Pediatric Cardiology

, Volume 40, Issue 3, pp 526–536 | Cite as

Differences in Right Ventricular Physiologic Response to Chronic Volume Load in Patients with Repaired Pulmonary Atresia Intact Ventricular Septum/Critical Pulmonary Stenosis Versus Tetralogy of Fallot

  • Andrew L. ChengEmail author
  • Abraham M. Kaslow
  • Jay D. Pruetz
  • Jimmy C. Lu
  • John C. Wood
  • Jon A. Detterich
Original Article



Patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS) have wide variation in right ventricle (RV) size, systolic function, and diastolic function at birth. Establishment of antegrade pulmonary blood flow creates the potential for RV dilation from chronic pulmonary insufficiency. Future surgical decisions are based on RV size and function, largely supported by longitudinal studies of patients with Tetralogy of Fallot (TOF). Given potential differences in RV physiology and lack of similar data in PAIVS/CPS, the objective of this study was to determine differences in RV size, systolic function, and diastolic function between patients with PAIVS/CPS versus TOF.


We retrospectively collected cardiovascular magnetic resonance (CMR) data in 27 patients with PAIVS/CPS (ages 13.3 ± 8.8 years) and 78 with TOF (11.4 ± 5.4 years). RV volumes, ejection fraction (EF), regurgitant fraction, end-diastolic forward flow across the pulmonary valve, and right atrial cross-sectional area were calculated.


There was no difference between the groups in RV end-diastolic volume (RVEDVi), RVEF, or pulmonary regurgitation. RVEF tended to decrease in TOF when RVEDVi exceeded 164 ml/m2. In PAIVS/CPS, RVEDVi less frequently reached 164 ml/m2 and was not associated with RVEF. There was worse RV diastolic dysfunction in PAIVS/CPS, with 1.5 times larger right atrial area and two times higher pulmonary end-diastolic forward flow (p < 0.0001).


Patients with PAIVS/CPS have similar RV size, systolic function, and pulmonary regurgitation as TOF. However, impaired RV diastolic function may limit extremes of RV dilatation and impact long-term management of PAIVS/CPS.


Pulmonary atresia Pulmonary stenosis Tetralogy of Fallot Cardiovascular magnetic resonance Echocardiography 



Jon Detterich was supported by a grant from the National Institutes of Health (5 K23 HL119627-03) during the conduct of this study.

Compliance with Ethical Standards

Conflict of interest

All authors declare no conflicts of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

For this type of study formal consent is not required.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Andrew L. Cheng
    • 1
    Email author
  • Abraham M. Kaslow
    • 2
  • Jay D. Pruetz
    • 1
  • Jimmy C. Lu
    • 3
  • John C. Wood
    • 1
    • 2
  • Jon A. Detterich
    • 1
    • 2
  1. 1.Division of Pediatric CardiologyChildren’s Hospital Los AngelesLos AngelesUSA
  2. 2.Keck School of MedicineUniversity of Southern CaliforniaLos AngelesUSA
  3. 3.Division of Pediatric Cardiology, C.S. Mott Children’s HospitalUniversity of MichiganAnn ArborUSA

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