Evaluating the response to cardiac resynchronization therapy performed with a new ventricular morphology-based strategy for congenital heart disease.

BACKGROUND: In cardiac resynchronization therapy (CRT) for patients with congenital heart disease (CHD) and a ventricular morphology other than a systemic left ventricle (LV), we previously proposed pacing sites that are different from those used for a systemic LV. The leads should be placed laterally on opposite sides of both ventricles in patients with short-axis dyssynchrony and a single ventricular physiology with two ventricles, whereas they should be placed at the farthest sites along the longitudinal direction in the right ventricle (RV) in patients with long-axis dyssynchrony of the RV. Moreover, in patients with interventricular dyssynchrony and a biventricular physiology with a systemic RV, they should be placed at sites that both ventricles can contract simultaneously. We retrospectively investigated 27 consecutive procedures in 24 patients with CHD who underwent CRT to evaluate the effectiveness of a new ventricular morphology-based CRT strategy. The responder rate was 63% (17/27). The reasons for a non-response to CRT in 10 cases were as follows: non-optimal lead positions during CRT, 4; no systemic ventricular conduction delay or heart failure symptoms before the CRT, 5; short follow-up periods after the CRT, 2; and an extremely dilated systemic RV, 1. The responder rate became 88% (14/16), after excluding the procedures without a ventricular conduction delay or heart failure symptoms and those with non-optimal lead positions. This new strategy for CRT can provide favorable results for CHD patients with a systemic ventricular conduction delay and heart failure.

Successful Truncal Valve Replacement After Truncal Valve Repairs.

The surgical management of severe truncal valvular dysfunction is still challenging in neonates with persistent truncus arteriosus. This report describes a 14-day-old neonate with severe truncal valve insufficiency successfully undergoing truncal valve repairs, and followed by valve replacement at the age of 4 years. The truncal valve was quadricuspid with two large and two small leaflets, and all leaflets had severe dysplastic and myxomatous changes. We performed leaflet extension and bicuspidization valvuloplasty for this valve. This patient obtained somatic growth for 4 years without heart failure symptoms, and safely underwent prosthetic valve replacement. This technique would be effective for truncal valve dysfunction in neonates as the life-saving and the bridging procedure to valve replacement.

Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries.

BACKGROUND: In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch. METHODS: Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock-Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years. RESULTS: There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I. CONCLUSION: Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Interventricular dyssynchrony in a patient with a biventricular physiology and a systemic right ventricle.

We describe of a unique pattern of cardiac dyssynchrony in a patient with a biventricular physiology and systemic right ventricle (RV): the interventricular dyssynchrony due to a contraction delay between the left ventricle and RV. In the present case, the cineangiography and intracardiac electrocardiography of the RV did not reveal intraventricular dyssynchrony of the RV, but revealed interventricular dyssynchrony. In addition, the pressure curves of the ventricles exhibited time phase differences between the two ventricles. The cardiac resynchronization therapy determining the pacing lead positions based on the idea of interventricular dyssynchrony induced reverse cardiac remodeling in this patient with systemic RV dysfunction.

Ductal stenting using side-branch cell dilation for aortic coarctation in high-risk patients with hypoplastic left heart syndrome.

For high-risk neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood operation, the strategy of bilateral pulmonary artery banding and ductal stenting is risky in case of coarctation of the aorta (CoA), often resulting in death. Therefore, we devised a new method of ductal stenting with side-branch cell dilation, which could overcome the constriction of the ductal arch with CoA in two HLHS patients. This is the first report that presents this method and the results. © 2015 Wiley Periodicals, Inc.

Optimal pacing sites for cardiac resynchronization therapy for patients with a systemic right ventricle with or without a rudimentary left ventricle.

AIMS: This study aims to assess the impact of pacing sites on the effectiveness of cardiac resynchronization therapy (CRT) in systemic right ventricle (sRV) patients with/without a rudimentary left ventricle (rLV). METHODS AND RESULTS: We evaluated 13 procedures in 11 sRV patients with a wide QRS (>150 ms). Based on the digitalization results of ventriculography, long-axis dyssynchrony (LD) was defined as extremely delayed right ventricular (RV) outflow tract movement: ≥100 ms delay from the RV apical contraction, and short-axis dyssynchrony (SD) was defined as a paradoxical contraction between the rLV and sRV caused by a conduction delay between the two ventricles. During the follow-up period (2.1 ± 1.9 years), the response rates were 71% (5/7) and 33% (2/6) in the sRV patients with and without an rLV, respectively (P = ns). Following the CRT, the QRS duration remained similar between the responders and nonresponders. Among five responders with an rLV, the leads were placed in the longitudinal RV direction in two with LD, longitudinal RV direction with fusion of the intrinsic QRS in two with LD + SD, and laterally on opposite sides of both ventricles in one with SD. Among two responders without an rLV, the leads were placed in the longitudinal RV direction in those two with LD. CONCLUSIONS: In sRV patients with LD with/without an rLV, the leads should be placed at furthest sites in the longitudinal RV direction. In patients with an rLV and SD, the leads should be placed laterally on opposite sides of both ventricles.

High Incidence of Dilated Cardiomyopathy After Right Ventricular Inlet Pacing in Patients With Congenital Complete Atrioventricular Block.

BACKGROUND: Some patients with congenital complete atrioventricular block (CCAVB) develop dilated cardiomyopathy (DCM) after pacemaker implantation (PMI). We evaluated the relationship between pacing site and DCM incidence. METHODS AND RESULTS: We retrospectively evaluated 38 patients with CCAVB; 8 (25%) of 32 patients who had PMI developed DCM/heart failure death (HFD) after PMI, although none of the 6 patients without PMI showed DCM/HFD. All DCM/HFD occurred within 50 months of PMI. Among the 32 patients with PMI, the DCM/HFD incidence was 55% (6/11) for right ventricular inlet (RVI), 18% (2/11) for RV apex (RVA), and 0% for left ventricle (LV) (P=0.013). At the endpoint, the LV ejection fraction and septal-to-posterior wall motion delay of patients with LV pacing were better than those for patients with other pacing sites. Among the 8 DCM/HFD patients, 2 in whom the pacing site was changed from RVI to LV apex or in whom therapy was upgraded to cardiac resynchronization remained alive with no heart failure symptoms, whereas the other 6 died of heart failure. CONCLUSIONS: A total of 25% of the patients who underwent PMI because of CCAVB, but none in the non-PMI group, developed DCM/HFD. DCM/HFD incidence was higher in patients with RVI pacing. Ventricular dyssynchrony related to pacing site may be one cause of DCM in patients with CCAVB. (Circ J 2016; 80: 1251-1258).

Early Single Institutional Experience of Berlin Heart EXCOR® Pediatric Ventricular Assist Device in Japan.

BACKGROUND: Since August 2015, the pediatric ventricular assist device (VAD), Berlin Heart EXCOR®, has been accepted for use in Japan.Methods and Results:Between August 2015 and July 2016, 4 pediatric patients with endstage heart failure underwent LVAD implantation with the EXCOR®device. The median age and body weight at operation were 8 months and 4.8 kg. During a median follow-up of 7.3 months (range, 5.0-10.3), all patients survived. Two patients went on to heart transplantation and the remaining 2 are on a waiting list with stable hemodynamics. CONCLUSIONS: The early outcomes of the Berlin Heart EXCOR®pediatric VAD were satisfactory. (Circ J 2016; 80: 2552-2554).

Steroids Improve Hemodynamics in Infants With Adrenal Insufficiency After Cardiac Surgery.

OBJECTIVE: To investigate whether steroid replacement therapy improved hemodynamics in infants after surgery for congenital heart disease only when they develop adrenal insufficiency. The authors retrospectively investigated adrenal function and evaluated hemodynamic responses to steroid replacement therapy in infants after surgery for congenital heart disease. DESIGN: Retrospective, cohort study. SETTING: Intensive care unit in the National Cerebral and Cardiovascular Center Hospital in Japan. PATIENTS: Thirty-two neonates and infants<3 months old who underwent cardiovascular surgery. INTERVENTIONS: The patients were divided into 2 groups based on corticotropin stimulation test results: group AI with adrenal insufficiency (baseline cortisol<15 µg/dL or incremental increase after testing of<9 µg/dL, with baseline cortisol of 15-34 µg/dL); and group N with normal adrenal function. The corticotropin stimulation test was performed by injecting 3.5 µg/kg of tetracosactide acetate. Hydrocortisone (1 mg/kg) was administered every 6 hours, and hemodynamics were compared before and after steroid administration between the groups. MEASUREMENTS AND MAIN RESULTS: Seven patients were classified into group AI, and demonstrated a mean blood pressure increase from 53±8 mmHg before treatment to 68±9 mmHg 18 hours after steroid administration (p<0.01). Urine output also increased, from 2.7±1.0 mL/kg/h to 4.8±1.9 mL/kg/h (p<0.05). In group N, neither mean blood pressure nor urine output increased after steroid administration. CONCLUSIONS: After surgery for congenital heart disease, one-fifth of infants developed adrenal insufficiency. Steroid replacement therapy improved hemodynamics only in the subgroup with adrenal insufficiency.

Efficacy of "Awake ECMO" for critical respiratory failure after pediatric open-heart surgery.

A 4-year-old boy with atrioventricular discordance, double-outlet right ventricle, pulmonary stenosis, and mitral regurgitation, was undergoing anatomical repair consisting of Senning, Rastelli, Damus-Kaye-Stansel procedures, and a mitral valve repair, complained of post-operative excessive airway tract secretion, which ultimately developed into acute respiratory distress syndrome (ARDS) 28 days after the operation. The cause of the ARDS was thought to be frequent manual positive pressure recruitment and prolonged inhalation of pure oxygen. At 45 days after the operation, hypercapnia and respiratory acidosis turned out to be irreversible, and therefore, veno-arterial extracorporeal membrane oxygenation (ECMO) was established utilizing the Endumo(®)4000 system. Pulmonic interstitial inflammation gradually improved while resting the lung under ECMO support; however, effective ventilation volume decreased critically because a massive pulmonary hemorrhage occurred at 2 and 9 days after the initiation of ECMO. To maximize the effectiveness of respiratory physical therapy, "Awake ECMO" was started and tidal volume dramatically increased with a regained cough reflex. Five days later, he was successfully weaned off from ECMO, and discharged 7 months after the operation without any neurological and physiological sequelae.

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.

Clinical Features and Surgical Outcomes of Coronary Sinus Orifice Atresia.

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

Acute rupture of chordae tendineae of the mitral valve in infants: a nationwide survey in Japan exploring a new syndrome.

BACKGROUND: Recently, infant cases of acute heart failure attributable to rupture of the mitral chordae tendineae have been reported. However, little is known about the pathogenesis and clinical course of this condition. METHODS AND RESULTS: Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. The clinical manifestations, management strategies, and prognosis were investigated. Eighty-one (85%) patients were between 4 and 6 months (median, 5 months) of age. In 63 (66%) patients, rupture occurred during the spring or summer. The underlying conditions before rupture included Kawasaki disease (10 cases), maternally derived anti-SSA antibodies (2 cases), and infective endocarditis (1 case). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations of the mitral valves and chordae (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%) and increased fibrous and myxoid tissue in 11 cases (39%), suggesting that nonbacterial infectious or autoimmune endocarditis and myxoid changes are involved in the pathogenesis. Eight patients (8.4%) died before (n=6) and shortly after (n=2) the operation, and significant neurological complications persisted in 10 cases (11%). CONCLUSIONS: Acute heart failure attributable to rupture of the mitral chordae tendineae in infants is a unique disease resulting from diverse causes. This condition should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.

Efficacy and safety of percutaneous transluminal balloon dilation to prevent progression of banding site stenosis after bilateral pulmonary artery banding.

OBJECTIVES: To investigate the efficacy and safety of percutaneous transluminal balloon dilation (PTBD) for the treatment of bilateral pulmonary artery banding (bil-PAB) site stenosis. BACKGROUND: Although bil-PAB is an alternative initial treatment for high-risk neonates with hypoplastic left heart syndrome (HLHS) or critical aortic stenosis (cAS), those patients often suffer from desaturation because of progressive stenosis of the bil-PAB sites during the interstage period. METHODS: We retrospectively evaluated the efficacy and safety of 11 consecutive PTBD procedures performed between 2006 and 2012 to treat bil-PAB site stenosis in four high-risk infants (three females) with HLHS or cAS. RESULTS: PTBD was repeated twice in two patients and three times in one patient over intervals. The mean balloon diameter (BD) and BD-to-band circumference (BC) ratio were 3.1 ± 0.5 mm and 0.31 ± 0.06, respectively. After the procedures, the mean minimum lumen diameter was dilated significantly from 1.1 ± 0.1 mm to 1.7 ± 0.3 mm (P < 0.01), and the mean peripheral oxygen saturation increased significantly from 75 ± 8% to 85 ± 4% (P < 0.01). All patients reached the next stage operation involving the Norwood & bidirectional Glenn or Ross procedure, after growth. No complications such as band rupture occurred. CONCLUSIONS: For progressive stenosis of bil-PAB sites, PTBD using a balloon size that did not exceed the BC (BD around 30% of the BC) was an effective and safe procedure.

Cardiac resynchronization therapy for various systemic ventricular morphologies in patients with congenital heart disease.

BACKGROUND: Cardiac resynchronization therapy (CRT) can result in functional improvement and reduced mortality in patients with medically refractory heart failure. Although CRT is reportedly effective in patients with congenital heart disease (CHD), it is still controversial in patients who have systemic right ventricle. METHODS AND RESULTS: Twenty CHD patients treated with CRT since 2006 were divided into 3 groups based on systemic ventricular (sysV) morphology (7 with left ventricle [sLV], 7 with right ventricle [sRV], and 6 with unbalanced 2 ventricles as a single-ventricular physiology [sBV]). The acute effects of CRT on hemodynamics and sysV function before device implantation was retrospectively evaluated and the chronic (≥6 months) effects of CRT on late outcomes was assessed. In our CHD populations, sysV volume index was reduced from 139±41 to 118±33 ml/m(2)(P=0.04) after CRT, and there was significant improvement in B-type natriuretic peptide levels (from 341±384 to 160±152 pg/ml, P=0.01) and New York Heart Association (NYHA) functional class (from 2.1±0.6 to 1.8±0.7, P=0.02) on a late outcome. The sRV group did not show a late sysV volume reduction despite significant QRS shortening, and an increase of sysV peak dP/dt in the acute study differed from that of other groups. CONCLUSIONS: CRT improves late hemodynamic and functional status in sLV and sBV CHD patients with a dyssynchronized sysV. However, an acute CRT effect cannot guarantee long-term benefit in sRV patients.

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.

Suspected cold agglutination during mild hypothermic pediatric open heart surgery: a report of two cases.

Cold agglutination was suspected in 2 pediatric open heart surgery cases during mild hypothermic cardiopulmonary bypass. The first patient was a 2-year-old boy with secundum atrial septal defect. Fifteen minutes after the initiation of mild hypothermic cardiopulmonary bypass, the inlet pressure of oxygenator suddenly elevated from 250 to over 500 mmHg, whereas outlet pressure was maintained. The blood flow rate decreased from 140 to 85 ml/kg/min. At that time, the arterial blood temperature was less than 32°C. Cold agglutinin was highly suspected, so patient was immediately warmed, and the inlet pressure of oxygenator decreased to 250 mmHg when the arterial blood temperature reached to 36°C. Second patient was a 3-year-old boy with secondary developed subvalvular pulmonary stenosis after the repair of double chambered right ventricle at 10 months of his age. Eighteen minutes after the initiation of mild hypothermic cardiopulmonary bypass, the inflow pressure suddenly elevated to 500 mmHg and transmission flow decreased to 55 ml/kg/min. Twenty-three minutes after warming, the pressure fell to a normal level and transmission flow was recovered. The operation continued with normo-thermic cardiopulmonary bypass and crystalloid cardioplegia. Both cases had no postoperative complications related to cold agglutinin such as myocardial infarction, cerebral infarction, or renal insufficiency.

Intrinsic obstruction in pulmonary venous drainage pathway is associated with poor surgical outcomes in patients with total anomalous pulmonary venous connection.

The aim of this study was to review the surgical outcomes of conventional repair in patients with total anomalous pulmonary venous connection (TAPVC). Between 1999 and 2012, 46 patients underwent conventional TAPVC repair; of those, 14 required emergent surgery within the first 24 h after their birth for coexisting intrinsic obstruction in pulmonary venous drainage pathway (Group 1). The remaining 32 were operated on after the first day of life and were divided into two groups: with (Group 2, n = 10) and without (Group 3, n = 22) subsequently progressed pulmonary venous obstruction (PVO) after birth. A follow-up was completed on all patients, and the mean follow-up period was 7 ± 4 years (range 0.9-14). Group 1 required a significantly prolonged nitric oxide inhalation (12 ± 11 days) and mechanical ventilation support (29 ± 36 days) compared to Group 2 and Group 3. The actuarial survival rate at 10 years was 69% in Group 1, 88% in Group 2, and 96% in Group 3 (Group 1 vs. Group 3: p = 0.05). Freedom from postoperative pulmonary vein stenosis at 10 years was 39% in Group 1, 70% in Group 2, and 86% in Group 3 (Group 1 vs. Group 3: p = 0.002). However, all the ten survivors in Group 1 showed an NYHA functional status of I or II, and 60% of survivors were free from medication. Outcomes of patients with TAPVC requiring surgical repair at the day of birth for coexisting intrinsic PVO were still dismal; however, the acceptable status of current survivors has encouraged us to treat this challenging group surgically.

The importance of hybrid stage I palliation for neonates with critical aortic stenosis and reduced left ventricular function.

The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment. A follow-up was completed on all patients and the median follow-up period was 3.3 years (max 16.0). The overall survival rate at 15 years was 67.1 %. Six of the seven patients with maintained LV function could go on to the definitive Ross or Konno-aortic valve replacement at the median duration of 311 days after initial BAV, without any mortality. Three of four patients with reduced LV function died before 2010 with conventional treatment. With use of a hybrid stage I palliation, one of two patients ultimately underwent Fontan completion at 38 months of age and the other successfully underwent the definitive Ross-Konno operation at 9 months of age after recovery of the LV function. Although a statistically significant improvement has not been observed yet, the application of hybrid stage I palliation following BAV would be a favorable alternative for patients with reduced LV function to avoid a high-risk neonatal Ross or Norwood-type operation, and also to determine further treatment carefully.

Myers' 3-sinus reconstruction for supravalvular aortic stenosis involving left coronary ostium: report of a case.

A 1-year-old boy with a bicuspid aortic valve, who had undergone successful repair of coarctation of the aorta by extended end-to-end direct anastomosis at the age of 1 month, was found to have mild supravalvular aortic stenosis involving the left coronary ostium. Because he was so young, we performed a modified Myers' all-autologous 3-sinus reconstruction to allow for potential growth. After transecting the ascending aorta just above the sinotubular junction, the superior wall of the left coronary ostium and the aortic root between both commissures were incised longitudinally, and then each of the incised parts was augmented by creating three flaps of distal aortic wall directly. Postoperatively, myocardial scintigram confirmed resolution of the pressure gradient at the supravalvular stenotic portion and improvement of the perfusion defect in the septal-anterior area.