The quadricuspid aortic valve (QAV) is a rare malformation; often isolated, sometimes associated with other heart diseases. Before the era of echocardiography, the diagnosis was made incidentally at autopsy or during surgery of valve replacement. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic approach is suitable for the diagnosis but, transesophageal echocardiography is a tool for the accurate definition of the valve anatomy. This review analyzes, after the presentation of a clinical case, the current knowledge on embryogenesis, classification, diagnosis and clinical course of QAV.

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Uhl's anomaly is characterized by absence of the myocardial layer of the right ventricle, with opposition of the endocardium and epicardium. It is rarely associated with other congenital malformation. Here, we reported the rare association of Uhl's anomaly with absent tricuspid valve in an infant.

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Aims: Three-dimensional speckle-tracking echocardiography (3D-STE) is a promising new technique to evaluate left ventricular (LV) mechanics. The feasibility and normal values of LV strain using 3D-STE have recently been established in adults and children. Unfortunately, no data are available in neonates. The aims of this study were to evaluate the feasibility and establish normal values of 3D LV volumes, ejection fraction (EF), and the 4 normal strains in healthy neonates. Materials and Methods: Of 50 consecutive newborns who were delivered at our hospital or returned to their first newborn follow-up within the first 3 weeks of life, 38 babies underwent full echocardiographic evaluation, including the acquisition of at least 3 full volume data sets from the apical window, while naturally sleeping. Data sets were analyzed offline. Global LV longitudinal, circumferential, and radial strain, as well as 3D LV volumes and EF, were measured using 3D-STE. Results: Of the 50 newborns, 2 patients were excluded because of significant intra-cardiac shunts, and in another 10 subjects, parents did not give consent. At least one data set was adequate for analysis in all the remaining subjects. Mean indexed LV diastolic, systolic volumes, and EF were 24.7 ± 3.6 ml/m ² , 9.2 ± 1.3 ml/m ² , and 63% ± 3.7%, respectively. Normal global longitudinal, circumferential, radial, and tangential 4D strain were −20.9% ± 2.8%, −32.4% ± 3.1%, 44.3% ± 3.4%, and −39.7% ± 3.4%, respectively. Conclusions: 3D-STE is feasible in newborns without the needed for sedation. Reference values of normal, regional, and global LV 4D strain and volumes were obtained.

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Supravalvular aortic stenosis, characterized by narrowing of the ascending aorta above the valve, is the least common form of left ventricular outflow tract obstruction and is usually associated with William's syndrome. We present a case of a 27-year-old male with isolated supravalvar aortic stenosis (SVAS) presenting with heart failure. This case underscores the fact that in rare cases sporadic SVAS can occur in isolation without the classic findings of William's syndrome and highlighting the importance of integration of clinical and echocardiographic recognition for definitive management.

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Mitral atresia is a rare congenital heart defect when compared to atresia of other valves and it is often associated with other cardiac malformation such as patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, pulmonary stenosis, shunt defects, cor triatriatum, chamber, and valvular anomalies. There have been few reports of mitral atresia, therefore, the case of a 5-month-old male child with complaint of difficulty with breathing and echocardiographic diagnosis of atretic mitral valves, atrial septal defect of 15 mms, ventricular septal defect (VSD) of 10 mms and hypoplastic left ventricle is reported.

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The unicuspid aortic valve (UAV) is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Here, we present a case of UAV with moderate aortic stenosis detected by transthoracic echocardiography in the infant.

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Left ventricular (LV) thrombus formation is a frequent complication in patients with acute anterior myocardial infarction (MI). Its incidence is lower with inferior wall MI. Risk factors for the development of LV thrombus are consistently irrespective of infarct treatment and include large infarct size, severe apical akinesia or dyskinesia LV aneurysm, and anterior MI.

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In the embryo, Eustachian valve is a crescent-shaped membrane extending from the lower margin of the inferior vena cava and the ostium of the coronary sinus into the right atrium toward fossa ovalis and tricuspid valve. At birth, after the functional closure of the foramen ovale, the Eustachian valve loses its function, reducing to an embryo remnant. According to growing evidence, a persistent Eustachian valve is a frequent finding in patients with a patent foramen ovale (PFO). By directing the blood from the inferior cava to the interatrial septum, it may prevent the spontaneous closure of PFO after birth and indirectly predispose to paradoxical embolism. Transesophageal contrast enhanced echocardiography (cTEE) is considered the gold standard to diagnose a PFO in postnatal life, but its accuracy maybe is not so high in the presence of a persistent Eustachian valve. In these cases, color Doppler TEE is more sensitive and simplifies the diagnostic process, reducing the duration of TEE and improving the patient compliance.

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