Intraoperative prebypass and postbypass epicardial color flow imaging in the repair of atrioventricular septal defects

The use of intraoperative epicardial Doppler echocardiography with color flow imaging both before and after cardiopulmonary bypass to assist repair of congenital heart defects in infants and small children has not been reported. To demonstrate its simplicity and utility, we obtained immediately prebypass and postbypass examinations from 18 consecutive patients undergoing repair of atrioventricular septal defects between March 1987 and March 1988 (aged newborn to 3 years, smallest 2.4 kg, 11 patients less than 1 year old). Direct application of a sterile transducer to the epicardial surface eliminates any restriction imposed by the transesophageal approach, and the routine use of color flow imaging enables the operating surgeon to directly evaluate intracardiac anatomy and flows in a variety of orientations. Average examination time was 3.95 +/- 1.96 minutes. Prebypass studies disclosed that a short-axis inspection through the common atrioventricular valve orifice produced a unique visualization of the dynamic commitments of atrioventricular valve tissue throughout systole and diastole that was helpful in planning valve allocation during repair. In addition, echocardiography demonstrated features not previously appreciated in seven of 18 patients (39%). In all, image quality and resolution were vastly superior to preoperative chest wall studies. Postbypass studies revealed significant residual interventricular shunts in two of 18 patients (11%). Views obtained from various orientations directed specific and efficient repair immediately so that all patients left the operating room with documented, surgically acceptable results. Comparison of ventricular function between prebypass and postbypass studies enabled appropriate application of pharmacologic agents in the operating room if necessary. All patients survived their operation. There have been two late deaths, and 16 patients are alive and doing well (follow-up: 9 to 21 months). These experiences indicate that intraoperative epicardial Doppler color flow imaging (1) can be easily learned and applied by the surgeon, (2) enhances the repair of atrioventricular septal defect by providing unique spatial, anatomic, and flow information in the beating heart at the time of repair, (3) increases confidence of a surgically acceptable repair before the patients leave the operating room, (4) guides specific surgical or anesthetic adjustments to optimize results, and (5) works as a valuable aid that may help reduce poor results in the repair of complex congenital cardiac lesions.     

Intraoperative transoesophageal echocardiography in a low birth weight neonate with atrioventricular septal defect

An 18-day-old male neonate (45 cm, 1.8 kg) with a history of cyanosis and congestive heart failure from an atrioventricular septal defect (AVSD) with a large left-to-right shunt was scheduled for surgical repair of the AVSD. After routine induction of anaesthesia with fentanyl and vecuronium, a 4.5-mm diameter transoesophageal echocardiography (TOE) probe was inserted into the oesophagus, and systematic echocardiographic evaluation was performed during surgery. After cardiopulmonary bypass was stopped, intraoperative TOE revealed mild residual mitral valve regurgitation. Because good left ventricular wall motion was confirmed and haemodynamic parameters were stable, cardiopulmonary bypass was not reinitiated. The patient's cardiac output was low in the postoperative intensive care unit. TOE was performed the next day to detect the source of this problem, revealed severe regurgitation compared with that observed intraoperatively. TOE was useful for evaluation of the residual mitral valve regurgitation, and we reconfirmed the importance of continuous monitoring even in a low birthweight neonate undergoing repair of a complete AVSD.     

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.     

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.     

Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect

BACKGROUND: Excellent surgical results have been reported for repair of incomplete atrioventricular septal defect; however, left atrioventricular valve regurgitation (ltAVVR) is a major cause of late morbidity. We reviewed our entire experience with incomplete atrioventricular septal defect in order to investigate long-term results of ltAVVR after repair and determine the factors influencing the progression of ltAVVR in late follow-up. METHODS: Between 1983 and 2002, 61 patients underwent surgical repair of incomplete atrioventricular septal defect, including 7 patients with intermediate forms. The age of operation ranged from 1 month to 62 years old (median 5.3 years old). Thirteen patients were less than 2 years old, including 7 infants, while there were 15 adult patients. All patients underwent patch closure of the ostium primum defect. Before 1995, the cleft was left open in 7 patients and partial closure of the cleft was done in 41 patients, whereas complete closure of the cleft was performed in 9 patients since 1996. Preoperative and postoperative ltAVVR at hospital discharge and late follow-up were graded 0-IV by echographic evaluation. RESULTS: There was 1 early death and 4 late deaths with a 91% 10-year actuarial survival rate. Preoperative ltAVVR grade was I in 25 patients, II in 31 patients, III in 4 patients, and IV in 1 patient. Postoperatively, ltAVVR deteriorated in 3 patients. Left AVVR decreased in 21 patients, whereas in 37 patients it remained the same at hospital discharge. Consequently, ltAVVR remained grade II in 18 patients, grade III in 2, and there was no patient with grade IV. During the long-term follow-up, 24 patients were noted to have increased ltAVVR, including grade III in 8 patients and grade IV in 4. Reoperations for ltAVVR were required in 5 patients (8.3% of hospital survivors); valve replacement in 3 patients and valve repair in 2. Actuarial freedom from reoperation for ltAVVR was 91% at 10 years, whereas actuarial freedom from postoperative ltAVVR grade III or more was 89% at 5 years and 78% at 10 years. Multivariate analysis indicated that postoperative ltAVVR grade II or more at hospital discharge (p = 0.0032, odds ratio = 7.41, 95%CI: 1.95-28.10) was the only independent variable related to late ltAVVR, whereas age at operation, preoperative grade of ltAVVR, and the method of cleft repair were not significant risk factors. CONCLUSIONS: Left AVVR is still a significant risk in long-term follow-up. Because the postoperative grade of ltAVVR is the only independent risk factor for late ltAVVR, more efforts should be focused on left atrioventricular valve repair so as to minimize residual regurgitation, even mild regurgitation.     

Intraoperative color flow mapping by real-time two-dimensional Doppler echocardiography for evaluation of valvular and congenital heart disease and vascular disease

We have developed real-time two-dimensional Doppler echocardiography, which can display color flow mapping on a two-dimensional echocardiogram. Intraoperative two-dimensional Doppler echocardiography was performed during cardiovascular operations before and after the definitive procedure in 33 patients, 14 with valvular disease, eight with congenital heart disease, and 11 with vascular disease. Its clinical usefulness was evaluated. In patients with valvular heart disease, 13 valve replacements, 10 valvuloplasties, and four untouched lesions were assessed. No paravalvular leakage and three instances of minimal physiological transvalvular leakage from St. Jude Medical valves in the mitral position were noticed. Regurgitation after valvuloplasty was graded by extent of the regurgitant flow. This grading, comparable to postoperative grading, indicated no need for an additional procedure. In congenital heart disease, preoperative diagnoses were confirmed. The effect of the corrective operation was evaluated and no significant leakage or stenosis was found. Interatrial shunt flow was shown to increase after operative balloon atrial septostomy in a patient with dextro-transposition of the great arteries. In 11 patients with dissecting aortic aneurysm, the aneurysm was totally visualized in the operative field, including the structure and flow dynamics. In two patients, the preoperative cineangiographic diagnosis regarding involvement of dissection was corrected. After the vascular procedure, sufficient flow in the major aortic branches was confirmed in all patients and minimal leakage at the suture line was noticed in four patients. In conclusion, intraoperative color flow mapping by two-dimensional Doppler echocardiography has enabled the precise diagnosis and the necessary operation to be determined before cardiopulmonary bypass. It has also allowed the effects of the operation to be assessed before chest closure.     

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.     

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.     

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.     

Direct closure of ostium primum defect in the repair of atrioventricular septal defect

BACKGROUND: Patch closure is generally performed for atrial septation of an atrioventricular septal defect. We recently developed a new surgical technique for repairing atrioventricular septal defects that avoids the use of any patch material for closing the atrial septal defect. We report our experience with this procedure. METHODS: Seven patients (complete type: 5, partial type: 2) underwent this new operation. The diameters of the atrial septal defects were measured by transesophageal echocardiography. The preoperative electrocardiograms were compared with those taken after the operations. RESULTS: Diameters of the atrial defects ranged from 3 to 10 mm. Electrocardiograms before and after the operations did not change. No significant atrioventricular valve regurgitation and no residual shunts were detected by postoperative echocardiography. CONCLUSIONS: This method simplifies the repair of atrioventricular septal defects. In the short-term results, no arrhythmia and no valve regurgitation was seen.     

Complete atrioventricular septal defect repair: Simplified single patch technique

Background: There has been a rekindling of interest in alternatives to conventional two patch technique for the repair of complete atrioventricular septal defect in infancy in the recent past. We applied the simplified single patch technique to 15 consecutive infants and herein report our intermediate term results. Methods: Between March 1998 and September 2001, fifteen patients underwent repair of complete atrioventricular septal defect with this technique (mean age 6 months, mean weight 5.4 kg). Downs syndrome was present in 11 patients. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum irrespective of the size of the ventricular septal component. The cleft in the anterior mitral leaflet was closed in all patients. The atrial septal component was closed by a pericardial patch. Results: There was no mortality. There were no pulmonary arterial hypertensive crises or heart block. The mean follow up was 13.2 months. One patient underwent mitral valve replacement after one year due to severe mitral regurgitation. The remaining fourteen patients had no significant mitral regurgitation, residual ventricular septal defect or left ventricular outflow tract obstruction on echocardiography. Conclusion: Simplified single patch technique is an easily reproducible method for surgical repair of complete atrioventricular septal defect. It is less time consuming and minimises ischaemic time. Atrioventricular valve function is preserved and there is no incidence of obstruction to left ventricular outflow tract. The intermediate term results are encouraging. Presented in the poster session of the 37^th Annual Meeting of Association for European Paediatric Cardiology (AEPC) at Porto, Portugal, May 2002     

Tricuspid valve repair. Operative and follow-up evaluation by Doppler color flow mapping

Severe tricuspid regurgitation may produce significant morbidity and mortality if not corrected, but commonly used methods of intraoperative assessment may be unreliable. Tricuspid regurgitation was evaluated by a new intraoperative technique, Doppler color flow mapping, in 85 patients before and after cardiopulmonary bypass. Regurgitation grade by intraoperative color Doppler mapping correlated well with right ventricular angiography (kappa value = 0.92, p less than 0.01; n = 8) and with preoperative color Doppler studies (kappa = 0.71, p less than 0.05; n = 51). The right atrial V wave correlated poorly with the severity of tricuspid regurgitation intraoperatively, both before (r = 0.30) and after (r = -0.05, p = no significant difference) cardiopulmonary bypass. Advanced (3+ or 4+) tricuspid regurgitation was found in 40% (21) of 52 patients requiring mitral valve repair or replacement. Tricuspid annuloplasty with a prosthetic ring provided a significant (greater than or equal to 2 grade) reduction in regurgitation severity in 94% (17/18; p less than 0.05). Without repair, tricuspid regurgitation decreased to a similar degree after mitral valve operations in 14% (5/36); only one of the five patients had advanced tricuspid regurgitation prepump. Fluid filling of the arrested right ventricle after the surgical procedure did not predict regurgitation severity (false negative rate 50%, 2/4; false positive rate 22%, 2/9). Regurgitation grade remained unchanged after the initial postpump study, up to 60 weeks postoperatively. In conclusion, color Doppler flow mapping provides more accurate intraoperative assessment of tricuspid regurgitation than the right atrial V wave or fluid filling of the right ventricle. This semiquantitative technique aids in the selection of patients appropriate for surgical repair of the tricuspid valve and is useful in judging the adequacy of tricuspid valve repair before chest closure. Advanced (3+ or 4+) tricuspid regurgitation is a common occurrence in patients undergoing mitral valve repair or replacement and rarely responds to conservative (nonoperative) management. Ring annuloplasty provides a highly effective and durable reduction in tricuspid regurgitation.     

Hemolytic anemia after atrioventricular septal defect repair without synthetic material

We report a rare case of severe hemolytic anemia following repair of a congenital heart defect without the use of prosthetic material. A review of the literature, diagnosis, and management are described. Although this is an unusual complication following congenital heart surgery, a high index of suspicion must be maintained and a possible mechanical cause should be sought and corrected.