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Tetralogy of Fallot With Pulmonary Stenosis Medication

  • Author: Michael D Pettersen, MD; Chief Editor: John Kupferschmid, MD  more...
 
Updated: Jan 08, 2014
 

Medication Summary

The majority of patients with tetralogy of Fallot (TOF) require no medical therapy prior to undergoing surgical repair. In rare instances, medications may be needed to treat ductal patency in severely cyanotic neonates and signs of congestive heart failure in patients with a minimal degree of right ventricular outflow tract obstruction.

Hypercyanotic spells are initially treated with nonpharmacologic means, including knee-chest position, supplemental oxygen, and volume expansion. In more severe episodes, additional pharmacologic interventions may include beta-blocker therapy to reduce right ventricular infundibular spasm and alpha-1 agonists to increase systemic vascular resistance.

In/outpatient medications

Most patients are on no medications prior to surgical repair. Beyond the initial postoperative period, most patients are also are on no medications. Select patients may require medication to treat ventricular dysfunction, congestive heart failure, or cardiac arrhythmias.

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Vasodilators

Class Summary

The use of a vasodilator will reduce systemic vascular resistance, allowing more forward flow, improving cardiac output.

Alprostadil (Prostin VR Pediatric)

 

Severely cyanotic neonates may require prostaglandin E1 to maintain ductal patency in order to provide adequate pulmonary blood flow.

Intravenous prostaglandin E1 may be continued until the time of surgical palliation.

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Diuretics

Class Summary

Loop diuretics decrease plasma volume and edema by causing diuresis. The reduction in plasma volume and stroke volume associated with diuresis decreases cardiac output and, consequently, blood pressure.

Furosemide (Lasix)

 

Patients with a minimal degree of right ventricular outflow tract obstruction may show signs and symptoms of pulmonary overcirculation. In these patients, diuretic therapy can be initiated to treat signs of congestive heart failure.

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Alpha1 Agonists

Class Summary

These agents improve the hemodynamic status by increasing myocardial contractility and heart rate, resulting in increased cardiac output. They also increase peripheral resistance by causing vasoconstriction. Increased cardiac output and increased peripheral resistance lead to increased blood pressure.

Phenylephrine

 

Phenylephrine is used to increase systemic vascular resistance.

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Beta-Blockers, Beta-1 Selective

Class Summary

These agents inhibit chronotropic, inotropic, and vasodilatory responses to beta-adrenergic stimulation.

Metoprolol (Lopressor, Toprol XL)

 

Beta-blocker therapy is used to reduce right ventricular infundibular spasm.

Hypercyanotic spells are initially treated with nonpharmacologic means, including knee-chest position, supplemental oxygen, and volume expansion. In more severe episodes, additional pharmacologic interventions may include beta-blocker therapy to reduce right ventricular infundibular spasm and phenylephrine to increase systemic vascular resistance.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Chief Editor

John Kupferschmid, MD Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio

John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

Jeffrey P Jacobs, MD, FACS, FACC, FCCP Clinical Associate Professor, Department of Surgery, University of South Florida College of Medicine; Medical Director, ECMO Program, Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/Bayfront Medical Center

Jeffrey P Jacobs, MD, FACS, FACC, FCCP is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Congenital Heart Surgeons Society, Society of Thoracic Surgeons, and Southern Thoracic Surgical Association

Disclosure: Nothing to disclose.

Robert DB Jaquiss, MD Professor of Surgery, University of Arkansas for Medical Sciences; Chief, Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital and Chief, Cardiothoracic Surgery, University of Arkansas for Medical Sciences

Robert DB Jaquiss, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, and Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School

Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Vibhuti N Singh, MD, MPH, FACC, FSCAI Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine; Director, Cardiology Division and Cardiac Catheterization Lab, Chair, Department of Medicine, Bayfront Medical Center, Bayfront Cardiovascular Associates; President, Suncoast Cardiovascular Research

Vibhuti N Singh, MD, MPH, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Florida Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine

Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

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