Pediatric Cardiology

, Volume 39, Issue 5, pp 967–975 | Cite as

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

  • Renuka E. Peterson
  • Grace Freire
  • Cynthia J. Marino
  • Saadeh B. Jureidini
Original Article


Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity–time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.


Pulmonary atresia with intact ventricular septum Critical pulmonary stenosis Right ventricular-dependent coronary circulation Coronary abnormalities 



The authors wish to thank Paula Buchanan, PhD for her guidance in the statistical analyses of the data and Barb Bequette for assistance with formatting and preparation of the images and tables for the manuscript.

Compliance with Ethical Standards

Conflict of interest

All authors declare that they have no financial or non-financial conflict of interest.

Ethical Approval

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

Informed Consent

Formal consent is not required.


  1. 1.
    Anderson RH, Spicer D (2010) Fistulous communications with the coronary arteries in the setting of hypoplastic ventricles. Cardiol Young 20(Suppl 3):86–91CrossRefPubMedGoogle Scholar
  2. 2.
    Burrows PE, Freedom RM, Benson LN, Moes CAF, Wilson G, Koike K, Williams WG (1990) Coronary angiography of pulmonary atresia, hypoplastic right ventricle and ventriculocoronary communications. AJR Am J Roentgenol 154:789–795CrossRefPubMedGoogle Scholar
  3. 3.
    Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE (1992) Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 86:1516–1528CrossRefPubMedGoogle Scholar
  4. 4.
    Lajos P, Love J, Salim MA, Wang W, Cardarelli MG (2004) Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum. Ann Thorac Surg 77:1088–1090CrossRefGoogle Scholar
  5. 5.
    Powell AJ, Mayer JE, Lang P, Lock JE (2000) Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol 86:1272–1274CrossRefPubMedGoogle Scholar
  6. 6.
    Kawaraguchi Y, Taniguchi A, Otomo T, Ota C, Uchida N (2006) Anesthetic management of bidirectional cavopulmonary shunt in a patient with pulmonary atresia with intact ventricular septum associated with sinusoidal communications. J Anesth 20(3):220–222CrossRefPubMedGoogle Scholar
  7. 7.
    O’Connor WN, Cottrill CM, Johnson GL, Noonan JA, Todd EP (1982) Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance. Circulation 65(4):805–809CrossRefPubMedGoogle Scholar
  8. 8.
    Fyfe DA, Edwards WE, Driscoll DJ (1986) Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 8(2):402–406CrossRefPubMedGoogle Scholar
  9. 9.
    Cheung EW, Richmond ME, Turner ME, Bacha EA, Torres AJ (2014) Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome. Ann Thorac Surg 98:1371–1377CrossRefPubMedGoogle Scholar
  10. 10.
    Sanders SP, Parness IA, Colan SD (1989) Recognition of abnormal connections of coronary arteries with the use of Doppler color flow mapping. J Am Coll Cardiol 13(4):922–926CrossRefPubMedGoogle Scholar
  11. 11.
    Garcia J, Zellers T, Weinstein E, Mahony L (1998) Usefulness of Doppler echocardiography in diagnosing right ventricular coronary arterial communications in patients with pulmonary atresia and intact ventricular septum and comparison with angiography. Am J Cardiol 81:103–104CrossRefPubMedGoogle Scholar
  12. 12.
    Leung M, Mok C, Hui P (1988) Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 12(3):719–725CrossRefPubMedGoogle Scholar
  13. 13.
    Satou GM, Perry SB, Gauvreau K, Geva T (2000) Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol 85:1319–1324CrossRefPubMedGoogle Scholar
  14. 14.
    Jureidini SB, Marino CJ, Waterman B, Rao PS, Balfour IC, Chen Su-chiung et al (1998) Transthoracic Doppler echocardiography of normally originating coronary arteries in children. J Am Soc Echocardiogr 11(5):409–420CrossRefPubMedGoogle Scholar
  15. 15.
    Jureidini SB, Marino CJ, Singh GK, Fiore A, Balfour IC (2000) Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography. J Am Soc Echocardiogr 13(4):255–263CrossRefPubMedGoogle Scholar

Copyright information

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

Authors and Affiliations

  • Renuka E. Peterson
    • 1
  • Grace Freire
    • 1
    • 2
  • Cynthia J. Marino
    • 1
  • Saadeh B. Jureidini
    • 1
  1. 1.Division of Pediatric CardiologySaint Louis University School of Medicine, SSM Health Cardinal Glennon Children’s HospitalSaint LouisUSA
  2. 2.Pediatric and Fetal Cardiology, Johns Hopkins All Children’s Heart InstituteJohns Hopkins All Children’s HospitalSt. PetersburgUSA

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