Congenitally Corrected Transposition Medication

  • Author: Arnold S Baas, MD, FACC, FACP; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Jul 2, 2010
 

Medication Summary

Medications include antibiotic prophylaxis for procedures or dental work and standard therapy for heart failure (diuretic drugs, digitalis, beta-blockers, and ACE inhibitors). All are helpful for symptomatic therapy, but none are demonstrated to improve mortality rates.

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Antibiotics

Class Summary

Empiric antimicrobial therapy should cover all likely pathogens in the context of this clinical setting.

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Amoxicillin (Amoxil, Trimox)

 

Interferes with synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria. Recommended prophylactic regimen for dental, oral, or upper respiratory procedures per American Heart Association guidelines.

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Ampicillin (Omnipen, Principen)

 

For prophylaxis in patients undergoing dental, oral, or respiratory tract procedures. Patients unable to take oral medications may be given ampicillin IV.

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Clindamycin (Cleocin)

 

Used in penicillin-allergic patients undergoing dental, oral, or respiratory tract procedures.

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Diuretics

Class Summary

These agents are used for treatment of pulmonary or hepatic congestion and peripheral edema due to heart failure.

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Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system in kidney, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Titrate dose according to response.

Available as 20-, 40-, and 80-mg tablets.

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Angiotensin converting enzyme inhibitors

Class Summary

These agents offer a mortality benefit in CHF and left ventricular dysfunction in patients with structurally normal hearts.

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Lisinopril (Prinivil, Zestril)

 

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion. Not recommended in patients with one kidney.

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Ramipril (Altace)

 

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

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Captopril (Capoten)

 

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

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Enalapril (Vasotec)

 

Competitive inhibitor of ACE. Reduces angiotensin II levels, decreases aldosterone secretion.

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Quinapril (Accupril)

 

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 inhibit sodium-potassium adenosine triphosphatase (ATPase), increasing intracellular calcium. Used in treatment of mild to moderately severe CHF.

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Digoxin (Lanoxin)

 

Cardiac glycoside with direct inotropic effects in addition to indirect effects on cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions through intracellular calcium release. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

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Beta-blockers

Class Summary

These agents have not been studied in patients with right systemic ventricle heart failure. Beta-blockers have mortality benefits in the general heart failure population and must be considered in the population of patients with complex congenital heart disease. Initiate beta-blockers only in patients whose condition is stable, without CHF symptoms, and titrate slowly.

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Metoprolol (Lopressor, Toprol XL)

 

Selective beta1-adrenergic receptor blocker that decreases automaticity of contractions. During IV administration, carefully monitor BP, heart rate, and ECG.

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Carvedilol (Coreg)

 

Used to reduce disease progression in CHF. Effects include beta-blockade, alpha1-blockade, and antioxidant properties.

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Contributor Information and Disclosures
Author

Arnold S Baas, MD, FACC, FACP  Assistant Professor of Medicine, Division of Cardiology, University of California, Los Angeles School of Medicine; Attending Physician, UCLA Santa Monica Hospital and UCLA Westwood Hospital

Arnold S Baas, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

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Subcostal view of a 1-year-old child with L-transposition of the great arteries, valvular and subvalvular pulmonic stenosis, and a moderate outlet ventriculoseptal defect (VSD). Note the ventriculoarterial discordance. Note the posterior, rightward position of the pulmonary artery. [PA = pulmonary artery, LV = left ventricle, RV = right ventricle].
Apical image revealing atrioventricular discordance. Note the pulmonary venous return into the left atrium, with sequential flow through the tricuspid valve to the right ventricle. The right ventricle is systemic. [LA = left atrium, RA = right atrium, LV = left ventricle, RV = right ventricle].
Post-Rastelli repair with left ventricle to aortic baffle through a ventriculoseptal defect (VSD) complicated by subaortic stenosis.
This image demonstrates a calcified pulmonary homograft anterior and adjacent to the chest wall (right ventricle to pulmonary artery bifurcation) with significant homograft stenosis and prior pulmonary valvular endocarditis (same patient as in Image 3).
 
 
 
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