The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies.

The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis.

Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome.

Background: Aortic branch aneurysms are not included in the diagnostic criteria for Marfan syndrome (MFS); however, their prevalence and eventual prognostic significance are unknown.

Objectives: The goal of this study was to assess the prevalence of aortic branch aneurysms in MFS and their relationship with aortic prognosis.

Methods: MFS patients with a pathogenic FBN1 genetic variant and at least one magnetic resonance or computed tomography angiography study assessing aortic branches were included.

Angiogenic Factors and Long-Term Cardiovascular Risk in Women That Developed Preeclampsia During Pregnancy.

Preeclampsia is caused by placental impairment with increased expression of sFlt-1 (soluble fms-like tyrosine kinase 1) and decreased PlGF (placental growth factor); it has been associated with cardiovascular morbidity and mortality later in life, but the underlying mechanism remains unknown. The aim of this study was to determine whether sFlt-1 and PlGF levels during preeclampsia are associated to long-term cardiovascular risk. We prospectively recruited 43 women with previous preeclampsia and 21 controls with uncomplicated pregnancies.

Proximal aorta longitudinal strain predicts aortic root dilation rate and aortic events in Marfan syndrome.

Aims: Life expectancy in Marfan syndrome patients has improved thanks to the early detection of aortic dilation and prophylactic aortic root surgery. Current international clinical guidelines support the use of aortic root diameter as a predictor of complications. However, other imaging markers are needed to improve risk stratification.

The current role of echocardiography in acute aortic syndrome.

Acute aortic syndrome (AAS) comprises a range of interrelated conditions caused by disruption of the medial layer of the aortic wall, including aortic dissection, intramural haematoma and penetrating aortic ulcer. Since mortality from AAS is high, a prompt and accurate diagnosis using imaging techniques is paramount. Both transthoracic (TTE) and transoesophageal echocardiography (TEE) are useful in the diagnosis of AAS.

Implications of Asymmetry and Valvular Morphotype on Echocardiographic Measurements of the Aortic Root in Bicuspid Aortic Valve.

Background: Transthoracic echocardiography (TTE) and magnetic resonance imaging (MRI) have yielded excellent results in aortic root diameter measurement in patients with tricuspid aortic valve. However, accuracy in bicuspid aortic valve (BAV), often associated with aortic root asymmetry, is not fully defined. The aim of this study was to determine the agreement between TTE and MRI in proximal ascending aortic diameters in patients with BAVs.

Rapid ultrasound score as an indicator of atherosclerosis' clinical manifestations in a population of hypertensives: the interrelationship between flow-mediated dilatation of brachial artery, carotid intima thickness, renal resistive index and retina resistive index of central artery.

Objective: Flow-mediated dilatation (FMD) of brachial artery, renal resistive index (RRI), retina resistive index of central artery (RRICA) and carotid intima-media thickness (IMT) have been used for ultrasound assessment of cardiovascular risk as good surrogate markers of pre-clinical atherosclerosis. We investigated the interrelationship of these four parameters and examined whether an integrated score is a good indicator of atherosclerotic disease in hypertensives.

Methods: One-hundred fifty-two consecutive subjects were enrolled in this study between April 2004 and April 2005.