Original paper
Pregnancy and delivery in women with congenital heart disease after cardiac surgery

Introduction
Material and methods
Data of 78 pregnant women aged 19-39 (mean age 26.1±4.6) with CHD operated on in the past (83 gestations), treated between 1995-2005 in the Adult Congenital Heat Disease Outpatient Clinic of 1st, Cardiology Department in Poznan were analysed. The studied population of women operated because of CHD consisted of: 18 women after surgery due to ventricular septal defect (VSD), 17 women after surgery due to atrial septal defect (ASD II), 11 women after patent ductus arteriosus (PDA) closure, 12 women after complete correction of tetralogy of Fallot (ToF), 6 women after surgery due to coarctation of the aorta (CoAo), 5 women after correction of atrial septal defect I (ASD I), 3 women after surgery of combined aortic valve disease (VAC), 2 women operated due to complete atrio-ventricular canal (CAVC), 2 women after correction of double outlet right ventricle (DORV), 2 women after anomalous pulmonary venous return (APVR), single patients were after Senning operation due to transposition of great arteries (dTGA), Fontan operation because of single ventricle physiologically and after mitral prosthesis implantation. Each woman was followed monthly, including a physical examination, ECG and echocardiography. Ten patients were hospitalised to determine the mode of pregnancy completion.
Forty eight women delivered spontaneously (57% of pregnancies), Caesarean section for mother’s indications (risk resulting from the mother’s heart defect) was performed in 24 pregnant women (29% of the group), Caesarean section for obstetric indications – in 7 women (8% of the group). The patient with common ventricle after Fontan surgery had a spontaneous abortion caused by paroxysmal atrial fibrillation, while atrial flutter was a direct cause of abortion in the woman after double outlet right ventricle. The patient with a history of previous mechanical mitral valve implantation lost her second pregnancy in this mechanism. One female after correction of Fallot’s tetralogy and implantation of cardioverter-defibrillator in the past had a spontaneous abortion in 22. week of gestation. (Table I). Most of the patients were primipara (57 females). The remaining 21 patients before registration in the Congenital Heart Diseases Outpatient Clinic for Adults had a history of 48 pregnancies in total. The patient after Fontan surgery had been pregnant 3 times before coming to our clinic, she was pregnant five times in total. The patients after correction of coarctation of the aorta, type II atrial septal defect and after type II atrial septal defect closure had four pregnancies each. The characteristics of patients with a history of more than one pregnancy are shown in Table II. In seven cases the observed pregnancies were their third ones, and in the remaining 28 – second ones. Table III lists cardiologic problems that occurred during the observed pregnancies. Fifteen pregnant women were affected, of those the majority had a significant left ventricular outflow tract obstruction. The only death was recorded in this group and occurred in 27. week of gestation during an urgent replacement of the aortic valve prosthesis due to valve thrombosis (anticoagulation therapy with heparin was administered in a local medical facility). At the time of the surgery a Caesarean section was performed and a 700-gram foetus was salvaged. Now the baby grows normally and suffers only from retinal lesions. In another patient with a mechanical aortic valve prosthesis the anticoagulation therapy administered according to the scheme: heparin, warfarin, heparin, was given in our hospital and the prosthesis function was not disturbed. Left hemiparesis was observed in a patient with homogenous aortic valve implanted due to bacterial endocarditis during a previous pregnancy. Arterial hypertension was diagnosed in a patient with operated coarctation of the aorta and was successfully treated with alpha-methyldopa. The patient with single ventricle physiology after Fontan surgery was classified as New York Heart Association II during her first pregnancy we followed (oxygen saturation – 88%). In this case, atrial fibrillation was the most probable cause of spontaneous abortion. The same patient, during the second pregnancy we observed (her fifth pregnancy), was recommended to reduce significantly her physical activity which probably helped her to maintain sinus rhythm and deliver a healthy newborn. Reduced exercise capacity (New York Heart Association class II) was observed in patients after correction of double outlet right ventricle (DORV) and the transposition of the great vessels after Senning surgery (TGA Senning); they were advised to reduce their physical activity and to take low doses of diuretics. Ventricular arrhythmias were found in two patients after repair of tetralogy of Fallot (one with implanted cardioverter-defibrillator). Supraventricular arrhythmias were recorded in one pregnant woman with surgically closed type II atrial septal defect. Those arrhythmias were successfully treated with cardioselective beta-blockers. Atrial flutter was followed by a spontaneous abortion in a patient after correction of DORV. First degree atrio-ventricular block was recorded in patients operated on for partial or complete atrioventricular canal. In the studied population, no bacterial endocarditis was noted. In 5 patients delivery was performed by Caesarean section in the operating room of the Cardiac Surgery Department with the assistance of cardiologists, obstetricians and neonatologists dealing with the issues of congenital heart diseases. Eight (10.1%) delivered children had congenital heart diseases. Three of them died early after the labour, showing symptoms of heart failure (Table IV).
Discussion
At present, most patients with CHD reach adulthood and, simultaneously, the prevalence of rheumatic heart disease is on a decline. This is why in developed countries up to 80% of pregnant women with cardiac problems are women with CHD [1, 4, 5, 8, 10, 11]. This group of patients is still very small and heterogeneous, so observations are sparse [1-12]. A large study from Sao Paulo [13], concerning 1000 pregnant women with heart disease, includes an analysis of 191 women with CHD (noncyanotic defects – 63.3%, cyanotic – 25%, Eisenmenger’s syndrome – 11.5%). In our population the most often represented defects were surgically repaired VSD and ASD. Together with patients after correction of CAVC, PDA closure and after APVR correction, they formed a low risk group (49 patients – 67% population). Our observation confirmed those made by others that the pregnancy course in these conditions is good [1, 3, 4, 6, 10-12, 14, 15]. Similarly to other authors, despite supraventricular extrasystole in one patient after ASD and a first degree atrio-ventricular block in a patient after correction of CAVC, we did not note other cardiac complications in this subgroup [7, 13]. Fourteen women in this subpopulation had had at least one labour before. One patient after VSD with postprocedural complete heart block had a DDD pacemaker implanted, and in accordance with the published data we did not observe any complication due to his fact [16, 17]. Another 10 followed women were after complete correction of ToF. Ventricular arrhythmias were noted in 2 of them. An AICD had been implanted due to MAS syndrome in one woman. This patient miscarried during first trimester. Published data do not confirm a connection between AICD and miscarriage rate in this rare group of patients [13, 16]. In patients after correction of ToF, if there are no severe pulmonary regurgitations or pulmonary restenoses, the gestation course is similar to that in normal population [3, 4, 8, 14]. Moderate exercise capacity limitation, as reported by others, was found in a patient after correction of DORV [8, 14]. The other women with the same condition presented recurrent episodes of atrial flutter that lead to a miscarriage in the first trimester, probably due to ischaemia of the foetus and placenta. Miscarriages during the first trimester in women with CHD occur more often than in healthy ones [3, 4, 8]. Six women in our group were after surgery due to CoAo. Moderate, treatable arterial hypertension occurred in one patient. In literature, the risk of aortic and Willis circle aneurisms rupture (augmented by hypertension) is described in 3.5% of patients. Deaths due to this mechanism are noted mainly in the first trimester and the first week after delivery [3, 4, 6, 14, 18]. Obviously, there were only few women after operated primary cyanotic defect in the studied population. One, after Senning operation due to TGA, was in the I/II functional class (NYHA). Because of the fact that the morphologically right ventricle acts as the systemic one, especially during increased workload as in pregnancy, there is a threat of reduction of its capacity. Moreover, past surgery favours supraventricular arrhythmias. Even though there are data of such complications in about 14-18% of such cases, we did not observe any in our patient [19-21]. The pregnant woman after Fontan operation due to physiologically single ventricle was a second described mother of two children [22]. She was pregnant five times, with three pregnancies unsuccessful because of supraventricular arrhythmias. As in other described women, her clinical state did not worsen. So far, there are no data on how pregnancy influences the survival rate in those patients. Hemodynamic changes observed during gestation – increased stroke volume, cardiac output and heart rate, with a parallel decrease in systemic vascular resistance, due to the essential dependence on venous return, and limited possibility to increase the cardiac output, are especially disadvantageous for women after Fontan operation. Additionally, supraventricular arrhythmias in these patients are severe cardiac complications [23-25]. Pregnant women with severe aortic stenosis are potentially high risk patients [7, 8, 14]. This defect was diagnosed in 3 patients. The only death in our observation occurred in 27th week of pregnancy in a patient with an urgent aortic prosthesis replacement due to its thrombosis. Such operations are rare: among 1000 women from the Sao Paulo study there were 8 successful prosthesis replacements [13]. Authors from the University Hospital in Rome reported about 69 such operations between 1958 and 1998, with the maternal mortality rate of 2.9% and 20% of the foetal mortality [26]. Another patient on antithrombotic therapy managed in our department, had no complication during pregnancy and delivery. Antithrombotic therapy during pregnancy is still controversial, but in patients with high daily warfarin demand (more than 5 mg, as in our patient) unfractioned heparin is indicated instead [7]. In our population spontaneous labour was achieved in 57% pregnancies, 38% was completed by Caesarean section. Theoretically, spontaneous labour is a lower threat than Caesarean section (blood loss 500 vs. 1000 ml, analgesia and sedation prevent an excessive increase of stroke volume) [1, 3, 4, 6]. But in cases with an elevated risk, Caesarean section is often preferred. Celia Oakley [12] an outstanding expert on this topic, believes that contemporary anaesthesia which does not lower blood pressure allows such therapeutic management. In 5 cases (since 1999) we decided to carry out the delivery in a tertiary centre. Because of a low number of pregnant patients with CHD, obstetricians in regional centres can manage only a few such cases in their career. It is thus important to refer those patients to specialised centres with cardiologists and obstetricians experienced in CHD management [1, 7, 8, 12]. Even though it is not precised in the ESC guidelines [7], as proposed by other authors we used infective endocarditis chemoprophylaxis in the perinatal period, being unable to predict if delivery would be uncomplicated [3-6, 12]. Eight newborns (10.1%) in the studied population had CHD and 3 died soon after birth. The noted inheritance rate is higher than reported by others. In the cited Sao Paulo population [13] there was a 5.2% inheritance rate of CHD, while other authors estimate it at 5-6.7% [5, 6, 27-28]. These values are significantly higher than in the general healthy population (0.4- 0.6%) [28, 29]. Our observation of pregnant women with CHD, although sparse, is one of the biggest accessible in literature [1-5, 8-11, 13]. It will be reasonable to base future guidelines on multicentre studies.
Conclusions
1. In the majority of women with CHD pregnancy and delivery are not essentially disturbed, but should be carefully monitored by experienced cardiologists and obstetricians. 2. The highest incidence of pregnancy and labour complications is observed in women with a significant left ventricular outflow tract obstruction. 3. CHD are transferred in 10.1% of cases.

References
1. Lupton M, Oteng-Ntim E, Ayida G, Steer PJ. Cardiac disease in pregnancy. Curr Opin Obstet Ginecol 2002; 14:137-43. 2. Somerville J. Grown-up congenital heart disease – medical demands look back, look forward 2000. Thorac Cardiovasc Surg 2001; 49: 21-6. 3. Colman JM, Serman M, Gareth P, Seaward R, Siu SC. Congenital heart disease in pregnancy. Cardiol Review 2000; 8: 166-73. 4. Siu SC, Colman JM. Heart disease and pregnancy. Heart 2001; 85: 710-15. 5. Siu S, Sermer M, Colman J. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation 2001; 104: 515-21. 6. Perloff JK, Child JS. Congenital heart disease in adult. 2ed. W.B. Saunders Company, Philadelphia, 1998. 7. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003; 24: 761-81. 8. Iserin L. Management of pregnancy in woman with congenital heart disease. Heart 2001; 85: 493-4. 9. Presbitero P, Rabajoli F, Somerville J. Pregnancy in patients with congenital heart disease. Schweiz Med Wochenschr 1995; 125: 311-5. 10. Gei AF, Hankins GD. Cardiac disease and pregnancy. Obstet Gynecol Clin North A 2001; 28: 485-512. 11. Ramsey PS, Ramin KD, Ramin SM. Cardiac disease in pregnancy. Am L Perinatol 2001; 18: 245-66. 12. Oakley CM. Pregnancy and congenital heart disease. Heart 1997; 78: 12-14. 13. Avila WS, Rossi EG, Ramirez JA, Grinberg M, Bortolotto MR, Zugaib M, et al. Pregnancy in patients with heart disease: experience with 1000 cases. Clin Cardiol 2003; 26: 135-42. 14. Bhagwat AR, Engel PJ. Heart disease and pregnancy. Cardiol Clin 1995; 13: 163-77. 15. Teerlink JR, Foster E. Valvular heart disease in pregnancy. Cardiol Clin 1998; 16: 573-97. 16. Gowda RM, Khan IA, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac arrhythmias in pregnancy: clinical and therapeutic considerations. Int J Cardiol 2003; 88: 129-33. 17. Jaffe R, Gruber A, Fejgin M, Altaras M, Ben-Aderet N. Pregnancy with an artificial pacemaker. Obstet Gynecol Surv 1987; 42: 137-9. 18. Beauchesne LM, Connolly HM, Ammash NM, Warnes CA. Coarctation of the aorta: outcome of pregnancy. J Am Coll Cardiol 2001; 38: 1728-33. 19. Genoni M, Jenni R, Hoerstrup SP, Vogt P, Turina M. Pregnancy after atrial repair for transposition of the great arteries. Heart 1999; 81: 276-7. 20. Clarcson P, Wilson M, Neutze J, North R, Calder A, Barrat-Bayes B. Outcome of pregnancy after the Mustard operation for transposition of the great arteries with intact ventricular septum. J Am Coll Cardiol 1994; 24: 190-3. 21. Lao TT, Sermer M, Colman MJ. Pregnancy following surgical correction for transposition of great arteries. Obstet Gynecol 1994; 83: 665-8. 22. Trojanarska O, Siwinska A, Markwitz W, Ochotny R. Pregnancy and labour in a patient after Fontan operation – a case report [Polish]. Kardiol Pol 2003; 58: 51-2. 23. Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85: 469-96. 24. Findlow D, Doyle E. Congenital heart disease in adults. Br J Anaesth 1997; 78: 416-30. 25. Pomini F, Mercogliano D, Cavalletti C, Caruso A, Pomini P. Cardiopulmonary bypass in pregnancy. Ann Thorac Surg 1996; 61: 259-68. 26. Gill HK, Splitt M, Sharland GK, Simpson JM. Patterns of recurrence of congenital heart disease: an analysis of 6,640 consecutive pregnancies evaluated by detailed fetal echocardiography. J Am Coll Cardiol 2003; 42: 923-9. 27. Roodpeyma S, Kamali Z, Afshar F, Naraghi S. Risk factors in congenital heart disease. Clin Pediatr (Phila) 2002; 41: 653-8. 28. Burn J, Brennan P, Little J, Holloway S, Coffey R, Somerville J, et al. Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study. Lancet 1998; 351: 311-6. 29. Hameed A, Karaalp IS, Tummala PP, Wani OR, Canetti M, Akhter MW, et al. The effect of valvular heart disease on maternal and fetal outcome of pregnancy. J Am Coll Cardiol 2001; 37: 893-9.