Changes in intracardiac hemodynamics after percutaneous catheter balloon valvuloplasty in different types of valvular stenosis of the pulmonary artery]

Percutaneous catheter balloon valvuloplasty was carried out in 75 patients with valvular pulmonary stenosis (VPS), whose ages ranged from 18 months to 38 years. In 40 of them (53.3%) VPS was complicated by infundibular stenosis of the right ventricle. After percutaneous catheter balloon valvuloplasty 13 patients received out-patient treatment with beta-adrenergic blocking agents in doses of 20 to 120 mg/24 hours. Control examination in periods of 6 months to 2 years after the operation was conducted in 35 patients among whom 10 patients had been given beta-adrenergic blocking agents in the postoperative period. A stable reduction of the right ventricle-pulmonary artery (RV-PA) gradient and positive dynamics of the cardiac volume indices were recorded in 25 patients after correction of isolated VPS and in 6 patients after percutaneous catheter balloon valvuloplasty and treatment with beta-adrenergic blocking agents. The residual RV-PA gradient in 4 patients after treatment with beta-adrenergic blocking agents remained within a range of 30 mm Hg. Percutaneous catheter balloon valvuloplasty is an effective method for the correlation of isolated VPS. Coexistence of VPS with infundibular right-ventricular stenosis is not a contraindication for the use of this method for correcting the anomaly. In such a case the roentgenosurgical intervention should be supplemented by treatment with beta-adrenergic blocking agents in individual doses.     

Complete atrioventricular canal and tetralogy of Fallot: surgical considerations

A review of 13 autopsy specimens and of 13 patients who were operated on for complete atrioventricular (AV) canal and tetralogy of Fallot between 1975 and 1985 revealed a number of anatomical details that are important in the successful repair of this combined lesion. A bridging anterior leaflet was present in 25 hearts. A septum primum was present and attached to leaflet tissue in 13. In all 26, a ventricular septal communication was present beneath the bridging anterior leaflet and extended anteriorly, but in 14 there was no ventricular septal defect underneath the posterior leaflet. Additional pathological features included the following: leaflet tissue deficiency (4 hearts), single left papillary muscle (3), accessory valve orifice (4), and left ventricular (4) or right ventricular (RV) (1) dominance. All 26 had infundibular stenosis, and 10 had hypoplastic pulmonary annuli. One had pulmonary atresia, and 6 had branch pulmonary artery stenosis. Surgical technique was modified to include incision of the septum primum in 7. Because of rightward displacement of the anterior ventricular septum and also to minimize the risk of causing subaortic stenosis, the bridging anterior leaflet was divided more toward the tricuspid orifice so as to parallel the crest of the ventricular septum. Transannular RV outflow patches were used in 10 patients, and a right ventricle-pulmonary artery conduit was placed in 1 patient. Three required repair of branch pulmonary artery stenosis. There were no hospital deaths. Three patients died late of residual AV valve regurgitation and branch pulmonary artery stenosis (2) and sepsis (1).(ABSTRACT TRUNCATED AT 250 WORDS)     

保留肺动脉瓣的改良REV手术治疗复杂型大动脉转位——附3例报道及文献综述

目的 介绍一种改良REV手术治疗合并室间隔缺损（VSD）、肺动脉瓣狭窄（PS）的复杂型大动脉转位或右心室双出口（DORV）。方法 2005年9月～2006年2月我院收治3例复杂型先天性心脏病患者，在经典REV手术的基础上，我们改良了REV手术，保留了肺动脉瓣和瓣环进行手术治疗。结果 2例痊愈，1例同时用体外膜式氧合器（ECMO）进行抢救后死亡。痊愈患者分别随访4个月和1个月，超声心动图检查提示肺动脉瓣跨瓣压差分别为15mmHg和5mmHg，结果满意。结论 保留肺动脉瓣的改良REV手术对治疗合并VSD、PS的复杂大动脉转位是一种较为理想的手术方法。     

Fontan type operation for complex lesions. Surgical considerations to improve survival

Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used. Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previous pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 +/- 6.5 torr), and hospital stay longer (31 days). In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 +/- 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlusion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13%). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing well. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valve closure, avoidance of valved conduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.     

The midterm and long-term results of the Mustard operation in patients with transposition of the great vessels and dynamic left ventricular outflow tract obstruction

Eight of 43 patients with transposition of the great vessels (TGV) and either an intact ventricular septum or very small ventricular septal defect were found to have dynamic left ventricular outflow tract obstruction (LVOTO). The preoperative left ventricle-pulmonary artery (LV-PA) pressure gradient ranged between 14 and 60 mm Hg. In 3 patients the pulmonary artery could not be catheterized. They had a left ventricular pressure of 43, 45, and 62 mm Hg, respectively, no evidence of pulmonary vascular disease, and either echocardiographic or angiocardiographic demonstration of LVOTO. The outflow tract was explored in 3 patients at the time of the Mustard operation. Exposure of the LVOT was difficult, particularly in patients less than 6 months of age. A shallow septal myectomy was performed in 1 patient; a thin fibrous endocardial scar was resected in 2; and no procedure was performed in the third. All 8 patients survived and remain asymptomatic 1 to 11 years (mean, 5 years) after operation. The LV-PA pressure gradient has either remained the same or has increased in every patient except 1 in whom there was a slight reduction in the gradient. We conclude that dynamic LVOTO persists after the Mustard operation and is not amenable to direct surgical relief. However, an excellent clinical result can be obtained for up to 11 years after operation.     

A balloon-dilatable pulmonary artery band in the dog. Results at one year

Increased pulmonary blood flow and pulmonary hypertension are frequent problems in infants with congenital heart disease. Although the use of pulmonary artery banding to limit pulmonary blood flow has decreased, the procedure may still be beneficial in certain forms of complex heart disease. The ability to noninvasively relieve the obstruction caused by the band may significantly reduce later operative complexity or even avoid reoperation entirely. The present study evaluated the effectiveness of a balloon-dilatable pulmonary artery band. Twenty 1-week-old dogs had a band of an absorbable suture material (Vicryl; Ethicon, Inc., Somerville, N.J.) placed around the main pulmonary artery. Eight dogs underwent angioplasty 6 months after band placement and also underwent follow-up catheterizations 3 and 6 months after angioplasty. Balloon angioplasty acutely reduced both the right ventricle-pulmonary artery pressure gradient (from 37 +/- 7 mm Hg to 3 +/- 1 mm Hg, p less than 0.001) and the right ventricular systolic pressure (from 62 +/- 8 mm Hg to 32 +/- 2 mm Hg, p less than 0.01). At follow-up the gradient remained low, measuring 4 +/- 1 mm Hg at 3 months and 3 +/- 1 mm Hg at 6 months. Twelve dogs did not undergo balloon dilatation until 12 months after band placement to determine whether any obstruction persisted and whether the band could be relieved after long-term placement. These 12 dogs had progressive increases in right ventricle-pulmonary artery gradient, from 27 +/- 3 mm Hg at 6 months to 43 +/- 4 mm Hg at 12 months. Ten of these dogs underwent dilation 1 year after pulmonary artery band placement. This dilation significantly reduced the right ventricular outflow tract gradient (from 43 +/- 4 mm Hg to 1 +/- 1 mm Hg, p less than 0.001). The remaining two dogs underwent successful partial dilation of the band 12 months after placement. This study demonstrated that a pulmonary artery band of absorbable suture material maintains effective right ventricular outflow tract obstruction for at least 1 year. Additionally, the effect of the pulmonary band can be successfully and persistently relieved.     

Gore-Tex片作单瓣重建右心室流出道的早、中期疗效

目的评价0.1mm厚的Gore-Tex片作肺动脉单瓣重建右心室流出道(RVOT)的早中期临床效果。方法2002年6月至2006年7月,对48例合并肺动脉狭窄或闭锁的先天性心脏病患者施行矫治手术,术中采用0.1mm厚的Gore-Tex片作肺动脉单瓣的Dacron血管片重建RVOT,其中包括法洛四联症合并肺动脉狭窄33例、型肺动脉闭锁8例、肺动脉瓣缺如3例,右心室双出口合并肺动脉狭窄2例,永存动脉干1例,完全型大动脉错位、室间隔缺损、肺动脉狭窄1例。结果本组无手术死亡。术后血氧饱和度达1.00,右心室/左心室收缩压比值0.22~0.65,右心室与左、右肺动脉压差<10mm Hg。所有患者(100%)均随访3~48个月,无死亡和并发症。彩色多普勒超声心动图检查提示:无肺动脉狭窄,肺动脉瓣轻度反流13例,中度反流5例,Gore-Tex膜具有活动功能40例。结论采用0.1mm厚的Gore-Tex片作单瓣重建RVOT,其早中期临床结果显示有较满意的血流动力学效果。     

Improved right ventricular function following late pulmonary valve replacement for residual pulmonary insufficiency or stenosis

Residual severe pulmonary insufficiency or stenosis may result in significant myocardial dysfunction late after repair of tetralogy of Fallot. Although pulmonary valve replacement has been advocated for selected patients, objective improvement in right ventricular function has been difficult to demonstrate. We undertook pulmonary valve replacement in 11 patients to treat residual insufficiency (n = 8) or stenosis (n = 3) and evaluated them before and after operation by radionuclide ventriculography and M-mode echocardiography. Patients' age at the original repair was 6.6 +/- 0.6 years (range 2 to 8 years) and at subsequent valve replacement was 14.6 +/- 1.5 years (range 5 to 20 years). Indications for pulmonary valve replacement were conduit stenosis indicated by a gradient greater than or equal to 75 mm Hg (n = 3), symptoms (n = 2), progressive cardiomegaly (n = 3), and new onset of tricuspid insufficiency (n = 3). Prior to pulmonary valve replacement, right ventricular ejection fraction was 0.29 +/- 0.12 (range 0.12 to 0.48) and rose to 0.35 +/- 0.10 (range 0.19 to 0.48) at a mean of 10.5 +/- 2.3 months after operation (p less than 0.05). Improvement (defined as an increase in ejection fraction greater than 0.05) was noted in seven patients whereas four demonstrated no change. Left ventricular ejection fraction before operation (0.55 +/- 0.12) was unchanged after pulmonary valve replacement (0.54 +/- 0.06). M-mode echocardiography demonstrated significant reduction in right ventricular dilatation. Right ventricular/left ventricular end-diastolic dimension fell from 1.03 +/- 0.30 to 0.73 +/- 0.13 after operation (p less than 0.01). Cardiothoracic ratio fell from 0.59 +/- 0.02 to 0.55 +/- 0.02 at a mean of 12 months after pulmonary valve replacement (p less than 0.01). Subjective improvement in exercise tolerance was noted in all seven patients who showed an increase in right ventricular ejection fraction. Of the remaining four patients, two had no improvement, one felt symptomatically improved, and one was too young for evaluation. These data demonstrate objective improvement in right ventricular function following pulmonary valve replacement and confirm the usefulness of this procedure in patients with significant right ventricular dysfunction secondary to residual pulmonary insufficiency and stenosis.     

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.     

Long-Term Sequential Hemodynamic Evaluation of Right Ventricular Outflow Tract Reconstruction Using a Valve Mechanism

From May, 1972, to May, 1978, right ventricular outflow tract reconstruction was performed in 20 patients with congenital heart disease. A monocusp patch constructed entirely of glutaraldehyde-stabilized calf pericardium, was employed in 19 patients, and a composite conduit consisting of a three-cusp pericardial xenograft valve in a Dacron tube was used in 1 patient.There were 2 hospital deaths (10%) and no late deaths. The follow-up ranged from 5 to 75 months (mean, 55.7 ± 4.6 months; total, 1,002 patient months). Eleven patients had completed 5 years of follow-up at the time of writing.Hemodynamic studies were performed in 10 patients at an average of 40 months after operation and the right ventricle-pulmonary artery systolic gradient was 9.3 ± 1.7 mm Hg. This was not significantly different in 6 patients who had a second postoperative catheterization at 51.8 ± 2.0 months following operation. Angiography showed fully mobile, thin valve cusps.These results compare well with those reported with other types of conduits, particularly with reference to relief of obstruction.     

Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. Surgical implications

Postoperative cardiac catheterization data of 74 patients with pulmonary insufficiency after tetralogy repair were analyzed. Two groups were identified: Group A, 26 patients with normal right ventricular function (ejection fraction 95% +/- 5.5%, end-systolic volume 110% +/- 17% of predicted normal) and Group B, 48 patients with right ventricular dysfunction (ejection fraction 80% +/- 18% [p less than 0.001], and end-systolic volume 218% +/- 75% of predicted normal [p less than 0.001]). There was no significant difference between the two groups with respect to frequency of previous palliative procedures, age at operative repair, operative techniques, methods of myocardial protection, and follow-up period. Right ventricular dysfunction in Group B was associated with significant distal pulmonary stenosis (right ventricle-pulmonary artery pressure gradient 28 +/- 13 torr in Group A versus 55 +/- 20 torr in Group B, p less than 0.001), moderate pulmonary regurgitation (regurgitant fraction 18% +/- 11% in Group A versus 32% +/- 10% in Group B, p less than 0.001), and large transannular outflow patch (ratio of patch diameter to descending aorta diameter 1.31 +/- 0.16 in Group A versus 2.50 +/- 0.28 in Group B, p less than 0.001). Pulmonary valve insertion was performed in 42 patients in Group B. Eighteen had subsequent cardiac catheterization. Right ventricular function recovered completely (end-systolic volume 122% +/- 24%, and ejection fraction 92% +/- 7% of predicted) in five of six patients (83%) who had valve insertion within the first 2 years after tetralogy repair. In contrast, right ventricular function remained abnormal in all 12 patients who had valve insertion later than 2 years after tetralogy repair (p less than 0.05). Patients with residual pulmonary stenosis and/or a large transannular outflow patch are at risk for the development of right ventricular dysfunction from pulmonary insufficiency after tetralogy repair. Early correction of these residual lesions and control of pulmonary insufficiency may prevent long-term deterioration in right ventricular function.     

The Use of the Right Atrial Flap in Total Cavopulmonary Connection

A technical modification of total cavopulmonary anastomosis (TCPC) is described. Inferior vena cava (IVC) channel is constructed from the right atrial wall in a fashion similar to Senning's operation. The use of Gore-TexR (W.L. Gore & Associates, Inc.) patch or tube is avoided. We have used the technique in 11 patients between 1988 and 1991 (six complex transpositions or double outlets with one hypoplastic ventricular chamber and/or straddling of the atrioventricular (AV) valve, four double inlet ventricles with pulmonary stenosis, and one tricuspid atresia). Superior vena cava (SVC)/IVC to pulmonary artery gradient was less than 2 mmHg in all patients. SVC/IVC pressure was 10-15 mmHg (mean 12.3 mmHg), transpulmonary gradient 5-8 mmHg (mean 6.4 mmHg). We have not observed any adverse effects such as arrhythmias or increased pleural drainage when we compared those patients with 85 children in whom the TCPC was performed with Gore-TexR patch/tube. The presented technique is simple, avoids the use of anticoagulants, and may have a possible long-term advantage in allowing growth of the IVC channel.     

Modified fontan operation

From November 1988 to October 1991 30 patients underwent a total extracardiac right heart bypass for complex cardiac anomalies by means of bidirectional cavopulmonary anastomosis and interposition of a conduit from the inferior vena cava to the pulmonary artery. Mean age at surgery was 6.4 years and mean weight 19.2 kg. There was 1 hospital death (3%) due to a borderline indication for a Fontan operation. 2 patients had further surgery: In 1 the repair was taken down due to the stenosis of the left pulmonary artery and the patient was left with a bidirectional cavopulmonary anastomosis only, the second patient required a revision of the cavopulmonary anastomosis due to a stenosis of the superior vena cavaright pulmonary artery junction. There were no late deaths and the survivors are in good clinical condition a mean of 15.1 months after the operation. We propose this technique as an alternative surgical option in candidates for a Fontan operation in whom atrial septation is hazardous including those with 1) hypoplasia or atresia of the left atrio-ventricular valve, 2) common atrioventricular valve, 3) anomalies of systemic and/or pulmonary venous return, or 4) auricular juxtaposition.     

Use of polytetrafluoroethylene (Gore-Tex) grafts for the construction of right ventricle-pulmonary artery conduits

Placement of a right ventricle-pulmonary artery conduit (18- or 20-mm grafts) made of Gore-Tex without a prosthetic valve was undertaken in 6 patients ranging in age from 8 1/2 to 32 years. Three patients had tetralogy of Fallot with pulmonary atresia, and the other 3 had transposition of the great vessels, ventricular septal defect, and pulmonary obstruction. Pulmonary pressure was low in all patients. The technique for implantation of this valveless, noncrimped type of prosthesis is described.     

Pulmonary homograft implantation for ventricular outflow tract reconstruction: early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryopreserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21-0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2-27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical correction of types II and III truncus arteriosus.

In truncus arteriosus types II and III, one or both pulmonary arteries arise independently from either side of the truncus. In the surgical correction of this anomaly, we have utilized on operative technique in which the essential features are as follows: ventricular septal defect (VSD) closure, which directs left ventricular outflow into the truncus: (2) anastomosis of a Dacron tube containing a glutaraldehyde-preserved procine aortic heterograft to the right ventriculotomy: (3) removal of a circumferential band of the truncus containing both pulmonary artery orifices; (4) tailoring of the band of truncus tissue into a generous cuff which is anastomosed to the distal end of the valved Dacron conduit; and (5) restoration of aortic continuity with a tubular Dacron graft. Since 1971, 4 children ages 2 to 9 years have undergone successful correction of truncus arteriosus types II or III by this technique. In one patient with marked pulmonary hypertension and congestive heart failure preoperatively, the pulmonary vascular resistance had reverted to normal by 3 years after the operation. In one patient in whom bronchial collaterals to the right pulmonary artery were present, postoperative left ventricular failure required reoperation for ligation of the collaterals. All 4 patients are asymptomatic and fully active 5 to 60 months postoperatively. None has evidence of stenosis or insufficiency of the heterograft valve.     

膨体聚四氟乙烯片作单瓣重建右室流出道术后早期并发症及处理

目的：评价膨体聚四氟乙烯片（Gore-Tex片）作肺动脉单瓣重建右室流出道（RVOT）的近期疗效。方法：2002年6月-2011年11月对75例先天性心脏病合并肺动脉狭窄或闭锁的患者旋行矫治手术,术中采用厚度0．1mm的Gore—Tex片作肺动脉单瓣重建RVOT,其中男42倒,女33例。法洛四联症合并肺动脉狭窄57例,l型肺动脉闭锁10例．肺动脉瓣缺如4例,右心室双出口台并肺动脉狭窄2例．完全型大动脉转位1例,室间隔缺损1例,肺动脉狭窄1例。结果：术后血氧饱和度为96％～100％。动脉血氧分压82～207mmHg,右室／左室收缩压比值0．22～0．70,右室与左、右肺动脉间的压力阶差小于10mmHg。左室射血分数（LVEF）0．53～O．80,右室射血分数（RVEF）0．52～071,左室舒张末期容积指数（LVEDI）0,28～0．62ml／m2。术后早期并发症：心包积液7例,低氧血症（氧合指数〈150）6例。心律失常5例,低心排血量综合征4例．残余室间隔缺损4例。术后随访3～48个月,无死亡和并发症的发生。其中62~Gore-Tex片作肺动脉单瓣重建右心室流出道（RVOT）早期瓣膜活动功能正常,13例单瓣固定在开放状态：72倒均无肺动脉单瓣狭窄．3例单瓣轻度狭窄;21倒肺动脉瓣轻度返流．5例中度返流。结论：采用厚度0．Imm的Gote-Tex片作单瓣重建RVOT,可获得满意的lI缶床和血流动力学效果．近期效果良好。膨体聚四氟乙烯片作单瓣重建右心室流出道术后早期并发症应引起高度熏视,加强术后监护及综合治疗措施,及时纠正术后低心输出量综合征．积极防治术后心律失常等均为提高手术成功率的重要因素。     

Surgical management of double-outlet right ventricle

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.     

Medtronic freestyle valves in right ventricular outflow tract reconstruction

BACKGROUND: Various pulmonary valve substitutes, with their inherent limitations, have been used in children and young adults. We chose the Medtronic Freestyle (Medtronics, Minneapolis, MN) valve because of its excellent hemodynamics, known durability in adults, and design features that allow modifications during implantation. METHODS: Over a 3 1/2 year period the Freestyle valve was implanted in 47 patients age 2 to 58 years (mean 14.2, median 12.0) in the pulmonary position. All patients had pulmonic stenosis and(or) insufficiency from previous operations for tetralogy (27), pulmonary atresia (6), truncus (4), or other diagnosis (10). The indication for surgery was pure pulmonary insufficiency in 11 patients, pulmonic stenosis in 3, and mixed stenosis and insufficiency in 33. Root replacement technique was used with additional enlargement of the pulmonary artery branches in 10 patients. RESULTS: Intraoperatively, one patient sustained a right ventricle tear and one a circumflex coronary artery injury during the dissection. There was one postoperative death. Two patients developed late subvalvular pannus formation, one of whom required reoperation. One patient was found to have an echo gradient of 95 mm Hg due to decreased leaflet motion and underwent cardiac catheterization at which the peak systolic gradient was determined to be 50 mm Hg. He has not required reintervention during his 3 1/2 years of follow-up. The remaining 43 patients have minimal gradients or insufficiency. All surviving patients are in New York Heart Association (NYHA) Class I. CONCLUSIONS: The Medtronic Freestyle valve is an attractive alternative for RVOT (right ventricular outflow tract) reconstruction in children. It is readily available, versatile, and has excellent hemodynamic characteristics. Although long term follow-up is not yet available, longevity of this prosthesis, and freedom from complications, will hopefully be superior to valves with stents.     

De Vega tricuspid annuloplasty for tricuspid regurgitation in children

BACKGROUND: Significant tricuspid valve regurgitation (TR) occurs with other congenital heart defects, typically after repair of right-sided obstructive lesions. Since 1991, we applied the De Vega tricuspid annuloplasty technique for TR in children. METHODS: Forty-one children, aged 5 months to 22.7 years (mean, 9.9 years) underwent 42 De Vega tricuspid annuloplasties for moderate or severe TR during correction of other heart defects. One child had a De Vega during primary ventricular septal defect repair. The remaining patients had prior repair of tetralogy of Fallot or pulmonary atresia, or both (19 patients), double-outlet right ventricle (6 patients), pulmonary stenosis (4 patients), pulmonary atresia and intact ventricular septum (3 patients), complete atrioventricular septal defect (3 patients), and other diagnoses (6 patients). At the time of the De Vega, 37 patients (88%) had pulmonary valve replacement or right ventricular to pulmonary artery conduit replacement. Other procedures included aortic or mitral repair or replacement (6 patients), atrial septal defect and ventricular septal defect closure (5 patients), pulmonary arterioplasty (6 patients), and tracheoplasty (1 patient). RESULTS: There were no deaths at follow-up of 3.4 +/- 2.1 years; 1 child required cardiac transplantation 17 months postoperatively. Early postrepair echocardiography quantified TR as absent or mild (34 patients; 81%), mild-to-moderate (4 patients), moderate (3 patients), and severe (1 patient). The most recent echocardiogram showed moderate TR in 11 patients and severe TR in 2 patients (both with recurrent right ventricular hypertension). One child required tricuspid valve replacement 3 years later and 1 child had redo De Vega at the time of conduit re-replacement. No other child has symptomatic TR, significant tricuspid stenosis, or De Vega-related pacemaker implantation. CONCLUSIONS: The De Vega tricuspid annuloplasty safely provides excellent relief of TR, usually in children undergoing pulmonary valve replacement or conduit replacement. Although echocardiographic TR tends to increase with time (especially with right ventricular hypertension), it rarely requires reintervention or causes symptoms.