Cases reported "Heart Defects, Congenital"

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1/290. Successful correction of double-outlet right ventricle with a ventricular D-l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect.

    The authors present the case of a fifteen-year old girl with double outlet right ventricle with ventricular d-loop and l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect, who was operated on successfully. This is the fourth case of double outlet right ventricle with l-position of the aorta that has been surgically corrected. The subaortic position of the interventricular defect favours the creation of the tunnel connecting the left ventricle with the aorta without obstructing the right ventricular outflow tract. The patient was doing well 11 months postoperatively.
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keywords = operative
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2/290. diagnosis of accessory mitral valve tissue by transesophageal echocardiography.

    Accessory mitral valve tissue is a rare cause of intracardiac mass and subvalvular left ventricular outflow tract obstruction. The preoperative diagnosis of this congenital anomaly has been facilitated by transthoracic two-dimensional and Doppler echocardiography. However, transthoracic two-dimensional echocardiography cannot identify or correctly diagnose all cases of accessory mitral valve tissue. We report a patient in whom an intracardiac mass detected by transthoracic echocardiography was definitively diagnosed as accessory mitral valve tissue by transesophageal echocardiography.
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keywords = operative
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3/290. Pediatric transesophageal color flow imaging 1990: the long and short of it.

    Noninvasive cardiac imaging has dramatically altered the practice of cardiology, specifically, the pediatric patient with congenital heart disease over the past decade. Since the introduction of transesophageal echocardiography nearly 15 years ago, addition of high-resolution cross-sectional imaging combined with Doppler color flow mapping has provided a new window to examine the heart. Recently, miniaturization of the transesophageal probe to "pediatric-size" has enabled its use in the smallest of infants to add significantly to the assessment of congenital heart malformations. Most recently, addition of a longitudinal-plane probe to the already-existent, transverse-plane probe has made biplane transesophageal echocardiography a reality with significant additional information being provided by orthogonal images of the cardiac structures. We used these probes in complementary fashion in 30 studies performed in 23 patients ages 1 day to 12 years with a mean of 35 months, weighing 2.6-40 kg (mean 12.4 kg). These studies were performed in the operating room, intensive care unit, cardiac catheterization laboratory, and outpatient department. Limitations of single-plane, transverse transesophageal echocardiography were overcome using the longitudinal-axis pediatric probe: left and right ventricular outflow tracts, distal pulmonary arteries, and all of the interventricular and atrial septa were easily visualized. Its use in the operating room and postoperative cardiac intensive care unit for continuous ventricular monitoring in otherwise-inaccessible patients also provided critical information. Transesophageal echocardiography in infants and small children is a valuable "noninvasive" imaging technique which, with addition of complementary longitudinal-plane views, offers important additional information regarding congenital heart malformations and their repair.
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ranking = 1
keywords = operative
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4/290. Subdivided left atrium mimicking a cardiac tumor.

    We describe a 25-year-old man with a subdivided left atrium. The lesion was misdiagnosed preoperatively as a cardiac tumor because echocardiographic and magnetic resonance imaging revealed a solid mass arising from the posterior wall of the left atrium. Cardiac surgery revealed a small accessory chamber draining the two left pulmonary veins. No membranous structure was evident between the chamber and the left atrium. The solid mass identified noninvasively was a hypertrophic muscle which formed a wall of the accessory chamber.
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keywords = operative
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5/290. drainage of the inferior vena cava to the left atrium.

    This report describes a 7-year-old girl with an anomalous connection between the inferior vena cava and the left atrium documented with intraoperative transesophageal color-coded Doppler flow echocardiography and angiography. This rare congenital disorder should be considered in the differential diagnosis in patients with cyanosis without cardiac murmurs. Operation is the only method for correction.
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ranking = 1
keywords = operative
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6/290. Heart/single-lung transplant for a "failed Fontan" with pulmonary A-V malformation.

    Pulmonary arteriovenous malformation can occur in patients with functional single ventricle after a cavopulmonary connection. There is no effective medical treatment for this complication. Pulmonary arteriovenous malformations may regress over time after heart transplantation, but may be a source of significant perioperative morbidity. We report the case of a woman with single ventricle, ventricular dysfunction, and bilateral pulmonary arteriovenous malformations who had successful treatment of both cardiac and pulmonary failure with en bloc heart and right lung transplantation.
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ranking = 1
keywords = operative
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7/290. Striae and acne following cardiac surgery in a child.

    We report a 13-year-old girl with extensive striae and an acneiform eruption following surgery for complex congenital heart disease. These findings were associated with elevated serum and urinary cortisol levels with loss of diurnal rhythm. The resolution of the eruption and the fading of her striae coincided in time with normalization of her blood parameters on day 72 postoperatively. We conclude that the cause of steroid excess in our patient was stress induced by the cardiac surgery and a complicated and protracted postoperative course. To our knowledge, this is the first report in the English language literature of skin changes due to endogenous hypercortisolaemia caused by intense physical and emotional stress.
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ranking = 2
keywords = operative
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8/290. Paired ventricular pacing: an alternative therapy for postoperative junctional ectopic tachycardia in congenital heart disease.

    Junctional ectopic tachycardia (JET) is one of the most life-threatening postoperative arrhythmias in children with congenital heart disease, and medical management is difficult. Paired ventricular pacing (PVP) may provide a safe alternative mode of management. We evaluated the safety and efficacy of PVP for the management of postoperative JET in patients with congenital heart disease. A retrospective collection of data was done from 1981-1995. PVP was successfully tried in five postoperative patients (age range: 37 days to 22 years, median: 10 months). Onset of JET was 3-60 hours (mean /- SD, 19 /- 23 hours) postoperatively. The maximal JET rate was 261 /- 39 beats/min. PVP was used as the first line of management in three patients and was successful in all patients. It resulted in an instantaneous increase in blood pressure from 66 /- 9 to 94 /- 15 mmHg (42% increase) and was required for 12 /- 14 hours (range 2-36 hours). No complications were noted. Therefore, in our experience, this is a safe alternative modality for the control of postoperative JET.
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ranking = 9
keywords = operative
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9/290. Reconstruction of the mitral and aortic annuli for advanced management of the Shone complex.

    Shone's complex is a congenital cardiac abnormality which consists of surpravalvular mitral ring, parachute mitral valve, subaortic stenosis and aortic coarctation. Initial operative management has traditionally proven difficult, with multiple procedures often necessary to control symptoms. Advanced management had required a careful, individual approach based on both clinical and anatomic patient presentation. We present the first patient in whom mitral and aortic annular reconstruction with bovine pericardial gussets was successful in managing the late sequelae of Shone complex.
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ranking = 1
keywords = operative
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10/290. Long-segment congenital tracheal stenosis: treatment by slide-tracheoplasty.

    BACKGROUND/PURPOSE: The prognosis of long-segment funnel-shaped congenital tracheal stenosis improved with surgical management by enlargement-tracheoplasty with a pericardial patch. However, the final outcome is not always satisfying mainly because of the complications related to the pericardial graft and because of misdiagnosed associated cardiovascular malformations. The objective of the report is to show a further improvement of the prognosis by a thorough preoperative planning and the use of a slide-tracheoplasty for the cure of the stenosis, to avoid the major drawbacks of enlargement tracheoplasties. methods: Two children, 7 months and 3 years old, underwent a bronchoesophagoscopy, spiral computed tomography, an echocardiogram, and a heart catheterization. The tracheal stenosis and the associated severe cardiovascular malformations were cured during a single operative session under cardiopulmonary bypass; the tracheal stenosis was corrected by a slide-tracheoplasty. RESULTS: The postoperative period was remarkably uneventful and the recovery extremely quick (hospital stay of 18 and 17 days). The midterm results are excellent, and the tracheal growth is not impaired (follow-up of 3 1/2 and 3 years). CONCLUSIONS: According to the authors' experience and to the literature, the slide-tracheoplasty seems to be the most efficient surgical procedure for correction of long-segment funnel-shaped congenital tracheal stenosis. But the success of the treatment depends also on a complete preoperative diagnosis allowing a 1-stage surgical treatment of all associated thoracic malformations.
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ranking = 4
keywords = operative
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