-2 :: Case - 12706 :: - Partial Anomalous Pulmonary Venous Return
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Tx and Followup:
The patient’s TEE and cardiac catheterization confirmed sinus venous atrial septal defect with bidirectional flow (predominantly right-to-left) suggesting Eisenmenger’s physiology. The right heart was moderately enlarged with right ventricular hypertrophy and pulmonary arterial hypertension (pulmonary arterial pressures were 60/25 mmHg). The patient underwent bovine pericardial baffle patch repair of the sinus venosus defect with re-implantation of the anomalous right pulmonary veins into the left atrium. At surgery, the interatrial defect measured almost 3cm. The right superior pulmonary vein drained anomalously into the posterior aspect of the superior cavo-atrial junction, and the right middle lobe segmental veins drained separately into the right atrium. The right inferior pulmonary vein and left pulmonary veins drained normally into the left atrium. The patient tolerated his surgery well and recovered uneventfully.

Additional Discussion:
This case demonstrates the classic findings of sinus venosus atrial septal defect (ASD), a rare congenital deficiency of the superior inter-atrial septum typically associated with partially anomalous right pulmonary venous return (PAPVR) to the superior vena cava and right atrium. The sinus venosus defect typically results in a left-to-right shunt at the level of the atria. The presence of PAPVR compounds the problem with additional left-to-right shunt pathways.

Most adult cases of ASD, including sinus venosus defects, remain unrecognized until the fourth to fifth decades of life when pulmonary arterial hypertension and right-to-left shunting develops. Consequently, patients with ASD are at risk for developing atrial dysrhythmias with an inherent risk of thromboembolic complications. Sinus venosus defect may carry a worse prognosis than other forms of ASD and may need to be treated at a younger age, via medical therapy and/or surgical closure. Surgical repair of ASD, including sinus venosus defect, in patients over 40 years of age, increases long-term survival and decreases the incidence of heart failure.

References
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1. Van Praagh S et al. Sinus venosus defects: unroofing of the right pulmonary veins – anatomic and echocardiographic findings and surgical treatment. Am Heart Journal 1994; 128: 365-79.
2. Miller SW. Cardiac Imaging: the Requisites, 2nd ed. Elsevier Mosby 2005: 36-38; 324-325.
3. Blom NA et. al. Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects. Am J Cardiology 2001; 87:305-09.
4. Sadley TW. Langman’s Medical Embryology, 7th ed. Williams and Wilkins 1995: 191-201.
5. Al Zaghal AM et. al. Anatomic criteria for the diagnosis of sinus venosus defects. Heart 1997; 78: 298-304.
6. Vogel M, Berger F, Kramer A, et. al. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart 1999; 82: 30-33.
7. Agrawal SK, Khanna SK, Tampe D. Sinus venosis atrial septal defects: Surgical follow-up. European J Cardiothoracic Surg 1997; 11(3): 455-7.
8. Konstantinides S et. al. A comparison of surgical and medical therapy for atrial septal defects in adults. NEJM 1995; 333(8): 469-73

Discussion Author: Matthew J Hoffman

Partial Anomalous Pulmonary Venous Return (left upper lobe pulmonary vein)

Partial anomalous venous return occurs when a pulmonary lobe drains into the right side of the circulation, usually a systemic vein. Anomalous venous drainage of the left upper lobe is usually an isolated congenital abnormality that has no clinical significance. Anomalous drainage of the right pulmonary vein to the inferior vena cava (scimitar syndrome) can present as a left to right shunt in patients with volume loss of the right hemithorax and a peculiar comma shaped density in the right lung base. Anomalous drainage of the right upper lobe is associated with a sinus venosus type of atrial septal defect. Left lower lobe pulmonary systemic drainage is associated with extralobar sequestration.   
   When the left upper lobe vein drains into the left brachiocephalic vein, a vertical vein is seen coursing lateral to the aortic arch and the AP window. This appearance must be distinguished from a persistent left superior vena cava. The distinction comes from following the entire course of the vein to its drainage, and if you see two vessels in the left hilum or just one. One is consistent with PAPVR while two indicates persistent left SVC (one vessel is svc other is normal left upper lobe vein.
   A persistent left SVC occurs in about 0.3% of the normal population with a substantially higher incidence in patients with congenital heart disease. This anomaly results from embryologic failure of regression of part of the left common and anterior cardinal veins. It at least drains the left jugular and subclavian veins. On CT a persistent left SVC is positioned immediately lateral to the left common carotid artery and anterior to the left subclavian artery. The anomalous vessel descends lateral to the aortic arch, in approximately the same coronal plane as the normal right SVC. It passes lateral to the main pulmonary artery and anterior to the left hilum. It typically drains into a dilated coronary sinus posterior to the left ventricle.
   

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