Cor triatriatum history and symptoms

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]

Overview

Symptomology of cor triatriatum hinges largely on the size of the opening in the septal wall and the development or presence of additional, associated congenital heart diseases. Patients may present in asymptomatic or symptomatic fashion with symptoms ranging from heart murmurs to fatigue to right ventricular failure.

Symptoms

Cor triatriatum sinistrum

The severity of symptoms depends upon the size of the hole in the diaphragm separating the two atrial chambers and the integrity of the interatrial septum. Patient may be asymptomatic, a murmer may be diagnosed incidentally, can also be detected during cardiac imaging for other purpose.

Common symptoms include:

Dyspea on exertion
Orthopnea
Tachypnea
Fatigue
Palpitation and signs of systemic thromboembolism
Hemoptysis
Markedly slowed physical growth of the body/growth failure

Cor triatriatum dextrum

Clinical presentation depends on the degree of blockade within right atrium. It may be asymptomatic and detected incidentally on cardiac imaging.

Common symptoms include:

Palpitation
Features of right heart failure such as ascites and edema

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines[1]

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines^[1]

Diagnostic Recommendations for Cor Triatriatum

Class I
1.Adults presenting with cor triatriatum sinister should be evaluated for other congenital abnormalities, particularly ASD, VSD, and anomalous pulmonary venous connection. (Level of Evidence B-NR)

Class IIa
1.In adults with prior repair of cor triatriatum sinister and recurrent symptoms, it is reasonable to evaluate for pulmonary vein stenosis. (Level of Evidence: B-NR)

References

↑ Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM; et al. (2019). “2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines”. J Am Coll Cardiol. 73 (12): 1494–1563. doi:10.1016/j.jacc.2018.08.1028. PMID 30121240.

Template:WikiDoc Sources

[pmid30121240-1] Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM; et al. (2019). “2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines”. J Am Coll Cardiol. 73 (12): 1494–1563. doi:10.1016/j.jacc.2018.08.1028. PMID 30121240.

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