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Progressive pulmonary hypertension after the arterial switch procedure
Progressive Pulmonary Hypertension After the Arterial Switch Procedure Naravanswami Sreeram, MRCP, Andrew Petros, MD, Ian Peat-t,MRCP, and Robert Arnold, FRCP’ rogressive pulmonary hyperten- nosed to have simple transposition sion resulting from pulmonary of the great arteries by 2-dimensionP vascular disease is well recognized al echocardiography. Balloon atria1 in patients with transposition of the great arteries.‘,* In early infancy, these complications occur predominantly in patients with an associated ventricular septal defect or large patent arterial duct,3,4although they have also been reported in infants without significant shunting at the ventricular or arterial leve1.5*6Early corrective surgery has been advocated to reduce the incidence of obstructive pulmonary vascular disease.(jq7 Since the introduction of the arterial switch procedure, early correction is now routinely performed where possible, and pulmonary vascular disease has been rarely encountered.8,9We report 3 patients in whom pulmonary hypertension and consequentright ventricular dysfunction developed at varying intervals after anatomicrepair of transposition. Patient 1: A 2-day-old male infant with central cyanosis was diagFrom the Heart Clinic, Royal Liverpool Children’s Hospital, Eaton Road, Liverpool LIZ 2AP, United Kingdom. Manuscript received June 4, 1993; revised manuscript received and acceptedJuly 13, 1993.
septostomy improved systemic oxygen saturation from 56 to 82%. Repeat cardiac catheterization at age 4 months revealed right and left ventricular systolic pressures of 82 and 59 mm Hg, respectively,without pulmonary stenosis. An arterial switch procedure was per&ormed, and the postoperative course was uneven@l. The patient presented3 months later with heart failure. At echocardiography, the right atrium and ventricle were dilated, with a high-velocity (4.5 mls) jet of tricuspid regurgitation. There was no evidence of pulmonary arterial stenoses, and the left ventricle appeared normal. The patient died suddenly 1 month later. Postmortem examination con$rmed an anatomically satisfactory repair, without distal pulmonary arterial stenoses.Histologic examination of lung tissue was not petiormed. Patient 2: A term female infant presenting at age 6 weeks with central cyanosis was diagnosed to have simple transposition of the great arteries. The arterial duct was widely patent. At cardiac catheterization, the right and left ventricular systolic
: i. Patient 2: 2dimensional echocardiogram in 4dmmbor view showing right vmtrlcular (RV) hypdmphy and psadoxical movement of intervmk pseptum &ma// arrows) toward left venkicle (LV). lnteratfial septum also toward left (bge arrow). IA q left atrium; RA = @ht athum.
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THE AMERICANJOURNALOF CARDIOLOGY VOLUME73
MARCH15,1994
pressures were similar (71 and 67 mm Hg, respectively}. An arterial switch operation was performed and the duct ligated. Follow-up electrocardiography showed persistent right ventricular hypertrophy. At age 2 years, the patient developeda new pansystolic murmur of tricuspid regurgitation. Echocardiography showed right atria1 dilatation with right ventricular dyskinesia and hypertrophy (Figure I), without outflow obstruction. By Doppler echocardiography, the peak velocity of tricuspid regurgitation was 5.5 mls. Cardiac catheterization was performed to evaluate the pulmonary vascular resistance.After induction of general anesthesia, the patient developedprofound hypotension and bradycardia, and could not be resuscitated. Postmortem examination showed Heath-Edwards grade 34 changes in all segments of the lung.tO Patient 3: This male infant presented at age 2 months with cyanosis. At echocardiography a diagnosis of transposition of the great arteries with a small ventricular septal defect, mild subpulmonary stenosis and a small arterial duct was made. At catheterization, the right and lef ventricular systolic pressures were II0 and 40 mm Hg, respectively, with a 20 mm Hg gradient from the left ventricle to pulmonary artery. Balloon atria1 septostomy resulted in a transient improvementin systemicoxygen saturation from 50 to 80%. Three days later, in view of increasing cyanosis, a modtfied right sided Blalock-Taussig shunt (4 mm Gore&x graft) was peformed, resulting in a systemic oxygen saturation of 83%. At age 6 months, cardiac catheterization revealed systolic ventricular pressures of 100 and 34 mm Hg in the right and left ventricles, respectively. The ventricular septal defect had closed, and subpulmonary stenosiswas mild (10 mm Hg). In view of right ventricular dysfunction, it was decided to per$orm a rapid, 2-stage, anatomic correction.” Therefore, banding of the pulmonary artery was performed, followed by anatomic repair 19 days later. After repair the patient could not be weanedfrom the respirator Tricuspid valve regurgitation could be detected by Doppler
echocardiography, with a peak velocity of regurgitation of 4.5 mls. At cardiac catheterization 3 weeks after repair, the right ventricular systolic pressure ranged between 70 (suhsystemic) and 120 (suprasystemic) mm Hg, and was responsive to both high inspired oxygen concentrations and nitric oxide inhalation.t2 Pulmonary arterial wedge and left ventricular end-diastolic pressures were increased (mean of 15 and 12 mm Hg, respectively). The patient was eventually weaned from the respirator 50 days after surgery, hut continues to show echocardiographic evidence of severe pulmonary hypertension at follow-up.
Obstructive pulmonary vascular diseasedevelops relatively early and progressesmore rapidly in patients with transposition of the great arteries than in those with other structural cardiac defects.‘*2,6This accelerated pulmonary vascular disease has usually been associatedwith the presenceof a large ventricular septal defect or arterial duct. These patients may also have persistence of pulmonary hypertension despite surgical correction.I3 In some cases, however, progressive pulmonary vascular disease may occur in the absenceof associatedshunts. Early surgical repair has been advocatedto prevent these complications and is now feasible with the use of the arterial switch operation. The only reported patient who developed pulmonary vascular disease after an atria1 correction procedure at age ~12 months was a 7-month-old infant in whom suprasystemicpulmonary arterial pressurewas recorded4 months after a Mustard procedure.” The preoperative pulmonary arterial pressure was normal. In a large series of patients undergoing the arterial switch operation, severe pulmonary hypertension was documented in 1 patient, 6 weeks after an arterial switch procedure performed at age 2 weeks.s9 The 3 patients described in this report have some of the unique features of the natural or postsurgical history of transposition of the great arteries. Patient 1 with simple transposition, who had initially been con-
sidered for an atria1repair, had persistent pulmonary hypertension at age 4 months. Although this enabled anatomic repair to be performed without preparatory banding of the pulmonary artery, pulmonary hypertension progressedafter repair, leading to death. In patient 2, in whom persistentductal patency was associated with pulmonary hypertension at age 6 weeks, anatomic repair at presentationdid not halt the progression of pulmonary vascular disease. In patient 3 in whom subpulmonary stenosisand normal pulmonary arterial pressurewere recorded at initial catheterization, a palliative shunt was needed initially. In view of the reported risk of pulmonary hypertension in associationwith large palliative shunts,’ a 4 mm diameter interposition graft was used to construct the shunt. Despite having a normal pulmonary arterial pressure at age 6 months, the patient developed severepulmonary hypertension within 3 weeks of repair. The determinantsof this early development of obstructive pulmonary vascular diseasein patients without an associatedshunt at the ventricular or arterial level are poorly understood. Many proposed mechanisms including polycythemia with increasedblood viscosityr4or recurrent pulmonary emboli, chronic hypoxemiar5 and increased pulmonary arterial floww6are operative before corrective surgery. Alternative factors that may explain the clinical course reported in these 3 cases include hyperreactivity of the pulmonary vascular bed (patient 3) inherent structural defects in the muscular pulmonary arteriesI and presence or persistence of extensive shunts through bronchial collateral arteries.‘* An inherent abnormality of the pulmonary vasculatureis further supported by a recentreport of histologic evidence of advanced pulmonary vascular disease in neonates with simple transposition of the great arteries.t9 In conclusion, obstructive pulmonary vascular disease may be progressive despite early definitive repair in a subset of patients with transposition of the great arteries. Clinical awareness and the routine
use of 2-dimensional Doppler echocardiography at follow-up evaluation should identify these patients early.
1. Ferguson DJ, Adams P, Watson D. Pulmonruy ateriosclerosis m transposition of the great vessels. Am J LX:, Cldd 1960,99:653~61, 2. Ferencz C. Transposition of the great vessels. Pathophysiologic considerations based upon a study of the lungs. Circulation 1966;33:232-241. 3. Newfeld EA, Paul MH, Muster AJ, ldriss FS. Pulmonluy vascular diseaz in complete transposition of the great arteries: a study of 200 patients. Am J Cardid 1974:34:7X32. 4. Waldman JD, Paul MH, Newfeld EA, Muster AI, Idriss FS. Transposition of the great arteries with intact venhicular septum and patent ductus arteriosus. Am J Curdiol
1977:39:232-23X.
5. Berman W, Whitman V, Pierce WS, Waldhausen JA. The development of pulmonary vascular obstructive disease after successful Mustard operation in early infancy. Cinvlan’on 1978;58:181-185, 8. Lakier JB, Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Early onset of pulmonary vascular obstruction in patients with aonopulmonary transposition and intact ventricular septum. Ciu u/urion I9755 I : x75-m. 7. Clark$on PM, Barrart-Boyes GB. Neutze JM, Lowe JB. Results over a ten-year period of palliation followed by corrective surgery for complete transposition of the great arteries. Ciwularion 197245: I25 I -I 25X. 8. Castaneda AR, Trusler GA, Paul MH, Blackstone EH, Kirklin JW. The early results of treatment of simple transposition in the current era. .I Thomc CnrdioI’US( surg 19X&95: 14-2X. 9. Losay J, Planche C. Geradin B, Lacour-Gayet F, Bnmiaux 1, Kachaner J. Midterm surgical results of arterial switch operation for transposition of the great ateties with intact septum. Cimdurion 1990;RZ(suppl 1v):Iv-l4Mv-l5O. 10. Heath D, Edwards JE. The pathology of hypertensive pulmonary vaxular disease: a description of six grades of structural changes in the pulmonary art&s with special reference to congenital cardiac septal defects. Circulation 1958: I X533-547. 11. Jonas RA. Giglia TM, Sanders SP. Wemovaky G, Nadal-Ginard B. Mayer JE Jr, Castaneda AR. Rapid, two-stage arterial switch for tramposition of the great arteries and intact ventricular septum beyond the neona1989;XO(suppl I):[-203%-20X tal paiod. Cifulorim 12. Robens JD. Lang P. Bigatello LM, Vlahakes GJ. Zap01 WM. Inhaled nitric oxide m congenital heart disease. Cirrulntron 1993:X7:447-453. 13. Shaher R, Kidd L. Effect of ventricul.ar septal defect and patent ductu\ arteriosus on left ventricular pressure in complete transposition of the great vessels. Cinulanon 196X:37:232-237. 14. Nihill MR. McNamara DG. Vick RL. The effects of increased blood viscaity on pulmonary vaa~lar rtistance. Am Heorr J 1976.92~65-72. 15. Naeye RL. Hypoxemia and pulmonary hypenension. Awh P&ho/ 1961:7 I :447WlS2. 16. Graham TP. Jarmakani JM, Canent RV, Jew&t PH. Quantification of left heati volume and systolic output in transposition of the great arteries. Cwcularim 197 1:44:89w%9. 17. Yamaki S, Tezuka F. Quantitative analysis of pulmonary vascular direase in complete transposition of the great arteries. Cimdation 1976:54:805~809 16. Aziz KU, Paul MH, Rowe RD. Bronchopulmonary circulation m d-transposmon of the great arteries: passible role m genesis of accelerated pulmonary vascular disease. An! .I Cardiol 1977:39:432-43X. 19. Kumar A, Taylor GP, Sandor GGS, Pattenon MWH. Pulmonary vascular disease in neonates with transporition of the great arteries and intact ventricuIx septum. Br Herr J 1993:69:442-445.
CASEREPORTS 621
septostomy improved systemic oxygen saturation from 56 to 82%. Repeat cardiac catheterization at age 4 months revealed right and left ventricular systolic pressures of 82 and 59 mm Hg, respectively,without pulmonary stenosis. An arterial switch procedure was per&ormed, and the postoperative course was uneven@l. The patient presented3 months later with heart failure. At echocardiography, the right atrium and ventricle were dilated, with a high-velocity (4.5 mls) jet of tricuspid regurgitation. There was no evidence of pulmonary arterial stenoses, and the left ventricle appeared normal. The patient died suddenly 1 month later. Postmortem examination con$rmed an anatomically satisfactory repair, without distal pulmonary arterial stenoses.Histologic examination of lung tissue was not petiormed. Patient 2: A term female infant presenting at age 6 weeks with central cyanosis was diagnosed to have simple transposition of the great arteries. The arterial duct was widely patent. At cardiac catheterization, the right and left ventricular systolic
: i. Patient 2: 2dimensional echocardiogram in 4dmmbor view showing right vmtrlcular (RV) hypdmphy and psadoxical movement of intervmk pseptum &ma// arrows) toward left venkicle (LV). lnteratfial septum also toward left (bge arrow). IA q left atrium; RA = @ht athum.
620
THE AMERICANJOURNALOF CARDIOLOGY VOLUME73
MARCH15,1994
pressures were similar (71 and 67 mm Hg, respectively}. An arterial switch operation was performed and the duct ligated. Follow-up electrocardiography showed persistent right ventricular hypertrophy. At age 2 years, the patient developeda new pansystolic murmur of tricuspid regurgitation. Echocardiography showed right atria1 dilatation with right ventricular dyskinesia and hypertrophy (Figure I), without outflow obstruction. By Doppler echocardiography, the peak velocity of tricuspid regurgitation was 5.5 mls. Cardiac catheterization was performed to evaluate the pulmonary vascular resistance.After induction of general anesthesia, the patient developedprofound hypotension and bradycardia, and could not be resuscitated. Postmortem examination showed Heath-Edwards grade 34 changes in all segments of the lung.tO Patient 3: This male infant presented at age 2 months with cyanosis. At echocardiography a diagnosis of transposition of the great arteries with a small ventricular septal defect, mild subpulmonary stenosis and a small arterial duct was made. At catheterization, the right and lef ventricular systolic pressures were II0 and 40 mm Hg, respectively, with a 20 mm Hg gradient from the left ventricle to pulmonary artery. Balloon atria1 septostomy resulted in a transient improvementin systemicoxygen saturation from 50 to 80%. Three days later, in view of increasing cyanosis, a modtfied right sided Blalock-Taussig shunt (4 mm Gore&x graft) was peformed, resulting in a systemic oxygen saturation of 83%. At age 6 months, cardiac catheterization revealed systolic ventricular pressures of 100 and 34 mm Hg in the right and left ventricles, respectively. The ventricular septal defect had closed, and subpulmonary stenosiswas mild (10 mm Hg). In view of right ventricular dysfunction, it was decided to per$orm a rapid, 2-stage, anatomic correction.” Therefore, banding of the pulmonary artery was performed, followed by anatomic repair 19 days later. After repair the patient could not be weanedfrom the respirator Tricuspid valve regurgitation could be detected by Doppler
echocardiography, with a peak velocity of regurgitation of 4.5 mls. At cardiac catheterization 3 weeks after repair, the right ventricular systolic pressure ranged between 70 (suhsystemic) and 120 (suprasystemic) mm Hg, and was responsive to both high inspired oxygen concentrations and nitric oxide inhalation.t2 Pulmonary arterial wedge and left ventricular end-diastolic pressures were increased (mean of 15 and 12 mm Hg, respectively). The patient was eventually weaned from the respirator 50 days after surgery, hut continues to show echocardiographic evidence of severe pulmonary hypertension at follow-up.
Obstructive pulmonary vascular diseasedevelops relatively early and progressesmore rapidly in patients with transposition of the great arteries than in those with other structural cardiac defects.‘*2,6This accelerated pulmonary vascular disease has usually been associatedwith the presenceof a large ventricular septal defect or arterial duct. These patients may also have persistence of pulmonary hypertension despite surgical correction.I3 In some cases, however, progressive pulmonary vascular disease may occur in the absenceof associatedshunts. Early surgical repair has been advocatedto prevent these complications and is now feasible with the use of the arterial switch operation. The only reported patient who developed pulmonary vascular disease after an atria1 correction procedure at age ~12 months was a 7-month-old infant in whom suprasystemicpulmonary arterial pressurewas recorded4 months after a Mustard procedure.” The preoperative pulmonary arterial pressure was normal. In a large series of patients undergoing the arterial switch operation, severe pulmonary hypertension was documented in 1 patient, 6 weeks after an arterial switch procedure performed at age 2 weeks.s9 The 3 patients described in this report have some of the unique features of the natural or postsurgical history of transposition of the great arteries. Patient 1 with simple transposition, who had initially been con-
sidered for an atria1repair, had persistent pulmonary hypertension at age 4 months. Although this enabled anatomic repair to be performed without preparatory banding of the pulmonary artery, pulmonary hypertension progressedafter repair, leading to death. In patient 2, in whom persistentductal patency was associated with pulmonary hypertension at age 6 weeks, anatomic repair at presentationdid not halt the progression of pulmonary vascular disease. In patient 3 in whom subpulmonary stenosisand normal pulmonary arterial pressurewere recorded at initial catheterization, a palliative shunt was needed initially. In view of the reported risk of pulmonary hypertension in associationwith large palliative shunts,’ a 4 mm diameter interposition graft was used to construct the shunt. Despite having a normal pulmonary arterial pressure at age 6 months, the patient developed severepulmonary hypertension within 3 weeks of repair. The determinantsof this early development of obstructive pulmonary vascular diseasein patients without an associatedshunt at the ventricular or arterial level are poorly understood. Many proposed mechanisms including polycythemia with increasedblood viscosityr4or recurrent pulmonary emboli, chronic hypoxemiar5 and increased pulmonary arterial floww6are operative before corrective surgery. Alternative factors that may explain the clinical course reported in these 3 cases include hyperreactivity of the pulmonary vascular bed (patient 3) inherent structural defects in the muscular pulmonary arteriesI and presence or persistence of extensive shunts through bronchial collateral arteries.‘* An inherent abnormality of the pulmonary vasculatureis further supported by a recentreport of histologic evidence of advanced pulmonary vascular disease in neonates with simple transposition of the great arteries.t9 In conclusion, obstructive pulmonary vascular disease may be progressive despite early definitive repair in a subset of patients with transposition of the great arteries. Clinical awareness and the routine
use of 2-dimensional Doppler echocardiography at follow-up evaluation should identify these patients early.
1. Ferguson DJ, Adams P, Watson D. Pulmonruy ateriosclerosis m transposition of the great vessels. Am J LX:, Cldd 1960,99:653~61, 2. Ferencz C. Transposition of the great vessels. Pathophysiologic considerations based upon a study of the lungs. Circulation 1966;33:232-241. 3. Newfeld EA, Paul MH, Muster AJ, ldriss FS. Pulmonluy vascular diseaz in complete transposition of the great arteries: a study of 200 patients. Am J Cardid 1974:34:7X32. 4. Waldman JD, Paul MH, Newfeld EA, Muster AI, Idriss FS. Transposition of the great arteries with intact venhicular septum and patent ductus arteriosus. Am J Curdiol
1977:39:232-23X.
5. Berman W, Whitman V, Pierce WS, Waldhausen JA. The development of pulmonary vascular obstructive disease after successful Mustard operation in early infancy. Cinvlan’on 1978;58:181-185, 8. Lakier JB, Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Early onset of pulmonary vascular obstruction in patients with aonopulmonary transposition and intact ventricular septum. Ciu u/urion I9755 I : x75-m. 7. Clark$on PM, Barrart-Boyes GB. Neutze JM, Lowe JB. Results over a ten-year period of palliation followed by corrective surgery for complete transposition of the great arteries. Ciwularion 197245: I25 I -I 25X. 8. Castaneda AR, Trusler GA, Paul MH, Blackstone EH, Kirklin JW. The early results of treatment of simple transposition in the current era. .I Thomc CnrdioI’US( surg 19X&95: 14-2X. 9. Losay J, Planche C. Geradin B, Lacour-Gayet F, Bnmiaux 1, Kachaner J. Midterm surgical results of arterial switch operation for transposition of the great ateties with intact septum. Cimdurion 1990;RZ(suppl 1v):Iv-l4Mv-l5O. 10. Heath D, Edwards JE. The pathology of hypertensive pulmonary vaxular disease: a description of six grades of structural changes in the pulmonary art&s with special reference to congenital cardiac septal defects. Circulation 1958: I X533-547. 11. Jonas RA. Giglia TM, Sanders SP. Wemovaky G, Nadal-Ginard B. Mayer JE Jr, Castaneda AR. Rapid, two-stage arterial switch for tramposition of the great arteries and intact ventricular septum beyond the neona1989;XO(suppl I):[-203%-20X tal paiod. Cifulorim 12. Robens JD. Lang P. Bigatello LM, Vlahakes GJ. Zap01 WM. Inhaled nitric oxide m congenital heart disease. Cirrulntron 1993:X7:447-453. 13. Shaher R, Kidd L. Effect of ventricul.ar septal defect and patent ductu\ arteriosus on left ventricular pressure in complete transposition of the great vessels. Cinulanon 196X:37:232-237. 14. Nihill MR. McNamara DG. Vick RL. The effects of increased blood viscaity on pulmonary vaa~lar rtistance. Am Heorr J 1976.92~65-72. 15. Naeye RL. Hypoxemia and pulmonary hypenension. Awh P&ho/ 1961:7 I :447WlS2. 16. Graham TP. Jarmakani JM, Canent RV, Jew&t PH. Quantification of left heati volume and systolic output in transposition of the great arteries. Cwcularim 197 1:44:89w%9. 17. Yamaki S, Tezuka F. Quantitative analysis of pulmonary vascular direase in complete transposition of the great arteries. Cimdation 1976:54:805~809 16. Aziz KU, Paul MH, Rowe RD. Bronchopulmonary circulation m d-transposmon of the great arteries: passible role m genesis of accelerated pulmonary vascular disease. An! .I Cardiol 1977:39:432-43X. 19. Kumar A, Taylor GP, Sandor GGS, Pattenon MWH. Pulmonary vascular disease in neonates with transporition of the great arteries and intact ventricuIx septum. Br Herr J 1993:69:442-445.
CASEREPORTS 621