Cerebrovascular Accident Rate Is Different Between Centrifugal and Axial-Flow Pumps, but Survival and Driveline Infection Rates Are Similar.

OBJECTIVES: We analyzed the outcome of patients with implantable left ventricular assist devices (LVADs) at the University of Tokyo Hospital to compare those with centrifugal pumps (CE group: Duraheart and Evaheart) and those with axial-flow pumps (AX group: Heartmate II and Jarvik 2000). METHODS: A total of 68 patients who underwent implantation of LVADs (Duraheart: n = 15; Evaheart: n = 23; Heartmate II: n = 22; Jarvik 2000: n = 8) as a bridge to transplantation at our institution from May 2011 to April 2015 were retrospectively reviewed. All patients were followed through December 2015. RESULTS: The mean follow-up time of the CE group was 1.95 ± 0.92 year (total 74.1 patient-years) and that of the AX group was 1.56 ± 0.56 year (total 46.8 patient-years). Whether the patients underwent centrifugal or axial-flow pump implantations was not associated with survival or driveline infection according to log-rank test (1-year survival rate: 89% vs 100% [P = .221]; 1-year freedom rate: 40% vs 43% [P = .952]). The rates of freedom from cerebrovascular accident (CVA) at 1 year after LVAD implantation in the CE and AX groups were 70% and 96%, respectively (P < .001). The CE group showed a higher frequency of CVA (0.472 vs 0.021 event per patient-year). CONCLUSIONS: Our findings indicate that overall survival and driveline infection rates are similar between centrifugal and axial-flow pumps, but they suggest that patients with centrifugal pumps are more likely to develop CVAs than those with axial-flow pumps.

Association of the Number of HLA-DR Mismatches With Early Post-transplant Acute Cellular Rejection Among Heart Transplantation Recipients: A Cohort Study in Japanese Population.

BACKGROUND: Although many risk factors are reported about graft rejection after heart transplantation (HTx), the effect of HLA mismatch (MM) still remains unknown, especially in the Japanese population. The aim of the present study was to investigate the influence of HLA MM on graft rejection among HTx recipients in Japan. METHODS: We retrospectively investigated the association of the number of HLA MM including class I (A, B) and class II (DR) (for each locus MM: 0 to 2, total MM: 0 to 6) and the incidence of moderate to severe acute cellular rejection (ACR) confirmed by endomyocardial biopsy (International Society for Heart and Lung Transplantation grade ≥ 3A/2R) within 1 year after HTx. RESULTS: Between 2007 and 2014, we had 49 HTx cases in our institute. After excluding those with insufficient data and positive donor-specific antigen, finally 35 patients were enrolled. Moderate to severe ACR was observed in 16 (45.7%) patients. The number of HLA-DR MM was significantly associated with the development of ACR (ACR+: 1.50 ± 0.63, ACR-: 1.11 ± 0.46, P = .029). From univariate analysis, DR MM = 2 was the only independent risk factor for ACR episodes (P = .017). The frequency of ACR within 1 year was significantly higher in those with DR MM = 2 (DR MM = 0 to 1: 0.3 ± 0.47, DR MM = 2: 1.17 ± 1.34 times, P = .007). CONCLUSIONS: The number of HLA-DR MMs was associated with the development and recurrence of ACR episodes among HTx recipients within 1 year after transplantation in Japanese population.

Radionuclide angiographic assessment of left ventricular diastolic filling in amyloid heart disease: a study of patients with familial amyloid polyneuropathy.

To assess left ventricular diastolic filling in amyloid heart disease, 17 patients with familial amyloid polyneuropathy and 20 normal subjects were examined by radionuclide angiography. None of the patients showed clinical evidence of restrictive cardiomyopathy. All but two patients had normal left ventricular ejection fraction. Peak filling rate was significantly lower and time to peak filling rate was significantly greater in patients than in normal subjects (2.60 +/- 0.52 versus 3.10 +/- 0.44 EDV/s, p less than 0.001, and 215 +/- 53 versus 147 +/- 18 ms, p less than 0.001, respectively). The mean left ventricular filling volume during rapid diastolic filling and atrial systole in patients was 54.5 +/- 19.5% and 44.2 +/- 21.6% of the stroke volume, respectively, compared with 83.8 +/- 6.6% (p less than 0.001) and 20.0 +/- 6.0% (p less than 0.001), respectively, in normal subjects. Although 10 of the 14 patients without clinical evidence of overt heart disease had normal ventricular wall thickness as well as normal ejection fraction, 8 of the 10 showed abnormal diastolic filling. In patients with familial amyloid polyneuropathy, indexes of diastolic filling were significantly related to ventricular wall thickness alone. The incidence and magnitude of abnormalities in time to peak filling rate and contribution of rapid filling as well as atrial systole to ventricular filling increased with age and duration of illness. Thus, abnormal diastolic filling can be seen even in the early stage of familial amyloid polyneuropathy and may be related to myocardial amyloid deposition as well as to fibrosis. Careful consideration should be given to age and duration of illness when diastolic filling is assessed in this disorder.

Iodine-123 metaiodobenzylguanidine scintigraphic assessment of myocardial sympathetic innervation in patients with familial amyloid polyneuropathy.

OBJECTIVES: This study attempted to assess myocardial sympathetic innervation using iodine-123 (I-123) metaidobenzylguanidine (MIBG) imaging in patients with familial amyloid polyneuropathy. BACKGROUND: Signs and symptoms of cardiac autonomic dysfunction are commonly seen in patients with cardiac amyloidosis. However, the incidence and magnitude of abnormalities in myocardial sympathetic nerve function by means of I-123 MIBG imaging and their relation to clinical findings, cardiac function and the results of thallium-201 (Tl-201) and technetium-99m pyrophosphate (Tc-99m PYP) myocardial scanning have not yet been clarified. METHODS: We performed M-mode, two-dimensional and Doppler echocardiography and I-123 MIBG, Tl-201 and Tc-99m PYP imaging of the heart in 12 patients with familial amyloid polyneuropathy and biopsy-proved cardiac amyloidosis. RESULTS: Ten of 12 patients had no clinical evidence of overt heart disease, but left ventricular (LV) wall thickening was observed in 4 of these 10. Left ventricular percent fractional shortening and Doppler transmitral flow velocity patterns were found to be normal in all 12 patients. Eight of 12 patients showed no myocardial MIBG accumulation, with limited uptake in the remaining 4 demonstrated only in the LV anterior wall. Diffuse but mild myocardial uptake of Tc-99m PYP occurred in only 4 of 12 patients, and all 12 had normal results on Tl-201 myocardial scanning. Complete defects on myocardial MIBG scans were found in five of eight patients with negative findings on Tc-99m PYP myocardial scanning. The incidence and magnitude of myocardial uptake of MIBG were independent of clinical findings, extent of endomyocardial amyloid deposition, electrocardiographic QRS voltage and ventricular wall thickness. CONCLUSIONS: Patients with familial amyloid polyneuropathy show a high incidence of myocardial adrenergic denervation with viable myocardium that can be identified very early in cardiac amyloidosis, before the development of clinically apparent heart disease, ventricular wall thickening, significant LV systolic and diastolic dysfunction and positive findings on Tc-99m PYP myocardial scanning.

Frequency analysis of signal-averaged electrocardiogram in patients with right ventricular tachycardia.

OBJECTIVES: The purpose of this study was to analyze the frequency content of signal-averaged electrocardiograms (ECGs) in patients with idiopathic ventricular tachycardia of right ventricular origin and in patients with arrhythmogenic right ventricular dysplasia. BACKGROUND: The late potentials in the time domains are usually found in patients with arrhythmogenic right ventricular dysplasia. They are not usually found in patients with idiopathic ventricular tachycardia of right ventricular origin. METHODS: Fast Fourier transform analysis of signal-averaged ECGs was performed with the use of a Blackman-Harris window in 43 subjects: 20 normal volunteers (group I), 12 patients with idiopathic ventricular tachycardia of right ventricular origin (group II) and 11 patients with arrhythmogenic right ventricular dysplasia (group III), and the frequency spectrum was displayed in a three-dimensional graph. Area ratio (ratio of the area under the spectral plot from 40 to 120 Hz to the area from 0 to 120 Hz) was calculated in all subjects. RESULTS: Area ratio was significantly higher in group II than in group I (243 +/- 45 vs. 196 +/- 15, p < 0.01) and significantly higher in group III (396 +/- 51) than in group I or II (p < 0.001). The high frequency components in group II were confined within the QRS complex in the three-dimensional graph, whereas those in group III extended outside the QRS complex. CONCLUSIONS: Frequency analysis of the signal-averaged ECG with fast Fourier transform analysis can detect the high frequency components in patients with right ventricular tachycardia, including idiopathic ventricular tachycardia and arrhythmogenic right ventricular dysplasia.

Comparison of electrocardiographic findings in patients with AL (primary) amyloidosis and in familial amyloid polyneuropathy and anginal pain and their relation to histopathologic findings.

To assess the prevalence of chest pain and ischemic electrocardiographic (ECG) changes and relate them to histopathologic findings of coronary arteries in cardiac amyloidosis, 33 patients with AL (primary) amyloidosis and 60 patients with familial amyloid polyneuropathy (FAP) were examined. Five patients (15%) with AL amyloidosis had recurrent anginal pain with exertion and 2 of them also experienced anginal pain after orthostatic hypotension. The chest pain was associated with transient downsloping or horizontal ST-segment depression with or without T-wave inversion in right precordial leads, whereas the remaining patients with AL amyloidosis and all patients with FAP did not show anginal pain or ischemic ST-T changes. Histologic sections of coronary arteries were obtained in 12 patients with AL amyloidosis, including 4 of the 5 patients who had angina pectaris and in 25 patients with FAP. Three patients with anginal pain had variable degrees of stenoses of the intramural coronary arteries by amyloid deposition predominantly in the media with normal or nearly normal epicardial arteries. One patient with AL amyloidosis who had effort angina showed marked stenosis and complete occlusion of the small coronary vessels by transmural amyloid deposition. The remaining 8 patients with AL amyloidosis and 25 with FAP without chest pain did not exhibit any stenosis or occlusion of both the epicardial and intramural vessels. These findings suggest that ischemic ST-T changes with chest pain are not so rare in patients with AL amyloidosis, and that markedly decreased myocardial oxygen supply due to diffuse stenotic or occlusive disease of the small coronary vessels by amyloid deposition contributes to the development of clinically significant ischemic heart disease in these patients.

Pulmonary arterial aneurysm with CATCH 22 syndrome--a case report.

An adult case of CATCH 22 syndrome with a pulmonary arterial aneurysm is presented. The chest radiograph showed a large massive lesion, which should be differentiated from lung cancer, mediastinal tumor, or aortic aneurysm. CATCH 22 syndrome is a developmental malformation caused by hemizygous deletion of 22q11 chromosome, which usually presents with cardiac malformation including tetralogy of Fallot, truncus arteriosus, patent arterial duct, and ventricular septal defect. Most patients with this syndrome usually die early in life.

Successful stenting of bilateral renal artery stenosis due to fibromuscular dysplasia assessed by use of pressure guidewire technique: a case report.

An 18-year-old woman with renovascular hypertension had stenosis due to fibromuscular dysplasia (FMD) of the bilateral renal arteries, and this did not respond to conventional balloon angioplasty owing to marked elastic recoil. Implantation of Palmaz-Schatz stents resulted in successful dilation of the stenosis and remission of hypertension. Transstenotic pressure gradients were measured by use of a pressure-monitoring guidewire. It is suggested that stenting is a very useful adjunct to balloon angioplasty in the treatment of renal artery stenosis due to FMD.

[Hemodynamic significance of diffuse lung uptake of 201Tl in heart diseases].

Clinical significance of diffuse lung uptake of 201Tl was studied in 244 cases of various heart diseases. The grade of lung uptake of 201Tl was assessed by total lung uptake ratio (L/T), maximal lung uptake ratio (Max/T), lung to myocardium or mediastinum counts ratio (M/H or M/Med) and visual grade. L/T, Max/T, M/H and M/Med were directly correlated with pulmonary arterial pressure (PCW), and inversely correlated with cardiac output (CO) and cardiac index (CI). Among them, L/T was the best index to assess PCW, that was correlated with PCW (r = 0.65), CO (r = -0.53) and CI (r = -0.45). The visual grade of 201Tl lung uptake was correlated with L/T, and the increased lung uptake was associated with a higher PCW and a lower CO as well as a lower CI. Diffuse increased lung uptake of 201Tl was observed in 29 of 41 cases (70.7%) with myocardial infarction, 25 of 29 cases (86.2%) with mitral valvular diseases and 9 of 10 cases (90.0%) with combined valvular diseases, in addition, in most cases with left heart failure and cases of NYHA 3 and 4, but that was not rarely observed in cases of NYHA 2 and without left heart failure. In heart diseases, 201Tl myocardial scintigraphy seems to be valuable for detection of left heart failure including pulmonary congestion and interstitial pulmonary edema. Total lung uptake ratio (L/T) may be useful for estimation of PCW, and the visual grade of 201Tl lung uptake may be applicable as routine index for grading of lung uptake of 201Tl.

[From arrhythmogenic right ventricular dysplasia/cardiomyopathy(ARVD/ARVC) to a broader concept of ABCDE syndrome].

The concept on the presence of a peculiar disease, arrhythmogenic right ventricular cardiomyopathy, has been widely recognized to be involved in the category of cardiomyopathy. The authors believe this disease entity should be further expanded, and propose the new term "ABCDE Syndrome", abbreviated from Arrhythmogenicity, Brugada syndrome, Competitive sports, Death(especially sudden death) and Electric disturbance type of cardiomyopathy(ECM), be incorporated to encompass all facets of this category.

Improved survival in patients with continuous-flow ventricular assist device for bridge to heart transplantation.

BACKGROUND: Contemporary continuous-flow ventricular assist devices (CFVADs) have greatly improved patient survival for indications of bridge to transplantation (BTT) and destination therapy. In Japan, CFVAD is limited for BTT use. The waiting period for heart transplantation (HT) is long owing to donor shortage. We examined the results of CFVAD for BTT indication. METHODS: Eighty-nine VAD treatments were performed among subjects whose preimplantation condition was profile 1 (n = 49) or profile 2 or 3 (n = 40). The device was the paracorporeal pulsatile Nipro VAD (n = 67) or CFVAD (n = 22). All CFVAD patients were profile 2 or 3. RESULTS: The median assist period was 529 days (Nipro VAD, 530; CFVAD, 528). Twenty-six patients were on the device for >2 years. Actuarial survival was 81.6%, 69.5%, and 61.1% at 1, 3, and 5 years. Survival in profile 1 was significantly worse than in profile 2 or 3. Survival of CFVAD patients was superior to that of paracorporeal VAD. Six-month mortality rate of 20% in cases before 2009 (n = 60) was dramatically improved to 3% among those after 2010 (n = 29). All patients with CFVAD were alive and discharged home. 26 patients were transplanted, 7 had been weaned from VAD and 27 were on a device. The rate of events requiring hospital admission was 0.98 per patient-year in CFVAD patients. CONCLUSIONS: Contemporary CFVADs have enabled advanced heart failure patients to await HT safely with an improved quality of life. The advent of CFVAD has also shifted their preimplantation condition to a less sick status. CFVADs were the safest, most reliable circulatory support devices for long-term waiting periods for the BTT indications.