RESUMO
Marfan syndrome (MFS) is an autosomal dominant condition that is caused by abnormal synthesis of connective tissue. The syndrome classically affects the ocular, musculoskeletal, and cardiovascular systems. The most common cardiovascular manifestations include mitral valve prolapse/regurgitation and aortic aneurysms at high risk of rupture and dissection. However, internal mammary artery (IMA) true aneurysms are rarely reported. In this case report, we describe a 43-year-old male patient with MFS and three previous thoracotomies referred for endovascular repair of bilateral IMA true aneurysms. To the best of our knowledge, there are no cases of endovascular treatment of bilateral IMA true aneurysms reported in the literature.
RESUMO
Kaposi sarcoma (KS) is a low-grade angioproliferative tumor associated with infection with human herpes virus 8 (HHV-8). The disease was named after Moritz Kaposi, a Hungarian dermatologist who first described it in 1872 as 'idiopathic multiple pigmented sarcoma of the skin.' HHV-8 infection is required for the development of KS, but not all infected persons develop the disease. KS is also considered an acquired immune deficiency syndrome (AIDS)-defining illness by the Centers for Disease Control and Prevention guidelines. According to data from the United States AIDS and cancer registries, both KS and non-Hodgkin lymphoma are the most common malignancies associated with human immunodeficiency virus (HIV) infection. However, the incidence of both malignancies has decreased dramatically since 1996 following the widespread utilization of highly active antiretroviral therapies. HIV-associated KS can involve virtually any site in the body including lymph nodes, gastrointestinal tract, respiratory system, heart, pericardium, bone marrow, and other visceral organs. However, cutaneous disease is the most common and is the usual initial presentation for KS. KS-related pericardial effusion can be a life-threatening emergency and should be considered in HIV/AIDS patients who present with signs and symptoms of pericardial effusion. The importance of diagnosing and differentiating KS-related pericardial effusion from other causes of pericardial effusion lies in the differences in the treatment and management in comparison to other etiologies of pericardial effusion. We report a case of a 54-year old man who presented to our hospital with a large pericardial effusion and was subsequently diagnosed to have HIV-related KS pericardial effusion. A brief review of the literature on the diagnosis and management is also presented.
RESUMO
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a genetic disease with an autosomal-dominant inheritance for which negative inotropes are the most widely used initial therapies. Observational studies and small randomised trials have suggested symptomatic and functional benefits using pacing and several theories have been put forward to explain why. Pacing, although not the primary treatment for HCM, could be beneficial to patients with relative or absolute contraindications to surgery or alcohol ablation. Several randomised controlled trials comparing pacing to other therapeutic modalities have been conducted but no Cochrane-style systematic review has been done. OBJECTIVES: To assess the effects of pacing in drug-refractory or drug-intolerant hypertrophic cardiomyopathy patients. SEARCH METHODS: We searched the following on the 14/4/2010: CENTRAL (The Cochrane Library 2010, Issue 1), MEDLINE OVID (from 1950 onwards ), EMBASE OVID (from 1980 onwards ), Web of Science with Conference Proceedings (from 1970 onwards). No language restrictions were applied. SELECTION CRITERIA: Randomised controlled trials of either parallel or crossover design that assess the beneficial and harmful effects of pacing for hypertrophic cardiomyopathy were included. When crossover studies were identified, we considered data only from the first phase. DATA COLLECTION AND ANALYSIS: Data from included studies were extracted onto a pre-formed data extraction paper by two authors independently. Data was then entered into Review Manager 5.1 for analysis. Risk of bias was assessed using the guidance provided in the Cochrane Handbook. For dichotomous data, relative risk was calculated; and for continuous data, the mean differences were calculated. Where appropriate data were available, meta-analysis was performed. Where meta-analysis was not possible, a narrative synthesis was written. A QUROUM flow chart was provided to show the flow of papers. MAIN RESULTS: Five studies (reported in 10 papers) were identified. However, three of the five studies provided un-usable data. Thus the data from only two studies (reported in seven papers) with 105 participants were included for this review. There was insufficient data to compare results on all-cause mortality, cost effectiveness, exercise capacity, Quality of life and Peak O2 consumption.When comparing active pacing versus placebo pacing on exercise capacity, one study showed that exercise time decreased from (13.1 ± 4.4) minutes to (12.6 ± 4.3) minutes in the placebo group and increased from (12.1 ± 5.6) minutes to (12.9 ± 4.2) minutes in the treatment group (MD 0.30; 95% CI -1.54 to 2.14). Statistically significant data from the same study showed that left ventricular outflow tract obstruction decreased from (71 ± 32) mm Hg to (52 ± 34) mm Hg in the placebo group and from (70 ± 24) mm Hg to (33 ± 27) mm Hg in the active pacing group (MD -19.00; 95% CI -32.29 to -5.71). This study was also able to show that New York Heart Association (NYHA) functional class decreased from (2.5 ± 0.5) to (2.2 ± 0.6) in the inactive pacing group and decreased from (2.6 ± 0.5) to (1.7 ± 0.7) in the placebo group (MD -0.50; 95% CI -0.78 to -0.22).When comparing active pacing versus trancoronary ablation of septal hypertrophy (TASH), data from one study showed that NYHA functional class decreased from (3.2 ± 0.7) to (1.5 ± 0.5) in the TASH group and decreased from (3.0 ± 0.1) to (1.9 ± 0.6) in the pacemaker group. This study also showed that LV wall thickness remained unchanged in the active pacing group compared to reduction from (22 ± 4) mm to (17 ± 3) mm in the TASH group (MD 0.60; 95% CI -5.65 to 6.85) and that LV outflow tract obstruction decreased from (80 ± 35.5) mm Hg in the TASH group to (49.3 ± 37.7) mm Hg in the pacemaker group. AUTHORS' CONCLUSIONS: Trials published to date lack information on clinically relevant end-points. Existing data is derived from small trials at high risk of bias, which concentrate on physiological measures. Their results are inconclusive. Further large and high quality trials with more appropriate outcomes are warranted.
