Discordant Atrioventricular Connections Research Articles

Long-term Outcomes of Surgical Repair for Corrected Transposition of the Great Arteries

BackgroundThis study investigated the long-term outcomes of physiological and anatomical repair for corrected transposition of the great arteries and double-outlet right ventricle with discordant atrioventricular connection. MethodsThis single-center retrospective study included 146 patients who underwent biventricular repair of corrected transposition of the great arteries or double-outlet right ventricle with discordant atrioventricular connections from 1972 to 2023. Survival rate, freedom from reoperation, New York Heart Association (NYHA) Functional Classification, and incidence of systemic ventricular dysfunction in the long-term were compared between physiological repair (PR) and anatomical repair (AR) groups. ResultsThe PR group consisted of 55 patients, with median age at repair of 10.3 years. Thirty-one patients underwent conventional Rastelli procedure, and 24 patients underwent atrial and/or ventricular septal defect closure. The AR group consisted of 91 patients, with median age at repair of 5.8 years. Seventy-two patients underwent atrial switch plus Rastelli procedure and 19 patients underwent atrial plus arterial switch operation. The 30-year survival was 63.5% in the PR group and 72.3% in the AR group (P = .448). The 30-year freedom from reoperation was 71.9% in the PR group and 62.2% in the AR group (P = .220). There was a significant difference in the incidence of systemic ventricular dysfunction between the groups (87.5% in the PR group and 35.3% in the AR group, P < .001) and in the NYHA classification of long-term survivors (mean NYHA class of 1.9 in the PR group and 1.5 in the AR group, P = .009). ConclusionsThe systemic ventricular function and general status in the long-term were significantly better in AR patients, suggesting the potential advantage of AR.

Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of "Double Outlet Left Atrium with three atrioventricular valves," which provided the necessary "left to right" shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.

Criss-cross heart: a clinical case of a rare congenital heart defect in a newborn

Introduction. Criss-cross syndrome is a rare congenital heart disease with an incidence of 8 cases per 1 million newborns. The purpose of the work is to demonstrate a rare CHD, a criss-cross heart diagnosed postnatally. The purpose of the work was to demonstrate a rare CHD, a criss-cross heart diagnosed postnatally. Materials and methods. A clinical observation of a newborn child with a rare congenital heart defect is presented. Medical documents with the results of clinical examination (physical examination) and instrumental examinations (X-ray examination, echocardiography, computed tomography) were used for analysis. Results. A child with a rare congenital heart defect (CHD) – criss-cross heart with discordant atrioventricular connections, transposition of the great vessels and preductal aortic coarctation was monitored from the time of delivery until surgical treatment. This malformation was diagnosed postnatally, based on the characteristic clinical symptoms of the detailed segmental analysis of the ultrasound picture of the heart, which is presented as illustrations. Timely diagnosis allowed the choice of adequate medication and surgical tactics of treatment, and the diagnosis was confirmed by computed tomography. Discussion. It is important for the clinician to remember that rare congenital heart diseases cannot always be diagnosed antenatally, and with the results of screening indicating the presence of more common malformations, the real postnatal heart picture may differ significantly. An additional difficulty is the differential diagnosis of the described malformation due to the rare anomalous mutual arrangement of the anatomical structures. Conclusion. Detailed segmental analysis during echocardiography immediately after birth reveals the main diagnostic marker of criss-cross syndrome – inability to simultaneously visualize all four heart chambers and both atrioventricular valves from a four-chamber position.

Transposition physiology in the setting of concordant ventriculo-arterial connections.

To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re-interventions, for the rare instances of transposition physiology with concordant ventriculo-arterial connections. We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo-arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Among reported cases, just over two-thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four-fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one-fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one-fifth of patients requiring pacemakers for surgically-induced heart block. The majority of survivors were in good functional state. The flow patterns produced by discordant atrioventricular and concordant ventriculo-arterial connections remain an important, albeit rare, indication for atrial redirection or hemi-Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long-term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.

Coronary‐to‐pulmonary collateral artery from a superdominant morphologically right coronary artery

We present a case of a 16-year-old cyanotic male patient with ventricular septal defect, pulmonary atresia and discordant atrioventricular connections where computed tomography angiography demonstrated a large tortuous coronary-to-pulmonary collateral artery arising from the superdominant morphologically right coronary artery and coursing cranially to supply the pulmonary parenchyma.

Third Atrial Chamber

A 10-MONTH-OLD BOY (weight: 5.8 kg; height: 66 cm; body mass index: 13) was taken to the cardiac catheterization laboratory before the creation of a bidirectional Glenn shunt. At 4 days of age, transthoracic echocardiography done for the evaluation of persistent cyanosis revealed heterotaxy (left atrial isomerism), situs ambiguous with levocardia, bilateral superior vena cava, atrial septal defect (9 mm), partial anomalous right pulmonary venous return to the morphologic right atrium, discordant atrioventricular connection and concordant ventriculoarterial connection, with a hypoplastic right ventricle. The child underwent stenting of the patent ductus arteriosus and balloon atrial septostomy on the 11th day of life. Pulmonary artery banding was done at the age of 1 month. During the current admission, a computerized tomography scan revealed the complex connections of the great vessels (Fig 1). A 4-chamber transesophageal echocardiography view displayed the morphologic left atrium, which was on the right side, and multiple coronary artery fistulae opening into an enlarged chamber located superiorly (Fig 2; Video 1). The atrial septal defect could be visualized in the bicaval view (Fig 3; Video 2). A modified midesophageal 4-chamber view displayed a large superior chamber, as well as the atrial septal defect (Fig 4). Agitated saline was injected into a right upper extremity vein. The agitated saline appeared first in the right internal jugular vein and then appeared in the upper atrial chamber, right side atrium (morphologic left atrium), followed by an appearance in the right ventricle (Fig 5, A-D; Video 3). Slight retraction of the probe from the 4-chamber view displayed a right superior vena cava on the right side that was moving into close juxtaposition with the morphologic left atrium (Fig 6; Video 4). Correlation with computerized tomography findings revealed the identity of the structure seen by transesophageal echocardiography into which the right superior vena cava and the coronary artery fistulae were draining. What is the diagnosis? Fig. 2(A) Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with (B) color Doppler displaying an enlarged chamber (white query sign) with multiple coronary artery fistulae opening into the chamber. mLA, morphologic left atrium; RV, right ventricle; TV, tricuspid valve View Large Image Figure Viewer Download Hi-res image Fig. 3Bicaval midesophageal transesophageal echocardiography view displaying the atrial septal defect. View Large Image Figure Viewer Download Hi-res image Fig. 4Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with color-flow Doppler displaying the atrial septal defect and a large upper chamber marked by white query sign. View Large Image Figure Viewer Download Hi-res image Fig. 5Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying (A) the agitated saline echo contrast injected into an upper limb vein entering the right superior vena cava, (B) the coronary sinus and the morphologic right atrium on the right side, (C) the morphologic left atrium on the right side, and (D) the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 6Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying the right superior vena cava and morphologic right atrium in close proximity. ASD, atrial septal defect; LAA, morphologic left atrial appendage; mLeft atrium, morphologic left atrium; mR, morphologic right atrium; RSVC, right superior vena cava; RV, right ventricle; TV, tricuspid valve. View Large Image Figure Viewer Download Hi-res image

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

Multiple Color Doppler Flow Jets Into The Atria in a Child With Heterotaxy Syndrome

A nine-month-old girl (weight: 7.92 kg; height: 70 cm) was taken to the operating room for the creation of a bilateral bidirectional Glenn shunt and atrial septectomy. Patent ductus arteriosus stenting had been done when the child was four days old. Transthoracic echocardiography showed heterotaxy (left atrial isomerism) with situs inversus, discordant atrioventricular and concordant ventriculoarterial connections (ventricular ‘noninversion’), a large ventricular septal defect, membranous pulmonary atresia with a hypoplastic main pulmonary artery, confluent branch pulmonary arteries, aorta anterior and to the right of the pulmonary artery, and bilateral superior vena cava with no bridging vein. The left superior vena cava was draining into the left-sided atrium (morphologic right atrium), while the right superior vena cava was draining into the same atrium via a massively dilated coronary sinus. The inferior vena cava was interrupted, with hemzygous continuation to the left superior vena cava. A computerized tomography scan revealed a right-sided stomach with a midline liver, and demonstrated the complex vascular communications (Fig 1, A and B). Transesophageal echocardiography confirmed the preoperative diagnosis (Fig. 2, Fig. 3; Videos 1 and 2). Color- flow Doppler mapping in the mid-esophageal view demonstrated multiple jets into both the atria and the right-sided ventricle (Fig 4, A and B; Videos 3 and 4). Pulse-wave Doppler of a dominant jet revealed a biphasic flow (Fig 5; Video 5). The intramyocardial origin of the jets was traced toward the aorta (Fig 6; Video 6). Fig. 2The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map showing demonstrating the anatomic abnormality in the child. View Large Image Figure Viewer Download Hi-res image Fig. 3The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map demonstrating the left superior vena cava. Left SVC, left superior vena cava. View Large Image Figure Viewer Download Hi-res image Fig. 4(A) The midesophageal transesophageal echocardiography views with color-flow Doppler map demonstrating jets (white arrows) into the left atrium. (B) The right atrium and the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 5The midesophageal pulse-wave Doppler signal interrogation of the jet by transesophageal echocardiography. View Large Image Figure Viewer Download Hi-res image Fig. 6The midesophageal transesophageal echocardiography view displaying the color-flow Doppler mapping delineating the origin of the jet (white arrows). View Large Image Figure Viewer Download Hi-res image

Femoral Artery–Systemic-Pulmonary Shunt–Femoral Vein Wire Loop to Assist Balloon Pulmonary Valvuloplasty in Infants with Discordant Atrioventricular and Ventriculoarterial Connection, Ventricular Septal Defect, and Extremely Severe Pulmonary Stenosis

Femoral Artery–Systemic-Pulmonary Shunt–Femoral Vein Wire Loop to Assist Balloon Pulmonary Valvuloplasty in Infants with Discordant Atrioventricular and Ventriculoarterial Connection, Ventricular Septal Defect, and Extremely Severe Pulmonary Stenosis

Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography.

To evaluate the imaging characteristics and associations in patients with twisted atrioventricular connections on multidetectorcomputed tomography (CT) angiography. We retrospectively reviewed 2605 CT angiography studies performed for suspected congenital heart diseases in our institution from January 2014 to December 2018. Twisted atrioventricular connections were diagnosed in 12 patients. Segmental sequential approach developed by Anderson et al was employed to characterize the complex congenital heart disease. Ventricular topology was also determined. CTA was also assessed to look for any associated intra- and extracardiac anomalies. Out of 12 patients with twisted atrioventricular connections, usual viscero-atrial arrangement was seen in nine patients, two patients had mirror-imaged viscero-atrial arrangement and one patient had mirror-imaged atrial arrangement. Right-sided heart was seen in four patients. Two patients had discordant atrioventricular connections, seven had concordant atrioventricular connections while three showed double-inlet right ventricle. Ventriculoarterial connections were concordant in two patients, discordant in one patient while nine patients had double-outlet right ventricle. Superior-inferior ventricular morphology with near-horizontal interventricular septum was seen in four patients. Left-sided aortic arch with normal branching pattern was observed in nine patients. No coronary anomaly was seen in any patient. Other associations included ventricular and atrial septal defects, pulmonic stenosis, hypoplasia/atresia of tricuspid valve, straddling of mitral valve, and duplicated superior caval veins. CT angiography is useful in diagnosis of twisted atrioventricular connections with accurate identification of viscero-atrial arrangement, atrioventricular and ventriculoarterial connections, and orientation and presence or absence of associated anomalies.

Congenitally corrected transposition of the great arteries: is it really a transposition? An anatomical study of the right ventricular septal surface

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital malformation which associates discordant atrioventricular and ventriculo-arterial connections. Although frequently associated with a ventricular septal defect (VSD), its anatomy remains controversial. This could be due in hearts with usual atrial arrangement to the apparently different anatomy of the left-sided right ventricle compared with a right-sided right ventricle. We wanted to compare the RV septal anatomy between ccTGA, transposition of the great arteries and normal heart and to determine the anatomy of the VSD in ccTGA. We analysed 102 human heart specimens: 31 ccTGA, 36 transpositions of the great arteries, 35 normal hearts. According to the last classification of VSD (ICD-11), VSD were classified as outlet if located above the superoseptalcommissure of the tricuspid valve and inlet if underneath. We measured the lengths of the superior and inferior limbs of the septal band and the angle between the two limbs. To assess the orientation of the septal band, we also measured the angle between superior limb and the arterial valve above. A VSD was present in 26 ccTGA (84%) and was an outlet VSD in 16 cases (61%). The mean angle between the two limbs of the septal band was 76.4° for ccTGA compared with 90.6° for transposition of the great arteries (P=0.011) and 76.1° for normal hearts (P= NS). The mean angle between the superior limb of the septal band and the arterial valve above was 70.6° for ccTGA compared with 90.6° for transposition of the great arteries (P=0.0004) and 69.1° for normal hearts (P= NS). The inferior limb of the septal band was significantly shorter in ccTGA (P<0.0003): SL/IL length ratio was 21.4 for ccTGA, 2.2 for transposition of the great arteries and 1.5 for normal hearts. The typical VSD in ccTGA is an outlet VSD. Its frequent misdiagnosis as an inlet VSD might be explained by the shortness of the inferior limb, which creates the illusion of a posterior VSD, and by the fact that the VSD is usually assessed from the left ventricular aspect. Surprisingly, the orientation of the septal band is similar in ccTGA and normal heart, despite the discordant atrioventricular connections, and different in ccTGA and transposition of the great arteries, despite the discordant ventriculo-arterial connections. These findings suggest that the mechanism leading to transposition in ccTGA and in TGA probably is different. The term 'double discordance' might therefore be more appropriate as a description of this complex anomaly.

P5685Abnormal anatomy of the coronary sinus in congenitally corrected transposition of the great arteries (double discordance): a pitfall for transvenous cardiac resynchronization therapy?

Abstract Background Congenitally corrected transposition of the great arteries (ccTGA) or double discordance is a rare congenital heart defect associating discordant atrioventricular and ventriculoarterial connections. Late prognosis depends on the progressive failure of the systemic right ventricle (RV). A possible cause for systemic RV dysfunction could be ventricular asynchrony. Cardiac resynchronization therapy (CRT) may thus be indicated in some cases. However, the cardiologists may experience some problems with the coronary sinus (CS) when implanting a 3-lead CRT transvenous system in these patients. Aim of the study To evaluate the anatomy of the CS and cardiac veins in specimens with ccTGA, in order to assess the feasibility of transvenous CRT. Material and methods Among the anatomic collection of the French Centre of Reference for complex CHD, 51 heart specimens had ccTGA with 2 ventricles. There were 33 post-natal and 18 fetal hearts. Hearts were reviewed with special attention paid to the course and drainage of the CS and cardiac veins. Segmental anatomy, location of the ventricular septal defect (VSD), status of the pulmonary outflow tract and anomalies of the atrioventricular valves were reviewed. Results Segmental anatomy was {S,L,L} in 46/51 hearts, {S,L,D} in 2 and {I,D,D} in 3. There was a VSD in 40 (outlet in 25, inlet in 11, both in 2, muscular in 2), pulmonary atresia in 13, subpulmonary stenosis in 6, abnormal tricuspid valve in 20/48 including Ebstein anomaly in 6, straddling in 9 (3 had replacement). The CS was always located behind the morphologically left atrium (LA). However, its anatomy was normal, with normal drainage into the morphologically right atrium, in only 25/51 (49% of cases). The CS was of reduced length with normal orifice in 17. Orifice was atretic with normal size CS in 2, and CS was completely absent in 6 with direct drainage of coronary veins into the LA. At least 1 available vein was found in all cases with patent CS orifice. Conclusion CS in ccTGA is always located behind the morphologically LA. However, its anatomy is abnormal in half of cases. The most frequent anomalies are reduced length (33%) and absent CS or atretic orifice (15.5%). The anatomy of CS should therefore be assessed by imaging techniques (multislice CT imaging or CS venography) before considering transvenous CRT in these patients.

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5years (range 36days to 22.7years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Management of the Adult with Arterial Switch.

Dextro-transposition of the great arteries (d-TGA) is a lethal congenital heart defect in which the great arteries-the pulmonary artery and aorta-are transposed to create ventriculoarterial discordance. Corrective surgical interventions have resulted in significant improvements in morbidity and mortality for this once-fatal congenital heart defect. The initial palliative surgery for d-TGA was the atrial switch operation, which provided physiological correction. The Mustard and Senning "atrial switch" procedures, in which an atrial baffle is created to produce a discordant atrioventricular connection on the existing discordant ventriculoarterial connection, showed preliminary success for the correction of d-TGA. However, follow-up evaluations demonstrated increasing complications from the right ventricle utilized as a systemic ventricle, resulting in progressive right ventricular dysfunction. Thus, the search continued for an anatomical correction of d-TGA to return the great arteries to their normal ventricular connections. The arterial switch operation (ASO), though attempted and theorized by many, was first successfully performed by Dr. Jatene and colleagues in 1975. For ASO, the distal main pulmonary artery and the distal ascending aorta are transected and then anastomosed to their respective ventricles with relocation of the coronary arteries to the neoaorta. The ASO has replaced the atrial switch operation since the 1980s and is now the standard surgical correction for d-TGA. As more patients who have undergone ASO are living into adulthood, late complications of this procedure have become more evident. The most common late postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis. Adults who have undergone ASO in childhood will need follow-up with surveillance imaging and evaluation of new symptoms or declining function to prevent and manage late postoperative complications. This review describes the management strategies for common late complications in patients who have undergone ASO.

Abstract 16788: Anatomical Repair Can Be Facilitated by Three-Dimensional Cardiac Printing in the Patients With Double Outlet Right Ventricle

Introduction: Determination of optimal strategy between single ventricle palliation (SVP) versus left ventricular recruitment (LVR) as a systemic ventricle is challenging in the patients with complicated double outlet right ventricle (DORV). We sought that three-dimensionally printed cardiac models (3DM) may enables the surgeons to make an intuitive preoperative decision potentially in favor of LVR. Hypothesis: Use of 3DM can extend the candidates for LVR and improve surgical outcomes. Methods: We retrospectively reviewed the patients with DORV from Jan 2008 to Jun 2018 in single center. The patients with discordant atrioventricular (AV) connection, complete AV septal defect and atresia of AV valves were excluded. The patients without at least one of LVR precluding factors such as left or right ventricular hypoplasia, mitral valve (MV) or tricuspid valve (TV) straddling, TV interposition in potential baffle course, huge ventricular septal defect (VSD), multiple VSDs and remote VSD were excluded. All patients were divided in SVP or LVR group except two patients without clear determination of strategy. Finally, 100 patients were enrolled and since 2016, 3DM was made in the patients with LVR precluding anatomy for better consensus formation whether LVR could be feasible in 26 patients. Risk factor analysis was done for SVP and adverse outcome. Results: Thirty-five patients enrolled as SVP strategy (Fontan operation in 14, Kawashima operation in 1, interstage mortality in 12, follow-up loss in 3 and waiting scheduled operation in 5) and 65 patients enrolled as LVR strategy (complete anatomical correction in 50, one and half ventricle repair in 6 and waiting scheduled operation in 9). In multivariate analysis, LV hypoplasia ( P =0.003) and MV straddling ( P =0.015) were significant LVR precluding factors and the use of 3DM was a favorable factor for LVR ( P =0.039, OR=0.212). Risk factor for mortality were LV outflow tract obstruction ( P =0.006) and LV hypoplasia ( P =0.043). The use of 3DM did not reach statistical significance on mortality ( P =0.132). Conclusions: In DORV patients, LVR can be precluded especially with LV hypoplasia and MV straddling however, LVR can be facilitated by direct-vision driven preoperative decision about complicated features with 3DM.

Cardiac Conduction System in Congenitally Corrected Transposition of the Great Arteries and Its Clinical Relevance

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculoarterial connections.1 The incidence has been reported to be around 1/33 000 live births, accounting for ≈0.05% of congenital heart malformations.2 Although a familial recurrence of heart defects in subjects with ccTGA has been reported,3 the etiology of this malformation is not currently known.1, 4 The morphological right atrium is connected to the morphological left ventricle across the mitral valve, with the left ventricle then connected to the pulmonary trunk; the morphological left atrium is connected to the morphological right ventricle across the tricuspid valve, with the morphological right ventricle connected to the aorta. Because of this “double discordance,” the systemic venous return is pumped to the lungs, while the pulmonary venous return is directed to the body. In the context of usual atrial arrangement (situs solitus), the morphological left ventricle is thus positioned to the right and the aorta, arising from the morphological right ventricle, is more commonly left‐sided. In mirror‐image atrial arrangement (situs inversus), the morphological left ventricle is left‐sided, and the aorta is usually positioned to the right. Most patients have at least 1 associated cardiac anomaly, mainly ventricular septal defect (VSD), subvalvular and/or valvular pulmonary stenosis, or abnormalities of the systemic (tricuspid) atrioventricular (AV) valve with coexisting accessory pathways.4 Because of the unusual position of the atrioventricular node and course of the atrioventricular conduction bundle, cardiac conduction disorders are frequent and markedly alter the natural history and management of the malformation.5, 6, 7 This article provides a comprehensive, up‐to‐date description of the characteristics of the cardiac conduction system in patients with ccTGA, their clinical sequelae, and their contemporary management.

Single Stage Extra Cardiac Conduit Total Cavopulmonary Connection in Atrial Situs Inversus and Levocardia

10-year female child was presented with complex congenital heart defect—atrial situs inversus, levocardia, discordant atrioventricular connections, double outlet right ventricle, ventricular septal defect, pulmonic stenosis and straddling of atrio-ventricular valves along with bilateral superior vena cava and large patent ductus arteriosus. We describe here the strategy adopted to perform single stage fenestrated extra cardiac conduit total cavo-pulmonary connections in this patient especially concerning cardiac malposition of atrial situs inversus and levocardia.

Double Outlet Right Ventricle with Discordant Atrioventricular Connections in a Newborn: Case Report and Brief Overview of the Literature

DOI: 10.4328/JCAM.1282 Received: 07.09.2012 Accepted: 19.09.2012 Printed: 01.01.2016 J Clin Anal Med 2016;7(1): 105-8 Corresponding Author: Ahmet Sert, Department of Pediatric Cardiology, Konya Training and Research Hospital, 42080, Konya, Turkey. T.: +90 3323236709 F.: +90 3323236723 E-Mail: ahmetsert2@hotmail.com Ozet Cift cikisli sag ventrikul butun dogumsal kalp defektlerinin nadir bir kardiyak malformasyondur. Anormal ventrikuloarteryal baglantilarin heterojen bir grubudur. Her iki buyuk arter (pulmoner arter ve aorta) esas olarak morfolojik sag ventrikulden cikmaktadir. Diskordan atriyoventrikuler baglantili cift cikisli sag ventrikul ve hipoplazik pulmoner arterleri olan bir yenidogani sunmaktayiz. Ekokardiyografi ile tani konulan nadir karmasik bir kardiyak malformasyonu bildirdik. Ekokardiyografi ile segmental yaklasim ve kardiyak segmentlerin ardisik analizi karmasik kardiyak malformasyonlarin tanisinda ipucudur.

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome.

Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach. To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up. A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections. Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors. Discussion Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Exercises in anatomy: cardiac isomerism.

It is well recognized that the patients with the most complex cardiac malformations are those with so-called visceral heterotaxy. At present, it remains a fact that most investigators segregate these patients on the basis of their splenic anatomy, describing syndromes of so-called asplenia and polysplenia. It has also been known for quite some time, nonetheless, that the morphology of the tracheobronchial tree is usually isomeric in the setting of heterotaxy. And it has been shown that the isomerism found in terms of bronchial arrangement correlates in a better fashion with the cardiac anatomy than does the presence of multiple spleens, or the absence of any splenic tissue. In this exercise in anatomy, we use hearts from the Idriss archive of Lurie Children's Hospital in Chicago to demonstrate the isomeric features found in the hearts obtained from patients known to have had heterotaxy. We first demonstrate the normal arrangements, showing how it is the extent of the pectinate muscles in the atrial appendages relative to the atrioventricular junctions that distinguishes between morphologically right and left atrial chambers. We also show the asymmetry of the normal bronchial tree, and the relationships of the first bronchial branches to the pulmonary arteries supplying the lower lobes of the lungs. We then demonstrate that diagnosis of multiple spleens requires the finding of splenic tissue on either side of the dorsal mesogastrium. Turning to hearts obtained from patients with heterotaxy, we illustrate isomeric right and left atrial appendages. We emphasize that it is only the appendages that are universally isomeric, but point out that other features support the notion of cardiac isomerism. We then show that description also requires a full account of veno-atrial connections, since these can seemingly be mirror-imaged when the arrangement within the heart is one of isomerism of the atrial appendages. We show how failure to recognize the presence of such isomeric appendages can lead to spurious diagnoses of discordant atrioventricular connections. Overall, we show that the finding of isomeric atrial appendages, guided by the finding of bronchial isomerism, is but the prelude to full segmental sequential analysis.