Results with the two-patch technique for repair of complete atrioventricular septal defect

From May, 1982, to September, 1983, 9 patients underwent repair of complete AV septal defect. They ranged in age from 11 months to 48 months and in weight from 5.3 kg to 16.5 kg. Seven patients were 24 months old or less. Previous operations included pulmonary artery banding in 1 patient and ligation of a patent ductus arteriosus with repair of coarctation in another. All patients had large left-to-right shunts (mean pulmonary to systemic flow ratio, 3.1), and the 7 young infants had marked pulmonary hypertension. Mitral regurgitation was absent in 2 patients, mild in 3, moderate in 2, and severe in 2. One patient had the right ventricular dominant form of complete AV septal defect. In all instances, repair was done using separate ventricular and atrial patches. Leaflet tissue was not divided, and a trileaflet mitral valve was left in each patient. Eight patients survived operation and are well 3 to 17 months after repair. The single operative death occurred in the patient with right ventricular dominance. Only 1 patient has mild residual heart failure 4 months after operation. Clinically, mitral regurgitation is absent in 4 patients and, at most, mild in the other 4. No patient has a conduction disturbance. Repair of complete AV septal defect is facilitated by using separate patches for the ventricular and atrial components of the defect. Less distortion is created, and a more accurate reconstruction of a competent trileaflet mitral valve can be done.     

Surgical repair of complete atrioventricular septal defect.

BACKGROUND: The objective of this study was to assess the outcome of complete atrioventricular septal defect repair from 1981 to 2000. METHODS: One hundred seventy-two consecutive patients with atrioventricular septal defect were operated on by a single surgeon using a consistent operative technique (single patch; "cleft" closure). The patients' age range was from 5 weeks to 9 years (mean, 10.8 +/- 1.2 months). RESULTS: Overall operative mortality was 15 of 172 (8.7%) and this decreased significantly from 12 of 73 (16.4%) in the first decade to 3 of 99 (3.0%) in the second decade (p = 0.0021) with no operative deaths in the last 51 patients. Operative mortality was related to decade of operation (p = 0.0021) and to use of crystalloid cardioplegia (p = 0.0047) by univariate analysis, and to decade of operation (p = 0.0016) and postoperative time on ventilator (p = 0.0023) by multivariate analysis. Actuarial long-term survival including operative deaths was 79.0% +/- 3.8% at 15 years. Ten of 157 (6.4%) operative survivors have undergone reoperation for late mitral regurgitation (9 mitral valve repair, 1 mitral valve replacement) with one death. Four of 8 patients surviving late mitral valve replacement have subsequently required mitral valve repair. Freedom from late reoperation for severe mitral regurgitation was 89.9% +/- 3.1% at 15 years. Freedom from late reoperation for mitral regurgitation did not decrease in the second decade (84.2% +/- 6.6% at 10 years) versus the first decade (94.5% +/- 3.1%) (p = 0.0679). CONCLUSIONS: Although operative mortality for repair of atrioventricular septal defect has decreased dramatically during the past decade, the incidence of late reoperation for mitral regurgitation has not improved, and better techniques to eliminate late mitral regurgitation are needed.     

完全性房室间隔缺损的外科治疗

目的回顾性总结手术治疗完全性房室间隔缺损的经验。方法112例病儿,≤6个月43例(38%,X组),>6个月69例(62%,Y组)。85例行心导管检查。Rastelli A型89例,Rastelli B型10例,Ras- telli C型13例。手术技术分单片法,双片法和简化单片法。术中经食管超声检查发现异常而即刻再次手术者7例(二尖瓣反流4例,二尖瓣狭窄2例,左室流出道梗阻1例)。术后入重症监护室,左房压8～21 mm Hg,中心静脉压7～12mm Hg。呼吸机平均应用47h,监护室平均滞留6.3d。结果室间隔缺损残余分流(直径>2mm)13例,二尖瓣中度反流12例,完全性房室传导阻滞4例。院内死亡6例(X组1例,Y组5例)。术后随访91例(81%),随访1～5年,平均2.3年。1例术后1年因肺炎心衰死亡,1例术后2年因二尖瓣中-重度反流而换瓣。结论院内死亡率提示,小于6月龄完全性房室间隔缺损病婴手术是安全的。随着年龄增大,瓣膜成形效果、肺动脉高压的预后可能会更差。双片法修补室间隔缺损较易发生残余漏(9例,18%),简化单片法出院时二尖瓣关闭不全发生率明显高于另外两种方法(6例,16%)。     

Single-stage repair of complete atrioventricular septal defect

We report our experience with 31 consecutive children who underwent single-stage repair of complete atrioventricular septal defect between 1984 and December 1991. Ages ranged from 2 months to 2.5 years, mean 11 months. 18 were classified Rastelli type A, 13 type C. 22 patients had Down's syndrome, 12 were Rastelli type C. 1 patch was used in Rastelli type A cases and 2 patches in type C patients, without incision of the atrioventricular valve tissue. In all but 1 case the left superior and inferior valve leaflets were approximated. 5 patients died postoperatively resulting in an overall mortality of 16.1%. In all survivors, good clinical results and sinus rhythm were seen, although all show some degree of mitral incompetence.     

Primary repair of complete atrioventricular septal defect in infancy.

Forty infants with complete atrioventricular septal defect have undergone primary repair within their 1st year of life. The mean age at time of surgery was 4 months (range 1-12 months) and the mean weight 4.2 kg (range 2.9-7.0 kg). Either the one- or the two-patch method was used. Four patients died (10%). There were no late deaths. Three patients needed reoperation due to mitral valve insufficiency. Postoperative complications were: 1 total atrioventricular block in an infant with an absent ventricular septum 3 weeks after surgery, 9 complete right bundle branch blocks, 3 small residual ventricular septal defects. Mean follow-up of the 36 survivors is 22 months (3-46 months). Thirty (83%) are in NYHA functional class I, 4 (11%) in NYHA class II and 2 (6%) in NYHA class III. No atrioventricular valve regurgitation is detectable by the color Doppler technique in 19 (53%) patients. Mitral incompetence is mild in 13 cases (36%) and moderate in 4 (11%). In ten recatheterized patients the average systolic pulmonary to systemic artery pressure ratio dropped from 1.0 to 0.42 and the average pulmonary arteriolar resistance was reduced from 5.5 to 3.9 U/m2. All but one patient gained weight and underwent adequate statomotoric development. Primary repair should be performed at the age of 5-6 months or even earlier, if pulmonary arteriolar resistance exceeds 5 U/m2.     

Surgical treatment of complete atrioventricular septal defect with the two-patch technique: early-to-mid follow-up

We report our results on surgical treatment of complete atrioventricular septal defects using the two-patch technique. Forty patients with complete atrioventricular septal defects were operated on in the period from November 1995 to January 2004 and retrospectively analyzed. The age at the time of surgery ranged from 4 months to 20 years (average=18.8+/-37 months). Their weights ranged from 3 to 39 kg (average=7.6+/-5.8 kg). Associated tetralogy of Fallot was present in 20% of the cases (8 patients). Monitoring was complete until January 2007, corresponding to a follow-up ranging from 36 to 135 months (average=74+/-33.7 months). The surgical mortality rate was 2.5% and the hospital mortality rate was 5%. A third patient died from a brain abscess two years after surgery. Over the long-term, two patients needed further operations: one was submitted to mitral plasty due to severe residual mitral insufficiency, one year later; the other underwent a resection of a sub-aortic membrane after three years. Differences were evaluated using the Student-t or Mann-Whitney tests. Surgical treatment of complete atrioventricular septal defect using the two-patch technique results in low morbidity and mortality in early-to-mid term follow-up.     

Correlation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair

 Open in a separate windowOBJECTIVESThere are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes.METHODSWe retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre. VSD height and left ventricular length on the four-chamber view were measured. Demographic details and early and late outcomes including reoperation and long-term survival were studied.RESULTSTwenty patients underwent modified single-patch repair and 25 patients underwent double-patch repair of CAVSD. VSD height in the modified single-patch group ranged from 4.2 to 11.7 mm and in the double-patch group ranged from 5.1 to 14.9 mm. Nine patients had a deep ‘scoop’ with a VSD height of >10 mm, (7 double patch, 2 modified single patch). VSD height did not correlate with a specific Rastelli classification. There was no significant difference in the VSD height (P = 0.51) or the VSD height-to-left ventricular length ratio (P = 0.43) between the 2 repair groups. There was no 30-day mortality. Eight patients required reoperation; however, VSD height was not a significant predictor of reoperation (hazard ratio 0.95, 95% confidence interval 0.69–1.33; P = 0.08).CONCLUSIONSThere was no correlation between VSD height and risk of reoperation after CAVSD repair. A deep ventricular scoop is uncommon in CAVSD patients.     

The simplified single patch repair of complete atrioventricular septal defect

Between May 1995 and November 2003, 88 consecutive patients referred to 2 consultant cardiac surgeons (GN and IN) for repair of complete AVSD had this technique used. The mean age at operation was 8.3 months (median 3.3 months, range 1 to 40 months). The mean weight at operation was 5.7 kg (range 2.3 to 16.8). Down syndrome was present in 63 patients (71.6%).The size of the ventricular septal defect was graded on preoperative echocardiography by cardiology review as restrictive (14 patients), moderate (21 patients), or large (47 patients). All patients had the described technique performed with no modifications, regardless of the size of the ventricular component.Nine patients had associated tetralogy of Fallot or pulmonary atresia anatomy. These patients were all initially treated with modified Blalock Taussig GoreTex shunts. Later repair of tetralogy (2 patients) or construction of RV-PA conduit (7 patients) at the time of AVSD repair was performed.Four patients had pulmonary artery banding due to small size and later had debanding and complete AVSD repair.There were 3 early deaths (3.4%). All 3 had other associated surgery at the time of AVSD repair (1 tetralogy repair, 1 RV-PA conduit for pulmonary atresia, and one aortic and pulmonary valvotomy for aortic stenosis and pulmonary stenosis).Of the survivors, post procedure echocardiography revealed mild or less mitral regurgitation in 73 patients (86%), moderate regurgitation in 7 (8%), and severe in 1 patient. Follow-up was performed on 78 of the 85 survivors at a median of 30 months (mean 36 months, range 0 to 97 months). Two patients required mitral valve replacement (2.5%). One of these patients had severe and one moderate mitral regurgitation recorded at initial post AVSD repair echocardiography. Of the remaining patients, 73 had mild or less mitral regurgitation (93.5%) and 3 had moderate regurgitation (4%).In general, this technique has lead to reduced cross clamp times and overall bypass time in our hands. It effectively eliminates an extra suture line used in other techniques. We have had good mitral valve function postoperatively and have not seen significant left ventricular outflow tract obstruction despite our initial theoretical concerns. We have applied this simplified technique to all of our AVSD patients regardless of size of ventricular defect.     

Primary repair of complete transposition of the great arteries with complete atrioventricular septal defect.

We successfully corrected complete transposition of the great arteries associated with complete atrioventricular septal defect in a 50-day-old infant in concomitant arterial switch operation and two-patch repair. The combination of these 2 complex anomalies is very rare, and primary anatomical repair is feasible in such patients.     

One stage slide cricotracheoplasty and repair of complete atrioventricular septal defect

Successful single stage repair of complete atrioventricular septal defect and cricotracheal stenosis in an 8-month-old baby with Trisomy 21 is described. The case is notable for rarity of cricoid stenosis being managed together with the long segment tracheal narrowing using the technique of slide cricotracheoplasty.     

Ideal timing of surgical repair of isolated complete atrioventricular septal defect

The ideal timing and optimal management of surgical repair for isolated complete atrioventricular septal defect (CAVSD) still remains controversial. To determine outcomes after the surgical repair of CAVSD, we reviewed 100 consecutive patients who underwent complete repair at our institute between January 1992 and August 2003. Among these 100 patients, 52 were female and 73 had Down's syndrome. Twelve had received preceding pulmonary artery banding. A two-patch repair was employed in all cases. The patients' median age and weight were 4.5 (1.2-48) months and 4.7 (2.5-12.5) kg, respectively. The mean stays in the intensive care unit and in the hospital were 5.3+/-3.8 and 25.4+/-18.1 days, respectively. The median duration of mechanical ventilation was 11.6 h. There were two in-hospital deaths, in patients 5.2 and 5.9 months of age. Both had underlying Down's syndrome, and significant pulmonary vascular obstructive disease (PVOD) was detected on postmortem specimen. The operative outcome of CAVSD was generally satisfactory. PVOD can progress rapidly from four to five months, especially with Down's syndrome. Therefore, in order to avoid progression to irreversible PVOD, surgical intervention within four months of birth may be appropriate in such patients.     

Two-patch repair of complete atrioventricular septal defect in the first year of life. Results and sequential assessment of atrioventricular valve function

Before January 1987, 62 infants underwent two-patch repair of complete (51) or intermediate (11) atrioventricular septal defect at the Royal Children's Hospital, Melbourne. Median age at repair was 4.3 months and median weight was 4.4 kg. Early deaths (3%) were confined to two infants with preoperative respiratory tract infections; a further two patients died during follow-up (late mortality rate 3%). Reoperation for severe postoperative mitral regurgitation was necessary in 10 infants (16%), two of whom subsequently required mitral valve replacement with a prosthesis. Preoperative atrioventricular valve regurgitation was assessed retrospectively in 49 patients from angiography or Doppler echocardiography and was found to be absent or mild in 33 (68%), moderate in 9 (18%), and severe in 7 (14%). At the time of latest review (at a mean of 2.4 years after repair), judged from a combination of clinical and echocardiographic criteria, mitral regurgitation was absent or mild in 49 (84%) of the 58 survivors; none of them had symptomatic regurgitation or were requiring continuing medical treatment. Analysis of sequential atrioventricular valve function in 46 of the 49 patients in whom objective preoperative data were available showed no relationship between the degree of preoperative and postoperative atrioventricular valve regurgitation. Infants without Down's syndrome, however, had a significantly higher reoperation rate for severe postoperative mitral valve regurgitation (50%) than those with Down's syndrome (10%) (p = 0.007). Complete atrioventricular septal defect can be repaired in early infancy with a low mortality rate and good intermediate term results.     

Complete repair of atrioventricular septal defect

We report our experience with 103 consecutive children who underwent repair of complete atrioventricular septal defect between 1971 and 1990. Ninety-one patients were less than 18 months old (mean age, 6.2 months; mean weight, 5.8 kg) and were repaired using deep hypothermia and circulatory arrest. There were 15 perioperative deaths. Twelve patients were older (mean age, 40.2 months; mean weight, 18.9 kg) and were repaired using moderate hypothermia and cardiopulmonary bypass. There were two perioperative deaths. Repairs were performed with the single-patch technique. Four younger patients required repeat repair to control residual mitral regurgitation. Two of the older children required late reoperation to replace one or both atrioventricular valves. Three younger children underwent pulmonary artery banding initially; 1 died after complete repair. Three older children underwent initial pulmonary artery banding; 2 died at definitive repair, and the survivor required pulmonary artery reconstruction, which was repeated subsequently. Since 1977 our policy has been to perform primary definitive repair whenever possible. Two patients died late from unrelated causes. At the most recent follow-up the majority of patients had no or minimal symptoms. We continue to advocate primary definitive repair whenever possible using the single-patch technique in symptomatic patients with complete atrioventricular septal defect.     

完全型房室间隔缺损的外科治疗

目的　总结完全型房室间隔缺损外科治疗的经验。方法　16例完全性房室间隔缺损患儿,平均年龄(1.2±0 .9)岁,平均体重(6.8±3 )kg。其中10例伴有Down综合征,1例合并法洛四联征。术前超声心动图显示房室瓣轻度反流12例,中度反流3例,重度反流1例。行单片法修补10例,双片法修补6例。结果　术后恢复顺利,无围手术期死亡。除1例患儿于出院4个月后因肺部感染合并心衰死亡外,余随访0 .3～5 .2年,经超声心动图检查显示房室瓣功能良好,未见明显反流。结论　完全型房室间隔缺损患者早诊断,早手术,可获良好疗效。     

Two-patch repair of complete atrioventricular septal defect using a small ventricular patch

Since 1992, 19 patients with an atrioventricular septal defect have undergone surgical treatment using a novel annuloplasty technique in which a small ventricular patch was used to reduce the anterior-posterior dimension of the atrioventricular orifice. All patients recovered uneventfully and needed no reoperation for the residual regurgitation or shunt. The results showed that the new annuloplasty technique was promising, although a long-term result is yet to be seen.     

Biventricular repair in children with complete atrioventricular septal defect and a small left ventricle.

OBJECTIVE: Biventricular repair of complete atrioventricular septal defect (CAVSD) with small left ventricle aims to restore the normal loading conditions of the left ventricle. This report retrospectively evaluates the outcome of biventricular repair in 19 children with CAVSD and a small left ventricle. METHODS: Our computer database was searched for all patients with CAVSD operated on between January 1988 and December 2005. Patients who underwent biventricular repair of CAVSD were considered for investigation if they had a preoperative left ventricle-to-right ventricle long axis ratio (LAR) of <1.1 as determined by cardiac catheterization. RESULTS: There were 259 patients who underwent surgical correction of complete atrioventricular septal defect. Nineteen (10.3%) of 184 patients who underwent biventricular repair had small left ventricle based on LAR measurements. These children had no other associated congenital heart anomalies and had no previous surgery. Nine of these children had associated Trisomy 21. There were 10 (52.6%) males and 9 (47.4%) females, with age of 1-210 (122+/-67) days and weight of 2.5-9.0 (5.26+/-1.7) kg at surgery. Mean LAR was 0.76+/-0.14. Two patients (10.5%) died on the 8th and 11th postoperative day, respectively. Both had very small left ventricle (LAR of 0.45 and 0.60, respectively) and received ECMO support for postoperative low output syndrome and intractable pulmonary hypertension. A patient with an LAR of 0.62 who had ECMO support for postoperative myocardial failure underwent successful heart transplantation on the 21st postoperative day. The long-term survivors (89.5%) with LAR>0.65 had an uncomplicated postoperative course, had undergone regular follow-up (65+/-36 months) with echocardiographic assessment of the left ventricle, and had good left ventricular function. There was no early reoperation for residual ventricular septal defect, left AV valve regurgitation, or left ventricular outflow tract obstruction. Late reoperation was performed in three patients (17.4%) who underwent mitral valve repair for significant regurgitation in the 18th, 59th, and 87th month postoperatively. CONCLUSIONS: Biventricular repair of CAVSD with small left ventricle in infants and children whose LAR is >0.65, although not without risks, improve patients' functional and clinical status even in long-term follow-up. Particular caution should be taken in patients with LAR of <0.65, since these are patients who may not be amenable to biventricular repair, but for whom univentricular palliation may be more suitable.