Anomalous Pulmonary Venous Return: Pearls and Pitfalls in multimodal imaging.

Congress:

ECR 2022

Poster Number:

C-10696

Type:

Educational Exhibit

Keywords:

Cardiac, Cardiovascular system, Paediatric, CT-Angiography, Echocardiography, MR-Angiography, Diagnostic procedure, Congenital

Authors:

F. A. Blanco, M. A. Cruz Marmolejo, D. S. Gonzalez Ontiveros, R. Pohls Vazquez, Z. Rios Garcia, K. Aguilar Zavala, I. C. Maldonado Jaimes, R. Cano, R. De La Mora Cervantes

DOI:

10.26044/ecr2022/C-10696

DOI-Link:

https://dx.doi.org/10.26044/ecr2022/C-10696

Learning objectives

1. Review the epidemiology, embryology, and classification of anomalous pulmonary venous connection. 2. Examine most common imaging findings in echocardiography, computed tomography and magnetic resonance of different types of anomalous venous connection.

Background

Abnormal embryonic pulmonary vein development may result in a wide spectrum of congenital anomalies [1], they are frequent in patients with congenital heart disease (CHD). The spectrum of findings can range from incidental findings to conditions that are lethal if left untreated. Accurate definition of the nature and extent of these abnormalities is critical for treatment decision making [2]. Abnormal pulmonary venous return was first described by Winslow in 1739 [3]. There’s a broad spectrum of pulmonary venous connections that deviate from the usual anatomical...

Findings and procedure details

Abnormalities of the pulmonary veins are classified as: 1- anomalous pulmonary venous drainage, total anomalous pulmonary venous return (TAPVR) and partial anomalous pulmonary venous return (PAPVR); 2- Stenosis, atresia, cor triatriatum, secondary stenosis; 3- Other or abnormal number of pulmonary veins [2]. TAPVR consists in all four pulmonary veins draining into systemic veins of the right atrium with or without pulmonary venous obstruction. Systemic and pulmonary venous blood mix in the right atrium. TAPVR is generally classified into 4 anatomical subtypes: supracardiac, cardiac, infracardiac, or...

Conclusion

Anomalous venous drainage of the pulmonary veins is an infrequent condition, which can be detected in pediatric patients and treated appropriately, or it can be diagnosed in adult life in patients with symptoms suggestive of pulmonary hypertension. Echocardiography, CT and MRI imaging allow adecuate delineation of pulmonary venous return.

Personal information and conflict of interest

F. A. Blanco: Nothing to disclose M. A. Cruz Marmolejo: Nothing to disclose D. S. Gonzalez Ontiveros: Nothing to disclose R. Pohls Vazquez: Nothing to disclose Z. Rios Garcia: Nothing to disclose K. Aguilar Zavala: Nothing to disclose I. C. Maldonado Jaimes: Nothing to disclose R. Cano: Nothing to disclose R. De La Mora Cervantes: Nothing to disclose

References

Dillman J. R., Yarram S. G., Hernandez R., J. Imaging of Pulmonary Venous Developmental Anomalies. AJR 2009; 192:1272–1285. Vyas H. V., Greenberg S. B., Krishnamurthy R. MR Imaging and CT Evaluation of Congenital Pulmonary Vein Abnormalities in Neonates and Infants. RadioGraphics 2012; 32:87-98. El-Kersha K., Homsyb E., Daniels C. J., Smith J. S., Partial anomalous pulmonary venous return: A case series with management approach. Respiratory Medicine Case Reports 2019; 27:100833. https://doi.org/10.1016/j.rmcr.2019.100833. Lyen S., et al. Anomalous pulmonary venous drainage: a pictorial essay with a CT...

Fig 1: Fig. 1. Most common pulmonary drainage pattern. Diagram shows four separate...

Fig 2: Fig.2. Diagram demonstrating the normal anatomic variants of the pulmonary...

$Fig 3: Fig. 3: Type III TAPVR Infracardiac. Coronal contrast-enhanced CTA MPR reconstructions (a) coronal MIP (b) VRT demonstrating TAPVR. Pulmonary veins (*) drain into a vertical confluence (black arrows) which itself drains to the ductus venosus (yellow arrow). ©Cardiovascular Imaging Department, Instituto Nacional de Cardiologia "Ignacio Chavez"/ Mexico 2022.$

Fig 3: Fig. 3: Type III TAPVR Infracardiac. Coronal contrast-enhanced CTA MPR...

$Fig 4: Fig. 4: Type III Infracardiac. Contrast-enhanced volume-rendered CT images in anterior (c) and posterior (d) views demonstrating four pulmonary veins join behind the left atrium to a common vertical descending vein (black asterisk) which in turn drain into the ductus venosus with a distal site of stenosis. RSPV (right superior pulmonary vein), RIPV (right inferior pulmonary vein), LSPV (left superior pulmonary vein), LIPV (left inferior pulmonary vein), CVDV (common vertical descending vein). ©Cardiovascular Imaging Department, Instituto Nacional de Cardiologia "Ignacio Chavez"/ Mexico 2022.$

Fig 4: Fig. 4: Type III Infracardiac. Contrast-enhanced volume-rendered CT images in...

Fig 5: Fig. 5: Scimitar syndrome is a combination of pulmonary hypoplasia and partial...

Fig 6: Fig. 6: Partial anomalous pulmonary vein return (PAPVR). Axial...

Fig 7: Fig. 7: Partial anomalous pulmonary vein return (PAPVR) with superior sinus...

Fig 8: Fig. 8: Partial anomalous pulmonary vein return (PAPVR). Axial (a), sagittal...

Fig 9: Fig. 9: A classic sign is the "snowman-shaped heart” in supra-cardiac form,...

$Fig 10: Echocardiographic examination of the pulmonary veins. [A-D] Transesophageal echocardiography of normal pulmonary veins. (A) Apical bicommissural 65º with visualization of the left atrial appendage (^), coumadin ridge (yellow arrow) and left superior pulmonary vein (*) assessed in 2D and color Doppler. (B) Middle esophageal at 117º with counterclockwise rotation with visualization of both left pulmonary veins (blue arrow). (C) Middle esophagus at 117º with clockwise rotation with visualization of both right pulmonary veins (green arrow). (D) Pulsed Doppler in the left superior pulmonary vein with reverse systolic flow due to severe mitral regurgitation. [E-H] Transthoracic echocardiographic approximations of pulmonary veins with obstruction in the connection. (E) Modified 4-chamber apical with 4 dilated pulmonary veins and an image suggestive of a membrane at the level of the connection site with the left atrium (*) that conditions flow acceleration (aliasing) by color Doppler visualized in 4 and 2 chambers (F and G ), and corroborated by pulsed Doppler (H), with continuous venous flow reaching a peak gradient of 45 mmHg. [I-L] Transthoracic echocardiographic approximations of TAPVR of the supracardiac variety. (I) Apical 4-chamber 2D with ostium secundum atrial septal defect. (J) Modified 4-chamber apical color Doppler image of the superior vena cava connected to the right atrium and pulsed Doppler assessment (K) showing continuous venous flow with a peak gradient of 21 mmHg. (L) 2D suprasternal approximation and color with dilated vertical vein (*) 30mm and its connection with the innominate vein (^). [M-N] Transthoracic echocardiographic approximations of TAPVR intracardiac variety. (M) Apical 4-chamber view modified posteriorly, visualizing drainage from the coronary sinus (*) to the right atrium, with pulmonary veins connected to it (arrow). (N) Modified parasternal approximation of the inflow tract of the right ventricle, with color Doppler on the connection of the pulmonary veins to the coronary sinus and aliased flow. [O-P] Transthoracic echocardiographic approximations of PAPVR, infracardiac variety. (O) Subcostal approximation with color Doppler, visualizing the right pulmonary vein connected to the inferior vena cava (^) with spectral flow by pulsed Doppler (P). © Echocardiography Department, Instituto Nacional de Cardiologia "Ignacio Chavez"/ Mexico 2022.$

Fig 10: Echocardiographic examination of the pulmonary veins. [A-D] Transesophageal...