Left ventricular twisting and untwisting motion in childhood cancer survivors

Background: Anthracycline has been shown to degrade titin that plays a role in myocardial twisting and untwisting. This study aimed to test the hypothesis that left ventricular (LV) twisting and untwisting motion may be altered in children after anthracycline therapy. Methods: Thirty‐six childhood leukemia survivors aged 15.6 ± 5.5 years and 20 healthy controls aged 16.8 ± 7.7 years (P = 0.54) were studied. LV twisting and untwisting motion was determined using speckle tracking imaging, whereas LV ejection fraction and systolic and diastolic mitral annular velocities were determined respectively by three‐dimensional and tissue‐Doppler echocardiography. Results: Compared with controls, patients had significantly lower LV ejection fraction (P = 0.01) but similar systolic and diastolic mitral annular velocities (all P > 0.05). Their peak LV torsion (P = 0.003), systolic twisting velocity (P < 0.001), and diastolic untwisting velocity (P = 0.04) were significantly lower than controls, which could be attributable to their reduced apical rotation (P = 0.03) and apical untwisting rate (P = 0.002). For the whole cohort, LV systolic torsion and twisting velocity correlated significantly with apical untwisting rate (P < 0.001) and LV diastolic untwisting velocity (P < 0.001). In patients, none of the twisting or untwisting parameters were found to correlate with cumulative anthracycline dose (all P > 0.05). Twenty‐eight (78%) patients had LV ejection fractions ≥50%. Although their systolic and diastolic mitral annular velocities were similar to those of controls, their peak LV torsion (P = 0.005), apical untwisting rate (P = 0.01), and LV systolic twisting velocity (P = 0.001) remained significantly lower. Conclusion: Impairment of LV twisting and untwisting motion is evident in children after anthracycline therapy, even in those with “normal” LV ejection fractions. (Echocardiography 2011;28:738‐745)     

Tissue Doppler and strain imaging: a new tool for early detection of cardiac amyloidosis

Using traditional echocardiography, the diagnosis of cardiac amyloidosis (CA) is often only possible in advanced stage when recommended therapies may have adverse effects. The aim of our study was to evaluate whether additional information can be derived from Tissue and strain Doppler imaging (TDI and SDI). Forty patients with systemic amyloidosis and 24 healthy subjects underwent traditional, tissue and strain Doppler echocardiography. Patients were classified having CA if mean wall thickness (mT), was half of the sum septum and posterior wall thickness, was ≥12 mm. The following parameters were evaluated: peak early diastolic velocity (Em) as index of ventricular relaxation, mitral E-wave to Em ratio (E/Em) as index of left ventricular (LV) filling pressure and mean LV strain peak curves (mSt) as global long-axis contraction index. In non cardiac amyloidosis (NCA), both Em and mSt were lower than in age matched controls (p < 0.01, p < 0.05, respectively) and higher than in CA (p < 0.01 and p < 0.01, respectively). Both Em and mSt were related to mT (p < 0.001). A significant (p < 0.01) nonlinear relation was observed between plasma terminal of pro B-natriuretic peptide and mT, Em, E/Em and mSt. TDI and SDI are able to detect amyloid myocardial involvement in such an early stage that cannot be evidenced by using traditional echocardiography.     

Myocardial Edema and Prognosis in Amyloidosis

Background

Prognosis in light-chain (AL) and transthyretin (ATTR) amyloidosis is influenced by cardiac involvement. ATTR amyloidosis has better prognosis than AL amyloidosis despite more amyloid infiltration, suggesting additional mechanisms of damage in AL amyloidosis.

Non-invasive predictors of survival in cardiac amyloidosis

BACKGROUND: Patients with cardiac amyloidosis (CA) have increased mortality. AIMS: Clinical, electrocardiographic, and echocardiographic parameters were assessed for risk-stratification of CA. METHODS AND RESULTS: CA was confirmed by endomyocardial biopsy in 59 patients (54.8+/-1.2 years) with light-chain (n = 43) or transthyretin amyloidosis (n = 16). Six patients without CA served as controls (NCA). Clinical symptoms, electrocardiographic, and echocardiographic parameters were analyzed for prognostic significance. Of the patients with light-chain amyloidosis, 14 died and 2 underwent heart transplantation. 1-/3-year survival was 68%/63%. Survival depended on left ventricular function (LV-EF), LV mass, radius/wall thickness, septum thickness, low voltage pattern (LVP), conduction delay, NYHA class, and stem cell transplantation. A multivariate model only contained LV-EF and LVP; the beneficial effect of stem cell transplantation was cancelled out as this treatment was withheld in patients with highest cardiac risk. Survival was most limited if both risk factors occurred. Cardiac involvement in transthyretin amyloidosis showed better survival (2 deaths, 1-/3-year survival 91%/83%). Analysis of prognostic risk factor utility in all amyloid patients (light-chain and transthyretin) again revealed LVP and LV-EF, and aetiology of amyloidosis as independent survival parameters. CONCLUSION: Prognosis of CA is poor, but aetiology of amyloid, LVP, and LV-EF allows identification of patients at highest risk of death, who may require individual treatment approaches (heart transplantation prior to causative therapy).     

Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosis

BACKGROUND: Patients with AL amyloidosis often present with signs of congestive heart failure. AIM: This study was prospectively designed to assess the significance of RV dysfunction in AL amyloidosis. METHODS AND RESULTS: Seventy-four patients with biopsy proven AL amyloidosis underwent a thorough echocardiographic evaluation. A tricuspid annular plane systolic excursion (TAPSE)<17 mm was taken as marker of RV dysfunction. Plasma NT-proBNP determinations were performed in all cases. RV function was normal in 60 patients and reduced in 14 patients. Patients with RV dysfunction had thicker left ventricular (LV) walls (p<0.01), lower LV end-diastolic volumes (p<0.01), lower LV ejection fraction (p<0.01) and more frequently a restrictive LV filling pattern (p<0.01). RV dimensions and RV free wall thickness were not significantly different in the two groups. A thick interventricular septum and a reduced TAPSE were associated with high NT-proBNP levels (both p<0.01). Seven patients died during a median follow-up period of 19 months; TAPSE<17 mm was the only echocardiographic parameter associated with poor survival. CONCLUSION: In patients with AL amyloidosis, RV dysfunction is associated with more severe involvement of the left ventricle, higher plasma levels of NT-proBNP and with poor prognosis.     

Multi-Imaging Characterization of Cardiac Phenotype in Different Types of Amyloidosis

BackgroundBone scintigraphy is extremely valuable when assessing patients with suspected cardiac amyloidosis (CA), but the clinical significance and associated phenotype of different degrees of cardiac uptake across different types is yet to be defined.ObjectivesThis study sought to define the phenotypes of patients with varying degrees of cardiac uptake on bone scintigraphy, across multiple types of systemic amyloidosis, using extensive characterization comprising biomarkers as well as echocardiographic and cardiac magnetic resonance (CMR) imaging.MethodsA total of 296 patients (117 with immunoglobulin light-chain amyloidosis [AL], 165 with transthyretin amyloidosis [ATTR], 7 with apolipoprotein AI amyloidosis [AApoAI], and 7 with apolipoprotein AIV amyloidosis [AApoAIV]) underwent deep characterization of their cardiac phenotype.ResultsAL patients with grade 0 myocardial radiotracer uptake spanned the spectrum of CMR findings from no CA to characteristic CA, whereas AL patients with grades 1 to 3 always produced characteristic CMR features. In ATTR, the CA burden strongly correlated with myocardial tracer uptake, except in Ser77Tyr. AApoAI presented with grade 0 or 1 and disproportionate right-sided involvement. AApoAIV always presented with grade 0 and characteristic CA. AL grade 1 patients (n = 48; 100%) had characteristic CA, whereas only ATTR grade 1 patients with Ser77Tyr had characteristic CA on CMR (n = 5; 11.4%). After exclusion of Ser77Tyr, AApoAI, and AApoAIV, CMR showing characteristic CA or an extracellular volume of >0.40 in patients with grade 0 to 1 cardiac uptake had a sensitivity and specificity of 100% for AL.ConclusionsThere is a wide variation in cardiac phenotype between different amyloidosis types across different degrees of cardiac uptake. The combination of CMR and bone scintigraphy can help to define the diagnostic differentials and the clinical phenotype in each individual patient.     

Comparison of different types of cardiac amyloidosis by cardiac magnetic resonance imaging

Objectives: We sought to determine cardiac morphological and functional differences between light-chain (AL), mutant-type transthyretin (ATTRmt) and wild-type TTR (ATTRwt) amyloidosis using contrast-enhancement cardiac magnetic resonance imaging (CE-CMR). Finally, we attempted to establish the diagnostic and prognostic impact of these findings. Introduction: The most common forms of cardiac amyloid are AL and ATTR amyloidosis, but the clinical courses of these variants are quite heterogeneous. While CE-CMR is used to evaluate patients with cardiac amyloidosis, its ability to predict prognosis in these patients is debatable.

Methods: About 130 patients with cardiac amyloidosis (AL, n?=?62; ATTRmt, n?=?30, ATTRwt, n?=?33) were assessed by CE-CMR (cardiac morphology, cardiac function, late gadolinium enhancement).

Results: Left ventricular (LV) mass, basal and mid-ventricular maximal wall thickness, and thickness of the inter-atrial septum were higher in ATTRwt when compared to AL and ATTRmt amyloidosis. Tricuspid annular excursion was lower in ATTRwt amyloidosis than in AL amyloidosis. CE was observed in 94.6% of the patients (AL 80.6%; ATTRmt 90%; ATTRwt 87.9%) with significant differences in quality and intensity between the groups. Differentiation of amyloid types was achieved by combination of age, number of organs, the presence of inferolateral CE-CMR, thickness of inter-atrial septum and troponin T. Overall 1-year-survival rates were 93.3, 93.9 and 70.5% in ATTRwt, ATTRmt and AL amyloidosis, respectively. LV mass, mitral annular excursion and NT-proBNP in AL amyloidosis, LV mass maximal apical wall thickness and troponin T in ATTRwt amyloidosis, and finally NT-proBNP and renal function in ATTRmt amyloidosis were independent predictors of outcome.

Conclusions: This study demonstrates that CE-CMR can highlight morphological and functional differences between different types of cardiac amyloidosis. In addition, CE-CMR and cardiac biomarkers provide useful prognostic information in patients with cardiac amyloidosis.     

Reduced and delayed untwisting of the left ventricle in patients with hypertension and left ventricular hypertrophy: a study using two-dimensional speckle tracking imaging.

AIMS: Newly developed two-dimensional ultrasound speckle tracking imaging allows measurements of left ventricular (LV) rotation and twist. Because LV untwisting predominantly occurs during the isovolumic relaxation period, its assessment reflects the process of LV relaxation. The aim of this study was to examine whether LV hypertrophy (LVH) adversely affects LV untwisting and abnormalities in LV untwisting could become a novel marker in assessing LV relaxation abnormalities. METHODS AND RESULTS: We acquired basal and apical LV short-axis images in 49 hypertensive patients. Using two-dimensional strain software, a time-domain speckle tracking was performed, and the mean value of LV rotation was obtained at each plane. LV twist was defined as apical rotation relative to the base. In order to adjust for inter-subject differences in heart rate, the time sequence was normalized to the percentage of systolic and diastolic duration. The degree of LV untwisting was calculated as the percentage of systolic twist : untwisting = (TwistES-Twistt/TwistES) x 100, where Twistt is twist at time t and TwistES is twist at end-systole. Although peak systolic twist was not different, early diastolic LV untwisting and untwisting rate during isovolumic relaxation period was significantly delayed and reduced in parallel to the severity of LVH, as assessed by LV mass index. CONCLUSION: The observed delayed and reduced diastolic untwisting during the isovolumic relaxation period noted in hypertensive patients with LVH may contribute towards the LV relaxation abnormality. Two-dimensional speckle tracking imaging is a novel tool which can be used for the non-invasive assessment of LV relaxation.     

Feasibility of 68Ga-Labeled Fibroblast Activation Protein Inhibitor PET/CT in Light-Chain Cardiac Amyloidosis

BackgroundSystemic amyloid light chain (AL) amyloidosis is the most common type of amyloidosis, leading to cardiomyocyte necrosis and interstitial fibrosis. Gallium-68-labeled fibroblast activation protein inhibitor 04 (⁶⁸Ga-FAPI-04) has recently been introduced for imaging fibroblast activation in cardiac diseases. To date, cardiac fibroblast and cardiac amyloidosis (CA) phenotype activities have not been mapped.ObjectivesThe aim of this study was to evaluate the feasibility of ⁶⁸Ga-FAPI-04 positron emission tomography (PET)/computed tomography (CT) in assessing AL CA.MethodsThirty consecutive patients (mean age: 59.1 ± 7.7 years; 20 men, 10 women) with biopsy-proven AL amyloidosis were enrolled prospectively (including 27 with AL CA and 3 without AL CA). All patients underwent ⁶⁸Ga-FAPI-04 PET/CT (107.4 ± 26.5 MBq). Global standardized uptake values and left ventricular (LV) molecular volume were calculated in correlation to echocardiography (n = 30), cardiac magnetic resonance (n = 18), and clinical biomarkers. Subsequently, the patients were categorized as having patchy (PET-patchy), extensive (PET-extensive), and negative (PET-negative) patterns.ResultsOf all patients, 80% (24 of 30) showed increased LV uptake (PET-patchy [n = 4] vs PET-extensive [n = 20]), whereas 6 patients did not show visible myocardial uptake. Standardized uptake value ratio and LV molecular volume were significantly higher in the PET-extensive than the PET-patchy group (2.79 mL ± 1.22 mL vs 1.53 mL ± 0.66 mL [P = 0.045] and 198.3 mL ± 59.97 mL vs 127.8 mL ± 25.82 [P = 0.005], respectively). Additionally, ⁶⁸Ga-FAPI-04 uptake was significantly correlated with clinical biomarkers (Mayo stage and N-terminal pro–brain natriuretic peptide), interventricular septal thickness, left ventricular ejection fraction (LVEF), LV end-systolic volume, extracellular volume, and LV global strain (P < 0.05).Conclusions⁶⁸Ga-FAPI-04 PET/CT is feasible in detecting myocardial fibroblast activation in patients with AL CA in correlation with myocardial remodeling. It might provide complementary information on cardiac molecular characterization and staging of disease.     

Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy.

OBJECTIVES: We investigated the diagnostic accuracy of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis. BACKGROUND: Differential diagnosis between TTR-related and AL amyloidosis is often complex and time-consuming. METHODS: Patients under routine observation with TTR-related/AL systemic amyloidosis and echocardiographic evidence of cardiac involvement were studied with 99mTc-DPD scintigraphy. RESULTS: Patients with cardiac involvement of TTR-related (group A; n = 15) and AL (group B; n = 10) etiology were comparable for left ventricular mass and renal function. Heart and heart/whole-body tracer retention were significantly higher (p < 0.05) in group A as compared with group B and with 10 unaffected controls. At visual scoring, cardiac 99mTc-DPD uptake was present in all group A patients and absent in all group B patients; thus, using genotyping/immunohistochemistry as the reference technique, the accuracy of 99mTc-DPD scintigraphy for distinction of TTR-related and AL etiology was 100%. Cardiac 99mTc-DPD uptake was also absent among unaffected controls. Using echocardiography as the reference standard for recognition of cardiac involvement, sensitivity and specificity of scintigraphy were both 100% for group A patients; in group B, sensitivity was 0% and specificity was 100% (accuracy, 50%). Eleven patients with myocardial 99mTc-DPD uptake underwent 99mTc-methylene diphosphonate (99mTc-MDP) scintigraphy; all patients showed a 99mTc-MDP myocardial visual score of 0. CONCLUSIONS: Etiology is a third major cause--in addition to type of organ-involved (soft-tissue/heart) and tracer type--of scintigraphic variability in cardiac amyloidosis. This is a highly relevant consideration for future studies. We conclude that 99mTc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.     

Orthotopic Heart Transplant Facilitated Autologous Hematopoietic Stem Cell Transplantation in Light-Chain Amyloidosis

Initial manifestations of light-chain amyloidosis (AL) are variable and often result in missed or delayed diagnosis. Survival in AL patients depends mainly on the severity of cardiac involvement. Dominant stage-III cardiac involvement due to primary systemic amyloidosis precludes effective AL treatment and is associated with an average survival of only 3–4 months. The following paper discusses the benefits of orthotopic heart transplantation and autologous hematopoietic stem cell transplantation to improve survival in patients with progressive cardiac AL.     

The six‐minute walk test in patients with AL amyloidosis: a single centre case series

The six‐minute walk test (6MWT) has been widely used as an objective evaluation of functional exercise capacity and response to medical intervention in cardiopulmonary diseases. However, little is known about the 6MWT in evaluating patients with AL amyloidosis. We performed a retrospective study of 120 adults with systemic AL amyloidosis (60 with cardiac involvement and 60 without cardiac involvement) who had their initial evaluation at the Amyloidosis Center between 2013 and 2015 and had undergone 6MWT as a measure of functional exercise capacity. Forty‐seven patients with cardiac involvement and 41 patients without cardiac involvement were included in the final analysis. The six‐minute walk distances (6MWD) were 368 ± 105 m and 420 ± 116 m (mean ± SD), respectively (P = 0·03). Among AL amyloidosis patients with cardiac involvement, the 6MWD was associated with New York Heart Association class (P < 0·001), B‐type natriuretic peptide (P = 0·003) and overall survival (hazard ratio 0·381, 95% confidence interval 0·215–0·676, P = 0·001). In conclusion, the 6MWT is a valuable tool in assessing functional exercise capacity in patients with AL amyloidosis.     

Plasma hepatocyte growth factor is a novel marker of AL cardiac amyloidosis

Abstract

Background: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics.

Methods: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n?=?26); (2) transthyretin (ATTR) cardiac amyloidosis (n?=?7); (3) left ventricular hypertrophy (LVH) (n?=?45); (4) systolic heart failure (n?=?42); and (5) non-cardiac systemic amyloidosis (n?=?7). Biomarkers were measured in stored plasma samples. Biomarkers' discrimination performance in predicting AL cardiac amyloidosis (i.e., Concordance index) was reported. A survival analysis was used to explore the relationship between HGF levels and mortality among AL cardiac amyloidosis patients.

Results: HGF levels were markedly elevated in patients with AL cardiac amyloidosis (median?=?622, interquartile range (IQR): 299–1228?pg/mL) compared with the other groups, including those with non-cardiac systemic amyloidosis (median?=?134, IQR: 94–163?pg/mL, p?<?0.001). HGF was not a specific marker for ATTR amyloidosis. Gal-3 was elevated in all groups with amyloidosis but could not differentiate between those with and without cardiac involvement. There was no difference in IL-6 or VEGF between those with AL cardiac amyloidosis compared to other groups (p?=?0.13 and 0.057, respectively).

Conclusions: HGF may be a specific marker that distinguishes AL cardiac amyloidosis from other cardiomyopathies with similar clinical or morphologic characteristics. Further studies are necessary to determine whether HGF levels predict the likelihood of survival.     

Echocardiographic features of an atypical presentation of rapidly progressive cardiac amyloidosis

We present the case of a 66 year old male who presented with dyspnea and reduced exercise tolerance. Echocardiography demonstrated impaired left ventricular (LV) function and restrictive diastolic function with pronounced concentric left ventricular hypertrophy (LVH) without a history of hypertension and no aortic valve stenosis. Differential diagnostics of concentric LVH are discussed in detail. In the current case, cardiac amyloidosis (AL) amyloidosis was diagnosed and confirmed by serum amyloid P (SAP) scintigraphy and abdominal fat aspiration biopsy. This case shows the rapid decline in clinical condition with progression of cardiac involvement of AL. As discussed in detail, cardiac involvement in AL-amyloidosis generally denotes a poor prognosis, regardless of the method of treatment.     

Amyloidosis and cardiac involvement

BACKGROUND: Amyloidosis is a rare disease characterized by the extracellular accumulation of a protein polysaccharide complex: amyloid. Cardiac involvement may occur with or without clinical manifestations, and is considered as a major prognostic factor. AIM OF THE STUDY: Firstly, to analyze the clinical, electrocardiographic, radiological and echocardiographic features in a group of patients with extracardiac biopsy-proven amyloid infiltration and evidence of echocardiographic amyloid heart disease. Secondly, to compare the survival of amyloidosis patients, with or without cardiac involvement. PATIENTS AND METHODS: We retrospectively analyzed the main echocardiographic features of 47 patients with biopsy proven amyloidosis. No clinical, electrocardiographic, radiological or scintigraphic criterium were selective for cardiac involvement. Thirty patients with echographic features of amyloid heart disease were identified and compared to 17 patients without echographic features of amyloid heart disease. RESULTS: Amyloid disease with heart involvement was AL in 25/30 (83%) patients and occurred more commonly in middle age men (mean age: 53+/-11 years). The main clinical presentation was congestive heart failure (59%), but 37% of patients had no clinical cardiac features. The electrocardiogram was abnormal in 86% and the cardiac silhouette was enlarged on chest roentgenogram in 27% of patients. The main echocardiographic findings were: diffuse ventricular wall thickening in 21 patients (70%) and isolated septal wall thickening in 9 patients (30%); restrictive pattern of left ventricular (LV) diastolic function in 17 patients (57%); pericardial effusion in 12 patients (40%); impaired LV systolic function in 8 patients (27%); atrial enlargement in 8 patients (27%); characteristic granular sparkling of LV myocardium in 8 patients (27%); mitral and/or aortic valve thickening in 4 patients (13%). Cardiac symptoms developed in 72% of the non symptomatic patients having echocardiographic evidence of cardiac involvement. Twenty-five patients died during the study period and the death was due to cardiac disease in 76%. Median survival time was 36 months from time of amyloidosis diagnosis, and it was 23 months from time of amyloid myocardiopathy diagnosis. It shortened to 6 months when congestive heart failure appeared. CONCLUSION: Patients with a histologically proven amyloidosis should be examined by echocardiography, because cardiac involvement is frequently found in patients with no clinical symptoms, and non symptomatic patients having echocardiographic evidence of cardiac involvement will almost always develop cardiac symptoms. Survival actuarial study confirms the significant adverse influence of cardiac involvement in amyloidosis.     

Autologous transplantation for primary systemic AL amyloidosis is feasible outside a major amyloidosis referral centre: the Calgary BMT Program experience

Recent reports from large amyloidosis referral centers suggest that primary systemic AL amyloidosis patients treated with high-dose melphalan (HDM) and autologous stem cell transplantation (ASCT) survive longer than historical controls treated with less intensive chemotherapy, despite high transplant-related mortality (TRM) rates of >10%. A retrospective review was conducted to determine if the outcome of ASCT for AL amyloidosis at our institution was similar to that reported at major amyloidosis referral centers. Over a 7 year period, we treated a total of 15 AL amyloidosis patients with ASCT, including four with poor prognosis cardiac or multisystem involvement. No TRM was observed. Overall, 10 patients (67%) achieved a complete hematological response and four patients (27%) achieved a complete organ response. The 4-year event-free and overall survival rates were 60% (95% CI 32-89%) and 75% (95% CI 50-100%), respectively. One patient, who presented with cardiac failure and multiorgan involvement with colonic bleeding currently remains in complete remission 62 months post-ASCT. In conclusion, ASCT for primary AL amyloidosis can safely be performed at experienced transplant centers that are not associated with major amyloidosis referral centers, and is feasible for patients who have multisystem involvement, particularly for motivated patients with good performance status.     

Outcome of patients with systemic light chain amyloidosis with concurrent renal and cardiac involvement

Cardiac involvement in systemic light chain amyloidosis (AL) is generally associated with a worse outcome, especially if other organs are also involved. We sought to determine whether concurrent cardiac and renal involvement were associated with a worse outcome than either organ alone. We identified 129 patients with AL, who received high‐dose chemotherapy followed by autologous hematopoietic stem cell transplantation (auto‐HCT) at our institution between 1997 and 2014. Ninety‐nine patients had either renal (group 1: n = 62, 62%), cardiac (group 2: n = 20, 20%), or both cardiac and renal (group 3: n = 17, 17%) involvement. The overall hematological response rate (CR+VGPR+PR) post‐auto‐HCT in groups 1, 2, and 3 was 69%, 74% and 82%, respectively (P = 0.62). Overall, organ response in groups 1, 2, and 3 was 39%, 42%, and 70%, respectively. The median PFS from auto‐HCT in groups 1, 2, and 3 was not reached (NR), 13.3 and 21 months, respectively (P = 0.02). The median OS in groups 1, 2, and 3 was 120, 46, and 60 months, respectively (P = 0.1). In conclusion, median PFS and OS in patients with concurrent cardiac and renal AL were comparable to patients with cardiac AL only, but worse than patients with renal AL.     

免疫球蛋白轻链型淀粉样变性病心脏受累的临床特征及预后

目的分析免疫球蛋白轻链型（AL型）淀粉样变性病累及心脏患者的临床特征、治疗和预后。方法回顾性分析我院近7年来确诊的AL型淀粉样变性病心脏受累的比例、心脏淀粉样变的临床特征以及治疗和预后。结果共有60例患者诊断为AL型淀粉样变,其中心脏受累36例（60％）。36例中有心功能不全表现者18例（50％）。心电图异常多表现为肢体导联低电压（47．2％）和伪心肌梗死改变（33．3％）。30例患者（83．3％）超声心动图有异常表现,多为室壁肥厚（63．9％）、心肌颗粒状回声（11．1％）、心房扩大（33．3％）及舒张功能不全（30．6％）。中位存活时间为13．9个月。结论AL型淀粉样变性病累及心脏并不少见,临床表现以室壁增厚、舒张功能不全为特征,预后不良,早期诊断尤为重要。