Cor triatriatum

Background

XC: anomalous upper chamber

• Congenital anomaly: heart with three atria
• Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum or cor triatriatum dextrum)
• Can be associated with other congenital syndromes including tetralogy of Fallot, double outlet RV, coarctation of the aorta, VSD, AV septal defect
• 0.1-0.4% incidence in USA

Clinical Features

• Sinistrum variant most associated with other congenital cardiovascular defects in symptomatic infants
• Can be incidental finding in adults as either isolated finding or in association with PFO, ASD, persistent left superior vena cava
• Symptoms depend on size of opening in accessory membrane between the atria
• Symptoms include dyspnea on exertion, orthopnea, easy fatiguability, low exercise tolerance, palpitations
• Increased risk of thrombus formation in LA
• Murmur
• Elevated JVD
• Hepatomegaly
• Ascites
• Peripheral edema

Differential Diagnosis

• Pericarditis
• Pulmonary Hypertension
• Right heart failure
• Atrial Myxoma
• Mitral Stenosis
• Tricuspid Stenosis

Congenital Heart Disease Types

• Cyanotic
  • Tetralogy of fallot
  • Total anomalous pulmonary venous return
  • Transposition of the great arteries
  • Tricuspid atresia
  • Truncus arteriosus
  • Ebstein anomaly
  • Hypoplastic left heart syndrome
• Acyanotic
  • AV canal defect
  • Atrial septal defect (ASD)
  • Ventricular septal defect (VSD)
  • Cor triatriatum
  • Patent ductus arteriosus (PDA)
  • Pulmonary/aortic stenosis
  • Coarctation of the aorta
• Differentiation by pulmonary vascularity on CXR
  • Increased pulmonary vascularity
    • Large ASD
    • Large VSD
    • Total anomalous pulmonary venous return
    • Transposition of the great arteries
    • Truncus arteriosus
  • Decreased pulmonary vascularity
    • Tetralogy of fallot
    • Rare heart diseases with pulmonic stenosis

Evaluation

• Initial workup may include:
  • ECG: Atrial premature complexes, Left or Right atrial abnormalities, Right axis deviation, Right heart strain
  • CXR
  • Echocardiography
  • Right heart cath

Management

• As associated with major cyanotic or acyanotic congenital heart lesions, mortality can be up to 75% in untreated symptomatic infants
• Treat increased pulmonary vascular resistance, heart failure
• Inotropic agents (eg, dopamine, dobutamine), diuretics
• Manage hypoxia, fluid status, rate in afib
• DVT/PE prophylaxis
  • Cardiac surgery consultation

See Also

• Congenital heart disease