Marfanův syndrom (MFS) je geneticky podmíněné onemocnění pojivové tkáně, které poprvé popsal francouzský pediatr Antoine Bernard-Jean Marfan v roce 1896 u pětileté dívky se svalovou atrofií a „nápadně“ dlouhými končetinami. Klinicky Marfanův syndrom zahrnuje celé spektrum příznaků. Nejdůležitější klinickou manifestací je dilatace ascendentní aorty, která nositele ohrožuje vznikem disekce, ruptury, či vznikem aortální regurgitace vedoucí k srdečnímu selhání. Preventivní náhrada postiženého úseku aorty kompozitním štěpem je zlatým standardem léčby. Po operaci přetrvává riziko pozdních komplikací, vyžadující provedení reoperace, která je ovšem spojená s horší prognózou a je zatížená vyšším rizikem perioperační mortality. V indikovaných případech se katetrizační procedury stávají plnohodnotnou alternativou chirurgických zákroků.
Marfan's syndrome (MFS) is genetic disorder of connective tissue. It was Antoine Bernard-Jean Marfan who first described disease in five-years old girl with muscular atrophy and „remarkable“ elongation of extremities. Clinically, MFS involves whole spectrum of signs. The most important clinical manifestation is dilatation of ascending aorta with possible life threatening conditions, such as dissection or rupture of the aorta, or severe regurgitation resulting in heart failure. Preventive composite valve replacement procedure is surgical gold standard. After successful aortic root replacement, there is still risk of late complications that require reoperation, which is associated with poor prognosis and higher perioperative mortality rates. Therefore, percutaneous procedures tend to be safe and effective alternative of surgical repair in indicated cases.
- MeSH
- Aorta * diagnostic imaging physiopathology pathology MeSH
- Heart Valve Prosthesis Implantation MeSH
- Adult MeSH
- Endoleak MeSH
- Humans MeSH
- Marfan Syndrome MeSH
- Aneurysm, False * diagnostic imaging therapy MeSH
- Reoperation MeSH
- Septal Occluder Device MeSH
- Cardiac Catheterization * methods MeSH
- Vascularized Composite Allotransplantation MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Male MeSH
- Publication type
- Case Reports MeSH
- MeSH
- Diagnostic Techniques, Cardiovascular classification utilization MeSH
- Diuretics administration & dosage pharmacology therapeutic use MeSH
- Drug Therapy methods utilization MeSH
- Cardiovascular Surgical Procedures methods utilization MeSH
- Humans MeSH
- Evidence-Based Medicine MeSH
- Heart Failure drug therapy therapy MeSH
- Vasodilator Agents administration & dosage pharmacology therapeutic use MeSH
- Check Tag
- Humans MeSH
BACKGROUND: Statins have been proved to be effective in reduction of mortality and morbidity when started in the early secondary prevention in stabilized patients after acute coronary syndrome (ACS). The safety and efficacy of statin administration directly in the first-line therapy in unstable ACS patients is not clear. The aim of our study was, therefore, to assess the effect of statin treatment initiated immediately at hospital admission of patients with ACS. METHODS: The trial was stopped prematurely after enrollment of one hundred and fifty-six patients with ACS that were randomized at admission to fluvastatin 80 mg (N = 78) or placebo (N = 78). Study medication was administered immediately after randomization and then once daily for 30 days; all patients were then encouraged to continue in open-label statin therapy and at the end of one-year follow-up 75% in the fluvastatin group and 78% in the placebo group were on statin therapy. RESULTS: We did not demonstrate any difference between groups in the level of C-reactive protein, interleukin 6, and pregnancy-associated plasma protein A on Day 2 and Day 30 (primary endpoint). Fluvastatin-therapy, however, significantly reduced one-year occurrence of major adverse cardiovascular events (11.5% vs. 24.4%, odds ratio (OR) 0.40, 95% CI 0.17-0.95, P = 0.038). This difference was caused mainly by reduction of recurrent symptomatic ischemia (7.7% vs. 20.5%, OR 0.32, 95% CI 0.12-0.88, P = 0.037). CONCLUSIONS: This study failed to prove the effect of fluvastatin given as first-line therapy of ACS on serum markers of inflammation and plaque instability. Fluvastatin therapy was, however, safe and it may reduce cardiovascular event rate that supports immediate use of a statin in patients admitted for ACS. TRIAL REGISTRATION: NCT00171275.
- MeSH
- Acute Coronary Syndrome drug therapy immunology mortality MeSH
- C-Reactive Protein metabolism MeSH
- Time Factors MeSH
- Double-Blind Method MeSH
- Risk Assessment MeSH
- Indoles administration & dosage adverse effects MeSH
- Interleukin-6 blood MeSH
- Kaplan-Meier Estimate MeSH
- Fatty Acids, Monounsaturated administration & dosage adverse effects MeSH
- Middle Aged MeSH
- Humans MeSH
- Inflammation Mediators blood MeSH
- Odds Ratio MeSH
- Placebo Effect MeSH
- Early Termination of Clinical Trials MeSH
- Patient Admission MeSH
- Prospective Studies MeSH
- Recurrence MeSH
- Chi-Square Distribution MeSH
- Drug Administration Schedule MeSH
- Secondary Prevention methods MeSH
- Aged MeSH
- Hydroxymethylglutaryl-CoA Reductase Inhibitors administration & dosage adverse effects MeSH
- Pregnancy-Associated Plasma Protein-A metabolism MeSH
- Sample Size MeSH
- Patient Selection MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
- Randomized Controlled Trial MeSH
- Geographicals
- Czech Republic MeSH
Uvedený přehled nabízí čtenářům informace o jednotlivých mechanických komplikacích akutního infarktu myokardu, jejich výskytu, diagnostice a léčbě. Přestože výskyt většiny uvedených komplikací je v dnešní době, kdy většinu pacientů léčíme reperfúzní terapií, málo častý, jejich závažnost a vysoká mortalita na ně nutí myslet při změně klinického stavu pacientů v časném poinfarktovém období. Rychlost diagnostiky a zahájení terapie je zásadní pro jejich přežití.
This overview provides readers with information of mechanical complications of an acute myocardial infarction, focusing on the incidence, diagnostic procedures and therapy. At present, when the majority of patients is treated with a reperfusion therapy, the incidence of mechanical complications decreased considerably in comparison to pre-reperfusion era data. Nevertheless, the high mortality of mechanical complications demands prompt diagnosis and well-chosen timing in the delivery of therapy; the both are proven to be critical for patients` survival.
Stresová kardiomyopatie je onemocnění charakterizované náhle vzniklou systolickou dysfunkcí levé komory srdeční, která je plně reverzibilní, nepostihuje bazální segmenty levé komory, není provázena významným postižením věnčitých tepen a předchází jí stresová situace. V tomto přehledném článku prezentujeme etiopatogenetické mechanizmy, které hrají roli při tomto onemocnění, dále možnosti diagnostické i terapeutické. V závěru uvádíme kazuistiku nemocné se vzácnější formou tohoto onemocnění – midventrikulární stresovou kardiomyopatií.
Stress cardiomyopathy is a disease characterized by an acute systolic dysfunction of the left ventricle of the heart which is fully reversible and does not affect basal segments of the left ventricle. It is not accompanied by significant stenoses of the coronary arteries and it is caused by a preceding stressful situation. This article provides an overview of the ethiopathogenesis, diagnostic and therapeutic methods. Finally, we present a case report of our patient who suffers from a rare form of the midventricular type of stress cardiomyopathy.
- MeSH
- Diagnosis, Differential MeSH
- Ventricular Dysfunction, Left diagnosis etiology physiopathology MeSH
- Echocardiography methods utilization MeSH
- Stress, Physiological complications physiopathology therapy MeSH
- Cardiomyopathies diagnosis etiology complications MeSH
- Coronary Angiography methods utilization MeSH
- Humans MeSH
- Radionuclide Ventriculography methods utilization MeSH
- Check Tag
- Humans MeSH
- Publication type
- Case Reports MeSH
