Surgical Correction of Unusual Double-Outlet Right Ventricle With Pulmonary Stenosis and Severe Tricuspid Insufficiency in the Presence of Dextrocardia with Ventricular Discordance W. C. P. Geldof, M.D., J. Rohmer, M.D., A. Oppenheimer-Dekker, M.D., Z. Aytug, M.D., and A. G. Brom, M.D. ABSTRACT This paper presents the case history of an 8-year-oldgirl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a BjorkShiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examinationrevealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made.
W
ith the continuing expansion of surgical possibilities, more and more patients with complicated congenital heart defects are being considered for operative repair. As early as 1968, Kiser and associates [6] described 6 patients who had dextrocardia with inversion of the ventricles and a double-outlet right ventricle with pulmonary stenosis, 3 of whom underwent repair. Depending on the degree of pulmonary stenosis and the size and location of the ventricular septa1 defect (VSD), it is necessary in some patients to use a valve-bearing tube for reconstruction of the outflow tract from the right ventricle to the pulmonary artery. This procedure was first described by Rastelli and colleagues [ 123 in 1965 for correction of pulmonary valve atresia and has since been used by many others for correction of a variety of congenital heart defects such as persistent truncus arteriosus [4, 8, 171, transposition of the great vessels with VSD and pulmonary stenosis [ l , 10, 111, and pulmonary atresia [7, 13, 14, 181. In view of the encouraging results of these corrections [9], it seems quite justifiable, after careful selection, to continue these reconstructive interventions for functional correction. It is for this reason that w e wish to present the case history of an 8-year-old girl with a complicated congenital anomaly of the heart From the Departments of Thoracic Surgery and Pediatric Cardiology and the Laboratory of Anatomy and Embryology, University Hospital, Leiden, The Netherlands. Accepted for publication Dec. 31, 1974. Address reprint requests to Dr. Brom, Department of Thoracic Surgery, University Hospital, Leiden, T h e Netherlands.
VOL. 20, NO. 4, OCTOBER, 1975
46 1
GELDOF ET AL. who underwent a successful operative correction but unfortunately died a few months later. The patient was a girl aged 8 years who was referred to us from Surinam for analysisof a previously diagnosed congenital heart defect. The history mentioned recurrent respiratory infections and recent complaints of dyspnea and poor appetite. Growth had been retarded. Examination disclosed marked cyanosis of the lips and clubbed fingers and toes. Her body weight was 2 1 kg for a height of 104cm. The pulse was regular and equal; pulse rate was 96 per minute. The blood pressure was 110/85.Auscultation revealed a grade 3-4/6 systolic murmur in the fourth intercostal space to the left and the third intercostal space to the right of the sternum. The left-sided liver was not enlarged. There were good femoral pulses. Laboratory studies showed hemoglobin concentration to be 19.3 gm per 100 ml, hematocrit SO%, and leukocyte count 6,100. On radiological examination there proved to be total situs inversus of the abdominal organs without an asplenic syndrome; scanning showed the spleen to be on the right side. The chest roentgenogram revealed dextrocardia and an aortic arch to the right. The electrocardiogram showed sinus rhythm with firstdegree atrioventricular block and hypertrophy of the right-sided ventricle. Heart catheterization and biplane cineangiocardiography yielded the following findings: the venae cavae and venous atrium were located on the left; the corresponding venous ventricle, which showed the anatomical structure of a left ventricle, emptied through a VSD. Both the aorta and the pulmonary artery originated side by side from the ventricle situated on the right, beneath the atrium into which the lung veins drained; this ventricle had the anatomical structure of a right ventricle. Marked valvular and subvalvular pulmonary stenosis was present. Finally, the two atria were found to communicate through an atrial septa1 defect and the aorta to descend on the right. It should be emphasized that no insufficiency of the two atrioventricular valves was noted, although review of the cineangiocardiograms showed in retrospect that the atrioventricular valve located on the right was undoubtedly insufficient. The systolic pressures in the two ventricles and the aorta were all about the same; the pulmonary artery was not reached.
Operation The approach was through a midline sternotomy. Inspection revealed the anatomical situation to be as expected from the catheterization findings (Fig. 1A). The two venae cavae were located on the left, as was the venous atrium with the corresponding anatomical left ventricle from which none of the great vessels arose. The atrium into which the lung veins drained was situated strikingly low on the right side, and from the corresponding anatomical right ventricle arose both the aorta, with its arch to the right, and the pulmonary artery. The diameter of the latter was less than half that of the ascending aorta. Another conspicuous finding
462
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
A
Unusual Double-Outlet Right Ventricle
B
FIG. 1 . (A) I n this patient the venae cavae and venous atrium were located on the left; the lung veins drained into an atrium situated strikingly low on the right side of the heart. There was ventricular discordance with marked pulmonary stenosis. The VSD and the insufficient tricuspid ualve are indicated. (B) Following repair the VSD and the proximal pulmonary artery stump have been closed. A Hancock prosthesis has been used to create a communication between the venous ventricle located on the left and the dktal segment of the pulmonary a r t q . The tricuspid valve has been replaced by a Bjork-Shiley prosthesis.
was a very marked thrill that was palpable on the atrium located low on the right side of the heart, indicating marked insufficiency of the tricuspid valve. After heparinization, the ascending aorta and the two venae cavae were cannulated and a decompression cannula was inserted into the ventricle located on the right. Circulation and oxygenation were then taken over by a heart-lung machine with a Temptrol oxygenator, and body temperature was reduced to a minimum of 18°C. A small atrial septa1 defect was closed, and the venous ventricle situated on the left was then opened with a transverse incision in the lateral wall. The other ventricle, from which the two great vessels arose, was then reached through an oval VSD measuring about 3.5 x 1.5 cm. The trunk of the pulmonary artery was dissected free all around and divided transversely immediately proximal to the stenosed valves. The proximal segment was closed in three layers, after which the VSD was closed with a Dacron patch. The location of the bundle of His could not be predicted with certainty. After enlarging the opening in the lateral wall of the anatomical left ventricle, in which an apparently sufficient mitral valve of normal appearance was found, a Hancock vascular prosthesis [2] was interposed between this opening and the distal end of the trunk of the pulmonary artery (Fig. 1B);the Hancock prosthesis had a porcine valve with a diameter of 18 mm. Immediately after this reconstruction the heart did not function well, and therefore it was decided to open the atrium located on the right into which all lung veins drained. The atrioventricular valves proved to be extremely deformed tricuspid valves that could not possibly close sufficiently. They were resected and replaced with a reversed Bjork-Shiley
VOL. 20. NO. 4, OCTOBER, 1975
463
GELDOF ET AL.
FIG. 2 . ( A ) Frontal and ( B ) lateral view of a control angiocardiogram (about three weeks after operation), showing good filling of the new outflow tract of the left-sided ventricle and the pulmonary artery.
prosthesis with a diameter of 27 mm. After careful venting 131 and defibrillation, the heart immediately took over and was soon able to maintain a good circulation independently. The ECG did show atrioventricular dissociation with periods of nodal rhythm but definitely excluded total block. As a precaution, pacemaker electrodes were sutured onto the heart.
Postoperative Course and Convalescence T h e postoperative course was uneventful, and after about three weeks the patient was transferred to a nursing home for further convalescence in anticipation of the return voyage to Surinam. At that time the electrocardiogram again showed a sinus rhythm with first-degree atrioventricular block and complete bundle-branch block. Follow-up catheterization disclosed normal oxygen saturations everywhere with a still slightly increased pressure in the right ventricle (60-40/0-5); the pressure in the pulmonary artery was 30/15. Angiocardiography revealed adequate filling of the pulmonary artery (Fig. 2), and the BjorkShiley prosthesis was functioning well (Fig. 3). Subsequent convalescence was likewise uneventful; specifically, no signs of cardiac decompensation occurred. Medication with digitalis and diuretics was discontinued, ultimately leaving the patient with anticoagulants as her sole medication. About two months after the operation her cardiac and general condition was such that a return to Surinam seemed justifiable. On the day before her departure the patient was very nervous, vomited a little on two occasions, and had tachycardia (1OO/min) and tachypnea (76/min), in view of which she was given sedatives. On her way to the aircraft she vomited once more and refused to walk unaided. She died suddenly on board the aircraft shortly after takeoff.
464
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Unusual Double-Outlet Right Ventricle
FIG. 3 . ( A ) Frontal and (B) lateral view of a control angiocardiogram (about three weeks after operation), showing good closure of the Bjork-Shiley valve prosthesis in the atrioventricular position of the right-sided ventricle.
Postmortem Findings N o anomalies other than those already established were found in the heart. An anatomically good operative result was evident. Both pleural spaces were found to contain about 300 ml of fluid, possibly indicating that cardiac decompensation had nevertheless existed. Otherwise, nothing was found that could explain the sudden death.
Anatomy and Embryology As Figure 1 shows, the aorta is transposed, ascending more or less vertically from the ventricle located on the right, in which the wide aortic orifice is situated on the right and slightly ventral to the narrow pulmonary infundibulum. Venous and arterial orifices are separated by a muscular band that morphologically resembles the crista supraventricularis but embryologically is the retained posteromedial portion of the conus cordis, usually called the embryonic conoventricular fold [ 151. The interventricular septum has a trabeculated surface. The ventricle situated on the right therefore has the anatomical characteristics of a right ventricle, as could be expected of a double-outlet ventricle. The ventricle located on the left, with its smooth septa1 surface and readily recognizable mitral valve, has the anatomical features of a left ventricle. The localization of the conducting system in the interventricular septum has not been investigated. The atria with the veins and coronary sinus, however, are in transposition (i.e., with the limbus fossae ovalis on the left side of the atrial septum) in accordance with the abdominal situs inversus. Adopting the terminology introduced by
VOL. 20, NO. 4, OCTOBER, 1975
465
GELDOF ET AL. Van Praagh and co-workers [ 161 and Hollman [5], this situation can be described as ventricular discordance: the position of the ventricles is not in accordance with the position of the atria and of the abdominal organs (the atria and abdominal situs are usually concordant owing to the embryological “bonds” between liver, inferior vena cava, and right atrium; in the rare instances of atrial discordance, therefore, the inferior vena cava is nearly always absent, and the link between liver and right atrium is lacking). Hollman [5] pointed out that in ventricular discordance the great arteries are always transposed. Frequent accompanying anomalies are pulmonary stenosis or atresia and VSD. In the patient under discussion there was in addition a double-outlet right ventricle with the arterial orifices side by side at about the same level. In normal embryological development the aortic canal, as part of the truncus arteriosus, is still situated entirely above the right ventricle in the fifth week. Early in the sixth week (10 to 15 mm crown-rump length) the left and right bulbar ridges grow in such a manner and direction that the aortic orifice communicates with the left ventricle obliquely behind the pulmonary orifice through the primary interventricular foramen (which at this point becomes the vestibule of the aorta). In a normal embryo of 18 to 19 mm crown-rump length, only a vestige of the interventricular communication is recognizable. In a defective embryo, at the start of the seventh week of gestation retarded development in the bulbus-truncus area appears to cause the aortic orifice to retain a position on the extreme right next to the pulmonary orifice and therefore above the right ventricle: double-outlet right ventricle with VSD. In this embryo the two orifices are still wide, but it is obvious that in this anomaly the pulmonary orifice, enclosed between the aortic orifice and ventricular septum, has a rather vulnerable position and runs a relatively high risk of becoming stenotic in the course of fetal development.
References 1 . Asfaou, E., Geldof, W., d e Villeneuve, V. H., Rohmer, J., and Brom, A. G. Traitement anatomique des transpositions des gros vaisseaux avec stenose pulmonaire. Ann Chir Thorac Cardiovasc 11:247, 1972. 2. Bowman, F. O., Hancock, W. D., and Malm, J. R. A valve-containing Dacron prosthesis. Arch Surg 107:724, 1973. 3. Geldof, W. C. P., Aytug, Z., and Brom, A. G. Die Entliiftung der Aorta nach extrakorporalem Kreislauf. Thoraxchirurgie 20:2 18, 1972. 4. Gomes M. M. R., and McGoon, D. C. Truncus arteriosus with interruption of the aortic arch: Report of a case successfully repaired. Mayo Clin Proc 46:40, 1971. 5. Hollman, A. Dextrocardia, Levocardia and Mesocardia. In H. Watson (Ed),Paedzatric Cardiology. London: Lloyd-Luke, 1968. P 668. 6. Kiser, J. C., Ongley, P. A., Kirklin, J. W., Clarkson, P. M., and McGoon, D. C. Surgical treatment of dextrocardia with inversion of ventricles and double-outlet right ventricle. J Thorac Cardiovasc Surg 55:6, 1968. 7. Kouchoukos, N. T., Barcia, A., Bargeron, L. M., and Kirklin, J. W. Surgical treatment of congenital pulmonary atresia with ventricular septa1 defect. J Thorac Cardiovasc Surg 61:70, 1971. 8. McGoon, D. C., Rastelli, G. C., and Ongley, P. A. An operation for the correction of truncus arteriosus.JAMA 205:59, 1968.
466
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Unusual Double-Outlet Right Ventricle
9. McGoon, D. C., Wallace, R. B., and Danielson, G. K. T h e Rastelli operation: Its indications and results. J Thorac Cardiovasc Surg 65:65, 1973. 10. Rastelli, G. C. A new approach to anatomic repair of transposition of the great arteries. Mayo Clin Proc 44: 1, 1969. 1 1 . Rastelli, G. C., McGoon, D. C., and Wallace, R. B. Anatomic correction of transposition of the great arteries with ventricular septa1 defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 58:545, 1969. 12. Rastelli, G. C., Ongley, P. A., Davis, G. D., and Kirklin, J. W. Surgical repair for pulmonary valve atresia with coronary-pulmonary artery fistula: Report of case. Mayo Clin Proc 40:521, 1965. 13. Ross, D. N., and Somerville, J. Correction of pulmonary atresia with a homograft aortic valve. Lancet 2:1446, 1966. 14. Somerville, J., and Ross, D. N. Long-term results of complete correction with homograft reconstruction in pulmonary outflow tract atresia. Br Heart J 34:29, 1972. 15. Van Mierop, L. H. S., and Gessner, I. H. Pathogenetic mechanisms in congenital cardiovascular malformations. Prog Cardiouasc Dzi 15:67, 1972. 16. Van Praagh, R., Van Praagh, S., Vlad, P., and Keith, J. D. Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications. A m J Cardiol 13:510, 1964. 17. Wallace, R. B., Rastelli, G. C., Ongley, P. A., Titus, J. L., and McGoon, D. C. Complete repair of truncus arteriosus. J Thorac Cardiouasc Surg 57:95, 1969. 18. Weldon, C. S., Rowe, R. D., and Gott, V. L. Clinical experience with the use of aortic valve homografts for reconstruction of the pulmonary artery, pulmonary valve and outflow portion of the right ventricle. Circulation 37, 38 (Suppl 11): 51, 1968.
VOL. 20, NO. 4, OCTOBER, 1975
467
W
ith the continuing expansion of surgical possibilities, more and more patients with complicated congenital heart defects are being considered for operative repair. As early as 1968, Kiser and associates [6] described 6 patients who had dextrocardia with inversion of the ventricles and a double-outlet right ventricle with pulmonary stenosis, 3 of whom underwent repair. Depending on the degree of pulmonary stenosis and the size and location of the ventricular septa1 defect (VSD), it is necessary in some patients to use a valve-bearing tube for reconstruction of the outflow tract from the right ventricle to the pulmonary artery. This procedure was first described by Rastelli and colleagues [ 123 in 1965 for correction of pulmonary valve atresia and has since been used by many others for correction of a variety of congenital heart defects such as persistent truncus arteriosus [4, 8, 171, transposition of the great vessels with VSD and pulmonary stenosis [ l , 10, 111, and pulmonary atresia [7, 13, 14, 181. In view of the encouraging results of these corrections [9], it seems quite justifiable, after careful selection, to continue these reconstructive interventions for functional correction. It is for this reason that w e wish to present the case history of an 8-year-old girl with a complicated congenital anomaly of the heart From the Departments of Thoracic Surgery and Pediatric Cardiology and the Laboratory of Anatomy and Embryology, University Hospital, Leiden, The Netherlands. Accepted for publication Dec. 31, 1974. Address reprint requests to Dr. Brom, Department of Thoracic Surgery, University Hospital, Leiden, T h e Netherlands.
VOL. 20, NO. 4, OCTOBER, 1975
46 1
GELDOF ET AL. who underwent a successful operative correction but unfortunately died a few months later. The patient was a girl aged 8 years who was referred to us from Surinam for analysisof a previously diagnosed congenital heart defect. The history mentioned recurrent respiratory infections and recent complaints of dyspnea and poor appetite. Growth had been retarded. Examination disclosed marked cyanosis of the lips and clubbed fingers and toes. Her body weight was 2 1 kg for a height of 104cm. The pulse was regular and equal; pulse rate was 96 per minute. The blood pressure was 110/85.Auscultation revealed a grade 3-4/6 systolic murmur in the fourth intercostal space to the left and the third intercostal space to the right of the sternum. The left-sided liver was not enlarged. There were good femoral pulses. Laboratory studies showed hemoglobin concentration to be 19.3 gm per 100 ml, hematocrit SO%, and leukocyte count 6,100. On radiological examination there proved to be total situs inversus of the abdominal organs without an asplenic syndrome; scanning showed the spleen to be on the right side. The chest roentgenogram revealed dextrocardia and an aortic arch to the right. The electrocardiogram showed sinus rhythm with firstdegree atrioventricular block and hypertrophy of the right-sided ventricle. Heart catheterization and biplane cineangiocardiography yielded the following findings: the venae cavae and venous atrium were located on the left; the corresponding venous ventricle, which showed the anatomical structure of a left ventricle, emptied through a VSD. Both the aorta and the pulmonary artery originated side by side from the ventricle situated on the right, beneath the atrium into which the lung veins drained; this ventricle had the anatomical structure of a right ventricle. Marked valvular and subvalvular pulmonary stenosis was present. Finally, the two atria were found to communicate through an atrial septa1 defect and the aorta to descend on the right. It should be emphasized that no insufficiency of the two atrioventricular valves was noted, although review of the cineangiocardiograms showed in retrospect that the atrioventricular valve located on the right was undoubtedly insufficient. The systolic pressures in the two ventricles and the aorta were all about the same; the pulmonary artery was not reached.
Operation The approach was through a midline sternotomy. Inspection revealed the anatomical situation to be as expected from the catheterization findings (Fig. 1A). The two venae cavae were located on the left, as was the venous atrium with the corresponding anatomical left ventricle from which none of the great vessels arose. The atrium into which the lung veins drained was situated strikingly low on the right side, and from the corresponding anatomical right ventricle arose both the aorta, with its arch to the right, and the pulmonary artery. The diameter of the latter was less than half that of the ascending aorta. Another conspicuous finding
462
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
A
Unusual Double-Outlet Right Ventricle
B
FIG. 1 . (A) I n this patient the venae cavae and venous atrium were located on the left; the lung veins drained into an atrium situated strikingly low on the right side of the heart. There was ventricular discordance with marked pulmonary stenosis. The VSD and the insufficient tricuspid ualve are indicated. (B) Following repair the VSD and the proximal pulmonary artery stump have been closed. A Hancock prosthesis has been used to create a communication between the venous ventricle located on the left and the dktal segment of the pulmonary a r t q . The tricuspid valve has been replaced by a Bjork-Shiley prosthesis.
was a very marked thrill that was palpable on the atrium located low on the right side of the heart, indicating marked insufficiency of the tricuspid valve. After heparinization, the ascending aorta and the two venae cavae were cannulated and a decompression cannula was inserted into the ventricle located on the right. Circulation and oxygenation were then taken over by a heart-lung machine with a Temptrol oxygenator, and body temperature was reduced to a minimum of 18°C. A small atrial septa1 defect was closed, and the venous ventricle situated on the left was then opened with a transverse incision in the lateral wall. The other ventricle, from which the two great vessels arose, was then reached through an oval VSD measuring about 3.5 x 1.5 cm. The trunk of the pulmonary artery was dissected free all around and divided transversely immediately proximal to the stenosed valves. The proximal segment was closed in three layers, after which the VSD was closed with a Dacron patch. The location of the bundle of His could not be predicted with certainty. After enlarging the opening in the lateral wall of the anatomical left ventricle, in which an apparently sufficient mitral valve of normal appearance was found, a Hancock vascular prosthesis [2] was interposed between this opening and the distal end of the trunk of the pulmonary artery (Fig. 1B);the Hancock prosthesis had a porcine valve with a diameter of 18 mm. Immediately after this reconstruction the heart did not function well, and therefore it was decided to open the atrium located on the right into which all lung veins drained. The atrioventricular valves proved to be extremely deformed tricuspid valves that could not possibly close sufficiently. They were resected and replaced with a reversed Bjork-Shiley
VOL. 20. NO. 4, OCTOBER, 1975
463
GELDOF ET AL.
FIG. 2 . ( A ) Frontal and ( B ) lateral view of a control angiocardiogram (about three weeks after operation), showing good filling of the new outflow tract of the left-sided ventricle and the pulmonary artery.
prosthesis with a diameter of 27 mm. After careful venting 131 and defibrillation, the heart immediately took over and was soon able to maintain a good circulation independently. The ECG did show atrioventricular dissociation with periods of nodal rhythm but definitely excluded total block. As a precaution, pacemaker electrodes were sutured onto the heart.
Postoperative Course and Convalescence T h e postoperative course was uneventful, and after about three weeks the patient was transferred to a nursing home for further convalescence in anticipation of the return voyage to Surinam. At that time the electrocardiogram again showed a sinus rhythm with first-degree atrioventricular block and complete bundle-branch block. Follow-up catheterization disclosed normal oxygen saturations everywhere with a still slightly increased pressure in the right ventricle (60-40/0-5); the pressure in the pulmonary artery was 30/15. Angiocardiography revealed adequate filling of the pulmonary artery (Fig. 2), and the BjorkShiley prosthesis was functioning well (Fig. 3). Subsequent convalescence was likewise uneventful; specifically, no signs of cardiac decompensation occurred. Medication with digitalis and diuretics was discontinued, ultimately leaving the patient with anticoagulants as her sole medication. About two months after the operation her cardiac and general condition was such that a return to Surinam seemed justifiable. On the day before her departure the patient was very nervous, vomited a little on two occasions, and had tachycardia (1OO/min) and tachypnea (76/min), in view of which she was given sedatives. On her way to the aircraft she vomited once more and refused to walk unaided. She died suddenly on board the aircraft shortly after takeoff.
464
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Unusual Double-Outlet Right Ventricle
FIG. 3 . ( A ) Frontal and (B) lateral view of a control angiocardiogram (about three weeks after operation), showing good closure of the Bjork-Shiley valve prosthesis in the atrioventricular position of the right-sided ventricle.
Postmortem Findings N o anomalies other than those already established were found in the heart. An anatomically good operative result was evident. Both pleural spaces were found to contain about 300 ml of fluid, possibly indicating that cardiac decompensation had nevertheless existed. Otherwise, nothing was found that could explain the sudden death.
Anatomy and Embryology As Figure 1 shows, the aorta is transposed, ascending more or less vertically from the ventricle located on the right, in which the wide aortic orifice is situated on the right and slightly ventral to the narrow pulmonary infundibulum. Venous and arterial orifices are separated by a muscular band that morphologically resembles the crista supraventricularis but embryologically is the retained posteromedial portion of the conus cordis, usually called the embryonic conoventricular fold [ 151. The interventricular septum has a trabeculated surface. The ventricle situated on the right therefore has the anatomical characteristics of a right ventricle, as could be expected of a double-outlet ventricle. The ventricle located on the left, with its smooth septa1 surface and readily recognizable mitral valve, has the anatomical features of a left ventricle. The localization of the conducting system in the interventricular septum has not been investigated. The atria with the veins and coronary sinus, however, are in transposition (i.e., with the limbus fossae ovalis on the left side of the atrial septum) in accordance with the abdominal situs inversus. Adopting the terminology introduced by
VOL. 20, NO. 4, OCTOBER, 1975
465
GELDOF ET AL. Van Praagh and co-workers [ 161 and Hollman [5], this situation can be described as ventricular discordance: the position of the ventricles is not in accordance with the position of the atria and of the abdominal organs (the atria and abdominal situs are usually concordant owing to the embryological “bonds” between liver, inferior vena cava, and right atrium; in the rare instances of atrial discordance, therefore, the inferior vena cava is nearly always absent, and the link between liver and right atrium is lacking). Hollman [5] pointed out that in ventricular discordance the great arteries are always transposed. Frequent accompanying anomalies are pulmonary stenosis or atresia and VSD. In the patient under discussion there was in addition a double-outlet right ventricle with the arterial orifices side by side at about the same level. In normal embryological development the aortic canal, as part of the truncus arteriosus, is still situated entirely above the right ventricle in the fifth week. Early in the sixth week (10 to 15 mm crown-rump length) the left and right bulbar ridges grow in such a manner and direction that the aortic orifice communicates with the left ventricle obliquely behind the pulmonary orifice through the primary interventricular foramen (which at this point becomes the vestibule of the aorta). In a normal embryo of 18 to 19 mm crown-rump length, only a vestige of the interventricular communication is recognizable. In a defective embryo, at the start of the seventh week of gestation retarded development in the bulbus-truncus area appears to cause the aortic orifice to retain a position on the extreme right next to the pulmonary orifice and therefore above the right ventricle: double-outlet right ventricle with VSD. In this embryo the two orifices are still wide, but it is obvious that in this anomaly the pulmonary orifice, enclosed between the aortic orifice and ventricular septum, has a rather vulnerable position and runs a relatively high risk of becoming stenotic in the course of fetal development.
References 1 . Asfaou, E., Geldof, W., d e Villeneuve, V. H., Rohmer, J., and Brom, A. G. Traitement anatomique des transpositions des gros vaisseaux avec stenose pulmonaire. Ann Chir Thorac Cardiovasc 11:247, 1972. 2. Bowman, F. O., Hancock, W. D., and Malm, J. R. A valve-containing Dacron prosthesis. Arch Surg 107:724, 1973. 3. Geldof, W. C. P., Aytug, Z., and Brom, A. G. Die Entliiftung der Aorta nach extrakorporalem Kreislauf. Thoraxchirurgie 20:2 18, 1972. 4. Gomes M. M. R., and McGoon, D. C. Truncus arteriosus with interruption of the aortic arch: Report of a case successfully repaired. Mayo Clin Proc 46:40, 1971. 5. Hollman, A. Dextrocardia, Levocardia and Mesocardia. In H. Watson (Ed),Paedzatric Cardiology. London: Lloyd-Luke, 1968. P 668. 6. Kiser, J. C., Ongley, P. A., Kirklin, J. W., Clarkson, P. M., and McGoon, D. C. Surgical treatment of dextrocardia with inversion of ventricles and double-outlet right ventricle. J Thorac Cardiovasc Surg 55:6, 1968. 7. Kouchoukos, N. T., Barcia, A., Bargeron, L. M., and Kirklin, J. W. Surgical treatment of congenital pulmonary atresia with ventricular septa1 defect. J Thorac Cardiovasc Surg 61:70, 1971. 8. McGoon, D. C., Rastelli, G. C., and Ongley, P. A. An operation for the correction of truncus arteriosus.JAMA 205:59, 1968.
466
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Unusual Double-Outlet Right Ventricle
9. McGoon, D. C., Wallace, R. B., and Danielson, G. K. T h e Rastelli operation: Its indications and results. J Thorac Cardiovasc Surg 65:65, 1973. 10. Rastelli, G. C. A new approach to anatomic repair of transposition of the great arteries. Mayo Clin Proc 44: 1, 1969. 1 1 . Rastelli, G. C., McGoon, D. C., and Wallace, R. B. Anatomic correction of transposition of the great arteries with ventricular septa1 defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 58:545, 1969. 12. Rastelli, G. C., Ongley, P. A., Davis, G. D., and Kirklin, J. W. Surgical repair for pulmonary valve atresia with coronary-pulmonary artery fistula: Report of case. Mayo Clin Proc 40:521, 1965. 13. Ross, D. N., and Somerville, J. Correction of pulmonary atresia with a homograft aortic valve. Lancet 2:1446, 1966. 14. Somerville, J., and Ross, D. N. Long-term results of complete correction with homograft reconstruction in pulmonary outflow tract atresia. Br Heart J 34:29, 1972. 15. Van Mierop, L. H. S., and Gessner, I. H. Pathogenetic mechanisms in congenital cardiovascular malformations. Prog Cardiouasc Dzi 15:67, 1972. 16. Van Praagh, R., Van Praagh, S., Vlad, P., and Keith, J. D. Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications. A m J Cardiol 13:510, 1964. 17. Wallace, R. B., Rastelli, G. C., Ongley, P. A., Titus, J. L., and McGoon, D. C. Complete repair of truncus arteriosus. J Thorac Cardiouasc Surg 57:95, 1969. 18. Weldon, C. S., Rowe, R. D., and Gott, V. L. Clinical experience with the use of aortic valve homografts for reconstruction of the pulmonary artery, pulmonary valve and outflow portion of the right ventricle. Circulation 37, 38 (Suppl 11): 51, 1968.
VOL. 20, NO. 4, OCTOBER, 1975
467
![[Unusual radiologic findings in hypertrophic cardiomyopathy with right ventricle obstruction].](/assets/img/hold.png)
