Claudia Visconti

Magnetic resonance in isolated noncompaction of the ventricular myocardium

UNLABELLED Non-compaction of the ventricular myocardium (LCVM) is a rare disorder of myocardial morphogenesis usually diagnosed in paediatric age. The diagnosis was echocardiographically made on the basis of a reported spongeous/compacted ratio >2 in one or more segments of the left ventricle during the diastolic period. We aimed to test the diagnostic accuracy of cardiovascular magnetic resonance (CMR) imaging in distinguishing pathological left ventricular non-compaction. METHODS We collected a consecutive series of 8 patients, 5 males and 3 females, with a mean age of 14.9 years with non-compaction of left ventricular myocardium. All patients were admitted in our divisions of cardiology. In all cases the diagnosis was performed by echocardiography. The diagnosis was obtained when the spongeous/compacted ratio was >2 in one or more segments of left ventricle, evaluated in systolic and diastolic period. In the end we completed the diagnosis by scanning with a Signa HD 1.5 T (GE, Milwaukee, USA) the same 8 patients affected by non compaction of ventricular myocardium. In all patients cardiac-gated T1 and T2 black-blood FSE images in short axis and in four-chamber horizontal long axis were obtained. Breath hold cine MR sequences (FIESTA) were performed, covering the whole left ventricle in short-axis plane and in four-chamber view. A segmented inversion-recovery fast gradient echo sequence (IR-FGE) was performed in the short-axis plane of the LV and in four-chamber-view after Gadolinium injection in 8 patients affected by non compaction of left ventricle. At the end of examination the spongeous/compacted ratio >2 was calculated in all involved segments of the left ventricle in diastole. RESULTS In all cases we demonstrated by echocardiography an involvement of the ventricular apex. In 3 cases the structural alterations involved also lateral wall of left ventricle. Magnetic resonance evaluation showed that involvement demonstrated by the echocardiogram was the same: ventricular apex involved in every patient, lateral wall in 3 and all segments in 2. However the spongeous/compacted ratio was >>2 in all patients, with a mean value of 3,1. CONCLUSIONS Although our data refer to a small population of patients and need further confirmation, they suggest that it seems reasonable increase the cut-off for spongeous/compacted ratio from a value of 2 to 2.5 for non-compaction diagnosis when high-resolution magnetic resonance is used.

Anticoagulant drugs in noncompaction: a mandatory therapy?

Background Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications. Methods The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation. Results The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcets disease (high risk of thrombi formation). Conclusion Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.

Ventricular dysfunction and number of non compacted segments in non compaction: non-independent predictors.

BACKGROUND Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC. METHOD To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Società Italiana di Ecografia Cardiovascolare) registry. The average age of patients was 41.5 years (range: 1-92 years), 137 were males and 101 females. In 122 cases we found ventricular systolic dysfunctions with an EF average of 34.6%. The number of affected segments by non-compactation and diastolic dysfunction were found to be non-independent predictors of LV systolic dysfunction. CONCLUSION From the analyses we carried out, it seems that ventricular dysfunction seems to be completely independent from the segment numbers of non compacted segments.

Asymptomatic carotid lesions add to cardiovascular risk prediction

Aim To show that subclinical atherosclerosis (subclinical-ATS) of carotid arteries [intima–media thickness (IMT) or asymptomatic carotid plaque (ACP)], may provide additional information for risk stratification, in asymptomatic patients, aged greater than 45 years, with a cluster of risk factors (RFs). Methods and results We studied 558 asymptomatic patients (235 males). RFs for atherosclerosis were assessed and the 10-year-risk was calculated according to the Italian risk score. Doppler ultrasound of carotid arteries identified the presence of IMT greater than 0.9 mm in 183 patients and ACP in 147 patients. One hundred and fifty-three patients developed cerebrovascular or cardiovascular (CV) events in the follow-up: 67 developed acute myocardial infarction, 39 developed angina, 25 had a stroke or transient ischemic attack, six died for CV events, and 16 underwent percutaneous or surgical revascularization. The incidence reflected the different risk profiles (4,14, and 20%, respectively). However, in patients with baseline subclinical-ATS the incidence of events increased to 35, 46, and 63%, respectively. In the multivariate analysis the incidence of events was significantly influenced by the presence of asymptomatic carotid lesions in each risk category. Conclusion In our experience, the incidence of CV events is enhanced in patients with subclinical-ATS. Increased IMT and ACP predict CV events and improve the risk stratification of asymptomatic patients aged greater than 45 years and with a cluster of RFs, in a long-term follow-up.

Early subclinical ventricular dysfunction in patients with insulin resistance.

Aims The aim of our study was to evaluate the relationship between insulin resistance and the detection of precocious echocardiographic signs of heart failure in patients with cardiovascular risk factors. Methods We enrolled 34 consecutive patients with cardiovascular risk factors. All patients underwent coronary angiography, echocardiography, and laboratory tests. Exclusion criteria were diabetes (fasting glucose greater than 126 mg/dl or treatment with insulin or oral hypoglycemic agents), coronary artery disease, creatinine above 1.5 mg/dl, left-ventricular hypertrophy, valvular heart disease, ejection fraction below 50%, atrial fibrillation, or other severe arrhythmia. The presence of insulin resistance was assessed by using the Homeostasis Model Assessment of Insulin Resistance (HOMA-IR). Ventricular function was investigated by echocardiography. Results Distinguishing patients with insulin resistance, based on the median value of HOMA-IR (<4.06 and >4.06), we observed that in the group with higher levels of HOMA-IR, there were echocardiographic signs of subclinical ventricular dysfunction statistically more frequent (E/A in group with HOMA <4.06: 1.159 + 0.33 vs. group with HOMA >4.06: 0.87 + 0.29, P = 0.0136; E/E’: 6.42 + 4 vs. 15.52 + 3.26, P = 0.001; Tei index: 0.393 + 0.088 vs. 0.489 + 0.079, P = 0.0029; S wave: 0112 + 0.015 vs. 0.114 + 0.027, P = 0.0001; ejection fraction 59.11 + 4.75 vs. 58.88 + 6.81, P = 0.9078). Grade II diastolic dysfunction was observed in 5 patients, grade I in 12 patients, and 17 patients had normal diastolic function. On multivariate analysis, HOMA-IR (P = 0.0092), hypertension (P = 0.0287), waist circumference (P = 0.0009), high-density lipoprotein (P = 0.0004), and fasting blood glucose (P = 0.0003) were variables independently associated with diastolic dysfunction. On analysis of covariance, we found that the variables that influence diastolic dysfunction are HOMA-IR, waist circumference, BMI, and age, and that the only variable that influences Tei index is HOMA-IR. Conclusion Insulin resistance is frequently associated with subclinical left-ventricular dysfunction. Patients with cardiovascular risk factors and increased HOMA-IR levels, although without diabetes mellitus, overt coronary artery disease, or hypertensive cardiomyopathy, may represent a target population for screening programs, recommended changes in lifestyle, and possibly the use of pharmacological interventions to prevent the onset of heart failure.

Atherosclerosis, Degenerative Aortic Stenosis and Statins

Aortic stenosis is the most common valvular heart disease among adult subjects in western countries The current treatment for aortic stenosis is aortic valve replacement. The possibility of a medical treatment that can slow the progression of aortic stenosis is very fascinating and statins have been tested to reduce the progression of degenerative aortic stenosis (DAS). The rationale for statin treatment in DAS has a deep pathophysiological substrate, in fact inflammation and lipid infiltration constitute the same histopathological pattern of both aortic stenosis and atherosclerosis and these two conditions have the same risk factors. Whether retrospective studies have shown some efficacy of statins in halting the progression of DAS, prospective trials have shown controversial results. A recently published large and randomized controlled trial SEAS found that statins have no significant effect on the progression of aortic stenosis, the ASTRONOMER, recently confirmed this data. The most plausible hypothesis is that coronary artery disease and DAS, have a common pathogenetic background and a distinct evolution due to different factors (mechanical stress, genetic factors, interaction between inflammatory cells and calcification mediators). Thus, treatment with statins is not recommended in patients with valvular aortic stenosis and without conventional indications to lipid-lowering treatment.

Takotsubo Syndrome After Mitral Valve Replacement: Case Report and Brief Review of the Literature

RANSIENT LEFT VENTRICULAR apical ballooningsyndrome, also known as Takotsubo or stress-inducedcardiomyopathy, is a cardiac disease characterized by transientleft ventricular dysfunction, electrocardiographic changes mim-icking an acute coronary syndrome, and release of myocardialcellular necrosis enzymes in the absence of significant lesionsof coronary arteries.

Pharmacological Therapy in Children with Atrial Fibrillation and Atrial Flutter

Heart rhythm disorders in children are not different, on electrocardiographic trace, from heart rhythm disorders in adults with the exception of incidence which is different according to the age. Paticularly, atrial flutter (FlA) and fibrillation (FA) are very uncommon arrhythmias in the general pediatric population. Generally atrial fibrillation and atrial flutter, in our experience, is a temporary heart rhythm disturbance connected to specifical and resovable reasons with the exception of Fontains surgical correction of congenital heart diseases or cardiopathies with dilatation of both atria. Presenting symptoms, symptom history (e.g., frequency, duration, and severity), risk assessment, previous response to alternative treatment options, convenience and patient preference for a specific treatment option, and costeffectiveness of a treatment option are among the many factors that should be considered. Treatment of atrial flutter and fibrillation in pediatric age involves several options: Pharmacological therapy, Transoesophageal atrial pacing (TEAP), Electrical cardioversion and Catheter ablation. In this review we evaluated the physiopathology, the clinical features and the current terapeutical strategies for these arrythmias in paediatric age.

Diagnosis and definition of biventricular non-compaction associated to Ebstein's anomaly

BACKGROUND Non-compaction of ventricular myocardium is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining the typical spongeous aspect. The diagnosis of non-compaction of ventricular myocardium is possible through the identification of morphological alterations by echocardiographic evaluation. Ebsteins anomaly is a rare congenital cardiac disease, defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. CASE REPORT We present a case of biventricular non-compaction and Ebsteins anomaly in a 29-year-old Italian man that was referred for chest pain. Diagnosis of Ebsteins anomaly was made during a medical control for military service through an echocardiographic evaluation which left the suspicion of myocardium non-compaction. We present the cardiac image of the 2D and 3D eco, RMN, scintigraphy and ventriculaography.

Neuromuscular disorders and non compaction: How much is the strength of the association and how can it be suspected?

In many reports Finsterer and Stöllberger reported a strong association between non compaction of the left ventricle and neuromuscular disorders. In the same report the authors described a neurological involvement in more than 50%. Recently we published our personal experience, about 21 paediatric patients: only 4 patients (19%) showed a neuromuscular disorder, and only 1 of them showed an increased plasmatic level of CK, and in particular of MM isoform, with a normal level of CK-MB. None presented high levels of troponine. Through the experience of 3 centres we collected 61 patients affected by non compaction that performed a neurological control, and only 14 (21%) were affected by neuromuscular disorders. A correlation between neuromuscular disorders and cardiac non compaction is present, even if, until today, genetic involvement has not been identified clearly. However, in our opinion, an estimated incidence of 50% of neuromuscular disorders in this population of patients could be too exaggerated. About the prognostic value of the CK elevation, it is interesting to consider that an increasing of CK plasmatic level is an expression of muscular disorders and not of cardiac alterations. In our experience only 1 patient in 21 patients (4%) with neuromuscular disorders showed an increasing of CK-MM plasmatic value.