Luis Otávio Cardoso Mocarzel

Cirrhotic Cardiomyopathy: Another Case of a Successful Approach to Treatment of Hepatorenal Syndrome

Hepatorenal syndrome (HRS) is defined as a failure of renal function, potentially reversible, in patients with liver cirrhosis and ascites. Recently, a component of cardiomyopathy associated with HRS was described, but the use of positive inotropic medicine as part of the treatment of the acute phase has not been extensively evaluated. We report a second case in our hospital of a patient with HRS type I without previous heart disease, with secondary hemodynamic decompensation due to liver disease, in which the abnormalities in systolic function by speckle-tracking echocardiography were observed and could be reversed by the use of inotropes. After partial response to current therapies, the patient presented a clinical and laboratorial response with improvement of renal function after infusion of dobutamine. Clinical studies are needed for the therapy approach to HRS taking into account myocardial dysfunction as a major contributing factor for renal dysfunction.

Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

Cirrhotic Cardiomyopathy: A New Clinical Phenotype

Hepatic cirrhosis is the final spectrum of several aggressions to the liver, with great relevance to public healthcare. National data estimate a prevalence of 0.14% to 0.35%, mortality of 3 to 35 per 100,000 individuals and an annual average of 30,000 hospital admissions in Brazil.1,2 With the ageing population, the prevalence of chronic liver diseases, in particular steatohepatitis associated to obesity and metabolic syndrome, results in an increase in the number of hepatic cirrhosis cases.3

A Rare Case of Ascites due to Peritoneal Amyloidosis

Patient: Male, 65 Final Diagnosis: Peritoneal amyloidosis Symptoms: Anasarca • Dyspnea • Orthopnea Medication: — Clinical Procedure: Paracentesis and peritoneal biopsy Specialty: Gastroenterology and Hepatology Objective: Unusual clinical course Background: The clinical manifestations of amyloidosis depend on the type of insoluble protein as well as the location of amyloid deposits in tissues or organs. In the gastrointestinal tract, the small intestine is the most common site of amyloid deposits, whereas peritoneal involvement and ascites are rare. Case Report: We report on a case of ascites due to peritoneal amyloidosis. A 65-year-old patient was admitted to our institution due to anasarca and pulmonary congestion, mimicking heart failure. We started the patient on diuretics and vasodilators. Despite improvement in pulmonary congestion and peripheral edema, his ascites was not reduced. Echocardiogram revealed restrictive cardiomyopathy and a speckle-tracking pattern suggestive of cardiac amyloidosis. Subcutaneous and peritoneal biopsies revealed amyloidosis. Conclusions: Amyloidosis is rare in the peritoneum and is usually asymptomatic. Ascites occurs in only 20% of patients with peritoneal amyloidosis. We searched PubMed using “ascites” and “amyloidosis” and identified only eight case reports of amyloidosis with ascites. Physicians should be particularly careful in heart failure and anasarca cases when ascites is disproportional or not responsive to diuretic treatment. To date, there is no specific treatment for peritoneal amyloidosis.

Systemic lupus erythematosus: review of cardiovascular aspects

Systemic lupus erythematosus (SLE) is an autoimmune condition with a complex pathophysiological process in which its inflammatory activity is an enhancer of coronary disease by systemic inflammation, endothelial dysfunction and predisposition to thrombosis. The cardiovascular involvement in SLE is not a diagnostic criterion and is considered only as damage established in the long-term of the disease. The objective of this study is to highlight the importance of clinical vision for the early identification of cardiovascular involvement in SLE. A critical analysis of the cardiac approach in SLE, with emphasis on clinical aspects, cardiovascular biomarkers and genetics and rational request of additional tests. The particularity of patients with lupus nephritis and antiphospholipid antibody syndrome is also highlighted. The perception of subclinical cardiac damage is critical for interrupting the cycle of myocardial injury and to avoid progression of heart disease.

Cirrhotic Patients with Child-Pugh C Have Longer QT Intervals

Fundamento e objetivos: A cardiomiopatia cirrotica tem sido usada para descrever a disfuncao cardiaca cronica em pacientes cirroticos sem doenca cardiaca estrutural previa. Alem disso, o prolongamento do intervalo QT e uma das alteracoes cardiacas mais importantes relacionadas a cirrose. Estudos previos sugerem que o prolongamento QT esta associado com uma taxa de mortalidade mais alta em pacientes cirroticos. O objetivo deste estudo foi analisar intervalos QTs segundo a gravidade da cirrose, medida pela classificacao Child-Plugh. Materiais e metodos: Em um estudo transversal, um total de 67 pacientes com cirrose nao alcoolica submeteu-se a avaliacao clinica e eletrocardiografica. A gravidade da cirrose foi classificada de acordo com o escore Child-Pugh. O intervalo QT foi medido por um eletrocardiograma de 12 derivacoes. Resultados: Os intervalos QTs foram mais longos em pacientes no grupo Child-Plugh C que nos grupos Child-Pugh A e B (459 ± 33 vs 436 ± 25 e 428 ± 34 ms, respectivamente, p = 0,004). Houve uma correlacao positiva entre o intervalo QT e o escore Child-Pugh em individuos com escore Child-Pugh ≥ 7 (r = 0,50; p < 0,05) e intervalos QT ≥ 440 ms (r = 0,46, p < 0,05). Conclusao: O presente estudo mostrou que pacientes com cirrose Child-Plugh C apresentam intervalos QTs mais longos, o que reforcou a relacao entre a gravidade da cirrose e achados eletrocardiograficos da cardiomiopatia cirrotica. Alem disso, esse resultado foi encontrado em pacientes sem sintomas cardiacos, o que destacou a importância de um metodo simples e nao invasivo, como o eletrocardiograma, para identificar pacientes cirroticos com cardiomiopatia. (Int J Cardiovasc Sci. 2017; [online].ahead print, PP.0-0)

Cirrhotic cardiomyopathy: we already know. What comes next?

Cirrhotic Cardiomyopathy is a recently recognized condition consisting of myocardial dysfunction manifested in cirrhotic patients in the absence of previous cardiac disease. The underlying pathogenetic mechanisms includes a “miocardiotoxic” environment that leads to a blunted heart response to stress, whether physical or pharmacological. This condition is now better understood and can be diagnosed supported clinical, by laboratory electrocardiography and echocardiography criteria. Clinically, systolic incompetence is most evident when cirrhotic patients are placed under stress, whether physical or pharmacological. Despite of being well understood, there is a lack of knowledge to a proper practical application. Recent case reports have suggested that CCM must be treated early because it may be an triggering factor for cirrhosis complications. Studies are needed to identify potential treatments that alter the natural history of heart disease in cirrhotic patients, especially in the phases in which the patients are asymptomatic. Correspondence to: Pedro Gemal Lanzieri, Department of Clinical Medicine, Antonio Pedro University Hospital, Fluminense Federal University, Rio de Janeiro, Brazil, Tel: +55-21-26299210; Email: pedrogemal@id.uff.br

Posterior Cord Syndrome and Trace Elements Deficiency as an Uncommon Presentation of Common Variable Immunodeficiency

Diarrhea is one of the most common symptoms in common variable immunodeficiency, but neurologic manifestations are rare. We presented a 50-year-old woman with recurrent diarrhea and severe weight loss that developed a posterior cord syndrome. Endoscopy found a duodenal villous blunting, intraepithelial lymphocytosis, and lack of plasma cells and magnetic resonance imaging of the spine was normal. Laboratory assays confirmed common variable immunodeficiency syndrome and showed low levels of trace elements (copper and zinc). Treatment was initiated with parenteral replacement of trace elements and intravenous human immunoglobulin and the patient improved clinically. In conclusion, physicians must be aware that gastrointestinal and neurologic disorders may be related to each other and remember to request trace elements laboratory assessment.

Recombinant Human Erythropoietin in Hemolytic Anemia in a Patient With Mechanical Heart Valve

The history of valve replacement surgery began more than a century ago, when Doyen, in 1913, performed the first surgical repair in a stenotic pulmonary valve. Despite all the implemented technology and efforts in recent years, there is no ideal valve replacement to date.1 The incidence of severe complications in patients with prosthetic heart valves is approximately 3% a year.2 The main complications include embolism, bleeding, mechanical valve thrombosis, fibrous tissue growth, paravalvular regurgitation, structural deterioration, infective endocarditis, prosthesis-patient mismatch and hemolytic anemia. Specifically, regarding hematological complications, the occurrence of hemolysis after valve replacement ranges from 5 to 15% of the patients3 and can lead to reoperation in up to 19% of cases.4 The main mechanism of hemolysis after valve replacement is mechanical, non-immune and the standard therapy in these cases is of support by prescribing oral iron and folate, as well as blood transfusions. In some patients, recurrent hemolysis refractory to supportive treatment may be an indication for valve replacement.3 However, with the aging of population, the number of patients at high risk for valve replacement surgery has increased. Thus, it is important to develop new palliative therapies for these individuals.5 Erythropoietin (EPO) is a hormone produced by the kidney that stimulates red-blood cell production in the bone marrow. It is classically indicated for the treatment of anemia secondary to chronic kidney disease. There are, in the literature, some reports of EPO being used for the treatment of hemolysis caused by heart valve prosthesis, and it is especially useful in patients in whom valve replacement surgery would be contraindicated, or for those that require multiple transfusions.5 In this article, we report a case of a patient receiving EPO for the treatment of hemolytic anemia secondary to the presence of mechanical prostheses in the aortic and mitral positions, with mitral paravalvular regurgitation, in whom heart surgery was contraindicated due to advanced heart failure.

Pulmonary Disease Secondary to Reflux Mimicking Interstitial Pneumonia in Systemic Sclerosis: Case Report and Literature Review

Systemic sclerosis is a complex disease due to the variety of clinical presentations, often superimposed on other conditions, related or not to the connective tissue. We report a 43-year-old Brazilian woman with limited systemic sclerosis and pulmonary symptoms secondary to gastroesophageal reflux disease, with a clinical presentation similar to a diffuse interstitial lung disease. Because of the frequency of interstitial lung injury due to systemic sclerosis, this was an important differential diagnosis, which could be excluded after optimized treatment of reflux disease, with clinical and radiological improvement. Clinical management of patients with collagen diseases requires clinician skills to identify the natural history and understand its nuances. This is a common situation in clinical practice, but with a few discussions in international literature.