Pediatric Cardiothoracic Surgery in Patients With Unilateral Pulmonary Agenesis or Aplasia

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

“…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 b of a median sternotomy as the surgical approach for repairing the associated heart diseases. In the literature, the left and right thoracotomy is used instead of median sternotomy for the correction of associated congenital heart diseases [1]. Our case differs from others in that the displacement and rotation of the organs was very high.…”

“…[1][2][3][4]. The condition is defined as complete unilateral absence of the lung tissue, bronchus, and pulmonary artery branch [1,4].…”

“…The surgical corrections of atrial and ventricular septal defects, double outlet right ventricle, interrupted aortic arch, patent ductus arteriosus, subaortic stenosis [1], total anomalous pulmonary venous connection [7], and tricuspid atresia [3] with unilateral lung agenesis are described in the literature. Through 2014, there are published 20 cases of repair different types of congenital heart malformations in combination with unilateral lung agenesis [1]. To the best of our knowledge, this case is the first English publication describing the combination of unilateral lung agenesis with tetralogy of Fallot.…”

Supportive pericardial suspension for surgical airway management of tracheobronchomalacia in unilateral pulmonary agenesis

“…Pulling up the overlying vessel or the airway itself anteriorly to the sternum can reduce the tracheobronchial collapse and improve respiratory function. In patients with right UPA, displacement and rotation of the heart to the hemithorax have the potential to cause aortic compression of the trachea, and our previous study showed that aortopexy or tracheopexy were also useful to prevent postoperative tracheomalacia [3]. However, the anterior border of the left lung often crosses the midline because of hyperinflation, which interrupts the route to the sternum for aortopexy or tracheopexy in patients with right UPA.…”

“…Patients with UPA present unique anatomic features such as an ipsilateral shift and rotation of the heart and mediastinum to the empty hemithorax, which results in displacement, distortion, and compression of the great vessels and airway. Tracheobronchial stenosis and malacia often accompany UPA and are associated with high morbidity and mortality [1][2][3]. We report here two cases of UPA with tracheobronchial problems that were successfully managed surgically using supportive pericardial suspension.…”