[Modified Fontan procedure for complex cardiac anomalies--problems in the cases of RV type systemic ventricle].
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Biomedical subjects
Publications and source records attributed to F Isobe.
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Ninety-one patients with tetralogy of Fallot underwent intracardiac repair between 1978 and 1981. One patient died from left-sided heart failure. Retrospective analyses of this death revealed a significant decrease of the left ventricular (LV) end-diastolic volume index (EDVI) of 21 ml/m2 (36% of normal). Results of early postoperative hemodynamic studies after total correction of this anomaly suggested that an EDVI of 30 ml/m2 is the minimal requirement for adequate cardiac output postoperatively. Based on these data, 3 patients with decreased LV volume with EDVI of around 30 ml/m2 were challenged with the primary repair with success, although they required atrial pacing and catecholamine support postoperatively to maintain adequate left atrial pressure and cardiac output. From these results, it is recommended that patients with tetralogy of Fallot and an EDVI of 30 ml/m2 or more can be considered as candidates for the primary repair, but that patients with an EDVI of less than 30 ml/m2 should be palliated once by systemic-to-pulmonary arterial shunt procedures. Subsequent total correction should be performed after sufficient LV growth for those patients.
We recently adopted the Rastelli operation as a one stage anatomical correction for three infants with transposition of the great arteries and associated with ventricular septal defect (TGA + VSD). In all, ventricular septal defect was enlarged by excision of the conus septum to avoid obstruction of intraventricular tunnel and Hancock valved conduits of 18 mm in diameter were used for reconstruction of pulmonary trunk. The postoperative function of the left ventricule proved to be better than that of the right ventricle, as the systemic ventricle, after the Mustard procedure for TGA + VSD. Our results show that the Rastelli operation is effective as a one stage anatomical correction in infants with TGA + VSD.
Sixty-six patients in whom atrial pacemaker (AAI) were implanted were followed for one year to 5 years for the occurrence of pacing failure, sensing problems, and later AV block. Pacing failure occurred in only one patient and sensing problems occurred in 15 patients but 10 of them improved after a change of sensing. Temporal change of AV conductivity was not recognized in the majority of patients. Eighteen patients developed transient decrease in AV conductivity. Two patients developed persistent decrease in AV conductivity and ended in clinical AV block for which the pacemaker was implanted. Out of 66 patients, 22 had a history of paroxysmal atrial flutter or fibrillation (AFF) prior to AAI implantation. They were divided into two groups. Group I consisted of 20 patients in whom paroxysmal AFF disappeared after AAI implantation. Group II consisted of 22 patients in whom paroxysmal AFF persisted after AAI implantation. Electrophysiological studies prior to the AAI implantation showed that sinus rate at control was significantly slower (36.3 +/- 10.1 beats per min in Group I, 57.1 +/- 10.8 beats per min in Group II), atrial fragmented activity zone was significantly narrower (62.7 +/- 32.9 msec in Group I, 88.1 +/- 19.7 msec in Group II), and the occurrence of PAC was less at an atrial pacing rate of 70 beats per min (8% in Group I, 67% in Group II) in Group I compared to Group II.(ABSTRACT TRUNCATED AT 250 WORDS)
The growth of pulmonary arteries following systemic-pulmonary arterial shunt procedures in infants with cyanotic heart disease is a determining factor to the prognoses of those patients. We assessed the validity of two-dimensional echocardiography (2DE) in evaluating pulmonary arterial growth following shunt procedures. Blood flow through the shunts was measured at the times of surgery, and the correlations between shunt flows and postoperative clinical courses were studied. 2DE was validated by its assessments of the right pulmonary artery (RPA) with an excellent correlation between the RPA diameter on 2DE and that on angiography (r = 0.97) and/or the actual RPA diameter measured at the times of surgery (r = 0.96) in 20 patients. Ten patients with tetralogy of Fallot and pulmonary atresia were studied by 2DE pre- and postoperatively. The RPA diameters of six patients with effective shunts increased from 5.7 +/- 1.7 mm to 8.5 +/- 1.5 mm during six months after the shunts and their left ventricular end-diastolic dimensions also increased significantly. But the RPA diameters in four patients with ineffective shunts did not show significant increases after the shunts. The shunt flows were correlated with the diameters of the anastomoses. The shunts which were obstructed within two years showed shunt flows of 83 +/- 26 ml/min, while the shunts which were patent for more than two years showed shunt flows of 320 +/- 126 ml/min. Serial measurements of RPA diameters by 2DE serve as a useful method of following patients after systemic-pulmonary shunts. The amounts of shunt flows were correlated with post-operative clinical courses over two year periods.
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The surgical results in patients with severe congenital heart disease, who underwent surgical treatment between 1978 and 1981 at the National Cardiovascular Center, Japan, were analyzed. The surgical mortality rates were 4% for cases of ventricular septal defect associated with pulmonary hypertension in patients under 2 years of age, 16.7% for complete atrio-ventricular canal, 11% for coarctation of the aorta associated with ventricular septal defect, 40% for pure pulmonary atresia without Ebstein anomaly, 44% for total anomalous pulmonary venous return, 14.8% for transposition of the great arteries, 44% for double outlet right ventricle and 1.1% for tetralogy of Fallot. The surgical results have been improving and postoperative residua and sequelae have been decreasing through our persistent efforts. Today's main problem is a relatively poor performance in cases which need surgical intervention early in life, i.e., under 3 months of age. Therefore, we think that our effort to improve the surgical results for new-borns or very young infants is most necessary.
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Partial Atrioventricular Canal Defect ( PAVC ) The operative results of this anomaly is generally acceptable. Between 1978 and 1982, 32 patients with PAVC underwent intracardiac repair. Two operative deaths occurred in 41 and 42 years old patients who were in NYHA class 4 preoperatively. Postoperative mitral insufficiency was either nil or mild except in one patient with mitral valve prolapse which was left unrepaired. Complete Atrioventricular Canal Defect ( CAVC ) Our operative procedures for the repair of CAVC are as follows, 1) all of accessory chordae are cut off and the bridging leaflet is incised into mitral and tricuspid components. 2) Ventricular septum, atrioventricular valve and atrial septum are constructed with our designed endocardial cushion prosthesis (Dacron prosthesis with pericardial brim of 3-4 mm width in each side). Between 1978 and 1982, 20 patients with CAVC containing 16 infants less than 2 years old underwent intracardiac repair using this prosthesis. Thirteen patients had no severe associated anomalies and only one death occurred in this group. Three patients were combined with tetralogy of Fallot (one died) and two patients with double outlet right ventricle (one died). The other two patients had either coarctation of the aorta (died) or interruption of the aortic arch (survived).
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