Surgical Treatment of Congenital Unilateral Pulmonary Arterial Stenosis With Contralateral Pulmonary Hypertension
LAWRENCE H. COHN, MD, FACC JOHN H. SANDERS, Jr., MD JOHN
J. COLLINS,
Jr.,
MD,
FACC
Boston, Massachusetts
This report describes the dlagnosis and surgical treatment of a 48 year old patient with congenital right pulmonary arterial ostial stenosis, no intraor extracardiac shunts and systemic levels of pressure in the main and left pulmonary artery with pulmonary vascular obstruction in the left lung. Operation consisted of a bypass graft of 10 mm woven Dacron from the main pulmonary artery to the distal right pulmonary artery producing an immediate 50 percent reduction in mean left pulmonary arterial pressure. Late postoperative evaluation revealed equal perfusion of both lungs, maintenance of the reduced pulmonary arterial pressure and improvement from New York Heart Association functlonal class IV to class I.
Congenital stenosis of the origin of one or both principal branches of the pulmonary arterial system has been reported only infrequently and is usually accompanied by other malformations of the heart and great vessels.l-6 The patient to be described demonstrated congenital stenosis of the origin of the right pulmonary artery with severe pulmonary hypertension and pulmonary vascular obstruction in the left lung and underwent successful surgical treatment of this unusual lesion. Case Report
From the Department of Surgery, Harvard Medical School, and Division of Thoracic and Cardiac Surgery, Peter Bent Brigham Hospital, Boston, Mass. Manuscript received May 5, 1975; revised manuscript received July 24. 1975, accepted July 29, 1975. Address for reprints: Lawrence H. Cohn, MD, Division of Thoracic and Cardiac Surgery, Peter Bent Brigham Hospital, 721 Huntington Ave., Boston, Mass. 02115.
A 48 year old woman was admitted to her local hospital for severe dyspnea that had progressed in the preceding five years. During the previous year, her condition had deteriorated until she was bedridden and in New York Heart Association functional class IV. Her previous medical history was essentially normal and she had no previous hospitalizations except for childbirth. She had been told she had a heart murmur when she was a child but there was no history of rheumatic fever or thrombophlebitis. She had a been a heavy cigarette smoker. Physical examination disclosed moderate cyanosis and clubbing of the fingernails. The lungs were clear. The jugular venous pressure was increased and there was a marked right ventricular impulse. The first heart sound was normal and was followed by an ejection click, the latter initiating a grade 2/S systolic ejection murmur in the pulmonary area. The second heart sound was narrowly split and the pulmonary component was markedly accentuated. The blood pressure was 140190 mm Hg, all peripheral arterial pulsations were within normal limits and there was no evidence of thrombophlebitis. Chest roentgenogram and fluoroscopy revealed that the heart was slightly enlarged with right ventricular prominence. The main left pulmonary artery was greatly enlarged but the right pulmonary artery could not be identified. The pulmonary vascular pattern was increased on the left and markedly decreased on the right (Fig. 1). The hemoglobin was 15.2 g and hematocrit 45 percent; the systemic arterial oxygen tension was 48 mm Hg and the oxygen saturation 89 percent. The electrocardiogram disclosed sinus rhythm, incomplete right bundle branch block and right ventricular and right atrial hypertrophy. The patient was referred to the Peter Bent Brigham Hospital where findings on physical examination were unchanged. A xenon-133 ventilation-perfusion lung scan (Fig. 2A) disclosed severely reduced perfusion of the right lung with normal perfusion of the left lung.
August 1976
The American Journal of CARDIOLOGY
Volume 38
257
PULMONARY ARTERIAL STENOSIS-COHN
ET AL
Right heart catheterization and pulmonary arterial angiography were performed. The main and left pulmonary arterial pressures were 160/60 mm Hg (mean 125), the right pulmonary artery could not be entered, the right ventricular pressure was 160/S and the right atrial pressure 6 mm Hg. The pulmonary arterial angiogram demonstrated a 95 percent stenosis of the right branch at its takeoff from the main pulmonary artery (Fig. 3A). Flow through the small right pulmonary artery to the right lung was severely reduced, but an appropriate number of peripheral pulmonary vessels in the right lung parenchyma was seen. Surgical treatment: Operation was advised to decompress the severe left-sided pulmonary arterial hypertension and reestablish flow to the right-sided pulmonary circulation. Operative findings were massively enlarged main and left pulmonary arteries, severe right ventricular hypertrophy and a soft, thin-walled but normal-sized right pulmonary artery
without intraluminal masses or thrills. After institution of total cardiopulmonary bypass, the aorta was retracted medially and the superior vena cava laterally to facilitate complete dissection of the right pulmonary artery beyond the first lobar branch. Before bypass, pressure in the distal right pulmonary artery was 20/15 mm Hg (mean 18) (Table I). Blood flow in the right pulmonary artery appeared to be entirely retrograde from the bronchial circulation. A main pulmonary artery-right pulmonary artery bypass was then accomplished with a 10 mm woven Dacron@ graft. The distal end to side anastomosis to the right pulmonary artery was performed first using running 5-O Prolene@; the graft was brought anterior to the aorta and anastomosed end to side to the dilated main pulmonary artery. Biopsy of the main pulmonary arterial wall demonstrated marked thickening and atherosclerotic changes in the intima and media. The patient was weaned off cardiopulmonary bypass without difficulty. Pressure measurements (Table I) off cardiopulmonary bypass showed an immediate decrease in the main pulmonary arterial pressure and a 10 mm Hg pressure stepdown from the main to the right pulmonary artery. Right and left lung biopsies were performed after the heparin was neutralized; the left lung showed hypertensive pulmonary
TABLE
I
Pulmonary and Systemic Arterial Pressure Measurements (mm Hg) Before and After Main to Right Pulmonary Artery Bypass Graft
Brachial Artery Preoperative Before cardiopulmonary bypass 1 hour after cardiopulmonary bypass 5 days postoperatively 8 months postoperatively
FIGURE 1. Posteroanterior chest roentgenogram. Note the oligemia of the right lung.
Figures in parentheses
160/130 (140) 120/80 (SO)
Main Pulmonary Artery
Right Pulmonary Artery
160/60 120150
(90) (70)
2Oliji18)
11 O/80 (75)
60/40
(55)
50/35
120170 130/70
60/35 50/20
(40) (35)
(80) (85)
(40)
indicate mean pressures.
FIGURE 2. Xenon-133 ventilation-perfusion posterior lung scans. A, preoperative. There is only faint opacification of the right lung. 6, postoperative. There is equal opacification of the right and left lungs.
250
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vascular disease whereas the right pulmonary arterioles were normal (Fig. 4). Postoperative course: Endotracheal intubation was maintained until the 3rd postoperative day because of a persistent reduction in arterial saturation that gradually increased with positive pressure respiration and diuretic therapy. Pressure in the main pulmonary artery on the fifth postoperative day was 50 percent of the simultaneously measured systemic pressure (Table I). The patient was discharged on her 14th postoperative day and did well in the follow-up period of 8 months. She noted relief of cyanosis and considerably more energy so that she was now in functional class I. A postoperative ventilation-perfusion scan showed equal perfusion of both lungs (Fig. 2B). Recatheterization 8 months postoperatively revealed that pressure was 50/2 mm Hg (mean 35) in the main pulmonary artery, 50/4 in the right ventricle and 4 (mean) in the right atrium; systemic pressure was 130/70 mm Hg (mean 85). Angiography demonstrated the patent prosthetic bypass graft from the main to the right pulmonary artery with excellent runoff in the right pulmonary vasculature (Fig. 3B).
Discussion Congenital stenosis of the peripheral branches of the pulmonary arterial tree has generally been associated with intracardiac shunts, the tetralogy of Fallot, and other congenital malformations.2-s Less common is the syndrome of multiple peripheral pulmonary arterial stenosis9 and only rarely described is stenosis of the right or left main pulmonary artery without associated abnormalities.7 Peripheral pulmonary arterial stenosis was first described pathologically by OppenheimerlO in 1938 and clinically by Moellerll in 1953 after angiography became available. This is an unusual condition; Delaney and Nadas12 found only 4 patients with this diagnosisin 3,250 patients undergoing right heart catheterization, whereas Weinberg et a1.4 found only 39 in 2,000 such patients. Incidence of contralateral pulmonary hypertension: In our patient, the contralateral (left) lung
FIGURE 3. Pulmonary arterial angiograms. A, preoperative. There is massive dilatation of the main (MPA) and left (LPA) pulmonary arteries and 95 percent stenosis at the origin of the right pulmonary artery (RPA). B, postoperative. There is a patent graft from the main to the distal ri@-rtpulrnonqry artery with perfusion of the right lung.
FIGURE 4. Photomicrographs of lung biopsy specimen. A, right lung, showing a normal pulmonary arteriole. B, left lung, showing medial hypertrophy of a pulmonary arteriole. (HematoxylinIeosin X 400 [A] and X 100 [B], reduced by 49 percent.)
August 1976
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Volume 38
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PULMONARY ARTERIAL STENOSIS-COHN ET AL.
received virtually the entire right ventricular output, resulting in pulmonary vascular obstructive disease of the left lung and severe hypertension in the main and left pulmonary arteries. This is an unusual finding since patients after pneumonectomy usually tolerate the total right ventricular output into one lung. Experimental and clinical observations by Vogel et al.ls showed that ligation of the right pulmonary artery in calves at sea level or at 5,200 feet (1,560 meters) above sea level led to severe pulmonary hypertensive changes in the left lung, but the reverse did not occur when the left pulmonary artery was ligated. l2 Observations in patients with agenesis of a major pulmonary artery are consistent with these findings. Severe pulmonary hypertension in the left lung and death occur within the 1st year of life in one third of patients who have no right pulmonary artery,14 but absence of the left pulmonary artery does not seem to result in pulmonary hypertension in the right pulmonary artery.14 The reported experimental and clinical observations and the clinical data in our patient probably relate to the larger volume of the right lung (55 percent) versus the left lung (45 percent)15 so that the left pulmonary vascular volume has less capacity. Hartmann et a1.16 found no significant increase in contralateral pulmonary arterial pressure in children with peripheral pulmonary stenosis followed up for 4 to 7 years; however, no child in their study was more than 12 years of age and the follow-up period was relatively short. Hartmann et al. suggested that since the condition appeared to be benign, surgical treatment was probably not indicated. The data in our patient, who presented at age 48 years, and the experimental and clinical observations of Pool et a1.14 suggest otherwise. The severe vascular obstructive changes in our patient were similar to the changes seen in patients with longstanding atria1 septal defect.17
Surgical treatment: The surgical treatment of peripheral pulmonary arterial stenosis depends on the site and number of stenoses. Stenosis may occur at the supravalvular level, at the pulmonary arterial bifurcation in either the main left or main right pulmonary artery, as in our patient, or in multiple peripheral branches.7 Single branch stenosis has been treated in patients with tetralogy of Fallot by dilatation6 or caval-pulmonary anastomosis8 and multiple peripheral stenosis by saphenous vein patch angioplasties.8,g One previous report1 describes an adult patient with right pulmonary arterial stenosis and agenesis of the left pulmonary artery treated with a Teflon@ bypass graft from the main pulmonary artery to the distal right pulmonary artery. The technique used was similar to that used in our patient. This patient later had pulmonary hypertension in the right lung. A synthetic graft bypass for this central obstruction offers an advantage over angioplasty, but angioplasty is the most appropriate technique for correction of multiple peripheral stenoses. This use of main pulmonary ,artery to right pulmonary artery bypass to relieve severe pulmonary hypertension in a patient with unilateral major pulmonary arterial stenosis and contralateral pulmonary arterial hypertension is apparently unique. Whether pulmonary hypertension will appear in the reperfused lung is uncertain although the maintenance of the low although not normal level of pressure in the main pulmonary artery, documented by cardiac catheterization studies 8 months postoperatively, and the excellent runoff in the right lung are encouraging,
Acknowledgment We thank George Daoud, MD, of the Good Samaritan Hospital, Cincinnati, Ohio and Eugene Braunwald, MD, of the Peter Bent Brigham Hospital for their work-up and referral of this patient and Richard Van Praagh, MD, for reviewing the manuscript.
References 1.
2.
3.
4.
5.
6.
7. 8. 9.
260
BaxterCF, BoothRW,Sirak HD:Surgical correction of congenital stenosis of the right pulmonary artery accompanied by agenesis of the left pulmonary artery. J Thorac Cardiovasc Surg 41:796-60 1, 1961 D’Cruz IA, Augustsson MH, Bicoff JP, et al: Stenotic lesions of the pulmonary arteries: clinical and hemodynamic findings in 64 cases. Am J Cardiol 13:441-450, 1964 Sanger PW, Taylor FH, Robicsek F, et al: Stenosis of the origin of the right pulmonary artery. J Thorac Cardiovasc Surg 46: 124-131, 1964 Weinberg M Jr, Agustsson MH, D’Cruz IA, et al: Stenosis of the branches of the pulmonary artery. J Thorac Cardiovasc Surg 47: 40-49, 1964 Gregoratos G, Jones RC, Jahnke EJ Jr: Unilateral peripheral pulmonic stenosis complicating tetralogy of Fallot. J Thorac Cardiovasc Surg 50:202-209. 1965 Roblcsek F, Sanger PW, Taylor FH, et al: Peripheral stricture of the pulmonary artery treated by cava-pulmonary anastomosis. Ann Surg 160:1066-1066, 1964 Edwards JE, Carey LS, Neufeld HW, et al: Congenital Heart Disease, Vol 2. Philadelphia, WB Saunders, 1965, p 673 &Goon DC, Kincald OW: Stenosis of branches of the pulmonary artery: surgical repair. Med Clin North Am 48:1083-1088, 1964 Smith GW, Thompson WM Jr, Muller WH Jr: Surgical treatment of pulmonary hypertension secondary to multiple bilateral pulmonary arterial stenosis. Circulation 29:Suppl IV:IV-152-IV-156,
August 1976
The American Journal of CARDIOLDGY
1964 10. Oppenheimer EH: Partial atresia of the main branches of the pulmonary artery occurring in infancy and accompanied by calcification of the pulmonary artery and aorta. Bull Johns Hopkins Hosp 63:261-276. 1938 11. Moeller T: A case of peripheral pulmonary stenosis. Acta Pediatr 42:390.1953 12. Delaney TB, Nadas AS: Peripheral pulmonic stenosis. Am J Cardiol 13:451-461, 1964 13 Vogel JHK, McNamara DG, Hallman G, et al: Effects of mild chronic hypoxia on the pulmonary circulation in calves with reactive pulmonary hypertension. Circ Res 21:661-669, 1967 14. Pool PE, Vogel JHK, Blount BG Jr: Congenital unilateral absence of a pulmonary artery: the importance of flow in pulmonary hypertension Am J Cardiol 10:706-732, 1962 15. Ma&son WH, Caldwell DR, Early NA, et al: The relationship of dry lung weight to pulmonary function in dogs and humans. J Surg Res 2:287-291, 1962 16. Hartmann AF Jr, Elliott LP, GolUrIng D: The course of peripheral pulmonary artery stenoses in children. J Pediitr 73:212-216, 1968 17. Cohn LH, Morrow AG, Braunwald E: Operative treatment of atrial septal defect: clinical and hemodynamic assessments in 175 patients. Br Heart J 29:725-734. 1967 18. Shumacker HB Jr, Lurle PR: Pulmonary valvotomy. J Thorac Surg 25:173-186, 1953
Volume 36
LAWRENCE H. COHN, MD, FACC JOHN H. SANDERS, Jr., MD JOHN
J. COLLINS,
Jr.,
MD,
FACC
Boston, Massachusetts
This report describes the dlagnosis and surgical treatment of a 48 year old patient with congenital right pulmonary arterial ostial stenosis, no intraor extracardiac shunts and systemic levels of pressure in the main and left pulmonary artery with pulmonary vascular obstruction in the left lung. Operation consisted of a bypass graft of 10 mm woven Dacron from the main pulmonary artery to the distal right pulmonary artery producing an immediate 50 percent reduction in mean left pulmonary arterial pressure. Late postoperative evaluation revealed equal perfusion of both lungs, maintenance of the reduced pulmonary arterial pressure and improvement from New York Heart Association functlonal class IV to class I.
Congenital stenosis of the origin of one or both principal branches of the pulmonary arterial system has been reported only infrequently and is usually accompanied by other malformations of the heart and great vessels.l-6 The patient to be described demonstrated congenital stenosis of the origin of the right pulmonary artery with severe pulmonary hypertension and pulmonary vascular obstruction in the left lung and underwent successful surgical treatment of this unusual lesion. Case Report
From the Department of Surgery, Harvard Medical School, and Division of Thoracic and Cardiac Surgery, Peter Bent Brigham Hospital, Boston, Mass. Manuscript received May 5, 1975; revised manuscript received July 24. 1975, accepted July 29, 1975. Address for reprints: Lawrence H. Cohn, MD, Division of Thoracic and Cardiac Surgery, Peter Bent Brigham Hospital, 721 Huntington Ave., Boston, Mass. 02115.
A 48 year old woman was admitted to her local hospital for severe dyspnea that had progressed in the preceding five years. During the previous year, her condition had deteriorated until she was bedridden and in New York Heart Association functional class IV. Her previous medical history was essentially normal and she had no previous hospitalizations except for childbirth. She had been told she had a heart murmur when she was a child but there was no history of rheumatic fever or thrombophlebitis. She had a been a heavy cigarette smoker. Physical examination disclosed moderate cyanosis and clubbing of the fingernails. The lungs were clear. The jugular venous pressure was increased and there was a marked right ventricular impulse. The first heart sound was normal and was followed by an ejection click, the latter initiating a grade 2/S systolic ejection murmur in the pulmonary area. The second heart sound was narrowly split and the pulmonary component was markedly accentuated. The blood pressure was 140190 mm Hg, all peripheral arterial pulsations were within normal limits and there was no evidence of thrombophlebitis. Chest roentgenogram and fluoroscopy revealed that the heart was slightly enlarged with right ventricular prominence. The main left pulmonary artery was greatly enlarged but the right pulmonary artery could not be identified. The pulmonary vascular pattern was increased on the left and markedly decreased on the right (Fig. 1). The hemoglobin was 15.2 g and hematocrit 45 percent; the systemic arterial oxygen tension was 48 mm Hg and the oxygen saturation 89 percent. The electrocardiogram disclosed sinus rhythm, incomplete right bundle branch block and right ventricular and right atrial hypertrophy. The patient was referred to the Peter Bent Brigham Hospital where findings on physical examination were unchanged. A xenon-133 ventilation-perfusion lung scan (Fig. 2A) disclosed severely reduced perfusion of the right lung with normal perfusion of the left lung.
August 1976
The American Journal of CARDIOLOGY
Volume 38
257
PULMONARY ARTERIAL STENOSIS-COHN
ET AL
Right heart catheterization and pulmonary arterial angiography were performed. The main and left pulmonary arterial pressures were 160/60 mm Hg (mean 125), the right pulmonary artery could not be entered, the right ventricular pressure was 160/S and the right atrial pressure 6 mm Hg. The pulmonary arterial angiogram demonstrated a 95 percent stenosis of the right branch at its takeoff from the main pulmonary artery (Fig. 3A). Flow through the small right pulmonary artery to the right lung was severely reduced, but an appropriate number of peripheral pulmonary vessels in the right lung parenchyma was seen. Surgical treatment: Operation was advised to decompress the severe left-sided pulmonary arterial hypertension and reestablish flow to the right-sided pulmonary circulation. Operative findings were massively enlarged main and left pulmonary arteries, severe right ventricular hypertrophy and a soft, thin-walled but normal-sized right pulmonary artery
without intraluminal masses or thrills. After institution of total cardiopulmonary bypass, the aorta was retracted medially and the superior vena cava laterally to facilitate complete dissection of the right pulmonary artery beyond the first lobar branch. Before bypass, pressure in the distal right pulmonary artery was 20/15 mm Hg (mean 18) (Table I). Blood flow in the right pulmonary artery appeared to be entirely retrograde from the bronchial circulation. A main pulmonary artery-right pulmonary artery bypass was then accomplished with a 10 mm woven Dacron@ graft. The distal end to side anastomosis to the right pulmonary artery was performed first using running 5-O Prolene@; the graft was brought anterior to the aorta and anastomosed end to side to the dilated main pulmonary artery. Biopsy of the main pulmonary arterial wall demonstrated marked thickening and atherosclerotic changes in the intima and media. The patient was weaned off cardiopulmonary bypass without difficulty. Pressure measurements (Table I) off cardiopulmonary bypass showed an immediate decrease in the main pulmonary arterial pressure and a 10 mm Hg pressure stepdown from the main to the right pulmonary artery. Right and left lung biopsies were performed after the heparin was neutralized; the left lung showed hypertensive pulmonary
TABLE
I
Pulmonary and Systemic Arterial Pressure Measurements (mm Hg) Before and After Main to Right Pulmonary Artery Bypass Graft
Brachial Artery Preoperative Before cardiopulmonary bypass 1 hour after cardiopulmonary bypass 5 days postoperatively 8 months postoperatively
FIGURE 1. Posteroanterior chest roentgenogram. Note the oligemia of the right lung.
Figures in parentheses
160/130 (140) 120/80 (SO)
Main Pulmonary Artery
Right Pulmonary Artery
160/60 120150
(90) (70)
2Oliji18)
11 O/80 (75)
60/40
(55)
50/35
120170 130/70
60/35 50/20
(40) (35)
(80) (85)
(40)
indicate mean pressures.
FIGURE 2. Xenon-133 ventilation-perfusion posterior lung scans. A, preoperative. There is only faint opacification of the right lung. 6, postoperative. There is equal opacification of the right and left lungs.
250
August 1976
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PULMONARY ARTERIAL STENOSIS-COHN ET AL.
vascular disease whereas the right pulmonary arterioles were normal (Fig. 4). Postoperative course: Endotracheal intubation was maintained until the 3rd postoperative day because of a persistent reduction in arterial saturation that gradually increased with positive pressure respiration and diuretic therapy. Pressure in the main pulmonary artery on the fifth postoperative day was 50 percent of the simultaneously measured systemic pressure (Table I). The patient was discharged on her 14th postoperative day and did well in the follow-up period of 8 months. She noted relief of cyanosis and considerably more energy so that she was now in functional class I. A postoperative ventilation-perfusion scan showed equal perfusion of both lungs (Fig. 2B). Recatheterization 8 months postoperatively revealed that pressure was 50/2 mm Hg (mean 35) in the main pulmonary artery, 50/4 in the right ventricle and 4 (mean) in the right atrium; systemic pressure was 130/70 mm Hg (mean 85). Angiography demonstrated the patent prosthetic bypass graft from the main to the right pulmonary artery with excellent runoff in the right pulmonary vasculature (Fig. 3B).
Discussion Congenital stenosis of the peripheral branches of the pulmonary arterial tree has generally been associated with intracardiac shunts, the tetralogy of Fallot, and other congenital malformations.2-s Less common is the syndrome of multiple peripheral pulmonary arterial stenosis9 and only rarely described is stenosis of the right or left main pulmonary artery without associated abnormalities.7 Peripheral pulmonary arterial stenosis was first described pathologically by OppenheimerlO in 1938 and clinically by Moellerll in 1953 after angiography became available. This is an unusual condition; Delaney and Nadas12 found only 4 patients with this diagnosisin 3,250 patients undergoing right heart catheterization, whereas Weinberg et a1.4 found only 39 in 2,000 such patients. Incidence of contralateral pulmonary hypertension: In our patient, the contralateral (left) lung
FIGURE 3. Pulmonary arterial angiograms. A, preoperative. There is massive dilatation of the main (MPA) and left (LPA) pulmonary arteries and 95 percent stenosis at the origin of the right pulmonary artery (RPA). B, postoperative. There is a patent graft from the main to the distal ri@-rtpulrnonqry artery with perfusion of the right lung.
FIGURE 4. Photomicrographs of lung biopsy specimen. A, right lung, showing a normal pulmonary arteriole. B, left lung, showing medial hypertrophy of a pulmonary arteriole. (HematoxylinIeosin X 400 [A] and X 100 [B], reduced by 49 percent.)
August 1976
The Arnerlcan Jwrnal of CARDIOLOGY
Volume 38
259
PULMONARY ARTERIAL STENOSIS-COHN ET AL.
received virtually the entire right ventricular output, resulting in pulmonary vascular obstructive disease of the left lung and severe hypertension in the main and left pulmonary arteries. This is an unusual finding since patients after pneumonectomy usually tolerate the total right ventricular output into one lung. Experimental and clinical observations by Vogel et al.ls showed that ligation of the right pulmonary artery in calves at sea level or at 5,200 feet (1,560 meters) above sea level led to severe pulmonary hypertensive changes in the left lung, but the reverse did not occur when the left pulmonary artery was ligated. l2 Observations in patients with agenesis of a major pulmonary artery are consistent with these findings. Severe pulmonary hypertension in the left lung and death occur within the 1st year of life in one third of patients who have no right pulmonary artery,14 but absence of the left pulmonary artery does not seem to result in pulmonary hypertension in the right pulmonary artery.14 The reported experimental and clinical observations and the clinical data in our patient probably relate to the larger volume of the right lung (55 percent) versus the left lung (45 percent)15 so that the left pulmonary vascular volume has less capacity. Hartmann et a1.16 found no significant increase in contralateral pulmonary arterial pressure in children with peripheral pulmonary stenosis followed up for 4 to 7 years; however, no child in their study was more than 12 years of age and the follow-up period was relatively short. Hartmann et al. suggested that since the condition appeared to be benign, surgical treatment was probably not indicated. The data in our patient, who presented at age 48 years, and the experimental and clinical observations of Pool et a1.14 suggest otherwise. The severe vascular obstructive changes in our patient were similar to the changes seen in patients with longstanding atria1 septal defect.17
Surgical treatment: The surgical treatment of peripheral pulmonary arterial stenosis depends on the site and number of stenoses. Stenosis may occur at the supravalvular level, at the pulmonary arterial bifurcation in either the main left or main right pulmonary artery, as in our patient, or in multiple peripheral branches.7 Single branch stenosis has been treated in patients with tetralogy of Fallot by dilatation6 or caval-pulmonary anastomosis8 and multiple peripheral stenosis by saphenous vein patch angioplasties.8,g One previous report1 describes an adult patient with right pulmonary arterial stenosis and agenesis of the left pulmonary artery treated with a Teflon@ bypass graft from the main pulmonary artery to the distal right pulmonary artery. The technique used was similar to that used in our patient. This patient later had pulmonary hypertension in the right lung. A synthetic graft bypass for this central obstruction offers an advantage over angioplasty, but angioplasty is the most appropriate technique for correction of multiple peripheral stenoses. This use of main pulmonary ,artery to right pulmonary artery bypass to relieve severe pulmonary hypertension in a patient with unilateral major pulmonary arterial stenosis and contralateral pulmonary arterial hypertension is apparently unique. Whether pulmonary hypertension will appear in the reperfused lung is uncertain although the maintenance of the low although not normal level of pressure in the main pulmonary artery, documented by cardiac catheterization studies 8 months postoperatively, and the excellent runoff in the right lung are encouraging,
Acknowledgment We thank George Daoud, MD, of the Good Samaritan Hospital, Cincinnati, Ohio and Eugene Braunwald, MD, of the Peter Bent Brigham Hospital for their work-up and referral of this patient and Richard Van Praagh, MD, for reviewing the manuscript.
References 1.
2.
3.
4.
5.
6.
7. 8. 9.
260
BaxterCF, BoothRW,Sirak HD:Surgical correction of congenital stenosis of the right pulmonary artery accompanied by agenesis of the left pulmonary artery. J Thorac Cardiovasc Surg 41:796-60 1, 1961 D’Cruz IA, Augustsson MH, Bicoff JP, et al: Stenotic lesions of the pulmonary arteries: clinical and hemodynamic findings in 64 cases. Am J Cardiol 13:441-450, 1964 Sanger PW, Taylor FH, Robicsek F, et al: Stenosis of the origin of the right pulmonary artery. J Thorac Cardiovasc Surg 46: 124-131, 1964 Weinberg M Jr, Agustsson MH, D’Cruz IA, et al: Stenosis of the branches of the pulmonary artery. J Thorac Cardiovasc Surg 47: 40-49, 1964 Gregoratos G, Jones RC, Jahnke EJ Jr: Unilateral peripheral pulmonic stenosis complicating tetralogy of Fallot. J Thorac Cardiovasc Surg 50:202-209. 1965 Roblcsek F, Sanger PW, Taylor FH, et al: Peripheral stricture of the pulmonary artery treated by cava-pulmonary anastomosis. Ann Surg 160:1066-1066, 1964 Edwards JE, Carey LS, Neufeld HW, et al: Congenital Heart Disease, Vol 2. Philadelphia, WB Saunders, 1965, p 673 &Goon DC, Kincald OW: Stenosis of branches of the pulmonary artery: surgical repair. Med Clin North Am 48:1083-1088, 1964 Smith GW, Thompson WM Jr, Muller WH Jr: Surgical treatment of pulmonary hypertension secondary to multiple bilateral pulmonary arterial stenosis. Circulation 29:Suppl IV:IV-152-IV-156,
August 1976
The American Journal of CARDIOLDGY
1964 10. Oppenheimer EH: Partial atresia of the main branches of the pulmonary artery occurring in infancy and accompanied by calcification of the pulmonary artery and aorta. Bull Johns Hopkins Hosp 63:261-276. 1938 11. Moeller T: A case of peripheral pulmonary stenosis. Acta Pediatr 42:390.1953 12. Delaney TB, Nadas AS: Peripheral pulmonic stenosis. Am J Cardiol 13:451-461, 1964 13 Vogel JHK, McNamara DG, Hallman G, et al: Effects of mild chronic hypoxia on the pulmonary circulation in calves with reactive pulmonary hypertension. Circ Res 21:661-669, 1967 14. Pool PE, Vogel JHK, Blount BG Jr: Congenital unilateral absence of a pulmonary artery: the importance of flow in pulmonary hypertension Am J Cardiol 10:706-732, 1962 15. Ma&son WH, Caldwell DR, Early NA, et al: The relationship of dry lung weight to pulmonary function in dogs and humans. J Surg Res 2:287-291, 1962 16. Hartmann AF Jr, Elliott LP, GolUrIng D: The course of peripheral pulmonary artery stenoses in children. J Pediitr 73:212-216, 1968 17. Cohn LH, Morrow AG, Braunwald E: Operative treatment of atrial septal defect: clinical and hemodynamic assessments in 175 patients. Br Heart J 29:725-734. 1967 18. Shumacker HB Jr, Lurle PR: Pulmonary valvotomy. J Thorac Surg 25:173-186, 1953
Volume 36
