Characteristics and long-term outcome for congenital left main coronary artery atresia.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

Early clinical outcomes of right ventricular outflow tract reconstruction with small caliber bovine jugular vein conduit (Contegra®) in small children.

This study reviewed early clinical outcomes of right ventricular outflow tract reconstruction with Contegra® valved conduits in pediatric patients. Between April 2013 and July 2014, thirteen pediatric patients underwent right ventricular outflow tract reconstruction with Contegra valved conduits. The size of the implanted conduits were 12 mm in 5 patients, 14 mm in 3, 16 mm in 3, and then 2 patients were implanted with bicuspidized conduits for downsizing the conduit to 9 and 10 mm in each. Follow-ups were completed in all patients. One conduit was explanted 7 days after a neonatal biventricular repair for Ebstein's anomaly and pulmonary atresia, timed to be at the point of conversion to a single ventricular palliation. Among the 5 patients who developed significant pulmonary insufficiency and/or conduit stenosis, 3 patients exhibited persistent pulmonary hypertension. Both the bicuspidized conduits resulted in early pulmonary insufficiency. One patient implanted with a ring-supported conduit developed coronary artery stenosis, caused by suppression between the ring of the implanted conduit and the annulus of the mechanical vale. Although the small caliber Contegra valved conduit might be an alternative to RVOT reconstruction, the indication should be carefully considered. More than mild pulmonary hypertension, and low body weight at operation of less than 3.0 kg caused early conduit dysfunction.

Impact of Ross Operation on Outcome in Young Female Adult Patients Wanting to Have Children.

BACKGROUND: The most appropriate valve substitute at aortic valve replacement (AVR) for young female adult patients wanting to have children is unclear. METHODS AND RESULTS: Between 1992 and 2013, 12 consecutive female patients aged >18 (median, 22.5 years; range, 18-34 years) underwent Ross operation (Ross group). Between 1984 and 2013, 9 consecutive female patients aged >18 (median, 30 years; range, 22-39 years) underwent AVR with bioprosthesis (bioprosthesis group). There was 1 late mortality in the bioprosthesis group, due to prosthetic valve endocarditis (PVE). Freedom from reoperation for aortic valve at 15 years was 90.0% in the Ross group, and 57.1% in the bioprosthesis group (log-rank, P=0.098). One in the Ross group underwent reoperation for aortic regurgitation (AR), whereas 4 in the bioprosthesis group did so for aortic stenosis (AS) in 2, combined AS and AR in 1, and PVE in 1. Five patients in the Ross group and 3 in the bioprosthesis group had 7 and 4 uneventful pregnancies, respectively. AR progressed during the perinatal period in a total of 7 of 11 pregnancies. No AS was seen at discharge, after 5 years, or during pregnancy in the Ross group. CONCLUSIONS: The long-term outcome of Ross operation for female patients wanting to have children is excellent. Although subclinical pulmonary autograft valve regurgitation during pregnancy was often observed, pulmonary autograft stenosis did not occur, therefore it would be an ideal option for patients wanting to have children.

Indication of Takeuchi technique for patients with anomalous origin of the left coronary artery from the pulmonary artery.

BACKGROUND: Intrapulmonary tunnel repair, called the Takeuchi technique, is a unique procedure for repairing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Since 1986, we have clearly defined the indication for the Takeuchi technique based on the location of the left coronary artery (LCA) orifice. METHODS AND RESULTS: From 1986 to 2011, 19 consecutive patients with ALCAPA underwent surgical repair; the dual-coronary system was reconstructed in 16 of these patients with either Takeuchi (n=9: 1 male; median age 14.7 years; median weight 42.7 kg) or a translocation procedure (n=7, 3 males; 0.4 years old, 5.6 kg). Takeuchi was performed in patients whose LCA arose far from the aorta (middle of posterior facing sinus in 3 patients, left side of posterior facing sinus in 2, non-facing sinus in 4). The mean postoperative follow-up period was 7.4±6.1 years in the Takeuchi group and 9.3±8.5 years in the Translocation group. Actuarial survival rate at 10 years was 87.5% and 71.4%, respectively, and the freedom from either reoperation or re-intervention rate at 10 years was 67.7% and 85.7%, respectively. All the patients who died had a preoperative left ventricular ejection fraction≤30%. CONCLUSIONS: The long-term outcome of the Takeuchi technique was acceptable. Although late reoperation and/or re-intervention cannot be disregarded, Takeuchi can be recommended when the LCA arises far from the aorta.

Clinical characteristics of acute mitral regurgitation due to ruptured chordae tendineae in infancy-experience at a single institution.

In infants, acute mitral regurgitation resulting from ruptured chordae tendineae is very rare, but often fatal. There are a few case reports, but the characteristics and etiology of chordae tendineae rupture have not been elucidated. Our aim was to determine the clinical characteristics of idiopathic acute mitral regurgitation due to chordal rupture in infancy. A retrospective analysis was performed on ten consecutive patients, with a mean onset age of 4.6 ± 1.3 months. Despite nonspecific initial symptoms, all patients developed respiratory distress and four required resuscitation within a few days (mean, 1.8 ± 1.8 days). Chest radiographs showed pulmonary congestion with a normal or mildly increased cardiothoracic ratio in all ten patients. Laboratory data and electrocardiograms showed nonspecific findings. Echocardiography revealed ruptured chordae in all patients; locations were anterior (50%), posterior (20%), and both (30%). Surgical intervention was performed within 24 h of admission in eight patients (mean, 3.6 ± 5.1 h). Pathological findings included inflammatory cells in six specimens and myxomatous degeneration in two. No bacteria were isolated from preoperative blood cultures, pathological tissues, or excised tissue cultures. Autoantibody levels were insignificant. Three preoperatively resuscitated patients developed neurological sequelae and arrhythmias occurred in four after mitral valve replacement. Acute onset and rapid deterioration in patients with ruptured chordae tendineae necessitates early surgical intervention to improve outcomes. Though the etiology remains unknown, onset is in infants approximately 4 months of age, suggesting a definite disease entity.

The clinical course and incidence of supraventricular tachyarrhythmias after extra-cardiac conduit Fontan procedures in relation to an atrial situs.

BACKGROUND: The extra-cardiac conduit Fontan (EC) has a lower incidence of tachyarrhythmias than other types of Fontan. However, some intrinsic arrhythmogenic conditions, such as atrial isomerism, have been associated with a high incidence of arrhythmias. METHODS AND RESULTS: We retrospectively reviewed the clinical course of the supraventricular tachyarrhythmias (SVTs) in 212 patients after an EC, compared the results according to the atrial situs and investigated the substrate of the SVTs. The atrial situs was a solitus or inversus (SS/SI) in 152 patients, right isomerism (RI) in 45 and left isomerism (LI) in 15. Twenty-four SVTs occurred in 22 (11%) patients ≥3 months after the EC. The freedom from SVT after the EC was 95%, 76% and 77% in the SS/SI, RI and LI patients at 5 years, respectively (P<0.0001). Among the 16 SVTs associated with atrial isomerism, 10 were not related to the atrioventricular (AV) node. For the atrial isomerism, the predictors of SVT ≥3 months after the EC were a history of an AV valve repair (risk ratio (RR) 1.9; P=0.02) and complications associated with sinus node dysfunction (RR 1.9; P=0.03). Death related to SVT occurred in 3 patients. CONCLUSIONS: Postoperative SVTs after the EC with an atrial isomerism were not uncommon, mostly did not involve the AV node and possibly were caused by atrial tissue damage. A comprehensive therapeutic strategy should be considered.

Mid- to long-term outcomes of cardiovascular tissue replacements utilizing homografts harvested and stored at Japanese institutional tissue banks.

PURPOSE: We reviewed our clinical experiences with cardiovascular homografts harvested and preserved at our institutional tissue banks. METHODS: Since our bank was first established in Japan in 1990, 74 patients have undergone various surgical procedures using homografts. We classified them into five groups according to the procedure: Group I, subcoronary implantation of a homograft aortic valve; Group II, homograft aortic root replacement for active native or prosthetic endocarditis; Group III, homograft aortic replacement for mycotic aortic aneurysms or infected grafts; Group IV, pulmonary homografts in the Ross operation; and Group V, pulmonary homograft conduits for complex congenital heart diseases. RESULTS: The 9- to 10-year survival rates were good and acceptable, respectively, for the patients in all five groups. The infection recurrence rate was low (8%). Cardiac event-free rates, including deaths, were 0.57 in Group I, 0.58 Group in II, 0.75 in Group III, 0.81 in Group IV, and 0.69 in Group V operations. The rates of structural homograft deterioration suggest that homografts deteriorate more rapidly after subcoronary implantation than aortic root replacements (P = 0.058). CONCLUSIONS: Subcoronary implantation should probably be abandoned for routine aortic valve replacement, but the continued use of homografts will provide valuable alternatives for patients with active infectious cardiovascular diseases. For the Ross operation, pulmonary valve homografts showed good durability.

Twenty-five-year outcome of pediatric coronary artery bypass surgery for Kawasaki disease.

BACKGROUND: The long-term outcome of pediatric coronary artery bypass for patients with severe inflammatory coronary sequelae secondary to Kawasaki disease is unknown. METHODS AND RESULTS: One hundred fourteen children and adolescents ranging in age from 1 to 19 (median, 10) years at operation were followed up for as long as 25 years with a median of 19 years. The number of distal anastomoses was 1.7+/-0.8 per patient, and the internal thoracic artery was used in all but 3, most frequently for left anterior descending artery lesions. Saphenous vein grafts were used in 24 patients, mostly for non-left anterior descending artery lesions. Patients underwent multiple angiograms to evaluate their coronary and graft status. There was no operative or hospital mortality. Both 20- and 25-year survival rates were 95% (95% confidence interval [CI], 88 to 98). Five deaths occurred, all cardiac in origin. Cardiac event-free rates at 20 and 25 years were 67% and 60% (95% CI, 46 to 72), respectively. Percutaneous coronary intervention and reoperation were the most common events. Overall, the 20-year graft patency rate was 87% (95% CI, 78 to 93) for internal thoracic artery grafts (n=154) and 44% (95% CI, 26 to 61) for saphenous vein grafts (n=30) (P<0.001), and the rate for non-left anterior descending artery lesions was also significantly better for arterial grafts (87% [95% CI, 73 to 94]; n=59) than for saphenous vein grafts (42% [95% CI, 23 to 60]; n=27) (P=0.002). Eighty-eight patients (77%) remain on medications, but all 109 survivors are presently symptom free in their daily activities. CONCLUSIONS: Although the 25-year survival was excellent after pediatric coronary bypass for Kawasaki disease, the event-free rate declined progressively. This reality mandated continued follow-up. Reinterventions successfully managed most cardiac events. An internal thoracic artery graft was the most favorable for children.

The fate of bioprostheses in middle-aged patients: the Japanese experience.

BACKGROUND AND AIM OF THE STUDY: Although the trend of bioprosthesis use has been evaluated extensively, the durability of currently available bioprostheses has not been determined in middle-aged patients. The study aim was to determine the long-term fate of bioprostheses implanted in patients aged < 60 years. METHODS: Valve implantation data were collected from 43 centers in Japan. The data included patient age at implantation, type of valve, implant position, follow up period, and cause of reoperation including structural valve deterioration (SVD) and non-SVD. Between 1975 and 2005, a total of 697 bioprostheses was implanted in the mitral position, and 247 in the aortic position. The mean follow up period was 9.2 years. Rates of freedom from SVD and reoperation were determined using an actuarial method. RESULTS: The mean age at implantation was 45 +/- 10.9 years. The 15-year freedom from SVD was 39% for those with valves implanted in the aortic position, and 27% in the mitral position (p = 0.004). For the same period, the actuarial freedom from reoperation was 31% for valves in the aortic position, and 24% in the mitral position (p = 0.178). The difference in actuarial freedom from SVD was not significant between age groups in the mitral position. However, there were differences in actuarial freedom from SVD in the aortic position for patients aged < 10 years when compared to the other age groups (p < 0.001). New-generation valves showed better long-term durability than older valves (p = 0.05). CONCLUSION: The long-term freedom from SVD in middle-aged patients was unfavorable for bioprostheses implanted in the aortic and mitral positions. Middle-aged patients must be made aware that reoperation will be necessary; consequently, the choice of bioprosthesis should be dictated by patient-surgeon preference.

Neurodevelopment in 1-year-old Japanese infants after congenital heart surgery.

BACKGROUND: The purpose of this study was to determine the prevalence of persistent neurodevelopmental sequelae in 1-year-old infants after open heart surgery for congenital heart disease, using the Bayley Scales of Infant Development second edition (BSID-II). A secondary objective was to confirm the applicability and usefulness of the BSID-II in Japanese infants. METHODS: Thirty-nine infants who underwent repair of a ventricular septal defect before 6 months of age and 108 normal Japanese infants at 1 year of age were assessed using the BSID-II. RESULTS: In normal infants, scores on the Mental Development Index and the Psychomotor Development Index components of the BSID-II ranged from borderline retardation to very superior following a normal distribution similar to those obtained for US controls. No problems were encountered, either in translation or in following the instructions when the BSID-II was used to evaluate the 1-year-old Japanese infants. On the other hand, the mean scores on the Mental Development Index and the Psychomotor Development Index were significantly lower in Japanese patients than in normal Japanese infants, particularly for gross motor development (P < 0.001). Linear regression analysis showed that a longer intensive care unit stay was associated with impaired cognitive development at 1 year of age (P= 0.03). CONCLUSIONS: Neurodevelopmental functions in 1-year-old infants with congenital heart disease were lower than those in normal infants, especially for gross motor function using the BSID-II. One risk factor that correlated with neurodevelopmental sequelae was the length of intensive care unit stay.

High prevalence of abnormal glucose metabolism in young adult patients with complex congenital heart disease.

BACKGROUND: Abnormal glucose metabolism (AGM) adversely impacts morbidity and mortality in patients with chronic heart failure. No data on AGM in adult patients with congenital heart disease (ACHD) are available. METHODS: To assess the AGM in ACHD and compare the results with their clinical characteristics, we performed a 75-g oral glucose tolerance test to detect AGM, that is, insulin resistance, impaired glucose tolerance, and diabetes mellitus, in 205 consecutive ACHD (24 +/- 8 years), including 16 unrepaired patients, 67 Fontan patients, 122 postbiventricular (BV) patients, and 27 healthy controls (27 +/- 5 years). RESULTS: All ACHD groups had a high prevalence of AGM (unrepaired, 43.8%; Fontan, 43.3%; BV, 46.7%; control, 3.7%; P < .001). In the 2 postoperative groups, the Matsuda index was decreased (P < .0001), and greater waist circumference, liver dysfunction, higher plasma renin activity, and diuretic use were associated with AGM. Although male gender was associated with AGM (P < .01), baseline glucose and lipid metabolic variables did not correlate with the 75-g oral glucose tolerance test-induced hyperglycemia (area under the plasma glucose curve [AUC-PG]) in the Fontan patients but did correlate in the BV patients. The AUC-PG correlated inversely with exercise capacity (P < .05) in the 2 postoperative ACHD groups, and the AGM ACHD had a high incidence of future cardiac events (P < .05), especially the Fontan patients with diabetes mellitus (P < .01). CONCLUSIONS: Complex ACHD have a high prevalence of AGM, and this newly recognized pathophysiology should be considered in managing long-term survivors of complex ACHD.

Is diabetic nephropathy a predicted risk factor?--Kaplan-Meier and multivariate analysis of confounding risk factors in off-pump coronary artery bypass grafting for chronic dialysis patients.

BACKGROUND: The purpose of this study was to investigate the preoperative risk factors of performing off-pump coronary artery bypass grafting (OPCAB) in patients on chronic dialysis. METHODS AND RESULTS: The 41 consecutive patients on chronic dialysis who underwent OPCAB from February 2000 to April 2006 at the National Cardiovascular Center were studied retrospectively. Of them, 29 had diabetic nephropathy (DN group) and the remaining 12 did not (NDN group). There were significant differences in the duration of dialysis before surgery (9.1 +/-7.5 years in NDN vs 4.2 +/-5.5 years in DN, P=0.028) and low cardiac function (left ventricular ejection fraction <30%), which was recognized only in the DN group (7/29, P=0.048). The early mortality rate was 6.9% (2/29) in the DN group and 16.7% (2/12) in the NDN group (P=0.349). The actuarial survival rates in the DN group were 85% at 1 year, 45% at 3 years, and 30% at 5 years, whereas in the NDN group they were 71%, 49%, and 49%, respectively (P=0.789). arteriosclerosis obliterans (ASO) and age (>65 YEARS) were independent risk factors of late death. CONCLUSIONS: For patients on chronic dialysis ASO and aging were predicted risk factors for OPCAB, whereas diabetic nephropathy was not.

Pulmonary valve replacement in adult patients with a severely dilated right ventricle and refractory arrhythmias after repair of tetralogy of fallot.

BACKGROUND: Refractory arrhythmias caused by right ventricular (RV) volume overload resulting from pulmonary regurgitation are the main concern in adult patients after repair of tetralogy of Fallot (TOF). Early pulmonary valve replacement (PVR) may prevent irreversible RV dysfunction and refractory arrhythmias, so the present study evaluated the PVR outcomes in adult patients with a severely dilated RV (d-RV) and refractory arrhythmias after TOF repair. METHODS AND RESULTS: Three TOF patients with a d-RV and tachyarrhythmias underwent PVR between the ages of 28 and 38 years. All had a d-RV (RV end-diastolic volume index (RVEDVI) >200 ml/m(2)) with a polymorphic nonsustained ventricular tachycardia (NSVT). Atrial tachycardia (AT) was identified in 2 patients and they underwent radiofrequency catheter ablation. The arrhythmias in all 3 were refractory to antiarrhythmic drugs. One year after PVR, the RVEDVI, left ventricular ejection fraction, right atrial pressure, cardiac index, cardiothoracic ratio, brain natriuretic peptide levels, and peak VO(2) improved in all, but without normalization. During the 2.6-3.8 year follow-up, all experienced tachyarrhythmias (NSVT or AT), which were controlled with medication. CONCLUSIONS: PVR may be beneficial for refractory arrhythmias, even in TOF patients with a d-RV, but it is difficult to completely normalize the hemodynamics and resolve the arrhythmogenicity.

Activation of cardiac progenitor cells through paracrine effects of mesenchymal stem cells.

Mesenchymal stem cells (MSC) transplantation has been proved to be promising strategy to treat the failing heart. The effect of MSC transplantation is thought to be mediated mainly in a paracrine manner. Recent reports have suggested that cardiac progenitor cells (CPC) reside in the heart. In this study, we investigated whether MSC had paracrine effects on CPC in vitro. CPC were isolated from the neonatal rat heart using an explant method. MSC were isolated from the adult rat bone marrow. MSC-derived conditioned medium promoted proliferation of CPC and inhibited apoptosis of CPC induced by hypoxia and serum starvation. Chemotaxis chamber assay demonstrated that MSC-derived conditioned medium enhanced migration of CPC. Furthermore, MSC-derived conditioned medium upregulated expression of cardiomyocyte-related genes in CPC such as beta-myosin heavy chain (beta-MHC) and atrial natriuretic peptide (ANP). In conclusion, MSC-derived conditioned medium had protective effects on CPC and enhanced their migration and differentiation.

[Application of the Bayley scales of infant development as a developmental test for Japanese infants with congenital heart disease].

The Bayley Scales of Infant Development 2nd edition (BSID-II) is used worldwide as a test for assessing development in infants with congenital heart disease (CHD); however, a Japanese version of these tests is not available. To introduce BSID-II in Japan, it was first used for 1-year-old Japanese infants with and without CHD (controls). The BSID-II scores of the control group were lower than American standards. In addition, the scores of the infants with CHD were lower than those of the controls, particularly the scores for motor development. Tsumori's Mental Development Test scores for the two groups were comparable; however, a diverse correlation was observed between BSID-II and Tsumori's Mental Development Test. No problems were encountered during the translation and use of BSID-II. These results suggest that BSID-II is useful for the assessment of development in Japanese infants.

Conservatively treated coronary malperfusion associated with acute type A dissection before aortic root replacement.

Coronary malperfusion associated with aortic dissection usually requires aggressive surgical treatment or catheter revascularization. Here, we report a case of conservatively treated coronary malperfusion associated with acute type A dissection before aortic root replacement. An 81-year-old woman was rushed to our hospital in a state of circulatory shock after developing chest pain. She was severely hypotensive on admission, and the electrocardiogram (ECG) revealed anterior and lateral ST elevation. However, the initial fluid resuscitation increased her blood pressure to a normal level, and the ischemic ECG changes disappeared in about 20 min. ECG-gated cardiac multidetector computed tomography showed a type A aortic dissection complicated with left main trunk dissection. A primary entry tear was located 5 mm below the left coronary ostium. The patient successfully underwent composite graft replacement of the aortic root in a stable hemodynamic condition. .

Abdominal aortic aneurysm with periaortic malignant lymphoma differentiated from aneurysmal rupture by clinical presentation and magnetic resonance imaging.

Abdominal aortic aneurysm (AAA) associated with periaortic malignant lymphoma is difficult to differentiate from aneurysmal rupture because of similarities in their clinical presentation and appearance on computed tomography images. We here report a case of AAA associated with periaortic malignant lymphoma diagnosed preoperatively with an absence of typical symptoms, showing that AAA in periaortic malignant lymphoma can present without any clinical correlates. Magnetic resonance imaging was used to confirm the diagnosis. The patient was treated by endovascular repair, which may be safer and more effective than open surgery for AAA associated with malignant lymphoma because of the tight adhesion between the aneurysm and the lymphoid tissue.

Long-term patency of internal thoracic artery grafts for coronary artery stenosis due to Kawasaki disease: comparison of early with recent results in small children.

BACKGROUND: The results of coronary artery bypass grafting using the internal thoracic artery (ITA) had been reported less satisfactory in patients < 12 years old with coronary artery lesions caused by Kawasaki disease. METHODS: Since 1983, 67 patients have undergone this operation in our hospital; their age at operation ranged from 1 to 59 years (median 11 years), and the total number of ITA grafts was 95. The interval from operation to latest graft patency as confirmed by angiogram or echocardiogram ranged from 4 months to 23 years (median 8 years). For analysis of graft patency rates, patients were divided into 4 groups based on year of coronary artery bypass grafting and age when grafted. The groups based on year were from 1983 to 1993 (early) and from 1994 to 2006 (later), whereas the age groups were age at operation < 12 years and age at operation > or = 12 years. From 1999, percutaneous transluminal balloon angioplasty was performed for postoperative anastomotic stenosis. RESULTS: Percutaneous transluminal balloon angioplasty for anastomotic stenosis in ITA graft was performed in 6 patients. When the age at operation was < 12 years, the 10-year patency rate in the later period was 94.4% (n = 18), significantly > the 70.0% (n = 30) seen in the earlier period (P < .05). CONCLUSION: Recent results of ITA grafts in patients < 12 years old have improved through the application of appropriate indications and percutaneous transluminal balloon angioplasty for anastomotic stenosis. Once good flow in the ITA 1 year after surgery is confirmed, graft patency will persist > 20 years.

Comparison of late post-operative cardiopulmonary responses in the Fontan versus ventricular septation for double-inlet left ventricular repair.

Ventricular septation (VS) and the Fontan procedure are alternatives for definitive repair in patients with double-inlet left ventricle; although VS is theoretically preferable, the current preference in practice is the Fontan procedure. However, the long-term outcomes of both procedures remain unclear. To address this issue, cardiopulmonary responses during exercise were measured in patients with double-inlet left ventricle, and the impact of the type of procedure performed, Fontan or VS, on long-term exercise capacity and late postoperative clinical profiles was assessed. Fourteen post-Fontan patients (mean age 17+/-6 years) and 13 VS patients (mean age 19+/-4 years) underwent exercise testing. Of the 13 VS patients, 5 required atrioventricular valve replacement (AVVR), and 7 required pacemaker implantation. Although no difference in peak oxygen uptake was found between the VS and Fontan patients, peak oxygen uptake was higher in VS patients without AVVR (30+/-8 ml/kg/min) than in VS patients with AVVR (19+/-1 ml/kg/min) and Fontan patients (22+/-6 ml/kg/min) (p<0.01). There was no significant difference in peak oxygen uptake between the VS patients with and without pacemaker implantation (p=0.09). The clinical profiles of the VS and Fontan patients were similar in terms of medication and freedom from tachyarrhythmias or reoperations during the follow-up period. In conclusion, the data suggest that VS without AVVR provides excellent future exercise capacity in selected patients with double-inlet left ventricle.

Possible role of adrenomedullin in the regulation of Fontan circulation: mature form of plasma adrenomedullin is extracted in the lung in patients with Fontan procedure.

OBJECTIVE: We investigated the pathophysiological significance of molecular forms of adrenomedullin (AM) in patients after the Fontan procedure. METHODS: Plasma concentrations of mature AM (AM-m), an active form, glycine-extended AM (AM-Gly), an inactive form, and total AM (AM-T: AM-m+AM-Gly) were measured by specific immunoradiometric assay in the femoral vein, pulmonary artery and femoral artery of 29 consecutive patients after the Fontan procedure. The eleven patients who had history of Kawasaki disease and have normal coronary and hemodynamics served as control. RESULTS: Patients who underwent Fontan procedure had significantly higher venous concentrations of AM-T, AM-Gly, and AM-m than age-matched normal controls (AM-T, 12.0+/-3.3 vs. 9.6+/-2.0; AM-Gly, 10.4+/-3.0 vs. 8.5+/-1.6; AM-m, 1.6+/-0.7 vs. 1.0+/-0.6 pmol/l, each p<0.05). In patients with Fontan procedure, there were no differences in plasma AM-T, AM-Gly or AM-m levels between the femoral vein and pulmonary artery, however, there was a significant step-down in the AM-m levels, but not in plasma AM-T or AM-Gly levels, between the pulmonary artery and femoral artery (1.3+/-0.6 to 1.0+/-0.6, p<0.05). The venous concentrations of AM-m correlated negatively with systemic blood flow (cardiac output) (r=-0.46, p<0.05). CONCLUSIONS: Results suggest that in Fontan circulation plasma AM-m is increased in parallel with those of AM-T and AM-Gly and that AM-m is extracted in the lung. Extracted AM-m may be involved in the regulation of pulmonary arterial tonus, although further studies are necessary to elucidate the exact role of AM in Fontan circulation.