Case reports Atypical phonocardiographic a patient with congenital
presentation of aortic insufficiency
Nicholas Kerin, M.D.* Berian Davis, M.D. Harold Schwartz, M.D. Cleveland, Ohio
The clinical and phonocardiographic findings of aortic regurgitation are straightforward and are surprisingly consistent whatever the etiology may be. Recently, we had the opportunity to study a case which revealed some puzzling auscultatory features. Case report A man, 24 years of age, was admitted to the Veterans Administration Hospital in Cleveland on Sept. 14, 1971, for cardiac evaluation. The discharge examination from the Air Force in June, 1971, revealed a diastolic murmur presumably due to aortic regurgitation. He lived a normal life and denied any history of scarlet fever, rheumatic fever, or syphilis. Because of difficulties in obtaining life insurance, he was admitted for cardiac catheterization. His family history was noncontributory. Physical examination revealed an alert, thin patient. His pulse rate was 75 beats per minute and regular. His blood pressure was 114/76 mm. Hg. Neck veins were not distended and the lungs were clear to percussion and auscultation. The apical impulse was active and located in the fifth left intercostal space at the midclavicular line. Heart sounds were normal. A loud, snapping ejection sound was heard in both infraclavicular fossae. A loud protodiastolic click followed by a high-pitched, blowing diastolic decrescendo murmur, grade III/IV, was heard in the second right intercostal space and along the left sternal border. The peripheral pulses in the upper and lower extremities were equal. The carotid pulse was bounding but no other peripheral arterial features of aortic incompetence were observed. The remainder of the physical examination was within normal limits. Laboratory findings. Complete blood count, urea nitrogen, fasting blood sugar, glutamic oxaloacetic transaminase, From the Cardiac Laboratory, Medical Service, Veterans Administration Hospital, and the Department of Medicine, Case Western Reserve University, Cleveland. Received for publication Aug. 20, 1973. Reprint requests to: Nicholas Kerin, M.D., Cardiopulmonary Laboratory, Mt. Sinai Hospital, 1800 E. 105 St., Cleveland, Ohio 44106. *Formerly, Fellow in Cardiology. Veterans Administration Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio. Present address: Assistant in Medicine, Cardiopulmonary Laboratory. Mt. Sinai Hospital, Cleveland, Ohio 44106.
July, 1975, Vol. 90, No. 1, pp. 75-77
cholesterol, uric acid, and chest roentgenogram were normal. The resting electrocardiogram (ECG) was within normal limits. Simultaneous recording of external phonocardiogram (PCG) with right carotid pulse tracing (CPTl (Fig. 1) was recorded. An ejection sound was recorded at the third left intercostal space at the left sternal border 30 msec. after the beginning of the carotid upstroke. The two major components of the second sound (A and P) are also clearly seen. A prominent protodiastolic click (DC) occurs 60 msec. after A2 and is followed by a high-medium frequency ( 100 to 500 c.p.s. 1 decrescendo diastolic murmur. This diastolic click occurs 10 msec. after the 0 point and precedes the peak of the rapid filling wave of the left apex cardiogram (ACG). Following premeditation with 100 mg. of Seconal, a right and left cardiac catheterization was performed, using the right brachial artery and its companion vein. Pressures in the right side of the heart were normal. Left-heart catheterization revealed a normal left ventricular pressure of 1 lo/80 mm. Hg. No systolic gradient across the pulmonic or aortic valve or diastolic gradient across the mitral valve were recorded. A left ventriculogram was performed in a 30” right anterior oblique position and showed a normal end-diastolic volume. Myocardial contractions were extremely vigorous and effected complete ventricular emptying with a normal end-systolic volume. The mitral valve opened normally. There was no evidence of left-ventricular outflow tract obstruction or mitral insufficiency. An aortic root angiogram was performed in a 60” left anterior oblique position. This demonstrated mild aortic insufficiency with some unusual features. While there was regurgitation of contrast material almost immediately after valve closure, this appeared to be maximal slightly later in diastole. The regurgitant stream was seen as a small jet of contrast material which was also unusual in that the jet was directed eccentrically. It appeared to originate from the anterior commissure between the right and left coronary cusps and was directed even more anteriorly rather than vertically in the outflow tract. It was thought that this slightly delayed and eccentric regurgitant stream could be consistent with a cusp prolapse, occurring slightly after the init.ial valve closure. None of the valve leaflets could be clearly identified as demonstrating prolapse. A second possibility is that of a fenestration in an aortic leaflet. An intracardiac phonocardiogram was recorded using a No. 5 F Stveltingis single-lumen phonocatheter. No murmur or protodiastolic click were recorded in the right ventricle. Intracardiac phonocardiogram
American
Heurt
Journal
75
Kerin,
Davis,
and
Schwartz
Fig. 1. External electrocardiogram murmur.
phonocardiogram (L,) and carotid
Fig. 2. Intracardiac phonocardiogram simultaneous L, electrocardiogram
recorded over pulse tracing,
third left intercostal space showing the protodiastolic
obtained in the outflow tract showing the aortic protodiastolic
of the left ventricle revealed a very prominent protodiastolic click which was followed by a diastolic murmur occupying mid- and late diastole. The murmur became more prominent as the catheter was withdrawn from inflow to outflow tract (Figs. 2 and 3) reaching a maximum intensity above the aortic valve (Fig. 3).
Discussion
The normal second sound consists of two separate components which are related to closure of the two semilunar valves’ and varies with respiration between 40 and 80 msec.2 In addition to these two components of the second sound, other vibrations (x and y compo-
76
(3 LICS), with simultaneous click followed by a diastolic
of the left ventricle (below aortic valve) click followed by a diastolic murmur.
with
nents) have been noted.3 The x-component occurs in the early or middle portion of the protodiastolic period.3 This component was thought to be a vibration produced by the relaxation of the aortic and ventricular walls as systolic contraction ceased.l, 5 In an experimental study in dogs, Mori and co-worker@ found a y-component following the pulmonary component by 40 to 120 msec. The vibration was recorded in aortic and pulmonary intravascular phonocardiograms as well. The mechanism of production of the y-component is uncertain. Coelho and Faleiro’ were able to correlate and to suggest that this y-compo-
July, 1975, Vol. 90, No. 1
&vpicnl
nent is a pulmonic protodiastolic click. This click was found in patients with pulmonary hypertension or increased pulmonary blood flow due to left-to-right shunts.? This case presents an unusual protodiastolic click probably originating from the aortic valve. The click was not recorded in the right ventricle. Its maximum recording was above and below the aortic valve, and it was followed by a diastolic murmur. The aortogram showed a moderate regurgitation of dye which appears to be maximal slightly after the beginning of diastole. These findings may explain why the click and the murmur do not start with the closure of the aortic valve. It was thought that this delayed and eccentric regurgitant stream could be consistent with a cusp prolapse or fenestrated valve. The possibility of artifacts produced by an intracardiac catheter was excluded in our case because of a similar recording which was obtained by external PCG. Furthermore, it was easily heard by the examiner. The differentiation between this aortic protodiastolic click and the mitral opening snap or the third sound is based on the fact that this sound does not coincide with the 0 point or the peak of rapid filling wave of the left apex cardiogram. It is not pulmonic or right ventricular in origin because it was not recorded in the right ventricle. It is possible that this aortic protodiastolic click is the same as the x-vibration found by Wiggers,‘3 Orias and Braun-Henendes,’ and Rappaport and Sprague.’ A search of the literature failed to reveal any reports of similar auscultatory findings.
ir~suficiertqv
REFERENCES 1. 9u. 3.
4.
5.
A patient with congenital aortic regurgitation in whom there was an abnormal aortic protodiastolic click and mid- and late-diastolic murmur is described.
7.
Journal
nortic
Fig. 3. Intracardiac phonocardiogram of the aorta (above aortic valve) with simultaneous (L1) electrocardiogram showing the aortic diastolic click followed by a prominent diastolic murmur. (All the recordings were obtained u..ine the same sensitii-ity.1
6.
Heart
of congenital
A
Summary
American
presentution
Leatham, A., and Towers, M.: Splitting of the second heart sound in health, Br. Heart J. 13:575, 1951. Leatham, A.: Splitting of the first and second heart sounds, Lancet 2:607, 1954. Wiggers. C. J.: Studies on the consecutive phases of the cardiac cycle. I. The duration of the consecutive phases of the cardiac cycle and the criteria for their precise determination, Am. J. Physiol. 56:415, 1921. Orias, O., and Braun-Henendes, E.: The heart sounds in normal and pathological conditions, London. 1939. Oxford University Press, p. 82. Rappaport, M. B., and Sprague, H. B.: The graphic registration of the normal heart sounds, AM. HEART d. 23:591, 1942. Mori, M., Shah, P. M., MacCanon, D. M.. and Luisada. A. A.: Hemodynamic correlates of the various components of the second heart sound, Cardiologia 44:65, 19fi4. Coelho, E. M., and Faleiro, L. L.: The pulmonary protodiastolic click studied by intracardiac phonocardiography, Acta Cardiol. 26:277, 1971.
77
presentation of aortic insufficiency
Nicholas Kerin, M.D.* Berian Davis, M.D. Harold Schwartz, M.D. Cleveland, Ohio
The clinical and phonocardiographic findings of aortic regurgitation are straightforward and are surprisingly consistent whatever the etiology may be. Recently, we had the opportunity to study a case which revealed some puzzling auscultatory features. Case report A man, 24 years of age, was admitted to the Veterans Administration Hospital in Cleveland on Sept. 14, 1971, for cardiac evaluation. The discharge examination from the Air Force in June, 1971, revealed a diastolic murmur presumably due to aortic regurgitation. He lived a normal life and denied any history of scarlet fever, rheumatic fever, or syphilis. Because of difficulties in obtaining life insurance, he was admitted for cardiac catheterization. His family history was noncontributory. Physical examination revealed an alert, thin patient. His pulse rate was 75 beats per minute and regular. His blood pressure was 114/76 mm. Hg. Neck veins were not distended and the lungs were clear to percussion and auscultation. The apical impulse was active and located in the fifth left intercostal space at the midclavicular line. Heart sounds were normal. A loud, snapping ejection sound was heard in both infraclavicular fossae. A loud protodiastolic click followed by a high-pitched, blowing diastolic decrescendo murmur, grade III/IV, was heard in the second right intercostal space and along the left sternal border. The peripheral pulses in the upper and lower extremities were equal. The carotid pulse was bounding but no other peripheral arterial features of aortic incompetence were observed. The remainder of the physical examination was within normal limits. Laboratory findings. Complete blood count, urea nitrogen, fasting blood sugar, glutamic oxaloacetic transaminase, From the Cardiac Laboratory, Medical Service, Veterans Administration Hospital, and the Department of Medicine, Case Western Reserve University, Cleveland. Received for publication Aug. 20, 1973. Reprint requests to: Nicholas Kerin, M.D., Cardiopulmonary Laboratory, Mt. Sinai Hospital, 1800 E. 105 St., Cleveland, Ohio 44106. *Formerly, Fellow in Cardiology. Veterans Administration Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio. Present address: Assistant in Medicine, Cardiopulmonary Laboratory. Mt. Sinai Hospital, Cleveland, Ohio 44106.
July, 1975, Vol. 90, No. 1, pp. 75-77
cholesterol, uric acid, and chest roentgenogram were normal. The resting electrocardiogram (ECG) was within normal limits. Simultaneous recording of external phonocardiogram (PCG) with right carotid pulse tracing (CPTl (Fig. 1) was recorded. An ejection sound was recorded at the third left intercostal space at the left sternal border 30 msec. after the beginning of the carotid upstroke. The two major components of the second sound (A and P) are also clearly seen. A prominent protodiastolic click (DC) occurs 60 msec. after A2 and is followed by a high-medium frequency ( 100 to 500 c.p.s. 1 decrescendo diastolic murmur. This diastolic click occurs 10 msec. after the 0 point and precedes the peak of the rapid filling wave of the left apex cardiogram (ACG). Following premeditation with 100 mg. of Seconal, a right and left cardiac catheterization was performed, using the right brachial artery and its companion vein. Pressures in the right side of the heart were normal. Left-heart catheterization revealed a normal left ventricular pressure of 1 lo/80 mm. Hg. No systolic gradient across the pulmonic or aortic valve or diastolic gradient across the mitral valve were recorded. A left ventriculogram was performed in a 30” right anterior oblique position and showed a normal end-diastolic volume. Myocardial contractions were extremely vigorous and effected complete ventricular emptying with a normal end-systolic volume. The mitral valve opened normally. There was no evidence of left-ventricular outflow tract obstruction or mitral insufficiency. An aortic root angiogram was performed in a 60” left anterior oblique position. This demonstrated mild aortic insufficiency with some unusual features. While there was regurgitation of contrast material almost immediately after valve closure, this appeared to be maximal slightly later in diastole. The regurgitant stream was seen as a small jet of contrast material which was also unusual in that the jet was directed eccentrically. It appeared to originate from the anterior commissure between the right and left coronary cusps and was directed even more anteriorly rather than vertically in the outflow tract. It was thought that this slightly delayed and eccentric regurgitant stream could be consistent with a cusp prolapse, occurring slightly after the init.ial valve closure. None of the valve leaflets could be clearly identified as demonstrating prolapse. A second possibility is that of a fenestration in an aortic leaflet. An intracardiac phonocardiogram was recorded using a No. 5 F Stveltingis single-lumen phonocatheter. No murmur or protodiastolic click were recorded in the right ventricle. Intracardiac phonocardiogram
American
Heurt
Journal
75
Kerin,
Davis,
and
Schwartz
Fig. 1. External electrocardiogram murmur.
phonocardiogram (L,) and carotid
Fig. 2. Intracardiac phonocardiogram simultaneous L, electrocardiogram
recorded over pulse tracing,
third left intercostal space showing the protodiastolic
obtained in the outflow tract showing the aortic protodiastolic
of the left ventricle revealed a very prominent protodiastolic click which was followed by a diastolic murmur occupying mid- and late diastole. The murmur became more prominent as the catheter was withdrawn from inflow to outflow tract (Figs. 2 and 3) reaching a maximum intensity above the aortic valve (Fig. 3).
Discussion
The normal second sound consists of two separate components which are related to closure of the two semilunar valves’ and varies with respiration between 40 and 80 msec.2 In addition to these two components of the second sound, other vibrations (x and y compo-
76
(3 LICS), with simultaneous click followed by a diastolic
of the left ventricle (below aortic valve) click followed by a diastolic murmur.
with
nents) have been noted.3 The x-component occurs in the early or middle portion of the protodiastolic period.3 This component was thought to be a vibration produced by the relaxation of the aortic and ventricular walls as systolic contraction ceased.l, 5 In an experimental study in dogs, Mori and co-worker@ found a y-component following the pulmonary component by 40 to 120 msec. The vibration was recorded in aortic and pulmonary intravascular phonocardiograms as well. The mechanism of production of the y-component is uncertain. Coelho and Faleiro’ were able to correlate and to suggest that this y-compo-
July, 1975, Vol. 90, No. 1
&vpicnl
nent is a pulmonic protodiastolic click. This click was found in patients with pulmonary hypertension or increased pulmonary blood flow due to left-to-right shunts.? This case presents an unusual protodiastolic click probably originating from the aortic valve. The click was not recorded in the right ventricle. Its maximum recording was above and below the aortic valve, and it was followed by a diastolic murmur. The aortogram showed a moderate regurgitation of dye which appears to be maximal slightly after the beginning of diastole. These findings may explain why the click and the murmur do not start with the closure of the aortic valve. It was thought that this delayed and eccentric regurgitant stream could be consistent with a cusp prolapse or fenestrated valve. The possibility of artifacts produced by an intracardiac catheter was excluded in our case because of a similar recording which was obtained by external PCG. Furthermore, it was easily heard by the examiner. The differentiation between this aortic protodiastolic click and the mitral opening snap or the third sound is based on the fact that this sound does not coincide with the 0 point or the peak of rapid filling wave of the left apex cardiogram. It is not pulmonic or right ventricular in origin because it was not recorded in the right ventricle. It is possible that this aortic protodiastolic click is the same as the x-vibration found by Wiggers,‘3 Orias and Braun-Henendes,’ and Rappaport and Sprague.’ A search of the literature failed to reveal any reports of similar auscultatory findings.
ir~suficiertqv
REFERENCES 1. 9u. 3.
4.
5.
A patient with congenital aortic regurgitation in whom there was an abnormal aortic protodiastolic click and mid- and late-diastolic murmur is described.
7.
Journal
nortic
Fig. 3. Intracardiac phonocardiogram of the aorta (above aortic valve) with simultaneous (L1) electrocardiogram showing the aortic diastolic click followed by a prominent diastolic murmur. (All the recordings were obtained u..ine the same sensitii-ity.1
6.
Heart
of congenital
A
Summary
American
presentution
Leatham, A., and Towers, M.: Splitting of the second heart sound in health, Br. Heart J. 13:575, 1951. Leatham, A.: Splitting of the first and second heart sounds, Lancet 2:607, 1954. Wiggers. C. J.: Studies on the consecutive phases of the cardiac cycle. I. The duration of the consecutive phases of the cardiac cycle and the criteria for their precise determination, Am. J. Physiol. 56:415, 1921. Orias, O., and Braun-Henendes, E.: The heart sounds in normal and pathological conditions, London. 1939. Oxford University Press, p. 82. Rappaport, M. B., and Sprague, H. B.: The graphic registration of the normal heart sounds, AM. HEART d. 23:591, 1942. Mori, M., Shah, P. M., MacCanon, D. M.. and Luisada. A. A.: Hemodynamic correlates of the various components of the second heart sound, Cardiologia 44:65, 19fi4. Coelho, E. M., and Faleiro, L. L.: The pulmonary protodiastolic click studied by intracardiac phonocardiography, Acta Cardiol. 26:277, 1971.
77
