Background-Aortopulmonary collaterals (APCs) have been associated with increased morbidity after the Fontan operation. We aimed to quantify APC flow after bidirectional cavopulmonary connections and Fontan completions, using phase-contrast MRI, and to identify risk factors for the development of APCs. Methods and Results-APC blood flow was quantifiable in 24 of 36 retrospectively analyzed MRI studies. Sixteen studies were performed after the bidirectional cavopulmonary connections (group A) and 8 after the Fontan operation (group B). APC blood flow was calculated by subtracting the blood flow volume through the pulmonary arteries from that through the pulmonary veins. The ratio of pulmonary to systemic blood flow (Qp/Qs) was 0.

Pulmonary vein pathologies often present a diagnostic challenge. Among the different imaging modalities used for the evaluation of pulmonary veins, magnetic resonance is the most comprehensive in assessing anatomy and pathophysiology at the same time. Bright blood cine sequences and contrast-enhanced magnetic resonance angiography outline the course and connections of the pulmonary veins. Phase-contrast velocity mapping measures flow patterns, velocities, and volumes throughout the pulmonary circulation. This paper reviews contemporary utilization of magnetic resonance in the evaluation of pulmonary venous abnormalities in children, based on our experience over the last 5 years and on that of other investigators. We summarize how magnetic resonance imaging enhances our understanding of pulmonary vein physiology and how it can influence the diagnostic approach to children and adults with a pulmonary venous pathology, and we discuss its limitations.

Purpose:To assess the feasibility of phase-contrast magnetic resonance (PCMR) in quantifying the pulmonary venous return in normal subjects. Materials and Methods:PCMR was performed in 12 healthy adult volunteers (mean age 38 years, range 27-60 years; 9 men; body surface area 1.81 Ϯ 0.15 m 2 ) for the ascending and descending aorta, caval veins, main and branch pulmonary arteries, and pulmonary veins. Two readers independently quantified blood flow in all subjects.Results: Intraobserver differences were Ϫ2.0% (95% confidence interval [CI]: Ϫ9.9% to 5.9%), Ϫ4.5% (95% CI: Ϫ15.6% to 6.5%), and Ϫ0.7% (95% CI: Ϫ4.5% to 3.0%) for all vessels, pulmonary veins, and other great vessels, respectively. Interobserver differences were Ϫ2.0% (95% CI: Ϫ10.6% to 6.6%), Ϫ3.1% (95% CI: Ϫ16.0% to 9.9%), and Ϫ1.4% (95% CI: Ϫ6.4% to 3.5%) for all vessels, pulmonary veins, and other great vessels, respectively. Pulmonary venous flow volume showed high correlations with the volumes of the pulmonary arterial flow, systemic arterial flow, and systemic venous flow (r ϭ 0.76 -0.92, P Ͻ 0.005). Conclusion:Flow quantification of normal pulmonary venous return using PCMR is feasible with high reproducibility and accuracy. FLOW QUANTIFICATION using the phase-contrast (or velocity-encoded) technique is a central part of comprehensive cardiac magnetic resonance (MR) imaging for congenital heart disease in children and adults. Phasecontrast MR imaging (PCMR) has been validated as accurate in evaluating velocity, volume, and pattern of pulsatile blood flow (1-5). Flow measurements using PCMR have been used at various great vessels, including the aorta, the pulmonary artery, and the superior and inferior vena cavae, in various clinical settings (6 -10). In contrast, feasibility of flow quantification of the pulmonary veins using PCMR has not been evaluated. As in systemic circulation, information on pulmonary venous flow in addition to pulmonary arterial flow is crucial for the full understanding of pulmonary circulation.Measurement of pulmonary venous flow is important in the following clinical scenarios: direct measurement of systemic collateral arterial flow to the lung; shunting involving the pulmonary vein; and alternative measurement of pulmonary arterial flow when it is not available for any reason. Attention with regard to evaluation of pulmonary vein abnormalities after radiofrequency ablation has also increased recently. However, it is very difficult to provide accurate quantification of pulmonary venous flow when using echocardiography and the catheter technique. This fact motivated our study because noninvasive PCMR has great potential in offering such pulmonary venous flow data. A few clinical studies have demonstrated the usefulness of quantification of pulmonary venous flow volume with PCMR in patients with anomalous pulmonary venous connection, stenotic pulmonary artery with or without a stent, and systemic collateral arteries supplying the lungs (11-13). The amount of systemic collateral arterial supply to the lungs can be calcula...

We report a case in which a meandering right pulmonary vein connecting to the left atrium is associated with hypoplasia of the right lung, horseshoe lung, abnormal pulmonary lobation, and abnormal branching of the pulmonary arteries. We discuss its relationship to the so-called scimitar variant, and to the scimitar syndrome itself.

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