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Right atrial enlargement—Cardiac imaging
Right Atrial Enlargement--Cardiac Imaging John B. Bingham, K. A. McKusick, and H. W. Strauss
A
60-yr-old female, known to have had a heart murmur from birth, had been noted to have thumbs which were nonopposable and which resembled fifth digits. A diagnosis of the HoltOrarn I syndrome had been made. She had developed exertional dyspnea and atrial fibrillation almost 20 yr prior to admission and had been maintained on digitoxin and diuretics. Two years previous to this admission she had had an embolus into a femoral artery, for which she had remained on anticoagulation. She recently had increasing symptoms and signs of florid congestive cardiac failure. Abnormal findings on examination included clubbing of the fingers, mild cyanosis, pedal edema, an irregularly irregular pulse, a raised jugular venous pressure, a right ventricular heave, and a grade 2 holosystolic murmur at the upper left sternal border and at the apex. The second heart sound was widely split, and was fixed. A gated cardiac scan using invivo labeled red ceils was obtained 2 (Fig. 1) in three projections. The right atrium was massively enlarged and noncontractile. The right ventricle was also enlarged, and showed an increased stroke volume compared to the left ventricle. The pulmonary outflow tract was markedly dilated. The left atrium and left ventricle were also enlarged, but apart from the abnormal anterior septal motion there was no abnormal regional wall motion. The left ventricular ejection fraction, calculated using an automatic edge detection program, was 50%. It is helpful to obtain lateral or posterior oblique views of the heart enabling the left atrial size to be assessed, so that the presence of mitral regurgitation is not overlooked. On the basis of catheterization studies, a diagnosis of an atrial septal defect and mitral regurgitation was made. Because of increasing cardiac decompensation, surgery was performed and a large atrial septal defect was closed and the mitral valve replaced. The Holt-Oram syndrome is associated with congenital heart anomalies? particularly of the
Seminars in Nuclear Medicine, Vol, X, No. 2 (April), 1980
Fig. 1. Three views of the heart obtained after in vivo labeling of red cells with ~ ' T c .
intraatriaI and intraventricular septum. Atrial septal defects are commonly accompanied by mitral valve abnormalities ranging from small clefts in the anterior mitral leaflet to undivided anterior and posterior atrioventricular leaflets. 4 Causes of right atrial enlargement: COMMON
1. Elevated right ventricular diastolic pressure due to: Common
a) pulmonary hypertension (eg COPD, primary pulmonary hypertension); b) left heart failure; Uncommon
a) right ventricular infarction; b) pulmonic stenosis. 2. Atrial fibrillation--this alone may be associated with mild atrial enlargement. 3. Left to right shunting as in atrial septal defect.
From the Massachusetts General Hospital Boston, Mass. Address reprint requests to H. W. Strauss, M. D., Massachusetts General Hospital, Boston, Mass. 02114. 9 1980 by Grune & Stratton, Inc. 0001-2998/80/1002-0009501.00/0
195
196
BINGHAM, McKUSICK, AND STRAUSS
UNCOMMON 1. T r i c u s p i d r e g u r g i t a t i o n , w h i c h often gives rise to m a s s i v e e n l a r g e m e n t . 2. T r i c u s p i d stenosis; the a b s e n c e of r i g h t v e n t r i c u l a r e n l a r g e m e n t and p u l m o n a r y h y p e r t e n s i o n suggests the p r e s e n c e of t r i c u s p i d stenosis. 3. M i t r a l stenosis or r e g u r g i t a t i o n w i t h pulmonary hypertension.
REFERENCES 1. Holt M, Oram S: Familial heart disease with skeletal malformations. Br Heart J 22:236, 1960 2. Pavel DG, Zimmer AM, Patterson VN: In vivo labeling of red blood ce~,ls with 99~Tc: A new approach to blood pool visualization. J Nucl Med 18:305, 1977 3. Brans YW, Lintemans JP: The upper limb~zardiovascular syndrome: A report of two African cases with a review of the literature. Am J Dis Child 124:779, 1972 4. Titus JL: Congenital malformations of the mitral and aortic valves and related structures. Dis Chest 55:358, 1969
A
60-yr-old female, known to have had a heart murmur from birth, had been noted to have thumbs which were nonopposable and which resembled fifth digits. A diagnosis of the HoltOrarn I syndrome had been made. She had developed exertional dyspnea and atrial fibrillation almost 20 yr prior to admission and had been maintained on digitoxin and diuretics. Two years previous to this admission she had had an embolus into a femoral artery, for which she had remained on anticoagulation. She recently had increasing symptoms and signs of florid congestive cardiac failure. Abnormal findings on examination included clubbing of the fingers, mild cyanosis, pedal edema, an irregularly irregular pulse, a raised jugular venous pressure, a right ventricular heave, and a grade 2 holosystolic murmur at the upper left sternal border and at the apex. The second heart sound was widely split, and was fixed. A gated cardiac scan using invivo labeled red ceils was obtained 2 (Fig. 1) in three projections. The right atrium was massively enlarged and noncontractile. The right ventricle was also enlarged, and showed an increased stroke volume compared to the left ventricle. The pulmonary outflow tract was markedly dilated. The left atrium and left ventricle were also enlarged, but apart from the abnormal anterior septal motion there was no abnormal regional wall motion. The left ventricular ejection fraction, calculated using an automatic edge detection program, was 50%. It is helpful to obtain lateral or posterior oblique views of the heart enabling the left atrial size to be assessed, so that the presence of mitral regurgitation is not overlooked. On the basis of catheterization studies, a diagnosis of an atrial septal defect and mitral regurgitation was made. Because of increasing cardiac decompensation, surgery was performed and a large atrial septal defect was closed and the mitral valve replaced. The Holt-Oram syndrome is associated with congenital heart anomalies? particularly of the
Seminars in Nuclear Medicine, Vol, X, No. 2 (April), 1980
Fig. 1. Three views of the heart obtained after in vivo labeling of red cells with ~ ' T c .
intraatriaI and intraventricular septum. Atrial septal defects are commonly accompanied by mitral valve abnormalities ranging from small clefts in the anterior mitral leaflet to undivided anterior and posterior atrioventricular leaflets. 4 Causes of right atrial enlargement: COMMON
1. Elevated right ventricular diastolic pressure due to: Common
a) pulmonary hypertension (eg COPD, primary pulmonary hypertension); b) left heart failure; Uncommon
a) right ventricular infarction; b) pulmonic stenosis. 2. Atrial fibrillation--this alone may be associated with mild atrial enlargement. 3. Left to right shunting as in atrial septal defect.
From the Massachusetts General Hospital Boston, Mass. Address reprint requests to H. W. Strauss, M. D., Massachusetts General Hospital, Boston, Mass. 02114. 9 1980 by Grune & Stratton, Inc. 0001-2998/80/1002-0009501.00/0
195
196
BINGHAM, McKUSICK, AND STRAUSS
UNCOMMON 1. T r i c u s p i d r e g u r g i t a t i o n , w h i c h often gives rise to m a s s i v e e n l a r g e m e n t . 2. T r i c u s p i d stenosis; the a b s e n c e of r i g h t v e n t r i c u l a r e n l a r g e m e n t and p u l m o n a r y h y p e r t e n s i o n suggests the p r e s e n c e of t r i c u s p i d stenosis. 3. M i t r a l stenosis or r e g u r g i t a t i o n w i t h pulmonary hypertension.
REFERENCES 1. Holt M, Oram S: Familial heart disease with skeletal malformations. Br Heart J 22:236, 1960 2. Pavel DG, Zimmer AM, Patterson VN: In vivo labeling of red blood ce~,ls with 99~Tc: A new approach to blood pool visualization. J Nucl Med 18:305, 1977 3. Brans YW, Lintemans JP: The upper limb~zardiovascular syndrome: A report of two African cases with a review of the literature. Am J Dis Child 124:779, 1972 4. Titus JL: Congenital malformations of the mitral and aortic valves and related structures. Dis Chest 55:358, 1969