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J Thorac Cardiovasc Surg 2006;132:704-705
© 2006 The American Association for Thoracic Surgery

Brief Communication

Extrapleural pneumonectomy for scimitar syndrome

David P. Mason, MD*, Tomislav Mihaljevic, MD, Peter J. Mazzone, MD, MPH, Sudish C. Murthy, MD, PhD, Thomas W. Rice, MD

Cleveland Clinic Foundation, Cleveland, Ohio.

Received for publication April 21, 2006; accepted for publication May 17, 2006.

* Address for reprints: David P. Mason, MD, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195 (Email: masond2{at}ccf.org).

Scimitar syndrome is a rare congenital anomaly characterized by partial or total anomalous pulmonary venous drainage of the right lung into the inferior vena cava. The description "scimitar" comes from the classic appearance resembling the Turkish sword of a crescent vascular density at the lower right heart border on the chest radiograph.1Go Scimitar syndrome may appear as early as infancy but has been discovered as late as the eighth decade of life. Treatment options are close observation, repair with an intra-atrial baffle, direct reimplantation of the anomalous drainage to the left atrium, or pneumonectomy.2,3Go This case presentation concerns a woman who had repair of scimitar syndrome at age 3 and who at age 34 years required right extrapleural pneumonectomy after multiple pulmonary infections. She may be the oldest patient documented in whom pneumonectomy was performed for scimitar syndrome and the only one in whom extrapleural pneumonectomy was carried out.

Clinical Summary

Our patient initially was brought for repair of scimitar syndrome at 3 years of age. The anomalous common right pulmonary vein drained into the inferior vena cava immediately above the diaphragm with no other anomalous blood. An intra-atrial diversion of the anomalous venous drainage to the left atrium was performed along with an atrial septectomy. For 10 years she did well, but a quantitative perfusion scan performed at this point revealed no perfusion to the right lung. She was presumed to have venous occlusion.

When the patient was first seen in our facility, she was having increasingly frequent respiratory infections with at least a dozen right-sided pneumonias necessitating hospitalization. A quantitative perfusion scan confirmed no perfusion to the right lung. A chest radiograph showed a mildly hypoplastic lung. A magnetic resonance angiogram demonstrated an atretic pulmonary artery with drainage to the left atrium via small collateral veins (Figure 1). Echocardiogram showed no evidence of a septal defect. Cardiac catheterization confirmed normal pulmonary artery pressures. Pulmonary function tests demonstrated a forced expiratory volume in 1 second of 1.52 L (49% of predicted normal); lung volumes showed moderate restriction and air trapping with a total lung capacity of 67% of predicted normal with air trapping.


Figure 1
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  		Figure 1. A, Chest radiograph showing hypoplastic right lung. B, Magnetic resonance angiogram with atretic right pulmonary artery.

 
Right pneumonectomy was recommended and accepted. A right anterolateral thoracotomy was performed through the fifth intercostal space. Complete pleural symphysis was immediately evident. An extrapleural dissection was carried out, working laterally to medially. The lung was dissected off the diaphragm in an extrapleural plane and the pericardium was preserved medially. The pulmonary artery was patent, approximately 1 cm in size, and was divided with a vascular stapler. Only small collateral vessels were identified and ligated in the region of the pulmonary vein. The airway was divided and covered with a pericardial fat pad. The patient did well and was discharged home on the fifth postoperative day. At early follow-up she is doing well with no recurrent respiratory symptoms.

Discussion

Scimitar syndrome typically appears early in life with symptoms of heart failure or respiratory distress. Symptoms at this stage are generally severe and must be addressed immediately. Repair focuses on returning the pulmonary drainage to the left side of the heart, although the repair may fail either early or over time.3Go Pneumonectomy is carried out in the setting of lung agenesis or failed repair.4Go Presentation is variable in adulthood and ranges from an incidental finding to severe symptoms—typically, recurrent respiratory symptoms in the affected lung or dyspnea owing to pulmonary hypertension from long-standing shunting. Indications for repair are (1) pulmonary hypertension owing to left-to-right shunt with a shunt fraction greater than 50% as determined by cardiac catheterization and (2) recurrent pulmonary infections.5Go

This case demonstrates the technical difficulty of pneumonectomy after failed repair of scimitar syndrome in the setting of recurrent infection. It points out that surgical resection of a lung should not be delayed when a propensity to become infected is evident. Inflammation can lead to dense pleural adhesions. Although an extrapleural approach may be radical, it potentially reduces the risk of postpneumonectomy space infection in patients with loculated fluid collections or ongoing parenchymal infection. Additionally, it may reduce postoperative bleeding from collateral blood supply. Preoperative imaging with magnetic resonance angiography of the vascular anatomy is critical to avoid intraoperative surprises. Pneumonectomy is unlikely to adversely affect pulmonary function and may actually improve it.

References

  1. Halasz NA, Halloran JH, Liebow AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 1956;14:826-846.[Abstract/Free Full Text]
  2. Dupuis C, Charaf LA, Breviere GM, Abou P, Remy-Jardin M, Helmius G. The "adult" form of the scimitar syndrome. Am J Cardiol 1992;70:502-507.[Medline]
  3. Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome. twenty years' experience and results of repair. J Thorac Cardiovasc Surg 1996;112:1161-1168discussion 1168-9.[Abstract/Free Full Text]
  4. Kamiyama M, Kamata S, Usui N. Scimitar syndrome treated with pneumonectomy. a case associated with bronchospastic attack. Pediatr Surg Int 2004;20:65-66.[Medline]
  5. Schramel FM, Westermann CJ, Knaepen PJ, van den Bosch JM. The scimitar syndrome. clinical spectrum and surgical treatment. Eur Respir J 1995;8:196-201.[Abstract]



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