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///2016 Abstract Details
2016 Abstract Details2019-07-15T10:10:51-05:00

Anesthetic management and peripartum outcomes of parturients with Loeys-Dietz phenotype with and without mutations in the transforming growth factor-β signaling pathway.

Abstract Number: F-38
Abstract Type: Original Research

Annemaria De Tina MD, FRCPC1 ; Anthony Chau MD, FRCPC2; Jean Marie Carabuena MD3; Lawrence C Tsen MD4

INTRODUCTION: Genetic mutations that alter transforming growth factor-β (TGF-β) signaling predispose individuals to cardiovascular, craniofacial and skeletal abnormalities. [1] These mutations are found in patients with Loeys-Dietz Syndrome (LDS), a connective tissue disease (CTD) first described in 2005. [2] Compared with Marfan syndrome, LDS patients have more aggressive vascular disease with dissection occurring at smaller aortic diameters and a mean age of death of 26.1. [1] During pregnancy, patients with LDS are at risk of progression of aortopathy and uterine rupture. [1] Some patients diagnosed with unspecified CTD are suspected to have LDS based on craniofacial and skeletal abnormalities but do not carry mutations in the TGF-β pathway. We sought to describe the anesthetic management and peripartum outcomes of parturients with unspecified CTD with LDS phenotype and those with genotype-confirmed LDS.

METHODS: We retrospectively reviewed the medical records of all patients diagnosed with unspecified CTD having LDS phenotype with (LDS+) and without (LDS-) mutations affecting TGF-β signaling over a 7-year period (Nov 2007 to Jan 2015).

RESULTS: Eleven pregnancies in nine parturients were analyzed. There were four LDS-, two LDS+, and three query LDS (qLDS) patients who not undergo genetic testing. Neither LDS+ patient had documented aggressive arterial disease, and three patients (1 LDS+, 2 LDS-) were on beta-blockers throughout gestation. The planned mode of delivery was vaginal for nine of eleven (82%) pregnancies. In the LDS- cases, three required vacuum or forceps assistance due to maternal fatigue and four cases ultimately required cesarean delivery (CD). A total of six CD’s (55%) were performed, four in the LDS- group and two in the qLDS group; one was performed emergently due to placental abruption, three were performed urgently due to fetal intolerance of labor; two were performed electively. In the LDS+ cases, both had successful term vaginal deliveries. All deliveries were performed with a neuraxial technique. All cases were managed with serial echocardiograms, which indicated initial aortic root diameters of ≤ 38 mm during and after pregnancy. Arterial blood pressure monitoring and telemetry were not used in any cases. No maternal or neonatal complications were observed in this cohort.

DISCUSSION: Parturients with unspecified CTD having LDS phenotype with or without mutations in the TGF-β pathway receiving care at our institution had good clinical outcomes. Despite being at high risk for anesthetic or obstetric complications, and encouraged to undergo early, elective CD [1], parturients with mutations in the TGF-β pathway without aggressive arterial disease can have successful term pregnancies, tolerate labor, and have vaginal and cesarean deliveries under neuraxial analgesia.

REFERENCES:

1. MacCarrick G et al, Genet Med, 2014

2. Loeys BL et al, Nat Genet, 2005

SOAP 2016