1/41. Divided left atrium in association with tricuspid atresia and discordant ventriculo-arterial connections.Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/41. Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn.This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin iii deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
3/41. tricuspid atresia with persistent truncus arteriosus.The association of tricuspid atresia and persistent truncus arteriosus is a very rare congenital anomaly. We report a newborn with a prenatal diagnosis of tricuspid atresia, in whom associated type II persistent truncus arteriosus was found by postnatal echocardiography. The patient had mild cyanosis and developed heart failure soon after birth. Balloon septostomy was performed to enlarge the interatrial communication. However, her condition rapidly deteriorated and she died of sepsis and heart failure at the age of 14 days.- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |
4/41. thromboembolism originated from the pulmonary artery stump after Fontan operation.Cerebral thromboembolism is a rare but serious complication after Fontan operation. This is the report of a patient who underwent a successful intracardiac thrombectomy for cerebral thromboembolism after Fontan operation. A 2-year-old girl was referred to us with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, and a ventricular septal defect. Although she underwent a successful Fontan operation and division of the main pulmonary artery, she developed a cerebrovascular event at 3 weeks after the operation. echocardiography demonstrated a large thrombus within the residue of the main pulmonary artery, and suggested that the thrombus had migrated into the systemic circulation by way of the ventricular septal defect. At 2 weeks after the cerebrovascular event, she underwent thrombectomy and excision of the pulmonary valve. Although she has developed slight left-sided hemiparesis, she is leading a normal life at 1 year after the operation.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
5/41. Diagnosis, management, and pathophysiology of post-Fontan hypoxaemia secondary to Glenn shunt related pulmonary arteriovenous malformation.An 8 year old child with tricuspid atresia had developed right sided pulmonary arteriovenous malformations following a previous classic Glenn procedure. These became clinically manifest immediately after Fontan conversion because of severe systemic desaturation. The pathophysiology and postoperative medical management of this case is described and related to current understanding of the aetiology of acquired pulmonary arteriovenous malformations following cavopulmonary shunt.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
6/41. Operative treatment of truncus arteriosus communis coexisting with tricuspid atresia.The coexistence of tricuspid atresia and common arterial trunk is extremely rare. We present a successful three-stage surgical treatment of this defect. The first stage included disconnection of the pulmonary arteries from the common trunk, atrial septectomy and systemic-to-pulmonary shunt; the second stage, a hemi-fontan procedure; and the third stage, a fenestrated Fontan completion. The child is now 8 years old and is developing well.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
7/41. Stent implantation in a central aorto-pulmonary shunt.A 5.5-week-old infant with tricuspid atresia presented with severe hypoxemia not responding to the placement of a central shunt (4 mm polytetrafluorethylene). The infant was taken to the catheterization laboratory, where an AVE stent was successfully implanted in a severe postoperative stenosis, at the pulmonary end of the anastomosis. The oxygen saturation improved significantly, however, the infant died due to renal failure. The post-mortem anatomical findings are shown.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
8/41. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.Absence of the pulmonary valve occurs usually in association with tetralogy of fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.- - - - - - - - - - ranking = 1.4keywords = atresia (Clic here for more details about this article) |
9/41. Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia.At 6 years of age, a girl with tricuspid atresia underwent a Bjork modified fontan procedure with implantation of a Carpentier Edwards bioprosthesis between the right atrium and the right ventricle. Ten years later she developed increasing edema, ascites and pleural effusions. The work-up showed severe stenosis of the bioprosthesis and protein losing enteropathy with a massive decrease of the albumin level to 14 g/l (normal 40-50 g/l). At 17 years of age, the bioprosthesis was replaced with a direct anastomosis between the cavoatrial junction and the right pulmonary artery. Within one month post-operatively, extensive thrombosis of the superior vena cava, anonymous and subclavian veins occurred. Protein-losing enteropathy persisted with an albumin level of 17 g/l. Parallel to the successful treatment of these thrombi with high molecular heparin and urokinase, protein losing enteropathy and hypoalbuminemia resolved completely as long as the antithrombotic treatment with high molecular heparin was continued. Oral anticoagulation was ineffective. Chronic antithrombotic treatment with high molecular heparin may thus be the treatment of choice in these forms of protein-losing enteropathy associated with venous thrombosis.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
10/41. Absorbable pulmonary artery banding in tricuspid atresia.A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
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