Dtsch Med Wochenschr 2004; 129: S35-S39
DOI: 10.1055/s-2004-824842
Kasuistiken

© Georg Thieme Verlag Stuttgart · New York

Schwangerschaft bei Eisenmenger-Patientinnen

Erfahrungen aus Vilnius 1967 - 2003Pregnancy in patients with Eisenmenger’s syndromeA. Dranenkiene1 , C. F. Opitz5 , L. Gumbiené1 , B. Doma2 , R. Ewert4 , K. Bühlmeyer3
  • 1Herzchirurgiezentrum der Vilnius Universität
  • 2Klinik für Geburtshilfe und Gynäkologie der Vilnius Universität
  • 3Landsberger Straße 33, 86938 Schondorf/Ammersee
  • 4Klinik und Poliklinik für Innere Medizin B, Ernst-Moritz-Arndt-Universität Greifswald
  • 5Klinik für Innere Medizin II - Kardiologie, DRK-Kliniken Westend, Berlin, Lehrkrankenhaus der Humboldt-Universität Berlin
Further Information

Publication History

eingereicht: 3.12.2003

akzeptiert: 1.4.2004

Publication Date:
10 May 2004 (online)

Summary

Pregnancy in patients with Eisenmenger}s syndrome is associated with high maternal and fetal mortality rates and effective contraception or termination of pregnancy is strongly recommended. When these patients decide to begin or continue a pregnancy against advice a coordinated multi-specialist care at a center for congenital heart disease is mandatory. So far, experience with this demanding group of patients is limited.

This report reviews the outcome of 30 pregnancies in 10 patients from a group of 35 women with Eisenmenger}s syndrome who have been treated at the University Hospital in Vilnius, Lithuania, between 1967 and 2003. The most frequent underlying lesion was a ventricular septal defect in 5 patients, their mean age was 26,2 ± 5,4 years and they were in functional class II or III (9/1) at the beginning of the pregnancy. In this group 13 pregnancies were terminated (43 %), 4 spontaneous abortions (13 %) occurred at less than 21 weeks of pregnancy and one stillbirth at 23 weeks of gestation. Twelve children were born alive, 10 were premature and 2 term deliveries. One maternal death occurred on the third day following an emergency delivery in week 36 and another patient decompensated immediately following delivery, but could be stabilized with intensive care therapy. During long-term follow-up all of these 9 patients were alive at 10/2003 despite a significant deterioration in functional class. These data are comparable to previously published observations in pregnant women with Eisenmenger}s syndrome.

Literatur

  • 1 Eisenmenger V. Die angeborenen Defecte der Kammerscheidewand des Herzens.  Z Klin Med. 1897;  32 (Suppl 3) 1-28
  • 2 Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt.  Br Med J. 1958;  46 701-709, 755 - 762
  • 3 Hopkins W E, Ochoa L L, Richardson G W, Trulock E P. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome.  J Heart Lung Transplant. 1996;  15 100-105
  • 4 Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A, Sorenson K, Kaemmer H, Thilen U, Bink-Boelkens M, Iserin L, Daliento L, Silove E, Redington A, Vouhe P, Priori S, Alonso M A, Blanc J J, Budaj A, Cowie M, Deckers J, Fernandez Burgos E, Lekakis J, Lindahl B, Mazzotta G, Morais J, Oto A, Smiseth O, Trappe H J, Klein W, Blomstrom-Lundqvist C, de Backer G, Hradec J, Mazzotta G, Parkhomenko A, Presbitero P, Torbicki A. Task Force on the Management of Grown Up Congenital Heart Disease, European Society of Cardiology; ESC Committee for Practice Guidelines . Management of grown up congenital heart disease.  Eur Heart J. 2003;  24 1035-1084
  • 5 Therrien J, Warnes C, Daliento L, Hess J, Hoffmann A, Marelli A, Thilen U, Presbitero P, Perloff J, Somerville J, Webb G D. Canadian Cardiovascular Society Consensus Conference 2001 update: recommendations for the management of adults with congenital heart disease part III.  Can J Cardiol. 2001;  17 1135-1158
  • 6 Cantor W J, Harrison D A, Moussadji J S, Connelly M S, Webb G D, Liu P, McLaughlin P R, Siu S C. Determinants of survival and length of survival in adults with Eisenmenger syndrome.  Am J Cardiol. 1999;  84 677-681
  • 7 Young D, Mark H. Fate of the patient with the Eisenmenger syndrome.  Am J Cardiol. 1971;  28 658-669
  • 8 Saha A, Balakrishnan K G, Jaiswal P K, Venkitachalam C G, Tharakan J, Titus T, Kutty R. Prognosis for patients with Eisenmenger syndrome of various aetiology.  Int J Cardiol. 1994;  45 199-207
  • 9 Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome: Factors relating to deterioration and death.  Eur Heart J. 1998;  19 1845-1855
  • 10 Siu S C, Sermer M, Colman J M, Alvarez A N, Mercier L A, Morton B C, Kells C M, Bergin M L, Kiess M C, Marcotte F, Taylor D A, Gordon E P, Spears J C, Tam J W, Amankwah K S, Smallhorn J F, Farine D, Sorensen S. Cardiac Disease in Pregnancy (CARPREG) Investigators . Prospective multicenter study of pregnancy outcomes in women with heart disease.  Circulation. 2001;  104 515-521
  • 11 Oechslin E N, Harrison D A, Connelly M S, Webb G D, Siu S C. Mode of death in adults with congenital heart disease.  Am J Cardiol. 2000;  86 1111-1116
  • 12 Daliento L, Rebellato L, Angelini A, Frescura C, Mazzotti E, Rotundo M, Thiene G. Fatal outcome in Eisenmenger Syndrome.  Cardiovasc Pathol. 2002;  11 221-228
  • 13 Kaemmerer H, Niesert S, Daniel W G, Lichtlen P R, Kallfelz H C. Schwangerschaft und angeborene Herzfehler.  Z Kardiol. 1994;  83 208-214
  • 14 Kandasarry R, Koh K F, Tharn S L, Reddy S. Anaesthesia for Caesarean section in a patient with Eisenmenger’s syndrome.  Singapore Med J. 2000;  41 356-358
  • 15 Schmaltz A A, Neudorf U, Winkler U H. Outcome of pregnancy in women with congenital heart disease.  Cardiol Young. 1999;  9 88-96
  • 16 Köhler F, Fotuhi P, Baumann G. Schwangerschaft und angeborene Herzfehler.  Z Kardiol. 2001;  90 30-35 (Suppl 4)
  • 17 Avila W S, Grinberg M, Snitcowsky R, Faccioli R, Da Luz P L, Bellotti G, Pileggi F. Maternal and fetal outcome in pregnant women with Eisenmenger’s syndrome.  Eur Heart J. 1995;  16 460-464
  • 18 Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger’s syndrome and pregnancy.  Obstet Gynecol Surv. 1979;  34 721-741
  • 19 Smedstad K G, Cramb R, Morison D H. Pulmonary hypertension and pregnancy: a series of eight cases.  Can J Anaesth. 1994;  41 502-512
  • 20 Presbitero P, Rabajoli F, Somerville J. Pregnancy in patients with congenital heart disease.  Schweiz Med Wochenschr. 1995;  125 311-315
  • 21 Colman J M, Siu S C. Pregnancy in adult patients with congenital heart disease.  Prog Pediatr Cardiol. 2003;  17 53-60
  • 22 Vongpatanasin W, Brickner M E, Hillis L D, Lange R A. The Eisenmenger syndrome in adults.  Ann Intern Med. 1998;  128 745-755
  • 23 Weiss B M, Hess O M. Pulmonary vascular disease and pregnancy: current controversies, management strategies, and perspectives.  Eur Heart J. 2000;  21 104-115
  • 24 Roberts N V, Keast P J. Pulmonary hypertension and pregnancy - a lethal combination.  Anaesth Intens Care. 1990;  18 366-374
  • 25 Henriksson P, Varendh G, Lundstrom N R. Haemostatic defects in cyanotic congenital heart disease.  Br Heart J. 1979;  41 23-27
  • 26 Altman R, Scazziota A, Rouvier J, Gurfinkel E, Favaloro R, Perrone S, Fareed J. Coagulation and fibrinolytic parameters in patients with pulmonary hypertension.  Clin Cardiol. 1996;  19 549-554
  • 27 Rabinovitch M, Andrew M, Thom H, Trusler G A, Williams W G, Rowe R D, Olley P M. Abnormal endothelial factor VIII associated with pulmonary hypertension and congenital heart defects.  Circulation. 1987;  76 1043-1052
  • 28 Oechslin E. Eisenmenger}s Syndrome. Churchill Livingstone, London In: Diagnosis and Management of Adult Congenital Heart Disease 2003
  • 29 Vitale N, DeFeo M, DeSanto L S, Pollice A, Tedesco N, Cotrufo M. Dose-dependent fetal complications of warfarin in pregnant women with mechanical heart valves.  J Am Coll Cardiol. 1999;  33 1637-1641
  • 30 Cole P J, Cross M H, Dresner M. Incremental spinal anaesthesia for elective Caesarean section in a patient with Eisenmenger’s syndrome.  Br J Anaesth. 2001;  86 723-726
  • 31 Kaemmerer H, Bauer U, Stein J -I, Lemp S, Bartmus D, Hoffmann A. et al . Pregnancy in congenital cardiac disease: an increasing challenge for cardiologists and obstetricians - a prospective multicenter study.  Z Kardiol. 2003;  92 16-23
  • 32 Barst R J, Rubin L J, Long W A, McGoon M D, Rich S, Badesch D B, Groves B M, Tapson V F, Bourge R C, Brundage B H. 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
  • 33 Rubin L J, Badesch D B, Barst R J, Galie N, Black C M, Keogh A, Pulido T, Frost A, Roux S, Leconte I, Landzberg M, Simonneau G. Bosentan therapy for pulmonary arterial hypertension.  N Engl J Med. 2002;  346 896-903
  • 34 Barst R J, Ivy D, Dingemanse J, Widlitz A, Schmitt K, Doran A, Bingaman D, Nguyen N, Gaitonde M, van Giersbergen P L. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension.  Clin Pharmacol Ther. 2003;  73 372-382
  • 35 Schulze-Neick I, Uhlemann F, Nurnberg J H, Bultmann M, Haas N A, Dahnert I, Alexi-Meshkishvili V, Opitz C, Pappert D, Rossaint R, Kleber F X, Hetzer R, Lange P E. Aerosolized prostacyclin for preoperative evaluation and post-cardiosurgical treatment of patients with pulmonary hypertension.  Z Kardiol. 1997;  86 71-80
  • 36 Rosenzweig E B, Kerstein D, Barst R J. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.  Circulation. 1999;  99 1858-1865
  • 37 Fernandes S M, Newburger J W, Lang P, Pearson D D, Feinstein J A, Gauvreau K, Landzberg M J. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology.  Am J Cardiol. 2003;  91 632-635
  • 38 Geohas C, McLaughlin V V. Successful management of pregnancy in a patient with eisenmenger syndrome with epoprostenol.  Chest. 2003;  124(3) 1170-3

Priv.-Doz. Alicija Dranenkiene

Head of the Outpatient-Clinic for Cardiovascular Surgery, Vilnius University, Hospital Santariskiu Clinics

Santariskiu st. 2

2021 Vilnius, Lithuania

Email: Alicija.Dranenkiene@santa.lt

    >