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Background Left ventricular (LV) remodeling in patients with severe aortic valve stenosis (AS) is a complex process that goes beyond hypertrophic response and may involve reparative/replacement fibrosis. Currently, cardiac magnetic resonance (CMR) is the gold-standard imaging technique for detecting focal myocardial fibrosis through late gadolinium enhancement (LGE). However, myocardial fibrosis prevalence and distribution is quite variable among series. Our goal was to assess LGE prevalence and distribution pattern in severe symptomatic high-gradient AS. Methodology Single-center prospective cohort of 132 patients with severe symptomatic high-gradient AS (mean age 73±11 years; 48% male, mean valvular transaortic gradient 60±20 mmHg; mean aortic valve area 0.7±0.2 cm2/m2; mean LV ejection fraction by 2D echocardiogram 58±9%), all with normal flow (except one) undergoing surgical aortic valve replacement. Those with previous history of acute myocardial infarction, ischemic cardiomyopathy or other cardiomyopathy were excluded. All patients performed 1.5T CMR assessment with LV myocardium tissue characterization prior to surgery. Segmental LGE presence was assessed by two independent operators and classified according to the AHA 16 segment model, using 5-standard deviations from remote myocardium as the signal intensity cut-off for LGE identification and quantification. Results Overall, 96 patients (74%) had non-ischemic LGE (median LGE mass 3.2 g [IQR 0.2–8.3] g; median percentage of LGE myocardial mass 2.5% [IQR 0.1–6.1]%); 22 patients [17%] with exclusively junctional LGE); in one patient an incidental ischemic scar (subendocardial distribution) was identified. No cases of subepicardial distribution were found. Intramyocardial LGE was most frequently observed in basal and mid-anterior and inferior interventricular septum – see Figure 1. In these segments, LGE was most often junctional at right-ventricular insertion points (54%), followed by mid-wall LGE (32%) or both sites involvement (14%). Conclusion LGE is frequent in symptomatic high-gradient AS patients with preserved left ventricular ejection fraction, most often presenting as junctional enhancement in basal/mid-anterior and inferior interventricular septum. Future studies may address whether distinct LGE patterns may impact patient prognosis. Funding Acknowledgement Type of funding sources: None.

Background Left ventricular (LV) hypertrophy is a common expected finding in aortic stenosis (AS) patients. Cardiac magnetic resonance (CMR) plays an important role as a non-invasive method for determining LV mass and volume, and to characterize the LV remodeling response in AS. Aim To assess the prevalence, to describe the patterns and evolution of LV remodeling (by CMR) in AS patients referred for surgical aortic valve replacement (AVR). Methods Single-center prospective cohort of 132 consecutive patients (73 years [68–77 years], 49% men] with severe AS: mean transaortic pressure gradient (AVmean): 61±1.5 mmHg; aortic valve area (AVA): 0.7±0.1 cm2, referred for surgical AVR, with no previous history of ischemic cardiomyopathy. Before surgery, all patients underwent electrocardiogram, complete transthoracic echocardiogram (TTE) and CMR for LV assessment and tissue characterization (mean LV indexed mass [LVMi]: 80.3±26.5 g/m2; mean end-diastolic LV indexed volume [LVEDVi]: 84.4±24.5 mL/m2 and median geometric remodeling ratio [M/V]: 0.95 g/mL [IQR 0. 81–1.08 g/mL]). Patterns of LV remodeling were investigated before and after AVR by CMR measurements of LVMi, LVEDVi and M/V. Besides normal LV ventricular structure, four other patterns were considered: concentric remodeling, concentric hypertrophy, eccentric hypertrophy, and adverse remodeling (Figure 1). Results Overall, 43% (n=58) of the patients had concentric hypertrophy, 30% (n=40) concentric remodeling, 22% (n=29) normal ventricular geometry, 4% (n=5) eccentric hypertrophy and in two patients we observed an adverse remodeling pattern. AVR was performed in 80 patients. At the 3rd to 6th month post-AVR assessment, LV remodeling changed to: normal ventricular geometry in 46%, concentric remodeling in 31%, concentric hypertrophy in 19%, eccentric hypertrophy in 3% and adverse remodeling in only one patient (Figure 1). Conclusions In this group of patients with severe aortic stenosis, concentric hypertrophy was not the sole pattern of LV remodeling and two out of every five still presented a normal ventricular geometry and mass as assessed by CMR. LV response was dynamic after AVR which stands for complex and multifactorial interaction in these group of patients despite similar valvular pathophysiology and therapeutic intervention. Funding Acknowledgement Type of funding sources: None.

Background Left ventricular (LV) hypertrophy is a known compensatory mechanism to pressure overload in aortic stenosis (AS) patients. However, by cardiac magnetic resonance (CMR) different patterns of LV adaptation are seen in this group of patients. Aim To describe the patterns of LV adaptation (by CMR) and to analyze its structure and function indexes in AS patients referred for surgical aortic valve replacement (AVR). Methods We prospectively studied 134 consecutive patients (age: 73y [IQR 68–77y], 49% men) with severe symptomatic AS - mean transaortic pressure gradient (AVmean): 61±1.5 mmHg; mean aortic valve area: AVA): 0.7±0.1 cm2, referred for surgical AVR with no previous history of ischemic cardiomyopathy or other. All patients underwent electrocardiogram, 2D transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) before surgery for LV assessment and tissue characterization. Five patterns of LV structure were considered: normal ventricular structure (normal LV mass/volume ratio [M/V], normal LVMi and normal indexed LV end-diastolic volume (LVEDVi); concentric remodeling: increased M/V, normal LVMi; concentric hypertrophy: increased M/V and LVMi; eccentric hypertrophy: increased LVMi and LVEDVi, normal M/V and ejection fraction; and adverse remodeling: dilated left ventricle, increased LVMi and normal M/V in the context of an impaired ejection fraction. Echocardiogram and CMR structural and functional indexes were compared between these groups. Results At baseline study, at CMR: mean LV indexed mass [LVMi]: 80.3±26.5 g/m2; mean end-diastolic LV indexed volume [LVEDVi]: 84.4±24.5 mL/m2 and median geometric remodeling ratio [M/V]: 0.95 g/mL [IQR 0. 81–1.08 g/mL]. Overall, 22% patients had normal LV structure, 30% concentric remodeling ventricular geometry, and two patients had an adverse remodeling pattern. LV hypertrophy was the most prevalent pattern and occurred in 48% of subjects (concentric 43%; eccentric 4%). In our cohort, the severity of AS (AVmean (p Conclusions In our cohort, AS patients presented several distinct patterns of LV remodeling. Disease severity, functional repercussion and loading conditions are distinct between them. Funding Acknowledgement Type of funding sources: None.

Background In patients with severe aortic stenosis (AS), left ventricular (LV) remodeling is believed to be a compensatory adaptive process which should reverse after aortic valve intervention. However, this is not always the rule and remodeling persistence may negatively impact post-procedural outcomes and survival. Aim To assess the prevalence and predictors of morphological LV reverse remodeling in severe symptomatic AS patients after surgical aortic valve replacement (AVR). Methods We prospectively studied 75 patients (72y [68–77y], 45% male) with severe symptomatic AS - mean gradient (AVM): 61±17mmHg; mean indexed aortic valve area (AVAi) 0.41±0.10 cm2/m2 with no previous history of ischemic cardiomyopathy, all with high gradient, 4 with low-flow, 81% with hypertension, 27% with type 2 diabetes mellitus and 35% patients with stage 3 chronic kidney disease: median MDR creat clearance: 70.4mL/min [40–102]. All patients performed pre-operative cardiac magnetic resonance (CMR) at a mean period of 3.4 months (0–17 months) before AVR and at the 3–6th months after AVR, for LV reverse remodeling assessment. It was defined as at least the occurrence of one of the following: >15% reduction in LVEDVi; >15% reduction in LVMi by CMR; >10% reduction in geometric remodeling ratio. Clinical, AV severity data, preoperative functional LV and tissue characterization data were analyzed at multivariate regression to predict the occurrence of LV reverse remodeling. Results Overall, at pre-operative CMR: mean LV indexed mass (LVMi): 82±28.9 g/m2; mean end-diastolic LV indexed volume (LVEDVi): 87.4±26.6 mL/m2; mean geometric remodeling (LV mass/end-diastolic volume): 0.92±0.2 g/mL. After AVR, at echocardiographic evaluation, no patient had prosthetic obstruction or prosthetic patient mismatch: median LV-Ao gradient 12mmHg [9.1–14 mmHg]; 5 of them had mild paravalvular regurgitation. LV reverse remodeling occurred in 65 patients (88%) (Figure 1A) and these were younger, had significantly smaller preoperative AVAi and higher valvular gradients (Figure 1B). At multivariate analysis, only preoperative AVAi remained an independent predictor (odds ratio 0.85, 95% CI 0.735–0.984, p=0.029). Conclusions In this prospective cohort of patients LV reverse remodeling after surgical AVR was highly frequent, occurring in almost nine out of every ten patients. Funding Acknowledgement Type of funding sources: None.

Background Myocardial fibrosis (MF) is a common finding and a potential adverse prognostic marker in several cardiac diseases, including in severe aortic stenosis (AS). While histological analysis obtained through endomyocardial biopsy remains the gold-standard for MF assessment, non-invasive cardiac imaging may offer surrogate biomarkers for fibrosis. We tried to assess the correlation between MF quantification at histopathology and cardiac magnetic resonance (CMR)-derived tissue characterization data in patients with severe AS. Methodology Single-center prospective cohort enrolling 71 patients with severe symptomatic high-gradient AS undergoing surgical aortic valve replacement (SAVR) (mean age 71±9 years; 49% male, mean valvular transaortic gradient 60±20 mmHg; mean left ventricle [LV] ejection fraction 58±9%). Those with past history of myocardial infarction or cardiomyopathy were excluded. All patients underwent pre-operative CMR study with LV tissue characterization and quantification. Normal T1 mapping value was defined as >1021ms as per center protocol. Myocardial tissue was obtained during SAVR either through myocardial biopsy at basal LV septum or harvested from surgical myectomy specimens. Masson's trichrome stain was used for collagen/fibrosis assessment. Automatic quantification was obtained at QuPathTM digital pathology software after applying a dedicated artificial intelligence algorithm on ultra-high-resolution digital slide scanning images. Results Histology-confirmed MF was observed in all patients (median percentage of fibrotic myocardial tissue 15% [IQR 9–22%]). Median global T1 mapping and extracellular volume (ECV) percentage was 1048ms (IQR 1027–1078) and 24% (IQR 20–30%), respectively. Late gadolinium enhancement (LGE) with a non-ischemic pattern was present in 42 patients (59%) with a median LGE mass of 5.8g [IQR 1.0–10.2]; median percentage of 3.7% [IQR 0.6–10.4]. While neither T1 mapping (global or basal LV septum), ECV nor LGE had any significant correlation with histology-confirmed MF (Figure 1) the vast majority had significantly elevated global and basal LV septum T1 mapping – 81% and 92%, respectively. Conclusion In this single-center prospective study, microscopic MF was present in all patients with severe symptomatic high-gradient AS, was accompanied by elevated T1 mapping values but no correlation was found between myocardial fibrosis at histopathology analysis and CMR-derived LV tissue characterization parameters. This may not only stem from sampling (single point biopsy vs. whole myocardial tissue assessment) but also from distinct evaluation of different types of fibrosis by different methods. Funding Acknowledgement Type of funding sources: None.

Background Relative apical sparing (RAS) of left ventricular (LV) longitudinal strain (LS) is a red flag marker for the suspicion of amyloid cardiomyopathy. However, it has also been described in patients with severe aortic stenosis (AS). Aim To assess the prevalence of RAS in patients with severe symptomatic AS referred for surgical aortic valve replacement (AVR), to evaluate its clinical significance and assess its presence after surgery. Methods We prospectively studied 135 consecutive patients (age: 73 y [IQR 68–77 y], 49% men) with severe symptomatic AS – mean transaortic pressure gradient (AVmean): 60.9±17.7 mmHg; mean aortic valve area: 0.7±0.2 cm2, referred for surgical AVR with no previous history of ischemic cardiomyopathy or other. Beyond 12 lead-ECG and transthoracic echocardiography (TTE), all patients underwent cardiac magnetic resonance (CMR) before surgery. RAS was defined by the ratio >1 of average LS at apical segments/sum of the average basal and mid LS at speckle tracking analysis. AVR with septal myocardial biopsy, for investigational purposes, was performed in 80 patients. AS severity indexes, LV remodeling and tissue characterization parameters were compared in both groups of patients, with and without RAS. LS deformation pattern was reassessed at 3–6 months after AVR. Results RAS was present in 24 patients (18%). In the whole cohort there were neither pseudoinfarct pattern or low voltage ECG criteria, nor infiltration suspicion from CMR (native T1 value 1053 ms [IQR 1025–1071 ms] for institutional reference values: 972–1070 ms; ECV 24% [IQR 21–27%]). None of the patients had amyloid deposition at histopathology. Overall, mean CMR LV ejection fraction (LVEF) was 59.6±10.5% and 98 patients (74%) had non-ischemic delayed enhancement, with a median fibrosis fraction of 4.1% [IQR 1.6–7.8%]. RAS cohort had a significantly higher AVmean gradient, relative wall thickness, maximum septal thickness, peak systolic dispersion, with lower global LS at TTE, as well as higher LV mass and lower LVEF at CMR. RAS group has also higher NT-proBNP ambulatory values (Table 1). Follow-up evaluation after AVR revealed RAS disappearance in 19 patients (79.2%). Conclusions RAS occurs in almost one-fifth of the patients in this cohort despite the absence of signs of myocardial infiltration. This deformation pattern elapses with worse indexes of LV remodeling consistent with a more advanced stage of the disease, being reversible after AVR, which stands for the absence of concomitant myocardial infiltration. Funding Acknowledgement Type of funding sources: None.

Background Left ventricular myocardial work (LVMW) is a novel method to evaluated left ventricular (LV) function using pressure-strain loops. It might correct global longitudinal strain (GLS) for afterload, being eventually useful to assess whether GLS reduction is due to reduced contractility (reflected as reduced myocardial work) or increased afterload (reflected as increased myocardial work). Aim To describe indices of LVMW in a group of patients with severe symptomatic aortic stenosis (AS). Methods We prospectively studied 104 consecutive patients (age: 71 years [IQR 66.5–75.5] years, 51% men) with severe symptomatic high gradient AS: mean transaortic pressure gradient: 56.5mmHg [IQR 46.8–67.8]; aortic valve area: 0.73cm2 [IQR 0.61–0.88]; indexed stroke volume: 47.7±1.3 mL/m2 (11 patients with low-flow AS), preserved LV ejection fraction (EV) (LVEF: 56.0% [51.0–61.3]; GLS: −14.5% [IQR −16.1 to −10.6]), with no previous coronary artery disease and no history of cardiomyopathy. Beyond complete transthoracic echocardiography, all patients underwent cardiac magnetic resonance for LV myocardium tissue characterization. As proposed for AS, LV systolic pressure was corrected adding the mean transaortic pressure gradient to non-invasive systolic blood pressure cuff measurement in the echocardiographic algorithm. Four LVMW indices were collected in 83 patients (patients excluded for atrial fibrillation, left bundle branch block or absence of non-invasive blood pressure registration) and correlated to LV function indexes, LV hypertrophy and remodeling, myocardial tissue characterization, BNP and troponin levels (Pearson or Spearman correlation). These same indexes were compared in patients with LV ejection fraction (EF) below and above 50%, normal and reduced flow and presence of replacement fibrosis. Results Global constructive work (GCW) (2658.6±76.4mmHg%), global myocardial work (GMW) (2218.7±74.9mmHg%) and global wasted work (GWE) (262.0mmHg% [198.8–339.5]) were high above normal with concomitant lower work efficiency (WE) (88.0% [83.2–91.8]. Weak correlations were found between LVMW indexes and parameters describing aortic valve severity, flow and LV function (table). Except for significant differences of LVMI in patients with reduced LV ejection fraction (GCW 2770.3±687.4 vs 2056.0±380.7mmHg%, p=0,014 and GMW 2362.5±657.9 vs 1621.3±319.9, p=0,021 in patients with LV EF>50% vs. LV EF Conclusions Global constructive and myocardial work are increased in these patients with severe aortic stenosis. This might reflect an increased afterload predominance rather than a LV functional impairment, particularly relevant in this group of patients with exclusive high gradient disease and preserved LVEF. Funding Acknowledgement Type of funding sources: None. Correlations between LVMI – LV function

Background Right ventricular (RV) function in aortic stenosis (AS) has been largely neglected. Recently it was demonstrated that right ventricular impairment may be influenced by left ventricular (LV) function and afterload, well before overt pulmonary hypertension development. Aim To describe the prevalence of RV dysfunction in a group of patients with severe symptomatic aortic stenosis (AS) and its relation to LV function parameters and afterload. Methods We prospectively studied 93 consecutive patients (age: 73 years [IQR 68–77] years, 55% women) with pure severe symptomatic high gradient aortic stenosis: mean transaortic pressure gradient: 57.0mmHg [IQR 46.9–71.1]; aortic valve area: 0.72cm2 [IQR 0.61–0.88]; indexed stroke volume: 48.8±1.5 mL/m2 (11 patients with low-flow AS), preserved LV ejection fraction (EV) (LVEF: 56.0% [51.0–61.3]; GLS: −14.5% [IQR −16.1 to −10.6]), with no previous coronary artery disease and no history of cardiomyopathy. Beyond complete transthoracic echocardiography, all patients underwent cardiac magnetic resonance (CMR) for LV myocardium tissue characterization (late gadolinium enhancement and extracellular volume). Normal RV function was defined according to TAPSE ≥17mm, tricuspid annular systolic velocity ≥12cm/s, mean free wall longitudinal strain ≤−20%. Patients were divided into four groups: (0) – all three RV parameters below normal (1.1%), (1) – 1 normal parameter (12.9%), (2) – 2 normal parameters (44.1%), (3) – 3 normal parameters (41.9%). Indexes of LV systolic and diastolic function, CMR derived LV geometric remodeling, hypertrophy and tissue characterization, aortic valve disease severity and afterload were compared across the 4 groups of patients. We tried to identify predictors of RV dysfunction (group 0, 1, 2 vs. group 3) at multivariate regression analysis. Results Left ventricular performance parameters, diastolic and myocardial work indexes were significantly different across the groups (Figure). Neither AV severity indexes nor LV tissue characterization were distinct. At multivariate analysis only global constructive work was an independent predictor of RV dysfunction. Conclusion RV dysfunction is common in this group of patients with severe high gradient aortic stenosis and preserved ejection fraction. RV impairment is significantly related to several LV systolic and diastolic parameters and also to LV afterload, probably accounting for RV-LV interdependence. Funding Acknowledgement Type of funding sources: None.

Aims: To describe cardiac remodeling in a population of male master athletes evaluated by transthoracic echocardiography and to analyse its relationship with several exercise-related characteristics.Methods and results: A total of 105 male master athletes aged ≥40 years old, mostly involved in endurance sports (81.0%) with a median training-volume of 66 [44; 103] METs/h/week, were studied. Left ventricular end-diastolic and end-systolic volumes were above the references in 84.8% and 75.8% athletes, decreasing in frequency when adjusted for BSA (26.3% and 23.2%). LV geometry was changed in more than half of the athletes (eccentric hypertrophy 28.3%, concentric remodelling 15.2% and concentric hypertrophy 8.1%) and several right ventricular (RV) dimensions were increased. Left atrium was dilated in 53.5% and right atrium in 37.4% athletes; only one athlete had a dilated aorta. Mean LV ejection fraction was 61±7% and global longitudinal strain -18.3±2.0%. Changes in LV geometry were more common in high intensity sports; LV dilation in athletes exercising >10 hours/week and in high intensity sports; RV dilation in athletes exercising >66 MET-hour/week and in endurance sports. In multivariate analysis high intensity sports remained an independent predictor of changes in LV geometry. There was a significant correlation between volume of exercise and cardiac structural adaptations.Conclusions: Cardiac structural adaptations were frequent in male master athletes, more pronounced in those involved in endurance sports, with high intensity and high volume of exercise. This data reinforces the concept that the characteristics of exercise are major determinants of cardiac remodeling and should be considered during athletes’ evaluation.

Methods We assessed 238 consecutive patients (154 male, mean age 64+/11 years) undergoing adenosine stress cardiac MR in a single center between January 2012 and July 2014. Significant incidental results were defined as any previously unknown non ischemic finding with potential clinical and/or therapeutic implications (all patients had an previous cardiac ultrasound done within the previous 5 months). The exams were performed on a 1.5T MR equipment and according to the SCMR guidelines for a adenosine stress cardiac MR. Interpretation was done by agreement between a Radiologist and a Cardiologist with level III equivalent certification.

Risk stratification has become increasingly important in the management of patients with suspected or known ischaemic heart disease (IHD). Recent guidelines recommend that these patients have their care driven by risk assessment. The purpose of this position statement is to sum- marize current evidence on the value of cardiac single-photon emission computed tomography, positron emission tomography, and hybrid imaging in risk stratifying asymptomatic or symptomatic patients with suspected IHD, patients with stable disease, patients after coronary revas- cularization, heart failure patients, and specific patient population. In addition, this position statement evaluates the impact of imaging results on clinical decision-making and thereby its role in patient management. The document represents the opinion of the European Association of Nuclear Medicine (EANM) Cardiovascular Committee and of the European Association of Cardiovascular Imaging (EACVI) and intends to stimu- late future research in this field.

The use of vascular ultrasonography (alone or combined with newer techniques like angio-magnetic resonance or angio-computed tomography) is an essential tool for the diagnosis and the assessment of vascular diseases. It is also useful for the follow-up after surgical or endovascular interventions (avoiding the need to use angiography in any therapeutic decision in most cases). The integration of two-dimensional echocardiography, colour flow imaging and spectral Doppler makes the morphological and functional assessment of vascular disease possible in almost every territory.For a long time, vascular ultrasonography was exclusively performed by non-cardiologists. Nowadays, in modern echo laboratories, vascular echography is frequently performed by cardiologists, often in cooperation with vascular surgeons and radiologists.In this chapter, we review the essential concepts of the use of vascular ultrasound imaging in the study of the territories that are most commonly evaluated: ◆ Cerebrovascular circulation. ◆ Abdominal circulation. ◆ Lower limb circulation (arterial and venous disease).In each of these sections, we describe the technical details of the ultrasonic examination, the normal recordings, the abnormal findings of specific diseases/syndromes affecting each territory, and the postoperative/post-interventional evaluation and follow-up.

Mesenchymal/stromal stem cells (MSC) have unique properties favorable to their future use in clinical practice and have been studied for cardiac repair. MSC are larger than coronary microvessels and there is controversy about the risk of embolization and microinfarctions, which could compromise the intracoronary route for their delivery. The index of microcirculatory resistance (IMR) is an invasive method for quantitatively assessing the coronary microcirculation status and could be applied for evaluation of the acute effects of MSC intracoronary infusion providing new insights for this open question. Our purpose was to examine heart microcirculation effects after MSC intracoronary injection with IMR. Methods and Results: Eighteen healthy swine were randomized to receive 30 x 106 MSC or saline by intracoronary infusion. In the heart catheterization laboratory hemodynamic, electrocardiographic and coronary flow parameters were monitored and there were no differences between groups. Thermodilution-derived coronary flow reserve (CFR) and IMR were assessed at baseline, 5 and 30 minutes post-delivery using a coronary pressure wire. CFR had a small although not significant decrease in animals receiving MSC. After cell infusion, IMR increased at 5 and 30 minutes with a significant difference from the control group at 30 minutes (Table). Conclusion: Intracoronary delivery of MSC is possible without a significant negative impact on hemodynamic parameters, electrical stability and epicardial coronary flow status. However, this study provides definitive evidence of microcirculatory disruption upon intracoronary administration of MSC, in a large animal model closely resembling human cardiac physiology, function and anatomy. | | CONTROL | MSC | p | || | Baseline CFR | 3.8 ± 1 | 4 ± 2 | 0.7 | | Baseline IMR (U) | 8.1 ± 1 | 6.7 ± 0.6 | 0.3 | | Post-delivery CFR - 5 min. | 3.6 ± 1 | 3 ± 2 | 0.5 | | Post-delivery CFR - 30 min. | 3.8 ± 2 | 2.3 ± 1.5 | 0.064 | | Post-delivery IMR - 5 min. (U) | 9.8 ± 1 | 15.3 ± 2.8 | 0.08 | | Post-delivery IMR - 30 min. (U) | 8.8 ± 1 | 13.2 ± 1.8 | 0.02 | * Coronary flow reserve (CFR), index of microcirculatory resistance (IMR) CFR and IMR mean values

TTR Met30 Familial Amyloidotic Polyneuropathy of the Portuguese type (FAP) is an incapacitating and lethal hereditary disorder that affects predominantly young adults of both genders. Portuguese type FAP patients have sensory, motor and autonomic polyneuropathy. The generalised systemic amyloid infiltration involves the heart, leading to the characteristic granular bright sparkling echocardiographic pattern. LV wall thickening occurs in the late phases of the disease. LV diastolic dysfunction has been reported in the absence of systolic dysfunction; an abnormal diastolic transmitral flow pattern assessed by pulsed wave Doppler (PW) was described. PW is very much dependent on load conditions. Tissue Doppler imaging (TDI) has been used as a more reliable method to assess long axis diastolic function.1--To identify the incremental value of TDI in the assessment of diastolic function in FAP. 2--To correlate diastolic pattern abnormalities and left ventricular mass index (LVMI) in FAP patients.We performed a prospective evaluation of 24 consecutive FAP patients and selected 14 (sinus rhythm, age45 years). Diastolic function was assessed by PW and classified as normal (GI-E/A1) or abnormal (GII-E/A1). TDI was performed in 4 sites of the mitral annulus (septum, lateral, inferior, anterior). Velocities of the rapid filling wave (E') and atrial contraction wave (A') were measured and E'/A' calculated. In each site we considered the TDI as normal (E'/A'1) or abnormal (E'/A'1). The LVMI was calculated by Devereux's formula.Age, gender and heart rate were similar in both groups. TDI at the septal mitral annulus was normal in all of the GI patients (E'/A': 1.29 +/- 0.19) and suggestive of abnormal LV relaxation in all of the GII patients (E'/A': 0.82 +/- 0.11, p0.0001). TDI revealed abnormal diastolic pattern when a restricted number of sites of the mitral annulus were assessed, even in GI patients and before PW abnormalities occurred. Fractional shortening (FS) and LVMI were similar in GI and GII (FS-GI: 45.5 +/- 5.3, GII 43.5 +/- 8.1%, p: NS; LVMI--GI: 66 +/- 9.3, GII: 67 +/- 3.0 g/m2 p: NS).The assessment of mitral annulus motion has introduced new data in the study of diastolic function of FAP patients. An abnormal LV relaxation pattern occurred early in the evolution of the disease in patients with normal LVMI and systolic function.

The assessment of the mitral annulus motion with tissue Doppler imaging is claimed to be an accurate method to quantify global left ventricular systolic and diastolic function. However, it is not yet perfectly defined which site of the annulus must be selected. Familial amyloidotic polyneuropathy of the Portuguese type (FAP) is an hereditary systemic disease in which diastolic dysfunction may occur.1--To determine if in FAP patients the mitral annulus motion is independent of the selected site. 2--To compare pulsed wave Doppler parameters with tissue Doppler parameters in the different annular sites.Of 24 FAP patients studied, 14 were included. In each patient we performed conventional transmitral pulsed wave Doppler and tissue Doppler in the 4 sites of the mitral annulus and measured the velocities of the rapid filling wave e, of the atrial contraction wave a and calculated e/a ratio.According to the transmitral inflow profile, patients were divided in 2 groups: Group I--normal global diastolic function and Group II--abnormal relaxation. Group I--33% of these patients showed e/a1 in the four sites and 67% showed e/a1 in at least 1, but not in all the sites. The rate of normal sites per patient was 3.1. Group II--25% of these patients showed e/a1 in the 4 sites of the annulus and 75% had e/a1 in at least 1, but not in all the sites analysed. The rate of abnormal sites/patient was 3.1. in this group. When conventional and tissue Doppler data were compared (bland and altman) the septal portion of the annulus was the one with the best correlation.1--The assessment of the mitral annulus motion with tissue Doppler imaging is dependent on the site selected for study. 2--The septal site was the one that showed the highest correlation and concordance between pulsed wave Doppler and tissue Doppler. 3--The relative number of normal versus abnormal sites was determinant of the transmitral pattern. 4--Tissue Doppler imaging identified: a) among patients until now classified as normal diastolic function, a subgroup of patients with abnormal function in some sites of the annulus and b) among patients with abnormal relaxation, a subgroup with normal diastolic function in some sites of the annulus.

Infective endocarditis continues to attract the attention of many physicians. The authors present a case report of a 64 year old man with a history of infective endocarditis of a native aortic valve complicated by a perivalvular abscess, mycotic aneurysm of a sinus of valsalva and acquired aorta-right ventricular shunt.

Tissue Doppler echocardiography (TDE) is a recent ultrasonic technique. One of its potential clinical uses is the assessment of regional diastolic function.To compare left ventricular global diastolic function with interventricular septum regional diastolic function in patients with hypertrophic cardiomyopathy with different levels of global diastolic dysfunction.10 normal subjects (group 1), eight hypertrophic cardiomyopathy patients with abnormal relaxation (group 2) and 12 patients with hypertrophic cardiomyopathy with restrictive pattern (group 3) on the mitral inflow profile (pulsed wave Doppler) were studied. Each patient performed an M-mode color; TDE of the basal septum in the four chamber view. Velocities during the rapid filling phase (RF), and during atrial contraction (AC) were measured and RF/AC ratio calculated in each patient.While in normal subjects (group 1) the RF/AC ratio was always higher than 1, in patients with hypertrophic cardiomyopathy patients with different levels of global diastolic dysfunction (groups 2 and 3), the RF/AC ratio was always lower than 1 (velocities during atrial contraction always higher than during rapid filling), despite the level of global diastolic dysfunction.A ratio between myocardial velocities during the rapid filling phase and during atrial contraction lower than one (RF/AC1) is a tissue Doppler echocardiography index of regional diastolic dysfunction, independently of the severity of global diastolic dysfunction.

As the number of adults surviving radiation therapy for Hodgkin's disease increases, the long-term clinical sequelae of such treatment is becoming increasingly more evident. Mediastinal radiation has been linked to acute and chronic pericarditis (with or without pleural effusion), coronary artery disease, myocardial fibrosis, valvular dysfunction and arrhythmias. The AA describe a case of a young patient with evidence of significant valvular disease following mediastinal irradiation, and review the radiation-induced cardiotoxicity, discussing aspects concerned with pathophysiology, diagnosis and therapy.

Purpose: To assess and compare the prognostic value of coronary CT angiography (CCTA) and exercise ECG (ExECG) in patients with suspected stable angina pectoris. Methods: Patients with suspected coronary artery disease (CAD) who had undergone both ECG exercise stress testing (ExECG) and CCTA (within 180 days) were identified retrospectively in a single center registry. The presence of 50% or greater coronary stenosis was assessed with CCTA. ExECG results were classified as normal, ischemic, or nondiagnostic. Patients were followed-up for major adverse cardiac events (MACE), defined as cardiac death, nonfatal myocardial infarction, unstable angina requiring hospitalization, and revascularization >90 days after index CCTA. Univariable and multivariable Cox regression analysis was used to determine the prognostic values, and discriminative power was assessed with continuous net reclassification improvement (cNRI). Results: Follow-up was completed for 467 patients (88%), with a median duration of 2.6 years (IQR 2.0-3.8). A total of 23 patients experienced MACE, including 3 deaths and 2 non-fatal myocardial infarctions. In the univariable model, modified CAD Consortium 2 score (HR 1.05, 95% CI 1.02-1.08), non-obstructive CAD (HR 5.63, 95% CI 1.25-25.43) and obstructive CAD (HR 15.43 CI 3.38-70.45) were predictors of MACE. In the multivariable model, only CCTA findings remained independently predictive of MACE. Addition of CCTA results to the clinical and ExECG model yielded a cNRI of 70% (222 reclassified upward, 245 downward). ![Figure][1] Conclusions: CCTA findings are strongly predictive of MACE. Its prognostic power seems superior and incremental to clinical and exercise ECG data. [1]: pending:yes

Purpose: To evaluate the performance of classic risk factors, alone or associated in scores, in identifying a higher than expected coronary atherosclerotic burden. Methods: We assessed 2069 consecutive patients (56% men, mean age 58±11 years) undergoing coronary CT angiography (CCTA) with Calcium Score (CaSc) for suspected coronary artery disease. A higher than expected atherosclerotic burden was defined as a CaSc >75th percentile (CaSc>p75) according to age and sex-adjusted nomograms. The ability of traditional risk factors to predict a CaSc>p75 was assessed in a customized logistic regression model (clinical score) and by the calculation of the HeartScore. The Population Attributable Risk (PAR) of the various risk factors for CaSc>p75 was also calculated. Results: The median CaSc was 3.0 [IQR 0.0-98.0], with 362 patients (17.5%) having CaSc>p75. The median HeartScore was 3.0 [IQR 1.0-4.0]. With the exception of hypertension, all classic risk factors were independent predictors of CaSc>p75: diabetes mellitus, dyslipidemia, smoking and family history (all OR 1.3-2.2, p≤ 0.026). The areas under the ROC curves to predict CaSc>p75 were 0.64 for the clinical score (95% CI 0.61-0.67, p p75 were in the two lower quartiles of the clinical score (Figure). Altogether, the various classical risk factors seem to explain only 56% of the prevalence of CaSc>p75 (adjusted PAR 0.56). Conclusion: Despite the statistical association of CV risk factors with a higher than expected atherosclerotic burden, they seem to justify only half of its prevalence. Even when integrated in scores, the predictive power of these risk factors is relatively modest, exposing the limitations of a risk stratification based solely on demographic and clinical risk factors.

Until a few years ago, therapy of refractory Hypertrophic Obstructive cardiomyopathy was mainly surgical--Morrow's myotomy/myectomy or mitral valve replacement. Despite the good results of these techniques, they are not free of mortality and morbidity. In the last years a new and promising therapy has been developed: the Dual Chamber Pacemaker. Technically easier and less invasive than surgery, this therapy has achieved better results and lower mortality and morbidity during the follow-up.

An evaluation was undertaken regarding two female patients suffering from obstructive hypertrophic cardiomyopathy, with high and strongly symptomatic gradients, as well as evidencing a resistance to medication with beta-blockers, verapamil and disopyramide when administered in maximal doses. These patients were provided with the implant of a definitive type DDD pacemaker, with an auricular electrocatheter placed on the right auricular appendix, and with a bipolar ventricular catheter placed on the apex of the right ventricle. The generator was programmed with a short AV so as to ensure that the ventricular stimulation would at any given time be the result of the ventricular contraction would be initiated at the apex portion of the right ventricle. With these therapeutics, we observed not only a reduction or even the gradient, but also the complete elimination of the existing symptoms--angor, dyspnea, dizziness, palpitations and fainting--with the resulting normalcy of the quality of life of the patients. All taken into account we are of the opinion that these therapeutics are, in the case of patients suffering from obstructive hypertrophic cardiomyopathy, a valid alternative for surgical treatment by means of myectomy or myotomy, but without the morbidity and mortality rates presented by such methods.

Authors report one case of myocardial infarction in a young man 23 years old. Prevalence of acute myocardial infarction under the age of 35-40 years is not negligible. Pathogenic mechanisms, risk factors, clinical presentation, prognosis, and findings in selective coronary angiography are reviewed.

Sudden death is a very important event in the natural history of hypertrophic cardiomyopathy. Although its physiopathology is not still fully understood, there are several potential mechanisms to explain it, such as electrical, ischemic and hemodynamic events. It is thought that these mechanisms vary from patient to patient and that more than one mechanism may coexist in a specific patient. Risk factors for sudden death in hypertrophic cardiomyopathy are clinical, genetical and electrical. Risk stratification implies aggressive investigation (electrophysiological study and/or forearm plethysmography during exercise and/or tilt test) in order to identify the most probable mechanism involved in each case and to select individualized preventive measures (pharmacological, surgical, implantable cardioverter defibrillator). The role of amiodarone is still controversial because of contradictory results with this drug in this illness.

Summary The aim of treatment in cases of hypertelorism is to obtain an aesthetically normal appearance rather than a normal interorbital distance. The new line of osteotomy to simplify the technique is presented; the operating time was much reduced, thus decreasing the operative risks, especially in children; bone grafts to the temporal region and nose are not necessary, no iliac crest or rib graft is employed; normal aesthetic appearance of the fronto-naso-orbital area is obtained.