Abstract
Pulmonary arterial hypertension (PAH) is a disease of unknown etiology that affects young and middle-aged woman with greater frequency than the rest of the population (1). It is characterized by an elevation in pulmonary vascular resistance (PVR) and a decrease in cardiac output (CO) that results in right-sided heart failure and reduced exercise capacity. Without treatment, it usually progresses to right heart failure and death. Recently developed therapies have improved exercise capacity and prolonged survival, but a cure for this debilitating disease does not yet exist. Fortunately, the disease is extremely rare. Unfortunately it usually strikes without warning and occurs most often in women who are otherwise healthy.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987;107:216–223.
Barnes PJ, Liu SF. Regulation of pulmonary vascular tone. Pharmacol Rev 1995;47:87–131.
Michelsen S, Otterstad JE. Blood pressure response during maximal exercise in apparently healthy men and women. J Intern Med 1990;227:157–163.
Hakim TS. Flow-induced release of EDRF in the pulmonary vasculature: site of release and action. Am J Physiol 1994;267: H363–H369.
Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987;107:216–223.
Weir EK, Olschewski A. Role of ion channels in acute and chronic responses of the pulmonary vasculature to hypoxia. Cardiovasc Res 2006;71:630–641.
Tandon HD, Kasturi J. Pulmonary vascular changes associated with isolated mitral stenosis in India. Br Heart J 1975;37:26–36.
Romberg E. Uebere Sklerose Der Lungenarterien. Dtsch Arch Klin Med 1981;48:197–206.
Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med 1951;11:686–705.
Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43:5S–12S.
Lee SD, Shroyer KR, Markham NE, Cool CD, Voelkel NF, Tuder RM. Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension. J Clin Invest 1998;101:927–934.
Palevsky HI, Schloo BL, Pietra GG, Weber KT, Janicki JS, Rubin E, Fishman AP. Primary pulmonary hypertension. Vascular structure, morphometry, and responsiveness to vasodilator agents. Circulation 1989;80:1207–1221.
Lumb AB. Pregnancy, neonates and children. In: Lumb AB ed. Nunn’s Applied Respiratory Physiology. New York: Elsevier Limited, 2005:229–230.
Weiss BM, Maggiorini M, Jenni R, Lauper U, Popov V, Bombeli T, Spahn DR. Pregnant patient with primary pulmonary hypertension: inhaled pulmonary vasodilators and epidural anesthesia for cesarean delivery. Anesthesiology 2000;92:1191–1194.
Robson SC, Hunter S, Boys RJ, Dunlop W. Serial changes in pulmonary haemodynamics during human pregnancy: a non-invasive study using Doppler echocardiography. Clin Sci 1991;80:113–117.
Dawkins KD, Burke CM, Billingham ME, Jamieson SW. Primary pulmonary hypertension and pregnancy. Chest 1986;89:383–388.
Wagenvoort CA and Wagenvoort N. Primary pulmonary hypertension. A pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation 1970;42:1163–1182.
Rubin LJ. Primary pulmonary hypertension. Chest 1993;104:236–250.
Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998;31:1650–1657.
Bitsch M, Johansen C, Wennevold A, Osler M. Maternal heart disease. A survey of a decade in a Danish university hospital. Acta Obstet Gynecol Scand 1989;68:119–124.
Okada K, Tanaka Y, Bernstein M, Zhang W, Patterson GA, Botney MD. Pulmonary hemodynamics modify the rat pulmonary artery response to injury. A neointimal model of pulmonary hypertension. Am J Pathol 1997;151:1019–1025.
De Canniere D, Stefanidis C, Brimioulle S, Naeije R. Effects of a chronic aortopulmonary shunt on pulmonary hemodynamics in piglets. J Appl Physiol 1994;77:1591–1596.
Karmochkine M, Cacoub P, Dorent R, Laroche P, Nataf P, Piette JC, Boffa MC, Gandjbakhch I. High prevalence of antiphospholipid antibodies in precapillary pulmonary hypertension. J Rheumatol 1996;23:286–290.
Welsh CH, Hassell KL, Badesch DB, Kressin DC, Marlar RA. Coagulation and fibrinolytic profiles in patients with severe pulmonary hypertension. Chest 1996;110:710–717.
Hassell KL. Altered hemostasis in pulmonary hypertension. Blood Coagul Fibrinolysis 1998;9:107–117.
Bjornsson J, Edwards WD. Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc 1985;60:16–25.
Christman BW, McPherson CD, Newman JH, King GA, Bernard GR, Groves BM, Loyd JE. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992;327:70–75.
Tuder, RM, Cool CD, Geraci MW, Wang J, Abman SH, Wright L, Badesch DB, Voelkel NF. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999;159:1925–1932.
Redman CW. Coagulation problems in human pregnancy. Postgrad Med J 1979;55:367–371.
Beller FK, Ebert C. The coagulation and fibrinolytic enzyme system in pregnancy and in the puerperium. Eur J Obstet Gynecol Reprod Biol 1982;13:177–197.
Rutherford S, Montoro M, McGehee W, Strong T. Thromboembolic disease associated with pregnancy: an 11-year review. Am J Obstet Gynecol 1991;164:286–286.
Toglia MR, Weg JG. Venous thromboembolism during pregnancy. N Engl J Med 1996;335:108–114.
Ribeiro A, Lindmarker P, Johnsson H, Juhlin-Dannfelt A, Jorfeldt L. Pulmonary embolism: one-year follow-up with echocardiography Doppler and five-year survival analysis. Circulation 1999;99:1325–1330.
Mata-Greenwood E, Meyrick B, Soifer SJ, Fineman JR, Black SM. Expression of VEGF and its receptors Flt-1 and Flk-1/KDR is altered in lambs with increased pulmonary blood flow and pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol 2003;285:L222–L231.
Mata-Greenwood E, Meyrick B, Steinhorn RH, Fineman JR, Black SM. Alterations in TGF-beta1 expression in lambs with increased pulmonary blood flow and pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol 2003;285:L209–L221.
Wedgwood S, Devol JM, Grobe A, Benavidez E, Azakie A, Fineman JR, Black SM. Fibroblast growth factor-2 expression is altered in lambs with increased pulmonary blood flow and pulmonary hypertension. Pediatr Res 2007;61:32–36.
Black SM, Bekker JM, Johengen MJ, Parry AJ, Soifer SJ, Fineman JR. Altered regulation of the ET-1 cascade in lambs with increased pulmonary blood flow and pulmonary hypertension. Pediatr Res 2000;47:97–106.
Rajakumar A, Michael HM, Rajakumar PA, Shibata E, Hubel CA, Karumanchi SA, Thadhani R, Wolf M, Harger G, Markovic N. Extra-placental expression of vascular endothelial growth factor receptor-1,(Flt-1) and soluble Flt-1 (sFlt-1), by peripheral blood mononuclear cells (PBMCs) in normotensive and preeclamptic pregnant women. Placenta 2005;26:563–573.
Evans PW, Wheeler T, Anthony FW, Osmond C. A longitudinal study of maternal serum vascular endothelial growth factor in early pregnancy. Hum Reprod 1998;13:1057–1062.
Wheeler T, Evans PW, Anthony FW, Godfrey KM, Howe DT, Osmond C. Relationship between maternal serum vascular endothelial growth factor concentration in early pregnancy and fetal and placental growth. Hum Reprod 1999;14:1619–1623.
Giaid A, Yanagisawa M, Langleben D, Michel RP, Levy R, Shennib H, Kimura S, Masaki T, Duguid WP, Stewart DJ. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993;328:1732–1739.
Sturrock A, Cahill B, Norman K, Huecksteadt TP, Hill K, Sanders K, Karwande SV, Stringham JC, Bull DA, Gleich M, Kennedy TP, Hoidal JR. Transforming growth factor-beta1 induces Nox4 NAD(P)H oxidase and reactive oxygen species-dependent proliferation in human pulmonary artery smooth muscle cells. Am J Physiol Lung Cell Mol Physiol 2006;290: L661–L673.
Hirose S, Hosoda Y, Furuya S, Otsuki T, Ikeda E. Expression of vascular endothelial growth factor and its receptors correlates closely with formation of the plexiform lesion in human pulmonary hypertension. Pathol Int 2000;50:472–479.
Benisty JI, McLaughlin VV, Landzberg MJ, Rich JD, Newburger JW, Rich S, Folkman J. Elevated basic fibroblast growth factor levels in patients with pulmonary arterial hypertension. Chest 2004;126:1255–1261.
Rajakumar A, Conrad KP. Expression, ontogeny, and regulation of hypoxia-inducible transcription factors in the human placenta. Biol Reprod 2000;63:559–569.
Cowden Dahl KD, Fryer BH, Mack FA, Compernolle V, Maltepe E, Adelman DM, Carmeliet P, Simon MC. Hypoxia-inducible factors 1alpha and 2alpha regulate trophoblast differentiation. Mol Cell Biol 2005;25:10479–10491.
Fryer BH, Simon MC. Hypoxia, HIF and the placenta. Cell Cycle 2006;5:495–498.
Schultz K, Fanburg BL, Beasley D. Hypoxia and hypoxia-inducible factor-1alpha promote growth factor-induced proliferation of human vascular smooth muscle cells. Am J Physiol Heart Circ Physiol 2006;290: H2528–H2534.
Yamakawa M, Liu LX, Date T, Belanger AJ, Vincent KA, Akita GY, Kuriyama T, Cheng SH, Gregory RJ, Jiang C. Hypoxia-inducible factor-1 mediates activation of cultured vascular endothelial cells by inducing multiple angiogenic factors. Circ Res 2003;93:664–673.
Holmgren L, Glaser A, Pfeifer-Ohlsson S, Ohlsson R. Angiogenesis during human extraembryonic development involves the spatiotemporal control of PDGF ligand and receptor gene expression. Development 1991;113:749–754.
Street ME, Seghini P, Ziveri MA, Fieni S, Volta C, Neri TM, Viani I, Bacchi-Modena A, Bernasconi S. Interleukin-6 and insulin-like growth factor system relationships and differences in the human placenta and fetus from the 35th week of gestation. Growth Horm IGF Res 2006;16:365–372.
Balasubramaniam V, Le Cras TD, Ivy DD, Grover TR, Kinsella JP, Abman SH. Role of platelet-derived growth factor in vascular remodeling during pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol 2003;284: L826–L833.
Humbert M, Monti G, Brenot F, Sitbon O, Portier A, Grangeot-Keros L, Duroux P, Galanaud P, Simonneau G, Emilie D. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med 1995;151:1628–1631.
Barker D, Williams S, Mason G, McLoughlin H, Schlosshan D, Tan L. Plasma brain natriuretic peptide may not be useful to assess possible cardiac symptoms during pregnancy: 248. Am J Obstet Gynecol 2006;193, (6):79.
Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, Pochettino A, Kotloff RM. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003;167:735–740.
D’Alonzo GE, Barst RJ, Ayers SM, et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 1991;115:343–349.
World Symposium on Primary Pulmonary Hypertension (Venice, Italy 2003).
Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992;327:76–81.
Galie N, Humbert M, Vachiery JL, Vizza CD, Kneussl M, Manes A, Sitbon O, Torbicki A, Delcroix M, Naeije R, Hoeper M, Chaouat A, Morand S, Besse B, Simonneau G. Arterial Pulmonary Hypertension and Beraprost European (ALPHABET) Study Group. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002;39:1496–502.
Higenbottam TW, Spiegelhalter D, Scott JP, Fuster V, Dinh-Xuan AT, Caine N, Wallwork J. Prostacyclin (epoprostenol) and heart-lung transplantation as treatments for severe pulmonary hypertension. Br Heart J 1993;70:366–370.
Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;334:296–302.
Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, Rich S, Barst RJ, Barrett PS, Kral KM, Jobsis MM, Loyd JE, Murali S, Frost A, Girgis R, Bourge RC, Ralph DD, Elliott CG, Hill NS, Langleben D, Schilz RJ, McLaughlin VV, Robbins IM, Groves BM, Shapiro S, Medsger TA Jr. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000;132:425–434.
McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002;106:1477–1482.
Yap RL, Mermel LA. Micrococcus infection in patients receiving epoprostenol by continuous infusion. Eur J Clin Microbiol Infect Dis 2003;22:704–705.
MMWR Centers for Disease Control and Prevention (CDC). Bloodstream infections among patients treated with intravenous epoprostenol or intravenous treprostinil for pulmonary arterial hypertension-seven sites, United States, 2003–2006. MMWR Morb Mortal Wkly Rep. 2007;56:170–172.
Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. American College of Chest Physicians. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:35S–62S.
Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, Keogh A, Oudiz R, Frost A, Blackburn SD, Crow JW, Rubin LJ; Treprostinil Study Group. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002;165:800–804.
Gomberg-Maitland M, Tapson VF, Benza RL, McLaughlin VV, Krichman A, Widlitz AC, Barst RJ. Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension. Am J Respir Crit Care Med 2005;172:1586–1589.
Opitz CF, Wensel R, Winkler J, Halank M, Bruch L, Kleber FX, Hoffken G, Anker SD, Negassa A, Felix SB, Hetzer R, Ewert R. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J 2005;26:1895–1902.
Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, Naeije R, Galie N. STRIDE-2 Study Group. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol 2006;47:2049–2056.
Galie’ N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, Frost AE, Zwicke D, Naeije R, Shapiro S, Olschewski H, Rubin LJ. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:529–535.
McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest 2006;36:10–15.
Klinger JR. The nitric oxide/cGMP signaling pathway in pulmonary hypertension. Clin Chest Med 2007;28:143–167.
Ghofrani, HA, Voswinckel, R, Reichenberger, F, et al. Differences in hemodynamic and oxygenation responses to three different phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension: a randomized prospective study. J Am Coll Cardiol 2004;44:1488–1496.
Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, Fleming T, Parpia T, Burgess G, Branzi A, Grimminger F, Kurzyna M, Simonneau G; Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148–2157.
Badesch DB, McLaughlin VV, Delcroix M, Vizza CD, Olschewski H, Sitbon O, Barst RJ. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:56S–61S.
Channick RN, Sitbon O, Barst RJ, Manes A, Rubin LJ. Endothelin receptor antagonists in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:62S–67S.
Ghofrani HA, Pepke-Zaba J, Barbera JA, Channick R, Keogh AM, Gomez-Sanchez MA, Kneussl M, Grimminger F. Nitric oxide pathway and phosphodiesterase inhibitors in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:68S–72S.
McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, Loyd JE. American College of Chest Physicians. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:14S–34S.
Mccaffrey RM, Dunn LJ. Primary pulmonary hypertension in pregnancy. Obstet Gynecol Surv 1964;19:567–591.
Breen TW, Janzen JA. Pulmonary hypertension and cardiomyopathy: anaesthetic management for caesarean section. Can J Anaesth 1991;38:895–899.
Martinez JM, Comas C, Sala X, Gratacos E, Torres PJ, Fortuny A. Maternal primary pulmonary hypertension associated with pregnancy. Eur J Obstet Gynecol Reprod Biol 1994;54:143–147.
Carro-Jimenez EJ, Lopez JE. Primary pulmonary hypertension and pregnancy. Bol Asoc Med P R 2005;97:328–333.
Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger's syndrome and pregnancy. Obstet Gynecol Surv 1979;34:721–741.
Sinnenberg RJ Jr. Pulmonary hypertension in pregnancy. South Med J 1980;73:1529–1531.
Roberts NV, Keast PJ. Pulmonary hypertension and pregnancy – a lethal combination. Anaesth Intensive Care 1990;18:366–374.
Easterling TR, Ralph DD, Schmucker BC. Pulmonary hypertension in pregnancy: treatment with pulmonary vasodilators. Obstet Gynecol 1999;93:494–498.
Smedstad KG, Cramb R, Morison DH. Pulmonary hypertension and pregnancy: a series of eight cases. Can J Anaesth 1994;41:502–512.
Elliot CA, Stewart P, Webster VJ, Mills GH, Hutchinson SP, Howarth ES, Bu'lock FA, Lawson RA, Armstrong IJ, Kiely DG. The use of iloprost in early pregnancy in patients with pulmonary arterial hypertension. Eur Respir J 2005;26:168–173.
3rd TRAX PMS Data Report to CPMP for the period from 24 May 2002–19 February 2003 (Actelion Pharmaceuticals, data on file).
Battenfeld R, Schuh W, Schobel C. Studies on reproductive toxicity of iloprost in rats, rabbits and monkeys. Toxicol Lett 1995;78:223–234.
Hunter S, Robson SC. Adaptation of the maternal heart in pregnancy.Br Heart J 1992;68:540–543.
Monnery L, Nanson J, Charlton G. Primary pulmonary hypertension in pregnancy; a role for novel vasodilators. Br J Anaesth 2001;87:295–298.
Bildirici I, Shumway JB. Intravenous and inhaled epoprostenol for primary pulmonary hypertension during pregnancy and delivery. Obstet Gynecol 2004;103:1102–1105.
Slomka F, Salmeron S, Zetlaoui P, Cohen H, Simonneau G, Samii K. Primary pulmonary hypertension and pregnancy: anesthetic management for delivery. Anesthesiology 1988;69:959–961.
Robinson DE, Leicht CH. Epidural analgesia with low-dose bupivacaine and fentanyl for labor and delivery in a parturient with severe pulmonary hypertension. Anesthesiology 1988;68:285–288.
Lam GK, Stafford RE, Thorp J, Moise KJ Jr, Cairns BA. Inhaled nitric oxide for primary pulmonary hypertension in pregnancy. Obstet Gynecol 2001;98:895–898.
Smedstad KG, Cramb R, Morison DH. Pulmonary hypertension and pregnancy: a series of eight cases. Can J Anaesth 1994;41:502–512.
Olofsson C, Bremme K, Forssell G, Ohqvist G. Cesarean section under epidural ropivacaine 0.75% in a parturient with severe pulmonary hypertension. Acta Anaesthesiol Scand 2001;45:258–260.
Badalian SS, Silverman RK, Aubry RH, Longo J. Twin pregnancy in a woman on long-term epoprostenol therapy for primary pulmonary hypertension. A case report. J Reprod Med 2000;45:149–152.
O'Hare R, McLoughlin C, Milligan K, McNamee D, Sidhu H. Anaesthesia for caesarean section in the presence of severe primary pulmonary hypertension. Br J Anaesth 1998;81:790–792.
Decoene C, Bourzoufi K, Moreau D, Narducci F, Crepin F, Krivosic-Horber R. Use of inhaled nitric oxide for emergency Cesarean section in a woman with unexpected primary pulmonary hypertension. Can J Anaesth 2001;48:584–587.
Mangano DT. Anaesthesia for the pregnant cardiac patient, In: Shnider SM, Levinson G, eds. Anaesthesia for Obstetrics, 3rd Edn. Baltimore: Williams and Wilkins, 1983: 502–508.
Roberts NV, Keast PJ. Pulmonary hypertension and pregnancy – a lethal combination. Anaesth Intensive Care 1990;18:366–374.
Hill LL, De Wet CJ, Jacobsohn E, Leighton BL, Tymkew H. Peripartum substitution of inhaled for intravenous prostacyclin in a patient with primary pulmonary hypertension. Anesthesiology 2004;100:1603–1605.
Stewart R, Tuazon D, Olson G, Duarte AG. Pregnancy and primary pulmonary hypertension: successful outcome with epoprostenol therapy. Chest 2001;119:973–975.
Vonk Noordegraaf A, Roeleveld RJ. Primary pulmonary hypertension in pregnancy. Br J Anaesth 2002;88:151.
Kiss H, Egarter C, Asseryanis E, Putz D, Kneussl, M. Primary pulmonary hypertension in pregnancy: a case report. Am J Obstet Gyn 1995;172:1052–1054.
Nootens M, Rich S. Successful management of labor and delivery in primary pulmonary hypertension. Am J Cardiol 1993;71:1124–1125.
Robinson JN, Banerjee R, Landzberg MJ, Thiet MP. Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension. Am J Obstet Gynecol 1999;180:1045–1046.
Nelson DM, Main E, Crafford W, Ahumada GG. Peripartum heart failure due to primary pulmonary hypertension. Obstet Gynecol 1983;62:58S–63S.
Sullivan JM, Ramanathan KB.Management of medical problems in pregnancy--severe cardiac disease. N Engl J Med 1985;313:304–309.
Weir EK. Diagnosis and management of primary pulmonary hypertension: In: Weir WK, Reeves JT eds. Pulmonary Hypertension. New York: Futura, 1984:142–143.
Acknowledgements
This paper was supported in part by NIH grant HL-088328-01 (JRK)
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2009 Humana Press, a part of Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Casserly, B., Klinger, J.R. (2009). Pulmonary Arterial Hypertension in Pregnancy. In: Rosene-Montella, K., Bourjeily, G. (eds) Pulmonary Problems in Pregnancy. Respiratory Medicine. Humana Press. https://doi.org/10.1007/978-1-59745-445-2_19
Download citation
DOI: https://doi.org/10.1007/978-1-59745-445-2_19
Published:
Publisher Name: Humana Press
Print ISBN: 978-1-934115-12-1
Online ISBN: 978-1-59745-445-2
eBook Packages: MedicineMedicine (R0)