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.
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.
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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Pediatric Cor Triatriatum. Long-axis parasternal view demonstrating a left atrial membrane separating pulmonary vein inflow from left ventricular (mitral valve) inflow. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
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Pediatric Cor Triatriatum. Long-axis parasternal view depicting a two-dimensional image of cor triatriatum sinister membrane and a color Doppler image of pulmonary venous flow through the orifice of the membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
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Pediatric Cor Triatriatum. Short-axis parasternal view depicting right and left pulmonary vein flow proximal to the cor triatriatum left atrial membrane and left atrial appendage orifice distal to the cor triatriatum left atrial membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
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Pediatric Cor Triatriatum. Subxiphoid coronal image of the posterior left atrial chamber that receives pulmonary venous flow separated from the rest of the left atrium by the cor triatriatum membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
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Pediatric Cor Triatriatum. Apical five-chamber view demonstrating a 4-5 mm left atrial membrane orifice with mild pulmonary venous inflow restriction. Note the presence of an associated perimembranous ventricular septal defect (VSD) with tricuspid septal aneurysmal tissue. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
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Pediatric Cor Triatriatum. This sonogram shows a mean Doppler gradient of 7-8 mmHg across the left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
