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The electrocardiogram in atrial septal defects and atrioventricular cushion defects
Annotations
The electrocardiogram and atrioventricular
in atrial septal cushion defects
The electrocardiogram has proved to be of considerable value in the differentiation of atria1 septal defects (ASD) of the ostium secundum type from those of the ostium primum type. Several recent excellent articles attest to this,‘-6 although some observers have failed to confirm this differential diagnostic value of the electrocardiogram.‘sR Atria1 septal defects of the ostium secundum type may be subdivided into three types: (a) sinus venosus (superior caval); (b) fossa ovalis (central); and (c) inferior caval.” It is not possible to distinguish between these subtypes electrocardiographically. Ostium primum defects occur in the lower portion of the atria1 septum in the region of the atrioventricular (-4-V) valves and are usually associated with a cleft in the anterior leaflet of the mitral valve and occasionally in the septal leaflet of the tricuspid valve. This type has also been called the partial form of persistent common X-\canal.‘Jo In the complete type of persistent common X-V canal, in addition to the ostium primum type of defect in the atria1 septum, there is a ventricular septal defect (VSD) and a common A-V valve or fusion of the mitral and tricuspid valves across the top of the ventricular septum with small communications beneath.4J0 The terms “endocardial cushion defects”l’ or “A-V cushion defects”’ have also been applied to both partial and complete common A-\. canal. Complete absence of the atrial septum is termed “common atrium” or “single auricle.“6J2~‘3 The electrocardiogram in .4SD of the ostium secundum type characteristically displays an rSR’ in the right precordial leads (VI, V~R), and right axis deviation (RAD) in the standard leads. This rSR’ pattern (QRS < 0.12 second) has been found to occur in about 65 per cent of the cases of ASD of the ostium secundum type.gJ The pattern of complete right bundle branch block (CRBBB) has been encountered in from 5 to 40 per cent of the cases.2JJ4 Other patterns which may occur in the right precordial leads include qR, rR’, Rs, rSr’ (normal crista supraventricularis pattern), and rS.lbJ The mean QRS axis in most cases lies between +60 and -150 degrees, with the majority lying between +90 and + 1.50 degrees.lb-3,6*6,‘6 The rSR’ pattern (QRS < 0.12 second) in the right precordial leads in ASD has been variousI> termed “incomplete right bundle branch block,“6J2-‘5 “diastolic o\-erload of the right ventricle,“‘6 and “right ventricular outflolv tract hypertroph!..“z
712
defects
Cardiac catheterization studies in ASD have demonstrated that there is no delay in the onset of right \zentricular systole, such as would occur if there were a true right bundle branch block.‘7 Many of the recent studies favor dilatation and/or hypertrophy of the crista supraventricularis region of the right ventricle as the erplanation for this pattern.2J,‘7J8 The rSR’ pattern is usually associated with normal or slightly elevated right ventricular and pulmonary arterial pressures. lb.3,li If pulmonary hypertension supervenes and concentric right ventricular hypertrophy develops, the right precordial leads often display a qR or Rs configuration.‘b~3~‘7~‘s The occurrence of a qR or Rs pattern might also suggest the presence of some associated anomaly, such as pulmonic stenosis or even mitral stenosis.6 The electrocardiographic pattern which has been found to be nearly pathognomonic of A-V cushion defects is left axis deviation (LAD) in the standard leads with the rSR’ pattern in the right precordial leads. The LrZD of the early QRS forces is usually in the range of 0 to -60 degrees,4j’Q whereas the mean QRS axis usually ranges from - 60 to * 180 degrees.1Z,6 Some workers have attributed the L&ID to left ventricular enlargement secondary to the mitral illsufficiency,“,zo,zl whereas others ha\.-e favored a parietal wall conduction defect’” or a congenital aberration of the left bundle branch system.3-6 Burchell, DuShane and Brandenburg4 have presented a critical review and rather convincing evidence in favor of the latter explanation. This distinctive electrocardiographic pattern of LAD has been described in 80 to 100 per cent of A-\’ cushion defects.‘&,2 3~9 Other workers, however, ha\e not found such a high incidence of L.4D, and ha\-e even encountered right axis deviation (RAD) in some cases.7,la Burchell and associates’ have encountered some cases of paitial or incomplete forms of II\-\’ cushion defects without the typical electrocardiographic pattern, but no cases of the complete form without the characteristic electrocardiogram. In =\-V cushion defects the right precordial leads may also display complete right bundle branch block or qR, Rs, rSr’, or rS patterns which are not diagnostically different from those of ASD of the ostium secundum type. In the presence of mitral insufficient)the left precordial leads may or may not demonstrate left ventricular overloading or hypertrophy. Atriovcntrirular cushion defects have
Anzotutions
beeu found to manifest q waves in left precordial leads twice as commonly (70 per cent) as do atria1 septal defects of the ostium secundum type (35 per cent).1”,4 The QRS xector loop in ;\SD of the ostium secundum type rotates clockwise in the frontal plane and is oriented below the isoelectric line (0 to 180 degrees), to the right and anteriorly.‘-l~?z ‘l‘he QRS vector loop in A-V cushion defects rotates c.onnterclocl;~vise in the frontal plane and is oriented superior to the isoelectric line (0 to 180 degrees), to the left and posteriorly.2-4~22 A horizontal ligure-ofeight pattern oriented along the isoelectric line, lvith the initial part of the loop rotating counterc-lockwlse, also has been described in .A-\- cushion (,efects,‘“.4-” The ventricular gradient (c) in ostium secundum defects tends to be \-ertical and oriented to the left of the mean QRS (&ns), whereas in A-\’ cushion defects. c is horizontal and to the rieht of &ns.? .~_“. Prolongation of the P-R interval occurs more c.ommonly in A-V cushion defects than in ostium secuudum defects.lbm” Atrial fibrillation has been described in about 10 per cent of the cases of .\SD,‘“J3 being uncommon in the young but more frequent in older persons.23 .Atrial fibrillation is like\vise uncommon iu A-V cushion defects.?s3 Tall I’ waves in Leads II, III, and a\-~, are surprising11 infrequent,2~“~14 although diphasic P v,yaves in Lead \:I (\‘?j which suggest right atrial enlargement have been described in about 50 per cent of the cases of hoth ASD and A-V cushion defects.ln,2 In patients with common atrium, LAD and counterclockwise rotation of the QRS loop identical to that in .X-V cushion defects have been encountered.5.6 .\nother interesting feature noted by Keith5 in cases of common atrium has been a P-wave ask of -60 degrees, together with a short P-R iuterval. It is worth emphasizing that ASD of the ostium secundum type may occur with a normal prerordial electrocardiogram (without the rSR’ pattern), with ;I normal axis, and, rarely, with LAD.‘“-3 The frontal I-ector ma\- also occasionally be oriented superiorI>above the -isoelectric line or horizontally in a figureof-eight pattern and have counterclockwise rotation.1b+6 Of considerable interest has been the observation that ostium secundum defects with acquired mitral insufficiency do not display L.qD but may show RAD and clockwise rotation of the frontal vector.4,5 Atrioventricular cushion defects occasionally ma! display a normal QRS axis and rarely even have 1~,4r)3,1 and c-lo&wise rotation of the frontal vector loop. Patients lvith pulmonic stenosis, patent ductus arteriosus, \-entriculnr septal defects, or total anomalous I-enous return present occasionally an electrocardiographic pattern which resembles that characteristically found in .\SD of the ostium secuudum type.6.F,21 Other types of congenital heart disease that may occasionally simulate the electrocardiographic pattern described in A-\’ cushion defects include: (1) VSD, especially those located posteriorly beneath the seotal triru~oid leaflet”: (2) combined ostium secund*um defect and VSDj,?‘; i3) origin of both the aorta and pulmonary artery from the right \‘en-
713
triclez6; (4) persistent truncus arteriosuslg; (5) single ventriclelg; (6) endocardial tibroelastosis.1” Cases of tricuspid atresia display LXD, but the initial forces, although horizontal, are not so leirward as in .A-\’ cushion defects, and the right prycordial leads do not exhibit the rSR’ pattern.‘” In conclusion, it appears that although there at-e significant exceptions, the electrocardiogram usualI! \vill permit a distinction to be made bctxveen ostillm secundum and .2-V cushion defects. Kulph C. Swtt, JI.L). 1.niwsify o,f Cinrimtrti Collcgc of Nediciue Cincinnati. Ohio REFERESCES la. Toscano-Barbosa, E., Brandenburg, IX. O., and Burchell, H. B.: Electrocardiographic studies of cases with intracardiac malformations of the atrioventricular canal, I’roc. Staff Meet. hlayo Clin. 31:.513, 1956. lb. Toscano-Barbosa, E., Brandenburg, Ii. O., and Swan, H. J. C.: Atrial septal defect. The electrocardiog;am and its hemodynaruic correlation in 100 moved cases. .4m. 1. Cardiol. 2:698. 1958. ’ 2. Burch, G. E., and DePasquale, X.: The electrocardiogram and ventricular gradient in atrial septal defect, AM. HEART J. 58:190, 1959. 3. Pryor, R., Woodwark, G. hr., aud Blount, S. G., Jr.: Electrocardiographic changes in atrial septal defects: ostium secundum defect versus ostium primum (endocardial cushion) defect, AM. HEART J. 58:689, 1959. 4. Burchell, H. B., DuShane, J. W., and Brandenburg, Ii. 0.: The electrocardiogram of patients with atrio\-entricular cushion defects (defects of the atrioventricular ranxl‘~, Xm. J. Card;ol. 6:575, 1960. .5 . Evans, J. R., Rowe, R. D., and Keith, J. U.: The clinical diagnosis of atrial septal defect in children, 1Zm. J. Med. 30:34S, 1961. 6. DuShane, J. \V., Weidman, \V. II., Brandcnburg, 12. O., and Kirklin, J. \I’.: Differentiation of interntrial com~nuIlications by clinical methods: ostium secundum, ostium primum, common atrium, and total anomalous pulmonar) venous connection, Circulation 21:.163, 1060. A. C.. and Ellison. R. G.: Diacnusit; 7. \Vitham. of ostium primum defects of the atrial septum, Am. J. Med. 22:593, 1957. I’.: The diagnosis of persistent ostium 8. h#Iounsey, Brit. Heart J. 20:270, 1958. primum, 9. Bedford, D. E.: The anatomical types of atrial septal defect: their incidence and clinical diagnosis, .1m. J. Cardiol. 6:568, 1960. C. S., and Edwards, J. E.: lk\eiop10. \\‘akai, mental and pathologic considerations in pcrsistent common atrioventricul,N canal, I’roc. Staff Meet. Mayo Clin. 3147, 1956. 11. Campbell, XI., and Missen, G. -1. Ii.: l
714
13.
14.
15.
16.
17.
18.
Annotations
Taussig, H. B.: Congenital malformations of the heart, 1’01. II, ed. 2, Cambridge, 1960, Harvard University Press, published for The Commonwealth Fund. Barber, J. M., Magidson, O., and Wood. I’.: Atria1 septal defect, with special reference to the electrocardiogram, the pulmonary artcr! pressure and the second heart sound, Brit. Heart J. 12:277, 1960. Sodi-Pallares, D., Pileggi, F., Cisneros, F., Ginefra, P., Portillo, B., Medrano, G., and Bisteni, A.: The mean manifest electric_al axis of the ventricular activation process (AQRSI in congenital heart disease: a new approach in electrocardiographic diagnosis, AM. HEART J. 55:681, 1958. Cabrera, E., and Gaxiola, A.: rl critical reevaluation of systolic and diastolic overloading patterns, Prog. Cardiovas. Dis. 2:219, 1959. Walker, W. J., Mattingly, T. it:., Pollock, B. E., Carmichael, D. B., Inmon, ‘1‘. W’., and Forrester, R. H.: Electrocardiographic and hemodynamic correlation in atria1 septal defect, ‘4~. HKZRT J. 52:547, 1956. Martins de Oh\-cira, J., and Zimmerman, II. -4.: The electrocardiogram in interatrial septal defects and its correlation with hemodynamics, AM. HIZ~RT J. 55:369, 1958.
Acute
cmgioneurotic
edemu
Contemporary literature is barren of references to this particular syndrome. Even Luisada’s massive Cardiology’ set has nothing on this topic. Although rare, this condition does exist, and its salient features are worth discussing. Dr. F. Tice diagnosed a case when I was his resident at the Cook County (Chicago, Ill.) Hospital. I have been searching for them since, and in this report, I present my third case. The young lady in question is now a 23-year-old medical student. She is married and has no children. Since infancy, she has been under constant, careful medical supervision because of various food allergies and asthma. Her family physician thought she had an attack of rheumatic fever when she was 6 years old. He sent her to Arizona, where she spent several years at a boarding school. No doctor ever recorded a cardiac murmur or any other evidence of cardiac pathology. Since her return to New York. she has been relatively free of symptoms but has continued under close medical observation. In 1958, before entering medical school, she underwent a battery of exhaustive tests. Since everything was considered to be normal, she was permitted to undertake the proposed course, Besides infrequent, trivial upper respiratory infections, the young lady has stayed well. Her last physical check-up was in the summer of 1960.
19.
Grant, R. l’., Sanders, K. J ., %Iorro\r, .\. (G.. and Braunm-ald, E.: Symposium on diagnostic methods in the study of Icft-to-right &unts. Circulation 16:791, 1957. 20. Blount, S. G., Jr., Balchum, 0. J., .md Gellsilli. G.: The persistent ostium primum atria1 septal defect, Circulation 13:499, 1956. 11. LIilnor, \V. K., and Bertrand, C. A.: The clcctrocardiogram in atria1 septal defect. A study of twenty-four cases, with obser\-ations on the RSR’-VI pattern, Am. J. bled. 22:223, 1957. 22. Burch, G. E., and DePasquale, S.: The spatial \-ectorcartlioaram in proved congenital atri,d septal defecty.4.w. Hr;.,i~r J. 58:319, 1959. 23. \Vood. I’.: Diseases of the heart and circulation. ccl. 2, Philadelphia, 1956, J. B. Lippincott Company. 24. Espino-\-ela, J.. Muracl-Netto, S., and Kubio.-2lvarez, \..: Differential diagnosis betweet) persistent atriovcntricular canal and the conbination of atria1 and ventricular septal defects, .Am. J. Cardiol. 6:589, 1960. 2.5. Neufeld, N. H., DuShane, J. \\‘., \Vood, 1-:. Il., Kirklin, J. W., and Edwards, J. E.: Origin of both great vessels from the right ventricle. I. \%‘ithout pulmonary stenosis, Circulation 23:399, 1961.
of the mitral
valve
On Thanksgiving Day of 1960, the patient woke up with a slight cold, but this did not prevent her from preparing the usual Thanksgiving meal. After washing the dishes and cleaning ~rp, she felt tired and so went to bed shortly after 8 P.M. However. the sense of fatigue became progressively worse, and she became very conscious of a pounding, rapid heart beat. At 9 P.M., when I saw her, she was sitting up in bed, badly frightened, and fighting to catch her breath. The pulse was 106, even, and full; oral temperature was 98°F.; the blood pressure was 94/62 mm. Hg; the lungs were still dry and clear, with excellent expansion. The heart presented an amazingly forceful diastolic murmur with presystolic accentuation; also, a maximal systolic murmur was heard; the apical pulsation was m,rrkecl and a diastolic thrill was felt. Because of the positive knowledge of the absence of all antecedent cardiac pathology, acute edema of the mitral valve was diagnosed. In the expectation of a rapid subsidence of symptoms, she was reassured maximally, given a sedative hypnotic c,;rpsule and an alcoholic beverage drink. She relaxed and fell asleep; I did tell the husband to call me immediately if her symptoms took a turn for the worse. I made clear that radical measures up to emergency cardiac operation could become imprr:ltive if closure of the mitral valve threatened. In the morning the patient was entirely romfort-
The electrocardiogram and atrioventricular
in atrial septal cushion defects
The electrocardiogram has proved to be of considerable value in the differentiation of atria1 septal defects (ASD) of the ostium secundum type from those of the ostium primum type. Several recent excellent articles attest to this,‘-6 although some observers have failed to confirm this differential diagnostic value of the electrocardiogram.‘sR Atria1 septal defects of the ostium secundum type may be subdivided into three types: (a) sinus venosus (superior caval); (b) fossa ovalis (central); and (c) inferior caval.” It is not possible to distinguish between these subtypes electrocardiographically. Ostium primum defects occur in the lower portion of the atria1 septum in the region of the atrioventricular (-4-V) valves and are usually associated with a cleft in the anterior leaflet of the mitral valve and occasionally in the septal leaflet of the tricuspid valve. This type has also been called the partial form of persistent common X-\canal.‘Jo In the complete type of persistent common X-V canal, in addition to the ostium primum type of defect in the atria1 septum, there is a ventricular septal defect (VSD) and a common A-V valve or fusion of the mitral and tricuspid valves across the top of the ventricular septum with small communications beneath.4J0 The terms “endocardial cushion defects”l’ or “A-V cushion defects”’ have also been applied to both partial and complete common A-\. canal. Complete absence of the atrial septum is termed “common atrium” or “single auricle.“6J2~‘3 The electrocardiogram in .4SD of the ostium secundum type characteristically displays an rSR’ in the right precordial leads (VI, V~R), and right axis deviation (RAD) in the standard leads. This rSR’ pattern (QRS < 0.12 second) has been found to occur in about 65 per cent of the cases of ASD of the ostium secundum type.gJ The pattern of complete right bundle branch block (CRBBB) has been encountered in from 5 to 40 per cent of the cases.2JJ4 Other patterns which may occur in the right precordial leads include qR, rR’, Rs, rSr’ (normal crista supraventricularis pattern), and rS.lbJ The mean QRS axis in most cases lies between +60 and -150 degrees, with the majority lying between +90 and + 1.50 degrees.lb-3,6*6,‘6 The rSR’ pattern (QRS < 0.12 second) in the right precordial leads in ASD has been variousI> termed “incomplete right bundle branch block,“6J2-‘5 “diastolic o\-erload of the right ventricle,“‘6 and “right ventricular outflolv tract hypertroph!..“z
712
defects
Cardiac catheterization studies in ASD have demonstrated that there is no delay in the onset of right \zentricular systole, such as would occur if there were a true right bundle branch block.‘7 Many of the recent studies favor dilatation and/or hypertrophy of the crista supraventricularis region of the right ventricle as the erplanation for this pattern.2J,‘7J8 The rSR’ pattern is usually associated with normal or slightly elevated right ventricular and pulmonary arterial pressures. lb.3,li If pulmonary hypertension supervenes and concentric right ventricular hypertrophy develops, the right precordial leads often display a qR or Rs configuration.‘b~3~‘7~‘s The occurrence of a qR or Rs pattern might also suggest the presence of some associated anomaly, such as pulmonic stenosis or even mitral stenosis.6 The electrocardiographic pattern which has been found to be nearly pathognomonic of A-V cushion defects is left axis deviation (LAD) in the standard leads with the rSR’ pattern in the right precordial leads. The LrZD of the early QRS forces is usually in the range of 0 to -60 degrees,4j’Q whereas the mean QRS axis usually ranges from - 60 to * 180 degrees.1Z,6 Some workers have attributed the L&ID to left ventricular enlargement secondary to the mitral illsufficiency,“,zo,zl whereas others ha\.-e favored a parietal wall conduction defect’” or a congenital aberration of the left bundle branch system.3-6 Burchell, DuShane and Brandenburg4 have presented a critical review and rather convincing evidence in favor of the latter explanation. This distinctive electrocardiographic pattern of LAD has been described in 80 to 100 per cent of A-\’ cushion defects.‘&,2 3~9 Other workers, however, ha\e not found such a high incidence of L.4D, and ha\-e even encountered right axis deviation (RAD) in some cases.7,la Burchell and associates’ have encountered some cases of paitial or incomplete forms of II\-\’ cushion defects without the typical electrocardiographic pattern, but no cases of the complete form without the characteristic electrocardiogram. In =\-V cushion defects the right precordial leads may also display complete right bundle branch block or qR, Rs, rSr’, or rS patterns which are not diagnostically different from those of ASD of the ostium secundum type. In the presence of mitral insufficient)the left precordial leads may or may not demonstrate left ventricular overloading or hypertrophy. Atriovcntrirular cushion defects have
Anzotutions
beeu found to manifest q waves in left precordial leads twice as commonly (70 per cent) as do atria1 septal defects of the ostium secundum type (35 per cent).1”,4 The QRS xector loop in ;\SD of the ostium secundum type rotates clockwise in the frontal plane and is oriented below the isoelectric line (0 to 180 degrees), to the right and anteriorly.‘-l~?z ‘l‘he QRS vector loop in A-V cushion defects rotates c.onnterclocl;~vise in the frontal plane and is oriented superior to the isoelectric line (0 to 180 degrees), to the left and posteriorly.2-4~22 A horizontal ligure-ofeight pattern oriented along the isoelectric line, lvith the initial part of the loop rotating counterc-lockwlse, also has been described in .A-\- cushion (,efects,‘“.4-” The ventricular gradient (c) in ostium secundum defects tends to be \-ertical and oriented to the left of the mean QRS (&ns), whereas in A-\’ cushion defects. c is horizontal and to the rieht of &ns.? .~_“. Prolongation of the P-R interval occurs more c.ommonly in A-V cushion defects than in ostium secuudum defects.lbm” Atrial fibrillation has been described in about 10 per cent of the cases of .\SD,‘“J3 being uncommon in the young but more frequent in older persons.23 .Atrial fibrillation is like\vise uncommon iu A-V cushion defects.?s3 Tall I’ waves in Leads II, III, and a\-~, are surprising11 infrequent,2~“~14 although diphasic P v,yaves in Lead \:I (\‘?j which suggest right atrial enlargement have been described in about 50 per cent of the cases of hoth ASD and A-V cushion defects.ln,2 In patients with common atrium, LAD and counterclockwise rotation of the QRS loop identical to that in .X-V cushion defects have been encountered.5.6 .\nother interesting feature noted by Keith5 in cases of common atrium has been a P-wave ask of -60 degrees, together with a short P-R iuterval. It is worth emphasizing that ASD of the ostium secundum type may occur with a normal prerordial electrocardiogram (without the rSR’ pattern), with ;I normal axis, and, rarely, with LAD.‘“-3 The frontal I-ector ma\- also occasionally be oriented superiorI>above the -isoelectric line or horizontally in a figureof-eight pattern and have counterclockwise rotation.1b+6 Of considerable interest has been the observation that ostium secundum defects with acquired mitral insufficiency do not display L.qD but may show RAD and clockwise rotation of the frontal vector.4,5 Atrioventricular cushion defects occasionally ma! display a normal QRS axis and rarely even have 1~,4r)3,1 and c-lo&wise rotation of the frontal vector loop. Patients lvith pulmonic stenosis, patent ductus arteriosus, \-entriculnr septal defects, or total anomalous I-enous return present occasionally an electrocardiographic pattern which resembles that characteristically found in .\SD of the ostium secuudum type.6.F,21 Other types of congenital heart disease that may occasionally simulate the electrocardiographic pattern described in A-\’ cushion defects include: (1) VSD, especially those located posteriorly beneath the seotal triru~oid leaflet”: (2) combined ostium secund*um defect and VSDj,?‘; i3) origin of both the aorta and pulmonary artery from the right \‘en-
713
triclez6; (4) persistent truncus arteriosuslg; (5) single ventriclelg; (6) endocardial tibroelastosis.1” Cases of tricuspid atresia display LXD, but the initial forces, although horizontal, are not so leirward as in .A-\’ cushion defects, and the right prycordial leads do not exhibit the rSR’ pattern.‘” In conclusion, it appears that although there at-e significant exceptions, the electrocardiogram usualI! \vill permit a distinction to be made bctxveen ostillm secundum and .2-V cushion defects. Kulph C. Swtt, JI.L). 1.niwsify o,f Cinrimtrti Collcgc of Nediciue Cincinnati. Ohio REFERESCES la. Toscano-Barbosa, E., Brandenburg, IX. O., and Burchell, H. B.: Electrocardiographic studies of cases with intracardiac malformations of the atrioventricular canal, I’roc. Staff Meet. hlayo Clin. 31:.513, 1956. lb. Toscano-Barbosa, E., Brandenburg, Ii. O., and Swan, H. J. C.: Atrial septal defect. The electrocardiog;am and its hemodynaruic correlation in 100 moved cases. .4m. 1. Cardiol. 2:698. 1958. ’ 2. Burch, G. E., and DePasquale, X.: The electrocardiogram and ventricular gradient in atrial septal defect, AM. HEART J. 58:190, 1959. 3. Pryor, R., Woodwark, G. hr., aud Blount, S. G., Jr.: Electrocardiographic changes in atrial septal defects: ostium secundum defect versus ostium primum (endocardial cushion) defect, AM. HEART J. 58:689, 1959. 4. Burchell, H. B., DuShane, J. W., and Brandenburg, Ii. 0.: The electrocardiogram of patients with atrio\-entricular cushion defects (defects of the atrioventricular ranxl‘~, Xm. J. Card;ol. 6:575, 1960. .5 . Evans, J. R., Rowe, R. D., and Keith, J. U.: The clinical diagnosis of atrial septal defect in children, 1Zm. J. Med. 30:34S, 1961. 6. DuShane, J. \V., Weidman, \V. II., Brandcnburg, 12. O., and Kirklin, J. \I’.: Differentiation of interntrial com~nuIlications by clinical methods: ostium secundum, ostium primum, common atrium, and total anomalous pulmonar) venous connection, Circulation 21:.163, 1060. A. C.. and Ellison. R. G.: Diacnusit; 7. \Vitham. of ostium primum defects of the atrial septum, Am. J. Med. 22:593, 1957. I’.: The diagnosis of persistent ostium 8. h#Iounsey, Brit. Heart J. 20:270, 1958. primum, 9. Bedford, D. E.: The anatomical types of atrial septal defect: their incidence and clinical diagnosis, .1m. J. Cardiol. 6:568, 1960. C. S., and Edwards, J. E.: lk\eiop10. \\‘akai, mental and pathologic considerations in pcrsistent common atrioventricul,N canal, I’roc. Staff Meet. Mayo Clin. 3147, 1956. 11. Campbell, XI., and Missen, G. -1. Ii.: l
714
13.
14.
15.
16.
17.
18.
Annotations
Taussig, H. B.: Congenital malformations of the heart, 1’01. II, ed. 2, Cambridge, 1960, Harvard University Press, published for The Commonwealth Fund. Barber, J. M., Magidson, O., and Wood. I’.: Atria1 septal defect, with special reference to the electrocardiogram, the pulmonary artcr! pressure and the second heart sound, Brit. Heart J. 12:277, 1960. Sodi-Pallares, D., Pileggi, F., Cisneros, F., Ginefra, P., Portillo, B., Medrano, G., and Bisteni, A.: The mean manifest electric_al axis of the ventricular activation process (AQRSI in congenital heart disease: a new approach in electrocardiographic diagnosis, AM. HEART J. 55:681, 1958. Cabrera, E., and Gaxiola, A.: rl critical reevaluation of systolic and diastolic overloading patterns, Prog. Cardiovas. Dis. 2:219, 1959. Walker, W. J., Mattingly, T. it:., Pollock, B. E., Carmichael, D. B., Inmon, ‘1‘. W’., and Forrester, R. H.: Electrocardiographic and hemodynamic correlation in atria1 septal defect, ‘4~. HKZRT J. 52:547, 1956. Martins de Oh\-cira, J., and Zimmerman, II. -4.: The electrocardiogram in interatrial septal defects and its correlation with hemodynamics, AM. HIZ~RT J. 55:369, 1958.
Acute
cmgioneurotic
edemu
Contemporary literature is barren of references to this particular syndrome. Even Luisada’s massive Cardiology’ set has nothing on this topic. Although rare, this condition does exist, and its salient features are worth discussing. Dr. F. Tice diagnosed a case when I was his resident at the Cook County (Chicago, Ill.) Hospital. I have been searching for them since, and in this report, I present my third case. The young lady in question is now a 23-year-old medical student. She is married and has no children. Since infancy, she has been under constant, careful medical supervision because of various food allergies and asthma. Her family physician thought she had an attack of rheumatic fever when she was 6 years old. He sent her to Arizona, where she spent several years at a boarding school. No doctor ever recorded a cardiac murmur or any other evidence of cardiac pathology. Since her return to New York. she has been relatively free of symptoms but has continued under close medical observation. In 1958, before entering medical school, she underwent a battery of exhaustive tests. Since everything was considered to be normal, she was permitted to undertake the proposed course, Besides infrequent, trivial upper respiratory infections, the young lady has stayed well. Her last physical check-up was in the summer of 1960.
19.
Grant, R. l’., Sanders, K. J ., %Iorro\r, .\. (G.. and Braunm-ald, E.: Symposium on diagnostic methods in the study of Icft-to-right &unts. Circulation 16:791, 1957. 20. Blount, S. G., Jr., Balchum, 0. J., .md Gellsilli. G.: The persistent ostium primum atria1 septal defect, Circulation 13:499, 1956. 11. LIilnor, \V. K., and Bertrand, C. A.: The clcctrocardiogram in atria1 septal defect. A study of twenty-four cases, with obser\-ations on the RSR’-VI pattern, Am. J. bled. 22:223, 1957. 22. Burch, G. E., and DePasquale, S.: The spatial \-ectorcartlioaram in proved congenital atri,d septal defecty.4.w. Hr;.,i~r J. 58:319, 1959. 23. \Vood. I’.: Diseases of the heart and circulation. ccl. 2, Philadelphia, 1956, J. B. Lippincott Company. 24. Espino-\-ela, J.. Muracl-Netto, S., and Kubio.-2lvarez, \..: Differential diagnosis betweet) persistent atriovcntricular canal and the conbination of atria1 and ventricular septal defects, .Am. J. Cardiol. 6:589, 1960. 2.5. Neufeld, N. H., DuShane, J. \\‘., \Vood, 1-:. Il., Kirklin, J. W., and Edwards, J. E.: Origin of both great vessels from the right ventricle. I. \%‘ithout pulmonary stenosis, Circulation 23:399, 1961.
of the mitral
valve
On Thanksgiving Day of 1960, the patient woke up with a slight cold, but this did not prevent her from preparing the usual Thanksgiving meal. After washing the dishes and cleaning ~rp, she felt tired and so went to bed shortly after 8 P.M. However. the sense of fatigue became progressively worse, and she became very conscious of a pounding, rapid heart beat. At 9 P.M., when I saw her, she was sitting up in bed, badly frightened, and fighting to catch her breath. The pulse was 106, even, and full; oral temperature was 98°F.; the blood pressure was 94/62 mm. Hg; the lungs were still dry and clear, with excellent expansion. The heart presented an amazingly forceful diastolic murmur with presystolic accentuation; also, a maximal systolic murmur was heard; the apical pulsation was m,rrkecl and a diastolic thrill was felt. Because of the positive knowledge of the absence of all antecedent cardiac pathology, acute edema of the mitral valve was diagnosed. In the expectation of a rapid subsidence of symptoms, she was reassured maximally, given a sedative hypnotic c,;rpsule and an alcoholic beverage drink. She relaxed and fell asleep; I did tell the husband to call me immediately if her symptoms took a turn for the worse. I made clear that radical measures up to emergency cardiac operation could become imprr:ltive if closure of the mitral valve threatened. In the morning the patient was entirely romfort-