Cor Triatriatum Dexter Associated With a Dysplastic Tricuspid Valve

•Cor triatriatum dexter results from persistence of the right valve sinus venosus.•A 3D evaluation can help differentiate CTD other congenital abnormalities.•Treatment options for include surgery, first line, or percutaneous resection. Cor (triatrial heart) is a rare abnormality in which either left atrium (cor sinister) (RA; cor [CTD]) divided into 2 separate chambers. The was described 1860s and named by Borst 1905.1Borst H. Ein triatriatum.Zentralble Allg Pathol. 1905; 16: 812-815Google Scholar,2Nassar P. Hamdan R. sinistrum: classification imaging modalities.Eur J Cardiovasc Med. 2011; 1: 84-87Crossref PubMed Google Scholar sinister related to malincorporation common pulmonary vein atrium, whereas distinct pathophysiologic failure regression venosus.2Nassar Scholar,3Dobbertin A. Warnes C. Seward J. an adult diagnosed transesophageal echocardiography: case report.J Am Soc Echocardiogr. 1995; 8: 952-957Abstract Full Text PDF typically presents childhood, with exertional cyanosis dyspnea, degree obstruction resultant shunting blood vena cavae coronary across foramen ovale.4Feld Shani Rudansky Rudyak E. Greengart Initial presentation 55-year-old woman.Am Heart 1992; 124: 788-791Crossref Scopus (33) has been associated patent ovale (PFO) atrial septal defects (ASDs) due toward ovale.5Schuchlenz Saurer G. Weihs W. Rehak Persisting eustachian adults: relation cerebrovascular events.J 2004; 17: 231-233Abstract (151) Scholar,6Vukovic Kosevic D. Milicic M. Jovovic L. Stojanovic I. Micovic S. defect 43-year-old woman.Tex Inst 2014; 41: 418-420Crossref (12) also heart abnormalities, including Ebstein anomaly, hypoplastic ventricle, stenosis atresia, tricuspid dysplasia, that are reduced flow through during embryonic development.6Vukovic We present 51-year-old woman referred clinic murmur found have regurgitation (TR) dysplastic (TV). A medical history spina bifida, chronic kidney disease stage 4 (with prior transplant 2012), Arnold-Chiari malformation, deep thrombosis inferior cava (IVC) filter, had removed, hypothyroidism, type diabetes mellitus, hypertension cardiology murmur, noted their nephrologist. patient they told as child but never echocardiogram further evaluation. recently having worsening dyspnea on exertion only able walk few dozen feet before stop catch breath. denied any chest pain tightness. patient’s physical examination notable grade 3 out 6 holosystolic loudest along sternal border increasing inspiration. complete count baseline anemia hemoglobin 11.1 g/dL, attributed disease, oxygen saturation 97% room air. transthoracic echo (TTE) performed showed TR acceleration, aliasing color-flow Doppler, RA diastole (Figure 1, Video 1), TTE technically difficult study. (TEE). TEE demonstrated normal ventricular size function mild mitral (small narrow central jet, A-wave dominant inflow, incomplete jet continuous-wave size). ventricle moderately dilated (same ventricle), there enlargement. large membranous structure visualized concerning giant Eustachian (EV) 2, 2). Color-flow Doppler illustrated TR, visually estimated severe, divergent jets 3, 3), one pointed membrane, diastolic TTE. There appeared be TV leaflet, prolapse, annular dilation, best three-dimensional (3D) (Video 4). No PFO, secundum ASD, venosus evidence partial anomalous venous return visible, although agitated saline contrast study not performed.Figure 3Two-dimensional TEE, midesophageal short-axis view (63°) demonstrates (arrows) systole (A) acceleration (arrow) (B).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Given concern versus EV, membrane because it unclear whether solely originating IVC orifice. Three-dimensional dividing went anterior wall superior single perforation present, allowed anterograde flow, measured around 11 mm. Pulsed-wave performed, small mm Hg gradient 5) membrane. Significant reversal seen pulsed-wave 5, 5).Figure 5Pulsed-wave spectral display obtained at fenestration mild, systolic TR.View currently undergoing multidisciplinary center excellence consideration surgical transcatheter resection replacement. involution venosus, forms fifth week embryogenesis.7Alboliras Edwards Driscoll dexter: two-dimensional echocardiographic diagnosis.J Coll Cardiol. 1987; 9: 334-337Crossref Scholar,8Moral Ballesteros Huguet Panaro Palet Evangelista Differential diagnosis clinical implications remnants venosus.J 2016; 29: 183-194Abstract (36) expands between sixth eighth embryogenesis functions direct fossa ovalis systemic circulation.8Moral involutes ninth 15th covers IVC, Thebesian valve, orifice sinus.8Moral These structures visible 65% 90% people current techniques autopsy studies.8Moral Failure spectrum anomalies, most minor presenting prominent EV resulting continued direction caval (present 13% patients cryptogenic stroke).8Moral Incomplete resorption result persistent Chiari network thin reticulated fibers RA.8Moral Persistence CTD, fenestrated separating upper lower cavity, cause necessitating repair.8Moral Importantly, remnant produces antegrade flow.8Moral Differentiation difficult, given exist spectrum. Specifically, confused, echocardiography assist diagnosis.8Moral produce useful determination potential RA, if would suggest CTD.8Moral divides chambers, origin insertion crista terminalis.8Moral originate same location, no attachments In past, discovered following studies, advances cardiovascular magnetic resonance imaging, become vital making diagnosis.9Roldan F. Barron Zavaleta N. Cardenas Keirns Antona et al.Cor 3-dimensional reconstruction.J 2001; 14: 634-636Abstract Both well visualize right-sided cardiac defects, PFO ASD assess obstruction, distinguishing aneurysms, masses, mistaken.9Roldan Scholar, 10Yavuz T. Nazli Kinay O. Kutsal Giant appearance atrium.Tex 2002; 336-338PubMed 11Ozeke Celik Basel caused interatrial aneurysm.J 2008; 21: 91.E1-91.E2Abstract 12Goldfarb Weinreb Daniel Kronzon membranes: Role 1989; 2: 350-353Abstract Once diagnosed, treatment recommended high defined causing symptoms manifestations present.13Bisinov Dieter Ballantyne Wolff Stein Echocardiographic catheter hypotension dexter.J 2003; 897-898Abstract (0) Typically, balloon dilation relieve 1 stent placement completed successfully.13Bisinov Scholar,14Derimay Gamondes Rioufol First correction isolated (or triatriatum) dexter.Can 2021; 37: 1867-1869Abstract Surgical resection, candidate, indicated valvular return. For this patient, closure relatively minimal led delay presentation. Larger degrees resulted more severe dysplasia right-to-left shunting, may earlier time Physical subsequent revealed, however, impairment function. restrict redirection ovale, anomalies. Preoperative knowledge essential will affect accessibility multiple defects.