Surgical Management of Infants with Congenital Lobar Emphysema and Concomitant Congenital Heart Disease

Abstract

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.

Ventricular Septal Defect Complicated by Infantile Lobar Emphysema

“…4) Conservative or non-operative treatment is possible in asymptomatic or mildly symptomatic cases, however, lobectomy is the universally accepted treatment in moderately or severely symptomatic cases. 1) Congenital heart disease can be associated with infantile lobar emphysema, however, there has been some debate as to the appropriate management of these infants, [2][3][4][5] regarding whether the correction of the cardiac defect should be performed first, or whether the lobectomy should be performed first, or whether they should be performed simultaneously.…”

“…It may be argued that pulmonary artery banding concomitant with a lobectomy might be less invasive than a total correction with a lobectomy. 2,3) However, a total correction of cardiac anomalies concomitant with a lobectomy simplifies the postoperative management.…”

“…1) Although the etiology of the condition is most commonly idiopathic, an association has been found between infantile lobar emphysema and congenital heart disease. [2][3][4] We encountered an infant who had ventricular septal defect (VSD) and atrial septal defect (ASD) and infantile emphysema of the right upper lobe. The infant was intubated due to respiratory distress and thereafter was dependent upon mechanical ventilation.…”

Management of Congenital Lobar Emphysema: The Current Challenges

“…Multislice CT with reconstructions of the bronchial tree and virtual bronchoscopy are important imaging tools for differential diagnosis of CLE and bronchial foreign bodies, as both may present with pulmonary hyperinsuflation findings and nonspecific clinical features [36].Presence of other congenital cardiac malformations may require further echocardiographic evaluation and/or cardiac catheterization [37].…”

Clinical Syndromes and Associations with Persistent Pulmonary Hypertension of the Newborn

“…CLE only rarely presents with PPHN at birth (Schwartz & Ramachandran, 1997). Concomitant cardiovascular disease however is not uncommon and should be investigated when CLE is detected (Dogan et al, 2004).…”