Learning about Cor Triatriatum

What is Cor Triatriatum?

First reported in 1868, cor triatriatum, that is, a heart with 3 atria (triatrial heart), is a congenital anomaly in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into 2 compartments by a fold of tissue, a membrane, or a fibromuscular band.

Classically, cor triatriatum presents in infancy with signs and symptoms of pulmonary hypertension and pulmonary venous obstruction. Due to low cardiac output, children can show poor growth and weight gain, feeding difficulties, respiratory distress, and tet spells.

Cause

Cor triatriatum dextrum is extremely rare and results from the complete persistence of the right sinus valve of the embryonic heart. The membrane divides the right atrium into a proximal (upper) and a distal (lower) chamber. The upper chamber receives the venous blood from both vena cava and the lower chamber is in contact with the tricuspid valve and the right atrial appendage.

Evaluation

The mainstay of evaluation and diagnosis includes imaging studies such as chest x-ray, ECG and echocardiography, angiography and left and right heart catheterization.

• Chest X-Ray
  A chest x-ray is the initial investigation of choice. The findings include:

-Pulmonary congestion with haziness (Kerley B-lines)
-Ground glass appearance of acute pulmonary edema
-Prominent pulmonary vessels
-Pleural effusion
-Left atrial enlargement
-Cardiomegaly

• Electrocardiography (ECG)
  ECG findings are non-specific in cor triatriatum. Can range from atrial fibrillation and no specific P-wave changes to right axis deviation due to pulmonary congestion and right ventricular hypertrophy.
• Echocardiography
  Echocardiography is the diagnostic modality of choice as it not only allows for definitive diagnosis, but the 3-dimensional reconstruction of echocardiographic images pinpoints the exact location of the defect and appendage, helping direct the surgical approach to the disease.
• Angiography
  Angiography helps determine the severity of obstruction and the time of surgical intervention needed.

Treatment
The heart disorder treatment is dependent on the type of patients.

• For Asymptomatic Patients
  Asymptomatic patients need no specific treatment. Observe the patients for the development of signs and symptoms. Schedule regular medical follow-ups.
• For Symptomatic Patients
  Treatment options for symptomatic patients includes both medical/conservative management and surgical repair.-Medical treatment
  This includes Hemodynamic stabilization of fluid overload and pulmonary edema, rate and rhythm control and anticoagulation for patients with atrial fibrillation, thromboembolic prophylaxis with anticoagulation against deep vein thrombosis, pulmonary embolism and stroke or obtaining surgical consultation-Surgical Management
  Surgery is the definitive treatment. Complete surgical resection of atrial appendage/accessory membrane through a midline sternotomy under cardiopulmonary bypass and closure of atrial septum with a pericardial patch provides the optimum cure. The 10-year survival rate following surgery is 83%, while patients with coexisting congenital heart diseases have a greater risk of adverse outcomes and a lower survival rate

Differential Diagnosis

Many congenital heart diseases can present similarly to cor triatriatum and should be taken into consideration when making a diagnosis of this disorder. These include:

• Supravalvular mitral stenosis
• Mitral stenosis
• Idiopathic pediatric pulmonary arterial hypertension
• Pulmonary venous hypertension
• Total anomalous pulmonary venous return
• Partial anomalous pulmonary venous return
• Atrial septal defect/ ventricular septal defect
• Ventricular septal defect
• Idiopathic pulmonary hypertension
• Tricuspid stenosis
• Atrial myxoma

An experienced surgical team should evaluate imaging results as high suspicion can accelerate diagnosis and delay or prevent unfavorable outcomes.

Complications

• Right-sided failure
• Pulmonary edema/hypertension
• Atrial arrhythmias
• Death

Considering the rarity of this congenital heart, we at Genesis Foundation have provided support for this heart disorder treatment to only one such child with Cor Triatrium.

Ekadant Ratheesh was operated for Cor Triatriatum

Three months old Ekadant Ratheesh weighs 5 kgs and is the second child of Aiswarya and Ratheesh from Ernakulam in Kerala. He has an elder sister aged seven years. When Ekadant was barely a month old, his parents noticed that he had difficulty in breathing. They consulted a pediatrician who suspected some heart defect. He was put on medication and the parents were advised to come after six months for a review. However, Ekadant’s condition showed no improvement and he had to be admitted to the hospital various times. Ratheesh is a decoration worker earning a humble salary of Rs 4000 per month. He had to take a loan of Rs 250,000 to bear the medical expenses. Ekadant was shown to another pediatrician who referred them to the Amrita Institute of Medical Sciences, Kochi. Ekadant had a resting saturation of 85%. A detailed Echo evaluation revealed that he was suffering from Fossa Ovalis ASD (Classic Cortriatriatum with Moderate Pulmonary Hypertension). In this condition all the Pulmonary veins open into a common chamber which is separated from the actual atrium by a diaphragm. Simultaneously there was an Atrial Septal Defect (ASD). The child required Cor Triatriatum repair through an open-heart surgery. His parents had run out of means to pay for their child’s surgery. The Hospital sought our help. Through our CSR partnership with ACRE we were able to pay for the child’s treatment. The State government insurance too contributed towards this surgery. The surgery was successful, and the baby is stable.

–  Contributed by R.Srivatsan