|Year : 2016 | Volume
| Issue : 2 | Page : 140-143
Cor triatriatum sinistrum in a Nigerian adult
Akinyemi Aje1, Oladimeji Adebayo2, Olugbenga Towoju2, Opeyemi Oni2, Abiodun M Adeoye3, Okechukwu S Ogah2, Adewole A Adebiyi3, Olulola O Oladapo4
1 Department of Medicine, Division of Cardiology, University College Hospital, University of Ibadan, Ibadan, Oyo State, Nigeria
2 Department of Medicine, University College Hospital, University of Ibadan, Ibadan, Oyo State, Nigeria
3 Department of Medicine, University College Hospital/College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria
4 Department of Anatomy; Department of Medicine, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria
|Date of Web Publication||4-Aug-2016|
Department of Medicine, Division of Cardiology, University College Hospital, PMB 5116, Ibadan, Oyo State
Source of Support: None, Conflict of Interest: None
Classical triatriatum sinistrum or cor triatriatum sinistrum is a rare congenital heart disorder arising from the left atrium being divided into two chambers and may present with undistinguishing features such as exertional dyspnea, hemoptysis, and orthopnea. It has been scarcely reported among adult Nigerians. The case being reported presented at an unusual age of 30 years. Most cases previously reported were in children. Definitive treatment is a corrective surgery with an excellent outcome. Nonintervention could have a catastrophic outcome. Therefore, there must be a high index of clinical suspicion and early diagnosis. This report is to increase awareness of such condition.
Keywords: Congenital, cor triatriatum, heart disease, Nigeria
|How to cite this article:|
Aje A, Adebayo O, Towoju O, Oni O, Adeoye AM, Ogah OS, Adebiyi AA, Oladapo OO. Cor triatriatum sinistrum in a Nigerian adult. Nig J Cardiol 2016;13:140-3
| Introduction|| |
Classical triatriatum sinistrum or cor triatriatum sinistrum (CTS) is a rare congenital heart disorder, in which the left atrium (LA) is divided into two chambers by a fenestrated membranous or fibro-muscular septum. It is rarely first diagnosed in adulthood. ,,,,
The usual presenting symptoms although not characteristic include exertional dyspnea, orthopnea, and hemoptysis. These symptoms can easily be mistaken for other cardiac disorders in low-resources settings where specialist care is not readily available. The harbinger of CTS can be due to complications such as atrial arrhythmias or cerebral and systemic embolic events.
To make early diagnosis, there must be a high index of suspicion. Aside the presenting symptoms, the most important physical findings include pulmonary congestion, diastolic murmur with a loud P2 when pulmonary hypertension is present, and absence of an opening snap or a loud S1 that marks the difference between CTS and mitral stenosis. Misdiagnosis can easily occur, but when promptly and adequately evaluated and treated, the clinical outcome will be excellent. 
| Case report|| |
The patient is a 30-year-old undergraduate Yoruba man. He presented with breathlessness of 1 month, which was insidious in onset, progressive in severity, and usually relieved with the use of diuretics. There was associated orthopnea, effort intolerance, paroxysmal nocturnal dyspnea, palpitation, and progressive pedal swelling. He had episodes of recurrent hemoptysis for 2/12.
There was hoarseness of voice at the onset of presentation; however, there was no palpitation, intermittent claudication, abdominal distension, and abdominal pain.
There was occasional chest pain, severity scale of 5/10, not associated with activity, and no known aggravating or relieving factor. There was no facial swelling, no frothiness of urine, but there was a slight diminution of urine output. There was no history of recurrent sore throat or frequent febrile illness in childhood. There was no reversal of sleep pattern.
He had only some relief in the symptoms when he visits the health care center.
There was no previous surgery or blood transfusion. He is not a drug injector. He neither smokes cigarette nor drinks alcoholic beverages. There was no history of multiple sexual partners. There is no history of drug reaction.
On examination, he was pale, afebrile, anicteric, no significant lymphadenopathy, no pedal edema. Pulse was 84 beat/min, large volume, regular, and collapsing. His jugular venous pressure was not elevated. The apex beat was located at the sixth left intercostal space anterior axillary line. He had an apical thrill and left parasternal heave. The first and second heart sounds were heard, and there was grade 4/6 mitral regurgitation and 3/6 tricuspid regurgitation. Diastolic murmur was heard at the left upper sternal edge and there was loud P2.
Other systemic examinations did not reveal additional significant finding.
His 12-lead echocardiogram (ECG) showed sinus rhythm with a ventricular rate of 93 beats/min and first degree atrioventricular (AV) block [Figure 1].
|Figure 1: Twelve-lead echocardiography of the patient showing sinus rhythm, left axis deviation, and first degree atrioventricular block|
Click here to view
ECG showed dilated LA. Two chambers were noted in the LA, which were separated by a pseudo-septum (medial portion LA measuring 4.4 cm and the lateral measuring 2.3 cm). The left ventricle (LV) systolic function appeared normal (LV ejection fraction - 65%). There was severe mitral regurgitation, tricuspid regurgitation, and moderate pulmonary regurgitation. The pericardium was free of excess fluid [Figure 2] and [Figure 3].
|Figure 2: Echocardiography of the patient. RA: Right atrium, RV: Right ventricle, LA-1: First left atrium, LA-2; Second left atrium, LV: Left ventricle, CT: Cor triatriatum|
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The patient was placed on standard heart failure treatment (which included diuretics, beta blocker angiotensin-converting enzyme inhibitors, and aldosterone antagonist) to which he responded very well.
He was discharged after a week on admission, and he is currently being prepared for surgical correction.
| Discussion|| |
Cor triatriatum accounts for about 0.1-0.4% of congenital heart defects. , The disorder usually involves the LA (cor triatriatum sinister) and less commonly the right atrium (cor triatriatum dexter).  In this case, we report a case of cor triatriatum sinister/CTS).
The condition was first described by Church in 1868, and the name "cor triatriatum" was given by Borst in 1905 to the condition. ,, Previous documented cases from Nigeria were diagnosed in childhood. ,,
The embryology is unclear. However, it has been suggested to be from the failure of developing pulmonary vein in utero to incorporate on the left side, providing the substrate for congenital division of the LA.  Other postulations include malseptation involving the septum primum, mal-incorporation of common pulmonary veins, and entrapment hypothesis, where the left horn of sinus venosus entraps the common pulmonary vein and thus, prevents incorporation into the LA.  The pathophysiology can be explained by the membrane within the LA, restricting pulmonary venous return and elevating pulmonary venous pressure. 
The most popular classification is the one developed by Loeffler in 1949, where it was divided into three distinct groups: Type 1 has no opening in the accessory membrane, with the proximal LA draining into the right atrium; Type 2 has one or more small restrictive openings (fenestrations), resulting in a significant left ventricular inflow obstruction; and Type 3 has a large (nonrestrictive) opening in the membrane. , Other classifications include those by Krabill and Lucas and Rodefeld et al. ,
The presenting features include exertional dyspnea, orthopnea, and ease in fatigability, which were found in our patient. Other symptoms may include palpitation, effort intolerance, and features suggestive of systemic thromboembolism.  Physical findings include murmur, pulmonary congestion, tachypnea, respiratory distress, pulmonary rales, pleural effusion, hypoxemia, and features of right-sided heart failure. 
Various cardiac anomalies are associated with the condition although they are usually in about 5%.  The associated condition include tetralogy of Fallot, coarctation of the aorta, ventricular septal defect, double outlet right ventricle, partial anomalous pulmonary venous connection, persistent left superior vena cava with unroofed coronary sinus, AV septal defect, and common AV canal. , Mitral stenosis can be associated with cor triatriatum in Wong anomaly (a rare variant of the disease). , No associated cardiac anomaly was found in our patient.
The condition may remain undiagnosed till adulthood (as the index case) until they present with complications such as heart failure, systemic embolization, or arrhythmias. The nonassociation of other cardiac congenital anomaly may explain the late presentation in our case.
ECG is the mainstay of diagnosis; however, trans-esophageal ECG is superior to transthoracic imaging in CTS.  Cardiovascular imaging such as computed tomography angiography is also useful in the diagnosis. 
Relevant differential diagnosis of CTS includes bronchogenic carcinoma, bronchial asthma, pulmonary vein stenosis, pulmonary veno-occlusive disease, supra-valvular mitral ring, atrial tumors, pulmonary tuberculosis, primary or secondary pulmonary hypertension, or mitral stenosis. ,,
Considering the manifestation of this disease, there must be a high index of clinical suspicion. This report is to increase awareness of such condition. The diagnosis should be considered in all patients presenting, particularly, with signs or symptoms of mitral stenosis, mitral stenosis, pulmonary hypertension, and heart failure. 
| Conclusion|| |
The report is that of a patient presenting in adulthood. This calls for a high index of suspicion considering the nonspecific presentation associated with the condition. Early diagnosis would aid better quality of life considering the curative surgical possibility.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]