View source for Cardiac disease in pregnancy and repaired congenital heart disease

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{{Pregnancy and heart disease}}

{{CMG}}; '''Associate Editor-In-Chief:'''  {{AC}}; '''Assistant Editor-In Chief:''' Ara Tachjian, MD [mailto:ara_tt@yahoo.com]

==Overview==
Despite successful repair of congenital heart disease, many patients will still have a residual defect. As a result, careful planning should be undertaken prior to pregnancy.<ref name="pmid8205680">{{cite journal| author=Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F| title=Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. | journal=Circulation | year= 1994 | volume= 89 | issue= 6 | pages= 2673-6 | pmid=8205680 | doi= | pmc= | url= }} </ref> <ref name="pmid10026351">{{cite journal| author=Zuber M, Gautschi N, Oechslin E, Widmer V, Kiowski W, Jenni R| title=Outcome of pregnancy in women with congenital shunt lesions. | journal=Heart | year= 1999 | volume= 81 | issue= 3 | pages= 271-5 | pmid=10026351 | doi= | pmc=PMC1728980 | url= }} </ref><ref name="pmid19038677">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e1-121 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref>
==Repaired Congenital Heart Disease==
===Tetralogy of Fallot===
Individuals with [[TOF]] usually present very early in life with [[cyanosis]] making it necessary to undergo surgical repair. The repair is is almost always done during infancy, particularly in developed countries. During the surgery, the [[pulmonary valve]] and [[right ventricular outflow tract]] are both surgically dilated.  This usually leads to [[pulmonary valve regurgitation]] as well as [[arrhythmias]] originating from the right side of the heart.  Therefore, subsequent [[right sided heart failure]] is sometimes observed.  20 years following repair of [[Tetralogy of Fallot]], 10-15% of patients develop late complications, the most important of which is [[right sided heart failure]]. <ref name="pmid15894783">{{cite journal| author=Meijer JM, Pieper PG, Drenthen W, Voors AA, Roos-Hesselink JW, van Dijk AP et al.| title=Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot. | journal=Heart | year= 2005 | volume= 91 | issue= 6 | pages= 801-5 | pmid=15894783 | doi=10.1136/hrt.2004.034108 | pmc=PMC1768963 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15894783  }} </ref> <ref name="pmid7688102">{{cite journal| author=Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM et al.| title=Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. | journal=N Engl J Med | year= 1993 | volume= 329 | issue= 9 | pages= 593-9 | pmid=7688102 | doi=10.1056/NEJM199308263290901 | pmc= | url= }} </ref> <ref name="pmid9350942">{{cite journal| author=Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B| title=Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. | journal=J Am Coll Cardiol | year= 1997 | volume= 30 | issue= 5 | pages= 1374-83 | pmid=9350942 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9350942  }} </ref>

Some [[TOF]] patients will have a palliative surgically-created systemic-pulmonary shunt such as [[Blalock-Taussig]] or Waterston, but without corrective surgery.<ref name="pmid15894783">{{cite journal| author=Meijer JM, Pieper PG, Drenthen W, Voors AA, Roos-Hesselink JW, van Dijk AP et al.| title=Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot. | journal=Heart | year= 2005 | volume= 91 | issue= 6 | pages= 801-5 | pmid=15894783 | doi=10.1136/hrt.2004.034108 | pmc=PMC1768963 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15894783  }} </ref> In such situations, pregnancy might worsen an already existing right-to-left shunt (through a [[VSD]]) by reducing the systemic vascular resistance, leading to worsening [[cyanosis]].  In previous studies, maternal NYHA functional class > 2 and [[cyanosis]] are identified as independent predictors of both maternal and fetal complications. <ref name="pmid9386139">{{cite journal| author=Siu SC, Sermer M, Harrison DA, Grigoriadis E, Liu G, Sorensen S et al.| title=Risk and predictors for pregnancy-related complications in women with heart disease. | journal=Circulation | year= 1997 | volume= 96 | issue= 9 | pages= 2789-94 | pmid=9386139 | doi= | pmc= | url= }} </ref>Therefore, worsening cyanosis is not favored in such patients and pregnancy should be avoided.

Elements to be evaluated for through history, physical exam, EKG and echocardiogram prior to pregnancy (in women with surgical correction):
1. Residual [[pulmonary regurgitation]] and severity
2. Residual [[right ventricular outflow tract obstruction]]
3. [[Right sided heart failure]]
4. [[Tricuspid regurgitation]]
5. Atrial or [[ventricular arrhythmias]]
6. [[VSD]] 

Generally, it is accepted that most women with corrected TOF can have non-restricted lifestyle and tolerate pregnancy. However, they still need to be evaluated by a cardiologist prior to and during pregnancy. Women with severe pulmonary regurgitation as a complication of the corrective surgery might benefit from pulmonary valve replacement prior to pregnancy to reduce possibility of complications. <ref name="pmid15894783">{{cite journal| author=Meijer JM, Pieper PG, Drenthen W, Voors AA, Roos-Hesselink JW, van Dijk AP et al.| title=Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot. | journal=Heart | year= 2005 | volume= 91 | issue= 6 | pages= 801-5 | pmid=15894783 | doi=10.1136/hrt.2004.034108 | pmc=PMC1768963 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15894783  }} </ref>

==References==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Obstetrics]]
[[Category:Cardiology board review]]