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Cardiomiopatía Hipertrófica , Muerte Súbita Cardíaca , Músculos Papilares , Humanos , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Muerte Súbita Cardíaca/etiología , Músculos Papilares/diagnóstico por imagen , Ecocardiografía/métodos , Valor Predictivo de las Pruebas , MasculinoAsunto(s)
Cardiomiopatía Hipertrófica , Humanos , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/complicaciones , Estado de Salud , Masculino , Femenino , Tabiques Cardíacos/diagnóstico por imagen , Persona de Mediana Edad , Pirimidinas/uso terapéutico , Bencilaminas , Uracilo/análogos & derivadosRESUMEN
This case report concerns a 48-year-old man with a history of ischemic stroke at the age of 41 who reported cardiac hypertrophy, registered in his twenties when explained by increased physical activity. Family history was positive for a mother with permanent atrial fibrillation from her mid-thirties. At the age of 44, he had a first episode of persistent atrial fibrillation, accompanied by left atrial thrombosis while on a direct oral anticoagulant. He presented at our clinic at the age of 45 with another episode of persistent atrial fibrillation and decompensated heart failure. Echocardiography revealed a dilated left atrium, reduced left ventricular ejection fraction, and an asymmetric left ventricular hypertrophy. Cardiac magnetic resonance was positive for a cardiomyopathy with diffuse fibrosis, while slow-flow phenomenon was present on coronary angiography. Genetic testing by whole-exome sequencing revealed three variants in the patient, c.309C > A, p.His103Gln in the ACTC1 gene, c.116T > G, p.Leu39Ter in the PLN gene, and c.5827C > T, p.His1943Tyr in the SCN5A gene, the first two associated with hypertrophic cardiomyopathy and the latter possibly with familial atrial fibrillation. This case illustrates the need for advanced diagnostics in unexplained left ventricular hypertrophy, as hypertrophic cardiomyopathy is often overlooked, leading to potentially debilitating health consequences.
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Fibrilación Atrial , Cardiomiopatía Hipertrófica , Hipertrofia Ventricular Izquierda , Humanos , Fibrilación Atrial/genética , Fibrilación Atrial/diagnóstico , Masculino , Persona de Mediana Edad , Cardiomiopatía Hipertrófica/genética , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/complicaciones , Hipertrofia Ventricular Izquierda/genética , Hipertrofia Ventricular Izquierda/diagnóstico , Accidente Cerebrovascular/genética , Accidente Cerebrovascular/diagnóstico , Ecocardiografía , Canal de Sodio Activado por Voltaje NAV1.5/genéticaAsunto(s)
Cardiomiopatía Hipertrófica , Neoplasias Cardíacas , Humanos , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/complicaciones , Masculino , Ecocardiografía , Femenino , Persona de Mediana Edad , Diagnóstico Diferencial , Miocardiopatía Hipertrófica ApicalRESUMEN
BACKGROUND: The adverse prognostic impact of diabetes on hypertrophic cardiomyopathy (HCM) is poorly understood. We sought to explore the underlying mechanisms in terms of structural and functional remodelling in HCM patients with coexisting diabetes (HCM-DM). METHODS: A total of 45 HCM-DM patients were retrospectively included. Isolated HCM controls (HCM patients without diabetes) were matched to HCM-DM patients in terms of maximal wall thickness, age, and gender distribution. Left ventricular (LV) and atrial (LA) performance were evaluated using cardiac magnetic resonance feature tracking strain analyses. The associations between diabetes and LV/LA impairment were investigated by univariable and multivariable linear regression. RESULTS: Compared with the isolated HCM controls, the HCM-DM patients had smaller end-diastolic volume and stroke volume, lower ejection fraction, larger mass/volume ratio and impaired strains in all three directions (all P < 0.05). In terms of the LA parameters, HCM-DM patients presented impaired LA reservoir and conduit strain/strain rate (all P < 0.05). Among all HCM patients, comorbidity with diabetes was independently associated with a low LV ejection fraction (ß = - 6.05, P < 0.001) and impaired global longitudinal strain (ß = 1.40, P = 0.007). Moreover, compared with the isolated HCM controls, HCM-DM patients presented with more myocardial fibrosis according to late gadolinium enhancement, which was an independent predictor of impaired LV global radial strain (ß = - 45.81, P = 0.008), LV global circumferential strain (ß = 18.25, P = 0.003), LA reservoir strain (ß = - 59.20, P < 0.001) and strain rate (ß = - 2.90, P = 0.002). CONCLUSIONS: Diabetes has adverse effects on LV and LA function in HCM patients, which may be important contributors to severe manifestations and outcomes in those patients. The present study strengthened the evidence of the prevention and management of diabetes in HCM patients.
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Función del Atrio Izquierdo , Cardiomiopatía Hipertrófica , Diabetes Mellitus , Imagen por Resonancia Cinemagnética , Valor Predictivo de las Pruebas , Volumen Sistólico , Función Ventricular Izquierda , Remodelación Ventricular , Humanos , Masculino , Femenino , Persona de Mediana Edad , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Estudios Retrospectivos , Anciano , Diabetes Mellitus/epidemiología , Diabetes Mellitus/fisiopatología , Diabetes Mellitus/diagnóstico , Factores de Riesgo , Adulto , Pronóstico , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiología , Comorbilidad , Remodelación AtrialAsunto(s)
Cardiomiopatía Hipertrófica , Diástole , Sístole , Humanos , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Ecocardiografía/métodos , Volumen Sistólico/fisiología , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/complicaciones , Masculino , FemeninoRESUMEN
Despite substantial advances in the management of hypertrophic cardiomyopathy, advanced heart failure remains a major cause of morbidity in this patient population. This narrative review presents the case of a patient with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation to frame a discussion of modern therapies for hypertrophic cardiomyopathy. The current treatment landscape includes medications, both old and new, and surgical and procedural interventions to relieve mechanical obstruction. Several promising new modalities for relieving obstruction are in the nascent stages of development.
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Técnicas de Ablación , Cardiomiopatía Hipertrófica , Humanos , Cardiomiopatía Hipertrófica/terapia , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/cirugía , Técnicas de Ablación/métodos , Masculino , Resultado del Tratamiento , Etanol/uso terapéutico , Persona de Mediana EdadRESUMEN
OBJECTIVE: This study aimed to provide a long-term follow-up of PRKAG2 syndrome and describe the new phenotypic aspects of the condition. PRKAG2 syndrome is a rare autosomal-dominant glycogen storage disease characterized by cardiac hypertrophy, ventricular pre-excitation, and conduction system disease. Fatal arrhythmias occur frequently. METHODS: A family cohort of 66 participants was recruited. Clinical and genetic analyses were performed. RESULTS: Median age of 36.97±17.28 years, with 69.9% being men. Nineteen subjects carried the deleterious variant p.K290I of the PRKAG2 gene. This group experienced many malignant events, including eight pacemaker implants, three sudden cardiac deaths, five aborted cardiac arrests, four strokes, four premature neonatal deaths, two spontaneous abortions, five forceps deliveries, and 12 cesarean procedures. Extracardiac involvement, such as in neurocognitive and psychiatric disorders, has been observed only in carriers of mutations. Palpitations, Syncope, atrial fibrillation, atrial flutter, sinus pauses, and bradycardia were strongly and significantly associated with major or severe adverse events (sudden cardiac death, aborted cardiac arrest, pacemaker use, stroke, and congestive heart failure). Early diagnosis and intervention through antiarrhythmic drugs, anticoagulation, pacemaker implantation, radiofrequency catheter ablation, and cesarean section surgery improved the symptoms and survival rates. Mutations carriers were advised to avoid pregnancy. CONCLUSION: This study identified that the p.K291I_PRKAG2 mutation is associated with poor prognosis, highlighting the need for early intervention. Further research may uncover the potential connections between intellectual disability, miscarriage, and neonatal death in individuals with this syndrome.
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Cardiomiopatía Hipertrófica , Humanos , Femenino , Masculino , Adulto , Estudios de Seguimiento , Adulto Joven , Persona de Mediana Edad , Cardiomiopatía Hipertrófica/genética , Cardiomiopatía Hipertrófica/complicaciones , Brasil/epidemiología , Adolescente , Mutación , Proteínas Quinasas Activadas por AMP/genética , Fenotipo , Niño , Arritmias Cardíacas/genética , Arritmias Cardíacas/etiología , Síndrome , AncianoRESUMEN
Noonan Syndrome with Multiple Lentigines (NSML-formerly known as LEOPARD syndrome) is a rare autosomal dominant condition that usually exhibits cardiac involvement with hypertrophic cardiomyopathy (HCM). Here we present a case of a NSML patient with an unusual and patchy cardiac hypertrophy distribution.
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Aneurisma Cardíaco , Hipertrofia Ventricular Izquierda , Síndrome LEOPARD , Humanos , Síndrome LEOPARD/complicaciones , Síndrome LEOPARD/genética , Síndrome LEOPARD/diagnóstico por imagen , Aneurisma Cardíaco/diagnóstico por imagen , Aneurisma Cardíaco/complicaciones , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/fisiopatología , Masculino , Femenino , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Fenotipo , Adulto , Función Ventricular IzquierdaAsunto(s)
Fibrilación Atrial , Cardiomiopatía Hipertrófica , Medios de Contraste , Gadolinio , Humanos , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/complicaciones , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/complicaciones , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Imagen por Resonancia Magnética/métodos , Factores de Riesgo , Imagen por Resonancia Cinemagnética/métodos , Masculino , FemeninoRESUMEN
BACKGROUND: Right ventricular (RV) involvement has been reported in one out of three patients with hypertrophic cardiomyopathy (HCM), however its prognostic significance remains unknown. We aimed to assess the prognostic value of RV involvement in patients with HCM through a systematic review and meta-analysis. METHODS: A literature search was performed on PubMed, ClinicalTrials.gov and Cochrane Library databases from inception through November 15, 2023. Original articles enrolling HCM patients >18 years old and evaluating the association of RV parameters routinely assessed in clinical practice through trans-thoracic echocardiography or cardiac magnetic resonance (i.e., RV hypertrophy, volumes, systolic function, and late gadolinium enhancement) and the risk of a pre-defined composite endpoint including i) all cause death; ii) cardiac death; iii) heart transplantation; iv) heart failure-related hospitalization; v) atrial fibrillation; vi) ventricular tachycardia; and vii) stroke were retrieved. We pooled the effect of RV imaging variables on the combined clinical endpoint in terms of hazard ratio (HR) with 95% confidence interval (CI). RESULTS: The meta-analysis included 12 articles and 4634 patients. The pooled analysis demonstrated that the presence of RV systolic dysfunction conveyed an increased risk of adverse outcomes (HR 2.46; 95% CI 1.80-3.35; P < 0.001), whereas other RV imaging parameters were not significantly associated with patients' prognosis, except for RV-fractional area change analyzed as a continuous variable (HR 0.96 per % increase; 95% CI 0.93-0.995; P = 0.025). CONCLUSIONS: Our results pinpoint a prognostic role of RV dysfunction, independent of left ventricular involvement, in patients with HCM, and future longitudinal studies, including multi-parametric RV assessment, are encouraged to provide clinically-relevant data to refine risk stratification in patients with HCM.
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Cardiomiopatía Hipertrófica , Humanos , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/complicaciones , Pronóstico , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/etiología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatologíaRESUMEN
BACKGROUND: Left ventricular hypertrophy (LVH), including hypertensive LVH, hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA), is a commonly encountered condition in cardiology practice, presenting challenges in differential diagnosis. In this study, we aimed to investigate the importance of echocardiographic evaluation of the inferior vena cava (IVC) in distinguishing LVH subtypes including hypertensive LVH, HCM, and CA. METHODS: In this retrospective study, patients with common causes of LVH including hypertensive LVH, HCM, and CA were included. The role of echocardiographic evaluation of IVC diameter and collapsibility in distinguishing these causes of LVH was assessed in conjunction with other echocardiographic, clinical, and imaging methods. RESULTS: A total of 211 patients (45% HCM, 43% hypertensive heart disease, and 12% CA) were included in our study. Their mean age was 56.6 years and 62% of them were male. While mean IVC diameter was significantly dilated in CA patients (13.4 mm in hypertensive LVH, 16.0 mm in HCM, and 21.1 mm in CA, p < .001), its collapsibility was reduced (IVC collapsible in 95% of hypertensive patients, 72% of HCM patients, and 12% of CA patients, p < .001). In the analysis of diagnostic probabilities, the presence of both hypovoltage and IVC dilation is significant for CA patients. Although it is not statistically significant, the presence of IVC dilation along with atrial fibrillation supports the diagnosis of HCM. CONCLUSION: In conclusion, although advances in imaging techniques facilitate the diagnosis of LVH, simple echocardiographic methods should never be overlooked. Our study supports the notion that IVC assessment could play an important role in the differential diagnosis of LVH.
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Ecocardiografía , Hipertrofia Ventricular Izquierda , Vena Cava Inferior , Humanos , Masculino , Femenino , Vena Cava Inferior/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/fisiopatología , Persona de Mediana Edad , Diagnóstico Diferencial , Ecocardiografía/métodos , Estudios Retrospectivos , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Amiloidosis/diagnóstico por imagen , Amiloidosis/complicaciones , Anciano , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/fisiopatologíaRESUMEN
BACKGROUND: The prognosis of hypertrophic cardiomyopathy (HCM) varies from mild disease with a normal life expectancy to heart failure and sudden cardiac death (SCD). The identification of patients who are at high risk for SCD remains challenging. AIMS: In this study, we evaluated the prognostic value of papillary muscle-free strain in HCM patients. METHODS AND RESULTS: Seventy-nine patients with a diagnosis of HCM were included in this study. Patients were divided into low/intermediate-risk (n = 57) and high-risk (n = 22) groups. Two-dimensional (2-D) echocardiography and strain imaging were performed for each patient. The mean age of the study population was 53.85 ± 15.88 years; 47 (59.5%) of them were male. During a mean follow-up duration of 74.45 ± 17.03 months, 12 patients died. A comparison of the low-intermediate and high-SCD risk groups revealed that patients in the high-SCD risk group had greater maximal wall thickness, interventricular septum thickness, posterior wall thickness, and left ventricular mass index (LVMI) and lower (less negative) global longitudinal, anterolateral papillary muscle (ALPM) and posteromedial papillary muscle (PMPM) free strain. Additionally, a history of syncope and ICD implantation were found to be more common in patients with high SCD risk scores. The SCD risk score was positively correlated with the global longitudinal strain, ALPM-free strain, and PMPM-free strain (r = .528, r = .658, and r = .600, respectively; p < .001 for all). Our results showed that the LVMI, presence of syncope, global longitudinal strain, and ALPM-free strain were predictors of death. CONCLUSIONS: Decreased papillary muscle-free strain values might have prognostic value in patients with HCM.
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Cardiomiopatía Hipertrófica , Ecocardiografía , Músculos Papilares , Humanos , Masculino , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Femenino , Músculos Papilares/diagnóstico por imagen , Músculos Papilares/fisiopatología , Persona de Mediana Edad , Pronóstico , Ecocardiografía/métodos , Reproducibilidad de los Resultados , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatologíaRESUMEN
Objective: To investigate the incidence of coronary artery tortuosity and its correlation with poor prognosis in patients with septal hypertrophic cardiomyopathy (HCM). Methods: This was a retrospective cohort study. Patients with septal HCM who were hospitalized in Fuwai Central China Cardiovascular Hospital and Zhengzhou University People's Hospital between December 1, 2017 and June 10, 2021 were selected. Non-HCM patients were matched by gender, age, and hypertension as control group. Septal HCM was divided into two groups based on the presence or absence of coronary artery tortuosity. Clinical baseline data and coronary angiography findings were compared using a multifactorial logistic analysis of the risk factors for coronary artery tortuosity. Patients were followed up until July 1, 2022, with the primary outcome being the composite endpoint of malignant arrhythmia, ischemic stroke and all-cause death. Incidence densities were compared between the coronary artery tortuosity and non-coronary artery tortuosity groups of septal HCM patients. The Cox risk-ratio model was used to analyze risk factors for primary outcomes in septal HCM patients. Results: There were 156 patients in the septal HCM group and 156 patients in the control group, both aged (57.0±11.4) years, and 75 (48.1%) were female. The incidence of coronary artery tortuosity was significantly higher in the septal HCM group than in the control group (63.5% vs. 36.5%, P<0.01), and the coronary artery tortuosity score was also higher in the septal HCM group than in the control group (P<0.01). Multiple logistic regression analysis showed that septal HCM was a risk factor for coronary artery tortuosity (OR=3.27, 95%CI: 2.02-5.29, P<0.01). In the septal HCM patients, after (2.5±1.2) years of follow-up, the incidence density of primary outcome was significantly higher in the coronary artery tortuosity group than in the non-coronary artery tortuosity group (P=0.02), while each on-point in coronary artery tortuosity score increased the risk of primary outcome by 53% for septal HCM patients (HR=1.53, 95%CI: 1.26-1.86, P<0.01). Conclusions: Patients with septal HCM are more prone to suffer coronary artery tortuosity and suffer from it to a greater extent. Coronary artery tortuosity is an important risk factor for adverse events in patients with septal HCM.
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Cardiomiopatía Hipertrófica , Vasos Coronarios , Humanos , Cardiomiopatía Hipertrófica/complicaciones , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Vasos Coronarios/diagnóstico por imagen , Vasos Coronarios/patología , Factores de Riesgo , Masculino , Femenino , Angiografía Coronaria , Anomalías de los Vasos Coronarios/epidemiología , China/epidemiología , IncidenciaRESUMEN
BACKGROUND: Percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) has been reported to be safe and effective at midterm follow-up to treat drug-refractory hypertrophic obstructive cardiomyopathy in a single center. However, data from other centers are lacking. This retrospective cohort study aimed to investigate the efficacy and safety of PIMSRA from another independent center. METHODS AND RESULTS: PIMSRA was performed in 76 patients with hypertrophic obstructive cardiomyopathy in our center from April 2020 to June 2023. The primary outcome was the reduction of left ventricular outflow tract gradient after 6 months or more post-PIMSRA. Secondary outcomes were periprocedural major adverse clinical events. Sixty-one patients returned to the hospital for follow-up 6 to 30 (median, 14) months after the procedure. At the last follow-up of the 61 patients, the maximum septal thickness decreased from a median of 23.6 (interquartile range, 20.5-26.4) to 19.1 (interquartile range, 16.0-22.1) mm (P<0.001) and the left ventricular outflow tract peak gradient at rest decreased from a median of 70.0 (interquartile range, 29.1-107.5) to 20.0 (interquartile range, 10.8-48.8) mm Hg (P<0.001). The percentage of patients with symptoms of New York Heart Association functional class III/IV decreased from 51% to 0%. Of all 76 patients, there was no in-hospital or 30-day death, no right or left branch block, and no permanent pacemaker implantation. Six (8%) patients had pericardial effusion, with 1 experiencing cardiac tamponade and ventricular fibrillation, and 1 (1%) patient developed septal branch aneurysm that was treated with coil occlusion. CONCLUSIONS: PIMSRA allows for the reduction in the left ventricular outflow tract gradient and enhances symptomatic improvement, with a limited incidence of adverse events and complications among patients with hypertrophic obstructive cardiomyopathy.
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Cardiomiopatía Hipertrófica , Tabiques Cardíacos , Humanos , Cardiomiopatía Hipertrófica/cirugía , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Tabiques Cardíacos/cirugía , Tabiques Cardíacos/diagnóstico por imagen , Anciano , Ablación por Catéter/métodos , Ablación por Catéter/efectos adversos , Factores de Tiempo , Ablación por Radiofrecuencia/métodos , Ablación por Radiofrecuencia/efectos adversos , Adulto , Función Ventricular Izquierda , Estudios de Seguimiento , EcocardiografíaRESUMEN
BACKGROUND: The cumulative burden of hypertrophic cardiomyopathy (HCM) is significant, with a noteworthy percentage (10%-15%) of patients with HCM per year experiencing major adverse cardiovascular events (MACEs). A current risk stratification scheme for HCM had only limited accuracy in predicting sudden cardiac death (SCD) and failed to account for a broader spectrum of adverse cardiovascular events and cardiac magnetic resonance (CMR) parameters. OBJECTIVES: This study sought to develop and evaluate a machine learning (ML) framework that integrates CMR imaging and clinical characteristics to predict MACEs in patients with HCM. METHODS: A total of 758 patients with HCM (67% male; age 49 ± 14 years) who were admitted between 2010 and 2017 from 4 medical centers were included. The ML model was built on the internal discovery cohort (533 patients with HCM, admitted to Fuwai Hospital, Beijing, China) by using the light gradient-boosting machine and internally evaluated using cross-validation. The external test cohort consisted of 225 patients with HCM from 3 medical centers. A total of 14 CMR imaging features (strain and late gadolinium enhancement [LGE]) and 23 clinical variables were evaluated and used to inform the ML model. MACEs included a composite of arrhythmic events, SCD, heart failure, and atrial fibrillation-related stroke. RESULTS: MACEs occurred in 191 (25%) patients over a median follow-up period of 109.0 months (Q1-Q3: 73.0-118.8 months). Our ML model achieved areas under the curve (AUCs) of 0.830 and 0.812 (internally and externally, respectively). The model outperformed the classic HCM Risk-SCD model, with significant improvement (P < 0.001) of 22.7% in the AUC. Using the cubic spline analysis, the study showed that the extent of LGE and the impairment of global radial strain (GRS) and global circumferential strain (GCS) were nonlinearly correlated with MACEs: an elevated risk of adverse cardiovascular events was observed when these parameters reached the high enough second tertiles (11.6% for LGE, 25.8% for GRS, -17.3% for GCS). CONCLUSIONS: ML-empowered risk stratification using CMR and clinical features enabled accurate MACE prediction beyond the classic HCM Risk-SCD model. In addition, the nonlinear correlation between CMR features (LGE and left ventricular pressure gradient) and MACEs uncovered in this study provides valuable insights for the clinical assessment and management of HCM.
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Cardiomiopatía Hipertrófica , Aprendizaje Automático , Imagen por Resonancia Cinemagnética , Valor Predictivo de las Pruebas , Humanos , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/mortalidad , Cardiomiopatía Hipertrófica/complicaciones , Masculino , Persona de Mediana Edad , Femenino , Adulto , Medición de Riesgo , Pronóstico , Factores de Riesgo , Estudios Retrospectivos , China/epidemiología , Dinámicas no Lineales , Reproducibilidad de los Resultados , Muerte Súbita Cardíaca/etiología , Factores de Tiempo , Técnicas de Apoyo para la Decisión , AncianoRESUMEN
Rest imaging in hypertrophic cardiomyopathy may underestimate or miss left ventricular outflow tract obstruction, leading to suboptimal management decisions that negatively affect symptomatic patients. The 2024 hypertrophic cardiomyopathy guidelines describe exercise stress testing as an important tool to determine overall exercise tolerance and latent, exercise-provoked left ventricular outflow tract obstruction.
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Cardiomiopatía Hipertrófica , Prueba de Esfuerzo , Obstrucción del Flujo Ventricular Externo , Humanos , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico , Prueba de Esfuerzo/métodos , Obstrucción del Flujo Ventricular Externo/fisiopatología , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/etiología , Masculino , Persona de Mediana Edad , Obstrucción del Flujo de Salida Ventricular IzquierdaRESUMEN
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a common inherited genetic cardiac disease during pregnancy. Studies of risk factors are of great significance for maternal and fetal outcomes. AIM: The aim of the study was to identify predictive risk factors for cardiac complications in pregnant women with HCM. METHODS: One hundred patients with HCM who delivered at the Shanghai obstetrical cardiology intensive care center between January 2000 and December 2022 were retrospectively reviewed. A logistic regression model was used to identify independent risk factors for cardiac complications. RESULTS: Twenty-one cases were obstructive HCM (21%), 16 with cardiac function grade I and 5 with grade II; 79 cases were non-obstructive HCM (79%), 67 with cardiac function grade I, 11 with grade II, and 1 with grade III. Ninety-one cases had abnormal electrocardiogram (ECG) (91%), mainly with ST-T changes (77%). The average interventricular septum was 19.39 ± 6.13 mm by echocardiography (21.75 ± 5.86 mm for obstructive HCM and 18.73 ± 6.08 mm for non-obstructive HCM). The main cardiac complications were maternal death (n = 2, 2%), heart failure (n = 7, 7%), and sustained ventricular tachyarrhythmia (n = 1, 1%). Cardiac complications occur commonly during the third trimester and postpartum period. Three independent risk factors to predict cardiac complications in pregnant women with HCM were obstructive HCM (P = 0.036), New York Heart Association (NYHA) class ≥II (P = 0.022), and previous history of syncope (P = 0.037). CONCLUSIONS: HCM increases the risk of maternal death, heart failure, and malignant arrhythmia. More attention should be given to risk assessment and pregnancy management. Early detection of risk factors can reduce the incidence of maternal mortality and cardiac complications.
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Cardiomiopatía Hipertrófica , Complicaciones Cardiovasculares del Embarazo , Humanos , Femenino , Embarazo , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/epidemiología , Adulto , Complicaciones Cardiovasculares del Embarazo/epidemiología , Factores de Riesgo , Estudios Retrospectivos , Electrocardiografía , Ecocardiografía , China/epidemiología , Insuficiencia Cardíaca/epidemiologíaRESUMEN
Chest pain, a common initial symptom in hypertrophic cardiomyopathy (HCM) patients, is closely linked to myocardial ischemia, despite the absence of significant coronary artery stenosis. This study explored microvascular dysfunction in HCM patients by employing angiography-derived microcirculatory resistance (AMR) as a novel tool for comprehensive assessment. This retrospective analysis included HCM patients with chest pain as the primary symptom and control patients without cardiac hypertrophy during the same period. The AMR was computed through angiography, providing a wire-free and adenosine-free index for evaluating microcirculatory function. Propensity score matching ensured balanced demographics between groups. This study also investigated the correlation between the AMR and clinical outcomes by utilizing echocardiography and follow-up data. After matching, 76 HCM patients and 152 controls were analyzed. While there was no significant difference in the incidence of epicardial coronary stenosis, the AMR of three epicardial coronary arteries was markedly greater in HCM patients. The criterion of an AMR ≥ 250 mmHg*s/m was that 65.7% of HCM patients experienced coronary microvascular dysfunction (CMD). Independent risk factors for CMD included increased left ventricular (LV) wall thickness (OR = 1.209, 95% CI 1.013-1.443, p = 0.036). Furthermore, an AMR_LAD ≥ 250 mmHg*s/m had an increased cumulative risk of the endpoint (log-rank p = 0.023) and was an independent risk factor for the endpoint (HR = 11.64, 95% CI 1.13-120.03, p = 0.039), providing valuable prognostic insights.