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Cardiac transplantation has been established as a therapeutic strategy for patients with end-stage heart failure. In Japan, however, cardiac transplantation has not been performed since the first case in 1968, and even now, after legislation for the approval of brain death was passed in 1997, it is still not performed regularly. Following long and steady efforts to enlighten Japanese society about the concept of brain death and the importance of organ transplantation, the first cardiac transplantation under the new legislation was successfully performed at Osaka University Hospital on February 1999. The patient was 47-year-old male in the dilated phase of hypertrophic cardiomyopathy who had been supported with an implantable left ventricular assist device. This article briefly reviews the situation prior to the first case of cardiac transplantation under the new legislation and discusses the current status of the therapy in Japan.

This study was designed to clarify the efficacy of coronary artery bypass grafting (CABG) on left ventricular (LV) function in 16 patients with a dilated LV due to myocardial infarction (LV end-systolic volume index: LVESVI >60 ml/m2). All had attained complete revascularization. To estimate the LV wall motion quantitatively using echocardiography, a wall motion score (WMS) was used (LV was divided into 17 segments with a four-point scale: akinesis=3, severe hypokinesis=2, hypokinesis=1, normal=0 and then summed). Exercise stress tests were performed after surgery, revealing that anginal symptoms had vanished in all the patients. In 5 patients with a preoperative end-systolic volume index (ESVI) >100 ml/m2, the ejection fraction (EF) did not change, and both were under 30% (before to after: 26+/-4 to 26+/-4%). Neither the ESVI (148+/-50 to 133+/-39 ml/m2) nor the end-diastolic volume index (end-diastolic volume index (EDVI): 198+/-62 to 180+/-37 ml/m2) changed; the WMS did not change (33+/-2 to 33+/-3). During exercise, in spite of the increase in heart rate (HR) (at rest, 81+/-20; HR during exercise, 111+/-21 beats/min, p

In any program for cardiac transplantation, appropriate recipient selection is critically important. The purpose of this study is to evaluate the prognosis of 42 patients with severe cardiac dysfunction who were referred to the Patient Referral Committee of the Osaka University Cardiac Transplant Program from August 1990 to July 1993. All of the patient profiles and clinical data were presented and discussed in the Committee Conference. The Committee classified the patients into three groups according to the following criteria: Class A; 14 patients judged to have a medical indication for heart transplantation. Class B; 7 patients with possible indications which required reevaluation for a definite indication after further intensive medical treatments, and Class C; 21 patients who did not have indications for heart transplantation or who required further clinical examinations and/or medical treatments before a final judgment. Twelve of the 14 Class A patients had a history of NYHA functional class IV and ejection fractions were 25% or less in all of the patients but one (18.5±1.7%). Six patients in Class A had a history of ventricular tachycardia. The one-year survival rate of Class A patients was 60%, and only 28% survived for 28 months. One patient underwent successful heart transplantation in the United States. If we assume that this patient would have died within a year without heart transplantation, the estimated one-year survival rate would fall to 48%, which is comparable to the survival rate of patients who have been accepted for transplant, but are being treated medically, in Western countries. This indicates that our criteria for recipient selection are comparable to those currently used in the Western countries, and therefore may be appropriate for future heart transplantation in Japan.

During the last 3 years, left or bi-ventricular support using a centrifugal pump as a ventricular assistance device was performed in 10 patients after open heart surgery. The basic lesions were coronary heart disease in 8 and valvular disease in 2 patients. Bypass support ranged in time from 33 to 240 h (average 114 h), and 3 patients received biventricular support. Six patients have survived in this group. Other supportive methods, in the form of emergency or elective use of portable cardiopulmonary bypass support, were used in 8 patients; 4 with cardiogenic shock and 4 for supported percutaneous coronary angioplasty. These assisted circulations appear to be useful and promising in the management of the critical cardiac patient.

We studied left atrial compliance and left atrial volume in 21 patients with mitral stenosis showing a normal sinus rhythm. 1) A significant (p less than 0.001) negative linear correlation of r = 0.72 was found between the mitral valve area (MVA) and left atrial mean pressure (LAm). 2) A significant (p less than 0.005) positive linear correlation of r = 0.60 was found between MVA and left atrial compliance (delta V/delta P). 3) A significant (p less than 0.001) hyperbolic correlation of r = 0.73 was found between LAm and delta V/delta P. 4) A significant (p less than 0.001) hyperbolic correlation of r = 0.65 was found between left atrial specific compliance (delta V/delta P/LAVmax. LAVmax: left atrial maximum volume) and left atrial mean volume. 5) These findings led to the conclusion that in patients with mitral stenosis showing a normal sinus rhythm, left atrial compliance was decreased in patients whose left atrial volume was increased, and the decrease of left atrial compliance was related to narrowing of the mitral valve area and elevation of the left atrial pressure.

Pre- and postoperative left ventricular (LV) mass and myocardial contractile state were evaluated in 62 patients with aortic regurgitation (AR) and in 20 patients with aortic stenosis (AS). Preoperatively, there was a significant inverse correlation between LV mass and end-systolic wall stress (ESS)/end-systolic volume index in AR patients and AS patients. In patients with preoperative LV mass exceeding 250 g/m2, ejection fraction failed to return to normal postoperatively, despite reduction in ESS close to the normal controls. Our results indicated that myocardial function was irreversible even in postoperative unloading conditions, when preoperative LV mass exceeded 250 g/m2 in AR patients as well as AS patients.

Mitral regurgitation is a significant complication of end-stage cardiomyopathy, and its existence predicts poor survival. In general, it is thought that mitral valve replacement (MVR) alone is ineffective; however, there are few detailed reports of the clinical course of patients who have undergone MVR. Five patients with mitral regurgitation whose preoperative left ventricular end-systolic volume index was more than 100 ml/m2 were studied. Although their prognosis late after MVR became poor, none of them died within 30 days of the operation. Postoperative cardiac catheterization was performed 6.3+/-1.1 months after surgery; the end-diastolic volume had reduced (before: 193+/-26 ml/m2; after: 166+/-34 ml/m2, p

A 72-year-old man was admitted with palpitations caused by sustained ventricular tachycardia. He had been followed for 27 years, with admissions on 7 occasions, on account of ventricular tachycardias, atrial fibrillation, and congestive heart failure. Electrophysiologic examination detected 2 points of origin of ventricular tachycardia in the right and left ventricles. During the examination a myocardial infarction led to death. At autopsy, most areas of the right ventricle and the left ventricular apex were found to have been replaced by fatty tissue, pointing to severe fatty infiltration into the myocardium. Thus, this patient represented a unique cause of arrhythmogenic right ventricular dysplasia (ARVD) with severe fatty degeneration causing progression of ARVD . It is important that a diagnosis of ARVD is made carefully and rigorously in elderly subjects. (Jpn Circ J 1997; 61: 872 - 876)

We re-evaluated the indications for mitral valve replacement and reconstruction, and attempted to determine the optimal time for aortic valve replacement. (1) The actuarial event-free survival rate in patients undergoing open mitral commissurotomy for mitral stenosis with far-advanced subvalvular deformities was 78 per cent at 11 years after surgery and similar in patients undergoing mitral valve replacement for mitral stenosis. Postoperative clinical improvements compared favorably with those in patients undergoing mitral valve replacement. If quality of postoperative life in patients not requiring anticoagulants is borne in mind, open mitral commissurotomy should be given strong consideration as the procedure of choice for the majority of patients with mitral stenosis. (2) The long-term results of reconstructive procedures for mitral regurgitation largely depend upon the pathological anatomies contributing to the development of mitral regurgitation. The results were less satisfactory in patients with fibrotic changes of valvular and subvalvular tissue than in patients undergoing mitral valve replacement for mitral regurgitation. (3) In aortic valve replacement, the postoperative prognosis was poor in patients with a preoperative left ventricular end-systolic volume index exceeding 200 ml/m2 and left ventricular ejection fraction less than 0.35. Operative management of patients with aortic valve disease should be considered, before severe left ventricular systolic dysfunction is evident.

The elective simple coronary revascularization in the last consecutive 200 patients were analyzed in this study. Despite of the increase in older patients who have more high risk factors, including low left ventricular ejection fraction (LVEF) and multiple vessel disease, there were no operative deaths in the second 100 patients. The use of calcium antagonist in patients with a history of spastic angina eliminated episodes of spastic angina in the perioperative period. In the late postoperative period, there were no cardiac deaths during the 7-year follow-up period. Angina remained in two of three patients with the patent grafts, who had a history of spastic angina. Hyperlipidemia and diabetes mellitus influenced occlusion of the grafts and progression of atherosclerosis of native coronary arteries and then four of five reoperations. Exercise tolerance in patients, even those with preoperative low LVEF, has been improved although their was some increment of left ventricular end-diastolic pressure during exercise. Patency rate in sequential grafting with saphenous vein grafts or internal mammary artery grafts were 92% in the average follow-up period of 11 months. The increase of flow rate with nitroglycerine and atrial pacing in internal mammary artery grafts (IMAG) were comparable with saphenous vein grafts.

There has been a continuing controversy between reconstructive valve surgery and replacement. In this paper, an analysis of clinical and hemodynamic late results of the patients undergoing open mitral commissurotomy (OMC, 135 patients), repair of mitral regurgitation (MAP, 32 patients), and combined valvuloplasty (62 patients) was presented. 1) OMC: The 10-year actuarial survival rate was 86.5% postoperatively. None of the patients required reoperation in this series. Clinical and hemodynamic improvements mainly depended upon the anatomical severity of a stenosed mitral valve. 2) MAP: The 10-year actuarial survival rate was 94% postoperatively. Especially, good to excellent results were obtained in the patients with mitral regurgitation due to ruptured chordae or dilated annulus. 3) Combined valvuloplasty: The 10-year actuarial survival rates postoperatively were 90% in OMC + tricuspid annuloplasty group (31 patients), 100% in MAP + tricuspid annuloplasty group (7 patients), and 87.2% in OMC + aortic commissurotomy group (24 patients). The reconstructive valve surgery provides very small operative risk, very low late mortality and morbidity. Therefore, it should be the treatment of choice for most of the patients with symptomatic valve disease.

Real-time flow imaging of pulmonary regurgitation (PR) by two-dimensional pulsed Doppler echocardiography was performed in 34 patients after repair of tetralogy of Fallot. The right ventricle and pulmonary artery were demonstrated in the parasternal short axis view and PR flow was visualized. The image was frozen when the velocity of regurgitant flow was maximum at the level of the pulmonary valve. Pulmonary regurgitant area index (PRAI) was calculated from planimetric measurement of the area where PR flow was expressed. Grading of PR (1 to 3) was independently attempted according to the extension of PR flow detected in pulmonary artery by a range-gated pulsed Doppler echocardiography. PRAI (cm2/m2) was 0.36 +/- 0.29 (mean +/- standard deviation) in grade 1, 1.48 +/- 0.46 in grade 2, and 2.80 +/- 0.94 in grade 3. Severity of PR (grade 1 to 3) was also rated on the basis of the pulmonary arteriography and compared with PRAI. PRAI was 0.64 +/- 0.60 in grade 1, 1.07 +/- 0.63 in grade 2, and 2.21 +/- 1.67 in grade 3, respectively. RPAI showed strong correlation with pulmonary regurgitant fraction measured by videodensitometric study of right ventriculography (r = 0.84). Quantitative evaluation of PR using real-time flow imaging system is a reliable and useful method.

Eighty-seven cases were studied in which IABP was used with open heart surgery, and 19 in which V-A bypass was employed. IABP was used in 31 patients with ischemic heart disease, which had the best survival rate (52%). Recent experience shows that the effects of IABP increase the operability of certain high-risk patients with severely impaired left ventricular function. In valvular heart disease, IABP was used with a survival rate of 34%. In congenital heart disease, the IABP survival rate was the worst among the groups studied because, in addition to left heart failure, many other factors also as cause the low cardiac output syndrome during corrective surgery for complicated cardiac anomalies. In total right heart bypass, or Fontan's procedure, three out of five cases were weaned from IABP; one of them is still alive. IABP was used with success in one case of valvular heart disease with almost no contraction of the right ventricle after repair. Therefore, IABP could be expected to improve the condition of severe postoperative right heart failure, or the equivalent condition, when there is subnormal left ventricular function in the postoperative period and pulmonary vascular resistance is normal. There has been only one successful V-A bypass lasting 11 hours, in valvular heart disease, and another case, in congenital heart disease, where the patient was weaned after V-A bypass lasting four days.

Ventricular volume measurements of single ventricle were carried out in 20 patients with or without palliation and in 2 after total correction. In the former 20 patients, 6 had a single left ventricle and 14 a single right ventricle, anatomically. Two having total correction were associated with undivided ventricle. In the 20 patients with or without palliation, ventricular end-diastolic volume ranged from 64 to 206 (115 +/- 42) percent of the sum of the normal left and right ventricular volumes. Ejection fraction of single ventricle ranged from 0.40 to 0.64 (0.55 +/- 0.06). There was no significant difference in ventricular volume and ejection fraction between patients with a single left and right ventricle. There was high correlation of ventricular volume with pulmonary to systemic blood flow ratio (r = 0.66, p less than 0.005). This indicates that systemic-to-pulmonary shunt should increase ventricular volume of single ventricle. In two patients after total correction, total ventricular volume was 136 and 166% of the sum of the normal left and right ventricular volumes. Total ejection fraction was 0.55 and 0.33 in the first and second patient, respectively. The prosthetic ventricular septum bulged toward the right ventricle during systole and this shift made the left ventricular volume increase and ejection fraction decrease. Right-to-left ventricular volume ratio at end-diastole was 0.74 and 0.67 in the first and second patient, respectively. These results suggest that 1) larger ventricular volume than the sum of the normal left and right ventricular volumes would be required for septation of single ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)

Age-related changes in right (RV) and left (LV) ventricular myocardium in tetralogy of Fallot (TF) are analyzed with regard to pre- and postoperative ventricular function. In RV, preoperative function was better in patients under 4 years of age in terms of ejection fraction and compliance, but no significant change was seen in LV function. Postoperatively, patients who underwent corrective surgery at age less than 4 years showed no significant difference compared to those at operative age above 4 years in terms of RV and LV functions at rest. However, stress tests for RV by isoproterenol and LV using methoxamine revealed some masked ventricular dysfunction in both ventricles in the older groups. The relationships between these functional abnormalities and previously described histological changes of right and left ventricular myocardium in TF are discussed.

The postoperative changes of pulmonary vascular resistance (PVR) of two age groups of children with complete atrioventricular canal (CAVC) were compared. Patients were divided by age at the time of primary repair; Group-1 (G-1, n = 4) with age below 2 years (average 13.8 mo.) and Group-2 (G-2, n = 5) with age over 2 years (average 44.0 mo.). All except one were Down's syndrome. Preoperatively, pulmonary to systemic resistance ratio (Rp/Rs) were 0.87 +/- 0.50 in G-1 and 0.41 +/- 0.13 in G-2 (n.s.), and postoperative study (average 6.5 mo.) showed no significant falls in Rp/Rs in both groups. However, G-2 showed significantly lower Rp/Rs postoperatively (0.79 +/- 0.19 in G-1 vs 0.27 +/- 0.12 in G-2, p less than 0.05). Mean pulmonary artery pressure (mPA) showed significant fall after surgery in only G-2 (66.8 +/- 6.6 to 31.8 +/- 11.6 mmHg, p less than 0.005). Anatomically, type-A showed better postoperative change in mPA compared to type-C. This study summarized the early progression of pulmonary vascular obstructive disease with poor postoperative improvement even the primary repair was done before 2 years of age. The less advanced pulmonary vascular disease in G-2 might have some relations to the natural selection of the disease.

The adequacy of hepatic circulation in terms of oxygen supply-demand relation was assessed by measuring hepatic venous oxygen saturation (Shvo2) in patients. Among those with congenital cardiac lesions (n = 11), Shvo2 during the early postoperative period was markedly low as less than 20% in Fontan operation group (n = 5) with subsequent clinical findings of acute hepatic dysfunction. Significant correlations were found between Shvo2 values early after surgery and subsequent peak values in serum hepatic enzymes. Serum total bilirubin and prothrombin time started to deteriorate when Shvo2 became below 30%. Cardiac index, hepatic perfusion pressure and mixed venous oxygen saturation showed positive linear correlations with Shvo2, and central venous pressure (CVP) with an inverse relation. In chronic valvular disease (n = 28), those with NYHA class IV patients showed lower Shvo2 (average; 47.4%) at cardiac catheterization than the others (class-I; 66.4%, class-II; 63.9%, p less than 0.05). These results indicate that Shvo2 monitoring appears to be useful to assess the hepatic perfusion in terms of oxygen supply-demand relation in acute and chronic heart failure, and Shvo2 of 30% seems to be a critical level.

The postoperative function of the right (systemic) ventricle (SV) was evaluated in eight pediatric patients who had undergone intra-atrial switching for simple transposition of the great arteries (TGA). SV function was evaluated at rest and during isoproterenol (ISP) infusion and compared with left ventricular function in the control group. The control group consisted of five patients including two with postoperative pulmonary stenosis, two with mild pulmonary stenosis and one with postoperative partial anomalous pulmonary venous drainage. SV-graphy was performed at rest. During ISP infusion, the TGA group showed a significant increase in cardiac index (3.50 +/- 0.68 (mean +/- SD) L/min/m2 to 4.94 +/- 1.53 L/min/m2, p less than 0.05) and no significant changes in stroke volume index (SVI). Similar changes were observed in the control group. Resting SV ejection fraction was 0.37 +/- 0.05 in the TGA group. In regard to the relationship between SV end-diastolic pressure and SVI, all but one of the TGA patients showed normal response to the stress. These results indicated that the right ventricle in TGA showed an acceptable response to ISP stress and acted as a systemic ventricle for at least two years after surgery.