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

AbstractObjectives. 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

702-1 Long-term (28-Year) Follow-up of Mustard Repair for Transposition of the Great Arteries with Intact Ventricular Septum: Freedom from Right Ventricular (Systemic) Heart Failure

113 30-day survivors of the Mustard repair undertaken between 1964 and 1982 were followed by serial cardiological review and a recent symptom questionnaire. Follow-up of late survivors was 10–28 years; 40 survive >20 years postoperatively. Right ventricular failure (RVF) was diagnosed if there were clinical signs and/or supportive radiological, echocardiographic or radionuclide evidence of progressive right heart dysfunction. Non-progressive RV dilatation was labelled RV dysfunction.ResultsActuarial survival was 93%, 90%, 84%, 80% and 80% at 5, 10, 15, 20 and 24 years respectively. Causes of the 19 deaths were: baffle obstruction 4; sudden, presumed arrhythmia 6; pulmonary vascular disease 2; miscellaneous 5; and RVF 2.Although RV dysfunction is frequently observed, actuarial freedom from RVF is 97% at 18 years.ConclusionProgressive right ventricular failure is rare following the Mustard operation for simple transposition with intact septum