Table !-Hemodynamic Data•
Situs lnversus Totalis Associated with Subaortic and Subpulmonic Stenosis*
Right atrium Mean
1969 10
1!172 12
Right ventricle
34/10
49/13
David E. Wells, M.S., F.C.C.P. and Benjamin Befeler, M.D., F.C.C.P.
Pulmonary artery Mean
34/14 21
50/11 32
Wedge pulmonary artery Mean
20
22
Left ventricle
136/35
141/3
Aorta
136/71
109/73
Brachial artery
140/75
122/73
Cardiac index
3.7
2.39 Lt/min/m2
The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnonnaHty (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasizes the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was faci6tated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outftow obstruction, rather than to coronary disease. inversus totalis is a rare congenital anomaly said S itus to occur one of 22,000 live births. Idiopathic in
1
hypertrophic subaortic stenosis is uncommon. 2 The association of both of these conditions in the same patient is For editorial comment, see page 4 extremely unusual and has been reported on only one previous occasion. Furthermore, the case reported here also demonstrated dynamic obstruction to the right ventricular outflow tract and presented clinically as angina pectoris. CASE REPORT
A 44-year-old man was referred to the Veterans Administration Hospital, Miami, Florida for cardiac evaluation because of severe angina pectoris. Dextrocardia and total sihts inversus were noted in 1945 during an Air Force induction examination. In 1959 he began to experience dyspnea with strenuous exertion. Examination in 1965 revealed a heart murmur for the first time. In 1969, he developed paroxysmal nocturnal dyspnea and orthopnea which were treated with digitalis and diuretics. Cardiac catheterization elsewhere that year revealed idiopathic subaortic stenosis, findings summarized in Table 1. No resting ventricular outBow tract gradient was found but a maximum gradient of 82 mm Hg was noted in post-PVC beats, a 35 mm gradient was induced during a Valsalva maneuver, and a 110 mm gradient was demonstrated with isoproterenol infusion. Left ventricular angiogram in the LAO projection revealed dynamic outflow obstruction. The coronary arterial tree was not °From the Cardiovascular Laboratory, Cardiology Section, Veterans Administration Hospital and the Department of Medicine, University of Miami, School of Medicine, Miami. This study was supported in part by Institutional Research Funds (Part II) Veterans Administration Hospital, Miami, and in part by a training grant from the USPHS, National Heart & Lung Institute ( HL-05463).
Reprint requests: Dr. Befeler, VA Hospital, 1201 NW 16th Street, Miami 33125
CHEST, 67: 1, JANUARY, 1975
Pulmonary vascular resistance
192 dynes/sec em·•
Systemic vascular resistance
1598
•Pressures in mm Hg studied during this catheterization. Diuretics and digitalis were stopped and propranolol begun. Symptoms remained unchanged until March 1971 when he experienced a severe, prolonged episode of chest pain resulting in hospitalization. Following this hospitalization, dyspnea on exertion, paroxysmal nocturnal dyspnea and pedal edema appeared. The patient was admitted to our hospital for reevaluation of coronary disease. Examination revealed blood pressure of 118172 mm Hg and a pulse of 68 per minute. The peripheral pulses were normal. The precordium was quiet, the PMI was in the 5th intercostal space midclavicular line in the right hemithorax; it was bifid. A loud fourth sound was heard along the entire right sternal border. A grade 4/6 systolic crescendo-decrescendo rough murmur was heard over the entire precordium; it radiated into the carotids. The standard electrocardiogram with additional right precordial leads demonstrated an inverted P wave in lead 1, a QRS axis of approximately 120 degrees, prominent septal forces, voltage and ST segment changes compatible with hypertrophy of a rightward and posteriorly located left ventricle ( Fig 1 ) . Chest x-ray film revealed moderate cardiomegaly with left ventricular configuration and complete situs inversus (Fig2). Cardiac catheterization was performed including coronary arteriography by a technique previously reported from our laboratory. 4 Findings are shown in Tables 1 and 2 and Fig 3. Left
Table 2-Dynamic Gradient• Induced by Hemodynamic and Pharmacologic Maneu11er• in MM Hg Right Heart (RV to PA)•
Left Heart (LV to BAJ•
Resting
5
27
Post-PVC beat
5
110
20
85
Amylnitrite inhalation
5
27
Isoproterenol infusion
15
27
Valsalva
• All gradients based upon simultaneously recorded pressures
SITUS INVERSUS TOTALIS 101
FIGURE I. Standard 12 lead electrocardiogram with additional right precordial leads. Tracing compatible with situs inversus totalis, septal and left ventricular hypertrophy. ventricular angiogram, 45• left anterior oblique (Fig 4), revealed a slit-like left ventricular cavity with generalized left ventricular muscular hypertrophy. No mitral regurgitation was noted. The left ventricular cavity in the right anterior oblique position showed dynamic narrowing of the subaortic outflow tract during systole, due to apposition of the anterior leaflet of the mitral valve to the hypertrophied muscular intraventricular septum. Selective coronary cine-
angiograms revealed angiographically normal coronary arteries. One subsequent hospitalization has occurred for viral pneumonitis. Though the patient is without sinus symptomatology, sinus x-ray fihng revealed thickening of the right maxillary antrum walls. This finding alone was not felt to be sufficient basis for diagnosing Kartagener' s syndrome, since bronchiectasis was not present,~> on bronchographic examination. DISCUSSION
The chance occurrence of total situs inversus and idiopathic hypertrophic stenosis must be extremely rare
FIGURE 2. Standard posterior-anterior chest film showing moderate cardiomegaly with a left ventricle configuration. Film was obtained during last admission.
102 WELLS, BEFELER
FIGURE 3. Simultaneous pressure recordings in left ventricle (LV) and brachial artery ( BA) at rest. Note small gradient at rest and its increase after a premature beat.
CHEST, 67: 1, JANUARY, 1975
6 Braunwald E, Lam brew C, Rockoff S, et a1: Idiopathic hypertrophic subaortic stenosis. Circ 29/30: ( supp 4) 1-213, 1964 7 Lewis R, Bristow J, Farrehi C, et al: Idiopathic left ventricular hypertrophy. Am J Med 38:842-852, 1965 8 Diamond G, Forrester J: Effect of coronary artery disease and acute myocardial infarction on left ventricular compliance in man. Circulation 40:11-19, 1972 9 Befeler B, Wells DE, Machado H, et al: Hemodynamic characteristics of obstructive cardiomyopathy of the right ventricle. Circulation 48:143 ( Suppl IV) 1973
FIGURE 4. Frame taken from 35 mm left ventricular angiogram, LAO 45°, during end-systole showing slit-like left ventricular hypertrophy. because both conditions are uncommon. The exact incidence of IHSS is not known, but cases with biventricular obstruction comprise about 20 percent of IHSS cases. 6 The technique of catheterization in this case was not more difficult than in patients with situs solitus, although some delay was experienced due to the necessity of performing all catheter maneuvers "backwards" from the left arm. The most striking abnormality demonstrated by catheterization was the elevated right and left ventricular enddiastolic pressures which have been related to decreased ventricular compliance in obstructive cardiomyopathy. 7 The calculated left ventricular compliance derived from the equation of Forrest and Diamond8 was strikingly abnormal and denoted marked stiffening of the ventricle. It has been estimated that 20 percent of patients with IHSS have dynamic right ventricular obstruction.A Our experience, however, utilizing simultaneous pressure recordings across the outflow tract suggests that bilateral obstruction may occur in a large number of cases. 9 In all likelihood, the diagnosis of IHSS in this patient would have been considered at an earlier date had not attention been directed only to the presence of total situs inversus. This case illustrates the fact that comprehensive evaluation of patients, even those with obvious anomalies, may uncover unusual associations. REFERENCES
2 3 4 5
Hollman A: Dextrocardia, levocardia and mesocardia. In Pediatric Cardiology, (Watson, Hamish, Eds) St. Louis, CV Mosby Co., 1968 Frank S, Braunwald E: Idiopathic hypertrophic subaortic stenosis. Circulation 37:759-788, 1968 KingS, Vogel J, BlountS: Idiopathic muscular subvalvular aortic stenosis with associated congenital cardiovascular lesions. Am J Cardiol 15:837-847, 1965 Wells DE, Befeler B, Winkler JS, et al. A simplified method for left heart catheterization including coronary arteriography. Chest 63 :959-962, 1973 Katz M, Benzier E, Nangerone L, et al: Kartagener's syndrome situs inversus, bronchiectasis, and chronic sinusitis. N Eng! J Med 248:730, 1963
CHEST, 67: 1, JANUARY, 1975
Idiopathic Hypertrophic Subaortic Stenosis in a Patient with Mirror-Image Dextrocardia* Paul T. Cochran, M .D., 00 and John L. Wanamaker, M.D.t
A 42-year-old man with mirror-image dextrocardia was found to have idiopathic hypertrophic subaortic stenosis. The coexistence of these uncommon congenital cardiovascular anomalies was previously reported in only one patient.
S
itus inversus with mirror-image dextrocardia is an uncommon congenital anomaly estimated to occur in from 1 in 7,000 to 1 in 12,000 persons.t In spite of the dramatic alteration in visceral symmetry, in only 5 to 10 percent of these people are additional cardiac malformations noted. 2 When other cardiac lesions coexist,
For editorial comment. see page 4 transposition of the great vessels has been the most frequently found. 3 The present report relates the findings in a man with situs inversus, mirror-image dextrocardia, and the familial form of idiopathic hypertrophic subaortic stenosis. The coexistence of these two defects is rare, having previously been reported in only one patient.• CASE REPORT
A 42-year-old caucasian man was transferred from Guam to the Malcolm Grow USAF Medical Center for evaluation of chest pain and shortness of breath. At 17 years of age, on induction into the military, examination by several physicians had revealed no physical abnormality other than situs inversus and mirror-image dextrocardia. He was physically active and in good health until he was 36 years of age when he began to experience shortness of breath on strenuous exertion. He was examined and found to have a systolic munnur that was believed functional. At 39 years of age he °From the Department of Cardiolojzy, Malcolm Grow USAF Medical Center, Andrews Air Force Base, Maryland. 00 Presently at University of New Mexico School of Medicine Albuquerque. ' tPresently at Guthrie Clinic, Sayre, Pennsylvania. Note : The opinions or assertions contained herein are those of the authors and are not to be constmed as official or reflecting the views of the Deparhnent of the Air Force. Reprint requests: Dr. Cochran, University of New Mexico School of Medicine, Albuquerque 87106
IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS 103
Situs lnversus Totalis Associated with Subaortic and Subpulmonic Stenosis*
Right atrium Mean
1969 10
1!172 12
Right ventricle
34/10
49/13
David E. Wells, M.S., F.C.C.P. and Benjamin Befeler, M.D., F.C.C.P.
Pulmonary artery Mean
34/14 21
50/11 32
Wedge pulmonary artery Mean
20
22
Left ventricle
136/35
141/3
Aorta
136/71
109/73
Brachial artery
140/75
122/73
Cardiac index
3.7
2.39 Lt/min/m2
The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnonnaHty (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasizes the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was faci6tated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outftow obstruction, rather than to coronary disease. inversus totalis is a rare congenital anomaly said S itus to occur one of 22,000 live births. Idiopathic in
1
hypertrophic subaortic stenosis is uncommon. 2 The association of both of these conditions in the same patient is For editorial comment, see page 4 extremely unusual and has been reported on only one previous occasion. Furthermore, the case reported here also demonstrated dynamic obstruction to the right ventricular outflow tract and presented clinically as angina pectoris. CASE REPORT
A 44-year-old man was referred to the Veterans Administration Hospital, Miami, Florida for cardiac evaluation because of severe angina pectoris. Dextrocardia and total sihts inversus were noted in 1945 during an Air Force induction examination. In 1959 he began to experience dyspnea with strenuous exertion. Examination in 1965 revealed a heart murmur for the first time. In 1969, he developed paroxysmal nocturnal dyspnea and orthopnea which were treated with digitalis and diuretics. Cardiac catheterization elsewhere that year revealed idiopathic subaortic stenosis, findings summarized in Table 1. No resting ventricular outBow tract gradient was found but a maximum gradient of 82 mm Hg was noted in post-PVC beats, a 35 mm gradient was induced during a Valsalva maneuver, and a 110 mm gradient was demonstrated with isoproterenol infusion. Left ventricular angiogram in the LAO projection revealed dynamic outflow obstruction. The coronary arterial tree was not °From the Cardiovascular Laboratory, Cardiology Section, Veterans Administration Hospital and the Department of Medicine, University of Miami, School of Medicine, Miami. This study was supported in part by Institutional Research Funds (Part II) Veterans Administration Hospital, Miami, and in part by a training grant from the USPHS, National Heart & Lung Institute ( HL-05463).
Reprint requests: Dr. Befeler, VA Hospital, 1201 NW 16th Street, Miami 33125
CHEST, 67: 1, JANUARY, 1975
Pulmonary vascular resistance
192 dynes/sec em·•
Systemic vascular resistance
1598
•Pressures in mm Hg studied during this catheterization. Diuretics and digitalis were stopped and propranolol begun. Symptoms remained unchanged until March 1971 when he experienced a severe, prolonged episode of chest pain resulting in hospitalization. Following this hospitalization, dyspnea on exertion, paroxysmal nocturnal dyspnea and pedal edema appeared. The patient was admitted to our hospital for reevaluation of coronary disease. Examination revealed blood pressure of 118172 mm Hg and a pulse of 68 per minute. The peripheral pulses were normal. The precordium was quiet, the PMI was in the 5th intercostal space midclavicular line in the right hemithorax; it was bifid. A loud fourth sound was heard along the entire right sternal border. A grade 4/6 systolic crescendo-decrescendo rough murmur was heard over the entire precordium; it radiated into the carotids. The standard electrocardiogram with additional right precordial leads demonstrated an inverted P wave in lead 1, a QRS axis of approximately 120 degrees, prominent septal forces, voltage and ST segment changes compatible with hypertrophy of a rightward and posteriorly located left ventricle ( Fig 1 ) . Chest x-ray film revealed moderate cardiomegaly with left ventricular configuration and complete situs inversus (Fig2). Cardiac catheterization was performed including coronary arteriography by a technique previously reported from our laboratory. 4 Findings are shown in Tables 1 and 2 and Fig 3. Left
Table 2-Dynamic Gradient• Induced by Hemodynamic and Pharmacologic Maneu11er• in MM Hg Right Heart (RV to PA)•
Left Heart (LV to BAJ•
Resting
5
27
Post-PVC beat
5
110
20
85
Amylnitrite inhalation
5
27
Isoproterenol infusion
15
27
Valsalva
• All gradients based upon simultaneously recorded pressures
SITUS INVERSUS TOTALIS 101
FIGURE I. Standard 12 lead electrocardiogram with additional right precordial leads. Tracing compatible with situs inversus totalis, septal and left ventricular hypertrophy. ventricular angiogram, 45• left anterior oblique (Fig 4), revealed a slit-like left ventricular cavity with generalized left ventricular muscular hypertrophy. No mitral regurgitation was noted. The left ventricular cavity in the right anterior oblique position showed dynamic narrowing of the subaortic outflow tract during systole, due to apposition of the anterior leaflet of the mitral valve to the hypertrophied muscular intraventricular septum. Selective coronary cine-
angiograms revealed angiographically normal coronary arteries. One subsequent hospitalization has occurred for viral pneumonitis. Though the patient is without sinus symptomatology, sinus x-ray fihng revealed thickening of the right maxillary antrum walls. This finding alone was not felt to be sufficient basis for diagnosing Kartagener' s syndrome, since bronchiectasis was not present,~> on bronchographic examination. DISCUSSION
The chance occurrence of total situs inversus and idiopathic hypertrophic stenosis must be extremely rare
FIGURE 2. Standard posterior-anterior chest film showing moderate cardiomegaly with a left ventricle configuration. Film was obtained during last admission.
102 WELLS, BEFELER
FIGURE 3. Simultaneous pressure recordings in left ventricle (LV) and brachial artery ( BA) at rest. Note small gradient at rest and its increase after a premature beat.
CHEST, 67: 1, JANUARY, 1975
6 Braunwald E, Lam brew C, Rockoff S, et a1: Idiopathic hypertrophic subaortic stenosis. Circ 29/30: ( supp 4) 1-213, 1964 7 Lewis R, Bristow J, Farrehi C, et al: Idiopathic left ventricular hypertrophy. Am J Med 38:842-852, 1965 8 Diamond G, Forrester J: Effect of coronary artery disease and acute myocardial infarction on left ventricular compliance in man. Circulation 40:11-19, 1972 9 Befeler B, Wells DE, Machado H, et al: Hemodynamic characteristics of obstructive cardiomyopathy of the right ventricle. Circulation 48:143 ( Suppl IV) 1973
FIGURE 4. Frame taken from 35 mm left ventricular angiogram, LAO 45°, during end-systole showing slit-like left ventricular hypertrophy. because both conditions are uncommon. The exact incidence of IHSS is not known, but cases with biventricular obstruction comprise about 20 percent of IHSS cases. 6 The technique of catheterization in this case was not more difficult than in patients with situs solitus, although some delay was experienced due to the necessity of performing all catheter maneuvers "backwards" from the left arm. The most striking abnormality demonstrated by catheterization was the elevated right and left ventricular enddiastolic pressures which have been related to decreased ventricular compliance in obstructive cardiomyopathy. 7 The calculated left ventricular compliance derived from the equation of Forrest and Diamond8 was strikingly abnormal and denoted marked stiffening of the ventricle. It has been estimated that 20 percent of patients with IHSS have dynamic right ventricular obstruction.A Our experience, however, utilizing simultaneous pressure recordings across the outflow tract suggests that bilateral obstruction may occur in a large number of cases. 9 In all likelihood, the diagnosis of IHSS in this patient would have been considered at an earlier date had not attention been directed only to the presence of total situs inversus. This case illustrates the fact that comprehensive evaluation of patients, even those with obvious anomalies, may uncover unusual associations. REFERENCES
2 3 4 5
Hollman A: Dextrocardia, levocardia and mesocardia. In Pediatric Cardiology, (Watson, Hamish, Eds) St. Louis, CV Mosby Co., 1968 Frank S, Braunwald E: Idiopathic hypertrophic subaortic stenosis. Circulation 37:759-788, 1968 KingS, Vogel J, BlountS: Idiopathic muscular subvalvular aortic stenosis with associated congenital cardiovascular lesions. Am J Cardiol 15:837-847, 1965 Wells DE, Befeler B, Winkler JS, et al. A simplified method for left heart catheterization including coronary arteriography. Chest 63 :959-962, 1973 Katz M, Benzier E, Nangerone L, et al: Kartagener's syndrome situs inversus, bronchiectasis, and chronic sinusitis. N Eng! J Med 248:730, 1963
CHEST, 67: 1, JANUARY, 1975
Idiopathic Hypertrophic Subaortic Stenosis in a Patient with Mirror-Image Dextrocardia* Paul T. Cochran, M .D., 00 and John L. Wanamaker, M.D.t
A 42-year-old man with mirror-image dextrocardia was found to have idiopathic hypertrophic subaortic stenosis. The coexistence of these uncommon congenital cardiovascular anomalies was previously reported in only one patient.
S
itus inversus with mirror-image dextrocardia is an uncommon congenital anomaly estimated to occur in from 1 in 7,000 to 1 in 12,000 persons.t In spite of the dramatic alteration in visceral symmetry, in only 5 to 10 percent of these people are additional cardiac malformations noted. 2 When other cardiac lesions coexist,
For editorial comment. see page 4 transposition of the great vessels has been the most frequently found. 3 The present report relates the findings in a man with situs inversus, mirror-image dextrocardia, and the familial form of idiopathic hypertrophic subaortic stenosis. The coexistence of these two defects is rare, having previously been reported in only one patient.• CASE REPORT
A 42-year-old caucasian man was transferred from Guam to the Malcolm Grow USAF Medical Center for evaluation of chest pain and shortness of breath. At 17 years of age, on induction into the military, examination by several physicians had revealed no physical abnormality other than situs inversus and mirror-image dextrocardia. He was physically active and in good health until he was 36 years of age when he began to experience shortness of breath on strenuous exertion. He was examined and found to have a systolic munnur that was believed functional. At 39 years of age he °From the Department of Cardiolojzy, Malcolm Grow USAF Medical Center, Andrews Air Force Base, Maryland. 00 Presently at University of New Mexico School of Medicine Albuquerque. ' tPresently at Guthrie Clinic, Sayre, Pennsylvania. Note : The opinions or assertions contained herein are those of the authors and are not to be constmed as official or reflecting the views of the Deparhnent of the Air Force. Reprint requests: Dr. Cochran, University of New Mexico School of Medicine, Albuquerque 87106
IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS 103
