract, and normal right ventricular shortening with mild dilation. Estimates of right ventricular pressure based upon the velocities in the right ventricular outflow tract, and of the mild tricuspid regurgitant jet, were less than one half of the patient’s measured blood pressure. The remaining patients likewise remain free of cardiac symptoms, have age appropriate growth, and, by noninvasive follow-up with echocardiography, have remained free of recurrent right ventricular outflow tract obstruction.
Fig 2. The proximally based pulmonary artery flap is turned down and its end is sutured to the upper margin of the ventriculotomy incision. The flap must be long enough to cover the anomalous coronary artery branch without compressing it.
1180
HOW TO DO IT REDDY DANDOLU ET AL ANOMALOUS CORONARY IN TETRALOGY
Ann Thorac Surg 1999;67:1178 – 80
vide adequate decompression of the right ventricle. Theoretically it has the advantage of potential growth of the surgically created outflow pathway that has been constructed in part from a flap of viable autologous vascular tissue. This technique shares with simple transannular patching the virtual certainty of some degree of pulmonic insufficiency. During follow-up ranging from 3 to 7 years, the only reintervention required was for muscular obstruction proximal to the right ventricular incision. Although the infundibular incision need not be very large, a concerted effort should be made at the time of repair to divide potentially obstructing right ventricular muscle bundles. This technique is similar to that described in 1995 by van Son [4], to whose report we add this encouraging intermediate follow-up data.
References
Fig 3. Postoperative angiocardiographic frame showing double outflow from the right ventricle to the branch pulmonary arteries.
Comment Use of this technique has made it possible to extend the policy of primary repair of tetralogy of Fallot in symptomatic infants to those with a variety of abnormalities of coronary artery anatomy. This method appears to pro-
1. Dabizzi RP, Caprioli G, Aiazzi L, et al. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circulation 1980;61:95–102. 2. Humes RA, Driscoll DJ, Danielson GK, Puga FJ. Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options. J Thorac Cardiovasc Surg 1987;94:784–7. 3. Hurwitz RA, Smith W, King H, Girod DA, Caldwell EL. Tetralogy of Fallot with abnormal coronary artery. J Thorac Cardiovasc Surg 1980;80:129–34. 4. Van Son JA. Repair of tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. J. Thorac Cardiovasc Surg 1995;110:561– 62.
A new technique to repair tetralogy of Fallot with an anomalous coronary artery crossing the right ventricular outflow tract is described, together with intermediate term follow-up. Using a pedicled flap of the anterior pulmonary artery wall as the floor, and a vascular or prosthetic patch as the roof, a composite conduit with the potential for growth is constructed. Together with the native outflow tract, this provides unobstructed egress
from the right ventricle to the branch pulmonary arteries. Since 1990, 4 infants aged 2-weeks to 6-months have undergone primary repair using this technique. Intermediate term follow-up shows adequate durability of the repair.
A
Technique
bnormal coronary artery anatomy, with a major coronary vessel crossing the outflow tract of the right ventricle, poses special problems during repair of tetralogy of Fallot. The most common of such anatomical variants is right coronary origin of the anterior descending artery, such that the anterior descending coronary artery crosses the outflow tract of the right ventricle [1]. Less commonly, there is a single coronary artery, or right coronary origin of the circumflex artery, or dual blood supply to the anterior septum with the portion nearer the base of the heart supplied by a vessel originating from the right coronary artery, and that portion nearer the apex supplied by a branch of the left coronary artery. Most often, symptomatic infants with these anatomical variants are managed initially by creation of a systemic to pulmonary artery shunt, and subsequently by a reparative procedure using an extra-cardiac conduit [2]. Alternatively, a tailored right ventricular incision and outflow patch reconstruction [3], or a primarily transatrial approach to relief of right ventricular outflow tract obstruction have been used. Consistent with our philosophy of accomplishing primary reparative surgery in symptomatic infants with tetralogy of Fallot, we have devised a technique that uses a viable flap of autologous vascular tissue as part of the right ventricular outflow tract reconstruction, and maintains antegrade flow through the native outflow tract (when patent) as well. We hypothesized that this double outflow technique would be associated with durable relief of right ventricular outflow tract obstruction, as there is the potential for growth of the surgically created outflow pathway.
Accepted for publication Sep 16 1998. Address reprint requests to Dr Jacobs, Section of Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134.
© 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
(Ann Thorac Surg 1999;67:1178 – 80) © 1999 by The Society of Thoracic Surgeons
Coronary anatomy is carefully inspected upon opening the pericardium, and is correlated with preoperative angiographic findings. Cardiopulmonary bypass with moderate hypothermia or with deep hypothermia and circulatory arrest is used. After cardioplegic arrest, a vertical infundibulotomy incision is made beginning a few millimeters below the level of the coronary artery that crosses the right ventricular outflow tract. Hypertrophied infundibular muscle bundles are divided and resected through this incision. The malalignment type ventricular septal defect is closed with a fabric patch and continuous monofilament suture. Parallel incisions are made in the anterior portion of the main pulmonary artery and may be carried on to the proximal left pulmonary artery if needed to provide adequate length to the autologous flap. The incisions are joined distally on the main pulmonary artery, or the proximal left pulmonary artery, creating a proximally based flap of pulmonary artery tissue (Fig 1). The length of this flap should be slightly greater than the distance between the base of the flap and the upper margin of the infundibular incision in the right ventricle. When appropriate, pulmonary valvotomy is performed through the opening in the main pulmonary artery. The flap is turned down onto the anterior surface of the infundibulum of the right ventricle such that it lies over the course of the anomalous coronary artery. The end of the flap is sutured to the superior margin of the ventriculotomy using continuous monofilament suture (Fig 2). Care should be taken to ascertain that the length of the flap is sufficient that it will not be stretched over the surface of the coronary and potentially compromise coronary flow. Cryopreserved pulmonary artery tissue is thawed and washed. A portion is excised to create a generous elliptically shaped patch. Alternatively the patch can be 0003-4975/99/$20.00 PII S0003-4975(99)00115-0
Ann Thorac Surg 1999;67:1178 – 80
Fig 1. The ventriculotomy incision is begun below the coronary artery, which crosses the right ventricular outflow tract. The ventricular septal defect is closed through this incision. The broken line shows the sites of incision in the anterior wall of the main pulmonary artery.
fashioned from autologous or xenograft pericardium, or from prosthetic material. Using monofilament suture, the patch is sutured to the edges of the opened main pulmonary artery (and left pulmonary artery), then to the edges of the autologous pulmonary artery flap, and finally to the remaining edges of the infundibular ventriculotomy. This can be carried out with a Hagar dilator or other calibrating device placed beneath the patch, to calibrate the size of the surgically created outflow pathway. At the end of this procedure, the right ventricle has two outflow tracts: a presumably restrictive pathway through the infundibulum and native pulmonary valve; and the new pathway created using the flap of the pulmonary artery wall and the roof of homograft tissue. Our first use of this technique was in a 3-day-old neonate in 1990. Since then, 3 additional infants aged 2 weeks, 3 weeks, and 7 months have undergone primary repair using this technique. All patients underwent echocardiography in the immediate postoperative period, and during the subsequent follow-up period at regular intervals. One patient who had undergone repair at age 2 weeks and weighed 2.6 kg, had evidence at one year of age of recurrent right ventricular outflow tract obstruction. This proved to be muscular obstruction, proximal to the surgically created outflow tract, and resulted in nearly systemic level pressure in the body of the right
HOW TO DO IT REDDY DANDOLU ET AL ANOMALOUS CORONARY IN TETRALOGY
1179
ventricle. This was managed by more proximal extension of the right ventricular incision, excision of the obstructing muscle bundle, and proximal augmentation of the outflow tract patch. The remainder of the previously surgically created outflow pathway was left undisturbed, and this revision resulted in a postoperative RV: LV pressure ratio of 0.4 to 1. An additional patient who had undergone repair at 3 weeks of age underwent elective cardiac catheterization at 2 years of age. Findings included right ventricular systolic pressure of 41 mm Hg, with a RV: LV pressure ratio of 0.39 to 1. The peak-topeak gradient between the body of the right ventricle and the branch pulmonary arteries was 19 mm Hg. Angiography demonstrated the double outflow from the right ventricle to the branch pulmonary arteries (Fig 3). The same patient’s most recent echocardiographic evaluation at 5 years of age revealed appropriate growth of the branch pulmonary arteries, a widely patent surgically created outflow tract, and normal right ventricular shortening with mild dilation. Estimates of right ventricular pressure based upon the velocities in the right ventricular outflow tract, and of the mild tricuspid regurgitant jet, were less than one half of the patient’s measured blood pressure. The remaining patients likewise remain free of cardiac symptoms, have age appropriate growth, and, by noninvasive follow-up with echocardiography, have remained free of recurrent right ventricular outflow tract obstruction.
Fig 2. The proximally based pulmonary artery flap is turned down and its end is sutured to the upper margin of the ventriculotomy incision. The flap must be long enough to cover the anomalous coronary artery branch without compressing it.
1180
HOW TO DO IT REDDY DANDOLU ET AL ANOMALOUS CORONARY IN TETRALOGY
Ann Thorac Surg 1999;67:1178 – 80
vide adequate decompression of the right ventricle. Theoretically it has the advantage of potential growth of the surgically created outflow pathway that has been constructed in part from a flap of viable autologous vascular tissue. This technique shares with simple transannular patching the virtual certainty of some degree of pulmonic insufficiency. During follow-up ranging from 3 to 7 years, the only reintervention required was for muscular obstruction proximal to the right ventricular incision. Although the infundibular incision need not be very large, a concerted effort should be made at the time of repair to divide potentially obstructing right ventricular muscle bundles. This technique is similar to that described in 1995 by van Son [4], to whose report we add this encouraging intermediate follow-up data.
References
Fig 3. Postoperative angiocardiographic frame showing double outflow from the right ventricle to the branch pulmonary arteries.
Comment Use of this technique has made it possible to extend the policy of primary repair of tetralogy of Fallot in symptomatic infants to those with a variety of abnormalities of coronary artery anatomy. This method appears to pro-
1. Dabizzi RP, Caprioli G, Aiazzi L, et al. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circulation 1980;61:95–102. 2. Humes RA, Driscoll DJ, Danielson GK, Puga FJ. Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options. J Thorac Cardiovasc Surg 1987;94:784–7. 3. Hurwitz RA, Smith W, King H, Girod DA, Caldwell EL. Tetralogy of Fallot with abnormal coronary artery. J Thorac Cardiovasc Surg 1980;80:129–34. 4. Van Son JA. Repair of tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. J. Thorac Cardiovasc Surg 1995;110:561– 62.