Introduction
Cor triatriatum is a clinical condition characterized by a heart with three atria. It was first reported in 1868. It is a congenital anomaly in which the atrium, left or right, is divided into two compartments by a membrane, a fold of tissue, or a fibromuscular band. It is extremely rare and can be misdiagnosed as other commonly known cardiac or pulmonary conditions like bronchial asthma or mitral stenosis. Studies have revealed no clear demarcation in incidence or clinical presentation among women or men. Cor triatriatum sinistrum is often associated with various major congenital defects of the cardiovascular system in symptomatic infants.
How Is It Caused?
Cor triatriatum dextrum is an extremely rare condition and is caused due to the complete persistence of the right sinus valve of the embryonic heart. This membrane divides the right atrium into upper and lower chambers and proximal and distal chambers, respectively. The upper chamber obtains the venous blood from both vena cavae, whereas the lower chamber remains in contact with the right atrial appendage and the tricuspid valve.
Mechanism:
The size of the communicating orifice lying between the upper and lower atrial chambers determines the natural history of this defect. The patient is considered critically ill if the communicating orifice is small and may get affected with congestive heart failure and pulmonary edema at a young age, more commonly during infancy. If the connection is larger, the patient may show symptoms like mitral stenosis during childhood or young adulthood. Cor triatriatum may also be detected accidentally when it is non-obstructive. The diagnosis can be made using ECHO and MRI.
What Are The Signs And Symptoms?
A heart murmur may be detected during the examination if the patient is asymptomatic. Cor triatriatum can be detected accidentally on routine cardiac imaging. An adult patient transforms from asymptomatic to symptomatic mainly due to fibrosis and calcification of the orifice in the accessory membrane. The other reason can be the development of mitral regurgitation and/or atrial fibrillation.
Following are the symptoms that the patient can experience:
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Orthopnea.
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Exertional dyspnea.
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Effort intolerance.
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Easily getting tired.
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Hemoptysis.
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Palpitation or atrial fibrillation.
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Systemic embolism.
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Systemic thromboembolism.
This can be observed especially in those individuals suffering from atrial fibrillation and/or restrictive orifice. Both these conditions lead to the formation of a thrombus in the left atrium.
Physical examination of the patient may show the following findings:
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Murmur.
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Tachypnea.
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Pulmonary congestion.
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Pleural effusion.
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Respiratory distress.
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Pulmonary rales.
How Can It Be Diagnosed?
The physician initially asks questions about the signs and symptoms you are facing, medical history, and personal history, including any deleterious habits. A physical examination will be conducted, and the signs mentioned above may be detected. As this condition is rare, the diagnosis can be delayed till the patient shows obvious signs and symptoms. Also, the symptoms of this condition mimic other commonly observed heart conditions, making the diagnosis more delayed. Your physician may recommend certain laboratory tests, which depend on the type of condition.
They have been mentioned below:
- Cor triatriatum sinistrum
- Electrocardiography
Electrocardiographic (ECG) shows the following findings:
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Coronary angiography.
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Right heart catheterization.
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Left heart catheterization.
Imaging Studies
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Chest radiography.
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CT scanning.
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MRI: Magnetic Resonance Imaging.
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Echocardiography.
How Can It Be Treated?
The management of this condition can be done using medical care or surgical care. The type of treatment depends on the type of this condition.
Medical Care
The following medical care is provided for symptomatic patients with cor triatriatum:
1) Stabilization of Hemodynamics
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This is done by controlling Hypoxemia, fluid overload, and pulmonary congestion.
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Establishment of anticoagulation prophylaxis against deep vein thrombosis and pulmonary embolism. This is done in those patients who have right-sided heart failure.
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Controlling ventricular rate in patients diagnosed with atrial fibrillation.
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Full anticoagulation is considered in patients with atrial fibrillation.
2) Diet and Activity:
Patients with a significant amount of fluid retention are advised to consume a low-salt diet. Symptomatic patients diagnosed with pulmonary congestion, significant right-sided heart failure, and pulmonary hypertension may require bed rest.
3) Surgery:
Cor triatriatum patients with symptomatic and significant gradients across the membrane are suitable candidates for surgical repair. Also, the resection of the accessory membrane has been proven to be successful. Another common surgical approach is the complete resection of the membrane and atrial septum closure with a pericardial patch. Also, associated congenital defects should be corrected along with this surgical management. Percutaneous treatment strategies are also preferred.
Cor Triatriatum Dextrum:
Patients diagnosed with asymptomatic cor triatriatum dextrum can be managed with observation alone. In symptomatic patients, fluid retention and rate control of atrial arrhythmias may be required. In some patients, a percutaneous technique of balloon septostomy of the accessory membrane has also been done.
How Is The Prognosis?
The highest mortality has been reported in untreated symptomatic infants diagnosed with cor triatriatum sinistrum related to complex congenital cardiac defects. Adults with isolated cor triatriatum show an excellent prognosis after surgical repair. Cor triatriatum sinistrum patients who are symptomatic in infancy show high morbidity and mortality. This is because of severely restricted opening in the accessory membrane and the relation with major cyanotic or acyanotic congenital heart lesions. Mortality may cross 75% in infants who are untreated and symptomatic. Significant sequelae is rare with cor triatriatum dextrum. This is because it is not commonly associated with life-threatening symptoms or any major congenital heart defects.
Are There Any Complications?
Following are the complications of cor triatriatum :
- Atrial arrhythmia.
- Right-sided heart failure.
- Pulmonary hypertension.
- Systemic and pulmonary embolism.
- Death.
Conclusion:
Cor triatriatum is a rare congenital heart condition affecting the heart's structure. However, the patient must not get worried as the condition is not life-threatening. Instead, the patient can consult a cardiologist to learn more about the condition and its management.
