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1.
Eur Heart J ; 45(30): 2697-2726, 2024 Aug 09.
Artículo en Inglés | MEDLINE | ID: mdl-38923509

RESUMEN

Cardiac sarcoidosis (CS) is a form of inflammatory cardiomyopathy associated with significant clinical complications such as high-degree atrioventricular block, ventricular tachycardia, and heart failure as well as sudden cardiac death. It is therefore important to provide an expert consensus statement summarizing the role of different available diagnostic tools and emphasizing the importance of a multidisciplinary approach. By integrating clinical information and the results of diagnostic tests, an accurate, validated, and timely diagnosis can be made, while alternative diagnoses can be reasonably excluded. This clinical expert consensus statement reviews the evidence on the management of different CS manifestations and provides advice to practicing clinicians in the field on the role of immunosuppression and the treatment of cardiac complications based on limited published data and the experience of international CS experts. The monitoring and risk stratification of patients with CS is also covered, while controversies and future research needs are explored.


Asunto(s)
Cardiomiopatías , Sarcoidosis , Humanos , Sarcoidosis/diagnóstico , Sarcoidosis/terapia , Sarcoidosis/complicaciones , Cardiomiopatías/diagnóstico , Cardiomiopatías/terapia , Inmunosupresores/uso terapéutico , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología
2.
N Engl J Med ; 384(1): 31-41, 2021 01 07.
Artículo en Inglés | MEDLINE | ID: mdl-33200890

RESUMEN

BACKGROUND: Interleukin-1 has been implicated as a mediator of recurrent pericarditis. The efficacy and safety of rilonacept, an interleukin-1α and interleukin-1ß cytokine trap, were studied previously in a phase 2 trial involving patients with recurrent pericarditis. METHODS: We conducted a phase 3 multicenter, double-blind, event-driven, randomized-withdrawal trial of rilonacept in patients with acute symptoms of recurrent pericarditis (as assessed on a patient-reported scale) and systemic inflammation (as shown by an elevated C-reactive protein [CRP] level). Patients presenting with pericarditis recurrence while receiving standard therapy were enrolled in a 12-week run-in period, during which rilonacept was initiated and background medications were discontinued. Patients who had a clinical response (i.e., met prespecified response criteria) were randomly assigned in a 1:1 ratio to receive continued rilonacept monotherapy or placebo, administered subcutaneously once weekly. The primary efficacy end point, assessed with a Cox proportional-hazards model, was the time to the first pericarditis recurrence. Safety was also assessed. RESULTS: A total of 86 patients with pericarditis pain and an elevated CRP level were enrolled in the run-in period. During the run-in period, the median time to resolution or near-resolution of pain was 5 days, and the median time to normalization of the CRP level was 7 days. A total of 61 patients underwent randomization. During the randomized-withdrawal period, there were too few recurrence events in the rilonacept group to allow for the median time to the first adjudicated recurrence to be calculated; the median time to the first adjudicated recurrence in the placebo group was 8.6 weeks (95% confidence interval [CI], 4.0 to 11.7; hazard ratio in a Cox proportional-hazards model, 0.04; 95% CI, 0.01 to 0.18; P<0.001 by the log-rank test). During this period, 2 of 30 patients (7%) in the rilonacept group had a pericarditis recurrence, as compared with 23 of 31 patients (74%) in the placebo group. In the run-in period, 4 patients had adverse events leading to the discontinuation of rilonacept therapy. The most common adverse events with rilonacept were injection-site reactions and upper respiratory tract infections. CONCLUSIONS: Among patients with recurrent pericarditis, rilonacept led to rapid resolution of recurrent pericarditis episodes and to a significantly lower risk of pericarditis recurrence than placebo. (Funded by Kiniksa Pharmaceuticals; RHAPSODY ClinicalTrials.gov number, NCT03737110.).


Asunto(s)
Pericarditis/tratamiento farmacológico , Receptores Tipo I de Interleucina-1/antagonistas & inhibidores , Proteínas Recombinantes de Fusión/uso terapéutico , Adolescente , Adulto , Anciano , Método Doble Ciego , Femenino , Humanos , Inyecciones Subcutáneas/efectos adversos , Interleucina-1alfa , Interleucina-1beta , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Proteínas Recombinantes de Fusión/efectos adversos , Recurrencia , Infecciones del Sistema Respiratorio/etiología , Adulto Joven
3.
Clin Genet ; 106(4): 394-402, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-38837338

RESUMEN

In the last decade, an incredible improvement has been made in elucidating the genetic bases of cardiomyopathies. Here we report the impact of either the European Society of Cardiology (ESC) guidelines or the use of whole exome sequencing (WES) in terms of a number of variants of uncertain significance (VUS) and missed diagnoses in a series of 260 patients affected by inherited cardiac disorders. Samples were analyzed using a targeted gene panel of 128 cardiac-related genes and/or WES in a subset of patients, with a three-tier approach. Analyzing (i) only a subset of genes related to the clinical presentation, strictly following the ESC guidelines, 20.77% positive test were assessed. The incremental diagnostic rate for (ii) the whole gene panel, and (iii) the WES was 4.71% and 11.67%, respectively. The diverse analytical approaches increased the number of VUSs and incidental findings. Indeed, the use of WES highlights that there is a small percentage of syndromic conditions that standard analysis would not have detected. Moreover, the use of targeted sequencing coupled with "narrow" analytical approach prevents the detection of variants in actionable genes that could allow for preventive treatment. Our data suggest that genetic testing might aid clinicians in the diagnosis of inheritable cardiac disorders.


Asunto(s)
Secuenciación del Exoma , Pruebas Genéticas , Humanos , Pruebas Genéticas/métodos , Pruebas Genéticas/normas , Femenino , Masculino , Adulto , Cardiopatías/genética , Cardiopatías/diagnóstico , Persona de Mediana Edad , Cardiología/normas , Cardiología/métodos , Europa (Continente) , Predisposición Genética a la Enfermedad , Adolescente , Anciano , Adulto Joven , Niño , Guías de Práctica Clínica como Asunto , Exoma/genética , Preescolar , Cardiomiopatías/genética , Cardiomiopatías/diagnóstico
4.
JAMA ; 332(13): 1090-1100, 2024 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-39235771

RESUMEN

Importance: Pericarditis accounts for up to 5% of emergency department visits for nonischemic chest pain in North America and Western Europe. With appropriate treatment, 70% to 85% of these patients have a benign course. In acute pericarditis, the development of constrictive pericarditis (<0.5%) and pericardial tamponade (<3%) can be life-threatening. Observations: Acute pericarditis is diagnosed with presence of 2 or more of the following: sharp, pleuritic chest pain that worsens when supine (≈90%); new widespread electrocardiographic ST-segment elevation and PR depression (≈25%-50%); a new or increased pericardial effusion that is most often small (≈60%); or a pericardial friction rub (<30%). In North America and Western Europe, the most common causes of acute pericarditis are idiopathic or viral, followed by pericarditis after cardiac procedures or operations. Tuberculosis is the most common cause in endemic areas and is treated with antituberculosis therapy, with corticosteroids considered for associated constrictive pericarditis. Treatment of acute idiopathic and pericarditis after cardiac procedures or operations involves use of high-dose nonsteroidal anti-inflammatory drugs (NSAIDs), with doses tapered once chest pain has resolved and C-reactive protein level has normalized, typically over several weeks. These patients should receive a 3-month course of colchicine to relieve symptoms and reduce the risk of recurrence (37.5% vs 16.7%; absolute risk reduction, 20.8%). With a first recurrence of pericarditis, colchicine should be continued for at least 6 months. Corticosteroids are often used if pericarditis does not improve with NSAIDs and colchicine. In certain patients with multiple recurrences, which can occur for several years, interleukin 1 (IL-1) blockers have demonstrated efficacy and may be preferred to corticosteroids. Conclusions: Acute pericarditis is a common cause of nonischemic chest pain. Tuberculosis is the leading cause of pericarditis in endemic areas and is treated with antitubercular therapy. In North America and Western Europe, pericarditis is typically idiopathic, develops after a viral infection, or develops following cardiac procedures or surgery. Treatment with NSAIDs and colchicine leads to a favorable prognosis in most patients, although 15% to 30% of patients develop recurrence. Patients with multiple recurrent pericarditis can have a disease duration of several years or more, are often treated with corticosteroids, and IL-1 blockers may be used for selected patients as steroid-sparing therapy.


Asunto(s)
Dolor en el Pecho , Colchicina , Pericarditis , Humanos , Enfermedad Aguda/epidemiología , Enfermedad Aguda/terapia , Antiinflamatorios no Esteroideos/uso terapéutico , Antituberculosos/uso terapéutico , Dolor en el Pecho/diagnóstico , Dolor en el Pecho/tratamiento farmacológico , Dolor en el Pecho/epidemiología , Dolor en el Pecho/etiología , Colchicina/uso terapéutico , Electrocardiografía , Pericarditis/diagnóstico , Pericarditis/tratamiento farmacológico , Pericarditis/epidemiología , Pericarditis/etiología , Medición de Riesgo , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/tratamiento farmacológico , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Tuberculosis/complicaciones , Tuberculosis/tratamiento farmacológico , Quimioterapia Combinada/métodos , Glucocorticoides/uso terapéutico
5.
J Pediatr ; 256: 18-26.e8, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-36470465

RESUMEN

OBJECTIVE: To analyze, in a cohort of pediatric patients with recurrent pericarditis undergoing anti-interleukin (IL)-1 treatment: the agent and dosing used as first-line treatment, the long-term efficacy of IL-1 blockers, the percentage of patients achieving a drug-free remission, and the presence of variables associated with drug-free remission. STUDY DESIGN: Data were collected from patients' charts. The annualized relapse rate (ARR) was used for evaluation of treatment efficacy, and bivariate logistic regression analysis was used for variables associated with drug-free remission. RESULTS: Fifty-eight patients, treated between 2008 and 2018, were included in the study (mean follow-up. 2.6 years). Of the 56 patients treated with first-line drugs, 14 not responsive patients were underdosed. Fifty-seven patients were treated with anakinra: the ARR before and during daily treatment was 3.05 and 0.28, respectively (P < .0001); an increase to 0.83 was observed after the reduction/withdrawal of treatment (P < .0001). The switch from anakinra to canakinumab (5 patients) was associated to an increase of the ARR (0.49 vs 1.46), but without statistical significance (P = .215). At last follow-up, only 9 of the 58 patients had withdrawn all treatments. With the limits of a retrospective study and the heterogeneity between the patients enrolled in the study, a shorter duration of treatment with anakinra was the only variable associated with drug-free remission. CONCLUSIONS: This study shows that most pediatric patients with recurrent pericarditis needing IL-1 blockade received an inadequate treatment with first-line agents. The effectiveness of anakinra is supported by this study, but few patients achieved drug-free remission. The different rate of response to anakinra and canakinumab may suggest a possible role of IL-1α in the pathogenesis of recurrent pericarditis.


Asunto(s)
Proteína Antagonista del Receptor de Interleucina 1 , Pericarditis , Humanos , Niño , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Estudios Retrospectivos , Interleucina-1/uso terapéutico , Nivel de Atención , Resultado del Tratamiento , Pericarditis/tratamiento farmacológico , Recurrencia
6.
Heart Fail Rev ; 28(1): 77-95, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-35536402

RESUMEN

Cardiac magnetic resonance (CMR) has become an essential tool for the evaluation of patients affected or at risk of developing cardiomyopathies (CMPs). In fact, CMR not only provides precise data on cardiac volumes, wall thickness, mass and systolic function but it also a non-invasive characterization of myocardial tissue, thus helping the early diagnosis and the precise phenotyping of the different CMPs, which is essential for early and individualized treatment of patients. Furthermore, several CMR characteristics, such as the presence of extensive LGE or abnormal mapping values, are emerging as prognostic markers, therefore helping to define patients' risk. Lastly new experimental CMR techniques are under investigation and might contribute to widen our knowledge in the field of CMPs. In this perspective, CMR appears an essential tool to be systematically applied in the diagnostic and prognostic work-up of CMPs in clinical practice. This review provides a deep overview of clinical applicability of standard and emerging CMR techniques in the management of CMPs.


Asunto(s)
Cardiología , Cardiomiopatías , Cardiopatías , Humanos , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/patología , Corazón , Cardiopatías/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Espectroscopía de Resonancia Magnética , Valor Predictivo de las Pruebas , Medios de Contraste , Imagen por Resonancia Cinemagnética/métodos
7.
Artículo en Inglés | MEDLINE | ID: mdl-37163222

RESUMEN

ABSTRACT: Recurrent pericarditis (RP) is the most troublesome complication of acute pericarditis reflecting an unresolving inflammation of the pericardial sac around the heart and associated with significant morbidity. Recent studies have shown interleukin-1 (IL-1) signaling to be central to the pathophysiology of cases of RP with evidence of activation of systemic inflammation. We herein review the literature and clinical trials discussing the utility of IL-1 blockade for RP. The early experience of IL-1 blockade with anakinra (Kineret) and its favorable safety profile paved the way for the clinical development of rilonacept (Arcalyst) and subsequent approval by the US FDA for RP. In patients with RP who have become colchicine-resistant and glucocorticoid-dependent, IL-1 blockade with rilonacept or anakinra effectively treats recurrences and prevents future flares, and significantly improves quality of life.

8.
Cardiology ; 148(3): 219-227, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36948161

RESUMEN

BACKGROUND: ECG is the initial diagnostic tool that in combination with typical symptoms often raises the suspicion of pericarditis. Echocardiography remains the first-line imaging modality for assessment of pericardial diseases, particularly effusion/tamponade, constrictive physiology, and assessment of regional wall motion abnormalities as differential diagnoses. However, cardiac CT and cardiac magnetic resonance may be necessary in complicated cases and to identify pericardial inflammation in specific settings (atypical presentation, new onset constriction), as well as myocardial involvement and monitoring the disease activity. SUMMARY: In acute pericarditis, the most commonly used ECG criteria recommended by international guidelines are the widespread ST-segment elevation or PR depression. However, the classic ECG pattern of widespread ST-segment elevation or PR depression can be seen in less than 60% of patients. In addition, ECG changes are often temporally dynamic, evolve rapidly during the course of disease, and may be influenced by a number of factors such as disease severity, time (stage) of presentation, degree of myocardial involvement, and the treatment initiated. Overall, temporal dynamic changes on ECG during acute pericarditis or myopericarditis have received limited attention. Hence, the aim of this brief clinical review was to increase awareness about the various ECG changes observed during the course of acute pericarditis. KEY MESSAGES: ECG may be normal at presentation or for days after the index episode of chest pain, but serial ECGs can reveal specific patterns of temporally dynamic ST elevation in patients with pericarditis or myopericarditis, particularly during new episodes of chest pain.


Asunto(s)
Miocarditis , Pericarditis , Humanos , Enfermedad Aguda , Arritmias Cardíacas/complicaciones , Dolor en el Pecho/etiología , Ecocardiografía , Electrocardiografía , Miocarditis/diagnóstico por imagen , Pericarditis/diagnóstico por imagen
9.
Eur Heart J ; 43(31): 2946-2957, 2022 08 14.
Artículo en Inglés | MEDLINE | ID: mdl-34528670

RESUMEN

Anti-interleukin (IL)-1 agents have been developed for the treatment of autoinflammatory and rheumatic conditions, where overproduction of IL-1 is an important pathophysiologic process. IL-1α and IL-1ß are the most studied members of the IL-1 family of cytokines and have the strongest proinflammatory effects. A naturally occurring antagonist (IL-1Ra) mitigates their proinflammatory effects. Overproduction of both IL-1α (released by inflamed/damaged pericardial cells) and IL-1ß (released by inflammatory cells) is now a well-recognized therapeutic target in patients with recurrent idiopathic pericarditis. Currently, there are three available anti-IL-1 agents: anakinra (recombinant human IL-1Ra), rilonacept (a soluble decoy receptor 'trap', binding both IL-1α and IL-1ß), and canakinumab (human monoclonal anti-IL-1ß antibody). For patients with corticosteroid-dependent and colchicine-resistant recurrent pericarditis with evidence of systemic inflammation, as evidenced by elevated C-reactive protein, the efficacy and safety of anakinra (2 mg/kg/day up to 100 mg/day subcutaneously usually for at least 6 months, then tapered) and rilonacept (320 mg subcutaneously for the first day followed by 160 mg subcutaneously weekly) have been clearly demonstrated in observational studies and randomized controlled clinical trials. Severe side effects are rare and discontinuation rates are very low (<4%). The most common reported side effect is injection site reactions (>50% of patients). In this article, we describe the historical and pathophysiological background and provide a comprehensive review of these agents, which appear to be the most significant advance in medical therapy of recurrent pericarditis in the last 5 years.


Asunto(s)
Cardiólogos , Pericarditis , Colchicina/uso terapéutico , Humanos , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Pericarditis/tratamiento farmacológico
10.
Eur J Clin Invest ; 52(10): e13815, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35598175

RESUMEN

BACKGROUND: Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization. Left ventricular peak global longitudinal strain (LV-GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF. METHODS: Data of patients undergoing cardiac magnetic resonance (CMR) for clinically suspected AM in seven European Centres (2013-2020) were retrospectively analysed. Patients with AM confirmed by CMR and LVEF ≥50% were included. LGE was visually characterized: localized versus. non-localized, subepicardial versus midwall. LV-GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including cardiac death, life-threatening arrhythmias, development of heart failure or of LVEF <50%. RESULTS: Of 389 screened patients, 256 (66%) fulfilled inclusion criteria: median age 36 years, 71% males, median LVEF 60%, median LV-GLS -17.3%. CMR was performed at 4 days from hospitalization. At 27 months, 24 (9%) patients experienced ≥1 ACE (71% developed LVEF <50%). Compared to the others, they had lower median LV-GLS values (-13.9% vs. -17.5%, p = .001). At Kaplan-Meier analysis, impaired LV-GLS (both considered as > -20% or quartiles), non-localized and midwall LGE were associated with ACEs. Patients with LV-GLS ≤-20% did not experience ACEs. LV-GLS remained associated with ACEs after adjustment for non-localized and midwall LGE. CONCLUSION: In AM presenting with LVEF ≥50%, LV-GLS provides independent prognostic value over LGE characterization, improving risk stratification and representing a rationale for further studies of therapy in this cohort.


Asunto(s)
Miocarditis , Función Ventricular Izquierda , Adulto , Medios de Contraste , Femenino , Gadolinio , Humanos , Imagen por Resonancia Cinemagnética , Espectroscopía de Resonancia Magnética , Masculino , Miocarditis/diagnóstico por imagen , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Volumen Sistólico
11.
Rev Cardiovasc Med ; 23(7): 228, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39076901

RESUMEN

Background and Aim of the Study: In patients with acute type A aortic dissection (A-AAD) whether initial repair should include also aortic arch replacement is still debated. We aimed to assess if extensive aortic repair prevents from reoperations patients with A-AAD. Methods: Outcomes after distal reoperation following repair of A-AAD (n = 285; 1977 to 2018) were analysed in 22 of 226 who underwent ascending aorta/hemiarch replacement (Group 1 R ) and 7 of 59 who had ascending aorta/arch replacement (Group 2 R ). Results: Distal reoperation was more common in Group 1 R (n = 22) than in Group 2 R (n = 0) (p < 0.001) while thoracic endovascular stenting was more frequent in Group 2 R (7 vs 3, p < 0.001). Indications for reoperation were pseudoaneurysm at distal anastomosis (n = 4, 18%) and progression of aortic dissection (n = 18, 82%) in Group 1 R . Indication for thoracic endovascular stenting was progressive aortic dissection in 3 patients of Group 1 R and in 6 of Group 2 R . Second reoperation was required in 2 patients from Group 1 R (2%) during a mean follow-up of 5 years. Median follow-up was 4 years in Group 1 R and 7 years in Group 2 R (p = 0.36). Hospital mortality was 14% in Group 1 R and 0% in Group 2 R (p = 0.3). Actuarial survival is 68 ± 10%, and 62 ± 11% for Group 1 R and 100% for Group 2 R at 5 and 10 years (p = 0.076). Conclusions: Distal reoperations after A-AAD repair have an acceptable mortality. An extensive initial repair has lower rate of reoperation and better mid-term survival and should be indicated especially for young patients in experienced centers.

12.
Echocardiography ; 39(9): 1259-1260, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35907783

RESUMEN

We describe an interventricular septum mass in 1 years old child, followed during 14 years. The mass did not grow up over time, the patient did not experienced any arrythmia, and did not developed heart failure. A complete diagnosis of interventricular Fibroma was made at the age of 14 years old when the patient underwent to cardiac MRI. A close follow up was in this case the winner strategy, saving him from an early unnecessary cardiac surgery.


Asunto(s)
Fibroma , Insuficiencia Cardíaca , Neoplasias Cardíacas , Tabique Interventricular , Adolescente , Niño , Preescolar , Fibroma/diagnóstico por imagen , Fibroma/cirugía , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Tabique Interventricular/diagnóstico por imagen , Tabique Interventricular/cirugía
13.
Echocardiography ; 39(9): 1158-1170, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-36029124

RESUMEN

Mitral valve prolapse (MVP) is the most frequent valvulopathy with a prevalence of 1.2%-2.4% in general population and it is characterized by a benign course. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) as ultimate expressions, are the most worrying. The estimated risk of SCD in MVP is between 0.2% and 1.9% per year including both MVP patients with left ventricular (LV) dysfunction due to severe MR and MVP patients without significant MR. The latter ones constitute a particular phenotype called "malignant MVP" characterized by bileaflet myxomatous prolapse, ECG repolarization abnormalities and complex VAs (c-VAs) with polymorphic/right bundle branch block morphology (RBBB) and LV fibrosis of the papillary muscles (PMs) and inferobasal wall secondary to mechanical stretching visualized as late gadolinium enhancement (LGE) areas by cardiac magnetic resonance (CMR). In MVP, the first diagnostic approach is transthoracic echocardiography (TTE) that defines the presence of mitral annular disjunction (MAD) which seems to be associated with "arrhythmic MVP" (AMVP). From an ECG point of view, AMVP is characterized by frequent premature ventricular contractions (PVCs) arising from one or both PMs, fascicular tissue, and outflow tract, as well as by T-wave inversion in the inferolateral leads. The aim of the present paper is to describe TTE red flags that could identify MVP patients at high risk to develop complex arrhythmias as supported by the corresponding findings of LGE-CMR and anatomy studies. TTE could be a co-partner in phenotyping high-risk arrhythmic MVP patients.


Asunto(s)
Prolapso de la Válvula Mitral , Arritmias Cardíacas/complicaciones , Arritmias Cardíacas/diagnóstico por imagen , Bloqueo de Rama/complicaciones , Medios de Contraste , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/patología , Ecocardiografía , Gadolinio , Humanos , Prolapso de la Válvula Mitral/complicaciones , Prolapso de la Válvula Mitral/diagnóstico por imagen , Músculos Papilares/diagnóstico por imagen , Músculos Papilares/patología , Fenotipo
14.
J Card Surg ; 37(10): 3389-3392, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35866184

RESUMEN

Cardiac sarcomas are rare and aggressive tumors that could have a multiorgan involvement and unfavorable prognosis. We present an extremely rare situation of cardiac sarcoma in a fragile elderly patient with a dramatic presentation of cardiogenic shock.


Asunto(s)
Infarto del Miocardio , Sarcoma , Anciano , Ventrículos Cardíacos/patología , Humanos , Infarto del Miocardio/patología , Pronóstico , Sarcoma/complicaciones , Sarcoma/diagnóstico , Sarcoma/cirugía , Choque Cardiogénico/etiología
15.
Eur Heart J ; 42(28): 2745-2760, 2021 07 21.
Artículo en Inglés | MEDLINE | ID: mdl-33961006

RESUMEN

Colchicine is a unique, sophisticated anti-inflammatory agent that has been used for decades for the prevention of acute inflammatory flares in gout and familial Mediterranean fever. In recent years, clinical trials have demonstrated its potential in a range of cardiovascular (CV) conditions. Colchicine is avidly taken up by leucocytes, and its ability to bind to tubulin and interfere with microtubular function affects the expression of cytokines and interleukins, and the ability of neutrophils to marginate, ingress, aggregate, express superoxide, release neutrophil extracellular traps, and interact with platelets. In patients with acute and recurrent pericarditis, clinical trials in >1600 patients have consistently shown that colchicine halves the risk of recurrence [relative risk (RR) 0.50, 95% confidence interval (CI) 0.42-0.60]. In patients with acute and chronic coronary syndromes, multicentre randomized controlled trials in >11 000 patients followed for up to 5 years demonstrated that colchicine may reduce the risk of CV death, myocardial infarction, ischaemic stroke and ischaemia-driven revascularization by >30% (RR 0.63, 95% CI 0.49-0.81). The use of colchicine at doses of 0.5-1.0 mg daily in CV trials has proved safe. Early gastrointestinal intolerance limits its use in ∼10% of patients; however, ∼90% of patients tolerate it well over the long term. Despite isolated case reports, clinically relevant drug interactions with moderate to strong CYP3A4 inhibitors/competitors or P-glycoprotein inhibitors/competitors are rare if this dosage of colchicine is used in the absence of advanced renal or liver disease. The aim of this review is to summarize the contemporary data supporting the efficacy and safety of colchicine in patients with CV disease.


Asunto(s)
Isquemia Encefálica , Pericarditis , Accidente Cerebrovascular , Antiinflamatorios/uso terapéutico , Colchicina/uso terapéutico , Humanos , Pericarditis/tratamiento farmacológico
16.
Eur Heart J ; 42(16): 1554-1568, 2021 04 21.
Artículo en Inglés | MEDLINE | ID: mdl-33825853

RESUMEN

Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.


Asunto(s)
Amiloidosis , Cardiomiopatías , Cardiopatías , Amiloidosis/diagnóstico , Amiloidosis/terapia , Cardiomiopatías/diagnóstico , Cardiomiopatías/terapia , Corazón , Cardiopatías/diagnóstico , Cardiopatías/terapia , Humanos , Miocardio
17.
Pak J Med Sci ; 38(8): 2373-2375, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36415240

RESUMEN

Fabry disease (FD) is a relatively rare X-linked hereditary disease caused by mutations in the GLA gene that results in deficient α-galactosidase A (α-Gal A) enzyme activity. The disturbed catabolism of the neutral sphingolipids globotriaosylceramide (Gb3) leads to its progressive lysosomal accumulation throughout the body. Multiple organs can be affected. The atypical late-onset cardiac variant is associated with a high burden of cardiac morbidity and mortality. The true burden of FD in Central and some South Asian countries is unknown. Lack of research studies and awareness, and misdiagnosis/underdiagnosis may be the reasons. Some possible explanations as well suggestions for a structured Fabry care and research possibilities in these WHO regions are offered.

18.
Pak J Med Sci ; 38(8): 2337-2344, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36415271

RESUMEN

Fabry disease (FD) is a relatively rare X-linked hereditary disease caused by mutations in the GLA gene that results in deficient α-galactosidase A (α-Gal A) enzyme activity. The disturbed catabolism of the neutral sphingolipids globotriaosylceramide (Gb3) leads to its progressive lysosomal accumulation throughout the body. Multiple organs can be affected. The atypical late-onset cardiac variant is associated with a high burden of cardiac morbidity and mortality. The aim of this work was to present an updated overview of the FD, with focus on cardiovascular manifestations and its management. Enzyme replacement therapy (ERT) is nowadays an established treatment of FD and is recommended as early as possible with or without chaperone therapy (migalastat) to prevent or delay the progression of renal, cardiac, and cerebrovascular complications. It improves quality of life and may further result in decrease in Left ventricular (LV) mass and to some extent LV function recovery. However, LV hypertrophy (LVH) does not always respond well to ERT despite successful Gb3 clearance. Furthermore, its impact on the hard clinical events is uncertain. Some possible reasons for this apparent discrepancy are discussed. ERT may be less effective in patients who have already developed fibrosis or irreversible organ damage. However, other confounding factors may be equally important.

19.
Eur J Clin Invest ; 51(11): e13602, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34050527

RESUMEN

BACKGROUND: Currently, we remain uncertain about which patients are at increased risk for recurrent pericarditis. We developed a risk score for pericarditis recurrence in patients with acute pericarditis. MATERIALS AND METHODS: We prospectively recruited 262 patients with a first episode of acute pericarditis. Baseline patients' demographics, clinical, imaging and laboratory data were collected. Patients were followed up for a median of 51 months (interquartile range 21-71) for recurrence. Variables with <10% missingness were entered into multivariable logistic regression models with stepwise elimination to explore independent predictors of recurrence. The final model performance was assessed by the c-index whereas model's calibration and optimism-corrected c-index were evaluated after 10-fold cross-validation. RESULTS: We identified six independent predictors for pericarditis recurrence, that is age, effusion size, platelet count (negative predictors) and reduced inferior vena cava collapse, in-hospital use of corticosteroids and heart rate (positive predictors). The final model had good performance for recurrence, c-index 0.783 (95% CI 0.725-0.842), while the optimism-corrected c-index after cross-validation was 0.752. Based on these variables, we developed a risk score point system for recurrence (0-22 points) with equally good performance (c-index 0.740, 95% CI 0.677-0.803). Patients with a low score (0-7 points) had 21.3% risk for recurrence, while those with high score (≥12 points) had a 69.8% risk for recurrence. The score was predictive of recurrence among most patient subgroups. CONCLUSIONS: A simple risk score point system based on 6 variables can be used to predict the individualized risk for pericarditis recurrence among patients with a first episode of acute pericarditis.


Asunto(s)
Antiinflamatorios no Esteroideos/uso terapéutico , Glucocorticoides/uso terapéutico , Pericardiocentesis , Pericarditis/terapia , Adulto , Factores de Edad , Anciano , Aspirina/uso terapéutico , Dolor en el Pecho/fisiopatología , Colchicina/uso terapéutico , Femenino , Fiebre/fisiopatología , Frecuencia Cardíaca/fisiología , Humanos , Ibuprofeno/uso terapéutico , Masculino , Persona de Mediana Edad , Pericarditis/sangre , Pericarditis/fisiopatología , Recuento de Plaquetas , Recurrencia , Medición de Riesgo , Factores de Riesgo
20.
Eur Heart J Suppl ; 23(Suppl C): C154-C163, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34456642

RESUMEN

The COVID-19 pandemic represents an unprecedented event that has brought deep changes in hospital facilities with reshaping of the health system organization, revealing inadequacies of current hospital and local health systems. When the COVID-19 emergency will end, further evaluation of the national health system, new organization of acute wards, and a further evolution of the entire health system will be needed to improve care during the chronic phase of disease. Therefore, new standards for healthcare personnel, more efficient organization of hospital facilities for patients with acute illnesses, improvement of technological approaches, and better integration between hospital and territorial services should be pursued. With experience derived from the COVID-19 pandemic,new models, paradigms, interventional approaches, values and priorities should be suggested and implemented.

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