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Robert H. Anderson - One of the best experts on this subject based on the ideXlab platform.
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atrioventricular septal defect with coexisting Tricuspid atresia
Cardiology in The Young, 2011Co-Authors: Vera Demarchi Aiello, Jorge Yussef Afiune, Samuel Menahem, Robert H. AndersonAbstract:We describe two infants having an atrioventricular septal defect in the setting of a double inlet atrioventricular connection, but with patency of the left-sided valvar orifice and an imperforate right-sided valvar component, and a further case with atrioventricular septal defect and an imperforate Ebstein's malformation, all producing the haemodynamic effect of Tricuspid atresia. We make comparisons with the arrangement in trisomy 16 mice, in whom deficient atrioventricular septation is seen at times with the common atrioventricular junction exclusively connected to the left ventricle, a situation similar to that seen in two of our infants. We also review previous reports emphasising the important theoretical implication of the findings despite their rarity.
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myoarchitecture and connective tissue in hearts with Tricuspid atresia
Heart, 1999Co-Authors: Damian Sanchezquintana, Vicente Climent, Robert H. AndersonAbstract:Objective—To compare the atrial and ventricular myoarchitecture in the normal heart and the heart with Tricuspid atresia, and to investigate changes in the three dimensional arrangement of collagen fibrils. Methods—Blunt dissection and cell maceration with scanning electron microscopy were used to study the architecture of the atrial and ventricular musculature and the arrangement of collagen fibrils in three specimens with Tricuspid atresia and six normal human hearts. Results—There were significant modifications in the myoarchitecture of the right atrium and the left ventricle, both being noticeably hypertrophied. The middle layer of the ventricle in the abnormal hearts was thicker than in the normal hearts. The orientation of the superficial layer in the left ventricle in hearts with Tricuspid atresia was irregular compared with the normal hearts. Scanning electron microscopy showed coarser endomysial sheaths and denser perimysial septa in hearts with Tricuspid atresia than in normal hearts. Conclusions—The overall architecture of the muscle fibres and its connective tissue matrix in hearts with Tricuspid atresia differed from normal, probably reflecting modelling of the myocardium that is inherent to the malformation. This is in concordance with clinical observations showing deterioration in pump function of the dominant left ventricle from very early in life. Keywords: Tricuspid atresia; congenital heart defects; connective tissue; fibrosis
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echocardiographic morphologic correlations in Tricuspid atresia
Journal of the American College of Cardiology, 1995Co-Authors: Joseph Orie, Christine Anderson, Jose A Ettedgui, James R Zuberbuhler, Robert H. AndersonAbstract:Objectives Our aim was to clarify the anatomic substrate in hearts diagnosed as having Tricuspid atresia by studying autopsy specimens and comparing the findings with those in twodimensional echocardiograms. Background Traditionally, Tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle. Methods We examined 39 autopsy specimens catalogued as having Tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of Tricuspid atresia. Results Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate Tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass. Conclusions Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.
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Tricuspid atresia presenting in infancy survival and suitability for the fontan operation
Circulation, 1993Co-Authors: Rodney C G Franklin, Robert H. Anderson, David Spiegelhalter, Ian D Sullivan, D G Thoele, Elliot A Shinebourne, J E DeanfieldAbstract:BACKGROUNDThe Fontan operation is the usual goal of therapy for children with Tricuspid atresia. The influences of morphology and different management strategies on survival and subsequent suitability for this procedure are crucial but unstudied in an unselected population during the Fontan era.METHODS AND RESULTSThe fates of 237 consecutive infants with Tricuspid atresia were reviewed (1972-1987; median follow-up, 8.0 years). Overall actuarial survival was 72% at 1 year, 53% at 5 years, and 46% at 10 years. Univariate risk factor analysis established that discordant ventriculoarterial connections (24% of the group; relative risk, 2.7), pulmonary atresia (14%, 2.3), aortic arch obstruction (7%, 2.9), and subaortic stenosis (8%, 4.2) were associated with poor survival, whereas pulmonary stenosis (60%, 0.52), balanced pulmonary blood flow (9%, 0.25), and older age at presentation (33%, 0.42) were beneficial. Multivariate analysis allowed the creation of predictive patient-specific survival curves and two ad...
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Atrial morphologic features in Tricuspid atresia.
The Journal of thoracic and cardiovascular surgery, 1991Co-Authors: David G. Thoele, Audrey Smith, Philip C. Ursell, Frederick O. Bowman, Welton M. Gersony, Robert H. AndersonAbstract:We studied the atrial morphology in 110 hearts removed at autopsy from patients with Tricuspid atresia. Ten of the patients had had a Fontan operation. We compared the findings in these hearts with those in 30 normal hearts. Prominent eustachian valves were common (40%). The Tricuspid "dimple," present in 33 of 100 specimens, denoted the membranous atrioventricular septum and was never related to the right ventricle. Most interatrial communications (85/100) were nonobstructive. A minority of patients with obstructive interatrial communications (13/100 = small, 2/100 = severely obstructive) would be expected to benefit from balloon atrial septostomy early in life. Right atrial hypertrophy was greatest in specimens with restrictive interatrial communications (severely obstructive = 3.2 +/- 0.3 mm, small = 2.1 +/- 0.6 mm) and in those that had been subjected to a Fontan operation (3.2 +/- 0.7 mm), but thickness was also increased in hearts with nonobstructive interatrial communications (1.6 +/- 0.5 mm) compared with normal hearts (1.1 +/- 0.2 mm; p less than 0.001 versus all groups). Of the patients in the series, 11 of 100 had left juxtaposition of the atrial appendages, which may have important implications for the Fontan operation.
Shuchien Huang - One of the best experts on this subject based on the ideXlab platform.
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modified norwood procedure for Tricuspid atresia transposition of great arteries and hypoplastic right arch with complete vascular ring
The Annals of Thoracic Surgery, 2016Co-Authors: Chanyang Hsu, Jinchung Shih, Shyhjye Chen, Yihsharng Chen, Shuchien HuangAbstract:We report a modified Norwood stage I procedure for Tricuspid atresia, transposition of great arteries, and hypoplastic right aortic arch with complete vascular ring. In this technique, we applied dual arterial cannulation to avoid circulation arrest during neoaortic reconstruction, and also corrected the arch laterality during the Norwood stage I palliation procedure. Pulmonary flow was supplied by the Blalock-Taussig shunt. Postoperative imaging revealed the patent left neoaortic arch, and the vascular ring was relieved with a patent tracheobronchial tree.
C Berg - One of the best experts on this subject based on the ideXlab platform.
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prenatal diagnosis of Tricuspid atresia intrauterine course and outcome
Ultrasound in Obstetrics & Gynecology, 2010Co-Authors: C Berg, Robert Lachmann, C Kaiser, P Kozlowski, R Stressig, M Schneider, B Asfour, U Herberg, Johannes Breuer, U GembruchAbstract:Objective To evaluate the intrauterine course and outcome of Tricuspid atresia detected in the fetus. Methods This was a retrospective review of all confirmed cases of Tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. Results Fifty-four cases of Tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46.3%) cases had a discordant ventriculoarterial connection of which 14 also had aortic outflow obstruction, six had pulmonary outflow tract obstruction and two had other associated intracardiac anomalies; and one (1.9%) had a common arterial trunk. The peak velocity index for veins in the ductus venosus was significantly elevated in 19 of the 37 (51.4%) cases assessed; however, this finding did not correlate with adverse intrauterine outcome. There were associated extracardiac anomalies in 12 cases: five with chromosomal anomalies, two with VACTERL association, one with unilateral renal agenesis, one with hypospadia, one with hydrothorax, one with megacystis and one with agenesis of the ductus venosus. Seventeen of the 54 (31.5%) cases underwent termination of pregnancy, two (3.7%) died in utero, two (3.7%) died in infancy and 33 (61.1%) children survived with a median follow-up of 26 (range, 12–120) months. Prenatal echocardiography correctly anticipated the postnatal course and the need for neonatal intervention in 29/35 (82.9%) continued pregnancies; in the remaining six (17.1%) cases the right outflow tract obstruction had been underestimated. Conclusions Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd.
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Tricuspid atresia with absent pulmonary valve and intact ventricular septum intrauterine course and outcome of an unusual congenital heart defect
Ultrasound in Obstetrics & Gynecology, 2010Co-Authors: K Lato, M Schneider, U Gembruch, A Geipel, R Lachmann, Viktor Hraska, C BergAbstract:The extremely rare syndrome including absent pulmonary valve associated with membranous Tricuspid atresia or severe Tricuspid stenosis, intact ventricular septum and patent ductus arteriosus has been reported sporadically in the postnatal literature. This cardiac defect is characterized by right ventricular dysplasia with asymmetrical ventricular septal hypertrophy, ventricular septum bulging into the left ventricle, small right ventricular cavity, membranous Tricuspid atresia or severe stenosis with abnormal papillary muscles and leaflets and absence of the pulmonary valve leaflets. The only prenatal case reported so far was diagnosed at 33 weeks of gestation and terminated shortly thereafter; the natural history of prenatally diagnosed cases is therefore unknown. We report on the intrauterine course of a case diagnosed at 17 weeks of gestation that had a favorable postnatal outcome after palliation.
Frank Cecchin - One of the best experts on this subject based on the ideXlab platform.
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ablation of atrioventricular nodal reentrant tachycardia in Tricuspid atresia
Journal of Cardiovascular Electrophysiology, 2004Co-Authors: P Khairy, Stephen P Seslar, John K Triedman, Frank CecchinAbstract:A 31-year-old woman with Tricuspid atresia and a palliative Waterston shunt presented with intractable poorly tolerated supraventricular tachycardia. Electrophysiologic evaluation was consistent with AV nodal reentrant tachycardia. The fast pathway region was mapped electroanatomically during tachycardia and with constant rate ventricular pacing. Successful radiofrequency ablation was achieved by placement of lesions in an area adjacent and inferior to the His-bundle electrogram on the left side of the septum.
Chanyang Hsu - One of the best experts on this subject based on the ideXlab platform.
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modified norwood procedure for Tricuspid atresia transposition of great arteries and hypoplastic right arch with complete vascular ring
The Annals of Thoracic Surgery, 2016Co-Authors: Chanyang Hsu, Jinchung Shih, Shyhjye Chen, Yihsharng Chen, Shuchien HuangAbstract:We report a modified Norwood stage I procedure for Tricuspid atresia, transposition of great arteries, and hypoplastic right aortic arch with complete vascular ring. In this technique, we applied dual arterial cannulation to avoid circulation arrest during neoaortic reconstruction, and also corrected the arch laterality during the Norwood stage I palliation procedure. Pulmonary flow was supplied by the Blalock-Taussig shunt. Postoperative imaging revealed the patent left neoaortic arch, and the vascular ring was relieved with a patent tracheobronchial tree.
