Hideaki Kado

Selective cerebral perfusion technique during aortic arch repair in neonates

We describe selective cerebral perfusion techniques for repair of the aortic arch in neonates. These techniques may help protect the brain from ischemic injury caused by a cessation of cerebral perfusion for aortic arch reconstruction in patients with hypoplastic left heart syndrome or interrupted aortic arch.

Elevated levels of plasma endothelin-1 in young patients with pulmonary hypertension caused by congenital heart disease are decreased after successful surgical repair.

R E F E R E N C E S 1. Hill PE. Complications of permanent transvenous car: diac pacing: a 14-year review of all transvenous pacemakers inserted at one community hospital. PACE Pacing Clin Electrophysiol 1987;10:564-70. 2. Ellis GL. Pacemaker twiddlers syndrome: a case report. Am J Emerg Med 1990;8:48-50. 3. Roberts JS, Wenger NK. Pacemaker twiddlers syndrome. Am J Cardiol 1989;63:1013-6. 4. Lal RB, Avery Rd. Aggressive pacemaker twiddlers syndrome: dislodgement of an active fixation ventricular pacing electrode. Chest 1990;97:756-7.

Clinical Results of the Staged Fontan Procedure in High-Risk Patients

BACKGROUND For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared. METHODS Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (> or =20 mm Hg), high pulmonary vascular resistance (> or =3 Wood units), small pulmonary artery (Nakata index <200 mm2/m2), atrioventricular valve incompetence (> or = moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same period, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group). RESULTS In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group). CONCLUSIONS A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.

Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients

BACKGROUND In the Fontan procedures total cavopulmonary connection with an extracardiac conduit is a concern. The potential benefits of an extracardiac conduit may be the avoidance of postoperative supraventricular arrhythmias over the long-term, hemodynamic benefits due to laminar flow, possibility of completion without anoxic arrest, and applicability to anomalous systemic or pulmonary venous return, or both anomalous systemic and pulmonary venous return. We demonstrate early to midterm results of total cavopulmonary connection with an extracardiac conduit. METHODS Between March 1994 and February 2000, a total of 100 patients underwent total cavopulmonary connection with an extracardiac conduit. In 27 patients, who underwent a single stage total cavopulmonary connection operation, 7 were done without palliation. Seventy-three patients had undergone a bidirectional Glenn shunt before completion of the total cavopulmonary connection. We used an expanded polytetrafluoroethylene tube graft as the extracardiac conduit. RESULTS Cardiopulmonary bypass time was 133.2+/-55.2 minutes. Myocardial ischemic time was 38.5+/-23.2 minutes in 40 patients who needed cardioplegic cardiac arrest for intracardiac procedures. Intraoperative fenestration was done in only 1 patient. There were no operative deaths. During follow-up of 37.3 months, there were 5 late deaths. When compared with the patients treated by the lateral tunnel technique in our institute, there was no significant difference in actuarial survival rate, but the event free rate of the extracardiac conduit group was significantly superior to the lateral tunnel group. CONCLUSIONS Total cavopulmonary connection with the extracardiac conduit produced good results in short to midterm follow-up.

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.

Clinical results of mitral valve repair by reconstructing artificial chordae tendineae in children

OBJECTIVE There are an increasing number of reports concerning mitral valve repair by reconstructing the chordae tendineae with the use of expanded polytetrafluoroethylene sutures in adults. However, little information is available about application or results of this technique in children. METHODS Between January 1995 and December 1997, 16 children who had from moderate to severe mitral regurgitation mainly as the result of a prolapse of the anterior leaflet (age range, 5 months-12.8 years) underwent mitral valve repair by reconstruction of artificial chordae. Either unilateral or bilateral Kay-Reed annuloplasty was also performed to correct annular dilatation in all patients. RESULTS No operative death or morbidity was observed. Before discharge, immediate postoperative echocardiography showed less than trivial mitral regurgitation in all patients. The follow-up was complete in all cases by a clinical examination and serial echocardiograms, and the median follow-up period was 14.8 months (range, 1.3-26.4 months). There were no valve-related events during the entire follow-up period. The degree of mitral regurgitation, estimated by echocardiography performed at recent follow-up period, was none in 5 patients, trivial in 10 patients, and mild in 1 patient. The diastolic and systolic dimensions of the left ventricle decreased and were 95.0% and 96.2% of the normal values, respectively. CONCLUSIONS Although further investigations and long-term results are still called for, mitral valve repair by reconstruction of the artificial chordae was found to be safe and effective even in infants and children.

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.

Mixed type of total anomalous pulmonary venous connection

BACKGROUND The mixed type of total anomalous pulmonary venous connection is a rare condition in which some diagnostic and surgical problems still remain to be solved. METHODS In 9 patients a single pulmonary vein was connected to the systemic vein at a site different from the drainage site of the confluence of three other pulmonary veins. In 2 other patients, four pulmonary veins made a confluence which had two drainage sites. Correct diagnosis was made in all 7 patients who received cardiac catheterization but only in 5 of the 9 patients by color Doppler echocardiography. Total correction was performed in 3 patients and the single anomalous pulmonary vein was left uncorrected in 8 other patients. RESULTS There were two in-hospital deaths. Seven patients with a single residual anomalous pulmonary vein have been in good condition without clinical symptoms of congestive heart failure or pulmonary hypertension. CONCLUSIONS Diagnosis of mixed type of total anomalous pulmonary venous correction by echocardiography is sometimes difficult. When a mixed type is suspected, cardiac catheterization is recommended if the condition of the patient permits it. A single anomalous pulmonary vein may be left uncorrected without serious complications, but close observation is needed to prevent congestive heart failure and pulmonary vascular obstructive disease.

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.