Abnormal Ventriculoarterial Connections Research Articles

Double-Outlet Left Ventricle: Case Series and Systematic Review of the Literature.

Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children's Hospital. A systematic search was developed in MEDLINE, Web of Science, and EMBASE databases to identify reports assessing the morphology and outcomes of DOLV between 1975 and 2023. RESULTS: Over a median follow-up of 9.9 years (IQR 7.8-11.7 y), four cases of DOLV were identified at our institution. Two patients were diagnosed with (S,D,D) DOLV subaortic VSD and pulmonary stenosis (PS): one patient had (S,D,D) DOLV with doubly committed VSD and hypoplastic right ventricle, and another patient had (S,D,L) DOLV with subaortic VSD and PS (malposition type). Pulmonary stenosis was the most commonly associated lesion (75%). LITERATURE REVIEW: After systematic evaluation, a total of 12 reports fulfilled the eligibility criteria and were included in our analysis. PS or right ventricular outflow tract obstruction was the most commonly associated lesion (69%, 95% CI 62-76%). The most common locations of VSD were subaortic (pooled prevalence: 75%, 95% CI 68-81), subpulmonary (15%, 95% CI 10-21), and doubly committed (7%, 95% CI 4-12). The position of the great arteries showed that d-transposition of the aorta was present in 128 cases (59% 95% CI 42-74), and l-transposition was present in 77 cases (35%, 95% CI 29-43).

Double Outlet Right Ventricle with Pulmonary Atresia and Antero-Posterior Malposition of Great Arteries: Transthoracic Echocardiographic Evaluation of a rare cyanotic congenital heart disorder

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle, This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We are reporting a case of seven year old deeply cyanotic child with DORV, subaortic VSD with rare anatomic features of antero-posterior malposition of great arteries (GA) and pulmonary vavular atresia, with conspicuous absence of patent ductusarteriosus (PDA) or major aorto-pulmonary collaterals (MAPCAs), despite the presence of atretic pulmonary valve. Keywords: DORV, Pulmonary atresia, TGA, VSD, subaortic VSD, overriding of Aorta, cyanotic congenital heart defects.

The 3-vessel view as a means of screening for conotruncal malformations

Prenatal detection of fetal anomalies allows the adequate counseling of the parents regarding the pregnancy and the neonatal prognosis. This is especially true in the case of congenital heart defects where counseling the parents is essential, given the high neonatal mortality of such anomalies. Conotruncal malformations are made up of a wide variety of congenital heart defects with abnormal ventriculoarterial connections and represent up to 30% of all cases of congenital heart defects. The most common types of conotruncal malformations are tetralogy of Fallot, transposition of great arteries, double outlet right ventricle, common arterial trunk, but numerous anatomical variants exist. The 3-vessel view can be used as an alternative to the ventricular outflow tract views to detect conotruncal malformations and may be used as a screening test. To test this hypothesis, we evaluated the 3-vessel view in both normal cases and cases of conotruncal malformations. As expected, in the 30 normal cases we reviewed, all the criteria for a normal 3-vessel view were met. In the 15 cases of conotruncal malformations (tetralogy of Fallot, transposition of great arteries, double outlet right ventricle, common arterial trunk) that we investigated, at least one criterion for a normal 3-vessel view was not met. This goes to show the utility the 3-vessel view can have in the screening for conotruncal malformations

Anatomy and Outcomes of Patients with Right Atrial Isomerism: A West Australian Perspective

Background: Right atrial isomerism (RAI) is a heterotaxy syndrome with poor long-term outcomes, early mortality with surgical intervention [ 1 Ota N. Fujimoto Y. Murata M. Tosaka Y. Ide Y. Tachi M. et al. Improving outcomes of the surgical management of right atrial isomerism. Ann Thorac Surg. 2018; 93: 832-839 Abstract Full Text Full Text PDF Scopus (35) Google Scholar , 2 Sadiq M. Stümper O. De Giovanni J.V. Wright J.G. Sethia B. Brawn W.J. Silove E.D. Management and outcome of infants and children with right atrial isomerism. Heart. 1996; 75: 314 Crossref PubMed Scopus (58) Google Scholar ], and low survival rates [ 3 Hashmi A. Abu-Sulaiman R. McCrindle B.W. Smallhorn J.F. Williams W.G. Freedom R.M. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol. 1998; 31: 1120-1126 Crossref PubMed Scopus (190) Google Scholar , 4 Eronen M.P. Aittomäki K.A.U. Kajantie E.O. Sairanen H.I. Pesonen E.J. The outcome of patients with right atrial isomerism is poor. Pediatr Cardiol. 2013; 34: 302-307 Crossref PubMed Scopus (31) Google Scholar ]. Patients with RAI have complex cardiac malformations, including total anomalous pulmonary venous return (TAPVR) [ 3 Hashmi A. Abu-Sulaiman R. McCrindle B.W. Smallhorn J.F. Williams W.G. Freedom R.M. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol. 1998; 31: 1120-1126 Crossref PubMed Scopus (190) Google Scholar , 4 Eronen M.P. Aittomäki K.A.U. Kajantie E.O. Sairanen H.I. Pesonen E.J. The outcome of patients with right atrial isomerism is poor. Pediatr Cardiol. 2013; 34: 302-307 Crossref PubMed Scopus (31) Google Scholar ], atrioventricular septal defect, ventricular hypoplasia, pulmonary stenosis/atresia, and abnormal ventriculo-arterial connections [ 2 Sadiq M. Stümper O. De Giovanni J.V. Wright J.G. Sethia B. Brawn W.J. Silove E.D. Management and outcome of infants and children with right atrial isomerism. Heart. 1996; 75: 314 Crossref PubMed Scopus (58) Google Scholar , 3 Hashmi A. Abu-Sulaiman R. McCrindle B.W. Smallhorn J.F. Williams W.G. Freedom R.M. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol. 1998; 31: 1120-1126 Crossref PubMed Scopus (190) Google Scholar ]. A number of extra–cardiac anomalies are also well defined, the most significant being asplenia [ [4] Eronen M.P. Aittomäki K.A.U. Kajantie E.O. Sairanen H.I. Pesonen E.J. The outcome of patients with right atrial isomerism is poor. Pediatr Cardiol. 2013; 34: 302-307 Crossref PubMed Scopus (31) Google Scholar ]. We aimed to review the anatomy and outcomes of patients born in Western Australia (WA) between 2003 and 2017 with RAI.

The trabecula septomarginalis (Leonardo's cord) in abnormal ventriculo-arterial connections: anatomic and morphogenetic implications.

BackgroundThe abnormal ventriculo-arterial connections in atrio-ventricular concordance and situs solitus with two well developed ventricles include the range from tetralogy of Fallot throughout the different forms of double outlet right ventricle to transposition of great arteries.The infundibular septum and the trabecula septomarginalis are the fundamental anatomical landmarks for the segmental analysis.In these abnormalities there is a pathological progressive counter-clockwise rotation of the infundibular septum which divorces from the antero-superior limb of the trabecula septomarginalis and achieves his identity. Is there any anatomical evidence of a simultaneous abnormal counter-clockwise rotation of the trabecula septomarginalis?MethodsMalposition of great arteries is a generic term since all relationships have to be expected.We present specimens with anatomical evidence of a progressive counter-clockwise rotation from 0° to about 180°of the plane passing throughout the trabecula septomarginalis’s limbs.ResultsWe can observe sequentially:1. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the ventriculo infundibular fold: (tetralogy of Fallot, double outlet right ventricle with sub-aortic ventricular septal defect, truncus arteriosus and doubly committed ventricular septal defect);2. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the infundibular septum (double outlet right ventricle with sub-pulmonary ventricular septal defect, transposition of great arteries).Conclusions1. The sequential-segmental analysis identify all the morphologies.2. The trabecula septomarginalis plane presents a progressive counter-clockwise twist on the long axis.3. Since the trabeculated portions of the ventricles are the oldest developmental components, our observations support the hypothesis that the abnormal ventriculo-arterial connections could be in relation with a pathological myocardial process during early cardio-genesis.We are promoting new studies to investigate our anatomical observations.

Landmarks for diagnosis and surgery in abnormal ventriculo-arterial connections with usual arrangement

Background The term “malposition of great arteries“ is commonly used for diagnosis and surgery in abnormal ventriculoarterial connections with usual arrangement and two well developed ventricles. In these abnormalities the variability of the arterial relationships is considerable and all relationships from normal to side by side to antero posterior have to be expected. Can we find other anatomical landmarks for the diagnosis and the subsequent corrective surgery? Methods We reviewed the specimens previously published and observations collected in three different institutions. Results There is a gradual transition from Tetralogy of Fallot to double outlet right ventricle with subaortic ventricular septal defect to Taussig-Bing hearts to transposition of great arteries. In this transition the normal aortic-mitral continuity is progressively lost with gradual development of pulmonary- mitral continuity. The key features determining the sequence of these abnormalities are the relationships between the outlet septum and the ventricular musculature. We may describe and group these malformations in function of the anticlockwise rotation of the outlet septum and the septomarginal trabeculation. Conclusion The term “malposition of great arteries“ is a generic term which is of little help for diagnosis and surgery, therefore, it should be avoided. We recommend a detailed description of the outlet septum and the septomarginal trabeculation in relation to the VSD. Careful attention to these structures dictates the diagnosis and the best surgical approach.

How Best Can We Define Double Outlet Right Ventricle When Describing Congenitally Malformed Hearts?

How Best Can We Define Double Outlet Right Ventricle When Describing Congenitally Malformed Hearts?

Deciphering the Mysteries of Crisscross Heart by Transthoracic Echocardiography

Accurate diagnosis of crisscross heart and its associated anomalies is important but problematic for cardiologists. This study aimed at identifying unique transthoracic echocardiographic features and common associated lesions of this complex condition. Clinical and echocardiographic features of 10 patients with crisscross anatomy were studied. Echocardiographic findings were verified by cardiac magnetic resonance imaging or surgical inspection. Crisscross anatomy (10 patients, age at diagnosis ranged from 1 month to 25 years, five female) was identified in 0.076% of patients with congenital heart diseases from 1985 to 2006. All patients had cyanosis and 80% of them were underweight. Superior-inferior ventricles (SIV) and crossed ventricular inflow streams were seen in 90% and 100% of patients, respectively. All patients had abnormal ventriculo-arterial (VA) connections: five with transposition of great artery (L-type: n = 2; D-type: n = 3) and five with double outlet right ventricle. Commonly associated anomalies included ventricular septal defects (100%), right ventricular outflow tract obstruction (60%), atrioventricular valves straddling or overriding (50%), atrial septal defect (40%), and right ventricular hypoplasia (30%). Seven patients received cardiac surgery for the relief of cyanosis. SIV and crossed inflow streams are important diagnostic features for crisscross heart by transthoracic echocardiogram. The hemodynamic consequences of abnormal VA connections and associated defects impact surgical management.

Anatomy of Common Atrioventricular Junction With Complex Associated Lesions

The essence of the lesion increasingly described as atrioventricular septal defect is the presence of a common atrioventricular junction. In most instances, the common junction is itself shared in more or less equal fashion between the cardiac chambers, producing the so-called balanced arrangement, which can be considered the default option. Complexity can be produced at various levels within this standard lesion. The most complex malformations are seen in the setting of visceral heterotaxy. Greatest complexity is seen with right isomerism. This always includes totally anomalous pulmonary venous connection, even when the pulmonary veins return to the heart. Still further complexity is often added by the presence of pulmonary stenosis or atresia. Imbalance can involve either the atrial or ventricular chambers. Imbalance at atrial level produces one form of double outlet atrium, but typically with balanced ventricles. Ventricular imbalance represents spectrums extending either to double inlet left or right ventricle through a common atrioventricular valve. Complexity at the level of the ventriculoarterial junctions is seen in the form of abnormal ventriculoarterial connections, notably tetralogy of Fallot or double outlet right ventricle. In these settings, the superior bridging leaflet is free-floating. Hypoplasia of the left atrioventricular valve is part of right ventricular dominance and is often associated with the so-called parachute malformation. Dual orifice is also a problem. In both these lesions, the zone of apposition between the bridging leaflets is the effective inlet to the left ventricle.

Use of the Lecompte procedure for ventriculoarterial connection abnormalities in infants and children with congenital heart diseases

The Lecompte (REV) procedure is used to correct abnormal ventriculoarterial connections in patients with congenital heart diseases; it avoids the need for an extracardiac conduit for pulmonary outflow tract reconstruction. The present study aimed to investigate effectiveness and criteria of the REV procedure in children with abnormal ventriculoarterial connections. Thirty-eight children (mean age, (2.2 +/- 1.7) years; mean weight, (11.5 +/- 3.8) kg) with abnormal ventriculoarterial connections who had an REV procedure in our hospital from January 1998 to May 2006 were studied. Only 10 patients had the usual anteroposterior relationship of the two great arteries. The infundibular septum between the two semilunar valves was aggressively resected to enlarge it and construct a straighter left ventricular outflow tract and a wide tunnel between the ventricular septal defect (VSD) and the aorta. Eighteen cases had the original REV procedure; 20 had a modified REV procedure. All patients are alive; none developed severe complications. The postoperative right ventricular (RV) to left ventricular (LV) pressure ratio was 0.20-0.45. Five patients had RV dysfunction; 2 patients had a pressure gradient in the RV ventricular outlet of 30.0-34.5 mmHg; 3 cases had a 37.5-47.3 mmHg pressure difference in the RPA. All patients had an RV pressure less than half the systemic pressure. These gradients' magnitudes in all patients were consistent with the post-operative RV to LV pressure ratio (P < 0.05). During the follow-up (mean, (4.2 +/- 0.6) years), 2 patients had an RPA pressure gradient of 24.0-29.3 mmHg which abated to less than 10 mmHg after two years. The REV procedure provides satisfactory short- to medium-term results. It may be superior to the Rastelli procedure for treating ventriculoarterial connection abnormalities; it allows early, complete anatomic repair and reduces the need for late re-operation, since no extracardiac conduit is needed. Longer follow-up is needed to determine long-term outcomes.

Clinical Use of Electrocardiography in Adults With Congenital Heart Disease

The prevalence of adult congenital heart disease (ACHD) has risen markedly over the past 2 decades, with the number of adults now rivaling the number of children with severe defects.1 This is, perhaps, not surprising given that current care allows nearly 90% of infants born with heart defects to thrive into their adult years.1,2 This remarkable triumph is tempered, however, by the realization that early interventions were reparative and not curative. Numerous complications may surface years after uneventful childhood courses, justifying vigilant clinical follow-up throughout adulthood. The 12-lead ECG remains an invaluable cornerstone in the clinical appraisal of adults with congenital heart disease that, in certain circumstances, provides diagnostic and/or prognostic information. The present review imparts a clinical perspective to ECG interpretation in ACHD, emphasizing practical and pathogenomonic findings in the more frequently encountered congenital defects in adults. Anatomic features of the conduction system relevant to ECG findings in ACHD are summarized, including variations in the location of the sinus node, atrioventricular (AV) node, and His-Purkinje system. Thereafter, pertinent ECG features are highlighted for common subtypes of ACHD (Table). Examples are provided throughout for illustration. View this table: Table. Typical ECG Features in Common Forms of ACHD ### Sinus Node In the morphologically normal heart, a crescent-shaped sinus node is characteristically located epicardially along the lateral aspect of the superior cavoatrial junction. It generates a P-wave axis typically between 15° and 75°. Most patients with ACHD have normally positioned atrial chambers, called atrial situs solitus, with normal sinus node location. The position of the sinus node may, however, vary with the atrial chambers and their appendages. #### Juxtaposition of the Atrial Appendages In juxtaposition of the atrial appendages, both appendages are on the same side of the arterial pedicle rather than each being ipsilateral to its respective atrium. Left juxtaposition, with left-sided atrial appendages, frequently accompanies tricuspid atresia and has …

Cardiorespiratory Responses to Exercise After Anatomic Repair of Atrioventricular Discordance with Abnormal Ventriculoarterial Connection

We evaluated exercise tolerance and cardiorespiratory responses to exercise in patients with atrioventricular discordance (AVD) and abnormal ventriculoarterial connection after anatomic repair. Cardiopulmonary treadmill exercise testing with gas measurement was done 62 times in 19 patients with AVD who had undergone anatomic repair at the National Cardiovascular Center. Exercise duration, oxygen uptake (V(O2)) and heart rate at anaerobic threshold and peak, and oxygen pulse during exercise were significantly lower in patients with AVD after anatomic repair than in controls. Carbon dioxide ventilatory equivalent during exercise was worse in patients with AVD after anatomic repair than in controls. Percentage peak V(O2) significantly correlated positively with percentage peak oxygen pulse and tended to correlate positively with the heart rate increments. Patients with AVD after anatomic repair exhibit impaired responses of heart rate and oxygen pulse with lower exercise capacity. Careful attention should be paid to patients with AVD after anatomic repair in terms of their functional capacity as well as other postoperative complications.

Traitement chirurgical de la transposition complète des gros vaisseaux

Among congenital heart defects, transposition of the great arteries is a frequent lesion. It includes an abnormal ventriculo arterial connection, the aorta arising from the right ventricle and the pulmonary artery from the left ventricle. To this basic anotomic and physiologic condition, several intra and/ or extra cardiac lesions may be associated. Without any intervention, it is quickly lethal. Early- and long-term prognosis has been thoroughly modified by the introduction of the arterial switch operation. This latter consists in reestablishment of normal anatomic ventriculo arterial connections, the aorta and coronary ostia arising from the left ventricle and the pulmonary artery from the right ventricle. In general and depending on the anatomic and clinical status, associated lesions are repaired during the same operative procedure. The single risk factor for operative mortality is the anatomy of coronary patterns to be transfered in aortic position. Early- and long-term results are excellent with 5 % of early mortality and a 15 years survival rate close to 90 %. At follow up, the vast majority of patients have a normal life without any medication. Three levels of postoperative complications can occur : right ventricular outflow tract obstruction, aortic valve insufficiency and myocardial ischemia due to coronary ostial stenosis . All these complications mandate a regular cardiologic follow-up.

Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft

Background. In patients with abnormal ventriculo-arterial connections, a mitral valve cleft different from an atrioventricular canal is occasionally associated. It may cause outflow obstruction, mitral regurgitation, and complicate biventricular repair. Methods. A retrospective review identified 21 patients operated upon with mitral valve cleft, abnormal ventriculo-arterial connections, and two well-developed ventricles. Eight patients had a ventricular outflow obstruction due to the mitral valve, whereas 2 had more than mild mitral regurgitation. One patient required initial mitral valve surgery. Eleven patients underwent biventricular repair, associated with mitral valve repair in 2 cases: arterial switch operation (n = 4), Senning operation (n = 3) associated with an arterial switch operation in one case, intraventricular repair (n = 3), and Rastelli-type extracardiac conduit repair (n = 1). Single-ventricle palliation was preferred in 10 patients with major mitral valve straddling (n = 5), outflow tract obstruction (n = 2), and noncommitted or multiple VSDs (n = 3). Results. There were three hospital deaths, two of which occurred after biventricular repair and one after an early reoperation after a bidirectional cavopulmonary anastomosis. Postoperatively after biventricular repair, 1 patient required permanent pacemaker implantation and 3 patients were reoperated on for subaortic stenosis (n = 1) and mitral regurgitation (n = 2), with one late death. By multivariate analysis, patients with a double-outlet right ventricle were at greater risk of death ( p = 0.04). After a mean follow-up period of 60.7 months (± 68.6 months), 16 patients are in New York Heart Association (NYHA) class I. One patient with a moderate mitral regurgitation on Doppler study is in NYHA class II. Conclusions. The surgical management remains controversial in patients with abnormal ventriculo-arterial connections and mitral valve cleft. Biventricular repair may not always be feasible, especially in cases of complex intracardiac anatomy associated with mitral valve straddling. Single-ventricle palliation can be achieved in these patients, although it is unknown whether the long-term results are as good as those obtained with biventricular repair.

Double-outlet right ventricle: Pathology and angiocardiography.

Double-outlet right ventricle is but one form of abnormal ventriculoarterial connection. The definition that more than half of each great artery originates above the morphologically right ventricle is arbitrary. As pointed out by Lecompte, those features that should be defined in hearts with the ventriculoarterial connection of double-outlet right ventricle (and indeed other forms of abnormal ventriculoarterial connection) include the nature of the infundibular septum, ventriculoinfundibular fold, trabeculoseptomargin-alis, attachments of infundibular septum to anterior or posterior limb of trabeculoseptomargin-alis, the size and position of the ventricular septal defect, the spatial relation of great artery(s) to the ventricular septal defect, the spatial relationship between the great, and the distance between the tricuspid and pulmonary valves and the semilunar valves. Copyright 2000 by W.B. Saunders Company

Prenatal diagnosis of congenital heart malformations: classification based on abnormalities detected by the four-chamber view

The aim of the study was to provide logical steps, alternative to the sequential chamber analysis, for diagnosing major congenital heart malformation prenatally. Between 1988 and 1995, of the 14,885 high-risk pregnancies screened at the Departments of Obstetrics and Paediatric Cardiology, University of Hong Kong, 92 fetuses with major congenital heart malformations were detected at 17-36 weeks' gestation (mean=24.6 weeks) by extended fetal echocardiographic screening. These were classified according to obvious abnormalities related to the partition of the heart or ventricular dimensions depicted by the four-chamber view. Subgrouping according to the presence of normal or abnormal ventriculo-arterial connection, with or without outflow tract anomalies, was further attempted. Five groups were identified: (1) septal defects with all four chambers identified (n=62, 67 per cent); (2) intact septation with dilatation of either the right or all four chambers of the heart (n=12, 13 per cent); (3) intact septation with hypoplasia of either one of the ventricles (n=11, 12 per cent); (4) a rudimentary interventricular septum giving an apparent three-chamber heart (n=6, 7 per cent); (5) miscellaneous causes (n=1, 1 per cent). Combined with the information related to the ventricular outflow tracts, the accuracy of arriving at a definitive diagnosis was 97 per cent, when compared with postnatal investigations, surgery and/or autopsies. This simple classification could provide logical steps to arrive at a definitive diagnosis for most major fetal heart malformations.

Management and Outcomes of Right Atrial Isomerism: A 26-Year Experience

Objectives. We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality.Background. Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality.Method. All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed.Results. A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001).Conclusions. Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.

Anatomic spectrum of abnormal ventriculoarterial connections: Surgical implications

To clarify the salient anatomic features of surgical significance, we investigated 33 specimens representing the spectrum of abnormal ventriculoarterial connections. In those with tetralogy of Fallot or double-outlet right ventricle with subaortic ventricular septal defect, the muscular outlet septum separating the subarterial outflow tracts was always inserted into (or in front of) the anterior limb of the septomarginal trabeculation (septal band). In those having double-outlet right ventricle with doubly committed ventricular septal defect, the outlet septum was lacking. When the ventricular septal defect was in subpulmonary position, with either double-outlet or discordant ventriculoarterial connections, the outlet septum was attached to the posterior limb of the septomarginal trabeculation. The outlet septum was deviated into the subpulmonary outlet in hearts with discordant ventriculoarterial connections and pulmonary stenosis. It is the interrelations between the septomarginal trabeculation, the outlet septum, and the ventriculoinfundibular fold that hold the key to the understanding of surgical anatomy and determine the optimal choice of procedure for definitive biventricular repair.

The pathological diagnosis of pulmonary atresia with abnormal ventriculoarterial connections in the fetus

AbstractPulmonary atresia with abnormal ventriculoarterial connections in the presence of an intact ventricular septum is rare even in postnatal life. Two cases are described in which this combination of lesions was diagnosed in previable fetuses at autopsy. Both pregnancies were terminated for gross structural abnormalities discovered on antenatal ultrasound scans. In the first case, the pulmonary atresia was associated with intact ventricular septum, a double outlet left ventricle and hypoplasia of the tricuspid valve, right ventricle and arterial duct, a combination of lesions that would appear to be unique. In the second case, there was complete transposition and a hypoplastic left ventricle. The severity of the cardiac lesions in both fetuses was such that it is unlikely that either would have survived to term. In the first case no other malformation was present, but in the second there was also a large encephalocoele secondary to early amnion rupture, and a single umbilical artery. The association of the cardiac lesion with early amnion rupture in this second fetus would appear to be a chance event.

Malaligned outlet septum with subpuhnonary 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.