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Arterial Switch Operation Surgery Surgical Solutions to Complex Problems

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Arterial Switch Operation Surgery Surgical Solutions to Complex Problems Pediatric Cardiovascular Arterial Switch Operation Surgery Surgical Solutions to Complex Problems Tom R. Karl, MS, MD The arterial switch operation is appropriate treatment for most forms of transposition of Andrew Cochrane, FRACS the great arteries. In this review we analyze indications, techniques, and outcome for Christian P.R. Brizard, MD various subsets of patients with transposition of the great arteries, including those with an intact septum beyond 21 days of age, intramural coronary arteries, aortic arch ob- struction, the Taussig-Bing anomaly, discordant (corrected) transposition, transposition of the great arteries with left ventricular outflow tract obstruction, and univentricular hearts with transposition of the great arteries and subaortic stenosis. (Tex Heart Inst J 1997;24:322-33) T ransposition of the great arteries (TGA) is a prototypical lesion for pediat- ric cardiac surgeons, a lethal malformation that can often be converted (with a single operation) to a nearly normal heart. The arterial switch operation (ASO) has evolved to become the treatment of choice for most forms of TGA, and success with this operation has become a standard by which pediatric cardiac surgical units are judged. This is appropriate, because without expertise in neonatal anesthetic management, perfusion, intensive care, cardiology, and surgery, consistently good results are impossible to achieve. Surgical Anatomy of TGA In the broad sense, the term "TGA" describes any heart with a discordant ven- triculoatrial (VA) connection (aorta from right ventricle, pulmonary artery from left ventricle). The anatomic diagnosis is further defined by the intracardiac fea- tures. Most frequently, TGA is used to describe the solitus/concordant/discordant heart. A heart with an additional discordant atrioventricular (AV) connection is termed "congenitally corrected" or "discordant" TGA. The Taussig-Bing anomaly is a double-outlet right ventricle with a subpulmonic ventricular septal defect (VSD). Strictly speaking, a heart with a double-outlet right ventricle does not have TGA, because 1 VA connection is concordant. However, anatomically and physi- ologically, the Taussig-Bing anomaly closely resembles TGA with a VSD and is Section editor: appropriately included in this discussion.' Transposition of the great arteries may Timothy F Feltes, MD also describe hearts with a univentricular AV connection (e.g., double-inlet left ventricle, tricuspid atresia) if an outlet chamber of right ventricular morphology Key words: Coronary vessel anomalies/surgery; heart gives rise to the aorta. The "TGA family" is illustrated in Figure 1. defects, congenital/surgery; Although the orientation of the great arteries varies considerably in TGA, a heart surgery/methods; nearly constant feature is that the coronary arteries arise from the aortic sinuses infant; infant, newborn; transposition of great that face the pulmonary artery, which greatly facilitates an ASO.2 The extraaortic vessels/surgery; vascular course of the coronary arteries is variable (Fig. 2), and the origin of 1 or both surgery/methods vessels may rarely be intramural and stenotic, posing special problems for the surgeon during an ASO.3'4 From: The Cardiac Surgical Unit, Royal Diagnosis of TGA Children's Hospital, Melbourne, Australia The diagnosis of TGA could be considered in any cyanotic newborn, but elec- trocardiogram, chest x-ray, and physical examination are not diagnostic. Two- dimensional echocardiography usually provides rapid and complete noninvasive Address for reprints: Tom R. Karl, MS, MD, diagnosis, so in the neonate, cardiac catheterization is rarely necessary. A weak Director, Victorian Pediatric area in imaging is the proximal coronary arteries, which may be difficult to accu- Cardiac Surgical Unit, rately describe with echocardiography or angiography.4 Prenatal echocardiographic Royal Children's Hospital, Flemington Road, Parkville diagnosis of TGA is now possible as well, although treatment remains problem- VIC 3052, Australia atic.5 322 Arterial Switch Volume 24, Number 4, 1997 ccTGA.IVS ccTGA.VSD (+l-LVOTO) Fig. 1 The transposition of the great arteries "family, " which includes all hearts with a discordant ventriculoarterial connection as well as the Taussig-Bing anomaly (double-outlet right ventricle and subpulmonic ventricular septal defect). AAO = aortic arch obstruction; Ao = aorta; AV = atrioventricular; cc = congenitally corrected; DORV = double-outlet right ventricle; IVS = intact ventricular septum; LVOTO = left ventricular outflow tract obstruction; RV = right ventricle; SAS = subaortic stenosis; Sub-PA = sub-pulmonary artery; TGA = transposition of the great arteries; VA = ventriculoarterial; VSD = ventricular septal defect Physiology and Natural History ent to maintain left ventricular pressure at system- The central physiologic problem is arterial hemoglo- ic levels. Therefore the optimal time for an ASO is bin desaturation, the severity of which varies with within the 1st 2 weeks of life, to avoid a "decondi- the degree of communication between the parallel tioned" left ventricle, which may contribute to low systemic and pulmonary circulations. The neonatal cardiac output postoperatively.6 Infants with TGA presentation varies from a healthy-appearing baby and arch obstruction (as well as other prostaglandin- to complete cardiovascular collapse. At the worst E1-dependent patients) usually require operation end of the clinical spectrum are those babies with a within the 1st few days of life.7 In TGA with a VSD, restrictive atrial septal defect (ASD) and/or aortic elective operation can usually be carried out at 1 to arch obstruction, who are often profoundly hypox- 3 months of age, if clinical condition remains stable. emic and acidotic. The natural history of all forms of TGA is ex- Surgery for TGA tremely unfavorable, and without some form of pal- The ASO has emerged as the procedure of choice for liative or definitive surgery, most neonates would most babies with TGA. The procedure employed at not survive infancy. In nearly all cases, the initial re- the Royal Children's Hospital (RCH) is technically suscitation efforts should include balloon atrial sep- demanding but logical and reproducible. It is a hy- tostomy, irrespective of surgical plan. brid of techniques proposed by Jatene, Lecompte, Timing of operation is critical, especially for ba- Quaegebeur, Mee, and others.2'84" The general tech- bies with TGA and an intact ventricular septum nique will be described briefly, along with modifi- (IVS). After the baby is born, pulmonary vascular cations that solve specific problems encountered in resistance drops from its fetal (systemic) level to a special cases. normal postnatal level within 2 weeks. This decline Our cardiopulmonary bypass strategy employs a is accompanied by an involution of left ventricular fresh, heparinized (noncitrate) blood prime and a mass, unless a patent ductus arteriosus, VSD, or left hollow fiber or membrane oxygenator.2 Alphastat ventricular outflow tract obstruction (LVOTO) is pres- pH strategy is followed for cooling to 26 to 28 'C. In Texas Heart InstituteJournal Arterial Switch 323 Sinus 2 (Left hand facing) A Leiden 1 LCx-2R Leiden 1 Cx-2LR Yacoub A LAD LAD Cx B p I - R o* I L A RCA RCA Leiden 1 R-2LCx Leiden 1 LR-2Cx Yacoub E Fig. 2 The Leiden classification of coronary anatomy in transposition of the great arteries. The right hand of an observer standing in the noncoronary sinus (facing the coronary ostia) points to sinus 1, the left hand to sinus 2. The most common pattern is 1 left, circumflex, 2 right. Ao = aorta; Cx = circumflex coronary artery; L = left; LAD = left anterior descending coronary artery; PA = pulmonary artery; R = right; RCA = right coronary artery (From: Karl TR. Transposition of the great arteries.2 Reproduced by permission of Mosby-Year Book, Inc.) most cases, full-flow cardiopulmonary bypass (150 aortic and bicaval cannulation and left-heart venting to 250 ml/kg) is used for the entire operation, with through the ASD. We use cold crystalloid cardiople- 324 Arterial Switch Volume 24, Number 4, 1997 gic solution and topical cold saline for myocardial pro- monary artery. The mobilized coronary arteries are tection, although many units use sanguineous car- translocated and sutured in place with running 8-0 dioplegic solution with good results. polypropylene sutures. We employ the Lecompte With the heart arrested, the aorta and pulmonary maneuver9 in nearly all cases, including those with artery are transected just above the commissures side-by-side great vessels.9" 2 The neoaortic anasto- (Fig. 3). The coronary arteries are excised with a mosis is completed by gathering tissue on the proxi- large cuff of sinus tissue, and medially based rectan- mal side to match the vessel sizes. At this point the gular flaps are cut into the facing sinuses of the pul- VSD, if present, is closed directly with pledgeted mat- D Neopulmonary a. Fig. 3 The arterial switch operation technique used at Royal Children's Hospital. We employ medially based rectangular flaps in the neoaortic sinuses for coronary translocation. The flaps decrease the arc of rotation and prevent tension on the anastomosis. Ao = aorta; LCA = left coronary artery; PA = pulmonary artery; RCA = right coronary artery (From: Karl TR. Transposition of the great arteries.2 Reproduced by permission of Mosby-Year Book, Inc.) Texas Heart InstituteJournal Arterial Switch 325 tress sutures and a Dacron patch, and the heart is tially suitable for an ASO, although some
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