- Email: [email protected]
Two successful pregnancies in a patient with single ventricle and transposition of the great arteries
International Journal of Cardiology, Elsevier Biomedical Press
1 (1982) 257-262
251
Two successful pregnancies .in a patient with single ventricle and transposition of the great arteries Guido
Leibbrandt,
Ulrich
Mtinch
Department of Medicine, Kantonsspital (Received
and Martin
Lucerne, Switzerland
16 April 1981; revised version received 29 September
Leibbrandt G, Munch U, Gander single ventricle and transposition 257-262.
Gander
1981; accepted
30 September
1981)
M. Two successful pregnancies in a patient with of the great arteries. Int J Cardiol 1982; 1:
A ZPyear-old female with transposition of the great arteries, single ventricle, a small open ductus arteriosus and a mild aortic incompetence experienced two successful pregnancies without major complications. The benign course of pregnancy is attributed to the relatively low pulmonary vascular resistance and to the fact that the patient has not yet developed an Eisenmenger syndrome.
Introduction Better diagnostic and therapeutic measures for patients with congenital heart disease have led to an increased number of female patients reaching adult life and being able to give birth to children [ 11. The cardiac complication rate of pregnancy in patients with common heart disease is well known [2,6]. In rare and hemodynamitally complex disorders, however, it may be difficult to evaluate the real cardiac and pulmonary reserves. This is particularly true for cyanotic heart defects with the exception of Fallot’s tetralogy [2,6]. Because of the high complication rate in these severe heart lesions women are discouraged to complete pregnancies. Experience with such patients is therefore limited and prognosis can only be worked out by comparing isolated case reports [3,5].
Case report Our patient, a 29-year-old female, was born of a normal pregnancy and delivery. Cyanosis and a heart murmur were present at birth. Because of marked exertional Ol67-5273/82/0000-0000/$02.75
0 1982 Elsevier Biomedical
Press
25X
m
259
dyspnea (N.Y.H.A. stage II-III) she was exempted from gymnastics at school. Later she worked fulltime in an office. In June 1970 the patient was seen for disability at the Department of Medicine, University Hospital, Zurich. On physical examination she (164 cm, 44.4 kg) showed marked central cyanosis and clubbing. The blood pressure was 130/80 mm Hg and the heart rate regular, 88/minute. The venous pulse revealed a prominent A wave. On palpation there was a marked precordial thrust and an apical impulse at the fifth intercostal space and the axillary line. On auscultation the first heart sound was normal and an accentuated single second heart sound was heard. A loud diamondshaped systolic and an early diastolic decrescendo murmur were present. The liver was not enlarged and there was no edema. The electrocardiogram showed sinus rhythm, a QRS axis of 70” and left ventricular hypertrophy which was confirmed by the vectorcardiogram. Chest X-rays demonstrated an enlarged heart, a small aorta, large pulmonary arteries and an augmented pulmonary vasculature (Fig. 1). Exercise testing was stopped at 50 W because of severe dyspnea at a heart rate of 160/minute. The angiocardiogram revealed a single ventricle, transposition of the great arteries, a small open ductus arteriosus and mild aortic incompetence. Further findings of the cardiac catheterization are shown in Table 1. From the oxygen saturation steps and an oxygen uptake of 180 ml/minute a pulmonary flow of 14.0 l/minute was calculated (cardiac output 3.1 l/minute, cardiac index 2.14 l/minute per m2). The pulmonary vascular resistance was 360 dyn . second. cme5, the systemic vascular resistance 2013 dyn . second. cm-‘. Against medical advice the patient refused sterilization and experienced a miscarriage in 1974 at 12 weeks of pregnancy. In 1975 she was admitted to our hospital at the 40th week of pregnancy. Delivery was obtained by cesarean section with peridural anesthesia without complications. The prepartal hemoglobin was 18.5 g/100 ml, and the hematocrit was 55%. The infant was a male weighing 1540g who had an Apgar rating of 7/9/10. The child did not show any malformations and developed well. The placenta demonstrated several infarctions.
TABLE
1
Cardiac
catheterization
data. Pressure
Mean pressure
(mm Hg)
(mm Hg)
Superior vena cava Inferior vena cava Right atrium Common ventricle Pulmonary artery PCP Ascending aorta
0=60=4 1IO/O-6 95/46 a = 10 0 = 10 102/66
2 70 7 80
Arterial oxygen saturation (W) 69.5 68.0 68.5 94.5 88.5 84.5
Fig. 2. The two-dimensional echocardiogram shows a univentricular heart from left ventricular type with rudimentary outlet chamber of the right ventricle and straddling atrioventricular valve. CV=common ventricle; RRC=rudimentary right chamber; RAVV=straddling right AV valve: LA= left atrium; RA= right atrium. (R. Jenny M.D., Department of Medicine, University Hospital. Zurich.)
Again the patient rejected sterilization, and after a second spontaneous abortion (12 weeks) she became once more pregnant in 1979. In the last trimester her activity was limited by fatigue and dyspnea on exertion. The patient was hospitalized in the 37th week of gestation and uneventfully delivered by cesarean section with peridural anesthesia. The prepartal hemoglobin was 18.5 g/100 ml, the hematocrit 60%. The arterial oxygen-saturation was 84%. The male newborn was again small-for-date (1520 g) and had an Apgar rating of 9/ lO/lO. No malformations were found and the child developed well. The placenta showed several infarctions. A postpartal fever
261
subsided without evidence of endocarditis, and the patient left our hospital 5 weeks after delivery. She is still able to perform her household duties herself. An echocardiogram was done on 13 November 1980 which confirmed the angiographic diagnoses (Fig. 2).
Discussion There is a close correlation between the incidence of complications during pregnancy in women with congenital heart disease and their hemodynamic findings, the shunt volumes and the pulmonary vascular resistance. A particularly high risk exists in patients with primary pulmonary hypertension or patients with Eisenmenger syndrome [ 1,2]. Patients with a single ventricle and transposition of the great arteries rarely reach adult life [4]. The present patient subject to this complex malformation carried two successful pregnancies which appears highly remarkable. She most probably succeeded due to a favorably balanced shunt brought about by a relatively low pulmonary vascular resistance. Even by the age of 29 years she has not developed an Eisenmenger syndrome [7], and has tolerated well her elevated cardiac output with diminishing peripheral resistance during both pregnancies. The increase of the hemoglobin during the pregnancies (1975: 18.2 g/100 ml, 1980: 19.3 g/100 ml) suggests a temporary increase in right-to-left shunt. Otherwise the hemodynamic situation remained fairly constant over a period of 10 years (1970-1980). Arterial oxygen saturation, hematocrit and heart size on chest X-ray remained unchanged. The two successful pregnancies in a patient with single ventricle and transposition of the great arteries are remarkable and, so far, not reported in the literature. In spite of this observation, the present attitude towards pregnancies in these patients should be retained [1,6]. Should a patient wish to complete her pregnancy, however, this may be possible without major complications provided the pulmonary vascular resistance is not fixed.
Acknowledgment We appreciate the help of Prof. M. Rothlin (Department of Medicine, University Hospital, Zurich) who allowed us to review the hemodynamic data and the angiogram of the patient.
References I Conradson TB, Werkb L. Management of heart disease in pregnancy. Prog Cardiovasc Dis 1974; 16: 407-4 19. 2 Jones AM. Howitt G. Eisenmenger syndrome in pregnancy. Br Med J 1965; 1: 1627-1631.
262
3 Juzpe AA, Johnson FL, Robinson JG. Successful pregnancy in a patient with single ventricle and other congenital cardiac anomalies. Can Med Assoc J 1970; 103: 1073-1075. 4 Newfeld EA, Paul MH, Muster AJ, Idriss FS. Pulmonary vascular disease in complete transposition of the great arteries: a study of 200 patients. Am J Cardiol 1974; 34: 75-82. 5 Seeds JW, Cefalo RC. Pregnancy with congenital heart disease: a two-chambered heart. Am J Obstet Gynecol 1977; 127: 213-214. 6 Szekely P, Snaith L. Heart disease and pregnancy. Churchill Livingstone, 1974. 7 Wood P. The Eisenmenger syndrome. Br Med J 1958; 2: 701-709.
1 (1982) 257-262
251
Two successful pregnancies .in a patient with single ventricle and transposition of the great arteries Guido
Leibbrandt,
Ulrich
Mtinch
Department of Medicine, Kantonsspital (Received
and Martin
Lucerne, Switzerland
16 April 1981; revised version received 29 September
Leibbrandt G, Munch U, Gander single ventricle and transposition 257-262.
Gander
1981; accepted
30 September
1981)
M. Two successful pregnancies in a patient with of the great arteries. Int J Cardiol 1982; 1:
A ZPyear-old female with transposition of the great arteries, single ventricle, a small open ductus arteriosus and a mild aortic incompetence experienced two successful pregnancies without major complications. The benign course of pregnancy is attributed to the relatively low pulmonary vascular resistance and to the fact that the patient has not yet developed an Eisenmenger syndrome.
Introduction Better diagnostic and therapeutic measures for patients with congenital heart disease have led to an increased number of female patients reaching adult life and being able to give birth to children [ 11. The cardiac complication rate of pregnancy in patients with common heart disease is well known [2,6]. In rare and hemodynamitally complex disorders, however, it may be difficult to evaluate the real cardiac and pulmonary reserves. This is particularly true for cyanotic heart defects with the exception of Fallot’s tetralogy [2,6]. Because of the high complication rate in these severe heart lesions women are discouraged to complete pregnancies. Experience with such patients is therefore limited and prognosis can only be worked out by comparing isolated case reports [3,5].
Case report Our patient, a 29-year-old female, was born of a normal pregnancy and delivery. Cyanosis and a heart murmur were present at birth. Because of marked exertional Ol67-5273/82/0000-0000/$02.75
0 1982 Elsevier Biomedical
Press
25X
m
259
dyspnea (N.Y.H.A. stage II-III) she was exempted from gymnastics at school. Later she worked fulltime in an office. In June 1970 the patient was seen for disability at the Department of Medicine, University Hospital, Zurich. On physical examination she (164 cm, 44.4 kg) showed marked central cyanosis and clubbing. The blood pressure was 130/80 mm Hg and the heart rate regular, 88/minute. The venous pulse revealed a prominent A wave. On palpation there was a marked precordial thrust and an apical impulse at the fifth intercostal space and the axillary line. On auscultation the first heart sound was normal and an accentuated single second heart sound was heard. A loud diamondshaped systolic and an early diastolic decrescendo murmur were present. The liver was not enlarged and there was no edema. The electrocardiogram showed sinus rhythm, a QRS axis of 70” and left ventricular hypertrophy which was confirmed by the vectorcardiogram. Chest X-rays demonstrated an enlarged heart, a small aorta, large pulmonary arteries and an augmented pulmonary vasculature (Fig. 1). Exercise testing was stopped at 50 W because of severe dyspnea at a heart rate of 160/minute. The angiocardiogram revealed a single ventricle, transposition of the great arteries, a small open ductus arteriosus and mild aortic incompetence. Further findings of the cardiac catheterization are shown in Table 1. From the oxygen saturation steps and an oxygen uptake of 180 ml/minute a pulmonary flow of 14.0 l/minute was calculated (cardiac output 3.1 l/minute, cardiac index 2.14 l/minute per m2). The pulmonary vascular resistance was 360 dyn . second. cme5, the systemic vascular resistance 2013 dyn . second. cm-‘. Against medical advice the patient refused sterilization and experienced a miscarriage in 1974 at 12 weeks of pregnancy. In 1975 she was admitted to our hospital at the 40th week of pregnancy. Delivery was obtained by cesarean section with peridural anesthesia without complications. The prepartal hemoglobin was 18.5 g/100 ml, and the hematocrit was 55%. The infant was a male weighing 1540g who had an Apgar rating of 7/9/10. The child did not show any malformations and developed well. The placenta demonstrated several infarctions.
TABLE
1
Cardiac
catheterization
data. Pressure
Mean pressure
(mm Hg)
(mm Hg)
Superior vena cava Inferior vena cava Right atrium Common ventricle Pulmonary artery PCP Ascending aorta
0=60=4 1IO/O-6 95/46 a = 10 0 = 10 102/66
2 70 7 80
Arterial oxygen saturation (W) 69.5 68.0 68.5 94.5 88.5 84.5
Fig. 2. The two-dimensional echocardiogram shows a univentricular heart from left ventricular type with rudimentary outlet chamber of the right ventricle and straddling atrioventricular valve. CV=common ventricle; RRC=rudimentary right chamber; RAVV=straddling right AV valve: LA= left atrium; RA= right atrium. (R. Jenny M.D., Department of Medicine, University Hospital. Zurich.)
Again the patient rejected sterilization, and after a second spontaneous abortion (12 weeks) she became once more pregnant in 1979. In the last trimester her activity was limited by fatigue and dyspnea on exertion. The patient was hospitalized in the 37th week of gestation and uneventfully delivered by cesarean section with peridural anesthesia. The prepartal hemoglobin was 18.5 g/100 ml, the hematocrit 60%. The arterial oxygen-saturation was 84%. The male newborn was again small-for-date (1520 g) and had an Apgar rating of 9/ lO/lO. No malformations were found and the child developed well. The placenta showed several infarctions. A postpartal fever
261
subsided without evidence of endocarditis, and the patient left our hospital 5 weeks after delivery. She is still able to perform her household duties herself. An echocardiogram was done on 13 November 1980 which confirmed the angiographic diagnoses (Fig. 2).
Discussion There is a close correlation between the incidence of complications during pregnancy in women with congenital heart disease and their hemodynamic findings, the shunt volumes and the pulmonary vascular resistance. A particularly high risk exists in patients with primary pulmonary hypertension or patients with Eisenmenger syndrome [ 1,2]. Patients with a single ventricle and transposition of the great arteries rarely reach adult life [4]. The present patient subject to this complex malformation carried two successful pregnancies which appears highly remarkable. She most probably succeeded due to a favorably balanced shunt brought about by a relatively low pulmonary vascular resistance. Even by the age of 29 years she has not developed an Eisenmenger syndrome [7], and has tolerated well her elevated cardiac output with diminishing peripheral resistance during both pregnancies. The increase of the hemoglobin during the pregnancies (1975: 18.2 g/100 ml, 1980: 19.3 g/100 ml) suggests a temporary increase in right-to-left shunt. Otherwise the hemodynamic situation remained fairly constant over a period of 10 years (1970-1980). Arterial oxygen saturation, hematocrit and heart size on chest X-ray remained unchanged. The two successful pregnancies in a patient with single ventricle and transposition of the great arteries are remarkable and, so far, not reported in the literature. In spite of this observation, the present attitude towards pregnancies in these patients should be retained [1,6]. Should a patient wish to complete her pregnancy, however, this may be possible without major complications provided the pulmonary vascular resistance is not fixed.
Acknowledgment We appreciate the help of Prof. M. Rothlin (Department of Medicine, University Hospital, Zurich) who allowed us to review the hemodynamic data and the angiogram of the patient.
References I Conradson TB, Werkb L. Management of heart disease in pregnancy. Prog Cardiovasc Dis 1974; 16: 407-4 19. 2 Jones AM. Howitt G. Eisenmenger syndrome in pregnancy. Br Med J 1965; 1: 1627-1631.
262
3 Juzpe AA, Johnson FL, Robinson JG. Successful pregnancy in a patient with single ventricle and other congenital cardiac anomalies. Can Med Assoc J 1970; 103: 1073-1075. 4 Newfeld EA, Paul MH, Muster AJ, Idriss FS. Pulmonary vascular disease in complete transposition of the great arteries: a study of 200 patients. Am J Cardiol 1974; 34: 75-82. 5 Seeds JW, Cefalo RC. Pregnancy with congenital heart disease: a two-chambered heart. Am J Obstet Gynecol 1977; 127: 213-214. 6 Szekely P, Snaith L. Heart disease and pregnancy. Churchill Livingstone, 1974. 7 Wood P. The Eisenmenger syndrome. Br Med J 1958; 2: 701-709.