scholarly journals 334 Hereditary transthyretin amyloidosis: main features and profiles of different clinical phenotypes

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Paolo Massa ◽  
Angelo Giuseppe Caponetti ◽  
Giulia Saturi ◽  
Albero Ponziani ◽  
Maurizio Sguazzotti ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Age Of Onset ◽  
Nyha Class ◽  
Clinical Signs ◽  
Left Ventricular ◽  
Single Amino Acid ◽  
Time Interval ◽  
Prognostic Features ◽  
Cardiac Phenotype ◽  
Significant Difference

Abstract Aims Hereditary transthyretin-related amyloidosis (h-ATTR) is a systemic infiltrative disease caused by a single amino acid mutation on the transthyretin (TTR) gene, which destabilizes the protein and can determine its deposition on multiple organs, including heart and peripheral nervous system. We aimed to characterize and compare clinical, instrumental, and prognostic features of patients affected by h-ATTR by dividing the population into the disease’s main phenotypes (unaffected carriers, cardiac, neurological or mixed phenotype). Methods and results Two hundred and eighty-five subjects of a single-centre cohort with a recognized pathogenic mutation on TTR gene were retrospectively included in the analysis. Phenotypes of disease were defined at baseline. Neurological phenotype (NP) was defined according to sensorimotor and/or autonomic dysfunction, while cardiac phenotype (CP) was defined in the presence of unexplained maximum wall thickness >12 mm and other typical echocardiographic findings. Unaffected carriers (UC) and mixed phenotypes (MP) presented none or both of the above-mentioned features, respectively. Two hundred and ten patients showed clinical signs of the disease, 37 (13%) with CP, 65 (23%) with NP and 108 (38%) with MP, while 75 subjects (26%) were UC. Ile68Leu was the most represented mutation (96 subjects, 34%), followed by Val30Met (21%) and Glu89Gln (13%). NP patients (mostly Val30Met) had mPND score >1 in 45% of patients, were younger at diagnosis (mean 47 years, P < 0.001 vs. CP/MP), and sex was equally distributed. In contrast, CP patients were older at diagnosis (mean 70 years, P < 0.001 vs. CP/MP), predominantly male (as well as in MP) with a higher incidence of tunnel carpal syndrome and a shorter time interval between onset of symptoms and diagnosis (mean 17 months, P < 0.001 vs. CP/MP). NYHA class, ECG findings, left ventricular wall thickness, and ejection fraction did not significantly differ between CP and MP. After a mean follow-up of 59 months, 98 (34%) patients died. On a Kaplan–Meier survival analysis, mean survival times were 208, 123, 150, and 95 months for UC, CP, NP, and MP, respectively, with a statistically significant difference in affected patients between NP and MP (P = 0.012). Conclusions H-ATTR is a rare systemic disorder whose natural history, including age of onset, clinical characteristics, and instrumental findings, is strongly influenced by primary phenotypes, ranging from the excellent prognosis of unaffected carriers to the inauspicious outcome of mixed phenotypes.

scholarly journals Analysis of characteristics and prognostic impact of phenotypes in hereditary ATTR

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M Sguazzotti ◽  
A G Caponetti ◽  
G Saturi ◽  
A Ponziani ◽  
P Massa ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Age Of Onset ◽  
Clinical Signs ◽  
Left Ventricular ◽  
Single Amino Acid ◽  
Time Interval ◽  
Prognostic Impact ◽  
Prognostic Features ◽  
Cardiac Phenotype ◽  
Significant Difference

Abstract Background Hereditary transthyretin-related amyloidosis (h-ATTR) is a systemic infiltrative disease caused by a single amino acid mutation on the transthyretin (TTR) gene, which destabilizes the protein and can determine its deposition on multiple organs, including heart and peripheral nervous system. Purpose We aimed to characterize and compare clinical, instrumental, and prognostic features of patients affected by h-ATTR by dividing the population into the disease's main phenotypes (unaffected carriers, cardiac, neurological or mixed phenotype). Methods Two hundred and eighty-five subjects of a single-centre cohort with a recognized pathogenic mutation on TTR gene were retrospectively included in the analysis. Phenotypes of disease were defined at baseline. Neurological phenotype (NP) was defined according to sensorimotor and/or autonomic dysfunction, while cardiac phenotype (CP) was defined in the presence of unexplained maximum wall thickness >12 mm and other typical echocardiographic findings. Unaffected carriers (UC) and mixed phenotypes (MP) presented none or both of the above-mentioned features, respectively. Results Two hundred and ten patients showed clinical signs of the disease, 37 (13%) with CP, 65 (23%) with NP and 108 (38%) with MP, while 75 subjects (26%) were UC. Ile68Leu was the most represented mutation (96 subjects, 34%), followed by Val30Met (21%) and Glu89Gln (13%). NP patients (mostly Val30Met) had mPND score >1 in 45% of patients, were younger at diagnosis (mean 47 years, p<0,001 vs CP/MP), and sex was equally distributed. In contrast, CP patients were older at diagnosis (mean 70 years, p<0,001 vs CP/MP), predominantly male (as well as in MP) with a higher incidence of tunnel carpal syndrome and a shorter time interval between onset of symptoms and diagnosis (mean 17 months, p<0,001 vs CP/MP). NYHA class, ECG findings, left ventricular wall thickness and ejection fraction did not significantly differ between CP and MP. After a mean follow-up of 59 months, 98 (34%) patients died. On a Kaplan-Meier survival analysis, mean survival times were 208, 123, 150 and 95 months for UC, CP, NP and MP, respectively, with a statistically significant difference in affected patients between NP and MP (p=0.012). Conclusions H-ATTR is a rare systemic disorder whose natural history, including age of onset, clinical characteristics and instrumental findings, is strongly influenced by primary phenotypes, ranging from the excellent prognosis of unaffected carriers to the inauspicious outcome of mixed phenotypes. FUNDunding Acknowledgement Type of funding sources: None.

6133Profile of cardiomyopathy patients in Cape Town - pilot phase the IMHOTEP Study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S M Kraus ◽  
P Samuels ◽  
N Laing ◽  
M Ntsekhe ◽  
A Chin ◽  
...  
Keyword(s):  
Age Of Onset ◽  
Communicable Disease ◽  
Beta Blockers ◽  
Nyha Class ◽  
Left Ventricular ◽  
Continuous Variables ◽  
Class Iii ◽  
Optimal Dosing ◽  
History Of ◽  
Centre Hospital

Abstract Background Cardiomyopathies pose a great challenge because of poor prognosis and high prevalence in LMIC with limited access to specialised care. Little is known about the clinical profile of cardiomyopathy in Africa. Purpose Delineation of clinical presentation and cardiovascular magnetic resonance (CMR) phenotypes of cardiomyopathy. Method The African Cardiomyopathy and Myocarditis Registry Program (IMHOTEP) is a prospective multi-centre, hospital-based study and aims to investigate the clinical characteristics, aetiology, genetics, management and outcomes of cardiomyopathies in Africans. Results Assessment of the first 99 adult cases showed that dilated cardiomyopathy (DCM; n=67) was commonest, followed by hypertrophic (HCM; n=13), left ventricular noncompaction (LVNC; n=11), restrictive (RCM; n=4) and arrhythmogenic (ARVC; n=4) cardiomyopathies. Idiopathic DCM (22%) and peripartum cardiomyopathy (16%) accounted for the majority (Figure). A family history of cardiomyopathy or SCD was reported in 20% of cases. Mean age of presentation was 37±12 years. Most patients (96%) were symptomatic at presentation. NYHA class III/IV was more frequently seen in DCM (61%), RCM (50%) and LVNC (64%), whereas syncope was more common in ARVC (50%) and HCM (23%). VT and aborted cardiac arrest were reported in 7% and 3%, respectively. Onset of symptoms in the peripartum period was observed in 47% of women. Beta-blockers and ACE-inhibitors were prescribed in 77% and 78%, respectively, however optimal dosing was achieved in ≤14% of patients at a median time of 5.4 months after symptom onset. CMR was performed in 67 (68%) cases (Table) and contributed diagnostically in a third of cases. Late gadolinium enhancement (LGE) was observed in 92%. In DCM, linear mid-wall and subendocardial patterns of LGE were seen in 95% and 8% of patients respectively – a much higher percentage than previously reported in the literature. CMR volumetric and functional assessment DCM, n=38 HCM, n=11 ARVC, n=3 RCM, n=4 LVNC, n=11 LVEF (%) 27±15 78±7 55±5 52±7 32±17 LVEDV/BSA (ml/m2) 150±40 80±17 98±12 59±13 155±52 LV mass/BSA (g/m2) 82±23 102±35 74±3 71±14 88±31 RVEF (%) 34±15 68±10 22±15 49±18 33±15 RVEDV/BSA (ml/m2) 104±37 68±15 189±27 56±8 106±51 All continuous variables presented as mean ± standard deviation. Cardiomyopathy diagnosis (n=99) Conclusion IMHOTEP is the first multi-centre registry for cardiomyopathy in Africa. Preliminary data suggests an earlier age of onset with female predominance compared to other cohorts, and DCM is the predominant form of cardiomyopathy in Africa. Acknowledgement/Funding NEWTON FUND NON-COMMUNICABLE DISEASE - South African Medical Research Council (SAMRC/GSK)

P3839Hypertension or hypertrophic cardiomyopathy? Using cardiovascular magnetic resonance imaging to unmask the great imitator

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Keteepe-Arachi ◽  
A Malhotra ◽  
J Basu ◽  
G Parry-Williams ◽  
B Ensam ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Wall Thickness ◽  
Lifestyle Modification ◽  
Left Ventricular ◽  
Grey Zone ◽  
Resonance Imaging ◽  
Hypertensive Patients ◽  
Significant Difference

Abstract Background Structural cardiac adaptations due to hypertension (HTN) present a diagnostic challenge when differentiating from hypertrophic cardiomyopathy (HCM), using traditional imaging techniques such as echocardiography (echo). Cardiac magnetic resonance imaging (CMR) offers reproducible anatomical, functional quantification and myocardial tissue characterisation which discriminates between hypertension and HCM. Purpose To identify hypertensive individuals with undiagnosed HCM using CMR imaging. Methods 100 consecutive hypertensive patients underwent CMR at a tertiary centre dedicated blood pressure clinic (55% male, mean age 51 years). In keeping with ESC guidelines, end diastolic wall thickness (EDWT) ≥15mm identified individuals within the “grey zone” between hypertension and with a potential HCM diagnosis. 19 individuals were referred on to the dedicated inherited cardiac conditions clinic for further evaluation. Four patients expressed a definitive LV phenotype and were diagnosed with HCM. CMR parameters were compared in three groups: Hypertensive (HTN), “grey zone” Hypertensive (GZH) and HCM. Results CMR demonstrated end diastolic wall thickness (EDWT) >11 mm in 50% of hypertensives. 73% of the referred patients were Afro-Caribbean (AC) and all 4 HCM patients were AC. All referrals demonstrated EDWTs ≥14mm, 9 (47%) demonstrated late gadolinium enhancement of which 3 (16%) had HCM. Three had asymmetrical septal hypertrophy – 2 were in the HCM cohort and one underwent endomyocardial biopsy confirming HTN. Left ventricular mass index (LVMI) was significantly higher in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0004). EDWT was significantly greater in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0002). There was no significant difference in these parameters between GZH and HCM. Table 1. P-values for CMR data in hypertensive (HTN), gray zone hypertensive (GZH) and hypertrophic cardiomyopathy (HCM) cohorts CMR Parameter HCM vs. HTN GZH vs. HTN GZH vs. HCM EDVI NS p=0.025 NS EDWT p=0.0002 p<0.0001 NS LVMI p=0.0004 p<0.0001 NS Conclusion This study reports a 4% prevalence of HCM among hypertensive patients - 20 x greater than in the general population - which would be left undiagnosed using echo alone. Screening hypertensive individuals with CMR is not routine but we advocate its use in these individuals especially in Afro Caribbeans and in those in the “grey zone”, to identify undiagnosed HCM, which has significant implications for lifestyle modification and family screening. Acknowledgement/Funding Cardiac Risk in the Young

Blood Pressure Control and Left Ventricular Hypertrophy in Long-Term Capd and Hemodialysis Patients: A Cross-Sectional Study

2003 ◽  
Vol 23 (6) ◽  
pp. 563-567 ◽  
Author(s):  
Ali Ihsan Günal ◽  
Erdogan Ilkay ◽  
Ercan Kirciman ◽  
Ilgin Karaca ◽  
Ayhan Dogukan ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Left Ventricular Hypertrophy ◽  
Wall Thickness ◽  
Left Atrial ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Volume Control ◽  
Left Ventricular Chamber ◽  
Significant Difference

Background It is still not clear whether hypertension and left ventricular hypertrophy (LVH) are more common in continuous ambulatory peritoneal dialysis (CAPD) than in hemodialysis (HD) patients. Methods To examine this subject, the indices of cardiac performance were compared between 50 HD and 34 CAPD patients. Patients were further divided into two subgroups [long-term (L) CAPD and L-HD] according to dialysis modality and duration of dialysis (more than 60 months’ duration). Results The blood pressure and cardiothoracic index of CAPD patients did not differ from HD patients. On average, the left atrial index was 2 mm/m2 higher in HD patients than in CAPD patients. Left ventricular chamber sizes, wall thickness, and left ventricular mass index (LVMI) in patients on CAPD were similar to those of HD patients. Isovolumic relaxation time (IVRT) of CAPD patients was insignificantly less than that of HD patients (101 ± 22 and 115 ± 27 msec respectively). There was no significant difference between the two subgroups (L-HD and L-CAPD) in blood pressure, left atrial diameter, left ventricular chamber size, wall thickness, LVMI, ejection fraction, or IVRT. Conclusion If normovolemia and normotension are obtained by strict volume control without using antihypertensive drugs, the effects of the two modalities of chronic dialysis treatment (HD and CAPD) on cardiac structure and function are not different from each other.

scholarly journals 383 ECG in biopsy-proven and clinically suspected myocarditis: morpho-functional correlates and prognostic implications

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Andrea Silvio Giordani ◽  
Alex Panzarella ◽  
Andrea Candelora ◽  
Davide Marcolongo ◽  
Chiara Merola ◽  
...  
Keyword(s):  
Nyha Class ◽  
Left Ventricular ◽  
Long Qt ◽  
Bundle Branch Block ◽  
Entire Cohort ◽  
Surrogate Outcome ◽  
Wave Inversion ◽  
Significant Difference ◽  
Ecg Alterations

Abstract Aims A distinction exists between biopsy-proven (BP) and clinically suspected (CS) myocarditis, the latter being an exclusion diagnosis based on clinical and instrumental findings. A clear diagnostic and prognostic role of the ECG in these two groups of patients has not yet been defined. (i) To describe frequency and characteristics of ECG findings in myocarditis, and to assess any difference between CS and BP myocarditis; (2) to identify morpho-functional correlates between ECG and cardiac magnetic resonance (CMR); and (iii) to evaluate the prognostic value of ECG findings. Methods and results 162 patients were included (median age 36 years, 70% male, median follow-up 32.9 months), 36 with BP and 126 with CS myocarditis. All patients underwent CMR; for ECG-CMR correlates, the ECG nearest in time to CMR was assessed. Surrogate outcome was defined as left ventricular (LV) ejection fraction (EF) &lt;50% and/or NYHA class &gt;I during follow-up. In the entire cohort ECG alterations were numerous: T-wave inversion (TWI) (82%), fragmented QRS (34%), low voltages (14%), ST elevation (STE) (13%). Compared to CS myocarditis, BP myocarditis patients showed higher frequency of non-sinus rhythm (17% vs. 2%, P &lt; 0.001), long QT (28% vs. 0%, P &lt; 0.001), lateral TWI (36% vs. 19%, P = 0.031) and bundle branch block (19% vs. 2%, P &lt; 0.001). BP myocarditis patients had worse clinical features at diagnosis: heart failure (64% vs. 6%, P &lt; 0.001), arrhythmic (14% vs. 4%, P = 0.029) and fulminant presentation (14% vs. 0%, P &lt; 0.001), and presented higher LV end-diastolic volume and lower LVEF by echocardiography and CMR (109.5 mL/m2 vs. 85.50 ml/m2, P &lt; 0.001; 31.5% vs. 59%, P &lt; 0.001). A correlation was observed between number of myocardial segments with oedema at CMR and low ECG voltages (P = 0.010) and between late gadolinium enhancement (LGE) mass at CMR and lateral STE (P = 0.004 and P = 0.049, respectively). Several ECG alterations correlated with the surrogate outcome: long QT (P = 0.029), lateral TWI (P = 0.006), left bundle branch block (P &lt; 0.001), ventricular ectopic beats (P = 0.020), and atrial fibrillation (P &lt; 0.001). Conclusions A significant difference in ECG findings between CS and BP myocarditis has been demonstrated: ECG alterations are more frequent and more severe in BP myocarditis and correlate with prognosis. Moreover, ECG alterations identified patients with pathologic morpho-functional correlates.

scholarly journals Patterns of cardiac involvement in patients with long COVID syndrome using cardiovascular magnetic resonance

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
V Puntmann ◽  
S Martin ◽  
B Vanchin ◽  
N Holm ◽  
E Giokoglu ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Late Complication ◽  
Left Ventricular ◽  
Time Interval ◽  
Ventricular Ejection Fraction ◽  
Native T1 ◽  
Significant Difference ◽  
Previous Hospitalization ◽  
Cmr Imaging

Abstract Background Long COVID (LC) is an increasingly recognized late complication of COVID-19 infection. Cardiovascular involvement has also been implicated, however, the type and extent of the underlying cardiovascular injury remains unknown. Purpose To evaluate the association between ongoing symptoms and findings with cardiovascular magnetic resonance (CMR) in consecutive patients recently recovered from COVID-19 illness. Methods Prospective observational cohort study of patients recently recovered from COVID-19 illness and no previously known cardiovascular disease were included between April 2020 and April 2021. Demographic characteristics, cardiac blood markers, and CMR imaging a minimum of 4 weeks from the diagnosis were obtained. Results Of the 389 included patients, 192 (49%) were male, the mean (±standard deviation) age was 44 (±13) years and 61 (16%) required hospitalization during the acute illness. The median (IQR) time interval between COVID-19 diagnosis and CMR was 94 (71–165) days. 298 (77%) of patients continued to experience ongoing cardiovascular symptoms (long COVID, LC), including dyspnea, palpitations, atypical chest pain and fatigue at the time of CMR at least 4 weeks after the infection. In most patients, the symptoms were only effort related 137 (46%), whereas in 98 (33%) the symptoms affected the activities of daily life; 10 (3%) had severe and debilitating symptoms at rest. Compared to those with no LC (NLC, n=91), LC patients were more commonly hospitalized, had significantly higher native T1, native T2, and showed pericardial enhancement and effusion (Figure 1). There were no differences in cardiac biomarkers, left ventricular (LV) and right ventricular ejection fraction and mass. Proportionally, men and women were similarly affected (n=144 (73%) vs. n=157 (80%), p=0.18). Previous hospitalization was associated with hypertension and ongoing detectable troponin. LC status was associated with previous hospitalization and CMR findings of raised native T1 and native T2, and in females also pericardial enhancement. Severity of symptoms were associated with increased native T1 and T2 and decreased end-diastolic volume, whereas cardiac function showed no significant difference. Conclusions In this cohort of patients recently recovered from COVID-19 infection, ongoing cardiovascular symptoms were common. The LC status was related to previous hospitalization and CMR imaging findings of myopericardial inflammation. The extent and type of cardiovascular findings was associated with the severity of symptoms. These findings indicate the need for ongoing investigation of the long-term cardiovascular consequences of COVID-19. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The German Heart Foundation (Deutsche Herzstiftung) and Bayer AG, Leverkusen, Germany Figure 1

scholarly journals Influence of Average Annual Mean Serum Ferritin Levels on Left Ventricular Function in Patients With β Thalassemia

2020 ◽  
Author(s):  
Yazdan Ghandi ◽  
Danial Habibi ◽  
Aziz Eghbali ◽  
Meysam Meraj Ein ◽  
Saeed Sadrnia
Keyword(s):  
Left Ventricular Function ◽  
Ventricular Function ◽  
Serum Ferritin ◽  
Clinical Signs ◽  
Left Ventricular ◽  
Cardiac Complications ◽  
Control Group ◽  
Doppler Image ◽  
Health Group ◽  
Significant Difference

Cardiac complications are the most important cause of mortality and morbidity in Beta-Thalassemia Major (B-TM). The aim of our study was to determine the influence of Average Annual Mean Serum Ferritin (AAMSF) levels on cardiac function in patients suffering from B-TM. In this cross-sectional study, 50 patients of 5-15 years with B-TM were enrolled in the absence of clinical signs and symptoms of cardiac dysfunction. Left ventricular function was studied by using tissue Doppler image (TDI), pulse wave Doppler (PWD). AAMSF level was measured for 12 months. Patient groups were subdivided into two groups, group A with AAMSF level below 1000 ng/ml and group with B with AAMSF level above 1000 ng/ml. The Case group included 24(48%) males and 26(52%) females which were compared with 50 healthy subjects composed of 27(54%) males and 23 (46%) females (control group). The AAMSF Levels were 1054.60±687.95 ng/ml. By using PDW, in patients with AAMSF below 1000 ng/ml, The Parameters of the E, A, E/A E-DT had no significant difference with health groups (P=0.808, P=0.820, P=0.231 and P=0.061, respectively). No significant difference was revealed in parameters of E’, IVCT and ET in patients with AAMSF below 1000 ng/ml by using TDI in comparison to control (P=0.155, P=0.683, P=0.222, respectively).however, other TDI indexes including A’, E’/A’, IVRT, S,’ LVMPI, E/E’ had a significant difference with health group (P=0.014, P=0.041, P=0.045, P=0.002, P=0.021, and P=0.002, respectively). At patients with above AAMSF 1000 ng/mL, both PDW and TDI parameters had significant differences with the health group. There was no correlation among AAMSF levels and PWD or TDI indexes. The evidence from this study suggests that subclinical systolic and diastolic LV dysfunction has happened with AAMSF level above 1000 ng/ml, and according to our result, TDI is found more useful than PWD for subclinical cardiac evaluation.

scholarly journals Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study

2021 ◽  
Author(s):  
Fabian Strodka ◽  
Jana Logoteta ◽  
Roman Schuwerk ◽  
Mona Salehi Ravesh ◽  
Dominik Daniel Gabbert ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Feature Tracking ◽  
Nyha Class ◽  
Radial Strain ◽  
Left Ventricular ◽  
Lv Function ◽  
Significant Difference ◽  
Lv Volumes

Abstract Purpose Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). Methods 54 SLV patients (11.4, 3.1–38.1 years) and 35 age-matched controls (12.3, 6.3–25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. Results The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR were not significantly different between DILV and TA patients. Conclusion Although most SLV patients had a preserved EF, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.

scholarly journals Relationship between myocardial edema and left ventricular wall thickness in acute myocardial ischemia: A Magnetic Resonance Imaging study

2014 ◽  
Vol 1 (2) ◽  
pp. 23
Author(s):  
Yoko Mikami ◽  
Andreas Kumar ◽  
Hassan Abdel-Aty ◽  
Matthias G. Friedrich
Keyword(s):  
Magnetic Resonance ◽  
Myocardial Ischemia ◽  
Wall Thickness ◽  
Myocardial Injury ◽  
Myocardial Edema ◽  
Left Ventricular ◽  
Acute Myocardial Ischemia ◽  
Ischemia And Reperfusion ◽  
Significant Difference ◽  
T2 Weighted Images

Purpose: We sought to assess the relationship between left ventricular regional end-diastolic myocardial wall thickness (EDWT) and myocardial edema defined using T2-weighted Cardiovascular Magnetic Resonance (CMR) after acute myocardial ischemia and reperfusion. Methods: T2-weighted and cine CMR images for 7 dogs at baseline, during coronary occlusion (mean 33 ± 4 minutes) and after reperfusion were studied. The EDWT was measured in segments with high signal intensity (SI) on T2-weighted images, adjacent segments and remote segments according to a 16-segment model. Results: The EDWT after reperfusion in segments with high SI on T2-weighted images was significantly increased compared to baseline (6.28 ± 1.06 mm and 5.51 ± 1.40 mm, p < 0.05), whereas EDWT after the reperfusion in adjacent and remote segments did not show significant difference compared to baseline (adjacent: 6.48 ± 1.55 mm and 6.38 ± 1.26 mm, p = N.S., remote: 6.41 ± 1.11mm and 6.42 ± 1.27mm, p = N.S.). The % increase in EDWT after reperfusion from baseline in segments with high SI on T2-weighted images was higher than those in adjacent and remote segments (19 ± 30%, 1.3 ± 15% and 1.5 ± 16%, respectively, p < 0.05). Conclusions: After a brief period of ischemia and reperfusion, edema as defined by high SI on T2-weighted CMR is related to an increase in EDWT. This increase however is too small to be clinically relevant to be used for the detection of acute myocardial injury. Edema imaging is more sensitive and is an essential part of the reliable assessment of acute ischemic myocardial injury.

scholarly journals Cardiac remodeling and contractile function in acid α-glucosidase knockout mice

2001 ◽  
Vol 5 (4) ◽  
pp. 171-179 ◽  
Author(s):  
JOEP H. J. KAMPHOVEN ◽  
RENE STUBENITSKY ◽  
ARNOLD J. J. REUSER ◽  
ANS T. VAN DER PLOEG ◽  
PIETER D. VERDOUW ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Contractile Function ◽  
Left Ventricular Pressure ◽  
Glycogen Storage ◽  
Left Ventricular ◽  
Suitable Model ◽  
Pompe’S Disease ◽  
Lv Dysfunction ◽  
Cardiac Phenotype ◽  
Pompe's Disease

Pompe’s disease is an autosomal recessive and often fatal condition, caused by mutations in the acid α-glucosidase gene, leading to lysosomal glycogen storage in heart and skeletal muscle. We investigated the cardiac phenotype of an acid α-glucosidase knockout (KO) mouse model. Left ventricular weight-to-body weight ratios were increased 6.3 ± 0.8 mg/g in seven KO compared with 3.2 ± 0.2 mg/g in eight wild-type (WT) mice ( P < 0.05). Echocardiography under ketamine-xylazine anesthesia revealed an increased left ventricular (LV) wall thickness (2.17 ± 0.16 in KO vs. 1.18 ± 0.10 mm in WT mice, P < 0.05) and a decreased LV lumen diameter (2.50 ± 0.32 in KO vs. 3.21 ± 0.14 mm in WT mice, P < 0.05), but LV diameter shortening was not different between KO and WT mice. The maximum rate of rise of left ventricular pressure (LV dP/d tmax) was lower in KO than in WT mice under basal conditions (2,720 ± 580 vs. 4,440 ± 440 mmHg/s) and during dobutamine infusion (6,220 ± 800 vs. 8,730 ± 790 mmHg/s, both P < 0.05). Similarly, during isoflurane anesthesia LV dP/d tmax was lower in KO than in WT mice under basal conditions (5,400 ± 670 vs. 8,250 ± 710 mmHg/s) and during norepinephrine infusion (10,010 ± 1,320 vs. 14,710 ± 220 mmHg/s, both P < 0.05). In conclusion, the markedly increased LV weight and wall thickness, the encroachment of the LV lumen, and LV dysfunction reflect cardiac abnormalities, although not as overt as in humans, of human infantile Pompe’s disease and make these mice a suitable model for further investigation of pathophysiology and of novel therapies of Pompe’s disease.

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