the eisenmenger syndrome and pregnancy. a report of two cases

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EUROP. J. OBSTET. GYNEC. REPROD. BIOL., 1975, 5/3, 187-190. EXCERPTA MEDICA REVIEW ARTICLE The Eisenmenger syndrome and pregnancy. A report of two cases G. C. L. Lachelin Deportment of Obstetrics and Gwecology, University College Hospitnl, London WC1, United Kingdom LACHELIN, G. C. L. (1975): The Eisenmenger syndrome and pregnancy. A report of two cases. Europ. J. Ohstet. Gynec. reprod. Biol., 5/3, 187-190. Three pregnancies in two patients with the Eisenmenger syndrome are reported, and the literature is reviewed. The major hazards of pregnancy in patients with this condition are emphasized, and the management of pregnancy and labour is discussed. Eisenmenger syndrome; congenital heart disease; pregnancy introduction Various definitions of the Eisenmenger complex and Eisenmenger syndrome have been made by different authors. The original patient described by Eisenmenger (1897) was cyanosed from infancy and was found at postmortem to have a ventricular septal defect with an overriding aorta, without pul- monary stenosis. Wood in 1958 proposed the following definition of the Eisenmenger complex: “pulmonary hypertension at systemic level due to a high pulmonary vascular resistance, with a reversed or bidirectional shunt, through a large ventricular septal defect.” He suggested that the term Eisen- menger syndrome should be used to embrace all congenital heart lesions, with shunts at other levels, which behave hemodynamically like the Eisen- menger complex. Other authors, however, have not always adhered to these definitions, and have re- ported cases which are not in the true Eisenmenger category. Case reports Case I A heart lesion had been discovered at birth and the patient’s childhood activities were limited by dyspnea. Cardiac catheterization at the age of 20 years had shown a pulmonary artery systolic pres- sure of 120 mm Hg, with a small bidirectional shunt at atria1 level. The patient was first seen at this hospital early in her first pregnancy in 1963, at the age of 25 years. She was breathless on climbing stairs. On examination there was no cyanosis and no clubbing. Hemoglobin level was 15.6 g per 100 ml. She was unmarried. Vaginal termination of pregnancy by dilatation and curettage was per- formed at 8 weeks’ gestation. She continued to bleed postoperatively, and evacuation of retained pro- ducts of conception was performed five days later. She made a satisfactory recovery. She took a com- bined estrogen and progestogen contraceptive pill from 1963 to 1965 and again in 1967.

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EUROP. J. OBSTET. GYNEC. REPROD. BIOL., 1975, 5/3, 187-190. EXCERPTA MEDICA

REVIEW ARTICLE

The Eisenmenger syndrome and pregnancy. A report of two cases

G. C. L. Lachelin

Deportment of Obstetrics and Gwecology, University College Hospitnl, London WC1, United Kingdom

LACHELIN, G. C. L. (1975): The Eisenmenger syndrome and pregnancy. A report of two cases. Europ. J. Ohstet. Gynec. reprod.

Biol., 5/3, 187-190.

Three pregnancies in two patients with the Eisenmenger syndrome are reported, and the literature is reviewed. The major hazards of pregnancy in patients with this condition are emphasized, and the management of pregnancy and labour is discussed.

Eisenmenger syndrome; congenital heart disease; pregnancy

introduction

Various definitions of the Eisenmenger complex and Eisenmenger syndrome have been made by different authors. The original patient described by Eisenmenger (1897) was cyanosed from infancy and was found at postmortem to have a ventricular septal defect with an overriding aorta, without pul- monary stenosis. Wood in 1958 proposed the following definition of the Eisenmenger complex: “pulmonary hypertension at systemic level due to a high pulmonary vascular resistance, with a reversed or bidirectional shunt, through a large ventricular septal defect.” He suggested that the term Eisen- menger syndrome should be used to embrace all congenital heart lesions, with shunts at other levels, which behave hemodynamically like the Eisen- menger complex. Other authors, however, have not always adhered to these definitions, and have re- ported cases which are not in the true Eisenmenger category.

Case reports

Case I A heart lesion had been discovered at birth and

the patient’s childhood activities were limited by dyspnea. Cardiac catheterization at the age of 20 years had shown a pulmonary artery systolic pres- sure of 120 mm Hg, with a small bidirectional shunt at atria1 level. The patient was first seen at this hospital early in her first pregnancy in 1963, at the age of 25 years. She was breathless on climbing stairs. On examination there was no cyanosis and no clubbing. Hemoglobin level was 15.6 g per 100 ml. She was unmarried. Vaginal termination of pregnancy by dilatation and curettage was per- formed at 8 weeks’ gestation. She continued to bleed postoperatively, and evacuation of retained pro- ducts of conception was performed five days later. She made a satisfactory recovery. She took a com- bined estrogen and progestogen contraceptive pill from 1963 to 1965 and again in 1967.

188 G. C. L. Lachelin, Eisenmenger syndrome and pregnancy

In 1968 she became pregnant again and elected to continue with the pregnancy in spite of the known hazards. When seen at 8 weeks’ gestation she was slightly cyanosed but not dyspneic on walking on flat ground. ‘Flow guide’ right heart catheterization showed a pulmonary artery pressure of 115/70 mm Hg. Brachial artery pressure was 115/80 mm Hg and arterial oxygen saturation was 87 per cent. Elective admission for rest was arranged at 23 weeks’ gestation. At 28 weeks the arterial p0, was 60 mm Hg without oxygen and 76 mm Hg with oxygen. She became progressively more cyanosed and dyspneic during her stay in hospital. She was digi- talized, and continuous oxygen was recommended (on the hypothesis that it might prevent a rise in pulmonary vascular resistance) but she was unable to tolerate the mask for much of the time. At 30 weeks the pOZ was 40 mm Hg without oxygen and 80 mm Hg with oxygen. At 32 weeks she developed systemic hypertension and albuminuria. These signs worsened in spite of sedation, and it was decided to effect delivery by cesarean section at 33 weeks. The 24-hour urinary estriol excretion levels had been satisfactory. The anesthetic technique employed was preoxygenation, methohexitone, suxametho- nium chloride, endotracheal intubation, nitrous oxide and oxygen, and pancuronium bromide. Routine lower segment cesarean section was per- formed. The baby, a female, weighed 1.73 kg and made satisfactory progress. The patient developed a wound hematoma which required evacuation under local anesthetic later on the day of operation. On the third day of the puerperium she had a syn- copal attack: it was thought that this might have been due to a pulmonary embolus or pulmonary thrombosis. She was therefore anticoagulated, using heparin and warfarin. The p02 remained be- tween 42 mm Hg and 52 mm Hg for the first ten days of the puerperium. She was kept in bed for two weeks and then gradually mobilized, being allowed home 6 weeks after delivery, still taking warfarin. For about a year she remained disabled with fatigue, dyspnea and exertional syncope, but these symptoms gradually resolved, and five years later she was well and working full time, and was only slightly cyanosed.

Case 2

A heart lesion had been discovered at birth. The

patient was slightly cyanosed during childhood but there had been little limitation of activity. She only became short of breath on strenuous exertion. In 1958 at the age of 18yearscardiaccatheterization had shown a pulmonary artery pressure of 110/65 mm Hg with an identical brachial artery pressure, and an arterial oxygen saturation of 89 per cent. She was warned of the risks of becoming pregnant. In 1966 the arterial oxygen saturation was 79 per cent. She married in 1968 and took a combined estrogen and progestogen contraceptive pill until 1972, with- out deterioration.

In 1971 she developed congestive cardiac failure after an attack of bronchitis and was treated suc- cessfully with digoxin and diuretics. She stopped taking the contraceptive pill in 1972 as she wished to become pregnant in spite of the known risks. Her menstrual cycle was very irregular after stop- ping the pill; she thought that her last period was in September 1972. When first seen in the antenatal clinic in January 1973, she was slightly breathless on exertion and had been using two pillows instead of one for a month. Her height was 6 feet and she weighed 66.5 kg. She was polycythemic (hemoglobin 18.6 g per 100 ml) and cyanosed. She had a high arched palate and arachnodactyly.

She was admitted for rest for two weeks in March, and then readmitted to hospital from the end of April until she died. She was slightly breathless on exertion and complained of palpitations at times, but otherwise she remained well during the preg- nancy.

J’n order to establish the length of gestation as accurately as possible serial ultrasound measure- ments of the fetal biparietal diameter were made in pregnancy. The predicted term date was the be- ginning of July. Fetal growth proceeded at a nor- mal rate, and the serial maternal urinary estriol ex- cretion levels remained satisfactory. The patients’ systemic blood pressure level remained between 105170 mm Hg and 120/85 mm Hg.

It was decided that elective cesarean section would be the safest method of delivery, and this was performed at an estimated 37weeks’ gestation. The patient was anesthetized using preoxygenation, thiopentone, suxamethonium chloride, endotra- ch.eal intubation, nitrous oxide and oxygen, and decamethonium. A routine lower segment cesarean section was performed with delivery of a live male

G. C. L. Lachelin, Eisenmenger syndrome and pregnancy 189

infant weighing 2.52 kg in good condition. He made satisfactory progress.

The patient was kept under careful observation postoperatively, and her postoperative course was entirely satisfactory until the eighth day when she collapsed and died as she got out of the bath. She had been allowed out of bed from the fourth day and had taken two baths since then without ex- periencing any untoward effects.

Postmortem examination revealed a large inter- ventricular septal defect measuring 3 cm by 2.8 cm involving the membranous and upper muscular portions of the septum, with a further 1 cm dia- meter defect near the ventricular apex. The foramen ovale was patent but the rest of the interatrial sep- tum was normal. The wall of the right ventricle was 12 mm thick, that of the left ventricle 16 mm thick. There were widespread atheromatous plaques in the major pulmonary arteries indicating long- standing severe pulmonary hypertension. There was no evidence of pulmonary embolism.

Discussion

Jones and Howitt (1965) in a comprehensive review of the literature found 21 cases of pregnancy in patients with the Eisenmenger syndrome as defined by Wood (1958) and added 16 of their own. There were 10 deaths in the combined series of 37 patients, giving a maternal mortality of 27 per cent which is far higher than that in any other type of congenital heart disease - 1.8 per cent with all other shunts, 4.2 per cent in Fallot’s tetralogy, and 4.3 per cent in coarctation of the aorta-and second only to that of 53 per cent in primary pul- monary hypertension (Jones and Howitt, 1965). Death most commonly occurs postpartum. In seven cases? as with one of our patients, death occurred suddenly between three and eleven days postpartum. No pulmonary emboli were demonstrated in the four of their patients who had a postmortem. Two other patients died shortly after cesarean section. The tenth patient died during labor.

The perinatal mortality is less easy to assess as the information in some cases described is inadequ- ate. In the series of Jones and Howitt (1965) 16 patients had 26 pregnancies. Seven of these mis- carried spontaneously and four were terminated. One patient died undelivered. There were 12 live

births and 2 still births from the 14 other pregnan- cies which went beyond 28 weeks.

Only relatively few cases of pregnancy in patients with the Eisenmenger syndrome have been described since 1965.

Neilson, Galea and Blunt (1971) reported the results of pregnancy in eight patients who had the the Eisenmenger syndrome as defined by Wood (1958). Two died in the puerperium, one died during an operation for a ruptured ectopic pregnancy, a.nd one died shortly after a vaginal hysterectomy per- formed 6 weeks postpartum, for sterilization. The other four patients survived one or more pregnan- cies.

Nanayakkara and Pieris (1964) and Curforth, Catchlove, Knight and Dudgeon (1968) each de- scribed a successful pregnancy in a patient with the Eisenmenger syndrome. Loffer (1967) uses a differ- ent definition of the Eisenmenger complex and his cases are not strictly comparable.

Batson (1974) in a recent article reported no maternal deaths in a series of eight patients with the Eisenmenger syndrome as defined by Wood (1958).

Seven maternal deaths in patients with the Eisenmenger syndrome were included in the 1967 to 1969 Report on Confidential Enquiries into Mater- nal Deaths in England and Wales (1972).

Certain points in the management of patients with the Eisenmenger syndrome are apparent from reading the literature. Firstly, it is important to make the diagnosis. In the series of Jones and Howitt (1965) this was not done in one patient until she complained of exertional dyspnea at 34 weeks gestation. She died four days after delivery. The diagnosis should be considered by obstetricians in all patients with a hemoglobin level of more than 15 g per 100 ml, with cyanosis, or with clubbing, or hemoptysis, and also in any patient who complains of dyspnea or syncope, both of which are of course common complaints in normal pregnancy. Patients with the Eisenmenger complex are typically cya- nosed and exhibit clubbing of the fingers, but both of these features may be absent. On examination of the heart there is usually a systolic murmur at the left sternal edge, but this is usually soft and short and may be absent. The second heart sound is characteristically loud and may be palpable.

Termination of pregnancy is a hazardous under- taking, but if performed vaginally before 10 weeks

190 G. C. L. Lachelin, Eisenmenger syndrome and pregnancy

it is probably less hazardous than continuing the pregnancy. Preeclampsia is a serious complication and may lead to rapid deterioration in the patient’s condition.

The functional capacity before pregnancy is not a good guide to the outcome of pregnancy in these patients. In Jones and Howitt’s series 2 of the 8 patients in functional class I died, and 3 of the 8 patients in functional class II died. Admission to hospital for a considerable part of the pregnancy is probably a wise precaution. The timing of delivery will depend on a continuous assessment of maternal and fetal well being during pregnancy. Different authors have different opinions on the best mode of delivery. The majority of patients described in the literature delivered vaginally. Only one of these died during labor. Crawford, Mills and Pentecost (1971) recommend epidural anesthesia during labor in the management of these patients. Our two pa- tients were delivered by cesarean section as it was felt that an electively timed procedure with adequate anesthetic and monitoring facilities offered the safest management for the patient. Avoidance of systemic hypertension aiid hypstension is of vital importance in these patients.

The puerperium is undoubtedly a particularly dangerous time, and most deaths occur postpartum. There is often some deterioration in the patients’ overall condition even if they do survive. Some writers advocate strict bed rest postpartum, but others suggest that this will increase the risk of deep vein thrombosis and embolism, and advocate early ambulation and return to normal activities such as bathing (Crawford et al., 1971). However, there must inevitably be some peripheral vasodilatation during bathing, and it may well be thought that this was a contributory factor in the death of our pa- tient.

The question of contraception is very important in these patients. Although a combined estrogen and progestogen contraceptive pill is usually thought to be contraindicated in patients with pul- monary hypertension, no deterioration occurred in the condition of either of our patients when they were taking the pill. The risks of pregnancy are pro- bably greater than those of oral contraception in patients with the Eisenmenger syndrome.

Acknowledgements

I would like to thank Professor D. V. I. Fair- weather, Dr. A. Hollman and Mr. J. M. Holmes for permission to report on their patients, and for their advice and encouragement in the preparation of this paper.

References

Batson, G. A. (1974): J. Obstet. Gynaec. Brit. Cwlth, 81, 549. Crawford, J. S., Mills, W. G. and Pentecost, B. L. (1971):

Brit. J. Anaesth., 43, 1091. Cutforth, R., Catchlove, B., Knight, L. W. and Dudgeon, G.

(1968): Austr. N. Z. J. Obstet. and Gynaec., 8, 202. Eisenmenger, V. (1897): Z. klin. Med., 32, Suppl., 1. Jones, A. M. and Howitt, G. (1965): Brit. med. J., 1, 1627. Loffer, F. D. (1967): Obstet. and Gynec., 29, 2.235. Nanayakkara, S. H. P. and Pieris, E. V. (1964): Postgrad.

med. J., 40, 670. Neilson, G., Galea, E. G. and Blunt, A. (1971): Med. J.

Aust., 1, 431. Wood, P. (1958): Brit. med. J., 2, 701.

Reprint requests to: Dr. G. C. L. Lachelin, Department of Obstetrics and Gynae- cology, University College Hospital, Huntley Street, London, W.C. I, England.