Results of the Senning procedure in infants with simple and complex transposition of the great arteries

Because of interest in the arterial switch operation, recent experience with the Senning operation in patients with simple and complex transposition of the great arteries (TGA) was examined. Between 1982 and 1985, 35 patients with simple TGA (group I) and 10 patients with complex TGA (group II) underwent a Senning operation. Mean duration of follow-up was 14 months for group I and 24 months for group II. In group I, 1 patient died early and no patient died late; infrequently, right ventricular dysfunction, tricuspid regurgitation, baffle obstruction or arrhythmias occurred. In group II, no patient died early and 3 died late. In addition, many patients required prolonged digoxin therapy. Because the arterial switch operation has a high early mortality risk and an undetermined long-term morbidity and mortality risk, the Senning operation (or other venous switch operations) is considered the preferred surgical approach for simple TGA. In contrast, an arterial switch operation is performed in patients with complex TGA (without subpulmonic obstruction). Comparison of the early and late morbidity and mortality of the arterial vs the venous switch operation may be helpful in selecting the appropriate surgical approach to complex TGA.     

Trans‐apical systemic tricuspid valve‐in‐ring replacement

Transcatheter valve replacement offers a safe and effective alternative to traditional surgical techniques in patients with congenital heart disease, especially those at high surgical risk. The most common causes of morbidity and mortality in patients with D‐transposition of the great arteries status post Senning or Mustard repair is severe tricuspid valve (TV) regurgitation. Replacement of the systemic TV may be useful in those without severe systemic ventricular dysfunction. We present a case of a patient with D‐loop transposition of the great arteries status post Mustard repair and TV ring placement with subsequent severe systemic TV regurgitation, at high surgical risk, who underwent a transcatheter valve replacement via a trans‐apical approach using an Edwards Sapien XT valve.     

Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.     

Arterial switch operation after Mustard procedures in adult patients with transposition of the great arteries: is it time to revise our strategy?

Background

After the Mustard or Senning procedure, adults with transposition of the great arteries may have right ventricular failure and require consideration of new therapies. A 2-stage arterial switch operation (ASO) may be performed as an alternative to heart transplantation. This procedure is relatively successful in children, but little is known about the 2-stage ASO in adults. We report our experience in adults undergoing pulmonary arterial banding as the first stage of a planned 2-stage arterial switch procedure after a failed Mustard operation.

Methods and results

Three adult patients with systemic right ventricular failure late after Mustard procedures embarked, through pulmonary artery banding, on a course toward a 2-stage arterial switch at the Toronto General Hospital. Baseline clinical characteristics as well as preoperative hemodynamics were reviewed. Immediate perioperative and postoperative events, hemodynamic measurements, and clinical outcomes were also recorded. Two patients were banded acutely such that their morphologic left ventricular to right ventricular (LV/RV) systolic pressure ratios were >0.65 after the initial banding procedure. The subpulmonary left ventricle failed in both cases. In contrast, the third patient had a more gradual approach to pulmonary artery banding (PAB), with an initial LV/RV pressure ratio of 0.5, which eventually led to a successful conversion to an arterial switch procedure.

Conclusions

Our evidence suggests that in adult patients expected to undergo a 2-stage arterial switch procedure after a failed Mustard operation, acute PAB achieving near-systemic subpulmonary LV pressure leads rapidly to ventricular failure and failure of this treatment strategy. A more gradual approach to PAB may be required to achieve a successful outcome.     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure)in infants and children with congenitally corrected transpositionof the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitallycorrected transposition underwent surgery at UCSF. Anatomicalrepair was performed in 11 of these patients, at ages rangingfrom 4·8 months to 7·8 years (median 3·2years). The remaining six patients did not undergo anatomicalrepair due to unfavourable anatomy (n=2), prior conduit repair(n=2), biventricular dysfunction (n=1), and isolated completeatrioventricular block (n=1). The 11 patients who underwentanatomical repair make up the study group for the present report.All 11 patients had a malalignment ventricular septal defect,while pulmonary outflow tract obstruction was present in ninepatients and significant tricuspid valve pathology or dysfunctionwas present in five. Anatomical repair was achieved with a Senning(n=7) or a Mustard (n=4) procedure combined with an arterialswitch operation plus ventricular septal defect closure (n=4),or a Rastelli procedure with left ventricle to aortic baffleand right ventricle to pulmonary artery conduit (n=7). Therewas one early death and no patients developed surgical completeatrioventricular block. At a median follow-up of 22 months,there were no late deaths. Two patients required a total ofthree late reoperations, and all patients were asymptomaticon no cardiac medication. Follow-up echocardiography revealednormal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achievedwith low rates of early mortality and surgical heart block,and favourable mid-term results. Long-term follow-up will benecessary to determine if the double switch approach improvesthe natural history of corrected transposition when comparedto less aggressive surgical approaches that leave the rightventricle in the systemic circulation.     

A case series of systemic right ventricular dysfunction post atrial switch for simple D-transposition of the great arteries: the impact of beta-blockade

BACKGROUND: Patients with atrial switch (Mustard or Senning) repair of D-transposition of the great arteries (D-TGA) are at increased risk for atrial arrhythmias, systemic right ventricular (RV) dysfunction and late mortality. OBJECTIVES: To evaluate case series from a single-centre experience with beta-blocker use in adult, post atrial switch, simple D-TGA patients. METHODS: The Adult Congenital Heart Disease Clinic (Halifax, Nova Scotia) database was used to identify patients with post atrial switch, simple D-TGA. Treatment effect of beta-blockade was evaluated. RESULTS: Eight patients were treated with beta-blockers for systemic RV dysfunction (n=2), arrhythmia (n=2) or both (n=4). Median follow-up was three years, at which time seven of eight patients were still on beta-blockade. Of those patients with complete data, two of five had improved systemic ventricular dysfunction, two of four had improved tricuspid regurgitation and four of six had improved functional capacity, as determined by history or exercise testing. Beta-blockade was well tolerated in seven of eight patients without any significant clinical deterioration. CONCLUSIONS: Beta-blockade was used infrequently in patients with a prior Mustard procedure. When beta-blockade was prescribed to patients with a prior atrial switch procedure, the drugs were well tolerated and were associated with trends toward improved symptoms, less tricuspid regurgitation and improved functional status in patients with reduced systemic RV function. These data support the need for a randomized trial of beta-blockade in patients with a previous Mustard or Senning operation and RV dysfunction.     

Transposition of the great arteries

Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.     

Double switch operation in a young infant

We performed a combined Senning and arterial switch operation on a 2-month-old patient with congenitally corrected transposition, Ebstein's malformation producing severe tricuspid regurgitation, ventricular septal defect, pulmonary hypertension, and congestive heart failure. The tricuspid regurgitation was improved. The double switch operation has the advantage of improving the function of the systemic atrioventricular valve, especially in newborns or young infants in whom the outcome of the valvar repair is poor.     

Subxiphoid two-dimensional echocardiographic identification of tricuspid valve abnormalities in transposition of the great arteries with ventricular septal defect

Tricuspid valve morphology was examined using subxiphoid 2-dimensional echocardiography (2-D echo) in 39 infants aged 2 years or younger who had transposition of the great arteries (TGA) and ventricular septal defect (VSD) (group I). Age-matched control groups were 21 patients with simple TGA (group II), 30 patients with VSD and normally related great arteries (group III), and 15 normal patients (group IV). Valve abnormalities, consisting of chordal attachments to the infundibular septum or ventricular septal crest, straddling, overriding or some combination of these, were identified in 25 of 39 patients (64%) in group I, no patients in groups II or IV and 6 of 30 patients (20%) in group III. Intraatrial baffle repair was performed in 27 patients in group I (median age at surgery 3.5 months) and 19 patients in group II (median age 4 months). Preoperative right ventricular angiography, performed in all patients with TGA, demonstrated tricuspid regurgitation (TR) with biventricular dysfunction in 1 patient in group I. After surgery, TR was present in 9 of 17 group I patients and none of the 8 group II patients who underwent catheterization. All patients in whom TR was not present preoperatively had abnormal chordal attachments; 3 required valve replacement. These results demonstrate that tricuspid valve abnormalities are common in patients with TGA and VSD and may be identified preoperatively using 2-D echo. Patients with abnormal chordal attachments are at increased risk for TR after intraatrial baffle repair and should be considered for arterial switch repair.     

Tricuspid Valve Imaging and Intervention in Pediatric and Adult Patients With Congenital Heart Disease

Tricuspid valve abnormalities in congenital heart disease comprise a wide spectrum, with the most common being Ebstein anomaly and tricuspid valve dysplasia. Tricuspid valve dysfunction may also be secondary to other types of congenital heart disease, including functional tricuspid regurgitation seen in right heart volume overload conditions, such as atrial septal defect and repaired tetralogy of Fallot with severe pulmonary valve regurgitation. Congenitally corrected transposition and Mustard and Senning procedures maintain the right ventricle as the systemic ventricle, and the tricuspid valve is subject to unique hemodynamic stress not typically seen in normal circulation. Surgical treatment of tricuspid valve disease remains the mainstay of therapy; primary catheter-based interventions are uncommon. However, once a tricuspid valve has been replaced with a bioprosthesis, the patient may be a candidate for tricuspid valve-in-valve catheter-based replacement.     

左心瓣膜置换术后远期三尖瓣关闭不全的外科治疗

目的:评价左心瓣膜置换术后三尖瓣重度关闭不全外科治疗效果。方法:对25例左心瓣膜置换术后,人工瓣膜功能正常,三尖瓣重度关闭不全患者行三尖瓣成形术或三尖瓣置换术;首次手术二尖瓣置换术17例,二尖瓣加主动脉瓣置换术8例,在首次手术中10例曾行三尖瓣DeVega法成形术。结果:三尖瓣成形术13例;三尖瓣置换术12例。术后早期死亡4例,病死率16%。随访7个月～8年,平均(5.1±2.6)年,2例三尖瓣置换患者死于心血管事件,长期生存15例,心功能Ⅱ级8例,Ⅲ级4例,Ⅳ级3例,多数仍需强心、利尿药维持,临床症状明显改善。结论:对左心瓣膜置换术后三尖瓣重度关闭不全患者外科手术是一种合适的选择。合理掌握手术指征、手术时机和良好的围手术期治疗是手术成功的关键。     

左心瓣膜置换术后孤立性三尖瓣关闭不全的外科治疗及危险因素

目的 明确左心瓣膜置换术后出现远期孤立性三尖瓣关闭不全（tricuspid regurgitation,TR）的外科治疗效果和危险因素.方法 分析2000年1月至2010年12月广东省人民医院因左心瓣膜术后发生远期孤立性重度TR而接受三尖瓣外科治疗的41例患者的资料.三尖瓣成形（tricuspid valve repair,TVP）7例,三尖瓣置换（tricuspid valve replacement,TVR）34例,比较两组的近、远期结果.并比较存活组与术后早期死亡组及术后远期死亡组的临床资料,分析手术早期及远期死亡的危险因素.结果 随访时间（6.3±3.4）年,随访率97％,死亡14例（34.1％）.TVP组术后死于右心功能衰竭1例（14.3％）,无远期死亡;因再发重度TR于术后3.5年行TVR1例.TVR组手术死亡8例,远期死亡5例,死亡率38.2％.因右心功能衰竭死亡9例,感染性心内膜炎、脑出血、猝死、结肠癌术后肝转移死亡各1例.TVP组死亡率有高于TVR组的趋势（P=0.43）.手术早期死亡组三尖瓣反流面积、肌酐值明显高于与存活组,差异有统计学意义（P＜0.05）;而术后远期死亡组右心室大小、肌酐值明显高于存活组,差异有统计学意义（P＜0.05）.结论 左心瓣膜置换术后远期孤立性TR的再次手术死亡率高,选择TVP将有助于降低死亡率.三尖瓣反流程度重、术前肌酐值升高是手术早期死亡的危险因素;右心室大小和术前肌酐值升高是术后远期死亡的危险因素.     

The vectorcardiogram in 4 children undergoing the arterial switch repair

Four children with complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections), aged 1.2 to 16.4 years, underwent a delayed repair by the arterial switch procedure from March 1985 to October 1986. Three children had undergone previous atrial diversion repairs in early childhood. Two of these children had developed right ventricular dysfunction with tricuspid incompetence as well as having recurrent and symptomatic supraventricular tachyarrhythmias. They underwent banding of the pulmonary trunk 0.7 and 1 year prior to the final repair. The third child had dynamic obstruction of the left ventricular outflow tract and prior banding was not performed. A fourth child had right ventricular dysfunction on initial assessment in early infancy and then had banding of the pulmonary trunk. Two patients survived the arterial switch operation. Vectorcardiograms were performed on these 4 children before arterial switch repair. The left maximal spatial voltage did not correlate with elevated left to right ventricular pressure ratio at cardiac catheterization before arterial switch repair nor did it relate to survival after operation. The horizontal loop was clockwise in all patients preoperatively. The vectorcardiogram in these children was not a useful indicator that the left ventricle was suitably prepared for the arterial switch repair.     

Experience with an adjustable pulmonary artery banding device in two cases: initial success--midterm failure

Retraining of the left ventricle in congenitally corrected TGA or after Senning or Mustard operation is necessary when right-ventricular failure is developing and an arterial switch operation is indicated. As these hearts have little tolerance of marginal overbanding, a long-term adjustable pulmonary artery banding device would lower stress and risk of training. Although the inserted device (Osypka) allowed convenient intraoperative pressure ratio adjustment, mid-term adjustment failed due to dysfunction of the system.     

Medium-term outcome of anatomically repaired congenitally corrected transposition: the double switch operation

BACKGROUND: The double switch operation is emerging as the procedure of choice for congenitally corrected transposition of the great arteries. However, rhythm disturbances in the postoperative period are rarely discussed. METHODS AND RESULTS: Eighteen survivors who underwent corrective surgery for congenitally corrected transposition of the great arteries were followed up. Patients in group I (n=8), who also had a ventricular septal defect and pulmonary stenosis, had undergone the Senning plus Rastelli operation. Patients in group II (n=10), who did not have pulmonary stenosis, had undergone the Senning and arterial switch operation. The patients were followed up by periodical clinical examination, echocardiography and 24-hour Holter monitoring. In group I, follow-up ranged from 24 to 66 months (mean 44 months). There was no late death and all the patients are symptom free. There was no significant atrioventricular valve regurgitation and left ventricular function was normal. There were no rhythm disturbances. In group II, follow-up ranged from 2 to 72 months (mean 48 months). There were 2 late deaths due to atrial tachyarrhythmia and residual pulmonary hypertension 36 and 8 months after the procedure, respectively. One patient had significant mitral regurgitation and required mitral valve replacement. Three patients had recurrent atrial/junctional tachyarrhythmia: one of them was lost to follow-up after 1 year while another died of resistant atrial tachyarrhythmia. The third patient underwent mitral valve replacement for severe mitral regurgitation and developed complete heart block necessitating a permanent pacemaker implantation. CONCLUSIONS: Though good long-term results are obtained following the double switch operation, the problem of atrial arrhythmias still needs to be addressed suitably.     

Ebstein malformation of the tricuspid valve: Current concepts in management and outcomes

Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.     

Ebstein's malformation. surgical treatment and results

Ebstein's malformation can be defined as an anomaly of the tricuspid valve existing in the setting of a right ventricular dysfunction. The technique introduced by Carpentier in 1980 is based on the concept of mobilization of the restrictive anterosuperior leaflet associated with a longitudinal plication of the inlet component of the right ventricle. From January 1980 to December 1999, 142 patients underwent surgery. The mean age was 25 +/- 15 years (1-65). Cyanosis was present in 48% and associated lesions in 64% of the patients. Patients were classified using a functional approach according to the severity of the lesions. Mild displacement of the septal leaflet, along with small size of the atrialized chamber was seen in 5% (referred to as Type A). Massive displacement of the septal leaflet, but with normal motion of the anterosuperior leaflet and an extensive atrialized chamber, was seen in 35% (Type B). In 51%, the mural (inferior) leaflet was absent, the anterosuperior leaflet was severely restricted by muscular trabeculations and very short tendinous cords, and the anterolateral papillary muscle was incorporated in the right ventricular wall. In these patients (Type C), the atrialized chamber was markedly enlarged and had dyskinetic walls. In such cases, the contractility of the distal (functional) right ventricle was also impaired, and some degree of stenosis of the tricuspid valve was present in one-fifth of them. In the most severe cases (8%), the leaflet tissue of the valve was extremely reduced and the right ventricular walls were thin and contracted poorly. This resulted in the so-called tricuspid sack arrangement (Type D). Valve replacement was needed in only 4 cases, with conservative surgery being achieved in 138 patients by means of mobilization of the anterosuperior leaflet and longitudinal plication of the inlet component of the right ventricle. Additional procedures included the use of a prosthetic ring (94 patients) and partial Glenn anastomosis (30 patients). The hospital mortality was 10%, mainly due to acute postoperative right ventricular failure. Actuarial survival was 75% at 10 years. After operation, 94% of the patients were in functional class I or II of the New York Heart Association, and 88% had no or mild tricuspid valve insufficiency as judged by echocardiography. The rate of reoperation was 9% with a mean delay of 3 years. A second repair was performed in 5 patients. Freedom from reoperation was 87% at 10 years. Sinus rhythm was present in 81%, and 8 pacemaker devices were implanted, 5 for surgically induced atrioventricular block, and 3 because of preoperative conduction disturbances. The use of the partial Glenn anastomosis was introduced recently in cases where the right ventricular contractility was severely impaired, and/or tricuspid valve repair was difficult, and/or permanent atrial fibrillation was present. In those patients with high risk, adding partial Glenn anastomosis reduced the operative mortality from 24% to 6%. Another benefit of the cavo-bipulmonary anastomosis was better functional tolerance of mild residual tricuspid valve incompetence. Those patients with the tricuspid sack arrangement had a high rate of reoperation (2/11) and valve replacement (3/11), but suffered no operative deaths. We conclude that tricuspid valvoplasty associated with longitudinal right ventricular plication is superior to valve replacement. The arrangement producing a tricuspid sack is not suitable for conservative surgery. An associated cavo-pulmonary anastomosis decreases the operative mortality in patients at high risk, and seems to preserve right ventricular function.     

Current results of management in transposition of the great arteries, with special emphasis on patients with associated ventricular septal defect

Two hundred forty-five patients less than 15 days of age with transposition of the great arteries with or without a ventricular septal defect or pulmonary stenosis were entered into an ongoing 20 institution treatment study between January 1, 1985 and June 1, 1986. Complete follow-up is available on all patients. The ventricular septal defect narrowed in only 1 of 36 patients with combined transposition of the great arteries and ventricular septal defect; pulmonary stenosis developed or worsened in 3 of these 36 patients and in 3 of the 187 patients with simple transposition. Twelve month overall survival among the 245 patients was 80%. No morphologic feature of transposition was a risk factor for death but major associated cardiac and noncardiac anomalies (more common in patients with combined transposition and ventricular septal defect) and low birth weight were risk factors. Neither arterial switch repair (n = 86), atrial switch (Mustard) repair (n = 21) nor atrial switch (Senning) repair (n = 39) was a risk factor for death, but results in all surgical groups were better in the last part of the experience. Death before repair was less frequent late in the study. Possibly, in low birth weight infants, survival was better with the arterial than with the atrial switch repair. These data suggest that survival at 1 year is similar with either the arterial or the atrial switch repair. The early results of repair of combined transposition of the great arteries and ventricular septal defect are as good as those of simple transposition. Special institutional efforts are required to attain good results with the arterial switch repair and to prevent death before repair.     

Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22-29 years).

BACKGROUND: Great concern exists about the ability of the anatomic right ventricle to sustain the systemic circulation in patients with transposition of the great arteries who have undergone a Mustard procedure. A prospective study was made to examine long-term survival, clinical outcome, and right ventricular function 25 years after surgery. METHODS: Ninety-one consecutive patients underwent the Mustard procedure between 1973 and 1980. After 14 years and again after 25 years (range 22-29 years), patients were studied with ECG, echocardiography, exercise testing, and Holter monitoring. RESULTS: The cumulative survival and event-free survival were 77% and 36%, respectively, after 25 years. Reoperation was necessary in 46%. No major loss of sinus rhythm was found. While all patients had good right ventricular function 14 years after repair, 61% of patients showed moderate-to-severe dysfunction after 25 years, when studied by echocardiography. Furthermore, the QRS complex widened and exercise capacity decreased. CONCLUSION: The anatomic right ventricle appears to be unable to sustain the systemic circulation at long-term follow-up and the clinical condition of patients late after Mustard repair is declining. We can expect more deaths or need for heart transplantation in the next decade.     

Use of multisite electroanatomic mapping to facilitate ablation of intra-atrial reentry following the Mustard procedure

Ablation of intra-atrial reentrant tachycardia following Mustard or Senning procedures has low success rates. The Biosense Carto system was used to map intra-atrial reentry in a 22-year-old woman who had undergone a Mustard procedure. A line of block was created connecting a Mustard baffle suture line to the tricuspid valve annulus, which terminated the arrhythmia and prevented its reinitiation. Multisite electroanatomic mapping was invaluable in defining atrial anatomy and the intra-atrial reentrant pathway, and in creating a contiguous line of block. This mapping may improve ablation success rates in patients following the Mustard or Senning repair.