Pediatr Cardiol 12:126-127, 1991
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991
Discordant Atrioventricular Connection and Concordant Ventriculoarterial C o n n e c t i o n in Situs Inversus: I s o l a t e d V e n t r i c u l a r N o n i n v e r s i o n S.S. Kothari, C.C. Kartha, C.G. Venkitachalam, R. Subramanyan, and K.G. Balakrishnan D e p a r t m e n t of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Kerala, India
SUMMARY. A case of discordant atrioventricular (AV) connection and concordant ventricuIoarterial (VA) connection (with aortomitral fibrous continuity and tricuspid pulmonary discontinuity) in situs inversus (I, D, I) is reported because of its rarity. This segmental combination with abnormally related great arteries in the absence of anomalies of venous connections and/or splenic anomalies is extremely rare. KEY WORDS: Congenital heart disease - - Discordant AV connection - - Ventricular noninversion - - Concordant VA connection
Hearts with discordant atrioventricular (AV) connection generally have either discordant ventriculoarterial (VA) connection (complete transposition of the great arteries) or double-outlet right ventricle (RV) with subaortic or bilateral conus. Concordant VA connection with discordant AV connection in visceroatrial situs solitus or visceroatrial situs inversus is very uncommon [6]. We report here a case of discordant (AV) connection and concordant (VA) connection in situs inversus, a segmental combination which has previously been termed isolated ventricular noninversion [2].
Case Report A 3-month-old female child was referred as a patient with visceroatrial situs i n v e r s u s , complete transposition of the great arteries, congestive heart failure, and respiratory infection. She was born after a full-term normal delivery (birth weight, 3.2 kg). D y s p n e a and c y a n o s i s were noticed since day 10 of age. On examination, a severe degree of c y a n o s i s and signs of congestive
Address offprint requests to: Dr. C.G. Venkitachalam, Departm e n t of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and T e c h n o l o g y , T r i v a n d r u m , Kerala, India 695011.
cardiac failure were evident. T h e cardiac apex was on the right side of the c h e s t and liver dullness was on the left side of the a b d o m e n . A single second heart s o u n d and grade II/VI pansystolic m u r m u r were heard over the precordium. Her c h e s t x-ray s h o w e d the cardiac shadow in the right chest with apex toward the right, gastric fundus gas shadow on the right side, and areas of patchy consolidation in the lungs. The electrocardiogram had inverted P w a v e s in lead I and a V L and upright P in aVR; q w a v e s were present in lead V4 and V5, and absent in the right-sided leads. The infant died shortly after admission to the hospital. At a u t o p s y , visceroatrial situs inversus totalis with a single well-formed spleen was seen. A trilobed lung in the left thorax and a bilobed one on the right was seen. There was atrial inversion as identified by typical morphology and the shape of appendages (Figs. 1 and 2). The systemic and pulmonary venous c o n n e c t i o n s were to the appropriate atria. The coronary sinus drained into the morphologically right atrium. Two well-formed ventricles with an interventricular s e p t u m were present. A large p e r i m e m b r a n o u s ventricular septal defect extended into the outlet septum. T h e morphologically right ventricle was on the right side and morphologically left ventricle on the left (right-hand ventricular topology). T h e left atrium was connected to the right ventricle (Fig. 1) and the right atrium to the left ventricle (Fig. 2). The tricuspid and mitral valves were normal. The pulmonary artery was c o n n e c t e d to the right ventricle (Fig. 3) and the aorta to the left ventricle. A well-formed m u s c u l a r infundibulum separated the tricuspid and p u l m o n a r y valves (Fig. 3). Aortomitral fibrous continuity was p r e s e n t on the left side. Both coronary arteries arose from the aorta. The great arteries were parallel to each other, with the p u l m o n a r y trunk slightly anterior and to the right of the aorta (Fig. 4). There were no other visceral anomalies.
Venkitachalam et al.: Isolated Ventricular Noninversion
127
Fig. 1. Specimen photograph showing morphologically left atrium (LA) connected to a coarsely trabeculated right ventricle (RV). PV, pulmonary vein. Fig. 2. Specimen photograph showing morphologically right atrium (RA) connected to left ventricle (LV). Fig, 3. Pulmonary artery (PA) connected to right ventricle (RV) with subpulmonic infundibulum is shown. Fig. 4. Specimen photograph showing parallel arrangement of the great arteries. Pulmonary trunk is slightly anterior and to the right of the aorta. PA, pulmonary artery; AO, aorta; LAA, left atrial appendage; RV, right ventricle.
Discussion
References
Hearts with discordant AV connection and concordant VA connection (with aorticomitral fibrous continuity and pulmonary tricuspid discontinuity) are very rare [I-4, 6, 7]. Such a segmental combination in situs inversus was previously termed isolated ventricular noninversion [2]. Patients with these anomalies present clinically like those with complete transposition of the great arteries, and may be incorrectly diagnosed as such. Associated defects are common. Total anomalous pulmonary venous connection [2], azygos continuation of inferior vena cava [1, 4], juxtaposition of atrial appendages [ 1], and complete common AV canal defect [4] have been reported. Most of the previously reported patients with anomalies of venous connection had atrial isomerism. Our patient did not have any anomalies of venous connection and the atria were inverted. Abnormally related great arteries in the segmental combination of situs inversus, discordant AV connection, and concordant VA connection are very rare. A recent review of all published cases did not reveal any such case in the English-language literature [5]. However, the case reported by Hazan et al. [4] is similar to the present case, with mirror image of normally related great arteries and isolated discordant AV connection in situs inversus [4]. That case in addition had complete common AV canal and underwent successful intracardiac repair [4].
1. Calcaterra, G, Anderson RH, Lau KC, Shinebourne EA (1979) Dextrocardia--Value of segmental analysis in its categorisation. Br Heart J 42:497-507 2. Espino-vela J, Dela Cruz MV, Munoz-Castellanos L, Plaza L, Allie F (1970) Ventricular inversion without transposition of the great vessels in situs inversus. Br Heart J 32:292-303 3. Fox LS, Kirklin JW, Pacifico AD, Waldo AL, Bargeron LM Jr (1976) Intracardiac repair of cardiac malformation with atrioventricular discordance. Circulation 54:123-127 4. Hazan E, Baillot F, Rey C, Dupuis C (1977) Isolated ventricular discordance and complete atrioventricular canal in situs inversus. Report of successful surgical repair. A m J Cardiol 40:463-466 5. Pasquini L, Sanders SP, Parness I, et al. (1988) Echocardiographic findings in atrioventricular discordance with ventricuIoarterial concordance. Am J Cardiol 62:1256-1262 6. Suider AR, Enderlein MA, Teitel DF, Hirji M, Heymann MA (1984) Isolated ventricular inversion. Two-dimensional echocardiographic findings and a review of literature. Pediatr. Cardiol. 5:27-33 7. Squarcia U, Ritler DG, Kincaid OW (1973) Dextrocardia-Angiographic study and classification. A m J Cardiol 32:965977
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991
Discordant Atrioventricular Connection and Concordant Ventriculoarterial C o n n e c t i o n in Situs Inversus: I s o l a t e d V e n t r i c u l a r N o n i n v e r s i o n S.S. Kothari, C.C. Kartha, C.G. Venkitachalam, R. Subramanyan, and K.G. Balakrishnan D e p a r t m e n t of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Kerala, India
SUMMARY. A case of discordant atrioventricular (AV) connection and concordant ventricuIoarterial (VA) connection (with aortomitral fibrous continuity and tricuspid pulmonary discontinuity) in situs inversus (I, D, I) is reported because of its rarity. This segmental combination with abnormally related great arteries in the absence of anomalies of venous connections and/or splenic anomalies is extremely rare. KEY WORDS: Congenital heart disease - - Discordant AV connection - - Ventricular noninversion - - Concordant VA connection
Hearts with discordant atrioventricular (AV) connection generally have either discordant ventriculoarterial (VA) connection (complete transposition of the great arteries) or double-outlet right ventricle (RV) with subaortic or bilateral conus. Concordant VA connection with discordant AV connection in visceroatrial situs solitus or visceroatrial situs inversus is very uncommon [6]. We report here a case of discordant (AV) connection and concordant (VA) connection in situs inversus, a segmental combination which has previously been termed isolated ventricular noninversion [2].
Case Report A 3-month-old female child was referred as a patient with visceroatrial situs i n v e r s u s , complete transposition of the great arteries, congestive heart failure, and respiratory infection. She was born after a full-term normal delivery (birth weight, 3.2 kg). D y s p n e a and c y a n o s i s were noticed since day 10 of age. On examination, a severe degree of c y a n o s i s and signs of congestive
Address offprint requests to: Dr. C.G. Venkitachalam, Departm e n t of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and T e c h n o l o g y , T r i v a n d r u m , Kerala, India 695011.
cardiac failure were evident. T h e cardiac apex was on the right side of the c h e s t and liver dullness was on the left side of the a b d o m e n . A single second heart s o u n d and grade II/VI pansystolic m u r m u r were heard over the precordium. Her c h e s t x-ray s h o w e d the cardiac shadow in the right chest with apex toward the right, gastric fundus gas shadow on the right side, and areas of patchy consolidation in the lungs. The electrocardiogram had inverted P w a v e s in lead I and a V L and upright P in aVR; q w a v e s were present in lead V4 and V5, and absent in the right-sided leads. The infant died shortly after admission to the hospital. At a u t o p s y , visceroatrial situs inversus totalis with a single well-formed spleen was seen. A trilobed lung in the left thorax and a bilobed one on the right was seen. There was atrial inversion as identified by typical morphology and the shape of appendages (Figs. 1 and 2). The systemic and pulmonary venous c o n n e c t i o n s were to the appropriate atria. The coronary sinus drained into the morphologically right atrium. Two well-formed ventricles with an interventricular s e p t u m were present. A large p e r i m e m b r a n o u s ventricular septal defect extended into the outlet septum. T h e morphologically right ventricle was on the right side and morphologically left ventricle on the left (right-hand ventricular topology). T h e left atrium was connected to the right ventricle (Fig. 1) and the right atrium to the left ventricle (Fig. 2). The tricuspid and mitral valves were normal. The pulmonary artery was c o n n e c t e d to the right ventricle (Fig. 3) and the aorta to the left ventricle. A well-formed m u s c u l a r infundibulum separated the tricuspid and p u l m o n a r y valves (Fig. 3). Aortomitral fibrous continuity was p r e s e n t on the left side. Both coronary arteries arose from the aorta. The great arteries were parallel to each other, with the p u l m o n a r y trunk slightly anterior and to the right of the aorta (Fig. 4). There were no other visceral anomalies.
Venkitachalam et al.: Isolated Ventricular Noninversion
127
Fig. 1. Specimen photograph showing morphologically left atrium (LA) connected to a coarsely trabeculated right ventricle (RV). PV, pulmonary vein. Fig. 2. Specimen photograph showing morphologically right atrium (RA) connected to left ventricle (LV). Fig, 3. Pulmonary artery (PA) connected to right ventricle (RV) with subpulmonic infundibulum is shown. Fig. 4. Specimen photograph showing parallel arrangement of the great arteries. Pulmonary trunk is slightly anterior and to the right of the aorta. PA, pulmonary artery; AO, aorta; LAA, left atrial appendage; RV, right ventricle.
Discussion
References
Hearts with discordant AV connection and concordant VA connection (with aorticomitral fibrous continuity and pulmonary tricuspid discontinuity) are very rare [I-4, 6, 7]. Such a segmental combination in situs inversus was previously termed isolated ventricular noninversion [2]. Patients with these anomalies present clinically like those with complete transposition of the great arteries, and may be incorrectly diagnosed as such. Associated defects are common. Total anomalous pulmonary venous connection [2], azygos continuation of inferior vena cava [1, 4], juxtaposition of atrial appendages [ 1], and complete common AV canal defect [4] have been reported. Most of the previously reported patients with anomalies of venous connection had atrial isomerism. Our patient did not have any anomalies of venous connection and the atria were inverted. Abnormally related great arteries in the segmental combination of situs inversus, discordant AV connection, and concordant VA connection are very rare. A recent review of all published cases did not reveal any such case in the English-language literature [5]. However, the case reported by Hazan et al. [4] is similar to the present case, with mirror image of normally related great arteries and isolated discordant AV connection in situs inversus [4]. That case in addition had complete common AV canal and underwent successful intracardiac repair [4].
1. Calcaterra, G, Anderson RH, Lau KC, Shinebourne EA (1979) Dextrocardia--Value of segmental analysis in its categorisation. Br Heart J 42:497-507 2. Espino-vela J, Dela Cruz MV, Munoz-Castellanos L, Plaza L, Allie F (1970) Ventricular inversion without transposition of the great vessels in situs inversus. Br Heart J 32:292-303 3. Fox LS, Kirklin JW, Pacifico AD, Waldo AL, Bargeron LM Jr (1976) Intracardiac repair of cardiac malformation with atrioventricular discordance. Circulation 54:123-127 4. Hazan E, Baillot F, Rey C, Dupuis C (1977) Isolated ventricular discordance and complete atrioventricular canal in situs inversus. Report of successful surgical repair. A m J Cardiol 40:463-466 5. Pasquini L, Sanders SP, Parness I, et al. (1988) Echocardiographic findings in atrioventricular discordance with ventricuIoarterial concordance. Am J Cardiol 62:1256-1262 6. Suider AR, Enderlein MA, Teitel DF, Hirji M, Heymann MA (1984) Isolated ventricular inversion. Two-dimensional echocardiographic findings and a review of literature. Pediatr. Cardiol. 5:27-33 7. Squarcia U, Ritler DG, Kincaid OW (1973) Dextrocardia-Angiographic study and classification. A m J Cardiol 32:965977
