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CardioNerd Amit Goyal, Cardio-OB series co-chair and University of Texas Southwestern Cardiology Fellow, Dr. Sonia Shah, and episode lead and Johns Hopkins University Cardiology Fellow, Dr. Anum Minhas, discuss pregnancy and aortic disorders with Dr. Nupoor Narula of Weill Cornell Medical College. Special introduction by Sukrit Narula.

In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery.

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Abstract • Pearls • Quotables • Notes • References • Guest Profiles • Production Team

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Episode Abstract – Pregnancy and Aortic Disorders

In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery.

Pearls – Pregnancy and Aortic Disorders

1. Assessment of aortic root and ascending aortic measurements should be performed prior to conception in women with known aortopathies, connective tissue diseases with high risk for aortopathies, bicuspid aortic valve or familial thoracic aortic syndromes. Dimensions should always be verified with multi-modality imaging prior to decision-making.

2. It is important to recognize that the immediate postpartum period is a high risk period for aortic dissection in women with aortopathies.

3. Goal systolic blood pressure is < 120 mmHg and diastolic blood pressure is < 80 mmHg in women with aortopathies. This goal should be discussed with the patient’s obstetric provider during pregnancy, however, to ensure a blood pressure target is chosen that is appropriate for optimal maternal and fetal outcomes. Beta-blockers followed by calcium channel blockers should be used for these targets. Aldosterone receptor antagonists are contraindicated during pregnancy.

4. Per the 2018 ESC guidelines, during pregnancy, women with aortic pathology should have an echocardiogram performed every 12 weeks if low risk, and every month (4 weeks) if high risk.

5. Women with type A dissection during pregnancy should be evaluated for urgent Caesarean section and aortic surgery if the fetus is viable. Women with a stable type B dissection may be managed medically if stable. All decisions should be made in a multidisciplinary fashion with consultation with Maternal Fetal Medicine and Cardiothoracic Surgery.

Quotables – Pregnancy and Aortic Disorders

1. “First and foremost, we must verify our dimensions. I’ll say that again. We must verify our dimensions because nothing could have greater impact.”

2. “You know, our most important role is to provide transparency in pre-pregnancy risk counseling. We have to present the data that are available. We need to present the gaps in literature.  We need to present the possibility that in the right individual, we can proceed through pregnancy safely with close clinical and imaging monitoring and follow up in a multidisciplinary construct.

Show notes – Pregnancy and Aortic Disorders

1. What is the risk of aortic dissection during pregnancy in Marfan syndrome?
   • Women who undergo pregnancy with prepartum counseling and close clinical and imaging follow-up usually maintain stability of aortic dimensions over time and risk of type A dissection appears low.
   • Type B dissection remains unpredictable.
   • Overall, the risk of aortic complications surrounding pregnancy remains eight-fold higher than in the non-pregnant state. The risk for dissection is higher with greater aortic root size, but there still remains a risk for dissection at diameters less than 40 mm.
   • Note, in the GenTAC registry (which included 184 women with Marfan’s Syndrome in whom pregnancy information was available), lack of knowledge of the underlying diagnosis of Marfan’s was not uncommon.

2. What is the underlying pathophysiology behind increased risk for aortic dissection during pregnancy?
   • Pregnancy is associated with both hemodynamic changes and alterations in tissue architecture which may both increase the risk of exacerbating an underlying aortopathy syndrome.
   • Physiologic changes during pregnancy include increased stroke volume and heart rate, resulting in increased cardiac output, with concomitant decrease in the peripheral resistance, particularly in the second and third trimesters and immediate postpartum period (Review our Initial Episode in the Cardio-Ob Series for more details).
   • Pregnancy-associated morphologic and histochemical changes of the aortic wall also occur.
   • In women with Marfan syndrome, at baseline aortic tissue tends to be weaker and more prone to dilatation and dissection.
   • Animal studies also implicate oxytocin in increasing dissection risk, with a suggestion that withholding lactation or administration of an oxytocin receptor antagonist may reduce dissection risk.

3. For patients with suspected or confirmed aortopathy or familial aortopathy syndromes, what evaluation is recommended prior to and during pregnancy?
   • Complete physical exam, including cardiac and vascular exams, should be performed, as well as review of personal and family medical/surgical history.
   • Echocardiogram should be performed to inform the safety of pregnancy.  Echocardiogram can be used to obtain aortic dimensions and identify any co-existing disease prior to conception.
   • Per the 2018 ESC guidelines, during pregnancy, women with aortic pathology should have echocardiogram performed every 12 weeks if low risk, and every month (4 weeks) if high risk.
   • Beta-blockers throughout pregnancy should be considered, along with fetal growth monitoring. 
   • While aldosterone receptor antagonists may normally be advised, these are contraindicated during pregnancy due to their teratogenic effects and should be withheld.
   • Collaboration with colleagues in Maternal Fetal Medicine for management decisions is critical.

4. What are some specific syndromes related to aortic pathology and the risks of each of these conditions?
   • Marfan syndrome
     • AHA/ACC guidelines recommend consideration of elective aortic surgery prior to pregnancy if aortic root is > 4.0 cm.
     • The 2018 ESC guidelines advise against pregnancy for aortic root diameter > 4.5 cm.
     • The decision to intervene prophylactically during pregnancy is highly individualized and complex, and we must weight patient factors including gestational age and rapidity of growth while balancing risk/benefits to the fetus and mother. 
   • Ehlers-Danlos syndrome (EDS)
     • Vascular EDS patients are at high risk of arterial and uterine rupture, and the 2018 ESC Guidelines recommend against pregnancy. If after shared-decision making a patient decides to proceed with pregnancy, the patient should be cared for in a specialized center with a multi-disciplinary Cardio-Ob team.
   • Loeys-Dietz syndrome
     • There is limited data is available, and it is insufficient to make any clear recommendations regarding pregnancy. Most centers will generally approach prophylactic aortic root surgery in Loeys-Dietz syndrome similar to Marfan disease and consider elective repair at 4.0 to 4.5 cm.
     • More frequent intrapartum echocardiograms may be considered given some suggestion of increased risk of dilatation during pregnancy.
   • Turner Syndrome
     • There is a strong association with bicuspid aortic valve (present in 15-30% of patients). Generally, we consider elective surgery in patients with an aortic size index (ASI) (ascending aortic root size indexed to body surface area) of > 2.5 cm/m² and that pregnancy should be avoided at this ASI cutoff. During pregnancy, prophylactic aortic surgery can be considered with a dilated aorta (> 2.5 cm/m² or rapid enlargement > 3mm)
     • Many women with Turner syndrome require assisted reproductive therapy (ART) and assisted reproductive therapy may increase the risk of cardiovascular complications.
     • Imaging of the thoracic aorta and heart are recommended within two years prior to pregnancy or assisted reproductive therapy.
     • Imaging should be performed at least once per 20 weeks if there is no increased ASI or other cardiovascular risk factors (e.g., family history of dissection or sudden death, bicuspid aortic valve, coarctation, elongation of the transverse arch).
     • Imaging should be performed every 4-8 weeks for those with ASI > 2.0 cm/m² or additional risk factors during pregnancy, and once during the first month after delivery. Subsequent imaging intervals depend on the severity of the aortic enlargement.
   • Bicuspid aortic valve (BAV)
     • Up to 50% of patients with BAV also have ascending aortic dilatation
     • ESC guidelines recommend elective aortic surgery if the ascending aorta is > 5.0 cm prior to pregnancy.
   • Familial Thoracic aortic aneurysms
     • Family history is present in 20% of patients.
     • A series in 53 women with ACTA2 mutations and total of 137 pregnancies showed 8 pregnancy-associated dissections (6 were type A) .
     • Evidence is limited, however, and no clear recommendations for pregnancy are available.

5. What are some delivery considerations for women with aortopathies?
   • The overall goal is to decrease the cardiovascular stress of the delivery process, and risk for both the mother and baby need to be considered jointly with the Maternal Fetal Medicine team.
   • For Marfan Syndrome and aortic root between 4-4.5 cm, vaginal delivery with an expedited second stage and regional anesthesia is typically the preferred mode of delivery to avoid spikes in blood pressure.
   • For patients with vascular EDS, consider C-section at 35-37 weeks’ gestation. There is a similar recommendation for patients with Marfan syndrome and aortic root > 4.5 cm and Turner syndrome patients with ASI > 2.5 cm/m².
   • For those with a type A dissection during pregnancy the approach depends on fetal viability. If viable, then urgent C-section and surgical aortic repair should be performed. If the fetus is currently non-viable, aortic repair is performed without delivery, acknowledging the high fetal risk in this context.
   • The approach to unstable type B dissection is as for type A dissections as above with C-section and aortic surgery if the fetus is viable or aortic surgery without deliver if the fetus is not viable at the time.  For stable type B dissection, medical therapy can be pursued.

References

Regitz-Zagrosek V et al. 2018 ESC Guidelines for the Management of Cardiovascular Diseases during Pregnancy: The Task Force for the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology. European Heart Journal. 2018;39(34):3165-3241. https://academic.oup.com/eurheartj/article/39/34/3165/5078465#186438440

Mehta et al. Cardiovascular Considerations in Caring for Pregnant Patients: A Scientific Statement from the American Heart Association. Circulation. 2020;141:e884–e903. https://www.ahajournals.org/doi/suppl/10.1161/CIR.0000000000000772

Guest Profiles

CardioNerds Cardioobstetrics Production Team

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