- Email: [email protected]
Isolated congenital right ventricular diverticulum with ventricular premature complexes
480
BRIEF REPORTS
Two of the 4 patients had a patent ductus arteriosus, which is a rather commonly associated finding when complex congenital malformations are explored but, except for its incidence of 50%, there is not enough evidence to consider it part of this syndrome. We suggest that if evidence of a patent ductus arteriosus is found during the course of evaluation of a patient with discrete subaortic stenosis, one should look further for the other malformations forming this morphologic complex.
1. Brandt PWT, Roche AHG, Barratt-Boyes BG. Lowe JB. Radiology of homograft oortic valves. Thorax 1969;24:129-141. 2. Higgins CB, Wexler L. Reversal of dominance of the coronary arterial system in isolated oortic stenosis and bicuspid oortic valve. C&lotion 1975:52:292-2.96. 3. Hutchins GM, Nazarian IH, Bulkley BH. Association of left dominant coronary arterial system with congenital bicuspid oortic stenosis. Am [ Cardial 1978;42:57-59. 4. Robicsek F, Snager PW, Daugherty KH, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Am J Cardiol ma:zxm-zw. 5. Rosenquist GC, Clark EB, McAllister HA, Bharati S, Edwards JE. Increased mitral-aortic separation in discrete subaortic stenosis. Circulation 1979;60:7074.
Isolated Congenital Right Ventricular Diverticulum with Ventricular Premature Complexes
the lesions were associated with other congenital cardiac anomalies, and only 1 case presented with ventricular arrhythmia.5 We describe 4 patients with muscular RV diverticula and no associated cardia anomalies, but with ventricular premature complexes.
KENJI HAMAOKA, MD, PhD TADASHI SAWADA, MD, PhD
The patients’ ages ranged from 5 to 32 years (mean They had been referred to our hospital for further examinations for intermittent palpitation and a precordial murmur. Only 1 patient (patient 1) had a soft systolic ejectional murmur at the left midsternal area. None of the chest roentgenograms revealed abnormal cardiac silhouette or cardiac enlargement. AJJelectrocardiograms showed monofocal ventricular premature complexes, and 1 patient (patient 3) had an intermittent accelerated idioventricular rhythm. Their
C
ongenital right ventricular (RV) diverticulum is a rare disorder in which a localized torus protrudes from the RV free wall due to congenital factors. There are 2 types of diverticula, muscular or fibrous. The lesion has been termed muscular or true diverticulum when it has a normal muscular structure or when it contracts functionally like a normal ventricle. Only 8 cases of muscular RV diverticula have been reported.lm6 In all, From Children’s Research Hospital and Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto 602, Japan. Manuscript received July 27, 1987; revised manuscript received September 18,1987, and accepted September 21.
Case 1
Case 2
8.5).
I
II ..~,.
,,-. 1
-1 /
III-
aVR
Case 3
,+y
Case 4
;.;.,;
~
~ ..,........::.:...:::..::..:..:-.
‘,.
FIGURE 1. Direct tracings from cineangiograms of representative right ventricular diverticula (arrows) in diastole.
FIGURE 2. Frequent monofocal ventricular premature beats (accelerated indioventricular rhythm) in patient 3.
February 15, 1988
ventricular premature complexes were estimated to occur from the RV anterior wall and the pulmonary conus from the QRS patterns of ventricular premature complexes. None of the 4 patients had any findings of ventricular or atrial overloading on the electrocardiograms. Echocardiography revealed no abnormal findings in the cardiac anatomy in any of the 4. In all 4 patients, cardiac catheterization showed normal intracardiac and pulmonary arterial pressures, and no intracardiac shunt. RV angiography showed a diverticulum in the outflow area of the right ventricle (Figure I), which contracted in synchrony with the remainder of the right ventricle. From the angiographic findings, we judged that the ventricular premature complexes in the 4 patients occurred from the diverticula of RV outflow tract. Surgical operation was performed in patient 3, who had an accelerated idioventricular rhythm (Figure 2) that could not be controlled by antiarrhythmic drugs. At operation, the diverticulum was observed in RV outflow tract. Histologically, the resected diverticulum had a normal muscular structure without any cell infiltration or fibrosis. Postoperatively, patient 3 showed normal reguJar sinus rhythm and no ventricular arrhythmia on the electrocardiogram. The 3 other patients had only simple ventricular premature complexes; thus, an operation was not recommended. We judged that the diver-
Influence of Sublingual Nitroglycerin on Diastolic Transmitral Flow Velocities in Normal Subjects BYRON F. VANDENBERG, MD JOHN A. RUMBERGER, PhD, MD RICHARD E. KERBER, MD
P
ulsed Doppler echocardiographic sampling of transmitral diastolic flow velocities provides a noninvasive method for the serial evaluation of left ventricular diastolic functi0n.l A reduction in the peak early filling (E) velocity and an increase in the peak late filling (A) velocity suggests reduced compliance of the left ventric1e.l Similar changes in the velocity-time profile occur after the administration of nitroglycerin,2 since diastolic function may be affected by changes in loading conditions3 independent of intrinsic myocardial relaxation abnormalities4 Our purpose was to From the Cardiovascular Center, Department of Internal Medicine, University of Iowa, Iowa City, Iowa. This study was supported in part by an Ischemic SCOR Grant, HL-32295, and by a Research Service Award HL-07379-01 (Dr. Vandenberg) from the National Institutes of Health, Bethesda, Maryland. Manuscript received July 27, 1987; revised manuscript received and accepted September 16,1987. Dr. Rumberger’s current address: Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota.
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume 61
481
ticula of these 3 patients were of the muscular type, because the patients had normal contractions on angiography. It has recently been noticed that ventricular arrythmias originating in RV outflow tract may be associated with some histologic abnormalities or organic heart lesions. In the 4 patients just described, the lesions were not associated with any other intracardiac anomalies. Each had complained of intermittent palpitation due to ventricular premature complexes or ventricular tachycardia related to the diverticula. The diverticula may have been an ectopic focus for ventricular premature complexes or a reentry circuit. 1. Magrassi P. Chartrand C, Guerin R, Kratz C, Stanley P. True diverticulum of the right ventricle. Two cases associated with teratology of Fallot. Ann Thorac Surg 1980;29:357-363. 2. Carter JB:Van TasselRA, Moller JH, Amplatz K, Edwards JE. Congenital diverticulum of the right ventricle. Associated with pulmonary stenosis and ventricular septal defect. Am J Cardiol 1971;28:478-482. 3. Bharati S. Rowen M, CamarataSJ,Ostermiller WE Jr, Singer M. Lev M. Diverticulum of the right ventricle. Arch Pathol 1975;89:383-386, 4. Copeland J, Higgino C. Hayden W, Stinson EB. Congenital diverticulum of the right ventricle. T Thorac Cardiovosc Sure 1975:70:536-538. 6. Nicod P. Laird WF’. Firth BG, Nicod L, F&r D. Congenital diverticula of the left and right ventricles: 3 cases. Am J Cardiol 1984;53:342-344. 6. Terai M, Ohta F. Kate T. Nakanishi T, Takahashi Y, Minami Y, Satomi G, ando M. Moma K, Takao a, Kurosawa H, Takanashi Y. Imai Y. Two cases of congenital right ventricular diverticulum (in lapanesel. Heart 1985:17:163168.
identify, in normal subjects, Doppler echocardiographic parameters of diastolic function that are not affected by preload reduction with sublingual nitroglycerin. We performed pulsed Doppler echocardiography in 9 healthy volunteers (mean age 27 years, range 24 to 29) with an American Technology Laboratory ultrasonoscope (3.0-MHz transducer). Sampling of transmitral flow velocities was obtained by placing the Doppler gate at the level of the mitral anulus from the apical 4-chamber view of the left ventricle. The transmitral velocity-time profiles were recorded at a paper speed of 100 mm/s. Sublingual nitroglycerin, 0.4 mg, was administered and the recordings were repeated after 3 to 5 minutes if heart rate increased by at least 10% from baseline. If heart rate did not increase by 10% above baseline (suggesting insignificant preload reduction), an additional 0.2 mg sublingual nitroglycerin was given. Thus heart rate, rather than systemic arterial pressure, was used to demonstrate the pharmacologic effect of nitroglycerin. E velocity, time from baseline to E velocity (acceleration time), time from E velocity to baseline (deceleration time), the E velocity-time integral (area under the E velocity-time profile), the A velocity and the E/A velocity ratio were averaged from 5 beats. The recordings were coded and measurements were made without knowledge of whether they were obtained at baseline or after sublingual nitroglycerin administration. Data are expressed as mean f standard deviation. Comparisons of measurements between control and nitroglycerin administration were performed with a paired t test; p <0.05 was considered significant.
BRIEF REPORTS
Two of the 4 patients had a patent ductus arteriosus, which is a rather commonly associated finding when complex congenital malformations are explored but, except for its incidence of 50%, there is not enough evidence to consider it part of this syndrome. We suggest that if evidence of a patent ductus arteriosus is found during the course of evaluation of a patient with discrete subaortic stenosis, one should look further for the other malformations forming this morphologic complex.
1. Brandt PWT, Roche AHG, Barratt-Boyes BG. Lowe JB. Radiology of homograft oortic valves. Thorax 1969;24:129-141. 2. Higgins CB, Wexler L. Reversal of dominance of the coronary arterial system in isolated oortic stenosis and bicuspid oortic valve. C&lotion 1975:52:292-2.96. 3. Hutchins GM, Nazarian IH, Bulkley BH. Association of left dominant coronary arterial system with congenital bicuspid oortic stenosis. Am [ Cardial 1978;42:57-59. 4. Robicsek F, Snager PW, Daugherty KH, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Am J Cardiol ma:zxm-zw. 5. Rosenquist GC, Clark EB, McAllister HA, Bharati S, Edwards JE. Increased mitral-aortic separation in discrete subaortic stenosis. Circulation 1979;60:7074.
Isolated Congenital Right Ventricular Diverticulum with Ventricular Premature Complexes
the lesions were associated with other congenital cardiac anomalies, and only 1 case presented with ventricular arrhythmia.5 We describe 4 patients with muscular RV diverticula and no associated cardia anomalies, but with ventricular premature complexes.
KENJI HAMAOKA, MD, PhD TADASHI SAWADA, MD, PhD
The patients’ ages ranged from 5 to 32 years (mean They had been referred to our hospital for further examinations for intermittent palpitation and a precordial murmur. Only 1 patient (patient 1) had a soft systolic ejectional murmur at the left midsternal area. None of the chest roentgenograms revealed abnormal cardiac silhouette or cardiac enlargement. AJJelectrocardiograms showed monofocal ventricular premature complexes, and 1 patient (patient 3) had an intermittent accelerated idioventricular rhythm. Their
C
ongenital right ventricular (RV) diverticulum is a rare disorder in which a localized torus protrudes from the RV free wall due to congenital factors. There are 2 types of diverticula, muscular or fibrous. The lesion has been termed muscular or true diverticulum when it has a normal muscular structure or when it contracts functionally like a normal ventricle. Only 8 cases of muscular RV diverticula have been reported.lm6 In all, From Children’s Research Hospital and Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto 602, Japan. Manuscript received July 27, 1987; revised manuscript received September 18,1987, and accepted September 21.
Case 1
Case 2
8.5).
I
II ..~,.
,,-. 1
-1 /
III-
aVR
Case 3
,+y
Case 4
;.;.,;
~
~ ..,........::.:...:::..::..:..:-.
‘,.
FIGURE 1. Direct tracings from cineangiograms of representative right ventricular diverticula (arrows) in diastole.
FIGURE 2. Frequent monofocal ventricular premature beats (accelerated indioventricular rhythm) in patient 3.
February 15, 1988
ventricular premature complexes were estimated to occur from the RV anterior wall and the pulmonary conus from the QRS patterns of ventricular premature complexes. None of the 4 patients had any findings of ventricular or atrial overloading on the electrocardiograms. Echocardiography revealed no abnormal findings in the cardiac anatomy in any of the 4. In all 4 patients, cardiac catheterization showed normal intracardiac and pulmonary arterial pressures, and no intracardiac shunt. RV angiography showed a diverticulum in the outflow area of the right ventricle (Figure I), which contracted in synchrony with the remainder of the right ventricle. From the angiographic findings, we judged that the ventricular premature complexes in the 4 patients occurred from the diverticula of RV outflow tract. Surgical operation was performed in patient 3, who had an accelerated idioventricular rhythm (Figure 2) that could not be controlled by antiarrhythmic drugs. At operation, the diverticulum was observed in RV outflow tract. Histologically, the resected diverticulum had a normal muscular structure without any cell infiltration or fibrosis. Postoperatively, patient 3 showed normal reguJar sinus rhythm and no ventricular arrhythmia on the electrocardiogram. The 3 other patients had only simple ventricular premature complexes; thus, an operation was not recommended. We judged that the diver-
Influence of Sublingual Nitroglycerin on Diastolic Transmitral Flow Velocities in Normal Subjects BYRON F. VANDENBERG, MD JOHN A. RUMBERGER, PhD, MD RICHARD E. KERBER, MD
P
ulsed Doppler echocardiographic sampling of transmitral diastolic flow velocities provides a noninvasive method for the serial evaluation of left ventricular diastolic functi0n.l A reduction in the peak early filling (E) velocity and an increase in the peak late filling (A) velocity suggests reduced compliance of the left ventric1e.l Similar changes in the velocity-time profile occur after the administration of nitroglycerin,2 since diastolic function may be affected by changes in loading conditions3 independent of intrinsic myocardial relaxation abnormalities4 Our purpose was to From the Cardiovascular Center, Department of Internal Medicine, University of Iowa, Iowa City, Iowa. This study was supported in part by an Ischemic SCOR Grant, HL-32295, and by a Research Service Award HL-07379-01 (Dr. Vandenberg) from the National Institutes of Health, Bethesda, Maryland. Manuscript received July 27, 1987; revised manuscript received and accepted September 16,1987. Dr. Rumberger’s current address: Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota.
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume 61
481
ticula of these 3 patients were of the muscular type, because the patients had normal contractions on angiography. It has recently been noticed that ventricular arrythmias originating in RV outflow tract may be associated with some histologic abnormalities or organic heart lesions. In the 4 patients just described, the lesions were not associated with any other intracardiac anomalies. Each had complained of intermittent palpitation due to ventricular premature complexes or ventricular tachycardia related to the diverticula. The diverticula may have been an ectopic focus for ventricular premature complexes or a reentry circuit. 1. Magrassi P. Chartrand C, Guerin R, Kratz C, Stanley P. True diverticulum of the right ventricle. Two cases associated with teratology of Fallot. Ann Thorac Surg 1980;29:357-363. 2. Carter JB:Van TasselRA, Moller JH, Amplatz K, Edwards JE. Congenital diverticulum of the right ventricle. Associated with pulmonary stenosis and ventricular septal defect. Am J Cardiol 1971;28:478-482. 3. Bharati S. Rowen M, CamarataSJ,Ostermiller WE Jr, Singer M. Lev M. Diverticulum of the right ventricle. Arch Pathol 1975;89:383-386, 4. Copeland J, Higgino C. Hayden W, Stinson EB. Congenital diverticulum of the right ventricle. T Thorac Cardiovosc Sure 1975:70:536-538. 6. Nicod P. Laird WF’. Firth BG, Nicod L, F&r D. Congenital diverticula of the left and right ventricles: 3 cases. Am J Cardiol 1984;53:342-344. 6. Terai M, Ohta F. Kate T. Nakanishi T, Takahashi Y, Minami Y, Satomi G, ando M. Moma K, Takao a, Kurosawa H, Takanashi Y. Imai Y. Two cases of congenital right ventricular diverticulum (in lapanesel. Heart 1985:17:163168.
identify, in normal subjects, Doppler echocardiographic parameters of diastolic function that are not affected by preload reduction with sublingual nitroglycerin. We performed pulsed Doppler echocardiography in 9 healthy volunteers (mean age 27 years, range 24 to 29) with an American Technology Laboratory ultrasonoscope (3.0-MHz transducer). Sampling of transmitral flow velocities was obtained by placing the Doppler gate at the level of the mitral anulus from the apical 4-chamber view of the left ventricle. The transmitral velocity-time profiles were recorded at a paper speed of 100 mm/s. Sublingual nitroglycerin, 0.4 mg, was administered and the recordings were repeated after 3 to 5 minutes if heart rate increased by at least 10% from baseline. If heart rate did not increase by 10% above baseline (suggesting insignificant preload reduction), an additional 0.2 mg sublingual nitroglycerin was given. Thus heart rate, rather than systemic arterial pressure, was used to demonstrate the pharmacologic effect of nitroglycerin. E velocity, time from baseline to E velocity (acceleration time), time from E velocity to baseline (deceleration time), the E velocity-time integral (area under the E velocity-time profile), the A velocity and the E/A velocity ratio were averaged from 5 beats. The recordings were coded and measurements were made without knowledge of whether they were obtained at baseline or after sublingual nitroglycerin administration. Data are expressed as mean f standard deviation. Comparisons of measurements between control and nitroglycerin administration were performed with a paired t test; p <0.05 was considered significant.