Commentary: Cor triatriatum dexter: Persistent tissue causing protean issues!

Abstract

Central MessageCor triatriatum dexter is a rare congenital cardiac anomaly with varied presentations. A high index of suspicion is necessary for accurate diagnosis and treatment.See Article page 254. Cor triatriatum dexter is a rare congenital cardiac anomaly with varied presentations. A high index of suspicion is necessary for accurate diagnosis and treatment. See Article page 254. Cor triatriatum dexter is a rare congenital cardiac anomaly caused by the abnormal persistence of embryonic tissue guarding the primitive sinoatrial orifice.1Doucette J. Knoblich R. Persistent right valve of the sinus venosus. So-called cor triatriatum dexter: review of the literature and report of a case.Arch Pathol. 1963; 75: 105-112PubMed Google Scholar,2Nakano S. Kawashima Y. Miyamoto T. Kitamura S. Manabe H. Supravalvular tricuspid stenosis resulting from persistent right sinus venosus valve.Ann Thorac Surg. 1974; 17: 591-595Abstract Full Text PDF PubMed Scopus (7) Google Scholar The pathophysiology is determined by a number of factors that come into interplay, including the size of the sinoatrial orifice, the presence of atrial communication, and the degree of tricuspid valve obstruction. The manifestations are indeed variable, with clinical presentation ranging from abdominal distension and cyanosis to incidental findings during correction of other cardiac malformations.3Alkhulaifi A.M. Serraf A. Planche C. Ascites and weight loss in a child: due to congenital division of the right atrium.Cardiol Young. 1999; 9: 335-337Crossref PubMed Google Scholar,4Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar The chronicity of this lesion only adds to its protean character as illustrated in this case report.5Kalangos A. Shatelen N. Demyanchuk V. Ruban N. Sfyridis P. Todurov B. et al.Cor triatriatum dexter in children: literature review and case report.J Thorac Cardiovasc Surg Tech. 2020; 4: 254-258Google Scholar Tricuspid regurgitation is an unusual manifestation of cor triatriatum dexter and probably related to repetitive trauma and consequent deformation of the tricuspid valve leaflets from the bulging membrane. Although the authors have done a commendable job of collating the various case reports on this rare congenital heart defect from literature, the disease is under-reported and not as exotic as this study suggests.5Kalangos A. Shatelen N. Demyanchuk V. Ruban N. Sfyridis P. Todurov B. et al.Cor triatriatum dexter in children: literature review and case report.J Thorac Cardiovasc Surg Tech. 2020; 4: 254-258Google Scholar Most cases are asymptomatic and others coexist as part of other complex congenital heart defects. The study also raises an important question regarding the risks of conservative management in a largely asymptomatic child with well-documented membrane in the right atrium. The risks to the tricuspid valve tissue should not be ignored and the surgical correction of the defect can become complicated. The challenge in management of this rare disorder is primarily related to its diagnosis, as the correction itself is usually straightforward. In addition to maintaining a high index of suspicion, the usage of a second modality of imaging such as cardiac magnetic resonance imaging can go a long way in making an accurate diagnosis.6Rao S. Suntharos P. Najm H. Komarlu R. Cor triatriatum dexter with right ventricular hypoplasia: role of multimodality imaging in decision making.Echocardiography. 2018; 35: 2113-2116Crossref PubMed Scopus (3) Google Scholar Cor triatriatum dexter in children: Literature review and case reportJTCVS TechniquesVol. 4PreviewCor triatriatum dexter (CTD) is one of the rarest congenital cardiac malformations in which there is an abnormal septation of the right atrium (RA) due to the failure of regression of the embryonic right-sided sinus venosus valve.1 We present our experience regarding a young patient with CTD associated with severe tricuspid regurgitation and review the 13 pediatric cases of CTD previously reported in the literature who underwent surgery (Table 1).1-12 Informed consent was received for our patient. Full-Text PDF Open Access