CR1-176

Single ventricle physiology in a pediatric patient with maternal Zika virus exposure: A case report and literature review regarding the anesthetic considerations of newborns and infants with congenital Zika syndrome

1Porter K, 2Bradshaw J, 3Heng R, 3Post-Martens K, 3Schrum S
1Mayo Clinic Rochester, Rochester, MN, USA; 2Mayo Clinic Jacksonville, Jacksonville, FL, USA; 3Nemours Children’s Specialty Care, Jacksonville, FL, USA

Introduction
This case regards a 10 month old male with congenital heart disease (CHD) and maternal Zika virus exposure. Discussion of his case and review of the current literature on the anesthetic considerations of Zika virus in newborns and infants follows. Parental consent and expedited IRB approval obtained.

Case Description
Our patient presented with single ventricle physiology from a large ventricular septal defect, subaortic stenosis, hypoplastic ascending aorta and transverse aortic arch, bidirectional patent ductus arteriosus, head sparing failure-to-thrive, and heart failure requiring diuretics. He underwent diagnostic cardiac catheterization and initial repair of his CHD with a Norwood procedure and use of a modified Blalock-Taussig shunt.

His mother had serologically confirmed Zika virus infection at 20+ weeks gestation. Her only presenting symptom was pruritus; she was tested for the virus due to its local prevalence and pregnancy. Exact time of viral inoculation is unknown. Our patient did not exhibit the usual constellation of birth defects seen with congenital Zika syndrome (CZS) though he was never tested for the virus. Due to maternal Zika virus exposure, the patient underwent extra fetal scans and an echocardiogram after birth which demonstrated his CHD. No family history of CHD was reported.

The Norwood procedure was completed successfully. His postoperative course was complicated by refractory hypoxemia and persistent need for inhaled nitric oxide, but extubation was eventually achieved.

Discussion and Lessons Learned
Though the full range of congenital defects related to Zika virus exposure is unknown, the currently accepted phenotype of CZS includes microcephaly, CNS and eye abnormalities, neural tube defects, joint contractures, and sensorineural deafness.1 Maternal Zika exposure does not always result in CZS, but CHD may be seen.2

Several perianesthetic considerations arise when treating CZS patients. Viremia is high in newborns and healthcare workers are at risk of occupational exposure. Personal protective equipment, safe needle handling practices, and proper hand hygiene should be used. Infants with CZS may present with micrognathia and other craniocervical defects which could make mask ventilation, direct laryngoscopy, or placement of a laryngeal mask airway challenging. Availability of video laryngoscopy and other advanced airway techniques is recommended during induction of anesthesia or neonatal resuscitation. Arthrogryposis is common with CZS and may present challenges with positioning and IV placement. In patients with craniosynostosis, medications which could increase intracranial pressure should be avoided or used with caution. Lastly, patients with CZS may exhibit epileptic activity and cautious use of medications that lower the seizure threshold is recommended. 3

1. Centers for Disease Control and Prevention (CDC). Zika Virus: Microcephaly & Other Birth Defects. (2017, August 9).
2. Di Cavalcanti et al. (2017). Echocardiographic findings of infants with presumed congenital Zika syndrome: Retrospective case series study. PLoS One, 12(4).
3. Tutiven et al. (2017). Zika Virus: Obstetric and Pediatric Anesthesia Considerations. Anesthesia and Analgesia, 124(6), 1918-1929.


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