Thorac Cardiovasc Surg 2016; 64 - OP129
DOI: 10.1055/s-0036-1571580

Cardiopulmonary Bypass (CPB) in Low-birth Weight Premature Neonate with Double Aortic Arch, Congenital Heart Disease and Tracheal Stenosis

M. Zaradzki 1, E. Fonseca-Escalante 2, J. Kolcz 2, P. Seppelt 2, M. Karck 1, W. Springer 3, M. Gorenflo 3, T. Loukanov 2
  • 1Universitätsklinikum Heidelberg, Klinik für Herzchirurgie, Heidelberg, Germany
  • 2Universitätsklinikum Heidelberg Klinik für Herzchirurgie, Sektion Kinderherzchirurgie, Heidelberg, Germany
  • 3Zentrum für Kinder- und Jugendmedizin, Pädiatrische Kardiologie und Angeborene Herzfehler, Heidelberg, Germany

Introduction: Prenatal diagnosis of aortic arch anomalies associated with critical tracheal stenosis is challenging and management is related to high morbidity and mortality. We present successful surgical treatment of low-birth weight premature neonate with vascular ring, congenital heart disease and tracheal stenosis.

Background: The patient was born in 33 + 0 week of pregnancy with body weight of 1600 g in a district hospital. After birth he presented with apnea and bradycardia (APGAR 1/1/8). He was intubated and after successful cardiopulmonary resuscitation, ventilatory support was commenced. Because of double-sided pneumothorax with suspicion of tracheal injury both pleurae were drained. Angio-CT revealed double aortic arch, VSD, ASD and long-segment tracheal stenosis. Preoperative bronchoscopy revealed funnel- trachea and tracheomalacia. The vascular ring and postintubation injury of the pars membranacea of the trachea coexisting with tracheal stenosis were diagnosed intraoperative. After transection of non-dominant part of the double aortic arch, the stenotic part of the trachea was resected and a slide-tracheoplasty was performed. In addition cardiac malformation was addressed. Postoperative care was uneventful.

Discussion: Vascular ring associated with funnel trachea is a rare constellation. The prenatal diagnosis of aortic arch anomalies is challenging especially if coexisting with other extra-cardiac anomalies. Presented case is according to our knowledge the smallest patient after successful treatment: slide- tracheoplasty concomitant with cardiac correction with CPB. Prenatal diagnosis of a vascular ring should lead to genetic screening (22q11 syndrome) and diagnosis of extra-cardiac anomalies. This can be crucial for successful perinatal management. In our case imprecise prenatal diagnosis led to iatrogenic complications. Nevertheless after stabilization and proper diagnosis, despite of low birth weight, the patient was successfully treated under CPB.