Transatrial-Transpulmonary Repair of Atrioventricular Septal Defect with Right Ventricular Outflow Tract Obstruction

A bstract Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence. ( J Card Surg 1993; 8:622–627 )     

Repair of complete atrioventricular septal defect with tetralogy of Fallot

Repair of complete atrioventricular canal with tetralogy of Fallot was performed in 9 patients. Ventricular septal defect was closed through the right atrium using a single polytetrafluoroethylene patch with ample anterior extension to avoid subaortic obstruction. The atrial septal defect was closed with a separate patch. Undivided atrioventricular valve leaflets were sandwiched between the two patches. Right ventricular outflow tract stenosis was relieved by pulmonary valvotomy and an infundibular patch in 7, a supravalvar patch (none transannular) in 6, and right ventricle-to-pulmonary artery conduit in 2. There was one hospital death (1/9, 11%) in a patient with persistent clinically significant postoperative pulmonary stenosis and low cardiac output requiring reoperation and right ventricle-to-pulmonary artery conduit insertion. There was no late mortality. All patients are asymptomatic 0.3 to 5.6 years after operation. Follow-up right ventricular outflow tract gradient ranged from 11 to 43 mm Hg and was 70 mm Hg in 1 patient who later had successful relief of obstruction. Three patients had mitral valve insufficiency; 1 needed reoperation. Aggressive relief of right ventricular outflow tract stenosis with maintenance of pulmonary valve competence and use of two separate patches for closure of the septal defects contribute to optimum immediate and long-term results after repair of this lesion.     

Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair.

This report describes our experience with 366 patients who had a transatrial, transpulmonary repair of tetralogy of Fallot between December 1980 and December 1991. Included in this group are patients with tetralogy of Fallot plus atrioventricular septal defect as well as patients displaying all degrees of aortic override (in the presence of subaortic ventricular septal defect and right ventricular outflow tract obstruction). Median age was 15.3 months and median weight, 12.3 kg. Of the 366 patients, 72% required a pericardial patch to reconstruct the main pulmonary artery or right ventricular outflow tract. Serious coronary anomalies were seen in 11 patients, without influencing surgical approach. There were two hospital deaths (0.5%; 70% confidence limits, 0.2% to 1.2%). Actuarial survival was 97.5% at 42 months (95% confidence limits, 95% to 99%) reflecting four late deaths over 1,129 patient-years of follow-up. Postoperative cardiac catheterization studies were performed in 61 patients at a mean follow-up interval of 23 months. Mean right ventricular/left ventricular systolic pressure ratio after repair was 0.46 (standard deviation, 0.28), and mean gradient across the right ventricular outflow tract was 15 mm Hg (standard deviation, 24 mm Hg). Actuarial freedom from reoperation for any reason has been 95% (95% confidence limits, 92% to 97%) at 5-year and 10-year follow-up. These early and medium-term results encourage us to continue with transatrial, transpulmonary repair of tetralogy of Fallot. We believe that this approach has an operative risk similar to or lower than transventricular repair, and that it will result in better preservation of right ventricular function in the long term.     

Aneurysm of pericardial right ventricular outflow tract patches.

Among 1,022 patients who underwent repair of tetralogy of Fallot, 252 received a pericardial patch of the right ventricular outflow tract; of these, 10 subsequently developed an aneurysm of the right ventricular outflow tract. Cardiac catheterization and angiography revealed moderate pulmonary insufficiency in all patients, a residual pressure gradient in the right ventricular outflow tract in 7, and a residual ventricular septal defect in 2 patients. Reoperation was indicated in 8 patients because of progressive distention of the aneurysm, residual infundibular or pulmonary artery stenosis, and recurrent ventricular septal defect. Reconstruction of the right ventricular outflow tract was accomplished by resection of the aneurysm and insertion of a woven Dacron patch in 5 patients, primary suture of the pulmonary artery in 2, and implantation of a woven Dacron conduit containing a Björk-Shiley cardiac valve prosthesis in 1 patient. There were no early or late deaths. When reconstruction of the right ventricular outflow tract is necessary, we recommend a woven Dacron patch because pericardium may form an aneurysm.     

Tetralogy of Fallot with subarterial ventricular septal defect

BACKGROUND: Tetralogy of Fallot with subarterial ventricular septal defect is frequently seen among Asians. Compared with infracristal ventricular septal defect, postoperative right ventricular outflow obstruction is more likely because of subpulmonary extension of the defect. Moreover the incidence of aortic regurgitation is a concern because of the absence of a supporting infundibulum. METHODS: Four hundred cases of classic tetralogy were reviewed, 61 of which had subarterial ventricular septal defect. RESULTS: Aortic regurgitation (of more than mild degree) was identified in 7 cases with subarterial and 7 with infracristal ventricular septal defects. The mechanism of infracristal defect was predominantly an annular dilation before surgery. In contrast 5 cases with subarterial defect had progression of aortic regurgitation after operation yielding an actuarial incidence of 29.7% at 20 years. In 2 patients the cause seemed to be fixation or plication of the aortic valve annulus by the ventricular septal patch. Compared with infracristal defect, subarterial defect was associated with increased incidence of reoperation (12.0% versus 1.9% at 10 years, p = 0.01), frequent use of transannular patch (70.5% versus 45.7%, p = 0.0004), and worse New York Heart Association (NYHA) functional class (p = 0.007). Right ventricular outflow obstruction was the reason for reoperation in 3 patients with subarterial defect and was associated with worse NYHA classification in the long-term, on multivariate analysis (p = 0.0002). CONCLUSIONS: Tetralogy with subarterial ventricular septal defect was associated with worse functional outcome. To prevent adverse outcomes, precise suturing of the distal ventricular septal patch, extensive infundibulectomy, lower threshold for transannular incision, and smaller-sized ventricular septal patch placement are warranted.     

Tetralogy of Fallot: Everything you wanted to know but were afraid to ask

Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow tract obstruction (RVOT) with an occurrence of 3.9 /10,000 births. The pathophysiologic effects in TOF are largely determined by the degree of RVOT and not the VSD. Intra‐operative anesthetic management is also dependent on the degree of RVOT obstruction and influenced by the extent of surgical RVOT repair.     

Absent pulmonary valve associated with tetralogy of Fallot and complete atrioventricular septal defect: report of a case.

A male infant with an extremely rare combination of absent pulmonary valve, tetralogy of Fallot and atrioventricular septal defect presented without symptoms of respiratory distress or congestive heart failure. He underwent successful primary repair at the age of 5 months. The procedure consisted of double-patch repair of the atrioventricular septal defect and right ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of a dilated pulmonary artery was not required. The patient is doing well without any symptoms 5 years after repair.     

Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects

BACKGROUND: Junctional ectopic tachycardia is a major cause of postoperative morbidity after surgery for congenital cardiac disease. To elucidate the mechanism of junctional ectopic tachycardia, surgical correlations were studied in four types of congenital heart defects involving closure of a ventricular septal defect, relief of right ventricular outflow tract obstruction, or both. METHODS: Between 1997 and 1999, a total of 343 consecutive patients underwent repair of tetralogy of Fallot (n = 114), common truncus arteriosus (n = 10), ventricular septal defect (n = 161), and atrioventricular septal defect (n = 58). Variables studied included demographic and bypass data, surgical approaches toward ventricular septal defect closure and relief of right ventricular outflow tract obstruction, and resection as opposed to division of muscle bundles. RESULTS: Junctional ectopic tachycardia occurred most frequently after repair of tetralogy of Fallot (n = 25; 21.9%), with no cases occurring after repair of common trunk, 6 occurring after repair of ventricular septal defect (3.7%), and 6 occurring after repair of atrioventricular septal defect (10.3%). Stepwise logistic regression revealed that resection of muscle bundles (P <.0001), higher bypass temperatures (P <.03), and relief of right ventricular outflow tract obstruction through the right atrium (P <.05) significantly and independently predicted postoperative junctional ectopic tachycardia. CONCLUSIONS: Relief of right ventricular outflow tract obstruction appears to be more important in the causation of junctional ectopic tachycardia than does ventricular septal defect closure, which may explain the higher incidence of this complication after tetralogy of Fallot repair. Muscular resection seems to be more arrhythmogenic than is simple division. Increased traction through the right atrium for relief of right ventricular outflow tract obstruction would fit the hypothesis that enhanced automaticity of the His bundle, the morphologic substrate for junctional ectopic tachycardia, may result from direct trauma or infiltrative hemorrhage of the conduction system. When feasible, techniques avoiding both extensive muscle resection and excessive traction should be applied during resection of right ventricular outflow tract obstruction.     

Dedicated transpulmonic approach for the repair of Fallot's tetralogy

Background To share our initial experience with cases of Tetralogy of Fallot (TOF) in whom transpulmonary artery route was exclusively used for repair of the defect. Material & Methods Twenty patients, 11 males and 9 females were carefully selected on the bases of anatomical configuration suitable for repair without trans annular patch. All underwent echocardiography. Angiocardiography was performed in patients. They had no other cardiac vascular anomaly. Results Of the 20 patients, there was one death following massive endotracheal bleeding. Four patients had low cardiac output and required prolonged inotropic support and recovered completely. All 19 survivors have been followed (1–56 months) are with echocardiography. There were no residual or recurrent ventricular septal defect (VSD), significant right ventricular (RV), outflow obstruction or tricuspid regurgitation. Four patient who underwent annualr enlargement have minimal pulmonary regurgitation. Conclusions In selected patients transpulmonic repair of TOF is feasible and safe.     

Simplified technique for pericardial patch of the right ventricular outflow tract

Pericardial patching to enlarge the right ventricular outflow tract is often required in congenital cardiac surgery in lesions such as tetralogy of Fallot, pulmonary atresia, pulmonary stenosis, and pulmonary artery repair following removal of a palliative pulmonary artery band. A method of simplifying the attachment of the pericardial patch onto the right ventricular outflow tract is described.     

Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options

Anomalous origin of the left anterior descending coronary artery from the right coronary artery can interfere with the location of the usual ventriculotomy during repair of tetralogy of Fallot. The purpose of this study was to compare the results of two operative techniques: (1) a "tailored" right ventricular incision and outflow patch reconstruction and (2) placement of a conduit from the right ventricle to the main pulmonary artery. We reviewed the records of 416 patients who had complete repair of tetralogy of Fallot at the Mayo Clinic from 1973 through 1984. Twenty (5%) (median age 6.5 years) had anomalous origin of the left anterior descending coronary artery from the right coronary artery. Twelve of these patients had right ventricular outflow patch reconstruction, and eight had placement of a conduit from the right ventricle to the pulmonary artery. Three deaths occurred, all during hospitalization, two in the patch reconstruction group and one in the conduit group. The average reduction in right ventricular pressure postoperatively was slightly but not significantly greater for the conduit group. These data indicate that correction of tetralogy of Fallot with anomalous origin of the left anterior descending coronary artery can be done with either patch reconstruction or conduit placement. Selection of the more appropriate procedure depends on the exact location and degree of tortuosity of the anomalous artery and the level and severity of right ventricular outflow obstruction.     

Complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: a report of successful surgical treatment

Six patients, 4 of whom had complete atrioventricular (AV) canal and tetralogy of Fallot (TOF) and 2 of whom had double-outlet right ventricle with subaortic ventricular septal defect (VSD) and right ventricular outflow tract obstruction, were treated. Two of the patients with complete AV canal and TOF had a shunt procedure only. The other 4 patients underwent complete repair. All 6 patients survived the operation. Complete repair was performed through the right atrium using the two-patch technique. The size and shape of the VSD patch is important. Residual VSD after repair was common. Two patients have undergone successful reoperation for this problem. Another patient has a small residual VSD.     

Surgical Treatment of Double-Outlet Left Ventricle in Situs Inversus {I,D, D}

A case of double-outlet left ventricle {I, D, D} with a subaortic ventricular septal defect and pulmonary stenosis is reported. The diagnosis was made at operation and was reconfirmed postoperatively by two-dimensional echocardiography. Repair was achieved by patch closure of the ventricular septal defect and enlargement of the functional right ventricular outflow tract using a valved patch. The postoperative course was uneventful except for a transient complete heart block. To our knowledge, this represents the second instance of surgical treatment of double-outlet left ventricle {I, D, D} reported in the literature.     

136例成人法乐四联症的外科治疗

目的探讨外科治疗成人法乐四联症的经验。方法从１９７５年４月至１９９７年１２月,对１３６例１４周岁以上成人法乐四联症施行根治术,最大年龄为５２岁。室间隔缺损修补材料中,１１２例采用涤纶补片,２４例采用自体心包补片。在１２６例使用补片扩大右心室流出道的病例中,９５例采用牛心包补片,３１例采用自体心包补片。结果住院死亡５例,住院病死率为３６８％。术后发生低心排综合征１７６％、心律失常１４０％、胸内出血需再次开胸止血１１８％、胸腔积液２１３％、心包积液５９％、肺部感染１２５％、感染性心内膜炎２２％。结论成人法乐四联症根治术有其自身特点,通过改进手术技术,加强术后监护处理,可以提高手术成功率     

Transcatheter pulmonary valve implantation in native pulmonary outflow tract using the Edwards SAPIEN? transcatheter heart valve

Percutaneous pulmonary valve implantation (PPVI) is now an accepted alternative option to conventional surgery for patients with dysfunctional conduits between the right ventricle and pulmonary artery. PPVI will reduce the total number of repeat operations in such patients. However, surgery remains the primary option in postoperative tetralogy of Fallot patients with severe pulmonary regurgitation who underwent transannular patch reconstruction of their right ventricular outflow tract (RVOT). Traditionally, an RVOT patch is considered a relative contraindication to PPVI, however, in selected patients PPVI was successfully performed. We report the case of a 12-year-old patient after neonatal repair of tetralogy of Fallot and pulmonary atresia, who developed advanced liver disease and severe pulmonary regurgitation. In this patient, the risk for surgical valve replacement was considered too high and he was treated with percutaneous implantation of the Edwards SAPIEN? transcatheter heart valve.     

Reoperation of tetralogy of Fallot for the transannular patch neo-intimal stenosis: a case report]

A 3-year-old male patient underwent right ventricular outflow tract reconstruction with a glutaraldehyde-preserved equine pericardium for tetralogy of Fallot. Because of progressive severe pulmonary restenosis with over systemic right ventricular pressure, tricuspid regurgitation, and abnormal high echoic shadow in the distal main pulmonary artery on echocardiogram, he required reoperation a year after the first correction. In the reoperative findings, the pseudointima was thickened heavily and detached from glutaraldehyde-preserved equine pericardial patch. The patch was removed and the right ventricular outflow was reconstructed widely to the pulmonary bifurcation with porcine pericardial patch again. Patho-histological findings showed foreign body giant cells and macrophages in the pseudointima. Four years after the reoperation, echocardiogram shows 41 mmHg for the right ventricular pressure and 22 mmHg for the pressure gradient of right ventricular outflow tract, and the patient is doing well now.     

Recent results in the surgical treatment of Fallot's tetralogy.

Tetralogy of Fallot consists essentially of two abnormalities: a large, unrestrictive ventricular septal defect and severe obstruction to the outflow of the right ventricle. A clear understanding of the principles described in this article should result in a mortality of under 5% after complete correction of tetralogy of Fallot.     

法洛四联症患儿术前镇静的研究进展

背景 法洛四联症(tetralogy of fallot,TOF)是一组心脏缺损性疾病,包括圆锥-心室室间隔缺损(ventricular septal defect,VSD)、主动脉骑跨、右心室肥厚和轻重不等的右心室流出道梗阻(right ventricular outflow tract,RVOT),合并不同程度的心脏畸形,病情随梗阻程度不同而异,多数患儿在1岁内行TOF根治术.对此类患儿,术前评估与恰当的术前镇静方案对减轻RVOT,减少右向左分流,避免术前缺氧发作是相当重要的,对麻醉诱导前病情的稳定以及麻醉诱导的安全过渡也是十分重要的. 目的 旨在了解TOF术前评估方法、术前镇静方案,为临床用药提供参考. 内容 阐述TOF患儿的病理生理、术前评估、现有术前镇静方案及不同镇静方案对TOF血流动力学的影响. 趋向 未来的研究方向应致力于更加安全、有效的术前镇静方案以及不同术前镇静药物对诱导用药的相互作用.     

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14–64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.     

Long-term results after early primary repair of tetralogy of Fallot.

OBJECTIVE: Early primary repair of tetralogy of Fallot has been routinely performed at Children's Hospital, Boston, since 1972. We evaluated the long-term outcome of this treatment strategy including the influence of a transannular patch. METHODS: Fifty-seven patients less than 24 months of age (median 8 months) underwent primary repair of tetralogy of Fallot between January 1972 and December 1977. Thirty-one patients had a transannular patch. Survival and freedom from reintervention were determined by the Kaplan-Meier method with 95% confidence intervals. RESULTS: There were 8 early deaths, and 1 patient died 24 years after initial repair. Recent follow-up was obtained for 45 of the 49 long-term survivors (92%). Median follow-up was 23.5 years. Ten patients underwent reintervention, 8 of whom underwent relief of right ventricular outflow tract obstruction. Right ventricular outflow tract obstruction occurred in 6 patients without a transannular patch and 2 with a transannular patch (33% vs 6%, P =.04). One pulmonary valve replacement was performed at another institution 20 years after the repair. Forty-one long-term survivors were in New York Heart Association class I and 4 were in class II. Actuarial survival was 86% at 20 years (95% confidence intervals = 80%-92%). Freedom from reintervention was 93% at 5 years (95% confidence intervals = 87%-99%) and 79% at 20 years (95% confidence intervals = 70%-86%). No significant differences were found between patients with and without a transannular patch (survival, P =.34; freedom from reintervention, P =.09, log-rank tests). CONCLUSIONS: Long-term survival is excellent and the freedom from reintervention is satisfactory after early primary repair of tetralogy of Fallot in the 1970s. Use of a transannular patch does not reduce late survival and is associated with a lower incidence of right ventricular outflow tract obstruction.