Acute coronay syndrome in a patient with severe hemophilia A: Dificult decisions.

Hemophilia A is an inherited coagulation disease characterized by factor VIII (FVIII) deficiency and is associated with high hemorrhagic risk, especially in its severe forms. As the average life expectancy of patients with hemophilia has increased, so has the prevalence of acute coronary events. There is however limited experience in dealing with them. The strategy of acting on acute coronary events in patients with hemophilia, as demonstrated in the present case, is a real challenge, not only due to the need for antiplatelet therapy (which is essential in the prevention of stent thrombosis, but increases hemorrhagic risk), but also due to the lack of specific recommendations related to the most adequate and safe replacement therapy in these situations. The authors describe the case of a 48-year-old man with unstable angina and a previous diagnosis of severe hemophilia A who underwent percutaneous coronary intervention under FVIII therapy without hemorrhagic complications.

Heart and brain interactions in heart failure: Cognition, depression, anxiety, and related outcomes.

BACKGROUND: Cognitive impairment, anxiety and depression are common in heart failure (HF) patients and its evolution is not fully understood. OBJECTIVES: To assess the cognitive status of HF patients over time, its relation to anxiety and depression, and its prognostic impact. METHODS: Prospective, longitudinal, single center study including patients enrolled in a structured program for follow-up after hospital admission for HF decompensation. Cognitive function, anxiety/depression state, HF-related quality of life (QoL) were assessed before discharge and during follow-up (between 6th and 12th month) using Montreal Cognitive Assessment (MoCA), Hospital Anxiety and Depression Scale (HADS) and Kansas City Cardiomyopathy Questionnaire, respectively. HF related outcomes were all cause readmissions, HF readmissions and the composite endpoint of all-cause readmissions or death. RESULTS: 43 patients included (67±11.3 years, 69% male); followed-up for 8.2±2.1 months. 25.6% had an abnormal MoCA score that remained stable during follow-up (22.6±4.2 vs. 22.2±5.5; p=NS). MoCA score <22 at discharge conferred a sixfold greater risk of HF readmission [HR=6.42 (1.26-32.61); p=0.025], also predicting all-cause readmissions [HR=4.00 (1.15-13.95); p=0.03] and death or all-cause readmissions [HR=4.63 (1.37-15.67); p=0.014]. Patients with higher MoCA score showed a greater ability to deal with their disease (p=0.038). At discharge, 14% and 18.6% had an abnormal HADS score for depression and anxiety, respectively, which remained stable during follow-up and was not related to MoCA. CONCLUSIONS: Cognitive function, anxiety and depressive status remain stable in HF patients despite optimized HF therapy. Cognitive status shall be routinely screened to adopt attitudes that improve management as it has an impact on HF-related QoL and prognosis.

Non-invasive telemonitoring improves outcomes in heart failure with reduced ejection fraction: a study in high-risk patients.

AIMS: Non-invasive telemonitoring (TM) in patients with heart failure (HF) and reduced left ventricular ejection fraction (HFrEF) may be useful in the early diagnosis of HF decompensation, allowing therapeutic optimization and avoiding re-hospitalization. We describe a TM programme in this population and evaluate its effectiveness during a 12 month period. METHODS AND RESULTS: We conducted a single-centre study of patients discharged from hospital after decompensated HF, allocated into three groups: prospective TM programme, prospective HF protocol follow-up programme (PFP) with no TM facilities, and retrospective propensity-matched usual care (UC). TM effectiveness was assessed by all-cause hospitalizations and mortality; HF-related hospitalization (HFH), days lost to unplanned hospital admissions/death, functional capacity and quality of life (New York Heart Association, Kansas City Cardiomyopathy Questionnaire, 6 min walk test, and plasma N-terminal pro-brain natriuretic peptide) were also evaluated. A total of 125 patients were included [65.9 ± 11.9 years, 32% female, left ventricular ejection fraction 27% (21-32)]. TM was similar to PFP regarding effectiveness; TM reduced all-cause hospitalization and mortality (HR 0.27; 95% CI 0.11-0.71; P < 0.01) and HFH (HR 0.29; 95% CI 0.10-0.89; P < 0.05) as compared with UC. TM reduced the average number of days lost due to unplanned hospital admissions or all-cause death as compared with PFP (5.6 vs. 12.4 days, P < 0.05) and UC (5.6 vs. 48.8 days, P < 0.01). Impact on quality of life was similar between TM and PFP (P = 0.36). CONCLUSIONS: In patients with HFrEF and recent HF hospitalization, non-invasive TM reduced 12 month all-cause hospitalization/mortality and HFH as compared with usual care. TM also reduced the number of days lost due to unplanned hospital admission/death as compared with either an optimized protocol-based follow-up programme or usual care.

Protocol-based follow-up program for heart failure patients: Impact on prognosis and quality of life.

INTRODUCTION: Heart failure is associated with high rates of readmission and mortality, and there is a need for measures to improve outcomes. This study aims to assess the impact of the implementation of a protocol-based follow-up program for heart failure patients on readmission and mortality rates and quality of life. METHODS: A quasi-experimental study was performed, with a prospective registry of 50 consecutive patients discharged after hospitalization for acute heart failure. The study group was followed by a cardiologist at days 7-10 and the first, third, sixth and 12th month after discharge, with predefined procedures. The control group consisted of patients hospitalized for heart failure prior to implementation of the program and followed on a routine basis. RESULTS: No significant differences were observed between the two groups regarding mean age (67.1±11.2 vs. 65.8±13.4 years, p=0.5), NYHA functional class (p=0.37), or median left ventricular ejection fraction (27% [19.8-35.3] vs. 29% [23.5-40]; p=0.23) at discharge. Mean follow-up after discharge was similar (11±5.3 vs. 10.9±5.5 months, p=0.81). The protocol-based follow-up program was associated with a significant reduction in all-cause readmission (26% vs. 60%, p=0.003), heart failure readmission (16% vs. 36%, p=0.032), and mortality (4% vs. 20%, p=0.044). In the study group there was a significant improvement in all quality of life measures (p<0.001). CONCLUSION: A protocol-based follow-up program for patients with heart failure led to a significant reduction in readmission and mortality rates, and was associated with better quality of life.

Profiles of hospitalized patients with valvular heart disease: Experience of a tertiary center.

INTRODUCTION: Valvular heart disease (VHD) is increasing worldwide, mostly because of aging. Percutaneous valve intervention is the preferred therapeutic option in high-risk patients. OBJECTIVE: To characterize the profiles of patients with VHD admitted to the cardiology ward at a tertiary referral center. METHODS: On the basis of ICD-9 codes for VHD, the discharge notes of 287 patients hospitalized over a 22-month period were reviewed and analyzed. One hundred characteristics were considered. RESULTS: Median age was 74 (23-93) years, and 145 (51%) were male. The admissions were elective (for valve intervention) in 36%. Heart failure (HF) was the reason for urgent admissions in 29.3%. Multiple comorbidities were observed in 53% of patients. Etiology of VHD was degenerative in 68%, functional in 15.3% and rheumatic (predominantly in women and younger patients) in 8.7%. Aortic valve disease was present in 63% (aortic stenosis in 56%), and was associated with HF (p=0.004), atrial fibrillation (AF) (p=0.01), and left ventricular (LV) dilatation (p=0.003) or hypertrophy (p<0.001). Mitral valve disease (51%), mostly mitral regurgitation (degenerative or functional), predominated in women, and was associated with HF, AF, LV dilatation (p<0.001) and reduced LV ejection fraction (p=0.003). Significant tricuspid regurgitation (34.8%) associated with the presence of previously implanted cardiac devices (p<0.001). Valve intervention (mostly transcatheter aortic valve implantation) was performed in 41% of patients. Mean length of hospital stay was 12±14.3 days and overall in-hospital mortality was 9.8%. CONCLUSIONS: Nowadays, the profiles of hospitalized patients with VHD are dominated by the elderly, with degenerative disease and multiple comorbidities, presenting with HF, AF and LV remodeling, who frequently undergo valve intervention, usually via a percutaneous approach. Mortality remains significant in this high-risk population.

Dangerous mistake: an accidental caffeine overdose.

Caffeine (1,3,7-trimethylxanthine) is a natural product commonly presented in food's composition, beverages and medicinal products. Generally, it is thought to be safe under normal dosage, yet it can be fatal in case of severe intoxication. We report a case of a healthy 32-year-old woman who went to the local emergency department (ED) 30 min after ingesting, accidentally, 5000 mg of anhydrous caffeine for a preworkout supplement. At the ED, she presented an episode of presyncope followed by agitation. ECG showed polymorphic broad complex QRS tachycardia and arterial blood gas revealed metabolic acidaemia with severe hypokalemia. The dysrhythmia was successfully treated with intravenous propranolol. Acid-base and hydroelectrolytic disorders were also corrected. A persistent sinus tachycardia was observed in the first 2 days in the ward and 5 days later she was discharged asymptomatic with internal medicine follow-up.

Complex phenotype linked to a mutation in exon 11 of the lamin A/C gene: Hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes.

The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Herein we describe a new phenotype associated with a mutation in exon 11 of the LMNA gene: hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes. A 64-year-old woman with hypertrophic cardiomyopathy and a point mutation in exon 11 of the LMNA gene (c.1718C>T, Ser573Leu) presented with severe symptomatic ventricular hypertrophy and left ventricular outflow tract obstruction. She underwent septal alcohol ablation, followed by Morrow myectomy. The patient was also diagnosed with severe dyslipidemia, diabetes and obesity, and fulfilled diagnostic criteria for metabolic syndrome. No other characteristics of LMNA mutation-related phenotypes were identified. The development of type III atrioventricular block with no apparent cause, and mildly depressed systolic function, prompted referral for cardiac resynchronization therapy. In conclusion, the association between LMNA mutations and different phenotypes is complex and not fully understood, and can present with a broad spectrum of severity.

Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report.

Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels. Resumo A hipertrigliceridemia grave tem sido associada de forma consistente ao aumento do risco cardiovascular e a outras complicações, nomeadamente, pancreatite aguda. Descrevemos um caso de uma mulher de 64 anos, com miocardiopatia hipertrófica e síndrome metabólica com valor sérico de triglicerídeos de 3260 mg/dL. Foi efectuada plasmaferese e optimizado o tratamento médico para alcançar uma redução rápida e efectiva dos níveis dos triglicerídeos, prevenindo complicações clínicas. Após três sessões de plasmaferese, verificou-se uma redução marcada dos triglicerídeos e do colesterol total. Existem alguns casos descritos na literatura demonstrado a importância da plasmaferese no tratamento da pancreatite aguda em contexto de hipertrigliceridemia grave. Os autores pretendem com este caso demonstrar a aplicabilidade desta técnica em contexto de prevenção primária em doentes com níveis de triglicerídeos extremamente aumentados.

Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report / Hipertrigliceridemia: há um papel para a aferese profilática? Relato de um caso

Abstract Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels.

Resumo A hipertrigliceridemia grave tem sido associada de forma consistente ao aumento do risco cardiovascular e a outras complicações, nomeadamente, pancreatite aguda. Descrevemos um caso de uma mulher de 64 anos, com miocardiopatia hipertrófica e síndrome metabólica com valor sérico de triglicerídeos de 3260 mg/dL. Foi efectuada plasmaferese e optimizado o tratamento médico para alcançar uma redução rápida e efectiva dos níveis dos triglicerídeos, prevenindo complicações clínicas. Após três sessões de plasmaferese, verificou-se uma redução marcada dos triglicerídeos e do colesterol total. Existem alguns casos descritos na literatura demonstrado a importância da plasmaferese no tratamento da pancreatite aguda em contexto de hipertrigliceridemia grave. Os autores pretendem com este caso demonstrar a aplicabilidade desta técnica em contexto de prevenção primária em doentes com níveis de triglicerídeos extremamente aumentados.

Severe rheumatic mitral stenosis: a 21st century medusa.

Although the prevalence of rheumatic fever has greatly decreased in developed countries, rheumatic mitral stenosis still causes significant morbidity and mortality. Symptomatic patients have a poor prognosis, with a 0 to 15% 10-year survival rate, particularly if percutaneous or surgical intervention are contraindicated or considered high risk. We present a case of severe rheumatic mitral stenosis with an evolution over 4 decades, in which exceptional venous distention has established.

Contrast-induced nephropathy following percutaneous coronary intervention: case report.

Angiography and percutaneous coronary and other vascular interventions are crucial for the diagnosis and treatment of vascular lesions, with a positive impact on patients' quality of life and prognosis. However, the use of intravascular contrast agents is the third leading cause of acute renal failure, accounting for considerable morbidity and mortality. The authors describe the case of a diabetic patient with no significant prior renal disease who developed severe oliguric acute renal failure requiring dialysis after the use of contrast for a percutaneous coronary intervention. The impact, risk factors and prophylaxis of contrast-induced nephropathy are reviewed.

Heart rate and blood pressure in mitral valve prolapse patients: divergent effects of long-term propranolol therapy and correlations with catecholamines.

OBJECTIVE: There is a well known association between mitral valve prolapse (MVP) and low blood pressure (BP), although patients often have high levels of catecholamines and high heart rate (HR). The main objective of our study was to evaluate the effects of long-term adrenergic beta-blockade on these parameters. METHODS: The study population consisted of 46 patients with MVP and the control group consisted of 20 normal individuals. The study had two phases: in the first phase, patients were free of medications. In the second phase, patients were under treatment with propranolol for 10 to 12 months. The tests were performed in normal individuals and patients in the first phase. Only patients underwent the same tests in the second phase. Measurement of urinary epinephrine and norepinephrine levels, by high performance liquid chromatography, was done. Rest HR was determined by electrocardiogram (ECG), and ambulatory blood pressure and HR were evaluated by 24 hours ambulatory blood pressure monitoring (ABPM) using the auscultatory method. RESULTS: The levels of epinephrine and norepinephrine were significantly higher in patients than in normal controls and decreased under propranolol. Rest and ambulatory HR were higher in patients and decreased under propranolol. The 24 hours systolic and diastolic BPs were lower in patients, and their values increased under propranolol. Heart rate decreasing and epinephrine levels reduction were positively correlated. No correlation was found between BP increase and catecholamine levels. CONCLUSION: The study results show divergent effects of propranolol on blood pressure, which increased, and on heart rate, that decreased, in patients with MVP. Heart rate decrease was an expected result and depends, namely, on b1 receptors blockade. Increase in BP is an unusual response to adrenergic beta-blockade in normal conditions, and this finding supports the preponderance of b2 receptors on the BP control in patients with MVP.

A ecografia no diagnóstico das lesões músculo-tendinosas do ombro / Ultrasonography findings of musculotendinous lesions of the shoulder

Este estudo propôs-se a revisar os aspectos relevantes da anatomia, técnica de exame e achados de imagens, com o objetivo de auxiliar o radiologista no diagnóstico diferencial frente às afecções músculo-tendinosas do ombro. Foram realizadas 34 ultra-sonografias da região do ombro, no período de janeiro a dezembro de 2001. A avaliação foi realizada com transdutores de alta freqüência (7,5-10 MHz). O exame foi normal em 23 por cento dos pacientes (oito casos). Dos 26 casos alterados, observaram-se oito com ruptura total (30 por cento), cinco com ruptura parcial (19 por cento), dez com tendinopatia (38 por cento), um com tendinopatia calcificada (3 por cento), um com tenossinovite bicipital (3 por cento) e um com tendinopatia associada a artrite gotosa (3 por cento). A falta de experiência e de conhecimento das principais alterações que acometem o ombro é um dos principais pontos limitantes do diagnóstico ultra-sonográfico das lesões do ombro. Portanto, o estudo dessas alterações deve ser realizado amplamente.

The purpose of this study is to review the anatomical structures, ultrasonography examination technique and imaging findings in patients with musculotendinous lesions of the shoulder with the aim of helping the radiologist in the differential diagnosis of these lesions. We performed ultrasonography examinations of the shoulder using high-resolution probes (7.5–10 MHz) in 34 patients from January to December, 2001. The examination was normal in eight patients (23%) whereas 26 patients presented abnormalities: eight patients had total rotator cuff tears (30%), five had partial rotator cuff (19%), ten had tendinopathy (38%), one had calcified tendinopathy (3%), one had biceps tendinitis, one had biceps tendon luxation (3%) and one had tendinopathy associated to crystal arthropathy (3%). The lack of experience and the insufficient knowledge about the main diseases of the shoulder are limiting factors for the diagnosis of these lesions. Therefore, a comprehensive study of the abnormalities of the shoulder should be carried out.

Dilated cardiomyopathy due to endocrine dysfunction.

Dilated cardiomyopathy can be idiopathic or be caused by many potentially treatable conditions. We report a complex case of peripartum heart failure associated with hypothyroidism and hypoparathyroidism. Myocardial biopsy suggested that hypothyroidism was the main cause for the dilated pattern, but hypocalcemia played a critical role in acute decompensation of heart failure during hospitalization. After a long and clinically complicated hospital stay, correction of hypothyroidism and hypocalcemia resulted in near-normal cardiac function.

MR imaging of hepatic metastases.

MR imaging is an excellent diagnostic tool for the detection and characterization of liver metastases. The most characteristic finding is peripheral ring enhancement on immediate postgadolinium images.

Post-treatment malignant liver lesions. MR imaging.

MR imaging is very accurate in the diagnosis and staging of tumors and in surgical planning. MR imaging is also an excellent method for evaluation of the liver after surgical resection, systemic or local tumor therapies, and liver transplantation. It permits early recognition of complications and the presence of recurrent tumor, providing an opportunity to repeat treatment or use alternative treatment. Surgical resection remains the standard therapy for treating liver metastases. The relatively small number of patients who are candidates for curative resection have provided impetus for the implementation and improvement of other techniques. The variety of techniques and the sensitivity for contrast enhancement have made MR imaging an ideal method to follow the response of tumors to various treatment approaches. The appearance of tumor recurrence and the response to treatment are relatively consistently shown on MR images; however, the time course of change in lesion appearance has not been fully elucidated, particularly in the setting of chemotherapy. Evaluating the response to chemotherapy is rendered complex because of the longer duration of the therapy, the types of response that various chemotherapeutic agents engender, the method of action of this therapy and the time of imaging in relation to therapy. The various local therapies share some general principles of action, and many have similar MR imaging findings. Some local therapies are effective only with certain malignancies (e.g., alcohol therapy and HCC), whereas other therapies are more limited because of the size of the tumor kill zone (e.g., interstitial laser therapy). We are in the early stages of using MR imaging to guide local therapies and to monitor response during treatment in real time. This appears to be an important future direction for MR imaging. The role of MR imaging in liver transplantation involves pre- and postoperative investigation of both donors (in the case of living-related transplantation) and recipients. These issues are described further in the section on MR imaging of liver transplantation.

Magnetic resonance imaging of pulmonary parenchymal disease using a modified breath-hold 3D gradient-echo technique: initial observations.

PURPOSE: To determine the potential of a modified breath-hold 3D gradient-echo technique for visualizing pulmonary parenchymal diseases. MATERIALS AND METHODS: Twenty-one magnetic resonance imaging (MRI) studies of the lungs were performed in 20 patients (15 male and 5 female; age range, 7.7-86.1 years) with pulmonary diseases comprising non-small-cell, small-cell, and bronchioalveolar carcinomas, endobronchial mucoepidermoid carcinoma, metastases, pneumonia, Wegener's granulomatosis, chronic obstructive pulmonary disease, arterio-venous malformation, and bronchogenic cyst. Confirmation of diagnoses was obtained via histopathology (14 patients) and follow-up (6 patients). MRI studies were performed at 1.5 T before and after administration of gadolinium using a modified volumetric interpolated breath-hold examination (VIBE) sequence. All images were evaluated prospectively regarding lesion detection and characterization. MR findings were correlated with final diagnoses. Retrospective grading (scores, 1-4) was performed for general image quality (rated "poor" to "excellent"); presence of artifacts ("negligible" to "severe"); and imaging quality of pulmonary lesions (conspicuity and contrast on pre- and postgadolinium images). RESULTS: Twenty-three solid pulmonary lesions, 25 infiltrates and segmental atelectases, and 1 cyst were detected and prospectively correctly diagnosed. Sizes ranged from 0.3-10 cm. The mean scores for image quality and presence of artifacts were 3.3 (SD, 0.7) and 1.8 (SD, 0.7), respectively. Conspicuity and contrast of pulmonary lesions received mean scores between 3.0 and 3.8 (SD, 0.4-0.7). CONCLUSION: This MRI technique allows imaging of a wide spectrum of solid and nonsolid pulmonary parenchymal diseases with reproducible high image quality, effective suppression of artifacts, high resolution, and visualization of gadolinium enhancement.