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Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease
Congenital Heart Disease
Pulmonary Atresia With Intact Ventricular Septum: Range of Morphology in a Population-Based Study
Abstracts
Daubeney PEF, Delany DJ, Anderson RH, et al., for the United Kingdom and Ireland Collaborative Study of Pulmonary Atresia With Intact Ventricular Septum. J Am Coll Cardiol 2002;39:1670 –9.
Adverse Neonatal and Cardiac Outcomes Are More Common in Pregnant Women With Cardiac Disease
Study Question: To describe the entire range of cardiac morphology of pulmonary atresia with intact ventricular septum (PA/IVS) from a prospective population-based study. Methods: A single investigator visited and reviewed the records, including imaging and autopsy reports of every child born with PA/IVS between 1991 and 1995 in the United Kingdom and Ireland. Results: The overall incidence of PA/IVS was 4.5 cases/ 100,000 live births. In addition, there were 86 fetal diagnoses with 53 pregnancy terminations. 38.2% (70/183) of infants have died postnatally. Atresia was membranous in 74.7% and muscular in 25.3% of cases. Median tricuspid valve size Z-score (the number of standard deviations from the mean of a normal population) was ⫺5.2 by echocardiography (interquartile range ⫺8.4 to ⫺2.6) and the median right ventricular inlet size Z-score was ⫺5.1 (interquartile range ⫺7.5 to ⫺2.8). Right ventricular morphology was unipartite (muscular obliteration of apical and infundibular portions of the right ventricle [RV]) in 7.7%, bipartite (muscular obliteration of the apex) in 33.6%, and tripartite (normal) in 58.7%. Only 71 patients (54.2%) had normal coronary arteries. Minor coronary fistulae were present in 15.3% and major fistulae in 18.0% patients and tended to occur in smaller ventricles as judged by Z-score, p⫽0.0011). 10 pts had an “RV-dependent” coronary circulation (fistulae with absent aortocoronary connection, coronary interruption, severe coronary stenoses, or marked coronary ectasia), 8 with membranous and 2 with muscular atresia. 45 patients (40.2%) had an acute angle take-off of the ductus arteriosus from the aorta, a reported indicator of pulmonary atresia occurring early in fetal development. This finding was associated with smaller ventricles, muscular atresia and coronary fistulae in contrast to a right or obtuse angle take-off of the ductus which was associated with membranous atresia, suggesting that pulmonary atresia was the result of pulmonary stenosis progressing to obstruction in the last trimester of pregnancy. Conclusion: The range of cardiac morphologies is described in infants with PA/IVS. Perspective: This meticulous study provides physicians with a standard by which they can compare study populations for this cardiac lesion. JK
Siu SC, Colman JM, Sorenson S, et al. Circulation 2002;105: 2179 – 84. Study Question: What are the neonatal, obstetric, and cardiac outcomes of pregnancy in women with cardiac disease as compared to prospective, contemporary controls? Methods: Prospective enrollment of 301 pregnant women (334 pregnancies) with heart disease (HD), (congenital 64%, acquired 28%, and arrhythmias 8%) and comparison to 572 women (578 pregnancies) contemporaneously enrolled without HD. Cardiac factors considered high risk were use of anticoagulants, maternal cyanosis, NYHA functional Class ⱖ III, or left heart obstructions. Maternal risk factors included active smoking, hypertension, and diabetes. Obstetric high risk indicators included history of premature delivery or rupture of membranes, incompetent cervix or intrauterine growth retardation, multiple gestation or bleeding after the first trimester. Results: In women between 20 and 35 yrs old who did not smoke, did not use anticoagulants during pregnancy, and had no obstetric risk factors, the incidence of neonatal complications (premature birth ⬍27 weeks gestational age, small-for-gestational age birth weight, respiratory distress syndrome, fetal death or neonatal death) was not different in women with HD and no cardiac risk factors vs. women with HD and ⱖ1 cardiac risk factors vs. controls (4% vs. 7% vs. 5%, p⫽N.S.). The neonatal event rate in women ⬍20 or ⬎35 years who smoked, used anticoagulants or had obstetric risk factors was higher in all groups, but was significantly higher in women with no cardiac risk factors (27%) and women with ⱖ1 cardiac risk factor (33%) than in controls (11%, p⬍0.001). The caesarean section rate was higher in the HD group than in controls (29% vs. 23%, p⫽0.032), primarily due to caesarean section performed for cardiac indications. Cardiac complications (heart failure, strokes, arrhythmias) occurred in 52 completed pregnancies (17%) with two postpartum deaths. Conclusion: The risk of neonatal complications in women with cardiac disease is no different than in controls when no high risk indicators of maternal or obstetric risk are present. However, the risk of neonatal complications is significantly increased in women who smoke, use anticoagulants during pregnancy, and have maternal or obstetric high-risk indicators. Perspective: This is the first study to highlight risk factors predictive of poor neonatal outcome for fetuses of women with cardiac disease. Minimizing maternal and obstetric risk factors are paramount in these patients to prevent neonatal complications. JK
Value of Clinical and Echocardiographic Features in Predicting Outcome in the Fetus, Infant, and Child with Tetralogy of Fallot With Absent Pulmonary Valve Complex Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH. Am J Cardiol 2002;89:1280 –5.
ACC CURRENT JOURNAL REVIEW Nov/Dec 2002
90
Pulmonary Atresia With Intact Ventricular Septum: Range of Morphology in a Population-Based Study
Abstracts
Daubeney PEF, Delany DJ, Anderson RH, et al., for the United Kingdom and Ireland Collaborative Study of Pulmonary Atresia With Intact Ventricular Septum. J Am Coll Cardiol 2002;39:1670 –9.
Adverse Neonatal and Cardiac Outcomes Are More Common in Pregnant Women With Cardiac Disease
Study Question: To describe the entire range of cardiac morphology of pulmonary atresia with intact ventricular septum (PA/IVS) from a prospective population-based study. Methods: A single investigator visited and reviewed the records, including imaging and autopsy reports of every child born with PA/IVS between 1991 and 1995 in the United Kingdom and Ireland. Results: The overall incidence of PA/IVS was 4.5 cases/ 100,000 live births. In addition, there were 86 fetal diagnoses with 53 pregnancy terminations. 38.2% (70/183) of infants have died postnatally. Atresia was membranous in 74.7% and muscular in 25.3% of cases. Median tricuspid valve size Z-score (the number of standard deviations from the mean of a normal population) was ⫺5.2 by echocardiography (interquartile range ⫺8.4 to ⫺2.6) and the median right ventricular inlet size Z-score was ⫺5.1 (interquartile range ⫺7.5 to ⫺2.8). Right ventricular morphology was unipartite (muscular obliteration of apical and infundibular portions of the right ventricle [RV]) in 7.7%, bipartite (muscular obliteration of the apex) in 33.6%, and tripartite (normal) in 58.7%. Only 71 patients (54.2%) had normal coronary arteries. Minor coronary fistulae were present in 15.3% and major fistulae in 18.0% patients and tended to occur in smaller ventricles as judged by Z-score, p⫽0.0011). 10 pts had an “RV-dependent” coronary circulation (fistulae with absent aortocoronary connection, coronary interruption, severe coronary stenoses, or marked coronary ectasia), 8 with membranous and 2 with muscular atresia. 45 patients (40.2%) had an acute angle take-off of the ductus arteriosus from the aorta, a reported indicator of pulmonary atresia occurring early in fetal development. This finding was associated with smaller ventricles, muscular atresia and coronary fistulae in contrast to a right or obtuse angle take-off of the ductus which was associated with membranous atresia, suggesting that pulmonary atresia was the result of pulmonary stenosis progressing to obstruction in the last trimester of pregnancy. Conclusion: The range of cardiac morphologies is described in infants with PA/IVS. Perspective: This meticulous study provides physicians with a standard by which they can compare study populations for this cardiac lesion. JK
Siu SC, Colman JM, Sorenson S, et al. Circulation 2002;105: 2179 – 84. Study Question: What are the neonatal, obstetric, and cardiac outcomes of pregnancy in women with cardiac disease as compared to prospective, contemporary controls? Methods: Prospective enrollment of 301 pregnant women (334 pregnancies) with heart disease (HD), (congenital 64%, acquired 28%, and arrhythmias 8%) and comparison to 572 women (578 pregnancies) contemporaneously enrolled without HD. Cardiac factors considered high risk were use of anticoagulants, maternal cyanosis, NYHA functional Class ⱖ III, or left heart obstructions. Maternal risk factors included active smoking, hypertension, and diabetes. Obstetric high risk indicators included history of premature delivery or rupture of membranes, incompetent cervix or intrauterine growth retardation, multiple gestation or bleeding after the first trimester. Results: In women between 20 and 35 yrs old who did not smoke, did not use anticoagulants during pregnancy, and had no obstetric risk factors, the incidence of neonatal complications (premature birth ⬍27 weeks gestational age, small-for-gestational age birth weight, respiratory distress syndrome, fetal death or neonatal death) was not different in women with HD and no cardiac risk factors vs. women with HD and ⱖ1 cardiac risk factors vs. controls (4% vs. 7% vs. 5%, p⫽N.S.). The neonatal event rate in women ⬍20 or ⬎35 years who smoked, used anticoagulants or had obstetric risk factors was higher in all groups, but was significantly higher in women with no cardiac risk factors (27%) and women with ⱖ1 cardiac risk factor (33%) than in controls (11%, p⬍0.001). The caesarean section rate was higher in the HD group than in controls (29% vs. 23%, p⫽0.032), primarily due to caesarean section performed for cardiac indications. Cardiac complications (heart failure, strokes, arrhythmias) occurred in 52 completed pregnancies (17%) with two postpartum deaths. Conclusion: The risk of neonatal complications in women with cardiac disease is no different than in controls when no high risk indicators of maternal or obstetric risk are present. However, the risk of neonatal complications is significantly increased in women who smoke, use anticoagulants during pregnancy, and have maternal or obstetric high-risk indicators. Perspective: This is the first study to highlight risk factors predictive of poor neonatal outcome for fetuses of women with cardiac disease. Minimizing maternal and obstetric risk factors are paramount in these patients to prevent neonatal complications. JK
Value of Clinical and Echocardiographic Features in Predicting Outcome in the Fetus, Infant, and Child with Tetralogy of Fallot With Absent Pulmonary Valve Complex Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH. Am J Cardiol 2002;89:1280 –5.
ACC CURRENT JOURNAL REVIEW Nov/Dec 2002
90