Cases reported "Pulmonary Valve Stenosis"

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11/309. trisomy iop. A report of two cases due to a familial translocation rcp (10;21) (pII;pII).

    trisomy for the short arm of chromosome number 10 was diagnosed (by a G-banding method) in two sisters with multiple congenital defects. Their mother and two other sisters showed a balanced translocation 46,XX rcp(10;21)(p11;p11), so the affected girls were the result of a maternal adjacent-1 meiotic segregation with a karyotype 46,XX, der(21), rcp(10;21)(p11;p11)mat. The concordant features in the abnormal patients constitute the following syndrome: severe psychomotor retardation, congenital microsomatia, mild hydrocephalus with cranium-face disproportion, low set ears with hypoplastic helix, ocular colobomata, pulmonary stenosis,flexion deformity of wrists and elbows, bilateral fifth finger clinodactyly and simian creases, hypoplastic dermal ridges, bilateral talipes, persistent icterus and delayed bone age. The phenotypical and cytogenetic findings permit the individualization of the 10p trisomy.
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keywords = stenosis
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12/309. Fetal hydrops due to a tachyarrhythmia progressing to organic pulmonary stenosis.

    We discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.
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13/309. Complete obstruction of the left main coronary artery associated with congenital pulmonary valvular stenosis.

    The surgical treatment of the completely obstructed left main coronary artery is aortocoronary saphenous-vein bypass to the left coronary artery system. Without surgical intervention, this lesion has an exceedingly poor prognosis. This report describes a patient with atherosclerotic obstruction of the left main coronary artery and congenital pulmonary valvular stenosis, an unusual combination of lesions not previously documented together. The patient remains asymptomatic 22 months after aortocoronary saphenous-vein bypass and pulmonary valvotomy.
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ranking = 5
keywords = stenosis
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14/309. aneurysm in the pulmonary trunk associated with atrial septal defect, a left coronary artery fistula to the pulmonary trunk, and valvular pulmonary stenosis.

    A 78-year-old woman with an aneurysm in the pulmonary trunk associated with an atrial septal defect, left anterior descending coronary artery fistula to the pulmonary trunk and valvular pulmonary stenosis is reported. The aneurysm showed gradual dilatation over 16 years and was successfully treated using aneurysmorrhaphy. Although there has been some controversy regarding the optimum management for a pulmonary artery aneurysm, surgical correction is thought to be essential for aneurysms associated with congenital cardiac anomalies because of the high incidence of rupture.
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keywords = stenosis
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15/309. Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up.

    BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. methods: We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS: There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebstein's anomaly. CONCLUSIONS: Biventricular repair of Ebstein's anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.
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keywords = stenosis
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16/309. Combined percutaneous atrial septal defect occlusion and pulmonary balloon valvuloplasty in adult patients.

    Severe pulmonary stenosis in association with a large atrial septal defect is uncommon. When these 2 conditions are present, significant left-to-right shunt is often prevented by the outflow obstruction, which protects the pulmonary bed until adulthood. This report shows our initial experience of percutaneous treatment of both congenital malformations, either staged or combined in the same procedure, in 2 adult patients whose treatments yielded effective atrial septal defect occlusion and right ventricular pressure relief that persisted at mid-term follow-up. Although these opposite procedures (opening and closing) have been applied as isolated methods of treatment, this preliminary experience appears to demonstrate the feasibility and effectiveness of a combined percutaneous treatment.
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ranking = 1
keywords = stenosis
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17/309. Bullous ichthyosiform erythroderma, developmental delay, aortic and pulmonary stenosis in association with a FRA12A.

    We present an 11-year-old female with bullous ichthyosiform erythroderma (BIE), learning disability, patent ductus arteriosus and mild stenosis of the aortic and pulmonary arteries. Chromosome analysis showed the expression of the rare folate-sensitive fragile site FRA12A at 12q13 in 8/20 (40%) of blood lymphocytes cultured in folate-deficient medium in the presence of trimethoprim. Her mother and maternal grandmother are phenotypically normal, but her mother shows expression of the same fragile site in 4/20 (20%) of cells cultured under the same conditions. lymphocytes from the grandmother only showed expression of the fragile site when cultured in the presence of methotrexate in folate deficient medium. Interestingly, two genes (keratin 1 and keratin 2e) which are known to cause BIE map to 12q13. Molecular data is presented excluding three candidate (CCG)n repeats within keratin 1 gene. We present a review of previously reported FRA12A cases and discuss possible molecular explanations for the clinical findings in this patient.
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ranking = 5
keywords = stenosis
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18/309. Pulmonary stenosis in recipient twins in twin-to-twin transfusion syndrome: report on 3 cases and review of literature.

    This report describes 3 cases of pulmonary stenosis in the recipient twin in twin-twin transfusion syndrome. Fetal echocardiography showed cardiomegaly, tricuspid valve regurgitation, and increased reverse flow in the inferior vena cava, as signs of congestive heart failure in all 3 cases. We diagnosed 2 cases of pulmonary stenosis by fetal echocardiography prenatally and confirmed our findings in all 3 cases postnatally. Two cases underwent postnatal balloon valvuloplasty to release the pulmonary valvular stenosis in neonatal period. The third one died soon after delivery and autopsy showed a slightly thickened pulmonary valve. One of the cases was diagnosed in the early second trimester (20 weeks of pregnancy), the earliest detection of fetal pulmonary stenosis reported in literature. The presence of high peak velocity of the pulmonary artery at 20 weeks of pregnancy preceded the development of pulmonary stenosis in this case. This supports the hypothesis that alterations in fetal hemodynamics may result in structural cardiac abnormality.
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ranking = 9
keywords = stenosis
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19/309. Intraluminal pulmonary artery fibroma in a 7-year-old boy.

    A supravalvar intraluminal pulmonary artery fibroma, presenting as pulmonary stenosis, is described. This is a previously unreported location for this type of tumor.
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ranking = 1
keywords = stenosis
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20/309. Cardiac malformations associated with the congenital nephrotic syndrome.

    The association of cardiac malformation with the congenital nephrotic syndrome (CNS) has been previously reported in only one family. We report four patients with CNS: three with pulmonary valve stenosis (one requiring valvuloplasty) and one with discrete subaortic stenosis requiring surgical resection. We conclude that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral to a cardiologist, as flow murmurs due to chronic anaemia may obscure cardiac pathology. It is important to diagnose any associated cardiac lesions as these may require intervention, and may also predispose to the development of bacterial endocarditis if surgical or dental procedures are undertaken without appropriate antibiotic prophylaxis.
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ranking = 2
keywords = stenosis
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