Obstructive Cor Triatriatum: A rare presentation
Surabhi Chandra, Hammad-ur-rehman Khan, Shrish Bhatnagar
Department of Pediatrics, Eras Medical College and Hospital, Lucknow, UP, India
Address for Correspondence
Dr. Surabhi Chandra, Lecturer, Department of Pediatrics, Era’s Medical College and Hospital, Sarfarazganj, Lucknow-226003 (UP), India.
Email
surabhi0329@gmail.com
An 8 years old girl presented with left-sided chest pain for 15 days. Examination was essentially normal except for systolic murmur in the tricuspid area. Her weight for age was 76%, height for age was 92.4% and body mass index was 14.6. Chest X ray and electrocardiogram were normal. Echocardiography was suggestive of cor triatriatum (obstructive type) with normal bi-ventricular dimensions and normal valves and left ventricular ejection fraction of 63%.
Cor triatriatum is a rare congenital cardiac anomaly, incidence being about 0.1%. (1) In this, a fibromuscular membrane divides the left atrium into two chambers. The proximal chamber communicates with the distal chamber (the true left atrium with left atrial appendage) through one or more openings. It may be obstructive or non-obstructive depending on the presence and size of fenestrations in the dividing membrane and obstruction to the pulmonary blood flow. It has a varied age of presentation from infancy to old age. (2) Clinical presentation depends on the size of the opening(s) in the membrane, as symptoms are directly related to the degree of obstruction to pulmonary venous drainage and pressure loading on the right side of the heart. Presentation in infancy mimics that of Ebstein’s anomaly and in adults mimics that of mitral stenosis. (3) Presentation in infancy is usually fatal with signs of pulmonary edema and hypertension. (4) In our case, initial presentation was at 8 years of age despite it being obstructive in nature. Also, the only complaint was chest pain. Association with other cardiac anomalies is also seen in 70 -80% cases, most common being patent foramen ovale and ostium-secundum atrial septal defect. (5) Electrocardiographic alterations are non-specific. (5) Echocardiography is required for definitive diagnosis. Medical treatment in cor triatriatum depends on symptoms. An incidental echocardiographic finding of a left atrial extra-membrane with no pressure gradient in an asymptomatic patient does not require medical management. Definitive treatment involves surgical excision of the abnormal fibromuscular membrane followed by closure of the inter-atrial septum with a pericardial baffle or patch. Long term prognosis depends on the underlying congenital heart disease (6) and is generally good after surgical correction in isolated cases.
Thus, obstructive varieties of this cardiac anomaly may not be limited only to infancy and an echocardiogram should definitely be performed when other investigations for chest pain are inconclusive, even when it is the only presenting complaint.
Funding
None
Conflict of Interest
None
References :
  1. Talner CN. Report of the New England Regional Infant Cardiac Program, by Donald C. Fyler, MD, Pediatrics, 1980; 65(suppl):375-461. Pediatrics. 1998;102 (1 Pt 2): 258-9
  2. Chen Q, Guhathakurta S, Vadalapali G, Nalladaru Z, Easthope RN, Sharma AK. CorTriatriatum in Adults: Three New Cases and a Brief Review. Tex Heart Inst J. 1999; 26(3): 206-210
  3. Fesslova V, Saracino A, Nuri H, Pomé G. Cor triatriatum dexter: unusual features in utero and after birth. Interact Cardiovasc Thorac Surg. 2012; 14(3):330-2
  4. Humpl T, Reineker K, Manlhiot C, Dipchand AI, Coles JG, McCrindle BW. Cor triatriatum sinistrum in childhood. A single institution's experience. Can J Cardiol. 2010; 26:371-376
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  6. Krasemann Z, Scheld HH, Tian TD, Krasemann T. Cor triatriatum: short review of the literature upon ten new cases. Herz. 2007; 32:506-510
Last Updated : Tuesday, April 01, 2014 Vol 11 Issue 2 Art #23
DOI: 10.7199/ped.oncall.2014.23
How to Cite URL :
Chandra S, Khan H, Bhatnagar S. Obstructive Cor Triatriatum: A rare presentation. Pediatric Oncall [serial online] 2014[cited 2014 April-June 1];11. Art #23. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=813&type=J&tid=&imgid=&reportid=429&tbltype=
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