A case of congenitally corrected transposition of the great arteries with rare but life-threatening ventricular tachycardia and a coincidental single coronary ostium

Congenitally corrected transposition of the great vessels (CCTGA) is a rare congenital heart defect. Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and congestive heart failure (CHF) being relatively common in older adults. The most common presenting feature is bradycardia due to high-degree atrioventricular (AV) blocks. Patients with CCTGA are increasingly subject to CHF with advancing age; this complication is extremely common by the fourth decade. The majority of patients have an inverted coronary arterial pattern.1 We report a case of a patient with CCTGA who presented with rare but life-threatening ventricular tachycardia (VT) leading to syncope, with preserved systemic ventricular function. Coincidentally, the patient also had a single coronary ostium.     

Congenitally corrected transposition of the great arteries treated by partial systemic ventriculectomy.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular (AV) and ventriculo-arterial discordance;(1) that is, the left ventricle supports the pulmonary circulation and the right ventricle supports the systemic circulation. The most common cardiac anomalies in CCTGA include ventricular septal defect, pulmonary outflow tract obstruction and abnormalities of the systemic AV valve. (1) The dysfunction of the systemic ventricle occurs with increasing frequency in older patients with CCTGA, independent of their commonly associated structural defects.(2-4) We report a patient with CCTGA undergone the partial ventriculectomy for his systemic ventricular dysfunction.     

Atrioventricular discordance. Clinico-surgical experience 1990-2000

OBJECTIVE: To know the incidence of ventriculoarterial connections combined with atrioventricular discordance, associated lesions and surgical results, including the first case with anatomical correction. METHOD: All patients with atrioventricular discordance by echocardiography from 1990 to March 2000 were analyzed. RESULTS: Thirty six patients with atrioventricular discordance were found. Ages ranged from 0.1 to 46 years, with a mean 9.2 years (SD 5.9 years). Atrial chambers were situs solitus in 88.9%, inversus in 11.2%. The ventriculoarterial connections were discordant in 28 (77.7%), double outlet right ventricle in 4 (11.1%) (one of them was a "criss cross" heart), single outlet (pulmonary atresia) in 4 (11.1%), and double outlet left ventricle in 1 (2.7%). Associated lesions: Ventricular septal defect with pulmonary stenosis or atresia was present in 21 (58%), ventricular septal defect with no pulmonary obstruction was observed in 10 (28%). Five had tricuspid regurgitation with right ventricular disfunction, (two adults). Surgical results: 22 (61%) required 28 surgical procedures: 8 (36%) were palliative and 19 (86%) were corrective, one of them was our first anatomical correction. Operative mortality in all was 40.1%, postoperative atrioventricular block was observed in 9 (40.1%). CONCLUSION: Right ventricular dysfunction is not uncommon. Surgical results revealed high mortality and high pacemaker requirement for atrioventricular block.     

Transposition of the great arteries

Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.     

Congenitally Corrected Transposition of the Great Arteries and Concomitant Coronary Artery and Valvular Disease in the Adult Patient

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.     

Congenitally corrected transposition of the great arteries: An update

Opinion statement  Congenitally corrected transposition of the great arteries is a rare condition in which systemic venous blood returns to normally positioned atria. However, the atria are connected to the opposite ventricle, right atrium to left ventricle, left atrium to right ventricle; so-called atrioventricular disordance. In addition, the ventricles are inverted (right to left change in position) and are connected to the opposite great artery, left ventricle to pulmonary artery, right ventricle to aorta; thus, forming ventricular-arterial discordance. The aorta is anterior and to the left of the pulmonary artery, L-transposed. Atrioventricular discordance plus ventricular-arterial discordance results in normal blood flow (ie, congenitally corrected). The right ventricle with the tricuspid valve is the systemic ventricle. Common associated conditions are ventricular septal defects (VSDs), pulmonary stenosis, and congenital heart block. Major issues related to management revolve around the status of the systemic right ventricle, which can develop dysfunction with increasing age and tricuspid regurgitation, which can increase in severity with age and contribute to ventricular dysfunction. One emerging treatment is the double switch operation. In patients with no pulmonary obstruction, it is possible to switch the systemic and pulmonary venous return using an atrial baffle procedure followed by an arterial switch procedure. This results in the anatomical left ventricle now functioning as the systemic ventricle. In those patients with associated pulmonary obstruction and a VSD, another type of double switch can be performed in which the left ventricle is tunneled through the VSD to the aorta, the right ventricle is connected to the pulmonary artery with a homograft or other conduit, and the atrial baffle procedure is performed. The most difficult challenge is choosing the patient who is a candidate for the double-switch operation and the timing of that operation, or the timing of a more classical operation for associated defects.     

Congenitally corrected transposition of the great arteries: Current treatment options

Opinion statement Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.     

Heart with criss-cross ventricles associated with an obstruction of the ejection pathway of the left ventricle and a mitral anomaly

A case of criss-cross heart with situs inversus associated with subvalvular aortic stenosis and mitral regurgitation in a 9 year old boy is reported. Surgical correction of the valvular abnormalities gave an excellent clinical and haemodynamic result. A review of the literature shows that this abnormality of rotation of the ventricles coexists with a concordant atrioventricular connection in 70 % cases; the ventriculoarterial connection is only discordant in 12 % cases. The right ventricle is always situated above the left ventricle. The most common associated lesions are usually accessible to surgery; ventricular septal defect (98 % cases); stenosis or atresia of the pulmonary artery (70% cases). However, right ventricular hypoplasia is also common (65 % cases). Six patients underwent surgical correction of the associated lesions with 4 successes and 2 deaths. Surgery is possible in these complex cardiac malformations with good results providing a precise preoperative anatomical diagnosis is made.     

"Straddling" tricuspid valve across an isolated ventricular septal defect. Apropos of 4 cases

Four cases of children from 10 months to 3 years of age with isolated ventricular septal defects and straddling tricuspid valve are reported. Three patients underwent surgery (mean age: 24 months). The lesion was diagnosed during operation in 2 cases. The VSD was a perimembranous defect with extension to the ventricular inlet in 2 cases, and in the other it was entirely muscular. The straddling involved all the septal leaflet of the tricuspid valve in 1 case. In two cases, there was doubling of the segment with one part crossing the VSD. The left ventricular attachment was a type B in 2 patients and type C in 1 patient. The tricuspid ring was normally situated in all patients (no overriding). In 2 patients, the diagnosis was made with the aid of complementary investigations, and, in particular, 2D echocardiography. One of these patients has not yet undergone surgery as the lesion is well tolerated and because of associated mitral regurgitation. In 3 patients, surgery consisted of repairing the ventricular septal defect, leaving the abnormal part of the tricuspid valve in the left ventricle. All patients survived; there were no cases of post-operative atrioventricular block or signs of tricuspid incompetence. With reference to these three cases, the authors review the main anatomical lesions, the diagnostic signs and different methods of treatment of this condition. Straddling tricuspid valve may present as a simple ventricular septal defect. This diagnosis should be considered in all cases of posterior ventricular septal defects.     

The feasibility of complete anatomical correction in the setting of discordant atrioventricular connections

OBJECTIVE: To evaluate the feasibility of anatomical correction based on morphological and echocardiographic findings in patients and preserved hearts with discordant atrioventricular connections. DESIGN: A retrospective study with clinicomorphological correlations to assess potential contraindications for anatomical correction in the setting of discordant atrioventricular connections. SETTING: A tertiary referral centre for congenital heart disease. MATERIAL: 25 specimens and 53 patients unified by presence of discordant atrioventricular connections. METHODS: The potential contraindications for anatomical correction were first evaluated on the basis of morphological findings in all 25 specimens with discordant atrioventricular connections collected in the department museum, including study of the major coronary arterial patterns in 20. These contraindications were then sought in a population of 53 patients examined echocardiographically between January 1992 and October 1997. RESULTS: At least one lesion was discovered that might have contraindicated anatomical correction in 14 of the specimens and in 16 of the patients. The most common lesions that might militate against the anatomical approach were severe Ebstein's malformation or straddling and overriding of the tricuspid valve, each when combined with hypoplasia of the morphologically right ventricle. Other potential contraindications were atrioventricular septal defect with common atrioventricular junction, and obstruction of the left ventricular outlet combined with a restrictive ventricular septal defect, although these may be overcome with increasing experience and expertise. CONCLUSIONS: According to the morphological and echocardiographic findings, at least 10 hearts and 37 patients would have produced no anatomical problems for the type of surgical correction in which the morphologically left ventricle is restored its rightful role as the systemic pumping chamber.     

Double discordance with ventricular septal defect and pulmonary artery hypertension. A study of 21 cases

This paper reports a retrospective study of 21 children with atrioventricular and ventriculo-arterial discordance, or double discordance, associated with a large ventricular septal defect responsible for pulmonary hypertension. Other associated congenital defects were: atrioventricular block (5 cases), coarctation of the aorta with neonatal cardiac failure (6 cases), tricuspid valve malformations responsible for significant tricuspid regurgitation (11 cases) and right ventricular hypoplasia (1 case). Two children died before any therapeutic intervention, one from syncope related to atrioventricular block and the other after a decision of therapeutic abstention. Three children underwent total correction with one good result (the only case of situs inversus), one late death and one lost to follow-up. The majority of patients (n = 16) underwent initial palliative surgery consisting in pulmonary artery banding occasionally associated with reconstruction of the aortic arch: there was no early mortality but there were 2 late deaths. Of the 14 survivors, 6 are well after a mean follow-up period of 31 months. Eight underwent open heart surgery with 1 operative death, 6 post-operative complete atrioventricular blocks requiring cardiac pacing and 5 poor results due to aggravation or secondary tricuspid regurgitation leading to 1 cardiac transplantation (death) and 2 reoperations for valvular surgery (1 plasty and 1 tricuspid valve replacement). The overall results of this series are poor: high mortality (33 per cent) and equally high morbidity when direct surgery is undertaken. Two major complications are observed: complete atrioventricular block (55 per cent) and regurgitation of the systemic atrioventricular valve (45 per cent), both of which often necessitate invalidating complementary procedures.(ABSTRACT TRUNCATED AT 250 WORDS)     

Radionuclide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries

Eight asymptomatic patients (mean age 19 years, range 7 to 32) with congenitally corrected transposition of the great arteries (CCTGA) underwent equilibrium gated radionuclide angiocardiography at rest and during supine bicycle exercise to assess systemic (morphologic right) and pulmonary (morphologic left) ventricular function. Five patients had normal intracardiac hemodynamic values, 2 had trivial atrioventricular valve regurgitation and 1 patient had trivial pulmonary ventricular outflow tract obstruction. Average exercise duration was 11 +/- 1 minute, with limitation due only to fatigue. At peak exercise, heart rate increased 225% and systolic blood pressure 152% over the rest value. Pulmonary ventricular ejection fraction at rest was 51 +/- 3% (mean +/- standard error of the mean); it did not change significantly at peak stress, 53 +/- 2%. Systemic ventricular ejection fraction was 48 +/- 4% at rest and increased to 64 +/- 4% at peak exercise (p less than 0.01). Count-based volume changes for the pulmonary chamber showed no significant change in end-diastolic or systolic counts at peak exercise (109 +/- 8% and 106 +/- 9% of rest value, respectively). However, end-diastolic counts decreased 13% (87 +/- 3% of rest value) and end-systolic counts 34% (62 +/- 7% of rest value) at peak exercise in the systemic ventricle. These data suggest normal systemic and impaired pulmonary ventricular function in patients with congenitally corrected transposition of the great arteries unaccompanied by significant associated lesions. These findings have important clinical implications in the setting of complex congenital heart disease in patients in whom a morphologic right ventricle functions as the systemic pumping chamber. Despite the pulmonary ventricular dysfunction, symptoms were not apparent at rest or during exercise.     

Cardiac involvement in childhood polyarteritis nodosa

In this report, we evaluated the cardiac findings of 15 children with polyarteritis nodosa. The age range of the patients was 4–14 years; with a mean of 10 years. All have had systemic involvement of the disease. The most common findings in cardiac evaluation were diminished left ventricular systolic functions and mild mitral and/or tricuspid valve regurgitation. One patient had pericardial thickening with no effusion. One had sinus tachycardia. There were no signs of myocardial infarction or ischemia clinically or electrocardiographically. In conclusion, we did not find cardiac complications, such as pericarditis or myocardial infarction, to be as frequent as in previous reports. However, even in asymptomatic patients, systolic dysfunction or valvular involvement were common findings in patients with polyarteritis nodosa, which were not reported previously. These findings may be due to the histological changes of the myocardium or atrioventricular valves. Although these were not severe and fatal lesions, long-term follow-up of these patients with echocardiography may help to determine the course of cardiac involvement.     

Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist

Objectives—To establish the prevalence of tricuspid valve abnormalities in children with a double discordant heart (or congenitally corrected transposition of the great arteries); to study the influence of the loading conditions induced by various surgical interventions on the right and left ventricle in patients with double discordance and an abnormal tricuspid valve; and to propose a rational surgical approach.
Methods—Case notes were reviewed of 141 consecutive patients admitted in the first year of life with various types of double discordance (intact ventricular septum (group 1), ventricular septal defect (group 2), ventricular septal defect and pulmonary obstruction (group 3)). A study group of 62 patients with an abnormal tricuspid valve was selected by cross sectional echocardiography. These were followed up through palliative and open heart procedures with grading of tricuspid regurgitation.
Results—Tricuspid valve abnormalities were more common in groups 1 and 2 (60% and 56%) than in group 3 (31%). Preoperative tricuspid regurgitation was more common in group 2 (90%) than in groups 1 and 3 (38% and 36%). Ten patients in groups 1 and 2 died in the neonatal period with severe tricuspid regurgitation, associated with coarctation of the aorta in 60%. Eight patients in group 1 had no surgery and are doing well, with a competent tricuspid valve. Palliative procedures were undertaken in 28 patients: 14 had pulmonary artery banding, which resulted in a decrease in tricuspid regurgitation, 12 in group 2 by reducing the pulmonary blood flow and two in group 1 by changing the septal geometry; 14 in group 3 had an aortopulmonary shunt, which induced tricuspid regurgitation in two. Twenty patients are still alive after palliation, with stable tricuspid valve function. Repair of the tricuspid valve was unsuccessful in the three patients who underwent conventional surgery, leaving the right ventricle facing the systemic circulation. In two patients with a competent but abnormal tricuspid valve, conventional surgery induced severe tricuspid regurgitation. Of the 15 patients who underwent conventional surgery, only 10 survived (mortality 33%): eight with a tricuspid valve prosthesis and two with severe residual tricuspid regurgitation. However, tricuspid regurgitation decreased after anatomical correction (nine patients), restoring a systemic left ventricle and a subpulmonary right ventricle, even when the tricuspid valve was not repaired (five patients). Eight patients are doing well after anatomical correction (mortality 11%).
Conclusions—Tricuspid valve function in double discordance with an abnormal tricuspid valve depends on the loading conditions of both ventricles and on the septal geometry. Interventions that increase right ventricular volume or decrease left ventricular pressure are likely to induce tricuspid regurgitation, while those that decrease right ventricular volume or increase left ventricular pressure are likely to improve tricuspid valve function. Repair of the tricuspid valve always failed when the right ventricle was left in a systemic position and always succeeded when the right ventricle was placed in a subpulmonary position. These results should be taken in to account when dealing with patients with double discordance and an abnormal tricuspid valve.

Keywords: transposition of the great arteries;  double discordance;  double switch procedures;  tricuspid valve;  paediatric cardiology;  congenitally corrected transposition     

Is systemic right ventricular function by cardiac MRI related to the degree of tricuspid regurgitation in congenitally corrected transposition of the great arteries?

Background and Methods

Systemic right ventricular dysfunction and tricuspid regurgitation (TR) are frequently encountered in patients with congenitally corrected transposition of the great arteries (CCTGA). Studies using echocardiography have suggested a relationship between the degree of TR and systemic right ventricular dysfunction; however, assessment of systemic right ventricular function by echocardiography is limited. Cardiac MRI (CMR) is the gold standard for volumetric assessment of the systemic right ventricle. We performed a retrospective cohort study at our center evaluating all adult patients with CCTGA who underwent a CMR between 1/1999 and 1/2013 to determine the relationship between the degree of TR and systemic right ventricular function.

Results

Of the 33 patients identified, 12 had ≤ mild TR (37%), 13 had moderate TR (40%), and 8 had severe TR (24%). Mean age at CMR was 38 years (23–64). Mean right ventricular ejection fraction (45% vs. 41% vs. 42%, p = 0.68) and mean indexed right ventricular end diastolic volume (122 ml/m² vs. 136 ml/m² vs. 138 ml/m²p = 0.36) were not significantly different for patients with ≤ mild TR, moderate TR or severe TR. The degree of TR was not associated with additional congenital lesions, prior procedures, presence of an intraventricular conduction delay, or decreased left ventricular function.

Conclusion

No association between the degree of TR and right ventricular volume or ejection fraction by CMR was identified. Failure to show worsening function or increased volume with greater degrees of TR suggests that the degree of regurgitation alone may not fully explain the heterogeneity in right ventricular size and function.     

Complete transposition of the great arteries: surgical concepts for patients with systemic right ventricular failure following intraatrial repair

One of the most serious late complications of the intraatrial baffle procedure (Mustard or Senning correction) in patients suffering from transposition of the great arteries, (TGA) is the late systemic right ventricular failure. Nearly all patients presenting with right ventricular dysfunction have severe associated tricuspid regurgitation. The surgical options for these patients include tricuspid valve reconstruction or replacement, staged conversion to the arterial switch operation and orthotopic heart transplantation. Review of 189 operative survivors who underwent the Mustard or Senning operation for TGA between 1970 and 1993 in our institution revealed 12 patients (6.3%) who died from severe systemic right ventricular dysfunction (mean follow-up 16+/-3.5 years), which was the most common cause of late death. All of them had concomitant severe tricuspid regurgitation. 7 patients (3.7%) died from sudden cardiac death. The actuarial survival at 10 years is 93% for simple TGA and 85% for TGA associated with ventricular septum defect or coarctation. At our institution, 4 adolescent or adult patients underwent tricuspid valve surgery; tricuspid valve replacement was performed in 2 patients and valve repair in 2 patients. In the mid-term follow-up, 2 of these patients died. Two additional patients underwent orthotopic heart transplantation, and one died on the waiting list. Staged conversion from the Senning/Mustard atrial repair to the arterial switch operation was initially reported by Mee. The procedure for pulmonary artery banding starts with inducing left ventricular reconditioning with subsequent arterial switch. The mortality of this two-staged procedure was as high as 20% to 30% in our early experience, and some of the candidates underwent heart transplantation. Tricuspid valve repair or replacement do not improve right ventricular function in patients with a failing right ventricle following the Mustard/Senning operation. Staged conversion to arterial switch may improve right ventricular function by decreasing the work load of the right ventricle and provides anatomic repair with left ventricle-to-aorta continuity. Orthotopic heart transplantation is the only alternative if the left ventricle does not respond to pulmonary artery banding.     

Ventricular Tachycardia

Congenitally Corrected Transposition and VT. Ventricular tachycardia (VT) is an uncommon finding in patients with congenitally corrected transposition of the great arteries (CCTGA). Cardiac death in patients with CCTGA has been attributed to complete heart block, systemic ventricular dysfunction, or severe AV valve regurgitation with heart failure. We descrihe the case of a patient who presented with palpitations and near-syncope that was associated with clinical episodes of VT. Programmed ventricular stimulation revealed easily inducible sustained VT that immediately degenerated to ventricular fibrillation and subsequently required therapy with an implantable cardioverter defibrillator.     

107例矫正型大动脉转位手术治疗中期结果分析

目的:报告并分析我院107例手术治疗矫正型大动脉转位(CCTGA)合并心内畸形患者的中期随访结果.方法:1996-01至2005-12,在我院手术治疗CCTGA合并心内畸形患者107例,其中男72例,女35例;年龄1.5～50岁,平均(11.5±8.4)岁;平均体重(31.6±4.7)kg.81例左位心,23例右位心,3例中位心.手术方式包括传统双心室功能矫治75例;单心室分期矫治14例;双心室解剖矫治18例.结果:术后早期死亡5例,死亡率为4.7%.传统双心室功能矫治死亡率为4.0%;单心室分期矫治死亡率为0%;心房动脉双调转手术死亡率为0%;心房心室双调转手术死亡率40.0%.手术死亡危险因素为复杂心内畸形合并肺动脉瓣狭窄(P<0.01),与其他危险因素相比差异有统计学意义.共随访99例,随访率为92.5%.随访期间死亡12例,死亡率为12.1%;其中10例(83.3%)属于传统双心室功能矫治患者,2例(16.7%)属于单心室矫治患者.双心室解剖矫治患者没有死亡.随访期死亡危险因素为传统功能矫治手术方式(P<0.05),心房心室双调转手术(P<0.05),与其他手术方式相比差异有统计学意义.结论:CCTGA传统手术方法的中期结果是令人失望的.对于适合单心室矫治的CCTGA病例,手术可以取得较好的中期结果.心房动脉双调转手术中期的死亡率低,并发症少,其手术结果是值得肯定的.     

Double outlet right ventricle with discordant atrioventricular connection. Clinical study

The clinical and anatomic findings were reviewed in 17 patients with double-outlet right ventricle and atrioventricular discordance. Ten cases had atrial situs solitus, seven with right-sided heart three with left-sided heart. Seven cases had atrial situs inversus, five with left-sided heart and two with right-sided heart. All cases presented ventricular septal defect, 13 subvalvar pulmonary stenosis, two tricuspid regurgitation and two complete atrioventricular block. The spatial relationship between the arterial valves are variable. Most cases in atrial situs solitus had a left-sided and anterior aorta and all patients in atrial situs inversus had a right-sided and anterior aorta. In this study we compared the anomalies found in our cases with double outlet right ventricle with those in 58 patients with corrected transposition. Absolute, relative and attributable risks were calculated for the presence of subvalvular pulmonary stenosis, ventricular septal defect, tricuspid regurgitation and atrioventricular block for each the two groups. We concluded that patients with double-outlet right ventricle are more prone to present ventricular septal defect and subvalvar pulmonary stenosis, while those with corrected transposition have a greater likelihood of presenting with tricuspid regurgitation and atrioventricular block. There is no typical clinical picture for the malformations. Symptoms depend upon the associated anomalies. The final diagnosis is best achieved by the echocardiographic and angiocardiographic studies, but electrocardiogram and chest radiograph may suggest the presence of a discordant atrioventricular connection.     

From tricuspid to double orifice Morphology: Percutaneous tricuspid regurgitation repair with the MitraClip device in congenitally corrected‐transposition of great arteries

Edge to edge transcatheter mitral valve repair with MitraClip (Abbott Vascular, Menlo Park, CA) is increasing for high‐risk surgical patients with significant mitral regurgitation. Patients with congenitally corrected transposition of the great arteries (CCTGA) presenting with tricuspid valve regurgitation of a systemic right ventricle may represent particularly challenging candidates for MitraClip given their anatomy. We report the case of a 67‐year‐old gentleman with CCTGA and severe tricuspid regurgitation who was referred for MitraClip implantation after heart team consensus. Successful implantation of one clip was performed, achieving a significant reduction of the regurgitation. Similarly, favorable findings were confirmed at 6 months, 1 and 2 years follow‐up and the patient had no recurrent heart failure admissions after 2‐year follow‐up. We describe the technical considerations and the importance of 3D‐transoesophageal echocardiography for performing the MitraClip of a trileaflet systemic atrioventricular valve. © 2016 Wiley Periodicals, Inc.