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Surgical repair of criss-cross heart with straddling atrioventricular valve
Surgical repair of criss-cross heart with straddling atrioventricular valve The criss-cross heart is a rare anomaly in which systemic and pulmonary bloodstreams cross at the atrioventricular (AV) level. Although diagnosed now with increasing frequency, successful surgical repair of associated anomalies has been reported only once. Straddling AV valve is another rare cardiac anomaly which only recently is being diagnosed consistently preoperatively and corrected surgically. This is the first report of successful repair of cardiac anomalies in a patient with the combination of straddling AV valve and criss-cross AV relationships. The uneventful postoperative course and good result on follow-up in this patient are gratifying and suggest that this combination of anomalies now can be repaired with results as good as those for less complex forms of congenital heart disease.
Gordon K. Danielson, M.D., Imad F. Tabry, M.D., Donald G. Ritter, M.D., and Richard E. Fulton, M.D., Rochester, Minn., and East Lansing, Mich.
T
he criss-cross heart, a term introduced independently by Anderson, t Ando," and their co-workers, is a rare cardiac anomaly in which the systemic and pulmonary bloodstreams cross at the atrioventricular (A V) level. The malformation is now being diagnosed with increasing frequency, but successful surgical repair of associated anomalies has been reported only once." Straddling A V valve is another rare cardiac anomaly which now is being diagnosed preoperativelyt" and corrected surgically." This is the first report of successful repair of cardiac anomalies in a patient with the combination of straddling A V valve and criss-cross A V relationshi ps.
Case report A IO-year-old white boy was first seen at the Mayo Clinic on May 23, 1977, for evaluation of congenital heart disease. Acyanotic at birth, he was a poor feeder and gained little weight. A murmur, first noted at age 6 weeks, prompted a cardiac catheterization at 3 months that revealed situs inversus totalis, ventricular septal defect, and a possible doubleoutlet right ventricle. Overt symptoms of congestive heart failure necessitated medical treatment. A repeat catheterizaFrom the Mayo Clinic/Mayo Foundation, Rochester, Minn. (Drs. Danielson, Ritter, and Fulton) and the Departmentof Surgery, Michigan State University, East Lansing, Mich. (Dr. Tabry). Received for publication Nov. I, 1978. Accepted for publication Dec. 15, 1978. Address for reprints: Dr. Gordon K. Danielson, Thoracic and Cardiovascular Surgery, Mayo Clinic, 200 First st., S.W., Rochester, Minn. 55901.
tion at age 22 months confirmed systemic pressures in the pulmonary artery; pulmonary artery banding resulted in resolution of congestive heart failure. Cyanosis appeared 18 months prior to admission. A sister also had situs inversus totalis with dextrocardia, but no known heart disease. When seen at the Mayo Clinc , the patient was still active and able to swim and ride a bicycle. However, cyanosis had increased over the past few months and exercise-related headaches had begun. Height was 140 cm. and weight 29 kilograms. Cyanosis and clubbing were present. There was a right thoracotomy scar and the right side of the chest was prominent. The second heart sound was split and had a loud pulmonary component. There was a Grade 4/6 harsh ejection systolic murmur at the upper right sternal border transmitted to the back and a Grade 2/6 diastolic murmur at the right apex. The liver was palpable 1.5 ern. below the left costal margin. The hemoglobin level was 17.4 Gm per deciliter and the hematocrit value was 48.1 percent. No Howell-Jolly bodies were seen in the peripheral blood smear. A roentgenogram of the chest showed situs inversus totalis with dextrocardia, normal vascularity of the lungs, and a cardiothoracic ratio of 0.46. The electrocardiogram revealed normal sinus rhythm, a QRS axis of 110 degrees, and right ventricular hypertrophy. Cardiac catheterization was carried out before operation. The systemic flow was 3.5 L. per minute per square meter and the pulmonary flow 4.5 L. per minute per square meter with a pulmonary resistance of 2.9 units meter squared and a pulmonary arteriolar resistance of 0.7 units meter squared (Table I). Angiocardiography demonstrated situs inversus totalis and dextrocardia with a horizontal and sagittally oriented ventricular septum separating an anterior, superior, and rightward morphologically right ventricle from a posterior, inferior, and leftward morphologically left ventricle (Figs. I and 2). Both great arteries arose from the right ventricle. A tight band was present at the pulmonary valve level. There
0022-5223/79/060847+05$00.50/0 © 1979 The C. V. Mosby Co.
847
The Journal of Thoracic and Cardiovascular Surgery
848 Danielson et al .
Fig. 1. Angiogram performed in morphologically right ventricle showing dextrocardia with a horizontal and sagittally oriented ventricular septum separating the anterior. superior, and rightward morphologically right ventricle from a posterior. inferior. and leftward morphologically left ventricle. A large ventricular septal defect is present and a tight band is demonstrated on the pulmonary artery just distal to the pulmonary valve.
Table I. Cardiac catheterization data Site
Superiorvena cava Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Femoral artery
I
Pressure (mm . Hg)
Saturation (%)
68 10/0 115/1-6 19/9 15/3 116/5-10 124/90
82 98 89
was a subaortic ventricular septal defect and a straddling right-sided A V valve (a morphologically mitral valve between the left atrium and left ventricle) (Fig . 3) . An 80 degree phased-array sector echocardiographic scan confirmed the presence of a straddling right-sided A V valve (Fig . 4) and an injection of contrast medium in the left atrium produced simultaneous appearance of contrast echoes in both ventricles via the straddling A V valve. Operation was performed on May 3, 1977. through a primary median sternotomy. The heart was dissected free from dense adhesions. The preoperative diagnoses were confirmed (Fig . 5, A) . There was a large ventricular septal defect encompassing nearly the entire ventricular septum . The rightsided and somewhat posterior A V valve (mitral) straddled the septum, having papillary muscles and chordae attached to the right ventricular side of the septum as well as other papillary muscles attached to the left ventricle . There were also common chordae and papillary muscles shared by both the mitral
Fig. 2. Angiocardiogram performed in morphologically left ventricle . and tricuspid valves. Both the aorta and pulmonary artery exited from the right ventricle. although there was fibrous continuity between the mitral valve and the aorta . The aortic valve was located anterior and slightly to the left of the pulmonary valve. The right ventricle was opened anteriorly. Electrophysiological mapping with the assistance of Dr. James Maloney showed that the specialized conduction tissue coursed down a muscle ridge posteriorly from the right side of the left-sided (tricuspid) A V valve. This tissue could be seen as a white thread running in the subendocardium and could be traced down between the ventricles posteriorly . Closure of the ventricular septal defect necessitated dividing the mitral components of the common chordae to the mitral and tricuspid valves (Fig. 5, B). This left the mitral leaflet flail in one portion and necessitated replacement of the valve with a 21 mm. glutaraldehyde-preserved porcine xenograft through an incision in the left atrium on the left side of the heart anterior to the left pulmonary veins . The ventricular septal defect was closed with a large patch anchored superiorly along several centimeters of the tricuspid anulus and placed so as to direct the left ventricular blood out the aorta (Fig . 5. B). The pulmonary artery band was resected and an end-to-end anastomosis of the pulmonary artery was accomplished. The distal pulmonary artery was noted to be small . The pressures measured before chest closure were as follows: right atrium 14/10 mm . Hg , right ventricle 70/8, main pulmonary artery 30 systolic, left atrium 22/15, left ventricle 90/5, aorta 75/55, and arterial 85/55 mm . Hg. The postoperative course was uneventful and the patient was dismissed on the eleventh postoperative day receiving Lanoxin and Dyazide . On follow-up examination 16 months later," the patient *We are indebted to Dr. Sidney Friedman for these data.
Volume 77 Number 6 June. 1979
Criss-cross heart with straddling AV valve
Fig . 3. Left . Angiocardiogram performed in left atrium. Right . Film made by radiographic subtraction technique on a subsequent frame demon strating simultaneous filling of the right ventricle and left ventricle through a straddling mitral valve .
Fig. 4. Left. Eighty-degree phased-array sector echocardiographic scan (courtesy of Dr. Jame s Seward). Right. Diagram illustrating scan . Chordae from the mitral valve are attached to the right ventricular side of the septum. I . Inferior. S. Superior. R, Right. L. Left. RV, Right ventricle . VS, Ventricular septum . LV. Left ventricle. MV. Mitral valve . LA. Left atrium . AS, Atrial septum.
84 9
850 Danielson et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 5. A. Diagram illustrating the cardiac anatomy. A horizontal and sagittally oriented ventricular septum separates an anterior, superior. and rightward morphologically right ventricle (RV) from a posterior, inferior. and leftward morphologically left ventricle (LV). Both great arterie s arise from the right ventricle . A tight band is present just distal to the pulmonary valve. There is a large ventricular septal defect, and the chordae and papillary muscles from the left-sided tricusp id valve (TV) cross to the right-sided right ventricle . The right-sided mitral valve (MV) straddles the septum , having papillary muscles and chordae attached to the right ventricular side of the septum as well as papillary muscles attached to the left ventricle . There are also common chordae and papillary muscles in the right ventricle which are shared by both the mitral and tricuspid valves . B. Diagram illustrating cardiac repair. Closure of the ventricular septal defect with a patch necessitated dividing the mitral components of the common chordae to the mitral and tricuspid valves. The patch was placed so as to direct left ventricular blood out the aorta. The mitral valve was replaced with a glutaraldehyde-preserved porcine xenograft. the pulmonary artery band was resected, and an end-to-end anastomosis of the pulmonary artery was accomplished . was fully active, had good color, and was free of symptoms referable to the cardiovascular system . His cardiac medications had been discontinued. Several wire sutures had been removed from the sternum because of local discomfort. The second heart sound at the base of the heart was of normal intensity and was intennittently reduplicated. There was a Grade 4/6 ejection systolic murmur at the base of the heart. loudest at the second right intercostal space. related to the small pulmonary artery distal to the site of pulmonary artery band removal. A rumbling, low-pitched, mid-diastolic murmur was present at the cardiac apex. related to the porcine xenograft. The liver was not palpable . Right and left heart cardiac catheterization revealed no intracardiac shunts and no obstruction of the left ventricular outflow to the aorta. Pressures in the right ventricle and pulmonary artery were half the systemic pressure. The prosthetic mitral valve was function ing well.
Comment Rare cardiac malformations in which systemic and pulmonary bloodstreams cross at the A V level were
described pathologically as early as 1961. 9 • 10 Anderson;' Ando," and their co-workers independently introduced the term "criss-cross heart" in 1974. Since these original reports, criss-cross hearts are being diagnosed with increasing frequency , not only at autopsy, but also before death." 1t-15 The criss-cross heart is charac terized by the strange situation in which the right atrium drains through its A V valve into a ventricle situated primarily on the contralateral side (in situs solitus of viscera and atria, the ventricle receiving right atrial blood lies to the left) and the left atrium drains oppositely (in situs solitus, to the ventricle on the right) . Thus the routes of ventricular inflow from the atria cross each other. Criss-cross hearts can occur with both concordant and discordant A V relationships. Most patients, but not all, have associated ventriculoarterial discordance. To our knowledge, all cases reported to date have had situs
Volume 77 Number 6 June. 1979
solitus, as opposed to our patient, who had situs inversus totalis. The morphogenesis of criss-cross heart is considered to be abnormal rotation either in a clockwise or counterclockwise direction. 1. 3 Total repair of associated anomalies has been attempted on several occasions,": 14 but a successful outcome has been reported only once." Straddling A V valve, another rare cardiac anomaly, occurs when a part of the support mechanism of the A V valve extends across a ventricular septal defect to insert into the inappropriate (opposite) ventricle. The chordae that cross the ventricular septal defect may be attached anywhere on the endocardial surface of the ventricular septum or free wall of the inappropriate ventricle. Only recently have the premortem diagnostic features of straddling A V valve been elucidated.':" Suspicion of straddling A V valve can be raised by angiocardiography and M-mode echocardiography. The condition is suggested by angiocardiographic appearance of an A V valve orifice overriding a ventricular septal defect so that the orifice connects directly with its appropriate ventricle and also with the opposite ventricle. With both angiographic and echocardiographic study, the demonstration of instantaneous flow of blood into both ventricles after injection of contrast agent into the appropriate atrium gives supportive but not always pathognomonic evidence of straddling A V valve. The diagnosis has been made with great reliability, however, with introduction of the 80 degree phased-array sector echocardiographic scanner. Straddling A V valve poses a considerable technical problem in the management of patients with ventricular septal defect, many of whom have other additional cardiac anomalies. Few operative reports have appeared in the literature." When straddling A V valve has occurred in association with criss-cross A V relationships, palliative procedures generally have been performed in view of the complexity or presumed impossibility of complete repair. 13 The uneventful postoperative course and good result on follow-up in this patient are gratifying and suggest the combination of straddling A V valve and criss-cross heart can now be repaired with results as good as those for less complex forms of congential heart disease. REFERENCES Anderson RH, Shineborne EA, Gerlis LM: Criss-cross atrioventricular relationships producing paradoxical atri-
Criss-cross heart with straddling AV valve
2
3 4 5 6 7 8
9 10
II 12
13 14 15
85 I
oventricular concordance or discordance. Circulation 50:176-180, 1974 Ando M, Takao A, Cho E, et al: Criss-cross heart by abnormal rotation of bulboventricular loop. Diagnostic considerations for complex cardiac anomaly. Proc Pediatr Circ Soc No.4, 1974 (in Japanese) Sato K, Ohara S, Tsukaguchi I, et al: A criss-cross heart with concordant atrioventriculo-arterial connections. Circulation 57:396-400, 1978 LaCorte MA, Fellows KE, Williams RG: Overriding tricuspid valve. Echocardiographic and angiocardiographic features. Am J CardioI37:911-919, 1976 Liberthson RR, Paul MH, Muster AJ, et al: Straddling and displaced atrioventricular orifices and valves with primitive ventricles. Circulation 43:213-226, 1971 Seward JB, Tajik AJ, Ritter DG: Echocardiographic features of straddling tricuspid valve. Mayo Clin Proc 50:427-434, 1975 Tandon R, Becker AE, Moller JH, Edwards JE: Double inlet left ventricle. Straddling tricuspid valve. Br Heart J 36:747-759, 1974 Tabry IF, McGoon DC, Wallace RB, Danielson GK, Tajik AJ, Seward 18: Surgical management of straddling atrioventricular valve. J THORAC CARDIOVASC SURG 77:191-201, 1979 Van Praagh R: The segmental approach to diagnosis in congential heart disease. Birth Defects. Original Article Series 8:4-23, 1972 Lev M, Rowlatt UF: The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 9:216-263, 1961 Ando M, Takao A, Nihmura I, Mori K: Crossing atrioventricular valves. Clinical study of 8 cases. Circulation 54:Suppl 2:90, 1976 Symons JC, Shinebourne EA, Joseph MC, et a1: Crisscross heart with congenitally corrected transposition. Report of a case with d-transposed aorta and ventricular preexcitation. Eur J Cardiol 5/6:493-505, 1977 Sieg K, Hagler OJ, Ritter 00, et al: Straddling right atrioventricular valve in criss-cross atrioventricular relationship. Mayo Clin Proc 52:561-568, 1977 Anderson KR, Lie IT, Sieg K, et al: A criss-cross heart. Detailed anatomic description and discussion of morphogenesis. Mayo Clin Proc 52:569-575, 1977 Guthaner D, Higgins CB, Silverman JF, et al: An unusual form of the transposition complex. Uncorrected levotransposition with horizontal ventricular septum. Report of two cases. Circulation 53: 190-195, 1976
Gordon K. Danielson, M.D., Imad F. Tabry, M.D., Donald G. Ritter, M.D., and Richard E. Fulton, M.D., Rochester, Minn., and East Lansing, Mich.
T
he criss-cross heart, a term introduced independently by Anderson, t Ando," and their co-workers, is a rare cardiac anomaly in which the systemic and pulmonary bloodstreams cross at the atrioventricular (A V) level. The malformation is now being diagnosed with increasing frequency, but successful surgical repair of associated anomalies has been reported only once." Straddling A V valve is another rare cardiac anomaly which now is being diagnosed preoperativelyt" and corrected surgically." This is the first report of successful repair of cardiac anomalies in a patient with the combination of straddling A V valve and criss-cross A V relationshi ps.
Case report A IO-year-old white boy was first seen at the Mayo Clinic on May 23, 1977, for evaluation of congenital heart disease. Acyanotic at birth, he was a poor feeder and gained little weight. A murmur, first noted at age 6 weeks, prompted a cardiac catheterization at 3 months that revealed situs inversus totalis, ventricular septal defect, and a possible doubleoutlet right ventricle. Overt symptoms of congestive heart failure necessitated medical treatment. A repeat catheterizaFrom the Mayo Clinic/Mayo Foundation, Rochester, Minn. (Drs. Danielson, Ritter, and Fulton) and the Departmentof Surgery, Michigan State University, East Lansing, Mich. (Dr. Tabry). Received for publication Nov. I, 1978. Accepted for publication Dec. 15, 1978. Address for reprints: Dr. Gordon K. Danielson, Thoracic and Cardiovascular Surgery, Mayo Clinic, 200 First st., S.W., Rochester, Minn. 55901.
tion at age 22 months confirmed systemic pressures in the pulmonary artery; pulmonary artery banding resulted in resolution of congestive heart failure. Cyanosis appeared 18 months prior to admission. A sister also had situs inversus totalis with dextrocardia, but no known heart disease. When seen at the Mayo Clinc , the patient was still active and able to swim and ride a bicycle. However, cyanosis had increased over the past few months and exercise-related headaches had begun. Height was 140 cm. and weight 29 kilograms. Cyanosis and clubbing were present. There was a right thoracotomy scar and the right side of the chest was prominent. The second heart sound was split and had a loud pulmonary component. There was a Grade 4/6 harsh ejection systolic murmur at the upper right sternal border transmitted to the back and a Grade 2/6 diastolic murmur at the right apex. The liver was palpable 1.5 ern. below the left costal margin. The hemoglobin level was 17.4 Gm per deciliter and the hematocrit value was 48.1 percent. No Howell-Jolly bodies were seen in the peripheral blood smear. A roentgenogram of the chest showed situs inversus totalis with dextrocardia, normal vascularity of the lungs, and a cardiothoracic ratio of 0.46. The electrocardiogram revealed normal sinus rhythm, a QRS axis of 110 degrees, and right ventricular hypertrophy. Cardiac catheterization was carried out before operation. The systemic flow was 3.5 L. per minute per square meter and the pulmonary flow 4.5 L. per minute per square meter with a pulmonary resistance of 2.9 units meter squared and a pulmonary arteriolar resistance of 0.7 units meter squared (Table I). Angiocardiography demonstrated situs inversus totalis and dextrocardia with a horizontal and sagittally oriented ventricular septum separating an anterior, superior, and rightward morphologically right ventricle from a posterior, inferior, and leftward morphologically left ventricle (Figs. I and 2). Both great arteries arose from the right ventricle. A tight band was present at the pulmonary valve level. There
0022-5223/79/060847+05$00.50/0 © 1979 The C. V. Mosby Co.
847
The Journal of Thoracic and Cardiovascular Surgery
848 Danielson et al .
Fig. 1. Angiogram performed in morphologically right ventricle showing dextrocardia with a horizontal and sagittally oriented ventricular septum separating the anterior. superior, and rightward morphologically right ventricle from a posterior. inferior. and leftward morphologically left ventricle. A large ventricular septal defect is present and a tight band is demonstrated on the pulmonary artery just distal to the pulmonary valve.
Table I. Cardiac catheterization data Site
Superiorvena cava Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Femoral artery
I
Pressure (mm . Hg)
Saturation (%)
68 10/0 115/1-6 19/9 15/3 116/5-10 124/90
82 98 89
was a subaortic ventricular septal defect and a straddling right-sided A V valve (a morphologically mitral valve between the left atrium and left ventricle) (Fig . 3) . An 80 degree phased-array sector echocardiographic scan confirmed the presence of a straddling right-sided A V valve (Fig . 4) and an injection of contrast medium in the left atrium produced simultaneous appearance of contrast echoes in both ventricles via the straddling A V valve. Operation was performed on May 3, 1977. through a primary median sternotomy. The heart was dissected free from dense adhesions. The preoperative diagnoses were confirmed (Fig . 5, A) . There was a large ventricular septal defect encompassing nearly the entire ventricular septum . The rightsided and somewhat posterior A V valve (mitral) straddled the septum, having papillary muscles and chordae attached to the right ventricular side of the septum as well as other papillary muscles attached to the left ventricle . There were also common chordae and papillary muscles shared by both the mitral
Fig. 2. Angiocardiogram performed in morphologically left ventricle . and tricuspid valves. Both the aorta and pulmonary artery exited from the right ventricle. although there was fibrous continuity between the mitral valve and the aorta . The aortic valve was located anterior and slightly to the left of the pulmonary valve. The right ventricle was opened anteriorly. Electrophysiological mapping with the assistance of Dr. James Maloney showed that the specialized conduction tissue coursed down a muscle ridge posteriorly from the right side of the left-sided (tricuspid) A V valve. This tissue could be seen as a white thread running in the subendocardium and could be traced down between the ventricles posteriorly . Closure of the ventricular septal defect necessitated dividing the mitral components of the common chordae to the mitral and tricuspid valves (Fig. 5, B). This left the mitral leaflet flail in one portion and necessitated replacement of the valve with a 21 mm. glutaraldehyde-preserved porcine xenograft through an incision in the left atrium on the left side of the heart anterior to the left pulmonary veins . The ventricular septal defect was closed with a large patch anchored superiorly along several centimeters of the tricuspid anulus and placed so as to direct the left ventricular blood out the aorta (Fig . 5. B). The pulmonary artery band was resected and an end-to-end anastomosis of the pulmonary artery was accomplished. The distal pulmonary artery was noted to be small . The pressures measured before chest closure were as follows: right atrium 14/10 mm . Hg , right ventricle 70/8, main pulmonary artery 30 systolic, left atrium 22/15, left ventricle 90/5, aorta 75/55, and arterial 85/55 mm . Hg. The postoperative course was uneventful and the patient was dismissed on the eleventh postoperative day receiving Lanoxin and Dyazide . On follow-up examination 16 months later," the patient *We are indebted to Dr. Sidney Friedman for these data.
Volume 77 Number 6 June. 1979
Criss-cross heart with straddling AV valve
Fig . 3. Left . Angiocardiogram performed in left atrium. Right . Film made by radiographic subtraction technique on a subsequent frame demon strating simultaneous filling of the right ventricle and left ventricle through a straddling mitral valve .
Fig. 4. Left. Eighty-degree phased-array sector echocardiographic scan (courtesy of Dr. Jame s Seward). Right. Diagram illustrating scan . Chordae from the mitral valve are attached to the right ventricular side of the septum. I . Inferior. S. Superior. R, Right. L. Left. RV, Right ventricle . VS, Ventricular septum . LV. Left ventricle. MV. Mitral valve . LA. Left atrium . AS, Atrial septum.
84 9
850 Danielson et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 5. A. Diagram illustrating the cardiac anatomy. A horizontal and sagittally oriented ventricular septum separates an anterior, superior. and rightward morphologically right ventricle (RV) from a posterior, inferior. and leftward morphologically left ventricle (LV). Both great arterie s arise from the right ventricle . A tight band is present just distal to the pulmonary valve. There is a large ventricular septal defect, and the chordae and papillary muscles from the left-sided tricusp id valve (TV) cross to the right-sided right ventricle . The right-sided mitral valve (MV) straddles the septum , having papillary muscles and chordae attached to the right ventricular side of the septum as well as papillary muscles attached to the left ventricle . There are also common chordae and papillary muscles in the right ventricle which are shared by both the mitral and tricuspid valves . B. Diagram illustrating cardiac repair. Closure of the ventricular septal defect with a patch necessitated dividing the mitral components of the common chordae to the mitral and tricuspid valves. The patch was placed so as to direct left ventricular blood out the aorta. The mitral valve was replaced with a glutaraldehyde-preserved porcine xenograft. the pulmonary artery band was resected, and an end-to-end anastomosis of the pulmonary artery was accomplished . was fully active, had good color, and was free of symptoms referable to the cardiovascular system . His cardiac medications had been discontinued. Several wire sutures had been removed from the sternum because of local discomfort. The second heart sound at the base of the heart was of normal intensity and was intennittently reduplicated. There was a Grade 4/6 ejection systolic murmur at the base of the heart. loudest at the second right intercostal space. related to the small pulmonary artery distal to the site of pulmonary artery band removal. A rumbling, low-pitched, mid-diastolic murmur was present at the cardiac apex. related to the porcine xenograft. The liver was not palpable . Right and left heart cardiac catheterization revealed no intracardiac shunts and no obstruction of the left ventricular outflow to the aorta. Pressures in the right ventricle and pulmonary artery were half the systemic pressure. The prosthetic mitral valve was function ing well.
Comment Rare cardiac malformations in which systemic and pulmonary bloodstreams cross at the A V level were
described pathologically as early as 1961. 9 • 10 Anderson;' Ando," and their co-workers independently introduced the term "criss-cross heart" in 1974. Since these original reports, criss-cross hearts are being diagnosed with increasing frequency , not only at autopsy, but also before death." 1t-15 The criss-cross heart is charac terized by the strange situation in which the right atrium drains through its A V valve into a ventricle situated primarily on the contralateral side (in situs solitus of viscera and atria, the ventricle receiving right atrial blood lies to the left) and the left atrium drains oppositely (in situs solitus, to the ventricle on the right) . Thus the routes of ventricular inflow from the atria cross each other. Criss-cross hearts can occur with both concordant and discordant A V relationships. Most patients, but not all, have associated ventriculoarterial discordance. To our knowledge, all cases reported to date have had situs
Volume 77 Number 6 June. 1979
solitus, as opposed to our patient, who had situs inversus totalis. The morphogenesis of criss-cross heart is considered to be abnormal rotation either in a clockwise or counterclockwise direction. 1. 3 Total repair of associated anomalies has been attempted on several occasions,": 14 but a successful outcome has been reported only once." Straddling A V valve, another rare cardiac anomaly, occurs when a part of the support mechanism of the A V valve extends across a ventricular septal defect to insert into the inappropriate (opposite) ventricle. The chordae that cross the ventricular septal defect may be attached anywhere on the endocardial surface of the ventricular septum or free wall of the inappropriate ventricle. Only recently have the premortem diagnostic features of straddling A V valve been elucidated.':" Suspicion of straddling A V valve can be raised by angiocardiography and M-mode echocardiography. The condition is suggested by angiocardiographic appearance of an A V valve orifice overriding a ventricular septal defect so that the orifice connects directly with its appropriate ventricle and also with the opposite ventricle. With both angiographic and echocardiographic study, the demonstration of instantaneous flow of blood into both ventricles after injection of contrast agent into the appropriate atrium gives supportive but not always pathognomonic evidence of straddling A V valve. The diagnosis has been made with great reliability, however, with introduction of the 80 degree phased-array sector echocardiographic scanner. Straddling A V valve poses a considerable technical problem in the management of patients with ventricular septal defect, many of whom have other additional cardiac anomalies. Few operative reports have appeared in the literature." When straddling A V valve has occurred in association with criss-cross A V relationships, palliative procedures generally have been performed in view of the complexity or presumed impossibility of complete repair. 13 The uneventful postoperative course and good result on follow-up in this patient are gratifying and suggest the combination of straddling A V valve and criss-cross heart can now be repaired with results as good as those for less complex forms of congential heart disease. REFERENCES Anderson RH, Shineborne EA, Gerlis LM: Criss-cross atrioventricular relationships producing paradoxical atri-
Criss-cross heart with straddling AV valve
2
3 4 5 6 7 8
9 10
II 12
13 14 15
85 I
oventricular concordance or discordance. Circulation 50:176-180, 1974 Ando M, Takao A, Cho E, et al: Criss-cross heart by abnormal rotation of bulboventricular loop. Diagnostic considerations for complex cardiac anomaly. Proc Pediatr Circ Soc No.4, 1974 (in Japanese) Sato K, Ohara S, Tsukaguchi I, et al: A criss-cross heart with concordant atrioventriculo-arterial connections. Circulation 57:396-400, 1978 LaCorte MA, Fellows KE, Williams RG: Overriding tricuspid valve. Echocardiographic and angiocardiographic features. Am J CardioI37:911-919, 1976 Liberthson RR, Paul MH, Muster AJ, et al: Straddling and displaced atrioventricular orifices and valves with primitive ventricles. Circulation 43:213-226, 1971 Seward JB, Tajik AJ, Ritter DG: Echocardiographic features of straddling tricuspid valve. Mayo Clin Proc 50:427-434, 1975 Tandon R, Becker AE, Moller JH, Edwards JE: Double inlet left ventricle. Straddling tricuspid valve. Br Heart J 36:747-759, 1974 Tabry IF, McGoon DC, Wallace RB, Danielson GK, Tajik AJ, Seward 18: Surgical management of straddling atrioventricular valve. J THORAC CARDIOVASC SURG 77:191-201, 1979 Van Praagh R: The segmental approach to diagnosis in congential heart disease. Birth Defects. Original Article Series 8:4-23, 1972 Lev M, Rowlatt UF: The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 9:216-263, 1961 Ando M, Takao A, Nihmura I, Mori K: Crossing atrioventricular valves. Clinical study of 8 cases. Circulation 54:Suppl 2:90, 1976 Symons JC, Shinebourne EA, Joseph MC, et a1: Crisscross heart with congenitally corrected transposition. Report of a case with d-transposed aorta and ventricular preexcitation. Eur J Cardiol 5/6:493-505, 1977 Sieg K, Hagler OJ, Ritter 00, et al: Straddling right atrioventricular valve in criss-cross atrioventricular relationship. Mayo Clin Proc 52:561-568, 1977 Anderson KR, Lie IT, Sieg K, et al: A criss-cross heart. Detailed anatomic description and discussion of morphogenesis. Mayo Clin Proc 52:569-575, 1977 Guthaner D, Higgins CB, Silverman JF, et al: An unusual form of the transposition complex. Uncorrected levotransposition with horizontal ventricular septum. Report of two cases. Circulation 53: 190-195, 1976