Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Autor:	Said SM; Department of Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN., Qureshi MY; Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN., Taggart NW; Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN., Anderson HN; Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN., O'Leary PW; Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN., Cetta F; Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN., Alrahmani L; Division of Obstetrics and Maternal Fetal Medicine, Mayo Clinic College of Medicine, Rochester, MN., Cofer SA; Division of Pediatric Otorhinolaryngology, Mayo Clinic College of Medicine, Rochester, MN., Segura LG; Division of Pediatric Anesthesiology, Mayo Clinic College of Medicine, Rochester, MN., Pike RB; Division of Cardiovascular Anesthesia, Mayo Clinic College of Medicine, Rochester, MN., Sharpe EE; Department of Anesthesiology and Perioperative Medicine, Mayo Clinic College of Medicine, Rochester, MN., Derleth DP; Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN., Nemergut ME; Division of Pediatric Anesthesiology, Mayo Clinic College of Medicine, Rochester, MN., Van Dorn CS; Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN., Gleich SJ; Division of Pediatric Anesthesiology, Mayo Clinic College of Medicine, Rochester, MN., Rose CH; Division of Obstetrics and Maternal Fetal Medicine, Mayo Clinic College of Medicine, Rochester, MN., Collura CA; Division of Neonatal Medicine, Mayo Clinic College of Medicine, Rochester, MN., Ruano R; Division of Obstetrics and Maternal Fetal Medicine, Mayo Clinic College of Medicine, Rochester, MN. Electronic address: ruano.rodrigo@mayo.edu.
Jazyk:	angličtina
Zdroj:	Mayo Clinic proceedings [Mayo Clin Proc] 2019 Feb; Vol. 94 (2), pp. 356-361.
DOI:	10.1016/j.mayocp.2018.08.004
Abstrakt:	Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases. (Copyright © 2018 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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