1/13. A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum.A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/13. Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect.patients with unrepaired pulmonary artery atresia and ventricular septal defect (PA/VSD) depend on aortoplumonary collaterals and surgically created shunts for pulmonary blood flow. These vessels frequently develop stenoses with time, leading to hypoperfusion of lung segments and systemic hypoxemia. The purpose of this article is to describe catheter palliation of hypoxemic patients with PA/VSD who were not candidates for surgical repair. We present our experience with stent implantation for stenosis of aortopulmonary collaterals and shunts in these patients. Three patients with hypoplastic pulmonary arteries underwent stent placement in aortopulmonary collateral arteries (APCAs) or their shunts. Technical aspects of the interventional catheterization procedure are discussed in detail. Case 1 underwent placement of five stents in collateral vessels and one stent in the Blalock-Taussig shunt (BT) with dramatic increase in vessel size and improvement in saturations from 70% to 89%. Case 2 underwent placement of two overlapping stents in a collateral vessel with an increase in diameter of the collateral vessel from 2.3 to 6 mm and an improvement in saturation from 68% to 88%. Case 3 underwent placement of three overlapping stents in a BT shunt with an increase in diameter of the shunt from 2.2 to 6.6 mm and an improvement in saturation from 71% to 89%. All three patients had excellent clinical improvement and stable saturation at follow-up. Stent placement for maintaining patency of APCAs and aortopulmonary shunts is feasible and safe.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
3/13. Monozygotic twins with chromosome 22q11 microdeletion and discordant phenotypes in cardiovascular patterning.Monozygotic twins with chromosome 22q11 microdeletions offer an ideal situation to observe the association of microdeletion and disrupted cardiovascular patterning. We report monozygotic twins concordant for 22q11.2 microdeletion but discordant for cardiovascular patterning. Both twins showed identical intracardiac defects including tetralogy of fallot with pulmonary atresia. Nevertheless, their great vessel patternings were variable. These twins show that the mispatterning of the great vessels may not correlate with intracardiac morphogenesis. The discordant development of the great vessels, especially in the pulmonary vascular system, has clinical significance for prognosis. The phenotypic variability of cardiovascular anomalies seen in 22q11 microdeletion cannot be explained on the basis of genotypic difference.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
4/13. pulmonary atresia with hypoplastic right ventricle. A clinical embryological study.An unusual case of pulmonary atresia with an aberrant karyotype of 46,XX,t(6;8)(p21.2;q11.2) is reported. Fetal ultrasonic examination at the 20th week of gestation revealed a hypoplastic right ventricle and an intact interventricular septum. Authors summarize their postnatal findings in fetal heart and the large adjacent vessels with special reference to the pathogenesis of this rare congenital heart defect. The observation delineates right-ventricular outflow tract obstruction associated with an abnormal pulmonary blood supply. The anatomy of the systemic pulmonary collaterals was studied and correlated with multifocal disorders in the system of the pharyngeal arch arteries in the early embryonic development.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/13. Neonates with congenital cardiac defects with decreased pulmonary blood flow.Lesions that cause decreased pulmonary blood flow are those that obstruct the RVOT. In general, the radiographic presentation includes the following: (1) Thin, poorly visualized pulmonary blood vessels with little extension beyond the perihilar region; (2) Dark, hyperlucent lung fields; and (3) Moderate to severe cardiomegaly, depending on the cardiac defect(s) present.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/13. Percutaneous occlusion of a pulmonary aneurysm causing hemoptysis in a patient with pulmonary atresia and aortopulmonary collaterals.A 28-year-old male was referred for cardiac catheterization because of recurrent severe hemoptysis necessitating resuscitation and subsequently preventing weaning from ventilation. He had a history of pulmonary atresia, ventricular septal defect, overriding aorta with right-sided aortic arch diagnosed at birth. Eisenmenger's syndrome ensued and he was not felt to be suitable for corrective cardiac surgery. He had multiple major aortopulmonary collateral vessels to both lungs with a large aneurysm in an artery to the right lower lobe, which was suspected to be the source of his bleeding. Occlusion of this aneurysm was achieved percutaneously using an Amplatzer septal occluder device. He had no further bleeding and was successfully weaned from ventilation. Six months later, he has recovered to his functional baseline and has not had any further episodes of hemoptysis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/13. Successful pregnancy in a woman with cyanotic congenital heart disease after a palliative pulmonary-systemic shunt. A case report.A 20-year-old woman with cyanotic congenital heart disease composed of corrected transposition of the great vessels, severe pulmonic stenosis, atresia of the left pulmonary artery and a large ventricular septal defect, had a successful pregnancy following a pulmonary-systemic shunt (Blalock-Taussig). The hemoglobin decreased from 21 to 16 g/dL following the operation. The antepartum course was complicated by intrauterine growth retardation and pregnancy-induced hypertension. A normal fetal nonstress test and biophysical profile permitted continuation of the pregnancy until 38 weeks' gestation, with delivery of a healthy infant.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/13. balloon occlusion scintigraphy of aortopulmonary collaterals.We evaluated two children with pulmonary atresia for coil embolization of aortopulmonary collateral vessels after placement of palliative aortopulmonary shunts. To determine vessel distribution and lung perfusion prior to collateral embolization, perfusion scintigraphy with technetium 99m-labeled macroaggregated albumin assessed pulmonary blood flow before and after balloon wedge catheter occlusion of the collaterals. In the first patient we found no perfusion defect during collateral occlusion, and we proceeded with embolization. In the second child, perfusion scintigraphy during occlusion of the collateral vessels demonstrated a filling defect, and embolization was not performed, thus avoiding the creation of a potential perfusion defect in this patient. Assessing the physiologic significance of aortopulmonary collateral vessels by utilizing temporary balloon occlusion of the collateral vessels and concurrent perfusion scintigraphy as an adjunct to selective angiography can provide a significant contribution to the safety and accuracy of coil embolization.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
9/13. pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature.The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect is highly variable. Several cases have been reported in the literature in which a coronary artery to pulmonary artery fistula, alone or in combination with additional vessels, supplies the pulmonary circulation. We report a case of successful repair during early infancy, with unique hemodynamic, clinical, and anatomic features. The literature is reviewed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/13. Avoiding hypoxemia during unifocalization.An 11-month-old child underwent unifocalization of the major aortopulmonary collateral arteries, but did not tolerate occlusion of both vessels simultaneously. Using a Y-shaped homograft tube, we translocated the vessels sequentially and avoided severe hypoxemia.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
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