Heart Failure: An overall assessment

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Διερεύνηση των παραμέτρων της δοκιμασίας κοπώσεως σε νέους ενήλικες άντρες με ετερόζυγο οικογενή υπερχολεστερολαιμία (hFH)

Η οικογενής ετερόζυγος υπερχοληστερολαιμία (heFH) οφείλεται κυρίως σε μεταλλάξεις του γονιδίου του υποδοχέα της χαμηλής πυκνότητας λιποπρωτεΐνης (LDL). Το λιπιδαιμικό προφίλ των ασθενών χαρακτηρίζεται κυρίως από αυξημένα επίπεδα ολικής και LDL χοληστερόλης. Η σημαντικότερη επιπλοκή της νόσου είναι η αυξημένη επίπτωση πρώιμης και επιταχυνόμενης αθηρωμάτωσης. Για την διάγνωση της νόσου έχουν ορισθεί κριτήρια, με βάση τα κλινικά, εργαστηριακά και γονιδιακά χαρακτηριστικά του ασθενούς. Η θεραπευτική προσέγγιση της FH στοχεύει φαρμακευτικά, στην αναστολή της ενδογενούς σύνθεσης της χοληστερόλης και στην παρεμπόδιση της απορρόφησής της από το έντερο, και στην LDL- μηχανική αφαίρεση από το αίμα. Η θεραπεία με μονοκλωνικά αντισώματα (αναστολή της PCSK9) επιτυγχάνει επιπρόσθετη μείωση. Η δοκιμασία κοπώσεως (ΔΚ) είναι η πιο ευρέως διαδεδομένη και χρησιμοποιούμενη μέθοδος για την διάγνωση και την διαστρωμάτωση κινδύνου ασθενών με στεφανιαία νόσο. Φυσιολογικά η αρτηριακή πίεση (ΑΠ), σημαντική παράμετρος της δοκιμασίας κοπώσεως, αυξάνεται αναλογικά με την πρόοδο της άσκησης. Για την απεικόνιση της λειτουργικότητας του μυοκαρδίου πέραν της κλασσικής διδιάστατης υπερηχογραφίας, η χρήση της τεχνικής speckle tracking, επιτρέπει την μέτρηση της συνολικής επιμήκους συστολικής παραμόρφωσης του μυοκαρδίου. Ο σκοπός της παρούσας μελέτης ήταν να εκτιμήσει την μεταβολή της συστολικής(ΣΑΠ) και διαστολικής (ΔΑΠ) αρτηριακής πίεσης, καθώς και την μεταβολή των υπόλοιπων παραμέτρων κατά τη ΔΚ σε κυλιόμενο τάπητα με πρωτόκολλο Bruce, σε νέους ενήλικες άνδρες με heFH σε σύγκριση με ομάδα ελέγχου υγιών ανδρών. Αφού εκτιμήθηκε η λειτουργικότητα της αριστερής κοιλίας (ΑΚ) με διδιάστατο υπερηχογράφημα καρδιάς, ακολούθησε μέτρηση της συνολικής επιμήκoυς συστολικής παραμόρφωσης του μυοκαρδίου (Global Longitudinal Strain) από τις κορυφαίες τομές 4, 3 και 2 κοιλοτήτων με την τεχνική speckle tracking. Από την συσχέτιση των ευρημάτων, φαίνεται ότι οι νέοι ενήλικες άνδρες με heFH παρουσίασαν μεγαλύτερη αύξηση της ΣΑΠ και ΔΑΠ στο μέγιστο της ΔΚ, σε σχέση με την ομάδα των υγιών ανδρών. Οι τιμές της συνολικής επιμήκους συστολικής παραμόρφωσης του μυοκαρδίου των νέων ενηλίκων ανδρών με heFH ήταν ελαφρώς μειωμένες, συγκρινόμενες με τις αντίστοιχες τιμές των υγιών μαρτύρων, ενώ παρατηρήθηκε στατιστικά σημαντική βελτίωση αυτών σε ποσοστό 84% των ασθενών μετά από μακροχρόνια λήψη υπολιπιδαιμικής αγωγής. Το κλάσμα εξώθησης της ΑΚ ήταν φυσιολογικό και στις δύο ομάδες. Συμπερασματικά, από την παρούσα μελέτη, φαίνεται ότι στους ασθενείς με heFH, από την μέτρηση της ΑΠ ηρεμίας καθώς και από την 2D υπερηχοκαρδιογραφική μελέτη ηρεμίας δεν αναδείχθηκαν παθολογικά ευρήματα. Απεναντίας, η χρήση της ΔΚ, με την αύξηση της ΣΑΠ και της ΔΑΠ, ανέδειξε μάλλον την ύπαρξη υποκλινικά αρτηριακής υπέρτασης. Παράλληλα, με την εφαρμογή των νεότερων υπερηχοκαρδιογραφικών τεχνικών, η μέτρηση της συνολικής επιμήκους συστολικής παραμόρφωσης του μυοκαρδίου ανέδειξε παθολογική τιμή παραμόρφωσης των μυοκαρδιακών ινών κατά τον επιμήκη άξονα της ΑΚ, γεγονός που σημαίνει μείωση της ελαστικής «δύναμης» των επιμήκων ινών. Η βελτίωση της μυοκαρδιακής παραμόρφωσης μετά από μακρό χρονικό διάστημα υπολιπιδαιμικής αγωγής ενισχύει τον ισχυρισμό.Familiar hypercholesterolemia (FH) is mainly due to mutations of the gene coding the LDL-receptor. The lipidemic profile of such patients is characterized by elevated levels of both total (TC), and LDL cholesterol. Elevated triglyceride (TG) and low HDL levels might also coexist. The most important consequence is the increased prevalence of early and accelerated atherosclerotic disease, particularly in the homozygotes, who develop coronary artery disease (CAD) before the age of 10. Nowadays, the management of FH includes a spectre of hypolipidemic drugs, aiming to the inhibition of the hepatic production or prevention of the intestinal absorption of cholesterol. The non pharmacological therapy includes LDL-apheresis, a procedure being able to directly remove LDL cholesterol from circulation with beneficial results mainly in homozygotes and severe eterozygotes as well. Monoclonal antibodies that inhibit PCSK9 are a new class of drugs that lower LDL cholesterol in patients that can not tolerate hypolipidemic drugs or when the LDL cholesterol reduction could not been achieved.Exercise treadmill testing (ETT) is a well-established, easy, safe and low-cost procedure, used for many decades as a noninvasive test to diagnose CAD. The normal response of the blood pressure in progressive exercise testing is the systolic blood pressure (SBP) to increase, while the diastolic blood pressure (DBP) to be maintained or to decrease slightly. Furthermore, the visual assessment of two-dimensional (2D) echocardiography provides a rapid evaluation of left ventricle (LV) systolic function. A more thorough evaluation of LV systolic function requires calculation of LV ejection fraction (LVEF) using Simpson's biplane method. Novel echocardiographic techniques allow the assessment of myocardial strain, which can measure myocardial deformation as an intrinsic mechanical property of the myocardium. In our study, we evaluated the change of the SBP and DBP during the ETT in asymptomatic adult heFH men without known CAD and with normal LVEF. Furthermore, we combined it with probable early detection of myocardial systolic abnormality assessed by measuring GLS. We found that heFH men showed a higher peak SBP and DBP at ETT and higher corresponding delta values, compared with healthy men. A slight but statistically significant reduction of GLS was also documented, although no difference in LVEF was identified. These findings lead to the assumption of preclinical cardiovascular impairment presence in a heFH male population

Transfusion-related acute lung injury: A case report

Transfusion-related acute lung injury is the most common cause of serious morbidity and mortality associated with the transfusion of plasma-containing blood components. The syndrome can be confused with other causes of acute respiratory failure. Herein, we describe a 71-year-old man who was transfused with fresh frozen plasma due to prolonged INR, and died of what was considered as transfusion-related acute lung injury, despite treatment

The emerging role of Cardiovascular Magnetic Resonance in the evaluation of hypertensive heart disease

Abstract Background Arterial hypertension is the commonest cause of cardiovascular death. It may lead to hypertensive heart disease (HHD), including heart failure (HF), ischemic heart disease (IHD) and left ventricular hypertrophy (LVH). Main body According to the 2007 ESH/ESC guidelines, the recommended imaging technique is echocardiography (echo), when a more sensitive detection of LVH than that provided by ECG, is needed. Cardiovascular Magnetic Resonance (CMR), a non-invasive, non-radiating technique, offers the following advantages, beyond echo: a) more reliable and reproducible measurements of cardiac parameters such as volumes, ejection fraction and cardiac mass b) more accurate differentiation of LVH etiology by providing information about tissue characterisation c) more accurate evaluation of myocardial ischemia, specifically if small vessels disease is present d) technique of choice for diagnosis of renovascular, aortic tree/branches lesions and quantification of aortic valve regurgitation e) technique of choice for treatment evaluation in clinical trials. The superiority of CMR against echocardiography in terms of reproducibility, operator independency, unrestricted field of view and capability of tissue characterization makes the technique ideal for evaluation of heart, quantification of aortic valve regurgitation, aorta and aortic branches. Conclusions CMR has a great potential in early diagnosis, risk stratification and treatment follow up of HHD. However, an international consensus about CMR in HHD, taking under consideration the cost-benefit ratio, expertise and availability, is still warranted

Reduced global longitudinal strain at rest and inadequate blood pressure response during exercise treadmill testing in male heterozygous familial hypercholesterolemia patients

Background: Heterozygous familial hypercholesterolemia (heFH) is a genetic disorder leading to premature coronary artery disease (CAD). We hypothesized that the subclinical pathophysiologic consequences of hypercholesterolemia may be detected before the occurrence of clinically overt CAD by stress testing and myocardial strain imaging. Patients-methods: We evaluated the treadmill tests (ETTs) of 46 heFH men without known arterial hypertension/diabetes mellitus/vasculopathy like CAD and of 39 healthy men matched for age, baseline systolic/diastolic blood pressure (BP) and heart rate (HR), using Bruce protocol. Global longitudinal strain (GLS) of the left ventricle (LV) additionally to ejection fraction was obtained. Results: heFH men reached a significantly higher peak systolic and diastolic BP compared to controls (p = 0.002 and p < 0.001, respectively). Mean rate pressure product was significantly higher in heFH patients (p = 0.038). Both duration of the ETT and workload in metabolic equivalents was lower in the heFH group (p < 0.001 and p < 0.001, respectively). Baseline to peak rise of systolic and diastolic BP in heFH men was higher (p = 0.008 and p < 0.001 for systolic and diastolic BP, respectively). Furthermore, heFH men had higher rise of HR from baseline to peak, compared to controls; (p = 0.047). GLS in heHF men was slightly decreased (p = 0.014), although the ejection fraction was similar in both groups. Conclusion: heFH men have a higher rise in systolic/diastolic BP during ETT, which may reflect early, preclinical hypertension. Furthermore, slight impairment of LV GLS is present, despite the absence of apparent myocardial dysfunction in conventional 2D echocardiography

Cardiovascular Magnetic Resonance as Pathophysiologic Tool in Diabetes Mellitus

Diabetes mellitus can independently contribute to cardiovascular disease and represents a severe risk factor for premature development of cardiovascular disease. A three-fold higher mortality than the general population has been observed in type 1 diabetes mellitus whereas a two- to four-fold increased probability to develop cardiovascular disease has been observed in type 2 diabetes mellitus. Cardiovascular magnetic resonance, a non-radiative modality, is superior to all other modalities in detecting myocardial infarction. The main cardiovascular magnetic resonance sequences used include a) balanced steady-state free precession (bSSFP) for function evaluation; b) T2-W for oedema detection; c) T1 W for ischemia detection during adenosine stress; and d) late gadolinium enhanced T1-W images (LGE), evaluated 15 min after injection of paramagnetic contrast agent gadolinium, which permit the diagnosis of replacement fibrosis, which appears white in the middle of suppressed, nulled myocardium. Although LGE is the technique of choice for diagnosis of replacement fibrosis, it cannot assess diffuse myocardial fibrosis. The application of T1 mapping (native or pre contrast and post contrast) allows identification of diffuse myocardial fibrosis, which is not detectable my other means. Native T1 and Contrast-enhanced T1 mapping are involved in the extracellular volume fraction (ECV) calculation. Recently, 1H-cardiovascular magnetic resonance spectroscopy has been applied to calculate the amount of myocardial triglycerides, but at the moment it is not part of the routine assessment of diabetes mellitus. The multifaceted nature of cardiovascular magnetic resonance has the great potential of concurrent evaluation of function and myocardial ischemia/fibrosis in the same examination and represents an indispensable tool for accurate diagnosis of cardiovascular disease in diabetes mellitus

Cardiovascular Magnetic Resonance Detects Inflammatory Cardiomyopathy in Symptomatic Patients with Inflammatory Joint Diseases and a Normal Routine Workup

Background. Patients with inflammatory joint diseases (IJD) are more likely to develop cardiovascular disease compared with the general population. We hypothesized that cardiovascular magnetic resonance (CMR) could identify cardiac abnormalities in patients with IJD and atypical symptoms unexplained by routine clinical evaluation. Patients-Methods. A total of 51 consecutive patients with IJD (32 with rheumatoid arthritis, 10 with ankylosing spondylitis, and 9 with psoriatic arthritis) and normal clinical, electrocardiographic and echocardiographic workups, were referred for CMR evaluation due to atypical chest pain, shortness of breath, and/or palpitations. Their CMR findings were compared with those of 40 non-IJD controls who were referred for the same reason. All participants were examined using either a 1.5 T or 3.0 T CMR system. For T1/T2 mapping, comparisons were performed separately for each field strength. Results. Biventricular systolic function was similar between groups. In total, 25 (49%) patients with IJD vs. 0 (0%) controls had replacement-type myocardial fibrosis (p &lt; 0.001). The T2 signal ratio, early/late gadolinium enhancement, and extracellular volume fraction were significantly higher in the IJD group. Native T1 mapping was significantly higher in patients with IJD independent of the MRI field strength (p &lt; 0.001 for both). T2 mapping was significantly higher in patients with IJD compared with controls only in those examined using a 1.5 T MR system&mdash;52.0 (50.0, 55.0) vs. 37.0 (33.5, 39.5), p &lt; 0.001. Conclusions. In patients with IJD and a mismatch between cardiac symptoms and routine non-invasive evaluation, CMR uniquely identified a significant proportion of patients with myocardial inflammation. A CMR examination should be considered in patients with IJD in similar clinical settings

Combined Brain/Heart Magnetic Resonance Imaging in Systemic Lupus Erythematosus

Cardiovascular Disease (CVD) in Systemic Lupus Erythematosus (SLE) and Neuropsychiatric SLE (NPSLE) has an estimated prevalence of 50% and 40%, respectively and both constitute major causes of death among SLE patients. In this review, we proposea combined brain/heart Magnetic Resonance Imaging (MRI) for SLE risk stratification has been proposed. The pathophysiologic background of NPSLE includes microangiopathy, macroscopic infarcts and accelerated atherosclerosis. Classic brain MRI findings demonstrate lesions suggestive of NPSLE in 50% of the NPSLE cases, while advanced MRI indices can detect pre-clinical lesions in the majority of them, but their clinical impact still remains unknown. Cardiac involvement in SLE includes myo-pericarditis, valvular disease/endocarditis, Heart Failure (HF), coronary macro-microvascular disease, vasculitis and pulmonary hypertension. Classic and advanced Cardiovascular Magnetic Resonance (CMR) indices allow function and tissue characterization for early diagnosis and treatment follow-up of CVD in SLE. Although currently, there are no clinical data supporting the combined use of brain/heart MRI in asymptomatic SLE, it may have a place in cases with clinical suspicion of brain/heart involvement, especially in patients at high risk for CVD/stroke such as SLE with antiphospholipid syndrome (SLE/APS), in whom concurrent cardiac and brain lesions have been identified. Furthermore, it may be of value in SLE with multi-organ involvement, NPSLE with concurrent cardiac involvement, and recent onset of arrhythmia and/or heart failure