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Marfan syndrome (MFS) and MFS-related diseases are inherited connective tissue disorders involving several organ systems. The diagnosis of MFS is difficult as the many symptoms overlap with those of other systemic connective tissue diseases. The phenotype is progressive. Effective surgical therapy and standardized follow-up programs have led to an improved lifespan for the affected individuals. Selective angiotensin II, type 1 (AT1) blockers may improve several manifestations of MFS, but the outcome of clinical trials is presently unknown. This review describes the importance of a coordinated strategy for diagnosis, treatment and follow-up.

Marfan syndrome (MFS) is a hereditary connective tissue disorder. Studies of MFS have established the critical contribution of fibrillin-1 deficiency to disease progression through altered cell-matrix interactions and dysregulated TGF-β signalling. It is now known that the disease is caused by altered regulation of TGF-β. As a result, the definition of MFS- and MFS-related diseases as the prototypical structural disorder of the connective tissue has changed to that of a developmental abnormality with broad and complex effects on the morphogenesis and tissue remodelling.

European guidelines do not recommend transesophageal echocardiography (TEE) during the course of an apparent uncomplicated treatment of infective endocarditis (IE). We report two cases where routine TEE at the end of treatment showed formation of pseudoaneurysms despite lack of any symptoms or signs of infection. We suggest that our finding be explained by a local dissection in the weakened but sterile paravalvular tissue. Therefore, TEE should be considered as a routine procedure in IE before antibiotic treatment is stopped, irrespective of the patient's course.

Introduction: The Danish Heart Registry (DHR) keeps track of all coronary angiographies (CATH), percutaneous coronary interventions (PCI), coronary artery bypass grafting (CABG), and adult heart valve surgery performed in Denmark. DHR is a clinical database established in order to follow the activity and quality of the procedures mentioned., Materials and Methods: Information concerning each procedure, age, gender, and co-morbidity of the patient was collected. Each patient was followed with respect to survival for 30 days by linkage to the central personal registry in Denmark. Mortality was estimated by the Kaplan-Meier method and comparisons of 30-day mortality between centres were carried out in Cox proportional hazard models., Results: The mortality within 30 days after PCI was 3.2% and closely related to the indication for PCI: ST-elevation myocardial infarction (STEMI) 6.8%; non-STEMI & unstable angina pectoris 1.9% and stable angina pectoris 0.5%. The 30-day mortality after PCI on the indication STEMI did not differ between the five centres, P=0.30. Mortality within 30 days after isolated CABG was 2.6% and was closely related to the EuroSCORE. The 30-day mortality after isolated CABG did not differ between the five centres, P=0.12., Conclusion: The 30-day mortality was closely related to the indication for PCI and the EuroSCORE for patients undergoing CABG. There were no significant differences in 30-day mortality between centres after either primary PCI or isolated CABG.

A Q wave has high specificity in identifying acute myocardial infarction and is associated with infarcts of a larger size. A considerable number of studies have addressed the prognostic value of the presence of a Q wave in the electrocardiogram before and after the introduction of thrombolytic therapy. Overall, these studies have demonstrated that a Q wave has slight, or even no, prognostic impact, especially in studies performed since the introduction of thrombolytic therapy. Some therapeutic choices have been suggested according to the presence or absence of Q waves. These suggestions depend basically on subgroup analysis with different results and hence have not documented that a Q wave has any value in therapeutic choices. A pathological Q wave has no significant prognostic value after a myocardial infarction and there is very doubtful documentation that a Q wave has any value for therapeutic choice.