Bilateral Partial Anomalous Pulmonary Venous Connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare and frequently under diagnosed entity in which one or more pulmonary veins are connected to the systemic venous return. Bilateral Partial anomalous pulmonary venous return is extremely unusual.

The authors present the case of a 27-year-old woman with a history of childhood asthma. She was symptomatic with progressive CFII-III dyspnea. Complementary studies performed showed an EKG with signs of right ventricular overload and chest radiography with moderate cardiomegaly and increased volume of the right atrium. Doppler echocardiography revealed dilatation of the right cavities and severely dilated RV, with no evidence of intracavitary shunts, indirect signs of moderate systolic pulmonary arterial hypertension, and good biventricular function. 

Angiography was used to diagnose the abnormal connection of the right superior pulmonary vein to the superior vena cava and the left superior pulmonary vein to the innominate vein. The authers diagnosed  bilateral partial anomalus pulmonary venous connection, and repair surgery was performed. The patient had an uneventful hospital course and was discharged on the fifth postoperative day, with good progress and an early return to normal activities. Follow-up CT scan showed good reconstruction of the pulmonary drainage.

The surgical indication in the PAPVC is controversial. The authors agree that if there is abnormal drainage of more than one vein, the hemodynamic consequences will be significant. In these cases, surgical repair is indicated. Each pulmonary vein contributes between 20-25% of venous flow, so the existence of more than one vein with an abnormal connection would mean a commitment greater than 50%. In the presented patient, two combined techniques were performed for the anatomical resolution of the anomaly, with a favorable result.

In conclusion, the case presented is a patient with bilateral anomalous partial drainage, a considerably particular entity, even more so in adult patients. The authors believe that intensive evaluation and prior planning of therapeutics is the fundamental basis for the resolution of highly unusual cases, in order to reduce morbidity and optimize long-term results.

References

1. ElBardissi AW, Dearani JA, Suri RM, Danielson GK. Left- sided partial anomalous pulmonary venous connections. Ann Thorac Surg. 2008;85:1007–1014.
2. Blake HA, Hall RJ, Manion WC. Anomalous pulmonary venous return. Circulation. 1965;32:406–414.
3. Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation. 1968;38:45–63.
4. Said SM, Burkhai HM, Schaff HV, Cetta, F Jr, Phillips SD, Barnes RD, et al. Single Patch-2 patch and Caval division techniques for repair of partial anomalous pulmonary venous connections: does it matter? J Thorac Cardiovasc Surg. 2012;143:896-903.
5. Wang T-L, Hung H-F, Lin C-C, Hsiung M-C, Wei J. Multiple anomalous left pulmonary venous connections detected with transthoracic echocardiography. J Cardiothorac Surg. 2013;8:130.
6. Majdalany,DS, Phillips, SD, Dearani, JA, Connolly, HM, Warnes CA. Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010;5:537–545.

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