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We report a case of CC-TGA with left AV mechanical prosthetic valve with systemic ventricular dysfunction who underwent successful transvenous permanent dual chamber pacing using coronary sinus.

OBJECTIVES In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries. METHODS Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Fragmented QRS was defined as ≥1 discontinuous deflections in narrow QRS complexes, and ≥2 in wide QRS complexes, in 2 contiguous electrocardiogram leads. The primary end point was a composite of all-cause mortality and systemic ventricular dysfunction. RESULTS A total of 74 patients were included. Among them, 30, 15 and 29 underwent the Senning arterial switch, the Senning Rastelli and the hemi-Mustard/bidirectional Glenn/Rastelli procedures, respectively. The primary end point occurred in 9 (12.2%) patients and included 7 late deaths and 2 cases of late-onset systemic ventricular dysfunction. Fragmented QRS and QRS prolongation were noted in 19 (25.7%) and 21 (28.4%) patients, respectively. In patients with the primary end point, QRS fragmentation (6/9 vs 10/65; P CONCLUSIONS Appearance of QRS fragmentation or QRS prolongation is associated with death or ventricular dysfunction in anatomically repaired corrected transposition of the great arteries. Although there is a trend that QRS fragmentation and QRS prolongation appear more frequently in patients who had the Senning-arterial switch operation, there is no statistically significant difference associated with these electrocardiogram features among varied procedures.

Right ventricular (RV) function is important for clinical status and outcomes in children and adults with congenital heart disease (CHD). In the normal RV, longitudinal systolic function is the major contributor to global RV systolic function. A variety of factors contribute to RV failure including increased pressure- or volume-loading, electromechanical dyssynchrony, increased myocardial fibrosis, abnormal coronary perfusion, restricted filling capacity and adverse interactions between left ventricle (LV) and RV. We discuss the different imaging techniques both at rest and during exercise to define and detect RV failure. We identify the most important biomarkers for risk stratification in RV dysfunction, including abnormal NYHA class, decreased exercise capacity, low blood pressure, and increased levels of NTproBNP, troponin T, galectin-3 and growth differentiation factor 15. In adults with CHD (ACHD), fragmented QRS is independently associated with heart failure (HF) symptoms and impaired ventricular function. Furthermore, we discuss the different HF therapies in CHD but given the broad clinical spectrum of CHD, it is important to treat RV failure in a disease-specific manner and based on the specific alterations in hemodynamics. Here, we discuss how to detect and treat RV dysfunction in CHD in order to prevent or postpone RV failure. ispartof: CARDIOVASCULAR DIAGNOSIS AND THERAPY vol:10 issue:5 pages:1625-1645 ispartof: location:China status: published

Congenitally corrected transposition of the great arteries is a rare congenital anomaly that can remain asymptomatic until adulthood, especially when there are no other associated congenital anomalies. We report two patients in their sixth decade of life with corrected transposition of the great arteries incidentally diagnosed by transthoracic echocardiography in a preventive medical check-up. The complementary use of cardiac computed tomography confirmed the diagnosis.

Background The outcomes of anatomical repair for patients with congenitally corrected transposed great arteries remain unclear and the indications for different procedures are poorly understood. Methods From January 2005 to February 2016, consecutive corrected transposition patients who underwent anatomical repair at the current institution were enrolled in this retrospective study. Varied types of anatomical repair were individually customised. Results A total of 85 patients were included. Fifty-one (51) and 35 patients presented with left ventricular outflow tract obstruction and cardiac malposition, respectively. Thirty-nine (39) patients presented with moderate-to-severe tricuspid regurgitation. Thirty-four (34), 19, and 32 patients underwent Senning arterial switch operations, Senning-Rastelli, and hemi-Mustard-Rastelli-bidirectional Glenn, respectively. Early after repair, there were five in-hospital deaths and nine re-operations. During 4.6 years (range, 0.5–10.3) of follow-up, seven late deaths were documented. Estimated overall survival rate after anatomical repair was 89.3%, 85.0%, and 85.0% at 1 year, 3 years, and 5 years, respectively. Instead of Senning-Rastelli, most (75.0%) early left ventricular dysfunctions were noted in patients who underwent Senning arterial switch procedures. However, all the late left ventricular dysfunctions were found in patients who underwent previous left ventricular retraining. In patients with left ventricular outflow tract obstruction, the hemi-Mustard-Rastelli-bidirectional Glenn shunt provided a lower early mortality (0% vs 15.8%, p = 0.047). Conclusions Favourable outcomes can be achieved for anatomical repair of corrected transposition. Left ventricular dysfunction was a significant postoperative issue. Hemi-Mustard-bidirectional Glenn-Rastelli procedure may provide benefits for patients with associated left ventricular outflow tract obstruction and cardiac malposition. Each procedure has its own advantages in varied anatomy.

A 51-year-old man, who had a history of open heart surgery for corrected transposition of great arteries, presented with palpitation due to atrial tachycardia. A propagation map using three-dimensional electroanatomical mapping (CARTO3) showed atrial flutter and underwent linear ablation successfully. This case highlights the difficulty of diagnosis before mapping following a complicated cardiac operation and the usefulness of three-dimensional mapping.

Congenitally corrected transposition of great arteries (CCTGA) is associated with increased risk for complete heart block. The morphologic right ventricle is at increased risk for pacing-induced cardiomyopathy and heart failure. In this report, we present a case of successful His bundle pacing (HBP) guided by 3-dimensional (3D) mapping in a patient with CGTGA.

Introduction: Physiologically corrected transposition of the great arteries [(S,L,L) TGA] is a rare congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Case Presentation: A 28-year-old man with (S,L,L) TGA, pulmonary valve (PV) stenosis, ventricular septal defect (VSD), and history of Kawasaki disease who presented for a gradual decline in exercise tolerance. Oxygen saturation was 96% at rest and dropped to 86% with ambulation. Hemoglobin was 17.7 g/dl, EKG showed no bradyarrhythmias. On stress test, peak heart rate was 159 beats/minute, and peak VO 2 22.6 ml/kg/min (83%, and 57% of predicted value respectively). Spirometry was normal. Transthoracic echocardiogram revealed biventricular hypertrophy with normal systolic function, a bicuspid PV with severe stenosis (peak and mean gradient of 155 and 90 mmHg respectively), large inlet VSD with bidirectional flow and mild systemic TV regurgitation. Coronary CTA showed no coronary artery aneurysms. Cardiac catheterization suggested hypoxia due to pulmonary-to-systemic shunting across the VSD with Q p /Q s = 0.86 (Figure). Consideration should be given to performing a high-risk surgical repair versus medical management. Conclusions: Natural history, symptoms, and timing of intervention are determined by the associated cardiac anomalies and the progressive dysfunction of the systemic right ventricle (SRV). Anatomic repair to restore the left ventricle as a systemic pump is a very high-risk procedure in adults. Functional repair maintains SRV, repair the VSD, and PV stenosis has a poor late outcome. Medical management ensures iron stores are repleted, provide empirical use of pharmacological heart failure therapy, and referral to heart transplantation when SRV systolic dysfunction ensues. Clinicians need to know the potential complications in both unoperated patients and following various surgical repairs to recommend appropriate treatment options.

Purpose To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up. Methods Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively. Conclusions ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life.

Right ventricular (RV) geometry is altered by septal shift after pulmonary artery banding. This may reduce tricuspid regurgitation (TR) and improve ventricular function in patients with corrected transposition of great arteries and systemic right ventricle. However, banding is risky in sick patients with severe RV failure. There are no predictive models in clinical practice to test this septal shift hypothesis before a risky surgery. A transcatheter model to mimic a pulmonary artery band is presented in corrected transposition of great arteries with failing right ventricle and severe TR.

The discordance between increased physiological demand during pregnancy and congenital cardiac pathology of a parturient is a perilous threat to the maternal-fetal well-being. Early involvement of a multidisciplinary team is essential in improving peripartum morbidity and mortality. Designing the most appropriate anesthetic care will require a concerted effort, with inputs from the obstetricians, obstetric and cardiac anesthesiologists, cardiologists, neonatologists, and cardiothoracic surgeons. We report the multidisciplinary peripartum care and anesthetic management for cesarean section (CS) of a 28-year-old primigravida who has partially corrected transposition of the great arteries, atrial and ventricular septal defect, dextrocardia, right ventricle hypoplasia, and tricuspid atresia.

Patient YB, 50 years old, female, with hypothyroidism, severe left ventricular dysfunction, uncompressed left ventricle and poorly tolerated ventricular tachycardia, submitted to implantation of a dual chamber implantable cardioverter-defibrillator (ICD) in February 2012 (generator Secura DR Medtronic, 4076 Medtronic atrial electrode, and Sprint Quattro 6947 Medtronic ventricular electrode). Returns asymptomatic nine months after the implant for routine evaluation.

Congenitally corrected transposition of the great arteries is a rare heart defect that can be associated with systemic ventricular dysfunction and conduction disturbances. The use of cardiac resynchronization therapy in patients with congenital heart disease is not fully established, and achievement of successful pregnancies after implantation of transvenous, biventricular system has never been described, and which resulted in a significant clinical improvement. We describe a 33-year-old female with congenitally corrected transposition of the great arteries, who achieved six pregnancies and successful vaginal deliveries. The two last pregnancies were achieved after cardiac resynchronization therapy for systemic ventricular dysfunction and complete heart block. A congenital cardiac disease has been identified in only one offspring.

Situs inversus with levocardia and congenitally corrected transposition of the great arteries represents a relatively very rare congenital condition and most patients are diagnosed in infancy or early age. This case report describes a 35-year old man with congenitally corrected transposition of the great arteries which presented with a five month history of exertional dyspnea. A diagnosis was confirmed by transesophageal echocardiogram, showing situs inversus, levocardia, atrioventricular and ventriculoarterial discordance. He underwent physiologic repair, and was discharged thirty five days after the operation, in a good general condition. Although management of the corrected transposition of the great arteries patients remains controversial, the recommendation is that physiologic repair may be the procedure of choice for some patients, particularly complicated cases.

Background A subset of patients with corrected transposition of the great arteries (CC-TGA) will require left ventricular (LV) retraining before undergoing a double-switch procedure. LV retraining results in an immediate increase in LV pressure but not in LV mass. The purpose of this study was to evaluate the relationship between LV pressure and mass during LV retraining. Methods This was a retrospective review of 36 patients with CC-TGA who were enrolled in LV retraining. The median age at enrollment was 12 months. The majority (82%) had an Ebstenoid tricuspid valve or moderate to severe tricuspid valve regurgitation before pulmonary artery banding. Results Twenty-seven of the 36 patients underwent LV retraining and a double switch, including 14 patients with a single pulmonary artery band (PAB), 12 patients with 2 PABs, and 1 patient with 3 PABs. There was no early or late mortality associated with these procedures. Ninety percent of the patients demonstrated a linear relationship between LV pressure and mass; however, 3 patients (10%) demonstrated a different LV retraining pattern characterized by excess LV mass compared to LV pressure. Two of these 3 patients are LV retraining failures, whereas the third patient did undergo a double switch. Conclusions The data show that the majority of patients enrolled in LV retraining have a predictable relationship between LV mass and pressure. A minority demonstrate an excess accrual of mass with respect to pressure load. These results suggest there may be an important distinction between normal and pathological hypertrophy during the LV retraining process.

This chapter presents data drawn from a number of interesting and informative cases focusing on patients with unusual congenital heart defects and/or intracardiac shunts.

Corrected transposition of the great arteries is a rare pathology with an incidence of 1% among the congenital heart diseases.It is thought to have multifactorial inheritance. It is mostly seen in men. If there is no concomitant pathology, the blood flow isphysiological and does not show symptoms until advanced ages. Life expectancy is close to normal in those with isolated pathology.Risk factors determining mortality include progressive right ventricular dysfunction, AV block, and severe tricuspid insufficiency. Inthis case, we present a CCTGA patient with dyspnea symptoms without any additional pathology.

Heart disease continues to be the leading cause of nonobstetric maternal morbidity and mortality. Early diagnosis and appropriate care can lead to prevention of complications and improvement of pregnancy outcome. This paper continues the review and provides recommendations for the approach to high-risk cardiovascular conditions during gestation. (C) 2016 by the American College of Cardiology Foundation.

Background Anatomic repair has become the preferred option in the subgroup of patients with congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary obstruction. We report our 14-year experience with this approach. Methods From April 2001 to February 2014, 22 patients with congenitally corrected transposition with ventricular septal defect and pulmonary obstruction underwent anatomic repair. Nineteen patients had a modified Senning-Rastelli procedure, 2 had a Mustard-Rastelli procedure, and one had a hemi-Mustard-Glenn-Rastelli procedure. The mean age was 10.9 years, and 8 (36.4%) patients were male. Results There were 2 early deaths from sepsis and ventricular failure at 18 and 81 days postoperatively, and 3 late deaths from ventricular failure at 4, 33, and 113 months postoperatively. Left ventricular failure with mitral valve regurgitation was present in 3 of the 5 patients who died. Among the survivors, 3 underwent 4 transcatheter interventions for right ventricular outflow tract obstruction and 3 underwent 4 reoperations for atrial pathway obstruction, left and right ventricular outflow tract obstruction, or residual shunt. At a median follow-up of 64 months (range 14–167 months), 15 of 17 survivors were in functional class I. One patient had severe mitral valve regurgitation and was awaiting valve replacement. Another patient had right ventricular outflow conduit obstruction and was scheduled for reoperation. Conclusions Results of atrial switch-Rastelli procedures in this subgroup of patients with corrected transposition are satisfactory but still imperfect. Mitral regurgitation might predict a poor outcome. Long-term follow-up is necessary.

Corrected transposition of the great arteries is a congenital heart defect that is rarely encountered in medical practice. The problem of diagnosing this abnormality of the cardiovascular system is due to the absence of hemodynamic disorders and clinical manifestations for a long time. The paper describes a clinical case of a complex heart defect occurring virtually asymptomatically. Echocardiography that allows timely diagnosis and adequate treatment plays a crucial role.

The hallmark of corrected transposition is discordance at atrio-ventricular and ventriculo-arterial level and that is defined as “double discordance”. This can occur as an isolated anomaly but more commonly has associated defects; most common being ventricular septal defect followed by tricuspid valve abnormalities. Other associated defects are pulmonary stenosis, systemic and pulmonary venous anomalies, univentricular physiology, ventricular dysfunction (morphological right ventricle facing systemic circulation) and association of conduction abnormalities. Echocardiography plays a pivotal role in defining the anatomy and planning the management. In this article we are discussing about role of echocardiography in evaluation of corrected transposition, diagnosing the lesion, role in immediate post-operative period and on follow up.

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch ( TGA ‐Mustard/Senning) and congenitally corrected TGA (cc TGA ). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty‐three adults with a systemic right ventricle (70% TGA ‐Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I‐ II ) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4‐stage free‐breathing bicycle test. They were compared with 12 healthy controls (39±10 years of age, 25% female, all New York Heart Association class I). TGA ‐Mustard/Senning patients had a higher global circumferential strain (−15.8±3.6 versus −11.2±5.2%, P =0.008) when compared with cc TGA , whereas global longitudinal strain and systemic right ventricle contractility during exercise were similar in both groups. Septal extracellular volume ( ECV ) in cc TGA was significantly higher than in TGA ‐Mustard/Senning (30.2±2.0 versus 27.1±2.7%, P =0.005). During exercise, TGA ‐Mustard/Senning had a fall in end‐diastolic volume and stroke volume (11% and 8%, respectively; both P ≤0.002), whereas cc TGA could increase their stroke volume in the same way as healthy controls. Because of a greater heart rate reserve in TGA ‐Mustard/Senning ( P for interaction=0.010), cardiac index and peak oxygen uptake were similar between both patient groups. Conclusions Caution should be exercised when evaluating pooled analyses of systemic right ventricle patients, given the differences in myocardial contraction pattern, septal extracellular volume, and the exercise response of TGA ‐Mustard/Senning versus cc TGA patients. Longitudinal follow‐up will determine whether abnormal exercise cardiac response is a marker of earlier failure.

Anatomic repair for congenitally corrected transposition requires thoughtful surgical planning at both initial (neonatal or late) presentation, and during definitive repair. An algorithmic approach to the overall management of this lesion, with its many associated intracardiac anomalies, is presented. Modified atrial switch with bidirectional superior cavopulmonary connection is commonly utilized and demonstrates favorable results through a 20-year experience. Herein, technical considerations learned during the implementation of this strategy are described and emphasize the concept, in selected cases, of native pulmonary root preservation by translocation as an adjunct that is uniquely suited by adopting this approach.

We describe the case of a 52-day-old child who was diagnosed with a rare combination of corrected transposition of great vessels – hypoplastic right ventricle with supracardiac total anomalous pulmonary venous connection.

The case of prenatal ultrasound diagnosis of combination corrected transposition of the great arteries (cTGA) with dextrocardia at 21 weeks of gestation is presented. The high-level ventricular septal defect and stenosis of pulmonary artery were associated cardiac pathologies. Another anomalies of the fetus were not detected. The fetus karyotype was normal, defined by classical cytogenetic analysis. The prenatal consultation assessed the prognosis for life and health as unfavorable. According to patient’s decision the pregnancy was terminated. Literature review of antenatal diagnostics of cTGA and combined cTGA with dextrocardia and the matters of short- and long-term prognosis for the fetus were presented in the paper. The issues of the difficulty of detecting cTGA in a fetus and prenatal counseling is discussed, especially in the presence of an abnormality of the intrathoracic location of the heart.

Dados clinicos Paciente evolui com dispneia aos esforcos habituais ha dois anos, com progressao a baixo debito cardiaco e sincope recentes, tratado na ocasiao com dobutamina e drogas anticongestivas habituais. Em uso atual de furosemida 40 mg, espironolactona 25 mg e losartana 12,5 mg. Exame fisico: Bom estado geral, eupneico, acianotico, pulsos normais nos 4 membros. Peso: 70 Kgs, Alt.: 160 cm, PAMSD: 90 x 60 mm Hg, FC: 94 bpm. […] Caso 3 / 2018 – Transposicao Corrigida das [...]

PURPOSE OF REVIEW: The right ventricle (RV) is uniquely at risk in many patients with repaired or palliated congenital heart disease (CHD) such as tetralogy of Fallot, corrected transposition, single right ventricle, and in those with pulmonary hypertension. These patients live with abnormal cardiac loading conditions throughout their life, predisposing them to right heart failure. RECENT FINDINGS: Standard heart failure therapies, developed to treat left ventricular failure, have failed to improve function or survival in patients with RV failure, suggesting a divergence in the molecular mechanisms of right versus left ventricular failure. As surgical techniques for repair of the most complex forms of RV-affecting CHDs continue to improve, more children with CHD will survive into adulthood. Long-term survival and quality of life will ultimately depend on our ability to preserve RV function. SUMMARY: The purpose of this review is to highlight the differences between the right and left ventricular responses to stress, our current knowledge of how the RV adapts to the unique hemodynamic stressors experienced by patients with CHD, and the need to better understand the molecular mechanisms of RV failure, providing new targets for the development of RV-specific heart failure therapeutics.

A 35 year old male, known case of corrected transposition of great arteries presented with exertional dyspnea and recurrent pre-syncope. 12 lead electrocardiogram revealed a regular rhythm at 75 beats per minute, P waves occurring on the upstroke of T waves and apparent 1:1 P-QRS relationship. The possibilities to be considered – complete AV block with junctional escape, junctional rhythm with 1:1 retrograde conduction, junctional rhythm with isorhythmic AV dissociation and prolonged PR interval have been discussed.