This article presents an anatomical variation of the very high take-off right coronary artery from the ascending aorta. Clinical care is complicated by the patient’s double outlet right ventricle and right aortic arch associated with this situation.

Surgical repair of channels between the ventricles is enhanced when the surgeon knows precisely where to place a patch, or baffle, so as to restore septal integrity. The paediatric cardiologist should provide the necessary information. Communication will be enhanced if the same words are used to account for the structures in question. Currently, however, the same term, namely “ventricular septal defect,” is used to account for markedly different areas within the heart. Closure of perimembranous defects found in hearts with concordant or discordant ventriculo-arterial connections restores the integrity of the ventricular septum, at the same time separating the systemic and pulmonary blood streams. When both arterial trunks arise from the right ventricle, in contrast, the surgeon when placing a baffle so as to separate the blood streams, does not close the channel most frequently described as the “ventricular septal defect.” In this review, we show that the perimembranous lesions as found in hearts with concordant or discordant ventriculo-arterial connections are the right ventricular entrances to the areas subtended beneath the hinges of the leaflets of the aortic or pulmonary valves. When both arterial trunks arise from the right ventricle, and the channel between the ventricles is directly subaortic, then the channel termed the “ventricular septal defect” is the left ventricular entrance to the comparable space subtended beneath the aortic root. We argue that recognition of these fundamental anatomical differences enhances the appreciation of the underlying morphology of the various lesions that reflect transfer, during cardiac development, of the aortic root from the morphologically right to the morphologically left ventricle.

We describe two cases of an unusual variant of double outlet right ventricle with intact ventricular septum diagnosed prenatally and confirmed by foetal autopsy in a case. The first case had mitral valve atresia, slit-like left ventricle, and normally related great arteries. The second case had mitral valve atresia, hypoplastic left ventricle, parallel outflows with an interrupted aortic arch.

Pregnancy in a woman with double outlet right ventricle, ventricular septal defect, and pulmonary stenosis is presented. Her previous two pregnancies ended in fetal losses. In the current pregnancy, she had thrombocytopenia, placenta previa, and severe fetal growth restriction. She underwent an emergency caesarean section followed by post-partum haemorrhage and COVID-19 infection. Literature review has been performed.

Background: Disagreement currently exists regarding the definition of aortic dextroposition. It is suggested that the term be used interchangeably with aortic overriding, along with suggestions that the aortic valve overrides in the normal heart. The dextroposed aorta, however, does not always override the crest of the muscular ventricular septum. It is incorrect to argue that the normal aortic valve overrides. It is the cavity of the right aortic valvar sinus, rather than the valvar orifice, that sits above the muscular septum when the septum itself is intact. Therefore, to circumvent these difficulties, those using the term “dextroposition” find it necessary to distinguish “true” as opposed to “false” categories. The problems arise because “dextroposition” is remarkably ill-suited as an alternative term for aortic valvar overriding. Methods and Results: In this review, combining developmental, morphologic, and clinical data, we show how aortic overriding is best considered on the basis of biventricular connection of the aortic root in the setting of deficient ventricular septation. When analysed in this manner, it becomes an easy matter to distinguish between one-to-one and double outlet ventriculo-arterial connections. Appreciation of these features emphasises the different spatial alignments of interventricular communications as opposed to the plane of deficient ventricular septation. The concept of overriding is applicable not only to biventricular connection of the aortic root, but also the pulmonary and common arterial roots. Conclusions: The diagnostic techniques now available to the paediatric cardiologist illustrate the features of arterial valvar overriding with exquisite accuracy, informing the discussions now required for optimal decision making.

Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.

We examined the echocardiograms of 40 patients with double oudet right ventricle, proven either at autopsy or during surgery. By way of the subcostal approach, it proved possible to determine the insertion of the outlet (infundibular) septum and to categorize the malformations within the classification proposed by de la Cruz and her colleagues. It was also possible to ascertain the relationships of the atrioventricular valves with one or with both infundibulums, and these features could be correlated with infundibular interrelationships. The interrelationship of the great arteries, in contrast, was not always in accordance with the infundibular interrelationships, indicating that the arterial interrelationship may not prove to be a good parameter for surgical decision-making. Of greatest importance was the visualization and echocardiographic appraisal of the spatial position and size of the ventriculoinfundibular fold, since this anatomic feature was the key to determining the optimal surgical approach. This is so because the ventriculoinfundibular fold constitutes one of the borders of the ventricular septal defect, and separates it from the plane of the leaflets of the arterial valves. Furthermore, the fold frequendy contributes to infundibular obstruction. Our echocardiographic studies, based on a better anatomic knowledge, permitted us to choose the most suitable surgical technique in our patients.

Form 1980 until January 1991, 76 patients with double outlet ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5± 5.0 years) underwent surgey at the A.N. Bakoulev Inste for Cardivascular Surgery. Associated heart defects were found in 77.6% patints. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infudibulum in four (the Tassisg-Bing anomaly) while, in 12 (15.8%), it opened under the outflow tracts of both great arteries. In two cases, (2.6%) there was a mno-committed ventricular septact. In one case (1.3%)a non-committed defect was diagnosed as coexisting with a non-commited defect was dignosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery.Another theree patients with the Taussig-Bing anomaly and obstructive pulmomnary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patiens with subaortic ventricular septal defects. The important factors, which increased the risk of correction and contributed to death were inappropriate pre-operative diagnosis of complex associated defecrs and techmical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significatly (one death among 34 patients undergoing surgery from 1986 through January 1991)

A ventricular septal defect is, almost always, an integral part of double outlet right ventricle and has been classified into the subaortic, subpulmonary, doubly committed and non-committed varieties. This study was performed to correlate the cross-sectional imaging characteristics of such ventricular septal defect in double outlet right ventricles using pathological specimens. The extent and the orientation of the outlet septum were the most important in the differentiation of the four varieties of ventricular septal defect. In the subaortic variety, the outlet septum fused with the left anterior margin of the defect, this being marked by the anterior limb of the septomarginal trabeculation. In the subpulmonary variety, the outlet septum fused with the right posterior margin of the defect, this being the posterior limb of the septomarginal trabeculation. The outlet septum was vestigial in case with doubly committed defects. In those with non-committed defects, the defect was not shown in those images or sections which demonstrated the outlet septum.

The Fontan operation was initially designed to provide a “physiological correction” of tricuspid atresia, but its indications have been extended to more complex forms of congenitally malformed hearts. In selected patients with two adequately sized ventricles, but highly complex intra cardiac anatomy which makes a biventricular repair more hazardous and which would require multiple reoperations, a univentricular repair using Fontan's principle has been proposed. Between 1978 and 1992, a modified Fontan operation was carried out in 34 such patients. Of the patients. 13 had double outlet right ventricle and 21 had transposed great arteries. Anomalies of ventricular loop were present in 14 patients. A non-committed ventricular septal defect was found in 26 whilst multiple defects were present in 10. All patients had anomalies of the atrioventricular valves, with straddling of the tricuspid valve,11 and/or of the mitral valve11, and abnormal tricuspid5, or mitral4 cordal attachments. Two patients had mild tricuspid hypoplasia, 1 had mitral hypoplasia and 3 had a cleft mitral valve. Fourteen patients had anomalies of both atrioventricular valve but only 6 had mild atrioventricular valvar incompetence. Pulmonary stenosis or atresia was seem in 26. All the patients had at least one or more (mean 2.35) intracardiac anomalies that preculded a biventricular repair. The Fontan operation was performed at a median age of 4.75 years. There were 4 early (11.4%, 70% CL: 5.1–19.8) and 2 late deaths, with a 4 year actuarial survival rate of 79.6±8.1%. Univariate analysis did not reveal any risk factor for early of late death. Follow-up at a mean interval of 3.6 ±2.6 years was available in all but 1 survivor. There were 6 early and 2 late reoperations, and 3 patients required implantation of a pacemaker. In conclusion, the modified Fontan operation can provide satisfactory early and mid term results in patients with two ventricles but with complex intracardiac anatomy which precludes a biventricular repair.

We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.

We report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

The authors achieved biventricular repair in a 1-year-old boy having double outlet right ventricle and unusual straddling of the mitral valve. There was a substantial abnormal muscular bar present within the right ventricle, and some of the tendinous cords of the mitral valve were attached to this structure. Preoperative echocardiography was particularly informative in enabling the surgeon to arrange the optional operative procedures. The postoperative course was smooth and uneventful.