Forms Of Double-outlet Right Ventricle Research Articles

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.

Double-Outlet Right Ventricle with Extremely Rare Conotruncal Morphology

We describe an extremely rare form of double-outlet right ventricle in a 1-year-old boy who had l-malposition of the great arteries with the definitive conotruncal features of false Taussig-Bing heart. Extraordinary conotruncal morphology raised several surgical options, however, the definitive anatomical repair was achieved by an arterial switch operation with a baffle from a ventricular septal defect to the pulmonary artery.

Aortic Translocation for the Management of Double-Outlet Right Ventricle and Pulmonary Stenosis With Dextrocardia: Technique to Avoid Coronary Insufficiency

The management of D-loop transposition of the great arteries with left ventricular outflow tract obstruction and related forms of double-outlet right ventricle is challenging. Association with right ventricular and tricuspid valve hypoplasia and dextrocardia represents a major obstacle to achieve a biventricular repair. The most commonly used technique to deal with this condition, which is the Rastelli operation, further reduces the potentially compromised right ventricular volume due to the left ventricle-to-aorta baffle. Because the Rastelli operation risks later development of left ventricular outflow tract obstruction, aortic translocation can be considered for these patients. We report a case of double-outlet right ventricle with left ventricular outflow tract obstruction in the setting of dextrocardia, left juxtaposition of the atrial appendages, and a small tricuspid valve, which was successfully managed with a biventricular repair by means of an aortic translocation technique.

The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle

Background: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. Methods: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. Results: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. Conclusion: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.J Thorac Cardiovasc Surg 2002;124:984-90

Long-term survival and functional follow-up in patients after the arterial switch operation

Background. For many years, the arterial switch operation (ASO) has been the therapy of choice for patients with transposition of the great arteries (TGA). Although excellent short- and mid-term results were reported, long-term results are rare.Methods. Between May 1983 and September 1997, ASO was performed on 285 patients with simple TGA (n = 171), TGA with ventricular septal defect (VSD) (n = 85), and Taussig-Bing (TB) anomaly (n = 29). This retrospective study describes long-term morbidity and mortality over a 15-year period.Results. Hospital mortality was 3.5% for simple TGA, 9.4% for TGA with VSD, and 13.8% for TB anomaly. Late death occured in 2 patients, 1 with simple TGA and 1 with TGA and VSD. The cumulative survival for all patients at 5 and 10 years is 93%, and at 15 years is 86%. Reoperations were required in 31 patients and were most common for stenosis of the right ventricular outflow tract (RVOT). However, no correlation was found between technical variations on pulmonary artery reconstruction and this type of complication. Forty-six patients underwent follow-up angiography, which revealed five cases with coronary occlusion or stenosis. Follow-up is complete in 96% of the patients from 1 to 15.2 years. Sinus rhythm is present in 97%; 88% of the patients show no limitations on exertion.Conclusions. The ASO can be performed with low early mortality, almost absent late mortality, and infrequent need for reoperation. The favorable long-term results demonstrate that the ASO can be considered as the optimal approach for patients with TGA and special forms of double-outlet right ventricle.

Coronary artery anatomy and transposition of the great arteries

Until recently, the anatomic variability of the coronary arteries in hearts with transposition of the great arteries (TGA) was only of academic interest. Shaher and Puddu [1] described the multiple variations in the origin and epicardial course of the coronary arteries as early as 1966. This marked diversity has been confirmed in other pathologic [2–5] and surgical series [6–19]. Additional classification schemes have been employed with the hope of simplifying the description of the many anatomic patterns, most notably by Gittenberger-de Groot et al. [20], Yacoub and Radley-Smith [21], and Pasquini et al. [22] and Mayer et al. [16]. With the current widespread application of the arterial switch operation for repair of TGA (and transposition-like forms of double-outlet right ventricle), detection of these anatomic variations has become clinically important. The improvement in surgical technique for the transfer of the coronary arteries to the neoaorta has been paralleled by the enhanced diagnostic capabilities of two-dimensional echocardiography [22,23] and modified aortography [24,25]. Recent reports detailing the outcome of the arterial switch operation indicate that coronary artery distribution probably plays an important role in the immediate and ultimate success of this surgical approach [7,9,16,17,26]. This article reviews 1) the embryology and numerous anatomic classification schemes proposed in the literature, 2) the various diagnostic methods used for the detection of coronary artery patterns, 3) the techniques of surgical transfer during the arterial switch operation, and 4) the midterm results of transfer of the coronary arteries in the neonate.

Malformations of the semilunar valves produced in chick embryos by mechanical interference with cardiogenesis. An experimental approach to the role of hemodynamics in valvular development.

The purpose of the present work was to analyze the role of hemodynamics in the morphogenesis and histogenesis of the semilunar valves. To achieve this goal we have studied the development of the chick semilunar valves in conditions of abnormal local flow. To obtain an abnormal pattern of local flow we have induced alterations of the cardiac septation process by mechanical interference of the development of the conus cordis. The malformations obtained by this procedure consisted of a spectrum of alterations in the process of incorporation of the aortic conus into the left ventricle. These malformations ranged from a simple widening of the outflow tract of the left ventricle to severe forms of double-outlet right ventricle and ventricular septal defects. Malformations of the semilunar valves consisting of extensive thickening of the leaflets and lack of maturation of the valve tissues were very often present in the malformed hearts. The malformation of the valve leaflets was more frequent and severe in the aortic valve at more advanced stages of development and in the hearts showing more severe alteration of the septation process. The absence of alterations in the semilunar valves of the control embryos and in the experimental embryos without alteration of the cardiac septation suggest a close relationship between the semilunar valves anomalies and the hemodynamic alterations present in the malformed hearts.

Transaortic repair of double-outlet right ventricle with situs inversus, 1-loop, 1-malposition (I,L,L), subaortic ventricular septal defect, and associated anomalies

Successful total correction of a rare form of double-outlet right ventricle (I.L.L.) is reported wherein transaortic construction of the ventricular baffle was used. Surgical approach to the defect was unsatisfactory through a right ventriculotomy. Excellent exposure was achieved through the aortic valve. Correction of partial anomalous venous return from the left lung, pulmonary debanding, and repair of the pulmonary artery were also done. Double superior venae cavae made special venous cannulation necessary. Cardiac catheterization at one year documented successful repair. This case illustrates the usefulness of the transaortic approach to a ventricular septal defect when repair through a ventriculotomy is difficult or impossible.

Double-outlet right ventricle with tunnel from left ventricle to aorta

The successful surgical correction of an unusual form of double-outlet right ventricle (DORV) in a 4-year-old boy is described. A long tunnel-like structure was present between the left ventricle and the aorta and also communicated with the right ventricle. hemodynamic and angiographic evaluation demonstrated restrictive communications between the tunnel, the aorta, and each of the ventricles. A pathological concept explaining the anatomic abnormality seen in this patient is discussed.

The conotruncus. II. Report of a case showing persistent aortic conus and lack of inversion of the truncus (a bulboventricular heart).

A primitive form of double-outlet right ventricle is described clinically and anatomically. A long tubular conus, arising exclusively from the right ventricle emptied into the transposed great vessels. Together with the presence of A-V canal malformation this heart represented a developmental arrest in the embryonic stage of the bulboventricular loop seen in horizon XV. Diagnosis is made on the basis of right ventricular angiography and theoretically, if this lesion is isolated, this conus malformation is surgically correctable.