The infundibular interrelationships and the ventriculoarterial connection in double outlet right ventricle. Clinical and surgical implications.

Fifty specimens of double outlet right ventricle were studied. The insertion of the outlet (infundibular) septum determines two types of infundibular interrelationships. In the first type, with anterior and posterior infundibulums, the outlet septum is inserted to the anterior limb of the septomarginal trabeculation; the posterior infundibulum is related with the atrioventricular orifices and the interventricular septum forms exclusively one of the walls of the posterior infundibulum. Therefore, the artery connected with the posterior infundibulum may be related with a subarterial ventricular septal defect. Of our material, 35 cases (70% of 50) had anterior and posterior infundibulums and, in 32, the aorta was connected with the posterior infundibulum (91.4% of 35). The ventricular septal defect was subaortic in 26 cases (81.2% of 32). In the second type, with side-by-side infundibulums, the outlet septum is inserted in to the ventriculo-infundibular fold in the proximity of the posterior limb of the septomarginal trabeculation. Both infundibulums are related with the atrioventricular orifices and the interventricular septum forms exclusively one of the walls of the medial infundibulum. Therefore, the artery connected with the medial infundibulum has the possibility of being related with a subarterial ventricular septal defect. Of our material, 13 cases (26% of 50) had side-by-side infundibulums. In all of these (100% of 13), the pulmonary trunk was connected with the medial infundibulum and the ventricular septal defect was subpulmonary in 12 cases (92.3% of 13). There were two cases (4% of 50) with a doubly committed ventricular septal defect. The insertion of the outlet septum permits one to determine the infundibular interrelationships, information which cannot be attained by taking into account the relationship of the great arteries with each other. Once the infundibular interrelationship is established, one must determine if the aorta is connected with the posterior or with the medial infundibulum, since, depending on the anatomical constitution of these infundibulums, there is the possibility of a ventricular septal defect being related with this artery. This information is indispensable before attempting the surgical correction of the double outlet right ventricle and it may be obtained by echocardiography or by angiocardiography.     

Transthoracic three-dimensional echocardiography for the assessment of straddling tricuspid or mitral valves

BACKGROUND: The advent of 3D echocardiography has provided a technique which, potentially, could afford significant additional information over conventional cross-sectional echocardiography in the assessment of patients with straddling atrioventricular valves prior to surgical correction. METHODS: Eight patients, aged from 1 month to 9.2 years, were examined with 3D echocardiography. All but three had discordant ventriculoarterial connections or double outlet right ventricle. Data suitable for reconstruction was acquired with transthoracic scanning. Right and left ventricular volumes were calculated in the 3D dataset. RESULTS: 3D echocardiography proved capable of defining the exact degree of straddling by imaging the proportion of tension apparatus attached to either side of the ventricular septum. It was able also to display the atrioventricular junction "en face", thus permitting identification of the precise site of insertion of the muscular ventricular septum relative to the atrioventricular junction. This made it possible first, to calculate the degree of valvar override, and second, to predict the location of the penetrating atrioventricular bundle. End-diastolic volume of the right ventricle in those with straddling tricuspid valves was 73 (61-83)% of normal, and, of the left ventricle in those with mitral valvar straddling 71 (40-97)% of normal. CONCLUSIONS: 3D echocardiography can aid in planning the optimal surgical procedure in patients with straddling or overrriding atrioventricular valves, as it provides diagnostic information superior to standard cross-sectional techniques. It also allows for exact measurement of the volumes of the respective ventricles.     

Morphological evaluation of atrioventricular septal defects by magnetic resonance imaging.

Twelve patients aged between 2 weeks and 22 months (median 6 weeks) with atrioventricular septal defects were examined with a 1.5 T, whole body, magnetic resonance imaging system. Ten patients had a common atrioventricular orifice (complete defect) while two patients had separate right and left valve orifices (partial defect). Associated cardiac malformations included the tetralogy of Fallot in two, isomerism of the right atrial appendages and pulmonary atresia in two, and right isomerism and double outlet right ventricle in one. All had previously been examined by cross sectional echocardiography. Eight subsequently had angiography and six underwent surgical correction. There was one operative death and three other deaths. Three of these patients underwent postmortem examinations. Small children and infants were scanned inside a 32 cm diameter head coil. Multiple electrocardiographically gated sections 5 mm thick, separated by 0.5 mm, were acquired using a spin echo sequence with echo time of 30 ms. A combination of standard and oblique imaging planes was used. Magnetic resonance imaging was more accurate than echocardiography in predicting the size of the ventricular component of the defect. It was also better than either echocardiography and angiography in identifying the presence of ventricular hypoplasia. All these findings were confirmed by surgical or postmortem examinations or both. Magnetic resonance imaging is capable of providing detailed morphological information in children with atrioventricular septal defects which is likely to be of value in their management.     

Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.     

Ventricular septal defect after myocardial infarction: assessment by cross sectional echocardiography with pulsed wave Doppler scanning.

Eight patients who developed a ventricular septal defect after myocardial infarction were assessed by cross sectional echocardiography and pulsed wave Doppler scanning. Cross sectional echocardiography visualised the defect in four patients and gave an accurate assessment of global and regional left ventricular function in all eight. In all patients pulsed wave Doppler scanning detected turbulent flow at the apex of the right ventricle or adjacent to a wall motion abnormality affecting the interventricular septum. Pulsed wave Doppler detected coexisting mitral regurgitation in one patient and tricuspid regurgitation in another two. In all patients a left to right shunt was confirmed by oximetry and the location of the defect was identified by angiography or at operation or necropsy. Cross sectional echocardiography in combination with pulsed wave Doppler scanning is useful in the rapid bedside evaluation of patients with ventricular septal defect after myocardial infarction.     

Double outlet right ventricle, non-committed ventricular septal defect and pulmonic stenosis. Anatomical and surgical considerations

A case of double outlet right ventricle with pulmonary stenosis, a non-committed muscular ventricular septal defect occupying the sinus and trabecular portions of the ventricular septum, an additional small subtricuspid ventricular septal defect, situs solitus, atrioventricular concordance and aortic d-position is described. Atrioventricular valvular canal type malformations, splenic, systemic or pulmonary venous anomalies, all frequently seen in double outlet right ventricle with uncommitted VSD, were not present.     

Positional abnormalities of atrioventricular valves in transposition of the great arteries including double outlet right ventricle, atrioventricular valve straddling and malattachment.

Positional abnormalities (straddling or overriding) of the atrioventricular (A-V) valves were studied using angiographic, sector scan echocardiographic and postmortem anatomic data in 10 patients with transposition of the great arteries including double outlet right ventricle. Group I included six patients with tricuspid valve abnormalities. This group was further classified into: (a) patients with anular straddling and abnormal attachment of portions of the tricuspid leaflets in the left ventricle (malattachment) or on the ventricular septum, or both (four patients with d-transposition of the great arteries and hypoplastic right ventricle); and (2) patients with malattachment of portions of the tricuspid valve leaflet to the crest or left ventricular surface of the septum (septal malattachment) without significant anular straddling (two patients with d-transposition of the great arteries).Group II included four patients with mitral valve abnormalities without significant anular straddling, further classified into: (1) patients with septal malattachment of the portions of the mitral valve to the crest and right ventricular surface of the ventricular septum (two patients with double outlet right ventricle); and (2) patients with ventricular malattachment of portions of the mitral valve leaflets to the papillary muscles in the right ventricle (two patients with ventricular septal defect and discordant criss-cross atrioventricular connections).On the basis of these observations and reported experience, two main types of positional abnormalities of the A-V valves were recognized: (1) anular straddling in which the A-V anulus straddled the ventricular septum above the contralateral ventricle, together with ventricular leaflet malattachments, and (2) leaflet malattachment of either the septal or ventricular type without significant straddling of the valve anulus. Angiocardiography and sector scan echocardiography helped to identify these abnormalities of the A-V valves.     

Atrioventricular discordance. Clinico-surgical experience 1990-2000

OBJECTIVE: To know the incidence of ventriculoarterial connections combined with atrioventricular discordance, associated lesions and surgical results, including the first case with anatomical correction. METHOD: All patients with atrioventricular discordance by echocardiography from 1990 to March 2000 were analyzed. RESULTS: Thirty six patients with atrioventricular discordance were found. Ages ranged from 0.1 to 46 years, with a mean 9.2 years (SD 5.9 years). Atrial chambers were situs solitus in 88.9%, inversus in 11.2%. The ventriculoarterial connections were discordant in 28 (77.7%), double outlet right ventricle in 4 (11.1%) (one of them was a "criss cross" heart), single outlet (pulmonary atresia) in 4 (11.1%), and double outlet left ventricle in 1 (2.7%). Associated lesions: Ventricular septal defect with pulmonary stenosis or atresia was present in 21 (58%), ventricular septal defect with no pulmonary obstruction was observed in 10 (28%). Five had tricuspid regurgitation with right ventricular disfunction, (two adults). Surgical results: 22 (61%) required 28 surgical procedures: 8 (36%) were palliative and 19 (86%) were corrective, one of them was our first anatomical correction. Operative mortality in all was 40.1%, postoperative atrioventricular block was observed in 9 (40.1%). CONCLUSION: Right ventricular dysfunction is not uncommon. Surgical results revealed high mortality and high pacemaker requirement for atrioventricular block.     

Atrioventricular discordance without ventriculo-arterial discordance. Apropos of 3 cases

Three cases of atrioventricular without ventriculo-arterial discordance are reported. The first case was a young woman with situs solitus, atrioventricular discordance and ventriculo-arterial concordance, large atrial and small ventricular septal defects. The functional tolerance was relatively good. The second case was an infant girl with dextrocardia by dextroversion and abdominal situs solitus. The child had atrioventricular discordance with ventriculo-arterial concordance and an ostium primum atrial septal defect. Cyanosis was mild and, in addition, complete heart block was diagnosed on the fourth day of life. The functional tolerance was good in this case too and growth was normal. The third case was a young girl with levocardia, atrial situs inversus, and abdominal situs inversus. A double outlet right ventricle was diagnosed at catheterisation with atrioventricular discordance, ventricular septal defect, and severe valvular and infundibular pulmonary stenosis. The first palliative procedure was performed at eight months: a left Blalock-Taussig anastomosis. As cyanosis has recurred a second procedure is being considered, the child now being 5 years old. Different forms of atrioventricular discordance without ventriculo-arterial discordance have been described: atrioventricular discordance with ventriculo-arterial concordance; atrioventricular discordance with double outlet right ventricle; atrioventricular discordance with double outlet left ventricle; atrioventricular discordance with a single vessel issuing from the right or left ventricle. With reference to their personal cases and those described in the literature, the authors describe the anatomy, physiopathology and the clinical consequences of the different forms encountered in their cases. The different surgical techniques for each anatomical form of this condition are discussed.     

Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.

OBJECTIVE--To report recent experience of patients with complete atrioventricular septal defect and tetralogy of Fallot, with emphasis on anatomical features, diagnosis, and management. DESIGN--Case notes were reviewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography. SETTING--Tertiary cardiothoracic referral centre. PATIENTS--Between 1987 and 1992 13 patients with atrioventricular septal defect and tetralogy of Fallot (12 with concordant and one with double outlet ventriculoarterial connections) underwent surgery; 10 underwent complete intracardiac repair. 11 patients had Down's syndrome. The complete diagnosis was established preoperatively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal short axis views was the diagnostic feature of atrioventricular septal defect, with tetralogy of Fallot diagnosed from the presence of anterocephalad deviation of the outlet septum producing subvalvar pulmonary stenosis as seen in subcostal right anterior oblique views. INTERVENTIONS--Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruction of the atrioventricular valves, and relief of the obstruction within the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Taussig shunts were performed in three patients, who await intracardiac repair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years. MAIN OUTCOME MEASURES--Diagnostic methods, surgical results, and functional state after complete correction. RESULTS--The presence of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck deformity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardiography. Of the 10 patients who underwent complete repair, nine are alive and one died 34 days after operation with adult respiratory distress syndrome. Examination at necropsy showed an excellent surgical correction. Mean (range) follow up was 23 (8 to 48) months. All nine patients are alive and well (New York Heart Association Class 1). CONCLUSION--Accurate diagnosis and staged management with improved surgical techniques have lowered mortality of this complex combination of cardiac defects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger than 2 years and total correction in older children.     

Ventricular septal defect after myocardial infarction: assessment by cross sectional echocardiography with pulsed wave Doppler scanning

Eight patients who developed a ventricular septal defect after myocardial infarction were assessed by cross sectional echocardiography and pulsed wave Doppler scanning. Cross sectional echocardiography visualised the defect in four patients and gave an accurate assessment of global and regional left ventricular function in all eight. In all patients pulsed wave Doppler scanning detected turbulent flow at the apex of the right ventricle or adjacent to a wall motion abnormality affecting the interventricular septum. Pulsed wave Doppler detected coexisting mitral regurgitation in one patient and tricuspid regurgitation in another two. In all patients a left to right shunt was confirmed by oximetry and the location of the defect was identified by angiography or at operation or necropsy. Cross sectional echocardiography in combination with pulsed wave Doppler scanning is useful in the rapid bedside evaluation of patients with ventricular septal defect after myocardial infarction.     

Morphological evaluation of atrioventricular septal defects by magnetic resonance imaging

Twelve patients aged between 2 weeks and 22 months (median 6 weeks) with atrioventricular septal defects were examined with a 1.5 T, whole body, magnetic resonance imaging system. Ten patients had a common atrioventricular orifice (complete defect) while two patients had separate right and left valve orifices (partial defect). Associated cardiac malformations included the tetralogy of Fallot in two, isomerism of the right atrial appendages and pulmonary atresia in two, and right isomerism and double outlet right ventricle in one. All had previously been examined by cross sectional echocardiography. Eight subsequently had angiography and six underwent surgical correction. There was one operative death and three other deaths. Three of these patients underwent postmortem examinations. Small children and infants were scanned inside a 32 cm diameter head coil. Multiple electrocardiographically gated sections 5 mm thick, separated by 0.5 mm, were acquired using a spin echo sequence with echo time of 30 ms. A combination of standard and oblique imaging planes was used. Magnetic resonance imaging was more accurate than echocardiography in predicting the size of the ventricular component of the defect. It was also better than either echocardiography and angiography in identifying the presence of ventricular hypoplasia. All these findings were confirmed by surgical or postmortem examinations or both. Magnetic resonance imaging is capable of providing detailed morphological information in children with atrioventricular septal defects which is likely to be of value in their management.     

Malaligned outlet septum with subpulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically

A retrospective analysis of 62 echocardiograms from 22 patients with malaligned outlet septum, subpulmonary ventricular septal defect and abnormal ventriculoarterial (VA) connection was performed and the results were confirmed by intraoperative inspection, cardiac catheterization and angiography, or pathologic specimen analysis in 21 of the 22 patients. The purpose of this study was to determine the relative incidence of the primary morphologic features; the correlation between the presence or absence of mitral valve to pulmonary valve fibrous continuity and the type of VA connection; the types and incidence of additional cardiovascular anomalies; and the correlation between the determination of the VA connection by echocardiography versus that ascertained at intraoperative and autopsy inspection. A spectrum of VA connections was found, ranging from double outlet right ventricle (23%) at one extreme, through an intermediate type (64%) in which the pulmonary artery was overriding the ventricular septum, thereby having no consistent and predominant connection to either ventricle, to a discordant VA connection (13%) at the opposite end of the spectrum. All three types of VA connection occurred whether or not there was mitral valve to pulmonary valve fibrous continuity. Additional cardiovascular anomalies were present in 95% of patients, the most common being aortic arch obstructive lesions in 59%, anomalous attachments of the atrioventricular valve tension apparatus in 55% and subaortic stenosis in 50%. A method of echocardiographic determination of the VA connection that correlated with that determined intraoperatively and from pathologic specimen analysis is described.     

Criss-cross heart with straddling right atrioventricular valve

Angiocardiographic appearance of a criss-cross heart with straddling tricuspid valve has been presented. Atrial situs was normal and the right atrium was connected to the morphological right ventricle situated superiorly and to the left of the left ventricle. The left atrium was connected to the morphological left ventricle situated inferiorly and to the right of the right ventricle. The interventricular septum was horizontal in position. Both great arteries arose from the right ventricle with the anterior aorta. The case was concluded as a criss-cross heart with concordant atrioventricular (A-V) connection and double outlet right ventricle (DORV). Straddling of the right A-V valve was recognized in another institution by two-dimensional (2-D) echocardiography and confirmed by right atrial angiography in our institution. The value of combined investigation with selective four-chamber angiography, especially including right atrial angiography and 2-D echocardiography in the diagnosis of such complex cardiac anomalies has been stressed.     

Double outlet from chambers of left ventricular morphology.

This series of 5 cases with double outlet of morphologically left ventricular chamber includes 4 found during a review of 1700 heart specimens (incidence 0.23%) and 1 found at operation and successfully corrected. Abnormal atrioventricular connection precluding total correction was present in the 4 anatomical cases. Clinical diagnosis may be difficult and it is suggested that axial cineangiography may make anatomical diagnosis easier. Absence of the infundibular septum and aortic laevoposition are frequent. As some cases can be surgically corrected, accurate information is required on the size of the right ventricle, the morphology and function of the atrioventricular valves, the presence, size, and position of the ventricular septal defect, and the degree and type of outflow tract obstruction.     

Double outlet from chambers of left ventricular morphology.

This series of 5 cases with double outlet of morphologically left ventricular chamber includes 4 found during a review of 1700 heart specimens (incidence 0.23%) and 1 found at operation and successfully corrected. Abnormal atrioventricular connection precluding total correction was present in the 4 anatomical cases. Clinical diagnosis may be difficult and it is suggested that axial cineangiography may make anatomical diagnosis easier. Absence of the infundibular septum and aortic laevoposition are frequent. As some cases can be surgically corrected, accurate information is required on the size of the right ventricle, the morphology and function of the atrioventricular valves, the presence, size, and position of the ventricular septal defect, and the degree and type of outflow tract obstruction.