[Bentall Operation for Pregnant Women with Marfan's Syndrome].

A 35-years-old pregnant woman with Marfan's syndrome visited the emergent department. She had sudden severe back pain. She was at the 20th week of gestation. An emergent chest computed tomo-graphy scan showed Stanford type B acute aortic dissection. After admission, strict blood pressure control was started. According to aortic valve insufficiency and fluid retention with pregnancy, acute heart and respiratory failure was getting worse. It seemed risky for both mother and the fetus to continue pregnancy. After sincere and detailed discussion between the patient and our multidisciplinary medical team, the patient decided to continue pregnancy. An urgent Bentall operation was performed. A careful attention was paid for the fetus during and after the surgery. Strict blood pressure control was also continued. The mother and the 30-week-gestation newborn recovered uneventfully. During four years of follow-up, thoracic and thoraco-abdominal aortic replacement was performed. The patient survived all of these procedures without any complication.

[Hybrid Treatment for Thoracoabdominal Aortic Aneurysm by Combined Graft Replacement and Stent-graft].

Graft replacement for thoracoabdominal aortic aneurysm(TAAA) is still an important technique, yet it has high risks of mortality, spinal cord ischemia, and pulmonary complications. In our hospital, thoracoabdominal aneurysm repair with grafting and endovascular treatment (TARGET) method was performed in patients with severe chronic obstructive pulmonary disease( COPD), severe pulmonary adhesions after descending aortic replacement, or those considered high risk from general condition to undergo a wide range replacement. In this method, thoracoabdominal aortic replacement near the diaphragm was followed by stent graft treatment of the residual proximal or distal lesions. Here the usefulness of this technique was reported.

Intracoronary autologous cardiac progenitor cell transfer in patients with hypoplastic left heart syndrome: the TICAP prospective phase 1 controlled trial.

RATIONALE: Hypoplastic left heart syndrome (HLHS) remains a lethal congenital cardiac defect. Recent studies have suggested that intracoronary administration of autologous cardiosphere-derived cells (CDCs) may improve ventricular function. OBJECTIVE: The aim of this study was to test whether intracoronary delivery of CDCs is feasible and safe in patients with hypoplastic left heart syndrome. METHODS AND RESULTS: Between January 5, 2011, and January 16, 2012, 14 patients (1.8±1.5 years) were prospectively assigned to receive intracoronary infusion of autologous CDCs 33.4±8.1 days after staged procedures (n=7), followed by 7 controls with standard palliation alone. The primary end point was to assess the safety, and the secondary end point included the preliminary efficacy to verify the right ventricular ejection fraction improvements between baseline and 3 months. Manufacturing and intracoronary delivery of CDCs were feasible, and no serious adverse events were reported within the 18-month follow-up. Patients treated with CDCs showed right ventricular ejection fraction improvement from baseline to 3-month follow-up (46.9%±4.6% to 52.1%±2.4%; P=0.008). Compared with controls at 18 months, cardiac MRI analysis of CDC-treated patients showed a higher right ventricular ejection fraction (31.5%±6.8% versus 40.4%±7.6%; P=0.049), improved somatic growth (P=0.0005), reduced heart failure status (P=0.003), and lower incidence of coil occlusion for collaterals (P=0.007). CONCLUSIONS: Intracoronary infusion of autologous CDCs seems to be feasible and safe in children with hypoplastic left heart syndrome after staged surgery. Large phase 2 trials are warranted to examine the potential effects of cardiac function improvements and the long-term benefits of clinical outcomes. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01273857.

Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle.

Heterotaxy syndrome is a mortality risk factor for patients with complex heart disease. These patients often have common atrioventricular valve (CAVV) morphology and significant atrioventricular valve regurgitation - yet another mortality risk factor in congenital heart disease. Thus, patients with both heterotaxy syndrome and atrioventricular valve regurgitation are at highest risk of mortality. A large number of patients with heterotaxy syndrome have CAVV and a functional single ventricle. These patients are more difficult to operate than patients with biventricular morphology because in the former, the CAVV is often less adapted to systemic afterload than the mitral valve. Herein, we present the results of atrioventricular valve repair surgeries performed at the Okayama University Hospital on patients with heterotaxy syndrome, atrial isomerism, and a functional single ventricle. In addition, we review the current literature in this typically challenging patient population. In-depth understanding of reasonable management strategies and resolution of technical issues will help guide surgeons during this procedure.

[Cardiac papillary fibroelastoma on the Chiari's network of a 3-month-old baby; report of a case].

A 3-month-old boy was transferred to our hospital because of a cardiac tumor in the right heart, which had been prenatally diagnosed by fetal echocardiography. During the operation, the tumor was found on the Chiari's network in the right atrium. The tumor was excised and the ventricular septal defect was repaired. Postoperative histological examination revealed that the tumor was a papillary fibroelastoma. He was transferred to a pediatric unit for postoperative care.

Anatomic Risk Factors for Reintervention After Arterial Switch Operation for Taussig-Bing Anomaly.

BACKGROUND: This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the arterial switch operation. METHODS: This retrospective study included 34 patients with TBA who underwent the arterial switch operation between 1993 and 2018. Preoperative anatomic and physiologic differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries with ventricular septal defect and TBA with an anterior and rightward aorta. RESULTS: At arterial switch operation, median age was 43 days (range, 16 to 102) and median body weight was 3.6 kg (range, 2.8 to 3.8 kg). Aortic arch obstruction and coronary anomalies were present in 64% and 41% of patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and the late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery to aortic annulus size ratio than patients not receiving reintervention (1.69 vs 1.41, P = .02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the transposition of great arteries/ventricular septal defect group. The PS-related reintervention was required more in the TBA group than in the transposition of great arteries/ventricular septal defect group. CONCLUSIONS: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomic relationship during the arterial switch operation.

The impact of pulmonary artery banding and cardiac resynchronization therapy for the adult patient with congenitally corrected transposition of the great arteries.

It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Cardiopulmonary exercise testing showed that the predictive value of anaerobic threshold and peak oxygen uptake improved from 65 to 99% and 59 to 92%, respectively. In conclusion, pulmonary artery banding and cardiac resynchronization therapy can be the first choice of surgery for severe tricuspid regurgitation with severely impaired systemic right ventricular function. After improving systemic right ventricular dysfunction tricuspid surgery could be the next choice as a surgical intervention in the future.

Acute mitral valve endocarditis at the 24th gestational week.

Infective endocarditis during pregnancy and subsequent cardiac surgery are rare and carry a high mortality risk for both the mother and fetus. We report our experience with a previously healthy, 22-year-old woman affected by acute active mitral endocarditis due to Streptococcus gordonii at the 24th gestational week, who wished to continue with the pregnancy. Due to cardiogenic shock, an intra-aortic balloon pump was inserted. Our patient successfully underwent mitral valve replacement with normothermic high-flow cardiopulmonary bypass and continuous intraoperative fetus monitoring. She delivered a 2524-g baby vaginally at the 38th gestational week. Both the mother and child were confirmed to be doing well at the 1-year follow-up. Although this was the first case, urgent cardiac surgery and a subsequent childbirth went well by prompt decision of each department.

Spontaneous ascending aortic rupture in a pregnant woman with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that affects 1 in 3000 individuals. Vascular involvement in NF-1 is a well-recognized, but rare, feature of this disease. In pregnant women, the risk of aortic dissection or rupture is elevated during pregnancy and the postpartum period. We report a pregnant woman who had a history of NF-1 with a spontaneous ascending aortic rupture. This rupture was successfully treated by emergent surgery. The mother and the 28-week-gestation newborn recovered uneventfully. During 7 years of follow-up, aorta of the patient shows no significant change. A review of the literature regarding the pathogenesis of this condition is also presented.

Repair of Ebstein's anomaly in neonates and small infants: impact of right ventricular exclusion and its indications.

OBJECTIVES: In cases of severe Ebstein's anomaly, it is essential to determine whether biventricular repair (BVR) or single-ventricle palliation is feasible. Since 1999, in our institution, we have used the novel technique comprising tricuspid valve (TV) closure and right ventricular and right atrial (RV/RA) exclusion to reduce the deleterious effects of an enlarged RV in patients with severe Ebstein's anomaly. However, in cases with good RV function, primary BVR is performed. In the present study, we describe our surgical strategy in the treatment of severely symptomatic neonates with Ebstein's anomaly. METHODS: From June 1999 to October 2011, 12 neonates with a severely symptomatic Ebstein's anomaly underwent surgical repair. The mean age at the first operation was 29 ± 25 (range, 5-92) days; and the mean body weight was 2.8 ± 0.5 (range, 2.0-4.1) kg. The associated anomalies included pulmonary atresia with an intact ventricular septum in 7, critical pulmonary stenosis in 1, ventricular septal defect in 3 and coarctation of the aorta in 1 patient. The mean cardio-thoracic ratio (CTR) was 80 ± 14% (range, 57-98%). Preoperatively, 9 patients had grade IV tricuspid regurgitation (TR), as detected by echocardiography, and 6 required ventilator support. RESULTS: Five patients underwent primary BVR. Seven patients underwent staged palliation using a modified Blalock-Taussig shunt (BT shunt) with/without RV/RA exclusion. There was 1 case each of hospital death and late death. The median follow-up duration in the present study was 6.5 years. Among the 8 patients who underwent TV repair, postoperative TR was trivial or mild in 6 patients, moderate in 1 and absent in 1. After surgery, the mean CTR and serum B-type natriuretic peptide levels decreased to 59 ± 14% (range, 45-70%) and 46 ± 28 (range, 12-83) pg/dl, respectively. CONCLUSIONS: Critically ill neonates with Ebstein's anomaly can be successfully treated using RV/RA exclusion combined with a modified BT shunt in cases where RV function is poor. However, in cases of good RV function, we recommend the use of primary BVR.

The effect of pulmonary root translocation on the left ventricular outflow tract.

We report the effect of pulmonary root translocation on the left ventricular outflow tract. A double switch operation with pulmonary root translocation was performed in a 6-year-old boy whose diagnosis was dextrocardia, congenitally corrected transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. Postoperative magnetic resonance imaging showed more natural left ventricular outflow than preoperatively (19 mm vs 22 mm in length between the top of the interventricular septum and the aortic valve). This technique does not require coronary transfer and enables preservation of the aortic root structure. The long-term results, including left ventricular outflow tract morphology, should be evaluated.

Radial incision for ventricular septal defect with membranous septal aneurysm.

Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the septal tricuspid leaflet carries a risk of tricuspid valve dehiscence and conduction disturbances when the septal leaflet is detached along the tricuspid annulus. To avoid these problems, we use a radial incision to expose and close perimembranous ventricular septal defects. We reviewed recent cases to determine the risks and benefits of this technique. From January 2005 through September 2008, 30 patients underwent closure of a perimembranous ventricular septal defect through a right atrial approach at our institution. The operation included radial incision of the membranous septal aneurysm to improve visualization of the perimembranous ventricular septal defect in 9 patients. There was no perioperative or late death. The operative and postoperative courses were uneventful in all cases. A residual leak was detected in only one patient. No patient had more than mild postoperative tricuspid valve insufficiency, none underwent reoperation, and no new arrhythmia or conduction disturbance was detected during follow-up. The radial incision for closure of a ventricular septal defect with a membranous septal aneurysm provides satisfactory exposure of the defect through the right atriotomy, for safe and accurate closure.

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure.

We describe here successful palliative repair of tricuspid atresia, hypoplastic right ventricle, transposition of the great arteries, and hypoplastic aortic arch in a neonate. The repair consisted of the Norwood procedure with a rudimentary right ventricle to pulmonary artery shunt, which was located on the right side of a neo-aorta. This procedure could be a useful adjunct to avoid left ventriculotomy and its subsequent dysfunction.

Effect of inhibition of glycogen synthase kinase-3 on cardiac hypertrophy during acute pressure overload.

OBJECTIVE: A large number of diverse signaling molecules in cell and animal models participate in the stimulus-response pathway through which the hypertrophic growth of the myocardium is controlled. However, the mechanisms of signaling pathway including the influence of lithium, which is known as an inhibitor of glycogen synthase kinase-3beta, in pressure overload hypertrophy remain unclear. The aim of our study was to determine whether glycogen synthase kinase-3beta inhibition by lithium has acute effects on the myocyte growth mechanism in a pressure overload rat model. METHODS: First, we created a rat model of acute pressure overload cardiac hypertrophy by abdominal aortic banding. Protein expression time courses for beta-catenin, glycogen synthase kinase-3beta, and phosphoserine9-glycogen synthase kinase-3beta were then examined. The rats were divided into four groups: normal rats with or without lithium administration and pressure-overloaded rats with or without lithium administration. Two days after surgery, Western blot analysis of beta-catenin, echo-cardiographic evaluation, left ventricular (LV) weight, and LV atrial natriuretic peptide mRNA levels were evaluated. RESULTS: We observed an increase in the level of glycogen synthase kinase-3beta phosphorylation on Ser 9. A significant enhancement of LV heart weight (P < 0.05) and interventricular septum and posterior wall thickness (P < 0.05) with pressure-overloaded hypertrophy in animals treated with lithium were also observed. Atrial natriuretic peptide mRNA levels were significantly increased with pressure overload hypertrophy in animals treated with lithium. CONCLUSIONS: We have shown in an animal model that inhibition of glycogen synthase kinase-3beta by lithium has an additive effect on pressure overload cardiac hypertrophy.

Pulmonary autograft patch aortoplasty for reconstruction of an interrupted aortic arch associated with an aortopulmonary window.

We present here an infant with an aortopulmonary window associated with an interrupted aortic arch. Single-stage repair with a pulmonary autograft patch for augmentation of the interrupted aortic arch repair was performed. Transpulmonary patch closure was used to repair the aortopulmonary window. A computed tomography scan at the 1-month follow-up demonstrated a reconstructed aortic arch without obstruction, kinking, or any sign of bronchial or branch pulmonary artery compression.

Aortic relocation for transposition complex with aortic arch obstruction.

We describe successful primary repair of 2 cases of transposition complex with aortic arch obstruction. A new aortic arch was reconstructed by direct anastomosis between the well-mobilized ascending aorta and the descending aorta. The neoaortic root with transferred coronary arteries was subsequently anastomosed to the undersurface of this new aortic arch. This technique deals with the significant size discrepancy between the 2 great arteries, and anomalous coronary artery patterns.

A cell-permeable NFAT inhibitor peptide prevents pressure-overload cardiac hypertrophy.

The activation of the calcineurin-nuclear factor of activated T cells cascade during the development of pressure-overload cardiac hypertrophy has been previously reported in a number of studies. In addition, numerous pharmacological studies involving calcineurin inhibitors such as FK506 and cyclosporine A have now demonstrated that these agents can prevent such hypertrophic responses in the heart. However, little is known regarding the roles of the calcineurin downstream effector--nuclear factor of activated T cells. Our present study has further examined the roles of nuclear factor of activated T cells in pressure-overload cardiac hypertrophy by employing a recently developed cell-permeable nuclear factor of activated T cells inhibitor peptide. Rat hearts were subjected to pressure overload attributable by 4 weeks of aortic banding, and then treated with this cell-permeable nuclear factor of activated T cells inhibitor peptide and a control peptide. Treatment with the inhibitor was found to significantly decrease the heart weight/body weight ratio, the size of cardiac myocytes, and the serum brain natriuretic peptide and atrial natriuretic peptide levels. These results suggest that nuclear factor of activated T cells functions in a key role in the development of cardiac hypertrophy during pressure overload. Inhibition of nuclear factor of activated T cells by a specific inhibitor peptide is a suitable method for characterization of the molecular mechanisms underlying cardiac hypertrophy as well as in the search for new promising therapies for disease.

Discrepancy between intraoperative transesophageal echocardiography and postoperative transthoracic echocardiography in assessing congenital valve surgery.

BACKGROUND: The purpose of this study was to investigate the discrepancy between intraoperative transesophageal and postoperative transthoracic echocardiography in assessing residual regurgitation in children undergoing valve repair. METHODS: Forty-two consecutive children (median age, 5.1 years) who underwent valve repair for valvar regurgitation from 2001 to 2004 were retrospectively analyzed. The patients were divided into two groups: atrioventricular valve group (n = 33) and aortic valve group (n = 9). Regurgitation grade, fractional shortening, and atrioventricular inflow velocity obtained by intraoperative transesophageal echocardiography were compared with those obtained by transthoracic echocardiography at discharge (median, 11 days) and at follow-up (median, 8 months). RESULTS: Intraoperative transesophageal echocardiography revealed specific residual lesions in 4 patients, leading to successful re-repair. Fractional shortening obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (p < 0.01). In the atrioventricular valve group, the regurgitation grade obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (0.7 +/- 0.8 versus 1.4 +/- 0.9; p < 0.01), and agreement between the two examinations was found in 12 patients (38%). Peak atrioventricular inflow velocity obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (p < 0.01). In the aortic valve group, there was no significant difference between the regurgitation grades in the two examinations (0.8 +/- 0.8 versus 1.1 +/- 0.9), and complete agreement in regurgitation grade was found in 5 (56%) of 9 patients. CONCLUSIONS: There were considerable discrepancies between the examinations in evaluation of residual atrioventricular valve regurgitation and potential atrioventricular valve stenosis: most of the residual regurgitations were underestimated by intraoperative transesophageal echocardiography. In contrast, reasonable agreement was found between the two examinations in evaluation of aortic valve regurgitation.