Cardiac disease in pregnancy and hypertrophic cardiomyopathy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Anjan K. Chakrabarti, M.D. [2]
Overview
Patients with pre-existing cardiomyopathies prior to pregnancy often have significant difficulty dealing with the physiologic and hemodynamic changes that occur during pregnancy, labor, and delivery. In addition to these patients, there is a subset of patients who will develop peripartum cardiomyopathy.
Pre-Existing Cardiomyopathies
Dilated Cardiomyopaty
This section will primarily focus on dilated cardiomyopathy (DCM) in pregnancy. For a more detailed discussion of dilated cardiomyopathy, click here.
Currently, DCM is responsible for "approximately 10,000 deaths and 46,000 hospital stays each year in the United States and is the most common indication for cardiac transplantation."[1] The cause often remains unknown, in up to 50% of cases.[2]
In general, women with dilated cardiomyopathy are advised to avoid pregnancy for the following reasons:
- Dilated cardiomyopathy has been associated with A-type lamin gene defects, which are associated with a high rate of heart failure and life-threatening arrhythmias, as predicted by NYHA functional class[3]
- The increase in intravascular volume and cardiac output during pregnancy leads to a greater risk for complications in women with dilated cardiomyopathy, especially in the 3rd trimester
- A history of cardiac events including previous episodes of heart failure, atrial fibrillation or atrial flutter, transient ischemic attack, or a history of cardiac events before pregnancy is predictive of negative pregnancy-related cardiac outcomes[4]
The following should be considered when managing heart failure during pregnancy:[5]
- Goals are similar to non-pregnant patients, including continuation of chronic therapies (with a few exceptions, including ACE Inhibitors, Angiotensin Receptor Blockers, and Aldosterone antagonists)
- Beta-blockers can and should be continued through pregnancy
- Vasodilator therapy can be achieved with Amlodipine and Hydralazine safely
- Sodium restriction and digoxin can both be used during pregnancy
- In the setting of acute decompansation, intravenous diuretics, vasodilators, and monitoring with right heart cathterization are all acceptable
Finally, the following should be considered during labor and delivery:[5]
- Multi-disciplinary approach is crucial
- Careful maternal continuous monitoring should be employed, including ECG monitoring, non-invasive blood pressure monitoring, and right heart catheterization/arterial line monitoring if necessary
- No official recommendation on timing of delivery is established; coordination between the obstetrician and cardiologist is necessary to deem what is safest for the patient
- Vaginal delivery generally poses less cardiovascular risk than cesarian section (less blood loss)
- Induction of labor with an unfavorable cervix should be avoided; induction with a favorable cervix can be achieved with oxytocin and artificial rupture of membranes
- Epidural anesthesia, can produce changes in preload and afterload that can be advantageous in the setting of reduced ventricular function, and should be considered along with intravenous opiates to provide analgesia and reduce the hemodynamic demands that accompany significant pain
- When in labor, the patient should be placed in a left lateral decubitus position to avoid IVC compression by the gravid uterus, and the first stage of labor should occur without maternal assistance to avoid the hemodynamic effects of the Valsalva maneuver
- The second stage of labor can be shortened via assistance with low forceps or by vacuum extraction as needed.
Hypertrophic Cardiomyopathy
This section will primarily focus on hypertrophic cardiomyopathy (HCM) in pregnancy. For a more detailed discussion of hypertrophic cardiomyopathy, click here.
HCM is an autosomal dominant disease due to mutations of the cardiac sarcomere proteins, which result in asymmetric hypertrophy of the left ventricle, a nondilated left ventricular cavity, and preserved systolic function with impaired diastolic function.[6] Left ventricular outflow tract (LVOT) obstruction is present at rest in approximately 20% of patients.[7]
Although most women with HCM with minimal or mild symptoms tolerate pregnancy well, risks in pregnancy include:[5]
- Sudden death, particularly in patients with severe outflow obstruction or other risk factors for sudden cardiac death
- Hemodynamic deterioration, particularly in those with moderate or severe symptoms before pregnancy
Management considerations during pregnancy, labor, and delivery include:[5]
- Beta-blockers and calcium channel blockers are safe in this population
- Avoid decreases in preload (straining, dehydration, etc.)
- Avoid inotropes and vasodilators
- Tachycardia and reduced preload from Valsalva can both adversely affect the HCM patient, so labor should be in a high-risk center
- Epidural anesthesia should be avoided due to the potential for hypotension
- Prostaglandins for the induction of labor is not advised, secondary to inherent vasodilatory effects
- General anesthesia is often employed, and asopressor agents and fluids can be given to treat hypotension
References
- ↑ Manolio TA, Baughman KL, Rodeheffer R, Pearson TA, Bristow JD, Michels VV; et al. (1992). "Prevalence and etiology of idiopathic dilated cardiomyopathy (summary of a National Heart, Lung, and Blood Institute workshop". Am J Cardiol. 69 (17): 1458–66. PMID 1590237.
- ↑ Felker GM, Thompson RE, Hare JM, Hruban RH, Clemetson DE, Howard DL; et al. (2000). "Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy". N Engl J Med. 342 (15): 1077–84. doi:10.1056/NEJM200004133421502. PMID 10760308.
- ↑ Pasotti M, Klersy C, Pilotto A, Marziliano N, Rapezzi C, Serio A; et al. (2008). "Long-term outcome and risk stratification in dilated cardiolaminopathies". J Am Coll Cardiol. 52 (15): 1250–60. doi:10.1016/j.jacc.2008.06.044. PMID 18926329.
- ↑ Siu SC, Colman JM, Sorensen S, Smallhorn JF, Farine D, Amankwah KS; et al. (2002). "Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease". Circulation. 105 (18): 2179–84. PMID 11994252.
- ↑ 5.0 5.1 5.2 5.3 Stergiopoulos K, Shiang E, Bench T (2011). "Pregnancy in patients with pre-existing cardiomyopathies". J Am Coll Cardiol. 58 (4): 337–50. doi:10.1016/j.jacc.2011.04.014. PMID 21757110.
- ↑ Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi P; et al. (2002). "Risk associated with pregnancy in hypertrophic cardiomyopathy". J Am Coll Cardiol. 40 (10): 1864–9. PMID 12446072.
- ↑ Spirito P, Autore C (2006). "Management of hypertrophic cardiomyopathy". BMJ. 332 (7552): 1251–5. doi:10.1136/bmj.332.7552.1251. PMC 1471918. PMID 16735335.