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1.
Circulation ; 149(25): 1938-1948, 2024 06 18.
Artigo em Inglês | MEDLINE | ID: mdl-38804148

RESUMO

BACKGROUND: Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification. METHODS: In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment. RESULTS: During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; P<0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (P=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (P=0.167) or valvular dysfunction. CONCLUSIONS: Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels. REGISTRATION: URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.


Assuntos
Valva Aórtica , Atorvastatina , Doença da Válvula Aórtica Bicúspide , Calcinose , Progressão da Doença , Doenças das Valvas Cardíacas , Inibidores de Hidroximetilglutaril-CoA Redutases , Humanos , Atorvastatina/uso terapêutico , Feminino , Masculino , Pessoa de Meia-Idade , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/patologia , Valva Aórtica/anormalidades , Valva Aórtica/efeitos dos fármacos , Calcinose/tratamento farmacológico , Calcinose/diagnóstico por imagem , Calcinose/patologia , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Doença da Válvula Aórtica Bicúspide/tratamento farmacológico , Doenças das Valvas Cardíacas/tratamento farmacológico , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/patologia , Adulto , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Dilatação Patológica/tratamento farmacológico , Seguimentos , Método Duplo-Cego , Resultado do Tratamento , Aorta/diagnóstico por imagem , Aorta/patologia , Aorta/efeitos dos fármacos , Valvopatia Aórtica/tratamento farmacológico , Estenose da Valva Aórtica
2.
J Cardiovasc Magn Reson ; 26(1): 100992, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38211655

RESUMO

BACKGROUND: The measurement of aortic dimensions and their evolution are key in the management of patients with aortic diseases. Manual assessment, the current guideline-recommended method and clinical standard, is subjective, poorly reproducible, and time-consuming, limiting the capacity to track aortic growth in everyday practice. Aortic geometry mapping (AGM) via image registration of serial computed tomography angiograms outperforms manual assessment, providing accurate and reproducible 3D maps of aortic diameter and growth rate. This observational study aimed to evaluate the accuracy and reproducibility of AGM on non-gated contrast-enhanced (CE-) and cardiac- and respiratory-gated (GN-) magnetic resonance angiographies (MRA). METHODS: Patients with thoracic aortic disease followed with serial CE-MRA (n = 30) or GN-MRA (n = 15) acquired at least 1 year apart were retrospectively and consecutively identified. Two independent observers measured aortic diameters and growth rates (GR) manually at several thoracic aorta reference levels and with AGM. Agreement between manual and AGM measurements and their inter-observer reproducibility were compared. Reproducibility for aortic diameter and GR maps assessed with AGM was obtained. RESULTS: Mean follow-up was 3.8 ± 2.3 years for CE- and 2.7 ± 1.6 years for GN-MRA. AGM was feasible in the 93% of CE-MRA pairs and in the 100% of GN-MRA pairs. Manual and AGM diameters showed excellent agreement and inter-observer reproducibility (ICC>0.9) at all anatomical levels. Agreement between manual and AGM GR was more limited, both in the aortic root by GN-MRA (ICC=0.47) and in the thoracic aorta, where higher accuracy was obtained with GN- than with CE-MRA (ICC=0.55 vs 0.43). The inter-observer reproducibility of GR by AGM was superior compared to manual assessment, both with CE- (thoracic: ICC= 0.91 vs 0.51) and GN-MRA (root: ICC=0.84 vs 0.52; thoracic: ICC=0.93 vs 0.60). AGM-based 3D aortic size and growth maps were highly reproducible (median ICC >0.9 for diameters and >0.80 for GR). CONCLUSION: Mapping aortic diameter and growth on MRA via 3D image registration is feasible, accurate and outperforms the current manual clinical standard. This technique could broaden the possibilities of clinical and research evaluation of patients with aortic thoracic diseases.


Assuntos
Aorta Torácica , Doenças da Aorta , Meios de Contraste , Imageamento Tridimensional , Angiografia por Ressonância Magnética , Variações Dependentes do Observador , Valor Preditivo dos Testes , Humanos , Reprodutibilidade dos Testes , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Aorta Torácica/diagnóstico por imagem , Idoso , Meios de Contraste/administração & dosagem , Doenças da Aorta/diagnóstico por imagem , Técnicas de Imagem de Sincronização Respiratória , Adulto , Fatores de Tempo , Interpretação de Imagem Assistida por Computador , Técnicas de Imagem de Sincronização Cardíaca
3.
J Clin Med ; 13(13)2024 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-38999432

RESUMO

Introduction: Non-ischemic dilated cardiomyopathy (NIDCM) is characterized by a reduced left ventricular (LV) ejection fraction (LVEF, <50%) and a high risk for heart failure (HF) and death. Echocardiography-derived hemodynamic forces (HDFs) may provide important information on LV mechanics, but their prognostic value is unknown. Aim: To explore the features of echocardiography-derived HDFs in NIDCM and their association with clinical endpoints. Methods: Asymptomatic, non-hospitalized NIDCM patients free from coronary artery disease and moderate or severe valvular heart disease were included in this single-center observational retrospective longitudinal study. Those with atrial fibrillation and a follow-up <12 months were excluded. Major adverse cardiovascular events (MACE) were defined as a composite of all-cause death, HF hospitalization, and ambulatory intravenous diuretics administration. LV HDFs were analyzed with a prototype software. Apex-base (HDFs-ab), lateral-septal (HDFs-ls), and HDFs-angle were computed. Results: Ninety-seven patients were included, sixty-seven (69%) were males, mean age was 62 ± 14 years, and mean LVEF was 39.2 ± 8.6%. During a median follow-up of 4.2 (3.1-5.1) years, 19 (20%) patients experienced MACE. These patients had a higher HDFs-angle (71.0 (67.0-75.0) vs. 68.0 (63.0-71.0)°, p = 0.005), lower HDFs-ls (1.36 (1.01-1.85) vs. 1.66 ([1.28-2.04])%, p = 0.015), but similar HDFs-ab (5.02 (4.39-6.34) vs. 5.66 (4.53-6.78)%, p = 0.375) compared to those without MACE. in a Cox regression analysis, HDFs-angle (HR 1.16 (95%-CI 1.04-1.30), p = 0.007) was associated with MACE, while other conventional echocardiography parameters, including LVEF and LV longitudinal strain, were not. Conclusions: HDFs-angle is associated with clinical endpoints in NIDCM. A higher HDFs-angle may be a marker of impaired myocardial performance in patients with reduced LVEF.

4.
Sci Rep ; 14(1): 18768, 2024 08 13.
Artigo em Inglês | MEDLINE | ID: mdl-39138245

RESUMO

Untargeted metabolomic analysis is a powerful tool used for the discovery of novel biomarkers. Chagas disease (CD), caused by Trypanosoma cruzi, is a neglected tropical disease that affects 6-7 million people with approximately 30% developing cardiac manifestations. The most significant clinical challenge lies in its long latency period after acute infection, and the lack of surrogate markers to predict disease progression or cure. In this cross-sectional study, we analyzed sera from 120 individuals divided into four groups: 31 indeterminate CD, 41 chronic chagasic cardiomyopathy (CCC), 18 Latin Americans with other cardiomyopathies and 30 healthy volunteers. Using a high-throughput panel of 986 metabolites, we identified three distinct profiles among individuals with cardiomyopathy, indeterminate CD and healthy volunteers. After a more stringent analysis, we identified some potential biomarkers. Among peptides, phenylacetylglutamine and fibrinopeptide B (1-13) exhibited an increasing trend from controls to ICD and CCC. Conversely, reduced levels of bilirubin and biliverdin alongside elevated urobilin correlated with disease progression. Finally, elevated levels of cystathionine, phenol glucuronide and vanillactate among amino acids distinguished CCC individuals from ICD and controls. Our novel exploratory study using metabolomics identified potential biomarker candidates, either alone or in combination that if confirmed, can be translated into clinical practice.


Assuntos
Biomarcadores , Doença de Chagas , Metabolômica , Humanos , Biomarcadores/sangue , Metabolômica/métodos , Masculino , Feminino , Doença de Chagas/sangue , Doença de Chagas/diagnóstico , Pessoa de Meia-Idade , Adulto , Estudos Transversais , Metaboloma , Cardiomiopatia Chagásica/sangue , Cardiomiopatia Chagásica/metabolismo , Idoso
5.
Eur Heart J Cardiovasc Imaging ; 25(7): 892-900, 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38568982

RESUMO

AIMS: To assess the current role of cardiac imaging in the diagnosis, management, and follow-up of patients with acute myocarditis (AM) through a European Association of Cardiovascular Imaging survey. METHODS AND RESULTS: A total of 412 volunteers from 74 countries responded to the survey. Most participants worked in tertiary centres (56%). All participants had access to echocardiography, while 79 and 75% had access to cardiac computed tomography angiography (CCTA) and cardiac magnetic resonance (CMR), respectively. Less than half (47%) had access to myocardial biopsy, and only 5% used this test routinely. CMR was performed within 7 days of presentation in 73% of cases. Non-ischaemic late gadolinium enhancement (LGE, 88%) and high-signal intensity in T2-weighted images (74%) were the most used diagnostic criteria for AM. CCTA was preferred to coronary angiography by 47% of participants to exclude coronary artery disease. Systematic prescription of beta-blockers and angiotensin-converting enzyme inhibitors was reported by 38 and 32% of participants. Around a quarter of participants declared considering LGE burden as a reason to treat. Most participants (90%) reported performing a follow-up echocardiogram, while 63% scheduled a follow-up CMR. The main reason for treatment discontinuation was improvement of left ventricular ejection fraction (89%), followed by LGE regression (60%). In two-thirds of participants, the decision to resume high-intensity sport was influenced by residual LGE. CONCLUSION: This survey confirms the high utilization of cardiac imaging in AM but reveals major differences in how cardiac imaging is used and how the condition is managed between centres, underlining the need for recommendation statements in this topic.


Assuntos
Imagem Multimodal , Miocardite , Humanos , Miocardite/diagnóstico por imagem , Masculino , Feminino , Europa (Continente) , Doença Aguda , Imagem Cinética por Ressonância Magnética/métodos , Angiografia por Tomografia Computadorizada , Adulto , Ecocardiografia , Pessoa de Meia-Idade , Sociedades Médicas , Inquéritos e Questionários , Angiografia Coronária
6.
Artigo em Inglês | MEDLINE | ID: mdl-39001760

RESUMO

BACKGROUND: Genetic disease has recently emerged as a cause of cardiac conduction disorders (CCDs), but the diagnostic yield of genetic testing and the contribution of the different genes to CCD is still unsettled. OBJECTIVES: This study sought to determine the diagnostic yield of genetic testing in young adults with CCD of unknown etiology requiring pacemaker implantation. We also studied the prevalence of rare protein-altering variants across individual genes and functional gene groups. METHODS: We performed whole exome sequencing in 150 patients with CCD of unknown etiology who had permanent pacemaker implanted at age ≤60 years at 14 Spanish hospitals. Prevalence of rare protein-altering variants in patients with CCD was compared with a reference population of 115,522 individuals from gnomAD database (control subjects). RESULTS: Among 39 prioritized genes, patients with CCD had more rare protein-altering variants than control subjects (OR: 2.39; 95% CI: 1.75-3.33). Significant enrichment of rare variants in patients with CCD was observed in all functional gene groups except in the desmosomal genes group. Rare variants in the nuclear envelope genes group exhibited the strongest association with CCD (OR: 6.77; 95% CI: 3.71-13.87). Of note, rare variants in sarcomeric genes were also enriched (OR: 1.73; 95% CI: 1.05-3.10). An actionable genetic variant was detected in 21 patients (14%), with LMNA being the most frequently involved gene (4.6%). CONCLUSIONS: Unrecognized rare genetic variants increase the risk of CCD in young adults with CCD of unknown etiology. Genetic testing should be performed in patients age ≤60 years with CCD of unknown etiology. The role of genetic variants in sarcomeric genes as a cause of CCD should be further investigated.

7.
Circ Genom Precis Med ; 17(2): e004404, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38353104

RESUMO

BACKGROUND: Less than 40% of patients with dilated cardiomyopathy (DCM) have a pathogenic/likely pathogenic genetic variant identified. TBX20 has been linked to congenital heart defects; although an association with left ventricular noncompaction (LVNC) and DCM has been proposed, it is still considered a gene with limited evidence for these phenotypes. This study sought to investigate the association between the TBX20 truncating variant (TBX20tv) and DCM/LVNC. METHODS: TBX20 was sequenced by next-generation sequencing in 7463 unrelated probands with a diagnosis of DCM or LVNC, 22 773 probands of an internal comparison group (hypertrophic cardiomyopathy, channelopathies, or aortic diseases), and 124 098 external controls (individuals from the gnomAD database). Enrichment of TBX20tv in DCM/LVNC was calculated, cosegregation was determined in selected families, and clinical characteristics and outcomes were analyzed in carriers. RESULTS: TBX20tv was enriched in DCM/LVNC (24/7463; 0.32%) compared with internal (1/22 773; 0.004%) and external comparison groups (4/124 098; 0.003%), with odds ratios of 73.23 (95% CI, 9.90-541.45; P<0.0001) and 99.76 (95% CI, 34.60-287.62; P<0.0001), respectively. TBX20tv was cosegregated with DCM/LVNC phenotype in 21 families for a combined logarythm of the odds score of 4.53 (strong linkage). Among 57 individuals with TBX20tv (49.1% men; mean age, 35.9±20.8 years), 41 (71.9%) exhibited DCM/LVNC, of whom 14 (34.1%) had also congenital heart defects. After a median follow-up of 6.9 (95% CI, 25-75:3.6-14.5) years, 9.7% of patients with DCM/LVNC had end-stage heart failure events and 4.8% experienced malignant ventricular arrhythmias. CONCLUSIONS: TBX20tv is associated with DCM/LVNC; congenital heart defect is also present in around one-third of cases. TBX20tv-associated DCM/LVNC is characterized by a nonaggressive phenotype, with a low incidence of major cardiovascular events. TBX20 should be considered a definitive gene for DCM and LVNC and routinely included in genetic testing panels for these phenotypes.


Assuntos
Cardiomiopatia Dilatada , Cardiopatias Congênitas , Masculino , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Feminino , Cardiomiopatia Dilatada/patologia , Cardiopatias Congênitas/genética , Arritmias Cardíacas , Fenótipo , Proteínas com Domínio T/genética
8.
Artigo em Inglês | MEDLINE | ID: mdl-39115499

RESUMO

BACKGROUND: Aortic stenosis (AS) is characterized by calcification and fibrosis. The ability to quantify these processes simultaneously has been limited with previous imaging methods. OBJECTIVES: The purpose of this study was to evaluate the aortic valve fibrocalcific volume by computed tomography (CT) angiography in patients with AS, in particular, to assess its reproducibility, association with histology and disease severity, and ability to predict/track progression. METHODS: In 136 patients with AS, fibrocalcific volume was calculated on CT angiograms at baseline and after 1 year. CT attenuation distributions were analyzed using Gaussian-mixture-modeling to derive thresholds for tissue types enabling the quantification of calcific, noncalcific, and fibrocalcific volumes. Scan-rescan reproducibility was assessed and validation provided against histology and in an external cohort. RESULTS: Fibrocalcific volume measurements took 5.8 ± 1.0 min/scan, demonstrating good correlation with ex vivo valve weight (r = 0.51; P < 0.001) and excellent scan-rescan reproducibility (mean difference -1%, limits of agreement -4.5% to 2.8%). Baseline fibrocalcific volumes correlated with mean gradient on echocardiography in both male and female participants (rho = 0.64 and 0.69, respectively; both P < 0.001) and in the external validation cohort (n = 66, rho = 0.58; P < 0.001). The relationship was driven principally by calcific volume in men and fibrotic volume in women. After 1 year, fibrocalcific volume increased by 17% and correlated with progression in mean gradient (rho = 0.32; P = 0.003). Baseline fibrocalcific volume was the strongest predictor of subsequent mean gradient progression, with a particularly strong association in female patients (rho = 0.75; P < 0.001). CONCLUSIONS: The aortic valve fibrocalcific volume provides an anatomic assessment of AS severity that can track disease progression precisely. It correlates with disease severity and hemodynamic progression in both male and female patients.

9.
Hellenic J Cardiol ; 2024 May 09.
Artigo em Inglês | MEDLINE | ID: mdl-38729346

RESUMO

OBJECTIVE: To evaluate the clinical characteristics, imaging findings, treatment, and prognosis of patients with type A acute aortic syndrome (AAS-A) presenting with shock. To assess the impact of surgery on this patient population. METHODS: The study included 521 patients with A-AAS enrolled in the Spanish Registry of Acute Aortic Syndrome (RESA-III) from January 2018 to December 2019. The RESA-III is a prospective, multicenter registry that contains AAS data from 30 tertiary-care hospitals. Patients were classified into two groups according to their clinical presentation, with or without shock. Shock was defined as persistent systolic blood pressure <80 mmHg despite adequate volume resuscitation. RESULTS: 97 (18.6%) patients with A-AAS presented with shock. Clinical presentation with syncope was much more common in the Shock group (45.4% vs 10.1%, p = 0.001). Patients in the Shock group had more complications at diagnosis and before surgery: cardiac tamponade (36.2% vs 9%, p < 0.001), acute renal failure (28.9% vs 18.2%, p = 0.018), and need for orotracheal intubation (40% vs 9.1%, p < 0.001). There were no significant differences in aortic regurgitation (51.6% vs 46.7%, p = 0.396) between groups. In-hospital mortality was higher among patients with shock (48.5% vs 27.4%, p < 0.001). Surgery was associated with a significant mortality reduction both in patients with and without shock. Surgery had an independent protective effect on mortality (OR 0.03, 95% CI (0.00-0.32)). CONCLUSION: Patients with AAS-A admitted with shock have a heavily increased risk of mortality. Syncope and pericardial effusion at diagnosis are strongly associated with shock. Surgery was independently associated with a mortality reduction in patients with AAS-A and shock.

10.
J Am Coll Cardiol ; 83(17): 1640-1651, 2024 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-38658103

RESUMO

BACKGROUND: Disease penetrance in genotype-positive (G+) relatives of families with dilated cardiomyopathy (DCM) and the characteristics associated with DCM onset in these individuals are unknown. OBJECTIVES: This study sought to determine the penetrance of new DCM diagnosis in G+ relatives and to identify factors associated with DCM development. METHODS: The authors evaluated 779 G+ patients (age 35.8 ± 17.3 years; 459 [59%] females; 367 [47%] with variants in TTN) without DCM followed at 25 Spanish centers. RESULTS: After a median follow-up of 37.1 months (Q1-Q3: 16.3-63.8 months), 85 individuals (10.9%) developed DCM (incidence rate of 2.9 per 100 person-years; 95% CI: 2.3-3.5 per 100 person-years). DCM penetrance and age at DCM onset was different according to underlying gene group (log-rank P = 0.015 and P <0.01, respectively). In a multivariable model excluding CMR parameters, independent predictors of DCM development were: older age (HR per 1-year increase: 1.02; 95% CI: 1.0-1.04), an abnormal electrocardiogram (HR: 2.13; 95% CI: 1.38-3.29); presence of variants in motor sarcomeric genes (HR: 1.92; 95% CI: 1.05-3.50); lower left ventricular ejection fraction (HR per 1% increase: 0.86; 95% CI: 0.82-0.90) and larger left ventricular end-diastolic diameter (HR per 1-mm increase: 1.10; 95% CI: 1.06-1.13). Multivariable analysis in individuals with cardiac magnetic resonance and late gadolinium enhancement assessment (n = 360, 45%) identified late gadolinium enhancement as an additional independent predictor of DCM development (HR: 2.52; 95% CI: 1.43-4.45). CONCLUSIONS: Following a first negative screening, approximately 11% of G+ relatives developed DCM during a median follow-up of 3 years. Older age, an abnormal electrocardiogram, lower left ventricular ejection fraction, increased left ventricular end-diastolic diameter, motor sarcomeric genetic variants, and late gadolinium enhancement are associated with a higher risk of developing DCM.


Assuntos
Cardiomiopatia Dilatada , Genótipo , Penetrância , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/fisiopatologia , Conectina/genética , Eletrocardiografia , Seguimentos , Espanha/epidemiologia , Estudos Retrospectivos
11.
Radiol Cardiothorac Imaging ; 6(3): e230247, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38900026

RESUMO

Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This retrospective study included patients with MVP without hemodynamically significant mitral regurgitation or left ventricular (LV) dysfunction undergoing late gadolinium enhancement (LGE) cardiac MRI between October 2007 and June 2020 in 15 European tertiary centers. The study end point was a composite of sustained ventricular tachycardia, (aborted) sudden cardiac death, or unexplained syncope. Unsupervised data-driven hierarchical k-mean algorithm was utilized to identify phenotypic clusters. The association between clusters and the study end point was assessed by Cox proportional hazards model. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 female, 230 male) with two phenotypic clusters were identified. Patients in cluster 2 (199 of 474, 42%) had more severe mitral valve degeneration (ie, bileaflet MVP and leaflet displacement), left and right heart chamber remodeling, and myocardial fibrosis as assessed with LGE cardiac MRI than those in cluster 1. Demographic and clinical features (ie, symptoms, arrhythmias at Holter monitoring) had negligible contribution in differentiating the two clusters. Compared with cluster 1, the risk of developing the study end point over a median follow-up of 39 months was significantly higher in cluster 2 patients (hazard ratio: 3.79 [95% CI: 1.19, 12.12], P = .02) after adjustment for LGE extent. Conclusion Among patients with MVP without significant mitral regurgitation or LV dysfunction, unsupervised machine learning enabled the identification of two phenotypic clusters with distinct arrhythmic outcomes based primarily on cardiac MRI features. These results encourage the use of in-depth imaging-based phenotyping for implementing arrhythmic risk prediction in MVP. Keywords: MR Imaging, Cardiac, Cardiac MRI, Mitral Valve Prolapse, Cluster Analysis, Ventricular Arrhythmia, Sudden Cardiac Death, Unsupervised Machine Learning Supplemental material is available for this article. © RSNA, 2024.


Assuntos
Prolapso da Valva Mitral , Fenótipo , Aprendizado de Máquina não Supervisionado , Humanos , Prolapso da Valva Mitral/diagnóstico por imagem , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sistema de Registros , Imagem Cinética por Ressonância Magnética/métodos , Arritmias Cardíacas/diagnóstico por imagem , Arritmias Cardíacas/fisiopatologia , Adulto , Imageamento por Ressonância Magnética
12.
Rev. esp. cardiol. (Ed. impr.) ; 75(12): 1059-1069, dic. 2022. ilus, tab
Artigo em Espanhol | IBECS (Espanha) | ID: ibc-212939

RESUMO

El miocardio no compactado es una entidad mal definida y en controversia, con una amplia expresividad fenotípica: desde un simple rasgo anatómico hasta una enfermedad con grave afección cardiaca. Los criterios diagnósticos actuales se basan únicamente en hallazgos morfológicos de hipertrabeculación y tienen una baja especificidad para identificar casos de miocardiopatía. El tratamiento del miocardio no compactado también es heterogéneo y no existen guías de práctica clínica específicas. La insuficiencia cardiaca, las arritmias ventriculares y las embolias sistémicas son las complicaciones cardiovasculares más frecuentes. En esta revisión, se tratan las limitaciones diagnósticas de los diferentes criterios disponibles y se propone una aproximación holística alternativa (que incluye variables funcionales por imagen, de caracterización tisular genética y estudio familiar) que puede ayudar en el diagnóstico diferencial de casos con hipertrabeculación. Se describe la genética de esta entidad y el solapamiento con otras miocardiopatías. Por último, se centra en aspectos debatidos del tratamiento clínico y se propone utilizar las mismas variables ya comentadas para la estratificación pronóstica e individualizar el seguimiento de los pacientes.(AU)


Left ventricular noncompaction is a poorly defined and controversial entity, with wide phenotypic expression: from a simple anatomical trait to a disease with overt cardiac affection. Current diagnostic criteria rely exclusively on morphologic features of hypertrabeculation, which have low specificity for identifying true cardiomyopathy cases. The management of left ventricular noncompaction is also heterogeneous, and there are no dedicated clinical practice guidelines. The most common cardiovascular complications are heart failure, ventricular arrhythmias, and systemic embolisms. In this review, we discuss the diagnostic limitations of the available criteria, and propose a comprehensive alternative approach (including functional imaging variables, tissue characterization, genetics, and family screening) that may help in the differential diagnosis of hypertrabeculation cases. We also describe the genetic background of the disease and discuss the overlap with other cardiomyopathies. Finally, we focus on controversial issues in clinical management and suggest the use of the previously-mentioned variables for risk stratification and for individualization of patient follow-up.(AU)


Assuntos
Humanos , Cardiomiopatias/diagnóstico , Cardiomiopatias/genética , Cardiomiopatias/terapia , Ventrículos do Coração , Diagnóstico Diferencial , Cardiopatias Congênitas , Cardiologia , Cardiopatias
20.
Rev. esp. cardiol. (Ed. impr.) ; 68(2): 129-135, feb. 2015. ilus
Artigo em Espanhol | IBECS (Espanha) | ID: ibc-132556

RESUMO

En este artículo se realiza una revisión de las aportaciones de las técnicas de imagen más relevantes a la cardiología que se han publicado durante este año. El ecocardiograma sigue siendo la piedra angular en el diagnóstico y el seguimiento de las valvulopatías, con un esfuerzo continuo para mejorar su cuantificación y obtener parámetros pronósticos de seguimiento. El estudio de la función miocárdica regional se afianza en el diagnóstico de la disfunción ventricular subclínica, y el ecocardiograma transesofágico tridimensional se ha convertido en el perfecto aliado del intervencionismo en las cardiopatías estructurales. La cardiorresonancia y la tomografía computarizada cardiaca acaparan la mayoría de las publicaciones en imagen cardiaca relativas a la cardiopatía isquémica, reflejo de unas técnicas más que consolidadas en la práctica clínica. La medicina nuclear destaca en el estudio de la viabilidad miocárdica tras el intervencionismo en el síndrome coronario agudo y refuerza su rendimiento en el diagnóstico de la cardiopatía isquémica (AU)


In this article, we review the contributions of the most important imaging techniques used in cardiology, reported in 2014. Echocardiography remains the cornerstone for diagnosing and monitoring valvular heart disease, and there has been a continuing effort to improve quantification of this condition and obtain prognostic parameters for follow-up. The study of regional myocardial function is anchored in the diagnosis of subclinical ventricular dysfunction, and 3-dimensional transesophageal echocardiography has become the perfect ally in interventional procedures for structural heart disease. Cardiac magnetic resonance imaging and cardiac computed tomography are the focus of most publications on cardiac imaging in ischemic heart disease, reflecting their consolidated use in clinical practice. Nuclear medicine excels in the study of myocardial viability after interventional treatment of acute coronary syndromes and its performance is validated in the diagnosis of ischemic heart disease (AU)


Assuntos
Humanos , Diagnóstico por Imagem/métodos , Cardiopatias/diagnóstico , Ecocardiografia/métodos , Tomografia Computadorizada por Emissão de Fóton Único de Sincronização Cardíaca/métodos , Tomografia Computadorizada por Raios X/métodos , Espectroscopia de Ressonância Magnética/métodos
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