|Year : 2014 | Volume
| Issue : 1 | Page : 40-42
Cor triatriatum dextrum in an adult Nigerian
Ejiroghene M Umuerri, Omotola C Ayandele
Department of Medicine, Delta State University Teaching Hospital, Oghara, Nigeria
|Date of Web Publication||7-Apr-2014|
Ejiroghene M Umuerri
Department of Medicine, Delta State University Teaching Hospital, P.M.B. 07 Oghara
Source of Support: None, Conflict of Interest: None
Cor triatriatum dextrum is an exceptionally rare congenital heart disease, in which the right atrium is partitioned into two chambers by a membrane to form a triatrial heart. It is caused by persistence of the right valve of sinus venosus. The aim of presenting this case is to highlight that cor triatriatum dextrum though rare can result in right heart failure, mimicking other common causes of heart failure such as mitral valve disease. 2D-echocardiography is an important tool in making early and accurate diagnosis. We report a case of an adult Nigerian with cor triatriatum dextrum presenting with pulmonary hypertension in heart failure that was initially misdiagnosed as pulmonary tuberculosis with right heart failure at the referring center, and then mixed mitral valve disease clinically by us prior to echocardiographic assessment.
Keywords: Adult, cor triatriatum dextrum, right heart failure
|How to cite this article:|
Umuerri EM, Ayandele OC. Cor triatriatum dextrum in an adult Nigerian. Nig J Cardiol 2014;11:40-2
| Introduction|| |
Cor triatriatum was first described by Church in 1868.  It is a rare congenital anomaly that occurs when the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is partitioned into two parts by a membrane, or a fibromuscular band.  It accounts for approximately 0.1% of all congenital heart diseases,  most being cor triatriatum sinistrum.
Cor triatriatum dextrum, or partitioning of the right atrium (RA), is an extremely rare congenital heart anomaly that results in a triatrial heart. It results from the persistence of the right valve of the sinus venosus.  Typically, the right atrial partition is due to exaggerated fetal eustachian and thebesian valves, which together form an incomplete septum across the lower part of the atrium, which may range from a reticulum to a substantial sheet of tissue. 
Cor triatriatum dextrum has varying clinical manifestations depending on the degree of partitioning or septation of the right atrium. Significant sequelae is unusual with cor triatriatum dextrum, and it may sometimes be misdiagnosed.
We here present a case of an adult Nigerian lady with right heart failure, cyanosis, and severe pulmonary hypertension.
| Case Report|| |
Our patient is a 32-year-old business-woman who presented with a 1-year history of recurrent cough. Cough was productive of copious non-foul smelling sputum and hemoptysis. There was also history of weight loss and excessive night sweats but no fever. Patient had a similar history of cough 3 years prior to presentation whilst she was pregnant. About 4 months prior to presentation, she developed bilateral leg swelling, insidious in onset initially at the feet but progressively involved the entire thighs, with associated abdominal swelling. She gets easily fatigued and breathless, even at rest. There was associated orthopnea and paroxysmal nocturnal dyspnea. There was no previous history of similar dyspnea or leg swelling in the past.
She was treated for pulmonary tuberculosis in a peripheral center for 9-months following a suggestive chest X-ray [Figure 1], despite persistently negative sputum microscopy and culture for Mycobacterium tuberculosis. However, there was no clinical improvement, necessitating her referral to our facility.
|Figure 1: Chest radiograph showing cardiomegaly with prominent pulmonary vasculature|
Click here to view
Physical examination revealed a chronically ill-looking young lady, dyspneic at rest with central cyanosis, grade 3 digital clubbing and marked bilateral pitting pedal edema extending to her thigh with sacral edema. There was no significant peripheral lymphadenopathy. She had distended neck veins and elevated jugular venous pressure, displaced thrusting apex beat with left parasternal heave. S3 gallop with loud P2 and systolic murmurs were heard. She had widespread rhonchi with bibasal crepitations. Her abdomen was uniformly distended with hepatomegaly and ascites.
An initial clinical assessment of congestive cardiac failure secondary to mixed mitral valve disease in NYHA grade 4 with pulmonary hypertension was made. Electrocardiography showed sinus rhythm with right axis deviation and right atrial enlargement.
Transthoracic echocardiography did not reveal any mitral valve disease. Rather, there was an incomplete membranous septum dividing the right atrium into 2 parts [Figure 2]. A final diagnosis of congestive cardiac failure secondary to cor triatriatum dextrum with pulmonary hypertension (RVSP = 114 mmHg, PAP = 52 mmHg) was then made. Patient is presently on anti-failure regime and has been counseled for surgical correction of the anomaly.
|Figure 2: 2D-transthoracic echocardiography apical four chamber view showing presence of a membrane separating the right atrium into two parts (RA1 Proximal Right Atrium, RA2 Distal Right Atrium, LA Left Atrium, RV Right Ventricle, LV Left Ventricle)|
Click here to view
| Discussion|| |
Cor triatriatum dextrum, an extremely rare form of cor triatriatum, accounting for 0.025% of all congenital heart disease.  It can occur as an isolated cardiac anomaly  or associated with other malformation of right heart structures such as pulmonary artery stenosis or atresia, tricuspid valve abnormality, ASD, and Ebstein anomaly.  Unlike cor triatriatum sinister, which carries a high mortality rate if not repaired, cor triatriatum dextrum has varying clinical manifestations depending on the degree of obstruction to venous flow ranging from asymptomatic to right-sided heart failure and elevated central venous pressures. The availability of flow across the obstructing membrane during systole as well as diastole is probably the major element in the surprisingly good tolerance of severe degrees of anatomic obstruction for long periods of time as is the case in our patient [Figure 3].
|Figure 3: Color Doppler echocardiography showing blood flow across the membranous partition of the right atrium (RA1 Proximal Right Atrium, RA2 Distal Right Atrium)|
Click here to view
Cor triatriatum dextrum may be misdiagnosed as mitral valve disease clinically as was the case in our patient. However, distinguishing features suggestive of the correct diagnosis of cor triatriatum dextrum rather than mitral stenosis would include the absence of an opening snap, the absence of a typical murmur of mitral stenosis, regular rhythm, and lesser evidence of left atrial enlargement in the presence of obvious pulmonary hypertension as in the case of the index patient who did not have the typical diastolic murmur of mitral stenosis and had regular sinus rhythm and right atrial enlargement on electrocardiography. Cyanosis, which was only detected at clinical examination but not reported by patient, is a very rare presentation of this condition. Similar to our patient, cyanosis have been reported , with cor triatriatum dextrum and assessment showed significant obstruction to right ventricular inflow.  Hemoptysis appears more frequently in cor triatriatum compared with mitral stenosis and when recurrent and/or massive can lead to pulmonary edema as in the index patient. Cor triatriatum is a rare cause of pulmonary hypertension due to increased resistance to pulmonary venous drainage. Common cardiac causes of pulmonary hypertension include diseases affecting the pericardium or mitral or aortic valves. In our patient, cor triatriatum dextrum was initially mistaken for pulmonary tuberculosis, a common cause of chronic cough, hemoptysis and pulmonary hypertension.
There are no pathognomonic chest X-ray or electrocardiographic findings in isolated cor triatriatum dextrum. Right heart catheterization may reveal elevated pressure in the proximal right atrial chamber with a gradient across the accessory membrane. Echocardiography (transthoracic and transesophageal) ,, and cardiac magnetic resonance imaging (MRI) , are usually diagnostic as it demonstrates the presence of a membrane within the right atrium. Since many patients are asymptomatic, transthoracic echocardiography being more readily available and affordable has a pivotal role as a means of ante-mortem diagnosis. In symptomatic patients when diagnosis is established, it is amenable to relatively simple surgical resection of the dividing membrane. Percutaneous catheter disruption of the membrane has been suggested as a preferred alternative to open heart surgery. 
| References|| |
|1.||Church WS. Congenital malformation of the heart: Abnormal septum in the left auricle. Trans Pathol Soc Lond 1868;19:188-90. |
|2.||Anderson RH. Understanding the nature of congenital division of the atrial chambers. Br Heart J 1992;68:1-3. |
|3.||Niwayama G. Cor triatriatum. Am Heart J 1960;59:291317. |
|4.||Hansing CE, Young WP, Rowe GG. Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 1972;30:559-64. |
|5.||Sánchez-Brotons JA, López-Pardo FJ, Rodríguez-Puras MJ, López-Haldón JE. Cor Triatriatum dexter in adults. Rev Esp Cardiol. 2010;63:998-9. |
|6.||Choudhary D, Sivasankaran S, Venkateshwaran S, Sasidharan B. Cor triatriatum dexter: A rare cause of isolated right atrial enlargement. Pediatr Cardiol 2013;34:198-9. |
|7.||Eroglu ST, Yildirir A, Simsek V, Bozbas H, Bilgi M, Ozin B, et al. Cor triatriatum dexter, atrial septal defect and Ebstein's anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogr 2004;17:780-2. |
|8.||Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA. Cor triatriatum dexter: A rare cause of neonatal cyanosis. Anesth Analg 2010;110:716-8. |
|9.||Zainudin AR, Tiong KG, Mokhtar SA. Cor triatriatum dexter: A rare cause of childhood cyanosis. Ann Pediatr Cardiol 2012;5:92-4. |
|10.||Bucholtz S, Jenni R. Doppler echocardiographic findings in 2 identical variants of a rare cardiac anomaly "subtotal" cor triatriatum: A critical review of the literature. J Am Soc Echocardiogr 2001;14:846-9. |
|11.||Fiorilli R, Argento G, Tomasco B, Serino W. Cor triatriatum dexter diagnosed by transesophageal echocardiography. J Clin Ultrasound 1995;23:502-4. |
|12.||Pennell DJ, Sechtem UP, Higgins CB, Manning WJ, Pohost GM, Rademakers FE, et al. Clinical indications for cardiovascular magnetic resonance (CMR): Consensus panel report. Eur Heart J 2004;25:1940-65. |
|13.||Masui T, Seelos KC, Kersting-Sommerhoff BA, Higgins CB. Abnormalities of the pulmonary veins: Evaluation with MR imaging and comparison with cardiac angiography and echocardiography. Radiology 1991;181:645-9. |
|14.||Savas V, Samyn J, Schreiber TL, Hauser A, O'Neill WW. Cor triatriatum dexter: Recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 1991;23:183-6. |
[Figure 1], [Figure 2], [Figure 3]