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PubMed This is a summary of 13 peer-reviewed journal articles Updated
Pediatric Cardiology · Pulmonary Atresia with Intact Ventricular Septum

Mapping the Heart: Diagnosis & Imaging for PA-IVS

At a Glance

The diagnosis of PA-IVS relies on postnatal imaging like echocardiograms, CT scans, and cardiac catheterization. These tests measure the tricuspid valve Z-score and check for right ventricle-dependent coronary circulation to help doctors determine the safest surgical pathway for your baby.

The journey to understanding your child’s heart starts with a series of sophisticated imaging tests. While a fetal echocardiogram (an ultrasound of the baby’s heart while in the womb) can often identify Pulmonary Atresia with Intact Ventricular Septum (PA-IVS), a postnatal diagnosis is essential to confirm the exact details of the heart’s anatomy [1].

Because every heart with PA-IVS is unique, doctors use these tests to create a “roadmap” that determines which surgical pathway is safest and most effective for your baby [2].

The Magic Number: The Tricuspid Valve Z-Score

One of the most important measurements the cardiologists will take is the Tricuspid Valve Z-score. A Z-score is a mathematical way of stating how much a valve’s size deviates from the “normal” average for a baby of the same size [3].

  • Why -3 matters: A Z-score of -3 is often considered a critical threshold in PA-IVS [4].
  • Z-score of -3 or higher: (For example, a Z-score of -2 or -1, meaning the valve is closer to a normal size). This generally suggests that the right ventricle is large enough to potentially handle a biventricular repair (using both sides of the heart) [5][1].
  • Z-score lower than -3: (For example, a Z-score of -4 or -5, meaning an even smaller valve). This often indicates the right ventricle is too small (hypoplastic), and the team may plan for a single-ventricle pathway or a “one-and-a-half” ventricle repair [6][5].

Key Imaging Tools

To get a complete picture, the medical team typically uses three main tools:

  1. Postnatal Echocardiogram: This is the first-line test after birth. It provides a detailed view of heart function, valve sizes, and initial clues about the coronary arteries [7].
  2. Coronary CT Angiography (CCTA): This is now considered a reliable, non-invasive “first-line” tool for looking specifically at the coronary arteries in neonates [8]. It helps doctors see if there are abnormal connections (sinusoids) or if the heart’s blood supply is dependent on the right ventricle [9].
  3. Cardiac Catheterization: If the CCTA or Echo results are unclear, or if the doctors need to perform an intervention (like opening the valve), they will use a “cath.” This is the “gold standard” for definitively ruling out Right Ventricle-Dependent Coronary Circulation (RVDCC) [10][8].

Checklist: What MUST be on the Imaging Report

When you review the imaging results with your cardiologist, ensure these four critical details have been clearly identified:

  • [ ] Tricuspid Valve (TV) Z-score: The specific measurement that helps guide the surgical pathway [3][4].
  • [ ] Right Ventricle (RV) Components: Does the RV have all three parts (the inlet, the apex/pump, and the outlet)? A “tripartite” (three-part) ventricle has the best growth potential [3].
  • [ ] Antegrade vs. Retrograde Coronary Flow: Is blood flowing “forward” from the aorta (normal) or is it being pushed “backward” from the right ventricle (RVDCC)? [11].
  • [ ] Coronary Stenosis or Atresia: Are there any narrowings or blockages in the coronary arteries that could affect the heart’s blood supply? [12].

Confirming these details—especially the presence or absence of RVDCC—is the most vital step in choosing the safest first procedure for your baby [13][6].

Common questions in this guide

What is the Tricuspid Valve Z-score in PA-IVS?
A Z-score is a measurement that shows how much your baby's tricuspid valve size differs from the normal average. A Z-score of -3 or higher often suggests the right ventricle may handle a biventricular repair, while a lower score may indicate the need for a single-ventricle surgical pathway.
What is Right Ventricle-Dependent Coronary Circulation (RVDCC)?
RVDCC happens when the heart's blood supply relies on the right ventricle pumping blood backward into the coronary arteries. Ruling this out with advanced imaging is a vital step in deciding the safest surgical approach for your baby.
Why does my baby need a CT scan or cardiac catheterization after an echocardiogram?
While an echocardiogram provides a detailed view of the heart, a Coronary CT Angiography (CCTA) or cardiac catheterization gives a clearer, definitive picture of the coronary arteries. These tests confirm if there are abnormal connections or blockages affecting the heart's blood supply.
What does it mean if my baby's right ventricle has all three components?
A right ventricle with all three components—the inlet, the apex or pump, and the outlet—is called a tripartite ventricle. This complete structure generally has the best potential for growth and function after surgical repair.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the exact postnatal Tricuspid Valve Z-score, and how does it affect the plan for my baby's first procedure?
  2. 2.Does my baby have all three components of the right ventricle (the inlet, the pump/apex, and the outlet)?
  3. 3.Has Right Ventricle-Dependent Coronary Circulation (RVDCC) been definitively ruled out? How was this confirmed (Echo, CCTA, or Catheterization)?
  4. 4.Can you show me on the imaging if the coronary arteries are receiving blood 'forward' from the aorta or 'backward' from the right ventricle?
  5. 5.Was there any evidence of coronary artery stenosis (narrowing) on the CCTA?

Questions For You

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References

References (13)
  1. 1

    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
  2. 2

    Pulmonary Atresia with Intact Ventricular Septum: Correlation of Preoperative Computed Tomography-Derived Parameters with Echocardiographic Tricuspid Valve Z-Score and Surgical Outcomes.

    Goo HW, Park SH, Goo SY

    Pediatric cardiology 2025; (46(6)):1560-1569 doi:10.1007/s00246-024-03570-1.

    PMID: 38953951
  3. 3

    Pulmonary Atresia With Intact Ventricular Septum With Borderline Tricuspid Valve: How Small Is Too Small.

    LaPar DJ, Bacha E

    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual 2019; (22()):27-31 doi:10.1053/j.pcsu.2019.02.007.

    PMID: 31027561
  4. 4

    Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

    Iliopoulos I, Mastropietro CW, Flores S, et al.

    Pediatric cardiology 2024; (45(4)):847-857 doi:10.1007/s00246-022-02954-5.

    PMID: 35751685
  5. 5

    Prenatal Echocardiographic Predictors of Postnatal Management Strategy in the Fetus with Right Ventricle Hypoplasia and Pulmonary Atresia or Stenosis.

    Cao L, Tian Z, Rychik J

    Pediatric cardiology 2017; (38(8)):1562-1568 doi:10.1007/s00246-017-1696-4.

    PMID: 28770306
  6. 6

    Pulmonary atresia with intact ventricular septum: Intended strategies.

    Sukhavasi A, McHugh-Grant S, Glatz AC, et al.

    The Journal of thoracic and cardiovascular surgery 2022; (164(5)):1277-1288 doi:10.1016/j.jtcvs.2021.11.104.

    PMID: 35414413
  7. 7

    Echocardiographic Assessment of Cardiac Phenotype Predicts Complications and Guides Intensive Care Management Following Pulmonary Valve Balloon Dilation in Neonates With Pulmonary Atresia/Critical Pulmonary Stenosis With Intact Ventricular Septum.

    Moras P, Luciano P, Campanale CM, et al.

    Echocardiography (Mount Kisco, N.Y.) 2025; (42(5)):e70182 doi:10.1111/echo.70182.

    PMID: 40367357
  8. 8

    Computed Tomographic Angiography Provides Reliable Coronary Artery Evaluation in Infants With Pulmonary Atresia Intact Ventricular Septum.

    Malone LJ, Browne LP, Morgan GJ, et al.

    Seminars in thoracic and cardiovascular surgery 2024; (36(3)):336-344 doi:10.1053/j.semtcvs.2022.10.003.

    PMID: 36244628
  9. 9

    Role of Computed Tomography Findings in Diagnosing Pulmonary Atresia With Intact Ventricular Septum and Right Ventricle-Dependent Coronary Circulation: A Case Report.

    Thurmann KE, Richardson RR

    Cureus 2025; (17(5)):e83914 doi:10.7759/cureus.83914.

    PMID: 40497184
  10. 10

    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
  11. 11

    Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

    Peterson RE, Freire G, Marino CJ, Jureidini SB

    Pediatric cardiology 2018; (39(5)):967-975 doi:10.1007/s00246-018-1846-3.

    PMID: 29516127
  12. 12

    Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

    Cheung EW, Mastropietro CW, Flores S, et al.

    The Annals of thoracic surgery 2023; (115(6)):1470-1477 doi:10.1016/j.athoracsur.2022.07.055.

    PMID: 36070807
  13. 13

    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

This page explains PA-IVS imaging and diagnostic terminology for educational purposes. Your pediatric cardiologist and surgical team are the best sources for interpreting your baby's specific heart anatomy and test results.

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