Type Of Double-outlet Right Ventricle Research Articles

Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies?

BackgroundThe term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning.ResultsA total of 50 patients diagnosed by echocardiography with DORV referred from our institute between September 2018 and August 2020 were enrolled. The location of the ventricular septal defect (VSD) to the great vessels and the alignment of the great vessels to each other as well as other associated malformations that affect the surgical planning were assessed by MDCT. The patients were retrospectively classified into subgroups according to the relative positions of the great arteries, the relationship between the great arteries and the VSD, and the presence of associated malformations according to the classification of Society of Thoracic Surgeons database. The diagnostic value of MDCT and transthoracic echocardiography (TTE) in evaluation of associated cardiac malformations apart from double outlet right ventricle was compared in 20 patients, whom surgeries were performed and surgical results were obtained. Fallot type of DORV was the most common clinical type which represented 48% of the patients in which the VSD is committed to the aorta. The second most common clinical type was Taussig–Bing anomaly which represented 30% of the patients and the VSD was seen committed to the pulmonary artery. The atrio-ventricular septal defect (AVSD)/uni-ventricular type represented 16% of the patients in the current study, with pulmonary and systemic venous anomalies, heterotaxy syndrome and situs anomalies were commonly associated. A total of 53 malformations apart from DORV were found in 20 patients whom performed surgical interventions. MDCT detected different vascular and situs anomalies in those patients with 100% sensitivity and 100% positive predictive value compared to 71.4% and 94% respectively in TTE. On the other hand, in our study TTE was superior to MDCT for detection of intracardiac anomalies with 100% sensitivity and 100% accuracy compared to 87.5% and 95% respectively in MDCT.ConclusionsOur study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV. It is superior to echocardiography in providing a comprehensive mapping of the anatomy of complex anomalies especially that of extra-cardiac structures such as aorta, systemic veins, pulmonary veins, and pulmonary arteries. However, it can miss tiny intracardiac malformations. So combining the results of MDCT and echocardiography would be beneficial to guarantee the accuracy of diagnosis of DORV.

Role of Computed Tomography in Pre- and Postoperative Evaluation of a Double-Outlet Right Ventricle.

Double-outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great arteries arise entirely or predominantly from the right ventricle. The morphology of DORV is characterized by a ventricular septal defect (location and relationship with the semilunar valve); bilateral coni and aortomitral continuity; the presence or absence of outflow tract obstruction; tricuspid-pulmonary annular distance; and associated cardiac anomalies. The surgical approach varies with the type of DORV and is based on multiple variables. Computed tomography (CT) is a robust diagnostic tool for the preoperative and postoperative assessment of DORV. Unlike echocardiography and magnetic resonance imaging (MRI), CT imaging is not limited by small acoustic window, need for anaesthesia and can be used in patients with metallic implants. Current generations CT scanners with high spatial and temporal resolution, wide detectors, high-pitch scanning mode, dose-reduction algorithms, and advanced three-dimensional post-processing tools provide a low-risk, high-quality alternative to diagnostic cardiac catheterization or MRI, and have been increasingly utilized in nearly every type of congenital heart defect, including DORV.

Application of three-dimensional printing technology in preoperative planning of double outlet right ventricle

Objective To assess the clinic value of three-dimensional(3D) printing models in operation scheme of double outlet right ventricle(DORV). Methods From September 2015 to December 2016, the imaging data of 29 patients (13 males and 16 females) with DORV were acquired using Dual Source CT.And then the cardiac models were generated using 3D printing technology.The cardiac models were used in diagnosing the type of DORV and guiding the surgery scheme.The 3D printed models were compared with two-dimensional imaging in diagnosis and surgical scheme of DORV patients. Results Both the two-dimensional imaging and 3D printed models were effective in the diagnosis and typing of DORV.According to 3D printing models, 28 cases were consistent with the real operations, and 1 case was inconsistent.According to the two-dimensional imaging data, 20 cases of surgical strategies were consistent with the real operations and 9 cases were inconsistent.For patients with DORV with non-committed ventricular septal defect (NC-VSD), 3D printing models were more accurate in the designing of surgical strategies. Conclusions 3D printing models can display 3D anatomical structures and it is helpful in the diagnosis and making preoperative planning for DORV especially for DORV with NC-VSD, which provides a new method for the assessment of DORV. Key words: Congenital heart disease; Double outlet right ventricle; Three-dimensional printing technology; Cardiac model

Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle.

Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO). Surgical approaches in 407 patients were based on their DORV subtype, as determined by echocardiography. We found that the optimal surgical management of patients classified as normal/committed/no RVOTO, normal/committed/RVOTO, and abnormal/committed/no RVOTO was, respectively, like that for patients with large ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries without RVOTO. Patients with abnormal/committed/RVOTO anatomy and those with abnormal/noncommitted/RVOTO anatomy underwent intraventricular repair and double-root translocation. For patients with other types of DORV, choosing the appropriate surgical approach and biventricular repair techniques was more complex. We think that our classification system accurately groups DORV patients and enables surgeons to select the best approach for each patient's cardiac anatomy.

Biventricular Repair of Double Outlet Right Ventricle: Preoperative Echocardiography and Surgical Outcomes.

To discuss the key anatomic features of double outlet right ventricle (DORV) assessed by preoperative echocardiography among patients treated with different types of biventricular repair. Surgical and echocardiographic databases were queried to identify patients who had undergone biventricular repair for DORV and had adequate preoperative echocardiographic imaging. All patients underwent pre- and postoperative echocardiography and clinical evaluation following discharge. Two hundred sixty-two patients with DORV met the inclusion criteria of the study. The patients were divided into two groups-intraventricular tunnel repair (IVR) to the aorta (194 [74%] patients) or to the pulmonary artery with either concomitant arterial switch operation or double-root translocation (68 [26%] patients). Among 68 patients undergoing IVR to the pulmonary artery, 50 patients with transposition of the great arteries (TGA) type of DORV and 7 patients with remote ventricular septal defect (VSD) type underwent IVR plus arterial switch operation and 6 patients with TGA type and 5 patients with remote VSD type underwent IVR plus double-root translocation. There were three hospital deaths and one late death (overall operative mortality: 1.5%). Preoperative echocardiography provided crucial data to estimate the feasibility of intraventricular tunnel creation to either the aorta or the pulmonary artery and to guide the selection of either arterial switch or double-root translocation. Biventricular repair could be achieved with favorable outcomes in most patients with DORV.

Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle

ObjectivesSurgical management of various forms of double-outlet right ventricle uses a variety of approaches depending on the underlying anatomic form. In this study, we sought to determine the risk factors of mortality and reoperation in those with double-outlet right ventricle undergoing biventricular repair, according to anatomic characteristics and initial surgical strategy. MethodsBetween 1992 and 2013, 433 patients were included in the study. Double-outlet right ventricle was classified as double-outlet right ventricle with subaortic ventricular septal defect associated with subpulmonary obstruction in 33% of patients (n = 141), with subaortic ventricular septal defect without subpulmonary obstruction in 30% of patients (n = 130), with subpulmonary ventricular septal defect in 32% of patients (n = 139), and with noncommitted ventricular septal defect in 5% of patients (n = 23). Three types of repairs were performed: (1) intraventricular baffle repair, n = 149 (34%); (2) intraventricular baffle repair with right ventricular outflow tract reconstruction, n = 163 (38%); and (3) intraventricular baffle repair with arterial switch operation, n = 121 (28%). ResultsThirty-day overall mortality was 7.4%. Early reoperation was needed in 6% of the cases. Early mortality was higher in the intraventricular baffle repair with arterial switch operation group (P = .01). Survival at 10 years was 86.2%, and freedom from reoperation at 10 years was 61.4%. At last follow-up (median, 5.7 years; 95% confidence interval, 4.5-6.6), mortality and reoperation rates were similar in the different surgical strategy groups. Late reoperation and late mortality were significantly higher in the double-outlet right ventricle with noncommitted ventricular septal defect group (P < .01). In multivariate analyses, risk factors for reoperation were concomitant surgical procedures (P = .03) and duration of cardiopulmonary bypass (P < .01). Risk factors for mortality were restrictive ventricular septal defect (P = .01), mitral cleft (P < .01), and associated coronary artery anomalies (P = .01). ConclusionsThose with the anatomic type of double-outlet right ventricle with noncommitted ventricular septal defect were at higher risk for reoperation and mortality. Intraventricular baffle repair with arterial switch operation was the surgical strategy in patients at higher risk of early death. Initial surgical strategy did not influence the late outcomes.

Tetralogy of Fallot with subarterial ventricular septal defect: Diagnostic and surgical considerations

Since 1964, 77 patients underwent repair for tetralogy of Fallot with subarterial ventricular septal defect. Median age at operation was 5 years. Cyanosis was commonly mild, and hypoxic episodes were infrequent. Accordingly, only 12 patients (15.58%) needed either palliative or corrective operations before the age of 2 years. Characteristic angiographic and echocardiographic features were observed, which allowed this entity to be differentiated from either classic tetralogy or other types of double-outlet right ventricle. The earliest series of operations (in which no right ventricular outflow patch was used) was followed by a prohibitive mortality (50%). Residual subpulmonary obstruction was the cause of all of these deaths. In a second series of patients, a transannular patch was frequently used (79.59%), with the rationale that closure of the ventricular septal defect would make restrictive the deficient infundibulum of these patients. In a later series, we observed that most of the patients had a nonrestrictive pulmonary anulus (80%); the patch therefore was limited to the ventriculotomy. In the absence of pulmonary annular hypoplasia, the need for patching of the right ventricular outflow tract in this entity has to be confined to preventing the subpulmonary obstruction induced by closure of the ventricular septal defect. A considerable improvement in the results followed our first series (4.3% mortality). Follow-up of the survivors (mean 7 years) has been satisfactory.

Repair of ventricular septal defect in the first year of life.

In the 7-year period 1976 through 1982, 48 infants underwent repair of ventricular septal defect (VSD). Their mean age was 5.3 (range 1-11) months and mean body weight 5.4 (range 3.1-9.6) kg. "Uncomplicated" VSD, located in the membranous septum, was present in 33 cases. "Complicated" VSD in terms of muscular/multiple (n = 6), subaortic type of double-outlet right ventricle (n = 5), AV-canal type (n = 2) and straddling chordae (n = 2) was present in 15 cases. The indication of surgery was progressive left ventricular dysfunction due to pulmonary hyperperfusion and pulmonary hypertension. Right trans-atrial exposure permitted repair, patch or tunnel in 43 of the 48 infants (90%), although temporary detachment of the septal tricuspid leaflet was required on six occasions. Co-existing patent ductus arteriosus (n = 6) and atrial septal defect (n = 22) were simultaneously closed. There were three postoperative deaths (6%). One infant required a second-stage repair for incomplete VSD closure and one sustained a permanent AV-block II. Repair via right atrial exposure was associated with few complications and only one death (2%).

Complete repair of the Taussig-Bing type of double-outlet right ventricle using the arterial switch operation without coronary translocation: Report of one successful case

We report one successful case of surgical repair of a Taussig-Bing type of double-outlet right ventricle (DORV) without pulmonary stenosis and with situs inversus. We used the arterial switch operation without coronary translocation, originally proposed by Damus, Kaye, Stansel, and Alvarez for treatment of dextro-loop transposition of the great arteries.

Conotruncal malformations: diagnosis in infancy using subxiphoid 2-dimensional echocardiography

Subxiphoid 2-dimensional echocardiography was performed and interpreted before the first cardiac catheterization in 109 of 113 infants less than 1 year of age with conotruncal malformations, situs solitus of the atria, and d-ventricular loop. The 4 remaining infants could not be examined using this technique for technical reasons. In 104 of 109 patients successfully examined a correct diagnosis was obtained with the use of Subxiphoid 2-dimensional echocardiography. All patients with d-transposition of the great arteries had a correct diagnosis, and in 10 of 11 patients various types of double-outlet right ventricle were recognized by direct visualization of ventriculoarterial connection. Tetralogy of Fallot was correctly diagnosed in 34 of 36 patients and tetralogy of Fallot and pulmonary atresia were correctly diagnosed in 8 of 9 patients, on the basis of the position of the infundibular septum and presence or absence of continuity between the main pulmonary artery and the right ventricle. Five of 6 patients with truncus arteriosus communis were correctly diagnosed by demonstrating a connection between the pulmonary arteries and the ascending portion of the single large semilunar root. Subxiphoid 2-dimensional echocardiography proved to be a sensitive and specific technique for diagnosing conotruncal malformations in infancy.

Double outlet right ventricle: experimental morphogenesis in the chick embryo heart.

Double outlet right ventricle (DORV) was produced experimentally in the chick embryo by preventing the incorporation of the posteromedial conus into the left ventricle. This was accomplished by placing a ligature around the caudal end of the conus at a stage in which it is exclusively continuous with the right ventricle. The embryos were divided into two groups according to the stage of development of the conal ridge. In one group, the conal ridges had not appeared (stages 17-18); in the other they had begun to develop (stage 22). Both groups were divided into two subgroups; in one the ligature was left in place up to the mature heart stage and in the other it remained for only 24 hours. In all the embryos where the ligature remained until the mature heart stage a DORV was obtained, while in the embryos where the ligature remained in place for only 24 hours a DORV was obtained in only two-thirds of the cases. Two different types of DORV were produced: 1) with anterior and posterior infundibula in which the subpulmonary infundibulum was anterior to the subaortic infundibulum and there was a subaortic infracristal ventricular septal defect (VSD); 2) with side-by-side infundibula in which the subpulmonary infundibulum was on the left side and the subaortic infundibulum on the right side; the VSD was far from both infundibula. Our experiments show that DORV is an essentially infundibular malformation. In DORV there are two types of infundibular relationships: a) with anterior and posterior infundibula, and b) with side-by-side infundibula. The crista supraventricularis in the normal heart and the infundibular septum in DORV are two different anatomic and embryologic structures.

Early Experience with Arterial Repair of Transposition

Major anomalies associated with isolated complete transposition of the great arteries (TGA) can produce systemic pressure in the left ventricle without fixed stenosis of the left ventricular outflow tract. In this situation, arterial repair may be advantageous. Eight children, 10 days to 15 years old, underwent arterial repair of TGA. Major associated anomalies included large patent ductus arteriosus, bulging intraventricular septum, ventricular septal defect (VSD), tricuspid atresia, and the Taussig-Bing type of double-outlet right ventricle. There were 2 operative deaths related to acute left ventricular failure. The survivors underwent postoperative echocardiographic, hemodynamic, and angiographic assessment; all were in sinus rhythm and well six months to 2 1/2 years after operation. Residual lesions have been frequent but mild: aortic insufficiency (5), stenosis of the right ventricular outflow tract (1), trivial VSD (1), and stenosis of the right coronary artery (1). Left ventricular function is “normal” in 4 and unchanged from moderately depressed preoperative status in 1.

Successful repair of double-outlet right ventricle with bilateral conus, 1-transposition of great arteries (S,D,L), and subpulmonary ventricular septal defect

Surgical correction was carried out successfully in a severely cyanotic 3-year-old Japanese girl who had a very rare type of double-outlet right ventricle. The malformation was associated with bilateral conus, 1-transposition of the great arteries, and subpulmonary ventricular septal defect without significant pulmonary stenosis in situs solitus. A large amount of subaortic conal musculature which separated the aortic valve from the subpulmonary ventricular septal defect was removed, as was the anterior rim of the ventricular septal defect. A tunnel, constructed with a woven Teflon prosthesis, was inserted in such a manner as to direct blood from the left ventricle through the defect and out to the aorta. The pulmonary outflow tract was reconstructed with a Teflon patch lined with pericardium. The patient's postoperative recovery was uneventful, and she was doing well 3 months postoperatively. To our knowledge, no identical case with a similar type of surgical correction has previously been reported.