Evaluation of the clinical features of an outpatient cohort with Marfan syndrome.

Marfan syndrome is a connective tissue disease with autosomal dominant inheritance and variable clinical presentation. The main clinical manifestations recognition could contribute to early diagnosis and cardiovascular complication prevention. We aimed to evaluate the clinical profile of a Marfan syndrome outpatient cohort. METHODS: Retrospective cross-sectional study was carried out with outpatients over 12 years of age whose electronic medical records contained the clinical information and complementary exams necessary for study inclusion. Data were analyzed using descriptive statistics and comparisons were performed using student's t-test and chi-square or Fisher's exact test. P-values<0.05 were considered statistically significant. RESULTS: 75 patients (29.5 ± 13.4 years) were included and 43(57 %) were female. Positive family history for the syndrome was observed in 55(73 %) patients and ectopia lentis in 37(49 %). Positive systemic score (≥7) was identified in 60(80 %) individuals and the most frequent score components were: skin striae in 64(85 %), scoliosis in 59(79 %), wrist and thumb sign in 45(60 %), moderate or severe myopia in 43(57 %) and plain flat foot in 40(53 %). Cardiovascular symptoms occurred in 17(23 %) patients: dyspnea in 10(13 %) and palpitations in 6(8 %). Mitral valve prolapse was observed in 32(43 %) participants and aortic root dilation (z-score ≥ 2) in 53(71 %), without significant difference between the groups with or without these alterations concerning sex, age, or symptom presence. CONCLUSION: Clinical profile of a Marfan syndrome outpatient cohort includes adolescents and young adults, most without cardiovascular symptoms and with a high incidence of skeletal, ophthalmological, and cardiovascular involvement. Recognizing these clinical signs could contribute to early disease diagnosis in the general population.

Chest Pain in Acute Myocardial Infarction and Its Association With the Culprit Artery and Fibrotic Segment Identified by Cardiac Magnetic Resonance.

Background: It is still very controversial whether the characteristics of pain in the acute myocardial infarction could be related to the culprit coronary artery. There are no data about associations of pain with the ST-segment elevation myocardial infarction (STEMI) and left ventricular (LV) fibrotic segments. Methods: Data from 328 participants who had STEMI and were included in the B and T Types of Lymphocytes Evaluation in Acute Myocardial Infarction (BATTLE-AMI) study were analyzed. The culprit artery was identified by coronary angiography and the injured myocardial segments by cardiac magnetic resonance. The statistical significance was established by P value < 0.05. Results: A total of 223 patients (68%) were selected. Association was not observed between chest pain and the culprit artery (P = 0.237), as well as between pain irradiation and the culprit artery (P = 0.473). No significant difference was observed in the pain localization in relation to the segments in the short axis basal, mid, apical, and long axis, except for the mid inferior segment. The data were not considered clinically relevant because this association was observed in only one of 17 segments after multiple comparisons. Conclusions: In patients with STEMI, no associations were observed between the location or irradiation of acute chest pain and/or adjacent areas and the culprit artery, or between pain and segmental myocardial fibrosis in the LV.

Premature atrial and ventricular complexes in outpatients referred from a primary care facility.

BACKGROUND: Premature complexes are common electrocardiographic findings in daily clinical practice that require further evaluation. Investigation may sometimes be complex and expensive. The aim of our study was to analyze variables associated with premature beats identified in outpatients referred from a primary care facility. MATERIALS AND METHODS: We performed a cross-sectional study of 407 outpatients (aged 55.8±11years; 56% women) who were followed by general practitioners and were referred for resting 12-lead electrocardiograms for a routine clinical follow-up. After signing informed consent, patients answered a questionnaire and underwent physical examinations, laboratory diagnostics, transthoracic echocardiograms and 24-hour Holter monitoring to evaluate for the presence of premature complexes. After the univariate analyses, logistic regression analyses were performed with adjustment for age, sex, and cardiovascular diseases. RESULTS: Premature complexes distribution revealed that they were frequent but with low density. Premature atrial complexes (≥ 4/hours) were associated with age (Odds Ratio (OD) = 1.030, Confidence Interval (CI) 95% = 1.002 ─ 1.059, p = 0.029), brain natriuretic peptide (BNP) levels > 20mg/dL (OR = 4.489, 95%CI = 1.918 ─ 10.507, p = 0.0005), intraventricular blocks (OR = 4.184, 95%CI = 1.816 ─ 9.406, p = 0.0005) and left atrial diameter (OR = 1.065, 95%CI = 1.001 ─ 1.134, p = 0.046). Premature ventricular complexes (≥ 5/hour) were related to age (OR = 1.032, 95%CI = 1.010 ─ 1.054, p = 0.004), the use of calcium channel blockers (OR = 2.248, 95%CI = 1.019 ─ 4.954, p = 0.045), HDL-cholesterol levels (OR = 0.971, 95%CI = 0.951 ─ 0.992, p = 0.007), BNP levels > 20mg/dL (OR = 2.079, 95%CI = 0.991 ─ 0.998, p = 0.033), heart rate (OR = 1.019, 95%CI = 1.001 ─ 1.038, p = 0.041), left ventricular hypertrophy (OR = 2.292, 95%CI = 1.402 ─ 3.746, p = 0.001) and left ventricular ejection fraction (OR = 0.938, 95%CI = 0.900 ─ 0.978, p = 0.002). CONCLUSIONS: Premature complexes had low density and were associated with BNP levels > 20mg/dL, lower levels of HDL-cholesterol, left atrial enlargement and ventricular hypertrophy. The identification of premature complexes on 24-hour Holter monitor recordings of outpatients in a primary public healthcare setting was associated with uncontrolled cardiovascular risk factors that may be addressed with medical advice and therapy in a primary care setting.