Outcome of pregnancy in patients with congenitally corrected transposition of the great arteries.

To assess maternal and fetal outcome of pregnancy in patients with congenitally corrected transposition of the great arteries, we reviewed 19 patients (18 retrospectively) who had 45 pregnancies. Their ages ranged from 18 to 40 years (mean 27) at the time of pregnancy. Thirty-six percent of the pregnancies were undertaken while patients were cyanosed, 7% in patients with unpaced complete heart block, and 16% were undertaken after surgical repair of the associated anomalies. Change in functional class and maternal cardiovascular complications during pregnancy were analyzed as well as number of live births, miscarriages, elective termination of pregnancies, timing of delivery, and incidence of cardiac defects in the live offspring. Five patients (26% of patients) developed cardiovascular complications during pregnancy, namely congestive heart failure (3 patients), worsening cyanosis (1 patient), and cerebrovascular accident (1 patient). No maternal deaths occurred. There were 27 live births (60%), 12 miscarriages (27%), and 6 elective terminations of pregnancy (13%). Cyanosis was a significant risk factor for miscarriage. One live offspring had congenital heart disease. Close follow-up of these patients during pregnancy, by a team of experienced physicians, is recommended.     

Successful pregnancies after transvenous cardiac resynchronization therapy in a woman with congenitally corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries is a rare heart defect that can be associated with systemic ventricular dysfunction and conduction disturbances. The use of cardiac resynchronization therapy in patients with congenital heart disease is not fully established, and achievement of successful pregnancies after implantation of transvenous, biventricular system has never been described, and which resulted in a significant clinical improvement.We describe a 33-year-old female with congenitally corrected transposition of the great arteries, who achieved six pregnancies and successful vaginal deliveries. The two last pregnancies were achieved after cardiac resynchronization therapy for systemic ventricular dysfunction and complete heart block. A congenital cardiac disease has been identified in only one offspring.     

Ebstein's anomaly: Outcome of pregnancy

Objectives. The outcome of pregnancy in Ebstein's anomaly was studied in 72 such patients (44 women, 28 men) who had had pregnancies or fathered children.Background. Patients with Ebstein's anomaly often reach childbearing age. Reports of the outcome of pregnancy in Ebstein's anomaly are available; however, the number of patients is small.Methods. The medical and surgical data bases at the Mayo Clinic were reviewed, and 145 patients (62 men, 83 women) of childbearing age with Ebstein's anomaly were located. All patients were contacted, and 72 patients (44 women, 28 men) with offspring were identified and reviewed in detail to assess the outcome of pregnancy.Results. Forty-four women had 111 pregnancies resulting in 85 live births (76%). Seventy-six deliveries (89%) were vaginal, and nine (11%) were by cesarean section. Twenty-three deliveries were premature. There were 19 spontaneously unsuccessful pregnancies, 7 therapeutic abortions and 2 early neonatal deaths. The mean birth weight of the infants born to cyanotic women was 2.53 kg, which was significantly lower than the mean birth weight of infants born to acyanotic women (3.14 kg [p < 0.001]). The overall incidence of congenital heart disease in the 158 offspring of parents with Ebstein's anomaly was 4% (6 of 158). The incidence of congenital heart disease was 6% (5 of 83) in the offspring of women with Ebstein's anomaly and 1% (1 of 75) in that of men. There was a 0.6% (1 of 158) incidence of familial Ebstein's anomaly. There were no serions pregnancy-related maternal complications, which included maternal death, stroke, congestive heart failure, arrhythmias or endocarditis.Conclusions. Pregnancy in women with Ebstein's anomaly is well tolerated. It is associated with an increased risk of prematurity, fetal loss and congenital heart disease in the offspring. In addition, a significantly lower birth weight is found in the offspring of cyanotic versus acyanotic women with Ebstein's anomaly. Paternal Ebstern's anomaly also seems to result in an increased risk of congenital heart disease in the offspring compared with the inddence in the general population.     

Outcome of pregnancy after the mustard operation for transposition of the great arteries with intact ventricular septum

Objectives. This study examined the outcome of pregnancy in patients with transposition of the great arteries and an intact ventricular septum after a Mustard operation.Background. Before the introduction of surgical treatment, most children with transposition of the great arteries died in early infancy. A number of these patients have now reached their reproductive years. There is little information about the effect of pregnancy on cardiovascular status, particularly the ability of the right ventricle to adjust to the hemodynamic changes of pregnancy. The outcome for the offspring and their risk of congenital heart disease are also unknown.Methods. Twenty-three female late survivors after the Mustard operation >15 years of age were reviewed in relation to the occurrence of pregnancy and its outcome. Serial echocardiographic estimates of right ventricular volume during pregnancy were made in three local patients.Results. Nine women had 15 pregnancies. They were asymptomatic before pregnancy and remained free from cardiac symptoms during each pregnancy. Right ventricular volume in the three patients studied increased during pregnancy but returned to normal at 8 to 11 weeks postpartum. There were 12 live births, 2 spontaneous abortions and 1 intrauterine death. None of the liveborn infants had evidence of congenital heart disease.Conclusions. In this small group of women with good quality late survival after a Mustard operation, pregnancy was well tolerated. We suspect that the incidence of congenital heart disease in infants of mothers with transposition of the great arteries will be at the lower end of the range for mothers with different types of congenital heart disease, but further data will be needed to confirm this.     

Etiologic correlates in a study of congenital heart disease in 56,109 births

From a prospective study of 56,109 total births some incidence factors and etiologic correlates for congenital heart disease have been identified. The risk of congenital heart disease in infants with Down's syndrome was found to be 1:3, for the offspring of mothers with congenital heart disease 1:20, for the offspring of diabetic mothers 1:39, and for twin infants 1:61. Data from the offspring of mothers with congenital heart disease and from offspring who are twins or siblings of twins suggest that there may be a relatively large element of inheritance in the etiology of pulmonary stenosis. The risk of a great vessel abnormality for offspring of diabetic mothers is reaffirmed. The role of maternal age in the production of congenital heart disease in the offspring is assessed, and several correlates of transposition of the great vessels are identified. Preliminary data are presented on the association of maternal viral infection with congenital heart disease in the offspring.     

Ressincronização cardíaca em doente com transposição congenitamente corrigida das grandes artérias

Congenitally corrected transposition of the great arteries is a rare heart defect that can be associated with systemic ventricular dysfunction and conduction disturbances.The use of cardiac resynchronization therapy in patients with congenital heart disease is not fully established.The authors report a case of cardiac resynchronization therapy in a 31‐year‐old woman with a history of congenitally corrected transposition of the great arteries and heart failure, who had undergone two heart surgeries and had a DDDR pacemaker implanted.To our knowledge this is the first case reported in Portugal of cardiac resynchronization therapy by transvenous access in a patient with congenitally corrected transposition of the great arteries.     

Congenitally corrected transposition of the great vessels: Localization of the site of complete atrioventricular block using His bundle electrograms

Complete atrioventricular block proximal to the bundle of His in a patient with congenitally corrected transposition of the great vessels was documented using His bundle electrograms. The spontaneous rhythm probably originated from the bundle of His and was responsive to carotid sinus massage, atropine and isometric and treadmill exercise. These electrophysiologic observations are consistent with recent anatomic studies of congenitally corrected transposition of the great vessels.     

Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: association with aortic arch obstruction

Aortic arch abnormalities are uncommon in patients with congenitally corrected transposition of the great arteries. Over a 20-year period, 10 patients with congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular (AV) valve were identified. Five neonates had severe systemic AV valve regurgitation with severe coarctation of the aorta (n = 4) or aortic atresia (n = 1) and presented with heart failure. Four died in the neonatal period and one who had coarctation repair died 7 months postoperatively. The remaining five patients with congenitally corrected transposition and Ebstein's anomaly had mild left AV valve regurgitation; none of these had aortic arch obstruction. In neonates who have coexisting Ebstein's anomaly of the systemic AV valve and congenitally corrected transposition, obstruction to aortic arch flow is common. Severe systemic AV valve regurgitation with a morphologic systemic right ventricle may have contributed to low anterograde flow in the ascending aorta prenatally and thereby to the aortic arch abnormality.     

Pregnancy outcomes after atrial repair for transposition of the great arteries

Increasingly, women born with complete transposition of the great arteries who have undergone atrial repair by either the Senning or the Mustard procedure are reaching childbearing age. This study reports on pregnancy outcomes after the atrial repair of transposition of the great arteries. Record review and standardized questionnaires were used to ascertain the outcomes of 70 pregnancies reported in 40 women (36 Mustard procedures, 4 Senning procedures). Of the 70 pregnancies, 54 resulted in 56 live births, 10 in miscarriages, and 6 in therapeutic abortions. At pregnancy, 31 women were in New York Heart Association class I, 8 were in class II, and 1 was in class III. Thirty-nine percent of the infants were delivered prematurely and weighed 2,714 +/- 709 g; 28% were delivered by cesarean section, 8 for cardiac indications. Maternal complications included arrhythmias in 5 women and hemoptysis in 2 women. Heart failure occurred in 6 women, developing during the second and third trimesters. Postpartum cardiac events developed 2 to 9 days postpartum: heart failure in 5 women, atrial fibrillation in 1 woman, and decreased oxygen saturation due to a new atrial baffle leak in 1 woman. Severe right ventricular (RV) failure led to cardiac transplantation after delivery in 1 woman; another developed heart failure and then died suddenly 1 month after delivery. There was 1 late death, 4 years after the patient's last pregnancy. In conclusion, pregnancy after atrial repair carries a moderate degree of risk and should be undertaken with caution.     

Accessory mitral valve tissue causing left ventricular outflow tract obstruction.

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.     

Accessory mitral valve tissue causing left ventricular outflow tract obstruction

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.     

Coarctation of the aorta: outcome of pregnancy.

OBJECTIVES: The study sought to determine the outcome of pregnancy in women with coarctation of the thoracic aorta. BACKGROUND: Patients with coarctation of the thoracic aorta are expected to reach childbearing age, but data on the outcome of pregnancy in this population are limited. METHODS: The Mayo Clinic database was reviewed for women of childbearing age (> or =16 years old) with a diagnosis of aortic coarctation evaluated from 1980 to 2000. Spectrum of cardiovascular disease, surgical history, and obstetrical and neonatal outcomes were determined. RESULTS: Fifty women with coarctation had pregnancies: 30 had coarctation repair before pregnancy, 10 had repair after pregnancy, 4 had repair both before and after pregnancy, and 6 had no history of repair. The 50 women had 118 pregnancies resulting in 106 births. There were 11 miscarriages (9%), 4 premature deliveries (3%), and 1 early neonatal death; 38 deliveries (36%) were by cesarean section. Of the 109 offspring, 4 (4%) had congenital heart disease. A patient with Turner syndrome died of a Stanford type A dissection at 36 weeks of pregnancy. Nineteen women (38%) were known to have hemodynamically significant coarctation during pregnancy (gradient > or =20 mm Hg). Fifteen women (30%) had hypertension during their pregnancy, 11 of whom (73%) had hemodynamically significant coarctation during that time (8 with native and 3 with residual/recurrent coarctation). CONCLUSIONS: Major cardiovascular complications were infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Systemic hypertension during pregnancy was common and related to the presence of a significant coarctation gradient.     

Pregnancy after atrial repair for transposition of the great arteries

OBJECTIVE: To investigate the risk of pregnancy in patients with transposition of the great arteries (TGA) who have undergone atrial repair. DESIGN: Retrospective analysis (1962-94) of 342 TGA patients who underwent atrial repair. Of 231 known late survivors, 48 were women over 18 years old who were interviewed about possible reproductive plans and previous pregnancies. As a control, comparison was made with data of 57 500 women (mean age 26 years) obtained from the Swiss Statistical Bank in Bern. RESULTS: Mean follow up was 13.7 years; 66% remained asymptomatic, 29% had mild to moderate cardiac symptoms, and 5% suffered from severe cardiac symptoms (New York Heart Association grade III-IV). Thirty six of the 48 women wished to bear children and, to date, there have been 10 live births, two spontaneous first trimester abortions, and one induced abortion at 16 weeks. During pregnancy there was one case of cardiac deterioration and two cases of pneumonia. There was no evidence of congenital heart disease in the children. CONCLUSIONS: In this relatively small series the completion of pregnancy in women with TGA who had undergone atrial repair and who had normal functional cardiac status was uncomplicated     

Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up.

OBJECTIVE--To assess the clinical course of adult patients with corrected transposition of the great arteries without associated anomalies. DESIGN--All patients with corrected transposition of the great arteries without associated anomalies were reviewed with complete clinical and echocardiographic assessment. The complications were evaluated in each decade. SETTING--Tertiary centre with a specific unit dealing with "grown-up" adolescent and adult congenital heart disease, designated as a quaternary centre and a general hospital with a referral centre for "grown-up" congenital heart disease. PATIENTS--18 patients (nine male and nine female) aged 16-61 years followed for 1-30 years (mean 10 years). RESULTS--There were no deaths. Six patients had a worsening ability index during follow up. Complications were: (a) complete heart block in seven, three of whom required pacemaker insertion; (b) significant left atrioventricular valve regurgitation in 50%, appearing only in the third decade (12%), with increasing frequency thereafter. Infective endocarditis was responsible for increasing left atrioventricular valve regurgitation in only one patient; (c) supraventricular arrhythmia appeared in the fifth decade, and occurred in all patients over the age of 60 years. One patient aged 61 had recurrent sustained ventricular tachycardia; and (d) congestive heart failure developed only after 50 years in 66%. One patient had severe left atrioventricular valve regurgitation; the function of the systemic ventricle was only moderately reduced in the other three. Three of the nine women had seven uneventful pregnancies. CONCLUSIONS--Patients with corrected transposition of the great arteries without associated defects may remain undiagnosed until adult life. Symptoms occur rarely before the fourth and fifth decades, when rhythm disturbance, left atrioventricular valve regurgitation, and moderately impaired systemic ventricular function cause congestive cardiac failure. The role of pacemaker insertion or surgery for left atrioventricular valve regurgitation needs further assessment.     

Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries.

AIMS: To investigate magnitude and determinants of risks during pregnancy in women with Mustard or Senning repair for complete transposition of the great arteries (TGA). METHODS AND RESULTS: Using a nationwide registry (CONCOR), 70 women with Senning (23%) or Mustard (77%) repair for TGA were enrolled. A total of 28 patients had 69 pregnancies (two twins), including 17 spontaneous miscarriages and three elective abortions. During 39 of the 49 completed pregnancies, complications were observed. The most important cardiac complication was clinically significant arrhythmia (n=11, 22%), especially occurring in patients with a prior history of arrhythmia. Important general pregnancy complications were preeclampsia (n=5, 10.2%) and pregnancy-induced hypertension (n=4, 8.2%). Obstetric complications included premature rupture of membranes (n=7, 14.3%), premature labour (n=12, 24.4%), premature delivery (n=16, 31.4%), and thrombo-embolic complications (n=2, 4.1%). Mean (singleton) pregnancy duration was 36+/-5 weeks. Eleven of the 51 children (21.6%) were small for gestational age. Foetal and neonatal mortality combined was 11.8% (n=6). No recurrence of congenital heart disease in the offspring was documented. CONCLUSION: In this largest report on pregnancy in women with atrial-corrected TGA to date, a high incidence of obstetric complications and mortality in the offspring was observed.     

A case of congenital cardiomyopathy with acute heart failure

A 45-year-old woman was admitted to our cardiology department for palpitations and dyspnoea. She had previously been investigated by echocardiography several times, resulting in a diagnosis of hypertrophic cardiomyopathy. However, a congenitally corrected transposition of the great arteries was diagnosed by our echocardiographic examination. The patient underwent electrophysiological evaluation and the accessory pathway was successfully ablated by applying radiofrequency pulses. This case report identifies that in patients with congenitally corrected transposition of the great arteries the primary diagnosis by echocardiography can sometimes be missed and that these patients are increasingly liable to develop Congestive Heart Failure with advancing age. In order to avoid diagnostic mistakes, more widespread dissemination of information about this congenital heart defect is essential.     

Complications of pregnancy and child development after cessation of treatment with 6-mercaptopurine for inflammatory bowel disease

PURPOSE: 6-Mercaptopurine (6-MP) has proven efficacy in the therapy of inflammatory bowel disease. Its teratogenicity is demonstrated in animal studies when used at very high doses, whereas human data suggest that 6-MP at maintenance doses is safe. We report the outcome of 72 pregnancies in patients with inflammatory bowel disease who were previously treated with 6-MP with three different doses of 50, 75, and 100 mg/d, for a median duration of 18 months, along with long-term follow-up of the children. METHODS: We have compared the outcome of pregnancies and development of the offspring in the following two groups: group 1, patients with inflammatory bowel disease who conceived 6 months to 22 years after stopping 6-MP (median 72 months); and group 2, patients with inflammatory bowel disease who never received 6-MP prior to conception. All pregnancies were evaluated in terms of outcome: live full-term birth, premature delivery, stillbirth, spontaneous abortion, ectopic pregnancy, and therapeutic dilatation and curettage. Data on children were obtained regarding birth weight, congenital anomalies, and development. RESULTS: Group 1 included 72 pregnancies carried by 29 women. There were 51 live births (4 premature), 16 spontaneous abortions, 1 stillbirth, 2 therapeutic abortions due to abnormal amniocentesis, and 2 ectopic pregnancies. The total incidence of fetal loss was 29.2%. In group 2, 75 women had 140 pregnancies resulting in 120 live births (8 premature), 18 spontaneous abortions, and 2 stillbirths. There were no cases of ectopic pregnancies or abnormal amniocentesis. The total incidence of fetal loss was 14.3%. There was no increase in the incidence of developmental defects when the mothers had been treated with 6-MP prior to pregnancy. CONCLUSIONS: The incidence of fetal loss is higher in women with inflammatory bowel disease who had been previously treated with 6-MP compared with those who had not. Whether this was related to the older age at conception in 6-MP group, longer duration of disease, initially more severe disease, or use of 6-MP we cannot tell.     

Safety of hydroxychloroquine in pregnant patients with connective tissue diseases: a study of one hundred thirty-three cases compared with a control group

OBJECTIVE: The use of hydroxychloroquine (HCQ) in pregnancy remains controversial. The recent demonstration that HCQ passes across the placenta, with cord blood concentrations nearly identical to those found in maternal blood, emphasizes the need for careful evaluation of pregnancies in women receiving HCQ. However, only small series of HCQ-treated pregnant women have been reported, and most of these studies had no control group. We now report our experience with 133 pregnancies in women being treated with HCQ, resulting in 117 live births. Results in the HCQ group are compared with those in a control group. METHODS: One hundred thirty-three consecutive pregnancies in 90 women treated with 200 mg of HCQ either twice daily (122 pregnancies) or once daily (11 pregnancies) were studied. These pregnancies were compared with 70 consecutive pregnancies in 53 women with similar disorders who did not receive HCQ. Electrocardiography was performed in 47 children of mothers treated with HCQ and in 45 children in the control group. RESULTS: Eighty-eight percent of pregnancies in the HCQ group and 84% of those in the control group ended successfully with a live birth. The outcomes of pregnancy were not statistically different between groups. One child in each group died of causes related to prematurity. Three malformations were observed in the HCQ group (1 hypospadias, 1 craniostenosis, and 1 cardiac malformation) versus 4 in the control group. On the electrocardiograms, the PR interval and the corrected QT interval were not statistically different between groups. No visual, hearing, growth, or developmental abnormalities were reported in any of the children at the last follow-up (ages 12-108 months; mean age 26 months). CONCLUSION: Our findings support preliminary evidence for the safety of HCQ therapy during pregnancy. This treatment probably should be maintained throughout pregnancy in patients with systemic lupus erythematosus.     

Medium-term outcome of anatomically repaired congenitally corrected transposition: the double switch operation

BACKGROUND: The double switch operation is emerging as the procedure of choice for congenitally corrected transposition of the great arteries. However, rhythm disturbances in the postoperative period are rarely discussed. METHODS AND RESULTS: Eighteen survivors who underwent corrective surgery for congenitally corrected transposition of the great arteries were followed up. Patients in group I (n=8), who also had a ventricular septal defect and pulmonary stenosis, had undergone the Senning plus Rastelli operation. Patients in group II (n=10), who did not have pulmonary stenosis, had undergone the Senning and arterial switch operation. The patients were followed up by periodical clinical examination, echocardiography and 24-hour Holter monitoring. In group I, follow-up ranged from 24 to 66 months (mean 44 months). There was no late death and all the patients are symptom free. There was no significant atrioventricular valve regurgitation and left ventricular function was normal. There were no rhythm disturbances. In group II, follow-up ranged from 2 to 72 months (mean 48 months). There were 2 late deaths due to atrial tachyarrhythmia and residual pulmonary hypertension 36 and 8 months after the procedure, respectively. One patient had significant mitral regurgitation and required mitral valve replacement. Three patients had recurrent atrial/junctional tachyarrhythmia: one of them was lost to follow-up after 1 year while another died of resistant atrial tachyarrhythmia. The third patient underwent mitral valve replacement for severe mitral regurgitation and developed complete heart block necessitating a permanent pacemaker implantation. CONCLUSIONS: Though good long-term results are obtained following the double switch operation, the problem of atrial arrhythmias still needs to be addressed suitably.     

Anticoagulation in pregnancy with mechanical heart valves: 10-year experience

Anticoagulation in pregnancy was evaluated in 33 women with a mechanical heart valve prosthesis who had 53 pregnancies between 1994 and 2006. Their mean age at valve operation was 24.4 +/- 5.4 years, and 22 (67%) had isolated mitral valve disease. Of these patients, 22 had a single pregnancy, 5 had 2 pregnancies, 3 had 3, and 3 had 4. In 43 pregnancies, the patients took warfarin throughout; in the other 10, heparin was used in the first trimester followed by warfarin until the last 15 days. Mean international normalized ratio and warfarin levels before, during, and after pregnancy were similar. Complications occurred in 3 (6%) women who had thrombosed valves: 2 (20%) in the heparin group and 1 (2%) who had warfarin only. Live births resulted from 37 (70%) pregnancies. There were significantly more abortions in the heparin group (6; 60%) than the warfarin group (8; 19%). Hemorrhage requiring transfusion occurred in 2 (5%) patients in the warfarin group. All live births resulted in healthy babies. It was concluded that anticoagulation with warfarin is safe during pregnancy in women with mechanical heart valves.