Pediatric Holt-Oram Syndrome Medication

Updated: Sep 06, 2019
  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Medication Summary

The specific cardiac defect and its effects dictate appropriate therapy. For example, treatment of congestive heart failure may include diuretics, an ACE inhibitor, and digoxin. Iron supplements are appropriate in patients with cyanotic heart disease.

Bacterial endocarditis prophylaxis is administered to patients with Holt-Oram syndrome (HOS) based on the specific cardiac condition. An isolated secundum atrial septal defect (ASD) does not require this treatment. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.



Class Summary

These agents eliminate retained fluid and lower preload.

Furosemide (Lasix)

Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. DOC in acute heart failure and in exacerbations of CHF. Used for the long-term management of CHF.

Spironolactone (Aldactone)

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

Amiloride (Midamor)

Potassium-sparing diuretic that acts directly on the distal renal tubule. Usually used along with a potassium-losing diuretic.


ACE inhibitors

Class Summary

These agents reduce afterload and decrease myocardial remodeling that worsen chronic heart failure.

Captopril (Capoten)

Widely accepted as an essential part of CHF treatment. Improves symptoms and prolongs survival. Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.


Cardiac glycosides

Class Summary

These agents improve symptoms, exert a positive inotropic effect on both the normal and failing heart, and are mediated through inhibition of transmembranous active transport of sodium and potassium. Clinically important actions are on the sinoatrial (SA) and AV nodes. Cardiac glycosides increase efferent vagal impulses, reflexly reduce sympathetic tone, and decrease the sinus rate. They decrease conduction velocity through the AV node.

Digoxin (Lanoxin)

Improves myocardial contractility, reduces heart rate, and lowers sympathetic stimulation in chronic heart failure.


Beta-adrenoceptor blockers

Class Summary

These agents relieve infundibular spasm in hypercyanotic spells.

Propranolol (Inderal)

Inhibits both beta1- and beta2-adrenergic receptors. The exact mechanism of benefit is uncertain, although it is believed to relieve infundibular spasm that precipitates hypercyanotic spells.