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

Sameh M. Said, Muhammad Yasir Qureshi, Nathaniel W. Taggart, Heather N. Anderson, Patrick W. O'Leary, Frank Cetta, Layan Alrahmani, Shelagh A. Cofer, Leal G. Segura, Roxann B. Pike, Emily E. Sharpe, Douglas P. Derleth, Michael E. Nemergut, Charlotte S. Van Dorn, Stephen J. Gleich, Carl H. Rose, Christopher A. Collura, Rodrigo Ruano

Research output: Contribution to journalArticle

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Abstract

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 utero Intrapartum 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.

Original languageEnglish (US)
Pages (from-to)356-361
Number of pages6
JournalMayo Clinic Proceedings
Volume94
Issue number2
DOIs
StatePublished - Feb 1 2019

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Atrial Septum
Hypoplastic Left Heart Syndrome
Fetus
Hydrops Fetalis
Therapeutics
Communication
Membrane Oxygenators
Pregnancy
Mortality
Pleural Effusion
Cardiopulmonary Bypass
Intensive Care Units
Echocardiography
Mothers
Recurrence

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum. / Said, Sameh M.; Qureshi, Muhammad Yasir; Taggart, Nathaniel W.; Anderson, Heather N.; O'Leary, Patrick W.; Cetta, Frank; Alrahmani, Layan; Cofer, Shelagh A.; Segura, Leal G.; Pike, Roxann B.; Sharpe, Emily E.; Derleth, Douglas P.; Nemergut, Michael E.; Van Dorn, Charlotte S.; Gleich, Stephen J.; Rose, Carl H.; Collura, Christopher A.; Ruano, Rodrigo.

In: Mayo Clinic Proceedings, Vol. 94, No. 2, 01.02.2019, p. 356-361.

Research output: Contribution to journalArticle

Said, SM, Qureshi, MY, Taggart, NW, Anderson, HN, O'Leary, PW, Cetta, F, Alrahmani, L, Cofer, SA, Segura, LG, Pike, RB, Sharpe, EE, Derleth, DP, Nemergut, ME, Van Dorn, CS, Gleich, SJ, Rose, CH, Collura, CA & Ruano, R 2019, 'Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum', Mayo Clinic Proceedings, vol. 94, no. 2, pp. 356-361. https://doi.org/10.1016/j.mayocp.2018.08.004
Said, Sameh M. ; Qureshi, Muhammad Yasir ; Taggart, Nathaniel W. ; Anderson, Heather N. ; O'Leary, Patrick W. ; Cetta, Frank ; Alrahmani, Layan ; Cofer, Shelagh A. ; Segura, Leal G. ; Pike, Roxann B. ; Sharpe, Emily E. ; Derleth, Douglas P. ; Nemergut, Michael E. ; Van Dorn, Charlotte S. ; Gleich, Stephen J. ; Rose, Carl H. ; Collura, Christopher A. ; Ruano, Rodrigo. / Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum. In: Mayo Clinic Proceedings. 2019 ; Vol. 94, No. 2. pp. 356-361.
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AU - Anderson, Heather N.

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AU - Cetta, Frank

AU - Alrahmani, Layan

AU - Cofer, Shelagh A.

AU - Segura, Leal G.

AU - Pike, Roxann B.

AU - Sharpe, Emily E.

AU - Derleth, Douglas P.

AU - Nemergut, Michael E.

AU - Van Dorn, Charlotte S.

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N2 - 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 utero Intrapartum 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.

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