Kyoichi Nishigaki

Cantrell's syndrome : a challenge to the surgeon

We present a case of partial Cantrells syndrome with ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with omphalocele. At the age of 20 months, the patient underwent a successful cardiac surgical procedure. To detect risk factors and to define therapeutic strategies, we analyzed the spectrum and the frequency of malformations described in 153 patients with Cantrells syndrome. Despite modern surgical standards, Cantrells syndrome represents a challenge to the surgeon because of the wide spectrum of anomalies, the severity of the abdominal and cardiac malformations, and the high mortality.

What factors affect ventricular performance after a Fontan-type operation

Postoperative conditions after a Fontan-type operation, particularly as they affect results in the early term, are thought to depend on factors such as the state of pulmonary circulation and ventricular function. In this study, we attempted to determine the factors that influence ventricular characteristics in the middle term after Fontan-type procedures. Catheterization was performed at a mean of 15 months after operation in 57 patients with univentricular atrioventricular connection who underwent the operation between 1.0 and 22.6 years of age. End-diastolic volume, end-systolic volume, ejection fraction, and end-diastolic pressure of the systemic ventricle were analyzed together with an estimation of the systemic flow index. These parameters were influenced significantly by the presence of atrioventricular valve insufficiency. The morphologically left ventricle showed a better ejection fraction than did the morphologically right ventricle, whereas the systemic flow index was greater in patients undergoing total cavopulmonary connection than in those receiving an atriopulmonary connection. Young age was significantly associated with a better postoperative contractility, whereas the potential for impaired ventricular compliance was suggested in several patients undergoing operation after 4 years of age. On the basis of our results, we conclude that total cavopulmonary connection performed at a young age should be the surgical procedure of choice and that atrioventricular insufficiency must be treated properly at, and even after, the initial definitive repair.

Surgery for congential heart disease Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

To extend the indications for corrective operation in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, surgical procedures were done to unify the blood sources for pulmonary perfusion. Since December 1985, 50 patients have undergone unifocalization at ages from 2 months to 26 years with a mean of 6 +/- 7 years. In total, 84 staged unifocalization procedures and 5 other palliative procedures were done in 49 patients. These included several operative procedures: simple ligation of major aortopulmonary collateral arteries in 8; pulmonary angioplasty in 29 including reconstruction of the pulmonary arterial tree by direct anastomosis or interposition between the central pulmonary arteries and the intrapulmonary arteries; construction of artificial central pulmonary arteries with use of a xenograft pericardial tube graft in 36 with no native central pulmonary arteries detected; and construction of supplemental central pulmonary arteries also with use of a pericardial tube graft in 10. The pericardial tube graft, if used, was anastomosed to the intrapulmonary arteries on one end and connected to a prosthetic tube on the other end so as to perfuse the reconstructed pulmonary arteries. The anastomosis was made inside the lung through the divided interlobar fissure. Five patients died after operation among those undergoing these 89 preparative operative procedures. Deaths were related either to bleeding caused by anticoagulation therapy administered to prevent thrombosis within the xenograft pericardial tube graft used or to progressive congestive heart failure as a result of an excessive amount of pulmonary blood flow. Twenty-six patients have undergone intracardiac repair after previous unifocalization. In 16 patients the artificial central pulmonary arteries surgically constructed were connected to each other and then an external conduit was placed. In another patient, intracardiac repair and unifocalization could be concomitantly achieved via a median sternotomy. The right ventricle to left ventricle systolic pressure ratio immediately after intracardiac repair in 27 patients ranged from 0.24 to 0.91 with a mean of 0.54 +/- 0.17. One patient (4%) died shortly after intracardiac repair because of thrombosis within the pulmonary arteries. Postoperative catheterization showed that pulmonary vascular resistance was correlated significantly with the number of pulmonary vascular segments functioning rather than with the condition of the central pulmonary arteries. We conclude that surgical unifocalization is a feasible procedure before subsequent intracardiac repair, even in patients with critically hypoplastic or absent central pulmonary arteries.

Biventricular repair in cardiac isomerism ☆ ☆☆ ★ ★★: Report of seventeen cases

Ninety-three patients with cardiac isomerism were treated surgically from July 1985 to June 1991. Among them, three patients with right and 14 with left isomerism underwent biventricular repair. Ages ranged from 4 months to 41 years (mean 4.8 years). Anatomic repair was accomplished in 15 patients and functional repair with the right ventricle used as the systemic ventricle in two patients. Methods of atrial septation to separate pulmonary venous flow from systemic venous flow included atrial partition with a straight patch in seven patients, intraatrial rerouting with a tailored baffle in five, and a Mustard-type atrial switch in five. One hospital death (5.8%) and two late deaths (12%) occurred. Two patients required reoperation (12%), one reconstruction of a stenotic systemic venous connection and one mitral valve replacement because of incompetence. Surgically induced complete atrioventricular block was not observed in any of the patients. Optimal atrial septation offers the possibility of biventricular repair for patients with acceptable intraventricular structure.

A Bicuspid Pulmonary Valve Is Not a Contraindication for the Arterial Switch Operation

There are no obvious criteria concerning the optimal repair for complete transposition with bicuspid pulmonary valve if neither the organic changes in the valve nor the pressure gradient between the left ventricle and the pulmonary trunk are severe. Instead of intraatrial switching or intraventricular rerouting in such circumstances, we have proceeded to the arterial switch procedure in 6 patients with an adequate diameter of the pulmonary valve (greater than 100% of the calculated normal aortic orifice). Postoperative catheterization (at approximately 8 months after the procedures) showed no pressure gradient between the left ventricle and the neoaorta except for a finding of 34 mm Hg difference in 1 patient who had undergone simultaneous subpulmonary myotomy. Echocardiography (7 years later in the longest follow-up) has shown no more than slight regurgitation across the bicuspid neoaortic valve, with no progressive increase of blood velocity across the valve. From these results in the middle term, we conclude that the arterial switch procedure remains an option of choice for patients with initially bicuspid pulmonary valve, providing there is no severe subpulmonary stenosis.

New surgical technique for total-defect aortopulmonary window.

A new technique is described to repair aortopulmonary window with total defect in an 8-day-old baby. Because we expected the future growth of aorta, we used the anterior wall of the pulmonary artery as a large flap to reconstruct the posterolateral aortic wall. An equine pericardial patch was used to repair the defect in the pulmonary artery. This is a logically effective method for aortic reconstruction in a neonate with a large aortopulmonary septal defect.

Bilateral pulmonary artery banding for extremely low birth weight infants with coarctation or interruption of the aorta weighing less than 1.0 kg

Surgical results for coarctation of the aorta (CoA) or interruption of the aorta (IAA) are now excellent, but the repair may still carry a high risk in extremely preterm neonates. Bilateral pulmonary artery banding (PAB) has been performed as a first palliation for hypoplastic left heart syndrome with a poor preoperative condition. This procedure also would be useful as a first palliation for extremely small patients with CoA or IAA. We successfully performed bilateral PAB followed by arch reconstruction and intracardiac repair in 2 extremely low birth weight infants.

Staged Starnes Operation Preserving Patent Ductus Arteriosus for Neonates with Ebstein's Anomaly and Pulmonary Atresia.

We herein reported 2 successful neonates with Ebstein’s anomaly and small pulmonary arteries undergoing Starnes operation preserving the patent ductus arteriosus. Subsequent Blalock-Taussig shunt was carried out 1 or 2 months after the first surgery. One case had already undergone a successful Fontan operation, and the other had a successful bidirectional Glenn shunt so far. This staged Starnes strategy might be a safe and simple choice for neonates with Ebstein’s anomaly and small pulmonary arteries.

Novel Technique for Implantation of a Cardioverter Defibrillator in Children

An 8-year-old boy with hypertrophic nonobstructive cardiomyopathy with ventricular fibrillation underwent implantation of an implantable cardioverter defibrillator. The lead was inserted through a pursestring suture in the right atrial appendage, and the tip of coil was placed in the right ventricular apex under fluoroscopic guidance. Another defibrillation coil was placed in the back of the left atrium and left ventricle by the transverse sinus. The device wrapped in a monofilament mesh sheet was placed in the intraperitoneal space. This case utilized a new technique for an implantable cardioverter defibrillator implantation in a small child.

Efficacy of Damus-Kaye-Stansel procedure in patients with univentricular heart associated with ventriculo-arterial discordance and excessive pulmonary blood flow

Pulmonary artery banding (PAB) and ventriculo-arterial discordance (VAD) were reported to be risk factors of subaortic stenosis in univentricular heart. The aim of this study was to evaluate efficacy of Damus-Kaye-Stansel (DKS) anastomosis. Of all 89 patients undergoing total cavo-pulmonary connection (TCPC) in our center since April 1996, 38 had VAD with high pulmonary blood flow, and had received PAB. Twenty-one of 38 had undergone DKS anastomosis due to subaortic stenosis or due to morphological hypertrophy of the outlet septum (DKS group); the other 17 had not yet (no-DKS group). Percentage end-systolic volume of the systemic ventricle and percentage subaortic lesion in both groups significantly decreased after TCPC (P<0.01). Pressure gradient across systemic outflow tract after TCPC was low in both groups at rest. The gradient in DKS group did not differ from those in control group with ventriculo-arterial concordance (VAC) (P>0.1). Ventricular outflow tract after DKS anastomosis might behave like that of VAC even when dobutamine is loaded, suggesting that the anastomosis should be carried out in many patients with this entity even if stenosis across systemic ventricular outflow is not significant, considering possible stenosis in the future.