Francesca Martone

Hypertrophic Cardiomyopathy in a Girl With Cornelia de Lange Syndrome Due to Mutation in SMC1A

Hypertrophic Cardiomyopathy in a Girl With Cornelia de Lange Syndrome Due to Mutation in SMC1A Giuseppe Limongelli,* Silvia Russo, Maria Cristina Digilio, Maura Masciadri, Giuseppe Pacileo, Fiorella Fratta, Francesca Martone, Valeria Maddaloni, Raffaella D’Alessandro, Paolo Calabro, Maria Giovanna Russo, Raffaele Calabro, and Lidia Larizza Department of Cardiology, Monaldi Hospital, Second University of Naples, Naples, Italy Istituto Auxologico, Milan, Italy Medical Genetics, Bambin Gesu’ Hospital, Rome, Italy Department of Medicine, Surgery and Dentistry, University of Milan, Milan, Italy

Acute and Chronic Effects of Noninvasive Ventilation on Left and Right Myocardial Function in Patients with Obstructive Sleep Apnea Syndrome: A Speckle Tracking Echocardiographic Study

In patients with obstructive sleep apnea syndrome (OSAS), repetitive hypoxia due to sleep‐induced apnea adversely affects the interaction between myocardial oxygen demand and supply, resulting in the development of subclinical cardiac dysfunction. The purpose of the study was to analyze the different involvement of left and right heart myocardial function in patients with OSAS treated with noninvasive ventilation (NIV).

Cardiac damage in athlete’s heart: When the “supernormal” heart fails!

Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete’s blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias. Athlete’s heart is a physiological condition and does not require a specific treatment. In some conditions, it is important to differentiate the physiological adaptations from pathological conditions, such as hypertrophic cardiomyopathy, arrhythmogenic dysplasia of the right ventricle, and non-compaction myocardium, for the greater risk of sudden cardiac death of these conditions. Moreover, some drugs and performance-enhancing drugs can cause structural alterations and arrhythmias, therefore, their use should be excluded.

Which is the correct management of patients with asymptomatic severe calcific aortic stenosis after symptomatic spontaneous calcium cerebral embolism

Elective surgery cannot be recommended in every patient with asymptomatic severe aortic stenosis, and predictors identifying high-risk patients need to be identified. In guidelines we read that elective surgery, at the asymptomatic stage, can only be recommended in selected patients, at low operative risk (see guidelines of European Society of Cardiology and American Heart Association), but we have not read any reference to patients with severe calcific aortic stenosis after symptomatic spontaneous calcium cerebral embolism. Because cardioembolic stroke is associated with poor prognosis compared to other stroke subtypes, in patients with asymptomatic severe aortic stenosis and spontaneous calcific embolic stroke valve replacement appears to offer the best hope of avoiding a recidivation of stroke and should be considered in most cases. On the contrary, is it still correct to consider as asymptomatic patients with severe aortic stenosis and cerebral thromboembolism from a calcified aortic valve?

Diagnosis, Management and Treatment of Septic Shock from Early Diagnosis to Infection Focus Control

Sepsis is a syndrome characterized by clinical signs and symptoms due to infection, with a high rate of mortality, especially if not recognized and treated promptly. In the last years, several definitions were explained about this syndrome. The aim of this review is to give a common and practical definition of septic shock, and to focus on diagnosis, early resuscitation and infection focus control.

Iatrogenic Delayed Cardiac Tamponade Secondary to Intrapericardial Hematoma after Dialysis Catheter Placement

An 84-year-old man affected by arterial hypertension, diabetes mellitus, chronic renal failure on dialysis, chronic ischemic heart disease, permanent atrial fibrillation, previous AICD implantation about one year before, was admitted to the intensive care unit because of typical chest pain and dyspnoea during dialysis treatment with nonspecific ST segment changes at ECG and mild increase of cardiac enzymes. A transthoracic echocardiogram was requested, and it demonstrated a big loculated paracardiac hematoma not easily distinguished from pleural source, with sprays of fibrin localized in correspondence of the free wall of the right ventricle determining a partial compression of the right ventricle with no signs of tamponade. Moreover, a chest CT scan with contrast medium was performed and it confirmed the presence of an intrapericardial hematoma located in correspondence of right heart chambers in the absence of contrast spearing post injection. Subsequently, during the following dialysis treatment the patient developed severe hypotension with chest pain, dyspnoea and paradox pulse treated with liquids infusion with prompt resolution of symptoms followed by a surgical drainage through midline sternotomy. The patient underwent operation and a large organizing thrombus was removed from the pericardial space anterolateral and inferior to the right atrium. The pericardium, which was not thickened, was not removed. His postoperative course was uneventful. This case shows that an intrapericardial hematoma several months following the initial bleeding can present with evolving clinical features and cause an impaired cardiac filling in condition of volume depletion such as dialysis treatment. Although two-dimensional echocardiography represents the first line diagnostic tool in this condition, chest CT scan with contrast medium is frequently used to evaluate patients for pericardial effusion and it is of value in these cases because it permits differentiation of an extracardiac from an intracavitary mass, precise determination of the extracardiac extent of a mass and characterization of a mass as a probable hematoma. Finally, we can conclude that a very low threshold for requesting complimentary imaging studies is essential for prompt diagnosis, and the selection of the diagnostic test depends on the urgency of the clinical presentation. Furthermore, the noninvasive information allowed the surgical team to plan an optimal approach to excising the mass.

Right Heart-Pulmonary Circulation Unit in Cardiomyopathies and Storage Diseases

Cardiomyopathies (CM) are a heterogeneous group of muscle heart diseases, divided into 3 main categories (dilated, hypertrophic, and restrictive). In addition to these subgroups, athletes heart and hypertensive cardiopathy are both the result of heart adaptation to increased loading conditions, making it possible to include them in the CM group. Right heart involvement is clear in some CM as arrhythmogenic CM, carcinoid syndrome, and endomyocardial fibrosis, whereas in others, like hypertrophic or dilated CM, it is known that the right heart has a prognostic impact but less clear is its pathogenic role.

Continuing Medical Education Activity inEchocardiographyAugust 2016

1Cardiology, Monaldi Hospital-AORN Ospedali dei Colli, Second University of Naples, Naples, Italy 2Division of Pneumology, Monaldi Hospital-AORN Ospedali dei Colli, Second University of Naples, Naples, Italy 3Institute of Biostructure and Bioimaging (IBB) of the Italian National Research Council, Naples, Italy 4Division of Cardiology, Evangelic Hospital Villa Betania, Naples, Italy 5Department of Cardiology and Cardiac Surgery, University Hospital San Giovanni di Dio e Ruggi d’Aragona, Salerno, Italy

ASSOCIATION BETWEEN CAROTID ARTERY STIFFNESS ESTIMATED WITH E-TRACKING AND DIASTOLIC DYSFUNCTION ASSESSED WITH TDI IN 105 PATIENTS WITH MASKED HYPERTENSION: PP.31.237

A normal blood pressure in the clinic or office but an elevated blood pressure out of the clinic is defined as masked hypertension. It may occurs in as many 10% of the general population. Increased arterial stiffness directly increase end-diastolic left ventricular pressure and compromise myocardial relaxation properties. Thanks to our study we can evaluate the role of carotid artery stiffness parameters on diastolic function in patients with masked hypertension. Methods: Observing 105 consecutive patients with masked hypertension (proved by 24 H- ambulatory blood pressure monitoring), we have noticed that diastolic early (E’) and late (A’) velocities were recorded by pulse wave tissue. Doppler imaging of the lateral mitral anulus in the apical 4- chamber view and carotid artery stiffness parameters were valued by e-tracking on the left carotid with ALOKA a 10 ultrasound system. Pulse wawe velocity (PWV), elastic pressure (Ep) and stiffness parameter (ß) were measured. Results: Pwv (m/s) 8+/-2, Ep (Kpa) 134+/-23, ß 12+/-2, normalized for age, were increased when compared with healthy subjects. Stiffness parameters correlate significantly with Tissue Doppler imaging diastolic parameters in particularly with E’ pwv (r = 0.68, p < 0.05), Ep (r = 0.60, p < 0.05) and ß (r = 0.68, p < 0.01). Conclusion: patients with masked hypertension increased arterial stiffness and abnormal left ventricular diastolic function (particularly with E’), was associated with arterial stiffness, expression of a greater contribution of atrial contraction to left ventricular filling for increased left ventricular end-diastolic pressure due to an high afterload.