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Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography


Review

. 2022 Oct 10;87:e549-e556.


doi: 10.5114/pjr.2022.120513.


eCollection 2022.

Affiliations

Item in Clipboard

Review

Amit Kumar Verma et al.


Pol J Radiol.


.

Abstract

Pulmonary venous anomalies occur as a result of failure in normal embryological development. These anomalies may present as a spectrum ranging from normal variation to partial anomalous pulmonary venous connection (PAPVC) and total anomalous pulmonary venous connection (TAPVC). Though not rare, PAPVC is an uncommon anomaly in which some of the pulmonary veins abnormally connect and drain into the vascular compartments other than the left atrium (LA); however, the others drain normally into the LA. The clinical presentation and severity of affected patients depend on the morphological heterogeneity of the disease. PAPVC associated with other complex conge-nital cardiac diseases present early and are more severe than isolated PAPVC-associated atrial septal defect only. This radiological review gives a detailed description of PAPVC in terms of morphological variability and associated anomalies along with a discussion of the role of multidetector dual-source computed tomography scan in the diagnostic assessment.


Keywords:

anomalous pulmonary venous drainage; atrial septal defect; congenital heart disease; multidetector CT angiogram.

Conflict of interest statement

The authors report no conflict of interest.

Figures



Figure 1

VR images in anterior and posterior view of a patient of right superior pulmonary vein partial anomalous pulmonary venous connection (PAPVC). The images show drainage of right superior pulmonary vein (PV) draining into lower part of superior vena cava (SVC). There is also a single accessary right middle pulmonary vein (*) draining near the cavoatrial confluence. The rest of the pulmonary veins are draining normally into the left atrium


Figure 2


Figure 2

A-B) VR images with clip plane in anterior and posterior view; (C) axial MIP image in a patient of right superior pulmonary vein partial anomalous pulmonary venous connection (PAPVC). The right superior pulmonary vein (PV) drains into superior vena cava (SVC) near the cavoatrial junction. The patient also has a superior sinus venosus atrial septal defect (double headed arrow)


Figure 3


Figure 3

A-C) Thin MIP axial and coronal and (D) VR images from a case of right partial anomalous pulmonary venous connection (PAPVC). The images show a sufficiently sized superior sinus venosus atrial septal defect (ASD). There is right-sided PAPVC with right superior pulmonary vein (PV) draining into the lower part of the superior vena cava (SVC) and inferior pulmonary veins draining at the cavoatrial junction. The left PV is draining normally into the left atrium (LA)


Figure 4


Figure 4

Thin MIP axial and coronal images from a case of right-sided cardiac partial anomalous pulmonary venous connection (PAPVC). The right superior and inferior pulmonary veins (PV) are draining into the right atrium (RA). The left PV have normal connection to the left atrium (LA). An atrial septal defect (*) is also present in superoposterior part with dilated right heart chambers (RA, RV)


Figure 5


Figure 5

A-C) Thin MIP (axial coronal) and (D, E) images from a case of cardiac partial anomalous pulmonary venous connection (PAPVC). The images show that the right-sided superior and superior pulmonary veins (PV) have a cardiac connection into the right atrium (RA). The left PV are draining normally into the left atrium (LA). A superior sinus venosus atrial septal defect (*) is present with dilated right heart chambers (RA, RV). The VR image compliments the same findings


Figure 6


Figure 6

A-C) VR images in different orientation; (D) axial thin MIP image of a patient with mixed type partial anomalous pulmonary venous connection (PAPVC). The right superior pulmonary vein (PV) is draining at the superior cavoatrial junction (CAJ) near the sinus venosus atrial septal defect (ASD) (double headed arrow). The left superior PV is draining into a left brachiocephalic vein (BCV) through an ascending vertical vein (VV). The right and left inferior PV are normally draining into the left atrium. There are also 2 accessary right middle pulmonary veins (*) draining into the LA


Figure 7


Figure 7

VR images with clip plane in different orientations. The images show partial anomalous pulmonary venous connection (PAPVC) of the right superior pulmonary vein (PV) draining into superior vena cava (SVC). There is a patent ductus arteriosus as shunt (*) [AO – aorta; PA – pulmonary artery]


Figure 8


Figure 8

These are images from a case of partial anomalous pulmonary venous connection (PAPVC) associated with heterotaxy syndrome (left isomerism) and pulmonary artery hypertension. A-C) VR images of the heart in posterior orientation and with clip plane showing right-sided PAPVC with a cardiac connection into the right atrium (RA). The left pulmonary veins (PV) are draining normally into the left atrium (LA). A small atrial septal defect is present (double head arrow) with dilated RA and RV. D) VR image showing a dilated pulmonary artery (PA) representing pulmonary artery hypertension. E, F) MnIP coronal and thin MIP coronal images show left bronchial pattern (B) and polysplenia (S)

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