Eastern Cardiothoracic Surgical Society

Surgical Treatment of Diffuse Complex Pulmonary Arteriovenous Malformations (PAVM) in Pediatric Patients
Stephanie Fuller1, Reed E. Pyeritz2, Raezelle Zinman1, Brian Hanna1, Jonathon J. Rome1, Scott O. Trerotola2. 1The Children’s Hospital of Philadelphia, Philadelphia, PA USA, 2The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA USA.

Objective: Diffuse-type PAVMs respond poorly to embolotherapy. Although initial response is favorable, progressive development of bronchial to pulmonary collaterals is common and incurs late morbidity and mortality. When PAVMs are limited, surgery can be curative and prevents the long-term sequelae of collateral formation and resultant hemoptysis.

Methods: Three hypoxemic pediatric patients ages 10, 13 and 15, with diffuse-type PAVMs limited to one lung were treated by resections including wedge, lobectomy and pneumonectomy. Two patients with hereditary hemorrhagic telangiectasia (HHT) had multiple embolotherapy treatments prior to surgery. The third had resection without embolization. The outcomes of embolotherapy and surgery were analyzed retrospectively.

Results: Embolotherapy was performed 4 times in one patient and 5 times in another using conventional techniques (coils, microcoils) starting as peripherally as possible and working centrally as previously described. Initial response to embolotherapy in these patients yielded improvement in O2 sat to >90%. Failures of embolotherapy were declared for hemoptysis (n=1) and intractable hypoxemia (n=3). Lung resection via thoracotomy was successful in all patients with normal postoperative O2 saturation on room air at follow-up. Hemoptysis has not occurred post-surgery.

Conclusions: Surgery is successful in treating limited diffuse-type PAVMs. With careful patient selection, curative resection may be considered front-line therapy in this rare patient population as it avoids repeated radiation exposure and eliminates the risk of bronchial collateral formation.