Fetal Doppler Echocardiographic Assessment Predicts Severe Postnatal Obstruction in Total Anomalous Pulmonary Venous Connection.

BACKGROUND: Obstructed total anomalous pulmonary venous connection (TAPVC) is a form of critical congenital heart disease that usually requires urgent postnatal intervention. Knowing which patients have severe obstruction can aid delivery planning. The authors previously developed a novel quantitative metric of pulmonary venous flow, the pulmonary venous variability index (PVVI). The aim of this study was to test the hypothesis that fetal PVVI and vertical vein Doppler velocities are associated with severe pulmonary vein obstruction postnatally. METHODS: A retrospective cohort study of neonates with prenatally diagnosed TAPVC was performed. Patients who underwent fetal echocardiography at the Children's Hospital of Philadelphia with Doppler interrogation of the vertical vein were included for analysis. Twenty-nine patients met criteria (21 with heterotaxy, 18 with supracardiac TAPVC). The latest gestation fetal echocardiogram was used. Severe pulmonary vein obstruction was defined as preoperative death or urgent surgery or catheter-based intervention (first day of life). Measurements of PVVI, defined as (maximum velocity - minimum velocity)/mean velocity, were made offline. Wilcoxon rank sum models were used to assess the associations of severe obstruction and PVVI and maximum, mean, and minimum velocities. RESULTS: The mean gestational age at the latest fetal echocardiographic examination was 35 weeks (range, 30-39 weeks). Twelve of the 29 patients (41%) met criteria for severe pulmonary vein obstruction. Lower PVVI was associated with greater risk for severe pulmonary venous obstruction (P = .008). The maximum, mean, and minimum velocities in the vertical vein were all significantly associated with severe pulmonary venous obstruction (P = .03, P = .03, and P = .007, respectively). Qualitative assessment of obstruction was not significantly associated with the outcome. Interobserver reliability for all vertical vein Doppler metrics was high (intraclass correlation coefficient > 0.9). CONCLUSIONS: Fetal PVVI and maximum, mean, and minimum velocities are associated with severe postnatal pulmonary vein obstruction in TAPVC. Accurate prediction of obstructed TAPVC could allow safer delivery planning. Further research with larger sample sizes is needed to identify the ideal cutoff values for these Doppler measures.

[Morphologic and functional assessment of vascular abnormalities of the pulmonary vasculature by breath-hold MR techniques]. / Morphologische und funktionelle Evaluation pulmonaler Gefässanomalien mittels schneller MR-Techniken.

PURPOSE: To evaluate breath-hold MR techniques for morphologic and functional assessment of vascular abnormalities of the pulmonary vasculature. PATIENTS AND METHODS: 13 patients aged 11 to 60 years with different vascular abnormalities of the pulmonary vasculature (5 patients with 16 arteriovenous malformations, 8 patients with partial anomalous pulmonary venous return) underwent MR imaging at 1.5 T. For morphological assessment, a contrast-enhanced 3D MR angiography (ce-MRA) was performed after a timing run. Segmented cine- and velocity-encoded GRE sequences were used for delineation of associated cardiac septal defects and for determination of systemic left-to-right or intrapulmonary shunt volumes. Selective intra arterial digital subtraction angiography, cardiac catheterization, and the intraoperative situs served as reference standards. RESULTS: Ce-MRA allowed for detection of all vascular abnormalities and for anatomic characterization of 14/16 arteriovenous malformations. Flow measurements in the feeding arteries allowed for determination of intrapulmonary shunt volumes in 4/5 patients. Flow measurements performed in the pulmonary arteries and the ascending aorta enabled determination of systemic left-to-right shunting in patients with anomalous pulmonary venous return. Cine-sequences clearly depicted associated cardiac septal defects. CONCLUSION: Breath-hold MR techniques allow for morphological and functional characterization of vascular anomalies of the pulmonary vasculature. Therefore, they are the non-invasive method of choice for planning further treatment.

Scimitar syndrome: MR assessment of hemodynamic significance.

We report a case of anomalous pulmonary venous drainage into the inferior vena cava (scimitar syndrome) in which the hemodynamic significance was noninvasively assessed by means of velocity-encoded cine MR. Left-to-right shunt was calculated from direct blood flow measurements performed in the ascending aorta, main pulmonary artery, and aberrant pulmonary vein.