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1.
Rev Port Cardiol ; 43(1): 13-19, 2024 Jan.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-37423311

RESUMEN

INTRODUCTION AND OBJECTIVES: Patients with hypertrophic obstructive cardiomyopathy (HOCM) that remain symptomatic despite optimized medical therapy often undergo alcohol septal ablation (ASA). One of the most frequent complications is complete heart block (CHB), requiring a permanent pacemaker (PPM) in variable rates of up to 20% of patients. The long-term impact of PPM implantation in these patients remains unclear. This study aimed to evaluate the long-term clinical outcomes in patients who implant PPM after ASA. METHODS: Patients who underwent ASA at a tertiary center were consecutively and prospectively enrolled. Patients with previous PPM or implantable cardio-defibrillator were excluded from this analysis. Patients with and without PPM implantation after ASA were compared based on their baseline characteristics, procedure data and three-year primary endpoint of composite of all-cause mortality and hospitalization and secondary endpoint of composite of all-cause mortality and cardiac cause hospitalization. RESULTS: Between 2009 and 2019, 109 patients underwent ASA, 97 of whom were included in this analysis (68% female, mean age 65.2 years old). 16 patients (16.5%) required PPM implantation for CHB. In these patients, no vascular access, pacemaker pocket or pulmonary parenchyma complications were noted. The baseline characteristics of comorbidities, symptoms, echocardiographic and electrocardiographic findings were identical in the two groups, with higher mean age (70.6±10.0 years vs. 64.1±11.9 years) and lower beta-blocker therapy rate (56% vs. 84%) in the PPM group. Procedure-related data showed higher creatine kinase (CK) peaks in the PPM group (1692 U/L vs. 1243 U/L), with no significant difference in the alcohol dose. At three years after ASA procedure, there were no differences in the primary and secondary endpoints between the two groups. CONCLUSIONS: Permanent pacemaker after ASA induced CHB do not affect long term prognosis in hypertrophic obstructive cardiomyopathy patients.


Asunto(s)
Técnicas de Ablación , Cardiomiopatía Hipertrófica , Marcapaso Artificial , Humanos , Femenino , Anciano , Persona de Mediana Edad , Anciano de 80 o más Años , Masculino , Tabiques Cardíacos/cirugía , Ecocardiografía , Cardiomiopatía Hipertrófica/cirugía , Marcapaso Artificial/efectos adversos , Bloqueo Cardíaco/etiología , Bloqueo Cardíaco/terapia , Resultado del Tratamiento , Técnicas de Ablación/efectos adversos , Técnicas de Ablación/métodos
2.
Acta Med Port ; 36(9): 598-602, 2023 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-37658721

RESUMEN

A 24-year-old man suffered a witnessed cardiac arrest after a padel game. Basic life support was immediately provided. The pre-hospital emergency services team continued the resuscitation efforts, and the patient was accepted for extracorporeal cardiopulmonary resuscitation. The return of spontaneous circulation was achieved in 45 minutes. The initial assessment revealed a ST-segment elevation in leads V4-V6 and a dilated left ventricle with severe systolic dysfunction. Coronary angiography was normal. An improvement in left ventricular systolic function was observed and extracorporeal cardiac support was discontinued after 48 hours. Cardiovascular magnetic resonance imaging demonstrated hypokinesia and subepicardial fatty infiltration of the left ventricle lateral wall. Genetic testing detected a variant of uncertain significance in the ANK2 gene. The diagnosis of arrhythmogenic left ventricular myocardiopathy did not fulfill all the current diagnostic criteria, but it is a very likely diagnosis. An implantable cardioverter-defibrillator was placed. The patient was discharged without physical or cognitive impairment.


Asunto(s)
Cardiomiopatías , Reanimación Cardiopulmonar , Disfunción Cognitiva , Paro Cardíaco , Masculino , Humanos , Adulto Joven , Adulto , Servicio de Urgencia en Hospital
3.
Eur Heart J Case Rep ; 7(3): ytad120, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36969513

RESUMEN

A 39-year-old female patient presents to the emergency department with palpitations and dyspnoea, with the admission electrocardiogram revealing atrial flutter. Step-by-step multimodality imaging techniques, including transthoracic echocardiography, computed tomography, cardiac magnetic resonance imaging, and intracardiac echocardiography-guided tissue biopsy, led to the final diagnosis of a right atrial angiosarcoma.

4.
Int J Cardiovasc Imaging ; 39(4): 793-809, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36543912

RESUMEN

Left ventricular hypertrophy (LVH) is a frequent imaging finding in the general population. In order to identify the precise etiology, a comprehensive diagnostic approach should be adopted, including the prevalence of each entity that may cause LVH, family history, clinical, electrocardiographic and imaging findings. By providing a detailed evaluation of the myocardium, cardiovascular magnetic resonance (CMR) has assumed a central role in the differential diagnosis of left ventricular hypertrophy, with the technique of parametric imaging allowing more refined tissue characterization. This article aims to establish a parallel between pathophysiological features and imaging findings through the broad spectrum of LVH entities, emphasizing the role of CMR in the differential diagnosis.


Asunto(s)
Corazón , Hipertrofia Ventricular Izquierda , Humanos , Hipertrofia Ventricular Izquierda/etiología , Valor Predictivo de las Pruebas , Imagen por Resonancia Magnética/métodos , Espectroscopía de Resonancia Magnética
5.
Rev Port Cardiol ; 41(7): 559-568, 2022 Jul.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-36065775

RESUMEN

BACKGROUND: Microvascular dysfunction is an often overlooked feature of hypertrophic cardiomyopathy (HCM). Our aim was to assess the association between microvascular dysfunction, wall thickness, tissue characteristics and myocardial deformation in HCM patients, by analyzing individual myocardial segments. METHODS: Prospective assessment including cardiac magnetic resonance to assess wall thickness, T1 and T2 mapping, extracellular volume, late gadolinium enhancement (LGE) and stress perfusion. Results were stratified according to the 16 American Heart Association segments. RESULTS: Seventy-five patients were recruited (1200 segments), 63% male, mean age 54.6±14.8 years, maximal wall thickness of 20.22±4.6 mm. Among the 424 segments (35%) with perfusion defects, 24% had defects only in the endocardial layer and 12% in both endocardial and epicardial layers. Perfusion defects were more often detected in hypertrophied segments (64%). Among the 660 segments with normal wall thickness, 19% presented perfusion defects. Independently of wall thickness, segments with perfusion defects had a higher T1 (ß-estimate 30.28, p<0.001), extracelluar volume (ß-estimate 1.50, p<0.001) and T2 (ß-estimate 0.73, p<0.001) and had late gadolinium enhancement more frequently (odds ratio 4.16, p<0.001). Higher values of circumferential strain (lower deformation) and lower values of radial strain were found in segments with perfusion defects (ß-estimate 2.76, p<0.001; and ß-estimate -10.39, p<0.001, circumferential and radial strain, respectively). CONCLUSION: While microvascular dysfunction was more prevalent in more hypertrophied segments, it also had a major presence in segments without hypertrophy. In this segmental analysis, we found an association between the presence of ischemia and tissue abnormalities, replacement fibrosis as well as impaired strain, independently of the segmental wall thickness.

6.
Rev Port Cardiol ; 41(7): 573-582, 2022 Jul.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-36065777

RESUMEN

INTRODUCTION: The management of acute coronary syndrome (ACS) in malignancy is challenging due to higher bleeding risk. METHODS: We analyzed patients with cancer (active or in the previous five years) prospectively included in the ProACS registry between 2010 and 2019. Our aim was to assess safety (major bleeding, primary endpoint) and secondary efficacy endpoints (in-hospital mortality and combined in-hospital mortality, reinfarction and ischemic stroke) of ACS treatment. Propensity score matching analysis (1:1) was further performed to better understand predictors of outcomes. RESULTS: We found 934 (5%) cancer patients out of a total of 18 845 patients with ACS. Cancer patients had more events: major bleeding (2.9% vs. 1.5%), in-hospital mortality (5.8% vs. 3.4%) and the combined endpoint (7.4% vs. 4.9%). The primary endpoint was related to cancer diagnosis (OR 1.97), previous bleeding (OR 7.09), hemoglobin level (OR 4.94), atrial fibrillation (OR 3.50), oral anticoagulation (OR 3.67) and renal dysfunction. Mortality and the combined secondary endpoint were associated with lower use of invasive coronary angiography and antiplatelet and neurohormonal blocker therapy. After propensity score matching (350 patients), there were no statistically significant differences in endpoints between the populations. CONCLUSION: Bleeding risk was not significant higher in the cancer population compared to patients with similar characteristics, nor were mortality or ischemic risk. The presence of cancer should not preclude simultaneous ACS treatment.

7.
Rev Port Cardiol ; 41(9): 761-767, 2022 Sep.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-36066271

RESUMEN

INTRODUCTION AND OBJECTIVES: Coronary microvascular dysfunction (CMD) is one of the most important pathophysiological features in hypertrophic cardiomyopathy (HCM). The index of microcirculatory resistance (IMR) is an invasive method to assess the coronary microcirculation. The aim was to assess CMD in patients with HCM by IMR. METHODS: Adult patients with HCM without epicardial coronary artery disease underwent cardiac catheterization for the assessment of CMD by IMR (normal cut-off value ≤22.0) and coronary flow reserve (CFR) (normal cut-off value ≥2). Cardiovascular magnetic resonance (CMR) was performed to assess the ischemic burden by perfusion imaging during regadenoson-induced hyperemia, and the extent of myocardial fibrosis was assessed by late gadolinium enhancement (LGE), native T1 mapping and extracellular volume (ECV). RESULTS: Fourteen patients were enrolled with a mean age of 62.8±6.2years, 8 (57.1%) males, of whom 9 (64.3%) had obstructive HCM. Using IMR, CMD was detected in 4 (29%) patients. Among four patients with an IMR>22.0, all had non-obstructive HCM and two had angina. CFR<2 was reported in eight patients (57%). Concordance between IMR and CFR (both normal or both abnormal) was verified in 6 patients (43%). Among four patients with IMR>22.0, perfusion defects were found in two of the three patients who underwent stress CMR. Increased ECV (>28%) was documented in two of the patients with IMR>22 and in three of the patients with IMR≤22.0. LGE was >15% in 2 of the patients with IMR>22 and in 4 with IMR≤22.0. CONCLUSIONS: IMR assessment in HCM is feasible and safe. Patients with abnormal IMR seemed to have more significant tissue abnormalities on CMR.

8.
Int J Cardiol ; 359: 61-68, 2022 07 15.
Artículo en Inglés | MEDLINE | ID: mdl-35427701

RESUMEN

BACKGROUND: Coronary microvascular dysfunction constitutes an important pathophysiological feature in hypertrophic cardiomyopathy (HCM). We aimed to assess the association between impaired coronary flow velocity reserve (CFVR) and ventricular systolic function and functional capacity. METHODS: Eighty-three patients with HCM were enrolled in this prospective cohort study. Patients underwent echocardiogram to evaluate ventricular performance and CFVR in the left anterior descending artery (LAD) and posterior descending artery (PD). Diastolic coronary flow velocity was measured in basal conditions and in hyperemia. CFVR was calculated as the ratio of hyperemic and basal peak diastolic flow velocities. Functional capacity was evaluated by cardiopulmonary exercise testing (CPET). The link between CFVR and biventricular systolic function and peak VO2 was studied. RESULTS: Age was 55.0(14.4)years, 50 patients (60%) were male; 59 patients (71%) had nonobstructive HCM. Mean CFVR LAD was 1.81(0.49) and CFVR PD was 1.73(0.55). Lower CFVR PD was associated with impaired global longitudinal strain (GLS) 2D (ß-estimate:-3.240,95%CI:-4.634;-1.846, p < 0.001), GLS 3D (ß-estimate:-2.559,95%CI:-3.932;-1.186, p < 0.001) and area strain (ß-estimate:-3.044,95%CI:-5.373;-0.716, p = 0.011). Lower values of CFVR PD related to worse global work index (ß-estimate:267.824,95%CI:75.964;459.683, p = 0.007), global constructive work (ß-estimate:217.300,95%CI:38.750;395.850, p = 0.018) and global work efficiency (ß-estimate:5.656,95%CI:2.229;9.084, p = 0.002). Impaired CFVR LAD (ß-estimate:2.826, 95%CI:0.913;4.739, p = 0.004) and CFVR PD (ß-estimate:2.801,95%CI:0.657;4.945, p = 0.011) were associated with lower TAPSE. Lower values of CFVR LAD (ß-estimate:2.580, 95%CI:0.169;4.991, p = 0.036) and CFVR PD (ß-estimate:3.163, 95%CI: 0.721;5.606, p = 0.012) were associated with worse peak VO2. CONCLUSION: Lower CFVR was associated with impairment in biventricular systolic function parameters and functional capacity assessed by pVO2.


Asunto(s)
Cardiomiopatía Hipertrófica , Circulación Coronaria , Velocidad del Flujo Sanguíneo/fisiología , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Circulación Coronaria/fisiología , Vasos Coronarios , Femenino , Humanos , Masculino , Estudios Prospectivos
9.
Front Cardiovasc Med ; 8: 761860, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34977179

RESUMEN

Aims: The aim of the study is to investigate the association between the degree of ischemia due to coronary microvascular dysfunction (CMD) and the left ventricular (LV) tissue characteristics, systolic performance, and clinical manifestations in hypertrophic cardiomyopathy (HCM). Methods and Results: This prospective study enrolled 75 patients with HCM without obstructive epicardial coronary artery disease. Each patient underwent cardiovascular magnetic resonance (CMR) including parametric mapping, perfusion imaging during regadenoson-induced hyperemia, late gadolinium enhancement (LGE) and three-dimensional longitudinal, circumferential, and radial strains analysis. Electrocardiogram, 24-h Holter recording, and cardiopulmonary exercise testing (CPET) were performed to assess arrhythmias and functional capacity. In total, 47 (63%) patients were men with the mean age of 54.6 (14.8) years, 51 (68%) patients had non-obstructive HCM, maximum wall thickness (MWT) was 20.2 (4.6) mm, LV ejection fraction (LVEF) was 71.6 (8.3%), and ischemic burden was 22.5 (16.9%) of LV. Greater MWT was associated with the severity of ischemia (ß-estimate:1.353, 95% CI:0.182; 2.523, p = 0.024). Ischemic burden was strongly associated with higher values of native T1 (ß-estimate:9.018, 95% CI:4.721; 13.315, p < 0.001). The association between ischemia and LGE was significant in following subgroup analyses: MWT 15-20 mm (ß-estimate:1.941, 95% CI:0.738; 3.143, p = 0.002), non-obstructive HCM (ß-estimate:1.471, 95% CI:0.258; 2.683, p = 0.019), women (ß-estimate:1.957, 95% CI:0.423; 3.492, p = 0.015) and age <40 years (ß-estimate:4.874, 95% CI:1.155; 8.594, p = 0.016). Ischemia in ≥21% of LV was associated with LGE >15% (AUC 0.766, sensitivity 0.724, specificity 0.659). Ischemia was also associated with atrial fibrillation or flutter (AF/AFL) (OR-estimate:1.481, 95% CI:1.020; 2.152, p = 0.039), but no association was seen for non-sustained ventricular tachycardia. Ischemia was associated with shorter time to anaerobic threshold (ß-estimate: -0.442, 95% CI: -0.860; -0.023, p = 0.039). Conclusion: In HCM, ischemia associates with morphological markers of severity of disease, fibrosis, arrhythmia, and functional capacity.

10.
Microcirculation ; 28(1): e12656, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32896949

RESUMEN

Myocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy. Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction. Since the direct visualization of coronary microcirculation in vivo is not possible, its function must be studied indirectly. Invasive and noninvasive techniques allow microcirculatory dysfunction to be evaluated, including echocardiography, magnetic resonance, positron emission tomography, and cardiac catheterization. Blunted myocardial blood flow and coronary flow reserve have been suggested to associate with unfavorable prognosis. Microcirculatory dysfunction may be one additional important parameter to take into account for risk stratification beyond the conventional risk factors.


Asunto(s)
Cardiomiopatía Hipertrófica , Circulación Coronaria , Ecocardiografía , Microcirculación , Microvasos , Isquemia Miocárdica , Tomografía de Emisión de Positrones , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Humanos , Microvasos/diagnóstico por imagen , Microvasos/fisiopatología , Isquemia Miocárdica/diagnóstico por imagen , Isquemia Miocárdica/fisiopatología
11.
Rev Port Cardiol (Engl Ed) ; 38(7): 473-480, 2019 Jul.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-31495717

RESUMEN

INTRODUCTION: In obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA) can lead to gradient reduction and symptom improvement. We aimed to assess the efficacy and safety of ASA in a long-term outcome study. METHODS: We analyzed patients who underwent ASA over a seven-year period in a tertiary center. The primary echocardiographic endpoint was >50% reduction in left ventricular outflow tract (LVOT) gradient within a year of the procedure. The primary clinical endpoints were improvement in functional capacity and a combined endpoint of cardiac death and rehospitalization for cardiac cause. The follow-up period was 4.17±2.13 years. RESULTS: A total of 80 patients, mean age 63.9±12.3 years, 30.0% male, were analyzed. Baseline LVOT gradient was 96.3±34.6 mmHg and interventricular septal thickness was 21.6±3.1 mm. Minor complications were observed in 6.3% and major complications in 2.5%, and 8.8% received a permanent pacemaker. The primary echocardiographic endpoint was achieved by 85.7%. At three-month follow-up, LVOT gradient was 25.8±26.0 mmHg in the successful procedure group, compared to 69.2±35.6 mmHg in the other patients (p=0.001). At six months, LVOT gradient was 27.1±27.4 vs. 58.2±16.6 mmHg (p=0.024). Among 74 patients in NYHA class III/IV before the procedure, 57 (77%) improved to NHYA class I/II. The combined primary clinical endpoint (cardiac death and rehospitalization for cardiac cause) was observed in 27.5% (n=22). In the unsuccessful group, the combined endpoint was observed in 54.5%, compared to only 22.7% in the successful group. Only two patients died of cardiac causes. CONCLUSION: ASA is a safe procedure with a high success rate. Patients who achieved significant reductions in LVOT gradient suffered less cardiac death and rehospitalization for cardiac cause.


Asunto(s)
Técnicas de Ablación/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiomiopatía Hipertrófica/cirugía , Etanol/farmacología , Obstrucción del Flujo Ventricular Externo/cirugía , Tabique Interventricular/cirugía , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico , Ecocardiografía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/diagnóstico , Obstrucción del Flujo Ventricular Externo/etiología , Tabique Interventricular/diagnóstico por imagen
12.
Rev Port Cardiol (Engl Ed) ; 38(1): 11-16, 2019 Jan.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-30686652

RESUMEN

INTRODUCTION: Implantable cardioverter-defibrillator (ICD) monitoring zones (MZ) provide passive features that do not interfere with the functioning of active treatment zones. However, it is not known for certain whether programming an MZ affects arrhythmia detection by the ICD. The aim of the present study is to assess the clinical relevance of MZ in a population of patients with ICDs. METHODS: In this retrospective analysis of patients with ICDs, with or without cardiac resynchronization therapy, for primary prevention under remote monitoring, the MZ was analyzed and recorded arrhythmias were assessed in detail. RESULTS: A total of 221 patients were studied (77% men; age 64±12 years). Mean ejection fraction was 30±12%. The mean follow-up was 63±35 months. One hundred and seventy-four MZ events were documented in 139 patients (62.9%): 74 of non-sustained ventricular tachycardia (NSVT), 42 of supraventricular tachycardia, 44 of atrial fibrillation/atrial flutter, and five cases of noise. Among the 137 patients who presented with arrhythmias in the MZ (excluding two cases with noise detection only), 22 (16.1%) received appropriate shocks and/or antitachycardia pacing (ATP), while of the other 84 patients, 15.5% received appropriate ICD treatment (p=NS). In patients who presented with NSVT in the MZ, 15 (20.5%) received appropriate shocks and/or ATP. In accordance with the MZ findings, physicians decided to change outpatient medication in 41.7% of all patients in whom arrhythmic events were reported. CONCLUSION: Ventricular and supraventricular arrhythmias are common findings in the MZ of ICD patients. Programming an MZ is valuable in the diagnosis of arrhythmias and may be a useful tool in clinical practice.


Asunto(s)
Arritmias Cardíacas/diagnóstico , Desfibriladores Implantables , Monitoreo Fisiológico/métodos , Telemedicina/métodos , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/terapia , Muerte Súbita Cardíaca/prevención & control , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Prevención Primaria/métodos , Reproducibilidad de los Resultados , Estudios Retrospectivos , Factores de Tiempo
13.
Int J Cardiol Heart Vasc ; 22: 31-34, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30555891

RESUMEN

BACKGROUND: In previous guidelines, acute coronary syndromes (ACS) with new or presumably new left bundle branch block (LBBB) were an indication for reperfusion treatment, preferably with primary angioplasty. Recent guidelines also included the presence of right bundle branch block (RBBB) in this recommendation. It was our objective to evaluate in a population of patients with ACS the differential impact of RBBB and LBBB in prognosis. METHODS: Consecutive patients included prospectively in a single-centre registry of ACS were included in the study. Patients were analyzed according to baseline ECG characteristics (normal QRS, LBBB or RBBB). Primary outcome was all-cause mortality at one-year follow-up. We used Cox-proportional hazards models to assess the predictive value for the primary outcome. RESULTS: A total of 3990 patients were included in, with a mean age of 64 (13) years, 72% males, 3.4% with LBBB and 4.3% with RBBB. Patients with BBB were older, with more previous history of myocardial infarction and coronary revascularization and higher prevalence of cardiovascular risk factors (except smoking). Medical treatment was similar but they were less often submitted to angioplasty. In univariate analysis, BBB patients had worst outcome (Log-rank, p < 0.001), but similar in LBBB and RBBB (Log-rank, p = 0.597). In multivariate analysis, only RBBB (HR 1.66, 95%CI 1.14-2.40, p = 0.007) is an independent predictor of all-cause mortality. CONCLUSIONS: Patients with BBB have worst outcome after an ACS, particularly with RBBB. For that reason, we should pay special attention and treat these patients as aggressively as patients with normal QRS duration or LBBB.

14.
Rev Port Cardiol (Engl Ed) ; 37(12): 1007.e1-1007.e5, 2018 Dec.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-30001957

RESUMEN

Uhl's disease, also known as Uhl anomaly, is a rare disease secondary to selective but uncontrolled apoptosis of right ventricular myocytes during the perinatal period, after complete cardiac development, leading to the absence of right ventricular myocardium and the direct apposition of endocardium to epicardium without a myocardial layer in between, resulting in right ventricular failure. The present paper describes a case of Uhl's disease with an uncommon presentation. A 28-year-old man was admitted with dyspnea and cyanosis. Transthoracic echocardiography showed severe dilation of the right chambers, impaired right ventricular systolic function and a large ostium secundum atrial septal defect (ASD). Cardiac catheterization revealed pulmonary hypertension, with increased pulmonary capillary wedge pressure (mean 19mmHg) and Qp:QS 0.88:1. At this point, the authors considered that a main diagnosis of ASD could not explain the clinical features and hemodynamic data. A primary disease of the right ventricle was the most likely hypothesis and cardiac magnetic resonance imaging was performed, which demonstrated an extremely thin-walled right ventricle, with almost complete absence of right ventricular free wall myocardium, compatible with Uhl's disease.


Asunto(s)
Cardiomiopatía Dilatada , Cardiopatías Congénitas , Adulto , Cardiomegalia/diagnóstico por imagen , Cardiomegalia/etiología , Cardiomegalia/patología , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/patología , Cardiomiopatía Dilatada/fisiopatología , Cianosis/etiología , Ecocardiografía , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/patología , Cardiopatías Congénitas/fisiopatología , Humanos , Hipertensión Pulmonar/etiología , Imagen por Resonancia Magnética , Masculino
15.
Rev Port Cardiol (Engl Ed) ; 37(5): 399-405, 2018 May.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-29776810

RESUMEN

AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction. RESULTS: We analyzed 154 CPET cases. There were significant differences between groups, with the lowest peak oxygen consumption (VO2) values seen in patients with cardiac shunts (39% with Eisenmenger physiology) (17.2±7.1ml/kg/min, compared to 26.2±7.0ml/kg/min in tetralogy of Fallot patients; p<0.001), the lowest percentage of predicted peak VO2 in complex heart defects (50.1±13.0%) and the highest minute ventilation/carbon dioxide production slope in cardiac shunts (38.4±13.4). Chronotropism was impaired in patients with complex defects. Eisenmenger syndrome (n=17) was associated with the lowest peak VO2 (16.9±4.8 vs. 23.6±7.8ml/kg/min; p=0.001) and the highest minute ventilation/carbon dioxide production slope (44.8±14.7 vs. 31.0± 8.5; p=0.002). Age, cyanosis, CPET duration, peak systolic blood pressure, time to anaerobic threshold and heart rate at anaerobic threshold were predictors of the combined outcome of all-cause mortality and hospitalization for cardiac cause. CONCLUSION: Across the spectrum of CHD, cardiac shunts (particularly in those with Eisenmenger syndrome) and complex defects were associated with lower functional capacity and attenuated chronotropic response to exercise.


Asunto(s)
Prueba de Esfuerzo , Cardiopatías Congénitas/fisiopatología , Cardiopatías/congénito , Cardiopatías/fisiopatología , Adulto , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías/diagnóstico , Humanos , Masculino , Estudios Retrospectivos
16.
Eur Heart J Acute Cardiovasc Care ; 7(3): 218-223, 2018 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-28617040

RESUMEN

PURPOSE: The aim was to characterise acute coronary syndrome patients with complete atrioventricular block and to assess the effect on outcome. METHODS: Patients admitted with acute coronary syndrome were divided according to the presence of complete atrioventricular block: group 1, with complete atrioventricular block; group 2, without complete atrioventricular block. Clinical, electrocardiographic and echocardiographic characteristics and prognosis during one year follow-up were compared between the groups. RESULTS: Among 4799 acute coronary syndrome patients admitted during the study period, 91 (1.9%) presented with complete atrioventricular block. At presentation, group 1 patients presented with lower systolic blood pressure, higher Killip class and incidence of syncope. In group 1, 86.8% presented with ST-segment elevation myocardial infarction (STEMI), and inferior STEMI was verified in 79.1% of patients in group 1 compared with 21.9% in group 2 ( P<0.001). Right ventricular myocardial infarction was more frequent in group 1 (3.3% vs. 0.2%; P<0.001). Among patients who underwent fibrinolysis complete atrioventricular block was observed in 7.3% in contrast to 2.5% in patients submitted to primary percutaneous coronary intervention ( P<0.001). During hospitalisation group 1 had worse outcomes, with a higher incidence of cardiogenic shock (33.0% vs. 4.5%; P<0.001), ventricular arrhythmias (17.6% vs. 3.6%; P<0.001) and the need for invasive mechanical ventilation (25.3% vs. 5.1%; P<0.001). After a propensity score analysis, in a multivariate regression model, complete atrioventricular block was an independent predictor of hospital mortality (odds ratio 3.671; P=0.045). There was no significant difference in mortality at one-year follow-up between the study groups. CONCLUSION: Complete atrioventricular block conferred a worse outcome during hospitalisation, including a higher incidence of cardiogenic shock, ventricular arrhythmias and death.


Asunto(s)
Síndrome Coronario Agudo/complicaciones , Bloqueo Atrioventricular/etiología , Estimulación Cardíaca Artificial/métodos , Angiografía Coronaria/métodos , Electrocardiografía , Intervención Coronaria Percutánea/métodos , Puntaje de Propensión , Síndrome Coronario Agudo/epidemiología , Síndrome Coronario Agudo/cirugía , Anciano , Bloqueo Atrioventricular/epidemiología , Bloqueo Atrioventricular/terapia , Estudios de Seguimiento , Humanos , Persona de Mediana Edad , Portugal/epidemiología , Prevalencia , Pronóstico , Estudios Prospectivos , Tasa de Supervivencia/tendencias
17.
Rev Port Cardiol ; 36(6): 475.e1-475.e3, 2017 Jun.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-28596047

RESUMEN

A 39-year-old woman underwent uneventful percutaneous occlusion of an ostium secundum atrial septal defect (ASD) with a 22 mm Ultrasept ASD Occluder®. Transesophageal echocardiography (TEE) performed two years after implantation revealed a de novo residual left-to-right shunt through the correctly implanted device. Three-dimensional transesophageal echocardiography (3D TEE) further clarified this finding by showing a perforation of the device membrane coating. The patient underwent transcatheter closure of the residual shunt with a 20 mm Ultrasept PFO® device. The procedure was guided by fluoroscopy and real-time 3D TEE. At the end of the procedure 3D TEE documented correct device deployment with complete defect coverage and absence of residual shunt.


Asunto(s)
Falla de Equipo , Defectos del Tabique Interatrial/cirugía , Dispositivo Oclusor Septal , Adulto , Procedimientos Quirúrgicos Cardíacos/métodos , Femenino , Humanos , Inducción de Remisión
18.
Rev Port Cardiol ; 36(3): 169-176, 2017 Mar.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-28238477

RESUMEN

INTRODUCTION: Cardiac rehabilitation (CR) has been demonstrated to improve exercise capacity in acute coronary syndrome (ACS), but not all patients derive the same benefit. Careful patient selection is crucial to maximize resources. OBJECTIVE: To identify in a heterogeneous ACS population which patients would benefit the most with CR, in terms of functional capacity (FC), by using cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis of consecutive ACS patients who underwent CR and CPET was undertaken. CPET was performed at baseline and after 36 sessions of exercise. Peak oxygen uptake (pVO2), percentage of predicted pVO2, minute ventilation/CO2 production (VE/VCO2) slope, VE/VCO2 slope/pVO2 and peak circulatory power (PCP) (pVO2 times peak systolic blood pressure) were assessed in two moments. The differences in pVO2 (ΔpVO2), %pVO2, PCP and exercise test duration were calculated. Patients were classified according to baseline pVO2 (group 1, <20 ml/kg/min vs. group 2, ≥20 ml/kg/min) and left ventricular ejection fraction (group A, <50% vs. group B, ≥50%). RESULTS: We analyzed 129 patients, 86% male, mean age 56.3±9.8 years. Both group 1 (n=31) and group 2 (n=98) showed significant improvement in FC after CR, with a more significant increase in pVO2, in group 1 (ΔpVO2 4.4±7.3 vs. 1.6±5.4; p=0.018). Significant improvement was observed in CPET parameters in group A (n=34) and group B (n=95), particularly in pVO2 and test duration. CONCLUSION: Patients with lower baseline pVO2 (<20 ml/kg/min) presented more significant improvement in FC after CR. CPET which is not routinely used in assessement before CR in context of ACS, could be a valuable tool to identify patients who will benefit the most.


Asunto(s)
Síndrome Coronario Agudo/rehabilitación , Rehabilitación Cardiaca , Síndrome Coronario Agudo/diagnóstico , Síndrome Coronario Agudo/fisiopatología , Prueba de Esfuerzo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Selección de Paciente , Estudios Retrospectivos , Resultado del Tratamiento
19.
Rev Port Cardiol ; 36(2): 77-83, 2017 Feb.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-28153630

RESUMEN

INTRODUCTION: There are barriers to proper implementation of risk stratification scores in patients with acute coronary syndromes (ACS), including their complexity. Our objective was to develop a simple score for risk stratification of all-cause in-hospital mortality in a population of patients with ACS. METHODS: The score was developed from a nationwide ACS registry. The development and internal validation cohorts were obtained from the first 31829 patients, randomly separated (60% and 40%, respectively). The external validation cohort consisted of the last 8586 patients included in the registry. This cohort is significantly different from the other cohorts in terms of baseline characteristics, treatment and mortality. Multivariate logistic regression analysis was used to select four variables with the highest predictive potential. A score was allocated to each parameter based on the regression coefficient of each variable in the logistic regression model: 1 point for systolic blood pressure ≤116 mmHg, Killip class 2 or 3, and ST-segment elevation; 2 points for age ≥72 years; and 3 points for Killip class 4. RESULTS: The new score had good discriminative ability in the development cohort (area under the curve [AUC] 0.796), and it was similar in the internal validation cohort (AUC 0.785, p=0.333). In the external validation cohort, there was also excellent discriminative ability (AUC 0.815), with an adequate fit. CONCLUSIONS: The ProACS risk score enables easy and simple risk stratification of patients with ACS for in-hospital mortality that can be used at the first medical contact, with excellent predictive ability in a contemporary population.


Asunto(s)
Síndrome Coronario Agudo/mortalidad , Anciano , Femenino , Mortalidad Hospitalaria , Humanos , Masculino , Portugal , Sistema de Registros , Medición de Riesgo , Factores de Tiempo
20.
J Heart Valve Dis ; 25(2): 130-138, 2016 03.
Artículo en Inglés | MEDLINE | ID: mdl-27989054

RESUMEN

BACKGROUND: Aortic valve replacement (AVR) is the treatment of choice in patients with severe symptomatic aortic stenosis (AS). Patients with a low left ventricular ejection fraction (LVEF) represent a challenge for evaluation and therapeutic decision. Dobutamine stress echocardiography (DSE) allows the distinction to be made between fixed low-flow/low-gradient (LF/LG) AS and pseudosevere AS. METHODS: Between 2001 and 2014 a retrospective analysis was conducted of patients who underwent DSE to investigate severe AS. DSE was performed in 3- to 5-min steps up to a maximum dose of 20 µg/kg/min. Parameters evaluated at baseline and at each step of DSE included: left ventricular enddiastolic volume (LVEDV) and left ventricular endsystolic volume (LVESV), maximum gradient (MaxG), mean gradient (MG) and aortic valve area (AVA). AS was considered to be severe if the AVA at peak dose was ≤1 cm2. Patients were allocated to two groups according to their therapy: group 1 received only medical treatment, while group 2 underwent AVR. The average follow up was 51.5 ± 4.4 months. RESULTS: A total of 41 patients (28 males, 13 females; mean age 71.7 ± 8.3 years) was analyzed. Severe AS was diagnosed in 34 patients (83%). Baseline echocardiographic characteristics were: AVA 0.7 ± 0.2 cm2, MaxG 42.0 ± 9.1 mmHg, MG 25.6 ± 6.4 mmHg, LVEF 33.1 ± 8.4%, LVEDV 149.6 ± 44.5 ml, and LVESV 104.0 ± 42.6 ml. At peak DSE, AVA was 0.8 ± 0.2 cm2, MaxG 62.7 ± 18.2 mmHg, MG 38.1 ± 11.6 mmHg, LVEF 42.2 ± 9.9%, LVEDV 142.6 ± 43.85 ml, and LVESV 89.7 ± 37.4 ml. Nineteen patients were allocated to group 1, and 22 to group 2. In group 2, two patients underwent transcatheter aortic valve implantation (TAVI) and 20 had surgery. Mortality in group 1 was significantly higher than in group 2 (78.9% versus 27.3%). A Cox proportional hazard model analysis showed that no-intervention was the only predictor of mortality (unadjusted to age; hazard ratio (HR) 5.13, 95% confidence interval (CI) 1.96-13.44, p = 0.001; adjusted to age - HR 4.01, 95% CI 1.46-11.01, p = 0.007). CONCLUSIONS: LF/LG AS has a poor prognosis without intervention. DSE allows the lesion severity to be established. In the present study intervention was a predictor of survival during follow up.


Asunto(s)
Agonistas de Receptores Adrenérgicos beta 1/administración & dosificación , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/diagnóstico por imagen , Dobutamina/administración & dosificación , Ecocardiografía Doppler , Ecocardiografía de Estrés/métodos , Hemodinámica , Anciano , Anciano de 80 o más Años , Válvula Aórtica/efectos de los fármacos , Válvula Aórtica/fisiopatología , Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/mortalidad , Estenosis de la Válvula Aórtica/fisiopatología , Estenosis de la Válvula Aórtica/terapia , Fármacos Cardiovasculares/uso terapéutico , Femenino , Implantación de Prótesis de Válvulas Cardíacas , Hemodinámica/efectos de los fármacos , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Volumen Sistólico , Factores de Tiempo , Reemplazo de la Válvula Aórtica Transcatéter , Resultado del Tratamiento , Función Ventricular Izquierda
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