Medication Summary
Medical management in cor triatriatum is targeted toward associated elevation in pulmonary vascular resistance and heart failure. It is continued in the postoperative period until the pulmonary resistance falls and right ventricular performance improves. Mainstays of treatment are inotropic agents and diuretics.
Inotropes are used in patients with low cardiac output secondary to heart failure. Agents such as dopamine predominate in the intensive are unit (ICU), whereas agents such as digoxin are sometimes used in the outpatient setting.
Diuretics are used in patients with pulmonary edema.
Patients with pulmonary edema are unlikely to respond to inhaled nitric oxide preoperatively because the pulmonary hypertension is secondary to mechanical obstruction.
Inotropic agents
Class Summary
Cardiac glycosides (eg, digoxin) increases myocardial contractility in patients with heart failure. Adrenergic and dopaminergic agents (eg, dopamine) provide myocardial support in the perioperative period for patients with heart failure. The more restrictive the connection between proximal and distal chambers, the more likely inotropic support is required. Numerous agents are available in this category.
Digoxin (Lanoxin)
Exerts its inotropic action by increasing the amount of intracellular calcium available during excitation-contraction coupling. It is one of numerous inotropic agents that can be used in infants with congenital cardiac defects. Other agents, such as dopamine (described below), are more appropriate for acute management of heart failure in ICU setting.
Dopamine (Intropin)
Adrenergic agonists are often used in the critical care setting for their rapid onset of action and rapid peak effect. They are, therefore, much easier to titrate to effect in acute settings. Their half-life is also much shorter than digoxin's, and their effects are rapidly lost when drug is discontinued.
Loop diuretics
Class Summary
These agents are used for management of right heart failure and pulmonary edema.
Furosemide (Lasix)
First-line drug for diuresis in newborns and infants and can be expected to be highly effective. It is a sulfonamide derivative that exerts its effects on the loop of Henle and distal renal tubule, thus inhibiting reabsorption of sodium and chloride.
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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.
