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
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
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
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
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
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
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
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
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