Pulmonary Atresia With Intact Ventricular Septum Treatment & Management

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

Medical Care

  • Initial treatment of pulmonary atresia with intact ventricular septum (PAIVS) consists of maintaining ductal patency with continuous intravenous prostaglandin E1 infusion.
  • To correct metabolic acidosis in a neonate, replace fluids and administer sodium bicarbonate.
  • Mechanical ventilation may be necessary if acidosis persists.
  • Patients ultimately require surgical palliation or therapeutic catheterization prior to hospital discharge.
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Surgical Care

Surgical algorithms for PAIVS depend on the size and morphology of both the tricuspid valve and the right ventricle, as well as the presence of abnormal coronary artery anatomy.

Mild tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections

  • Perform a surgical valvotomy or transannular patch, with or without a systemic-to-pulmonary artery shunt, or a transcatheter valvotomy, with or without stenting of the patent ductus arteriosus.
  • If the right ventricle and tricuspid valve grow, a 2-ventricle correction is probable in the future.
  • One-stage definitive repair has been described in 2 infants.[4] The repair comprised resection of hypertrophied muscles in the outflow and trabecular portions of the right ventricle (right ventricular overhaul technique), surgical valvotomy or transannular patch, and adjustable snare-closure of the foramen ovale.
  • A study by Chubb et al summarized long-term followup of 39 patients with PA/IVS who underwent valve perforations with or without stenting of the arterial duct. There were 8 deaths (21%), and 25 patients (83% of survivors) had a biventricular circulation despite small median tricuspid valve Z-scores.[5]

Moderate-to-severe tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections

  • Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus.
  • Future univentricular (Fontan) repair is likely.

Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections but no stenoses or interruption

  • Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus.
  • Future univentricular (Fontan) repair is likely.

Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections and proximal stenoses or interruption

  • Perform a systemic-to-pulmonary artery shunt or stenting of the patent ductus arteriosus.
  • Future univentricular (Fontan) repair or heart transplant is likely.
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Consultations

  • Pediatric cardiologist
  • Pediatric cardiothoracic surgeon
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Diet

  • Patients with PAIVS require increased caloric density during infancy to provide 120-130 kcal/kg/d for approximately 6 months.
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Activity

  • No specific activity restrictions are necessary.[6]
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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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