Title: Screening and Anesthetic Management of Hereditary Hemorrhagic Telangiectasia in Parturients

Nicholas Cirrone, MD; Mohammad Nawaz, MD; Christine Chen, MD

Background: 

• Hereditary hemorrhagic telangiectasia (HHT): vascular dysplasia characterized by telangiectasias and arteriovenous malformations (AVMs).

• Affects 1 in 5000 people.

• Most commonly in the lung (40%) and more rarely in the spine (<1%).1 

• Pregnancy can worsen AVMs from increased blood volume and cardiac output

• Guidelines recommend screening, multidisciplinary planning, and risk assessment.2 

• HHT raises concern for spinal AVMs and neuraxial bleeding, while general anesthesia carries risk from pulmonary AVM rupture and right-to-left shunting.1,2 

• We report two patients with HHT and review evaluation, anesthetic management, and outcomes.

Cases:

• 32-year-old G1P0 (IVF) with prior pulmonary AVM embolization (2022) and stable residual pulmonary AVMs on CTA (2024). Brain (2022) and spine (2025) MRI were negative. History of mild epistaxis. Presented December 2025 for premature rupture of membranes, received uncomplicated combined spinal-epidural (CSE) labor analgesia using saline loss of resistance with in-line air filters. She later underwent cesarean delivery for category II tracing without hemorrhagic or neurologic complications and was discharged postoperative day 3.
  
  

• 37-year-old G2P1 longstanding HHT, two prior pulmonary AVM embolizations (2018), and negative brain (2017) and lumbar (2016) MRI. History intermittent epistaxis only and a prior uncomplicated labor CSE in 2021. She declined neuraxial analgesia despite being cleared by anesthesia during her recent labor in October 2025 and delivered vaginally without complications or postpartum hemorrhage.

Teaching Points:

• HHT creates high-stakes anesthetic decisions due to pulmonary shunting and possible neuraxial vascular lesions. 

• These cases support a risk-stratified approach using focused history, prior imaging review, pulmonary/CNS AVM assessment, and multidisciplinary planning. 

• When neuraxial anesthesia is used, saline loss of resistance to reduce paradoxical embolism, strict avoidance of intravascular air, and early analgesia may mitigate preventable risk.3 

• Standardizing these steps promotes safe neuraxial use and provides practical teaching points.

References: 

1. Brady S, Tan T, O’Flaherty D. Hereditary haemorrhagic telangiectasia and neuraxial anaesthesia in pregnancy: when should magnetic resonance imaging be performed? Anaesth Rep. 2023;11:e12227. doi:10.1002/anr3.12227

2. Faughnan ME, Mager JJ, Hetts SW, et al. Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia. Ann Intern Med. 2020;173(12):989-1001. doi:10.7326/M20-1443

3. Lomax S, Edgcombe H. Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia. Can J Anaesth. 2009;56(5):374-384. doi:10.1007/s12630-009-9076-z

4. Jaskolka J, Wu L, Chan RP, Faughnan ME. Imaging of hereditary hemorrhagic telangiectasia. AJR Am J Roentgenol. 2012;183(2). doi:10.2214/ajr.183.2.1830307

Figure 1. Example of arteriovenous malformations (arrow) posterior to spinal cord in a patient diagnosed with HHT. Image is from a sagittal spin-echo T1-weighted MRI.4