1/47. Left ventricle to pulmonary artery conduit in treatment of transposition of great arteries, restrictive ventricular septal defect, and acquired pulmonary atresia.Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved.- - - - - - - - - - ranking = 1keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
2/47. 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.- - - - - - - - - - ranking = 1.0076228780451keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
3/47. One day old infant with acyanotic congenital heart disease: critical aortic stenosis.Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis.- - - - - - - - - - ranking = 0.00025426863776115keywords = valve (Clic here for more details about this article) |
4/47. Doppler echocardiographic evaluation of the hemodynamics in absent aortic valve.hemodynamics were estimated by Doppler echocardiography in a neonate with an absent aortic valve and absent or extremely hypoplastic mitral valve. The coronary blood flow depended on the increased end-diastolic pressure of the left ventricle. Pulmonary venous congestion, which was also due to the increased end-diastolic pressure of the left ventricle, may induce decreased oxygen saturation and, subsequently, further myocardial hypoxia, poor contraction and increased end-diastolic pressure of the left ventricle. Finally, hypoxic blood was supplied to each organ from the pulmonary artery through the ductus arteriosus, which induced severe acidosis and differential cyanosis after birth.- - - - - - - - - - ranking = 0.00076280591328345keywords = valve (Clic here for more details about this article) |
5/47. Device closure of an atrial septal defect following successful balloon valvuloplasty in a neonate with critical pulmonary valve stenosis and persistent cyanosis.Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.- - - - - - - - - - ranking = 0.030510089271647keywords = pulmonary valve, valve (Clic here for more details about this article) |
6/47. Occlusion of azygos vein via direct percutaneous puncture of innominate vein following cavopulmonary anastomosis.A 2-year-10-month-old boy was diagnosed with a complex congenital heart disease: right atrial isomerism, left superior vena cava (LSVC), complete atrioventricular septal defect, secundum type atrial septal defect, transposition of the great arteries with pulmonary atresia, patent ductus arteriosus, absence of a right superior vena cava (RSVC), and dextrocardia. He had received a left Blalock-Taussig (BT) shunt at the age of 3 months and a left bidirectional Glenn shunt one year after BT shunt. Progressive cyanosis was noted after the second operation and cardiac catheterization showed a functional Glenn shunt with an engorged azygos vein, which was inadvertently skipped for ligation. Because of the absence of RSVC, transcatheter occlusion of the azygos vein was performed successfully via direct puncture of the innominate vein.- - - - - - - - - - ranking = 0.25keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
7/47. Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? A 5th aortic arch with a systemic-to-pulmonary arterial connection.Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.- - - - - - - - - - ranking = 1.5keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
8/47. Postsurgical use of amplatzer septal occluder in cyanotic patients with pulmonary atresia/intact ventricular septum: significance of cor triatriatum dexter and dilated right atrium.Percutaneous closure of secundum atrial septal defects (ASDs) has been shown to be safe and effective. However, its role after surgery in patients with cyanotic congenital heart disease who may have associated cor triatriatum dexter and a dilated right atrium has not been established. This article reports on successful closure in such patients, including precautions and results.- - - - - - - - - - ranking = 1keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
9/47. Rapid onset of intrapulmonary arteriovenous shunting after surgical repair of tetralogy of fallot with pulmonary atresia.We describe a 2-year-old girl with tetralogy of fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.- - - - - - - - - - ranking = 1.25keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
10/47. Endovascular stent implantation in a coronary artery to pulmonary artery fistula in a patient with pulmonary atresia with ventricular septal defect and severe cyanosis.A 31-year-old male with pulmonary atresia, ventricular septal defect presented with exercise intolerance and severe cyanosis. A restrictive coronary-pulmonary artery fistula was identified as the main source of pulmonary blood flow. We report transcatheter stent implantation in the fistula to augment pulmonary flow as a palliative management option in the adult patient with complex congenital heart disease.- - - - - - - - - - ranking = 1.25keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
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