Topic 13.3. Cardiac considerations for women planning for pregnancy
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
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The Clinical Management Guidelines for Friedreich ataxia (‘Guidelines’) are protected by copyright owned by the authors who contributed to their development or said authors’ assignees.
These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
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Intended Use
These Guidelines are made available as general information only and do not constitute medical advice. These Guidelines are intended to assist qualified healthcare professionals make informed treatment decisions about the care of individuals with Friedreich ataxia. They are not intended as a sole source of guidance in managing issues related to Friedreich ataxia. Rather, they are designed to assist clinicians by providing an evidence-based framework for decision-making.
These Guidelines are not intended to replace clinical judgment and other approaches to diagnosing and managing problems associated with Friedreich ataxia which may be appropriate in specific circumstances. Ultimately, healthcare professionals must make their own treatment decisions on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise.
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The authors of this document gratefully acknowledge the support of the Friedreich Ataxia Research Alliance (FARA). The views and opinions expressed in the Guidelines are solely those of the authors and do not necessarily reflect the official policy or position of FARA.
13.3 Cardiac considerations for women planning for pregnancy
Roger Peverill and Aarti Patel
There is little data available about pregnancy in women with FRDA and reduced left ventricular ejection fraction (LVEF) who are either asymptomatic or have heart failure (HF) symptoms. Based upon data in other cardiac conditions (6), it should be assumed that there is a high risk of maternal and fetal complications in women with FRDA and either reduced LVEF or a history of HF; therefore, avoidance of pregnancy should be recommended in such individuals (7).
Women with FRDA and reduction in LVEF should be advised that pregnancy could result in cardiac decompensation and greater fetal risk and that it is therefore advised against. Pre-pregnancy counseling including consultation with a multidisciplinary team, including a cardiologist and an obstetrician, may assist in decision making.
Women with reduced ejection fraction planning pregnancy
QUESTION: Should advice on avoiding pregnancy versus pursuing or not avoiding pregnancy be used for high risk pregnancy (patients with reduced ejection fraction with or without heart failure) with Friedreich ataxia?
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[/sg_popup]STRENGTH OF RECOMMENDATION: ↑
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RECOMMENDATION: We conditionally recommend that women with Friedreich ataxia with reduced ejection fraction with or without heart failure be advised of the risks of mortality and morbidity associated with commencing or proceeding with a pregnancy.
JUSTIFICATION: Women with Friedreich ataxia with reduced ejection fraction with or without heart failure are at significant risk of mortality and morbidity. Evidence for this is apparent from women with other (non-Friedreich ataxia-related) cardiomyopathies and associated reduced ejection fraction with or without heart failure.
SUBGROUP CONSIDERATION: This recommendation is for women with Friedreich ataxia with reduced ejection fraction with or without heart failure.
Evidence to Recommendation Table PDFLay summary of clinical recommendation for planning pregnancy for women with Friedreich ataxia with reduced ejection fraction
Why this recommendation?
This recommendation suggests that women with Friedreich ataxia with reduced ejection fraction (that is where muscles of the left ventricle of the heart do not pump well) who are either contemplating pregnancy or are pregnant should be advised of the risks of significant health issues for them and their baby if they become pregnant or continue a pregnancy.
What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?
If you are thinking about becoming pregnant or you are already pregnant, it might be important for you to speak with your healthcare professional about Friedreich ataxia and pregnancy and what it means for you.
Who is this recommendation specifically for?
This recommendation is specifically for women with Friedreich ataxia and reduced ejection fraction with or without heart failure.
Aarti Patel, MD
Associate Professor of Medicine, University of South Florida, Tampa, Florida, USA
Email: apatel15@usf.edu
Roger E. Peverill, MBBS, PhD
Cardiologist, MonashHeart, Monash Health, Clayton, Victoria, Australia
Email: roger.peverill@monash.edu
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These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
It is our expectation that going forward individual topics can be updated in real-time in response to new evidence versus a re-evaluation and update of all topics simultaneously.