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CORRESPONDENCE

References 1. Baciewicz FA. Anomalous aortic origin of the right coronary artery (letter). Ann Thorac Surg 2015;99:1491. 2. Izumi K, Wilbring M, Stumpf J, et al. Direct reimplantation as an alternative approach for treatment of anomalous aortic origin of the right coronary artery. Ann Thorac Surg 2014;98:740–2.

Isolated Unilateral Absence of Pulmonary Artery: Challenges in Treatment To the Editor:

respiratory tract infection, or pulmonary hypertension [3, 4]. Treatment options include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow, or a primary versus staged pulmonary artery anastomosis. Hemoptysis may be self-limiting over many years, but massive hemoptysis can be treated only by selective embolization of the systemic collaterals [3]. In patients who have delayed diagnosis and delayed surgical interventions, irreversible hypoplasia and regression of the affected pulmonary artery may develop, and these patients have a less favorable outcome. Therefore, it is very important to create a guideline for the surgical treatment of this serious disease. Supported by a grant from the European Regional Development Fund—Project FNUSA-ICRC (no. CZ.1.05/1.1.00/02.0123).

Peter Kruzliak, MD, MS Department of Cardiovascular Diseases International Clinical Research Center St. Anne’s University Hospital and Masaryk University Pekarska 53 Brno 656 91, Czech Republic e-mail: [email protected]

References 1. Topcu S, Ozbay S, Akgul AG, et al. Unusual etiologic factor of massive hemoptysis: unilateral absence of pulmonary artery. Ann Thorac Surg 2014;98:346. 2. Szwast A, Tian Z, McCann M, et al. Anatomic variability and outcome in prenatally diagnosed absent pulmonary valve syndrome. Ann Thorac Surg 2014;98:152–8. 3. Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 2013;106:448–54. 4. Welch K, Hanley F, Johnston T, Cailes C, Shah MJ. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg 2005;79:1399–402.

MISCELLANEOUS

Topcu and colleagues [1] reported a very interesting case of unilateral absence of pulmonary artery that presented as massive hemoptysis. I would like to provide some comments regarding the surgical treatment of this serious condition. Unilateral absence of pulmonary artery is a rare pulmonary vascular malformation usually associated with other cardiac anomalies, such as tetralogy of Fallot, ventricular septal defect, or patent ductus arteriosus [2, 3]. There is no consensus regarding the surgical treatment of isolated unilateral absence of pulmonary artery. Early surgical intervention may potentially preserve the affected lung vasculature and prevent morbidity and mortality in children. Early reestablishment of pulmonary blood flow may allow the affected lung to develop more normally and improve pulmonary hypertension [3, 4]. The surgical approaches include either creating an aortopulmonary shunt or connecting the affected pulmonary artery to the main pulmonary artery [3, 4]. If the intrapulmonary arteries are well developed, a primary anastomosis with the central pulmonary artery can be made. If the intrapulmonary branches are small, a modified BlalockTaussig shunt can be used. For older patients, revascularization is not encouraged or even feasible because the intrapulmonary arteries have been found to be obstructed by fibrosis [3, 4]. In patients with progressive irreversible pulmonary hypertension, the only chance for survival is heart-lung transplantation. No treatment is required for adult patients without any evidence of cardiopulmonary dysfunction. They should be followed up on a regular basis. Treatment should be reserved for the small number of adult patients who have hemoptysis, recurrent lower

Ann Thorac Surg 2015;99:1488–92

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

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