Surgical Results of Arterial Switch Operation for Taussig-Bing Anomaly: Is Position of the Great Arteries a Risk Factor?

Size: px
Start display at page:

Download "Surgical Results of Arterial Switch Operation for Taussig-Bing Anomaly: Is Position of the Great Arteries a Risk Factor?"

Transcription

1 Surgical Results of Arterial Switch Operation for Taussig-Bing Anomaly: Is Position of the Great Arteries a Risk Factor? Mark D. Rodefeld, MD, Mark Ruzmetov, MD, PhD, Palaniswamy Vijay, PhD, MPH, Andrew C. Fiore, MD, Mark W. Turrentine, MD, and John W. Brown, MD Section of Cardiothoracic Surgery, James W. Riley Hospital for Children and Indiana University School of Medicine, Indianapolis, Indiana; and Section of Cardiothoracic Surgery, St. Louis University School of Medicine, St. Louis, Missouri Background. A variety of definitive operations have been used to manage patients with double-outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). This study identifies the impact of the position of the great arteries and use of a staged surgical approach on the outcome after the arterial switch operation in children with Taussig-Bing anomaly. Methods. From 1986 through July 2005, 34 patients with Taussig-Bing anomaly underwent the arterial switch operation. The median age at operation was 21 days. Based on position of the great arteries, patients were divided into group I (side by side; n 16) and group II (anteroposterior; n 18). Aortic arch obstruction was present in 18 patients (53%), of whom 16 had prior repair with aortic arch reconstruction. Abnormal coronary artery patterns were present in 9 patients (27%). Results. There were 4 early deaths and 1 late death (3 from group I and 2 from group II). The actuarial survival rate was 85% at 15 years (81% in group I and 89% in group II). Right ventricular outflow tract obstruction (mean gradient, mm Hg) developed in 5 cases (2 from group I and 3 from group II). One patient underwent reoperation for residual aortic arch obstruction. Freedom from reoperation was 80% at 15 years, and thereafter 85% in group I and 75% in group II. Statistical analysis of potential risk factors revealed no significant identifiers for death or need for reoperation between groups. Conclusions. The arterial switch operation remains our preferred choice of treatment for children with Taussig- Bing anomaly. The position of the great arteries has no effect on postoperative morbidity and mortality. In the presence of aortic arch obstruction, staged arch reconstruction followed soon thereafter by early intracardiac repair has yielded excellent outcomes in our experience. (Ann Thorac Surg 2007;83:1451 7) 2007 by The Society of Thoracic Surgeons In 1949, Taussig and Bing [1] reported on a morphologic syndrome consisting of transposed aorta, subpulmonary ventricular septal defect (VSD), and levoposition of the pulmonary artery. They emphasized that overriding of the pulmonary artery was an integral part of the malformation. Lev and associates [2] coined the term Taussig-Bing heart in 1950 and subsequently introduced the concept of a spectrum of Taussig-Bing hearts depending on the degree of pulmonary artery override (right-sided, intermediate, and left-sided). Despite this controversy, most authors of surgical reviews describe the Taussig-Bing heart as double-outlet right ventricle (DORV), subpulmonary VSD, and subarterial conus, perhaps more because of established usage rather than an endorsement of one interpretation over the other. Whatever the definition, the goal of therapy is anatomic correction whereby the resultant repair connects the Accepted for publication Oct 27, Presented at the Poster Session of the Fifty-second Annual Meeting of the Southern Thoracic Surgical Association, Orlando, FL, Nov 10 12, Address correspondence to Dr Rodefeld, Section of Cardiothoracic Surgery, Indiana University School of Medicine, 545 Barnhill Dr, EH 215, Indianapolis, IN ; rodefeld@iupui.edu. morphologic left ventricle to the aorta (or neoaorta) and the morphologic right ventricle to the pulmonary artery (or neopulmonary artery). The options for anatomic repair are determined by great artery orientation, internal cardiac morphology [3], and associated lesions, which include aortic coarctation, small ascending aorta relative to the pulmonary artery, multiple VSDs, straddling mitral valve, and tricuspid septal leaflet attachment to the interventricular septum. The evolution of surgical repair for the Taussig-Bing DORV has progressed from atrial baffle operations with intraventricular partition [4], Damus-Kaye-Stansel procedure [5, 6], and Rastelli-type intracardiac baffle and right ventricle-pulmonary artery conduit repair [4, 7, 8] to the arterial switch operation (ASO) with VSD closure [9 12] and intraventricular repair [10, 13, 14]. In addition, Fontan-type procedures may be proposed as a solution for even more complex forms. Of the intraventricular repairs, one (Patrick-McGoon operation) has been used for anteroposterior great artery anatomy by tunneling left ventricular flow anterior to the pulmonary valve, whereas the other (Kawashima operation) is used for side-by-side great artery anatomy by 2007 by The Society of Thoracic Surgeons /07/$32.00 Published by Elsevier Inc doi: /j.athoracsur

2 1452 RODEFELD ET AL Ann Thorac Surg SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 2007;83: Table 1. Associated Cardiac Anomalies Anomaly Number of Patients Percent Intracardiac Secundum atrial septal defect Multiple ventricular septal defects 2 6 Bicuspid aortic valve 1 3 Subaortic stenosis 1 3 Extracardiac Coarctation of aorta Interrupted aortic arch 4 12 Hypoplastic aortic arch 1 3 tunneling left ventricular flow posterior to the pulmonary valve [13, 15]. Because of intraventricular geometric factors that can lead to tunnel stenosis, the Patrick-McGoon operation has been largely replaced by the ASO. The Kawashima intraventricular repair is still favored by some surgeons for patients with side-by-side orientation of the great arteries [13, 15]. Since 1986, we have performed ASO to manage all patients presenting with Taussig-Bing anomaly (side-byside or anteroposterior great artery relationships). In the presence of aortic arch obstruction, we have utilized an early staged approach to repair these patients. The purpose of this retrospective study is to identify the impact of anatomic relationship of the great arteries and use of an early staged approach on outcomes after ASO in children with Taussig-Bing anomaly. There were 20 boys (59%) and 14 girls (41%). The mean age at the time of surgery was months, with a range of 3 days to 16 years (median age, 21 days). Twenty-six patients (77%) were neonates (n 21) or infants younger than 3 months (n 5). Associated anatomic lesions are listed in Table 1. Aortic arch obstruction, manifested as aortic coarctation (n 14) or interrupted aortic arch (n 4), was common (18 of 34 patients; 53%). Two patients had an additional muscular VSD. Prior palliative and concomitant cardiovascular operations are shown in Table 2. Twenty-two patients (65%) underwent previous palliative procedures. All patients (except 1 with coarctation and 1 with interruption of the aorta) with a coarctation of the aorta and with an interrupted aortic arch underwent early two-stage repair. All patients had favorably facing aortic and pulmonary artery sinuses of Valsalva for coronary artery transfer regardless of great artery relationship. The coronary artery anatomy was sinus I (left anterior descending, circumflex) sinus II (right coronary artery) in 25 (type A, according to Yacoub and Radley-Smith [16]); sinus I (right, left anterior descending, and circumflex) in 2 (type B); sinus I (left anterior descending) sinus II (circumflex, right coronary artery) in 5 (type D); and sinus I (right, left anterior descending) sinus II (circumflex) in 2 (type E). Operative Technique In the presence of associated aortic arch obstruction, we have approached the majority of these patients with a closely staged combined operative approach. If aortic Material and Methods Study Patients Between January 1986 and July 2005, 34 infants and children underwent ASO surgical repair of Taussig-Bing DORV at James W. Riley Hospital for Children at Indiana University School of Medicine. After obtaining approval from our Institutional Review Boards and waiving the need to obtain patient consent for this study, a retrospective review of medical records was performed with regard to initial clinical features, pathophysiologic findings, surgical treatment, and hospital mortality. Data from outpatient visits and from patients who died after hospital discharge were obtained from physician records, hospital records, or death certificates. Taussig-Bing anomaly is defined as an anomalous ventriculoarterial connection with VSD and DORV. The VSD is subpulmonary in location and the outlet septum is malaligned. In addition, tricuspid-aorta discontinuities are present. The aorta may be positioned rightward and anterior (D-transposition) or alongside the pulmonary trunk (side-by-side orientation). The assigned anatomic diagnosis was based on a combination of twodimensional echocardiographic and angiographic evidence (by cardiologists) and surgical inspection (by surgeons). Based on position of the great arteries, patients were divided into group I (side-by-side; n 16) and group II (anteroposterior; n 18). Table 2. Previous and Concomitant Cardiovascular Operation Procedure Group I (n 16) Group II (n 18) Previous PAB 1 1 PAB with atrial septostomy 1 1 IAA repair with atrial septostomy 1 1 Atrial septostomy 1 2 CoA repair with PDA ligation 4 6 CoA repair with PDA ligation 2 0 and PAB IAA repair with PAB and atrial 0 1 septostomy Total Concomitant ASD closure 9 11 ASD closure with PDA ligation 3 4 ASD and additional VSD closure 1 1 CoA repair with ASD closure and 1 0 PDA ligation IAA repair with ASD closure and 1 0 PDA ligation Subaortic resection 1 0 Lecompte maneuver ASD atrial septal defect; CoA coarctation of the aorta; IAA interrupted aortic arch; PAB pulmonary artery banding; PDA pattern ductus arteriosus; VSD ventricular septal defect.

3 Ann Thorac Surg RODEFELD ET AL 2007;83: SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 1453 arch obstruction is present, we have performed early arch repair through thoracotomy, followed closely (1 to 3 weeks) by intracardiac repair through median sternotomy. This has the advantage of simplifying the overall repair at each procedure, yet does not preclude ASO within the neonatal time period. The surgical technique for ASO is well outlined in several previous reports. We perform the ASO using continuous full-flow bypass at 20 C. Myocardial protection is accomplished with intermittent dose of cold 4:1 blood: crystalloid cardioplegia administered every 20 to 30 minutes at 10 to 15 cc/kg. Cardioplegia is given into the aortic root before the aorta is opened and directly into the coronaries while the neoaorta is reconstructed and coronary transfer is being accomplished. The neoaorta and coronary anastomoses are carried out with 7-0 absorbable monofilament sutures in running fashion. Once the neoaortic anastomosis has been completed, the aortic cross-clamp is released, allowing the aortic root to distend. The coronary artery buttons are excised from their respective sinuses taking most of sinus tissue. The coronary buttons were mobilized for a distance of 4 to 6 mm and allowed to rotate to the location on the distended neoaortic root where they will reside without torsion or tension. The ideal location on the neoaortic root is marked with a fine suture. A stab wound is made at the mark, taking care not to injure the previously marked anterior neoaortic commissure. The aortic cross-clamp is reapplied while a 2.5-mm aortic punch is used to create a site for coronary implantation. The location of the neoaortic commissure is confirmed, and the opening is enlarged if necessary to accommodate the coronary buttons. Selecting the ideal location for the coronary buttons on the closed and distended neoaortic root has greatly reduced the incidence of left ventricular dysfunction that was observed early (1985 to 1989) in our experience, and subsequently dramatically improved our mortality risk. During this phase, the coronary buttons were reimplanted as the neoaortic anastomosis was completed. The change in coronary implantation technique has eliminated our need to reposition the coronary buttons once implanted. Once coronary artery button transfer is complete, the aortic cross-clamp is removed, and the patient is rewarmed while the neopulmonary valve is reconstructed with a generous pantaloon-shaped patch of fresh autologous pericardium 2.5 to 3.5 times larger than the combined area of the transferred coronary buttons. Using a large pericardial patch to reconstruct the neopulmonary root has greatly reduced our incidence of late supravalvar pulmonary artery stenosis. In 18 patients (14 coarctation and 4 interrupted aortic arch), the surgical procedures included prior (n 16) or concomitant (n 2) aortic arch repair: in 8, subclavian flap type of coarctation repair; in 7, direct anastomosis between the ascending and descending aortic segments; in 2 patients (with interrupted aortic arch), the left common carotid artery was divided, spatulated appropriately and anastomosed as a reverse flap to the descending thoracic aorta; and in 1 patient, synthetic patch aortoplasty of a primary anastomosis. After aortic reconstruction, the pulmonary artery was banded (n 13) to reduce the distal pulmonary artery pressure to 50% or less of systemic pressure. The VSD was closed through the right atrium in 7 patients and through the pulmonary artery (neoaorta) in 27 patients. Our approach the Taussig-Bing VSD is from the left side of patient. After dividing the aorta and pulmonary artery for the switch approach, the VSD is closed by starting the patch with a running 6-0 polypropylene sutute on the right inferior aspect of the VSD and running the suture line from right to left, sewing away from the crest of the septum and away from the conduction tissue. The surgeon then carries the suture line anterior and superior to the base of the neoaortic valve. The Lecompte maneuver was performed in all but 1 patient. No tension was noted on the left pulmonary artery in this subset of patients. One had restrictive VSD that was enlarged anteriorly. One patient with subaortic stenosis underwent resection of a hypertrophic infundibular septum during repair. Statistical Analysis Variables including demographics, previous palliative procedures, morphology, coronary artery pattern, and operative procedure related variables were assessed by means of univariate analysis utilizing 2 test and multivariate logistic regression analysis. In the analysis of risk factors for early death, variables with significance levels of 0.1 in univariate analysis were admitted to a multivariate logistic regression model. Factors with p values of less than 0.05 were considered significantly related to early death. Kaplan-Meier analysis was used for the actuarial survival rates and freedom from reoperation rate. Differences in survival curves were assessed by log-rank test. All analyses were performed with standard commercially available statistical software (SPSS, Chicago, Illinois). Early mortality was defined as death during initial hospitalization or within 30 days of operation. Any deaths later than that were defined as late mortality. In this series, all early deaths occurred during the initial hospitalization and all late deaths occurred after discharge from the initial hospitalization. Results Early and Late Mortality There were 4 early deaths (3 from group I and 1 from group II (12%, 4 of 34). One of them had a single coronary artery (type B), 1 had type E coronary artery anatomy and multiple VSDs, and 1 had interrupted aortic arch. The first patient (from group II) died early in the series owing to myocardial ischemia related to coronary insufficiency. The other child, who had anteroposterior relation of great vessels and redundant atrioventricular valve tissue, died of multisystem organ failure. This patient could not be weaned from cardiopulmonary bypass and was placed on extracorporeal membrane oxygenation (ECMO) support for 23 days. The third death occurred in a 4-week-old

4 1454 RODEFELD ET AL Ann Thorac Surg SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 2007;83: patient who previously underwent repair of interrupted aortic arch. This patient had side-by-side orientation of the great vessels and type B coronary arterial pattern. An ASO was performed, and this patient died 6 hours later. Postmortem examination revealed a recent infarct of the entire left ventricle due to left coronary ostial stenosis. The fourth death was of a patient from group I who underwent ASO but during the postoperative period suffered a cardiac arrest with cardiopulmonary resuscitation and then continuing ECMO support. Sixteen days later, orthotopic cardiac transplantation was performed. The immediate postoperative course was complicated by development of refractory hypoxemia with persistent acidosis and pulmonary hypertension, and this patient subsequently died. There was 1 late death in group II at a mean follow-up of years (range, 6 months to 16 years). This patient had undergone ASO repair at 21 days of age. This death occurred 1 year after initial repair and was due to viral pneumonia. Overall survival including operative mortality according to Kaplan-Meier analysis was 85% at 1, 5, and 15 years. Figure 1 shows the actuarial survival, including operative mortality, which was 81% and 89% at 15 years in patients in group I and group II, respectively. Statistical analysis did not reveal any significant risk factors for early, late, or overall mortality between both groups. Reoperations Reoperation was necessary in 6 survivors without operative mortality (6 of 30; 20%). All patients were successfully reoperated at a mean interval of years after primary repair. Right ventricular outflow tract obstruction (mean gradient, mm Hg) developed in 5 patients (2 from group I and 3 from group II). Two patients underwent right ventricular outflow tract (RVOT) obstruction reconstruction for pulmonary stenosis with Gore-Tex (W.L. Gore & Assoc, Flagstaff, Arizona) patch arterioplasty, and 1 patient required residual VSD closure in addition to Gore-Tex patch arterioplasty. Two had late pulmonary artery stenosis; both had side-byside great vessel orientation. The stenoses were located Fig 1. Kaplan-Meier estimated 15-year survival, including hospital mortality. (Diamonds overall; squares group I; triangles group II.) Fig 2. Kaplan-Meier estimated 15-year freedom from reoperation. (Diamonds overall; squares group I; triangles group II.) distally at the branch level, and reconstruction of the RVOT was performed with Gore-Tex patch with resection of an RVOT muscle bundle. One patient (from group II) underwent revision of coarctation repair owing to aortic arch obstruction. Overall freedom from reoperation was 93% at 1 year, 80% at 5 and 75% at 15 years (Fig 2) and thereafter 85% in group I and 75% in group II. Statistical analysis of contributing factors revealed no significant risk factors for need for reoperation between groups. The incidence of significant RVOT obstruction was not influenced by the presence of aortic arch obstruction for side-by-side (p 0.68) or anteroposterior great arteries (p 1.00), the side-by-side relationship of the great arteries (p 0.92), the anteroposterior relationship of the great arteries (p 0.71), or by the right coronary artery crossing the infundibulum for side-by-side (p 0.84) or anteroposterior great arteries (p 0.86). The difference between neonatal repair versus older repair was significant in patients with aortic arch obstruction (p 0.001), but was not significant between single-stage versus staged repair (p 0.88). Follow-Up and Functional Status of Survivors Mean follow-up was years (range, 6 months to 16 years). Follow-up included sequential noninvasive evaluations: two-dimensional echocardiography, Doppler study, electrocardiography, and radiographic studies. Twenty-six patients have had postoperative echocardiography only, and 3 patients have had both postoperative echocardiography and cardiac catheterization. All 29 survivors are in New York Heart Association functional class I, without medication and are in normal sinus rhythm. At last follow-up, Holter examination performed in 2 patients did not reveal major abnormalities, and Doppler studies showed no subaortic obstruction in 28 and a mild subaortic obstruction (gradient at rest less than 30 mm Hg) in 1 patient. Neoaortic incompetence was moderate in 3 (10%; 3 of 29), and trivial or mild in 26 patients (90%; 26 of 29). Right ventricular outflow tract obstruction (echocardiographic gradient more than 30 mm Hg) was present only in 2 patients (7%). There is no significant difference in neoaortic or neopulmonary obstruction and incompetence between groups.

5 Ann Thorac Surg RODEFELD ET AL 2007;83: SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 1455 Table 3. Arterial Switch Operation With Ventricular Septal Defect Closure for Taussig-Bing Anomaly Patients (Literature Review) First Author Year No. of Patients Great Artery Anatomy Death A/P S/S Reoperation Early Late Serraf [7] Mavroudis [10] Comas [9] Masuda [25] Takeuchi [11] Wetter [12] Rodefeld (current) A/P anteroposterior; S/S side-by-side. Comment The ASO has become our procedure of choice for the Taussig-Bing DORV and all other types of D-transposition of the great arteries. Some authors suggest that the Kawashima intraventricular repair is preferable for Taussig-Bing patients when the great vessels are side by side. Our experience with the Kawashima operation is limited. Our results indicate that there was no difference in Taussig-Bing patient outcome based on great vessel orientation. A literature survey showing the results of the ASO versus the intraventricular repair for Taussig-Bing repair is shown in Tables 3 and 4. Because individual patient factors must be taken into consideration to determine the best overall surgical approach, the decision as to which patients are selected for ASO versus intraventricular procedure can be difficult. Intraventricular repair was first performed in 1968 by Patrick and McGoon [14], and Williams [17] first reported the ASO for Taussig-Bing in Serraf and colleagues [7] reported 20 patients undergoing ASO and 7 patients having intraventricular repair. Kawashima and colleagues [13] reported 10 patients having intraventricular repair and 13 having ASO. Mavroudis and coworkers [10] reported 16 patients undergoing ASO and 4 having intraventricular repair. In aggregate, ASO with VSD closure appears to be the more frequently applied method of repair. Intraventricular diversion of left ventricular blood flow to the aorta may provide a physiologic repair; however, this operation is technically difficult to perform. It involves the insertion of a patch through the VSD to the aortic conus, with hypertrophied muscle in the RV possibly necessitating resection of the aortic conal septum and a significant suture load to stably affix the patch in the RV. Snoddy and associates [18] have reported that tunnel repair of the VSD in the small RV may result in RVOT obstruction. In this situation, a valved external conduit from the RV to the pulmonary artery may provide good hemodynamic results. Recently, Binet and colleagues [19] reported treating physiologically complete transposition of the great arteries by closing the VSD and repairing the transposition by means of the Damus-Kaye-Stansel technique. The long-term outcome of this approach is limited, however, by the suboptimal durability of valved external conduits. Reoperation will eventually be necessary due to structural deterioration of biological valved conduits in children [20]. The ASO, which was introduced as a corrective technique for simple transposition of the great arteries, has become the procedure of choice for patients with all forms of D-transposition including those with Taussig- Bing DORV. At our institution, ASO is currently the most commonly used technique for two-ventricle repair of Taussig-Bing DORV. The ASO is always feasible; however, intraventricular repair seems more attractive as it preserves the native aortic valve and avoids coronary dissection. That has led Yacoub and Radley-Smith [21] to propose a classification according to the extracardiac anatomy: when great arteries are side by side, an intraventricular repair may be performed; when the great arteries relation is more or less anteroposterior, arterial switch may be performed. Table 4. Kawashima Intraventricular Repair for Taussig-Bing Anomaly Patients (Literature Review) Great Artery Anatomy Death First Author Year No. of Patients A/P S/S Reoperation Early Late Serraf [7] Kawashima [13] Mavroudis [10] A/P anteroposterior; S/S side-by-side.

6 1456 RODEFELD ET AL Ann Thorac Surg SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 2007;83: These conclusions were then discussed by Sakata and Lecompte [3], who could not define any strict relation between the intervalvular distances and the relation of the great arteries. Van Praagh [22] commented that the surgical significance is that when the great arteries are anteroposterior, the aortic valve is very anterior and the VSD-to-aorta conduit (for Rastelli-type conduit repair or intraventricular repair) would have to run anteriorly and would cause iatrogenic pulmonary stenosis. With sideby-side great arteries, the conduit can pass posteriorly and allow unimpeded flow to the pulmonary valve. Based on this previous work, we have developed our guidelines for surgical decision making. Our approach is to first consider the relationship of the great arteries: if anteroposterior, an arterial switch is performed with closure of the VSD. If side-by-side orientation of the great vessels is present, then the tricuspid-pulmonary valve distance is evaluated; if less than aortic valve diameter or if there are abnormal tricuspid valve chordae present, ASO and VSD closure are performed; if this distance is greater, pulmonary artery banding is performed before 1 year of age to permit growth, and then a Rastelli-type intraventricular conduit repair is subsequently performed beyond 1 year of age. The coronary distribution in Taussig-Bing DORV is often quite different than that in transposition of the great arteries and may be a source of technical difficulties in ASO. In patients in the ASO group, coronary patterns were normal (Yacoub type A) when the great vessels were in an anteroposterior relationship. But when the great vessel orientation is side by side, almost exclusively, other types of coronary artery distribution are encountered (Yacoub type B, D or E). In a detailed pathologic study, Uemura and associates [23] found that a single coronary artery (type B) was present in 27% of hearts with a side-by-side great artery relationship. Associated aortic arch anomalies are frequently observed in Taussig-Bing DORV [7, 10, 23, 24]. Repair of the aortic arch obstruction in a one- or two-stage procedure remains controversial, although the one-stage procedure has recently become increasingly popular [9, 10]. However, analysis of the overall mortality of patients with associated aortic arch anomalies in this series in which a closely sequenced two-stage repair was utilized, the existence of aortic arch anomalies was not an incremental risk factor, provided that an adequate aortic arch repair was achieved. If aortic arch reconstruction is complicated by an inadequate luminal diameter and pulmonary artery banding is performed, biventricular outflow tract obstruction will result. Finally, as we have performed in several patients in the latter part of our series, a onestage repair, including aortic arch reconstruction, ASO, and closure of the VSD can certainly be successfully performed in selected patients. A one-stage approach has the advantage of avoiding multiple operations, the development of pulmonary vascular disease, and may be performed in a neonate with suitable ventricular function. However, the optimal strategy utilized depends upon an overall assessment of the patients intracardiac anatomy and severity of associated extracardiac anomalies. Patients with Taussig-Bing DORV face a higher risk of neoaortic incompetence from a mismatch of the large pulmonary artery and a small aorta. Minor neoaortic regurgitation is common after ASO, with reported rates ranging from 5% to 55% [24]. It has been speculated that resection of tissue of the neononcoronary sinus may lead to dilatation of the sinus of Valsalva and adjacent leaflet prolapse, which in turn leads to neoaortic insufficiency [7]. Moreover, the increased preoperative flow across the anatomic pulmonary valve may result in dilatation of the pulmonary valve annulus and postoperative neoaortic insufficiency, or reimplantation of the abnormal coronary arteries into the neoaorta may distort the architecture of the valve. Because the degree of neoaortic regurgitation present in most of our patients was hemodynamically insignificant, further follow-up will be required to determine whether trivial or mild regurgitation will eventually become clinically relevant. We conclude that Taussig-Bing DORV can be repaired by ASO and VSD closure in neonates and young infants with an excellent outcome. Staged correction of associated cardiac anomalies (especially aortic arch obstruction) closely followed by early intracardiac repair (1 to 3 weeks) had no detrimental influence on survival and later outcome. Therefore, we prefer to perform a twostage complete repair with each stage closely timed in the neonatal period. The position of the great arteries has no effect on postoperative morbidity and mortality. Right ventricular outflow tract obstruction often is the most commonly observed late complication and is a leading cause for reintervention. To determine secondary effects of neoaortic regurgitation and their clinical relevance, continued follow-up is mandatory after ASO repair of Taussig-Bing DORV. References 1. Taussig HB, Bing RJ. Complete transposition of the aorta and levoposition of the pulmonary artery. Clinical, physiological, and pathological findings. Am Heart J 1949;37: Lev M, Volk BM. The pathologic anatomy of the Taussig- Bing heart: riding pulmonary artery. Report of case. Bull Int Assoc Med Museums 1950;31: Sakata R, Lecompte Y, Batisse A, Borromee L, Durandy Y. Anatomical repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. I. Criteria of surgical decision. J Thorac Cardiovasc Surg 1986;92: Pacifico AD, Kirklin JK, Colvin EV, Bargeron LM. Intraventricular tunnel repair for Taussig-Bing heart and related cardiac anomalies. Circulation 1986;74(Suppl 1): DeLeon SY, Ilbawi MN, Tubeszewski K, Wilson WR, Idriss FS. The Damus-Stansel-Kaye procedure: anatomical determinants and modifications. Ann Thorac Surg 1991;52: Lui RC, Williams WG, Trusler GA, et al. Experience with the Damus-Kaye-Stansel procedure for children with Taussig- Bing heart or univentricular hearts with subaortic stenosis. Circulation 1993;88: Serraf A, Lacour-Gayet F, Bruniaux J, et al. Anatomic repair of Taussig-Bing hearts. Circulation 1991;84(Suppl 3): Kawashima Y, Matsuda H, Taniguchi K, Kobayashi J. Additional aortopulmonary anastomosis for subaortic obstruction in

7 Ann Thorac Surg RODEFELD ET AL 2007;83: SURGICAL RESULTS OF TAUSSIG-BING ANOMALY 1457 the Rastelli-type repair for the Taussig-Bing malformation. Ann Thorac Surg 1987;44: Comas JV, Mignosa C, Cochrane AD, Wilkinson JL, Karl TR. Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome. Eur J Cardiothorac Surg 1996;10: Mavroudis C, Backer CL, Muster AJ, Rocchini AP, Rees AH, Gevitz M. Taussig-Bing anomaly: arterial switch versus Kawashima intraventricular repair. Ann Thorac Surg 1996; 61: Takeuchi K, McGowan FX, Moran AM, et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg 2001;71: Wetter J, Sinzobahamvya N, Blaschczok HC, et al. Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly. Ann Thorac Surg 2004;77: Kawashima Y, Matsuda H, Yagihara T, et al. Intraventricular repair for Taussig-Bing anomaly. J Thoracic Cardiovasc Surg 1993;105: Patrick DL, McGoon DC. An operation for double-outlet right ventricle with transposition of the great arteries. J Cardiovasc Surg 1968;9: Kawahira Y, Yagihara T, Uemura H, et al. Ventricular outflow tracts after Kawashima intraventricular rerouting for double outlet right ventricle with subpulmonary ventricular septal defect. Eur J Cardiothorac Surg 1999;16: Yacoub MH, Radley-Smith R. Anatomy of the coronary arteries in transposition of the great arteries and methods for their transfer in anatomical correction. Thorax 1978;33: Williams WG, Freedom RM, Culhan JAG, et al. Early experience with arterial repair of transposition. Ann Thorac Surg 1981;32: Snoddy JW, Parr EL, Robertson LW, Mauck HP, McCue CM, Lower RR. Successful intracardiac repair of the Taussig-Bing malformation in 2 children. Ann Thorac Surg 1978;25: Binet JP, Lacour-Gayet F, Conso JF, Dupuis C, Bruniaux J. Complete repair of the Taussig-Bing type of double-outlet right ventricle using the arterial switch operation without coronary translocation. J Thorac Cardiovasc Surg 1983;85: Kanter K, Anderson R, Lincoln C, Firmin R, Rigby M. Anatomic correction of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing anomaly). Ann Thorac Surg 1986;41: Yacoub MH, Radley-Smith R. Anatomic correction of the Taussig-Bing anomaly. J Thorac Cardiovasc Surg 1984;88: Van Praagh R. What is the Taussig-Bing malformation? Circulation 1968;38: Uemura H, Yagihara T, Kawashima Y, et al. Coronary arterial anatomy in double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg 1995;59: Masuda M, Kado H, Shiokawa Y, et al. Clinical results of arterial swith operation for double-outlet right ventricle with subpulmonary VSD. Eur J Cardiothorac Surg 1999; 15: The Society of Thoracic Surgeons: Forty-Fourth Annual Meeting Please mark your calendars for the Forty-Fourth Annual Meeting of The Society of Thoracic Surgeons, to be held in Fort Lauderdale, Florida, from January 28 30, The program will provide in-depth coverage of surgical topics selected to enhance and broaden the knowledge of cardiothoracic surgeons. Attendees will benefit from traditional Abstract Presentations, as well as Surgical Forums, Breakfast Sessions, and Surgical Motion Pictures. Parallel sessions will focus on specific subspecialty interests. Advance registration forms, hotel reservation forms, and details regarding transportation arrangements, as well as the complete meeting program, will be mailed to Society members this fall. Also, complete meeting information will be available on the Society s Web site at Nonmembers who wish to receive information on the Annual Meeting may contact the Society s secretary, Douglas E. Wood. Douglas E. Wood, MD Secretary The Society of Thoracic Surgeons 633 N. Saint Clair St, Suite 2320 Chicago, IL Telephone: (312) Fax: (312) sts@sts.org website: by The Society of Thoracic Surgeons Ann Thorac Surg 2007;83: /07/$32.00 Published by Elsevier Inc

Surgical Treatment for Double Outlet Right Ventricle. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery

Surgical Treatment for Double Outlet Right Ventricle. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery for Double Outlet Right Ventricle Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery 1 History Intraventricular tunnel (Kawashima) First repair of Taussig-Bing anomaly (Kirklin) Taussig-Bing

More information

The Rastelli procedure has been traditionally used for repair

The Rastelli procedure has been traditionally used for repair En-bloc Rotation of the Truncus Arteriosus A Technique for Complete Anatomic Repair of Transposition of the Great Arteries/Ventricular Septal Defect/Left Ventricular Outflow Tract Obstruction or Double

More information

In 1980, Bex and associates 1 first introduced the initial

In 1980, Bex and associates 1 first introduced the initial Technique of Aortic Translocation for the Management of Transposition of the Great Arteries with a Ventricular Septal Defect and Pulmonary Stenosis Victor O. Morell, MD, and Peter D. Wearden, MD, PhD In

More information

Surgical Results in Patients With Double Outlet Right Ventricle: A 20-Year Experience

Surgical Results in Patients With Double Outlet Right Ventricle: A 20-Year Experience Surgical Results in Patients With Double Outlet Right Ventricle: A 20-Year Experience John W. Brown, MD, Mark Ruzmetov, MD, Yuji Okada, MD, Palaniswamy Vijay, PhD, MPH, and Mark W. Turrentine, MD Section

More information

The arterial switch operation has been the accepted procedure

The arterial switch operation has been the accepted procedure The Arterial Switch Procedure: Closed Coronary Artery Transfer Edward L. Bove, MD The arterial switch operation has been the accepted procedure for the repair of transposition of the great arteries (TGA)

More information

Double Outlet Right Ventricle with Anterior and Left-Sided Aorta and Subpulmonary Ventricular Septal Defect

Double Outlet Right Ventricle with Anterior and Left-Sided Aorta and Subpulmonary Ventricular Septal Defect Case Report Double Outlet Right Ventricle with Anterior and Left-Sided rta and Subpulmonary Ventricular Septal Defect Luciana Braz Peixoto, Samira Morhy Borges Leal, Carlos Eduardo Suaide Silva, Sandra

More information

TGA Surgical techniques: tips & tricks (Arterial switch operation)

TGA Surgical techniques: tips & tricks (Arterial switch operation) TGA Surgical techniques: tips & tricks (Arterial switch operation) Seoul National University Children s Hospital Woong-Han Kim Surgical History 1951 Blalock and Hanlon, atrial septectomy 1954 Mustard et

More information

14 Valvular Stenosis

14 Valvular Stenosis 14 Valvular Stenosis 14-1. Valvular Stenosis unicuspid valve FIGUE 14-1. This photograph shows severe valvular stenosis as it occurs in a newborn. There is a unicuspid, horseshoe-shaped leaflet with a

More information

"Giancarlo Rastelli Lecture"

Giancarlo Rastelli Lecture "Giancarlo Rastelli Lecture" Surgical treatment of Malpositions of the Great Arteries Pascal Vouhé Giancarlo Rastelli (1933 1970) Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième

More information

Double outlet right ventricle: navigation of surgeon to chose best treatment strategy

Double outlet right ventricle: navigation of surgeon to chose best treatment strategy Double outlet right ventricle: navigation of surgeon to chose best treatment strategy Jan Marek Great Ormond Street Hospital & Institute of Cardiovascular Sciences, University College London Double outlet

More information

The Double Switch Using Bidirectional Glenn and Hemi-Mustard. Frank Hanley

The Double Switch Using Bidirectional Glenn and Hemi-Mustard. Frank Hanley The Double Switch Using Bidirectional Glenn and Hemi-Mustard Frank Hanley No relationships to disclose CCTGA Interesting Points for Discussion What to do when. associated defects must be addressed surgically:

More information

Introduction. Study Design. Background. Operative Procedure-I

Introduction. Study Design. Background. Operative Procedure-I Risk Factors for Mortality After the Norwood Procedure Using Right Ventricle to Pulmonary Artery Shunt Ann Thorac Surg 2009;87:178 86 86 Addressor: R1 胡祐寧 2009/3/4 AM7:30 SICU 討論室 Introduction Hypoplastic

More information

Techniques for repair of complete atrioventricular septal

Techniques for repair of complete atrioventricular septal No Ventricular Septal Defect Patch Atrioventricular Septal Defect Repair Carl L. Backer, MD *, Osama Eltayeb, MD *, Michael C. Mongé, MD *, and John M. Costello, MD For the past 10 years, our center has

More information

Current Technique of the Arterial Switch Procedure for Transposition of the Great Arteries

Current Technique of the Arterial Switch Procedure for Transposition of the Great Arteries Current Technique of the Arterial Switch Procedure for Transposition of the Great Arteries EDWARD L. BOVE, M.D. Section of Thoracic Surgev, C.S. Mott Children's Hospital, The University of Michigan Medical

More information

SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE

SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE Mr. W. Brawn Birmingham Children s Hospital. Aims of surgery The aim of surgery in congenital heart disease is to correct or palliate the heart

More information

Perimembranous VSD: When Do We Ask For A Surgical Closure? LI Xin. Department of Cardiothoracic Surgery Queen Mary Hospital Hong Kong

Perimembranous VSD: When Do We Ask For A Surgical Closure? LI Xin. Department of Cardiothoracic Surgery Queen Mary Hospital Hong Kong Perimembranous VSD: When Do We Ask For A Surgical Closure? LI Xin Department of Cardiothoracic Surgery Queen Mary Hospital Hong Kong Classification (by Kirklin) I. Subarterial (10%) Outlet, conal, supracristal,

More information

LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT

LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT 10-13 March 2017 Ritz Carlton, Riyadh, Saudi Arabia Zohair AlHalees, MD Consultant, Cardiac Surgery Heart Centre LEFT VENTRICULAR

More information

Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions

Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions Tier 1 surgeries AV Canal Atrioventricular Septal Repair, Complete Repair of complete AV canal (AVSD) using one- or two-patch or other technique,

More information

Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016

Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016 1 Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016 DISCLOSURES I have no disclosures relevant to today s talk 2 Why should all echocardiographers

More information

Since first successfully performed by Jatene et al, the

Since first successfully performed by Jatene et al, the Long-Term Predictors of Aortic Root Dilation and Aortic Regurgitation After Arterial Switch Operation Marcy L. Schwartz, MD; Kimberlee Gauvreau, ScD; Pedro del Nido, MD; John E. Mayer, MD; Steven D. Colan,

More information

The first report of the Society of Thoracic Surgeons

The first report of the Society of Thoracic Surgeons REPORT The Society of Thoracic Surgeons National Congenital Heart Surgery Database Report: Analysis of the First Harvest (1994 1997) Constantine Mavroudis, MD, Melanie Gevitz, BA, W. Steves Ring, MD, Charles

More information

The modified Konno procedure, or subaortic ventriculoplasty,

The modified Konno procedure, or subaortic ventriculoplasty, Modified Konno Procedure for Left Ventricular Outflow Tract Obstruction David P. Bichell, MD The modified Konno procedure, or subaortic ventriculoplasty, first described by Cooley and Garrett in1986, 1

More information

Coronary Arterial Anatomy in Double-Outlet Right Ventricle With Subpulmonary VSD

Coronary Arterial Anatomy in Double-Outlet Right Ventricle With Subpulmonary VSD Coronary Arterial Anatomy in Double-Outlet Right Ventricle With Subpulmonary VSD Hideki Uemura, MD, Toshikatsu Yagihara, MD, Yasunaru Kawashima, MD, Kyoichi Nishigaki, MD, Tetsuro Kamiya, MD, Siew Yen

More information

Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease

Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease TIRONE E. DAVID, MD ; SEMIN THORAC CARDIOVASC SURG 19:116-120c 2007 ELSEVIER INC. PRESENTED BY INTERN 許士盟 Mitral valve

More information

The Arterial Switch Operation for Transposition of the Great Arteries

The Arterial Switch Operation for Transposition of the Great Arteries The Arterial Switch Operation for Transposition of the Great Arteries Jan M. Quaegebeur, M.D., Ph.D. A Journey of 60 Years Transposition of the Great Arteries First description: M. BAILLIE The morbid anatomy

More information

Cardiac Catheterization Cases Primary Cardiac Diagnoses Facility 12 month period from to PRIMARY DIAGNOSES (one per patient)

Cardiac Catheterization Cases Primary Cardiac Diagnoses Facility 12 month period from to PRIMARY DIAGNOSES (one per patient) PRIMARY DIAGNOSES (one per patient) Septal Defects ASD (Atrial Septal Defect) PFO (Patent Foramen Ovale) ASD, Secundum ASD, Sinus venosus ASD, Coronary sinus ASD, Common atrium (single atrium) VSD (Ventricular

More information

Tetralogy of Fallot (TOF) with absent pulmonary valve

Tetralogy of Fallot (TOF) with absent pulmonary valve Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome Karl F. Welke, MD, and Ross M. Ungerleider, MD, MBA Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS) occurs in 5%

More information

Long-Term Results After the Rastelli Repair for Transposition of the Great Arteries

Long-Term Results After the Rastelli Repair for Transposition of the Great Arteries PEDIATRIC CARDIAC SURGERY: The Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member

More information

SURGICAL APPROACHES FOR DOUBLE-OUTLET RIGHT VENTRICLE OR TRANSPOSITION OF THE GREAT ARTERIES ASSOCIATED WITH STRADDLING ATRIOVENTRICULAR VALVES

SURGICAL APPROACHES FOR DOUBLE-OUTLET RIGHT VENTRICLE OR TRANSPOSITION OF THE GREAT ARTERIES ASSOCIATED WITH STRADDLING ATRIOVENTRICULAR VALVES SURGICAL APPROACHES FOR DOUBLE-OUTLET RIGHT VENTRICLE OR TRANSPOSITION OF THE GREAT ARTERIES ASSOCIATED WITH STRADDLING ATRIOVENTRICULAR VALVES Alain Serraf, MD ~ Tomohiro Nakamura, MD ~ Fran ois Lacour-Gayet,

More information

The pulmonary valve is the most common heart valve

The pulmonary valve is the most common heart valve Biologic versus Mechanical Valve Replacement of the Pulmonary Valve After Multiple Reconstructions of the RVOT Tract S. Adil Husain, MD, and John Brown, MD Indiana University School of Medicine, Department

More information

Appendix A.2: Tier 2 Surgical Procedure Terms and Definitions

Appendix A.2: Tier 2 Surgical Procedure Terms and Definitions Appendix A.2: Tier 2 Surgical Procedure Terms and Definitions Tier 2 surgeries Anomalous Systemic Venous Connection Anomalous Systemic Venous Connection Repair Repair includes a range of surgical approaches,

More information

Pediatric Echocardiography Examination Content Outline

Pediatric Echocardiography Examination Content Outline Pediatric Echocardiography Examination Content Outline (Outline Summary) # Domain Subdomain Percentage 1 Anatomy and Physiology Normal Anatomy and Physiology 10% 2 Abnormal Pathology and Pathophysiology

More information

BIVENTRICULAR REPAIR FOR AORTIC ATRESIA OR HYPOPLASIA AND VENTRICULAR SEPTAL DEFECT

BIVENTRICULAR REPAIR FOR AORTIC ATRESIA OR HYPOPLASIA AND VENTRICULAR SEPTAL DEFECT BIVENTRICULAR REPAIR FOR AORTIC ATRESIA OR HYPOPLASIA AND VENTRICULAR SEPTAL DEFECT Richard G. Ohye, MD a Koji Kagisaki, MD a Lisa A. Lee, MD b Ralph S. Mosca, MD a Caren S. Goldberg, MD b Edward L. Bove,

More information

Surgical Treatment for Atrioventricular Septal Defect. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery

Surgical Treatment for Atrioventricular Septal Defect. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery Surgical Treatment for Atrioventricular Septal Defect Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery 1 History Rastelli classification (Rastelli) Pulmonary artery banding (Muller & Dammann)

More information

Damus-Kaye-Stansel Procedure: Midterm Follow-up and Technical Considerations

Damus-Kaye-Stansel Procedure: Midterm Follow-up and Technical Considerations Damus-Kaye-Stansel Procedure: Midterm Follow-up and Technical Considerations Thomas L. Carter, MD, Richard D. Mainwaring, MD, and John J. Lamberti, MD Division of Cardiac Surgery, Children's Hospital and

More information

Complete Transposition of the Great Arteries

Complete Transposition of the Great Arteries 1 Complete Transposition of the Great Arteries Contents Introduction 2 Anatomy 3 Complete Transposition of the Great Arteries, with or without Ventricular Septal Defect 5 Indication for Surgery 5 Approach

More information

The evolution of the Fontan procedure for single ventricle

The evolution of the Fontan procedure for single ventricle Hemi-Fontan Procedure Thomas L. Spray, MD The evolution of the Fontan procedure for single ventricle cardiac malformations has included the development of several surgical modifications that appear to

More information

Common Defects With Expected Adult Survival:

Common Defects With Expected Adult Survival: Common Defects With Expected Adult Survival: Bicuspid aortic valve :Acyanotic Mitral valve prolapse Coarctation of aorta Pulmonary valve stenosis Atrial septal defect Patent ductus arteriosus (V.S.D.)

More information

FATE OF THE NEOPULMONARY VALVE AFTER THE ARTERIAL SWITCH OPERATION IN NEONATES

FATE OF THE NEOPULMONARY VALVE AFTER THE ARTERIAL SWITCH OPERATION IN NEONATES FATE OF THE NEOPULMONARY VALVE AFTER THE ARTERIAL SWITCH OPERATION IN NEONATES Shunji Nogi, MD a Brian W. McCrindle, MD, FACC a Christine Boutin, MD a William G. Williams, MD, FACC b Robert M. Freedom,

More information

Commissural Malalignment of Aortic-Pulmonary Sinus in Complete Transposition of Great Arteries

Commissural Malalignment of Aortic-Pulmonary Sinus in Complete Transposition of Great Arteries Commissural Malalignment of Aortic-Pulmonary Sinus in Complete Transposition of Great Arteries Soo-Jin Kim, MD, Woong-Han Kim, MD, Cheong Lim, MD, Sam Se Oh, MD, and Yang-Min Kim, MD Departments of Pediatric

More information

Single-Stage Correction for Taussig Bing Anomaly Associated With Aortic Arch Obstruction

Single-Stage Correction for Taussig Bing Anomaly Associated With Aortic Arch Obstruction DOI 10.1007/s00246-017-1694-6 ORIGINAL ARTICLE Single-Stage Correction for Taussig Bing Anomaly Associated With Aortic Arch Obstruction Kai Luo 1 Jinghao Zheng 1 Shunmin Wang 1 Zhongqun Zhu 1 Botao Gao

More information

The need for right ventricular outflow tract reconstruction

The need for right ventricular outflow tract reconstruction Polytetrafluoroethylene Bicuspid Pulmonary Valve Implantation James A. Quintessenza, MD The need for right ventricular outflow tract reconstruction and pulmonary valve replacement is increasing for many

More information

Repair of Complete Atrioventricular Septal Defects Single Patch Technique

Repair of Complete Atrioventricular Septal Defects Single Patch Technique Repair of Complete Atrioventricular Septal Defects Single Patch Technique Fred A. Crawford, Jr., MD The first repair of a complete atrioventricular septal defect was performed in 1954 by Lillehei using

More information

Children with Single Ventricle Physiology: The Possibilities

Children with Single Ventricle Physiology: The Possibilities Children with Single Ventricle Physiology: The Possibilities William I. Douglas, M.D. Pediatric Cardiovascular Surgery Children s Memorial Hermann Hospital The University of Texas Health Science Center

More information

Congenital Heart Defects

Congenital Heart Defects Normal Heart Congenital Heart Defects 1. Patent Ductus Arteriosus The ductus arteriosus connects the main pulmonary artery to the aorta. In utero, it allows the blood leaving the right ventricle to bypass

More information

Anatomy of the coronary arteries in transposition

Anatomy of the coronary arteries in transposition Thorax, 1978, 33, 418-424 Anatomy of the coronary arteries in transposition of the great arteries and methods for their transfer in anatomical correction MAGDI H YACOUB AND ROSEMARY RADLEY-SMITH From Harefield

More information

Perioperative Management of DORV Case

Perioperative Management of DORV Case Perioperative Management of DORV Case James P. Spaeth, MD Department of Anesthesia Cincinnati Children s Hospital Medical Center University of Cincinnati Objectives: 1. Discuss considerations regarding

More information

The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly

The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly Original Article The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly Shusheng Wen, Jianzheng Cen, Jimei Chen, Gang Xu, Biaochuan He, Yun Teng, Jian

More information

Heart and Lungs. LUNG Coronal section demonstrates relationship of pulmonary parenchyma to heart and chest wall.

Heart and Lungs. LUNG Coronal section demonstrates relationship of pulmonary parenchyma to heart and chest wall. Heart and Lungs Normal Sonographic Anatomy THORAX Axial and coronal sections demonstrate integrity of thorax, fetal breathing movements, and overall size and shape. LUNG Coronal section demonstrates relationship

More information

Absent Pulmonary Valve Syndrome

Absent Pulmonary Valve Syndrome Absent Pulmonary Valve Syndrome Fact sheet on Absent Pulmonary Valve Syndrome In this condition, which has some similarities to Fallot's Tetralogy, there is a VSD with narrowing at the pulmonary valve.

More information

Neonatal arterial switch operation: coronary artery patterns and coronary events 1

Neonatal arterial switch operation: coronary artery patterns and coronary events 1 European Journal of Cardio-thoracic Surgery 11 (1997) 810 817 Neonatal arterial switch operation: coronary artery patterns and coronary events 1 Daniel Tamisier, Ruth Ouaknine, Philippe Pouard, Philippe

More information

Although most patients with Ebstein s anomaly live

Although most patients with Ebstein s anomaly live Management of Neonatal Ebstein s Anomaly Christopher J. Knott-Craig, MD, FACS Although most patients with Ebstein s anomaly live through infancy, those who present clinically as neonates are a distinct

More information

Repair of very severe tricuspid regurgitation following detachment of the tricuspid valve

Repair of very severe tricuspid regurgitation following detachment of the tricuspid valve OPEN ACCESS Images in cardiology Repair of very severe tricuspid regurgitation following detachment of the tricuspid valve Ahmed Mahgoub 1, Hassan Kamel 2, Walid Simry 1, Hatem Hosny 1, * 1 Aswan Heart

More information

TGA, VSD, and LVOTO. Cheul Lee, MD. Department of Thoracic and Cardiovascular Surgery Sejong General Hospital

TGA, VSD, and LVOTO. Cheul Lee, MD. Department of Thoracic and Cardiovascular Surgery Sejong General Hospital Surgical Management of TGA, VSD, and LVOTO Cheul Lee, MD Department of Thoracic and Cardiovascular Surgery Sejong General Hospital TGA, VSD, and LVOTO Incidence : 0.7% of all CHD 20% of TGA with VSD 4%

More information

Anatomic repair of complex transposition with en bloc rotation of the truncus arteriosus: 10-year experience

Anatomic repair of complex transposition with en bloc rotation of the truncus arteriosus: 10-year experience European Journal of Cardio-Thoracic Surgery 49 (2016) 176 182 doi:10.1093/ejcts/ezv056 Advance Access publication 19 February 2015 ORIGINAL ARTICLE Cite this article as: Mair R, Sames-Dolzer E, Innerhuber

More information

Partial anomalous pulmonary venous connection to superior

Partial anomalous pulmonary venous connection to superior Cavo-Atrial Anastomosis Technique for Partial Anomalous Pulmonary Venous Connection to the Superior Vena Cava The Warden Procedure Robert A. Gustafson, MD Partial anomalous pulmonary venous connection

More information

CMR for Congenital Heart Disease

CMR for Congenital Heart Disease CMR for Congenital Heart Disease * Second-line tool after TTE * Strengths of CMR : tissue characterisation, comprehensive access and coverage, relatively accurate measurements of biventricular function/

More information

The Clinical Outcomes of Damus-Kaye-Stansel Procedure According to Surgical Technique

The Clinical Outcomes of Damus-Kaye-Stansel Procedure According to Surgical Technique Korean J Thorac Cardiovasc Surg 24;4:344-349 ISSN: 2233-6X (Print) ISSN: 293-656 (Online) Clinical Research http://dx.doi.org/.59/kjtcs.24.4.4.344 The Clinical Outcomes of Damus-Kaye-Stansel Procedure

More information

Indications for the Brock operation in current

Indications for the Brock operation in current Thorax (1973), 28, 1. Indications for the Brock operation in current treatment of tetralogy of Fallot H. R. MATTHEWS and R. H. R. BELSEY Department of Thoracic Surgery, Frenchay Hospital, Bristol It is

More information

Ebstein s anomaly is characterized by malformation of

Ebstein s anomaly is characterized by malformation of Fenestrated Right Ventricular Exclusion (Starnes Procedure) for Severe Neonatal Ebstein s Anomaly Brian L. Reemtsen, MD,* and Vaughn A. Starnes, MD*, Ebstein s anomaly is characterized by malformation

More information

3/14/2011 MANAGEMENT OF NEWBORNS CARDIAC INTENSIVE CARE CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 WITH HEART DEFECTS

3/14/2011 MANAGEMENT OF NEWBORNS CARDIAC INTENSIVE CARE CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 WITH HEART DEFECTS CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 MANAGEMENT OF NEWBORNS WITH HEART DEFECTS A NTHONY C. CHANG, MD, MBA, MPH M E D I C AL D I RE C T OR, HEART I N S T I T U T E C H I LDRE N

More information

Congenital heart disease involving the coronary artery

Congenital heart disease involving the coronary artery Anomalous Coronary Artery With Aortic Origin and Course Between the Great Arteries: Improved Diagnosis, Anatomic Findings, and Surgical Treatment Eldad Erez, MD, Vincent K. H. Tam, MD, Nancy A. Doublin,

More information

M/3, cc-tga, PS, BCPC(+) Double Switch Operation

M/3, cc-tga, PS, BCPC(+) Double Switch Operation 2005 < Pros & Cons > M/3, cc-tga, PS, BCPC(+) Double Switch Operation Congenitally corrected TGA Atrio-Ventricular & Ventriculo-Arterial discordance Physiologically corrected circulation with the morphologic

More information

Recent technical advances and increasing experience

Recent technical advances and increasing experience Pediatric Open Heart Operations Without Diagnostic Cardiac Catheterization Jean-Pierre Pfammatter, MD, Pascal A. Berdat, MD, Thierry P. Carrel, MD, and Franco P. Stocker, MD Division of Pediatric Cardiology,

More information

Corrective Repair of Complete Atrioventricular

Corrective Repair of Complete Atrioventricular Corrective Repair of Complete Atrioventricular Canal Defects and Major Associated Cardiac Anomalies A. D. Pacifico, M.D., A. Ricchi, M.D., L. M. Bargeron, Jr., M.D., E. C. Colvin, M.D., J. W. Kirklin,

More information

S. Bert Litwin, MD. Preface

S. Bert Litwin, MD. Preface 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

More information

Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy Tom R. Karl, MS, MD he most commonly reported coronary artery malformation leading to sudden death in children and young

More information

World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions

World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions All surgeries are Tier 2 surgeries unless otherwise noted. Anomalous Systemic Venous Connection

More information

Coarctation of the aorta is a congenital narrowing of the

Coarctation of the aorta is a congenital narrowing of the Operative Risk Factors and Durability of Repair of Coarctation of the Aorta in the Neonate Walter H. Merrill, MD, Steven J. Hoff, MD, James R. Stewart, MD, Charles C. Elkins, MD, Thomas P. Graham, [r,

More information

An anterior aortoventriculoplasty, known as the Konno-

An anterior aortoventriculoplasty, known as the Konno- The Konno-Rastan Procedure for Anterior Aortic Annular Enlargement Mark E. Roeser, MD An anterior aortoventriculoplasty, known as the Konno-Rastan procedure, is a useful tool for the cardiac surgeon. Originally,

More information

Case 47 Clinical Presentation

Case 47 Clinical Presentation 93 Case 47 C Clinical Presentation 45-year-old man presents with chest pain and new onset of a murmur. Echocardiography shows severe aortic insufficiency. 94 RadCases Cardiac Imaging Imaging Findings C

More information

Tetralogy of Fallot (TOF) with atrioventricular (AV)

Tetralogy of Fallot (TOF) with atrioventricular (AV) Tetralogy of Fallot with Atrioventricular Canal Defect: Two Patch Repair Sitaram M. Emani, MD, and Pedro J. del Nido, MD Tetralogy of Fallot (TOF) with atrioventricular (AV) canal defect is classified

More information

The successful application of the Fontan operation for

The successful application of the Fontan operation for Modified Norwood Operation for Single Left Ventricle and Ventriculoarterial Discordance: An Improved Surgical Technique Ralph S. Mosca, MD, Hani A. Hennein, MD, Thomas J. Kulik, MD, Dennis C. Crowley,

More information

The Edge-to-Edge Technique f For Barlow's Disease

The Edge-to-Edge Technique f For Barlow's Disease The Edge-to-Edge Technique f For Barlow's Disease Ottavio Alfieri, Michele De Bonis, Elisabetta Lapenna, Francesco Maisano, Lucia Torracca, Giovanni La Canna. Department of Cardiac Surgery, San Raffaele

More information

Patients with congenitally corrected transposition of the great arteries

Patients with congenitally corrected transposition of the great arteries Surgery for Congenital Heart Disease Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great

More information

Anomalous Systemic Venous Connection Systemic venous anomaly

Anomalous Systemic Venous Connection Systemic venous anomaly World Database for Pediatric and Congenital Heart Surgery Appendix B: Diagnosis (International Paediatric and Congenital Cardiac Codes (IPCCC) and definitions) Anomalous Systemic Venous Connection Systemic

More information

Long-term results (22 years) of the Ross Operation a single institutional experience

Long-term results (22 years) of the Ross Operation a single institutional experience Long-term results (22 years) of the Ross Operation a single institutional experience Authors: Costa FDA, Schnorr GM, Veloso M,Calixto A, Colatusso D, Balbi EM, Torres R, Ferreira ADA, Colatusso C Department

More information

Twenty-five years ago Jatene and colleagues1 first described the. Twenty-five years experience with the arterial switch operation

Twenty-five years ago Jatene and colleagues1 first described the. Twenty-five years experience with the arterial switch operation Surgery for Congenital Heart Disease Twenty-five years experience with the arterial switch operation P. A. Hutter, MD D. L. Kreb, MD S. F. Mantel, MD J. F. Hitchcock, MD, PhD E. J. Meijboom, MD, PhD, FACC

More information

World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions

World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions World Database for Pediatric and Congenital Heart Surgery Appendix A: Surgical Procedure Terms and Definitions All surgeries are Tier 2 surgeries unless otherwise noted. Anomalous Systemic Venous Connection

More information

In conventional repair of the associated malformations

In conventional repair of the associated malformations Intermediate Results of the Double-Switch Operations for Atrioventricular Discordance Masahiro Koh, MD, Toshikatsu Yagihara, MD, Hideki Uemura, MD, Koji Kagisaki, MD, Ikuo Hagino, MD, Toru Ishizaka, MD,

More information

T who has survived first-stage palliative surgical management

T who has survived first-stage palliative surgical management Intermediate Procedures After First-Stage Norwood Operation Facilitate Subsequent Repair Richard A. Jonas, MD Department of Cardiac Surgery, Children s Hospital, Boston, Massachusetts Actuarial analysis

More information

Data Collected: June 17, Reported: June 30, Survey Dates 05/24/ /07/2010

Data Collected: June 17, Reported: June 30, Survey Dates 05/24/ /07/2010 Job Task Analysis for ARDMS Pediatric Echocardiography Data Collected: June 17, 2010 Reported: Analysis Summary For: Pediatric Echocardiography Exam Survey Dates 05/24/2010-06/07/2010 Invited Respondents

More information

Obstructed total anomalous pulmonary venous connection

Obstructed total anomalous pulmonary venous connection Total Anomalous Pulmonary Venous Connection Richard A. Jonas, MD Children s National Medical Center, Department of Cardiovascular Surgery, Washington, DC. Address reprint requests to Richard A. Jonas,

More information

3 Aortopulmonary Window

3 Aortopulmonary Window 0 0 0 0 0 Aortopulmonary Window Introduction Communications between the ascending aorta and pulmonary artery constitute a spectrum of malformations which is collectively designated aortopulmonary window,

More information

Coarctation of the aorta

Coarctation of the aorta T H E P E D I A T R I C C A R D I A C S U R G E R Y I N Q U E S T R E P O R T Coarctation of the aorta In the normal heart, blood flows to the body through the aorta, which connects to the left ventricle

More information

A Unique Milieu for Perioperative Care of Adult Congenital Heart Disease Patients at a Single Institution

A Unique Milieu for Perioperative Care of Adult Congenital Heart Disease Patients at a Single Institution Original Article A Unique Milieu for Perioperative Care of Adult Congenital Heart Disease Patients at a Single Institution Ghassan Baslaim, MD, and Jill Bashore, RN Purpose: Adult patients with congenital

More information

was judged subjectively. The left ventricle was considered to be slightly hypoplastic when the cardiac

was judged subjectively. The left ventricle was considered to be slightly hypoplastic when the cardiac British Heart J7ournal, 1976, 38, 1124-1132. Double outlet right ventricle Study of 27 cases A. H. Cameron, F. Acerete, M. Quero, and M. C. Castro From the Department of Patlology, Children's Hospital,

More information

Index. cardiology.theclinics.com. Note: Page numbers of article titles are in boldface type.

Index. cardiology.theclinics.com. Note: Page numbers of article titles are in boldface type. Index Note: Page numbers of article titles are in boldface type. A ACHD. See Adult congenital heart disease (ACHD) Adult congenital heart disease (ACHD), 503 512 across life span prevalence of, 504 506

More information

September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical)

September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical) September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical) Advances in cardiac surgery have created a new population of adult patients with repaired congenital heart

More information

Will we face a big problem with the aortic valve/root after ASO?

Will we face a big problem with the aortic valve/root after ASO? Will we face a big problem with the aortic valve/root after ASO? Laurence Iserin Unité médico-chirurgicale de Cardiologie Congénitale Adulte Hôpital Universitaire Européen Georges Pompidou APHP, Université

More information

Hypoplastic Left Heart Syndrome: Echocardiographic Assessment

Hypoplastic Left Heart Syndrome: Echocardiographic Assessment Hypoplastic Left Heart Syndrome: Echocardiographic Assessment Craig E Fleishman, MD, FACC, FASE Director, Non-invasive Cardiac Imaging The Hear Center at Arnold Palmer Hospital for Children, Orlando SCAI

More information

Congenital Heart Disease An Approach for Simple and Complex Anomalies

Congenital Heart Disease An Approach for Simple and Complex Anomalies Congenital Heart Disease An Approach for Simple and Complex Anomalies Michael D. Pettersen, MD Director, Echocardiography Rocky Mountain Hospital for Children Denver, CO None Disclosures 1 ASCeXAM Contains

More information

Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum

Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum Kagami MIYAJI, MD, Akira FURUSE, MD, Toshiya OHTSUKA, MD, and Motoaki KAWAUCHI,

More information

Complete atrioventricular septal defect with tetralogy

Complete atrioventricular septal defect with tetralogy Atrioventricular Septal Defect With Tetralogy of Fallot: Results of Surgical Correction Stacey B. O Blenes, MD, David B. Ross, MD, Maurice A. Nanton, MD, and David A. Murphy, MD Divisions of Cardiovascular

More information

Congenital heart disease: When to act and what to do?

Congenital heart disease: When to act and what to do? Leading Article Congenital heart disease: When to act and what to do? Duminda Samarasinghe 1 Sri Lanka Journal of Child Health, 2010; 39: 39-43 (Key words: Congenital heart disease) Congenital heart disease

More information

Surgical Procedures. Direct suture of small ASDs Patch repair Transcatheter closure with a prosthetic device called occluder

Surgical Procedures. Direct suture of small ASDs Patch repair Transcatheter closure with a prosthetic device called occluder PEDIATRIC Review Surgical Procedures Atrial Septal Defect repair: Direct suture of small ASDs Patch repair Transcatheter closure with a prosthetic device called occluder Balloon atrial septostomy (Rashkind)

More information

Surgery for Congenital Heart Disease. The aortic translocation (Nikaidoh) procedure: Midterm results superior to the Rastelli procedure

Surgery for Congenital Heart Disease. The aortic translocation (Nikaidoh) procedure: Midterm results superior to the Rastelli procedure The aortic translocation (Nikaidoh) procedure: Midterm results superior to the Rastelli procedure Thomas Yeh Jr, MD, PhD, Claudio Ramaciotti, MD, Steven R. Leonard, MD, Lonnie Roy, PhD, and Hisashi Nikaidoh,

More information

Adult Echocardiography Examination Content Outline

Adult Echocardiography Examination Content Outline Adult Echocardiography Examination Content Outline (Outline Summary) # Domain Subdomain Percentage 1 2 3 4 5 Anatomy and Physiology Pathology Clinical Care and Safety Measurement Techniques, Maneuvers,

More information