Valved Conduit Repair
Operations are carried out with cardiopulmonary bypass, moderate hypothermia, aortic clamping, cardioplegia, and profound local cardiac cooling. In most cases, repair is performed with a valved conduit placed in the right of the heart otherwise residual pulmonary hypertension that may be present after surgery may result in significant pulmonary regurgitation and right heart failure. 17-1-1. Repair with Porcine-Valved Conduit These conduits are readily available in various sizes however, the...
Apical Left Ventricle to Ascending Aorta Conduit
In small infants, when an apical-aortic conduit is required, there may be inadequate space in which to work to attach the conduit to the descending aorta when working through a median sternotomy. Here the conduit is attached to the ascending aorta. Figure 14-74. Through a median sternotomy, a small ascending aorta is seen. The procedure is performed with cardiopulmonary bypass, moderate hypothermia, aortic clamping, and cardioplegic arrest plus profound local cooling. Figure 14-74. Through a...
Supramitral Ring
In the presence of this anomaly, a fibrous membrane located immediately above the mitral valve annulus causes symptoms similar to mitral stenosis. The pulmonary veins and left atrial appendage enter the left atrium above the membrane. Figure 13-8. After the cardiopulmonary bypass is established, the left atrium along the right heart border is entered. A thick membrane is present immediately above the mitral valve, which is not yet visualized. Figure 13-8. After the cardiopulmonary bypass is...
Muscular Ventricular Septal Defect
These defects can occur in any part of the muscular ventricular septum. Preoperative definition of the number of VSDs and the precise location of each by echocardiography or angiography is useful to the surgeon at the time of repair. The best exposure for most muscular defects is through a right atriotomy. An unusual location may warrant a high or an apical right ventriculotomy. In the presence of multiple low VSDs an apical left ventriculotomy may be optimal. Figure 5-34. Cardiopulmonary...
DoubleSwitch Operation
When a VSD is present, the double-switch operation is becoming popular for anatomic repair of corrected transposition. With both a venous switch procedure and a ventriculoarterial connection switch, the morphologic left ventricle is placed in the systemic circulation and the morphologic right ventricle in the pulmonary circulation. I prefer the Senning operation for the venous switch over the Mustard procedure in that the former uses natural landmarks to position various suture lines. This...
With a Ventricular Septal Defect and Pulmonic Stenosis
Figure 16-1. The ascending aorta is anterior and toward the left, while the main pulmonary artery is posterior and toward the right. The right coronary artery is located on the pulmonary ventricle outflow tract. Figure 16-1. The ascending aorta is anterior and toward the left, while the main pulmonary artery is posterior and toward the right. The right coronary artery is located on the pulmonary ventricle outflow tract. Figure 16-2. Looking through a right atriotomy the right-sided mitral valve...
Obstruction of Bulboventricular Foramen
In the presence of single ventricle with d- or 1-transposition of the great arteries and obstruction of a bulboventricular foramen that separates the ventricle and aorta, ventricular hypertension occurs that must be relieved to preserve ventricular function. Two techniques are used. In the first, the proximal-divided main pulmonary is attached to the ascending aorta Damus procedure , creating a double outlet ventricle to bypass the obstruction. With the second technique, subaortic obstruction...
Right Superior Vena Cava Draining to Roof of Left Atrium
The preoperative diagnosis in this patient was sinus venosus ASD with possible partial anomalous pulmonary venous connection. Figure 1-6. The child has been placed on cardiopulmonary bypass and the superior vena cava is cannulated directly and remotely from the caval atrial junction. An atriotomy is made in the mid right atrium. The ASD is identified in the high lateral septum in the sinus venosus region. The right upper and middle pulmonary veins were seen draining to the left atrium...
Recruitment of Multiple Anomalous Collateral Arteries
In infants born with severe hypoplasia or absence of the pulmonary arteries, the lungs are usually supplied by multiple collateral arteries. Recruitment of these to construct branch pulmonary arteries can be followed by total repair in many cases. For most, staged thoracotomies offer the best chance of successful pulmonary artery reconstruction. The exposure is optimal through a lateral approach and this allows for precise anastomoses and recruitment of all anomalous vessels. A Blalock shunt is...
Cor Triatriatum
In the presence of this rare anomaly, a membrane separates the left atrium into two chambers. The proximal chamber communicates with the pulmonary veins, while the distal chamber communicates with the atrial appendage and mitral valve. orifice to distal chamber of left atrium Figure 13-1. After the cardiopulmonary bypass is established, the dilated proximal left atrial chamber is entered along the right heart border. Pulmonary veins enter this chamber, which is separated from the distal chamber...
Rastelli Operation
This procedure is used in the correction of d-transposition of the great arteries associated with a ventricular septal defect VSD and pulmonary stenosis. By working through the right ventricle, a patch is placed over the VSD and the aortic valve to create a tunnel to establish flow from the left ventricle through the VSD and into the aorta. The procedure is usually not performed in small infants, because an adequate working area is needed for the extensive tunneling procedure in the right...
Color Atlas of Congenital Heart Surgery
Director Emeritus, Cardiothoracic Surgery, Herma Heart Center, Children's Hospital of Wisconsin Clinical Professor of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA S. Bert Litwin, MD Director Emeritus Cardiothoracic Surgery Herma Heart Center Children's Hospital of Wisconsin and Clinical Professor of Surgery Medical College of Wisconsin Milwaukee, WI USA Library of Congress Control Number 2006930105 ISBN-10 0-387-35415-8 e-ISBN-10 0-387-49925-3 ISBN-13 978-0-387-35415-6...
Supravalvar Stenosis
In the presence of this anomaly, there is usually a severe stenosing ring, at or immediately above the aortic valve commissures. A simple incision across the area of stenosis with patch angioplasty is often inadequate to relieve the obstruction. The stenosing ring must be incised into at least two sinuses of Valsalva. Three effective repair techniques are described. Figure 14-29. An external view of the heart shows the narrow proximal ascending aorta at the site of supravalvar stenosis. Figure...
Arterial Switch Jatene Operation
Arterial switch repair is the preferred operation for d-transposition of the great arteries without severe anatomic left ventricular outflow tract narrowing or when such narrowing can be treated with resection. Other surgical repairs are rarely performed. The traditional contraindication to its use is the presence of an abnormal pulmonary valve, which would become the aortic valve after repair, although today one would consider combining a form of the Ross procedure and arterial switch...
Valvular Pulmonary Stenosis
Pulmonary valvotomy is performed using cardiopulmonary bypass, including left ventricular venting and aortic cross-clamping although in unusual circumstances, as in very small neonates, it is most expeditious to avoid the use of a left ventricular vent. In these cases, care must be taken not to allow air from the opened right heart to enter the left heart through an intracardiac communication as a patent foramen ovale. Aortic clamping may also be optional but its use helps in reducing blood...
Pulmonary Artery Banding and Band Removal at Subsequent Total Repair
In the presence of a large left-to-right shunt, pulmonary artery banding may be indicated to protect the lungs and to avoid development of pulmonary vascular obstructive disease when total repair of intracardiac anomalies is not possible or should be delayed until an older age. Banding can be performed through a left or right thoracotomy or median sternotomy, regardless of the position of the great vessels. Ideally, it is carried out through a fourth interspace lateral thoracotomy on the side...
Pulmonary Vein Stenosis
This rare anomaly occurs at the entrance of pulmonary veins to the left atrium. Because of the severe consequences of pulmonary venous hypertension, including congestive heart failure and or pulmonary vascular obstructive disease, surgery to repair this lesion is indicated immediately after discovery, regardless of the age of the patient. Direct repair of the pulmonary vein is performed in the presence of mild-to-moderate stenosis. An alternative repair is sutureless with stitches placed in...
Aortic Root Enlargement Procedures
Figure 14-47. With cardiopulmonary bypass, aortic clamping, and cardioplegia with profound local cardiac cooling, a proximal ascending aortotomy is made. A bicuspid stenotic and dysplastic valve is seen. After it is determined that valvuloplasty is not possible, the valve leaflets are excised. Figure 14-47. With cardiopulmonary bypass, aortic clamping, and cardioplegia with profound local cardiac cooling, a proximal ascending aortotomy is made. A bicuspid stenotic and dysplastic valve is seen....
Ventricular Septum
Infants born with this anomaly have inadequate pulmonary blood flow caused by pulmonary valve atresia and severe right ventricular hypertension, unless tricuspid insufficiency allows decompression of the ventricle. Initial resuscitation includes maintenance of ductus arteriosus patency with prostaglandin E Right ventricular size may vary from severe hypoplasia to a near normal size, depending on when in fetal development the pulmonary valve became atretic. If it was late in cardiac development,...
Total Repair
Figure 11-1. Working through a median sternotomy, cardiopulmonary bypass is established. Repair is carried out with aortic clamping, cardioplegia, and profound local cardiac cooling. A high longitudinal right ventriculotomy is made extending cephalad into the outflow tract chamber which is atretic at its upper end. The ventricular septal defect VSD is subcristal and nonrestrictive. Figure 11-1. Working through a median sternotomy, cardiopulmonary bypass is established. Repair is carried out...
Palliation and Repair of Tetralogy of Fallot with Absent Pulmonary Valve
Infants born with this rare syndrome may develop aneurysmal dilatation of the main pulmonary artery and or its branches this may cause tracheal and bronchial obstruction resulting in severe respiratory distress. In the rare case when totally corrective surgery cannot be undertaken, airway obstruction may be relieved by translocating the offending right or left pulmonary artery from the posterior to the anterior mediastinum. This operation is largely of historic interest but emphasizes the...
Repair of Shunts
Previously constructed systemic-to-pulmonary artery shunts must be closed at the time of total corrective surgery. 9-5-1. Classic Right Blalock-Taussig Shunt Figure 9-51. The subclavian artery trunk of the right Blalock-Taussig shunt is usually easy to identify medial to the superior vena cava while working within the pericardial space. Figure 9-51. The subclavian artery trunk of the right Blalock-Taussig shunt is usually easy to identify medial to the superior vena cava while working within...
FirstStage Fontan Procedure
Before cardiopulmonary bypass, extensive dissection is performed. The ascending aorta and main pulmonary artery are freed from surrounding tissue to allow division of the proximal main pulmonary artery. The left pulmonary artery is mobilized, and the right pulmonary artery is dissected to facilitate the superior vena cava-right pulmonary arterial anastomosis. The entire superior vena cava is dissected to near the inominate vein, allowing high caval cannulation and lower caval exposure for the...
Subaortic Stenosis Fibromuscular Obstruction
Figure 14-5. Repair of this anomaly is performed with cardiopulmonary bypass and aortic clamping, cardioplegia, and profound local cardiac cooling, working through a proximal ascending aortotomy. Typically, the aortic valve is normal as seen here. There are three leaflets without commissural stenosis. Figure 14-5. Repair of this anomaly is performed with cardiopulmonary bypass and aortic clamping, cardioplegia, and profound local cardiac cooling, working through a proximal ascending aortotomy....
Late Pulmonary Valve Placement after Tetralogy Repair with a Transannular Patch
Placement of a transannular patch is necessary in some patients during tetralogy repair but this results in pulmonary regurgitation. Although many patients tolerate this indefinitely, 30 to 40 will require later valve placement. A pulmonary homograft valved conduit is the replacement of choice. Indications for surgery include progressive right ventricular dilatation and dysfunction, paradoxical septal motion, and flattening of the septum, which may result in left ventricular dysfunction and or...
Isolated Secundum Atrial Septal Defect
Figure 2-1. The child has been placed on cardiopulmonary bypass, and a mid right atriotomy is made. The region of the cavoatrial junction is spared to avoid injury to the sinoatrial S A node. A large secundum atrial septal defect can be seen in the region of the fossa ovalis. A metal sucker is in the defect. Because the septal tissue that surrounds the ASD is substantial, it can be closed primarily. It is important to inspect the atrial chambers to rule out other anomalies and the orifices of...
Total Anomalous Pulmonary Venous Connection
Total repair is carried out soon after establishing the diagnosis and medical stabilization of the patient. The procedure can be emergent shortly after birth when there is obstruction of the common pulmonary venous channel as with a subdiaphragmatic connection , or in the early days of life when there is obstruction at the atrial septal level supracardiac or intracardiac connection . When there is no obstruction to pulmonary venous return, surgery is required in the early weeks of life because...
Total Repair of Simple Tetralogy of Fallot Transventricular
Surgery is carried out with cardiopulmonary bypass, moderate hypothermia, aortic clamping with cardioplegia, and profound local cardiac cooling. Repair of pulmonary stenosis and the ventricular septal defect VSD are performed through a short high right ventriculotomy in the infundibulum, which is positioned to avoid division of coronary branches. If the pulmonary valve annulus is restrictive and a transannular patch is anticipated, a longitudinal or oblique ventriculotomy is made that is...
Infundibular Stenosis and Double Chamber Right Ventricle
Repair is performed with cardiopulmonary bypass, aortic clamping, cardioplegic arrest, and profound local cardiac cooling. Figure 7-3. In this patient, after cardiopulmonary bypass is established, a proximal main pulmonary arteriotomy is made. There are three leaflets with stenotic commissures, and the valve is dysplastic. All leaflets are fibrous with limited mobility. Incising the stenotic commissures alone will not relieve the obstruction, because the leaflets are bulky and will not...
Palliative Shunts and Outflow Tract Reconstruction
9-1-1. Classic Blalock-Taussig Subclavian Artery to Pulmonary Artery Shunt This operation is rarely performed because most surgeons prefer the more predictable modified Blalock shunt. It may still be useful in cases when the shunt is the end point of treatment because the subclavian artery trunk will grow with the patient. This procedure is ideally performed by working through a lateral fourth intercostal space thoracotomy on the side ipsilateral to the innominate artery. Postoperative...
Double Outlet Right Ventricle
Figure 10-1. In this infant, the great vessels and semilunar valves are oriented side by side and at the same level. Figure 10-2. After placing the child on bypass with aortic clamping and cardioplegia, a high longitudinal right ventriculotomy is made and extended cephalad into the proximal main pulmonary artery. There is a common annulus between the semilunar valves and both arise from the right ventricle. The ventricular septal defect is subaortic. Figure 10-2. After placing the child on...
Primum Atrial Septal Defect
ceph a tad rim of atrial septal defect mitral chord supports cephaiad part of anterior mitral leaflet cleft in mitral leaflet extends to annulus thickening along margin of mitral valve cleft Figure 4-1. The patient is on cardiopulmonary bypass, and a right atriotomy has been made. The low primum atrial septal defect ASD allows excellent exposure of the anterior mitral leaflet, which is lifted into the field. There is a cleft that separates the leaflet into cephaiad and caudad components, and it...
Valvular Stenosis
Figure 14-1. This photograph shows severe valvular stenosis as it occurs in a newborn. There is a unicuspid, horseshoe-shaped leaflet with a single posterior commissure. Leaflet tissue is thick, fibrous, and myxomatous. Successful valvotomy can be performed by creating a second commissure. Significant aortic regurgitation is rare due to the fibrous nature of the valve that prevents redundancy. Although typically there is residual stenosis after surgery, many infants will do surprisingly well...
Repair with Conus Left Anterior Descending Coronary Artery
A critical part of this repair is relief of infundibular pulmonary stenosis without damage to the left anterior descending coronary artery which traverses the right ventricular outflow tract after originating from the main right coronary. Division of the coronary vessel followed by aorta to coronary artery bypass grafting is a poor option in children because of the small size and the lack of growth potential of currently acceptable grafts. Depending on the location of the abnormal coronary...
Subpulmonary Ventricular Septal Defect
Subpulmonary ventricular septal defects are located high in the ventricular septum and immediately below the pulmonary valve. Myocardial relaxation with cardioplegic arrest allows the upper septum to be retracted inferiorly, so that many subpulmonary defects can be closed completely through the transatrial approach. In many cases, an aortic valve cusp is intimate with the rim of this ventricular defect and care must be used to avoid damage to the valve. If exposure through the atrium is not...
Repair of Hypoplastic Right Ventricular Outflow Tract
Although there are advantages and disadvantages of outflow tract patching across the pulmonary valve annulus, adequate relief of outflow tract blockage is an important component of total repair operations for tetralogy of Fallot. A competent pulmonary valve with minimal or no residual outflow tract blockage is ideal. Significant residual pulmonary stenosis results in increased morbidity and mortality, so that a transanular patch is indicated when the valve annulus is severely restrictive....
Inlet Ventricular Septal Defect
The inlet ventricular septal defect is located in the endocardial cushion position and is also known as a perimembranous inlet or Type III ventricular septal defect. Figure 4-12. The septal and anterior tricuspid valve leaflets are retracted to expose the VSD. The defect is located primarily beneath the septal leaflet. This position places it caudad to the location of a typical Type II or perimembranous VSD. Exposure through the right atrium is enhanced due to the more caudad location of the...
Anomalous Systemic Venous Return
Abnormal connections between the inferior vena cava, or right or left superior venae cavae to the right or left atrium may occur. A right-side anomaly e.g., persistent left superior vena cava to the coronary sinus or azygos continuation of the inferior vena cava may be of no consequence and require no treatment, although when other intracardiac anomalies require repair, the right-side abnormality may require an alteration in cardiopulmonary bypass cannulation techniques. When systemic veins...
Tricuspid Valve Anomalies
In the pediatric population, surgical repair of the tricuspid valve may be required for tricuspid insufficiency or stenosis, Ebstein's anomaly, or other rare lesions. Operations are carried out working through a right atriotomy using cardiopulmonary bypass, moderate hypothermia 26 C , aortic cross-clamping and cardioplegia, profound local cardiac cooling, and left ventricular venting. Children with tricuspid atresia require single ventricle surgery and this is covered in Chapter 6. Figure 3-1....
Complete Atrioventricularis Communis or AtrioVentricular Canal
ventricular septal defect beneath AV valve leaflets Figure 4-15. Exposure is through a right atriotomy. A primum ASD is in the lower or caudad part of the atrial septum, and it is in continuity with a high VSD located beneath the flaccid AV valve leaflets. In this child, a secundum ASD is also present. ventricular septal defect beneath AV valve leaflets Figure 4-15. Exposure is through a right atriotomy. A primum ASD is in the lower or caudad part of the atrial septum, and it is in continuity...
Common Atrium
Figure 4-8. The child has been placed on cardiopulmonary bypass, and the view is looking through a right atriotomy. There is no atrial septum, and the orifices of the left atrial appendage and right pulmonary veins are seen in the posterior wall of the common atrial chamber. The mitral valve is anterior to the left atrial appendage. Figure 4-8. The child has been placed on cardiopulmonary bypass, and the view is looking through a right atriotomy. There is no atrial septum, and the orifices of...
Atrial Septal Defect with Partial Anomalous Pulmonary Venous Connection
When partial anomalous pulmonary venous connection is present, abnormal pulmonary veins from the right upper and middle lobes commonly drain to the superior vena cava or the right atrium near the caval atrial junction. This is usually associated with a sinus venosus ASD located opposite the anomalous pulmonary veins. Figure 2-11. Anomalous pulmonary veins are seen entering the high and lateral right atrium and low superior vena cava adjacent to the cava atrial junction. Figure 2-11. Anomalous...
Left Superior Vena Cava Draining to Roof of Left Atrium
cannula in anomalous eft superior vena cava Figure 1-1. The patient is on cardiopulmonary bypass and cannulae are placed in the right superior and inferior venae cavae. The right atrium is opened, and a large secundum atrial septal defect ASD is seen. A third caval cannula passes through the ASD and is placed in the left superior vena cava, which enters the upper posterior wall of the left atrium. Figure 1-1. The patient is on cardiopulmonary bypass and cannulae are placed in the right superior...
and Techniques
Some techniques and principles of operative exposure and cardiopulmonary bypass that I currently prefer are worth mentioning. No doubt different techniques used by other surgeons are just as effective in their hands. Most open heart operations are performed through a standard median sternotomy with a longitudinal skin incision. For female patients of all ages who undergo simpler operations and in whom I do not anticipate a repeat sternotomy or the need to leave the sternum open, my preference...
Preface
Because of the wide variety of anomalies encountered in congenital heart surgery, a broad understanding of the pathologic anatomy of defects is vitally important to the surgeon. More than in many other fields of surgery, a feel for three-dimensional spatial relationships of anomalies is helpful in allowing the operating surgeon to improvise technical details of a procedure. Precisely shaping and sizing an intraventricular baffle or patch, or correctly placing a long intra-atrial suture can make...