Long term results of heart transplantation in patients with amyloid heart disease

OBJECTIVE—To determine the outcome of heart transplantation for end stage amyloid heart disease in patients treated at a single centre.
DESIGN—Records of all patients with amyloid heart disease who underwent heart transplantation were examined to determine survival, graft involvement by amyloid, the course of systemic amyloid disease, and the cause of death.
PATIENTS—10 patients, mean (SD) age 54 (8) years, received transplants in the 13 year period 1984 to 1997.
RESULTS—Two patients, both with AL amyloid (primary systemic amyloidosis), died perioperatively. Mean follow up in the remaining eight patients was 49.9 (39.5) months (range 3-116 months). Amyloid deposits in the grafts became evident histologically in five patients with AL amyloid at 5, 11, 12, 28, and 30 months after transplantation, and in one patient with familial amyloid at 60 months. Echocardiography showed no evidence of left ventricular systolic impairment at the time of recurrence. Seven patients died, at 3, 11, 26, 32, 49, 85, and 116 months after transplantation; four of these deaths were related to amyloidosis. Actuarial survival at one and two years was 60% and at five years, 30%.
CONCLUSIONS—Heart transplantation for amyloid heart disease remains controversial because of the scarcity of hearts for transplantation, the systemic nature of amyloidosis, and the potential for amyloid deposition in the graft. Postoperative mortality was high (20%), reflecting extracardiac amyloid. Heart transplantation for end stage cardiac amyloidosis is feasible but, without treatment of the underlying process, it is a palliative procedure.


Keywords: heart transplantation; amyloid heart disease; heart failure     

替米沙坦联合辛伐他汀治疗慢性充血性心力衰竭的疗效

目的:观察替米沙坦联合辛伐他汀治疗慢性充血性心力衰竭(CHF)的疗效。方法:将132例CHF患者随机分为替米沙坦联合辛伐他汀治疗组(联合治疗组,44例)、辛伐他汀组(44例)和常规对照组(44例)3组,随访180d,观察治疗前后NYHA心功能分级,同时以超声动图检测左室舒张末期内径(LVEDD)、左室收缩末期内径(LVESD)、左室射血分数(LVEF)及E/A来评估治疗效果。结果:联合治疗组疗效优于辛伐他汀组和常规对照组(P0.05)。辛伐他汀组各指标均优于常规对照组,差异有统计学意义(P0.05)。LVEF(%)、E/A联合治疗组均优于辛伐他汀组,差异有统计学意义(P0.05)。结论:替米沙坦联合辛伐他汀治疗CHF比单用辛伐他汀的临床疗效更好。     

冠心病和充血性心力衰竭患者的心率变异性

本文采用标准差法及心率变指数法分析了冠心病、充血性心力衰竭及正常人各５０例的心率变异性，结果发现冠心病及充血性心力衰竭者的心率变异值显著低于正常人。心衰组中，心衰程度越重者，心率弈异赵低。且心率变异与心胸比值及ＰＥＰ／ＬＶＥＴ呈负相关，与心脏指数呈正相关。急性心肌梗死者的心率变趔氏于陈旧性心肌梗死和心绞痛者。２４例心肌梗死者查心室晚电位，晚电位阳性者的心率变异值显著低于晚电位阴性者，提示两者结合可     

慢性心力衰竭患者心率震荡检测及临床意义

目的观察慢性心力衰竭患者窦性心率震荡(HRT)现象的特征并探讨其临床意义。方法50例慢性心衰患者(心衰组)和30例非器质性心脏病患者(对照组)均接受24hHolter检查,分别计算HRT的初始值(TO)、震荡斜率(TS)及心率变异性的SDNN、SDANN、rMSSD值,并进行相关性分析。结果心衰组TO明显高于对照组(0.65±3.60%与-1.89±2.48%,P<0.01);心衰组TS明显低于对照组(2.96±1.23与10.24±4.47,P<0.001)。对照组TS与SDNN、SDANN、rMSSD的相关系数分别为-0.426、-0.385、-0.372(P均<0.05);心衰组TO与SDNN、SDANN、rMSSD的相关系数分别为0.489、0.465、0.436(P均<0.01),TS与SDNN、SDANN、rMSSD的相关系数分别为-0.745、-0.686、-0.597(P均<0.001)。结论慢性心衰患者中HRT现象明显减弱,TO与SDNN、SDANN、rMSSD值呈正相关,TS与SDNN、SDANN、rMSSD值呈负相关。     

Restoration of heart rate turbulence by titrated beta-blocker therapy in patients with advanced congestive heart failure: positive correlation with enhanced vagal modulation of heart rate

INTRODUCTION: Heart rate turbulence (HRT) is a powerful novel predictor for cardiovascular mortality. Chronic congestive heart failure is associated with abnormal HRT. Whether antiadrenergic beta-blocker therapy can restore control of HRT in patients with chronic congestive heart failure is unknown. METHODS AND RESULTS: A 24-hour Holter ECG recording was obtained before and 1 and 3 months after titrated addition of atenolol therapy in 10 consecutive patients with advanced congestive heart failure. Two parameters derived from HRT, turbulence slope (TS) and turbulence onset (TO), and time- and frequency-domain heart rate variability (HRV) parameters (SDNN, RMSSD, VLF, LF, HF) from 24-hour ECG were compared before and after beta-blocker therapy, together with the same parameters in age-matched normal control. Results showed that TS (3.1 +/- 2.2 vs 6.2 +/- 3.0; P = 0.001) and all HRV parameters were increased after 3 months of atenolol treatment. No changes in TO were evident (0.6 +/- 0.5 vs -0.2 +/- 1.3; P = 0.13). The improvement of TS and the vagally mediated parameters of mean R-R interval, RMSSD, and the HF component of HRV were positively correlated. CONCLUSION: Abnormal HRT caused by chronic congestive heart failure can be restored by beta-blocker therapy. The evolution of TS was positively correlated with measures of vagal modulation of heart rate.     

慢性心力衰竭患者心率震荡的检测及其临床意义

目的观察慢性心力衰竭患者窦性心率震荡（HRT）现象的特征并探讨其临床意义。方法48例慢性心力衰竭患者（心力衰竭组）和35例非器质性心脏病患者（对照组）均接受24h动态心电图检查，分别计算HRT的初始值（T0）、震荡斜率（TS）。结果心力衰竭组T0明显高于对照组[（0．76±3．42）％与（-1．61±2．05）％，P〈0．01）]；心力衰竭组TS明显低于对照组（2．53±1．21与9．56±4．03，P〈0．001）。结论慢性心力衰竭患者中HRT现象明显减弱。     

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.     

Cardiac Catheterization and Selective Angiography in Infants with a New Flow-Directed Catheter

Preliminary experience with a new flow-directed pediatric anglography catheter in 35 infants indicates that catheterization of all cardiac chambers and both great vessels can be accomplished without risk of perforation or major arrhythmia. Antegrade access to the aorta from the left ventricle via the foramen ovale reduced the need for retrograde arterial catheterization. The maneuverability of this balloon-tipped catheter coupled with the ability to perform safely selective anglography at any site entered establishes a unique advantage over standard cardiac catheters now in use. The success with this catheter in performing right and left heart studies and the safety in its use promise to significantly reduce the risk of mechanical and angiographic accidents during the intracardiac investigation of critically ill Infants with congenital heart disease.     

Heart transplant for dilated cardiomyopathy associated with polymyositis

Cardiac involvement is one of the most significant factors in the poor clinical outcome of polymyositis. The case of a 39 year old African American woman with polymyositis, cardiomyopathy, and severe heart failure who had orthotopic heart transplantation is described. Review of the literature reveals that cardiac manifestations of polymyositis are frequent and include conduction system abnormalities, myocarditis, cardiomyopathy, coronary artery atherosclerosis, valvar disease, and pericardial abnormalities.


Keywords: polymyositis; cardiomyopathy; heart failure; heart transplantation     

Hyperhomocysteinemia in patients with heart failure referred for cardiac transplantation: preliminary observations

BACKGROUND: Hyperhomocysteinemia is becoming recognized as a risk factor for cardiovascular disease, yet there are limited data on the prevalence of hyperhomocysteinemia in patients with heart failure. HYPOTHESIS: The purpose of this study was to examine the prevalence of hyperhomocysteinemia in patients with severe heart failure and to correlate serum homocysteine levels with factors that may affect homocysteine metabolism. METHODS: Serum homocysteine levels were measured at the time of cardiac transplant evaluation in 89 consecutive patients with severe heart failure. Homocysteine levels for patients with ischemic cardiomyopathy (ICM) were compared with levels obtained in patients with nonischemic cardiomyopathy (NICM), and homocysteine levels were correlated with demographic and hemodynamic parameters as well as functional status. RESULTS: The mean plasma homocysteine level was increased (14.3 +/- 5.3 micromol/l, normal <9.0 micromol/l) and was equivalent between patients with ICM versus NICM (14.7 +/- 5.8 micromol/l vs. 13.8 +/- 4.5 micromol/l, p = 0.44). Elevated homocysteine levels were seen in a large proportion (89%) of patients and were equally common to patients with NICM (94%) and ICM (85%). Serum homocysteine levels correlated with serum creatinine (r = 0.51, p < 0.001), with a history of diabetes (p = 0.028), and with a history of peripheral vascular disease (p = 0.045). Only 6% of patients were receiving folic acid therapy at the time of transplant referral. CONCLUSION: Hyperhomocysteinemia is common in patients with severe heart failure, and plasma homocysteine levels are uniformly elevated regardless of the etiology of heart failure. Elevated plasma homocysteine levels are likely a consequence of heart failure-related renal insufficiency.