Cases reported "Heart Defects, Congenital"

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1/59. Stent implantation to create interatrial communications in patients with complex congenital heart disease.

    A restrictive interatrial communication can complicate the management of complex congenital heart disease. The purpose of this report is to present a new technique to achieve a patent and reliable interatrial communication by using an endovascular stent. A stent was successfully implanted across a fenestrated extracardiac conduit in two patients with low cardiac output after Fontan operations and across the interatrial septum in a patient with double inlet left ventricle and severe left atrioventricular stenosis. The procedures were uncomplicated and all patients showed immediate hemodynamic improvement. Cathet. Cardiovasc. Intervent. 47:310-313, 1999.
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2/59. Divided left atrium associated with supravalvar mitral ring.

    Reported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.
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3/59. Acardiac fetus in a triplet pregnancy: ultrasound pitfalls. A case report.

    This communication aims at illustrating ultrasound diagnostic difficulties in early pregnancy with acardiac fetus. Our case concerns a spontaneously conceived triplet pregnancy. It was diagnosed as a twin pregnancy at 11 weeks of amenorrhea. One and a half months later the patient was referred to our center for spontaneous premature rupture of membranes with the diagnosis of a fetal demise in a triplet pregnancy. The definite diagnosis of acardia was assessed sonographically by the presence of a reverse blood flow through the umbilical cord, reflex movements, limbs anomalies and discordance between femoral and crown-rump length. Two days after admission, the patient developed chorioamnionitis and the three fetuses were expelled.
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4/59. magnetic resonance imaging of unroofed coronary sinus: three cases.

    Unroofed coronary sinus is a rare cardiac anomaly in which communication occurs between the coronary sinus and the left atrium due to the partial or complete absence of the roof of the coronary sinus. It is usually associated with other cardiovascular anomalies, especially persistent left superior vena cava. It is often not discovered during cardiac catheterization without clinical suspicion. We report three cases of unroofed coronary sinus which were incidentally detected by magnetic resonance imaging.
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5/59. Bifurcation of the left superior pulmonary vein, associated with an anomalous connection with the left atrium.

    An unusual case of bifurcation of the left superior pulmonary vein (LSPV) just before it enters the pericardium is described. The LSPV, which at the hilus of the lung originated from normal confluence of a superior and inferior root, bifurcated near the left atrium (LAt) of the heart into anterior (AB) and posterior (PB) branches that, separately invaginating the parietal pericardium, formed two individual serous sheaths. The PB coursed almost horizontally and opened, as usual, into the supero-dorsal wall of the LAt. The AB turned downward, reached the superior margin of the left auricle (LAu) and emptied into it. Thus, the AB was interposed between the pulmonary trunk and the LAt obstructing on the left side the communication between the transverse sinus of the pericardium and the pericardial cavity. The auricular opening of the AB was avalvular, but, unlike those of the normal pulmonary veins (PVs) which are surrounded by a large smooth inner surface, was, except for a narrow smooth-walled zone, close to the pectinate muscles. Moreover, an inferior muscular ridge at the inferior margin of its orifice of entrance into the LAu, separated it from the cavity of the LAt. It is well known that in development the PVs arise from convergence of capillaries belonging to the mediastinal part of the primitive splanchnic plexus and drain this into the systemic (cardinal and vitello-umbilical) veins of the embryo. As a consequence, it might be hypothesized that the AB of the LSPV probably represents a partial remnant either of a pulmonary-cardinal anastomotic mediastinal vein, or of a diverging vessel of the mediastinal plexus from which the PVs originate. In either case the AB became absorbed by the LAu, which, while it was developing on the left side of the primitive truncus arteriosus, drew the AB forward and downward, in the direction of its movement. The influence of such an anomaly of the PVs for altered intracardiac hemodynamics of the oxygenated blood flow has to be emphasized. Furthermore, the particular location of the AB, obstructing the communication between transverse sinus and pericardial cavity, can be a hindrance during cardio-pulmonary surgery.
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6/59. Noninvasive diagnosis and surgical management in total anomalous pulmonary venous return draining into the coronary sinus: report of 2 cases.

    Total anomalous pulmonary venous return results from nondevelopment of the common pulmonary vein, with consequent enlargement of embryonic collaterals between the lungs and the systemic veins. In this report, two patients with this anomaly draining into the coronary sinus who presented in infancy are described. One of the patients was referred because of growth and development failure and chronic constipation, while the other had tachypnea as the presenting problem. Both were diagnosed during the echocardiographic examination. Typical echocardiographic findings were a small left atrium, a sausage-shaped dilated coronary sinus receiving the pulmonary veins in the subcostal short axis, and flying seagull configuration in the subcostal long axis views. Both had a large interatrial communication. The patients underwent corrective surgery. The aim of the presentation is to emphasize the role of segmental echocardiography in the differential diagnosis.
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7/59. Absence of the ductus venosus with direct connection between the umbilical vein and right atrium.

    Abnormalities of the ductus venosus are rare. We present the case of a dichorionic, diamniotic twin pregnancy in which complete absence of the ductus venosus, with direct communication between the umbilical vein and right atrium, was detected in one infant on antenatal ultrasonography. autopsy confirmed the aberrant course of the umbilical vein, which also had an abnormal histological structure. Associated congenital anomalies included an ostium secundum type atrial septal defect, absent inferior vena cava, single left pulmonary vein, stenosed right pulmonary artery, proliferation of the hepatic arterioles with reduction of portal venules, and a duplex ureter. The literature is reviewed and pathogenesis relevant to this case is discussed.
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8/59. Aortico-left ventricular tunnel: two new cases with a long-term follow-up.

    Aortico-left ventricular tunnel is a rare congenital communication between the ascending aorta and the left ventricle. Its hemodynamic effect is severe aortic incompetence. Surgery is the only treatment and should be performed before aortic incompetence or ventricular dilation develops. Two neonates with aortico-left ventricular tunnel were operated on at our institution, with closure of the aortic end of the tunnel with a Gore-Tex patch. The 2 patients were discharged in good conditions, and at 112 and 42-month follow-up respectively they are in good health, without medication and with a normal echocardiographic pattern. Aortico-left ventricular tunnel should be treated surgically as soon as possible in order to prevent any damage to the aortic valve and the left ventricle. The operative risk is not low, but results are very encouraging.
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9/59. Telangiectatic fistula between the conal branch of the left coronary artery and the pulmonary trunk.

    A rare case of telangiectatic communication between the conal branch of the left coronary artery and the pulmonary trunk in a 50-year-old woman is reported. Unusual features included the presence of clear-cut angina on effort, unstable auscultatory findings and a RSR' pattern in lead V1, probably related to concommitant diffuse coronary atherosclerosis. Ten previously reported cases of the condition are briefly reviewed.
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10/59. Chromosome 22q11 deletion syndrome (CATCH 22): neuropsychiatric and neuropsychological aspects.

    Twenty children and young adults (age range 5 to 33 years, 12 females and eight males) with genetically confirmed 22q11 deletion syndrome (CATCH 22: Cardiac anomaly, Anomalous face, Thymus hypoplasia/aplasia, cleft palate, and Hypocalcaemia), recruited from a large ongoing study, were given comprehensive assessments with a view to determining the pattern of neuropsychiatric and neuropsychological deficits thought to be part of the syndrome in many cases. IQ ranged between 46 and 100 with a mean score of 70. Half the group had an IQ <70. In 13 individuals, attention-deficit-hyperactivity disorder (ADHD), mainly inattentive or combined type in most cases, and/or autism spectrum problems were diagnosed. Many participants, even among those who had an IQ within the normal range and had neither ADHD nor autistic spectrum problems, showed a characteristic and pronounced behavioural profile with low mental energy, initiation difficulties, deficits in sustained attention, and social interaction (often augmented by limited facial expression and communication and speech problems).
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