|Year : 2017 | Volume
| Issue : 2 | Page : 160-162
Cor triatriatum dexter
Manoj Kumar Dubey1, Avinash Mani2, Vineeta Ojha3, Punam Dubey4
1 Department of Medicine, PMCH, Dhanbad, Jharkhand, India
2 Department of Medicine, Medical College, Kolkata, West Bengal, India
3 Department of Radiodiagnosis, Medical College, Kolkata, West Bengal, India
4 Department of Obstetrics and Gynaecology, Central Hospital, Dhanbad, Jharkhand, India
|Date of Web Publication||28-Aug-2017|
Manoj Kumar Dubey
M. P. Heart Centre, Dhaiya, P. O. ISM, Dhanbad - 826 004, Jharkhand
Source of Support: None, Conflict of Interest: None
Cor Triatriatum, otherwise known as triatrial heart, is a rare congenital heart defect. This report describes a case of a 45 yrs old female who was sent to us for evaluation of exertional shortness of breath and swelling of feet. She was found to be in incipient heart failure. Transthoracic echocardiography revealed a membrane separating the right atrium into 2 chambers and dilated Right heart and pulmonary artery with features of pulmonary hypertension. The 2 portions of Right atrium had adequate communication between them, hence her paucity of complications and survival. But perhaps the onset of pulmonary hypertension had aggravated her symptoms forcing her to seek medical attention. Early diagnosis and prompt referral are mandatory to prevent mortality from the defect that is amenable to simple corrective surgery.
Keywords: Cor triatriatum dexter, pulmonary hypertension, right heart failure
|How to cite this article:|
Dubey MK, Mani A, Ojha V, Dubey P. Cor triatriatum dexter. J Indian Acad Echocardiogr Cardiovasc Imaging 2017;1:160-2
| Introduction|| |
The triatrial heart, cor triatriatum, is when one of the atrial chambers is partitioned into two by an abnormal fibromuscular membrane resulting into a triatrial heart. It represents 0.1% of all congenital cardiac anomalies. It results from complete persistence of right sinus valve of embryonic life whose function is to direct flow from inferior vena cava to the foramen ovale, thus bypassing the pulmonary circulation. Usually, this network regresses and leaves the crista terminalis superiorly, Eustachian valve More Details, and Thebesian valve inferiorly. In cor triatriatum, persistence of the right sinus valve results in separation of smooth and trabeculated portion of the atrium.
Cor triatriatum can be associated with several congenital anomalies such as ventricular septal defect, atrial septal defect, tetralogy of Fallot, anomalous pulmonary venous connection, and bicuspid pulmonary valve. Presence of these anomalies is the reason for its incidental detection. Clinical presentation is dependent on the affected atrium, the size of communication between the subdivided compartments, and presence of associated anomalies. Septation is brought about by a fold of tissue membrane or a fibromuscular band.
The consequence will depend on the communication between the two compartments (Loeffler classification for cor triatriatum sinistrum in 1949).
- Type 1 – No opening in accessory membrane
- Type 2 – One or more restrictive opening (fenestration)
- Type 3 – Large nonrestrictive opening in the membrane.
Depending on the opening and associated anomalies, the patient may remain asymptomatic for long and may be detected as an incidental finding or may present with palpitation (recurrent supraventricular tachycardia), ascites, edema, and features of right heart failure.
| Case Report|| |
Our patient was a 45-year-old female who was referred for evaluation of breathlessness and swelling of feet. Clinically, she was hemodynamically stable. No dyspnea at rest. Clinical examination revealed raised jugular venous pressure, systolic murmur at parasternal edge, moderate ascites, and bilateral pedal edema. Her chest X-ray and electrocardiogram were normal. There was no history of angina or chronic obstructive pulmonary disease.
Transthoracic echocardiography revealed a fibromuscular band in the right atrium (RA) dividing it into two compartments, which communicated with each other through a narrow orifice between the band and the interatrial septum [Figure 1] and [Figure 2].
|Figure 2: Communication between two parts of right atrium and intact interatrial septum|
Click here to view
As the communication between the two compartments was narrow and inferior vena caval flow was in jet, the blood flow from RA to right ventricle was with increased gradient despite a normal tricuspid valve. Tricuspid velocity Vmax = 2.11 m/s MaxPG = 21.36 mmHg. Features of right ventricular hypertrophy and early enlargement were seen [Figure 3].
|Figure 3: Blood moving in jet across communication and tricuspid valve, right ventricular hypertrophy seen|
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Short axis view showed a dilated pulmonary artery with normal valve, normal forward flow (PS flow) of Vmax 0.6 m/s and a significant pulmonary regurgitation (PR) with Vmax 2.5 m/s. This resulted in a moderate tricuspid regurgitation (TR) with Vmax 3.05 m/s and MaxPG of 37.24 mmHg. There was insignificant mitral regurgitation (MR) and normal left atrium cavity size suggestive of the fact that this was not a reactive pulmonary hypertension secondary to MR [Figure 4],[Figure 5],[Figure 6].
Hence, this patient had cor triatriatum dexter with communication between the two compartments with pulmonary hypertension with severe PR and moderate TR.
| Discussion|| |
Diagnosis of this rare anomaly is mostly an incidental finding at any age with slight male preponderance. Second, it may present as right heart failure. Here, age of detection will depend on the degree of communication between the two compartments and the resultant hemodynamic changes. Finally, cor triatriatum can be associated with other congenital lesions, and sometimes, they can be the reason for the incidental detection. Diagnosis with symptoms, especially at early age, speaks of poor prognosis. Since the morbidity and mortality is determined by the severity of pulmonary hypertension and right heart failure, both of which are inversely related to the degree of communication between the two compartments, this alone determines the onset, severity of symptoms, and age of presentation.
In our case, right heart failure was the reason to seek medical attention and presentation in the fourth decade speaks of moderately inadequate communication which leads to the development of right heart failure. It was the development of pulmonary hypertension which led to TR and right heart failure. The mechanisms of pulmonary hypertension appeared to be increased pulmonary flow secondary to increased right ventricular outflow. The pulmonary artery in this case was grossly dilated with apparently normal left heart associated with trivial mitral regurgitation which alone cannot lead to pulmonary hypertension. Our patient who had right heart failure was treated with conservative management to which she responded and did not require surgery.
A similar case of cor triatriatum with pulmonary hypertension and congenital mitral regurgitation presenting as congestive heart failure has been reported by Asani and Gambo from Nigeria in 2016. That patient presented with tachypnea, hypoxia, and severe right heart failure. The patient died within 2 days of diagnosis.
Long-term prognosis for isolated cor triatriatum dexter is good. However, there is a scarcity of case reports on those associated with pulmonary hypertension and right heart failure. Moreover, there cannot be generalized prediction of prognosis as the communicating orifice is the sole determinant of the progression to sequelae. Hence, course and prognosis will vary from case to case.
| Conclusion|| |
In view of the above findings, a high index of suspicion is necessary to identify such rare congenital heart lesion. This entity should always be kept in mind when any patient with isolated right heart failure is being evaluated by echocardiography. Early diagnosis with two-dimensional echocardiography will facilitate prompt referral of patient with this defect that is amenable to simple corrective surgery.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]