Even though pulmonary atresia with intact ventricular septum is an infrequent defect, accounting for less than 1% of congenital heart defects, it is the 3 rd most frequent cyanotic heart defect in the neonatal period 1 . Its morphological spectrum is broad with cases ranging from extremely hypoplastic tricuspid valves and right ventricles to ventricular cavities of almost normal dimensions [2][3][4] . Ebstein's anomaly of the tricuspid valve and extreme ventricular dilation have also been reported, and they are associated with a poor prognosis 5 . In addition, communication between the right ventricular cavity and the coronary arteries is a relatively common finding, which is sometimes associated with coronary circulation partially or totally dependent on the right ventricle [6][7][8] . Due to this anatomic heterogeneity, the therapeutic algorithm should be individualized 6 . The final objective is always to attain biventricular correction with total separation between systemic and pulmonary circulations 6 . However, this is sometimes impossible, and correction with a 1½ ventricle or of the univentricular type (Fontan) is necessary [6][7][8][9] . Cardiac transplantation should also be considered for treating cases with severe stenoses or multiple interruptions in the coronary arteries and secondary left ventricular dysfunction 6,8 . The initial therapeutic approach in the neonatal period should, whenever possible (if the coronary circulation pattern allows), open the pulmonary valve to decompress the right ventricle and stimulate its growth 3,[6][7][8] . During the last decade, perforation of the pulmonary valve to establish continuity between the right ventricle and the pulmonary artery with the aid of interventional catheterization became a reality [10][11][12][13][14][15] , even in Brazil 16. We report 2 percutaneous techniques of valve perforation, which were recently introduced into clinical practice, and their advantages and disadvantages are discussed. Case reportWe report the cases of 3 patients referred to our service from other neonatal units for investigation or treatment of cyanotic congenital heart defects. The clinical, echocardiographic, and hemodynamic data, are listed in tables I, II, and III. It is worth noting that patient 2 had a previous diagnosis of critical pulmonary stenosis. All patients had mild to moderate cyanosis under continuous infusion of prostaglandin; on auscultation, the 2 nd cardiac sound was single and low, and was followed by a mild systolic murmur in the dorsum. On chest X-ray, the cardiac silhouette was slightly enlarged, mainly because of the right atrium, and the pulmonary flow was reduced. The electrocardiogram showed sinus rhythm and left ventricular hypertrophy in all patients. QRS axes ranged from 90º to 120º. In regard to the echocardiographic findings, all patients had situs solitus, pulmonary atresia with imperforate pulmonary valve, and intact ventricular septum. The right ventricle was hy-
We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel.Based on the publication of results of convincing experimental studies 1 , the new Amplatzer device for percutaneous occlusion of ductus arteriosus (AGA Medical Corporation, Golden Valley, MN, USA) has been shown to be safe and highly effective in clinical practice [2][3][4][5] . Due to the versatility of the implantation system and the characteristics of the prosthesis, it has also been used for occlusion of other defects and vascular malformations, such as coronary and pulmonary arteriovenous fistulae, systemic-pulmonary shunts (Blalock-Taussig), venovenous collaterals after Glenn surgery, tubes, fenestrated Fontan, and others 6-8 . We report a case in which this device was successfully used for closure of a large systemic-pulmonary collateral vessel originating from the descending aorta. Case ReportThe patient is a 17-year-old male who underwent surgical repair of pulmonary atresia, ventricular septal defect, and systemic-pulmonary collaterals at the age of 7 years. The collaterals were focalized, the ventricular septal defect was occluded with a patch of bovine pericardium and an 18-mm Dacron tube graft, which was used to reestablish the continuity between the right ventricle and the pulmonary artery (Rastelli technique). The patient complained of mild fatigue on exertion, and on physical examination an ejective systolic murmur in the middle left sternal margin and a continuous murmur in the dorsum could be heard. On chest X-ray, slight cardiomegaly and increased pulmonary flow to the right middle and lower lobes were observed. The electrocardiogram showed sinus rhythm and biventricular hypertrophy. The twodimensional Doppler color flow echocardiogram showed signs of obstruction of the tube with rectification of the ventricular septum during systole. Right ventricular pressure was estimated in approximately 3/4 of the arterial blood pressure through tricuspid reflux. Moderate pulmonary insufficiency also existed. The patient was referred for cardiac catheterization for diagnostic complementation, and the examination, performed with the patient under local anesthesia, showed the following pressure levels (in mm Hg): right atrium, 9; right ventricle, 90/10; pulmonary artery, 30/11; left ventricle, 110/12; and aorta, 110/ 60. Right ventriculography revealed obstruction of the tube in its proximal portions, next to the right ventricular musculature, and mild to moderate systolic dysfunction. The aortogram, followed by selective injection in the vessel, sho...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.