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1/299. Reversion to sinus rhythm 11 years after surgically induced heart block.

    A patient is presented in whom the heart reverted spontaneously to sinus rhythm 11 years after surgical closure of a ventricular septal defect complicated by complete heart block. It seems unlikely that regeneration of fibres in the bundle of his, if these had indeed been destroyed, could account for the restoration of sinus rhythm after so long an interval.
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2/299. High origin of the right coronary artery with congenital heart disease.

    We encountered a case of anomalous high origin of the right coronary artery associated with ventricular septal defect and patent ductus arteriosus. The right coronary artery originated from the distal part of the ascending aorta resulting in unsuccessful induction of cardiac arrest by cardioplegia. We describe this rare case with anomalous origin of the right coronary artery.
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3/299. Sudden death due to ventricular septal defect.

    Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16-31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and (1/2) months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.
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4/299. Aortic root dilation in apparent Lujan-Fryns syndrome.

    We present a patient and his maternal uncle who have a subaortic ventricular septal defect and aortic root dilation. They both have physical anomalies, characteristic behaviors, and cognitive disabilities that are consistent with the diagnosis of Lujan-Fryns syndrome (LFS). Although there have been 4 cases reported in the literature with heart findings, ventricular septal defect and aortic root dilation have not been previously reported in LFS. Differentiation between LFS and marfan syndrome (MS) is discussed. The pathophysiology of LFS as a connective tissue disorder is also considered.
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5/299. Accessory mitral valve tissue causing severe subaortic stenosis with dextrocardia in a premature newborn.

    We report an unusual case of left-ventricular outflow obstruction caused by accessory mitral valve tissue associated with dextrocardia and ventricular septal defect in a seven-day-old, 2200 grams premature infant, who was referred with a heart murmur. The diagnosis was made by two-dimensional and Doppler echocardiography which demonstrated the accessory tissue as well as a 100 mmHg peak systolic gradient between the left ventricle and the aorta. Ten days after the presentation the infant underwent emergency surgery after respiratory arrest and recurrent episodes of syncope. The accessory mitral valve tissue and its fibrous extension were excised and the ventricular septal defect was closed. We believe that surgical treatment of patients with accessory mitral valve tissue should be performed early because of the possibility of acute deterioration. Combined aortotomy and interatrial approach is very helpful in evaluating the anatomy and the mitral valve function as well as delineating the tissue to be excised.
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keywords = heart, murmur
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6/299. cyanosis due to diastolic right-to-left shunting across a ventricular septal defect in a patient with repaired tetralogy of fallot and pulmonary atresia.

    cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.
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7/299. Obstruction of the right ventricular outflow tract caused by a tuberculoma in a patient with ventricular septal defect and aneurysm of the membranous septum.

    Reported here is an obstruction of the right ventricular outflow tract caused by a tuberculoma in a 15-year-old boy who presented with a ventricular septal defect. The obstruction was discovered at surgery and the tuberculous aetiology was only demonstrated histologically. To the authors' knowledge, this is the first report of a tuberculoma of the heart associated with congenital heart disease.
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8/299. Bivalvation for common atrioventricular valve regurgitation in cyanotic heart disease.

    We describe the case of a 15-year-old boy with complex heart disease, in whom we successfully performed bivalvation for common atrioventricular valve regurgitation and bilateral, bidirectional cavopulmonary shunts. Postoperative Doppler echocardiography revealed significant reduction of atrioventricular valve regurgitation.
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keywords = heart
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9/299. Atrioventricular septal defect following blunt chest trauma.

    The authors describe an acquired atrioventricular septal defect that has resulted from a blunt chest trauma. Besides being an uncommon traumatic heart injury, this case has the particularities of the non-involvement of other adjacent anatomical structures and the long delay between the accident and the occurrence of the myocardial rupture.
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10/299. Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis.

    Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.
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