Pediatric cardiac surgery guided by echocardiography. Established indications and new trends

Cardiac surgery in 602 children was not preceded by cardiac catheterization, the diagnosis being based on clinical findings and two-dimensional and Doppler echocardiography. In the 355 operations without cardiopulmonary bypass there were nine major and seven minor diagnostic errors (2.5% and 2%). Among the 247 cases with open-heart surgery there were no major and eight (3.2%) minor errors. The malformations most suitable for nonbypass surgery without catheterization seem to be those with reduced pulmonary blood flow requiring systemic-pulmonary artery shunt, aortic coarctation and patent ductus arteriosus. For open-heart surgery without invasive investigation, atrial septal defect, partial atrioventricular canal, aortic and pulmonary stenosis, cardiac tumor and isolated valve disorder are 'classic' candidates. Recent experience indicated that selected cases of complete atrioventricular canal, tetralogy of Fallot, truncus arteriosus, total anomalous pulmonary venous connection and transposition of the great arteries may safely undergo primary repair without cardiac catheterization. Because of its diagnostic potentialities, pediatric cardiac surgeons must become familiar with echocardiography.     

Deep hypothermia in cardiovascular surgery

Recent experimental studies and clinical application of deep hypothermia for cardiovascular surgery are reviewed. At most institutions, surface hypothermia alone or in combination with limited cardiopulmonary bypass has been employed. Circulatory dynamics were well maintained following prolonged cardiac arrest at 20°C. Some degree of acidosis usually developed after the arrest period but was gradually corrected during rewarming. Total circulatory occlusion could be maintained for at least one hour at 20°C. without evidence of cerebral damage in infants. Many complex congenital cardiac anomalies, including transposition of the great arteries, total anomalous pulmonary venous return, ventricular septal defect, and tetralogy of Fallot, have been successfully corrected in the first few weeks of life. Less encouraging results have been achieved in patients with infradiaphragmatic total anomalous pulmonary venous return, complete atrioventricular canal, or pulmonary atresia.     

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.     

Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.     

Open-heart surgery in pediatric patients with end-stage liver disease

Little is known about the safety of pediatric cardiac surgery in children with end-stage liver disease. We reviewed our experience with 4 patients with biliary atresia or Alagille's syndrome who underwent repair of ventricular septal defect and tricuspid regurgitation, atrioventricular canal, subaortic stenosis, or supravalvular aortic stenosis. One patient died on postoperative day 2. All other patients survived to discharge. At follow-up, 1 patient died at home awaiting liver transplantation and the remaining patients are doing well. One patient received a successful liver transplant. Pediatric cardiac surgery in children with end-stage liver disease can be done safely, albeit with a higher mortality.     

Early and late results of total correction of congenital cardiac anomalies in infancy

OBJECTIVE: We evaluated long-term results of surgical correction of congenital cardiac anomalies in infancy. METHODS: We reviewed cases of 856 patients who underwent complete correction of major cardiac anomalies in the first year of life during last 24 years at our institution, and analyzed results. Surgery involved ventricular septal defect (n = 453), tetralogy of Fallot (n = 92), atrioventricular canal defect (n = 93), and complete transposition of the great arteries (n = 218). RESULTS: Operative mortality was 2.2% in ventricular septal defect, 0% in tetralogy of Fallot, 8.6% in atrioventricular canal defect, and 4.1% in complete transposition of the great arteries. Freedom from reoperation at 20 years was 96.5 +/- 2.0% and actuarial survival was 94.2 +/- 1.3% in ventricular septal defect. Freedom from reoperation at 15 years was 90.5 +/- 3.9% in tetralogy of Fallot and 86.6 +/- 4.4% in atrioventricular canal defect. Actuarial survival at 15 years was 97.8 +/- 1.6% in tetralogy of Fallot, 85.7 +/- 3.7% in atrioventricular canal defect, and 89.9 +/- 2.2% in complete transposition of the great arteries. Actuarial survival in complete transposition of the great arteries was significantly better in arterial than in atrial switch operations. CONCLUSIONS: Total correction of ventricular septal defect, tetralogy of Fallot, atrioventricular canal defect, and complete transposition of the great arteries in infancy was conducted safely, but the incidence of reoperation in late follow-up must be reduced.     

Pulmonary artery banding

Between January 1981 and December 1983 22 children underwent pulmonary artery banding at the Red Cross War Memorial Children's Hospital, Cape Town. The indications for banding in this group were control of pulmonary overperfusion and severe congestive cardiac failure. The underlying cardiac defects were tricuspid atresia with ventricular septal defect (VSD), transposition of the great arteries and univentricular heart, large or multiple VSDs, double-outlet right ventricle, atrioventricular canal defect, persistent truncus arteriosus and the VSD-patent ductus arteriosus-coarctation syndrome. Three children died (13,6%) in the first 30 days postoperatively and another 3 died 5-7 weeks after surgery.     

Patient whose surgery was postponed due to complete atrioventricular block on arrival at operating theater

An 86-year-old woman with low cardiac function was scheduled to undergo hip fracture surgery. Preoperative electrocardiogram showed complete left bundle brunch block, first degree atrioventricular block, left axis deviation and bigeminy. However, her electrocardiogram had changed to complete atrioventricular block on arrival at operating theater. ACC/AHA guideline on perioperative cardiovascular evaluation and care for non cardiac surgery indicates the assessment of both the urgency of the surgery and cardiac complications. Because complete atrioventricular block is classified to "active cardiac conditions", we decided to postpone the surgery for more detailed evaluation and treatment of cardiac conditions. In spite of the discontinuation of digoxin and carvegilol, complete atrioventricular block continued for a week, and the permanent pacemaker was inserted. The surgery was performed 2 weeks following the insertion of the pacemaker without any problems under combined general and lumbar epidural anesthesia.     

Evaluation of a new finger-type intraoperative epicardial echocardiographic probe for infants and small children

A new finger-type epicardial echocardiographic probe was evaluated in 26 children with complex cardiac anomalies during open heart surgery. The probe is a small 3.75 MHz phased array transducer for 2-dimensional echo and color Doppler imaging. The tip is slightly angled to facilitate the apical approach through a median sternotomy. The patients included 4 neonates and 8 infants, and the major cardiac lesions were complete atrioventricular (AV) canal, transposition of the great arteries, double outlet right ventricle, and others. The intraoperative study was possible in most of the patients facilitating various approaches for the assessment of left-sided AV valve function, left ventricular outflow obstruction, residual shunt and intraatrial anatomy. Four patients received subsequent operative procedures after the assessment. This transducer appears to be useful in the intraoperative study during surgery for complex cardiac anomalies in neonates and small infants.     

Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease

We present the case of 52-day-old girl with a common atrioventricular canal. Severe liver dysfunction persisted following complete repair of the cardiac defect. A patent ductus venosus appeared to be the source of the hemodynamic disturbance responsible for hepatic dysfunction. Given her critical condition, coil embolization of the ductus venosus was performed, after which the patient improved rapidly. The ductus venosus should be tested for patency when liver dysfunction persists after the corrective cardiac surgery, and coil embolization is the treatment of choice in gravely ill children.     

无顶冠状静脉窦综合征合并房室通道的临床分析

目的 分析无顶冠状静脉窦综合征(UCSS)合并房室通道的病理学特点和外科手术治疗方法.方法 1999年9月至2007年10月,20例UCSS合并房室通道患者接受外科手术治疗.男性10例,女性10例;年龄0.5～38.0岁,平均(11.4±11.0)岁;体重6.7～73.0 Kg,平均(28.4±21.3)kg.其中合并完全型房室通道2例,部分型房室通道18例,同时合并单心房12例.复杂型UCSS合并左上腔静脉(LSVC)直接汇入左心房,即UCSS Ⅰ型共11例,其中10例采用行心房内板障术或心内隧道术将LSVC引流入右心房,直接结扎LSVC 1例.简单型UCSS 9例用其他方法矫治.所有患者同期行房室通道畸形矫治术.结果 全组手术死亡1例,死于术后肺部感染;其余19例手术效果满意.随访14例,随访时间4个月～3年,无死亡病例和并发症.结论 房室通道合并LSVC及单心房时,要警惕UCSS的存在.根据UCSS的不同病理类型选择不同的手术方法,可获得满意的手术效果.     

Mitral valve replacement after atrioventricular canal repair in adults: Report of three cases

Three cases of adults who required a mitral valve replacement (MVR) following repair of an atrioventricular (AV) canal are presented herein. In adult patients with an AV canal, the deformity of the mitral valve is sometimes too advanced for adequate repair, and therefore, residual mitral regurgitation frequently causes intractable cardiac failure despite appropriate medical management. Thus, for adults with an AV canal associated with severe valvular deformities, MVR should be considered from the initial operation to obtain a significantly better outcome.     

Late recovery of atrioventricular conduction after pacemaker implantation for complete heart block associated with surgery for congenital heart disease

OBJECTIVES: Pacemaker implantation is a standard recommendation for patients with persistent complete heart block following surgery for congenital heart disease. This study was performed to determine the incidence and clinical significance of late recovery of atrioventricular conduction following pacemaker implantation. METHODS: Between 1990 and 2001, 5662 open cardiac procedures for congenital heart defects were performed at our institution. The postoperative course of all patients with complete heart block in whom a permanent pacemaker was implanted was followed on a monthly basis, by either clinical or transtelephonic follow-up. RESULTS: A total of 72 patients with persistent postoperative complete heart block underwent pacemaker implantation. After insertion of the pacemaker, recovery of atrioventricular conduction was recognized in 7 of 72 patients (9.6%) at a median of 41 days (18-113 days) after the initial cardiac operation. These included 3 patients with ventricular septal defect, 2 with ventricular inversion or single ventricle, and 1 each with left ventricular outflow tract obstruction and atrioventricular septal defect. During a mean follow-up of 4.4 +/- 2.6 years, there was no late recurrence of heart block. Three patients had residual right bundle branch block and 1 had first-degree atrioventricular block. CONCLUSIONS: Atrioventricular conduction may return in a small but significant percentage of patients following pacemaker implantation for complete heart block associated with congenital heart surgery. When recovery of atrioventricular conduction occurs within the first months after surgery it appears reliable, which suggests that lifelong cardiac pacing may not be necessary in these individuals.     

Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients

Double-orifice mitral valve is an uncommon but surgically important condition. The experience in 25 cases of double-orifice mitral valve associated with atrioventricular canal defects was reviewed. This constituted 4.3% of the 581 cases of atrioventricular canal defects operated upon between 1961 and July, 1984. The combined mitral orifice area ranged from 85% to 91% of normal in those patients whose valves were sized intraoperatively. Ten associated cardiac defects were repaired in six patients. Of 23 patients having cleft mitral valve, 21 had partial closure of the cleft. There was one operative death (4.0%), which occurred early in the series in a patient in whom the tissue bridge was severed and massive mitral regurgitation resulted. In the remaining 24 patients the tissue bridge was left intact, and all survived operation. No patient had clinically significant mitral stenosis during a follow-up of 1 to 14 years (mean 4.9 years). Two patients (8%) developed progressive mitral regurgitation and required mitral valve replacement 3 and 11 years postoperatively. One of these patients died and a second death occurred suddenly 2 years following operation. All survivors are in Functional Class I or II. The noncleft orifice of a double-orifice mitral valve usually is competent and rarely requires closure. The cleft, because it constitutes a type of parachute (single papillary muscle) valve, should be closed partially so as to relieve valve incompetence without causing undue stenosis. The incidence of late development of mitral regurgitation is similar to that of atrioventricular canal without double-orifice mitral valve. Repair of atrioventricular canal associated with double-orifice mitral valve can be achieved with a low operative mortality and excellent late results.     

Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.     

Concomitant repair of complete atrioventricular canal defect and transposition of the great arteries in an infant

The case of an infant who was born with the rare presentation of complete atrioventricular canal defect and transposition of the great arteries is described. The patient underwent pulmonary banding at the age of 2 months, followed by concomitant arterial switch and repair of the atrioventricular canal defect at 20 months. Palliative banding allowed for the infant to grow and thus facilitated the later successful repair of these congenital defects.     

Mitral valve replacement in the first year of life

From 1973 through 1987 25 patients underwent mitral valve replacement in the first year of life for mitral stenosis and mitral regurgitation. The patients with mitral stenosis included two with mitral arcade, two with supravalvular mitral stenosis with hypoplastic mitral valve, and one with parachute mitral valve. Included in the group of patients with mitral regurgitation were 12 with atrioventricular canal defect, six with chordal and leaflet defects, one with Marfan's syndrome, and one with bacterial endocarditis. Prostheses included 12 Bj?rk-Shiley (17 mm), seven St. Jude Medical (19 mm in four, 21 mm in three), five stent-mounted dura mater valves (12 mm to 16 mm), and one porcine xenograft (19 mm). In four patients the valves were placed in the left atrium in a supraannular location. There were nine operative (atrioventricular canal defect seven, mitral regurgitation two) and five late (atrioventricular canal defect four, mitral stenosis one) deaths, giving actuarial 1- and 5-year survival rates of 52% and 43%, respectively. All 6 patients with tissue valves died; the four with supraannular mitral valve replacement survived. Since 1983 operative mortality has been reduced to 0% (70% confidence limits 0% to 24%). Nine patients required a second mitral valve replacement for prosthetic stenosis 5 to 69 (mean 30) months after the original mitral valve replacement (one operative death). Because of improvements in repair of atrioventricular canal defect in infancy, the need for mitral valve replacement at atrioventricular canal defect repair has decreased. Although valvuloplasty has been advocated for repair of congenital mitral valve disease and is applicable in some infants with mitral regurgitation, mitral valve replacement is frequently unavoidable for congenital mitral disease and can now be accomplished at a low operative risk, even when the prosthesis has to be positioned supraannularly.     

A tribute to Giancarlo Rastelli

Giancarlo Rastelli (1933-1970) was a pioneer cardiac surgeon who developed a classification of atrioventricular canal and a novel surgical procedure that revolutionized the management of children with congenital heart disease. Rastelli lived a short, yet fascinating life. His work was ahead of its time and laid the foundation for the treatment of complex congenital cardiac anomalies.     

Spinal anesthesia in a patient with congenital complete atrioventricular block

Congenital complete atrioventricular block is a rare cardiac conduction abnormality that predisposes to arrhythmias and cardiac arrest. Recommendations for the anesthetic management of patients with this condition are based on the few case reports that have been published. We describe the successful use of spinal anesthesia for ankle osteosynthesis in a patient with asymptomatic congenital complete atrioventricular block detected during preoperative assessment.     

Cor Triatriatum Sinistrum: Surgical Experience in Taiwan

Purpose Cor triatriatum sinister, more commonly referred to as cor triatriatum (CT), is characterized by a common pulmonary venous chamber (proximal chamber) separated from the left atrium (distal chamber) by a fibromuscular septum. This report of our experience updates the surgical results of this anomaly in the Asian population. Methods Between January 1992 and May 2005, nine patients with cor triatriatum underwent surgical correction at Chang Gung Memorial Hospital. We retrospectively analyzed their cardiac anatomy, clinical data, surgical procedures and follow-up data. Results The mean age at surgery was 260 ± 215 days (range, 20–790 days), with the exception of one patient who underwent surgery at 31 years of age. Three patients had a partial anomalous pulmonary venous connection, one had a complete atrioventricular canal defect, and one had another major complex cardiac anomaly. Eight corrective operations and one palliative operation were performed. There was no surgical mortality. The mean follow-up time was 52.1 ± 43.6 months (range, 17–139 months). Conclusion Our findings show that surgical correction is efficient and safe for this rare cardiac anomaly.