Aspergilloma Resection in a Partial Anomalous Pulmonary Venous Connection of the Left Upper Lobe

Partial anomalous pulmonary vein connection (PAPVC) is due to the failure of one or more of the pulmonary veins to be incorporated into the left atrium, thus flowing into the systemic circulation (1). The incidence of PAPVC at autopsy is between 0.6% - 0.7%, and on the right side is twice as common as PAPVC from the left lung. The most common form of PAPVC is the one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava (2). Anomalous left lung veins flow into derivatives of the left cardinal vein, most often the left innominate vein or the coronary sinus. Many factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs), and its magnitude (Qp:Qs ratio) causes symptoms and complications (3).

Children with PAPVC are usually asymptomatic; symptoms can be observed in older patients and may be due to right-sided volume overload or pulmonary vascular obstructive disease. Pulmonary vascular disease can occasionally occur in older adults; cyanosis is not observed in the presence of an intact atrial septum (4).

Medical therapy of partial anomalous pulmonary venous connection is not indicated for asymptomatic patients. While PAPVC with shunt ratios below 1.5:1 are usually asymptomatic and can only be followed up if the shunt fraction is greater than 1.5:1, surgical closure is indicated. The type of surgical repair depends on the site of anomalous drainage and the coexistence of any other form of heart disease. Anomalous left pulmonary veins may be re-anastomosed to the left atrial appendage while right-sided anomalous veins are often anastomosed to the right atrium and connected to the left atrium with a patch or baffle through a pre-existing or surgically created atrial septal defect. Patients who have been operated on usually present very good outcomes (1).

Here the authors report the case of a 50-year-old woman suffering from lymphoblastic leukemia, developing left upper lobe aspergilloma during aplastic chemotherapy, requiring orotracheal intubation for acute respiratory failure, and long-term mechanical ventilation. The patient subsequently recovered from respiratory failure and the authors were asked by their hematologists to remove the aspergilloma, a formal contraindication to therapy for hematologic disease. The patient underwent total muscle-sparing lateral left thoracotomy and extensive pneumolysis; the anomalous left upper pulmonary vein was isolated as well as the mediastinal branch of the left upper lobe artery; the vascular anatomy was slightly different from normal but without any dangerous anomalies. The left upper pulmonary vein was transected by a 30 mm gold vascular stapler and then the first mediastinal branch of the left upper lobe artery was similarly transected. Pulmonary resection was then accomplished by multiple loads of parenchymal staplers. The postoperative course was uneventful and the patient was discharged on postoperative day four.

References

1. Schiau C, Manole S. Anomalous pulmonary venous connection: the journey from theory to practice! ECR 2016. http://dx.doi.org/10.1594/ecr2016/C-1245.
2. Ho VB, Reddy GP; Cardiovascular Imaging. Vol I. St. Louis, MO. Elsevier Saunders; 2001:625-638.
3. Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Wiliams WG, Caldarone CA, et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation. 2007;115:1591-1598.
4. Monesha Gupta. Partial Anomalous Pulmonary Venous Connection Clinical Presentation. Medscape.com. http://emedicine.medscape.com/article/897686-clinical#b4. Published January 27, 2015. Accessed October 17, 2019.