Medication Summary
Medical management is ineffective in the treatment of sinus venosus defects. The rare patient who presents in congestive heart failure can be stabilized medically with diuretics and inotropic support.
Inotropic agents
Class Summary
These agents provide myocardial support in patients with dysfunction secondary to pulmonary overcirculation from left-to-right shunting. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure (CHF). Some may also increase or decrease the heart rate (ie, positive or negative chronotropic agents), provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances.
Digoxin (Lanoxin)
Exerts its inotropic effects by increasing amount of intracellular calcium available during excitation-contraction coupling. One of numerous inotropic agents that can be used in infants with congenital cardiac defects. Generally used for long-term administration and is rarely drug of choice for acute management of heart failure in ICU setting.
Dopamine (Intropin)
Adrenergic agonists often are used for inotropic support in critical care setting for their rapid onset of action and rapid time to peak effect, which make them easier to titrate to effect
Loop diuretics
Class Summary
These agents are used for management of right heart failure and pulmonary edema. They promote excretion of water and electrolytes by the kidneys.
Furosemide (Lasix)
Highly effective first-line diuretic in newborns and infants. A sulfonamide derivative, it exerts its effects on the loop of Henle and distal renal tubule, inhibiting reabsorption of sodium and chloride.
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Panel A. Transesophageal echocardiogram (transverse view) of a patient with a sinus venosus defect of the superior vena cava (SVC) type. The original defect (white star burst) has been repaired by placing a baffle (arrows), which directs blood from the anomalously connected right upper pulmonary vein into the left atrium (LA). In this patient, the baffle was redundant so at a more rostral level (Panel B), it could be seen (black open arrows) to bulge into the superior vena cava (SVC)–right atrial (RA) junction (trio of white arrows). The remainder of the atrial septum is denoted by the duo of white open arrows. Panel C is a transesophageal echocardiogram, sagittal view. Doppler color flow mapping verifies that the protruding baffle (white closed arrows) results in a narrowing of the pathway from the SVC to the RA. The quartet of white open arrows points to the remainder of the atrial septum.
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Panel A is a transesophageal echocardiogram, transverse view. The white star burst shows the sinus venosus defect of the inferior vena cava (IVC) type, lying adjacent to the IVC junction with the right atrium (RA). The remainder of the atrial septum is just out of the view of this sector but is represented by the white open arrowheads. The leaflets of the closed tricuspid valve (TV) are visible. RV = right ventricle. Panel B is a transesophageal echocardiogram, sagittal view. This is the same patient as in Panel A. This view proves that the rostral portion of the atrial septum (which would be missing in a patient with a sinus venosus defect of the SVC type) is intact. ct = crista terminalis; svc = superior vena cava.
