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| {{Pregnancy and heart disease}}
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| {{CMG}}; '''Associate Editor-In-Chief:''' {{AC}}
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| ==Overview==
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| [[Pulmonary hypertension]], defined as mean pulmonary artery pressure of greater than 25 mmHg at rest or 30 mmHg with exercise, carries a higher mortality when it is associated with pregnancy. It carries a significant risk to mother and child during pregnancy; as a result, mothers require careful monitoring.<ref name="pmid19223169">{{cite journal| author=Madden BP| title=Pulmonary hypertension and pregnancy. | journal=Int J Obstet Anesth | year= 2009 | volume= 18 | issue= 2 | pages= 156-64 | pmid=19223169 | doi=10.1016/j.ijoa.2008.10.006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19223169 }} </ref>
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| ==Physiologic Considerations in Pregnancy==
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| As reviewed in [[Pregnancy and heart disease pathophysiology|Physiologic Changes Associated with Pregnancy]], maternal blood volume increases throughout pregnancy until between 28 and 34 weeks of gestation, and circulating blood volume is increased to between 30% and 50% above the non-pregnant state. Red blood cell mass increases to approximately 25% above the non-pregnant state. [[Cardiac output]] increases through various mechanisms, and [[hypercoagulability]] is noted in the postpartum state due to relative resistance to activated [[protein C]], reduced serum levels of [[protein S]] and increased levels of factors I, II V, VII, VIII, X and XII.<ref name="pmid14484810">{{cite journal| author=PECHET L, ALEXANDER B| title=Increased clotting factors in pregnacy. | journal=N Engl J Med | year= 1961 | volume= 265 | issue= | pages= 1093-7 | pmid=14484810 | doi=10.1056/NEJM196111302652205 | pmc= | url= }} </ref>
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| All of these changes can be particularly deleterious in patients with [[PAH]]. It can be very harmful if a thrombus forms or embolizes to an already compromised pulmonary circulation. Such hematological changes present a significant risk, and mortality is between 30% and 50% for pregnant women with idiopathic [[PAH]].<ref name="pmid9626847">{{cite journal| author=Weiss BM, Zemp L, Seifert B, Hess OM| title=Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. | journal=J Am Coll Cardiol | year= 1998 | volume= 31 | issue= 7 | pages= 1650-7 | pmid=9626847 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9626847 }} </ref>
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| ==Specific Issues with PAH and Pregnancy==
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| *Longterm elevation of pulmonary vascular resistance may cause [[right ventricular hypertrophy]] or dilatation, [[tricuspid regurgitation]] or arrhythmias, leading to intolerance of the increased heart rate and circulating blood volume of pregnancy.
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| *[[Cardiac output]] may already be reduced by pulmonary hypertension, and the heart may not be able to increase cardiac output in proportion to the reduced [[systemic vascular resistance]] that occurs during pregnancy.
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| *Normal adaptive changes in the lungs to accommodate the increased pulmonary blood flow may be prevented by fixed vascular remodeling.
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| *[[Hypercoagulability]] may increase the tendency for thrombus formation, which may be poorly tolerated (as above).
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| *Patients are at risk for [[sudden cardiac death]] from a pulmonary hypertensive crises, malignant [[arrhythmias]] or [[pulmonary thromboembolism]].
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| *Patients can sustain a [[stroke]] as a result of intracardiac shunting in pre-existing [[Eisenmenger's syndrome|Eisenmenger’s syndrome]] or if there is a persistent [[patent foramen ovale]]<ref name="pmid11108754">{{cite journal| author=Jaigobin C, Silver FL| title=Stroke and pregnancy. | journal=Stroke | year= 2000 | volume= 31 | issue= 12 | pages= 2948-51 | pmid=11108754 | doi= | pmc= | url= }} </ref>
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| ==Outcomes and Recommendations==
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| In a review of [[PAH]] and pregnancy outcomes between 1978 and 1996, Weiss and colleagues<ref name="pmid9626847">{{cite journal| author=Weiss BM, Zemp L, Seifert B, Hess OM| title=Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. | journal=J Am Coll Cardiol | year= 1998 | volume= 31 | issue= 7 | pages= 1650-7 | pmid=9626847 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9626847 }} </ref> found a maternal mortality rate of 30% in idiopathic PAH, 36% in Eisenmenger’s syndrome and 56% in pulmonary hypertension secondary to a variety of other conditions including liver disease, connective tissue disease, chronic thromboembolic events and ingestion of weight loss agents. Of note, the highest mortality was in the first month after delivery.
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| As a result, many authors recommend pregnancy should be avoided in the setting of maternal PAH.<ref name="pmid10637084">{{cite journal| author=Weiss BM, Hess OM| title=Pulmonary vascular disease and pregnancy: current controversies, management strategies, and perspectives. | journal=Eur Heart J | year= 2000 | volume= 21 | issue= 2 | pages= 104-15 | pmid=10637084 | doi=10.1053/euhj.1999.1701 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10637084 }} </ref>. See guidelines below.
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| #REDIRECT [[Cardiac disease in pregnancy and pulmonary hypertension]] | | #REDIRECT [[Cardiac disease in pregnancy and pulmonary hypertension]] |
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| ==Management of Patients with PAH During Pregnancy==
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| A multidisciplinary team should be involved in management, and specific therapeutic options include:
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| *Oxygen to maintain maternal PaO2 above 70 mmHg
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| *[[Diuretics]] for patients with [[PAH]] who develop [[right heart failure]], under careful supervision<ref name="pmid12790853">{{cite journal| author=Al-Mobeireek AF, Almutawa J, Alsatli RA| title=The nineteenth pregnancy in a patient with cor pulmonale and severe pulmonary hypertension: a management challenge. | journal=Acta Obstet Gynecol Scand | year= 2003 | volume= 82 | issue= 7 | pages= 676-8 | pmid=12790853 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12790853 }} </ref>
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| *Anticoagulation, often with the assistance of a hematologist. The agent of choice is a [[low-molecular-weight heparin]] with serial measurement of anti-factor Xa levels.<ref name="pmid15383488">{{cite journal| author=Bates SM, Greer IA, Hirsh J, Ginsberg JS| title=Use of antithrombotic agents during pregnancy: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy. | journal=Chest | year= 2004 | volume= 126 | issue= 3 Suppl | pages= 627S-644S | pmid=15383488 | doi=10.1378/chest.126.3_suppl.627S | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15383488 }} </ref>
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| *Pulmonary vasoactive agents:
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| :#Endothelin receptor antagonists are contraindicated during pregnancy because of risks of teratogenicity. Alternative agents include:
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| :#[[Sildenafil]]<ref name="pmid15121629">{{cite journal| author=Lacassie HJ, Germain AM, Valdés G, Fernández MS, Allamand F, López H| title=Management of Eisenmenger syndrome in pregnancy with sildenafil and L-arginine. | journal=Obstet Gynecol | year= 2004 | volume= 103 | issue= 5 Pt 2 | pages= 1118-20 | pmid=15121629 | doi=10.1097/01.AOG.0000125148.82698.65 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15121629 }} </ref>
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| :#Intravenous [[Epoprostenol]] ([[Prostacyclin]])<ref name="pmid10214821">{{cite journal| author=Easterling TR, Ralph DD, Schmucker BC| title=Pulmonary hypertension in pregnancy: treatment with pulmonary vasodilators. | journal=Obstet Gynecol | year= 1999 | volume= 93 | issue= 4 | pages= 494-8 | pmid=10214821 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10214821 }} </ref>
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| :#In the acute situation, inhaled [[Nitric Oxide]]<ref name="pmid11704193">{{cite journal| author=Lam GK, Stafford RE, Thorp J, Moise KJ, Cairns BA| title=Inhaled nitric oxide for primary pulmonary hypertension in pregnancy. | journal=Obstet Gynecol | year= 2001 | volume= 98 | issue= 5 Pt 2 | pages= 895-8 | pmid=11704193 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11704193 }} </ref>
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| *Atrial septostomy<ref name="pmid1722434">{{cite journal| author=Nihill MR, O'Laughlin MP, Mullins CE| title=Effects of atrial septostomy in patients with terminal cor pulmonale due to pulmonary vascular disease. | journal=Cathet Cardiovasc Diagn | year= 1991 | volume= 24 | issue= 3 | pages= 166-72 | pmid=1722434 | doi= | pmc= | url= }} </ref>
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| ==References==
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| {{Reflist|2}}
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| [[Category:Cardiology]]
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| [[Category:Obstetrics]]
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| [[Category:Disease]]
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| {{WH}}
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| {{WS}}
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