Cases reported "Pulmonary Atresia"

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1/50. Right ventricular thrombosis early after bidirectional Glenn shunt.

    thrombosis in the right ventricle occurred early after bidirectional superior cavopulmonary shunt in 2 patients with pulmonary atresia with intact ventricular septum and major right ventricular coronary artery communication, and perioperative brain infarction occurred in 1 patient. Clinicians should be aware of the hazards of this potentially lethal complication, and transfusion of platelets and fresh plasma should be minimized. Although the hemodynamic state is good, echocardiography should be performed frequently and strict anticoagulation should be started as early as possible.
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2/50. Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

    The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.
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3/50. 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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4/50. Pathologic finding of restenosis in stent-implemented right ventricle-pulmonary artery extracardiac conduit.

    We describe an excised specimen of a stent-implanted valved equine pericardial extracardiac conduit in the right heart. It appears from careful pathologic examination that the stent acted as a nidus for thrombus formation followed by thick neo-intimal development over the stent, which caused restenosis. Restenosis occurred despite anticoagulation.
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5/50. pulmonary atresia with intact ventricular septum--a report of two cases with review of literature.

    Two perinatal autopsy cases are reported where a rare congenital anomaly, namely pulmonary atresia with intact ventricular septum in association with tricuspid stenosis and a hypoplastic right ventricle was encountered.
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6/50. Congenitally corrected transposition with pulmonary atresia and intact ventricular septum.

    We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.
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7/50. pulmonary atresia with intact ventricular septum, antegrade coronary-right ventricular sinusoidal communication, and wolff-parkinson-white syndrome.

    A fenestrated Fontan operation was performed successfully in a patient with pulmonary atresia with intact ventricular septum, an antegrade sinusoidal communication, and wolff-parkinson-white syndrome. Unlike most cases, blood flow in the sinusoidal communication was antegrade, from the left anterior descending artery to the right ventricle. This is the first report of the combination of pulmonary atresia with intact ventricular septum and wolff-parkinson-white syndrome.
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8/50. association of pulmonary atresia with intact ventricular septum and aortic valve stenosis. prenatal diagnosis.

    A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81 mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.
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9/50. Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction.

    BACKGROUND: Valved conduits from the right ventricle to the pulmonary artery are frequently used in paediatric cardiac surgery. However, stenosis and insufficiency of the conduit usually occur in the follow-up and lead to reoperations. Conduit stenting can delay surgical replacement, but it aggravates pulmonary insufficiency. We developed an innovative system for percutaneous stent implantation combined with valve replacement. methods: A 12-year-old boy with stenosis and insufficiency of a prosthetic conduit from the right ventricle to the pulmonary artery underwent percutaneous implantation of a bovine jugular valve in the conduit. FINDINGS: angiography, haemodynamic assessment, and echocardiography after the procedure showed no insufficiency of the implanted valve, and partial relief of the conduit stenosis. There were no complications after 1 month of follow-up, and the patient is presently in good physical condition. INTERPRETATION: We have shown that percutaneous valve replacement in the pulmonary position is possible. With further technical improvements, this new technique might also be used for valve replacement in other cardiac and non-cardiac positions.
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keywords = ventricle
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10/50. prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature.

    We report on a case of absent pulmonary valve syndrome in a woman with a history of one healthy child and one child with tetralogy of fallot with absent pulmonary valve. The diagnosis was missed at the first ultrasound examination performed at 13 5 weeks of gestation and correctly diagnosed at 21 5 weeks. Re-evaluation of the ultrasound examination recorded at 13 5 weeks exhibited severe insufficiency of the pulmonary valve at this time. However, neither dilatation of the right and left pulmonary arteries nor asymmetry of the ventricles were present at that time. The pregnancy was terminated at 22 1 weeks of gestation when autopsy confirmed the diagnosis of absent pulmonary valve syndrome. karyotyping of the fetus after termination of pregnancy revealed normal chromosomes. echocardiography of the parents and the healthy sibling revealed normal results.
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