Yuko Takeda

Surgical Results of Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta Including Reoperation for Infrequent Complications

BACKGROUND We evaluated the results of surgery for an anomalous origin of the right pulmonary artery from the ascending aorta. METHODS From August 1986 to December 2005, 8 children (6 neonates) aged 7 to 180 days (mean, 35 +/- 59 days) with anomalous origin of the right pulmonary artery from the ascending aorta underwent surgical repair at our institute. All except one child, who had the distal form, had the proximal form. Cardiac catheterization showed that the left pulmonary artery to systemic pressure ratio was 1.0 or more. Surgery was performed by direct anastomosis in 7 patients and by graft interposition in 1. RESULTS There were no operative or late deaths. All patients postoperatively underwent cardiac catheterization that showed decreased left pulmonary artery to systemic pressure ratio ranging from 0.2 to 0.6. Follow-up periods ranged from 2 months to 13 years. We undertook reoperations for two infrequent postoperative causes. One patient exhibited significant supravalvar aortic stenosis and required patch enlargement of the ascending aorta 3 years after operation. The other patient (with the distal form) needed a reoperation after 1 month because of progressive stenosis at the anatomic site. Graft interposition was performed, and histopathologic examination showed that the tissue from the stenotic region looked like that of a ductus. CONCLUSIONS We undertook surgical repair for anomalous origin of the right pulmonary artery from the ascending aorta. Pulmonary hypertension was improved in all patients. Careful follow-up was necessary to detect supravalvar aortic and anastomotic stenosis early and late after operation.

Pulmonary Artery Banding for Functionally Single Ventricles: Impact of Tighter Banding in Staged Fontan Era

BACKGROUND In this study, we assessed our surgical strategy, tighter pulmonary artery banding (PAB) during the neonatal period, as an initial step followed by early application of bidirectional cavopulmonary shunts (BCPS) in infancy, to treat functionally single ventricles with unobstructed pulmonary blood flow. METHODS On the basis of our surgical strategy, 68 consecutive patients underwent PAB and were divided into two groups, group 1 (January 1990 to June 2003; n = 30) and group 2 (July 2003 to August 2008; n = 38). The median age at PAB was 45 days in group 1 and 9 days in group 2. The circumference of the bands was significantly shorter in group 2 than in group 1, corresponding to the patients weight in kg plus 19.0 +/- 0.6 mm in group 1 or 17.0 +/- 0.3 mm in group 2 (p = 0.003). RESULTS Cardiac catheterization before the right heart bypass operation showed that the pulmonary artery index (group 1, 322 +/- 29; group 2, 283 +/- 27 mm(2)/m(2); p = 0.01), pulmonary resistance index (group 1, 2.4 +/- 0.2; group 2, 1.9 +/- 0.1 U x m(2); p = 0.03), and ventricular end-diastolic volume (group 1, 212 +/- 19%; group 2, 166 +/- 9%; p = 0.04) were significantly different between the two groups. The rates for achievement of right heart bypass at 12 months (group 1, 19%; group 2, 81%; p < 0.01) and survival at 3 years (group 1, 70%; group 2, 87%; p = 0.04) were significantly higher in group 2 than in group 1. CONCLUSIONS Our present strategy could prevent volume overload and improve the achievement and survival rates of right heart bypass operations.

Rapid Two-Stage Starnes Procedure for a Symptomatic Neonate With Ebstein Anomaly

We present a rapid two-stage Starnes procedure for a seriously symptomatic neonate with the prenatal diagnosis of Ebstein anomaly. At 16 hours after birth, we performed an emergency operation consisting of main pulmonary artery ligation, plication of the right atrial and right ventricular wall, modified Blalock-Taussig shunt, and patent ductus arteriosus ligation, without cardiopulmonary bypass. At age 12 days, we then performed the Starnes procedure using a glutaraldehyde-treated autologous pericardial patch with a 4-mm fenestration to close the tricuspid valve orifice. The infants postoperative course was excellent. A rapid two-stage Starnes procedure is useful for treating a seriously symptomatic neonate with Ebstein anomaly.

Extracorporeal life support after cardiac surgery in children: outcomes from a single institution.

Extracorporeal life support (ECLS) is used after congenital heart surgery for several indications, including failure to separate from cardiopulmonary bypass, postoperative low cardiac output syndrome, and extracorporeal cardiopulmonary resuscitation. Here, we assessed the outcomes of ECLS in children after cardiac surgery at our institution. Medical records of all children who required postoperative ECLS at our institution were reviewed. Between 2003 and 2011, 36 (1.4%) of 2541 pediatric cardiac surgical cases required postoperative ECLS. Median age of patients was 64 days (range: 0 days-4.1 years). ECLS was in the form of either extracorporeal membrane oxygenation (ECMO; n = 24) or ventricular assist system (VAS; n = 12). Mean duration of ECLS was 4.9 ± 4.2 days. Overall, 21 patients (58%) were weaned off ECLS, and 17 patients (47%) were successfully discharged from the hospital. Patients with biventricular heart (BVH) had higher survival-to-hospital discharge rates compared with those with univentricular heart (UVH) (P = 0.019). Regarding ECLS type, UVH patients who received VAS showed higher rates of device discontinuation than UVH patients who received ECMO (P = 0.012). However, rates of hospital discharge were not significantly different between UVH patients who received VAS or ECMO. Surgical interventions, such as banding of Blalock-Taussig shunt to reduce pulmonary blood flow or placing bidirectional cavopulmonary shunt to minimize ventricular volume overload, were effective for weaning off ECLS in patients with UVH. ECLS is beneficial to children with low cardiac output after cardiac surgery. Rates of survival-to-hospital discharge were higher in BVH patients than UVH patients. Additional interventions to reduce ventricular volume load may be effective for discontinuing ECLS in patients with UVH.

Impact of 3-mm Blalock–Taussig shunt in neonates and infants with a functionally single ventricle

Functionally single ventricle (f-SV) is susceptible to volume overload. Atrioventricular valve regurgitation (AVVR) tends to develop and ventricular function deteriorates due to excessive pulmonary blood flow following modified Blalock-Taussig shunt (mBTS). On the other hand, a small caliber graft has risks of early obstruction and poor growth of pulmonary vascular beds. We assessed the effect of mBTS with a 3-mm graft to circumvent volume overload in f-SV on achievement of the right heart bypass. Eleven neonates and infants with f-SV at the median age of 24 days underwent mBTS using a 3-mm graft between August 2004 and June 2007. There were no early deaths, but there was one late death. All survivors achieved bidirectional cavopulmonary shunt (BCPS) at 4.2 months after mBTS. Cardiac catheterization demonstrated sufficient growth of the pulmonary artery (pulmonary artery index, 268+/-98 cm(2)/m(2)), low pulmonary vascular resistance (1.4+/-0.9 U.m(2)). The AVVR remained mild or less. Ventricular end-diastolic volume and ejection fraction were 171+/-61% of the normal value and 64+/-6%, respectively. We conclude that a 3-mm mBTS was useful in preventing f-SV from volume overload and was effective for growing good pulmonary vasculature and achieving a right heart bypass.

Arch reconstruction without circulatory arrest in neonates.

Between May 2000 and December 2002, 10 neonates underwent arch reconstruction without circulatory arrest. Age at surgery ranged from 1 to 18 days, and body weight ranged from 1.62 to 3.38 kg. The diagnosis was interrupted aortic arch in 4, hypoplastic left heart syndrome in 3, and coarctation complex in 3. A 3 mm polytetrafluoroethylene graft was anastomosed to the innominate artery, and the brain was perfused via this graft while the aortic arch was reconstructed. Regional cerebral oxygen saturation and the right and left radial artery pressures were monitored. There were 2 deaths: one because of low cardiac output syndrome after a Norwood operation; another from multiple organ failure due to preoperatively undetected congenital biliary atresia. Regional cerebral oxygen saturation was kept constant at over 40% during regional cerebral perfusion. There were no neurologic sequelae observed postoperatively. It was concluded that the regional cerebral perfusion technique can be safely applied during neonatal aortic arch reconstruction, and deep hypothermic circulatory arrest should be avoided.

Pulmonary Artery Sling Associated with Tetralogy of Fallot

We describe a rare case of pulmonary artery sling occurring simultaneously with tetralogy of Fallot. This report describes the successful concomitant repair of both intracardiac anomalies.

Total cavopulmonary connection via a thoracotomy

We report a patient who underwent a total cavopulmonary connection with an extracardiac conduit through a right thoracotomy. The thoracotomy approach was useful in circumventing possible hazardous complications at the sternal reentry for the completion of a staged Fontan due to previous mediastinitis in this patient.

Surgical treatment of an infant with myocardial ischemia due to an anomalous origin of the right coronary artery from the main pulmonary artery: report of a case.

An anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare cardiac malformation, and only three cases of isolated ARCAPA in patients younger than 2 years of age have been reported. This report describes the surgical treatment of a 12-month-old girl with myocardial ischemia due to ARCAPA. The diagnosis was made by echocardiography. A reimplantation of the aberrant coronary artery was performed, and the patient had a successful postoperative course.

Bland-White-Garland syndrome with ventricular septal defect: late presentation

Bland-White-Garland syndrome has been reported generally to occur in an isolated lesion. Here, we report a case of Bland-White-Garland syndrome associated with a ventricular septal defect. Late onset of myocardial ischemia was noted in this patient, which we think is related to increased coronary steal due to regression of pulmonary hypertension caused by narrowing of the ventricular septal defect. At surgery, we temporally occluded the left main coronary trunk to cease the coronary steal phenomenon and augment the left coronary flow. Direct implantation of the left coronary artery and closure of the ventricular septal defect were performed. The postoperative course was excellent with no evidence of myocardial ischemia.