Single Ventricle Medication

Updated: Aug 09, 2018
  • Author: Alvin J Chin, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Medication Summary

Preoperatively, administer alprostadil (ie, intravenous [IV] prostaglandin E1). Early postoperatively, warfarin is largely protective against venous thrombosis; the optimal late postoperative anticoagulation regimen has not yet been determined. Angiotensin-converting enzyme inhibitors, although popularly utilized, have not been shown to improve resting or exercise cardiac index. 

The medical treatment of postoperative patients with supraventricular arrhythmias is frequently complex, because many patients who have undergone a Fontan operation have sinus node dysfunction and can only be safely administered antiarrhythmic agents if a pacemaker is placed first. Catheter versus surgical therapy for atrial tachyarrhythmias appears to be preferred over medical therapy.

Digoxin, furosemide, spironolactone, and sildenafil are all used in long-term survivors. Various "maintenance drug regimens" are empirically used for patients who have undergone a Fontan operation, including the following:

  • No medications

  • Furosemide

  • Spironolactone

  • Angiotensin-converting enzyme (ACE) inhibitor (ACEI)

  • Sildenafil

  • Digoxin

  • Warfarin (for the first 3 months after fenestrated Fontan)

  • Aspirin (although no evidence suggests this helps for prophylaxis of venous thrombosis)

  • Combinations of the above medications

In an analysis of the Pediatric Heart Network Infant Single Ventricle public use dataset to evaluate associations between digoxin and survival, transplant-free survival, and change in weight-for-age Z (WAZ) before superior cavopulmonary connection (SCPC) and at 14 months in a mixed group of infants with single ventricle, investigators did not find an association between digoxin and improved survival during the interstage or at 14 months in the mixed group, but they noted a trend toward improved interstage transplant-free survival in infants who underwent the Norwood procedure. [62] In addition, digoxin was associated with poorer weight gain.



Class Summary

Alprostadil (PGE1) is used for treatment of ductal-dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.

Alprostadil IV (Prostin VR)

Used to maintain patency of the ductus arteriosus in neonates with ductal–dependent congenital heart disease until surgery can be performed. Has direct vasodilatation action on the ductus arteriosus and vascular smooth muscle.



Class Summary

These agents prevent recurrent or ongoing thromboembolic occlusion.

Warfarin (Coumadin)

Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor dose to maintain an INR in the range of 2-3.


Angiotensin-converting enzyme (ACE) inhibitors

Class Summary

The pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload.

Enalapril (Vasotec)

Competitive inhibitor of angiotensin-converting enzyme. Reduces angiotensin II levels, decreasing aldosterone secretion.

Captopril (Capoten)

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion. Rapidly absorbed, but bioavailability is significantly reduced with food intake. It achieves a peak concentration in an hour and has a short half-life. The drug is cleared by the kidney. Impaired renal function requires reduction of dosage. Absorbed well PO. Give at least 1 h before meals. If added to water, use within 15 min. Can be started at low dose and titrated upward as needed and as patient tolerates.

Lisinopril (Prinivil, Zestril)

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion.



Class Summary

These agents are indicated for management of edema.

Spironolactone (Aldactone)

For management of edema and hepatic congestion resulting from excessive aldosterone excretion. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Aldosterone inhibitors help block the renin-angiotensin system and help prevent potassium loss in the distal tubules. The body conserves potassium, and less PO potassium supplementation is needed.


Phosphodiesterase (type 5) Enzyme Inhibitor

Class Summary

These agents decrease pulmonary arterial pressure by causing vasodilation in the pulmonary vasculature.

Sildenafil (Revatio)

Promotes selective smooth muscle relaxation in lung vasculature possibly by inhibiting phosphodiesterase type 5 (PDE5). This results in subsequent reduction of blood pressure in pulmonary arteries and increase in cardiac output.