Cases reported "Heart Septal Defects"

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1/9. Usefulness of selective myocardial contrast echocardiography in percutaneous transluminal septal myocardial ablation: a case report.

    A 67-year-old woman with hypertrophic obstructive cardiomyopathy that was refractory to medical treatment underwent percutaneous transluminal septal myocardial ablation (PTSMA). The septal branch supplying the myocardium involved in the left ventricular outflow tract (LVOT) obstruction was identified by selective myocardial contrast echocardiography (MCE). MCE for the third and largest septal branch opacified the right side of the mid-septal myocardium and MCE for the second septal branch opacified the right side of the basal portion of the septal myocardium. Finally, contrast agent was injected into the first, small branch, which opacificied the myocardium protruding into the LVOT. Subsequently, septal myocardial ablation for this vessel with intracoronary alcohol was performed, followed by a reduction of the LVOT gradient and successful, dramatic improvement in the patient's clinical condition. Selective MCE was very useful to identify the appropriate septal branch for PTSMA and enabled maximal effect of this treatment with minimal myocardial damage.
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2/9. trisomy 5 mosaicism detected prenatally with an affected liveborn.

    This paper reports a case of chromosomal mosaicism for trisomy 5 recovered from amniotic fluid cells and from skin fibroblasts of a liveborn dysmorphic male. Routine amniocentesis was performed at 16 weeks' gestation because of parental concern. trisomy 5 cells were measured from 25 per cent of amniocytes from two culture vessels. No further invasive testing was performed until 32 weeks' gestation, at which time ultrasound examination showed a fetus with intrauterine growth retardation. fetal blood sampling was then performed, with only karyotypically normal cells recovered. At birth, the child was found to have multiple dysmorphic features and congenital anomalies, including an eventration of the diaphragm and ventricular septal defect, both of which required surgical correction. Chromosomal analysis of cord blood lymphocytes indicated 46,XY; however, 20 per cent of the cultured fibroblasts obtained from the chest skin at the incision site for diaphragmatic repair had a 47,XY, 5 karyotype. trisomy 5 mosaicism may be another example of tissue-limited mosaicism. fetal blood sampling can then be falsely reassuring. Furthermore, because some cell lines rarely appear in lymphocyte populations, cytogenetic analysis of multiple tissues is warranted as part of the evaluation of individuals with developmental delay and dysmorphic features.
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3/9. heart transplantation in repaired transposition of the great arteries.

    Cardiac transplantation was carried out in a 40-year-old man with the diagnosis of repaired transposition of the great arteries and right-sided aortic arch who had end-stage cardiac failure due to myopathy of the ventricles. Because of several previous surgical repairs and the orientation of the great vessels, the operation presented some technical problems. Therefore, modifications of operative procedures were used, including recipient hypothermia, circulatory arrest, and changes in the donor heart implantation. The removal of the donor heart was modified in such a way that the graft included the aortic arch and both pulmonary arteries. With the extra length of ascending aorta and transverse arch, the innominate, left carotid, and left subclavian vessels were excised as a button, thereby leaving the distal orifice of the aorta in the superior portion of the transverse arch. For the recipient, the operation was performed using hypothermic total circulatory arrest to dissect free the huge pulmonary artery and the short right-sided aortic arch to place the clamp. Implantation of the donor heart was modified accordingly. The technical results were confirmed one and a half months later on a control digital angiogram. Thirty-five days postoperatively the patient was discharged. Six months after operation, the patient is doing better than ever before in his life. Our findings suggest that a complicated conotruncal development does not preclude cardiac transplantation.
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4/9. Anomalous origin and malposition of the pulmonary arteries (crisscross pulmonary arteries) associated with complex congenital heart disease.

    Anomalous origin and malposition of the branch pulmonary arteries is an uncommon malformation that has previously been noted only at the time of pathologic examination. Two infants with complex congenital cardiac disease had malposition of the branch pulmonary arteries (crisscross pulmonary arteries) detected by angiography and confirmed during surgery and in one case, at autopsy. The presence of this clinically unsuspected lesion complicated the accurate interpretation of both the two-dimensional echocardiographic appearance of the great vessels, and the catheter course and location during cardiac catheterization. angiography clearly defined the crisscrossing pattern of the branch pulmonary arteries. The recognition of this malformation was important in planning and executing the surgical palliation or repair for these infants.
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5/9. Prenatal closure of the foramen ovale in complete transposition of great vessels. Observations in a case with intact ventricular septum and normally developed left heart chambers.

    A rare case is reported of premature closure of the foramen ovale in d-transposition of the great vessels with intact ventricular septum and normal development of the left side of the heart. asphyxia neoatorum and cardiac failure were the manifestations in the early neonatal period. Balloon atrial septostomy during cardiac catheterization would be unsuccessful in infants with this condition, and closed atrial septectomy (Blalock-Hanlon procedure) would be necessary for survival.
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6/9. verapamil for refractory ventricular fibrillation during cardiac operations in patients with cardiac hypertrophy.

    calcium-entry blockers prevent ventricular fibrillation during acute myocardial ischemia in laboratory animals. They may be useful as an adjunct to cold cardioplegia by preserving the myocardium during cardiopulmonary bypass. Their use may limit myocardial infarct size. However, the clinical application of calcium-entry blockers for ventricular dysrhythmias associated with myocardial ischemia has been little explored, as yet. We describe four patients, all of whom had significant cardiac hypertrophy (two had idiopathic hypertrophic subaortic stenosis; one had transposition of great vessels; and one had aortic stenosis) and in whom ventricular fibrillation developed after rewarming or shortly after the termination of bypass. The dysrhythmias were refractory to multiple attempts at defibrillation and conventional pharmacologic interventions. However, in each case, defibrillation was successful after administration of verapamil.
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7/9. Single coronary artery with the right coronary artery arising from the first septal perforator.

    A 50-year-old woman with chest pain and an exercise thallium-201 scintigram positive for focal ischemia was found on coronary arteriography to have a heretofore unreported variant of single left coronary artery with the right coronary artery originating as a branch from the first septal perforator. Proximally, the aberrant vessel coursed through the ventricular septum at the level of the right ventricular outflow tract. A conus artery was absent and this is a possible basis for the focal basal ventricular ischemia and the patient's symptoms.
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8/9. Anomalous origin of the right coronary artery with aortopulmonary window: functional and surgical considerations.

    A rare case of anomalous origin of the right coronary artery from the pulmonary artery associated with a large aortopulmonary window in a 4-month-old boy is reported. The right coronary artery is exposed to systemic pressure and carries fairly well-oxygenated blood to the myocardium. Angiographic diagnosis could be difficult because of the simultaneous filling of both great arteries which obscures the origin of the anomalous vessel. Closure of the aortopulmonary window alone could result in acute myocardial ischaemia. A plastic procedure for correction of this association of defects, which should allow normal growth, is described.
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9/9. Anatomic correction of transposition of the great vessels.

    We present a new approach for anatomic correction of transposition of the great arteries. The two coronary arteries, with a piece of the aortic wall attached, are transposed to the posterior artery. The two aortic openings are closed with a patch. The aorta and pulmonary artery are transected, contraposed, ant then anastomosed. The interventricular septal defect is closed with a patch, through a right ventriculotomy approach, because the right ventricle is no longer part of the systemic circulation. Two patients, aged 3 months and 40 days weighing 4,200 and 3,700 grams, respectively, were operated upon with deep hypothermia and total circulatory arrest. There was good recovery from the operation, with normal cardiocirculatory conditions. Renal failure developed in the first patient, and she died on the third postoperative day. During this time the cardiocirculatory conditions were good. The second patient made an uneventful recovery. Hemodynamic studies 20 days after the operation showed complete correction of the malformation. Five and one-half months after the operation, he weighs 7,500 grams, and his development is very good. We believe that this operation will be reproducible by most cardiovascular septal defect and pulmonary hypertension.
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