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Pediatric Holt-Oram Syndrome Medication

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Aug 05, 2015
 

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.

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Diuretics

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.

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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.

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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.

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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.

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Contributor Information and Disclosures
Author

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

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Photograph showing hypoplastic right thumb of the right hand of a 6-month-old infant with Holt-Oram syndrome.
Photograph of the left hand of a 6-month-old infant with Holt-Oram syndrome showing total aplasia of the left thumb.
Plain radiograph of the right forearm and hand of a 5-month-old infant with Holt-Oram syndrome showing hypoplastic radius and ulna and only 4 metacarpals.
A 2-dimensional echocardiographic picture taken from subxiphoid window showing a large secundum atrial septal defect (arrow) in a 7-year-old boy with Holt-Oram syndrome. ASD = Atrial septal defect; RA = Right atrium; RV = Right ventricle; LA = Left atrium; LV = Left ventricle.
Color Doppler echocardiographic picture taken from subxiphoid window showing the large left-to-right flow of blood (arrow) across the atrial septal defect. The red color pattern depicts flow direction from left atrium (LA) to right atrium (RA). ASD = Atrial septal defect; RA = Right atrium; RV = Right ventricle; LA = Left atrium; LV = Left ventricle.
 
 
 
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