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European Radiology

, Volume 28, Issue 11, pp 4598–4606 | Cite as

How many versus how much: comprehensive haemodynamic evaluation of partial anomalous pulmonary venous connection by cardiac MRI

  • Neil Seller
  • Shi-Joon Yoo
  • Brian Grant
  • Lars Grosse-Wortmann
Cardiac
  • 90 Downloads

Abstract

Objectives

The objective of this study was to investigate the effect of location and number of anomalously connected pulmonary veins and any associated atrial septal defect (ASD) on the magnitude of left-to-right shunting in patients with partial anomalous pulmonary venous connection (PAPVC), and how that influences right ventricular volume loading.

Methods and results

The cardiac magnetic resonance (CMR) and echocardiography examinations of 26 paediatric patients (mean age, 11.2 ± 5.1 years) with unrepaired PAPVC were analysed. Fourteen patients had right-sided, 11 left-sided and 1 patient bilateral PAPVC. An ASD was present in 11 patients, of which none had a Qp/Qs < 1.5 and 8 had a Qp/Qs≥ 2.0. No patient with isolated left upper PAPVC experienced a Qp/Qs ≥ 2.0 compared to 9/12 patients with right upper PAPVC. Qp/Qs correlated with indexed right ventricle (RV) end-diastolic volume (RVEDVi, r = 0.59, p = 0.002) by CMR and with echocardiographic right ventricular end-diastolic dimension (RVED) z-score (r = 0.68, p = 0.003). A RVEDVi >124 ml/m2 by CMR and a RVED z-score >2.2 by echocardiography identified patients with a Qp/Qs ≥1.5 with good sensitivity and specificity.

Conclusions

An asymptomatic patient with a single anomalously connected left upper pulmonary vein and without an ASD is unlikely to have a significant left-to-right shunt. On the other hand, right-sided PAPVC is frequently associated with a significant left-to-right shunt, especially when an ASD is present.

Key Points

Patients with PAPVC and ASD routinely have a significant left-to-right shunt.

Patients with right PAPVC are likely to have a significant left-to-right shunt.

Patients with left PAPVC are unlikely to have a significant left-to-right shunt.

CMR is helpful in decision-making for patients with PAPVC.

Keywords

Children Pulmonary veins Heart septal defects, atrial Scimitar syndrome Magnetic resonance imaging 

Abbreviations

ASD

Atrial septal defect

CMR

Cardiac magnetic resonance

LPA

Left branch pulmonary arteries

LUPV

Left upper pulmonary vein

PAPVC

Partial anomalous pulmonary venous connection

PC CMR

Phase contrast cardiac magnetic resonance imaging

RMPV

Right middle pulmonary vein

RPA

Right branch pulmonary arteries

RUPV

Right upper pulmonary vein

RV

Right ventricle

RVED

Right ventricular end-diastolic dimension

SVC

Superior vena cava

TPBF

Total pulmonary blood flow

Notes

Funding

The authors state that this work has not received any funding.

Compliance with ethical standards

Guarantor

The scientific guarantor of this publication is Dr. Shi-Joon Yoo.

Conflict of interest

The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Statistics and biometry

One of the authors has significant statistical expertise.

No complex statistical methods were necessary for this paper.

Ethical approval

Institutional Review Board approval was obtained.

Informed consent

Written informed consent was waived by the Institutional Review Board.

Methodology

• retrospective

• cross-sectional study, diagnostic or prognostic study, observational

• performed at one institution

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Copyright information

© European Society of Radiology 2018

Authors and Affiliations

  1. 1.The Labatt Family Heart Centre, Department of PaediatricsThe Hospital for Sick Children, University of TorontoTorontoCanada
  2. 2.Department of Diagnostic ImagingThe Hospital for Sick Children, University of TorontoTorontoCanada

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