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.

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.

Two cases of bronchial asthma after treatment with amiodarone.

Amiodarone is a highly effective antiarrhythmic agent for the prevention of life-threatening arrhythmias. Two cases are described of patients who developed bronchial asthma after treatment with amiodarone. The bronchial asthma resolved after the dose of amiodarone was decreased in both patients. To our knowledge, an association between amiodarone and severe bronchial asthma has previously been reported only once in the medical literature. Physicians should note that amiodarone may cause bronchospasm in susceptible 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 prevention of recurrent ventricular fibrillation by intravenous isoproterenol in a patient with Brugada syndrome.

Intravenous administration of isoproterenol restored the ST-segment configuration to nearly normal in the right precordial leads and completely prevented spontaneous VF attacks in a patient with Brugada syndrome. The formation of a Brugada-type ECG has been attributed to the transmural dispersion of repolarization of the right ventricular epicardium and related to modulation of the autonomic nervous system. Our case may provide clues to the pathophysiological mechanism of this syndrome.

Biventricular hypertrophic cardiomyopathy with right ventricular outflow tract obstruction associated with Noonan syndrome in an adult.

This report describes an adult patient with Noonan syndrome accompanied by biventricular hypertrophic cardiomyopathy causing isolated right ventricular outflow tract obstruction. Biventricular hypertrophic cardiomyopathy causing right- and/or left-side outflow tract obstruction, as well as valvular pulmonary stenosis, is relatively common in infants with Noonan syndrome. However, this condition without a dysplastic pulmonary valve, or indeed any polyvalvular dysplasia, is rare in adults with Noonan syndrome. Treatment with a beta-adrenergic receptor blocking agent improved the patient's symptoms. Because neither the etiologic and prognostic relationship nor the genetic linkage between hypertrophic cardiomyopathy associated with Noonan syndrome and non-syndromic hypertrophic cardiomyopathy is clearly defined, clinicopathological findings and further follow-up may provide important evidence for the pathogenesis of hypertrophic cardiomyopathy.

[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.

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.

Familial sick sinus syndrome with atrioventricular conduction disturbance.

A family with sick sinus syndrome is presented: a mother and her 2 daughters affected with sinus node dysfunction. Electrophysiological studies revealed abnormal atrioventricular conduction in the 2 daughters. All 3 individuals were implanted with a permanent pacemaker to relieve their symptoms. Endomyocardial biopsy from the right ventricle of one daughter showed disarrangement, degeneration, myocyte necrosis and interstitial fibrosis suggestive of myocardial disease.

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.

Surgical treatment of patients with psychiatric disorders: a review of 21 patients.

Between January 1985 and September 1994, 21 patients with psychiatric disorders underwent various forms of surgery at our hospital. There were 12 men and 9 women with an average age of 57.6 years. The coexisting psychiatric disorders were schizophrenia in 15 patients, depression in 2, dementia in 2, mental retardation with epilepsy in 1, and Parkinson's disease in 1. All the patients had been receiving neuroleptic medications for a long period. The indications for surgery were: cholelithiasis in 6 patients, acute appendicitis in 4, perforation of the small intestine in 3, incarceration of an inguinal hernia in 2, and esophageal cancer, stomach cancer, bleeding from a gastric ulcer, perforation of a duodenal ulcer, strangulating ileus, and burns in 1 patient each, respectively. All of the patients who underwent elective surgery were given epidural anesthesia with or without general anesthesia. Antipsychotic medications were given until just prior to surgery and recommenced concurrent with the first meal. Abnormal behavior was observed in 11 patients (52.4%) postoperatively, but all the patients were discharged in accordance with recovery from their surgical disorder. Intra- and postoperative hypotension resistant to intravenous catecholamine administration was recognized in 9 patients (42.9%), and this peculiar complication should be borne in mind when patients with psychiatric disorders require surgical management.

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.

Heart rate variability in patients with familial amyloid polyneuropathy.

The purpose of this study was to evaluate heart rate variability (HRV) in patients with familial amyloid polyneuropathy (FAP) using the time- and frequency-domain analysis. The study population consisted of 19 patients with FAP, and 19 age and sex matched normal volunteers. The 24-hour Holter recordings of all subjects in sinus rhythm and off medication were analyzed. Five time-domain indices of HRV were computed. The frequency component of HRV was calculated by fast Fourier transform analysis of the RR intervals. The power spectrum of the low frequency (LF) between 0.04-0.15 Hz and high frequency (HF) between 0.15-0.40 Hz and the LF/HF ratio was calculated. Global measures of HRV including the standard deviation of the mean of RR intervals (SDNN) and the standard deviation of 5-minute mean RR intervals (SDANN) were decreased in patients with FAP. Specific vagal influences on HRV including the proportion of RR intervals more than 50 milliseconds different (pNN50) and the HF power on spectral analysis were less in patients with FAP. LF power and LF/HF ratio were more decreased in patients with FAP at the advanced stage than at the early stage. In conclusion, HRV was significantly decreased in patients with FAP at the early stage, and sympathetic activity was more decreased in patients at the advanced stage. These findings suggest that the decrease of the HRV is an indicator of this disease and the power spectral analysis of the HRV is beneficial in assessing the severity of the autonomic dysfunction.

Radiofrequency catheter ablation of supraventricular tachycardias in patients with hypertrophic cardiomyopathy.

Supraventricular tachycardias, as well as ventricular tachycardia, are critical patients with hypertrophic cardiomyopathy. Anti-arrhythmic agents often induce arrhythmias, known as pro arrhythmia. Radiofrequency catheter ablation has become a well-established therapy for the treatment of supraventricular tachycardia. The reported success rate is over 90%, although catheter mapping in the left ventricle is difficult in patients with hypertrophic cardiomyopathy because of the marked left ventricular wall thickening associated with bizarre myocardial hypertrophy with disorganization, the abundant capillary muscle networks, and annoying ventricular arrhythmias. This report documents catheter mapping technique of radio frequency ablation in patients with hypertrophic cardiomyopathy and supraventricular tachycardias.

Diagnostic clues from the surface ECG to identify idiopathic (fascicular) ventricular tachycardia: correlation with electrophysiologic findings.

INTRODUCTION: An RS interval > 100 msec in precordial leads has been recently described for the diagnosis of ventricular tachycardia (VT). The aim of this study was to assess the value of this criterion when applied to patients with right bundle branch block pattern, left-axis deviation (fascicular) VT sensitive to verapamil. METHODS AND RESULTS: Eleven patients (mean age 31 +/- 11 years; range 16 to 51) had a mean heart rate of 164 +/- 37 beats/min (range 107 to 230) during VT. The QRS complex axis was -92 degrees +/- -15 degrees (range -80 to -115). The mean QRS duration was 121 +/- 9 msec (range 105 to 140). The mean RS interval was 67 +/- 9 msec (range 60 to 80). Fusion beats were present in 2 patients (18%), and AV dissociation confirmed by electrophysiologic study was found on ECG in 8 (73%) of 11. During tachycardia, the QRS-H' interval was 19 +/- 10 msec (range 10 to 30) in 6 of 11 patients. In seven patients, a fast, unique (or double) presystolic potential lasting 32 msec (range 12 to 40) occurring before the onset of the QRS complex was found at the site of origin of VT, localized in the inferior apical left ventricular septum. In all cases, VT was successfully treated by catheter ablation. CONCLUSION: A wide QRS complex tachycardia with right bundle branch block and left-axis deviation sensitive to verapamil observed in a young patient without structural heart disease should not be confused with supraventricular tachycardia with aberrancy but rather suggests the presence of fascicular VT. As opposed to VT associated with structural heart disease, the RS interval is < 80 msec in all precordial leads in all cases. Independent of this parameter, AV dissociation detectable on surface ECG has a sensitivity of 73%, which increases to 82% in the presence of fusion beats.

Time- and frequency-domain analyses of the signal-averaged ECG in patients with arrhythmogenic right ventricular dysplasia.

BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by recurrent ventricular tachycardia of right ventricular origin and a cardiomyopathy with hypokinetic areas involving the free wall of the right ventricle. Subjects have a risk of sudden cardiac death, particularly during sports and strenuous exercise. Routine clinical examinations may be normal, but fragmented or delayed electrograms are usually recorded in the right ventricle of these patients. However, the frequency with which late potentials are detected by conventional time-domain analysis of the signal-averaged ECG (SAECG) is not high. This study evaluated the usefulness of the frequency-domain analysis of the SAECG in addition to the conventional time-domain analysis for a screening test to detect patients with ARVD. METHODS AND RESULTS: SAECG was recorded by using a bipolar X, Y, and Z lead system in 28 patients with ARVD (mean age, 38 +/- 13 years) and 35 age-matched normal subjects (mean age, 35 +/- 11 years). The conventional time-domain analysis of the SAECG was performed at two different high-pass filter settings, 25 and 40 Hz, and the low-pass cutoff frequency was fixed at 250 Hz. The fast-Fourier transform analysis of SAECG was performed using a Blackman-Harris window. Area ratio 1 (area of 20 to 50 Hz)/(area of 0 to 20 Hz) and area ratio 2 (area of 40 to 100 Hz)/(area of 0 to 40 Hz) were calculated. In the conventional time-domain analysis, 20 (71%) and 18 (64%) patients had positive criteria at filter settings of 25 and 40 Hz, respectively. In the frequency-domain analysis, 18 (64%) and 20 (71%) patients had abnormal values in area ratios 1 and 2, respectively. Combining the time- and frequency-domain analyses, all patients were judged positive, with a sensitivity of 100% and a specificity of 94%. CONCLUSIONS: Each result of the time- and frequency-domain analyses revealed that both methods had equivalent value. Combining the two domain analyses improved the sensitivity without reducing the specificity. These findings suggest that combining the time- and frequency-domain analyses of the SAECG may be useful as a screening test to detect patients with ARVD.

Effects of heart rate on phasic coronary blood flow pattern and flow reserve in patients with normal coronary arteries: a study with an intravascular Doppler catheter and spectral analysis.

To assess the effects of pacing-induced tachycardia on phasic coronary blood flow pattern and flow reserve of left anterior descending artery, we examined 16 patients with chest pain and angiographically normal coronary arteries by using an intravascular Doppler catheter with spectral analysis of the velocity signal. The heart rate was increased from a mean of 68 +/- 11 beats/min during sinus rhythm to 100 beats/min and again to 120 beats/min. Cross-sectional area of the epicardial artery and resting systolic and diastolic coronary blood flows increased progressively, resulting in an elevation of total coronary flow from 142 +/- 54 ml/min during sinus rhythm to 190 +/- 66 ml/min at 100 beats/min (p < 0.05) and to 219 +/- 69 ml/min at 120 beats/min (p < 0.01). During maximal hyperemia with intracoronary injection of 10 to 12 mg of papaverine, there was an increase in the systolic coronary blood flow with a decrease in the diastolic flow, resulting in no significant change in the total flow. These alterations led to progressive reductions in coronary flow reserve from 3.9 +/- 0.7 during sinus rhythm to 2.9 +/- 0.9 at 100 beats/min (p < 0.01) and to 2.3 +/- 0.3 at 120 beats/min (p < 0.001). Thus careful consideration should be given to the effects of heart rate when phasic coronary blood flow pattern and flow reserve are assessed.

Body surface potential maps in patients with familial amyloid polyneuropathy.

The purpose of this study was to evaluate the characteristics of body surface potential maps in patients with cardiac amyloidosis. The study population consisted of 30 patients with familial amyloid polyneuropathy and 50 age-matched normal volunteers. The patients were classified into one of the following three stages: stage I, peripheral neuropathy limited to the lower limbs; stage II, neuropathy involving both the lower and upper limbs; and stage III, bedridden because of extensive progressive neuropathy. Electrodes for the body surface potential maps were placed at 87 points (59 anterior and 28 posterior) on the chest. To analyze these body surface electrocardiograms, isopotential maps, isochrone maps, and isointegral maps were used. The mean values of the positive potential were significantly lower in the advanced stage (1.9 +/- 0.2 mV in stage I, 1.0 +/- 0.2 mV in stage II, and 0.7 +/- 0.2 mV in stage III). Prolongation of ventricular activation time was observed on the anterior and lateral chest. The mean QRST isointegral maps of the patients in the advanced stage of cardiac amyloidosis showed a large negative area over the anterior and left lateral chest, the positive areas were small and their potentials were very low. In addition, 18 (60%) of the 30 patients had a multipolar pattern in the QRST isointegral maps. The changes of the body surface potential maps correlated with clinical staging and echocardiographic findings.