Cor triatriatum dexter (CTD) is an extremely rare finding resulting from the persistence of the right valve of sinus venosus. It is a congenital cardiac anomaly defined by an abnormal septation of the atrium leading to inflow obstruction to the respective ventricle. Multimodal diagnostic modalities are necessary to characterize it for an optimal patient management. We report the case of a 68-year-old woman who presented to our clinic for further feedback of ventricular ectopic beats

Chieppa, Domenico Riccardo Rosario

De Michele, Francesca

Forte, Valentina

Guglielmi, Giuseppe

Nemore, Francesco

Paparella, Maria Teresa

English

Archivio Istituzionale della Ricerca- Università degli Studi di Foggia

beats, we performed a contrast CMR to eventually demonstrate the presence of ventricular fibrosis with the Late Gadolinium Enhancement (LGE) technique, which ruled out the presence of fibrosis. The sequences cine SSFP, most commonly used to study the cardiac morphology, demonstrated the presence of a mem-brane and an accessory chamber in greater detail, which showed the presence of CTD (Figure 1).Atrial chambers were dilatated and the ventricles were of normal dimensions with a normal functionally parameters. In the absence of any heart hemodynamic distubances CTD can be considered a rare incidentaloma. The patient was discharged with beta blockers therapy and the strategy of watchful observation was adopted.Case ReportA 68-year-old woman was referred to our clinic for further feedback of ventricular ectopic beats. Physical exam revealed irregular rhythm and no signs of heart failure. Transthoracic echocardiogram was performed and showed preserved left ventricular ejection fraction, dilated left atrium, and severely dilated RA. We didn’t find any interatrial communication. Mild tricuspid and mitral regurgitation was reported. A diagnostic study was requested by means of cardiac magnetic resonance (CMR) to obtain in the first case a response to ven-tricular ectopic beats. To answer to the diagnostic sus-picious of cardiologists about the ventricular ectopic Acta Biomed 2021; Vol. 93, Supplement 1: e2022093 DOI 10.23750/abm.v93iS1.12585 © Mattioli 1885C a s e  r e p o r tCor triatriatum dexter: a rare incidentalomaDe Michele Francesca1, Paparella Maria Teresa1, Forte Valentina2, Chieppa Domenico Riccardo Rosario3, Nemore Francesco2, Guglielmi Giuseppe1,41Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, Foggia, Italy; 2Radiology Unit, ‘’San Nicola Pellegrino Hospital’’,Trani, Italy; 3Cardiology Unit, “Bonomo” Hospital, Andria, Italy; 4Radio-logy Unit, Barletta University Campus UNIFG, “Dimiccoli” Hospital, ItalySummary. Cor triatriatum dexter (CTD) is an extremely rare finding resulting from the persistence of the right valve of sinus venosus. It is a congenital cardiac anomaly defined by an abnormal septation of the atrium leading to inflow obstruction to the respective ventricle. Multimodal diagnostic modalities are necessary to characterize it for an optimal patient management. We report the case of a 68-year-old woman who presented to our clinic for further feedback of ventricular ectopic beats.Key words: cor triatriatum dexter (CTD), atrial sept defect, cardiac magnetic resonanceIntroductionCor triatratum dexter (CTD) is a partitioning of the right atrium (RA) to form a triatrial heart. It is an extremely rare congenital anomaly caused by the persistence of the right valve of the sinus venosus (1). The prevalence of CTD is only approximately 0.025% of cases of all congenital heart diseases (2). It has no gender predilection and commonly presents in infancy but may remain undetected till death (3). Symptomatology may mimic tricuspid stenosis, producing signs of systemic venous congestion (4). This paper presents a case of CTD in a 68-year-old woman without hemodynamic disturbances.Acta Biomed 2021; Vol. 93, Supplement 1: e20220932Discussion: Cor triatriatum dexter is a rare con-genital heart disease characterized by a separation be-tween the smooth posterior part and the trabeculated anterior part of the RA due to the persistence of the right valve of the right horn of the sinus venosus. The right horn of the sinus venosus is incorporated into the right atrium, giving rise to the posterior smooth part of the right atrium called sinus venarum, the orifices of the superior vena cava and inferior vena cava. During development, the right valve of the si-nus venosus divides the right atrium and then ends up leaving the crista terminalis, eustichian valve, and thebasian valve (5, 6). The membrane in CTD may range from a reticulum to a substantial sheet of tis-sue. The most common location of the membrane is at the right of superior vena cava, coronary sinus, and inferior vena cava, and the next common location is at the left of coronary sinus and to the right of su-perior and inferior vena cava (7). Right ventricular hypoplasia, pulmonary valve atresia, pulmonary valve stenosis, and Ebstein anomaly are communally associ-ated with CTD (8,9). Multimodal imaging modalities with echocardiography, transesophageal echography and CMR are necessary to confirm this condition and may be required to delineate and differentiate it from others cardiac anomalies. Moreover, they also directly visualize the presence of the membrane that divides the atrial chamber into two separate chambers. CMR could play an important role where the transthoracic echocardiography can’t show the real cardiac morphol-ogy. The degree of septation within the right atrium decides the age and severity of clinical manifestations, thus it may mimic tricuspid stenosis with signs of sys-temic venous congestion or when the septation is mild, the condition is often asymptomatic and represents an incidental finding frequently made at postmortem ex-amination. In symptomatic patients the mainstay of treatment for CTD is surgical resection or percuta-neous catheter disruption of the dividing membrane (10,11), conversely treatment is not required when the septum appears to have no hemodynamic significance.Conflict of Interest: Each author declares that he or she has no commercial associations (e.g. consultancies, stock ownership, equity interest, patent/licensing arrangement etc.) that might pose a con-flict of interest in connection with the submitted article.Figure 1. Four chamber visioncine SSFP. It is possible to view the presence of cor triatrium dexter during the atrial diastole and atrial systole.Acta Biomed 2021; Vol. 93, Supplement 1: e2022093 3References1. Hansing CE, Young WP, Rowe GG. Cor triatriatum dex-ter. Persistent right sinus venosus valve. Am J Cardiol. 1972 Oct; 30(5):559-64.2. Moral S, Ballesteros E, Huguet M, Panaro A, Palet J, Evan-gelista A. Differential Diagnosis and Clinical Implications of Remnants of the Right Valve of the Sinus Venosus. J Am Soc Echocardiogr. 2016 Mar;29(3):183-94.3. Jha AK, Makhija N. Cor Triatriatum: A Review. Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):178-185.4. Choudhary D, Sivasankaran S, Venkateshwaran S, Sasid-haran B. Cor triatiratum dexter: a rare cause of isolated right atrial enlargement. Pediatr Cardiol. 2013;34: 198-199.5. Goldfarb A, Weinreb J, Daniel WG, Kronzon I. A patient with right and left atrial membranes: the role of transesoph-ageal echocardiography and magnetic resonance imaging in diagnosis. J Am SocEchocardiogr. 1989;2:350-353.6. Trento A, Zuberbuhler JR, Anderson RH, Park SC, Siewers RD. Divided right atrium (prominence of the eustachian and thebesian valves). J ThoracCardiovascSurg. 1988;96: 457-463.7. Doucette J, Knoblich R. Persistent right valve of the sinus venosus. ArchPathol. 1963;75:105-112.8. Verel D, Pilcher J, Hynes DM. Cor triatriatumdexter. Br Heart J. 1970;32:714-716.9. Gerlis LM, Anderson RH. Cor triatriatumdexter with im-perforate Ebstein’s anomaly. Br Heart J. 1976;38:108-111.10. Feld H, Shani J, Rudansky HW, Rudyak E, Greengart A. Initial presentation of cor triatriatum in a 55-year-old woman. Am Heart J. 1992 Sep;124(3):788-91.11. Vukovic PM, Kosevic D, Milicic M, Jovovic L, Stojanovic I, Micovic S. Cor triatriatum dexter and atrial septal defect in a 43-year-old woman. Tex Heart Inst J. 2014 Aug 1;41(4):418-20.CorrespondenceArrived: 17/11/21Accepted: 17/11/21Giuseppe Guglielmi, MDProfessor of Radiology,Department of Clinical and Experimental Medicine,Foggia University School of Medicine, Viale L. Pinto 1, 71121 Foggia, ItalyE-mail: giuseppe.guglielmi@unifg.it

Cor triatriatum dexter: a rare incidentaloma

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Cor triatriatum dexter: a rare incidentaloma

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