Surgical Management of Infants with Congenital Lobar Emphysema and Concomitant Congenital Heart Disease
Objective: Congenital lobar emphysema (CLE) is an uncommon cause of infantile respiratory distress. It is diagnosed on the basis of evidence of lobar overaeration, mediastinal shift, and compression of the adjacent lobe. Concomitant congenital heart disease (CHD) and CLE is not uncommon. In the literature a 12% to 20% concomitance rate is given. The optimal treatment of respiratory symptoms associated with CLE and CHD is not clear; however, there has been a great deal of progress in the treatment of CLE and CHD. The aim of this study was to evaluate a clinical experience with and long-term follow-up of the surgical treatment of 13 patients with concomitant CLE and CHD.
Material and Methods: We reviewed the cases of 13 patients with concomitant CLE and CHD. The medical records were evaluated with reference to age, type of CHD, pulmonary artery pressure, clinical symptoms, and results of surgical management.
Results: One patient died. This patient had ventricular septal defect (VSD) and left upper lobe emphysema in the postoperative period. The remaining patients undergoing follow-up were clinically well at the final evaluation. Postoperative thoracic computed tomography revealed complete spontaneous regression of emphysema 3 months after division of ductus arteriosus in 1 patient. Pulmonary hypertensive episode was seen in 3 patients after the early postoperative period. Five of the patients were discharged with bronchodilator treatment after surgery. Six patients needed positive inotropic support. Among the patients with pulmonary hypertension and those with VSD who had undergone cardiopulmonary bypass, we found a greater need for inotropic support, a higher risk of postoperative infection, and a longer intubation period. Echocardiography in the late postoperative revealed decreased pulmonary artery diameter and pressure; myocardial performance was normal. Results of blood gas analyses revealed increased oxygen saturation and decreased partial pressure of carbon dioxide. Normal exercise activity was found in all patients.
Discussion: The presence of CHD, especially in infants with unusual respiratory distress symptoms, should be kept in mind, and echocardiography and/or cardiac catheterization should be considered in the diagnosis. In patients with high pulmonary artery pressure, palliative or corrective surgery for CHD in addition to lobectomy can be considered. We believe that for lesions without high pulmonary artery pressure, such as small atrial septal defect and patent foramen ovale, clinical follow-up is sufficient treatment after lobectomy. If the cause of CLE is compression of large ductus arteriosus, only division of the patent ductus arteriosus may be considered before lobectomy and clinical and radiologic follow-up. The cardiac lesion should be assessed as to severity and ease of management. A corrective procedure can be carried out at lobectomy. Because of the technical ease with which the cardiac operation can be performed at the time of lobectomy, we suggest that in addition to lobectomy, operative treatment of cardiac lesions be performed.
Ozcelik U, Göçmen A, Kiper N, Dogru D, Dilber E, Yalçin EG. 2003. Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center. Pediatr Pulmonol 35:384-91.nPierce WS, DeParedes CG, Friedman S, Waldhausen JA. 1970. Concomitant congenital heart disease and lobar emphysema in infants: incidence, diagnosis, and operative management. Ann Surg 172:951-6.nRoghair GD. 1972. Nonoperative management of lobar emphysema lobar emphysema. Radiology 102:125-7.nRoguin N, Peleg H, Lemer J, et al. 1980. The value of cardiac catheterization and cineangiography in infantile lobar emphysema. Pediatr Radiol 10:71-4.nRudolph AM. 1969. Commentary: pulmonary complications of congenitally heart disease. Pediatrics 43:757.nStigers KB, Woodring JH, Konga JF. 1992. The clinical and imaging spectrum of findings in patients with congenital lobar emphysema. Pediatr Pulmonol 14:160-70.nStranger P, Lucas PV, Edwards JE. 1969. Anatomic factors causing respiratory distress in acyanotic congenitally cardiac disease: special reference to bronchial obstruction. Pediatrics 43:760-9.nDogan R, Demircin M, Sarigul A, Pasaoglu I, Gocmen A, Bozer AY. 1997. Surgical treatment of congenital lobar emphysema. Turkish J Pediatr 39:35-44.nElmaci TT, Guler N, Aydogan U, Onursal E. 2001. Infantile lobar emphysema and tracheal bronchus in a patient with congenitally heart disease. J Pediatr Surg 36:1596-8.nGordon I, Dempsey JE. 1990. Infantile lobar emphysema in association with congenital heart disease. Clin Radiol 4:48-52.nGross RE, Lewis JE Jr. 1945. Defect of the anterior mediastinum successful repair. Surg Gynecol Obstet 80:549-54.nIsojima A, Yuasa H, Kusagawa M, et al. 1978. Surgical treatment of infantile lobar emphysema in cardiovascular disease with left to right shunt. Jpn J Surg 8:57-65.nJones J, Almond CH, Snyder HM, et al. 1965. Lobar emphysema and congenital heart disease in infancy. J Thorac Cardiovasc Surg 49:1-10.nKennedy JH, Rothman BF. 1965. The surgical treatment of congenital lobar emphysema. Surg Gynecol Obstet 121:253-60.nLincoln JC, Stark J, Subramanian S, et al. 1971. Congenital lobar emphysema. Ann Surg 173:55-62.nLitwin SB, Rosenthal A, Fellows K. 1973. Surgical management of young infant with tetralogy of Fallot, absence of the pulmonary valve, and respiratory distress. J Thorac Cardiovasc Surg 65:552-8.nMan DW, Hamdy MH, Hendry GM, Bisset WH, Forfar JO. 1983. Congenital lobar emphysema: problems in diagnosis and management. Arch Dis Child 58:709-11.nMurray GF. 1967. Congenital lobar emphysema. Surg Gynecol Obstet 124:611-25.nMurray GF. 1971. Congenital lobar emphysema in infants. Br J Radiol 44:692-6.n