One-Stage Midline Unifocalization With Complete Repair of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collaterals

The patient was a 5-month-old, 4.7 kg Infant with pulmonary atresia, ventricular septal defect (VSD), and absent native intra-pericardial pulmonary arteries. All bronchopulmonary segments were supplied by four large major aortopulmonary collaterals (MAPCA). Associated defects were severe ascending aorta dilation, mild bicuspid aortic valve stenosis, right aortic arch with mirror image branching, and multiple thoracic hemivertebral anomalies.

Preoperative evaluation included transthoracic echocardiography, cardiac computed tomography (CT) scan, and cardiac catheterization to demonstrate her complete anatomy. Through a median sternotomy, complete unifocalization of all MAPCAs were performed followed by completion of the repair with patch closure of the ventricular septal defect, suture closure of the patent foramen ovale, and establishment of right ventricular to neopulmonary confluence using a decllularized valved aortic homograft. She remained in normal sinus rhythm and was weaned off cardiopulmonary bypass without difficulty. She was extubated on postoperative day two and required a subxiphoid pericardial window for drainage of pericardial effusion. She was dismissed a month later on aspirin.

Follow-up echocardiography showed good biventricular function, widely patent biventricular outflow tracts, tiny residual ventricular level shunt, and good flow in the pulmonary conduit and main branches. CT scan showed the reconstructed pulmonary vascular bed. Her right ventricular pressure was 40% of the systemic blood pressure. She continued to do well at her last follow-up.

References

  1. Reddy VM1, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 1995 May;109(5):832-844.
  2. Malhotra SP1, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009:145-151.
  3. Carrillo SA1, Mainwaring RD2, Patrick WL1, Bauser-Heaton HD3, Peng L3, Reddy VM1, et al. Repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg. 2015 Aug;100(2):606-614.
  4. Said SM. Commentary: unifocalization revision: patches, patches, and more patches…. J Thorac Cardiovasc Surg. 2019 Aug;158(2):546-547.

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