Mixomas Cardíacos, 13 Anos de Experiência no seu Diagnóstico Ecocardiográfico

INTRODUCTION: Transthoracic echocardiography is the method of choice for the diagnosis of cardiac myxomas, but the transesophageal approach provides a better definition of the location and characteristics of the tumor. The authors review their thirteen years' experience on the echocardiographic diagnosis of this pathology. METHODS: From 1994 to 2007, 41 cardiac tumors were diagnosed in our echocardiographic laboratory, of which 27 (65.85%) were cardiac myxomas. The exams and the patients' clinical files were retrospectively reviewed. RESULTS: Of the 27 patients, 22 (81.5%) were female, with a mean age of 62.1 +/- 13.6 years (25-84 years). The predominant clinical features were due to the obstruction caused by the tumor in more than two thirds of the patients, followed by constitutional symptoms in one third and embolic events in 30%. In the lab results, anemia was found in three patients and elevated sedimentation rate and CRP in two. In two patients the myxoma was found by chance. All the cases were of the sporadic type, although we found a prevalence of thyroid disease of 14% (4 patients). All patients underwent urgent surgical resection except one, in whom surgery was refused due to advanced age and comorbidities. The myxomas followed a typical distribution with 24 (88.8%) located in the left atrium, 18 of them attached to the atrial septum (AS) and two to the mitral valve. In one patient, the tumor involved both atria. The other two cases originated in the right atrium at the AS. Embolic phenomena were more frequent in small tumors (p = 0.027) and in those with a villous appearance (p = 0.032). Obstructive manifestations were associated with larger tumors (p = 0.046) and larger left atria (p = 0.048). In our series, there were no deaths during hospitalization or in the follow-up period of 5.2 +/- 3.7 years in 19 patients. There were two recurrences, both patients being successfully reoperated. CONCLUSION: Myxoma is the most common cardiac tumor. Transesophageal echocardiography provides excellent morphologic definition, aiding in diagnosis and follow-up. Most clinical manifestations are obstructive and are associated with larger tumors. Small tumors with a friable appearance have a higher chance of embolization. Surgical resection is usually curative and the long-term prognosis is excellent.

Resultados da Angioplastia Primária num Centro de Referência. Evolução Intra-Hospitalar

INTRODUCTION: Primary angioplasty is accepted as the preferred treatment for acute myocardial infarction in the first 12 hours. However, outcomes depend to a large extent on the volume of activity and experience of the center. Continuous monitoring of methods and results obtained is therefore crucial to quality control. OBJECTIVE: To describe the demographic, clinical and angiographic characteristics as well as in-hospital outcomes of patients undergoing primary PCI in a high-volume Portuguese center. We also aimed to identify variables associated with in-hospital mortality in this population. METHODS: This was a retrospective registry of consecutive primary PCIs performed at Santa Marta Hospital between January 2001 and August 2007. Demographic, clinical, and angiographic characteristics and in-hospital outcomes were analyzed. Independent predictors of in-hospital mortality were identified by multivariate logistic regression analysis. RESULTS: A total of 1157 patients were identified, mean age 61+/-12 years, 76% male. Mean pain-to-balloon time was 7.6 hours and primary angiographic success was 88%. Overall in-hospital mortality was 6.9%, or 5.5% if patients presenting in cardiogenic shock were excluded from the analysis. Previous history of heart failure, cardiogenic shock on admission, invasive ventilatory support, major hemorrhage, and age over 75 years were found to be associated with increased risk of in-hospital death. Conclusions: In this center primary PCI is effective and safe. Angiographic success rates and in-hospital mortality and morbidity are similar to other international registries. Patients at increased risk for adverse outcome can be identified by simple clinical characteristics such as advanced age, cardiogenic shock on admission, mechanical ventilation and major hemorrhage during hospitalization.

Terapêutica Trombolítica no Enfarte Agudo do Miocárdio: Motivos de Inelegibilidade

STUDY OBJECTIVE: to establish the reasons of ineligibility for thrombolytic therapy (TL) in a group of patients with acute myocardial infarction (AMI). DESIGN: retrospective analysis of protocols and clinical records. SETTING: the medical intensive care unit (ICU) of a tertiary care hospital. PATIENTS AND METHODS: we studied the records from patients with AMI admitted to the ICU during a five-year period (1987-91) and excluded from TL, to determine the cause(s) of ineligibility. RESULTS: we found 1669 patients with AMI, 89 of which were excluded from the study. Of the remaining 1580 patients, 1274 (80.6%) did not receive TL. Mean age was 64.4 years; 66.4% were men. Mortality was 24.6%. Mean duration of chest pain was 19.4 hours. Chief reasons for exclusion from TL were advanced age (43.1% of patients) and delayed presentation (55.7%); one of these was present in 79.2%. CONCLUSIONS: this study confirmed the high mortality of patients with AMI who do not receive TL. Advanced age and delayed presentation were the main causes of ineligibility. As age is being abandoned as an exclusion criterion, efforts for expansion of TL should center on the earlier arrival of patients to centers where it is available.

Symptom Clusters and Survival in Portuguese Patients with Advanced Cancer

This study aimed to identify clusters of symptoms, to determine the patient characteristics associated with identified, and determine their strength of association with survival in patients with advanced cancer (ACPs). Consecutively eligible ACPs not receiving cancer-specific treatment, and referred to a Tertiary Palliative Care Clinic, were enrolled in a prospective cohort study. At first consultation, patients rated 9 symptoms through the Edmonton Symptom Assessment System (0-10 scale) and 10 others using a Likert scale (1-5). Principal component analysis was used in an exploratory factor analysis to identify. Of 318 ACPs, 301 met eligibility criteria with a median (range) age of 69 (37-94) years. Three SCs were identified: neuro-psycho-metabolic (NPM) (tiredness, lack of appetite, lack of well-being, dyspnea, depression, and anxiety); gastrointestinal (nausea, vomiting, constipation, hiccups, and dry mouth) and sleep impairment (insomnia and sleep disturbance). Exploratory factor analysis accounted for 40% of variance of observed variables in all SCs. Shorter survival was observed for patients with the NPM cluster (58 vs. 23, P < 0.001), as well as for patients with two or more SCs (45 vs. 21, P = 0.005). In a multivariable model for survival at 30-days, age (HR: 0.98; 95% CI: 0.97-0.99; P = 0.008), hospitalization at inclusion (HR: 2.27; 95% CI: 1.47-3.51; P < 0.001), poorer performance status (HR: 1.90, 95% CI: 1.24-2.89; P = 0.003), and NPM (HR: 1.64; 95% CI: 1.17-2.31; P = 0.005), were associated with worse survival. Three clinically meaningful SC in patients with advanced cancer were identifiable. The NPM cluster and the presence of two or more SCs, had prognostic value in relation to survival.

Perinatal Death: Epidemiology and Etiology

Perinatal mortality rate is an important mark to evaluate women and perinatal health care. It is of utmost importance to know causes and the evolution of its two components aiming to improve health care in different fields – sanitary conditions, diagnosis and treatment of infectious disease, immunisations, diagnosing and caring for medical diseases induced by pregnancy or directly related to it, providing skilled birth attendance, preventing birth asphyxia, preventing preterm birth complications and infections. In high-income countries the epidemiology varies mainly with social and economic conditions; in low-income countries, paired with poverty, undernutrition, superstition, lack of medical care, deficient basic sanitary conditions are also found. Also, in rich countries, responsible for 1% of deaths, data are published and improvements evaluated, while in low-income countries responsible for 99% of deaths numbers and causes are unknown, making difficult to implement cost effective interventions, a reason why “stillbirth rates in low-income countries are now where they were in high-income countries 50 to 100 years ago”. Knowledge on causes of death are very important as often what is needed are “simple” measures as improvement of sanitary conditions and immunisation programmes rather than high technologies. About four million babies dye each year in the first 28 days of life and another 3 million dye before birth in the third-trimester, with 98% occurring in low-income and middle income countries and more than 1 million occurring during labour and delivery. Classically stillbirths are the major component of perinatal mortality rate. Causes of death are even more difficult to know. In low-income countries a great proportion of women give birth at home. Worldwide the main causes of stillbirth are asphyxia due to obstructed labour, eclampsia, abruption placenta and umbilical cord complications - making valid the assumption that skilled birth attendance would decrease stillbirth; and infection - chorioamnioitis, syphilis and malaria. In high-income countries placental pathology and infection, congenital anomalies, complications of preterm birth and post term delivery, are the most common. If in low-income countries famine and lack of provisions and health care are common, in high-income countries, advanced maternal age and diabetes, obesity, hypertension, smoking, are frequent findings.

Mastocytosis: a Rare Case of Anaphylaxis in Paediatric Age and Literature Review

The term “mastocytosis” denotes a heterogeneous group of disorders characterised by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Symptoms result from MC chemical mediator’s release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem to contribute to an autonomous growth, but somatic c-kit D816V mutation is more frequently encountered, especially in systemic disease. We present a literature review of mastocytosis and a rare case report of an 18 month-old-girl with a bullous dermatosis, respiratory distress and anaphylaxis, as clinical manifestations of mastocytosis. The developments of accepted classification systems and novel useful markers allowed a re-evaluation and updating of the classification of mastocytosis. In paediatric age cutaneous forms of disease prevail and may regress spontaneously. SM is more frequently diagnosed in adults and is a persistent(clonal) disease of bone marrow. The clinical course in these patients is variable.Today diagnostic criteria for each disease variant are reasonably well defined. There are, however, peculiarities, namely in paediatric age, that makes the diagnostic approach difficult. Systemic disease may pose differential diagnostic problems resulting from multiple organ systems involvement. Coversly, the “unexplained” appearance of those symptoms with no skin lesions should raise the suspicion of MC disease. This case is reported in order to stress the clinical severity and difficult diagnostic approach that paediatric mastocytosis may assume.

Ponseti Method: Does Age at the Beginning of Treatment Make a Difference?

The Ponseti method is reportedly effective for treating clubfoot in children up to 9 years of age. However, whether age at the beginning of treatment influences the rate of successful correction and the rate of relapse is unknown. We therefore retrospectively reviewed 68 consecutive children with 102 idiopathic clubfeet treated by the Ponseti technique in four Portuguese hospitals. We followed patients a minimum of 30 months (mean, 41.4 months; range, 30–61 months). The patients were divided into two groups according to their age at the beginning of treatment; Group I was younger than 6 months and Group II was older than 6 months. All feet(100%) were initially corrected and no feet required extensive surgery regardless of age at the beginning of treatment. There were no differences between Groups I and II in the number of casts, tenotomies, success in terms of rate of initial correction, rate of recurrence, and rate of tibialis anterior transference. The rate of the Ponseti method in avoiding extensive surgery was 100% in Groups I and II; relapses occurred in 8% of the feet in younger and older children. Level of Evidence: Level II, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Amniocentesis in HIV Pregnant Women: 16 Years of Experience

The iatrogenic risk of HIV vertical transmission, calculated in initial epidemiologic studies, seemed to counterindicate invasive prenatal diagnosis (PND) procedures. The implementation of highly active antiretroviral therapy (HAART) represented a turning point in PND management, owing to a rapid and effective reduction of maternal viral load (VL). In the present study, we identified cases of vertical transmission in HIV-infected pregnant women who did amniocentesis in the second trimester of pregnancy (n = 27), from 1996 to 2011. We divided our sample into Group A--women under HAART when submitted to amniocentesis (n = 20) and Group B--women without antiretroviral therapy before amniocentesis (n = 7). We had 1 case of vertical transmission in Group B. Preconceptional or early first trimester HIV serology is essential to avoid performing an amniocentesis without antiretroviral therapy or viral suppression. When there is an indication for amniocentesis in an HIV-infected pregnant woman, it should be done if the patient is on HAART and, if possible, when VL is undetectable. Nowadays, with combined first trimester screening test to select pregnancies with high risk of aneuploidies, advanced maternal age is a less frequent indication to perform PND invasive procedures, representing an outstanding gain in prenatal diagnosis of this population.

Locally Advanced and Metastatic Prostate Cancer Treated with Intermittent Androgen Monotherapy or Maximal Androgen Blockade: Results from a Randomised Phase 3 Study by the South European Uroncological Group

BACKGROUND: Few randomised studies have compared antiandrogen intermittent hormonal therapy (IHT) with continuous maximal androgen blockade (MAB) therapy for advanced prostate cancer (PCa). OBJECTIVE: To determine whether overall survival (OS) on IHT (cyproterone acetate; CPA) is noninferior to OS on continuous MAB. DESIGN, SETTING, AND PARTICIPANTS: This phase 3 randomised trial compared IHT and continuous MAB in patients with locally advanced or metastatic PCa. INTERVENTION: During induction, patients received CPA 200 mg/d for 2 wk and then monthly depot injections of a luteinising hormone-releasing hormone (LHRH; triptoreline 11.25 mg) analogue plus CPA 200 mg/d. Patients whose prostate-specific antigen (PSA) was <4 ng/ml after 3 mo of induction treatment were randomised to the IHT arm (stopped treatment and restarted on CPA 300 mg/d monotherapy if PSA rose to ≥20 ng/ml or they were symptomatic) or the continuous arm (CPA 200 mg/d plus monthly LHRH analogue). OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: Primary outcome measurement was OS. Secondary outcomes included cause-specific survival, time to subjective or objective progression, and quality of life. Time off therapy in the intermittent arm was recorded. RESULTS AND LIMITATIONS: We recruited 1045 patients, of which 918 responded to induction therapy and were randomised (462 to IHT and 456 to continuous MAB). OS was similar between groups (p=0.25), and noninferiority of IHT was demonstrated (hazard ratio [HR]: 0.90; 95% confidence interval [CI], 0.76-1.07). There was a trend for an interaction between PSA and treatment (p=0.05), favouring IHT over continuous therapy in patients with PSA ≤1 ng/ml (HR: 0.79; 95% CI, 0.61-1.02). Men treated with IHT reported better sexual function. Among the 462 patients on IHT, 50% and 28% of patients were off therapy for ≥2.5 yr or >5 yr, respectively, after randomisation. The main limitation is that the length of time for the trial to mature means that other therapies are now available. A second limitation is that T3 patients may now profit from watchful waiting instead of androgen-deprivation therapy. CONCLUSIONS: Noninferiority of IHT in terms of survival and its association with better sexual activity than continuous therapy suggest that IHT should be considered for use in routine clinical practice.

Food Allergy and Anaphylaxis in Infants and Preschool-Age Children

Food allergy (FA) prevalence data in infants and preschool-age children are sparse, and proposed risk factors lack confirmation. In this study, 19 children’s day care centers (DCC) from 2 main Portuguese cities were selected after stratification and cluster analysis. An ISAAC’s (International Study of Asthma and Allergies in Childhood) derived health questionnaire was applied to a sample of children attending DCCs. Outcomes were FA parental report and anaphylaxis. Logistic regression was used to explore potential risk factors for reported FA. From the 2228 distributed questionnaires, 1217 were included in the analysis (54.6%). Children’s median age was 3.5 years, and 10.8% were described as ever having had FA. Current FA was reported in 5.7%. Three (0.2%) reports compatible with anaphylaxis were identified. Reported parental history of FA, personal history of atopic dermatitis, and preterm birth increased the odds for reported current FA. A high prevalence of parental-perceived FA in preschool-age children was identified. Risk factor identification may enhance better prevention.

Percutaneous Treatment of Mitral Valve Regurgitation: Initial Experience with the MitraClip Device

INTRODUCTION: Mitral regurgitation (MR) is the most common valvular disease and has recently become the target of a number of percutaneous approaches. The MitraClip is virtually the only device for which there is considerable experience, with more than 20,000 procedures performed worldwide. OBJECTIVE: To describe our initial experience of the percutaneous treatment of MR with the MitraClip device. METHODS: We describe the first six MitraClip cases performed in this institution (mean age 58.5 ± 13.1 years), with functional MR grade 4+ and New York Heart Association (NYHA) heart failure class III or IV (n=3), with a mean follow-up of 290 ± 145 days. RESULTS: Procedural success (MR ≤ 2+) was 100%. Total procedure time was 115.8 ± 23.7 min, with no in-hospital adverse events and discharge between the fourth and eighth day, and consistent improvement in the six-minute walk test (329.8 ± 98.42 vs. 385.33 ± 106.95 m) and in NYHA class (three patients improved by two NYHA classes). During follow-up there were two deaths, in two of the four patients who had been initially considered for heart transplantation. CONCLUSION: In patients with functional MR the MitraClip procedure is safe, with both a high implantation and immediate in-hospital success rate. A longer follow-up suggests that the clinical benefit decreases or disappears completely in patients with more advanced heart disease, namely those denied transplantation or on the heart transplant waiting list.