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Spontaneous Closure of Atrial Septal Defect With Cardiac Failure in Infancy
test ( PPD-S) induced no induration. Bleeding measurements, including platelet count, prothrombin time, and partial thromboplastin time, were all within normal limits. Thoracocentesis revealed grossly hemorrhagic fluid containing WBCs, 1,350/cu mm, and RBCs, 580,000/cu mm. The protein concentration was 4.9 gm/100 ml; glucose was 84 mg/100 ml; and lactic dehydrogenase, 965 IU/liter. Cope needle biopsy showed only chronic inflammation on histologic examination of the pleura. Stains for fungi and tubercle bacilli were negative. Cultures of the pleural fluid and the biopsy material were negative for aerobic and anaerobic bacteria, Mycobacterium tuben:uloris, and fungi. Cytologic examination findings were negative for malignant cells. The patient underwent an elective thoracotomy, and a 3cm 6rm mass was found within the right lower lobe. The surgeon noted &brous adhesions but no other speci&c pleural pathologic findings. On inspection, prior to lobectomy, the two feeder arteries were identi&ed and ligated. Pathologic examination showed the mass to be atelectatic parenchyma surrounded by a dense 6brous wall. No communication between the mass and the bronchial tree was demonstrable. The structure was not lined by bronchial epithelium. No bleeding site was found. DISCUSSION
Bloody pleural effusions are commonly associated with puhnonary infarction, malignant involvement of the pleura, and chest trauma. Although a bloody effusion may be associated with other diseases, eg, pancreatitis and uremia, its presence in a 50-year-old smoker with a mass lesion seen on chest roentgenogram almost always signifies malignancy. A careful review of the literature revealed only two previous reports of bloody effusions associated with intralobar sequestration. Each of those cases was characterized by the sudden development of a massive hemothorax, and immediate thoracotomy was required in both patients to effect hemostasis. 1 •8 This case is the first report of an association between an intralobar sequestration and a subacute bloody, exudative, pleural effusion. Since there was no clinical or pathologic evidence for any of the recognized causes of hemothorax, we can only assume its occurrence was related to the sequestration. It is attractive to speculate that clinically inapparent trauma caused one of these thin abnonnal vessels, which have been described as lacking the usual musculature of systemic arteries, to leak blood into the pleural space.' Intralobar puhnonary sequestration may be associated with other congenital abnonnalities, including situs inversus, the scimitar or venolobar syndrome, and diaphragmatic hernias. 5 In the absence of other anomalies, intralobar sequestration is discovered in the symptomatic patient almost exclusively because of complicating infection.8 However, when an undiagnosed roentgenographic • abnonnality is compatible with an intralobar sequesqation, the presence of a bloody pleural effusion should neither eliminate this benign process from consideration, nor deter one from obtaining the appropriate diagnostic studies.
fu:FmtENCJ!'S 1 Spencer H: Pathology of the Lung. Pbi1adelphia, WB Saunders, 1977 p 84
CHEST, 75: 3, MARCH, 1979
2 Zumbro GL, Green DC, Brott W, et al: Pulmonuy ~equestration with spontaueons intrapleural hemorrhage. J Thorac Cardiovasc Surg 68:673-674, 1974 3 Oxman LM: Intralobar sequestration causinl hemoptysis and hemot:hotu. NY State J Med 74:961-96i, 1974 4 Pryce DM: Lower accessory pulmonuy aartery with intra· lobar sequestration of lung: A report of seven cases. I Pathol Bact 58:457-467, 1946 5 Felson B: Chest Roentgenology. Philadelphia, WB Saunders,1973 6 Marks C, Wiener SN, Reydman IC: Pulmonuy sequestration. Chest 61:253-257, 1972
Spontaneous Closure of Atrial Septal Defect With Cardiac Failure in Infancy* Andrea C.V. Giardina. M.D.;•• Arthur S. .Raptoulil, M.D.;t Mary Allen Engle, M.D., F.C.C.P.;t and Aaron R. Leofn, M.D.§
Two serial eardiae catheterlzatlou before the ap of four months and another at sb: yean con&rmed I(IOntaneons dosnre of a laqe atrial septal defec:t wblda cansed eon.. gestlve heart fallnn in infaney. bile atrial septal defect (ASD) is one of the most W frequently encountered congenital malformations
of the heart, it is not commonly diagnosed in infancy, and it rarely causes problems early in life. Nonetheless, this usually benign lesion may produce congestive heart failure and even death during infancy and childhood.1 •1 Another uncommon aspect of the natural history of ASD is its spontaneous_, closure, and quite rare is spontaneous closure of an isolated secundum-type of ASD with heart failure in infancy. 8 • 8 This report presents serial cardiac catheterization data that demonstrated such an occurrence.
The patient seemed well for his &rst two months, then tachypnea with wheezing appeared. He was foUDd to be in cardiac failure with tachycardia, tachypnea, cardiomegaly, and hepatomegaly, but no cyanosis. A grade 2/6 systolic mur~ mur with midsystolic accentuation and early systolic ejection sound was heard at the second right and left int8rcoltal ~. in the suprasternal notch, and over both lung&elds posteriorly, •From the Divflion of Pediatric Cardiology. The New York Hospital-Cornell University Medical Centmf New York. ••Assistant AttendinJr in Pediatrics, Beth lsrae Medical Center. ~ent of Pediatrics, Beth Israel Medical Center, New YOrk. tAttending Ph~cian in Pediatric Cardiology, Orlando Regional MediCal Centm, the Depa$1ent of Pediatrics, Orlando Regional Medical Center, Florida. :tProfessor of Pediatrics, The New York Hospital-Cornell University Medical Center.
§Professor of Pediatrics, The New York Hospital-Cornell
University Medical Center. .Rsprint reqU6818: Dr. Engls, 'NBW Yorlc HOif'lltJZ, 'NBW York Cifr/100!1
SPONTANEOUS CLOSURE OF ASD -
Tahle l-Henaodynamlc DGIG Cardiac Catheterization
LA RA RV, Flowa (Jiter/min/sq m) Mean, Mean, MPA, Qpl Qsl Qp:Qs mmHg mmHg mmHg mmHg
LV, mmHg
10.2
11.2
4.2
2.60:1
14.0
8.5
58/4
53/10
125/11
0.33
9.8
10.8
5.4
1.94:1
14.5
10.5
60/14
57/10
100/10
0.94
12.7
8.1
8.1
1.00:1
8.0
31/8
28/11
110/7
Age
BSA,. sqm
Hemoglobin, gm/100ml
3mo
0.33
2
3~mo
3
8yr
...
Rp, FA, mm Hg/Liter/ mmHg min/sqm
98/70
2.1 2.2
115/85
1.8
*Abbreviations: BSA, body surface area; LA, left atrium; RA, right atrium; RV, right ventricle; MPA, main pulmonary artery; LV,Ieft ventricle; FA, femoral artery; and Rp, right pulmonary.
The second heart sound was narrowly split No diastolic murmur was heard. Femoral pulses felt less. strong than those in the arms. Electrocardiogram demoustrated sinus tachycardia with frequent atrial premature contractioDS, right axis deviation, right ventricular hypertrophy, and conduction disturbance. Chest roentgenograms showed cardiomegaly (cardiapulmonary thoracic ratio of 62 percent) with in~ vascularity. The findings suUested a left-to-right shunt and obstruction to left ventricular outflow. Digitalization controlled the cardiac failure; heart rate slowed, and rhythm became normal. At three months, cardiac catheterization demonstrated (Table I) a moderately large left-to-right shunt at atrial level, comprising 62 percent of pulmonary blood flow. Right ventricular and pulmonary arterial pressures were moderately elevated, as was pulmonary vascular resistance. Pulmonary artery was twice the size of the aorta. Pulmonary veins drained into the left atrium. Left ventricular systolic and enddiastolic pressures were elevated, and a 17 mm Hg peak systolic gradient was recorded between the left ventricle and femoral artery on simultaneously measured pressures. This suggested mild aortic stenosis and/or coarctation of the aorta, but the left ventricular outflow tract and aorta were not clearly delineated angiocardiographically. Because of that, as well as the fact that the pulmonary veins were not entered during this study to rule aut cor triatriatum contn'buting to pulmonary hyperteusion, a second cardiac catheterization was performed two weeks later (Table 1). A large, secundum-type ASD with moderate pulmonary hypertension was again demoustrated. There was no pulmonary "capillary" to left atrial gradient on sequential measurements and no pres• sure gradient across the mitral valve; this time, left ventricular and aortic pressures were normal. Angiocardiography ruled out a third atrial chamber, anomalous or obstructed pulmonary venous return, aortic stenosis, and coarctation of the aorta. The left-to-right shunt across the ASD comprised 49 percent of pulmonary blood flow. He steadily improved. and at one year of age, he had normal height and weight, pulses and blood pressure, and only a soft systolic murmur. Electrocardiogram and heart size were normal ( cardiothoracic ratio of 49 percent). Pulmonary vascularity was average. He remained asymptomatic when digitalis was discontinued. The systolic murmur disappeared. The third cardiac catheterization at six years of age showed normal hemodynamics and no evidence of atrial left-to-right shunting. DISCUSSION
Because it is so unusual for a simple secundum-type ASD to cause the heart to fail in infancy, we had sought evidence of associated obstruction to left abial inflow or left ventricular out:ftow. Although the first cardiac catheterization suggested such obstruction, the second study
-
GIARDINA ET AL
revealed no other anomaly than the isolated ASD. The reasons are not cl~ why a shunt at atrial level that increased J>u!monary flow and pressure to twice normal should cause the heart to fail Possibly, there was some early neonatal underdevelopment of left ventricular outflow or of the isthmus of the aorta, as the pressures at first catheterization suggested. With continued beating of the heart and molding of these structures, the contours assumed the normal, unobstructing conflguration. Only rarely have ASDs in symptomatic infants been noted to have closed spontaneously. Hoffman et al8 presented serial hemodynamic data on four infants who had functional closure of an ASD during infancy, but only one of their patients had cardiac symptoms. Timmis et al7 documented closure of a small ASD in a patient who had signs of heart failure attributed to myocarditis. Cardiac catheterization took place at 8 and 14 months, and at three years of age. CaylerB presented serial catheterization data showing spontaneous functional closure of large ASDs in three symptomatic infants, one of whom had no other cardiac anomaly. Watson and Dark described five cases with spontaneous closure; one had been in heart failure." Hoffman et al' reported 24 infants with ASD and no other lesion, who had spontaneous closure of the defect. Of 6ve with congestive failure, three had documentation of closure by a second catheterization. Menon and Wagner& described four cases of spontaneous closure, two of them in infants with heart failure. The mechanisms for spontaneous closure of a secundum-type ASD are not known. Edwards10 postulated that postnatal growth of the heart caused a change in shape of the defect from oval to elliptical until the margins drew together and sealed. CaylerB suggested that continued downward growth of the septum sellUDdum obliterated the defect. Timmis et al7 thought that myocarditis might produce destruction with subsequent repair by fibrosis and sealing of the defect. Menon and Wagner& cited Rodbard's theory11 that hydraulic forces of high-velocity flowing across an ASD might produce valve formation and cushions that tended to close the • defect, especially during infancy when intracardiac structures are rapidly growing. Our observations suggest the possibility that fetal and neonatal slight hypoplasia of the outflow tract of the left ventricle or of the aorta may offer temporary obstruction to left ventricular outlow and favor left-to-right shunting through a patulous foramen ovale or defect in the abial septum. Postnatal
CHEST, 75: 3, MARCH, 1979
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
Bloody pleural effusions are commonly associated with puhnonary infarction, malignant involvement of the pleura, and chest trauma. Although a bloody effusion may be associated with other diseases, eg, pancreatitis and uremia, its presence in a 50-year-old smoker with a mass lesion seen on chest roentgenogram almost always signifies malignancy. A careful review of the literature revealed only two previous reports of bloody effusions associated with intralobar sequestration. Each of those cases was characterized by the sudden development of a massive hemothorax, and immediate thoracotomy was required in both patients to effect hemostasis. 1 •8 This case is the first report of an association between an intralobar sequestration and a subacute bloody, exudative, pleural effusion. Since there was no clinical or pathologic evidence for any of the recognized causes of hemothorax, we can only assume its occurrence was related to the sequestration. It is attractive to speculate that clinically inapparent trauma caused one of these thin abnonnal vessels, which have been described as lacking the usual musculature of systemic arteries, to leak blood into the pleural space.' Intralobar puhnonary sequestration may be associated with other congenital abnonnalities, including situs inversus, the scimitar or venolobar syndrome, and diaphragmatic hernias. 5 In the absence of other anomalies, intralobar sequestration is discovered in the symptomatic patient almost exclusively because of complicating infection.8 However, when an undiagnosed roentgenographic • abnonnality is compatible with an intralobar sequesqation, the presence of a bloody pleural effusion should neither eliminate this benign process from consideration, nor deter one from obtaining the appropriate diagnostic studies.
fu:FmtENCJ!'S 1 Spencer H: Pathology of the Lung. Pbi1adelphia, WB Saunders, 1977 p 84
CHEST, 75: 3, MARCH, 1979
2 Zumbro GL, Green DC, Brott W, et al: Pulmonuy ~equestration with spontaueons intrapleural hemorrhage. J Thorac Cardiovasc Surg 68:673-674, 1974 3 Oxman LM: Intralobar sequestration causinl hemoptysis and hemot:hotu. NY State J Med 74:961-96i, 1974 4 Pryce DM: Lower accessory pulmonuy aartery with intra· lobar sequestration of lung: A report of seven cases. I Pathol Bact 58:457-467, 1946 5 Felson B: Chest Roentgenology. Philadelphia, WB Saunders,1973 6 Marks C, Wiener SN, Reydman IC: Pulmonuy sequestration. Chest 61:253-257, 1972
Spontaneous Closure of Atrial Septal Defect With Cardiac Failure in Infancy* Andrea C.V. Giardina. M.D.;•• Arthur S. .Raptoulil, M.D.;t Mary Allen Engle, M.D., F.C.C.P.;t and Aaron R. Leofn, M.D.§
Two serial eardiae catheterlzatlou before the ap of four months and another at sb: yean con&rmed I(IOntaneons dosnre of a laqe atrial septal defec:t wblda cansed eon.. gestlve heart fallnn in infaney. bile atrial septal defect (ASD) is one of the most W frequently encountered congenital malformations
of the heart, it is not commonly diagnosed in infancy, and it rarely causes problems early in life. Nonetheless, this usually benign lesion may produce congestive heart failure and even death during infancy and childhood.1 •1 Another uncommon aspect of the natural history of ASD is its spontaneous_, closure, and quite rare is spontaneous closure of an isolated secundum-type of ASD with heart failure in infancy. 8 • 8 This report presents serial cardiac catheterization data that demonstrated such an occurrence.
The patient seemed well for his &rst two months, then tachypnea with wheezing appeared. He was foUDd to be in cardiac failure with tachycardia, tachypnea, cardiomegaly, and hepatomegaly, but no cyanosis. A grade 2/6 systolic mur~ mur with midsystolic accentuation and early systolic ejection sound was heard at the second right and left int8rcoltal ~. in the suprasternal notch, and over both lung&elds posteriorly, •From the Divflion of Pediatric Cardiology. The New York Hospital-Cornell University Medical Centmf New York. ••Assistant AttendinJr in Pediatrics, Beth lsrae Medical Center. ~ent of Pediatrics, Beth Israel Medical Center, New YOrk. tAttending Ph~cian in Pediatric Cardiology, Orlando Regional MediCal Centm, the Depa$1ent of Pediatrics, Orlando Regional Medical Center, Florida. :tProfessor of Pediatrics, The New York Hospital-Cornell University Medical Center.
§Professor of Pediatrics, The New York Hospital-Cornell
University Medical Center. .Rsprint reqU6818: Dr. Engls, 'NBW Yorlc HOif'lltJZ, 'NBW York Cifr/100!1
SPONTANEOUS CLOSURE OF ASD -
Tahle l-Henaodynamlc DGIG Cardiac Catheterization
LA RA RV, Flowa (Jiter/min/sq m) Mean, Mean, MPA, Qpl Qsl Qp:Qs mmHg mmHg mmHg mmHg
LV, mmHg
10.2
11.2
4.2
2.60:1
14.0
8.5
58/4
53/10
125/11
0.33
9.8
10.8
5.4
1.94:1
14.5
10.5
60/14
57/10
100/10
0.94
12.7
8.1
8.1
1.00:1
8.0
31/8
28/11
110/7
Age
BSA,. sqm
Hemoglobin, gm/100ml
3mo
0.33
2
3~mo
3
8yr
...
Rp, FA, mm Hg/Liter/ mmHg min/sqm
98/70
2.1 2.2
115/85
1.8
*Abbreviations: BSA, body surface area; LA, left atrium; RA, right atrium; RV, right ventricle; MPA, main pulmonary artery; LV,Ieft ventricle; FA, femoral artery; and Rp, right pulmonary.
The second heart sound was narrowly split No diastolic murmur was heard. Femoral pulses felt less. strong than those in the arms. Electrocardiogram demoustrated sinus tachycardia with frequent atrial premature contractioDS, right axis deviation, right ventricular hypertrophy, and conduction disturbance. Chest roentgenograms showed cardiomegaly (cardiapulmonary thoracic ratio of 62 percent) with in~ vascularity. The findings suUested a left-to-right shunt and obstruction to left ventricular outflow. Digitalization controlled the cardiac failure; heart rate slowed, and rhythm became normal. At three months, cardiac catheterization demonstrated (Table I) a moderately large left-to-right shunt at atrial level, comprising 62 percent of pulmonary blood flow. Right ventricular and pulmonary arterial pressures were moderately elevated, as was pulmonary vascular resistance. Pulmonary artery was twice the size of the aorta. Pulmonary veins drained into the left atrium. Left ventricular systolic and enddiastolic pressures were elevated, and a 17 mm Hg peak systolic gradient was recorded between the left ventricle and femoral artery on simultaneously measured pressures. This suggested mild aortic stenosis and/or coarctation of the aorta, but the left ventricular outflow tract and aorta were not clearly delineated angiocardiographically. Because of that, as well as the fact that the pulmonary veins were not entered during this study to rule aut cor triatriatum contn'buting to pulmonary hyperteusion, a second cardiac catheterization was performed two weeks later (Table 1). A large, secundum-type ASD with moderate pulmonary hypertension was again demoustrated. There was no pulmonary "capillary" to left atrial gradient on sequential measurements and no pres• sure gradient across the mitral valve; this time, left ventricular and aortic pressures were normal. Angiocardiography ruled out a third atrial chamber, anomalous or obstructed pulmonary venous return, aortic stenosis, and coarctation of the aorta. The left-to-right shunt across the ASD comprised 49 percent of pulmonary blood flow. He steadily improved. and at one year of age, he had normal height and weight, pulses and blood pressure, and only a soft systolic murmur. Electrocardiogram and heart size were normal ( cardiothoracic ratio of 49 percent). Pulmonary vascularity was average. He remained asymptomatic when digitalis was discontinued. The systolic murmur disappeared. The third cardiac catheterization at six years of age showed normal hemodynamics and no evidence of atrial left-to-right shunting. DISCUSSION
Because it is so unusual for a simple secundum-type ASD to cause the heart to fail in infancy, we had sought evidence of associated obstruction to left abial inflow or left ventricular out:ftow. Although the first cardiac catheterization suggested such obstruction, the second study
-
GIARDINA ET AL
revealed no other anomaly than the isolated ASD. The reasons are not cl~ why a shunt at atrial level that increased J>u!monary flow and pressure to twice normal should cause the heart to fail Possibly, there was some early neonatal underdevelopment of left ventricular outflow or of the isthmus of the aorta, as the pressures at first catheterization suggested. With continued beating of the heart and molding of these structures, the contours assumed the normal, unobstructing conflguration. Only rarely have ASDs in symptomatic infants been noted to have closed spontaneously. Hoffman et al8 presented serial hemodynamic data on four infants who had functional closure of an ASD during infancy, but only one of their patients had cardiac symptoms. Timmis et al7 documented closure of a small ASD in a patient who had signs of heart failure attributed to myocarditis. Cardiac catheterization took place at 8 and 14 months, and at three years of age. CaylerB presented serial catheterization data showing spontaneous functional closure of large ASDs in three symptomatic infants, one of whom had no other cardiac anomaly. Watson and Dark described five cases with spontaneous closure; one had been in heart failure." Hoffman et al' reported 24 infants with ASD and no other lesion, who had spontaneous closure of the defect. Of 6ve with congestive failure, three had documentation of closure by a second catheterization. Menon and Wagner& described four cases of spontaneous closure, two of them in infants with heart failure. The mechanisms for spontaneous closure of a secundum-type ASD are not known. Edwards10 postulated that postnatal growth of the heart caused a change in shape of the defect from oval to elliptical until the margins drew together and sealed. CaylerB suggested that continued downward growth of the septum sellUDdum obliterated the defect. Timmis et al7 thought that myocarditis might produce destruction with subsequent repair by fibrosis and sealing of the defect. Menon and Wagner& cited Rodbard's theory11 that hydraulic forces of high-velocity flowing across an ASD might produce valve formation and cushions that tended to close the • defect, especially during infancy when intracardiac structures are rapidly growing. Our observations suggest the possibility that fetal and neonatal slight hypoplasia of the outflow tract of the left ventricle or of the aorta may offer temporary obstruction to left ventricular outlow and favor left-to-right shunting through a patulous foramen ovale or defect in the abial septum. Postnatal
CHEST, 75: 3, MARCH, 1979
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