Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report

We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management. Received: November 5, 2001 / Accepted: February 5, 2002     

Systemic AL amyloidosis in Gaucher disease. A case report and review of the literature

Chronic Gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic AL amyloidosis diagnosed by bone marrow diffuse plasmacytosis, serum monoclonal IgA-lambda, severe total proteinuria with monoclonal IgA-lambda, Bence-Jones-lambda and amyloid deposits in bone marrow, liver, spleen and kidney biopsy specimens. Treatment with melphalan and prednizolon has dramatically decreased both levels of serum M-IgA and proteinuria and also improved the clinical symptoms of amyloidosis. He died from restrictive cardiac disease 30 months after the diagnosis of amyloidosis. Previously reported cases of GD in association with AL amyloidosis are reviewed.     

瓣膜性心脏病伴缺血性心脏病的外科治疗

目的 :总结瓣膜性心脏病伴缺血性心脏病手术治疗的早期效果和经验 ,以期提高疗效。方法 :11例患者中 ,行主动脉瓣置换术 4例 ,二尖瓣置换术 2例 ,二尖瓣和主动脉瓣双瓣置换术 1例 ,主动脉带瓣管道置换 2例 ,二尖瓣成形术 1例和三尖瓣成形术 1例 ;搭 1支桥 4例 ,搭 2支桥 1例 ,3支桥 3例 ,4支桥 3例 ,平均 (2 .5±1.3)支。结果 :11例无手术早期死亡 ,痊愈出院 ;随访 2～ 16 (平均 6 .3)个月 ,心绞痛症状消失 ,心功能明显改善。结论 :对年龄 >5 0岁瓣膜病患者或具有冠心病高危因素患者 ,应行冠状动脉造影检查 ;彻底纠正心脏病变 ,加强心肌保护 ;妥善处理术后并发症 ,手术疗效满意     

The eyes are the window to the heart: one case of cardiac amyloidosis with eyelid swelling as the initial symptom

Cardiac amyloidoses (CA), a disease caused by the precipitation of amyloid in myocardial extracellular matrix, is difficult to diagnose due to lack of specificity of the clinical manifestations, and can be easily misdiagnosed. The patient was a 59-year-old man showed symptoms of double eyelid swelling and chest tightness without obvious causes. Electrocardiograph (ECG) for many times reveal low voltage in limb leads and poor progression of R waves in V1-V3. Furthermore, the levels of brain natriuretic peptide (BNP) and cardiac Troponin I (cTnI) are increased. The results of echocardiography suggested symmetric thickening of the left ventricle (IVS: 12mm, LVPW: 12mm), granular echoes in myocardium, left ventricular diastolic dysfunction (level III) (EF57%). Urine K light chain<0.1mg/l (0-7.1mg/L), urine λ light chain: 30.39mg/l increment (0-3.9 mg/L). The bone marrow results suggested no significant plasma cell dyscrasia. Since the gold standard for CA diagnosis is histopathological examination, skin and muscle biopsies of both eyelids were completely examined, hematoxylin and eosin (HE) and Congo red staining, suggesting positive result of Congo red staining. The results of polarized light examination suggested the presence of birefringence of apple green. Immunofixation electrophoresis was carried out for the serum, Bence-Jones protein electrophoresis was carried out for the urine sample, and results suggested M-proteinemia of LAM. The initial symptom of this case was manifested in the eyelid, after excluding orbital disease, coronary heart disease, lung disease and hypertrophic cardiomyopathy, the patient was diagnosed as cardiac amyloidosis through eyelid biopsy, fully demonstrating that “the eyes are the window to the heart”.     

Wild type transthyretin cardiac amyloidosis in a young individual: A case report

Rationale:Senile systemic amyloidosis, a disease of elderly is caused by amyloid deposition of wild-type transthyretin. The symptoms often overlap with other heart diseases. Hence it is either misdiagnosed or considered as a normal aging process in majority of cases.Patient concerns:We present a young patient of wild-type transthyretin amyloidosis, contradicting its only senile presence. The 34-year-old man presented with dyspnoea on exertion. He was suffering from hypertension for consecutive 3 years.Diagnosis:Echocardiography demonstrated left ventricular hypertrophy with reduced global longitudinal strain and apical sparing. Congo red staining and immuno-histochemical staining of the abdominal fat biopsy confirmed transthyretin amyloid deposition. Genetic analysis revealed absence of any mutant variant/s of transthyretin gene, confirming wild-type transthyretin amyloidosis.Intervention:A combination of amlodipine 5 mg, telmisartan 40 mg, and chlorthalidone 12.5 mg once daily was given to control the blood pressure of the patient.Outcome:Blood pressure was controlled but he continued to have exertional dyspnoea. The patient expired in December 2019.Lessons:A systematic diagnosis for wild type transthyretin amyloid cardiomyopathy (ATTR-CM) shall be considered in young cardiac patients suffering from cardiac distress with unknown etiology.     

Evaluation of ischemic heart disease and viability by cardiac MRI

In ischemic heart disease, cardiac MRI, besides being the gold standard for evaluation of quantitative ventricular function, enables evaluation of myocardial wall thickness, T2-weighted imaging for myocardial edema and infarct quantification and transmurality. Delayed hyperenhancement sequences are highly predictive of scar formation, being associated with myocyte necrosis. The extent and transmurality of delayed hyperenhancement has prognostic implications and is inversely proportional to the degree of functional recovery after acute myocardial infarction. A greater transmural extent of infarction (eg, hyperenhancement involving >50% of the wall thickness) can predict regions that are less likely to improve in function after therapy. The ultimate focus of MRI in ischemic heart disease is in diagnosis, quantification of myocardium at risk, salvageable myocardium, perfusion defects and differentiation of viable myocardium from non viable myocardium to enable prognostication.     

老年慢性肺原性心脏病合并冠心病临床分析

目的：探讨老年慢性肺心病合并冠心病的的临床特点。方法：回顾分析福建省老年医院2002年1月至2006年12月期间82例老年慢性肺心病合并冠心病患者（A组）和195例老年单纯慢性肺心病患者（B组）的临床资料，比较分析两组患者的临床特点。结果：两组比较是易患因素高血压、糖尿病和肥胖比例有显著差别（P〈0．05），临床表现陈旧性心肌梗塞史、完全性左束枝传导阻滞（LBBB）、心界向左或向左下扩大、持续缺血的ST—T改变、Ⅱ～Ⅲ度房室传导阻滞、电轴右偏和重度右心衰竭有显著差别（P〈0．05）。结论：慢性肺心病患者若有陈旧性心肌梗塞史、高血压、糖尿病、肥胖或LBBB、心界向左或左下扩大应考虑合并冠心病。     

心肌淀粉样变性的临床特点

目的　心肌淀粉样变性 ( cardiac amyloidosis CAL)是临床上相对少见的疾病 ,对其临床特点认识不足。本文分析CAL患者的临床特征 ,为正确诊断疾病奠定基础。方法　对近 2年来确诊为 CAL患者的临床、心电图、超声心动图和病理学特点进行了分析。结果　 3例患者确诊为 CAL。患者为老年 ( x=70岁 ) ,临床表现为肾病综合征 ( 3例 )和充血性心力衰竭 ( 2例 )。心电图显示肢体导联低电压 ( 3例 ) ,胸前、肢体导联异常 Q波 ( 3例 )。心肌电压和心肌团块比值下降。超声心动图显示左心室后壁和室间隔增厚 ( 3例 ) ;左心室舒张内径减小 ( 2例 ) ;左心房增大 ( 3例 ) ;E/ A比值倒置 ;心内膜闪耀的颗粒样物质 ( 1例 ) ;心包积液 ( 2例 ) ;左心室收缩功能正常 ( 3例 )。肾脏病理显示基底膜、小动脉大量微细纤维淀粉样物质沉积。结论　老年充血性心力衰竭、心脏舒张功能受损同时合并肾病综合征等提示 CAL的可能。当心脏外病理确诊为淀粉样变性后 ,心电图和超声心动图的特异改变可帮助确诊 CAL     

Aspartate aminotransferase and mortality in patients with ischemic heart disease

Background and aimsEvidence on the association between aspartate aminotransferase (AST) activity and mortality of patients with ischemic heart disease (IHD) is limited. We investigated whether there is an association between AST activity and mortality in IHD patients.Methods and resultsThe study included 6857 patients with coronary angiography-proven IHD and AST activity within the reference range. AST activity measurements were available in all patients. The primary outcome was 3-year cardiac mortality. Patients were categorized in groups according to the AST activity tertiles: a group with AST within the 1st tertile (AST < 17.0 U/L), a group with AST within the 2nd tertile (AST > 17–24.5 U/L) and a group with AST within the 3rd tertile (AST > 24.5 U/L). Cardiac death (n = 297) occurred in 109, 69 and 119 patients in the 1st to 3rd AST tertiles (Kaplan–Meier estimates of mortality: 5.3%, 3.6% and 5.9%; univariable hazard ratio [HR] = 1.75, 95% confidence interval [CI] 1.30–2.36, P < 0.001 for tertile 3 vs. 2; HR = 1.13 [0.87–1.46], P = 0.370 for tertile 3 vs. 1; and HR = 0.65 [0.48–0.87], P = 0.004 for tertile 2 vs. 1). The association between AST and cardiac mortality was U-shaped. AST values <15 U/L (HR = 1.118 [1.009–1.238]) and >23 U/L (HR = 1.029 [1.003–1.056]) were associated with higher cardiac mortality compared with the reference value (21 U/L). After adjustment, the association between AST and cardiac mortality was attenuated (P = 0.133) but remained non-linear (P = 0.047).ConclusionsIn patients with IHD, AST activity was associated with the risk of cardiac mortality with a U-shaped relationship. After adjustment, the association between AST and mortality was attenuated.     

A review of troponins in ischemic heart disease and other conditions

Measuring cardiac troponin (cTn) I and T levels is currently considered to be a cornerstone for making the diagnosis of acute coronary syndrome (ACS).Based on current literature, cTnI and cTnT are known to be very sensitive and specific for myocardial damage, regardless of the underlying cause. Lately, it has been found that cTns can be elevated and reflect worse prognoses in many situations where ACS is excluded. Such information can affect the validity of cTns as markers for ACS without classic symptoms. This may call for a revision of the troponin cutoff values to make a diagnosis of ACS. Furthermore, it opens a new field of study to determine appropriate management of patients with elevated cTn levels in whom ACS has been excluded.     

Hemodynamic effects of sildenafil in patients with stable ischemic heart disease

Background: Recently the new specific phosphodiesterase-5 inhibitor sildenafil was introduced into therapy for erectile dysfunction. The hemodynamic effects of sildenafil may be potentially hazardous for patients with cardiac disease. Sildenafil has been reported to augment the hypotensive effects of nitrates. There is sparse information regarding the systemic and pulmonary hemodynamic effects of a single oral dose of sildenafil in patients with stable angina. Methods: Male patients referred for coronary angiography with diagnosis of chronic stable angina were enrolled in this study to assess the acute hemodynamic effects of sildenafil. Patients receiving long-acting or sublingual nitrates for the last 6 h before the study were excluded. Hemodynamic measurement were taken during right and left heart catheterization in the basal state and 60 min after 50 mg of oral sildenafil. Results: Twelve patients (age 53±7 years) were studied. All had stable angina CCS class II or III. Four had previous myocardial infarction. By coronary angiography, seven patients had at least one coronary artery with >70% stenosis, four had at least one with 50–70% stenosis, and one had only intimal irregularities. There were no significant effects of sildenafil on systemic or pulmonary arterial pressure, left ventricle endiastolic pressure, cardiac output, and systemic or pulmonary vascular resistance (P>0.05 for all). No adverse events were observed. Conclusion: A single oral dose of sildenafil had no significant hemodynamic effect in supine patients with stable angina. Isolated administration of sildenafil does not appear to be associated to adverse cardiovascular effects.     

缺血性心脏病基因和细胞治疗的研究概况

基因和细胞治疗能有效地保护高危心肌,减少心肌炎症反应,挽救缺血、梗死心肌,并且已经初步应用于临床,成为目前治疗冠心病有潜力的方法,二者的联合治疗可能更有希望。     

误诊为冠心病的甲状腺功能减退性心脏病一例

1病例资料 患者男性,62岁,因“反复胸闷1年,加重1月”入院。胸闷位于心前区,本人手掌大小范围,伴冷汗,无心悸、气促、抽搐、晕厥,持续3～5min后稍缓解,伴肌肉僵硬、疼痛,声音低哑。反复发作,多于劳累、感冒时发作,休息后可缓解。     

原发性淀粉样变病1例

病例患者,女,44岁。因腹胀6年,双下肢水肿2个月入院。2002年患者无明显诱因反复出现腹胀,无胸闷、胸痛、发热。2007年8月患者出现厌油、食欲减退,曾到当地医院行乙肝全套及胸片检查均正常。08年4月腹胀加重,曾多次在医院行B超、CT检查提示肝大。于08年7月厌油、纳差症状加重,     

Stable ischemic heart disease in the older adults

Ischemic heart disease (IHD) is caused by atherosclerotic and/or thrombotic obstruction of coronary arteries. Clinical spectrum of IHD expands from asymptomatic atherosclerosis of coronary arteries to acute coronary syndromes (ACS) including unstable angina, acute myocardial infarction (non-ST elevation myocardial infarction and ST elevation myocardial infarction). Stable IHD (SIHD) refers to patients with known or suspected IHD who have no recent or acute changes in their symptomatic status, suggesting no active thrombotic process is underway. These patients include those with i) recent-onset or stable angina or ischemic equivalent symptoms, such as dyspnea or arm pain with exertion; ii) post-ACS stabilized after revascularization or medical therapy; and iii) asymptomatic IHD diagnosed by abnormal stress tests or imaging studies. This review summarizes clinical features and management of SIHD in the older adult. ACS in older adults is not considered in this review.     

Cardiac amyloidosis: A report of two cases

Cardiac amyloidosis is a manifestation of multisystem disorder. The condition is rare, difficult to diagnose and invariably fatal. We report 2 cases of amyloidosis associated with plasma cell dyscrasia. A high index of clinical suspicion, echocardiographic clues (ventricular thickening, diastolic dysfunction, biatrial enlargement) and elevated cardiac biomarkers led to the diagnosis. Early institution of amyloid specific treatment should be the aim. Cardiac treatment is supportive and results are often disappointing.     

The long-term effects of individual cardiac rehabilitation in patients with coronary artery disease

BackgroundThe positive effects of cardiac rehabilitation have been repeatedly described and are well-known over the short- and middle-term periods. However there is less knowledge about long-term outcomes in patients with chronic stable coronary artery disease.AimThe aim of this study was to evaluate the long-term outcome of individual cardiac rehabilitation in patients with coronary artery disease.MethodsOne hundred fifty-two patients with stable coronary artery disease were retrospectively divided into two groups according to their adherence to individual physical activity recommendations, regardless of their participation in guided cardiac rehabilitation training. The IT+ group which participated in individual exercise programmes according to recommendations was compared with patients who declined these activities (the IT− group). The median follow-up period was 12.7 years.ResultsThe individual training had no long-term effect on survival after being checked for other possible contributing factors, but the multivariate analysis showed a significant association with the occurrence of cardiac events like myocardial infarction, unstable angina, coronary revascularization and hospitalization for heart failure: HR (95% CI) 0.51 (0.30–0.89); p = 0.017.ConclusionHome based cardiac rehabilitation and regular physical activity significantly improve long-term cardiac morbidity in patients with coronary artery disease.     

Prevalence of cardiac amyloidosis among elderly patients with systolic heart failure or conduction disorders

Objective: Cardiac amyloid infiltration can lead to systolic heart failure (HF) or to conduction disorders (CD). Patients with transthyretin (ATTR) amyloidosis are particularly exposed. We sought to determine the prevalence of ATTR and AL among patients >60?years admitted with CD or unexplained systolic HF and increased wall thickness.

Materials and Methods: We studied 143 patients (57% males, 79?±?9?years) with HF (N?=?28) or CD requiring pacemaker implantation (N?=?115). In total, 139 (97%) patients (28 with HF and 111 with CD) underwent ^99mTc-DPD scintigraphy to detect ATTR, and 105 (73%; 19 HF and 86?CD) underwent AL screening.

Results: Five patients (4%; 95%CI:0–7%) exhibited wild-type ATTR (ATTRwt) amyloidosis, 2 (2%; 95%CI:0–4%) had CD and 3 (11%; 95%CI:0–23%) HF. No patient showed AL. The 2 ATTRwt patients with CD were previously asymptomatic, did not show classical ECG signs and exhibited mild LV hypertrophy with preserved LVEF. By contrast, all ATTRwt patients with HF had ECG and echocardiographic signs of amyloid. During a mean follow-up of 18?±?11?months, 3(60%) patients with ATTRwt amyloidosis (1?CD and 2 HF) and 14(10.4%) without died.

Conclusion: Prevalence of ATTRwt amyloidosis in patients with CD requiring pacemaker is low. Although, additional studies are needed, prevalence seems to be higher in elderly patients with systolic HF.