Cor triatriatum dexter in children: Literature review and case report
2020; Elsevier BV; Volume: 4; Linguagem: Inglês
10.1016/j.xjtc.2020.08.024
ISSN2666-2507
AutoresAfksendiyos Kalangos, Nataliia Shatelen, Vitaly Demyanchuk, N. A. Ruban, Panagiotis G. Sfyridis, Б.М. Тодуров,
Tópico(s)Cardiovascular and Diving-Related Complications
ResumoCentral MessageCor triatriatum dexter is a rare congenital defect. Early cyanosis, Budd–Chiari syndrome, and progressive dyspnea are the most frequent symptoms. Membrane resection is indicated for symptomatic patients.See Commentaries on pages 259 and 261. Cor triatriatum dexter is a rare congenital defect. Early cyanosis, Budd–Chiari syndrome, and progressive dyspnea are the most frequent symptoms. Membrane resection is indicated for symptomatic patients. See Commentaries on pages 259 and 261. Cor 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.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 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).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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar, 4Nakano 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, 5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar, 6Kolouskova S. Lebl J. Snajderova M. Zemkova D. Hrobonova V. Honek T. et al.Growth in children with the exudative enteropathy syndrome due to a congenital heart defect—cor triatriatum dexter.Cas Lek Cesk. 1996; 135: 59-61PubMed Google Scholar, 7Alkhulaifi 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, 8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar, 11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar, 12Rao 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 Informed consent was received for our patient.Table 1Reported surgical cases of cor triatriatum dexter in childrenStudyAge at surgery and sexAdditional malformationsSymptomsSurgeryOutcomeDoucette and Knoblich, 19631Doucette 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 Scholar6 wk, MPA, HRV, HTV, PFO, ASDCyanosisModified Potts shuntDeath after surgeryFolger, 19682Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar9 y, FPA, HRV, HTVPDA, ASDCyanosis from 3 moPulmonary valvulotomy and tricuspid valvulotomyDeath after surgeryFolger, 19682Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar5½ mo, MSupravalvular and pulmonary valve stenosis, PDA, ASDCyanosis and dyspnea on exertionASD closure and opening of supravalvular PSDeath after surgeryJones and Niles, 19683Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar4 mo, MHRV, tricuspid atresia, ASD, VSDCyanosisPotts shunt at 4 moGlenn procedure at 8 yDeath at 10 y due to obstruction of RA flow by the large membraneNakano et al, 19744Nakano 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 Scholar7 y, FASD, peri-membranous VSD, HTVCyanosis early in life, fatigability and progressive dyspnea on severe exertion after the first year of lifeComplete membrane resectionDirect closure of VSDAliveMazzuco et al, 19835Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar5 mo, FLSVC draining into the LA, separate draining of hepatic veins into the RACyanosisComplete membrane resectionPericardial patch reconstruction of a new interatrial septumAlive 5 y after surgeryKolouskova et al, 19966Kolouskova S. Lebl J. Snajderova M. Zemkova D. Hrobonova V. Honek T. et al.Growth in children with the exudative enteropathy syndrome due to a congenital heart defect—cor triatriatum dexter.Cas Lek Cesk. 1996; 135: 59-61PubMed Google Scholar6 y, M–Exudative enteropathy syndromeComplete membrane resectionAliveAlkhulaifi et al, 19997Alkhulaifi 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 Scholar12 y, M–AscitesComplete membrane resectionAliveBarrea et al, 20098Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar4 mo, FLarge PFOCyanosisComplete membrane resectionAliveGalli et al, 20099Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar1 mo, FASD, PDACyanosisComplete membrane resection and ASD closureAlive at 16 mo after surgeryHoye et al, 201010Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar5 d, FPFO, SVC stenosisCyanosisComplete membrane resection, PFO closure and pericardial patch enlargement of SVCAliveAlghamdi, 201611Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar15 d, F–CyanosisComplete membrane resection at 15 d after unsuccessful disruption of the membrane by catheterizationAliveRao et al, 201812Rao 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 Scholar10 mo, MLarge ASD, PS, mild HRV, LSVCCyanosisPulmonary balloon valvulotomy at 4 moComplete membrane resection, ASD closure with autologous pericardiumAlivePresent case, 20209 y, FSevere TRFatigability and dyspnea on exertionComplete membrane resection and TV repairAliveM, Male; PA, pulmonary atresia; HRV, hypoplastic right ventricle; HTV, hypoplastic tricuspid valve; PFO, patent foramen ovale; ASD, atrial septal defect; F, female; PDA, patent ductus arteriosus; VSD, ventricular septal defect; RA, right atrium; LSVC, left superior vena cava; LA, left atrium; SVC, superior vena cava; PS, pulmonary stenosis; TR, tricuspid regurgitation; TV, tricuspid valve. Open table in a new tab M, Male; PA, pulmonary atresia; HRV, hypoplastic right ventricle; HTV, hypoplastic tricuspid valve; PFO, patent foramen ovale; ASD, atrial septal defect; F, female; PDA, patent ductus arteriosus; VSD, ventricular septal defect; RA, right atrium; LSVC, left superior vena cava; LA, left atrium; SVC, superior vena cava; PS, pulmonary stenosis; TR, tricuspid regurgitation; TV, tricuspid valve. A female child was initially seen at our institution at the age of 5 years of age because of frequent episodes of palpitation on exertion. Her electrocardiogram exhibited a normal sinus rhythm with a heart rate of 100 beats/min. Peripheral arterial oxygen saturation was normal (99%). Transthoracic echocardiography (TTE) detected the presence of a wide unrestrictive membrane in an enlarged RA and revealed mild-to-moderate tricuspid regurgitation with no evidence of Ebstein disease or any intracardiac shunting. At regular clinical examinations, the patient began demonstrating progressive dyspnea, palpitations, and fatigue on moderate exertion. Her latest TTE control, at the age of 9 years, confirmed the presence of this wide membrane in the RA, which was protruding toward the tricuspid valve (TV) during diastole and also revealed severe central regurgitation during systole (Video 1). At surgery, the RA was opened and a nonobstructive membrane extending across the RA—which was dividing it into 2 chambers, with both caval veins and the coronary sinus on one side and the TV and RA appendage on the other—was exposed (Figure 1). This membrane, which was completely covering the TV leaflets, was carefully resected starting from the upper and lower parts of its anterior unobstructed sinoatrial orifice. After resection, the TV was exposed and its analysis confirmed the suspicion of annular dilatation as well as the presence of a retracted fibrotic septal leaflet with limited mobility. Tricuspid annuloplasty with a Carpentier Edwards tricuspid ring (Edwards Lifesciences, Irvine, Calif) size 28, and a stitch closure of the anteroseptal commissure were performed. Postrepair transesophageal echocardiography confirmed the satisfactory surgical correction with no residual leak. The postoperative course was uneventful. Eustachian and thebesian valves as remnants of the sinus venosus valves are not uncommon.13Chiari H. Uber Netzbildungen in rechten Vorhofedes Herzens.Beit Path Anat. 1897; 22: 1-10Google Scholar They rarely produce clinical symptoms in children without any other significant structural heart defects.14Sehra R. Ensing G. Hurwitz R. Persistent Eustachian valve in infants: course and management in symptomatic patients.Pediatr Cardiol. 1998; 19: 221-224Crossref PubMed Scopus (11) Google Scholar,15Martinez-Quintana E. Rodriguez-Gonzalez F. Focusing on cor triatriatum dexter and atrial septal defects.Tex Heart Inst J. 2014; 41: 567-568Crossref PubMed Scopus (2) Google Scholar In contrast, the septation of the RA with a membrane between its smooth and trabeculated portions may lead to subsequent malformations of the right heart structures in utero. In anatomical studies of hypoplastic right heart structures, a prominent eustachian valve directing the abnormal fetal flow across the atrial septal defect (ASD) has been documented in up to 80% of cases, including CTD in 25% of them.16Schutte D. Rowland D. Allen H. Schutte D. Rowland D. Allen H. et al.Prominent venous valves in hypoplastic right hearts. Prominent venous valves in hypoplastic right hearts.Am Heart J. 1997; 134: 527-531Crossref PubMed Scopus (31) Google Scholar However, it cannot clearly be determined whether this abnormal fetal flow due to the persistent presence of the RA remnants promotes the genesis of hypoplastic right heart structures or vice versa the presence of hypoplastic right heart structures potentially contributes to the persistence of these RA remnants. Based on the reported cases presented in Table 1, the time of onset and the severity of the various clinical manifestations depend primarily on the following 6 factors in children. (1) The size of the sinoatrial orifice(s), the sinus portion of the RA receiving the superior, and inferior venae cavae blood flow return. The size of this orifice determines the degree of blood flow obstruction from the smooth sinus part of the RA toward its trabeculated part, which contains the TV. (2) The presence of an associated ASD, its size determining the quantity of right-to-left shunting in correlation with the degree of obstruction of the sinoatrial orifice(s).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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar, 4Nakano 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, 5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar,8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar,12Rao 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 (3) The presence of associated RV and TV hypoplasia resulting in limited RV compliance and therefore increasing right-to-left shunting.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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar,12Rao 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 (4) The presence of functional TV obstruction created by the protrusion of the membrane through the tricuspid orifice during diastole4Nakano 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,8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar,9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar,12Rao 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 or the presence of TV insufficiency due to fibrosis and deformation induced by the repetitive traumatic effect of the membrane on the TV leaflets, as in our case. (5) The presence of associated right ventricular outflow tract obstruction at different levels.2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar,12Rao 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,17Haboub M. Drighil A. Successful balloon valvuloplasty of a subpulmonic membrane associated with cor triatriatum dexter: a case report.J Med Case Rep. 2019; 13: 291Crossref PubMed Scopus (1) Google Scholar (6) Predominant obstruction of inferior vena cava flow by the RA membrane with no associated ASD.6Kolouskova S. Lebl J. Snajderova M. Zemkova D. Hrobonova V. Honek T. et al.Growth in children with the exudative enteropathy syndrome due to a congenital heart defect—cor triatriatum dexter.Cas Lek Cesk. 1996; 135: 59-61PubMed Google Scholar,7Alkhulaifi 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 Eleven of the 14 pediatric cases reported in the literature presented with cyanosis early in life due to the presence of obstructive sinoatrial orifice(s) and an associated ASD of varying sizes.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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar, 4Nakano 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, 5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar,8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar, 11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar, 12Rao 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 Two of these 14 cases also presented with Budd–Chiari syndrome with exudative enteropathy in one case6Kolouskova S. Lebl J. Snajderova M. Zemkova D. Hrobonova V. Honek T. et al.Growth in children with the exudative enteropathy syndrome due to a congenital heart defect—cor triatriatum dexter.Cas Lek Cesk. 1996; 135: 59-61PubMed Google Scholar and ascites in the other7Alkhulaifi 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 due to obstruction of the inferior vena cava flow by the RA membrane, in the context of an intact interatrial septum. As a sole symptom, progressive fatigue and dyspnea on exertion is consistent with associated TV dysfunction in the context of CTD and can contribute to its incidental diagnosis by echocardiography, especially in cases of unobstructive sinoatrial orifice(s) and no associated ASD, as in our case. Children with unobstructed sinoatrial orifice(s) are usually asymptomatic and generally do not need any treatment of their CTD unless they would undergo cardiac surgery or percutaneous interventions for other reasons. Eight of 11 cases that presented with cyanosis early in life were operated on during the first 6 months after birth1Doucette 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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar,5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar,8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar, 11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar and the other 3 cases at the ages of 10 months,12Rao 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 7 years,4Nakano 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 and 9 years.2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar In cases before 1980, the diagnosis was often missed and either revealed during or after surgery at postmortem examination,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, 2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar, 3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar, 4Nakano 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 although clinical and especially cardiac catherization findings, in retrospect, were suggestive of CTD.4Nakano 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,5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar Three of the 5 cases of this early period, with cyanosis after birth and a missed preoperative diagnosis of CTD, were operated on during the first 6 months of life. Two of the patients died just after surgery1Doucette 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,2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar and the other patient 10 years later due to the obstructive membrane.3Jones R.N. Niles N.R. Spinnaker formation of sinus venosus valve.Circulation. 1968; 38: 468-473Crossref PubMed Scopus (26) Google Scholar The other 2 cases2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar,4Nakano 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 of this early period were operated on at a relatively advanced age despite the early onset of cyanosis after birth. One of them died after surgery due again to the missed prompt diagnosis of CTD2Folger G.M. Supravalvular tricuspid stenosis.Am J Cardiol. 1968; 21: 81-87Abstract Full Text PDF PubMed Scopus (9) Google Scholar and the other one survived thanks to the resection of the membrane, which was incidentally discovered during surgery.4Nakano 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 After 1980, the reported 9 cases, including ours, survived thanks to the complete resection of the membrane at the time of surgery.4Nakano 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, 5Mazzuco A. Bortolotti U. Gallucci V. Del Torso S. Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy.J Thorac Cardiovasc Surg. 1983; 85: 140-145Abstract Full Text PDF PubMed Google Scholar, 6Kolouskova S. Lebl J. Snajderova M. Zemkova D. Hrobonova V. Honek T. et al.Growth in children with the exudative enteropathy syndrome due to a congenital heart defect—cor triatriatum dexter.Cas Lek Cesk. 1996; 135: 59-61PubMed Google Scholar, 7Alkhulaifi 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, 8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar, 11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar, 12Rao 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 In the last 6 cases including ours, which were reported after 2009, accurate preoperative diagnosis of CTD relied entirely on TTE performance.8Barrea C. Rubay J. Wagner K. Ovaert C. Cor triatriatum dexter mimicking Ebstein disease.Circulation. 2009; 120: e86-e88Crossref PubMed Scopus (12) Google Scholar, 9Galli M.A. Galletti L. Schena F. Salvini L. Mosca F. Danzi G.B. A rare case of neonatal cyanosis due to "cor triatriatum dexter" and a review of the literature.J Cardiovasc Med. 2009; 10: 535-538Crossref Scopus (8) Google Scholar, 10Hoye D.J. Wilson E.C. Fyfe D.A. Guzzetta N.A. Cor triatriatum dexter: a rare cause of neonatal cyanosis.Anesth Analg. 2010; 110: 716-718Crossref PubMed Scopus (14) Google Scholar, 11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar, 12Rao 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 Nowadays, cardiac magnetic resonance imaging may be very helpful in the diagnostic assessment of CTD, as an additional valuable tool, to delineate the membrane and assess better right ventricular volume and function.12Rao 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 Although percutaneous catheter disruption of the obstructive membrane has been suggested as an alternative to surgical resection, this is not always technically feasible or possible.18Savas V. Samyn J. Schreiber T.L. Hauser A. O'Neil W.W. Cor triatriatum dexter: recognition and percutaneous transluminal correction.Catheter Cardiovasc Diagn. 1991; 23: 183-186Crossref PubMed Scopus (37) Google Scholar For example, Alghamdi11Alghamdi M.H. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period.Ann Pediatr Cardiol. 2016; 9: 46-48Crossref PubMed Scopus (2) Google Scholar reported a case of unsuccessful disruption that required surgical resection 1 week later. On the contrary, percutaneous ASD closure can be an alternative treatment to surgery in unobstructive CTD cases.19Eckersley L.G. Clements B. Shipton S. Exercise-induced hypoxia secondary to an atrial septal defect and cor triatriatum dexter.Cardiol Young. 2016; 26: 793-795Crossref PubMed Scopus (2) Google Scholar In conclusion, CTD with associated ASD must be considered as a cause of persistent hypoxia after birth in newborns and infants and a rare cause of exercise-induced hypoxia later in life. Budd–Chiari syndrome in childhood can in exceptional cases also be a consequence of CTD. Although percutaneous catheter disruption of the membrane has recently been reported, surgical resection of the RA and excision of the membrane still remains the preferred treatment for symptomatic patients. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI5NzNjNTU1MmQzOTkwYzQxZTY4ZWZhOGRlMTU0ZDJhNyIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjMxMjM4MDA5fQ.KI3oZqYR1twRpUFBYdIEcdSal4HnjKm-8dwzajGbLsOH95OwsHUZudXEpK9xROPW0yELKW2_FljSrTllhGnOUR1zQHjo6197CR9i66j9z0oEOVbUeEnmDtfbWuq_SzyzM_yh5LjrOxvJClu5gUIoIlHL7B7hirQUCr63qT1K07UtC37WrCM06LEGmS6pvueCrMlV9tABErFD3nHHTbxazyi84QkDLJnzSXYEllQey6cGjFUSCF_NcjcvQiC5PhdBXGys3SnNnShexoEcIU-_XEaT6MuOJZpalXIPzgKdxcpLANrTJtqbZ1nuUDazuqG8wZvwifKMlMdtNQ_U93Rmyg Download .mp4 (1.79 MB) Help with .mp4 files Video 1Preoperative 2-dimensional transthoracic echocardiography demonstrating the presence of a membrane separating the right atrium into 2 parts that was protruding into the right ventricle through the tricuspid orifice during diastole. The septal tricuspid leaflet is thickened and immobile (A). Severe central tricuspid insufficiency during systole with color Doppler (B). Video available at: https://www.jtcvs.org/article/S2666-2507(20)30399-0/fulltext.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJjOWM4MWM5YTVlMTEyMzAxODAwNjAyNzE3NjBkOGZlOSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjMxMjM4MDA5fQ.HGn51dsX-3HaRNBoATDLUggXxKVoFNK57gOnbfadlRJ1u_f92ALHVXqfUK_zb9y_7siFNPEKs1h7hcJvO0kzCSTjVPD1ji__qfN6Kv8rfHxc4ZKpQAla1h0YWkcWc0Mjyd2ddnXKU0GaQ7kWL79sUkhyF53Ei8zPjxMuoo9EnmyU7jMT2sY1xXSgKa71HBvk0_OCscik_rizpgb8x3iAteqhSBNIHbRjqW2ooxd4wVR8XZX8P3k_6rlKylJNDZownnol-FC1RFp2WQTKu8c7u-qzVKj_97wN3_KDvmQ1NUu8cc9DWJSN-daRCg1KnCRO1WQbcaTFGHbEHC6SdOnCeQ Download .mp4 (2.03 MB) Help with .mp4 files Video 1Preoperative 2-dimensional transthoracic echocardiography demonstrating the presence of a membrane separating the right atrium into 2 parts that was protruding into the right ventricle through the tricuspid orifice during diastole. The septal tricuspid leaflet is thickened and immobile (A). Severe central tricuspid insufficiency during systole with color Doppler (B). Video available at: https://www.jtcvs.org/article/S2666-2507(20)30399-0/fulltext.
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