Cases reported "Pulmonary Atresia"

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1/15. Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death.

    A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistuious communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.
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2/15. 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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3/15. 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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4/15. Vascular aneurysm producing divided right atrium in a patient with pulmonary atresia and intact ventricular septum.

    We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.
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5/15. 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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6/15. New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum.

    We report new percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum, in 3 newborns who had this birth defect. There was mild to moderate hypoplastic right ventricle, a patent infundibulum, and no coronary-cavitary communications. We succeeded in all cases, and no complications related to the procedure occurred. The new coaxial radiofrequency system was easy to handle, which simplified the procedure. Two patients required an additional source of pulmonary flow (Blalock-Taussig shunt) in the first week after catheterization. All patients had a satisfactory short-term clinical evolution and will undergo recatheterization within 1 year to define the next therapeutic strategy. We conclude that this technique may be safely and efficiently performed, especially when the new coaxial radiofrequency system is used, and it may become the initial treatment of choice in select neonates with pulmonary atresia and intact ventricular septum.
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7/15. Biventricular repair for pulmonary atresia with intact ventricular septum associated with sinusoidal communications.

    pulmonary atresia with intact ventricular septum (PA-IVS) is very difficult to treat due to the variety of right ventricular hypoplasia and coronary artery anomalies. Biventricular repair is viewed as possible in patients with mild right ventricular hypoplasia but not in those with severe right ventricular hypoplasia or with sinusoidal communication. We report a case of a 17-day-old boy with PA-IVS and severe right ventricular hypoplasia and large sinusoidal communications. We successfully conducted biventricular repair, ligated coronary artery and right ventricular fistulas, and implemented right ventricular outflow tract reconstruction. He now enjoys good health and attends elementary school. Sinusoidal communications are not always a limiting factor in biventricular repair for PA-IVS if the right ventricle can be decompressed without inviting ventricular dysfunction by ligating fistulas.
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8/15. Regression of cardiac enzyme and ventriculocoronary communication in an infant with pulmonary atresia and intact ventricular septum after radiofrequency valvulotomy and valvuloplasty.

    I report on a 3-month-old infant with pulmonary atresia-intact ventricular septum and ventriculocoronary communication (VCC) who underwent percutaneous radiofrequency valvulotomy and valvuloplasty (RFVV). The patient's cardiac troponin-I, creatine kinase (CK), and myocardial fraction of (CK-MB) were elevated before RFVV and were gradually regressed to normal levels 12 days after RFVV. The VCC disappeared after RFVV. The transvalvular pressure gradients across the pulmonary valve were less than 30 mmHg in the follow-up echocardiography at 4-12 months of age. oxygen saturation was approximately 90% in room air. dipyridamole-thallium myocardial scintigraphy showed positive reperfusion over the apex and interventricular septum.
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9/15. Catheter closure of tube graft fenestration in extracardiac Fontan operation.

    As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would become useful. We describe a simple and safe technique of fenestration amenable to coil occlusion, which can be carried out in most cardiac catheterization laboratories.
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10/15. Anesthetic management of bidirectional cavopulmonary shunt in a patient with pulmonary atresia with intact ventricular septum associated with sinusoidal communications.

    pulmonary atresia with intact ventricular septum (PAIVS) is sometimes associated with coronary artery anomalies, including right ventricle (RV)-to-coronary artery fistulas (sinusoidal communications), coronary artery stenoses, and coronary artery occlusions. In some cases, the coronary circulation depends entirely or partly on the desaturated systemic venous blood supply from the RV. Under these circumstances, decompression of the RV can result in fatal myocardial ischemia. A 6-month-old boy, diagnosed with PAIVS associated with sinusoidal communications, underwent a bidirectional cavopulmonary shunt procedure under venoarterial cardiopulmonary bypass (CPB). During CPB, to prevent RV decompression, we maintained right atrial pressure above 5 mmHg and used a pump perfusion rate of 30%-40% of the calculated value based on body surface area. Although electrocardiography showed slight ST depression and bradycardia, myocardial contractility after weaning from CPB was adequate to maintain the circulation with the administration of dobutamine and atrial pacing. In patients with PAIVS and RV-dependent coronary circulation, it is important to maintain coronary artery perfusion throughout the period of anesthesia.
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