Pediatric Cor Triatriatum Treatment & Management

Updated: Dec 18, 2020
  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD  more...
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Treatment

Medical Care

The goal of medical care in cor triatriatum is to reduce the symptoms caused by pulmonary venous congestion until definitive surgical therapy can be performed.

Patients presenting in extremis should be operated on immediately after resuscitation, without time spent for prolonged medical therapy.

On occasion, extracorporeal membrane oxygenation is initiated to stabilize a patient.

Consultations

Obtain consultations with a pediatric cardiologist and a pediatric cardiac surgeon.

Transfer

Admit patients with cor triatriatum who have undergone surgical treatment to a pediatric ICU experienced in dealing with congenital cardiac defects.

Provide postoperative treatment of heart failure and pulmonary hypertension until pulmonary vascular resistance normalizes.

Diet and activity

No specific dietary restrictions are recommended.

Physical activity should not be limited in patients with early and complete correction.

Patients with persistent pulmonary or cardiac dysfunction secondary to long-standing disease may have moderate restriction of exercise tolerance.

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Surgical Care

Surgery is the treatment of choice for cor triatriatum. In relatively recent years, interventional catheterization techniques have evolved and been used successfully in some patients. [25]

Surgical correction

Open correction is currently preferred over closed (percutaneous) procedures.

The procedure is performed on cardiopulmonary bypass through an atrial incision with complete resection of the diaphragm.

Interventional cardiology

The role of percutaneous balloon dilation in managing this condition remains to be determined.

Postoperative care

Admit patients with cor triatriatum who have undergone surgical treatment to a pediatric ICU experienced in dealing with congenital cardiac defects.

Provide postoperative treatment of heart failure and pulmonary hypertension until pulmonary vascular resistance normalizes.

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Long-Term Monitoring

Successful surgical correction of cor triatriatum allows a return to a normal lifestyle without restriction of activity or need for medications.

Serial echocardiography is a reliable and effective method for following patients on an outpatient basis. Late complications are rare.

Recurrent membrane stenosis from incomplete surgical resection can occur and is well demonstrated by echocardiography. This imaging modality may also demonstrate residual pulmonary vein stenosis.

Postoperative function and exercise tolerance should approach normal. Long-term activity restrictions are usually unnecessary.

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