Cardiac disease in pregnancy and hypertrophic cardiomyopathy: Difference between revisions

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Revision as of 19:46, 18 April 2012

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Anjan K. Chakrabarti, M.D. [2]

Overview

Patients with pre-existing cardiomyopathies such as dilated cardiomyopathy and hypertrophic cardiomyopathy, 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.

Pathophysiology

Hypertrophic cardiomyopathy (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[[.^[1]

Left ventricular outflow tract obstruction (LVOT) is present at rest in approximately 20% of patients.^[2]

Complications

Although most women with HCM with minimal or mild symptoms tolerate pregnancy well, risks in pregnancy include:^[3]

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.

Treatment

Management considerations during pregnancy, labor, and delivery include:^[3]

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.

Related Chapter

This section will primarily focus on hypertrophic cardiomyopathy (HCM) in pregnancy. For a more detailed discussion of hypertrophic cardiomyopathy, click here.

References

↑ 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.CS1 maint: PMC format (link)
↑ ^3.0 ^3.1 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.

Template:WH Template:WS

[pmid12446072-1] 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.

[pmid16735335-2] 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.CS1 maint: PMC format (link)

[pmid21757110-3] 3.0 ^3.1 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.

[1]

[2]

[3]

@@ Line 6: / Line 6: @@
 Patients with pre-existing [[Cardiomyopathy|cardiomyopathies]] such as [[dilated cardiomyopathy]] and [[HOCM|hypertrophic cardiomyopathy]], 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|peripartum cardiomyopathy]].
-==Dilated Cardiomyopathy==
+==Pathophysiology==
-====Incidence====
+[[Hypertrophic cardiomyopathy]] ([[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[[.<ref name="pmid12446072">{{cite journal| author=Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi P et al.| title=Risk associated with pregnancy in hypertrophic cardiomyopathy. | journal=J Am Coll Cardiol | year= 2002 | volume= 40 | issue= 10 | pages= 1864-9 | pmid=12446072 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12446072  }} </ref>
-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."<ref name="pmid1590237">{{cite journal| author=Manolio TA, Baughman KL, Rodeheffer R, Pearson TA, Bristow JD, Michels VV et al.| title=Prevalence and etiology of idiopathic dilated cardiomyopathy (summary of a National Heart, Lung, and Blood Institute workshop. | journal=Am J Cardiol | year= 1992 | volume= 69 | issue= 17 | pages= 1458-66 | pmid=1590237 | doi= | pmc= | url= }} </ref>
-====Cause====
+[[Left ventricular outflow tract obstruction]] ([[LVOT]]) is present at rest in approximately 20% of patients.<ref name="pmid16735335">{{cite journal| author=Spirito P, Autore C| title=Management of hypertrophic cardiomyopathy. | journal=BMJ | year= 2006 | volume= 332 | issue= 7552 | pages= 1251-5 | pmid=16735335 | doi=10.1136/bmj.332.7552.1251 | pmc=PMC1471918 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16735335  }} </ref>
-The cause often remains unknown, in up to 50% of cases.<ref name="pmid10760308">{{cite journal| author=Felker GM, Thompson RE, Hare JM, Hruban RH, Clemetson DE, Howard DL et al.| title=Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. | journal=N Engl J Med | year= 2000 | volume= 342 | issue= 15 | pages= 1077-84 | pmid=10760308 | doi=10.1056/NEJM200004133421502 | pmc= | url= }} </ref>
-====Reasons why Women with Dilated Cardiomyopathy are Advised to Avoid Pregnancy====
-*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<ref name="pmid18926329">{{cite journal| author=Pasotti M, Klersy C, Pilotto A, Marziliano N, Rapezzi C, Serio A et al.| title=Long-term outcome and risk stratification in dilated cardiolaminopathies. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 15 | pages= 1250-60 | pmid=18926329 | doi=10.1016/j.jacc.2008.06.044 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18926329  }} </ref>
-*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<ref name="pmid11994252">{{cite journal| author=Siu SC, Colman JM, Sorensen S, Smallhorn JF, Farine D, Amankwah KS et al.| title=Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease. | journal=Circulation | year= 2002 | volume= 105 | issue= 18 | pages= 2179-84 | pmid=11994252 | doi= | pmc= | url= }} </ref>
-====Management of Heart Failure====
-The following should be considered when managing [[heart failure]] during pregnancy:<ref name="pmid21757110">{{cite journal| author=Stergiopoulos K, Shiang E, Bench T| title=Pregnancy in patients with pre-existing cardiomyopathies. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 4 | pages= 337-50 | pmid=21757110 | doi=10.1016/j.jacc.2011.04.014 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21757110  }} </ref>
-*Goals are similar to non-pregnant patients, including continuation of chronic therapies (with a few exceptions, including [[Congestive heart failure ACE inhibitors|ACE inhibitors]], [[Congestive heart failure angiotensin receptor blockers|angiotensin receptor blockers]], and [[Congestive heart failure aldosterone antagonists|aldosterone antagonists]]).
-*[[Congestive heart failure beta blockers|Beta-blockers]] can and should be continued through pregnancy.
-*[[Congestive heart failure vasodilators|Vasodilator therapy]] can be safely achieved with [[amlodipine]] and [[hydralazine]].
-*Sodium restriction and [[Congestive heart failure positive inotropics|digoxin]] can both be used during pregnancy.
-*In the setting of acute decompansation, [[Congestive heart failure diuretics|intravenous diuretics]], [[Congestive heart failure vasodilators|vasodilators]], and monitoring with [[Congestive heart failure invasive monitoring|right heart cathterization]] are all acceptable.
-====Management during Labor & Delivery====
-Finally, the following should be considered during labor and delivery:<ref name="pmid21757110">{{cite journal| author=Stergiopoulos K, Shiang E, Bench T| title=Pregnancy in patients with pre-existing cardiomyopathies. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 4 | pages= 337-50 | pmid=21757110 | doi=10.1016/j.jacc.2011.04.014 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21757110  }} </ref>
-*Multi-disciplinary approach is crucial.
-*Careful maternal continuous monitoring should be employed, including [[EKG|EKG 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 [[left ventricular dysfunction|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|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.
-====Related Chapter====
-This section will primarily focus on [[dilated cardiomyopathy]] ([[DCM]]) in pregnancy. For a more detailed discussion of dilated cardiomyopathy, click [[Dilated Cardiomyopathy|here]].
-===[[Hypertrophic Cardiomyopathy|Hypertrophic Cardiomyopathy]]===
-This section will primarily focus on hypertrophic cardiomyopathy (HCM) in pregnancy.  For a more detailed discussion of hypertrophic cardiomyopathy, click [[Hypertrophic Cardiomyopathy|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.<ref name="pmid12446072">{{cite journal| author=Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi P et al.| title=Risk associated with pregnancy in hypertrophic cardiomyopathy. | journal=J Am Coll Cardiol | year= 2002 | volume= 40 | issue= 10 | pages= 1864-9 | pmid=12446072 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12446072  }} </ref>  Left ventricular outflow tract (LVOT) obstruction is present at rest in approximately 20% of patients.<ref name="pmid16735335">{{cite journal| author=Spirito P, Autore C| title=Management of hypertrophic cardiomyopathy. | journal=BMJ | year= 2006 | volume= 332 | issue= 7552 | pages= 1251-5 | pmid=16735335 | doi=10.1136/bmj.332.7552.1251 | pmc=PMC1471918 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16735335  }} </ref>
-Although most women with HCM with minimal or mild symptoms tolerate pregnancy well, risks in pregnancy include:<ref name="pmid21757110">{{cite journal| author=Stergiopoulos K, Shiang E, Bench T| title=Pregnancy in patients with pre-existing cardiomyopathies. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 4 | pages= 337-50 | pmid=21757110 | doi=10.1016/j.jacc.2011.04.014 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21757110  }} </ref>
-*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
+==Complications==
+Although most women with [[HCM]] with minimal or mild symptoms tolerate pregnancy well, risks in pregnancy include:<ref name="pmid21757110">{{cite journal| author=Stergiopoulos K, Shiang E, Bench T| title=Pregnancy in patients with pre-existing cardiomyopathies. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 4 | pages= 337-50 | pmid=21757110 | doi=10.1016/j.jacc.2011.04.014 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21757110  }} </ref>
+*[[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.
+==Treatment==
 Management considerations during pregnancy, labor, and delivery include:<ref name="pmid21757110">{{cite journal| author=Stergiopoulos K, Shiang E, Bench T| title=Pregnancy in patients with pre-existing cardiomyopathies. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 4 | pages= 337-50 | pmid=21757110 | doi=10.1016/j.jacc.2011.04.014 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21757110  }} </ref>
-*Beta-blockers and calcium channel blockers are safe in this population
+*[[Beta-blockers]] and [[calcium channel blockers]] are safe in this population.
-*Avoid decreases in preload (straining, dehydration, etc.)
+*Avoid decreases in [[preload]] (straining, dehydration, etc.).
-*Avoid inotropes and vasodilators
+*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
+*[[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
+*[[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
+*[[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
+*[[General anesthesia]] is often employed, and asopressor agents and fluids can be given to treat [[hypotension]].
+==Related Chapter==
+*This section will primarily focus on [[hypertrophic cardiomyopathy]] ([[HCM]]) in pregnancy. For a more detailed discussion of hypertrophic cardiomyopathy, click [[Hypertrophic Cardiomyopathy|here]].
 ==References==
 {{Reflist|2}}
 [[Category:Cardiology]]
 [[Category:Obstetrics]]
 [[Category:Disease]]
 {{WH}}
 {{WS}}