title: Norwood Procedure Performed on a Patient With Trisomy 13.
abstract: Trisomy 13 is associated with a variety of congenital anomalies, some of which are life-threatening and related to poor prognosis. Therefore, cardiac surgery is rarely offered to these patients, especially to those with complex cardiac anomalies. We report the case of a neonate weighing 2324 g who was born with severe congenital heart defects. Transthoracic echocardiography revealed the diagnoses of asplenia, single ventricle, aortic stenosis, coarctation of the aorta, hypoplastic aortic arch, and total anomalous pulmonary venous return. She was hemodynamically unstable. Palliative Norwood procedure with right ventricle-pulmonary artery conduit (RV-PA conduit) was performed at the age of 1 day to save her life. On postoperative day 7, chromosome analysis revealed trisomy 13. Echocardiography revealed good heart function; stable hemodynamic status was achieved with minimal amounts of inotropic agents. However, she developed anuria, which did not improve despite situational possible interventions, including peritoneal dialysis and continuous hemodiafiltration. On postoperative day 37, she succumbed to sudden cardiorespiratory failure. Nevertheless, this case indicates that a neonate with trisomy 13 can have a better chance at survival with cardiac surgery such as the Norwood procedure with an RV-PA conduit.
authors.affiliation: Department of Cardiovascular Surgery, Kitasato University School of Medicine.
authors.forename: Norihiko
authors.initials: N
authors.lastname: Oka
authors.forename: Takamichi
authors.initials: T
authors.lastname: Inoue
authors.forename: Miyuki
authors.initials: M
authors.lastname: Shibata
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yoshii
authors.forename: Yuki
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Haruna
authors.initials: H
authors.lastname: Araki
authors.forename: Yoshikiyo
authors.initials: Y
authors.lastname: Matsunaga
authors.forename: Tomoki
authors.initials: T
authors.lastname: Tamura
authors.forename: Keiichi
authors.initials: K
authors.lastname: Itatani
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Horai
authors.forename: Tadashi
authors.initials: T
authors.lastname: Kitamura
authors.forename: Shinzo
authors.initials: S
authors.lastname: Torii
authors.forename: Kagami
authors.initials: K
authors.lastname: Miyaji
mesh_terms: D006328:Cardiac Catheterization; D025063:Chromosome Disorders / Q000175:diagnosis / Q000601:surgery*; D002882:Chromosomes, Human, Pair 13; D004452:Echocardiography; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D058327:Norwood Procedures / Q000379:methods*; D016896:Treatment Outcome; D014314:Trisomy / Q000175:diagnosis; D000073839:Trisomy 13 Syndrome