Yorikazu Harada

Do we need fenestration when performing two-staged total cavopulmonary connection using an extracardiac conduit?

Between August 1999 and December 2007, 72 consecutive patients with single ventricle physiology underwent a modified Fontan procedure after a bidirectional Glenn shunt using an extracardiac polytetrafluoroethylene conduit without fenestration. Nitric oxide gas inhalation was commenced just after cardiopulmonary bypass together with intravenous phosphodiesterase III inhibitor administration. After oral intake was started, pulmonary vascular dilators such as beraprost, sildenafil, bosentan were given orally according to amount of chest drainage and patients condition. After discharge, oxygen therapy at home was continued for three months. No hospital death occurred after surgery. All patients were followed by our institute and follow-up period was 44.2+/-26 (36-106.8) months. One late death occurred during this follow-up period after re-operation. Cardiac catheterization after the Fontan completion showed transpulmonary gradient of 5.9+/-2.4 mmHg, systemic output of 3.4+/-2.1 l/min m2. Arterial oxygen saturation (SaO(2)) at the latest outpatient visit was 94.4+/-3.8%. According to our clinical experience with two-staged total cavopulmonary connection using an extracardiac conduit without fenestration, fenestration in the Fontan circuit is not necessary when performing the Fontan completion. Two-staged extracardiac total cavopulmonary connection without fenestration can be satisfactorily completed with the aid of pulmonary vasodilation therapy.

Modified Starnes operation for neonatal Ebstein’s anomaly

We report the case of a severely symptomatic neonate with Ebsteins anomaly. A modified Starnes operation was performed, but insufficient drainage of venous blood returning through thebesian veins caused overdistention of the right ventricle and severe left ventricular dysfunction. Urgent reestablishment of right ventricular-right atrial communication successfully resolved these problems.

Absent aortic valve : Successful palliation in the neonate

A successful palliation of a neonate with absent aortic valve in hypoplastic left heart syndrome is reported.

Normal values for cardiac and great arterial dimensions in premature infants by cross-sectional echocardiography

The number of neonates who undergo intracardiac repair for congenital heart disease has increased in recent years. In considering appropriate surgical options in such circumstances, normal values for various cardiovascular dimensions are needed. We measured 19 parameters for the heart and great arteries (four valves, pulmonary tree, aortic arch, ascending and descending aorta, ventricular septum, left ventricular internal and posterior wall dimensions) using cross-sectional echocardiography in 55 premature and full-term infants (ranging in gestational age from 23 to 41 weeks, in body weight from 543 to 3966 g). The increase in each dimension correlated closely with body weight (r=0.62 to 0.87) and was found to follow a linear regression. The diameter of the isthmus was found to be smaller, and the size of mitral valve annulus larger, than previously published values.

Second-Stage Palliation After Bilateral Pulmonary Artery Bands for HLHS and its Variants—Which is Better, Modified Norwood or Norwood Plus Bidirectional Glenn?

Background. The purpose of this study was to evaluate the surgical outcomes and pulmonary artery (PA) development associated with a new strategy wherein the modified Norwood (N) procedure is performed at 1-2 months after bilateral pulmonary artery banding (PAB). Methods. Between January 2008 and February 2010, 16 patients underwent Norwood-type operation after previous bilateral PAB. For analysis, patients were divided into two groups. Group I (n = 11) underwent modified Norwood procedure with either right modified Blalock Taussig (RMBT) shunt (n = 4) or right ventricle to pulmonary artery (RV-PA) conduit (n = 7). Group II (n = 5) underwent Norwood procedure plus bidirectional Glenn anastomosis. Diagnoses were hypoplastic left heart syndrome in 6 and its variants in 10. Results. There was no surgical death and no late death. Pulmonary artery interventions were performed at the time of the Norwood procedure in 27% in Group I and in 100% in Group II (p < 0.05). Additional PA interventions were performed during the period of follow-up in 4 cases in Group I (36.4%), and in 4 cases in Group II (80.0%). Additional Blalock Taussig shunts were performed in 7 patients, resulting in significant increase in PA index. In all, four patients have reached total cavopulmonary connection, and one has undergone biventricular repair. Eight patients in Group I and one patient in II Group reached bidirectional Glenn anastomosis. In Gp II, two patients showed LPA narrowing or obstruction with PA index of 80 ± 12 mm2/m2. Conclusions. Regarding the second-stage palliation after bilateral PAB, modified Norwood procedure with either RMBT or RV-PA conduit has some advantages compared with Norwood plus BDG with respect to subsequent pulmonary artery development. Additional BT shunt may contribute to PA development, even in the patients with Norwood procedure with RV-PA conduit.

Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis

A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.

Successful neonatal balloon aortic valvoplasty following prenatal diagnosis of critical aortic stenosis

We report a successful neonatal balloon aortic valvoplasty after prenatal diagnosis of critical aortic stenosis with severe left ventricular dysfunction. The infant was delivered by cesarean section at 34 weeks of gestation and had balloon valvoplasty via the right common carotid artery 67 minutes after birth. Left ventricular contraction improved immediately after the procedure. Scheduled balloon aortic valvoplasty immediately after birth subsequent to prenatal diagnosis can improve the outcome of critical aortic stenosis.

Interventional treatment for fetus and newborn infant with congenital heart disease.

Abstract Background : Catheter intervention has become popular not only in adult patients but in younger patients with congenital heart disease. The early neonatal catheter interventional treatment has also been attempted in conjunction with the development of prenatal diagnosis of the congenital heart disease.

Current status of the hybrid approach for the treatment of hypoplastic left heart syndrome

The hybrid approach for hypoplastic left heart syndrome (HLHS), consisting of bilateral pulmonary artery banding and ductal stenting, has emerged as an alternative to the traditional Norwood approach. This approach defers open heart surgery to beyond the neonatal period, which is believed to reduce postoperative mortality and morbidity and improve neurological development as compared with the conventional approach. However, there have been no scientific studies supporting these hypotheses. Recently, there seems to be a tendency that many centers recommend the hybrid approach as an interim procedure to rescue preoperative high-risk patients. Currently, the decision to adopt the hybrid approach or the Norwood approach seemed to be based on the preference of congenital heart surgeons and cardiologists. Further investigation including a randomized multi-center study would allow a scientific decision as to which approach is more appropriate for the patient with HLHS.

Aortic arch reconstruction for interrupted aortic arch using an aberrant right subclavian artery

An aberrant right subclavian artery can be used in a variety of ways in complex aortic arch reconstructions, including reconstruction of an interrupted aortic arch. Here, we described the case of a 4-month-old female infant with a type B interrupted aortic arch, who underwent arch reconstruction using an aberrant right subclavian artery.