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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.

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.

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.