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1.
Kidney Int ; 101(5): 1027-1038, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35124011

RESUMEN

Long-term adaptive immune memory has been reported among immunocompetent individuals up to eight months following SARS-CoV-2 infection. However, limited data is available in convalescent patients with a solid organ transplant. To investigate this, we performed a thorough evaluation of adaptive immune memory at different compartments (serological, memory B cells and cytokine [IFN-γ, IL-2, IFN-γ/IL12 and IL-21] producing T cells) specific to SARS-CoV-2 by ELISA and FluoroSpot-based assays in 102 convalescent patients (53 with a solid organ transplants (38 kidney, 5 liver, 5 lung and 5 heart transplant) and 49 immunocompetent controls) with different clinical COVID-19 severity (severe, mild and asymptomatic) beyond six months after infection. While similar detectable memory responses at different immune compartments were detected between those with a solid organ transplant and immunocompetent individuals, these responses were predominantly driven by distinct COVID-19 clinical severities (97.6%, 80.5% and 42.1%, all significantly different, were seropositive; 84% vs 75% vs 35.7%, all significantly different, showed IgG-producing memory B cells and 82.5%, 86.9% and 31.6%, displayed IFN-γ producing T cells; in severe, mild and asymptomatic convalescent patients, respectively). Notably, patients with a solid organ transplant with longer time after transplantation did more likely show detectable long-lasting immune memory, regardless of COVID-19 severity. Thus, our study shows that patients with a solid organ transplant are capable of maintaining long-lasting peripheral immune memory after COVID-19 infection; mainly determined by the degree of infection severity.


Asunto(s)
COVID-19 , Trasplante de Órganos , Anticuerpos Antivirales , Humanos , Memoria Inmunológica , Trasplante de Órganos/efectos adversos , SARS-CoV-2 , Receptores de Trasplantes
2.
Transpl Int ; 35: 10454, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35497887

RESUMEN

Objectives: To analyze the efficacy and tolerability of diflunisal for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. Methods: We performed a retrospective longitudinal study of prospectively collected data for all domino liver transplant recipients with acquired amyloid neuropathy who received diflunisal at our hospital. Neurological deterioration was defined as an score increase of ≥2 points from baseline on the Neurological Impairment Scale/Neurological Impairment Scale-Lower Limbs. Results: Twelve patients who had received compassionate use treatment with diflunisal were identified, of whom seven had follow-up data for ≥12 months. Five patients (71.4%) presented with neurological deterioration on the Neurological Impairment Scale after 12 months (p = 0.0382). The main adverse effects were cardiovascular and renal, leading to diflunisal being stopped in five patients and the dose being reduced in two patients. Conclusion: Our study suggests that most domino liver transplant recipients with acquired amyloid neuropathy will develop neurological deterioration by 12 months of treatment with diflunisal. This therapy was also associated with a high incidence of adverse effects and low treatment retention. The low efficacy and low tolerability of diflunisal treatment encourage the search for new therapeutic options.


Asunto(s)
Neuropatías Amiloides , Diflunisal , Diflunisal/uso terapéutico , Humanos , Estudios Longitudinales , Estudios Retrospectivos , Receptores de Trasplantes
3.
Eur J Orthop Surg Traumatol ; 32(3): 575-581, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34050818

RESUMEN

INTRODUCTION: Approximately 75% of patients with carpal tunnel syndrome (CTS) are diagnosed as idiopathic. Despite this, the presence of an underlying cause such as an anatomical variant or a systemic disease must always be suspected, especially in cases of bilateral presentation without an obvious cause, recurrence or complications. The anatomical variant known as the bifid median nerve (BMN) is a very rare abnormality that can occasionally lead to CTS. On the other hands, transthyretin-associated amyloidosis (ATTR) is one of the possible causes of bilateral CTS. We report a case where these two very rare pathologies converge as the cause of bilateral CTS and a review of the literature. CASE REPORT: We report a 71-year-old male with prior history of lumbar canal stenosis, bilateral trigger finger, rupture of the supraspinatus muscle tendon and of the long portion of the right biceps brachial. He also had 8-year-old bilateral CTS that recurred after CTS surgery. He was surgically re-intervened and was diagnosed incidentally with BMN and an ultrasound of the other hands also showed BMN. Because of all the prior musculoskeletal history, a biopsy of the transverse carpal ligament was taken showing ATTR deposits that led to the diagnosis of cardiac ATTR wild type. CONCLUSIONS: This case highlights the natural history of the multiple musculoskeletal manifestations related to ATTR and the importance of performing intraoperative biopsies in patients with CTS surgery as this can lead to early diagnosis of cardiac ATTR.


Asunto(s)
Neuropatías Amiloides Familiares , Síndrome del Túnel Carpiano , Cirujanos , Anciano , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/diagnóstico , Síndrome del Túnel Carpiano/diagnóstico , Síndrome del Túnel Carpiano/etiología , Síndrome del Túnel Carpiano/cirugía , Niño , Humanos , Masculino , Nervio Mediano , Prealbúmina
4.
Am J Transplant ; 21(1): 372-381, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32705768

RESUMEN

Domino liver transplantation (DLT) has been used widely in patients with hereditary amyloid transthyretin (ATTR) amyloidosis. New-onset polyneuropathy in recipients of DLT has been reported, but there are few cases of cardiac involvement reported. We aimed to perform a cross-sectional study for ATTR amyloidosis with cardiomyopathy (ATTR-CM) in DLT recipients. We evaluated 23 living DLT recipients a median of 9 years since DLT at 2 referral centers with a systematic cardiac evaluation, including bone scintigraphy. Median age was 72 years, 91% had hypertension, 35% had diabetes mellitus, 67% had chronic renal failure, and 8 patients (35%) developed new-onset polyneuropathy. Only 13% had a normal electrocardiogram and a normal echocardiography, and most of them showed some conduction disturbance or increase in left ventricular wall thickness, but only 1 patient with a Glu89Lys mutation developed ATTR-CM diagnosed by bone scintigraphy and endomyocardial biopsy. None of the recipients of a DLT with Val30Met mutation showed cardiac involvement by bone scintigraphy. In conclusion, DLT from Val30Met donors seems to be safe regarding the development of ATTR-CM. Evaluation of cardiomyopathy in DLT recipients is challenging due to concomitant comorbidities and in this context, bone scintigraphy can be helpful to evaluate ATTR-CM.


Asunto(s)
Neuropatías Amiloides Familiares , Cardiomiopatías , Trasplante de Hígado , Anciano , Neuropatías Amiloides Familiares/genética , Cardiomiopatías/etiología , Estudios Transversales , Humanos , Trasplante de Hígado/efectos adversos
5.
Am J Transplant ; 21(8): 2749-2761, 2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-33756051

RESUMEN

The description of protective humoral and T cell immune responses specific against SARS-CoV-2 has been reported among immunocompetent (IC) individuals developing COVID-19 infection. However, its characterization and determinants of poorer outcomes among the at-risk solid organ transplant (SOT) patient population have not been thoroughly investigated. Cytokine-producing T cell responses, such as IFN-γ, IL-2, IFN-γ/IL-2, IL-6, IL-21, and IL-5, against main immunogenic SARS-CoV-2 antigens and IgM/IgG serological immunity were tracked in SOT (n = 28) during acute infection and at two consecutive time points over the following 40 days of convalescence and were compared to matched IC (n = 16) patients admitted with similar moderate/severe COVID-19. We describe the development of a robust serological and functional T cell immune responses against SARS-CoV-2 among SOT patients, similar to IC patients during early convalescence. However, at the infection onset, SOT displayed lower IgG seroconversion rates (77% vs. 100%; p = .044), despite no differences on IgG titers, and a trend toward decreased SARS-CoV-2-reactive T cell frequencies, especially against the membrane protein (7 [0-34] vs. 113 [15-245], p = .011, 2 [0-9] vs. 45 [5-74], p = .009, and 0 [0-2] vs. 13 [1-24], p = .020, IFN-γ, IL-2, and IFN-γ/IL-2 spots, respectively). In summary, our data suggest that despite a certain initial delay, SOT population achieve comparable functional immune responses than the general population after moderate/severe COVID-19.


Asunto(s)
COVID-19 , Trasplante de Órganos , Anticuerpos Antivirales , Formación de Anticuerpos , Convalecencia , Humanos , SARS-CoV-2 , Linfocitos T
6.
Transpl Int ; 34(5): 882-893, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33482021

RESUMEN

Male patients are at increased risk for developing malignancy postheart transplantation (HT); however, real incidence and prognosis in both genders remain unknown. The aim of this study was to assess differences in incidence and mortality related to malignancy between genders in a large cohort of HT patients. Incidence and mortality rates were calculated for all tumors, skin cancers (SCs), lymphoma, and nonskin solid cancers (NSSCs) as well as survival since first diagnosis of neoplasia. 5865 patients (81.6% male) were included. Total incidence rates for all tumors, SCs, and NSSCs were lower in females [all tumors: 25.7 vs. 44.8 per 1000 person-years; rate ratio (RR) 0.68, (0.60-0.78), P < 0.001]. Mortality rates were also lower in females for all tumors [94.0 (77.3-114.3) vs. 129.6 (120.9-138.9) per 1000 person-years; RR 0.76, (0.62-0.94), P = 0.01] and for NSSCs [125.0 (95.2-164.0) vs 234.7 (214.0-257.5) per 1000 person-years; RR 0.60 (0.44-0.80), P = 0.001], albeit not for SCs or lymphoma. Female sex was associated with a better survival after diagnosis of malignancy [log-rank p test = 0.0037; HR 0.74 (0.60-0.91), P = 0.004]. In conclusion, incidence of malignancies post-HT is higher in males than in females, especially for SCs and NSSCs. Prognosis after cancer diagnosis is also worse in males.


Asunto(s)
Trasplante de Corazón , Neoplasias , Neoplasias Cutáneas , Estudios de Cohortes , Femenino , Trasplante de Corazón/efectos adversos , Humanos , Incidencia , Masculino , Neoplasias/epidemiología , Neoplasias/etiología , Pronóstico , Factores de Riesgo , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/etiología
7.
Eur Heart J ; 41(18): 1720-1729, 2020 05 07.
Artículo en Inglés | MEDLINE | ID: mdl-32016393

RESUMEN

AIM: Cardiotoxicity (CTox) is a major side effect of cancer therapies, but uniform diagnostic criteria to guide clinical and research practices are lacking. METHODS AND RESULTS: We prospectively studied 865 patients, aged 54.7 ± 13.9; 16.3% men, scheduled for anticancer therapy related with moderate/high CTox risk. Four groups of progressive myocardial damage/dysfunction were considered according to current guidelines: normal, normal biomarkers (high-sensitivity troponin T and N-terminal natriuretic pro-peptide), and left ventricular (LV) function; mild, abnormal biomarkers, and/or LV dysfunction (LVD) maintaining an LV ejection fraction (LVEF) ≥50%; moderate, LVD with LVEF 40-49%; and severe, LVD with LVEF ≤40% or symptomatic heart failure. Cardiotoxicity was defined as new or worsening of myocardial damage/ventricular function from baseline during follow-up. Patients were followed for a median of 24 months. Cardiotoxicity was identified in 37.5% patients during follow-up [95% confidence interval (CI) 34.22-40.8%], 31.6% with mild, 2.8% moderate, and 3.1% with severe myocardial damage/dysfunction. The mortality rate in the severe CTox group was 22.9 deaths per 100 patients-year vs. 2.3 deaths per 100 patients-year in the rest of groups, hazard ratio of 10.2 (95% CI 5.5-19.2) (P < 0.001). CONCLUSIONS: The majority of patients present objective data of myocardial injury/dysfunction during or after cancer therapy. Nevertheless, severe CTox, with a strong prognostic relationship, was comparatively rare. This should be reflected in protocols for clinical and research practices.


Asunto(s)
Disfunción Ventricular Izquierda , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Sistema de Registros , Volumen Sistólico , Disfunción Ventricular Izquierda/inducido químicamente , Disfunción Ventricular Izquierda/epidemiología , Función Ventricular Izquierda
8.
Clin Transplant ; 34(8): e13994, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32463124

RESUMEN

Vasoplegic syndrome (VS) is associated with poor outcomes after heart transplantation (HT). Our aim was to determine whether SAC/VALS is associated with VS after HT. We retrospectively analyzed all consecutive HT performed in three centers between January 2017 and August 2018. VS was defined as vasopressor need (norepinephrine or epinephrine >.5 mcg/kg/min or vasopressin) for more than 24 hours to maintain a mean arterial pressure >70 mm Hg. Ninety-six recipients underwent HT in the study period: 60 elective HT with no LVAD, 5 elective HT on long term LVAD, and 31 emergent HT: 3 on long-term LVAD and 28 on temporary mechanical circulatory support. Fourteen patients were on SAC/VALS treatment at the time of transplant, and 82 were not. The global incidence of VS was 15.6%, with no significant differences between the groups (7.14% in with SAC/VALS vs 17.07% in no-SAC/VALS). In conclusion, in our small cohort SAC/VALS was not associated with VS development.


Asunto(s)
Trasplante de Corazón , Corazón Auxiliar , Vasoplejía , Aminobutiratos , Compuestos de Bifenilo , Combinación de Medicamentos , Trasplante de Corazón/efectos adversos , Humanos , Incidencia , Estudios Retrospectivos , Valsartán , Vasoplejía/tratamiento farmacológico , Vasoplejía/epidemiología , Vasoplejía/etiología
9.
Clin Transplant ; 34(12): e14096, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32978995

RESUMEN

The study of gender differences may lead into improvement in patient care. We have aimed to identify the gender differences in heart transplantation (HT) of adult HT recipients in Spain and their evolution in a study covering the years 1993-2017 in which 6740 HT (20.6% in women) were performed. HT indication rate per million inhabitants was lower in women, remaining basically unchanged during the 25-year study period. HT rate was higher in men, although this decreased over the 25-year study period. Type of heart disease differed in men versus women (p < .001): ischemic heart disease 47.6% versus 22.5%, dilated cardiomyopathy 41.3% versus 34.6%, or other 36% versus 17.8%, respectively. Men were more frequently diabetics (18% vs. 13.1% p < .001), hypertensives (33.1% vs. 24% p < .001), and smokers (21.7% vs. 12.9% p < .001), respectively. Women had more pre-HT malignancies (7.1% vs. 2.8% p < .001), and their clinical status was worse at HT due to renal function and mechanical ventilation. Adjusted survival (p = .198) and most of the mortality-related variables were similar in men and women. Death occurred more frequently in women due to rejection (7.9% vs. 5.1% p < .001) and primary failure (18.2% vs. 12.5% p < .001) and in men due to malignancies (15.1% vs. 6.6% p < .001).


Asunto(s)
Trasplante de Corazón , Caracteres Sexuales , Adulto , Femenino , Humanos , Masculino , Sistema de Registros , España/epidemiología , Tasa de Supervivencia , Factores de Tiempo
10.
Curr Opin Cardiol ; 33(3): 334-340, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29543671

RESUMEN

PURPOSE OF REVIEW: Iron overload cardiomyopathy (IOC) is an important predictor of prognosis in a significant number of patients with hereditary hemochromatosis and hematologic diseases. Its prevalence is increasing because of improved treatment strategies, which significantly improve life expectancy. We will review diagnosis, treatment, and recent findings in the field. RECENT FINDINGS: The development of preclinical translational disease models during the last years have helped our understanding of specific disease pathophysiological pathways that might eventually change the outcomes of these patients. SUMMARY: IOC is an overlooked disease because of the progressive silent disease pattern and the lack of physicians' expertise. It mainly affects patients with hemochromatosis and hematologic diseases and its prevalence is expected to increase with the improvement in life expectancy of hematologic disorders. Early diagnosis of IOC in patients at risk by means of biochemical parameters and cardiac imaging can lead to early treatment and improved prognosis. The mainstay of treatment of IOC is conventional heart failure treatment, combined with phlebotomies or iron chelation in the context of anemia. The development of preclinical models has provided a comprehensive look into specific pathophysiological pathways with potential treatment strategies that must be sustained by future randomized trials.


Asunto(s)
Cardiomiopatías , Manejo de la Enfermedad , Quelantes del Hierro/uso terapéutico , Sobrecarga de Hierro , Hierro/sangre , Biomarcadores/sangre , Cardiomiopatías/diagnóstico , Cardiomiopatías/tratamiento farmacológico , Cardiomiopatías/etiología , Humanos , Sobrecarga de Hierro/sangre , Sobrecarga de Hierro/complicaciones , Sobrecarga de Hierro/tratamiento farmacológico
11.
BMC Cardiovasc Disord ; 18(1): 206, 2018 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-30382817

RESUMEN

BACKGROUND: Iron deficiency (ID) in patients with chronic heart failure (CHF) is considered an adverse prognostic factor. We aimed to evaluate if ID in patients with CHF is associated with increased mortality and hospitalizations. METHODS: We evaluated ID in patients with CHF at 3 university hospitals. ID was defined as absolute (ferritin < 100 µg/L) or functional (transferrin Saturation index < 20% and ferritin between 100 and 299 µg/L). We excluded patients who received treatment with intravenous Iron or Erythropoietin during follow-up. We evaluated if ID was a predictor of death or hospitalization due to heart failure or any cause using univariate and multivariate cox regression analysis. RESULTS: We included 1684 patients, 65% males, 38% diabetics, median age of 72 years, 37% in functional class III-IV and 30% of patients with a left ventricular ejection fraction > 45%. Patients were well treated, with 87% and 88% of patients receiving renin-angiotensin inhibitors and beta-blockers, respectively. Median transferrin saturation index was 20%, median ferritin 155 ng/mL and median haemoglobin 13 g/dL. ID was present in 53% of patients; in 35% it was absolute and in 18% functional. Median follow-up was 20 months. ID was a predictor of death, hospitalization due to heart failure or to any cause in univariate analysis but not after multivariate analysis. No differences were found between absolute or functional ID regarding prognosis. CONCLUSION: In a real life population of patients with CHF and a high prevalence of heart failure with preserved ejection fraction, ID did not predict mortality or hospitalizations after adjustment for comorbidities, functional class and neurohormonal treatment.


Asunto(s)
Anemia Ferropénica/mortalidad , Insuficiencia Cardíaca/mortalidad , Admisión del Paciente , Anciano , Anemia Ferropénica/sangre , Anemia Ferropénica/diagnóstico , Anemia Ferropénica/terapia , Biomarcadores/sangre , Causas de Muerte , Enfermedad Crónica , Comorbilidad , Femenino , Ferritinas/sangre , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/fisiopatología , Hospitales Universitarios , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Prevalencia , Pronóstico , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , España/epidemiología , Volumen Sistólico , Factores de Tiempo , Función Ventricular Izquierda
12.
Hum Mol Genet ; 24(13): 3638-50, 2015 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-25801283

RESUMEN

Protein aggregate myopathies (PAMs) define muscle disorders characterized by protein accumulation in muscle fibres. We describe a new PAM in a patient with proximal muscle weakness and hypertrophic cardiomyopathy, whose muscle fibres contained inclusions containing myosin and myosin-associated proteins, and aberrant distribution of microtubules. These lesions appear as intact A- and M-bands lacking thin filaments and Z-discs. These features differ from inclusions in myosin storage myopathy (MSM), but are highly similar to those in mice deficient for the muscle-specific RING finger proteins MuRF1 and MuRF3. Sanger sequencing excluded mutations in the MSM-associated gene MYH7 but identified mutations in TRIM63 and TRIM54, encoding MuRF1 and MuRF3, respectively. No mutations in other potentially disease-causing genes were identified by Sanger and whole exome sequencing. Analysis of seven family members revealed that both mutations segregated in the family but only the homozygous TRIM63 null mutation in combination with the heterozygous TRIM54 mutation found in the proband caused the disease phenotype. Both MuRFs are microtubule-associated proteins localizing to sarcomeric M-bands and Z-discs. They are E3 ubiquitin ligases that play a role in degradation of sarcomeric proteins, stabilization of microtubules and myogenesis. Lack of ubiquitin and the 20S proteasome subunit in the inclusions found in the patient suggested impaired turnover of thick filament proteins. Disruption of microtubules in cultured myotubes was rescued by transient expression of wild-type MuRF1. The unique features of this novel myopathy point to defects in homeostasis of A-band proteins in combination with instability of microtubules as cause of the disease.


Asunto(s)
Proteínas Musculares/genética , Debilidad Muscular/genética , Mutación , Agregación Patológica de Proteínas/genética , Ubiquitina-Proteína Ligasas/genética , Adulto , Anciano , Animales , Femenino , Humanos , Masculino , Ratones , Ratones Noqueados , Persona de Mediana Edad , Células Musculares/metabolismo , Proteínas Musculares/metabolismo , Debilidad Muscular/metabolismo , Músculo Esquelético/metabolismo , Linaje , Agregado de Proteínas , Agregación Patológica de Proteínas/metabolismo , España , Proteínas de Motivos Tripartitos , Ubiquitina-Proteína Ligasas/metabolismo
13.
Europace ; 19(2): 267-274, 2017 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-28175266

RESUMEN

Aims: The aim of this article is to evaluate the impact of a coronary chronic total occlusion in an infarct-related artery (IRA-CTO) on the occurrence of ventricular arrhythmias (VAs) in patients implanted with an implantable cardioverter defibrillator (ICD) for primary prevention. Methods and Results: The study includes a prospective cohort of 108 consecutive patients with ischaemic cardiomyopathy, in whom an ICD was implanted for primary prevention and a coronary angiography performed before ICD implantation. About 49 patients (45%) had a CTO and 34 (31%) had an IRA-CTO. Patients with IRA-CTO did not differ from the rest of the population in terms of basal characteristics and severity of cardiac disease. Median follow-up was 33 months (interquartile range 46). Infarct-related artery-CTO was associated with higher rates of any VA (53 vs. 26%, P = 0.006) and fast ventricular tachycardia (fast VT, cycle length <300 ms) or ventricular fibrillation (VF) (47 vs. 19%, P = 0.002). At multivariate Cox regression, IRA-CTO was the only independent predictor of any VA [hazard ratio (HR) 3.64, P = 0.002] and fast VT/VF (HR 3.36, P = 0.008). On the contrary, CTO not associated with a prior infarction in their territory did not increase the risk of VA. Infract-related artery-CTO was also an independent predictor of cardiac mortality or heart transplantation (HR 3.46, P = 0.022). Conclusion: In ischaemic patients implanted with an ICD for primary prevention, a CTO associated with a previous infarction in its territory is an independent predictor of VA and, especially, of fast VT/VF, identifying a subgroup of patients with a very high rate of arrhythmic events at follow-up.


Asunto(s)
Cardiomiopatías/terapia , Oclusión Coronaria/epidemiología , Desfibriladores Implantables , Infarto del Miocardio/terapia , Taquicardia Ventricular/epidemiología , Fibrilación Ventricular/epidemiología , Anciano , Cardiomiopatías/etiología , Enfermedad Crónica , Estudios de Cohortes , Angiografía Coronaria , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Infarto del Miocardio/complicaciones , Isquemia Miocárdica , Prevención Primaria , Modelos de Riesgos Proporcionales , Estudios Prospectivos , Taquicardia Ventricular/prevención & control , Fibrilación Ventricular/prevención & control
15.
J Heart Lung Transplant ; 43(7): 1059-1073, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38518863

RESUMEN

Hemodynamic derangements are defining features of cardiogenic shock. Randomized clinical trials have examined the efficacy of various therapeutic interventions, from percutaneous coronary intervention to inotropes and mechanical circulatory support (MCS). However, hemodynamic management in cardiogenic shock has not been well-studied. This State-of-the-Art review will provide a framework for hemodynamic management in cardiogenic shock, including a description of the 4 therapeutic phases from initial 'Rescue' to 'Optimization', 'Stabilization' and 'de-Escalation or Exit therapy' (R-O-S-E), phenotyping and phenotype-guided tailoring of pharmacological and MCS support, to achieve hemodynamic and therapeutic goals. Finally, the premises that form the basis for clinical management and the hypotheses for randomized controlled trials will be discussed, with a view to the future direction of cardiogenic shock.


Asunto(s)
Hemodinámica , Unidades de Cuidados Intensivos , Choque Cardiogénico , Choque Cardiogénico/terapia , Choque Cardiogénico/fisiopatología , Humanos , Hemodinámica/fisiología , Corazón Auxiliar
16.
Neurol Ther ; 2024 May 10.
Artículo en Inglés | MEDLINE | ID: mdl-38727765

RESUMEN

INTRODUCTION: Acquired amyloid neuropathy is an iatrogenic disease that appears years after a domino liver transplant. The objectives of our study are to analyze the efficacy and tolerability of tafamidis for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. This post-authorization, prospective, longitudinal study included seven domino liver transplant recipients with acquired amyloid neuropathy who received treatment with tafamidis for 18 months. METHODS: The primary endpoints were the response rate, defined as those patients with an increase of < 2 points on the Neurological Impairment Score (NIS) from baseline, and the change in the NIS score from baseline. Secondary endpoints included the Quantitative Sensory Test, 10-m walk test, quality of life (Norfolk), and disability (Rasch-built Overall Disability Scale). As safety parameters, the evidence of graft rejection, changes in immunosuppressive trough levels and changes in antiviral and allogeneic cellular immunity before and 12 months after tafamidis treatment were also assessed. RESULTS: Six patients (85.7%) had responded at 18-months. Compared to baseline, we observed non-statistically significant improvement in mean NIS score at 6 months (- 2.54 points, CI - 5.92 to 0.84), 12 months (- 3.25 points; CI - 6.63 to 0.13), and 18 months (- 2.35 points; CI - 5.74 to 1.02). Changes in the Quantitative Sensory Test, 10-m walk tests and the quality of life and disability questionnaires were not statistically significant. The use of tafamidis did not induce relevant side effects or drug interactions. Also, no acute rejections events nor changes in functional adaptive immunity were observed. CONCLUSION: Our study supports the safety and tolerability of tafamidis for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. Tafamidis shows promise as a useful treatment in the clinical management of these patients. Future randomized placebo-controlled clinical trials with longer follow-up durations are needed.

17.
Rev Esp Cardiol (Engl Ed) ; 77(1): 39-49, 2024 Jan.
Artículo en Inglés, Español | MEDLINE | ID: mdl-37217134

RESUMEN

INTRODUCTION AND OBJECTIVES: We aimed to describe the clinical outcomes of the use of the CentriMag acute circulatory support system as a bridge to emergency heart transplantation (HTx). METHODS: We conducted a descriptive analysis of the clinical outcomes of consecutive HTx candidates included in a multicenter retrospective registry who were treated with the CentriMag device, configured either for left ventricular support (LVS) or biventricular support (BVS). All patients were listed for high-priority HTx. The study assessed the period 2010 to 2020 and involved 16 transplant centers around Spain. We excluded patients treated with isolated right ventricular support or venoarterial extracorporeal membrane oxygenation without LVS. The primary endpoint was 1-year post-HTx survival. RESULTS: The study population comprised 213 emergency HTx candidates bridged on CentriMag LVS and 145 on CentriMag BVS. Overall, 303 (84.6%) patients received a transplant and 53 (14.8%) died without having an organ donor during the index hospitalization. Median time on the device was 15 days, with 66 (18.6%) patients being supported for> 30 days. One-year posttransplant survival was 77.6%. Univariable and multivariable analyses showed no statistically significant differences in pre- or post-HTx survival in patients managed with BVS vs LVS. Patients managed with BVS had higher rates of bleeding, need for transfusion, hemolysis and renal failure than patients managed with LVS, while the latter group showed a higher incidence of ischemic stroke. CONCLUSIONS: In a setting of candidate prioritization with short waiting list times, bridging to HTx with the CentriMag system was feasible and resulted in acceptable on-support and posttransplant outcomes.


Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Humanos , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/cirugía , Estudios Retrospectivos , Corazón Auxiliar/efectos adversos , Trasplante de Corazón/efectos adversos , Factores de Tiempo , Resultado del Tratamiento
18.
Rev Esp Cardiol (Engl Ed) ; 77(4): 290-301, 2024 Apr.
Artículo en Inglés, Español | MEDLINE | ID: mdl-37516313

RESUMEN

INTRODUCTION AND OBJECTIVES: Repetitive ambulatory doses of levosimendan are an option as a bridge to heart transplantation (HT), but evidence regarding the safety and efficacy of this treatment is scarce. The objective of the LEVO-T Registry is to describe the profile of patients on the HT list receiving levosimendan, prescription patterns, and clinical outcomes compared with patients not on levosimendan. METHODS: We retrospectively reviewed all patients listed for elective HT from 2015 to 2020 from 14 centers in Spain. RESULTS: A total of 1015 consecutive patients were included, of whom 238 patients (23.4%) received levosimendan. Patients treated with levosimendan had more heart failure (HF) admissions in the previous year and a worse clinical profile. The most frequent prescription pattern were fixed doses triggered by the patients' clinical needs. Nonfatal ventricular arrhythmias occurred in 2 patients (0.8%). No differences in HF hospitalizations were found between patients who started levosimendan in the first 30 days after listing and those who did not (33.6% vs 34.5%; P=.848). Among those who did not, 102 patients (32.9%) crossed over to levosimendan after an HF admission. These patients had a rate of 0.57 HF admissions per month before starting levosimendan and 0.21 afterwards. Propensity score matching analysis showed no differences in survival at 1 year after listing between patients receiving levosimendan and those who did not (HR, 1.03; 95%CI, 0.36-2.97; P=.958) or in survival after HT (HR, 0.97; 95%CI, 0.60-1.56; P=.958). CONCLUSIONS: Repetitive levosimendan in an ambulatory setting as a bridge to heart transplantation is commonly used, is safe, and may reduce HF hospitalizations.


Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Piridazinas , Humanos , Simendán/uso terapéutico , Cardiotónicos/uso terapéutico , Estudios Retrospectivos , Resultado del Tratamiento , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/cirugía , Hidrazonas/uso terapéutico , Piridazinas/uso terapéutico
19.
Rev Esp Cardiol (Engl Ed) ; 77(1): 69-78, 2024 Jan.
Artículo en Inglés, Español | MEDLINE | ID: mdl-37926340

RESUMEN

Heart transplant (HT) remains the best therapeutic option for patients with advanced heart failure (HF). The allocation criteria aim to guarantee equitable access to HT and prioritize patients with a worse clinical status. To review the HT allocation criteria, the Heart Failure Association of the Spanish Society of Cardiology (HFA-SEC), the Spanish Society of Cardiovascular and Endovascular Surgery (SECCE) and the National Transplant Organization (ONT), organized a consensus conference involving adult and pediatric cardiologists, adult and pediatric cardiac surgeons, transplant coordinators from all over Spain, and physicians and nurses from the ONT. The aims of the consensus conference were as follows: a) to analyze the organization and management of patients with advanced HF and cardiogenic shock in Spain; b) to critically review heart allocation and priority criteria in other transplant organizations; c) to analyze the outcomes of patients listed and transplanted before and after the modification of the heart allocation criteria in 2017; and d) to propose new heart allocation criteria in Spain after an analysis of the available evidence and multidisciplinary discussion. In this article, by the HFA-SEC, SECCE and the ONT we present the results of the analysis performed in the consensus conference and the rationale for the new heart allocation criteria in Spain.


Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Adulto , Humanos , Niño , España/epidemiología , Insuficiencia Cardíaca/cirugía , Consenso , Choque Cardiogénico
20.
ESC Heart Fail ; 10(5): 2875-2881, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37991427

RESUMEN

AIMS: The prevalence of advanced heart failure (HF) is increasing due to the growing number of patients with HF and their better treatment and survival. There is a scarcity of data on the accuracy of HF web-based risk scores in this selected population. This study aimed to assess mortality prediction performance of the Meta-Analysis Global Group in Chronic HF (MAGGIC-HF) risk score and the model of the Barcelona Bio-HF Risk Calculator (BCN-Bio-HF) containing N terminal pro brain natriuretic peptide in HF patients receiving intermittent inotropic support with levosimendan as destination therapy. METHODS AND RESULTS: Four hundred and three advanced HF patients from 23 tertiary hospitals in Spain receiving intermittent inotropic support with levosimendan as destination therapy were included. Discrimination for all-cause mortality was compared by area under the curve (AUC) and Harrell's C-statistic at 1 year. Calibration was assessed by calibration plots comparing observed versus expected events based on estimated risk by each calculator. The included patients were predominantly men, aged 71.5 [interquartile range 64-78] years, with reduced left ventricular ejection fraction (27.5 ± 9.4%); ischaemic heart disease was the most prevalent aetiology (52.5%). Death rate at 1 year was 26.8%, while the predicted 1-year mortality by BCN-Bio-HF and MAGGIC-HF was 17.0% and 22.1%, respectively. BCN-Bio-HF AUC was 0.66 (Harrell's C-statistic 0.64), and MAGGIC-HF AUC was 0.62 (Harrell's C-statistic 0.61). CONCLUSIONS: The two evaluated risk scores showed suboptimal discrimination and calibration with an underestimation of risk in advanced HF patients receiving levosimendan as destination therapy. There is a need for specific scores for advanced HF.


Asunto(s)
Insuficiencia Cardíaca , Función Ventricular Izquierda , Femenino , Humanos , Masculino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/epidemiología , Sistema de Registros , Factores de Riesgo , Simendán , Volumen Sistólico , Persona de Mediana Edad , Anciano
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