Ebsteins anomaly of the tricuspid valve natural history, complications & prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Claudia P. Hochberg, M.D.; Priyamvada Singh, MBBS [2]
Overview
The symptoms of Ebstein's anomaly vary in severity, with some patients experiencing either no symptoms or very mild symptoms and others experiencing symptoms that may worsen over time such as (cyanosis), heart failure, heart block, or other tachyarrhythmias or bradyarrhythmias. Paradoxical embolization, brain abscesses and pulmonary embolism may also occur.The prognosis of Ebstein's anomaly of the tricuspid valve is extremely variable and depends on the spectrum of the severity of disease.The one-year survival is 67%, and the ten year survival is 59%.
Natural History
- The earlier the symptoms develop, the more severe the disease.[1][2][3]
- Infants generally have poor prognosis.
Complications
- Severe tricuspid regurgitation may lead to dilation of the right ventricle, hepatic congestion and congestive heart failure.[4][5]
- Other complications may include:
- Arrhythmias, including tachyarrhythmias, bradyarrhythmias, and heart block
- Paradoxical embolization due to an associated ASD
- Brain abscess
- Sudden cardiac death
- Bacterial endocarditis
- Transient ischemic attacks
- Stroke
Prognosis
- The prognosis of Ebstein's anomaly of the tricuspid valve is extremely variable and depends on the spectrum of the severity of disease.
- The mortality depends upon the cardiac output.
- Intrauterine mortality is up to 85%, among newborns the mortality 70% if cyanosis is present versus 15% if cyanosis is absent.
- The one-year survival is 67%, and the ten year survival is 59%.
References
- ↑ Kim HY, Jang SY, Moon JR, Kim EK, Chang SA, Song J, Huh J, Kang IS, Yang JH, Jun TG, Park SW (November 2016). "Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation". J. Korean Med. Sci. 31 (11): 1749–1754. doi:10.3346/jkms.2016.31.11.1749. PMC 5056206. PMID 27709852.
- ↑ Radford DJ, Graff RF, Neilson GH (November 1985). "Diagnosis and natural history of Ebstein's anomaly". Br Heart J. 54 (5): 517–22. doi:10.1136/hrt.54.5.517. PMC 481939. PMID 4052292.
- ↑ Watson H (May 1974). "Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international co-operative study of 505 cases". Br Heart J. 36 (5): 417–27. doi:10.1136/hrt.36.5.417. PMC 458838. PMID 4134860.
- ↑ Walsh EP (October 2018). "Ebstein's Anomaly of the Tricuspid Valve: A Natural Laboratory for Re-Entrant Tachycardias". JACC Clin Electrophysiol. 4 (10): 1271–1288. doi:10.1016/j.jacep.2018.05.024. PMID 30336873.
- ↑ Freeman A, Byard RW (May 2018). "Ebstein Anomaly and Sudden Childhood Death". J. Forensic Sci. 63 (3): 969–971. doi:10.1111/1556-4029.13652. PMID 28940541.