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PubMed This is a summary of 18 peer-reviewed journal articles Updated
Pediatric Cardiology · Pulmonary Atresia with Intact Ventricular Septum

First Steps: Initial Stabilization and Interventions

At a Glance

The immediate priority for a baby born with PA-IVS is stabilizing blood flow to the lungs. Doctors typically use an IV medication called Prostaglandin E1 (PGE1) to keep the ductus arteriosus open. Following stabilization, procedures like PDA stenting or a BT shunt secure long-term blood flow.

The moments following the birth of a baby with Pulmonary Atresia with Intact Ventricular Septum (PA-IVS) are a whirlwind of activity. Your baby will be quickly moved to a specialized unit, often a Neonatal Intensive Care Unit (NICU) or a Cardiac Intensive Care Unit (CICU) [1]. While the environment can feel overwhelming—with monitors, IV lines, and a large team of specialists—the immediate goal is straightforward: stabilization and keeping the blood flowing to the lungs [2].

The Lifeline: Prostaglandin E1 (PGE1)

In the womb, all babies have a natural tunnel between their heart vessels called the ductus arteriosus [3]. Normally, this tunnel closes shortly after birth. However, for a baby with PA-IVS, this tunnel is their lifeline—it is the only way oxygen-poor blood can reach the lungs [4].

To keep this tunnel open, doctors will start an IV infusion of a medication called Prostaglandin E1 (PGE1 or Alprostadil) [3][5].

  • What to expect: PGE1 is highly effective, but it has common side effects. The most notable is apnea (pauses in breathing), which may require the baby to be placed on a ventilator (a breathing machine) [6].
  • Other effects: It is also common for babies on PGE1 to develop a low-grade fever or low blood pressure, both of which are carefully managed by the CICU team [6][7].

Emergency Relief: Balloon Atrial Septostomy

As discussed in the Anatomy section, blood blocked from exiting the right side of the heart must cross over to the left side through a hole between the upper chambers (the atrial septum). If this hole is too small (restrictive), blood gets dangerously backed up [8]. To relieve this pressure, the team may perform an emergency Balloon Atrial Septostomy (BAS) in the catheterization lab—using a balloon to safely enlarge the hole [9].

Initial Interventions: Opening the Path

Once the baby is stable on PGE1, the medical team will perform detailed imaging to determine the safest next step. Depending on the heart’s anatomy, they will choose one of several initial interventions:

1. Opening the Pulmonary Valve

If the right ventricle is large enough and Right Ventricle-Dependent Coronary Circulation (RVDCC) has been definitively ruled out, doctors may try to open the blocked valve [10][11]. This is often done in the cardiac catheterization lab using:

  • Radiofrequency Perforation: Using a specialized wire to create a tiny hole in the blocked valve [12].
  • Balloon Valvuloplasty: Using a small balloon to stretch the valve open, allowing blood to finally flow from the right ventricle to the lungs [12][9].

2. Securing Pulmonary Blood Flow

If opening the valve isn’t possible or isn’t enough, the team must create a more permanent “bridge” than the PGE1 infusion. There are two main ways to do this:

  • PDA Stenting: A small, mesh tube (stent) is placed inside the ductus arteriosus to keep it propped open [1]. This is a less invasive procedure and often leads to shorter hospital stays [13][14].
  • Surgical Shunt (BT Shunt): A surgeon places a small synthetic tube to create a permanent connection between the heart’s vessels and the lungs [15]. While more invasive than a stent, it is a time-tested method for ensuring consistent blood flow [16].

The CICU Environment and Feeding

Parents often wonder when they can take their baby home. The initial CICU stay usually lasts several weeks, depending on the baby’s stability and procedures [1].

During this time, feeding can be challenging. Babies on PGE1 or awaiting a shunt often need a feeding tube (NG tube) passed through their nose to their stomach to ensure they get nutrition without spending too much energy [1][17]. The team will heavily support breast milk pumping or formula choices to ensure your baby is nourished while they stabilize [18].

Common questions in this guide

Why does my baby with PA-IVS need Prostaglandin E1 (PGE1)?
PGE1 is an IV medication used to keep a natural blood vessel connection called the ductus arteriosus open. This tunnel is the only way for oxygen-poor blood to reach your baby's lungs until a more permanent procedure can be performed.
What is a Balloon Atrial Septostomy and why is it needed?
A Balloon Atrial Septostomy is an emergency catheter procedure used when blood gets dangerously backed up in the right side of the heart. A doctor uses a small balloon to safely enlarge the hole between the heart's upper chambers to relieve the pressure.
How do doctors open the blocked pulmonary valve?
If the heart's right ventricle is large enough, doctors may use a cardiac catheterization procedure like radiofrequency perforation or balloon valvuloplasty. These techniques use a specialized wire or small balloon to stretch or create an opening in the blocked valve.
What is the difference between a PDA stent and a surgical BT shunt?
A PDA stent is a small mesh tube placed inside the ductus arteriosus to keep it propped open, which is less invasive. A BT shunt is a surgical procedure where a synthetic tube is placed to create a permanent connection between the heart's vessels and the lungs.
How will my baby be fed while in the intensive care unit?
Babies in the cardiac intensive care unit often receive breast milk or formula through a feeding tube (NG tube) that goes from their nose to their stomach. This ensures they get essential nutrition without spending too much energy while they stabilize.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What dose of PGE1 is my baby receiving, and are we currently using the lowest effective dose to minimize side effects?
  2. 2.Has my baby experienced any apnea since starting the medication, and what is the plan if they do?
  3. 3.Given my baby's ductal anatomy, would you recommend a PDA stent or a surgical shunt (BT shunt)?
  4. 4.If the plan is to open the pulmonary valve, how was RVDCC definitively ruled out beforehand?
  5. 5.What specific monitors and life-support machines will be connected to my baby in the CICU?

Questions For You

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References

References (18)
  1. 1

    Comparison of Ductal Stent Versus Surgical Shunt as Initial Intervention for Neonates with Pulmonary Atresia with Intact Ventricular Septum.

    Puente BN, Mastropietro CW, Flores S, et al.

    Pediatric cardiology 2024; doi:10.1007/s00246-024-03529-2.

    PMID: 38842558
  2. 2

    Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty.

    Haddad RN, Hanna N, Charbel R, et al.

    Cardiology in the young 2019; (29(4)):492-498 doi:10.1017/S1047951119000118.

    PMID: 31030705
  3. 3

    Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines?

    Vari D, Xiao W, Behere S, et al.

    Cardiology in the young 2021; (31(1)):63-70 doi:10.1017/S1047951120003297.

    PMID: 33140712
  4. 4

    Stenting of the ductus arteriosus for ductal-dependent pulmonary blood flow-current techniques and procedural considerations.

    Aggarwal V, Petit CJ, Glatz AC, et al.

    Congenital heart disease 2019; (14(1)):110-115 doi:10.1111/chd.12709.

    PMID: 30811792
  5. 5

    Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease.

    Aykanat A, Yavuz T, Özalkaya E, et al.

    Pediatric cardiology 2016; (37(1)):131-4 doi:10.1007/s00246-015-1251-0.

    PMID: 26260095
  6. 6

    Effectiveness of Alprostadil for Ductal Patency.

    Gordon CM, Tan JT, Carr RR

    The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2024; (29(1)):37-44 doi:10.5863/1551-6776-29.1.37.

    PMID: 38332962
  7. 7

    Cumulative Dose of Prostaglandin E1 Determines Gastrointestinal Adverse Effects in Term and Near-Term Neonates Awaiting Cardiac Surgery: A Retrospective Cohort Study.

    Ofek Shlomai N, Lazarovitz G, Koplewitz B, Eventov Friedman S

    Children (Basel, Switzerland) 2023; (10(9)) doi:10.3390/children10091572.

    PMID: 37761532
  8. 8

    Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.

    Liu L, Wang H, Cui C, et al.

    Medicine 2019; (98(42)):e17492 doi:10.1097/MD.0000000000017492.

    PMID: 31626103
  9. 9

    Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter.

    Lefort B, Saint-Etienne C, Soulé N, et al.

    Congenital heart disease 2019; (14(5)):814-818 doi:10.1111/chd.12812.

    PMID: 31290594
  10. 10

    Commentary: Despite best intentions: Developing better strategies for patients with pulmonary atresia with intact ventricular septum.

    Barron DJ, Vanderlaan RD

    The Journal of thoracic and cardiovascular surgery 2022; (164(5)):1289-1290 doi:10.1016/j.jtcvs.2021.12.038.

    PMID: 34998589
  11. 11

    Transcatheter radiofrequency pulmonary valve perforation in newborns with pulmonary atresia/intact ventricular septum: Echocardiographic predictors of biventricular circulation.

    Yoldaş T, Örün UA, Doğan V, et al.

    Echocardiography (Mount Kisco, N.Y.) 2020; (37(8)):1258-1264 doi:10.1111/echo.14811.

    PMID: 32762137
  12. 12

    Radiofrequency Perforation of an Atretic Pulmonary Valve with a Modified Coronary Wire and Electrocautery Pencil.

    Mejia E, Soszyn N, Morgan GJ, Leahy RA

    Pediatric cardiology 2024; (45(7)):1607-1609 doi:10.1007/s00246-023-03270-2.

    PMID: 37606651
  13. 13

    Comparison of Immediate Intensive Care Outcomes of Patent Ductus Arteriosus Stenting Versus Modified Blalock-Taussig-Thomas Shunt in Infants With Ductal-Dependent Pulmonary Circulation.

    Sirisani JD, Haranal M, Soo KW, et al.

    Pediatric cardiology 2024; doi:10.1007/s00246-024-03591-w.

    PMID: 39073479
  14. 14

    Patent Ductus Arteriosus Stent Versus Surgical Aortopulmonary Shunt for Initial Palliation of Cyanotic Congenital Heart Disease with Ductal-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-Analysis.

    Tseng SY, Truong VT, Peck D, et al.

    Journal of the American Heart Association 2022; (11(13)):e024721 doi:10.1161/JAHA.121.024721.

    PMID: 35766251
  15. 15

    Blalock-Taussig Shunt versus Ductal Stenting as Palliation for Duct-Dependent Pulmonary Circulation.

    Al Kindi H, Al Harthi H, Al Balushi A, et al.

    Sultan Qaboos University medical journal 2023; (23(Spec Iss)):10-15 doi:10.18295/squmj.12.2023.073.

    PMID: 38161753
  16. 16

    Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

    Valencia E, Staffa SJ, Kuntz MT, et al.

    Journal of the American Heart Association 2023; (12(17)):e030528 doi:10.1161/JAHA.123.030528.

    PMID: 37589149
  17. 17

    Systemic Venous Hypertension and Low Output Are Prevalent at Catheterization in Adults with Pulmonary Atresia and Intact Ventricular Septum Regardless of Repair Strategy.

    Jain CC, Egbe AC, Stephens EH, et al.

    Seminars in thoracic and cardiovascular surgery 2022; (34(4)):1312-1319 doi:10.1053/j.semtcvs.2021.10.006.

    PMID: 34688901
  18. 18

    Myocardial ischemia risk in Fontan candidates with pulmonary atresia with intact ventricular septum.

    Masaki N, Tatewaki H, Matsuo S, et al.

    General thoracic and cardiovascular surgery 2025; (73(9)):675-681 doi:10.1007/s11748-025-02131-4.

    PMID: 40032811

This page explains initial stabilization and NICU interventions for infants with PA-IVS for educational purposes. Always consult your pediatric cardiologist or neonatologist regarding your baby's specific care plan.

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