Two patients underwent an arterial switch procedure for the relief of severe pulmonary venous obstruction complicating a Mustard procedure. Without preparatory pulmonary banding, both patients had adequate left ventricular function due to secondary pulmonary hypertension. At 8 and 4 years after the procedure, both patients are in New York Heart Association functional class I, with echocardiographic evidence of good left and right ventricular function

Bogers, A.J.J.C. (Ad)

Bos, E. (Egbert)

Jong, P.L. (Peter) de

Witsenburg, M. (Maarten)

Erasmus University Digital Repository

Ann Thorac Surg CASE REPORT DE JONG ET AL 1005 1995;59:1005-7 ARTERIAL SWITCH AFTER MUSTARD PROCEDURE References 1. Mork Jp, Gersony WM. Progressive atrioventricular regurgi- tation in single ventricle. Am J Cardiol 1987;59:658. 2. Yutaka O, Miki S, Kusuhara K, et al. Annuloplastic recon- struction for common atrioventricular valvular egurgitation in right isomerism. Ann Thorac Surg 1989;47:302-4. Arterial Switch for Pulmonary Venous Obstruction Complicating Mustard Procedure Peter L. de Jong, MD, Ad J. J. C. Bogers, MD, PhD, Maarten Witsenburg, MD, PhD, and Egbert Bos, MD, PhD Departments of Thoracic Surgery and Pediatric Cardiology, Sophia/Dijkzigt University Hospital, Rotterdam, the Netherlands Two patients underwent an arterial switch procedure for the relief of severe pulmonary venous obstruction com- plicating a Mustard procedure. Without preparatory pul- monary banding, both patients had adequate left ventric- ular function due to secondary pulmonary hypertension. At 8 and 4 years after the procedure, both patients are in New York Heart Association functional class I, with echocardiographic evidence of good left and right ven- tricular function. (Ann Thorac Surg 1995;59:1005-7) C urrently, the arterial switch operation is the opera- tion of choice for transposition of the great arteries [1]. In earlier times, atrial rerouting as in the Mustard procedure, was performed with good early and medium- term results. However, right ventricular dysfunction may be troublesome in the long term [2, 3]. The pulmonary venous obstruction that can arise after a Mustard proce- dure is a serious complication [1, 41. Elimination of the obstruction through the placement of a patch carries the risk of restenosis. To overcome the risks of recurrent pulmonary venous obstruction after atrial repair and the long-term complications of the atrial switch procedure, an arterial switch operation should be considered, espe- cially in patients with secondary pulmonary hyperten- sion due to pulmonary venous obstruction. These pa- tients may have a well-trained left ventricle that is capable of support ing the systemic irculation. Two pa- tients underwent a single-stage conversion from a Mus- tard construction to an arterial switch for the relief of Accepted for publication Aug 3, 1994. Address reprint requests o Dr Bogers, Department of Thoracic Surgery, Thoraxcentre, Bd156, Dijkzigt University Hospital, Dr. Molewaterplein 40, 3015 GD Rotterdam, the Netherlands. Table I. Variables Before Arterial Switch Procedure Variable Patient 1 Patient 2 Weight (kg) Electrocardiogram Biventricular Biventricular hypertrophy hypertrophy Echocardiography Diastolic left 8 (95th 6 (75th ventricular percentile) percentile) posterior wall thickness (ram) Shortening fraction 0.30 0.29 Cardiac catheterization Ejection fraction 0.70 0.65 Left ventricular 80/5-10 71/1-7 pressure (mm Hg) Pulmonary artery 69/36 68/39 pressure (ram Hg) Mean capillary wedge 29 30 pressure (mm Hg) Right ventricular 91/0-10 95/1-8 pressure (rnm Hg) Left ventricular-to- 0.88 0.75 right ventricular ratio symptomatic pulmonary venous obstruction, without the need for preparatory pulmonary artery banding. Case Reports Patient 1 This patient underwent a Mustard procedure in 1976 at the age of 6 weeks for the repair of transposit ion of the great arteries with an intact ventricular septum and atrial septum defect, after unsuccessful balloon atrial septos- tomy. A Mustard procedure with a Teflon baffle was performed using hypothermia and circulatory arrest. The patient was reoperated on twice in the first year of life because of pulmonary venous obstruction. In the first reoperation, a dura mater patch was placed. The second reoperation involved the use of a Teflon patch. In 1985, obstruction recurred with secondary pulmonary hyper- tension. This caused the patient o be in New York Heart Association functional class IV and ventilator dependent.  Preoperative lectrocardiography showed biventricular hypertrophy. Echocardiography showed dilatation of the pulmonary veins. The size of the left (pulmonary) ventri- cle was equal to that of the right (systemic) ventricle, with the left ventricular posterior wall thickness and shorten- ing fraction within normal imits for a systemic ventricle. These findings suggested the existence of severe left ventricular pressure overload (Table 1). Cardiac catheter- ization showed a high pulmonary capillary wedge pres- sure, elevated left ventricular and pulmonary arterial pressures, and good left ventricular contractility (see Table 1). After left femoral artery cannulation and venous can- nulation of the right pulmonary artery, because of the © 1995 by The Society of Thoracic Surgeons 0003-4975/95/$9.50 0003-4975(94}00736-Q 1006 CASE REPORT DE JONG ET AL Ann Thorac Surg ARTERIAL SWITCH AFTER MUSTARD PROCEDURE 1995;59:1005-7 inaccessibility of the caval veins, extracorporeal circula- tion was started. Under conditions of deep hypothermia (16°C) and circulatory arrest, an arterial switch using the Lecompte procedure was performed [5]. After excision of the Teflon patch placed during the Mustard procedure, an atrial septum was reconstructed using a USCI patch (C.R. Bard, Billerica, MA). The patient's postoperative course was complicated by a cerebrovascular ccident that left her with temporary hemiplegia nd pseudobul-  bar paresis. After a long period of rehabilitation, she recovered completely. Eight years later she is in New York Heart Association functional class I. The electrocar- diogram shows a junctional rhythm, and echocardiogra- phy shows normal left ventricular function, with still some right ventricular hypertrophy and mild aortic in- sufficiency. Patient 2 This patient was admitted with persisting cyanosis after birth, and the diagnosis of transposition of the great arteries, an intact ventricular septum, and open ductus arteriosus was made. A balloon atrial septostomy was performed 2 days after birth. In 1985, at the age of 4 months, the infant underwent a Mustard procedure un- der circulatory arrest and hypothermia that involved the use of a Teflon baffle. In 1988 exertional dyspnea with a period of pneumonia occurred. Echocardiography re- vealed distended pulmonary veins and severe narrowing of the communication to the pulmonary venous atrium. The size of the left ventricle was almost equal to that of the right, and both the shortening fraction and the left ventricular posterior wall thickness were within the nor- mal limits for a systemic ventricle (see Table 1). Cardiac catheterization showed pulmonary venous obstruction with elevated left ventricular and pulmonary arterial pressures due to secondary pulmonary hypertension. Left ventricular contractility was good (see Table 1). Subsequently, an arterial switch was performed. With the patient on extracorporeal circulation and under con- ditions of hypothermia (20°C) and cardioplegic arrest, an arterial switch procedure was done. The Teflon baffle was excised and the atrial septum was reconstructed with a Gore-Tex patch (W. L. Gore, Elkton, MD). The patient had an uneventful postoperative course, and 4 years later he is in excellent clinical condition (New York Heart Association functional class I) and in sinus rhythm. Echocardiography has confirmed that left and right ventricular contractility is normal, but there is trivial aortic regurgitation. Comment Before the era of anatomic repair procedures, children with transposition of the great arteries were treated with a physiologic repair that involved redirecting the sys- temic and pulmonary flow at the atrial level [1, 6]. In the treatment of transposit ion with an intact ventricular septum, the atrial switch operation has been found to be associated with a minimal early mortality at many cen- ters, and the actuarial survival results over the first 10 years have been excellent. However, late complications such as rhythm disturbances, right ventricular dysfunc- tion, tricuspid valve incompetence, and sudden death have been reported [2, 3, 7]. This possible long-term outcome after atrial repair is a major argument in favor of the arterial switch procedure. The arterial switch repair can be accomplished with equally good or even better early results and better intermediate r sults [1, 8]. This is the basis for considering an arterial switch procedure when managing the complications of the atrial switch operation. Cochrane and associates [2] and Mee [3] described the technique for converting from an atrial switch to an arterial switch for the treatment of right ventricular failure. This was done in two stages, with the first stage consisting of banding the pulmonary artery to retrain the left ventricle. Once adequate left ventricular pressure developed, the arterial switch operation was performed. We describe a one-stage procedure of converting from an atrial switch to an arterial switch for the treatment of pulmonary venous obstruction. Pulmonary venous ob- struction is a serious complication after a Mustard atrial switch [1, 4]. Repair using a patch may result in resteno- sis, as i l lustrated by patient 1. The pulmonary venous obstruction with secondary pulmonary hypertension may cause the left ventricle to be well trained and thus able to support he systemic irculation without the need for preparatory pulmonary artery banding, as was shown in both our patients (see Table 1). Echocardiography appeared to be a very valuable tool in the selection of candidates for the one-stage approach. After a successful atrial repair for transposition of the great arteries, the left ventricle is flattened due to its low systemic pressure. Echocardiography showed that, in both our patients, the left and right ventricular volumes were comparable. Left ventricular posterior wall thickness had increased to within normal limits for a systemic ventricle. The left ventricular posterior wall shortening fraction was in the lower normal range. However, we believe that the deci- sion to perform a one-stage repair in this setting can only be made adequately with invasively obtained hemody- namic data, using as the cutoff a minimal eft ventricular- to-right ventricular pressure ratio of 0.75, as suggested by Mee [3] and Cochrane and colleagues [2]. We conclude that the arterial switch operation is fea- sible for correction of pulmonary venous obstruction after a Mustard procedure. In this regard, we think that the one-stage arterial switch operation is the operation of choice in patients with adequate left ventricular function. References 1. Kirklin JW, Barratt-Boyes BG. Cardiac surgery, 2nd ed. New York: Churchill Livingstone, 1993:1383-467. 2. Cochrane AD, Karl TR, Mee RBB. Staged conversion to arterial switch for late failure of the systemic right ventricle. Ann Thorac Surg 1993;56:854-62. Ann Thorac Surg CASE REPORT DELEON ET AL 1007 1995;59:1007-10 ROSS PROCEDURE MODIFICATIONS 3. Mee RBB. Severe right ventricular failure after Mustard or Senning operation. J Thorac Cardiovasc Surg 1986;92:385-90. 4. Driscoll DJ, Nihill MR, Vargo TA, Mullins CE, McNamara DG. Late development of pulmonary venous obstruction following Mustard's operation using a Dacron baffle. Circu- lation 1977;55:484-8. 5. Lecompte Y, Zannini L, Hazan E, et al. Anatomic orrection of transposition of the great arteries. J Thorac Cardiovasc Surg 1981;82:629-31. 6. Mustard WT. Successful two-stage correction of transposition of the great vessels. Surgery 1964;55:469-72. 7. Williams WG, Trusler GA, Kirklin JW, et al. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988;95:717-26. 8. Norwood WI, Dobell AR, Freed MD, Kirklin JW, Blackstone EH, the Congenital Heart Surgeons Society. Intermediate results of the arterial switch repair. J Thorac Cardiovasc Surg 1988;96:854-63. INV ITED COMMENTARY This article reports 2 patients who underwent late arterial switch repair after a Mustard procedure performed in infancy, and in whom pulmonary venous obstruction with secondary pulmonary hypertension developed. Be- cause the left ventricular pressure was high, it was not necessary to carry out prel iminary pulmonary artery banding. This is an interesting and novel approach to the man- agement of patients with pulmonary venous obstruction after a Mustard procedure, and fits well with our philos- ophy of recommending conversion to an arterial switch for all patients who have undergone Senning or Mustard procedures, and who clearly require further revisional operations in whom conversion to an arterial switch is a feasible opt ion- -e i ther  as a one-stage or two-stage pro- cedure. In these patients, the presence of well-main- tained right ventricular function puts them into a differ- ent category from that of many patients who become symptomatic and need to be considered for further operation, which, if an arterial switch is not feasible, may well involve transplantation. The fact that the Mustard baffle can be removed in its entirety as part of the conversion to an arterial switch, likely makes it the most satisfactory long-term solution to the problem of pulmo- nary venous obstruct ion--which in 1 of the 2 patients described recurred despite two previous attempts at revision. James L. Wilkinson, FACC Director of Cardiology Victorian Paediatric Cardiac Surgical Unit Royal Children's Hospital Flemington Rd Parkville, Victoria, 3052 Australia Use of the Native Aortic Valve as the Pulmonary Valve in the Ross Procedure Serafin Y. DeLeon, MD, Jose A. Quinones, MD, Ronald H. Miles, MD, Joanne Hofstra, MS, Timothy J. Bell, MD, Elizabeth A. Fisher, MD, and Roque Pifarr6, MD Departments of Thoracic-Cardiovascular Su gery and Pediatrics, Loyola University Medical Center, Maywood, Illinois The placement of a foreign valve in the pulmonary position using the Ross procedure requires reoperation. To circumvent this problem, we devised a method of reimplanting the native aortic valve in the pulmonary position, and successfully performed this procedure in a 12-year-old iabetic boy operated on for the treatment of aortic insufficiency. Although diseased, the reimplanted aortic valve functioned well, with trivial stenosis and insufficiency. This modification offers patients with aor- tic valve disease a potentially curative operation. (Ann Thorac Surg 1995;59:1007-10) T he Ross procedure requires the implantation of a foreign valve in the pulmonary position using a valve that has longevity. Currently, a cryopreserved ho- mograft is preferred for this purpose, although other materials uch as autologous fascia lata and pericardial valves have been used [1]. Valveless conduits also have been used successfully in the pulmonary position, but right ventricular dysfunction is a consequence of this. Although cryopreserved homografts are preferred and last longer in the pulmonary position, replacement nev- ertheless will be necessary [2]. Early failures have also been reported and have been attributed to possible immunologic rejection [3]. To circumvent the need for pulmonary valve replace- ment with the Ross procedure, we devised a method of implanting the native aortic valve in the pulmonary position. This was based on our belief that a diseased aortic valve, especially when the main problem is aortic insufficiency, should function reasonably well in the pulmonary position because of the lower pulmonary vascular pressure and resistance involved. We report on our experience with this method. A 12-year-old boy was referred to us for the treatment of congenital aortic insufficiency. Cardiac catheterization performed when he was 4 years old had shown a tricus- pid aortic valve with mild incompetence. The aortic insufficiency had worsened considerably over the years. Accepted for publication Aug 5, 1994. Address reprint requests to Dr DeLeon, Loyola Universi~ Medical Center, 2160 S First Ave, Maywood, IL 60153. © 1995 by The Society of Thoracic Surgeons 0003-4975/95]$9.50 0003-4975(94)00738-S 

Arterial switch for pulmonary venous obstruction complicating mustard procedure

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Arterial switch for pulmonary venous obstruction complicating mustard procedure

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