Primary repair of tetralogy of Fallot combined with unilateral left lung agenesis with crossed-ectopic right lower lobe

Abstract

Unilateral lung agenesis is a rare congenital anomaly often combined with congenital malformations of different systems. A case of tetralogy of Fallot repair with unilateral left lung agenesis via median sternotomy is described for the first time. In this case, the choice of surgical approach to the heart could be affected by the fears of not getting optimal surgical exposition via median sternotomy due to severe displacement of the chest organs. The case demonstrates the median sternotomy as an optimal surgical approach even in high degrees of organ displacement caused by a combination of left pulmonary agenesis and cardiac lesions.

Surgical management in tetralogy of Fallot with rare unilateral pulmonary anomalies: A literature review

“…Because it allows adequate exposure of ascending aorta and vena cavae. 7,22 Malyshev et al 6 did a different surgical approach in an 8-year-old male with TOF and left pulmonary agenesis with a crossed-ectopic lower lobe of the right pulmonary. The patient underwent a successful primary repair of TOF with a median sternotomy approach.…”

“…The surgical approach through median sternotomy can be considered a universal and optimal approach in any left pulmonary agenesis with cardiac anomalies. 6…”

“…(1) the distal segment of the trachea; (2) the single right bronchus; (3) the crossed-ectopic right lower lobe; (4) the bronchus of the crossed-ectopic right lower lobe; (5) the right branch of the pulmonary artery. 6…”

“…(1) the expanded right lung, (2) the anterior surface of the pericardium, (3) the right pleural sac. 6…”

“…Pulmonary tissue that can be seen in ipsilateral hemithorax space comes from the herniated or cross-ectopic contralateral lung tissue. 6 The absence of lung volume in the ipsilateral hemithorax causes displacement of the heart and mediastinum and causes distortion and compression of the large vessels and airways. 7 This phenomenon occurs to fill the empty ipsilateral hemithorax.…”