AUCTORES
Case Report
*Corresponding Author: Ramachandran Muthiah, President of all Nations Morning Star Hospital, Enayam Thoppu, Kanykumari District, India
Citation: Ramachandran Muthiah, (2024), Surgical aspects of “Acyanotic Taussig-Bing heart”, Journal of Thoracic Disease and Cardiothoracic Surgery; Journal of Thoracic Disease and Cardiothoracic Surgery, 5(6): DOI: 10.31579/2693-2156/111
Copyright: © 2024 Ramachandran Muthiah, this is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited
Received: 05 August 2024 | Accepted: 16 September 2024 | Published: 04 October 2024
Keywords: acyanotic; embryologically; ventricular
A 14-year-old acyanotic boy presented with severe left-sided AV valve regurgitation and bradycardia. ECG revealed left sided morphologic right ventricular hypertrophy (RVH) as evidenced by a loss of septal Q waves in left precordial leads suggesting ventricular inversion. X-ray chest revealed a straight upper right cardiac border due to loss of normal relationship of great vessels and cardiomegaly due to both left atrial and morphologic right ventricular enlargements suggesting a left-sided regurgitant lesion
Aim: To report a case of acyanotic Taussig-Bing heart, anatomically consistent with L-transposition and rheumatic Left AV valve regurgitation associated with complete AV block in an adolescent male.
Introduction: Taussig-Bing heart is one of the conotruncal malformation, characterized by double-outlet right ventricle (DORV) and a subpulmonary VSD. Embryologically, abnormal cardiac looping and malalignment of conotruncal septum determines its complexity and great artery relationships.
Case Report: A 14-year-old acyanotic boy presented with severe left-sided AV valve regurgitation and bradycardia. ECG revealed left sided morphologic right ventricular hypertrophy (RVH) as evidenced by a loss of septal Q waves in left precordial leads suggesting ventricular inversion. X-ray chest revealed a straight upper right cardiac border due to loss of normal relationship of great vessels and cardiomegaly due to both left atrial and morphologic right ventricular enlargements suggesting a left-sided regurgitant lesion. Echocardiography revealed the ventricular inversion, primary origin of both L-transposed great arteries from the left-sided morphologic right ventricle suggesting a ‘double-outlet morphologic right ventricle’ with ‘double discordance’ and a subpulmonary VSD of Taussig-Bing type. The left-sided morphologic tricuspid valve is severely regurgitant due to rheumatic process resulting in heart failure which was improved with anti-failure measures and penicillin prophylaxis. Conclusion: The presence of left-sided regurgitation associated with bradycardia is a suspicion of ‘double discordance’ and ‘double switch’ procedure remains the mainstay of its surgical correction. Left AV valve replacement with intraventricular repair is preferred in this child since the malformation is consistent with Taussing-Bing type of DORV with L-transposition.
‘” Taussig-Bing heart” is a morphological subtype of double-outlet right ventricle (DORV) with subpulmonary VSD (ventricular septal defect) [1]. It was first described in 1949 by two outstanding physicians, Helen Brooke Taussig and Richard John Bing, who worked together at the John Hopkins Hospital in Baltimore. The original “Taussig-Bing heart” [2] may be summarized as a double-outlet right ventricle (DORV) with semilunar valves side-by-side and approximately at the same height, a bilateral conus, and a subpulmonary VSD. This original description has been broadened to include all kinds of double-outlet right ventricle with subpulmonary VSD [3],[4]. The morphological variability of “Taussig-Bing heart” is guided by the relationship between great arteries [5] and ventricles that are associated with a bilateral conus [6],[7] and so this case had been reported.
A 14-year-old acyanotic boy was admitted with features of heart failure in the intensive care unit. His pulse rate was 50 bpm and blood pressure 110/70 mmHg. He had a history of rheumatic fever during childhood and blood chemistry revealed a positive ASO (anti-streptolysin O) titer. Auscultation revealed a grade 4/6 pansystolic murmur over the apex with a shift of apical impulse to left 6th space towards mid- axillary line. ECG revealed high-grade AV block with features of ventricular inversion and strain pattern due to morphologic RVH as shown in Figures 1 and 2, compared to a normal right-sided pattern as in Figure 3 with normal echocardiography as in Figure 4 of a normal adolescent male of similar age. X-ray chest revealed a narrow vascular pedicle with left atrial and morphologic right ventricular enlargement as shown in Figure 5. Transthoracic echocardiography revealed a VSD with inlet extension as in Figure 6 and a discordant relationship between atria and ventricles and a left-sided Ebstenoid morphologic tricuspid valve as shown in Figure 7 (apical four chamber view), but mRV (morphologic tricuspid valve) is normally attached as in Figures 8 and 9 (subcostal views). The leaflets are showing the features of rheumatic etiology as in Figures 10 to 14 and severely regurgitant as shown in Figures 15 to 18.
The child was treated with anti-failure medications such as diuretics, angiotensin-converting enzyme inhibitors (enalapril) with penicillin prophylaxis for a period of one and a half months and the regurgitant lesion became decreased as shown in Figures 19 and 20 and M-mode mRV study showing the marked improvement in ejection fraction from 36% to 46% as shown in Figures 21 and 22.
Since the great arteries are primarily originating from morphologic RV as in Figure 23 and it is L-transposed with side-by-side relationship as in Figure 24 with a subpulmonary VSD, it is consistent with Taussig-Bing anomaly of L-transposition, a Kawashima intraventricular repair was advised if further deterioration of mRV function occurs on periodic follow-up with a continuation of medical treatment along with lifelong penicillin prophylaxis. Palliative procedures are not indicated in this boy since there is no aortic arch obstruction and outflow tract lesions as shown in Figures 25 and 26.
Figure 1: ECG showing complete AV block with narrow QRS (QRS duration < 120>
Figure 2: ECG showing the QS complexes in inferior leads (III, aVF) and a QS pattern in right-sided chest leads (V2-V6), compared to a normal ECG of right-sided leads in the same age of a male boy as in Figure 3.
Figure 3: ECG showing RS complexes in the right sided leads of a normal 14-year-old boy.
Figure 4: X-ray chest (postero-anterior) view showing the narrow vascular pedicle (right upper arrow) since great arteries are not border forming, appears straight due to loss of normal relationship between arterial trunks as ascending aorta is typically not visualized on the right side, convexities from descending aortic knob and pulmonary trunk are absent on the left side due to posteriorly positioned pulmonary artery suggesting L-transposition. The cardiomegaly is due to aneurysmal Left atrial (LA) enlargement and a dilated morphologic right ventricle (mRV). The visceral situs is normal. Left upper arrow- ‘hump-shaped’ due to inverted RV infundibulum. Left middle arrow- giant left atrium (GLA). Left lower arrow – ‘septal notch’ corresponding to apical position of interventricular groove.
Figure 5: Apical view showing the normal atrioventricular relationships, AV valves (mitral and tricuspid) attachments and the shape of ventricles in a normal 14-year-old boy. Arrow- long, elongated anterior mitral leaflet (AML) as a feature of mitral valve.
Figure 6: Apical view showing the VSD (ventricular septal defect) with inlet extension. Upper arrow- moderator band. Lower arrow- subpulmonary VSD (ventricular septal defect) with inlet extension.
Figure 7: Apical view showing the ‘Ebstenoid malformation” of left-sided morphologic tricuspid valve (mTV) due to adherence of septum (IVS) to STL. The mTV is identified by a long and elongated posterior leaflet which is short in mitral valve as in Figure 5: of a normal 14-year-old boy and a discordant AV connection. Upper arrow- moderator band. Lower right arrow- IVS (interventricular septum) touching the STL (septal tricuspid leaflet). Left middle arrow- long, elongated posterior tricuspid leaflet (PTL). Lower left arrow- Ebstenoid malformation due to touching of IVS to STL seen as apical displacement of STL and mimicking as Left-sided Ebstein’s anomaly.
Figure 8: Subcostal view showing the normally attached rheumatic morphologic tricuspid valve and the pulmonary valve.
Figure 9: Subcostal view showing the normal attachments of morphologic tricuspid (mTV) and mitral valves (mMV) to the annulus. Arrow- normally attached STL (septal tricuspid leaflet)
Figure 10: Subcostal view-long sweep –showing the thick, calcified morphologic tricuspid valve (mTV) with commissural fusion due to rheumatic etiology. Upper arrow- subpulmonary VSD (ventricular septal defect) in perimembranous location. Right lower arrow- ‘commissural fusion’ due to rheumatic process. Left lower arrow- pulmonary to tricuspid discontinuity as a feature of DORV (double-outlet right ventricle).
Figure 11: Subcostal view-showing the calcification and thickening which is more prominent at leaflet tips due to rheumatic involvement, the normal tricuspid annulus (30 mm) and a dilated morphologic right ventricle (mRV) (65 mm)
Figure 12: Subcostal view showing the marked rheumatic involvement of posterior leaflet of the morphologic tricuspid valve (mRV). Right arrow- thick, calcified posterior tricuspid leaflet (PTL) due to rheumatic process. Left upper arrow- subpulmonary VSD (ventricular septal defect). Left lower arrow- pulmonary to tricuspid discontinuity as a feature of DORV (double-outlet right ventricle)
Figure 13: Subcostal view showing the “sail-like” deformity of posteriot leaflet of morphologic tricuspid valve. Upper arrow- subpulmonary VSD (ventricular septal defect). Lower arrow - “sail-like” posterior tricuspid leaflet.
Figure 14: Subcostal view- long sweep showing the subpulmonary conus. Right upper arrow- subpulmonary VSD (ventricular septal defect). Left upper arrow- subpulmonary conus (infundibulum). Left lower arrow- pulmonary to tricuspid discontinuity.
Figure 15: Apical view showing the severe tricuspid regurgitation (TR). Upper arrow- ‘coarse trabeculations’ as a feature of morphologic right ventricle (mRV). Lower arrow- severe tricuspid regurgitation (TR).
Figure 16: Apical view showing the tricuspid regurgitation jet, originating from the posterior tricuspid leaflet (PTL) swirling around the interatrial septum. Upper arrow- moderator band. Lower arrow- posterior tricuspid leaflet (PTL).
Figure 17: Apical view showing the tricuspid regurgitation jet, originating from the septal tricuspid leaflet (STL) swirling around the lateral wall of left atrium. Upper arrow- moderator band as a feature of morphologic right ventricle (mRV).
Figure 18: Subcostal view showing the severe tricuspid regurgitation originating from the posterior tricuspid leaflet (PTL) swirling around the interatrial septum. Arrow- Tricuspid regurgitation (TR).
Figure 19: Apical view showing the decrease in regurgitant jet due to aggressive medical treatment and left-sided Ebstenoid malformation due to adherence of septum (IVS) to STL. Upper arrow- moderator band. Lower arrow- tricuspid regurgitation (TR) jet.
Figure 20: Subcostal view showing the marked decrease in regurgitant jet due to aggressive medical treatment. Right arrow- PTL (posterior tricuspid leaflet). Left arrow- tricuspid regurgitation (TR).
Figure 21: M-mode mRV study (green line) showing the severe dysfunction of morphologic right ventricle (mRV). EF – 36 %.
Figure 22: M-mode mRV study (green line) showing the improvement in RV function following aggressive medical treatment - EF 46%
Figure 23: Subcostal view-short sweep showing the primary origin of great vessels from the morphological right ventricle (mRV) and it is side-by-side (L-transposition)
Figure 24: Basal short axis view showing the great arteries seen as circles. Right arrow- “posterior sausage” caused by main pulmonary artery. Left arrow- Aorta is left, anterior and superior to pulmonary artery (PA).
Figure 25: Showing the dilated pulmonary trunk in Taussig-Bing heart and there is no obstructive lesions. MPA- dilated main pulmonary artery.
Figure 26: Suprasternal view showing the normal aortic arch. Upper arrow- left common carotid artery. Lower arrow- Left subclavian artery.
3.Discussion:
Review of literature:
Taussig-Bing suspected a totally different malformation at autopsy in a five-and-a-half-year-old girl who had cyanosis since birth and died during angiocardiography after a third intravenous injection of contrast material, the diodrast, the child sat bolt upright and the heart stopped. The malformation consists of a transposed aorta, a large pulmonary artery which arises primarily from the right ventricle and partially overrides the ventricular septum, a high ventricular septal defect, and right ventricular hypertrophy. Maurice Lev was one of the first to call this anomaly as “Taussig-Bing syndrome” [8].
This anomaly was initially described in 1949 by Taussig and Bing as transposition of the great arteries. The important difference between Taussig-Bing anomaly and transposition of the great arteries were described by Richard Van Praagh in his editorial review and stressed that the absence of pulmonary-mitral continuity is the characteristic feature of this anomaly. Taussig and Bing emphasized that the overriding of pulmonary artery was an integral part of this malformation. This anomaly was eventually named as “Taussig-Bing heart” in 1950 by Lev and associates [9] and subsequently introduced the spectrum of Taussig-Bing hearts depending on the degree of pulmonary artery overriding (right-sided, intermediate, and left sided) [10]. The malalignment of infundibular septum as a fundamental requisite to define these hearts, collectively termed as “Taussig-Bing complex”.
Etiopathogenesis
The Taussig-Bing anomaly is relatively rare and it accounted for approximately 8% of cases in Sridaromont series [11]. In these cases, the pulmonary stenosis does not occur, the pulmonary trunk is markedly dilated as in Figure 25 and there is a high VSD immediately subjacent to the pulmonary valve (subpulmonary) and the pulmonary valve is not in continuity with AV (atrioventricular) valve because of interposed sub pulmonary conal free wall musculature as in Figure 14. The anatomic feature of Taussig-Bing malformation appears to result from marked and symmetrical development of both conal free walls. The semilunar valves have been protruded anteriorly to about the same extent by the underlying conal musculature and remain side-by-side, so that both great arteries arise above the anterior (right) ventricle and the failure of complete absorption of the conus or coni result in fibrous discontinuity with atrioventricular valves.
During the development of the heart, the outflow tracts initially connect exclusively with the primitive right ventricle and undergo extensive remodeling to divide into aorta and pulmonary artery with connection to left and right ventricles respectively. Goor and Edwards {12} stated that double-outlet right ventricle appears to represent a primitive embryologic condition as failure to achieve conotruncal inversion and leftward shift of conus results in persistence of this condition with complete origin of both great arteries from the morphologic right ventricle. The primitive cardiac tube anchored at one end by the sinus venosus and at the other end by truncus arteriosus. The chambers of the heart develop by ballooning from the primary heart tube as atrial appendage ballooning in parallel to the atrial component of the tube and the apical component of the ventricle balloons in series from the inlet and outlet parts of the ventricular loop. The ‘looping’ of the straight heart tube during the 3rd week of gestation is one of the key embryologic processes leading to anatomic alignment of chambers of the heart and it normally ‘loops’ to the right (D-loop) resulting in normal morphological position of right ventricle to the right side. The abnormal cardiac looping to the left side (L-loop) brings the morphologic right ventricle to the left and the ventricles are reversed (ventricular inversion), the atrioventricular valves ‘follow’ the ventricles and the two great arteries are transposed or exit from the ‘wrong’ ventricle, but the systemic venous blood flows from the morphologic left ventricle to the pulmonary artery and the systemic arterial blood flows from the morphologic right ventricle to the aorta and so the circulation is corrected. Thus, during embryologic development, left-handed looping of the heart tube results in AV (atrioventricular) discordance and the aorto-pulmonary septum fails to rotate 180⁰ , resulting in ventriculo-arterial discordance. This heart defect is called as ‘corrected transposition’ by Baron Rokitansky in 1875 and described as ‘Rokitansky’s malformation’ [13] in 1957 or L-transposition (Levo-transposition) or ‘ventricular inversion’ and subsequently designated as ‘congenitally corrected transposition’(ccTGV) by Schiebler since the correction was a gift of God, rather than the gift of the surgeon [14]. Abnormalities of the morphologic tricuspid valve (left AV valve) was seen in 90% of L-transposition in autopsy series and significant Ebstein-like malformation of left AV valve, ie, the septal leaflet is displaced inferiorly towards the cardiac apex [15] as in Figure 7, 17 mm apical to the right-sided mitral valve (normally the distance between the tricuspid and mitral annulus is < 8>Figure 13 which is rarely seen in left-sided Ebstein’s malformation. The unusual disposition of the conduction tissue that predisposes to spontaneous AV block [18] as in Figure 1, occurs in 1/10thof infants born with this anomaly [19],[20] with an incidence of 2%/year [21], as 45% will have developed third-degree AV block on 20-year follow up [22] and it reaches a prevalence of 10 to 15% by adolescence and 30% in adulthood [23]. In older patients, fibrotic invasion of proximal and distal bundle branches was involved in its pathogenesis.
The tricuspid valve tends to remain competent during the first decade of life, but progressively become incompetent during the 2nd to 5th decade in 20 to 50% of patients and it has been implicated as a cause of ventricular dysfunction due to volume overload. Since the morphologic right ventricle is in the systemic position, it is unable to generate the necessary forces to sustain the increasing work demand, such as exercise, similar to normal systemic left ventricle [24] due to the loss of special twisting motion during systole and early diastole [25] as it lacks the helical myocyte arrangement seen in left ventricle. The RV muscle mass increase significantly since it is subjected to systemic pressure, leading to right ventricular hypertrophy (RVH) as in ECG shown in Figure 1.
The VSD is neither membranous nor conal septal defect and it is due to its malalignment of well-developed distal conal free walls, the parietal and septal bands may separate or dissociate widely in Taussig-Bing malformation. In L-TGA, the VSDs occur in 60-80% of cases, usually perimembranous, located below the pulmonary valve, extends posteriorly and inferiorly towards the crux of the heart and opening into the inlet of the morphologic left ventricle as in Figure 6. Endocardial cushions in the outflow tract are responsible for the formation of semilunar valves and the development of conal septum (the portion of ventricular septum between the distal ventricular outflow tracts). During conus malrotation, changes in position of anterior portion of the muscular interventricular septum and differential conus absorption leads to malposition of great artery relations. In S.L.L. type of ccTGV [26] (S-situs solitus of the viscera and atriaI, L-loop ventricle, L-transposition of great arteries), deviation in the normal development of interventricular septum as the ventral limb of the left interventricular sulcus on the primary heart tube gives rise to a left-sided crista supraventricularis, which, determines, in part, the right ventricular morphology of the left-sided ventricle. The dorsal limb spirals towards the atrioventricular canal, giving rise to a malpositioned interventricular septum, and displaces the embryologic right ventricle to the left. The course of outlet septum establishes the alignment of VSD with great arteries. If the outflow tract is straight and parallel to the trabecular septum, the VSD becomes subpulmonary and the extreme leftward rotation of the aorta resulting the great arteries nearly in a side-by-side relationship, with the aorta to the left.
The molecular and biological forces that dictate the abnormal cardiac looping in humans are not clear. Cytogenetic analysis identified the discrete chromosomal lesions in the morphogenesis of the heart and appears to be associated with subtypes of DORV. Type 1 DORV (with subaortic VSD) is associated with chromosome 22q11 deletion, the type II (with subpulmonary VSD (supracristal) - Taussig Bing type) with trisomy 13 or 18. The transcription factors such as Nkx 2.5, Gate 4 and Tbx 20 are postulated to influence the right ventricular development via IsI 1, Mef2c and Hand 2 pathways and Pitr 2, Fgf8, Fgf 10 are essential for the development of great arteries [27]. Type 1 DORV might reflect the abnormalities of neural crest cells necessary for the normal outflow tract development and the TGFβ superfamily genes (activin type IIB receptor, noggin, TGFβ2), Sox4 [28], NF1, RAR genes, Endothelin converting enzyme genes (ECE-1, ECE-2) and Pax 3 genes also play a role. Mutations of Gate 4 gene has a potential for the development of type II DORV. Genes determining the body plans such as Pitx 2, CFC1, Cx43 (left-right axis determination), Hox 1.5 (rostral-caudal specification) and jefty 1, inversin (abnormalities of situs and looping) for type 111 DORV (with doubly-committed VSD) and mutations in several other genes had been implicated in few cases of human DORV pertinent to animal models [29],[30]. Recently, it is noted that there is a relationship between primary ciliary dyskinesis (PCD) and ccTGV [31]. The association between cilia and cardiogenesis was made in a baby chick and Willaredt et al [32] found a link between Sonic hedgehog signaling at the primary cilium and the role of bone morphogenic protein in the cardiogenesis of endocardial cushions.
Animal studies also implicate diverse teratogens, the commonly used drugs (ephedrine, theophylline, sodium valproate, retinoic acid/vitamin A, tedral (combination of theophylline, ephedtine, phenobarbitone), other agents including bis-diamine, bromodeoxyuridine), copper citrate, environmental factors such as hair dye, air pollutants [33] and maternal diabetes are enhancing the susceptibility of DORV phenotypes.
Echocardiographic features
Transthoracic echocardiography is the first line modality to assess the specific relations of the different segments of the heart and to determine the presence of any associated malformations. The anatomical designation is first assigned by demonstrating atrial position, ventricular and arterial loopings. The morphology of right ventricle is determined by the presence of course trabeculations, moderator band and the level of tricuspid valve, which is inferior (apical) to mitral valve, also give a clue to ventricular inversion as shown in Figure 7. The morphology of tricuspid valve is characterized by a long, elongated posterior leaflet and a short septal or anterior leaflet in comparison to mitral valve, in which the posterior leaflet is short and the anterior leaflet is long and elongated as in Figure 5. The morphologic TV (tricuspid valve) is a systemic AV valve and its septal leaflet is abnormally displaced inferiorly. This systemic AV valve (mTV) is anatomically abnormal, that predicts the occurrence of regurgitation and transthoracic echocardiography is the technique of choice to evaluate the etiology of TR (tricuspid regurgitation) and in most cases, it is so called “functional” (secondary TR (tricuspid regurgitation) due to dilatation of annulus (diastolic diameter > 40 mm or 21 mm/m2)) as a consequence of RV dilation secondary to pulmonary hypertension. In 15 to 20% of cases, the injury to tricuspid valve can be organic and generally of rheumatic in origin. The morphologic TV (mTV) is thickened and calcified as in Figure 7, more prominent at leaflet tips as in Figure 11 with commissural fusion as in Figure 10, the posterior tricuspid leaflet is predominantly affected as in Figure 12 and it is severely regurgitant as shown in Figures 15 to 18, the annulus diameter is 30 mm (< 40>Figure 11.
The anatomic objective such as “double discordance”, ie, discordant connection at both atrioventricular and ventriculo-arterial junctions can be defined by echocardiography. The discordance between the atrium and ventricle as left atrium is connected to the morphologic Right ventricle (mRV) and the right atrium is connected to the morphologic left ventricle (mLV) was shown in Figure 7.
The origin of great arteries in relation to the septum and its spatial relationships are best visualized in subcostal coronal views. Figure 23 shows the origin of both great arteries primarily from the morphologic RV (mRV), it is parallel to each other (normally cross each other) and the pulmonary trunk is identified by its bifurcation into right and left branches. The great arteries appear as double circles in basal short axis view as in Figure 24 with posterior ‘sausage’ caused by the main pulmonary artery segment, transected tangentially and an anterior circle which is the aorta, positioned leftward, anterior and superior to the pulmonary artery [34] suggesting L-transposition. The subcostal coronal view also helps to determine the lack of fibrous continuity between the posterior semilunar valve (pulmonary) and the anterior tricuspid leaflet as in Figures 11 and 12 and suggesting double-outlet right ventricle with a subpulmonary VSD (Taussig-Bing type). In about 50% of cases of Taussig-Bing anomaly, the congenital malformation of the aorta including coarctation, isthmic hypoplasia, interruption, patent ductus arteriosus and subaortic conus may occur [35],[36]. The aorta is identified by its brachiocephalic branches, but the aortic arch appears normal in this patient as shown in Figure 26.
Management
Systemic AV valve regurgitation is a potential risk factor for the development of dysfunction of the morphologic RV and early heart failure [37] and those patients without such complications (ie, in the absence of hemodynamic lesions) appear to function well into late adulthood in ccTGV [38],[39]. Medical management with diuretics, afterload reduction with angiotensin converting enzyme inhibitors such as captopril and digoxin (inotropic) is adapted for the failing systemic right ventricle [40], depending on the severity of associated malformations [41]. The role of angiotensin receptor blocker with losartan was evaluated and found to have no improvement in exercise capacity and no reduction in neurohormonal levels in patients with systemic right ventricle [42]. Beta blockers did not improve outcome in a randomized trial included pediatric patients with systemic RV dysfunction [43] and must be used cautiously in ccTGV patients as they may precipitate AV block, but an improvement in systemic ventricular function occurred after carvedilol (a vasodilatory beta blocker) administration in a 80-year old woman with ccTGV [44], Aggressive medical treatment with afterload reduction is indicated for patients with ventricular enlargement and early symptoms. The benefits of prophylactic use of vasodilators are unproven to delay or prevent systemic ventricular dysfunction.
Interventional therapy
In patients with widened QRS complex (QRS duration > 120-140 ms, sometimes >200ms) due to bundle branch blocks, the electromechanical dyssynchrony create insufficiency in ventricular ejection and restoration of electrical synchrony with transvenous permanent para-Hisian pacing (cardiac resynchronization therapy) reduces the QRS duration and improves the hemodynamics of the failing systemic right ventricle [45],[46], but is technically challenging [47]
Complete heart block is also a common cause of death [48]. Early pacemaker placement is recommended in the setting of complete heart block if there is RV dysfunction, symptoms of bradycardia or heart failure and urgently done if it occurs during or after the surgical intervention when the bradycardia is intolerable. It can be technically challenging pertaining to the complex heart anatomy [49] such as modification in position of ventricular septum that induces a septal shift and failure of tricuspid valve coaptation [50] leading to worsening of systemic ventricular function and AV valve regurgitation.
Surgical therapy
The evolution of surgical repair for the Taussig-Bing anomaly has progressed from atrial baffle procedures to arterial switch with VSD closure and intraventricular repair. The need for surgical interventions varies according to the associated defects and several options are available as
It represents a group of procedures in which AV discordance is corrected by an inflow, baffling procedure at atrial level, called as atrial or venous switch (Mustard or Senning) and the ventriculo--arterial discordance is corrected by either an outflow procedure at arterial or ventricular level, called as arterial or ventricular switch or by translocation of the aortic root (Jatane procedure) and collectively termed as “double-switch”. The choice of procedures depending on the underlying anatomy of LVOT/or morphology of VSD [56]. A modification of atrial switch procedures to a hemi-Mustard technique can be performed to overcome the high early morbidity and mortality in more complex ccTGV patients to decrease the surgical challenge [57].
The indication for anatomic repair for those with a “balanced” systemic and pulmonary blood flow without clinically significant cyanosis is still unclear. Current indication for ‘double switch’ operation includes patients who have developed symptoms attributed to tricuspid regurgitation or RV dysfunction or both [58]. The anatomic repair is currently the first choice for most surgeons in treating ccTGV [59], only meant for patients with a favourable anatomy [60] and it remains the best choice for TGA type of DORV [61]. Early prophylactic pulmonary banding [62] as a bridge to ‘double switch surgery’ related to LV retraining in selected asymptomatic young infants with isolated ccTGA is a challenging practice to expand anatomic repair in those patients who suffer from persistent late LV failure due to LV retraining.
For patients with Taussig-Bing type of DORV, the ‘arterial switch’ operation still appears to be the procedure of choice and can be performed in the neonatal period [63] and also in patients with all types of great artery anatomy without ventriculotomy. The arterial switch for Taussig-Bing heart was first reported in 1981 [64]. The issue of great artery anatomy was first addressed by Yacoub and Radley- Smith [65] and many surgeons have thought that the side-by-side great arteries are most suitable for intraventricular repair (Kawashima repair) [66],[67], which is also applicable to unusual coronary artery anatomy or a pulmonary valve that is not considered adequate to function as a systemic valve. The antero-posterior great arteries are most suitable for arterial switch with a closure of VSD. The distance between the tricuspid and pulmonary valve for an intraventricular tunnel [68] is more important than the great artery relations and in side-by-side great arteries, the conduit can pass posteriorly and allow unimpeded flow to the pulmonary valve, ie, by tunneling the left ventricular flow posterior to the pulmonary valve. The operative mortality for arterial switch is 13%, 3% for intraventricular repair and late mortality for both series is 5 to 6%.
Outcome:
In patients without tricuspid valve regurgitation, the survival rate at 20 years is > 90% and it is < 50> 40%, the patients do reasonably well. In addition, a number of reports of ccTGA in adults come to medical attention for either cardiac or non cardiac reasons in 6th to 8th decade, indeed, the right ventricle can function as a systemic ventricle for many tears [70].
Case Analysis:
The 14-year-old boy was presented with features of “double discordance” (Figure 7) with primary origin of L-transposed great arteries from the left-sided morphologic right ventricle (Figure 23) and a pulmonary-tricuspid discontinuity with well-developed pulmonary conus (Figure 14) and a malaligned subpulmonary VSD (Figure 10), suggesting a “Taussig-Bing heart” with L-transposition. The visceral situs was normal (Figure 4) and there Is a marked RV dilatation (Figure 11) with severe tricuspid regurgitation (Figures 15 to 18) due to rheumatic etiology (Figures 8 to 10) and so it is designated as “Rheumatic Taussig-Bing heart”.
The septal malalignment in situ solitus alters the cardiac hemodynamics and conduction pathways, leading to increased risk of complications. The atrial and ventricular septa are malaligned and the AV node cannot penetrate the ventricular mass, indeed, a secondary node penetrates the anterior septum, predisposing to high grade atrioventricular block [71] (Figure 1) with a tolerable heart rate (50 bpm) due to congenital etiology. The septal malalignment in situs solitus also leads to a high prevalence of Ebstein-like anomalies and 50% of ccTGV patients had abnormal tricuspid valve, compared to normal in situs inversus group. The left-sided Ebstenoid-like malformation of mTV as in Figure 7 (apical four chamber view) is due to septal malalignment, mimicking as apical displacement of septal tricuspid leaflet which is normally attached to annulus as in Figures 8 and 9 shown in subcostal views with a subpulmonary VSD (Figure 10) as the result of this septal malalignment.
On aggressive medical management with antifailure measures and penicillin prophylaxis, the severe tricuspid regurgitation decreases as shown in Figures 19 and 20. It is a primary tricuspid regurgitation due to rheumatic involvement of mTV and the tricuspid annulus is not dilated (< 40>Figure 11. The mRV function improves from the severe dysfunction (Figure 21- EF 36%) to a moderate degree (Figure 22- EF 46%).
The boy remains acyanotic with an improvement in hemodynamic status, the medical measures are continued with lifelong penicillin prophylaxis and advised periodic echocardiographic evaluation to assess the ventricular function. Since the left-sided morphologic RV is a systemic pumping chamber, it is hypertrophied (Figure 1) and may deteriorate in function on long-term follow-up. Since the both ventricles are adequate without any outflow tract obstructions (Figures 25, 26) and the great arteries are in side-by-side, the Kawashima intraventricular repair is the preferred surgical option with tricuspid valve replacement in this boy. The pacemaker implantation is necessary when the heart rate becomes intolerable especially during surgical procedures.
Taussig-Bing heart is one of the variants of double-outlet right ventricle (DORV) [72] and it constitues < 10>
Clearly Auctoresonline and particularly Psychology and Mental Health Care Journal is dedicated to improving health care services for individuals and populations. The editorial boards' ability to efficiently recognize and share the global importance of health literacy with a variety of stakeholders. Auctoresonline publishing platform can be used to facilitate of optimal client-based services and should be added to health care professionals' repertoire of evidence-based health care resources.
Journal of Clinical Cardiology and Cardiovascular Intervention The submission and review process was adequate. However I think that the publication total value should have been enlightened in early fases. Thank you for all.
Journal of Women Health Care and Issues By the present mail, I want to say thank to you and tour colleagues for facilitating my published article. Specially thank you for the peer review process, support from the editorial office. I appreciate positively the quality of your journal.
Journal of Clinical Research and Reports I would be very delighted to submit my testimonial regarding the reviewer board and the editorial office. The reviewer board were accurate and helpful regarding any modifications for my manuscript. And the editorial office were very helpful and supportive in contacting and monitoring with any update and offering help. It was my pleasure to contribute with your promising Journal and I am looking forward for more collaboration.
We would like to thank the Journal of Thoracic Disease and Cardiothoracic Surgery because of the services they provided us for our articles. The peer-review process was done in a very excellent time manner, and the opinions of the reviewers helped us to improve our manuscript further. The editorial office had an outstanding correspondence with us and guided us in many ways. During a hard time of the pandemic that is affecting every one of us tremendously, the editorial office helped us make everything easier for publishing scientific work. Hope for a more scientific relationship with your Journal.
The peer-review process which consisted high quality queries on the paper. I did answer six reviewers’ questions and comments before the paper was accepted. The support from the editorial office is excellent.
Journal of Neuroscience and Neurological Surgery. I had the experience of publishing a research article recently. The whole process was simple from submission to publication. The reviewers made specific and valuable recommendations and corrections that improved the quality of my publication. I strongly recommend this Journal.
Dr. Katarzyna Byczkowska My testimonial covering: "The peer review process is quick and effective. The support from the editorial office is very professional and friendly. Quality of the Clinical Cardiology and Cardiovascular Interventions is scientific and publishes ground-breaking research on cardiology that is useful for other professionals in the field.
Thank you most sincerely, with regard to the support you have given in relation to the reviewing process and the processing of my article entitled "Large Cell Neuroendocrine Carcinoma of The Prostate Gland: A Review and Update" for publication in your esteemed Journal, Journal of Cancer Research and Cellular Therapeutics". The editorial team has been very supportive.
Testimony of Journal of Clinical Otorhinolaryngology: work with your Reviews has been a educational and constructive experience. The editorial office were very helpful and supportive. It was a pleasure to contribute to your Journal.
Dr. Bernard Terkimbi Utoo, I am happy to publish my scientific work in Journal of Women Health Care and Issues (JWHCI). The manuscript submission was seamless and peer review process was top notch. I was amazed that 4 reviewers worked on the manuscript which made it a highly technical, standard and excellent quality paper. I appreciate the format and consideration for the APC as well as the speed of publication. It is my pleasure to continue with this scientific relationship with the esteem JWHCI.
This is an acknowledgment for peer reviewers, editorial board of Journal of Clinical Research and Reports. They show a lot of consideration for us as publishers for our research article “Evaluation of the different factors associated with side effects of COVID-19 vaccination on medical students, Mutah university, Al-Karak, Jordan”, in a very professional and easy way. This journal is one of outstanding medical journal.
Dear Hao Jiang, to Journal of Nutrition and Food Processing We greatly appreciate the efficient, professional and rapid processing of our paper by your team. If there is anything else we should do, please do not hesitate to let us know. On behalf of my co-authors, we would like to express our great appreciation to editor and reviewers.
As an author who has recently published in the journal "Brain and Neurological Disorders". I am delighted to provide a testimonial on the peer review process, editorial office support, and the overall quality of the journal. The peer review process at Brain and Neurological Disorders is rigorous and meticulous, ensuring that only high-quality, evidence-based research is published. The reviewers are experts in their fields, and their comments and suggestions were constructive and helped improve the quality of my manuscript. The review process was timely and efficient, with clear communication from the editorial office at each stage. The support from the editorial office was exceptional throughout the entire process. The editorial staff was responsive, professional, and always willing to help. They provided valuable guidance on formatting, structure, and ethical considerations, making the submission process seamless. Moreover, they kept me informed about the status of my manuscript and provided timely updates, which made the process less stressful. The journal Brain and Neurological Disorders is of the highest quality, with a strong focus on publishing cutting-edge research in the field of neurology. The articles published in this journal are well-researched, rigorously peer-reviewed, and written by experts in the field. The journal maintains high standards, ensuring that readers are provided with the most up-to-date and reliable information on brain and neurological disorders. In conclusion, I had a wonderful experience publishing in Brain and Neurological Disorders. The peer review process was thorough, the editorial office provided exceptional support, and the journal's quality is second to none. I would highly recommend this journal to any researcher working in the field of neurology and brain disorders.
Dear Agrippa Hilda, Journal of Neuroscience and Neurological Surgery, Editorial Coordinator, I trust this message finds you well. I want to extend my appreciation for considering my article for publication in your esteemed journal. I am pleased to provide a testimonial regarding the peer review process and the support received from your editorial office. The peer review process for my paper was carried out in a highly professional and thorough manner. The feedback and comments provided by the authors were constructive and very useful in improving the quality of the manuscript. This rigorous assessment process undoubtedly contributes to the high standards maintained by your journal.
International Journal of Clinical Case Reports and Reviews. I strongly recommend to consider submitting your work to this high-quality journal. The support and availability of the Editorial staff is outstanding and the review process was both efficient and rigorous.
Thank you very much for publishing my Research Article titled “Comparing Treatment Outcome Of Allergic Rhinitis Patients After Using Fluticasone Nasal Spray And Nasal Douching" in the Journal of Clinical Otorhinolaryngology. As Medical Professionals we are immensely benefited from study of various informative Articles and Papers published in this high quality Journal. I look forward to enriching my knowledge by regular study of the Journal and contribute my future work in the field of ENT through the Journal for use by the medical fraternity. The support from the Editorial office was excellent and very prompt. I also welcome the comments received from the readers of my Research Article.
Dear Erica Kelsey, Editorial Coordinator of Cancer Research and Cellular Therapeutics Our team is very satisfied with the processing of our paper by your journal. That was fast, efficient, rigorous, but without unnecessary complications. We appreciated the very short time between the submission of the paper and its publication on line on your site.
I am very glad to say that the peer review process is very successful and fast and support from the Editorial Office. Therefore, I would like to continue our scientific relationship for a long time. And I especially thank you for your kindly attention towards my article. Have a good day!
"We recently published an article entitled “Influence of beta-Cyclodextrins upon the Degradation of Carbofuran Derivatives under Alkaline Conditions" in the Journal of “Pesticides and Biofertilizers” to show that the cyclodextrins protect the carbamates increasing their half-life time in the presence of basic conditions This will be very helpful to understand carbofuran behaviour in the analytical, agro-environmental and food areas. We greatly appreciated the interaction with the editor and the editorial team; we were particularly well accompanied during the course of the revision process, since all various steps towards publication were short and without delay".
I would like to express my gratitude towards you process of article review and submission. I found this to be very fair and expedient. Your follow up has been excellent. I have many publications in national and international journal and your process has been one of the best so far. Keep up the great work.
We are grateful for this opportunity to provide a glowing recommendation to the Journal of Psychiatry and Psychotherapy. We found that the editorial team were very supportive, helpful, kept us abreast of timelines and over all very professional in nature. The peer review process was rigorous, efficient and constructive that really enhanced our article submission. The experience with this journal remains one of our best ever and we look forward to providing future submissions in the near future.
I am very pleased to serve as EBM of the journal, I hope many years of my experience in stem cells can help the journal from one way or another. As we know, stem cells hold great potential for regenerative medicine, which are mostly used to promote the repair response of diseased, dysfunctional or injured tissue using stem cells or their derivatives. I think Stem Cell Research and Therapeutics International is a great platform to publish and share the understanding towards the biology and translational or clinical application of stem cells.
I would like to give my testimony in the support I have got by the peer review process and to support the editorial office where they were of asset to support young author like me to be encouraged to publish their work in your respected journal and globalize and share knowledge across the globe. I really give my great gratitude to your journal and the peer review including the editorial office.
I am delighted to publish our manuscript entitled "A Perspective on Cocaine Induced Stroke - Its Mechanisms and Management" in the Journal of Neuroscience and Neurological Surgery. The peer review process, support from the editorial office, and quality of the journal are excellent. The manuscripts published are of high quality and of excellent scientific value. I recommend this journal very much to colleagues.
Dr.Tania Muñoz, My experience as researcher and author of a review article in The Journal Clinical Cardiology and Interventions has been very enriching and stimulating. The editorial team is excellent, performs its work with absolute responsibility and delivery. They are proactive, dynamic and receptive to all proposals. Supporting at all times the vast universe of authors who choose them as an option for publication. The team of review specialists, members of the editorial board, are brilliant professionals, with remarkable performance in medical research and scientific methodology. Together they form a frontline team that consolidates the JCCI as a magnificent option for the publication and review of high-level medical articles and broad collective interest. I am honored to be able to share my review article and open to receive all your comments.
“The peer review process of JPMHC is quick and effective. Authors are benefited by good and professional reviewers with huge experience in the field of psychology and mental health. The support from the editorial office is very professional. People to contact to are friendly and happy to help and assist any query authors might have. Quality of the Journal is scientific and publishes ground-breaking research on mental health that is useful for other professionals in the field”.
Dear editorial department: On behalf of our team, I hereby certify the reliability and superiority of the International Journal of Clinical Case Reports and Reviews in the peer review process, editorial support, and journal quality. Firstly, the peer review process of the International Journal of Clinical Case Reports and Reviews is rigorous, fair, transparent, fast, and of high quality. The editorial department invites experts from relevant fields as anonymous reviewers to review all submitted manuscripts. These experts have rich academic backgrounds and experience, and can accurately evaluate the academic quality, originality, and suitability of manuscripts. The editorial department is committed to ensuring the rigor of the peer review process, while also making every effort to ensure a fast review cycle to meet the needs of authors and the academic community. Secondly, the editorial team of the International Journal of Clinical Case Reports and Reviews is composed of a group of senior scholars and professionals with rich experience and professional knowledge in related fields. The editorial department is committed to assisting authors in improving their manuscripts, ensuring their academic accuracy, clarity, and completeness. Editors actively collaborate with authors, providing useful suggestions and feedback to promote the improvement and development of the manuscript. We believe that the support of the editorial department is one of the key factors in ensuring the quality of the journal. Finally, the International Journal of Clinical Case Reports and Reviews is renowned for its high- quality articles and strict academic standards. The editorial department is committed to publishing innovative and academically valuable research results to promote the development and progress of related fields. The International Journal of Clinical Case Reports and Reviews is reasonably priced and ensures excellent service and quality ratio, allowing authors to obtain high-level academic publishing opportunities in an affordable manner. I hereby solemnly declare that the International Journal of Clinical Case Reports and Reviews has a high level of credibility and superiority in terms of peer review process, editorial support, reasonable fees, and journal quality. Sincerely, Rui Tao.
Clinical Cardiology and Cardiovascular Interventions I testity the covering of the peer review process, support from the editorial office, and quality of the journal.
Clinical Cardiology and Cardiovascular Interventions, we deeply appreciate the interest shown in our work and its publication. It has been a true pleasure to collaborate with you. The peer review process, as well as the support provided by the editorial office, have been exceptional, and the quality of the journal is very high, which was a determining factor in our decision to publish with you.
The peer reviewers process is quick and effective, the supports from editorial office is excellent, the quality of journal is high. I would like to collabroate with Internatioanl journal of Clinical Case Reports and Reviews journal clinically in the future time.
Clinical Cardiology and Cardiovascular Interventions, I would like to express my sincerest gratitude for the trust placed in our team for the publication in your journal. It has been a true pleasure to collaborate with you on this project. I am pleased to inform you that both the peer review process and the attention from the editorial coordination have been excellent. Your team has worked with dedication and professionalism to ensure that your publication meets the highest standards of quality. We are confident that this collaboration will result in mutual success, and we are eager to see the fruits of this shared effort.
Dear Dr. Jessica Magne, Editorial Coordinator 0f Clinical Cardiology and Cardiovascular Interventions, I hope this message finds you well. I want to express my utmost gratitude for your excellent work and for the dedication and speed in the publication process of my article titled "Navigating Innovation: Qualitative Insights on Using Technology for Health Education in Acute Coronary Syndrome Patients." I am very satisfied with the peer review process, the support from the editorial office, and the quality of the journal. I hope we can maintain our scientific relationship in the long term.
Dear Monica Gissare, - Editorial Coordinator of Nutrition and Food Processing. ¨My testimony with you is truly professional, with a positive response regarding the follow-up of the article and its review, you took into account my qualities and the importance of the topic¨.
Dear Dr. Jessica Magne, Editorial Coordinator 0f Clinical Cardiology and Cardiovascular Interventions, The review process for the article “The Handling of Anti-aggregants and Anticoagulants in the Oncologic Heart Patient Submitted to Surgery” was extremely rigorous and detailed. From the initial submission to the final acceptance, the editorial team at the “Journal of Clinical Cardiology and Cardiovascular Interventions” demonstrated a high level of professionalism and dedication. The reviewers provided constructive and detailed feedback, which was essential for improving the quality of our work. Communication was always clear and efficient, ensuring that all our questions were promptly addressed. The quality of the “Journal of Clinical Cardiology and Cardiovascular Interventions” is undeniable. It is a peer-reviewed, open-access publication dedicated exclusively to disseminating high-quality research in the field of clinical cardiology and cardiovascular interventions. The journal's impact factor is currently under evaluation, and it is indexed in reputable databases, which further reinforces its credibility and relevance in the scientific field. I highly recommend this journal to researchers looking for a reputable platform to publish their studies.
Dear Editorial Coordinator of the Journal of Nutrition and Food Processing! "I would like to thank the Journal of Nutrition and Food Processing for including and publishing my article. The peer review process was very quick, movement and precise. The Editorial Board has done an extremely conscientious job with much help, valuable comments and advices. I find the journal very valuable from a professional point of view, thank you very much for allowing me to be part of it and I would like to participate in the future!”
Dealing with The Journal of Neurology and Neurological Surgery was very smooth and comprehensive. The office staff took time to address my needs and the response from editors and the office was prompt and fair. I certainly hope to publish with this journal again.Their professionalism is apparent and more than satisfactory. Susan Weiner
My Testimonial Covering as fellowing: Lin-Show Chin. The peer reviewers process is quick and effective, the supports from editorial office is excellent, the quality of journal is high. I would like to collabroate with Internatioanl journal of Clinical Case Reports and Reviews.
My experience publishing in Psychology and Mental Health Care was exceptional. The peer review process was rigorous and constructive, with reviewers providing valuable insights that helped enhance the quality of our work. The editorial team was highly supportive and responsive, making the submission process smooth and efficient. The journal's commitment to high standards and academic rigor makes it a respected platform for quality research. I am grateful for the opportunity to publish in such a reputable journal.
My experience publishing in International Journal of Clinical Case Reports and Reviews was exceptional. I Come forth to Provide a Testimonial Covering the Peer Review Process and the editorial office for the Professional and Impartial Evaluation of the Manuscript.