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Pregnancy in women with coarctation of the thoracic aorta
MEDICAL/SURGICAL
CONSIDERATIONS
larged or unstable aorta, and in women with a severe obstructive lesion, such as coarctation.
PERSPECTIVE
Pregnancy in Women With Coarctation of the Thoracic Aorta
Incidence of Congenital Heart Disease in Offspring The incidence of congenital heart disease in the general population is approximately 1%. In general, the offspring of women with congenital heart disease have a 5 to 6% incidence of congenital heart disease. Usually the congenital anomaly affecting the offspring is not the same lesion as that affecting the mother. Fetal echocardiography is routinely used in women with a congenital cardiac anomaly, to anticipate the presence of congenital heart disease in the offspring.
Heidi M.Connolly, MD, Mayo Clinic and Mayo Foundation, Rochester,Minnesota Coarctation of the thoracic aorta accounts for approximately 2% of congenital heart disease. It is characterized by a discrete narrowing usually located distal to the left subclavian artery, and encompasses a spectrum of disease. Approximately 80% of cases are diagnosed in infancy or childhood (1,2). Surgery to correct coarctation of the aorta has been successfully carried out since 1944, and largely due to this, survival into adulthood is common. Most women with coarctation of the thoracic aorta reach child-bearing age. Previous data suggested a substantial maternal and fetal risk of pregnancy in women with coarctation, particularly when unoperated (3-7). The estimated maternal mortality during pregnancy was reported to be 3 to 8%. In addition, even in the absence of aortic disease, there is an increased risk of aortic dissection during pregnancy, attributable primarily to the hemodynamic and hormonal changes that normally occur. In women <40 years of age, 50% of aortic dissections occur during pregnancy, most commonly during the third trimester as well as during the peripartum and postpartum periods. Coarctation and hypertension further increase the risk of aortic dissection, and the risk is unpredictable. Significant hemodynamic and physiologic changes occur during pregnancy. Some of these changes may adversely affect the hemodynamic status of the patient with congenital heart disease, particularly obstructive lesions such as significant coarctation. The hemod}mamic changes of pregnancy include a substantial increase in blood volume, cardiac output, stroke volume, heart rate and circulating catecholamine concentrations and a decrease in the peripheral vascular resistance (8). Although these changes are usually well tolerated, cardiovascular disease may be first recognized during pregnancy, or previously minimally symptomatic patients may develop severe symptoms.
Presentation of Coarctation Coarctation of the aorta may present initially during pregnancy. The usual presenting sign of coarctation in adults is systemic hypertension. In young patients (beyond the age of 1 year) the initial finding usually is a murmur. The characteristic physical finding associated with coarctation (radial artery' to femoral arte U pulse delay) is not always present. In addition, other characteristic clinical findings such as reduced lower extremity blood pressure, and rib notching on the chest radiograph may also be absent. Current commonly used diagnostic modalities include invasive angiography, two-dimensional and Doppler echocardiography, and magnetic resonance imaging. Risk In the assessment of the patient with coarctation of the aorta, one must also consider associated anomalies and conditions such as bicuspid aortic valve, mitral valve abnormalities, cerebral aneurysms and systemic hypertension. More than 70% of patients with a history of coarctation, even after surgical intervention will have hypertension. In addition, this patient population is at increased risk for premature coronary artery disease, congestive heart failure, intracerebral hemorrhage and aortic dissection and rupture (9). Aortic dissection and rupture are unpredictable complications of coarctation and can occur after repair. The risk of aortic dissection is further increased during pregnancy as pre~i ously outlined. Thus, patients with coarctation not only require regular cardiovascular follow-up but clearly are at increased risk during pregnancy. With appropriate counseling and patient selection, the complication rate of pregnancy is acceptable and the outcome of pregnancy appears more optimistic than previously described. In the past, many women with coarctation have been discouraged from having children, however, this should no longer be universal practice. We recently reviewed the outcome of 87 pregnancies in 39 women with coarctation, primarily but not exclusively after surgical intervention. We found that 94% of all preg-
Delivery Route Vaginal deliveU is usually the preferred route of delivery from the hemodynamic and cardiac standpoint in women with cardiovascular disease. Cesarean section, however, remains the suggested mode of delivery for obstetrical reasons, and when spontaneous labor occurs in the presence of full Coumadin anticoagulation. Cesarean section should also be considered in women with significant pulmonary' hypertension (who refuse termination of pregnancy), those with Marfan's syndrome or other conditions resulting in an en-
ACC CURRENT JOURNAL REVIEx& May/June 1997 © 1997 by the American College of CardiologD" Published b} Elsevier Science tnc
55
1062-1458/97t517.00 PlI S1062-1458(97)00022-6
MEDICAL/SURGICAL
CONSIDERATIONS
nancies resulted in live birth, and that 33 % of deliveries were by cesarean section. There were no maternal pregnancyrelated deaths or life-threatening complications; however, 49% of women either reported significant hypertension during pregnancy or were on antihypertensive therapy before pregnancy. Hypertension was particularly common among unoperated patients or those with recoarctation after repair. The miscarriage and premature birth rates were not higher than rates noted for the general population. Similarly the mean birth weights were not significantly different than birth weights of infants born to women without cardiovascular disease, and there was no significant difference between the birth weights of offspring born to unoperated versus repaired patients. The incidence of congenital heart disease in the offspring was 4%. Overall our retrospective study suggests optimism regarding the outcome of pregnancy in women with coarctation of the thoracic aorta (10).
vention if significant coarctation exists in a woman who wishes to become pregnant. Balloon dilatation of the aorta is an effective form of therapy in a subset of patients with coarctation; however, it is contraindicated in women considering pregnancy as the mechanism of aortic dilatation is intimal disruption and is often associated with localized dissection. Recommendations It is recommended that once pregnant, patients with coarctation of the aorta be monitored closely by a multidisciplinary team that includes obstetricians, cardiologists and obstetric anesthesiologists. In select cases cardiac surgeons are also involved in the management of these patients. With the exception of the repaired patient with coarctation who has minimal or no residual obstruction and no other sequelae, all patients with a history of coarctation of the aorta should be followed and delivered at tertiary centers where congenital cardiac surgical expertise is available. Care must be taken not only to avoid rapid volume shifts during parturition but also to facilitate the second stage of labor. In select cases delivery by cesarean section is suggested. Endocarditis prophylaxis should be given to the high-risk patient at the time of delivery. In addition those patients with systemic hypertension must be closely monitored with meticulous blood pressure control throughout pregnancy and delivery, as well as in the postpartum period. Beta-blocker therapy is now the preferred antihypertensive medication during pregnancy in a patient with hypertension related to coarctation.
Evaluation of the Patient With Coarctation The patient with coarctation of the aorta who wishes to pursue pregnancy requires a comprehensive cardiovascular evaluation including a thorough anatomic, hemodynamic and functional assessment as well as evaluation of rest and exercise blood pressure. The evaluation should be carried out before pregnancy, whenever possible. The best diagnostic procedure in patients with coarctation is currently disputed. The detection of an anatomic narrowing in the aorta establishes the diagnosis of coarctation. The assessment of the clinical significance of a documented coarctation, however, depends on determining its hemodynamic effect. Twodimensional and Doppler echocardiography allows excellent evaluation of the cardiac structure, function and the intracardiac hemodynamics. The evaluation of the descending thoracic aorta, however, is somewhat difficult by surface echocardiography, particularly in the adult the region of coarctation is often difficult to visualize. The Dopplerderived gradient across the region of coarctation may be inaccurate, particularly in patients who have developed collateral vessels. Exercise Doppler evaluation improves diagnostic accuracy. Invasive angiography is well established as an excellent diagnostic modality; however, the measured gradient may also be falsely low when collateral vessels are present. Angiography has the additional disadvantages of requiring an invasive procedure and using radiation. There is recent optimism regarding the use of magnetic resonance angiography in patients with coarctation, and this may become the procedure of choice in the evaluation of the patient with suspected or confirmed coarctation. Importantly magnetic resonance imaging can also be performed during pregnancy. It is imperative to confirm the diagnosis of coarctation, and to assess the severity of obstruction. It may be necessary in select cases to suggest surgical inter-
Other Considerations A large number of women with congenital heart disease are now reaching child-bearing age and considering pregnancy. This is due primarily to early diagnosis of congenital heart disease, advances in surgical and medical management and an increase in the genetic pool. In the complex patient with congenital heart disease, both before and after surgical intervention, uncertainty remains regarding the ability to conceive, the effects of pregnancy on the maternal cardiac disease and the effects of the maternal cardiac disease on fetus. An intricate part of the management of such patients should include counseling regarding potential pregnancy, the genetic implications of congenital heart disease on the fetus and the systematic assessment of the anatomy and physiology of the cardiac anomaly, preferably before pregnancy. Summary In summary, significant coarctation of the aorta during pregnancy in the unoperated setting carries an increased risk of morbidity and mortality both to mother and fetus and therefore, is contraindicated until adequate surgical intervention has been carned out. Previously operated patients
ACC CURRENTJOURNAL REVIEW May/June 1997
56
MEDICAL/SURGICAL
need to be carefully screened from the cardiovascular standpoint before pregnancy. The risk of pregnancy is substantially reduced after successful coarctation repair, however, it is not negligible.
CONSIDERATIONS
10), and mitral valve replacement (n = 2) were performed selectively. Results: There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class I). Late postoperative deaths occurred m five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect m one and severe mitral regurgitation in the other. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity. Conclusions: An ostium primum defect can be repaired in adult patients with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function and despite the presence of atrial fibrillation or moderately elevated pulmonary vascular resistance.
REFERENCES l. Warnes CA, Fuster V, McGoon DC. Coarctation of the aorta. Mayo Clinic Textbook (Part V111)45:1572-80. 2. Stratford MA, Gri~ths SP, Gersony WM. Coarctation of the aorta: a study in delayed detection. Pediatrics 1982;69:159- 63 3. Rosenthal L. Coarctation of the aorta and pregnancy. Bnt Med J 1955; 1:16-7. 4. Goodwin JF. Pregnancy and coaretation of the aorta. Clin Obstet Gynecol 1961;4:645-52. 5. Wachtel HL, et al. Coarctation of the aorta and pregnancy. Am Heart J 1966;72(2):251-4. 6. Hillestad L. Aortic coarctation and pregnancy. Acta Obstet Gy~ec Scand 1972;51:95-8. 7. Benny PS, et al. Pregnancy and coarctation of the aorta. BrJ Obstet Gyn 1980;87:1159-61. 8. Gianopoulos JG. Cardiac disease in pregnancy. Med Clin N Am 1989; 73(3):639-51.
Should Atrial Septal Defects in Adults Be Closed? M~ Gatzoulis,A.N. Redington,J. Somerville,D.F. Shore.RoyalBromptonHospital, NationalHeartand LungInstitute,London,England.Ann ThoracSurg1996;61: 657-9. Background: By assessing current surgical outcome and
9. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of aorta. Long-term follow up and prediction of outcome after surgical correction. Circulation 1989;80(4):840-5. 10. Connolly HM, Ammash NM, Wames CA. Pregnancy in women with coarctation of the aorta. JACC 1996;27:43A. Address correspondence and repnnt requests to Heidi M. Connolly, MD, Mayo Clinic, 200 First Street, S.W., Rochester, MN 55905.
symptomatic relief, this study attempts to answer whether atrial septal defects in adults should be closed. Methods: Thirty-nine adult patients aged 35.3 + 13.6 years underwent operation for an atrial septal defect between June 1988 and June 1994. Indications for closure were symptoms (33 patients) or a significant left-to-right atrial shunt (6 patients). Data were obtained from hospital records, and the latest status of the patients was determined by a written questionnaire. Results: There were no deaths. Pulmonary embolism in 1 patient was the only complication observed. The QRS duration on the surface electrocardiogram decreased immediately (p < 0.001), and the cardiothoracic ratio on chest radiographs was significantly lower 3 to 6 months after operation (p < 0.001), both findings reflecting improved hemodynamics. No residual shunts were seen on follow-up (mean follow-up, 3.3 + 2.2 years). Twenty-seven (81.8%) of the 33 symptomatic patients improved clinically in terms of exercise performance, atrial arrhythmias, or both. Three (50%) of the 6 previously asymptomatic patients reported improved functional capacity postoperatively. Conclusions: Today, operation for atrial septal defects in adults can be performed with no mortality and low morbidity and results in symptomatic improvement in the majority of patients. Clinical improvement was seen even in patients who considered themselves asymptomatic preoperatively. We advocate closure of atrial septal defects in adult patients with symptoms or significant atrial shunts.
Considerations Relative to Atrial Septal Defect Closure Long-term FolLow-up After Surgical Repair of Ostium Primum Atrial Septal Defect in Adults I~P. Burke,IC Horvath,M. Land~berg,P. Hyde,J.J.CollinsJr., LH. Cohn.Divisionof CardiacSurgery,Brighamand Women'sHospital;Boston,MA.J Am CoilCardiol 1996;27:696-9. Objectives: We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect. Background: The natural history- of patients undergoing such surgical repair in adulthood remains unclear. Methods: We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were > 50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association function class > II) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure > 25 mm Hg or pulmonary, vascular resistance > 4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral val~qaloplasty, consisting of cleft repair (n =
ACC CURRENTJOURNALREVIEW May/June 1.997
57
CONSIDERATIONS
larged or unstable aorta, and in women with a severe obstructive lesion, such as coarctation.
PERSPECTIVE
Pregnancy in Women With Coarctation of the Thoracic Aorta
Incidence of Congenital Heart Disease in Offspring The incidence of congenital heart disease in the general population is approximately 1%. In general, the offspring of women with congenital heart disease have a 5 to 6% incidence of congenital heart disease. Usually the congenital anomaly affecting the offspring is not the same lesion as that affecting the mother. Fetal echocardiography is routinely used in women with a congenital cardiac anomaly, to anticipate the presence of congenital heart disease in the offspring.
Heidi M.Connolly, MD, Mayo Clinic and Mayo Foundation, Rochester,Minnesota Coarctation of the thoracic aorta accounts for approximately 2% of congenital heart disease. It is characterized by a discrete narrowing usually located distal to the left subclavian artery, and encompasses a spectrum of disease. Approximately 80% of cases are diagnosed in infancy or childhood (1,2). Surgery to correct coarctation of the aorta has been successfully carried out since 1944, and largely due to this, survival into adulthood is common. Most women with coarctation of the thoracic aorta reach child-bearing age. Previous data suggested a substantial maternal and fetal risk of pregnancy in women with coarctation, particularly when unoperated (3-7). The estimated maternal mortality during pregnancy was reported to be 3 to 8%. In addition, even in the absence of aortic disease, there is an increased risk of aortic dissection during pregnancy, attributable primarily to the hemodynamic and hormonal changes that normally occur. In women <40 years of age, 50% of aortic dissections occur during pregnancy, most commonly during the third trimester as well as during the peripartum and postpartum periods. Coarctation and hypertension further increase the risk of aortic dissection, and the risk is unpredictable. Significant hemodynamic and physiologic changes occur during pregnancy. Some of these changes may adversely affect the hemodynamic status of the patient with congenital heart disease, particularly obstructive lesions such as significant coarctation. The hemod}mamic changes of pregnancy include a substantial increase in blood volume, cardiac output, stroke volume, heart rate and circulating catecholamine concentrations and a decrease in the peripheral vascular resistance (8). Although these changes are usually well tolerated, cardiovascular disease may be first recognized during pregnancy, or previously minimally symptomatic patients may develop severe symptoms.
Presentation of Coarctation Coarctation of the aorta may present initially during pregnancy. The usual presenting sign of coarctation in adults is systemic hypertension. In young patients (beyond the age of 1 year) the initial finding usually is a murmur. The characteristic physical finding associated with coarctation (radial artery' to femoral arte U pulse delay) is not always present. In addition, other characteristic clinical findings such as reduced lower extremity blood pressure, and rib notching on the chest radiograph may also be absent. Current commonly used diagnostic modalities include invasive angiography, two-dimensional and Doppler echocardiography, and magnetic resonance imaging. Risk In the assessment of the patient with coarctation of the aorta, one must also consider associated anomalies and conditions such as bicuspid aortic valve, mitral valve abnormalities, cerebral aneurysms and systemic hypertension. More than 70% of patients with a history of coarctation, even after surgical intervention will have hypertension. In addition, this patient population is at increased risk for premature coronary artery disease, congestive heart failure, intracerebral hemorrhage and aortic dissection and rupture (9). Aortic dissection and rupture are unpredictable complications of coarctation and can occur after repair. The risk of aortic dissection is further increased during pregnancy as pre~i ously outlined. Thus, patients with coarctation not only require regular cardiovascular follow-up but clearly are at increased risk during pregnancy. With appropriate counseling and patient selection, the complication rate of pregnancy is acceptable and the outcome of pregnancy appears more optimistic than previously described. In the past, many women with coarctation have been discouraged from having children, however, this should no longer be universal practice. We recently reviewed the outcome of 87 pregnancies in 39 women with coarctation, primarily but not exclusively after surgical intervention. We found that 94% of all preg-
Delivery Route Vaginal deliveU is usually the preferred route of delivery from the hemodynamic and cardiac standpoint in women with cardiovascular disease. Cesarean section, however, remains the suggested mode of delivery for obstetrical reasons, and when spontaneous labor occurs in the presence of full Coumadin anticoagulation. Cesarean section should also be considered in women with significant pulmonary' hypertension (who refuse termination of pregnancy), those with Marfan's syndrome or other conditions resulting in an en-
ACC CURRENT JOURNAL REVIEx& May/June 1997 © 1997 by the American College of CardiologD" Published b} Elsevier Science tnc
55
1062-1458/97t517.00 PlI S1062-1458(97)00022-6
MEDICAL/SURGICAL
CONSIDERATIONS
nancies resulted in live birth, and that 33 % of deliveries were by cesarean section. There were no maternal pregnancyrelated deaths or life-threatening complications; however, 49% of women either reported significant hypertension during pregnancy or were on antihypertensive therapy before pregnancy. Hypertension was particularly common among unoperated patients or those with recoarctation after repair. The miscarriage and premature birth rates were not higher than rates noted for the general population. Similarly the mean birth weights were not significantly different than birth weights of infants born to women without cardiovascular disease, and there was no significant difference between the birth weights of offspring born to unoperated versus repaired patients. The incidence of congenital heart disease in the offspring was 4%. Overall our retrospective study suggests optimism regarding the outcome of pregnancy in women with coarctation of the thoracic aorta (10).
vention if significant coarctation exists in a woman who wishes to become pregnant. Balloon dilatation of the aorta is an effective form of therapy in a subset of patients with coarctation; however, it is contraindicated in women considering pregnancy as the mechanism of aortic dilatation is intimal disruption and is often associated with localized dissection. Recommendations It is recommended that once pregnant, patients with coarctation of the aorta be monitored closely by a multidisciplinary team that includes obstetricians, cardiologists and obstetric anesthesiologists. In select cases cardiac surgeons are also involved in the management of these patients. With the exception of the repaired patient with coarctation who has minimal or no residual obstruction and no other sequelae, all patients with a history of coarctation of the aorta should be followed and delivered at tertiary centers where congenital cardiac surgical expertise is available. Care must be taken not only to avoid rapid volume shifts during parturition but also to facilitate the second stage of labor. In select cases delivery by cesarean section is suggested. Endocarditis prophylaxis should be given to the high-risk patient at the time of delivery. In addition those patients with systemic hypertension must be closely monitored with meticulous blood pressure control throughout pregnancy and delivery, as well as in the postpartum period. Beta-blocker therapy is now the preferred antihypertensive medication during pregnancy in a patient with hypertension related to coarctation.
Evaluation of the Patient With Coarctation The patient with coarctation of the aorta who wishes to pursue pregnancy requires a comprehensive cardiovascular evaluation including a thorough anatomic, hemodynamic and functional assessment as well as evaluation of rest and exercise blood pressure. The evaluation should be carried out before pregnancy, whenever possible. The best diagnostic procedure in patients with coarctation is currently disputed. The detection of an anatomic narrowing in the aorta establishes the diagnosis of coarctation. The assessment of the clinical significance of a documented coarctation, however, depends on determining its hemodynamic effect. Twodimensional and Doppler echocardiography allows excellent evaluation of the cardiac structure, function and the intracardiac hemodynamics. The evaluation of the descending thoracic aorta, however, is somewhat difficult by surface echocardiography, particularly in the adult the region of coarctation is often difficult to visualize. The Dopplerderived gradient across the region of coarctation may be inaccurate, particularly in patients who have developed collateral vessels. Exercise Doppler evaluation improves diagnostic accuracy. Invasive angiography is well established as an excellent diagnostic modality; however, the measured gradient may also be falsely low when collateral vessels are present. Angiography has the additional disadvantages of requiring an invasive procedure and using radiation. There is recent optimism regarding the use of magnetic resonance angiography in patients with coarctation, and this may become the procedure of choice in the evaluation of the patient with suspected or confirmed coarctation. Importantly magnetic resonance imaging can also be performed during pregnancy. It is imperative to confirm the diagnosis of coarctation, and to assess the severity of obstruction. It may be necessary in select cases to suggest surgical inter-
Other Considerations A large number of women with congenital heart disease are now reaching child-bearing age and considering pregnancy. This is due primarily to early diagnosis of congenital heart disease, advances in surgical and medical management and an increase in the genetic pool. In the complex patient with congenital heart disease, both before and after surgical intervention, uncertainty remains regarding the ability to conceive, the effects of pregnancy on the maternal cardiac disease and the effects of the maternal cardiac disease on fetus. An intricate part of the management of such patients should include counseling regarding potential pregnancy, the genetic implications of congenital heart disease on the fetus and the systematic assessment of the anatomy and physiology of the cardiac anomaly, preferably before pregnancy. Summary In summary, significant coarctation of the aorta during pregnancy in the unoperated setting carries an increased risk of morbidity and mortality both to mother and fetus and therefore, is contraindicated until adequate surgical intervention has been carned out. Previously operated patients
ACC CURRENTJOURNAL REVIEW May/June 1997
56
MEDICAL/SURGICAL
need to be carefully screened from the cardiovascular standpoint before pregnancy. The risk of pregnancy is substantially reduced after successful coarctation repair, however, it is not negligible.
CONSIDERATIONS
10), and mitral valve replacement (n = 2) were performed selectively. Results: There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class I). Late postoperative deaths occurred m five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect m one and severe mitral regurgitation in the other. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity. Conclusions: An ostium primum defect can be repaired in adult patients with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function and despite the presence of atrial fibrillation or moderately elevated pulmonary vascular resistance.
REFERENCES l. Warnes CA, Fuster V, McGoon DC. Coarctation of the aorta. Mayo Clinic Textbook (Part V111)45:1572-80. 2. Stratford MA, Gri~ths SP, Gersony WM. Coarctation of the aorta: a study in delayed detection. Pediatrics 1982;69:159- 63 3. Rosenthal L. Coarctation of the aorta and pregnancy. Bnt Med J 1955; 1:16-7. 4. Goodwin JF. Pregnancy and coaretation of the aorta. Clin Obstet Gynecol 1961;4:645-52. 5. Wachtel HL, et al. Coarctation of the aorta and pregnancy. Am Heart J 1966;72(2):251-4. 6. Hillestad L. Aortic coarctation and pregnancy. Acta Obstet Gy~ec Scand 1972;51:95-8. 7. Benny PS, et al. Pregnancy and coarctation of the aorta. BrJ Obstet Gyn 1980;87:1159-61. 8. Gianopoulos JG. Cardiac disease in pregnancy. Med Clin N Am 1989; 73(3):639-51.
Should Atrial Septal Defects in Adults Be Closed? M~ Gatzoulis,A.N. Redington,J. Somerville,D.F. Shore.RoyalBromptonHospital, NationalHeartand LungInstitute,London,England.Ann ThoracSurg1996;61: 657-9. Background: By assessing current surgical outcome and
9. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of aorta. Long-term follow up and prediction of outcome after surgical correction. Circulation 1989;80(4):840-5. 10. Connolly HM, Ammash NM, Wames CA. Pregnancy in women with coarctation of the aorta. JACC 1996;27:43A. Address correspondence and repnnt requests to Heidi M. Connolly, MD, Mayo Clinic, 200 First Street, S.W., Rochester, MN 55905.
symptomatic relief, this study attempts to answer whether atrial septal defects in adults should be closed. Methods: Thirty-nine adult patients aged 35.3 + 13.6 years underwent operation for an atrial septal defect between June 1988 and June 1994. Indications for closure were symptoms (33 patients) or a significant left-to-right atrial shunt (6 patients). Data were obtained from hospital records, and the latest status of the patients was determined by a written questionnaire. Results: There were no deaths. Pulmonary embolism in 1 patient was the only complication observed. The QRS duration on the surface electrocardiogram decreased immediately (p < 0.001), and the cardiothoracic ratio on chest radiographs was significantly lower 3 to 6 months after operation (p < 0.001), both findings reflecting improved hemodynamics. No residual shunts were seen on follow-up (mean follow-up, 3.3 + 2.2 years). Twenty-seven (81.8%) of the 33 symptomatic patients improved clinically in terms of exercise performance, atrial arrhythmias, or both. Three (50%) of the 6 previously asymptomatic patients reported improved functional capacity postoperatively. Conclusions: Today, operation for atrial septal defects in adults can be performed with no mortality and low morbidity and results in symptomatic improvement in the majority of patients. Clinical improvement was seen even in patients who considered themselves asymptomatic preoperatively. We advocate closure of atrial septal defects in adult patients with symptoms or significant atrial shunts.
Considerations Relative to Atrial Septal Defect Closure Long-term FolLow-up After Surgical Repair of Ostium Primum Atrial Septal Defect in Adults I~P. Burke,IC Horvath,M. Land~berg,P. Hyde,J.J.CollinsJr., LH. Cohn.Divisionof CardiacSurgery,Brighamand Women'sHospital;Boston,MA.J Am CoilCardiol 1996;27:696-9. Objectives: We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect. Background: The natural history- of patients undergoing such surgical repair in adulthood remains unclear. Methods: We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were > 50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association function class > II) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure > 25 mm Hg or pulmonary, vascular resistance > 4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral val~qaloplasty, consisting of cleft repair (n =
ACC CURRENTJOURNALREVIEW May/June 1.997
57