scholarly journals Lingual liability: macroglossia and dyspnoea as the harbinger of systemic AL (light-chain) cardiac amyloidosis

2018 ◽  
Vol 11 (1) ◽  
pp. e225923 ◽  
Author(s):  
Michael Uncle Williams ◽  
Caroline E Murphy ◽  
Rosco Steven Gore ◽  
Emilio Fentanes
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Systemic Disease ◽  
Supportive Therapy ◽  
Left Ventricular ◽  
The Body ◽  
Left Ventricular Chamber ◽  
Patient Reported ◽  
Ventricular Wall Thickness

A 58-year-old man presented with a chief complaint of tongue indentations and discomfort. Otolaryngology treated him for oral thrush with counselling to avoid tongue biting. In addition, the patient reported dyspnoea described as a decrease in tolerance of his physical activities. Due to continued increase in tongue size and worsening dyspnoea, he underwent a tissue biopsy with findings consistent with amyloidosis. Further evaluation with a bone marrow biopsy revealed underlying multiple myeloma. Echocardiography revealed abnormal ventricular wall thickness, with a reduced left ventricular chamber size, dilated atria and Doppler findings with restrictive filling patterns indicative of cardiac amyloidosis. The patient was initiated on chemotherapy for his multiple myeloma and supportive therapy for his cardiac amyloidosis. Light-chain amyloidosis (AL) is a systemic disease characterised by irreversible deposition of amyloid in tissues throughout the body; when there is cardiac involvement, it can result in heart failure with a poor prognosis. Early diagnosis of cardiac amyloidosis can lead to prolonged survival.

Cardiac Amyloidosis Without Increased Left Ventricular Wall Thickness

2014 ◽  
Vol 89 (6) ◽  
pp. 781-789 ◽  
Author(s):  
Ga Yeon Lee ◽  
Kihyun Kim ◽  
Jin-Oh Choi ◽  
Seok Jin Kim ◽  
Jung-Sun Kim ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Cardiac Amyloidosis ◽  
Left Ventricular ◽  
Left Ventricular Wall ◽  
Ventricular Wall ◽  
Left Ventricular Wall Thickness ◽  
Ventricular Wall Thickness

scholarly journals Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis

PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0255487
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Mordehay Vaturi ◽  
Iuliana Vaxman ◽  
Zaza Iakobishvili ◽  
Noa Rhurman-Shahar ◽  
...  
Keyword(s):  
Heart Failure ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Clinical Laboratory ◽  
Left Ventricular ◽  
Male Predominance ◽  
Cardiac Outcomes ◽  
Lv Mass ◽  
Artery Disease

Aims To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients’ contemporary cardiac outcomes. Methods Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients’ cohort (years 2013–2020). Results Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p<0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm2 vs. 27 (IQR 23, 30)cm2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m2 vs. 82 (IQR 72, 98)grams/m2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Conclusion Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.

Abstract 10961: QRS Duration and Left Bundle Branch Block Do Not Deter Assessment of Low Voltage in Cardiac Amyloidosis

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Brett W Sperry ◽  
Michael N Vranian ◽  
Hariom Joshi ◽  
Rory Hachamovitch ◽  
Mazen Hanna
Keyword(s):  
Left Bundle Branch Block ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Left Ventricular ◽  
S Wave ◽  
Bundle Branch Block ◽  
Propensity Matching ◽  
History Of ◽  
Ventricular Wall Thickness ◽  
Qrs Duration

Introduction: Left bundle branch block (LBBB) with QRS duration > 120 msec is typically associated with increased ECG voltage without a necessary increase in left ventricular wall thickness. Studies assessing voltage criteria in amyloidosis and other cardiomyopathies have excluded patients with LBBB. We sought to assess the effects of QRS duration and LBBB on voltage criteria in cardiac amyloidosis. Methods: We performed a retrospective analysis of patients with newly diagnosed cardiac amyloidosis at our institution from 2001-2014. Low voltage in the precordial leads was defined by the Sokolow criteria (S wave in V1 plus R wave in V5 or V6 ≤ 15mm). Limb lead voltage was calculated by the sum of the entire QRS complex voltage of leads I, II and III with low voltage being defined as each lead ≤ 5 mm. Patients with left bundle branch block were propensity matched to assess voltage criteria based upon age, sex, history of HTN, amyloid type (AL vs TTR), anteroseptal thickness and BSA to those with QRS < 120msec. Results: In 299 subjects (age 69.6 +/- 11.7 years, male 69%, HTN 53%, AL 53%, IVS 18.1 +/- 4.1 mm, BSA 1.95 +/- 0.26, EF 48 +/- 14%), mean QRS duration was 107 +/- 25 msec with 71 patients (24%) having a QRS duration greater than 120 msec (17 LBBB). The average limb voltage was 15.1 +/- 7.3 mm with low limb lead voltage seen in 62% of patients. Low precordial lead voltage was found in 73% of patients with a mean Sokolow voltage of 12.9 +/- 7.5 mm. After propensity matching the 17 patients with LBBB on a 1:3 basis with patients with a narrow complex QRS, there was no difference in any measure of voltage criteria. Conclusion: LBBB is not associated with higher voltage and does not hinder the ability to detect low voltage in patients with cardiac amyloidosis.

Abstract 15734: Left Atrial Reservoir Strain as a Novel Prognostic Marker in Biopsy-proven Light-chain Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Peter Huntjens ◽  
Kathleen Zhang ◽  
Yuko Soyama ◽  
Maria Karmpalioti ◽  
Daniel Lenihan ◽  
...  
Keyword(s):  
Light Chain ◽  
Left Atrial ◽  
Prognostic Value ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Strain Imaging ◽  
Left Ventricular ◽  
Lv Function ◽  
Al Amyloidosis

Introduction: Light chain cardiac amyloidosis (AL) has a variable but usually poor prognosis. Left ventricular (LV) function measures including LV strain imaging for global longitudinal strain (GLS) have shown clinically prognostic value in AL. However, the utility of novel left atrial (LA) strain imaging and its associations with LV disease remains unclear. Hypothesis: LA strain is of additive prognostic value to GLS in AL. Methods: We included 99 consecutive patients with AL. Cardiac amyloidosis either confirmed by endocardial biopsy (25%) or by non-cardiac tissue biopsy and imaging data supportive of cardiac amyloidosis. Peak LA reservoir strain was calculated as an average of peak longitudinal strain from apical 2- and 4-chamber views. GLS and apical sparing ratio were assessed using the 3 standard apical views. All-cause mortality was tracked over a median of 5 years. Results: Echocardiographic GLS and peak longitudinal LA strain were feasible in 96 (97%) and 86 (87%) of patients, respectively. There were 48 AL patients who died during follow-up. Patients with low GLS (GLS < median; 10.3% absolute values) had worse prognosis than patients with high GLS group (p<0.001). Although peak longitudinal LA strain was correlated with GLS (R=0.65 p<0.001), peak longitudinal LA strain had additive prognostic value. AL patients with low GLS and low Peak LA strain (<13.4%) had a 8.3-fold increase in mortality risk in comparison to patients with high GLS (95% confidence interval: 3.84-18.03; p<0.001). Multivariable analysis showed peak longitudinal LA strain was significantly and independently associated with survival after adjusting for clinical and echocardiographic covariates (p<0.01). Conclusions: Peak longitudinal LA strain was additive to LV GLS in predicting prognosis in patients with biopsy confirmed AL amyloidosis. LA strain imaging has potential clinical utility in patients with AL cardiac amyloidosis.

scholarly journals Left Ventricular Structure and Function in Transthyretin-Related Versus Light-Chain Cardiac Amyloidosis

Circulation ◽  
2014 ◽  
Vol 129 (18) ◽  
pp. 1840-1849 ◽  
Author(s):  
Candida Cristina Quarta ◽  
Scott D. Solomon ◽  
Imran Uraizee ◽  
Jenna Kruger ◽  
Simone Longhi ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Left Ventricular ◽  
Structure And Function ◽  
Left Ventricular Structure ◽  
Ventricular Structure ◽  
And Function

scholarly journals An Unusual Cause of Generalized Swelling

2018 ◽  
Vol 26 (2) ◽  
pp. 173-177
Author(s):  
Sabrina Shahrin ◽  
Md Aminul Islam ◽  
Md Ehsan Uddin Khan ◽  
Parvez Iftekhar Ahmed ◽  
Md Nazrul Islam ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Systemic Disease ◽  
Light Chains ◽  
Al Amyloidosis ◽  
Medical College ◽  
Multiple Organs ◽  
Single Clone ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Amyloidosis and multiple myeloma are included in the same spectrum of clonal plasma cell disorder. Amyloidosis can present with localized deposits or manifest as systemic disease involving multiple organs such as kidney, heart, intestine. Free Ig subunits ,mostly light chains secreted by a single clone of B cells, are the cause of the most frequent and severe amyloidosis affecting the kidney. The incidence of AL amyloidosis is nine per 1 million populations per year. Amyloid deposists are found in approximately 10% of all patients with myeloma and in 20% of those with pure light chain myeloma[1].Here we are reporting a case who initially presented with generalized swelling and subsequently found to have AL amyloidosis involving multiple organs due to multiple myeloma.J Dhaka Medical College, Vol. 26, No.2, October, 2017, Page 173-177

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