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)     

Late reoperations after repair of tetralogy of Fallot.

Twenty-two patients underwent 23 late reoperations after total correction of tetralogy of Fallot from 1965 to 1990. Indications for reoperation included: isolated ventricular septal defect (VSD) in 9 patients (41%), isolated right ventricular outflow tract (RVOT) obstruction in 3 patients (13.7%), VSD associated with a RVOT obstruction in 7 patients (31.8%), aneurysm of the pericardial RVOT patch in 1 patient (4.5%), aortic insufficiency with a residual VSD in 1 patient (4.5%), and tricuspid regurgitation in 1 patient (4.5%). The reoperation consisted of closure of a residual VSD in 17 patients, relief of a RVOT gradient in 11, insertion of a RVOT valve in 4, tricuspid valve replacement in 1 (reoperated twice), aortic valve replacement in 1, and excision of a RVOT aneurysm in 1. Two patients died in hospital (9%) but there were no early deaths in the 11 patients reoperated upon after 1978. Mean follow-up period was 135 months. There were 2 late deaths. The actuarial 20-year survival was 87%. Of the surviving patients, 16 (89%) were in New York Heart Association class I, 1 (5.5%) was in class II, and one (5.5%) was in class III. One patient required a second reoperation for tricuspid bioprosthesis degeneration and 1 patient had moderate recurrent RVOT gradient due to calcified pulmonary bioprosthesis. This study tends to support the policy of recommending reoperation in the presence of surgically significant residual defects. Reoperation is associated with a low early mortality and good long-term results.     

Morphosurgical correlation of outcomes in complete double outlet right ventricle

Even though surgical experience with wide spectrum of double outlet right ventricle (DORV) is available, the experience with extreme form of complete DORV where both great arteries completely arise from the right ventricle is limited. We present our experience with this unique subset where the systemic outflow is far removed from the interventricular foramen and hence, the systemic ventricle. In this situation, biventricular repair can be technically demanding and challenging. Between June 2002 and February 2006, 12 patients underwent biventricular repair of this subset. The VSD was subaortic in all; eight patients had infundibular and valvar obstruction. Aorta was anterior and to the right, with the pulmonary artery far posterior and to the left. The interventricular foramen was patched with a long Gore-Tex patch to route the LV flow to the aorta. Eight patients had infundibular resection and right ventricular outflow tract (RVOT) enlargement with an autologous monocusp pericardial patch. No patient required a valved conduit. There was no operative mortality. In one patient, there was a small residual VSD that was not of haemodynamic significance. There was no RVOT and left ventricular outflow tract obstruction and no RV inflow obstruction. The early and mid-term results are good.     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.     

Repair of ventricular septal defect after pulmonary artery banding.

Since 1964, 90 patients have undergone two-stage surgical repair of ventricular septal defect (VSD) with pulmonary artery banding (PAB) in early infancy and total repair at an average age of 4 years. Reconstruction of the pulmonary artery was accomplished with a pericardial patch, woven Dacron patch, or transverse angioplasty. The VSD was closed with a knitted Dacron patch in 75 patients and by primary suture technique in 13 patients. The VSD closed spontaneously in 2 patients. The mortality rate for patients who had repair and debanding was 9 per cent (8 patients), including 4 deaths due to severe pulmonary hypertensive disease, 3 from congestive heart failure, and one from atrioventricular block. Twenty patients underwent repeat cardiac catheterization several months to 7 years after total repair. This study revealed no shunt in 16 patients and a minimal shunt not requiring operation in the other 4 children. Slight residual stenosis of the pulmonary artery was found in 2 patients and a residual infundibular stenosis in another 2 patients. We believe two-stage surgical treatment of VSD in severely ill infants under one year of age is safe and reliable.     

787例小于6月龄先天性心脏病患者外科治疗的临床分析

目的回顾性总结婴幼儿先天性心脏病的手术时机和手术治疗方法,以进一步提高手术成功率和远期疗效。方法1988年1月～2003年6月,手术纠治年龄小于6个月的先天性心脏病患者787例。主要病种包括完全性大动脉错位109例,完全性肺静脉异位引流51例,肺动脉闭锁16例,主动脉缩窄33例,室间隔缺损伴肺动脉高压299例,法洛四联症44例,右心室双出口23例,室间隔完整型肺动脉闭锁9例等;对787例患者根据不同病种采取相应的手术方法纠治。结果手术死亡77例,手术死亡率9.78%(77/787)。随着手术方法的不断改进,手术总死亡率从1988～1995年的25%降至2003年的4.11%。随访完全性大动脉错位患者中发生VSD残余漏1例,术后3个月再次手术治愈;随访中发生肺动脉和主动脉瓣上狭窄2例。完全性肺静脉异位引流心内型患者中2例分别在术后4d和2个月出现肺静脉回流梗阻,1例死亡,1例再次手术解除梗阻。室间隔缺损患者中发生残余漏5例,分流量小,不需再次手术。其余病例随访资料不完整。结论对婴幼儿先天性心脏病施行手术治疗的时间非常重要,危重复杂型先天性心脏病患者如不早期手术,将失去手术机会,增加术后危险性和死亡率。     

Long-term outcome after total correction of tetralogy of Fallot in adolescent and adult age

Although most patients with tetralogy of Fallot (TOF) undergo radical repair during infancy and childhood, patients remaining undiagnosed and untreated until adulthood can still be treated. These patients have either a previous palliative or natural collateral circulation to the lung or a mild form of right ventricular outflow tract (RVOT) obstruction. The aim of this study is to analyze the perioperative and long-term results of radical corrective procedures in patients who reached adult ages. Two hundred and seven patients with TOF underwent complete correction between 1985-and 2002, 64 (30.9%) of whom were aged 14 years or more. The mean age at corrective repair for this group was 20.6 +/- 7.5 years (range 14 to 49 years). Only two patients had previous modified Blalock-Taussig shunts. In 44 patients (68.7%) besides infundibular resection, a transannular gluteraldehyde-treated pericardial patch was used to reconstruct right ventricular outflow tract (RVOT). Only infundibular patching was used in 15 patients (23.4%) and infundibular muscular resection with primary closure of right ventricle was performed in five patients (7.8%). Hospital mortality was 3.1% with two patients. Four patients (6.2%) underwent reoperation because of recurrent ventricular septal defect (VSD) with/without residual obstruction or pulmonary regurgitation. All survivors were in NYHA class I (42) or II (17). Late mortality was recorded in two patients and 16-year actuarial survival was 89.2%+/- 4.9%. The significant negative predictors of late survival determined by univariate analysis were reoperation <0.018) and associated cardiac anomalies <0.011). Multivariate analysis showed that there was no negative predictor of late-term mortality. Corrective procedures in adult patients with TOF can be performed successfully compared to patients who underwent operation during infancy and childhood.     

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.     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

Repair of aortic coarctation in the first year of life

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.     

婴儿早期法洛四联症的外科治疗

目的总结婴儿早期法洛四联症（TOF）外科治疗经验,探讨TOF早期根治术的相关问题。方法回顾性分析上海市儿童医院2008年6月至2010年8月收治21例婴儿早期（〈6个月）TOF患者的临床资料,其中男14例,女7例;年龄4.86±1.15个月;体重6.84±1.33 kg。均经心脏彩色超声心动图确诊,有4例行CT或磁共振成像（MRI）或右心导管造影术。McGoon比值1.86±0.41,肺动脉指数（PAI）142.54±59.46 mm2/m2。经右心房（19例）或右心室（2例）自体心包补片连续缝合修补室间隔缺损;对肺动脉瓣环Z值〈-1者采用心包补片跨瓣扩大成形（18例）;对接近或基本达到正常Z值者保留瓣环,用心包补片分别扩大右心室流出道（RVOT）和肺总动脉（3例）。结果术后第15 d因心力衰竭死亡1例;1例术后第2 d拔除气管内插管后出现喉头水肿,再插管辅助通气,3 d后顺利撤机;其余患者术后均顺利恢复。18例获得随访,随访9.89±6.47个月,心功能改善（Ross分级Ⅰ~Ⅱ级）;随访心脏超声心动图提示：RVOT压差为21.20±12.27 mm Hg（8.10~45.14 mm Hg）;肺动脉瓣反流（PI）轻度10例,中度5例,无重度患者;2例早期残余室间隔缺损已闭合。与术后早期相比,RVOT压差和PI程度差异均无统计学意义（P〉0.05）,右心功能良好。结论婴儿早期行TOF根治术可取得良好的手术效果;经右心房矫正心内畸形,并保留肺动脉瓣环有利于术后心功能保护。     

Outcome after one-stage repair of tetralogy of Fallot

AIM: The purpose of this study was to evaluate the outcome after one-stage repair of tetralogy of Fallot (TOF). METHODS: Between May 1997 and December 2002, 240 patients with a median age of 9 months (1 month-48 years) underwent one-stage repair of TOF. Closure of ventricular septal defect (VSD) was accomplished through the right atrium in 171 (71.3%) patients and through the right ventricle in 69 (28.7%) patients. For the reconstruction of the right ventricular outflow tract (RVOT), transannular repair was performed in 151 (62.9%) patients, and non-transannular repair was performed in 89 (37.1%) patients. Follow-up was complete, averaging 40+/-17.6 months (3 months-5.8 years). RESULTS: There were 2 (0.8%) operative deaths. Between early repair group (age under 6 months) and late repair group (age above 6 months), there were no differences in the method of RVOT reconstruction (transannular vs non-transannular) and the need for branch pulmonary artery angioplasty. Early repair group had more transventricular VSD closure than late repair group (46% vs 22%, P < 0.05). Duration of inotropic support and intensive care unit (ICU) stay were longer in the early repair group (P < 0.05). Five patients required reoperations due to RVOT obstruction (n = 3), and residual VSD (n = 2). Kaplan-Meier freedom from reoperation at 5 years was 98.3+/-1%. Nine patients underwent catheter intervention for branch pulmonary artery stenosis. Freedom from reintervention at 5 years was 95.4+/-1.5%. All survivors are currently asymptomatic. CONCLUSIONS: One-stage repair of TOF could be performed with low mortality and morbidity. Especially, early one-stage repair in symptomatic infant could be performed with low risk, eliminating the need for palliative procedures.     

冠状动脉畸形对复杂先心病右室流出道重建的影响

目的探讨冠状动脉畸形对复杂先心病右室流出道（RVOT）重建的影响。方法自1989年4月至2004年5月治疗18例合并冠状动脉畸形的法洛四联症（14例）和右室双出口（4例）患者。右冠状动脉开口缺如7例,左冠状动脉开口缺如4例,左前降支起自右冠状动脉6例,左右冠状动脉交通支形成蔓状血管丛1例。均在全身麻醉低温体外循环下手术,1例行姑息手术,17例行根治术,RVOT重建的方法：游离畸形冠状动脉从其下方做切口,RVOT心包补片扩大术4例;3例在异常交通支下方平行切口修补室间隔缺损（VSD）,交通支上方纵切口加宽RVOT和肺动脉瓣环;4例行右心房切口修补VSD,RVOT小切口和肺动脉切口心包补片扩大术;3例肺动脉主干与RVOT直接吻合;3例同种带瓣主动脉重建RVOT。结果术后早期死于顽固性低心排血量综合征1例,无严重出血并发症和呼吸道并发症,姑息手术1例,血氧饱和度由术前的68．0％升至82.0％。16例随访10个月～8年,1例术后1年不明原因猝死;超声心动图示轻度残余漏3例,残余梗阻3例。结论复杂先天性心病并冠状动脉畸形RVOT重建时,应根据冠状动脉畸形特点选择手术方式。     

The results of probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classic tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure with a principal theoretical aim of minimizing structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to a right ventricular approach. However, it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients were reviewed who had repair of isolated TOF by this technique from 1993 to 2001. The mean age of patients was 5 +/- 2 years. Transatrial-transpulmonary approach was performed for all patients. To make the infundibular muscle-bundle resection easier and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. Tricuspid valve detachment in order to improve the exposure was done in 11 patients. All patients were followed up in our clinic at regular six-month intervals by echocardiography. RESULTS: There was no early or late mortality nor reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSIONS: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases gives excellent early and mid-term results.     

Results of the probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classical tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure in the goal to minimize structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to the right ventricular approach. However it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients who had repair of isolated TOF using this technique from 1993 to 2001 were reviewed. The mean age of patients were 5 +/- 2 years. Transatrial-transpulmonary approach were performed for all patients. To make easier the infundibular muscle bundles resection and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. To improve exposure, tricuspid valve detachment was performed in 11 patients. All patients were followed-up in our clinic every 6 months using echocardiography. RESULTS: There were no early or late deaths, and no reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA Class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSION: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases shows excellent early and mid-term results.     

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.     

双心室矫治室间隔缺损远离型右室双出口单中心15年经验

目的总结单中心双心室矫治室间隔缺损远离型右室双出口的15年经验,探讨双心室矫治的合适方法,分析再手术的危险因素。方法回顾性分析2005~2019年于我院连续入组162例接受双心室矫治的室间隔缺损远离型右室双出口患儿的临床资料。根据内隧道建立的路径将患儿分为两组:110例患儿术中行室间隔缺损连接至主动脉内隧道[A组,男75例、女35例,平均年龄(3.6±3.2)岁];52例患儿行室间隔缺损连接至肺动脉内隧道[B组,男30例、女22例,平均年龄(2.8±2.7)岁]。为了建立通畅的心室内隧道,同期进行的操作包括室间隔缺损扩大、圆锥肌肉切除、三尖瓣腱索或乳头肌转移等。结果全组行双心室矫治的患儿早期死亡9例(5.6%),早期心室内隧道梗阻6例(3.7%)。经过(7.5±7.0)年的随访,8例(4.9%)患儿发生晚期死亡,A组的1年、5年、10年、15年生存率分别为92.7%、91.1%、91.1%、85.4%,B组分别为92.2%、85.2%、85.2%、85.2%,两组差异无统计学意义(P=0.560)。随访发现10例(6.2%)迟发性心室内隧道梗阻,8例接受了再次手术。两组比较A组患儿有更多的迟发性心室内隧道梗阻(A组9例vs.B组1例,P=0.017)及总体心室内隧道梗阻(A组15例vs.B组1例,P=0.001)。两组患儿的早期死亡率和晚期死亡率差异无统计学意义(P=0.386、0.223)。A组中同期进行三尖瓣操作46例,其中1例发生左室流出道梗阻,是否同期行三尖瓣操作的术后左室流出道梗阻发生率差异具有统计学意义(1/46 vs.15/64,P=0.004),并不会造成三尖瓣反流或狭窄。Rastelli术后患儿因右室流出道病变再手术率明显高于REV手术和双根部调转术,两者差异具有统计学意义(5/14 vs.0/38,P<0.001)。结论双心室矫治室间隔缺损远离型右室双出口可以取得令人满意的远期结果。室间隔缺损连接至主动脉内隧道较室间隔缺损连接至肺动脉内隧道左室流出道梗阻发生率更高。同期进行三尖瓣处理可降低内隧道梗阻发生率。     

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 )     

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.     

Tetralogy of Fallot with subpulmonary ventricular septal defect

Among a total of 608 patients with tetralogy of Fallot, 35 with tetralogy and subpulmonary ventricular septal defect (VSD) have undergone intracardiac repair at the Children's Hospital, Buenos Aires. The crista supraventricular was absent in 62.9% of these patients; it was hypoplastic in 37.1%. Only 3 patients (8.6%) were severely cyanotic and required operation in infancy. The main and left and right pulmonary arteries were relatively large in all but 1 patient. Nevertheless, patch-graft enlargement across the pulmonary valve ring was necessary in 20 (57.1%). We believe that closure of the subpulmonary VSD increase the apparent severity of the pulmonary stenosis and necessitates patch-graft enlargement in patients in whom the appearance of the right ventricular outflow tract before repair would not have suggested its need.