HER2-Positive Inflammatory Breast Cancer Challenges of Clinical Practices.

HER2-positive inflammatory breast cancer (IBC) is associated with poor overall survival. Targeted therapies have led to improved outcomes. IBC is underrepresented in clinical trials due to its rareness. This case reports a 52-year-old woman diagnosed with IBC of 119x89mm, axillar node-positive, hormone receptor-negative, HER2 positive. The patient underwent neoadjuvant chemotherapy with dual HER2 blockage. Mastectomy histology showed pathological complete response. After two cycles of adjuvant trastuzumab, the patient developed asymptomatic cardiotoxicity leading to the therapeutic suspension. Early recurrence and persisting cardiac alterations prevented treatment with anti-HER2 therapy. At the time of brain recurrence, with cardio-oncology collaboration, it was possible to start TDM-1, with a reduction of 71% of brain lesions size, after two cycles. This case highlights the effectiveness of anti-HER therapy in IBC and the importance of multidisciplinary discussion in treatment choice and toxicity management.

Estratégias de Revascularização em Doentes com Infarto Agudo do Miocárdio em Choque Cardiogênico - Resultados do Registo Português de Síndromes Coronárias Agudas / Revascularization Strategies in Patients with Acute Myocardial Infarction and Cardiogenic Shock: Results from the Portuguese Registry on Acute Coronary Syndromes

Resumo Fundamento: Em doentes com infarto agudo do miocárdio (IAM), choque cardiogênico (CC) e doença multivaso (DMV) persistem dúvidas sobre a intervenção nas artérias não responsáveis. Objetivos: 1) caracterizar a amostra de doentes com IAM, CC e DMV incluídos no Registo Nacional Português de Síndromes Coronárias Agudas (RNSCA); 2) comparar os eventos associados a diferentes estratégias de revascularização; e 3) identificar preditores de mortalidade intra-hospitalar nesta amostra. Métodos: Estudo observacional retrospetivo de doentes com IAM, CC e DMV incluídos no RNSCA entre 2010 e 2018. Compararam-se duas estratégias de revascularização: completa durante o procedimento índice (grupo 1); e completa diferida ou incompleta durante o internamento (grupo 2-3). O endpoint primário foi a ocorrência de reinfarto ou morte intra-hospitalar. A significância estatística foi definida por um valor p < 0,05. Resultados: Identificaram-se 127 doentes com IAM, CC e DMV (18,1% no grupo 1 e 81,9% no grupo 2-3), com idade média de 70 ± 12 anos e 92,9% com IAM com supradesnivelamento do segmento ST. O endpoint primário ocorreu em 47,8% dos doentes do grupo 1 e em 37,5% do grupo 2-3 (p = 0,359). As taxas de mortalidade intra-hospitalar, reinfarto, acidente vascular cerebral e hemorragia major foram também semelhantes nos dois grupos. Os preditores de mortalidade intra-hospitalar nesta amostra foram a presença na admissão de disfunção ventricular esquerda (OR 16,8), bloqueio completo de ramo direito (OR 7,6) e anemia (OR 5,2), (p ≤ 0,02). Conclusões: Entre os doentes com IAM, CC e DMV, incluídos no RNSCA, não se verificou diferença significativa entre revascularização completa no evento índex e completa diferida ou incompleta durante o internamento, relativamente à ocorrência de morte intra-hospitalar ou reinfarto. (Arq Bras Cardiol. 2021; 116(5):867-876)

Abstract Background: In patients with acute myocardial infarction (MI), cardiogenic shock (CS), and multivessel disease (MVD) questions remain unanswered when it comes to intervention on non-culprit arteries. Objective: This article aims to 1) characterize patients with MI, CS and MVD included in the Portuguese Registry on Acute Coronary Syndromes (ProACS); 2) compare different revascularization strategies in the sample; 3) identify predictors of in-hospital mortality among these patients. Methods: Observational retrospective study of patients with MI, CS and MVD included in the ProACS between 2010 and 2018. Two revascularization strategies were compared: complete during the index procedure (group 1); and complete or incomplete during the index hospitalization (groups 2-3). The primary endpoint was a composite of in-hospital death or MI. Statistical significance was defined by a p-value <0.05. Results: We identified 127 patients with MI, CS, and MVD (18.1% in group 1, and 81.9% in groups 2-3), with a mean age of 7012 years, and 92.9% of the sample being diagnosed with ST-segment elevation MI (STEMI). The primary endpoint occurred in 47.8% of the patients in group 1 and 37.5% in group 2-3 (p = 0.359). The rates of in-hospital death, recurrent MI, stroke, and major bleeding were also similar. The predictors of in-hospital death in this sample were the presence of left ventricle systolic dysfunction on admission (OR 16.8), right bundle branch block (OR 7.6), and anemia (OR 5.2) (p ≤ 0.02 for both). Conclusions: Among patients with MI, CS, and MVD included in the ProACS, there was no significant difference between complete and incomplete revascularization during the index hospitalization regarding the occurrence of in-hospital death or MI. (Arq Bras Cardiol. 2021; 116(5):867-876)

Revascularization Strategies in Patients with Acute Myocardial Infarction and Cardiogenic Shock: Results from the Portuguese Registry on Acute Coronary Syndromes. / Estratégias de Revascularização em Doentes com Infarto Agudo do Miocárdio em Choque Cardiogênico ­ Resultados do Registo Português de Síndromes Coronárias Agudas.

BACKGROUND: In patients with acute myocardial infarction (MI), cardiogenic shock (CS), and multivessel disease (MVD) questions remain unanswered when it comes to intervention on non-culprit arteries. OBJECTIVE: This article aims to 1) characterize patients with MI, CS and MVD included in the Portuguese Registry on Acute Coronary Syndromes (ProACS); 2) compare different revascularization strategies in the sample; 3) identify predictors of in-hospital mortality among these patients. METHODS: Observational retrospective study of patients with MI, CS and MVD included in the ProACS between 2010 and 2018. Two revascularization strategies were compared: complete during the index procedure (group 1); and complete or incomplete during the index hospitalization (groups 2-3). The primary endpoint was a composite of in-hospital death or MI. Statistical significance was defined by a p-value <0.05. RESULTS: We identified 127 patients with MI, CS, and MVD (18.1% in group 1, and 81.9% in groups 2-3), with a mean age of 7012 years, and 92.9% of the sample being diagnosed with ST-segment elevation MI (STEMI). The primary endpoint occurred in 47.8% of the patients in group 1 and 37.5% in group 2-3 (p = 0.359). The rates of in-hospital death, recurrent MI, stroke, and major bleeding were also similar. The predictors of in-hospital death in this sample were the presence of left ventricle systolic dysfunction on admission (OR 16.8), right bundle branch block (OR 7.6), and anemia (OR 5.2) (p ≤ 0.02 for both). CONCLUSIONS: Among patients with MI, CS, and MVD included in the ProACS, there was no significant difference between complete and incomplete revascularization during the index hospitalization regarding the occurrence of in-hospital death or MI. (Arq Bras Cardiol. 2021; 116(5):867-876).

FUNDAMENTO: Em doentes com infarto agudo do miocárdio (IAM), choque cardiogênico (CC) e doença multivaso (DMV) persistem dúvidas sobre a intervenção nas artérias não responsáveis. OBJETIVOS: 1) caracterizar a amostra de doentes com IAM, CC e DMV incluídos no Registo Nacional Português de Síndromes Coronárias Agudas (RNSCA); 2) comparar os eventos associados a diferentes estratégias de revascularização; e 3) identificar preditores de mortalidade intra-hospitalar nesta amostra. MÉTODOS: Estudo observacional retrospetivo de doentes com IAM, CC e DMV incluídos no RNSCA entre 2010 e 2018. Compararam-se duas estratégias de revascularização: completa durante o procedimento índice (grupo 1); e completa diferida ou incompleta durante o internamento (grupo 2-3). O endpoint primário foi a ocorrência de reinfarto ou morte intra-hospitalar. A significância estatística foi definida por um valor p < 0,05. RESULTADOS: Identificaram-se 127 doentes com IAM, CC e DMV (18,1% no grupo 1 e 81,9% no grupo 2-3), com idade média de 70 ± 12 anos e 92,9% com IAM com supradesnivelamento do segmento ST. O endpoint primário ocorreu em 47,8% dos doentes do grupo 1 e em 37,5% do grupo 2-3 (p = 0,359). As taxas de mortalidade intra-hospitalar, reinfarto, acidente vascular cerebral e hemorragia major foram também semelhantes nos dois grupos. Os preditores de mortalidade intra-hospitalar nesta amostra foram a presença na admissão de disfunção ventricular esquerda (OR 16,8), bloqueio completo de ramo direito (OR 7,6) e anemia (OR 5,2), (p ≤ 0,02). CONCLUSÕES: Entre os doentes com IAM, CC e DMV, incluídos no RNSCA, não se verificou diferença significativa entre revascularização completa no evento índex e completa diferida ou incompleta durante o internamento, relativamente à ocorrência de morte intra-hospitalar ou reinfarto. (Arq Bras Cardiol. 2021; 116(5):867-876).

Global longitudinal strain as a potential prognostic marker in patients with chronic heart failure and systolic dysfunction.

INTRODUCTION AND OBJECTIVE: The prognostic value of myocardium deformation measurements in chronic heart failure (CHF) is still poorly addressed. The purpose of this study was to evaluate the correlation of left ventricular (LV) global longitudinal strain (GLS) with clinical and prognostic indicators in patients with CHF and systolic dysfunction. METHODS: Ambulatory patients with CHF and LV ejection fraction (LVEF) <45% were studied by two-dimensional and Doppler transthoracic echocardiogram with assessment of GLS. An indication of prognostic status was obtained by the Seattle Heart Failure Model (SHFM) prognostic estimates for life expectancy. RESULTS: We included 54 CHF patients (mean age 55±12 years; 80% male). GLS was significantly correlated with NYHA functional class (r=0.41, p=0.002), BNP levels (r=0.47, p=0.001), LVEF (r=-0.69, p<0.001) and LV end-diastolic pressure, assessed by E/e' ratio (r=0.35, p<0.014) and left atrial maximal volume index (r=0.57, p<0.001). A significant correlation was found between GLS and SHFM prognostic estimates for life expectancy (r=-0.41, p=0.002). The multivariate logistic regression analysis showed that GLS independently predicted an estimated life expectancy <10 years (OR 2.614 [95% CI 1.010-6.763]). The corresponding area under the ROC curve was 0.802 (0.653-0.951) and the best obtained threshold was -9.5 (80% sensitivity, 65% specificity, p=0.003). CONCLUSIONS: GLS was strongly associated with a higher disease severity status and predicted a lower prognostic estimate for life expectancy.

Large right ventricular thrombus.

Right ventricular thrombosis is a rare yet potentially fatal condition. It has been described in association with hypercoagulability states, autoimmune diseases and dilated cardiomyopathy. Echocardiography constitutes the election tool for diagnosis and characterization of these entities, allowing for the differentiation between the various types of thrombi. We present a case of a patient with alcoholic dilated cardiomyopathy admitted for congestive heart failure and lower respiratory infection. In the diagnostic approach, a routine echocardiography revealed a large mural right ventricular thrombus in association with severe biventricular dysfunction. The patient was proposed for anticoagulation strategy, which he refused.

A formação de trombos no ventrículo direito corresponde a uma situação rara porém potencialmente fatal. Tem sido descrita em associação a estados de hipercoagulabilidade, doenças autoimunes e cardiopatia dilatada. A ecocardiografia constitui o método de eleição para o diagnóstico e caracterização destas estruturas, permitindo, inclusive, a diferenciação entre os diversos tipos de trombos possíveis. Apresentamos o caso de um doente com miocardiopatia dilatada de etiologia alcoólica que foi admitido por insuficiência cardíaca congestiva e infeção respiratória baixa. Durante a marcha diagnóstica, o ecocardiograma de rotina revelou a presença de um volumoso trombo mural no ventrículo direito, em associação a disfunção sistólica biventricular de grau severo. Foi proposto o início de hipocoagulação oral, estratégia que o doente recusou.

Conservative management of a prosthetic valve thrombosis--report of a successful case.

Prosthetic valve thrombosis (PVT) refers to the presence of non infective material in valvular apparatus, interfering with its function. It is a potentially fatal complication of valvular replacement surgery. Treatment options include surgery, fibrinolysis and anticoagulation optimisation. The authors present the case of a young man, carrier of an aortic prosthetic mechanical valve, who didn't take his anticoagulant medicine, admitted for an acute obstructive PVT, with evidence of a large thrombotic mass on the aortic valve (> 1cm(2)). The patient refused surgical treatment and eventually presented a complete resolution of the acute PVT with anticoagulation optimisation.

Iron deficiency status irrespective of anemia: a predictor of unfavorable outcome in chronic heart failure patients.

OBJECTIVE: To assess the prognostic significance of iron deficiency (ID) in a chronic heart failure (CHF) outpatient population. METHODS AND RESULTS: We prospectively evaluated 127 patients with stable CHF and left ventricular ejection fraction ≤45%. Clinical and analytical data as well as information regarding the occurrence of the composite endpoint of overall mortality and nonfatal cardiovascular events were assessed. Among the 127 patients enrolled [81% men, median age: 62 years (25th-75th percentile: 53-68)], 46 (36%) patients had ID. Women, patients with higher plasma brain natriuretic peptide levels (>400 pg/ml) and with right ventricular systolic dysfunction presented ID more frequently (p < 0.05 for all). At 225 ± 139 days of follow-up, the composite endpoint occurred in 15 (12%) patients. It was more frequent in ID (24 vs. 5%, p = 0.001) and anemic patients (25 vs. 8%, p = 0.014). In a Cox regression analysis, ID was associated with a higher likelihood of composite endpoint occurrence (HR 5.00, 95% CI 1.59-15.78, p = 0.006). In a multivariable analysis adjusted for clinical variables, including the presence of anemia, ID remained a significant predictor of the composite endpoint (HR 5.38, 95% CI 1.54-18.87, p = 0.009). CONCLUSION: In a CHF outpatient population, ID carried a higher risk of unfavorable outcome, irrespectively of the presence of anemia.

Post-cardiac injury syndrome following transvenous pacing: case report.

Post-cardiac injury syndrome (PCIS) is an inflammatory process involving the pericardium secondary to cardiac injury. It can develop after cardiac trauma, cardiac surgery, myocardial infarction, and, rarely, after certain intravascular procedures. We report a rare case of an iatrogenic cardiac rupture followed by PCIS with delayed inflammatory pericardial effusion after pacemaker implantation. A comprehensive literature review on this topic is provided.

The usefulness of the head-up tilt test in patients with suspected epilepsy.

PURPOSE: It is estimated that approximately 20-30% of patients diagnosed with epilepsy have been misdiagnosed, and neurocardiogenic syncope (NCS) might frequently be the real cause of transient loss of consciousness (TLOC) episodes. We assessed the role of the head-up tilt test (HUTT) in patients previously diagnosed with refractory epilepsy to evaluate the ability of this test to correctly diagnose patients with NCS. METHOD: We retrospectively analysed the clinical records of 107 consecutive patients with a previous diagnosis of refractory epilepsy that were taking antiepileptic drugs and who were referred for HUTT between January 2000 and December 2010. During the subsequent follow-up, we recorded the treatments performed and the recurrence of symptoms. RESULTS: Complete follow-up data were available for 94 (88%) patients, and the mean follow-up period was 80±36 months. The HUTT was positive in 54% of patients. Thirty-one (33%) patients were misdiagnosed with epilepsy, and 20 (21%) patients had a dual diagnosis of NCS and epilepsy. The recurrence of TLOC was reported in 55% of the patients, but it was significantly lower in the misdiagnosed group (42% versus 64%; P=0.039). CONCLUSION: NCS is an important cause of epilepsy misdiagnosis. The HUTT is often critical for making an accurate diagnosis and subsequently selecting the appropriate treatment for patients presenting with TLOC. The diagnostic overlap between epilepsy and NCS is not uncommon, suggesting that electroencephalographic monitoring during a HUTT may play an important role in diagnosing patients with recurrent, undiagnosed TLOC episodes.

Spirito-Maron echocardiographic score: a marker for morphological and physiological assessment of patients with hypertrophic cardiomyopathy.

AIMS: The heterogeneous distribution of hypertrophy in hypertrophic cardiomyopathy (HCM) limits the echocardiographic conventional measurements accuracy in the evaluation of left ventricular hypertrophy (LVH). The aim of this study was to assess the correlation of the echocardiographic Spirito-Maron score (SMS) with left ventricle (LV) mass quantification by cardiac magnetic resonance (CMR) and with LV diastolic function. METHODS AND RESULTS: Left ventricle diastolic function parameters, SMS, LV mass (American Society of Echocardiography formula), and maximal wall thickness (MWT) were evaluated by two-dimensional (2D) transthoracic echocardiography. The SMS was obtained by adding the MWT of 4 LV segments, at the mitral valve or papillary muscles short-axis views. Echocardiographic parameters of LVH, including SMS, were correlated with LV mass obtained by CMR and with E/e' ratio. We included 45 patients (60% male, mean age 48 ± 18 years), who underwent 2D echocardiography. Twenty-two of them performed a CMR study. A positive correlation was found between SMS and CMR LV mass (r = 0.80; P < 0.001), whereas MWT (r = 0.62; P = 0.002) and the 2D LV mass (r = 0.60; P = 0.011) presented a lower correlation with CMR LV mass. The SMS was significantly correlated with E/e' ratio (r = 0.60; P = 0.007), whereas a nonsignificant correlation was found with MWT (r = 0.41; P = 0.081) and 2D LV mass (r = 0.22; P = 0.400). CONCLUSION: Spirito-Maron score presents a highly positive correlation with CMR LV mass and with diastolic dysfunction severity in HCM patients. SMS is a reliable quantitative LVH measurement method and seems to provide more comprehensive morphological and physiological information than 2D echocardiographic conventional parameters used to estimate LVH.

Acute coronary syndrome in a patient with multifocal coronary vasospasm.

Vasospastic angina results from temporary spasm of one or more coronary segments. Although prognosis of patients presenting with coronary vasospasm appears to be generally good, multivessel coronary vasospasm may increase the risk of life-threatening cardiac events. We present a case of a 51-year-old man admitted to the emergency room due to severe retrosternal pain, who was documented with multifocal coronary vasospasm. .

Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities.

Sub-infundibular stenosis is a rare form of congenital heart disease, difficult to precisely diagnose, especially in adult patients. We report a case of an adult patient, referred to our hospital with long-standing dyspnea on exertion. She was finally diagnosed with sub-infundibular stenosis. During cardiovascular magnetic resonance imaging, right-sided aortic arch and left pulmonary artery agenesis were also identified. .