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BACKGROUND: ISCHEMIA (International Study of Comparative Health Effectiveness With Medical and Invasive Approaches) did not find an overall reduction in cardiovascular events with an initial invasive versus conservative management strategy in chronic coronary disease; however, there were conservative strategy participants who underwent invasive coronary angiography early postrandomization (within 6 months). Identifying factors associated with angiography in conservative strategy participants will inform clinical decision-making in patients with chronic coronary disease. METHODS: Factors independently associated with angiography performed within 6 months of randomization were identified using Fine and Gray proportional subdistribution hazard models, including demographics, region of randomization, medical history, risk factor control, symptoms, ischemia severity, coronary anatomy based on protocol-mandated coronary computed tomography angiography, and medication use. RESULTS: Among 2591 conservative strategy participants, angiography within 6 months of randomization occurred in 8.7% (4.7% for a suspected primary end point event, 1.6% for persistent symptoms, and 2.6% due to protocol nonadherence) and was associated with the following baseline characteristics: enrollment in Europe versus Asia (hazard ratio [HR], 1.81 [95% CI, 1.14-2.86]), daily and weekly versus no angina (HR, 5.97 [95% CI, 2.78-12.86] and 2.63 [95% CI, 1.51-4.58], respectively), poor to fair versus good to excellent health status (HR, 2.02 [95% CI, 1.23-3.32]) assessed with Seattle Angina Questionnaire, and new/more frequent angina prerandomization (HR, 1.80 [95% CI, 1.34-2.40]). Baseline low-density lipoprotein cholesterol <70 mg/dL was associated with a lower risk of angiography (HR, 0.65 [95% CI, 0.46-0.91) but not baseline ischemia severity nor the presence of multivessel or proximal left anterior descending artery stenosis >70% on coronary computed tomography angiography. CONCLUSIONS: Among ISCHEMIA participants randomized to the conservative strategy, angiography within 6 months of randomization was performed in <10% of patients. It was associated with frequent or increasing baseline angina and poor quality of life but not with objective markers of disease severity. Well-controlled baseline low-density lipoprotein cholesterol was associated with a reduced likelihood of angiography. These findings point to the importance of a comprehensive assessment of symptoms and a review of guideline-directed medical therapy goals when deciding the initial treatment strategy for chronic coronary disease. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01471522.
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Angiografia por Tomografia Computadorizada , Tratamento Conservador , Angiografia Coronária , Doença da Artéria Coronariana , Valor Preditivo dos Testes , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Fatores de Tempo , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/terapia , Tratamento Conservador/efeitos adversos , Resultado do Tratamento , Fatores de Risco , Medição de Risco , Doença CrônicaRESUMO
New or preexisting atrial fibrillation (AF) is frequent in patients undergoing aortic valve replacement. We evaluated whether the presence of AF during transcatheter aortic valve implantation (TAVI) or surgical aortic valve replacement (SAVR) impacts the length of stay, healthcare adjusted costs, and inpatient mortality. The median length of stay in the patients with AF increased by 33.3% as compared with those without AF undergoing TAVI and SAVR (5 [3 to 8] days vs 3 [2 to 6] days, p <0.0001 and 8 [6 to 12] days vs 6 [5 to 10] days, p <0.0001, respectively). AF increased the median value of adjusted healthcare associated costs of both TAVI ($46,754 [36,613 to 59,442] vs $49,960 [38,932 to 64,201], p <0.0001) and SAVR ($40,948 [31,762 to 55,854] vs $45,683 [35,154 to 63,026], p <0.0001). The presence of AF did not independently increase the in-hospital mortality. In conclusion, in patients undergoing SAVR or TAVI, AF significantly increased the length of stay and adjusted healthcare adjusted costs but did not independently increase the in-hospital mortality.
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Estenose da Valva Aórtica/cirurgia , Fibrilação Atrial/epidemiologia , Custos de Cuidados de Saúde/estatística & dados numéricos , Mortalidade Hospitalar , Tempo de Internação/estatística & dados numéricos , Substituição da Valva Aórtica Transcateter , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/epidemiologia , Comorbidade , Feminino , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Fatores de Risco , Resultado do TratamentoRESUMO
Intramyocardial dissection is a rare complication of myocardial infarction, trauma, and percutaneous intervention. It is usually caused by hemorrhagic dissection among the spiral myocardial fibers. We hereby report the case of a patient with left ventricular intramyocardial dissection who presented with acute decompensated heart failure. (Level of Difficulty: Advanced.).
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Importance: Traditional time-to-event analyses rate events occurring early as more important than later events, even if later events are more severe, eg, death. Days alive out of hospital (DAOH) adds a patient-focused perspective beyond trial end points. Objective: To compare DAOH between invasive management and conservative management, including invasive protocol-assigned stays, in the International Study of Comparative Health Effectiveness with Medical and Invasive Approaches (ISCHEMIA) randomized clinical trial. Design, Setting, and Participants: In this prespecified analysis of the ISCHEMIA trial, DAOH was compared between 5179 patients with stable coronary disease and moderate or severe ischemia randomized to invasive management or conservative management. Participants were recruited from 320 sites in 37 countries. Stays included overnight stays in hospital or extended care facility (skilled nursing facility, rehabilitation, or nursing home). DAOH was separately analyzed excluding invasive protocol-assigned procedures. Data were collected from July 2012 to June 2019, and data were analyzed from July 2020 to April 2021. Interventions: Invasive management with angiography and revascularization if feasible or conservative management, with both groups receiving optimal medical therapy. Main Outcomes and Measures: The hypothesis was formulated before data lock in July 2020. The primary end point was mean DAOH per patient between randomization and 4 years. Initial stays for invasive protocol-assigned procedures were prespecified to be excluded. Results: Of 5179 included patients, 1168 (22.6%) were female, and the median (interquartile range) age was 64 (58-70) years. The average DAOH was higher in the conservative management group compared with the invasive management group at 1 month (30.8 vs 28.4 days; P < .001), 1 year (362.2 vs 355.9 days; P < .001), and 2 years (718.4 vs 712.1 days; P = .001). At 4 years, the 2 groups' DAOH were not significantly different (1415.0 vs 1412.2 days; P = .65). In the invasive management group, 2434 of 4002 stays (60.8%) were for protocol-assigned procedures. There were no clear differences at any time point in DAOH when protocol-assigned procedures were excluded from the invasive management group. There were more hospital and extended care stays in the invasive management vs conservative management group during follow-up (4002 vs 1897; P < .001). Excluding protocol-assigned procedures, there were fewer stays in the invasive vs conservative group (1568 vs 1897; P = .001). Cardiovascular stays following the initial assigned procedures were lower in the invasive management group (685 of 4002 [17.1%] vs 1095 of 1897 [57.8%]; P < .001) due to decreased spontaneous myocardial infarction stays (65 [1.6%] vs 123 [6.5%]; P < .001) and unstable angina stays (119 [3.0%] vs 216 [11.4%]; P < .001). Conclusions and Relevance: DAOH was higher for patients in the conservative management group in the first 2 years but not different at 4 years. DAOH was decreased early in the invasive management group due to protocol-assigned procedures. Hospital stays for myocardial infarction and unstable angina during follow-up were lower in the invasive management group. DAOH provides a patient-focused metric that can be used by clinicians and patients in shared decision-making for management of stable coronary artery disease. Trial Registration: ClinicalTrials.gov Identifier: NCT01471522.
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Tratamento Conservador/métodos , Doença da Artéria Coronariana/terapia , Gerenciamento Clínico , Isquemia Miocárdica/terapia , Revascularização Miocárdica/métodos , Pacientes Ambulatoriais/estatística & dados numéricos , Idoso , Angiografia Coronária , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/mortalidade , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Tempo de Internação/tendências , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/diagnóstico , Isquemia Miocárdica/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Fatores de Tempo , Estados Unidos/epidemiologiaRESUMO
Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic heart muscle disease characterized by hypertrophy with preserved or increased ejection fraction in the absence of secondary causes. However, recent studies have demonstrated that a substantial proportion of individuals with HCM also have comorbid diabetes mellitus (~10%). Whether genetic variants may contribute a combined phenotype of HCM and diabetes mellitus is not known. Here, using next-generation sequencing methods, we identified novel and ultrarare variants in adiponectin receptor 1 (ADIPOR1) as risk factors for HCM. Biochemical studies showed that ADIPOR1 variants dysregulate glucose and lipid metabolism and cause cardiac hypertrophy through the p38/mammalian target of rapamycin and/or extracellular signal-regulated kinase pathways. A transgenic mouse model expressing an ADIPOR1 variant displayed cardiomyopathy that recapitulated the cellular findings, and these features were rescued by rapamycin. Our results provide the first evidence that ADIPOR1 variants can cause HCM and provide new insights into ADIPOR1 regulation.
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OBJECTIVES: This study aimed to examine the concordance of coronary computed tomographic angiography (CCTA) assessment of coronary anatomy and invasive coronary angiography (ICA) as the reference standard in patients enrolled in the ISCHEMIA (International Study of Comparative Health Effectiveness with Medical and Invasive Approaches). BACKGROUND: Performance of CCTA compared with ICA has not been assessed in patients with very high burdens of stress-induced ischemia and a high likelihood of anatomically significant coronary artery disease (CAD). A blinded CCTA was performed after enrollment to exclude patients with left main (LM) disease or no obstructive CAD before randomization to an initial conservative or invasive strategy, the latter guided by ICA and optimal revascularization. METHODS: Rates of concordance were calculated on a per-patient basis in patients randomized to the invasive strategy. Anatomic significance was defined as ≥50% diameter stenosis (DS) for both modalities. Sensitivity analyses using a threshold of ≥70% DS for CCTA or considering only CCTA images of good-to-excellent quality were performed. RESULTS: In 1,728 patients identified by CCTA as having no LM disease ≥50% and at least single-vessel CAD, ICA confirmed 97.1% without LM disease ≥50%, 92.2% with at least single-vessel CAD and no LM disease ≥50%, and only 4.9% without anatomically significant CAD. Results using a ≥70% DS threshold or only CCTA of good-to-excellent quality showed similar overall performance. CONCLUSIONS: CCTA before randomization in ISCHEMIA demonstrated high concordance with subsequent ICA for identification of patients with angiographically significant disease without LM disease.
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Angiografia , Angiografia por Tomografia Computadorizada , Humanos , Isquemia , Valor Preditivo dos TestesRESUMO
Aim: To study sex-related differences in acute coronary syndrome (ACS) presentation, management and in-hospital outcomes. Materials & methods: We studied 621 ACS patients (150 women, 471 men) of low socio-economic status from South India from February 2015 to January 2016. Multivariable logistic regression methods were used to assess sex differences in the in-hospital outcomes. Adjudicated major adverse cardiovascular events (MACE) included in-hospital cardiac arrest, cardiogenic shock, heart failure, re-infarction, stroke, major bleeding and mortality. Results & conclusion: Mean age in women was 60.97 ± 11.23 years versus 54.5 ± 10.87 years in men (p < 0.001). Women had higher prevalence of hypertension and diabetes and presented with more non-ST elevation ACS. There were no differences in the use of antiplatelets, statins and other pharmacotherapy except for the higher use of nitrates in women. There were no differences in MACE rates between women and men (15.3 vs 9.6%; adjusted odds ratio: 1.43; CI: 0.76-2.69).
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Síndrome Coronariana Aguda , Síndrome Coronariana Aguda/epidemiologia , Idoso , Feminino , Humanos , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Caracteres Sexuais , Fatores Sexuais , Fatores Socioeconômicos , Resultado do TratamentoRESUMO
AIMS: To study the incidence, clinical and angiographic characteristics, management and outcomes of coronary artery perforation (CAP) during percutaneous coronary intervention (PCI) at a high volume center in South-east Asia. METHODS: Data from patients who had CAP during PCI from January 2016 to December 2019 at our center were collected. Clinical features, angiographic and procedural characteristics, their management and outcomes were analyzed retrospectively. RESULTS: A total of 40,696 patients underwent PCI during the study period and the incidence of CAP was 0.13% (n = 51). Mean age was 60.0 ± 10.8 years and 69% were males. CAP cases involved complex type B2/C lesions in 73%, calcified lesions in 58%, and chronic total occlusions in 25%. Majority of patients presented as acute coronary syndrome (65%) and STEMI was the most frequent indication for PCI (33%). Most of the CAPs were Ellis type II (33%) and III (55%). CAP most frequently occurred during post dilation (n = 20) and wire manipulation (n = 17). Majority were treated by prolonged balloon inflation (53%) and covered stents (33%). Pericardiocentesis was required in 19 patients to alleviate tamponade. In one patient coil embolisation was done and two patients required bail-out emergency cardiac surgery. Periprocedural myocardial infarction occurred in 6% and in-hospital mortality was 10%. All-cause mortality accrued to 14% at 30 days and 16% at 6 months. CONCLUSION: Although incidence of CAP in contemporary interventional practice remains low, the morbidity and mortality are considerable. Early recognition and management strategies tailored to the severity of perforation play a key role in achieving better outcomes.
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Angiografia Coronária/métodos , Doença da Artéria Coronariana/cirurgia , Vasos Coronários/lesões , Hospitais com Alto Volume de Atendimentos/estatística & dados numéricos , Complicações Intraoperatórias , Intervenção Coronária Percutânea/efeitos adversos , Lesões do Sistema Vascular/epidemiologia , Doença da Artéria Coronariana/diagnóstico , Vasos Coronários/diagnóstico por imagem , Feminino , Seguimentos , Mortalidade Hospitalar , Humanos , Incidência , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Lesões do Sistema Vascular/diagnóstico , Lesões do Sistema Vascular/etiologiaRESUMO
AIMS: The aim of this study was to demonstrate the safety and efficacy of the next-generation balloon-expandable Myval transcatheter heart valve (THV) in an intermediate- or high-risk patient population with severe symptomatic native aortic stenosis. METHODS AND RESULTS: MyVal-1 was a first-in-human, prospective, multicentre, single-arm, open-label study. Between June 2017 and February 2018, a total of 30 patients were enrolled at 14 sites across India. Mean age was 75.5±6.7 years; 43.3% had coronary artery disease. The mean Society of Thoracic Surgeons score was 6.4±1.8% and 100% of the patients were in New York Heart Association (NYHA) functional Class II/III/IV pre-procedure. The six-minute walk test and Kansas City Cardiomyopathy Questionnaire (KCCQ) scores were recorded. After successful implantation of the Myval THV, 96.6% and 100% were in NYHA functional Class I/II at 30-day and 12-month follow-up, respectively. Outcomes of the six-minute walk test (148.0±87.4 vs 336.0±202.9 m) and KCCQ score (36.6±11.0 vs 65.9±11.4) improved from baseline to 12-month follow-up. The effective orifice area (0.6±0.2 vs 1.8±0.3 cm2, p<0.0001), mean aortic valve gradient (47.4±8.8 vs 12.0±3.3 mmHg, p<0.0001), peak aortic valve gradient (71.7±13.0 vs 20.3±5.9 mmHg, p<0.0001) and transaortic velocity (4.5±0.4 vs 2.2±0.4 m/s, p<0.0001) improved substantially from baseline to 12 months post procedure. Four all-cause mortality cases were reported up to 12 months. Moreover, there was no other moderate/severe paravalvular leak, aortic regurgitation or need for new permanent pacemaker (PPM) up to 12-month follow-up. CONCLUSIONS: The MyVal-1 study demonstrated the primary safety and efficacy of the Myval THV with no new PPM requirement up to 12-month follow-up. However, future trials with a larger number of patients and long-term follow-up are warranted to establish the safety and efficacy of the device.
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Estenose da Valva Aórtica/cirurgia , Próteses Valvulares Cardíacas , Substituição da Valva Aórtica Transcateter/efeitos adversos , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/cirurgia , Humanos , Índia , Estudos Prospectivos , Desenho de Prótese , Resultado do TratamentoRESUMO
AIMS: Although the proof of concept of the bioresorbable vascular scaffold (BRS) is well documented, device-related adverse outcomes with first-generation BRS indicate longer-term surveillance. The current study provides insights into the safety and performance of the MeRes100, a novel second-generation sirolimus-eluting BRS, beyond one-year up to three-year follow-up (FU). METHODS AND RESULTS: A total of 108 enrolled patients with de novo coronary artery lesions who underwent implantation of MeRes100 as part of the first-in-human MeRes-1 trial were followed up clinically beyond one year at two and three years and with multiple modality imaging at six months and two years. At three-year FU, the cumulative major adverse cardiac events rate was 1.87%, in the form of two ischaemia-driven target lesion revascularisations. No scaffold thrombosis was reported. Between six months and two years at quantitative coronary angiography, in-segment late lumen loss (LLL) (0.15±0.22 mm vs. 0.23±0.32 mm; p=0.18) and in-scaffold LLL (0.13±0.22 mm vs. 0.24±0.34 mm; p=0.10) changed insignificantly. IVUS subset analysis revealed a non-significant reduction in mean lumen area (6.17±1.28 mm2 vs. 5.47±1.50 mm2; p=0.21) and minimum lumen area (5.14±1.19 mm2 vs. 4.05±1.42 mm2; p=0.10) at two years compared to post-procedural measurements. OCT subset analysis demonstrated 99.24±2.27% neointimal strut coverage. CONCLUSIONS: The extended outcomes of the MeRes-1 trial demonstrated sustained efficacy and safety of the MeRes100 BRS with maintained lumen patency up to two years by multimodality imaging and no very late scaffold thrombosis up to three-year clinical FU.The MeRes-1 trial is registered at the Clinical Trials Registry-India. CTRI Number: CTRI/2015/04/005706.
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Implantes Absorvíveis , Fármacos Cardiovasculares/administração & dosagem , Doença da Artéria Coronariana/terapia , Vasos Coronários/efeitos dos fármacos , Stents Farmacológicos , Everolimo/administração & dosagem , Intervenção Coronária Percutânea/instrumentação , Sirolimo/administração & dosagem , Fármacos Cardiovasculares/efeitos adversos , Angiografia Coronária , Doença da Artéria Coronariana/diagnóstico por imagem , Vasos Coronários/diagnóstico por imagem , Everolimo/efeitos adversos , Seguimentos , Humanos , Índia , Intervenção Coronária Percutânea/efeitos adversos , Sirolimo/efeitos adversos , Resultado do TratamentoRESUMO
AIMS: The MeRes-1 trial sought to study the safety and effectiveness of a novel sirolimus-eluting bioresorbable vascular scaffold (MeRes100 BRS) in treating de novo native coronary artery lesions by clinical evaluation and using multiple imaging modalities. METHODS AND RESULTS: The MeRes-1 first-in-human trial was a single-arm, prospective, multicentre study, which enrolled 108 patients with de novo coronary artery lesions (116 scaffolds were deployed to treat 116 lesions in 108 patients). At six months, quantitative coronary angiography revealed in-scaffold late lumen loss of 0.15±0.23 mm with 0% binary restenosis. Optical coherence tomography demonstrated minimum scaffold area (6.86±1.73 mm2) and percentage neointimal strut coverage (99.30%). Quantitative intravascular ultrasound analysis confirmed a 0.14±0.16 mm2 neointimal hyperplasia area. At one year, major adverse cardiac events, a composite of cardiac death, any myocardial infarction and ischaemia-driven target lesion revascularisation, occurred in only one patient (0.93%) and there was no scaffold thrombosis reported. At one year, computed tomography angiography demonstrated that all scaffolds were patent and in-scaffold mean percentage area stenosis was 11.33±26.57%. CONCLUSIONS: The MeRes-1 trial demonstrated the safety and effectiveness of MeRes100 BRS. The favourable clinical outcomes and effective vascular responses have provided the basis for further studies in a larger patient population. The MeRes-1 trial is registered at the Clinical Trials Registry-India.
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Implantes Absorvíveis , Fármacos Cardiovasculares/uso terapêutico , Infarto do Miocárdio/tratamento farmacológico , Poliésteres/uso terapêutico , Sirolimo/uso terapêutico , Idoso , Vasos Coronários/diagnóstico por imagem , Everolimo/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Intervenção Coronária Percutânea/métodos , Estudos Prospectivos , Tomografia de Coerência Óptica/métodos , Resultado do TratamentoRESUMO
BACKGROUND: Congenital left ventricle to right atrial communications (Gerbode defects) are extremely rare (0.08%) type of ventricular septal defects. They were traditionally closed by surgery in the past. There are few case reports and small series of acquired and congenital Gerbode defects, closed with various types of devices. Aim of our study is to assess the feasibility, efficacy, and complications of transcatheter closure of congenital Gerbode defects with Amplatzer duct occluder II (ADO II). MATERIAL: Twelve consecutive cases of Gerbode defects, age ranging from 10 months to 16 years (mean 6.7 years), weight ranging from 6.5 kg to 34 kg (mean 19.3 kg), were diagnosed on transthoracic echocardiography. RESULTS: Transcatheter closure of Gerbode defects was done successfully through retrograde approach with ADO II. No aortic or tricuspid regurgitation or residual shunt occurred in any of the patients. One patient developed transient complete heart block needing temporary pacing. DISCUSSION: The soft low profile, easily trackable ADO II appears to be ideal for closure of Gerbode defects, as the central cylinder fits in the defect and the soft retention discs on either side, without polyester material, do not impinge on either aortic, mitral, or tricuspid valve. We report the successful transcatheter closure of twelve cases of congenital Gerbode defects with ADO II. CONCLUSIONS: Transcatheter closure of congenital Gerbode defects with ADO II is safe, effective, and an attractive alternative to surgical closure. ADO II appears to be tailor made for Gerbode defects, as the success rate is very high and complication rate is very low.
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Cateterismo Cardíaco/métodos , Átrios do Coração/anormalidades , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/terapia , Ventrículos do Coração/anormalidades , Dispositivo para Oclusão Septal , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estudos de Coortes , Ecocardiografia , Ecocardiografia Doppler em Cores/métodos , Feminino , Seguimentos , Átrios do Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Masculino , Segurança do Paciente , Desenho de Prótese , Estudos Retrospectivos , Medição de Risco , Resultado do TratamentoRESUMO
Left ventricular (LV) thrombus formation is a frequent complication in patients with acute anterior myocardial infarction (MI). Its incidence is lower with inferior wall MI. Risk factors for the development of LV thrombus are consistently irrespective of infarct treatment and include large infarct size, severe apical akinesia or dyskinesia LV aneurysm, and anterior MI.
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Viper venom toxicities comprise mainly bleeding disorders and nephrotoxicity. Cardiotoxicity is a rare manifestation of viper bite. We describe the case of a previously healthy 35-year-old man who developed coagulopathy and sinus node dysfunction following a viper bite. Electrocardiography showed sinus arrest and junctional escape rhythm. This is the first account of sinus node dysfunction caused by a viper bite.
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Síndrome do Nó Sinusal/etiologia , Mordeduras de Serpentes/complicações , Venenos de Víboras , Viperidae , Adulto , Animais , Antivenenos/uso terapêutico , Eletrocardiografia , Parada Cardíaca/diagnóstico , Parada Cardíaca/etiologia , Hemodinâmica , Humanos , Masculino , Síndrome do Nó Sinusal/diagnóstico , Síndrome do Nó Sinusal/tratamento farmacológico , Síndrome do Nó Sinusal/fisiopatologia , Mordeduras de Serpentes/diagnóstico , Fatores de Tempo , Resultado do TratamentoRESUMO
Coarctoplasty with stenting is often an effective strategy in cases of recoarctation following surgical repair. The potential benefit of coarctoplasty in a patient with Eisenmenger's syndrome is unknown. We describe the case of a 21-year-old male who presented with claudication of lower limbs. He was known to have congenital heart disease, consisting of ventricular septal defect, patent ductus arteriosus (PDA), and coarctation of the aorta. Coarctation repair and PDA ligation had been done at two months of age. At the time of presentation for evaluation of claudication, echocardiography revealed severe coarctation and evidence of Eisenmenger's syndrome. This patient subsequently underwent balloon angioplasty and stenting of coarctation without any increase in cyanosis.
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Coartação Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Complexo de Eisenmenger/diagnóstico , Comunicação Interventricular/diagnóstico , Stents , Coartação Aórtica/diagnóstico , Ecocardiografia , Comunicação Interventricular/cirurgia , Humanos , Masculino , Tomografia Computadorizada Multidetectores , Adulto JovemRESUMO
A 4-month-old male infant presented with recurrent cough for 2 months. He had a shortened right upper limb with absent right thumb and continuous murmur in the left parasternal area. The X-ray showed an absent radius and the first metacarpal and phalyngeal bones on the right side. Echocardiogram revealed aortopulmonary window and small secundum atrial septal defect. Aortopulmonary window was successfully treated by device closure. Holt-Oram syndrome with aortopulmonary window is an extremely rare association.
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Anormalidades Múltiplas/diagnóstico , Defeito do Septo Aortopulmonar/diagnóstico , Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Comunicação Interatrial/diagnóstico , Deformidades Congênitas das Extremidades Inferiores/diagnóstico , Deformidades Congênitas das Extremidades Superiores/diagnóstico , Defeito do Septo Aortopulmonar/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Diagnóstico Diferencial , Eletrocardiografia , Humanos , Lactente , Masculino , Radiografia TorácicaRESUMO
A 21-year-old pregnant female of known rheumatic heart disease presented to us for evaluation of central cyanosis during her late pregnancy. Though she was investigated for any associated congenital heart disease or pulmonary arteriovenous fistula, but incidentally she was diagnosed of having acquired methemoglobinemia. Her serum methemoglobin level was 33% which was far above the normal range. Ultimately, she was managed conservatively and delivered through elective caesarean section. Though the delivered baby was lethargic after birth, but later he was improved and discharged. This was the first case reported so far that a pregnant patient of rheumatic heart disease developed cyanosis due to methemoglobinemia.