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.     

Ventricular outflow tracts after Kawashima intraventricular rerouting for double outlet right ventricle with subpulmonary ventricular septal defect.

OBJECTIVE: To determine whether or not the ventricular outflow tracts can be efficiently constructed in patients with double outlet right ventricle with subpulmonary ventricular septal defect by the Kawashima intraventricular rerouting in which the morphologically right ventricular outlet is divided into two, one for the systemic and the other for the pulmonary circulations. METHODS: The intraventricular rerouting procedure was carried out in nine patients with this particular malformation. Age at repair ranged from 35 days to 3 years old. The distance between the attachments of the tricuspid and the pulmonary valves was 10 mm or greater in all except one patient in whom the measured value was 3 mm. Resecting subaortic musculature appropriately, a tailored patch, either oval-shaped (in seven) or heart-shaped (in two), was placed to construct an unobstructed channel for the left ventricular outflow tract with its diameter greater than that of the anticipated normal aortic orifice at the time of repair. For an unobstructed channel to the pulmonary arteries, enlargement of the right ventricular outflow tract was carried out using a patch in six. RESULTS: All patients survived the operative procedure. On postoperative catheterization, mean pulmonary arterial pressure was 15 +/- 8 mmHg, and cardiac index was calculated as 3.3 +/- 0.6 l/min per m2. It proved that the constructed left ventricular outflow tract can become larger in the longer term. Pressure gradient across the left ventricular outflow tract was greater than 20 mmHg in two patients in the intermediate term. One of these two underwent reoperation for the obstruction 10 years after the initial repair. It was suspected that use of a heart-shaped internal conduit, which seems to result from inadequate conal resection, was one of the possible causes of such obstruction in the longer term. Pressure gradient of 47 mmHg was seen across the right ventricular outflow tract in one patient, although this patient has undergone no reoperation. Enlargement of the right ventricular outflow tract could minimize postoperative obstruction for the pulmonary pathway. CONCLUSIONS: The intraventricular rerouting remains one of the attractive surgical options for repair in this particular setting, in terms of successful construction of the ventricular outflow tracts.     

Late results of total correction of tetralogy of Fallot in adults

Between 1966 and 1986, 30 patients underwent total correction of the tetralogy of Fallot. Preoperative presenting features were: dyspnoea on exertion, clubbing, cyanosis and polycythaemia. Twenty-six patients had one or more palliative procedures prior to definitive repair. Preoperatively, all patients had a significant gradient across the right ventricular outflow tract (mean gradient 70 ± 46 mmHg). Peak right ventricle to left ventricle systolic pressure ratio (pRV/LV) was 0.9 ± 0.2. A functioning Blalock Taussig shunt was ligated in 11 patients prior to the institution of cardiopulmonary bypass. All patients had a patch closure of the ventricular septal defect. An additional muscle bundle resection from the right ventricular outflow tract was performed in 15, pulmonary valvotomy in 6 and enlargement of the right ventricular outflow tract in 2 patients. There was a significant fall in pRV/LV ratio postoperatively (P < 0.05). There were 3 early and 2 late deaths. Mild right ventricular outflow tract obstruction has persisted in all survivors. Four patients have remained on antiarrhythmic drugs. Long term results after definitive repair were satisfactory in this group of adult patients who have survived due to palliative procedures performed during childhood.     

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.     

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.     

A valved conduit replacement for a calcified homograft 12 years following a Rastelli operation: a case report

A 25 year-old male with transposition of great arteries, ventricular septal defect and pulmonary stenosis successfully underwent Rastelli procedure with aortic homograft in 1969. After the procedure he grew up and became an engineer without any complaints. However, gradually his homograft degenerated with calcification and the pressure gradient between pulmonary artery and right ventricle increased to 77 mmHg in systolic phase. Twelve years after the conduit repair, we operated upon and enlarged the ventricular septal defect, repaired the residual interventricular shunt, removed his homograft and, replaced it with the Hancock valved conduit from the right ventricular outflow tract through cardiopulmonary bypass. On postoperative cardiac catheterization, the pressure gradient decreased, and the patient returned to his work.     

Noonan syndrome associated with anomalous coronary artery and other cardiac defects

This case-report describes an 11-month-old infant, who had been diagnosed with Noonan syndrome with a variety of associated anomalies. Multiple cardiac anomalies were present, consisting of dysplastic pulmonary valve, symmetric biventricular hypertrophy, atrial septal defect and right ventricular outflow tract (RVOT) obstruction, in which the pressure gradient measured 73 mmHg, and anomalous coronary artery. Systolic anterior motion of the mitral valve (SAM) was present, without remarkable clinical significance. RVOT transannular repair with non-cusped xenograft along with resection of hypertrophied right ventricular outflow myocardium. Left ventricular outflow tract (LVOT), which had no clinical sign of obstruction, was left untouched, expecting the RVOT repair also effectively release LVOT dynamic obstruction. The postoperative echocardiography revealed residual SAM without significant pressure gradient through LVOT.     

Noonan syndrome associated with anomalous coronary artery and other cardiac defects.

This case-report describes an 11-month-old infant, who had been diagnosed with Noonan syndrome with a variety of associated anomalies. Multiple cardiac anomalies were present, consisting of dysplastic pulmonary valve, symmetric biventricular hypertrophy, atrial septal defect and right ventricular outflow tract (RVOT) obstruction, in which the pressure gradient measured 73 mmHg, and anomalous coronary artery. Systolic anterior motion of the mitral valve (SAM) was present, without remarkable clinical significance. RVOT transannular repair with non-cusped xenograft along with resection of hypertrophied right ventricular outflow myocardium. Left ventricular outflow tract (LVOT), which had no clinical sign of obstruction, was left untouched, expecting the RVOT repair also effectively release LVOT dynamic obstruction. The postoperative echocardiography revealed residual SAM without significant pressure gradient through LVOT.     

Complete Surgical Repair of Tetralogy of Fallot in Adults,Is It Ever Too Late?

A minority of patients with Tetralogy of Fallot (TF) survive into adulthood. These patients have been subjected to a prolong period of cyanosis and hypoxia. The aim of this study is to assess the benefits of total correction of TF in this adult population. From August 1995 to March 2005, fifty one patients underwent total correction of TF. The mean age was 22.2 years (range 16 to 38 years). There were 31 males and 20 females. Twenty two percent of patients were in NYHA functional class III prior to the operation. The mean gradient across the right ventricular outflow tract was 81.7 mmHg (range 30 to 130 mmHg). The operation was performed through the right ventricle in the majority of patients. Transannular patch was used in 33 patients. The mean follow-up period was 42 months ranging from 1 to 116 months. Hospital mortality was 1.9% (1 patient), and one patient died three months after the operation. Post-operatively 87.3% of patients were in NYHA functional class I. During the follow-up period four patients required re-operation; two for residual ventricular septal defect, one for residual pulmonary stenosis and one had pulmonary valve replacement for severe pulmonary regurgitation. Complete repair of TF in adults is feasible with acceptable mortality and morbidity. The main benefit of the operation is functional improvement in this patient population.     

Early primary repair of tetralogy of Fallot

Young age, low weight, and the requirement for transannular patch reconstruction of the right ventricular outflow tract (RVOT) are thought to adversely affect intracardiac repair of tetralogy of Fallot. Forty patients underwent complete repair between January, 1984, and January, 1987. Only infants with pulmonary atresia, complete atrioventricular canal, or coronary artery anomalies were shunted initially. The malalignment ventricular septal defect was closed with a Dacron patch. Infundibular resection was minimized. All atrial communications were left open. Thirty-four patients (85%) had a transannular RVOT patch, and 2 patients (5%) had a nontransannular patch. All 10 infants weighing 3.4 to 9.6 kg had a transannular RVOT patch at 7 weeks to 12 months of age. An RVOT patch was used in 26 of 30 children operated on between 1 and 6 years of age (median age, 24 months). No patient undergoing intracardiac repair died. Postoperative RVOT pullback gradients were between 0 and 35 mm Hg (mean, 18.5 mm Hg). Postoperative pulmonary artery saturation (mean, 73%) did not reveal any residual ventricular septal defect. The right ventricular/arterial pressure ratio was always less than 0.6 (mean, 0.4). All children are doing well at follow-up from two to 37 months. Serial echocardiograms reveal no residual ventricular septal defect and only 1 moderate RVOT gradient. Follow-up cardiac catheterization in 15 patients revealed no residual ventricular septal defect and RVOT gradients between 5 and 35 mm Hg. The right ventricular/left ventricular pressure ratio was always less than 0.6 (mean, 0.48). The early and late results justify continued primary repair of tetralogy of Fallot in symptomatic children, regardless of age or weight.(ABSTRACT TRUNCATED AT 250 WORDS)     

Transatrial relief of diffuse subaortic stenosis after ventricular septal defect closure

Transatrial enlargement of the left ventricular outflow tract for serious obstruction was performed in 3 patients with previous ventricular septal defect closure. Two patients had recurrent subaortic stenosis as resection had already been performed at initial operation. In all patients, the obstruction was located below the ventricular septal defect patch. Patch enlargement of the left ventricular outflow tract was carried out by opening the ventricular septal defect patch through the tricuspid valve and extending the incision downward through the area of obstruction and the left ventricular body. All patients had uneventful postoperative course and effective relief of left ventricular outflow tract obstruction. We feel that the approach is simple and effective; it avoids a right ventriculotomy and provides a viable option in certain patients with left ventricular outflow tract obstruction.     

Surgical management of tunnel-like subaortic stenosis via ventricular septal defect in a patient with the interrupted aortic arch

A 24-day-old male with interrupted aortic arch (type B), ventricular septal defect, and tunnel-like subaortic stenosis underwent a one-stage surgical treatment The operative procedure comprised reconstruction of the aortic arch, transatrial excision of the subaortic fibromuscular tissue via the ventricular sepatal defect, and patch closure of the defect. The patient tolerated the procedure well and the postoperative echocardiography demonstrated a residual pressure gradient across the left ventricular outflow tract of 20 mmHg. Our result suggests that the transatrial surgical management of subaortic stenosis via the ventricular sepatal defect produces a safe and promising surgical option.     

Midterm restenosis of freestyle valved conduit in an adult tetralogy of Fallot

We report a case of midterm restenosis of freestyle valved conduit implanted between the right ventricle and the pulmonary artery. A 69-year-old woman visited our hospital with dyspnea and general fatigue and was diagnosed as tetralogy of Fallot. Total corrective surgery including patch closure of ventricular septal defect was performed using the freestyle valve with the Hemashield prothesis to reconstruct the right ventricle to the pulmonary artery. The catheterization revealed almost normal right ventricular pressure on 40th postoperative day, but the opening of the valve was limited. Twenty-two months later she visited our hospital with severe right heart failure. The pressure gradient over the valve was 122 mmHg and the leaflet of the freestyle valve was markedly thickened causing severe stenosis. Use of the freestyle valve for the right ventricular out flow tract reconstruction has to be further studied.     

Ventricular septal defect with tricuspid pouch with and without transposition. Anatomic and surgical considerations.

In a 10-year review, patients operated on for ventricular septal defect and tricuspid valve pouch were divided into two groups, because the effect of the tricuspid valve pouch is influenced by which ventricle has the higher pressure. Group I comprised patients with ventricular septal defect without transposition of the great arteries and group II, ventricular septal defect with transposition. In 72 of 392 group I patients, the septal tricuspid valve leaflet was incised to expose the edges of the hidden ventricular septal defect to accomplish proper anatomic repair. Forty-eight patients had a tricuspid valve pouch, the diagnosis being established by angiography, echocardiography, or at operation. Ages at operation ranged from 5 months to 22 years and the pulmonary-systemic flow ratio ranged from 1 to 3.4, with 16 being less than 1.5. In one patient the pouch produced a 40 mm Hg pressure gradient in the right ventricular outflow tract. At operation, through a transatrial approach, the tricuspid valve pouch was opened radially, the actual ventricular septal defect patched, and the tricuspid valve leaflet repaired. There were no deaths, no significant intraoperative or postoperative morbidity, and no tricuspid valve dysfunction. The average postoperative hospital stay was 4.8 days. In group II, six of 83 patients operated on for transposition with ventricular septal defect had significant left ventricular outflow tract obstruction from the tricuspid valve pouch. Five of six had a Mustard procedure, two requiring a left ventricular-pulmonary artery conduit, and in two of the six the ventricular septal defect was closed through the pulmonary artery. One patient had heart transplantation after a Mustard repair and tricuspid valve replacement. The sixth patient in group II had a successful arterial switch at 9 years of age, after the presence of left ventricular outflow tract obstruction was proved to be due to the pouch. The presence of a tricuspid valve pouch in group I may lead the surgeon to close false small openings produced by the pouch rather than the actual ventricular septal defect. Incising the pouch is safe and essential for proper exposure and secure closure of the true defect. In group II, the systemic right ventricular pressure can push the pouch into the left ventricular outflow tract, causing significant obstruction, and may contribute to tricuspid valve insufficiency after atrial baffle repair. Arterial switch is preferred because it returns the obstructive tricuspid valve pouch and abnormal tricuspid leaflet to the lower pressure pulmonic right ventricle.     

Surgical repair of double-chambered right ventricle with or without ventriculotomy

Double-chambered right ventricle is an uncommon congenital anomaly consisting of obstruction of the trabecular zone of the ventricle by an elevated, hypertrophied moderator band, with or without additional muscle bundles(s). During an 18 month period (August 1984 to February 1986) 12 patients underwent repair of double-chambered right ventricle. Their ages ranged from 13 months to 12 years (mean 4.4 years). Associated lesions were ventricular septal defect in 11 patients (three closed spontaneously), atrial septal defect in two, left superior vena cava in three, aortic valve prolapse in one, and mild subaortic stenosis in two. Right ventricular outflow tract gradients were from 30 to 165 mm Hg (mean 77.2 +/- 37.3 mm Hg). The initial six patients (Group 1) were treated in the "classical" manner with a right ventriculotomy for muscle resection and ventricular septal defect closure (4/6). All patients survived. Five of the six required pharmacologic support for signs of mild to moderate right ventricular failure. The right ventricular outflow tract gradient postoperatively was 0 mm Hg in five patients and 25 mm Hg in one patient. Oxygen saturations were checked in the right atrium and pulmonary artery in all six patients, and one patient had a step-up of more than 10%. In the next six patients (Group 2) a previously unreported right atrial-pulmonary artery approach was used to avoid a ventriculotomy. The right ventricular outflow tract gradient was 0 mm Hg in five patients and 5 mm Hg in the sixth. A ventricular septal defect was closed in four of the six patients. There were no deaths, and no patient required inotropic support. Follow-up has been from 2 to 20 months. There have been no late deaths, and all patients are asymptomatic. One patient in Group 1 still has signs of a residual ventricular septal defect at 1 year. No patient has echocardiographic evidence of right ventricular outflow tract obstruction. All patients have remained in sinus rhythm with right bundle branch block. No patient has been recatheterized. Repair of double-chambered right ventricle is easily performed without a ventriculotomy. The atrial exposure of the ventricular septal defect is excellent. The muscle bundle(s) can be visualized easily through an initial transverse pulmonary arteriotomy with completion of resection through the tricuspid valve.     

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 )     

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.     

大动脉转换术在复杂先天性心脏病治疗中的应用

目的 回顾总结我院近3年采用大动脉转换术治疗小儿复杂先天性心脏病(先心病)的临床经验。方法2000年1月至2003年5月,采用大动脉转换术(Switch手术)治疗小儿复杂先心病61例。其中完全性大血管错位(TGA)45例,包括室间隔完整型(TFGA-IVS)26例,伴室间隔缺损(TGA,VSD)19例;右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)16例。结果TGA,IVS死亡2例;TGA-VSD死亡4例;Taussig-Bing死亡4例;总手术病死率16％。术后随访3个月-3年,所有病例青紫症状消失,活动能力明显增强。1例Taussig-Bing术前二尖瓣轻～中度返流,术后仍为中度返流。2例TGA术后出现主动脉和肺动脉瓣上狭窄,压差40mmHg,1例肺动脉瓣下狭窄和残余室间隔缺损,3个月后再次手术治愈。结论大动脉转换术应用于完全性大血管错位的纠治,手术效果满意;应用于右室双出口肺动脉瓣下室间隔缺损的纠治,不但可早期纠治,防止其肺血管阻塞性病变发生,而且避免了心内修补左室流出道梗阻的远期并发症。     

A report of two cases of absent pulmonary valve syndrome

Two infants, aged 8 and 18 months, with absent pulmonary valve syndrome unaccompanied by severe pulmonary symptoms or roentgenographic evidence of emphysema underwent a corrective operation consisting of infundibular muscle resection, patch closure for ventricular septal defect (perimembranous outlet type), and right ventricular outflow tract reconstruction with a monocusp patch. The patients tolerated pulmonary regurgitation relatively well, although aneurysmal dilatation of the pulmonary arteries was present preoperatively. Since the postoperative course was uneventful, we think that, in milder cases of this syndrome, a pulmonary valve prosthesis, the insertion of which is advocated by some authors, is not essential, and that right ventricular outflow tract reconstruction with a monocusp patch may suffice in selected cases.     

Postoperative functional assessment of a modified surgical approach to repair of tetralogy of Fallot

Since February 1982, we have used a modified technique to repair tetralogy of Fallot with transatrial closure of the ventricular septal defect, a short infundibular incision with avoidance of muscle resection, and anterior expansion of the right ventricular outflow tract using a patch in every case. The pulmonary anulus is approached in the usual manner. Twenty-six patients have had repair with this technique without mortality, and 20 patients (Group 1) have been evaluated 1 to 3 years postoperatively. Their results are compared with those of all patients followed up in our division who had had repair by the traditional technique in the 5 years before February 1982, with the same evaluation within 4 years of operation (Group 2, n = 22). Ten of 20 patients in Group 1 and 14 of 22 in Group 2 required a transannular patch. At cardiac catheterization, right ventricular outflow tract obstruction was effectively relieved in both groups (right ventricular systolic pressure [mean +/- standard error], Group 1 versus Group 2, 45 +/- 5 versus 49 +/- 4 mm Hg). No patient with the modified technique had a residual ventricular septal defect. By M-mode echocardiography, right ventricular-left ventricular end-diastolic dimension ratio was significantly lower for Group 1 patients (0.58 +/- 0.03 versus 0.78 +/- 0.04; normal less than 0.45; p less than 0.001). With radionuclide ventriculography, right ventricular ejection fractions were significantly higher for Group 1 patients with the modified repair (38% +/- 2.2% versus 33% +/- 1.8%; normal greater than or equal to 45%; p less than 0.05). Because the incidence of obligatory pulmonary insufficiency caused by transannular patch is similar in both groups, these results cannot be directly attributed to the presence of pulmonary insufficiency alone. With ambulatory electrocardiographic monitoring, no patient in Group 1 had any ventricular premature contractions whatsoever, whereas six of 22 patients in Group 2 had demonstrable ventricular premature contractions. These results suggest that in children with tetralogy of Fallot, right ventricular outflow tract obstruction can be effectively relieved with this modified approach with improved early preservation of right ventricular function.