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Differential Diagnosis of Pulmonic Stenosis by Means of Intracardiac Phonocardiography

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Differential Diagnosis of Pulmonic Stenosis by Means of Intracardiac Phonocardiography Differential Diagnosis of Pulmonic Stenosis by Means of Intracardiac Phonocardiography Tadashi KAMBE, M.D., Tadayuki KATO, M.D., Norio HIBI, M.D., Yoichi FUKUI, M.D., Takemi ARAKAWA, M.D., Kinya NISHIMURA,M.D., Hiroshi TATEMATSU,M.D., Arata MIWA, M.D., Hisao TADA, M.D., and Nobuo SAKAMOTO,M.D. SUMMARY The purpose of the present paper is to describe the origin of the systolic murmur in pulmonic stenosis and to discuss the diagnostic pos- sibilities of intracardiac phonocardiography. Right heart catheterization was carried out with the aid of a double- lumen A.E.L. phonocatheter on 48 pulmonic stenosis patients with or without associated heart lesions. The diagnosis was confirmed by heart catheterization and angiocardiography in all cases and in 38 of them, by surgical intervention. Simultaneous phonocardiograms were recorded with intracardiac pressure tracings. In valvular pulmonic stenosis, the maximum ejection systolic murmur was localized in the pulmonary artery above the pulmonic valve and well transmitted to both right and left pulmonary arteries, the superior vena cava, and right and left atria. The maximal intensity of the ejection systolic murmur in infundibular stenosis was found in the outflow tract of right ventricle. The contractility of the infundibulum greatly contributes to the formation of the ejection systolic murmur in the outflow tract of right ventricle. In tetralogy of Fallot, the major systolic murmur is caused by the pulmonic stenosis, whereas the high ventricular septal defect is not responsible for it. In pulmonary branch stenosis, the sys- tolic murmur was recorded distally to the site of stenosis. Intracardiac phonocardiography has proved useful for the dif- ferential diagnosis of various types of pulmonic stenosis. Additional Indexing Words: Right heart catheterization Pulmonic valvular stenosis Infundibular stenosis Pulmonary branch stenosis NTRACARDIAC phonocardiography, introduced by Yamakawa in 1953,1) was a breakthrough in the realm of clinical phonocardiography. Since the advent of this method, it has served as a useful tool for differential diagno- From the Third Department of Internal Medicine, Nagoya University, School of Medicine , Tsurumai-cho 65, Showa-ku, Nagoya 466, Japan. Received for publication December 8, 1975. 691 Jap.N Heart J. 692 KAMBE, ET AL. ovember, 1976 sis, thus contributing to the analysis of the origin of the cardiac murmurs and sounds in heart chambers or great vessels. In general, conventional external phonocardiography is non-invasive and simple, but may not always correctly reflect the acoustic events occurring in the cardiac chambers or great vessels. In contrast, intracardiac phono- cardiography can localize the origin of cardiac murmurs or sounds in a wide variety of cardiovascular diseases and may detect the result of small eddies that are usually inaudible on the chest surface. Since the introduction of intracardiac phonocardiography, many pub- lications2)-10) have discussed the origin of the systolic murmurs in pulmonic stenosis. Recently, Lequime et al11) reported on the origin of systolic mur- murs in pulmonic stenoses of different types, comparing intracardiac phono- cardiography with angiocardiography. The purpose of the present study is to describe the origin and transmission of the systolic murmurs in pulmonic stenosis of various types, and to discuss the diagnostic possibilities of intracardiac phonocardiography. MATERIAL AND METHODS Right heart catheterization using intracardiac phonocardiography was carried out on 48 pulmonic stenosis patients with or without associated cardiac anomalies. The age ranged from 3 to 41 years. The cardiac diagnosis of the subjects, as shwon in Table I, was confirmed by heart catheterization and angiocardiography. Of Table I. Materials Abbreviations: PS: pulmonic stenosis, ASD: atrial septal defect, VSD: ventricular septal defect, PLSVC: persistent left superior vena cava, PAPVD: partial anomalous pulmonary venous drainage, T/F: tetralogy of Fallot, Pul.: pulmonic. * The manufacturer of the phonocatheter is A .E.L. (American Electric Laboratories Inc.), P.O. Box 552, Lansdale, PA. 19446, U.S.A. Vol.17 No.6 INTRACARDIAC PHONOCARDIOGRAPHY 693 the 48 patients, the diagnosis of 38 was eventually verified by surgical intervention. A double-lumen phonocatheter of A.E.L.,* with barium titanate on the tip, was introduced into the left axillary vein and advanced to the pulmonary artery via superior vena cava, right atrium and ventricle. All procedures of heart catheteriza- tion were performed in the sedated and postabsorptive state after informed consent was obtained. In the majority of cases, a simultaneous recording of intracardiac and external phonocardiograms was made in conjunction with intracardiac pres- sure tracing with the aid of a polygraph (Fukuda-denshi EMR-100R) and a photo- graphic recorder (Sanei-sokki 100A). A pressure transducer TM-1 was used for the pressure tracing and a contact microphone (Fukuda-denshi MA-250 or PM-1) was applied to the chest surface where the maximum murmur was audible. Paper speed was 100mm/sec in the majority of cases, and intracardiac murmurs were usually investigated in the pulmonary artery of both sides from the main trunk to the periphery, right ventricle, right atrium and superior vena cava. In 9 cases the left atrium was entered and a systolic murmur was recorded. During the recording of systolic murmurs, the phonocatheter was manipulated with great care and the withdrawal from the pulmonary artery into the right ventricle was repeated very slowlywith caution, since the outflow tract may produce artificial extrasounds. RESULTS I. Valvular Pulmonic Stenosis In all subjects with valvular pulmonic stenosis, the maximum ejection Fig.1. A simultaneous recording of intracardiac and external phono- cardiograms with pressure tracing of main pulmonary artery in a 19-year-old male (N.S.). The intracardiac phonocardiogram indicates a localization of the maximum ejection systolic murmur in the pulmonary artery just above the pulmonic valve. Abbreviations: I-PCG=intracardiac phonocardiogram , PCG=external phonocardiogram, PA=pulmonary artery, 2L=the second left intercostal space, SM=systolic murmur, IIP=the pulmonary component of the second heart sound. Jap. HeartJ. 694 KAMBE, ET AL. November, 1976 Fig.2. A 19-year-old female with moderate pulmonic stenosis and func- tional infundibular stenosis. Note the ejection systolic murmur in the pulmo- nary artery just above the valve (upper panel), whereas a late systolic murmur is found in the outflow tract of right ventricle (lower panel). The latter is thought to be due to the functional infundibular stenosis. Abbreviations: RV=right ventricle, 3L=the third left intercostal space. Other abbreviations as in Fig.1. systolic murmur was recorded in the pulmonary artery just above the pul- monic valve, radiating from the main pulmonary trunk to the periphery of left and right pulmonary arteries. Fig.1 demonstrates an example of mild valvular stenosis (19-year-old male, N.S.). Simultaneous registration of in- tracardiac and external phonocardiograms was made together with the pres- sure tracing of the main pulmonary artery. The intracardiac phonocardio- gram (I-PCG) indicates a localization of the maximum ejection systolic murmur in the pulmonary artery just above the pulmonic valve. The interval of the second heart sound splitting amounted to 80 msec, and the pressure gradient across the pulmonic valve was 37mmHg in systole. There was no systolic murmur in the outflow tract of right ventricle. Moreover, right Vol.17 INTRACARDIAC PHONOCARDIOGRAPHY 695 No.6 Fig.3. A mode of propagation of systolic murmur from the pulmonary artery to the right atrium (upper panel) and superior vena cava (lower panel) in a 26-year-old female with valvular pulmonic stenosis associated with partial pulmonary venous return draining into left innominate vein through vertical vein. In the right atrium and superior vena cava, there are ejection systolic murmurs which are considered to be propagated from the pulmonary artery. Abbreviation: RA=right atrium, SVC=superior vena cava. Other ab- breviations as in Fig.1. ventriculography did not prove the presence of functional infundibular steno- sis. In moderate or severe valvular pulmonic stenosis, a late systolic murmur was demonstrated in the outflow tract of right ventricle, which corresponded to the findings obtainded by the selective angiocardiography of right ven- tricle. Fig.2 shows a simultaneous registration of intracavitary and external phonocardiograms with intracardiac pressure in a 19-year-old female with moderate valvular pulmonic stenosis and functional infundibular stenosis (Y.S.). The diagnosis was corroborated by open heart surgery, and the systolic pressure gradient across the pulmonic valve was found to be 80mmHg. The intracardiac phonocardiogram revealed that the largest ejection systolic murmur was just above the pulmonic valve, whereas a late systolic murmur Jap. Heart J. 696 KAMBE, ET AL. November, 1976 was found in the outflow tract of right ventricle. The latter can be attributed to a functional infundibular stenosis. In valvular pulmonic stenosis with or without associated heart anomalies, an ejection systolic murmur was also recorded in the superior vena cava in 15 out of 19 cases, and in the right atrium in 18 out of 25 patients. This phenomenon is explained by a transmission from the pulmonary artery, due to their close relationship. Fig.3 illustrates a mode of propagation of systolic murmur from the pulmonary
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