molding of these structures might then lessen the obstruction and lead to disappearance of the left-to-right shunt. This report, along with others in the literature, illustrates that a secundum ASD, with a significant amount of shunting and congestive heart failure in infancy, can close spontaneously. We believe that intensive medical measures to control failure should be employed in such a baby, and surgery should be delayed in order to allow for possible spontaneous decrease in size or closure.
1 Dimich F, Steinfeld F, Park SC: Symptomatic atrial septal defect in infants. Am Heart J 85:601-604, 1973 2 Toews WH, Nora JJ, Wolfe RR: Presentation of atrial septal defect in infancy. JAMA 234:1250-1251, 1975 3 Cayler GG : Spontaneous functional closure of symptomatic atrial septal defects. N Engl J Med 276:65-73, 1967 4 Hoffman JF, Rudolph AM, Danilowicz D: Left-to-right atrial shunts in infants. Am J Cardiol 30:868-875, 1972 5 Mody MR: Serial hemodynamic observations in secundum atrial septal defect with special reference to spontaneous closure. Am J Cardiol32:978-981, 1973 6 Menon VA, Wagner HR: Spontaneous closure of secundum atrial septal defect. NY State J Med, 1068-1077, 1975 7 Timmis GC, Gordon S, Reed JO: Spontaneous closure of an atrial septal defect, J AMA 196: 137-139, 1966 8 Hoffman JF, Danilowicz D, Rudolph AM: Hemodynamics, clinical features and course of atrial shunts in infancy. Circulation 31 & 32: 11-113, 1965 9 Watson GH, Dark JF: The spontaneous disappearance of interatrial shunts in infancy. Proc Roy Soc Med 61:30001,1968 10 Edwards JE : Congenital malformation of the heart and great vessels. In Gould SE ( ed) : Pathology of the Heart. Springfield, lll, Charles C Thomas Publishers, 1953, p
~e
congenital malformation of •aortopulmonary win-
.I dow" is well known.1 It consists of an opening
between the aorta and the pulmonary artery so that a shunt between these two vessels develops. It is common belief that the opening has no length; Blieden et al, 2 for instance, in a study of 17 patients, made a statement to that effect. Occasionally, however, this communication may develop in such a way that it forms what can almost be considered a separate vessel. This variant does not appear to be well known. We would, therefore, like to present two such cases.
CASE!
The first patient was a baby boy weighing 1.8 kg ( 3.9 lb) at birth. At five days of age, he became tachypneic ( respirations, 60 per minute) with chest retraction. A heart murmur was heard at the left sternal border. A chest x-ray film at the age of seven days showed moderate cardiomegaly and increased pulmonary vascularity. Cardiac catheterization was performed at 14 days of age, followed by right and left ventricular angiograms which showed the presence of an atrial ( ASD), ventricular septal defect ( VSD), a right aortic arch, anomalous origin of the left subclavian artery from the pulmonary artery, and aortopulmonary communication whose site could not be accurately defined. The pulmonary artery was not entered. Recatheterization a few days later ( Fig 1 ) with pulmonary and aortic injections showed an aortopulmonary communication, and a diagnosis of aortopulmonary "window" was made. Surgery was delayed until the age of ten weeks when the child weighed slightly over 2 kg ( 4.4 lb). At operation, the presence of a large aortopulmonary communication was con-
288 11 Rodbard S: Physical forces and vascular lining. Ann Intern Med 50:1339, 1959
Aortopulmonary Window or Aortopulmonary Communication?* Christian L. Belcourt, M.B., B.S.; 0 ° Kurt Alerman, M.D.,t D. A. Gillis, M.D.;t tmd Dougl& L. Roy, M.D.§
Two patients bad a vessel-like aortopa1moaary "window" wbicb could be lipted witllout die instltudoa of cardiopulmoaary bypass. WbDe embryologlally and faaedoa. ally these commaulcatioiiS are identical to tbe dassic aortopulmoaary "wiadow," it is sageRed that tbe term "aortopolmoaary commaaicatioa" Is more appropriate. °From the Departments of Pathology Cardio~. and Surgery, Izaak Walton Killam HospiW for Children, and Dalhousie University, Halifax. Nova Scotia. Canada. 0 0 Assistant Professor. tProfessor of Pathology. tAssociate Professor of Surgery. §Professor of Pediatrics (Cardiology). Reprint reguem: Dr. Belcourt, De,1artment qf ltDdlolollJI, I%001c Walton Killllm Ho,ittJl, Htdifa, N.S., B3]3G9,
Canada
CHEST, 75: 3, MARCH, 1979
F'wt111B 1. Angiogram-injection in the aortopulmonary communication "stencilled." Note right aortic arch. Origin of left subclavian is in fact from the pulmonary artery (confirmed at surgery and autopsy); it is superimposed over the aorta. 1, Aorta; 2, pulmonary artery; and 3, aberrant left subclavian arising from pulmonary artery.
AORTOPULMONARY WINDOW OR COMMUNICAnON? 397
4
FiGURE 2. Surgery at age ten weeks. Chest has been opened and heart exposed. An umbilical tape has been passed behind the aortopulmonary communication. The ·right atrium has been retracted. 1, aortopulmonary commUnication; 2, aorta; 3, pulmonary artery; 4, right ventricle; and 5 right atrium.
firmed in the form of a vessel 1.5 em long and of approximately the same width as the aorta. It joined the left posterior aspect of the aorta to the anterior aspect of the pulmonary artery (Fig 2). It was possible to place a tape around this vessel, and there was more length to it than had been anticipated. It extended transversely from the aorta to the pulmonary artery at a point situated about 1 em distal to the aortic valve. While surgical occlusion of this vessel might have been possible without the aid of cardiopulmonary bypass, the latter was obviously going to be required for management of the intracardiac abnormality. A vascular clamp could be placed across the communication prior to the institution of cardiopulmonary bypass. The communication was then divided close to the pulmonary artery, and the defect on the latter was closed with a fine running suture. The aortic side was similarly closed, and the previously placed clamp was then removed. A small atrial septal defect was closed by direct suture. Through a right ventriculotomy, a subcristal ventricular septal defect was closed by a Dacron patch after discontinuation of the bypass. At the time of sternal closure, however, the patient's cardiac output diminished and her condition from that time onwards was poor and resistant to treatment. The infant died a few hours following the procedure from continued low cardiac output Necropsy confirmed the anomalous origin of the right subclavian artery and the presence of a right-sided aortic arch. The closure of the aortic pulmonary window and the repaired ventricular septal defect were considered satisfactory.
CASE2 The second patient was a boy first seen at the age of five weeks when he weighed 3.6 kg (7.9 lb). For the previous
398 BELCOURT ET Al
two weeks, he had been taking digitalis for cardiac failure that had developed soon after birth. On admission, he had a continuous murmur in the left precordial region and a systolic thrill was felt in the left fourth anterior intercostal space. His facial features were suggestive of the rubella syndrome. The ECG showed biventricular hypertrophy with right ventricular dominance. The pulse rate was 150 beats per minute, respirations 48 per minute, and the blood pressure was 80 mm Hg, systolic. The liver was felt to be 3 to 4 em below the costal margin. He was believed to have a patent ductus arteriosus ( PDA) which was surgically ligated without complication at the age of six weeks. A loud murmur persisted, however, after operation, and there was clinical evidence of a persistent significant left-to-right shunt. Chest x-ray films showed the persistence of cardiomegaly and increased pulmonary vascularity. Cardiac catheterization and angiocardiography indicated the presence of a large shunt from the proximal aorta to the proximal main pulmonary artery. A second surgical procedure was therefore carried out two weeks following ligation of the patent ductus through a midsternal incision, with preparation for cardiopulmonary bypass should this be required. The right ventricle and the pulmonary artery were found to be large, and a coarse thrill was palpable over the proximal part of the main stem of the pulmonary artery. Further dissection confirmed the presence of a large vessel connecting the proximal aorta to the proximal main stem of the pulmonary artery (Fig 3). This "vessel" appeared to originate from the posterior surface of the proximal aorta from where it curved upwards and to the left to connect to the posterior medial aspect of the pulmonary arterial stem. The vessel was approximately 1.5 em long and had a diameter of 3 to 4 mm. It was easily isolated and doubly ligated. A bypass was not required. Ligature of the vessel abolished the thrill. The operation was uncomplicated and recovery was uneventful. The child has remained well. DISCUSSION
The main purpose of this presentation is to draw attention to the existence of a variant of the "aortopulmonary window" which does not appear to be widely known. Takao et al8 have classified aortopulmonary
FiGURE 3. At surgery, looking at exposed heart, right atrium has been retracted. An umbilical tape has been passed behind the long aortopulmonary communication. 1, Aortopulmonary communication; 2, aorta; 3, pulmonary artery; 4, right ventricle; and 5, right atrium.
CHEST, 75: 3, MARCH, 1979
windows into three types. Type 1 is proximal "typical." Type 2 is distal. Type 3 is a combination of types 1 and .2. The two cases of aortopulmonary COIIlDlunication presented here were of type 1 of the classification of Takao et al, 8 but in both instances, the communication had acquired the morphologic configuration almost of an independent vessel. The term "window" would hardly be justified or appropriate for this variant. R. Van Praagh, M.D., stated that the aortopulmonary window "does indeed appear to have-does have-some length" (written communication, December, 1977). This is a .point which should be emphasized, and which does raise the question whether the term "window" should not be replaced by the term "communication." There can, however, be little doubt that the vessel-like anomaly described here is merely a variant of the better known aortopulmonary "window" whose basis is a defect in the development of the aortopulmonary septum. 4 This anomaly is frequently (35 percent) associated with other congenital malformations of the heart, G including VSD (7.7 percent), right aortic arch (6.8 percent), anomalous origin of the left coronary artery from the pulmonary artery (5.1 percent), and PDA (11 percent). One of the two patients presented here had a PDA, and the other one had a right aortic arch ASD and VSD. The associated cardiac anomalies may make the diagnosis difiicult, G,e and indeed, our first patient who had a coexistent PDA was not suspected of the presence of an aortopulmonary communication until after the ligation of the patent ductus had demonstrated the persistence of a significant shunt. The preferred surgical approach appears to be via the aorta. There does not appear to be any reason why, in suitable cases, the vessel could not be isolated and ligated without the institution of cardiopulmonary bypass as in the two cases presented here. Clearly, the correct approach will be influenced by the underlying pathologic changes and will have to be decided upon on an individual basis.
REFERENCES 1 Neufeld HN, Lester RG, Adams P Jr, et al: Aorticopulmonary septal defect. Am J Cardiol 9: 12-25, 1962 2 Blieden LC, Moller JH: Aorticopulmonary septal defect: An experience with 17 patients. Br Heart J 36:630-635, 1974 3 Takao A, Mori K, Konno S, et al: Anatomical classification of aorticopulmonary window. In: Proceedings of the World Congress of Cardiology (abstract 396), Buenos Aires, Advances in Congenital Heart Disease, 1974 4 Edward JE: Congenital malformation of the heart and great vessels. In Gould SE: Pathology of the Heart and Blood Vessels, 3rd ed. Springfield, m, Charles C Thomas Publisher, 1968, pp 411-412 5 Faullcner SL, Oldham RR, Atwood GF, et al: Aortopulmonary window, ventricular septal defect, and membranous pulmonary atresia with a diagnosis of truncus arteriosus. Chest 65:351-353, 1974 6 Fisher EA, DuBrow IW, Eckner FAO, et al: Aorticopulmonary septal defect and interrupted aortic arch: A diagnostic challenge. Am J Cardiol34:356-359, 1974
CHEST, 75: 3, MARCH, 1979
Calcific Pulmonic Stenosis in Adulthood* Treatment by Valve Replacement (Porcine Xenograft) With Postoperative Hemodynamic Evaluation Edgar A. CovarrubiDs, M.D.; Mahar U. Sheikh, M.D.; Jeffrey M. Isner, M.D.; Marlo Gomes, M.D., F.C.C.P.; Charles A. Hufnagel, M.D., F.C.C.P.; and William C. Roberts, M.D., F.C.C.P•
COnical and morphologic: features are described iD a 56year-old man 'in whom severe, isolated pulmonic: valve stenosis was Created by valve replacement with a porc:ioe prosthesis. The c:alc:ific: deposits were located on the ventricular aspect of the pnlmonle valve, opposite the location (arterial aspect) of c:alc:ific: deposits on stenotfe aortic: valves, and c:alc:ific: deposibl also were present in the tricuspid valve anulus.
J solated pulmonic valve stenosis of severe degree in Calcific persons over 50 years of age is rare. 1 -4
deposits in stenotic pulmonic valves also are rare, 24 and calcific deposits in the tricuspid valve anulus are even more rare. G,o Treatment of isolated pulmonic valve stenosis by valve replacement has not been reported. Although each of the above occurrences is rare, all of them occurred together in our patient. In addition, the patient had postoperative hemodynamic evaluation after pulmonic valve replacement with a porcine xenograft. CASE REPoRT
A 56-year-old man was easily fatigued during his teens and was denied military service because of a "heart murmur." He was asymptomatic until age 46 years when exertional dyspnea occurred, and he was digitalized. At age 52, atrial fibrillation was noted, and cardioversion was done. During the next four years, the exertional dyspnea gradually progressed so that by age 56, he had considerable dyspnea when walking three flat blocks at a normal pace or climbing one flight of stairs. Examination in November 1977 (age 56) disclosed a prominent "a" wave in the jugular veins, no precordial thrills, a decreased pulmonic component of the second heart sound at the base, a grade 3/6 harsh, systolic precordial murmur loudest over the upper left sternal border, and no organomegaly or subcutaneous edema. Electrocardiogram showed sinus rhythm and right bundle branch bloclc. Chest roentgenogram (Fig 1 ) disclosed cardiomegaly and heavy calcific deposits in the pulmonic valve. The catheterization data are summarized in Table 1. At operation on Feb 2, 1978, palpation via the right atrium disclosed calcific deposits in the tricuspid valve anular region. The pulmonary trunk was dilated, and a thrill was palpated. °From the Georgetown Cardiology Service, District of Columbia Hospital, and the Deparbnents of Surge!'Y1 Medicine, and Pathology, Georgetown University Hospital, Washington, DC; and the Pathology Branch, Nationiil Heart, Lung, and Blood Institute. NatioDallnstitutes of Hea1th, Bethesda. Reprint te~f.!68fs: Dr. Robem, Chief, Patholo_sy_Branch. 1;¥HLBI1 Btag lOA, Boom 3E30, 9000 Rockmlle PUce, Betheaaa 20014
CALCIFIC PULMONIC STENOSIS IN ADULTHOOD 399
1 Dimich F, Steinfeld F, Park SC: Symptomatic atrial septal defect in infants. Am Heart J 85:601-604, 1973 2 Toews WH, Nora JJ, Wolfe RR: Presentation of atrial septal defect in infancy. JAMA 234:1250-1251, 1975 3 Cayler GG : Spontaneous functional closure of symptomatic atrial septal defects. N Engl J Med 276:65-73, 1967 4 Hoffman JF, Rudolph AM, Danilowicz D: Left-to-right atrial shunts in infants. Am J Cardiol 30:868-875, 1972 5 Mody MR: Serial hemodynamic observations in secundum atrial septal defect with special reference to spontaneous closure. Am J Cardiol32:978-981, 1973 6 Menon VA, Wagner HR: Spontaneous closure of secundum atrial septal defect. NY State J Med, 1068-1077, 1975 7 Timmis GC, Gordon S, Reed JO: Spontaneous closure of an atrial septal defect, J AMA 196: 137-139, 1966 8 Hoffman JF, Danilowicz D, Rudolph AM: Hemodynamics, clinical features and course of atrial shunts in infancy. Circulation 31 & 32: 11-113, 1965 9 Watson GH, Dark JF: The spontaneous disappearance of interatrial shunts in infancy. Proc Roy Soc Med 61:30001,1968 10 Edwards JE : Congenital malformation of the heart and great vessels. In Gould SE ( ed) : Pathology of the Heart. Springfield, lll, Charles C Thomas Publishers, 1953, p
~e
congenital malformation of •aortopulmonary win-
.I dow" is well known.1 It consists of an opening
between the aorta and the pulmonary artery so that a shunt between these two vessels develops. It is common belief that the opening has no length; Blieden et al, 2 for instance, in a study of 17 patients, made a statement to that effect. Occasionally, however, this communication may develop in such a way that it forms what can almost be considered a separate vessel. This variant does not appear to be well known. We would, therefore, like to present two such cases.
CASE!
The first patient was a baby boy weighing 1.8 kg ( 3.9 lb) at birth. At five days of age, he became tachypneic ( respirations, 60 per minute) with chest retraction. A heart murmur was heard at the left sternal border. A chest x-ray film at the age of seven days showed moderate cardiomegaly and increased pulmonary vascularity. Cardiac catheterization was performed at 14 days of age, followed by right and left ventricular angiograms which showed the presence of an atrial ( ASD), ventricular septal defect ( VSD), a right aortic arch, anomalous origin of the left subclavian artery from the pulmonary artery, and aortopulmonary communication whose site could not be accurately defined. The pulmonary artery was not entered. Recatheterization a few days later ( Fig 1 ) with pulmonary and aortic injections showed an aortopulmonary communication, and a diagnosis of aortopulmonary "window" was made. Surgery was delayed until the age of ten weeks when the child weighed slightly over 2 kg ( 4.4 lb). At operation, the presence of a large aortopulmonary communication was con-
288 11 Rodbard S: Physical forces and vascular lining. Ann Intern Med 50:1339, 1959
Aortopulmonary Window or Aortopulmonary Communication?* Christian L. Belcourt, M.B., B.S.; 0 ° Kurt Alerman, M.D.,t D. A. Gillis, M.D.;t tmd Dougl& L. Roy, M.D.§
Two patients bad a vessel-like aortopa1moaary "window" wbicb could be lipted witllout die instltudoa of cardiopulmoaary bypass. WbDe embryologlally and faaedoa. ally these commaulcatioiiS are identical to tbe dassic aortopulmoaary "wiadow," it is sageRed that tbe term "aortopolmoaary commaaicatioa" Is more appropriate. °From the Departments of Pathology Cardio~. and Surgery, Izaak Walton Killam HospiW for Children, and Dalhousie University, Halifax. Nova Scotia. Canada. 0 0 Assistant Professor. tProfessor of Pathology. tAssociate Professor of Surgery. §Professor of Pediatrics (Cardiology). Reprint reguem: Dr. Belcourt, De,1artment qf ltDdlolollJI, I%001c Walton Killllm Ho,ittJl, Htdifa, N.S., B3]3G9,
Canada
CHEST, 75: 3, MARCH, 1979
F'wt111B 1. Angiogram-injection in the aortopulmonary communication "stencilled." Note right aortic arch. Origin of left subclavian is in fact from the pulmonary artery (confirmed at surgery and autopsy); it is superimposed over the aorta. 1, Aorta; 2, pulmonary artery; and 3, aberrant left subclavian arising from pulmonary artery.
AORTOPULMONARY WINDOW OR COMMUNICAnON? 397
4
FiGURE 2. Surgery at age ten weeks. Chest has been opened and heart exposed. An umbilical tape has been passed behind the aortopulmonary communication. The ·right atrium has been retracted. 1, aortopulmonary commUnication; 2, aorta; 3, pulmonary artery; 4, right ventricle; and 5 right atrium.
firmed in the form of a vessel 1.5 em long and of approximately the same width as the aorta. It joined the left posterior aspect of the aorta to the anterior aspect of the pulmonary artery (Fig 2). It was possible to place a tape around this vessel, and there was more length to it than had been anticipated. It extended transversely from the aorta to the pulmonary artery at a point situated about 1 em distal to the aortic valve. While surgical occlusion of this vessel might have been possible without the aid of cardiopulmonary bypass, the latter was obviously going to be required for management of the intracardiac abnormality. A vascular clamp could be placed across the communication prior to the institution of cardiopulmonary bypass. The communication was then divided close to the pulmonary artery, and the defect on the latter was closed with a fine running suture. The aortic side was similarly closed, and the previously placed clamp was then removed. A small atrial septal defect was closed by direct suture. Through a right ventriculotomy, a subcristal ventricular septal defect was closed by a Dacron patch after discontinuation of the bypass. At the time of sternal closure, however, the patient's cardiac output diminished and her condition from that time onwards was poor and resistant to treatment. The infant died a few hours following the procedure from continued low cardiac output Necropsy confirmed the anomalous origin of the right subclavian artery and the presence of a right-sided aortic arch. The closure of the aortic pulmonary window and the repaired ventricular septal defect were considered satisfactory.
CASE2 The second patient was a boy first seen at the age of five weeks when he weighed 3.6 kg (7.9 lb). For the previous
398 BELCOURT ET Al
two weeks, he had been taking digitalis for cardiac failure that had developed soon after birth. On admission, he had a continuous murmur in the left precordial region and a systolic thrill was felt in the left fourth anterior intercostal space. His facial features were suggestive of the rubella syndrome. The ECG showed biventricular hypertrophy with right ventricular dominance. The pulse rate was 150 beats per minute, respirations 48 per minute, and the blood pressure was 80 mm Hg, systolic. The liver was felt to be 3 to 4 em below the costal margin. He was believed to have a patent ductus arteriosus ( PDA) which was surgically ligated without complication at the age of six weeks. A loud murmur persisted, however, after operation, and there was clinical evidence of a persistent significant left-to-right shunt. Chest x-ray films showed the persistence of cardiomegaly and increased pulmonary vascularity. Cardiac catheterization and angiocardiography indicated the presence of a large shunt from the proximal aorta to the proximal main pulmonary artery. A second surgical procedure was therefore carried out two weeks following ligation of the patent ductus through a midsternal incision, with preparation for cardiopulmonary bypass should this be required. The right ventricle and the pulmonary artery were found to be large, and a coarse thrill was palpable over the proximal part of the main stem of the pulmonary artery. Further dissection confirmed the presence of a large vessel connecting the proximal aorta to the proximal main stem of the pulmonary artery (Fig 3). This "vessel" appeared to originate from the posterior surface of the proximal aorta from where it curved upwards and to the left to connect to the posterior medial aspect of the pulmonary arterial stem. The vessel was approximately 1.5 em long and had a diameter of 3 to 4 mm. It was easily isolated and doubly ligated. A bypass was not required. Ligature of the vessel abolished the thrill. The operation was uncomplicated and recovery was uneventful. The child has remained well. DISCUSSION
The main purpose of this presentation is to draw attention to the existence of a variant of the "aortopulmonary window" which does not appear to be widely known. Takao et al8 have classified aortopulmonary
FiGURE 3. At surgery, looking at exposed heart, right atrium has been retracted. An umbilical tape has been passed behind the long aortopulmonary communication. 1, Aortopulmonary communication; 2, aorta; 3, pulmonary artery; 4, right ventricle; and 5, right atrium.
CHEST, 75: 3, MARCH, 1979
windows into three types. Type 1 is proximal "typical." Type 2 is distal. Type 3 is a combination of types 1 and .2. The two cases of aortopulmonary COIIlDlunication presented here were of type 1 of the classification of Takao et al, 8 but in both instances, the communication had acquired the morphologic configuration almost of an independent vessel. The term "window" would hardly be justified or appropriate for this variant. R. Van Praagh, M.D., stated that the aortopulmonary window "does indeed appear to have-does have-some length" (written communication, December, 1977). This is a .point which should be emphasized, and which does raise the question whether the term "window" should not be replaced by the term "communication." There can, however, be little doubt that the vessel-like anomaly described here is merely a variant of the better known aortopulmonary "window" whose basis is a defect in the development of the aortopulmonary septum. 4 This anomaly is frequently (35 percent) associated with other congenital malformations of the heart, G including VSD (7.7 percent), right aortic arch (6.8 percent), anomalous origin of the left coronary artery from the pulmonary artery (5.1 percent), and PDA (11 percent). One of the two patients presented here had a PDA, and the other one had a right aortic arch ASD and VSD. The associated cardiac anomalies may make the diagnosis difiicult, G,e and indeed, our first patient who had a coexistent PDA was not suspected of the presence of an aortopulmonary communication until after the ligation of the patent ductus had demonstrated the persistence of a significant shunt. The preferred surgical approach appears to be via the aorta. There does not appear to be any reason why, in suitable cases, the vessel could not be isolated and ligated without the institution of cardiopulmonary bypass as in the two cases presented here. Clearly, the correct approach will be influenced by the underlying pathologic changes and will have to be decided upon on an individual basis.
REFERENCES 1 Neufeld HN, Lester RG, Adams P Jr, et al: Aorticopulmonary septal defect. Am J Cardiol 9: 12-25, 1962 2 Blieden LC, Moller JH: Aorticopulmonary septal defect: An experience with 17 patients. Br Heart J 36:630-635, 1974 3 Takao A, Mori K, Konno S, et al: Anatomical classification of aorticopulmonary window. In: Proceedings of the World Congress of Cardiology (abstract 396), Buenos Aires, Advances in Congenital Heart Disease, 1974 4 Edward JE: Congenital malformation of the heart and great vessels. In Gould SE: Pathology of the Heart and Blood Vessels, 3rd ed. Springfield, m, Charles C Thomas Publisher, 1968, pp 411-412 5 Faullcner SL, Oldham RR, Atwood GF, et al: Aortopulmonary window, ventricular septal defect, and membranous pulmonary atresia with a diagnosis of truncus arteriosus. Chest 65:351-353, 1974 6 Fisher EA, DuBrow IW, Eckner FAO, et al: Aorticopulmonary septal defect and interrupted aortic arch: A diagnostic challenge. Am J Cardiol34:356-359, 1974
CHEST, 75: 3, MARCH, 1979
Calcific Pulmonic Stenosis in Adulthood* Treatment by Valve Replacement (Porcine Xenograft) With Postoperative Hemodynamic Evaluation Edgar A. CovarrubiDs, M.D.; Mahar U. Sheikh, M.D.; Jeffrey M. Isner, M.D.; Marlo Gomes, M.D., F.C.C.P.; Charles A. Hufnagel, M.D., F.C.C.P.; and William C. Roberts, M.D., F.C.C.P•
COnical and morphologic: features are described iD a 56year-old man 'in whom severe, isolated pulmonic: valve stenosis was Created by valve replacement with a porc:ioe prosthesis. The c:alc:ific: deposits were located on the ventricular aspect of the pnlmonle valve, opposite the location (arterial aspect) of c:alc:ific: deposits on stenotfe aortic: valves, and c:alc:ific: deposibl also were present in the tricuspid valve anulus.
J solated pulmonic valve stenosis of severe degree in Calcific persons over 50 years of age is rare. 1 -4
deposits in stenotic pulmonic valves also are rare, 24 and calcific deposits in the tricuspid valve anulus are even more rare. G,o Treatment of isolated pulmonic valve stenosis by valve replacement has not been reported. Although each of the above occurrences is rare, all of them occurred together in our patient. In addition, the patient had postoperative hemodynamic evaluation after pulmonic valve replacement with a porcine xenograft. CASE REPoRT
A 56-year-old man was easily fatigued during his teens and was denied military service because of a "heart murmur." He was asymptomatic until age 46 years when exertional dyspnea occurred, and he was digitalized. At age 52, atrial fibrillation was noted, and cardioversion was done. During the next four years, the exertional dyspnea gradually progressed so that by age 56, he had considerable dyspnea when walking three flat blocks at a normal pace or climbing one flight of stairs. Examination in November 1977 (age 56) disclosed a prominent "a" wave in the jugular veins, no precordial thrills, a decreased pulmonic component of the second heart sound at the base, a grade 3/6 harsh, systolic precordial murmur loudest over the upper left sternal border, and no organomegaly or subcutaneous edema. Electrocardiogram showed sinus rhythm and right bundle branch bloclc. Chest roentgenogram (Fig 1 ) disclosed cardiomegaly and heavy calcific deposits in the pulmonic valve. The catheterization data are summarized in Table 1. At operation on Feb 2, 1978, palpation via the right atrium disclosed calcific deposits in the tricuspid valve anular region. The pulmonary trunk was dilated, and a thrill was palpated. °From the Georgetown Cardiology Service, District of Columbia Hospital, and the Deparbnents of Surge!'Y1 Medicine, and Pathology, Georgetown University Hospital, Washington, DC; and the Pathology Branch, Nationiil Heart, Lung, and Blood Institute. NatioDallnstitutes of Hea1th, Bethesda. Reprint te~f.!68fs: Dr. Robem, Chief, Patholo_sy_Branch. 1;¥HLBI1 Btag lOA, Boom 3E30, 9000 Rockmlle PUce, Betheaaa 20014
CALCIFIC PULMONIC STENOSIS IN ADULTHOOD 399
