J Thorac Cardiovasc Surg 2006;132:1237-1238
© 2006 The American Association for Thoracic Surgery
Surgical repair of anomalous pulmonary venous connection shunting from left atrium to superior vena cava
Masamichi Ono, MDa,*,
Dietmar Boethig, MDb,
Heidi Goerler, MDa,
Thomas Breymann, MDa
a Division of Thoracic and Cardiovascular Surgery, Hannover Medical School, Hannover, Germany
b Division of Pediatric Cardiology and Intensive Medicine, Hannover Medical School, Hannover, Germany.
Received for publication July 29, 2006; accepted for publication August 7, 2006.
* Address for reprints: Dr. Masamichi Ono, MD, PhD, Division of Thoracic and Cardiovascular Surgery, Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany. (Email: Ono.Masamichi{at}MH-Hannover.de).
Partial anomalous pulmonary venous connection (PAPVC) causes a left-to-right shunt from the anomalous pulmonary vein (PV) to a systemic vein. We herein report an extremely uncommon case of PAPVC in which the right upper PV drained into both the superior vena cava (SVC) and the left atrium (LA), demonstrating retrograde shunting from the LA to the SVC via the anomalous PV. The anomaly was surgically repaired.
Clinical Summary
A 5-year-old boy with a systolic heart murmur at routine pediatric checkups was admitted to our hospital. Echocardiogram showed a volume-loaded right ventricle and right atrium, a patent foramen ovale, and patent ductus arteriosus. Color Doppler echocardiogram demonstrated retrograde blood flow in the right upper PV. To clarify the exact anatomy, a cardiac catheterization was performed, which revealed a PAPVC from the right upper lobe into the SVC. Angiograms projected in the LA demonstrated retrograde blood flow from LA to SVC through the right upper PV (Figure 1). Moderate left-to-right shunt (pulmonary/systemic flow ratio 2.4) was due to the shunt from the LA to the SVC. Interventional closure of the patent ductus arteriosus was done.
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Figure 1. A, Angiogram of LA injection showed retrograde blood flow from LA to SVC via the RUPV (white triangles). B, Diagram of the anatomy of venous return. LA, Left atrium; RUPV, right upper pulmonary vein; SVC, superior vena cava.
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The elective operation was performed at the age of 6 years. After right lateral thoracotomy and longitudinal pericardectomy, we encountered a Y-shaped right upper PV draining into both the SVC and the LA (Figure 2,
A). The anomalous PV was dissected and divided (Figure 2, B).
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Figure 2. Intraoperative drawings. A, There are Y-shaped RUPV connections both to SVC and to LA. B, Anomalous RUPV connected to SVC was dissected and divided. RA, Right atrium; RUPV, right upper pulmonary vein; SVC, superior vena cava; RPA, right pulmonary artery. *Anomalous pulmonary vein connected to SVC.
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Discussion
PAPVC of the right upper PV is most commonly attached to the low SVC or the SVC–right atrial junction.1-3
In this case, the right upper PV connects both to the SVC and to the LA, showing a moderate left-to-right shunt through retrograde flow from the LA.4 To the best of our knowledge, there has been no prior report of surgical repair for such cases. Accurate diagnosis and hemodynamic assessments by cardiac catheterization were necessary before surgical repair. Surgical repair was successfully done by division of the anomalous PV without cardiopulmonary bypass. Our case showed the possibility of coexistence of normal and anomalous PVs in the common situation of PAPVC of the right upper PV. Surgeons must keep in mind this rare anomaly and its repair.
Conclusion
We reported surgical repair of PAPVC of the right upper PV, which connected both to the SVC and to the LA, showing retrograde blood flow from the LA to the SVC.
References
- Bahnson HT, Spencer FC, Neill CA. Surgical treatment of thirty-five cases of drainage of pulmonary veins to the right side of the heart. J Thorac Surg 1958;36:777-779.[Medline]
- Lewis FJ. High defects of the atrium. J Thorac Cardiovasc Surg 1958;36:1-5.[Medline]
- Swan HJ, Kirklin JW, Becu LM, Wood EH. Anomalous connection of right pulmonary veins to superior vena cava with intra-atrial communications: hemodynamic data in eight cases. Circulation 1957;16:54-66.[Medline]
- Beck C, Wessel A, Yelbuz TM, Bertram H. Unusual case of anomalous pulmonary venous return with left atrial to systemic venous shunt. Circulation 2006;113:e840-e841.[Free Full Text]
