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Cardiac hydatid cyst with clinical features resembling subaortic stenosis
Volume 117 Number 6
of Doppler values and the continuity equation.’ In our patient, the estimates of aortic valve area were similar whether derived from the continuity equation or from the simplified Gorlin equation5 using the invasively obtained cardiac output and Doppler-derived valve velocity, and these estimates were consistent with significant, but not critical aortic stenosis. Unfortunately, we were unable to compare these values with those classically obtained by cardiac catheterization. To our knowledge, there has been only one previous report* in which continuous-wave Doppler was used to monitor the course of a pregnancy complicated by aortic stenosis. At various intervals, peak instantaneous valve gradients were obtained in a young woman with a deteriorating aortic valve prosthesis. Aortic valve area was unreported. The authors rightfully cautioned misinterpretation of Doppler-derived estimates of valve gradients in view of known pregnancy-related hemodynamic changes. However, estimates of aortic valve area, which take into account the effect of flow, should be valid. We await the establishment of normal Doppler values for pregnancy and further investigation in associated pathologic states is needed. In spite of these limitations, Doppler-derived data obtained in the overall noninvasive evaluation of this patient played a vital role in outlining the appropriate peripartum management. The preferred method of delivery in these patients remains controversial. The major objective is to avoid volume depletion and hypotension, which can lead to catastrophic hemodynamic collapse. This can be precipitated by peripartum blood loss, supine vena caval obstruction, anesthetic-related vasodilatation, and even myocardial infarction..1~6~7In view of this patient’s persistent excellent LV systolic function, Doppler estimates of noncritical aortic stenosis, and normal right heart and PCWPs, we elected to allow her to undergo labor and to deliver entirely in the left lateral decubitus position with continuous fetal and right heart pressure monitoring. In conclusion, we describe the safe and uneventful labor and delivery of an otherwise healthy young woman with congenital aortic valve stenosis. The degree of stenosis was originally considered critical when only peak aortic valve velocity and estimated gradient were considered. However, the Doppler-derived estimates of aortic valve area, which should take into account the hyperdynamic flow state known to exist during pregnancy, were not consistent with critical valve stenosis. Doppler values reflecting LV function can assist in differentiating critical from “pseudo-critical” aortic stenosis. REFERENCES
1. Arias F, Pineda J. Aortic stenosis and pregnancy. J Reprod Med 1978;20:229. 2. Hatle L. Noninvasive assessment and differentiation of left ventricular outflow obstruction with Doppler ultrasound, Circulation 1981;64:380. 3. Currie PJ, *Seward JB, Reeder GS, Vlietstra RE, Bresnahan DR, Bresnahan JF, Smith HC, Hagler DJ, Tajik AJ. Continuous wave Doppler echocardiographicassessmentof severity of aortic stenosis: a simultaneous Doppler-catheter correlative study in 100 adult patients. Circulation 1985;71:1162.
Brief Communications
4.
1385
Otto CM, Pearlman AS, ComessKA, Reamer RP, Janko CL, Huntsman LL. Determination of the stenotic aortic valve area in adults using Doppler echocardiography. J Am Co11
Cardiol 19&X7:509. Teirstein P, Yeager M, Yolk PG, Popp RL. Doppler echocardiographic measurement of aortic valve area in aortic stenosin: an&cation of the Gorlin formula. J Am ___ -a noninvasive . ~.. Co11 Cardiol 1986;8:i659. 6. Sullivan JM, Ramanathan KB. Management of medical problems in pregnancy-severe cardiac disease. N Engl J Med 1985;313:304. 7. Clark SL. Labor and delivery in the patient with structural cardiac disease. Clin Perinatol 1986;13:695. RH, Wyner J, Cohen S, Ueland K. 8. Rose BI, Holbrook Efficacy of Doppler echocardiography in the evaluation of aortic stenosis during pregnancy. Obstet Gynecol 1987; 69:431. 5.
Cardiac hydatid cyst with clinical features resembling subaortic stenosis Giovanni Russo, MD, Corrado Tamburino, MD, Salvatore CuscunB, MD, Giuseppe Arcidiacono, MD, Rosario Foti, MD, Domenico R. Grimaldi, MD, Valeria Calvi, MD, Salvatore Felis, and
Giuseppe Giuffrida, MD. Catania, Italy Cardiac involvement in hydatid disease is rare, occurring in less than 2% of cases.’ The most common site of involvement is the left ventricle, although involvement of the right ventricle and right atrium has also been reported.2 We report a case in which a large cyst located in the interventricular septum was indicative of clinical subaortic stenosis. The patient, a 50-year-old man, had been treated surgically at 20 years of age for a right pulmonary hydatid cyst and at age 38 years for a cyst in the left lobe of the liver. When he was 42 years of age, clinical and echocardiographic findings accidentally revealed obstructive hypertrophic cardiomyopathy, and he was treated with beta blockers (propanolol, 40 mg 3 times day). He was hospitalized because of a 2-month history of fatigue, dizziness, and dyspnea after slight physical exertion. At the time of admission the patient was in good clinical condition. The apical precordial impulse was displaced laterally (at the fifth intercostal
space, 2 cm externally
to the left midclav-
icular line and was forceful and enlarged. A presystolic apical impulse was present. No systolic thrill was palpable on the precordial area. The first heart sound was normal and the second heart sound was normally split. A 3/6 crescendo-decrescendo, blowing, systolic murmur was heard between the apex and the left sternal border of the third intercostal space. lt began well after the first heart sound and radiated to the axillae. No diastolic murmur was heard. From the Istituto di Cardiologia, Cattedra di Cardiochirurgia, tituto di Clinica Medica II, UniversitL di Cat&a. Reprint requests: Giovanni Russo, MD, Via S. Nico16,322-95045, anco (CT), Italy.
and the IsMisterbi-
1366
June 1989 American Heart Journal
Brief Communications
1. Apical four-chamber view of two-dimensional echocardiogram showing large cystic mass in interventricular septum (arrows) extends into right and left ventricular cavities. LA, left atrium; RA, right atrium; L V, left ventricle; RV, right ventricle. Fig.
Results of chest x-ray examination showed slight enlargement of the left ventricle. The ECG showed sinus bradicardia (45 beats/min), left anterior hemiblock, left ventricular hypertrophy, and inverted T waves in leads Vb, Vs, and Vs. Results of a hemoagglutinin test were negative. A twodimensional echocardiogram showed a large cystic image located in the middle third of the interventricular septum, which extended into the right and left ventricular cavities but protruded more into the left ventricle (Fig. 1). Beta blocker treatment was discontinued. Results of Holter monitoring 10 days later showed no atrioventricular conduction defects or significant arrhythmias. Heart rate ranged from 52 to 78 beats/min. Results of computed tomography confirmed the presence of the intraseptal mass and excluded the presence of cerebral, renal, hepatic, or pulmonary cysts. Results of hemodynamic examination showed subaortic stenosis with a peak systolic gradient of 40 mm Hg at rest with a left ventricular systolic pressure of 170 mm Hg. No abnormality was observed during right-heart catheterization. Results of angiography showed a wide calcified mass located in the interventricular septum causing a large filling defect and narrowing in the outflow tract of the left ventricle (Fig. 2, A, B, and C). Coronary arteriographic findings were normal. Fatigue and dizziness disappeared after propanolol therapy was discontinued. Inasmuch as the patient’s only persistent complaint was dyspnea after marked physical exertion, he was discharged without treatment. Surgery was not performed because massive calcification (as was present in our patient) indicates disappearance of the cyst and spontaneous recovery. In addition, surgical removal of the large intraventricular cyst could have posed more risks than did obstruction to left
c Fig. 2. Left ventricular angiogram in right anterior oblique projection. A, before contrast injection; 6, diastole; C, systole. Wide calcified cystic mass is evident (A); large filling defect resulting from cyst (6) causesnarrowing of left ventricular outflow tract (C).
Volume 117 Number 6
Brief
ventricular outflow, which did not present a heavy hemodynamic load. At follow-up examination, 6 months later the patient’s only symptom was dyspnea after marked physical exertion, and no clinical changes were observed. Results of repeated echocardiographic examinations and Holter monitoring showed no alterations. The patient has resumed his job as a carpenter. The clinical picture of hydatid disease depends on location and size of the cyst. Cardiac hydatid cysts may be asymptomatic or may provoke ECG signs of subepicardial ischemia3 and conduction disturbance of the His bundle or its branches.4 Pulmonary2 and mitral stenosis5 and tricuspid involvement” have been described. Massive pulmonary embolism, hydatid pulmonary hypertension, systemic embolism, and anaphylactic reactions have been reported as a result of rupture of the cyst.2 In our patient the cyst in the interventricular septum caused left anterior hemiblock and a clinical picture of subaortic stenosis; the latter is unusual in hydatid disease. REFERENCES
1.
Murphy TE, Kean BH, Venturine A, Lillekei CW. Echinococcus cyst of the left ventricle: report of a case with review of the pertinent literature. J Thorac Cardiovasc Surg 19’70;61: 443-5.
2.
3.
4.
5.
6.
Peres-Gomez F, Duran H, Tamames S, Perrote JL, Blanes A. Cardiac echinococcosis: clinical picture and complications. Br Heart J 1973;35:1326-31. Olivier JM, Perez-Benito L, Ferrufino 0, Sotillo JF, Nunez L. Cardiac hydatid cyst diagnosed by two-dimensional echocardiography: A:u HEART J 1982;104:164-5. De Los Amos E, Madurga MP, Perez-Leon J, Martinez JL, Urquia M. Hydatid cyst of interventricular septum causing left anterior ihemiblock. Br Heart J 1971;33:623-5. Munoz-Femandez J, Martinez-Ferrer J, Cia-Ruiz J, VedeperzTorrubia JL. Arcas-Meca R. Merino-An&o A. Embolia hidatica femoropoplitea y posterior emboli&ion multiple en un paciente con hidatidosis miocardica recidivante. Rev Esp Cardiol 1984:;37:371-4. Mancuso L, 13ondi F, Marchi S, Iacona MA, Guarnera S, Patane L. Cardiac hydatid disease with clinical features resembling tricuspid stenosis. AM HEART J 1987;113:1235-6.
Successful pregnancy following modified Fontan procedure in a patient with tricuspid atresia and recurrent atrial flutter Derek A. Fyfe, MD, PhD>b Paul C. Gillette, MD, J. Stephen Jones, MD,b and Gordon K. Danielson, MD: Charleston,
S. C., and Rochester,
Minn.
From South Carolina Children’s Heart Center, Divisions of Pediatric Cardiology,” Obstetrics, and Gynecology,b Medical University of South Carolina; and Division of Thoracic and Cardiovascular Surgery,’ Mayo Clinic Foundation. Reprint requests: IDerek A. Fyfe, MD, PhD, Division of Pediatric Csrdiology, Medical University of South Carolina, 171 Ashley Ave., Charleston, SC 29425.
Communications
1387
Successful pregnancy in patients with operated congenital heart disease is being reported with increasing frequency.l*z However, there is little experience in the world literature with patients becoming pregnant who have congenital heart disease that has been palliated by the modified Fontan operation. Additionally, as supraventricular arrhythmias emerge as frequent sequelae of this procedure, the influence of the hemodynamic burden of pregnancy on the control of these arrhythmias is important to define. We recently cared for a young woman who experienced a successful pregnancy after palliation of tricuspid atresia with the Fontan procedure and who was being maintained on antiarrhythmic drugs for treatment of recurring atria1 flutter. T.T. is a 24-year-old woman who underwent modified Fontan procedure at age 22 years. She was previously palliated for tricuspid atresia, pulmonary stenosis, and normally related great vessels with a right modified Blalock-Taussig shunt at age 2 years and a Potts’ anastomosis at age 5 years. She had done well prior to the Fontan procedure except for increasing cyanosis, polycythemia and fatigue, and although several episodes of subjectively rapid heart rates were reported, there were no episodes documented. Preoperatively before the Fontan procedure, echocardiography showed her left ventricular end-diastolic dimension (LVEDD) to be 7.6 cm with a shortening fraction of 34 % , Her modified Fontan procedure required closure of the Potts’ anastomosis from inside the pulmonary artery and direct anastomosis of the right atrium to the main pulmonary artery with augmentation using bovine pericardium. Two weeks postoperatively, she developed runs of atria1 flutter and was treated with digoxin, 0.125 mg twice daily. This eventually required treatment with DC cardioversion. Atria1 flutter subsequently recurred with 1:l conduction and a ventricular rate of 300 beatsfmin, and quinidine sulfate, 300 mg three times a day, was begun. Her postoperative LVEDD was 5.9 cm with fractional shortening (FS) of 34%. After 3 months, an attempt to discontinue quinidine resulted in recurrence of atria1 flutter within 48 hours. Invasive electrophysiologic catheterization documented atria1 flutter with 2:l atrioventricular block. Quinidine was restarted following cardioversion, and the patient has remained in sinus rhythm for the past l-‘/2 years. Contraception with a barrier method was recommended and was provided. At examination 6 months prior to pregnancy, she appeared well with no cyanosis. Her weight was 93 lbs. Her precordial examination was normal; a grade II/VI soft, continuous murmur was audible at the left upper sternal border. No hepatosplenomegaly or edema was present. The chest x-ray film showed a cardiothoracic ratio of 50%, and the electrocardiogram showed sinus rhythm. Two-dimensional, and color Doppler echocardiography showed normal left ventricular size and contractility, with an LVEDD of 5.5. cm and an FS of 34%. Blood flow in the pulmonary arteries was of a low velocity, 50 cmlsec, and was biphasic but with a continu-
of Doppler values and the continuity equation.’ In our patient, the estimates of aortic valve area were similar whether derived from the continuity equation or from the simplified Gorlin equation5 using the invasively obtained cardiac output and Doppler-derived valve velocity, and these estimates were consistent with significant, but not critical aortic stenosis. Unfortunately, we were unable to compare these values with those classically obtained by cardiac catheterization. To our knowledge, there has been only one previous report* in which continuous-wave Doppler was used to monitor the course of a pregnancy complicated by aortic stenosis. At various intervals, peak instantaneous valve gradients were obtained in a young woman with a deteriorating aortic valve prosthesis. Aortic valve area was unreported. The authors rightfully cautioned misinterpretation of Doppler-derived estimates of valve gradients in view of known pregnancy-related hemodynamic changes. However, estimates of aortic valve area, which take into account the effect of flow, should be valid. We await the establishment of normal Doppler values for pregnancy and further investigation in associated pathologic states is needed. In spite of these limitations, Doppler-derived data obtained in the overall noninvasive evaluation of this patient played a vital role in outlining the appropriate peripartum management. The preferred method of delivery in these patients remains controversial. The major objective is to avoid volume depletion and hypotension, which can lead to catastrophic hemodynamic collapse. This can be precipitated by peripartum blood loss, supine vena caval obstruction, anesthetic-related vasodilatation, and even myocardial infarction..1~6~7In view of this patient’s persistent excellent LV systolic function, Doppler estimates of noncritical aortic stenosis, and normal right heart and PCWPs, we elected to allow her to undergo labor and to deliver entirely in the left lateral decubitus position with continuous fetal and right heart pressure monitoring. In conclusion, we describe the safe and uneventful labor and delivery of an otherwise healthy young woman with congenital aortic valve stenosis. The degree of stenosis was originally considered critical when only peak aortic valve velocity and estimated gradient were considered. However, the Doppler-derived estimates of aortic valve area, which should take into account the hyperdynamic flow state known to exist during pregnancy, were not consistent with critical valve stenosis. Doppler values reflecting LV function can assist in differentiating critical from “pseudo-critical” aortic stenosis. REFERENCES
1. Arias F, Pineda J. Aortic stenosis and pregnancy. J Reprod Med 1978;20:229. 2. Hatle L. Noninvasive assessment and differentiation of left ventricular outflow obstruction with Doppler ultrasound, Circulation 1981;64:380. 3. Currie PJ, *Seward JB, Reeder GS, Vlietstra RE, Bresnahan DR, Bresnahan JF, Smith HC, Hagler DJ, Tajik AJ. Continuous wave Doppler echocardiographicassessmentof severity of aortic stenosis: a simultaneous Doppler-catheter correlative study in 100 adult patients. Circulation 1985;71:1162.
Brief Communications
4.
1385
Otto CM, Pearlman AS, ComessKA, Reamer RP, Janko CL, Huntsman LL. Determination of the stenotic aortic valve area in adults using Doppler echocardiography. J Am Co11
Cardiol 19&X7:509. Teirstein P, Yeager M, Yolk PG, Popp RL. Doppler echocardiographic measurement of aortic valve area in aortic stenosin: an&cation of the Gorlin formula. J Am ___ -a noninvasive . ~.. Co11 Cardiol 1986;8:i659. 6. Sullivan JM, Ramanathan KB. Management of medical problems in pregnancy-severe cardiac disease. N Engl J Med 1985;313:304. 7. Clark SL. Labor and delivery in the patient with structural cardiac disease. Clin Perinatol 1986;13:695. RH, Wyner J, Cohen S, Ueland K. 8. Rose BI, Holbrook Efficacy of Doppler echocardiography in the evaluation of aortic stenosis during pregnancy. Obstet Gynecol 1987; 69:431. 5.
Cardiac hydatid cyst with clinical features resembling subaortic stenosis Giovanni Russo, MD, Corrado Tamburino, MD, Salvatore CuscunB, MD, Giuseppe Arcidiacono, MD, Rosario Foti, MD, Domenico R. Grimaldi, MD, Valeria Calvi, MD, Salvatore Felis, and
Giuseppe Giuffrida, MD. Catania, Italy Cardiac involvement in hydatid disease is rare, occurring in less than 2% of cases.’ The most common site of involvement is the left ventricle, although involvement of the right ventricle and right atrium has also been reported.2 We report a case in which a large cyst located in the interventricular septum was indicative of clinical subaortic stenosis. The patient, a 50-year-old man, had been treated surgically at 20 years of age for a right pulmonary hydatid cyst and at age 38 years for a cyst in the left lobe of the liver. When he was 42 years of age, clinical and echocardiographic findings accidentally revealed obstructive hypertrophic cardiomyopathy, and he was treated with beta blockers (propanolol, 40 mg 3 times day). He was hospitalized because of a 2-month history of fatigue, dizziness, and dyspnea after slight physical exertion. At the time of admission the patient was in good clinical condition. The apical precordial impulse was displaced laterally (at the fifth intercostal
space, 2 cm externally
to the left midclav-
icular line and was forceful and enlarged. A presystolic apical impulse was present. No systolic thrill was palpable on the precordial area. The first heart sound was normal and the second heart sound was normally split. A 3/6 crescendo-decrescendo, blowing, systolic murmur was heard between the apex and the left sternal border of the third intercostal space. lt began well after the first heart sound and radiated to the axillae. No diastolic murmur was heard. From the Istituto di Cardiologia, Cattedra di Cardiochirurgia, tituto di Clinica Medica II, UniversitL di Cat&a. Reprint requests: Giovanni Russo, MD, Via S. Nico16,322-95045, anco (CT), Italy.
and the IsMisterbi-
1366
June 1989 American Heart Journal
Brief Communications
1. Apical four-chamber view of two-dimensional echocardiogram showing large cystic mass in interventricular septum (arrows) extends into right and left ventricular cavities. LA, left atrium; RA, right atrium; L V, left ventricle; RV, right ventricle. Fig.
Results of chest x-ray examination showed slight enlargement of the left ventricle. The ECG showed sinus bradicardia (45 beats/min), left anterior hemiblock, left ventricular hypertrophy, and inverted T waves in leads Vb, Vs, and Vs. Results of a hemoagglutinin test were negative. A twodimensional echocardiogram showed a large cystic image located in the middle third of the interventricular septum, which extended into the right and left ventricular cavities but protruded more into the left ventricle (Fig. 1). Beta blocker treatment was discontinued. Results of Holter monitoring 10 days later showed no atrioventricular conduction defects or significant arrhythmias. Heart rate ranged from 52 to 78 beats/min. Results of computed tomography confirmed the presence of the intraseptal mass and excluded the presence of cerebral, renal, hepatic, or pulmonary cysts. Results of hemodynamic examination showed subaortic stenosis with a peak systolic gradient of 40 mm Hg at rest with a left ventricular systolic pressure of 170 mm Hg. No abnormality was observed during right-heart catheterization. Results of angiography showed a wide calcified mass located in the interventricular septum causing a large filling defect and narrowing in the outflow tract of the left ventricle (Fig. 2, A, B, and C). Coronary arteriographic findings were normal. Fatigue and dizziness disappeared after propanolol therapy was discontinued. Inasmuch as the patient’s only persistent complaint was dyspnea after marked physical exertion, he was discharged without treatment. Surgery was not performed because massive calcification (as was present in our patient) indicates disappearance of the cyst and spontaneous recovery. In addition, surgical removal of the large intraventricular cyst could have posed more risks than did obstruction to left
c Fig. 2. Left ventricular angiogram in right anterior oblique projection. A, before contrast injection; 6, diastole; C, systole. Wide calcified cystic mass is evident (A); large filling defect resulting from cyst (6) causesnarrowing of left ventricular outflow tract (C).
Volume 117 Number 6
Brief
ventricular outflow, which did not present a heavy hemodynamic load. At follow-up examination, 6 months later the patient’s only symptom was dyspnea after marked physical exertion, and no clinical changes were observed. Results of repeated echocardiographic examinations and Holter monitoring showed no alterations. The patient has resumed his job as a carpenter. The clinical picture of hydatid disease depends on location and size of the cyst. Cardiac hydatid cysts may be asymptomatic or may provoke ECG signs of subepicardial ischemia3 and conduction disturbance of the His bundle or its branches.4 Pulmonary2 and mitral stenosis5 and tricuspid involvement” have been described. Massive pulmonary embolism, hydatid pulmonary hypertension, systemic embolism, and anaphylactic reactions have been reported as a result of rupture of the cyst.2 In our patient the cyst in the interventricular septum caused left anterior hemiblock and a clinical picture of subaortic stenosis; the latter is unusual in hydatid disease. REFERENCES
1.
Murphy TE, Kean BH, Venturine A, Lillekei CW. Echinococcus cyst of the left ventricle: report of a case with review of the pertinent literature. J Thorac Cardiovasc Surg 19’70;61: 443-5.
2.
3.
4.
5.
6.
Peres-Gomez F, Duran H, Tamames S, Perrote JL, Blanes A. Cardiac echinococcosis: clinical picture and complications. Br Heart J 1973;35:1326-31. Olivier JM, Perez-Benito L, Ferrufino 0, Sotillo JF, Nunez L. Cardiac hydatid cyst diagnosed by two-dimensional echocardiography: A:u HEART J 1982;104:164-5. De Los Amos E, Madurga MP, Perez-Leon J, Martinez JL, Urquia M. Hydatid cyst of interventricular septum causing left anterior ihemiblock. Br Heart J 1971;33:623-5. Munoz-Femandez J, Martinez-Ferrer J, Cia-Ruiz J, VedeperzTorrubia JL. Arcas-Meca R. Merino-An&o A. Embolia hidatica femoropoplitea y posterior emboli&ion multiple en un paciente con hidatidosis miocardica recidivante. Rev Esp Cardiol 1984:;37:371-4. Mancuso L, 13ondi F, Marchi S, Iacona MA, Guarnera S, Patane L. Cardiac hydatid disease with clinical features resembling tricuspid stenosis. AM HEART J 1987;113:1235-6.
Successful pregnancy following modified Fontan procedure in a patient with tricuspid atresia and recurrent atrial flutter Derek A. Fyfe, MD, PhD>b Paul C. Gillette, MD, J. Stephen Jones, MD,b and Gordon K. Danielson, MD: Charleston,
S. C., and Rochester,
Minn.
From South Carolina Children’s Heart Center, Divisions of Pediatric Cardiology,” Obstetrics, and Gynecology,b Medical University of South Carolina; and Division of Thoracic and Cardiovascular Surgery,’ Mayo Clinic Foundation. Reprint requests: IDerek A. Fyfe, MD, PhD, Division of Pediatric Csrdiology, Medical University of South Carolina, 171 Ashley Ave., Charleston, SC 29425.
Communications
1387
Successful pregnancy in patients with operated congenital heart disease is being reported with increasing frequency.l*z However, there is little experience in the world literature with patients becoming pregnant who have congenital heart disease that has been palliated by the modified Fontan operation. Additionally, as supraventricular arrhythmias emerge as frequent sequelae of this procedure, the influence of the hemodynamic burden of pregnancy on the control of these arrhythmias is important to define. We recently cared for a young woman who experienced a successful pregnancy after palliation of tricuspid atresia with the Fontan procedure and who was being maintained on antiarrhythmic drugs for treatment of recurring atria1 flutter. T.T. is a 24-year-old woman who underwent modified Fontan procedure at age 22 years. She was previously palliated for tricuspid atresia, pulmonary stenosis, and normally related great vessels with a right modified Blalock-Taussig shunt at age 2 years and a Potts’ anastomosis at age 5 years. She had done well prior to the Fontan procedure except for increasing cyanosis, polycythemia and fatigue, and although several episodes of subjectively rapid heart rates were reported, there were no episodes documented. Preoperatively before the Fontan procedure, echocardiography showed her left ventricular end-diastolic dimension (LVEDD) to be 7.6 cm with a shortening fraction of 34 % , Her modified Fontan procedure required closure of the Potts’ anastomosis from inside the pulmonary artery and direct anastomosis of the right atrium to the main pulmonary artery with augmentation using bovine pericardium. Two weeks postoperatively, she developed runs of atria1 flutter and was treated with digoxin, 0.125 mg twice daily. This eventually required treatment with DC cardioversion. Atria1 flutter subsequently recurred with 1:l conduction and a ventricular rate of 300 beatsfmin, and quinidine sulfate, 300 mg three times a day, was begun. Her postoperative LVEDD was 5.9 cm with fractional shortening (FS) of 34%. After 3 months, an attempt to discontinue quinidine resulted in recurrence of atria1 flutter within 48 hours. Invasive electrophysiologic catheterization documented atria1 flutter with 2:l atrioventricular block. Quinidine was restarted following cardioversion, and the patient has remained in sinus rhythm for the past l-‘/2 years. Contraception with a barrier method was recommended and was provided. At examination 6 months prior to pregnancy, she appeared well with no cyanosis. Her weight was 93 lbs. Her precordial examination was normal; a grade II/VI soft, continuous murmur was audible at the left upper sternal border. No hepatosplenomegaly or edema was present. The chest x-ray film showed a cardiothoracic ratio of 50%, and the electrocardiogram showed sinus rhythm. Two-dimensional, and color Doppler echocardiography showed normal left ventricular size and contractility, with an LVEDD of 5.5. cm and an FS of 34%. Blood flow in the pulmonary arteries was of a low velocity, 50 cmlsec, and was biphasic but with a continu-