Pulmonary Atresia With Intact Ventricular Septum Follow-up

  • Author: John R Charpie, MD, PhD; Chief Editor: Stuart Berger, MD   more...
 
Updated: May 8, 2012
 

Further Inpatient Care

  • Admit patients with pulmonary atresia with intact ventricular septum (PAIVS) for future preoperative testing and surgical interventions.
  • Pulmonary valve replacement constitutes most late reoperations.
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Further Outpatient Care

  • Carefully monitor medication doses and adverse effects.
  • Monitor adequacy of repair/palliation with periodic echocardiography.
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Inpatient & Outpatient Medications

  • Possible discharge medications include digoxin, furosemide, and aspirin.
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Transfer

  • Transfer may be required for specialized diagnostic evaluation and surgical intervention.
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Complications

  • Congestive heart failure (CHF)
  • Sudden death
  • Arrhythmia
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Prognosis

  • Prognosis depends on the specific anatomy and type of intervention (univentricular or biventricular correction).
  • Overall survival is approximately 64-76% at age 5 years; however, single institutions report improved intermediate-term outcomes in relatively small series.
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Patient Education

  • Provide cardiopulmonary resuscitation (CPR) instruction to family members.
  • Educate family members about congenital heart disease (CHD).
  • Consider genetics counseling for future pregnancies.[7]
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Contributor Information and Disclosures
Author

John R Charpie, MD, PhD  Professor and Director, Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan Medical Center

John R Charpie, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles I Berul, MD  Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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 Pediatric 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, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References

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