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1.
Int J Cardiol ; 2021 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-33571563

RESUMO

BACKGROUND: Pregnant women with repaired tetralogy of Fallot (rTOF) are at increased risk of adverse outcomes. Although pre-pregnancy pulmonary valve replacement (PVR) may be considered in some women to attenuate risk, published data to support this practice are lacking. Our objective was to explore the impact of pre-pregnancy PVR on pregnancy outcomes in rTOF. METHODS: Women with rTOF and cardiovascular magnetic resonance imaging (CMR) before and after pregnancy were included if CMR studies were completed within 3 years of pregnancy. Subjects were compared according to presence (+) or absence (-) of PVR at pre-pregnancy CMR. Pregnancy outcomes (cardiovascular, obstetric, and fetal/neonatal) were documented. RESULTS: Of the 29 study women identified, 7 were PVR+ and 22 were PVR-. Post-pregnancy, the PVR- group demonstrated interval increase in indexed right ventricular end-diastolic volumes (RVEDVi) (157 ± 28 versus 166 ± 33 ml/m2, p = 0.003) and end-systolic volumes (RVESVi) (82 ± 17 versus 89 ± 20 ml/m2, p = 0.003) as compared with pre-pregnancy, but no significant change in RV ejection fraction, RV mass, or left ventricular measurements. In the PVR+ group, there were no interval changes in RV measurements pre-versus post pregnancy. Interval rate of change in RVESVi of PVR- exceeded PVR+ women (+3.7 ± 5.0 versus -2.2 ± 5.0 ml/m2/year, p = 0.03). Pregnancy outcomes did not differ in PVR+ versus PVR- women. CONCLUSIONS: Pregnancy outcomes did not differ according to PVR status in our cohort. While RV volumes remained unchanged in PVR+ women, interval RV dilation was observed in PVR- women. Additional study of a larger population with longer follow-up may further inform clinical practice regarding pre-pregnancy PVR.

4.
Artigo em Inglês | MEDLINE | ID: mdl-33551194

RESUMO

Muscular dystrophies (MDs) are a group of inherited disorders caused by mutations that interfere with muscular structure, contraction, or relaxation. As the cardiac sarcomeric unit shares multiple proteins with the skeletal muscle unit, the heart is affected in several MDs, sometimes without apparent musculoskeletal involvement. Early detection of MD-related cardiomyopathy is crucial as timely initiation of cardioprotective therapy can slow adverse cardiac remodeling. Although transthoracic echocardiography is widely used for the evaluation of cardiac morphology and function, it has limitations in terms of reproducibility and image quality. The need for an optimal acoustic window may be particularly challenging to obtain in patients with MDs given their body habitus and position. Cardiac magnetic resonance (CMR) imaging has emerged as a useful tool in the evaluation of patients with MDs. Its superb tissue characterization capability through late gadolinium enhancement, T1 mapping, extracellular volume fraction quantification, and edema imaging detects early cardiac involvement, even when echocardiography and electrocardiogram are unremarkable. MDs that frequently present with cardiac involvement include Duchenne MD, Becker MD, Emery Dreifuss MD, Limb-Girdle MDs, and myotonic dystrophy. The purpose of this review article is to briefly describe the pathophysiology of these entities, discuss their clinical presentation and expected evolution, explain the role of CMR in the diagnosis and follow-up of these patients, and portray the different CMR findings present in MD patients.

5.
Ann Am Thorac Soc ; 2021 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-33433272

RESUMO

RATIONALE: Patients with hematologic malignancies (HM) requiring mechanical ventilation have historically experienced poor outcomes. OBJECTIVES: We aimed to determine whether body composition characteristics derived from thoracic Computed-Tomography (CT) were associated with time to liberation from mechanical ventilation. METHODS: We evaluated mechanically ventilated patients with HM admitted between 2014-2018. We included patients with a thoracic-CT completed between one month prior and 48 hours after ICU admission. We assessed the association of carinal skeletal muscle cross-sectional area (CSA), subcutaneous fat CSA and fat index (fat/skeletal muscle) with time to liberation from mechanical ventilation within 28-days. We accounted for the competing event of death within 28-days of mechanical ventilation. RESULTS: 156 patients were included across whom the mean age was 57 years (SD 14) and 39% were female. Thirty-seven percent had received a hematopoietic stem cell transplant and median PaO2/FiO2 ratio was 134mmHg(IQR 92-205). Median skeletal muscle CSA was 68cm2(IQR 54-88) and subcutaneous fat CSA was 38cm2(IQR 27-52). Forty-two percent of patients were liberated from mechanical ventilation within 28-days and 56% died in the ICU. Subcutaneous fat CSA (subdistribution Hazard Ratio [sHR] 0.81,95% CI-0.68-0.97) and fat index (sHR 0.81,95% CI-0.68-0.97) were significantly associated with longer time to liberation from mechanical ventilation. Skeletal muscle CSA was not associated with time to liberation from ventilation (sHR 1.08, 95% CI-0.94-1.23). CONCLUSION: Body composition measurements based on thorax-CT scan were associated with time to liberation from ventilation. These could represent novel surrogate markers of physical frailty in patients with hematologic malignancies receiving mechanical ventilation.

6.
Heart Fail Clin ; 17(1): 109-120, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33220880

RESUMO

Disorders of the pericardium are common and can result in significant morbidity and mortality. Advances in multimodality imaging have enhanced our ability to diagnose and stage pericardial disease and improve our understanding of the pathophysiology of the disease. Cardiovascular MRI (CMR) can be used to define pericardial anatomy, identify the presence and extent of active pericardial inflammation, and assess the hemodynamic consequences of pericardial disease. In this way, CMR can guide the judicial use of antiinflammatory and immune modulatory medications and help with timing of pericardiectomy. CMR can also be used to diagnose congenital disorders of the pericardium. Furthermore, CMR can be used to define pericardial masses and understand their malignant potential.


Assuntos
Imagem Cinética por Ressonância Magnética/métodos , Derrame Pericárdico/diagnóstico , Pericardite Constritiva/diagnóstico , Pericárdio/patologia , Humanos
7.
Artigo em Inglês | MEDLINE | ID: mdl-33248971

RESUMO

OBJECTIVES: This study examined fibrosis progression in hypertrophic cardiomyopathy (HCM) patients, as well as its relationship to patient characteristics, clinical outcomes, and its effect on clinical decision making. BACKGROUND: Myocardial fibrosis, as quantified by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR), provides valuable prognostic information in patients with HCM. METHODS: A total of 157 patients with HCM were enrolled in this study, with 2 sequential CMR scans separated by an interval of 4.7 ± 1.9 years. RESULTS: At the first CMR session (CMR-1), 70% of patients had LGE compared with 85% at CMR-2 (p = 0.001). The extent of LGE extent increased between the 2 CMR procedures, from 4 ± 5.6% to 6.3 ± 7.4% (p < 0.0001), with an average LGE progression rate of 0.5% ± 1.0%/year. LGE mass progression was correlated with higher LGE mass and extent on CMR-1 (p = 0.0017 and 0.007, respectively), greater indexed left ventricular (LV) mass (p < 0.0001), greater LV maximal wall thickness (p < 0.0001), apical aneurysm at CMR-1 (p < 0.0001), and lower LV ejection fraction (EF) (p = 0.029). Patients who were more likely to have a higher rate of LGE progression presented with more severe disease at baseline, characterized by LGE extent >8% of LV mass, indexed LV mass >100 g/m2, maximal wall thickness ≥20 mm, LVEF ≤60%, and apical aneurysm. There was a significant correlation between the magnitude of LGE progression and future implantation of insertable cardioverter-defibrillators (p = 0.004), EF deterioration to ≤50% (p < 0.0001), and admission for heart failure (p = 0.0006). CONCLUSIONS: Myocardial fibrosis in patients with HCM is a slowly progressive process. Progression of LGE is significantly correlated with a number of clinical outcomes such as progression to EF ≤50% and heart failure admission. Judicious use of serial CMR with LGE can provide valuable information to help patient management.

8.
Magn Reson Imaging ; 74: 195-202, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33010378

RESUMO

BACKGROUND: Investigation of a simple, precision optimized, identical pre-/post-contrast modified look locker inversion recovery (MOLLI) protocol employing Composite inversion group (IG) fitting in a clinical cardiomyopathy population. METHODS: Cardiac magnetic resonance imaging (MRI) was performed at 3 Tesla in 36 patients (48.0 years [IQR: 35.7, 58.2 years]) with known/suspicion of hypertrophic cardiomyopathy. T1 mapping was performed pre-/post-contrast (0.15 mmol/kg Gadobutrol) using a standard 3-parameter fit (STANDARD) and an optimized (OPTIMAL) single-protocol Composite-IG fitting MOLLI approach. The OPTIMAL protocol was based on a simulation study (for 11hb acquisitions) with cost metric analysis across the range of expected T1 values (300-1400 ms) and heart rates (50-80 bpm). All maps were generated offline based on motion corrected source images. Based on region of interest analysis, the precision of both approaches was assessed using a previously validated propagation of errors technique for pre-/post-contrast T1 mapping as well as calculated ECV (based on point-of care hematocrit measurements. Furthermore, respective T1 and ECV values were calculated. Statistical methods included Wilcoxon Signed-Rank tests and Student's paired t-test. RESULTS: A total of ~9000 11hb inversion groupings were simulated with a 4(0)2(0)2(0)2(0)1 grouping providing the optimal precision across the specified T1/heart rate range. In comparison to standard pre-contrast 5(3)3 MOLLI, this OPTIMAL protocol demonstrated a significantly improved pre-contrast precision (9.1 [6.2, 9.9]ms vs. 9.4 [7.3, 10.8]ms; P < 0.001) while no significant differences were found for post-contrast T1 mapping (4.5 [2.6, 5.3]ms vs. 4.2 [2.8, 5.1]ms; P = 0.25) and EVC mapping (0.38 [0.28, 0.45]ms vs. 0.35 [0.25, 0.44]ms; P = 0.07) or reproducibility (0.16 [0.14, 0.19] vs. 0.19 [0.13, 0.23]P = 0.53). Direct comparison of resulting T1/ECV values demonstrated no significant differences between STANDARD and OPTIMAL techniques for pre-contrast T1 (1178 [1158, 1199]ms vs. 1173 [1143, 1195]ms; P = 0.46) and significant differences for post-contrast T1 (466 [446, 506]ms vs. 456 [433, 503]ms; P = 0.04) and ECV (23.1 [20.8, 25.1]% vs. 23.9 [22.3, 26.4]%; P = 0.001). CONCLUSIONS: A single optimized Composite-IG fitting protocol for pre-/post-contrast T1 mapping demonstrated improved precision over standard MOLLI techniques. It enables a simplified workflow with reduction of potential sources of error especially with respect to image data co-registration easing advanced post-processing for generation of patient specific ECV maps.

9.
Heart ; 2020 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-32748799

RESUMO

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

10.
AJR Am J Roentgenol ; : 1-7, 2020 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-32755161

RESUMO

OBJECTIVE. Cardiac involvement is the leading cause of mortality in Fabry disease. Noninvasive markers of cardiac involvement are needed to identify patients at high risk. The purpose of this study was to evaluate the diagnostic potential of segmental native T1 spread as an imaging biomarker in Fabry disease. SUBJECTS AND METHODS. In this prospective study, 43 patients with confirmed Fabry disease (mean ± SD age, 46±14 years; 70% women) and 17 healthy control subjects (mean ± SD age, 44 ±13 years; 53% women) underwent 3-T cardiac MRI including modified Look-Locker inversion recovery T1 mapping. Segmental native T1 spread was calculated as the difference between maximum and minimum segmental native T1 values, expressed as an absolute value and as a relative percentage of global native T1. RESULTS. Absolute and relative segmental native T1 spreads were significantly higher in patients with Fabry disease than in healthy control subjects (absolute median, 115 vs 98 ms [p = 0.004]; relative median, 9.9% vs 8.0% [p < 0.001]) and correlated positively with quantitative late gadolinium enhancement (absolute, r = 0.434, p < 0.001; relative, r = 0.436, p < 0.001), indexed left ventricular mass (absolute, r = 0.316, p = 0.01; relative, r = 0.347, p = 0.007), and global longitudinal strain (absolute, r = 0.289, p = 0.03; relative, r = 0.277, p = 0.03). Relative segmental native T1 spread differentiated patients with Fabry disease from healthy control subjects (odds ratio, 1.44 [95% CI, 1.10-1.89]; p = 0.009). Interob-server agreement was excellent for both absolute (intraclass correlation coefficient, 0.932) and relative (intraclass correlation coefficient, 0.926) segmental native T1 spread. CONCLUSION. Increased native T1 spread is a reproducible imaging biomarker of cardiac involvement in Fabry disease and may be particularly useful in the evaluation of patients who cannot undergo late gadolinium enhancement imaging.

11.
J Thorac Imaging ; 2020 Aug 25.
Artigo em Inglês | MEDLINE | ID: mdl-32852417

RESUMO

PURPOSE: Accurate and reproducible assessment of left ventricular mass (LVM) is important in Fabry disease. However, it is unclear whether papillary muscles should be included in LVM assessed by cardiac magnetic resonance imaging (MRI). The purpose of this study was to evaluate the reproducibility and predictive value of LVM in patients with Fabry disease using different analysis approaches. MATERIALS AND METHODS: A total of 92 patients (44±15 y, 61 women) with confirmed Fabry disease who had undergone cardiac MRI at a single tertiary referral hospital were included in this retrospective study. LVM was assessed at end-diastole using 2 analysis approaches, including and excluding papillary muscles. Adverse cardiac events were assessed as a composite end point, defined as ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death. Statistical analysis included Cox proportional hazard models, Akaike information criterion, intraclass correlation coefficients, and Bland-Altman analysis. RESULTS: Left ventricular end-diastolic volume, end-systolic volume, ejection fraction, and LVM all differed significantly between analysis approaches. LVM was significantly higher when papillary muscles were included versus excluded (157±71 vs. 141±62 g, P<0.001). Mean papillary mass was 16±11 g, accounting for 10%±3% of total LVM. LVM with pap illary muscles excluded had slightly better predictive value for the composite end point compared with LVM with papillary muscles included based on the model goodness-of-fit (Akaike information criterion 140 vs. 142). Interobserver agreement was slightly better for LVM with papillary muscles excluded compared with included (intraclass correlation coefficient 0.993 [95% confidence interval: 0.985, 0.996] vs. 0.989 [95% confidence interval: 0.975, 0.995]) with less bias and narrower limits of agreement. CONCLUSIONS: Inclusion or exclusion of papillary muscles has a significant effect on LVM quantified by cardiac MRI, and therefore, a standardized analysis approach should be used for follow-up. Exclusion of papillary muscles from LVM is a reasonable approach in patients with Fabry disease given slightly better predictive value and reproducibility.

12.
J Am Coll Radiol ; 17(5S): S55-S69, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32370978

RESUMO

Chest pain is a frequent cause for emergency department visits and inpatient evaluation, with particular concern for acute coronary syndrome as an etiology, since cardiovascular disease is the leading cause of death in the United States. Although history-based, electrocardiographic, and laboratory evaluations have shown promise in identifying coronary artery disease, early accurate diagnosis is paramount and there is an important role for imaging examinations to determine the presence and extent of anatomic coronary abnormality and ischemic physiology, to guide management with regard to optimal medical therapy or revascularization, and ultimately to thereby improve patient outcomes. A summary of the various methods for initial imaging evaluation of suspected acute coronary syndrome is outlined in this document. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

13.
J Thorac Imaging ; 2020 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-32251236

RESUMO

PURPOSE: The purpose of this study was to compare thoracic aortic measurements between computed tomography (CT), magnetic resonance imaging (MRI), and transthoracic echocardiography (TTE). MATERIALS AND METHODS: A total of 127 patients (mean age: 45±18 y, 49% male) who had undergone CT and MRI evaluation of the thoracic aorta at a single tertiary referral hospital within a 6-month interval between 2007 and 2017 were included in this retrospective study. TTE studies performed within the same 6-month interval were also evaluated. Thoracic aortic measurements were blindly evaluated using multiple techniques and were compared between modalities. RESULTS: There was no significant difference in maximum aortic root diameter between CT and MRI when using the inner lumen-to-inner lumen technique (mean difference: 0.2±1.4 mm, P=0.51) or the outer lumen-to-outer lumen technique (mean difference: 0.5±1.4 mm, P=0.07). There were no significant differences between CT and MRI at any other level except for the distal descending aorta (20.2±4.6 vs. 19.8±4.6 mm, P<0.001). However, aortic root measurements by TTE using the leading edge-to-leading edge technique were significantly smaller compared with maximum aortic root diameters using the inner lumen-to-inner lumen and outer lumen-to-outer lumen techniques by both CT (mean difference: 4.9±2.7 mm, P<0.001 and 7.4±2.8 mm, P<0.001, respectively) and MRI (mean difference: 4.8±3.2 mm, P<0.001 and 8.2±3.0 mm, P<0.001, respectively). CONCLUSIONS: There is excellent agreement in thoracic aortic measurements between CT and MRI. However, TTE significantly underestimates maximum aortic root diameter compared with CT and MRI. Therefore, caution should be used when interpreting small apparent changes in aortic root diameters between TTE and CT or MRI.

14.
J Thorac Imaging ; 35(5): 334-339, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32195888

RESUMO

PURPOSE: To evaluate the diagnostic utility of the Look Locker inversion time (TI) sequence on cardiac magnetic resonance imaging in patients with suspected cardiac amyloidosis and to evaluate whether there are differences in the nulling pattern between amyloid types. MATERIALS AND METHODS: A total of 144 patients with suspected cardiac amyloidosis who had undergone cardiac magnetic resonance imaging were included in this retrospective study. Sixty-four had cardiac amyloidosis (62.1±9.2 y, 70.3% male, 68.8% had light chain amyloid [AL], 18.8% had familial transthyretin amyloid caused by mutant genes [ATTRm], and 12.5% had wild-type transthyretin amyloid [ATTRwt]) and 80 did not have cardiac amyloidosis (61.3±13.3 y, 58.8% male). Time to myocardial and blood pool nulling on the Look Locker TI sequence was classified as normal if blood pool nulled before myocardium or abnormal if blood pool nulling was coincident with or after myocardial nulling. RESULTS: The nulling pattern was abnormal in 26 patients with cardiac amyloidosis compared with none of the patients without cardiac amyloidosis (40.6% vs. 0.0%, P<0.0001). Abnormal nulling had 40.6% sensitivity and 100% specificity for cardiac amyloidosis (area under the receiver operating characteristic curve: 0.703, 95% confidence interval: 0.642-0.764). All patients with cardiac amyloidosis with an abnormal nulling pattern demonstrated late gadolinium enhancement. Among patients with cardiac amyloidosis, there was no significant difference in abnormal nulling between AL, ATTRm, and ATTRwt amyloid types (31.8%, 58.3%, 62.5%, respectively, P=0.10). CONCLUSIONS: An abnormal nulling pattern on the Look Locker TI sequence is highly specific for cardiac amyloidosis when present. However, abnormal nulling is a late finding with low sensitivity and does not differentiate between amyloid types.

15.
Circ Genom Precis Med ; 13(2): e002748, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32150461

RESUMO

Background Genetic testing is helpful for diagnosis of hypertrophic cardiomyopathy (HCM) mimics. Little data are available regarding the yield of such testing and its clinical impact. Methods The HCM genetic database at our center was used for identification of patients who underwent HCM-directed genetic testing including at least 1 gene associated with an HCM mimic (GLA, TTR, PRKAG2, LAMP2, PTPN11, RAF1, and DES). Charts were retrospectively reviewed and genetic and clinical data extracted. Results There were 1731 unrelated HCM patients who underwent genetic testing for at least 1 gene related to an HCM mimic. In 1.45% of cases, a pathogenic or likely pathogenic variant in one of these genes was identified. This included a yield of 1% for Fabry disease, 0.3% for familial amyloidosis, 0.15% for PRKAG2-related cardiomyopathy, and 1 patient with Noonan syndrome. In the majority of patients, diagnosis of the HCM mimic based on clinical findings alone would have been challenging. Accurate diagnosis of an HCM mimic led to change in management (eg, enzyme replacement therapy) or family screening in all cases. Conclusions Genetic testing is helpful in the diagnosis of HCM mimics in patients with no or few extracardiac manifestations. Adding these genes to all HCM genetic panels should be considered.

16.
J Cardiovasc Magn Reson ; 22(1): 9, 2020 Jan 30.
Artigo em Inglês | MEDLINE | ID: mdl-31996239

RESUMO

BACKGROUND: Investigation of the myocardial strain characteristics of the left ventricular non-compaction (LVNC) phenotype with cardiovascular magnetic resonance (CMR) feature tracking. METHODS: CMR cine balanced steady-state free precession data sets of 59 retrospectively identified LVNC phenotype patients (40 years, IQR: 28-50 years; 51% male) and 36 healthy subjects (39 years, IQR: 30-47 years; 44% male) were evaluated for LV volumes, systolic function and mass. Hypertrabeculation in patients and healthy subjects was evaluated against established CMR diagnostic criteria. Global circumferential strain (GCS), global radial strain (GRS) and global longitudinal strain (GLS) were evaluated with feature-tracking software. Subgroup analyses were performed in patients (n = 25) and healthy subjects (n = 34) with normal LV volumetrics, and with healthy subjects (n = 18) meeting at least one LVNC diagnostic criteria. RESULTS: All LVNC phenotype patients, as well as a significant proportion of healthy subjects, met morphology-based CMR diagnostic criteria: non-compacted (NC): compacted myocardial diameter ratio > 2.3 (100% vs. 19.4%), NC mass > 20% (100% vs. 44.4%) and > 25% (100% vs. 13.9%), and NC mass indexed to body surface area > 15 g/m2 (100% vs. 41.7%). LVNC phenotype patients demonstrated reduced GRS (26.4% vs. 37.1%; p < 0.001), GCS (- 16.5% vs. -20.5%; p < 0.001) and GLS (- 14.6% vs. -17.1%; p < 0.001) compared to healthy subjects, with statistically significant differences persisting on subgroup comparisons of LVNC phenotype patients with healthy subjects meeting diagnostic criteria. GCS also demonstrated independent and incremental diagnostic value beyond each of the morphology-based CMR diagnostic criteria. CONCLUSIONS: LVNC phenotype patients demonstrate impaired strain by CMR feature tracking, also present on comparison of subjects with normal LV volumetrics meeting diagnostic criteria. The high proportion of healthy subjects meeting morphology-based CMR diagnostic criteria emphasizes the important potential complementary diagnostic value of strain in differentiating LVNC from physiologic hypertrabeculation.


Assuntos
Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Função Ventricular Esquerda , Adulto , Bases de Dados Factuais , Feminino , Humanos , Interpretação de Imagem Assistida por Computador , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Estudos Retrospectivos
17.
Radiology ; 294(1): 42-49, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31660802

RESUMO

Background Cardiac involvement is the leading cause of mortality in patients with Fabry disease. Identification of imaging findings that predict adverse cardiac events is needed to enable identification of high-risk patients. Purpose To establish the prognostic value of cardiac MRI findings in men and women with Fabry disease. Materials and Methods Consecutive women and men with gene-positive Fabry disease who had undergone cardiac MRI at a single large tertiary referral hospital between March 2008 and January 2019 were included in this retrospective cohort study. Evaluators of cardiac MRI studies were blinded to all clinical information. Adverse cardiac events were assessed as a composite end point, defined as ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death. Statistical analysis included Cox proportional hazard models adjusted for age and Mainz Severity Score Index (a measure of the severity of Fabry disease). Results Ninety patients (mean age, 44 years ± 15 [standard deviation]; 59 women) were evaluated. After a median follow-up period of 3.6 years, the composite end point was reached in 21 patients (incidence rate, 7.6% per year). Left ventricular hypertrophy (LVH) and late gadolinium enhancement (LGE) were independent predictors of the composite end point in adjusted analysis (LVH hazard ratio [HR], 3.0; 95% confidence interval [CI]: 1.1, 8.1; P = .03; and LGE HR, 7.2; 95% CI: 1.5, 34; P = .01). Patients with extensive LGE (≥15% of left ventricular mass) were at highest risk (HR, 12; 95% CI: 2.0, 67; P = .006). Sex did not modify the relationship between the composite end point and any of the cardiac MRI parameters, including LVH (P = .15 for interaction term) and LGE (P = .38 for interaction term). Conclusion Cardiac MRI findings of left ventricular hypertrophy and late gadolinium enhancement can be used to identify patients with Fabry disease who are at high risk of adverse cardiac events. © RSNA, 2019 See also the editorial by Zimmerman in this issue.


Assuntos
Doença de Fabry/complicações , Gadolínio/farmacocinética , Insuficiência Cardíaca/complicações , Hipertrofia Ventricular Esquerda/complicações , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Aumento da Imagem/métodos , Imagem por Ressonância Magnética/métodos , Adulto , Estudos de Coortes , Meios de Contraste/farmacocinética , Feminino , Seguimentos , Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
19.
Am J Cardiol ; 124(10): 1636-1642, 2019 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-31540664

RESUMO

The cardiovascular magnetic resonance imaging (CMR) features of adults with repaired truncus arteriosus (rTA) are largely undefined. We sought to explore CMR characteristics in rTA and to identify associations between imaging findings and cardiovascular outcomes. Adults with rTA and CMR were identified and anatomic subtypes (1-4) were assigned (Collett and Edwards classification). CMR characteristics, clinical data at last follow-up and adverse cardiovascular outcome were recorded. Twenty-seven adults (19% male) were studied (median age at cardiovascular magnetic resonance 26 years [interquartile range 18 to 40]) over 5.2-year duration [interquartile range 2.5 to 7.5]. With the exception of mildly increased RV mass (30 ± 12 g/m2), cardiac chamber measurements were within the normal range. In CMR measurements, only pulmonary artery peak velocity differed in subtypes (highest in subtype 3, 318 ± 26 cm/s, p = 0.029). Number of cardiovascular interventions in adulthood was moderately correlated with left ventricular end-diastolic volume (r = 0.463, p = 0.015), left ventricular ejection fraction (r = 0.425, p = 0.027) and neoaortic root size (r = 0.398, p = 0.039). Cardiovascular events (nonmutually exclusive) in 5 of 27 patients (19%) included death (n = 1), heart failure (n = 1), ventricular tachycardia (n = 1), and atrial tachycardia (n = 3). Increased cardiovascular risk was associated with decreased right ventricular ejection fraction (odds ratio 1.153, confidence interval 1.003 to 1.326, p = 0.046) and smaller ascending aorta diameter (odds ratio 1.758, confidence interval 1.037 to 2.976, p = 0.036). In conclusion, decreased right ventricular ejection fraction and smaller ascending aorta on cardiovascular magnetic resonance were associated with adverse events in rTA.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Persistência do Tronco Arterial/cirurgia , Tronco Arterial/diagnóstico por imagem , Adolescente , Adulto , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Persistência do Tronco Arterial/diagnóstico , Persistência do Tronco Arterial/fisiopatologia , Adulto Jovem
20.
J Cardiovasc Magn Reson ; 21(1): 45, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31366357

RESUMO

BACKGROUND: Cardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. METHODS: In this prospective study, 38 FD patients (45.0 ± 14.5 years, 37% male) and 8 healthy controls (40.1 ± 13.7 years, 63% male) underwent 3 T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis. RESULTS: Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (- 15.3 ± 3.5% vs. - 16.3 ± 1.5%, p = 0.45), GCS (- 19.4 ± 3.0% vs. -19.5 ± 2.9%, p = 0.84) or base-to-apex LS gradient (7.5 ± 3.8% vs. 9.3 ± 3.5%, p = 0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 ± 3.7% vs. 6.5 ± 2.2%, p = 0.002) and native T1 (1170.2 ± 37.5 ms vs. 1239.0 ± 18.0 ms, p < 0.001). Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy controls (OR 0.42, 95% CI: 0.20 to 0.86, p = 0.019), even after controlling for native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p = 0.049). In a nested logistic regression model with native T1, model fit was significantly improved by the addition of base-to-apex CS gradient (χ2(df = 1) = 11.04, p < 0.001). Intra- and inter-observer agreement were moderate to good for myocardial strain parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). CONCLUSIONS: CMR reproducibly identifies myocardial strain abnormalities in FD. Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in FD, with independent and incremental value beyond native T1.


Assuntos
Cardiomiopatias/diagnóstico , Meios de Contraste/administração & dosagem , Doença de Fabry/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/diagnóstico , Imagem Cinética por Ressonância Magnética , Contração Miocárdica , Compostos Organometálicos/administração & dosagem , Função Ventricular Esquerda , Remodelação Ventricular , Adulto , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Doença de Fabry/fisiopatologia , Feminino , Humanos , Hipertrofia Ventricular Esquerda/fisiopatologia , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes
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