Case Challenge #2

August 17, 2012 9:47 pm / Leave a Comment /

Prepared by:   Juan C Batlle, MD

Solved by:   Jacobo Kirsch, MD

 

18 year old female with history of congenital heart disease status post repair of previous ASD. Currently presents with shortness of breath and desaturation.

Figure 1. Frontal chest radiograph

Figure 2. Relatively normal ventilation nuclear scan.

Figure 3. Perfusion nuclear scan, with no substantial lung uptake.

Figure 4a. Bright blood SSFP axial images.

Figure 4b. Bright blood SSFP axial images.

Figure 5a. Coronal MIP Time-Resolved Angiography.

Figure 5b. Coronal MIP Time-Resolved Angiography.

Figure 6a. Time-resolved MR angiography, early phase. Axial dataset.

Figure 6b. Time-resolved MR angiography, early phase. Axial dataset.

Figure 7a. Time-resolved MR angiography, mid phase. Axial dataset.

Figure 7b. Time-resolved MR angiography, early phase. Axial dataset.

Figure 8a. Time-resolved MR angiography, late phase. Axial dataset.

Figure 8b. Time-resolved MR angiography, late phase. Axial dataset.

Figure 9. Time-resolved MR angiography, last phase. Axial dataset.

This patient has complex great vessel anatomy. She was born with two separate upper pulmonary vein anomalies. On the right, she had an anomalous right upper pulmonary vein (RUPV) that drained to the SVC. As is often the case, she had a sinus venosus ASD in association with the anomalous pulmonary venous return. On the left, she had an anomalous vein that drained from the brachiocephalic vein to the left upper pulmonary vein (LUPV). This so-called levoatrial cardinal vein is a very rare variant. The right-sided issues were corrected in childhood, with baffling of the flow from the RUPV to the left atrium via patch as well as Warden procedure to create an anastomosis between a ligated upper SVC and the right atrial appendage. Thus, the SVC and RUPV no longer communicate, with the RUPV draining oxgenated blood via a baffle into the LA and the SVC dumping deoxygenated blood into the RA via the RAA.
A common complication of the Warden procedure is stenosis of the SVC/RAA connection, sometimes because of inadequate resection of RAA trabeculae at the time of surgery. In our case, the SVC/RAA Warden pathway was nearly completely closed off, with only a trickle of flow at catheter exam and with no appreciable pulmonary flow on V/Q scan (injection of Tc-99m-MAA). It is unclear why the levoatrial cardinal vein was never addressed until this time. At Miami Children’s Hospital, an embolic plug was used to shut off the right to left shunt. Before the intervention, the Qp/Qs was 1 to 1.5. The SVC/RAA narrowing was balloon dilated and stented, restoring drainage.

Warden procedure, operative approach.

Warden Procedure

A companion case is provided by Dr. Jacobo Kirsch.
  • An anomalous vein connecting either the left atrium or the pulmonary vein to a systemic vein was first described by MacIntosh in 1926, and Edwards and DuShane designated this type of malformation as a levoatrial cardinal vein in 1950.
  • Reports of the levoatrial cardinal vein typically described normal pulmonary connections and hypoplasia of the left heart and suggested that the channel resulted from the persistence of primitive connections between the embryonic pulmonary venous primordial and the cardinal vein. Therefore, this abnormal vessel would represent an important route of drainage for pulmonary venous blood in the context of left heart malformation.

Volume rendering demonstrates abnormal venous communication between brachiocephalic vein and LUPV.

Volume rendering demonstrates abnormal venous communication between brachiocephalic vein and LUPV. Zoomed view.

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