Toshihide Nakano

Midterm surgical results of total cavopulmonary connection : clinical advantages of the extracardiac conduit method

OBJECTIVE We evaluated the midterm surgical outcomes of intra-atrial lateral tunnel and extracardiac conduit total cavopulmonary connection to clarify the clinical superiority. METHODS Patients (n = 167) underwent total cavopulmonary connection (88 with lateral tunnel and 79 with extracardiac conduit) from November 1991 to March 1999. Survival, incidence of reoperation and late complications, exercise tolerance, hemodynamic variables, and plasma concentration of natriuretic peptide type A were compared. In the lateral tunnel group, time-related change in lateral tunnel size was investigated for its relationship to postoperative arrhythmias. RESULTS The 8-year survival was 93.2% in the lateral tunnel group and 94.9% in the extracardiac conduit group. Seven reoperations were performed in the lateral tunnel group but none in the extracardiac conduit group. Supraventricular arrhythmias developed in 14 patients (15.9%) in the lateral tunnel group and in 4 patients (5.1%) in the extracardiac conduit group (P =.003). Freedom from cardiac-related events was 72.5% in the lateral tunnel group and 89.8% in the extracardiac conduit group at 8 years (P =.0098). Hemodynamic variables and exercise tolerance were similar in both groups but plasma natriuretic peptide type A concentration, a parameter of atrial wall tension, was higher in the lateral tunnel group. In the lateral tunnel group, intra-atrial tunnel size increased by 19.4% during the 44.2-month interval and the percent increase in tunnel size was an independent predictor of supraventricular arrhythmias. CONCLUSIONS The midterm survival, hemodynamic variables, and exercise tolerance were similar and satisfactory in both lateral tunnel and extracardiac conduit groups; however, the incidence of cardiac-related events was significantly less frequent in the extracardiac conduit group. In the lateral tunnel group, careful observation is required to monitor the relationship of the dilating tendency of the intra-atrial tunnel and the development of late complications.

Results of extracardiac conduit total cavopulmonary connection in 500 patients

OBJECTIVES This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

Twenty-eight years' experience of arterial switch operation for transposition of the great arteries in a single institution †

OBJECTIVES We reviewed our 28 years of experience of arterial switch operation (ASO) for transposition of the great arteries to investigate late sequelae of this procedure. METHODS 387 patients who underwent ASO from 1984 to 2010 were included in this retrospective study. The longitudinal data were estimated by the Kaplan-Meier method and compared using a log-rank test. Risk factors for late sequelae were analysed by the multivariable Cox proportional hazards model. RESULTS The mean follow-up time was 10.0 years. There were 13 early deaths and 17 late deaths. All late deaths were within 1 year, except for three patients. Actuarial survival was 92.2 and 91.6% at 10 and 20 years, respectively. Sixty-six patients (17.1%) had developed pulmonary stenosis (PS) and 29 patients (7.5%) had developed moderate or more aortic insufficiency (AI) during follow-up. Selective coronary angiography was performed in 210 patients (54.3%) at 9.6 ± 5.1 years after ASO. Left main tract occlusion was found in 2 patients (2/210; 1.0%) and hypoplastic left coronary artery was found in 10 patients (10/210; 4.8%). Among these 12 patients, 8 patients were asymptomatic. Re-operation was performed in 76 patients (19.6%), pulmonary artery plasty for PS in 58 patients (15.0%), aortic valve replacement for AI including two Bentall operations in 9 patients (2.3%) and others. Freedom from re-operation was 78.2 and 62.8% at 10 and 20 years, respectively. The risk factor for PS was the use of equine pericardium for reconstruction (P < 0.0001). Factors associated with moderate or more AI was the presence of left ventricular outflow tract obstruction (P = 0.004). There were no risk factors for late coronary lesions. Three hundred and forty surviving patients (340/357; 95.2%) were in NYHA functional class I. Treadmill test, which was performed on 217 patients (56.1%) at 14.3 ± 5.4 years after ASO, revealed that the maximum heart rate was 97.5 ± 7.6% of normal and peak oxygen consumption was 105.2 ± 20.5% of normal. CONCLUSIONS ASO was performed with satisfactory results in the overall survival and functional status. PS was the main reason for re-operation. Coronary lesions can appear late without any symptoms. Benefits of ASO can be achieved by long-term follow-ups of PS, AI and coronary lesions.

Early results of bilateral pulmonary artery banding for hypoplastic left heart syndrome

OBJECTIVE To compare the haemodynamics and perioperative course of initial palliation with bilateral pulmonary artery banding (PAB) and the Norwood procedure. METHODS Between April 2004 and December 2007, 43 consecutive children with hypoplastic left heart syndrome (HLHS) or a variant underwent initial palliation (PAB, n=18; Norwood, n=25). Clinical perioperative data were analysed. In the PAB group, lipo-prostaglandin E1 administration was continued with hospitalisation until stage 2 palliation with a bi-directional Glenn shunt and the Norwood procedure. RESULTS There were no significant differences in the age and operative weight of patients who received stage 1 palliation (PAB, 12+/-9 days, 2.7+/-0.6 kg; Norwood, 12+/-8 days, 2.8+/-0.4 kg). The PAB group had more high-risk patients than the Norwood group (PAB, 83%; Norwood, 48%, p=0.04). Increased early and inter-stage mortality were observed in patients who underwent the Norwood procedure (early mortality with PAB, 6% vs Norwood, 12%; inter-stage mortality, 6% vs 27%, respectively). Mortality between stages 1 and 2 was 11% for the PAB group and 36% for the Norwood group. The Kaplan-Meier survival estimate at 1 year did not differ between groups (77% for the PAB group, 64% for the Norwood group). Ductal stenosis was found in one patient in the PAB group during the follow-up period. Twenty-eight patients underwent stage 2 reconstruction, and the patients in the PAB group were younger at the time of surgery (PAB, 116 days; Norwood, 224 days). There were no significant differences between groups in pulmonary artery index regarding body surface area (BSA) (PAB, 179 mm(2)BSA(-1); Norwood, 194 mm(2)BSA(-1)) and the incidence of ventricular dysfunction after stage 2 construction (PAB, 21%; Norwood, 21%). CONCLUSIONS Bilateral PAB with continuous lipo-prostaglandin E1 administration may improve early and intermediate mortality in infants with HLHS. Intimate care with hospitalisation may contribute to the results.

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

AbstractObjective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm2/m2; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity.

Surgical results of double-orifice left atrioventricular valve associated with atrioventricular septal defects.

BACKGROUND Double-orifice left atrioventricular valve (LAVV) is a rare but surgically important anomaly, which is regarded as a risk factor for surgical correction of atrioventricular septal defects (AVSDs). METHODS Of 209 consecutive patients with AVSDs, double-orifice LAVV was identified in 19 patients (9.1%, including 7 infants). Preoperative LAVV function, surgical procedures and results, incidence of postoperative LAVV dysfunction and reoperations were reviewed and compared between patients with this valve malformation (group I, n = 19) and those without it (group II, n = 190). RESULTS There were no operative or late deaths in group I. Preoperative LAVV function was similar in both groups. The cleft was totally closed in 77.2% of group II and 47.1% of group I (p < 0.01). In partial AVSDs, freedom from postoperative LAVV insufficiency was 77.0% in group II versus 30.5% in group I at 5 years (p = 0.009) and freedom from reoperation was 89.9% in group II versus 58.3% in group I at 5 years (p = 0.012); however, there was no difference in complete AVSDs. None of the infants in group I underwent total cleft closure and 4 of them showed more than moderate LAVV insufficiency postoperatively. CONCLUSIONS Double-orifice LAVV is a significant predictor for postoperative LAVV incompetence and reoperation in partial AVSDs, but not in complete AVSDs. Surgical procedures for the cleft should be individualized with careful intraoperative evaluation of the structure and function of this abnormal valve, especially in partial AVSDs and infants.

The yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract

OBJECTIVE To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT). METHODS Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and -9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus-Kaye-Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years. RESULTS There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients. CONCLUSIONS The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates.

The impact of extracardiac conduit-total cavopulmonary connection on apicocaval juxtaposition

OBJECTIVES Modifications of the Fontan procedure are applied to a wide range of complex congenital heart defects with single ventricle physiology. We examined the pathway and the clinical results of extracardiac conduit-total cavopulmonary connection (EC-TCPC) for a malpositioned heart with apicocaval juxtaposition. METHODS Of the 365 patients who underwent EC-TCPC since 1994, 56 patients with a malpositioned heart with apicocaval juxtaposition were included in this retrospective study (group 1). The pathway for the EC was selected after careful consideration of the results of preoperative angiography and computed tomography, as well as intra-operative findings. A concurrent group of 299 patients undergoing EC-TCPC without apicocaval juxtaposition was used as a control group (group 2). The mean follow-up periods for groups 1 and 2 were 5.5 years (range: 0-12 years) and 5.5 years (range: 0-14 years), respectively. RESULTS The mean age at operation was 4.2±3.2 years and the median size of the conduit was 18 mm (range: 16-20mm). In 30 patients, the conduit was placed between the inferior vena cava (IVC) and the opposite side of the pulmonary artery crossing the vertebra. In another 25 patients, the conduit was positioned behind the ventricle between the IVC and on the same side as the pulmonary artery (PA). There was one patient who had a Y-style conduit placed between the IVC and right and left PAs behind the ventricle. In group 1, there were no early deaths; three patients died in the intermediate term due to gastric bleeding in one, haemoptysis in another and sudden death in yet another. None of the patients developed conduit stenosis or pulmonary venous obstruction, and no patient required re-operation. Three patients developed late complications, including arrhythmias requiring medication in two and subdural haematoma in one. The incidence of death or late complications did not differ among the pathways of conduits. In group 2, there were eight late deaths, and 27 patients developed late complications. The Kaplan-Meier survival rate was 93.5% at 5 and 10 years in group 1, and 97.3% at 5 years and 96.1% at 10 years in group 2 (log-rank test, P=0.29). The haemodynamics in groups 1 and 2 during the intermediate term were identical with respect to IVC pressure (8.9 ± 2.5 in group 1 and 9.6 ± 2.6mmHg in group 2), left ventricular end-diastolic pressure (4.4 and 4.3 mmHg, respectively), cardiac index (3.3 and 3.4 l min(-1)m(-2), respectively) and arterial oxygen saturation (94 and 94.2%, respectively). No patient in either group had a pressure gradient >2 mmHg between the IVC and central PA. Postoperative catheterisation data showed no significant differences in haemodynamics between the conduit pathways in group 1. CONCLUSIONS EC-TCPC can be performed in children with apicocaval juxtaposition with excellent mid-term outcomes compared with other Fontan candidates. Based on individual cardiac anatomy, the pathway of the EC behind the ventricle or crossing the vertebra can be used without conduit stenosis or pulmonary venous obstruction.

A 17-year experience with mitral valve repair with artificial chordae in infants and children

OBJECTIVES We sought to examine our long-term results of mitral valve (MV) repair with expanded polytetrafluoroethylene (ePTFE) sutures and to determine the predictors for the outcome of this procedure. METHODS Between 1995 and 2011, MV repair with chordal reconstruction by artificial chordae was achieved in 78 patients (34 males and 44 females). Median age at repair was 1.5 years (range 3.6 months-13.4) and weight was 9.1 kg (2.5-31.4). The mean follow-up was 8.3 years. A Cox proportional hazards model was used to analyse the risk factors for a composite outcome of death, conversion to other MV repair techniques or MV replacement, reoperation on MV and recurrent mitral regurgitation (MR). RESULTS According to Carpentier classification, 65 (83.3%) patients were Type 2 and 13 (16.7%) were Type 3. Mitral annuloplasty was performed in all cases, except 2. During MV repair, 8 (10.3%) patients were ineffective with artificial chordae and converted to other techniques. Six (7.7%) patients underwent MV reoperation (three repairs and three replacements). Freedom from MV reoperation was 92.5 and 90.4% at 5 and 10 years, respectively. There was 1 in-hospital death. At the latest follow-up, moderate or more MR was observed in 3 (3.8%) patients. Risks for the composite outcome were low body weight at operation and Carpentier classification Type 3. CONCLUSIONS MV repair with artificial chordae in infants and children is safe and effective and associated with a low reoperation rate. Further investigation into the long-term durability and biological adaptation of ePTFE sutures after patient growth is mandatory.

“Spiral-Cuff” Technique for Repair of Anomalous Left Coronary Artery From the Pulmonary Artery

We describe a new technique for coronary transfer in the repair of anomalous origin of the left coronary artery from the pulmonary artery. The left coronary artery is elongated with the native pulmonary artery wall to form a spiral-shaped coronary cuff to construct an unstretched new left coronary artery system from the ascending aorta. A postoperative angiographic scan showed good flow of the left coronary artery without any kinking or narrowing. This technique is considered useful when an anomalous coronary artery arises a long way from the ascending aorta.