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Outcomes in patients with cor triatriatum sinister
Margaret M Fuchs1, Heidi M Connolly1, Sameh M Said2
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Cor triatriatum sinister (CTS) typically shows stable left atrial obstruction, particularly in isolated cases diagnosed in adulthood. Surgical resection of the CTS membrane is safe and effective, with a low recurrence risk.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Cor triatriatum sinister (CTS) is a rare congenital heart defect characterized by a membrane dividing the left atrium.
- Understanding the natural history and outcomes of CTS is crucial for patient management.
Purpose of the Study:
- To describe the clinical outcomes and natural history of patients diagnosed with cor triatriatum sinister (CTS).
- To evaluate the effectiveness and safety of surgical intervention for CTS.
Main Methods:
- Retrospective review of 57 patients with CTS from 1990 to 2016.
- Analysis of clinical data, operative reports, and multimodality imaging (echocardiogram, MRI, CT, cardiac catheterization).
Main Results:
- Diagnosis was often delayed, with 61% diagnosed in adulthood.
- 47% of patients underwent surgical resection of the CTS membrane, with younger age and associated congenital heart disease (CHD) being predictors for surgery.
- Surgery was associated with one perioperative mortality and no late mortality or recurrence; conservative management also showed stability.
Conclusions:
- CTS generally follows a stable course without progressive obstruction, especially in isolated cases or those diagnosed later in life.
- Surgical membrane resection for symptomatic CTS is safe and effective, offering excellent long-term outcomes with minimal recurrence.
- Multimodality imaging plays a key role in CTS diagnosis and management planning.
Objective:
To describe outcomes in patients with cor triatriatum sinister (CTS).
Design:
Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization.
Results:
Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of 57 (58%) patients had additional congenital heart disease (CHD) diagnosis. A total of 27 (47%) patients required surgical resection of CTS membrane during median follow-up of 76 months, and these patients were younger at the time of CTS diagnosis (26 vs 41, P = 0.01) and more likely to have associated CHD (55% vs 45%, P = 0.02). There was one perioperative mortality and no late mortality. There was no recurrence of CTS membrane obstruction in the patients that underwent surgery. Similarly there was no significant increase in CTS membrane gradient in the patients that were managed conservatively.
Conclusions:
The natural history of CTS is stability without progressive left atrial obstruction, especially in patients with isolated CTS and in those with initial CTS diagnosis made in adulthood. In patients requiring surgical membrane resection due to flow obstruction, surgery is safe and effective with very low risk of recurrence.
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