Congenital Corrected Transposition Of Great Arteries Research Articles

Permanent Pacemaker Implantation in a Patient with Congenitally Corrected Transposition of Great Arteries and Mesocardia: An Infrequent Happenstance

We present a compelling case of a 37-year-old male who, after three decades of asymptomatic living, was diagnosed with congenitally corrected transposition of great arteries (CCTGA). Initially presenting with troubling symptoms of dizziness and syncope, further evaluation revealed complete heart block, a significant finding necessitating intervention. Despite the rarity of symptomatic presentation in CCTGA, the severity of the heart block prompted the implantation of a DDD pacemaker to ensure adequate management. With meticulous care and precision, the implantation procedure was successfully executed, offering hope for improved cardiac function and symptom relief. Remarkably, the patient remained asymptomatic during subsequent follow-up visits, highlighting the effectiveness of the intervention in mitigating the risks associated with high atrioventricular block in CCTGA. This case serves as a testament to the importance of vigilant monitoring and timely intervention in managing complex cardiac anomalies, ultimately enhancing patient outcomes and quality of life.

A Case of Late Diagnosis of Anatomically Corrected Malposition of the Great Arteries

Anatomically corrected malposition of the great arteries (ACMGA) is a rare form of congenital heart disease. This abnormality belongs to conotruncal anomalies. ACMGA is an anomaly in which aorta arises from the left ventricle and pulmonary artery arises from the right ventricle but great arteries are located in parallel relationships. Therefore, diagnosis of this disease is complicated due to frequent diagnostic errors.
 The aim. To share our experience of late diagnosis of ACMGA in a patientwho was previously mistakenly diagnosed with congenitally corrected transposition of great arteries (CC-TGA).
 Case report. We present a case of late diagnosis of ACMGA. An 11-day-old newborn was admitted to the Ukrainian Children’s Cardiac Center in 2007. After clinical examination, which included ultrasound examination, the diagnosis of CC-TGA, pulmonary stenosis, ventricular septal defect and atrial septal defect was conϐirmed. The patient was in a stable condition and there were no signs of heart failure. That is whythe patient was discharged. In 2017, cardiac catheterization was performed, and there were doubts about the diagnosis because of untypical anatomical features.
 In 2022, we performed computed tomography and magnetic resonance imaging. The diagnosis of ACMGA was made.
 Conclusions. ACMGA is an unusual form of congenital heart disease. The diagnosis of ACMGA is challenging, because its anatomical features are similar to those of CC-TGA. The difference of both these diagnoses is normal anatomy and hemodynamics but abnormal relationship in ACMGA, in contrast to abnormal anatomy in CC-TGA.

Percutaneous edge-to-edge repair in the setting of a severe systemic tricuspid regurgitation complicating a congenitally-corrected transposition of great arteries

This study sought to report the specific considerations and implications of percutaneous edge-to-edge repair in one case of severe systemic tricuspid regurgitation (STR). In congenitally-corrected transposition of great arteries (ccTGA) tricuspid valve results spontaneously in systemic position. In this situation, progressive STR and systemic right ventricular (SRV) dysfunction are frequently observed, leading ultimately to altered functional capacities and pulmonary edema. In this setting surgical valve repair or replacement is challenging given the underlying SRV failure, therefore heart transplant is usually preferred. However, recent studies have underlined the reliability of edge-to-edge repair for secondary severe tricuspid and mitral regurgitation. Few data are currently available concerning the percutaneous management of STR. We report about the case of a 59 year-old man presenting with dyspnea and mild peripheral edema. He was known for non-operated ccTGA with SRV ejection fraction (EF) of 25%, and severe STR, defined as an effective regurgitant orifice area of 40 mm 2 . The patient was in theory eligible for heart transplant but a percutaneous approach was first attempted. The procedure was performed under general anesthesia, transesophageal echocardiography guidance and through a femoral vein route. A MitraClip XTR device (Abbott Vascular, Santa Clara, CA) was successfully implanted between the septal and the anterior leaflets, without any complication ( Fig. 1 ). After the procedure the STR improved dramatically from grade IV to I. After 2 years follow-up the patient is still asymptomatic and free from severe STR, concomitantly SRV EF improved up to 32% under optimal medical treatment. Percutaneous edge-to-edge therapy of STR in ccTGA seems to be feasible, safe and effective to reduce regurgitation grade, to limit SRV dilatation and to improve patient symptoms.

B-PO01-108 ATRIAL TACHYCARDIA ABLATION AT THE PULMONIC VALVE IN A PATIENT WITH CONGENITALLY CORRECTED TRANSPOSITION OF GREAT ARTERIES

Patients with congenitally-corrected transposition of great arteries (ccTGA) have anatomical differences with clinical ramifications for catheter ablation. To review the relevant anatomical structures in patients with ccTGA for atrial tachycardia (AT) ablation. N/A A 50 year old man with ccTGA with palpitations, refractory to medical management, presented for catheter ablation. Baseline ECG demonstrated AT with biphasic narrow P-waves in V1, suggestive of a para-Hisian origin. During electrophysiologic study, overdrive pacing at various cycle lengths yielded a fixed return cycle length favoring a micro-reentrant tachycardia. Right and left atrial mapping demonstrated early activation along the right atrial (RA) septum, 40 msec preceding the surface P-wave. Ablation at this site resulted in 2:1 exit block, with subsequent return of AT. Mapping of the mitral annulus and pulmonic outflow tract delineated the His-Purkinje system (Figure). Using intracardiac echocardiography, the pulmonic valve was visualized directly opposite to the earliest activation site in the RA (pulmonic and aortic valve locations are reversed in ccTGA). Mapping inside the pulmonic valve demonstrated a double-potential preceding the surface P-wave by 120 msec. Ablation resulted in immediate termination of AT, with no recurrence despite aggressive programmed stimulation, burst pacing, and isoproterenol infusion. We demonstrated the anatomic mapping and ablation of incessant AT in a patient with ccTGA. It is important to appreciate the anatomic relationship between the RA, pulmonic outflow tract, and conduction system to avoid inadvertent injury.

Three-dimensional mapping–guided permanent His bundle pacing in a patient with corrected transposition of great arteries

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.

Successful ablation for premature ventricular contraction originating from moderator band of morphologic right ventricle in congenitally corrected transposition of great arteries

A 54-year-old man with congenitally corrected transposition of great arteries (CCTGA) was referred to our hospital for palpitation. 24-hour Holter ECG showed frequent premature ventricular contraction (PVC) and we performed catheter ablation for this PVC. Pace-mapping was performed in morphologic right ventricle (RV) by transaortic approach. Perfect pace-map was achieved in morphologic RV midpart lateral and ablation at this site could eliminate the clinical PVC. After the ablation, by integrating ablation site and 3D mapping, we diagnosed that the clinical PVC was originated from the moderator band (MB) of morphologic RV.

A Rare Case of Congenitally Corrected Transposition of Great Arteries (CCTGA)

A Rare Case of Congenitally Corrected Transposition of Great Arteries (CCTGA)

Dilatable pulmonary artery band: Safety and efficacy of balloon dilatation.

Review early and midterm results of dilatable pulmonary artery band (PAB). PAB is performed in various conditions: as a transient stage before ventricular septal defect (VSD) closure, as a palliative procedure for multiple VSD or to prepare the left ventricle (LV) before switch or double switch operations in transposition of the great artery (TGA) or congenitally corrected transposition of great arteries (CCTGA). All children with dilatation of PAB were reviewed. Reason for PAB and for cardiac catheterization, hemodynamics, and outcomes were analyzed. Between 2002 and 2014, 28 patients were identified. Reason for interventional dilatation of the PAB was supra systemic right ventricular (RV) pressure in four patients, aortic recoarctation in one patient, cyanosis in seven patients, RV failure in one patient, suprasystemic LV pressure in two CCTGA patients and spontaneous closure of VSD in other patients. All patients underwent successful balloon dilatation using high (n = 21) or low pressure balloons with a mean diameter of 12 mm. 23 Patients had no further interventions. Five patients had reintervention: one needed two dilatations of PAB and four had surgery (one double switch, one vicious pulmonary artery (PA) band removal, one in a context of severe RV dysfunction with tricuspid regurgitation, and one for VSD closure). One patient died of RV failure following surgery. Balloon dilatation of dilatable PAB carries good results with achievement of hemodynamic aim in most of the cases and definitive treatment in the majority of patients. Careful and sequential dilatation with shunt measurement is mandatory in patients with significantly residual VSD. © 2016 Wiley Periodicals, Inc.

Anesthetic experience of two consecutive caesarean sections in a parturient with congenitally corrected transposition of great arteries and complete heart block: A case report

We describe the clinical course of two subsequent caesarean sections in the same parturient with congenitally corrected transposition of great arteries (CCTGA) complicated with complete heart block. CCTGA represents <1% of all forms of congenital heart diseases. In such patients, the hemodynamic effects of pregnancy and surgery may trigger systemic ventricular failure. In these parturients, ventricular dysfunction will also progress rapidly with subsequent pregnancies. The literature review showed no evidence describing the anesthetic management of these high-risk parturients. We used combined spinal epidural anesthesia uneventfully for both the caesarean sections in this patient. This case report will discuss the details of this rare congenital heart disease and the anesthetic management.

Transversus abdominis plane block as sole anaesthetic technique for inguinal hernia repair in two patients having complex medical conditions

Sir, Transversus abdominis plane (TAP) block has been used as a sole anaesthetic technique for the patients with multiple co-morbidities presenting for emergency laparotomy.[1,2] We share our experience of using TAP block for the surgical anaesthesia in two patients scheduled for inguinal hernioplasty who had associated complex medical conditions. First patient was a 52-year-old male, known case of congenitally corrected transposition of great arteries (CCTGA) associated with severe pulmonary stenosis and a pressure gradient of 64 mm Hg, large non-restrictive ventricular septal defect (VSD) and right ventricular (i.e., physiologic left ventricle) dysfunction. He was stable on treatment with tablet torsemide. Second case was a 54-year-old male known case of Takayasu's arteritis (TA) and hypertension on treatment with tablet metoprolol and losartan. The patient had the history of claudication and weakness of left upper limb. The peripheral pulses in left upper limb were feeble and blood pressure (B.P) was lower compared to right upper limb (112/70 mm Hg vs. 172/73 mm Hg). For surgery, ultrasound guided (USG) TAP block was administered in both cases using in-plane posterior approach with 20 ml of 0.5% bupivacaine and 10 ml of 2% lignocaine. For visceral analgesia, the TAP block was supplemented with infusions of dexmedetomidine at the loading dose of 1 μg/kg over 10 min followed by 0.5-0.6 μg/kg/min infusion and fentanyl at 75-100 μg/h, respectively, in the first and the second case. In the first patient, T10 -L1 dermatomes were blocked whereas in second patient L1 was spared; only T10 -T12 blockade was achieved as checked by pin prick. The incision line was infiltrated with 2% lignocaine in the second case before surgery. The anaesthetic monitoring in both cases included continuous electrocardiogram, continuous invasive B.P monitoring, pulse oximetry, and temperature. The intraoperative vital parameters were stable and surgery was completed uneventfully in both cases. CCTGA is an uncommon congenital cardiac anomaly characterized by TGA along with the inversion of both ventricles and thus there is both atrio-ventricular and ventriculo-great vessel discordance. The anaesthetic haemodynamic goals in these cases are to maintain systemic vascular resistance (SVR) to avoid right to left shunt by VSD and to preserve myocardial contractility.[3] TA involves a focal stenotic process in the aorta and proximal segment of its main branches. In our second case, though angiography was not done, based on the history of claudication and findings of feeble pulses and decreased B.P. in left arm compared to right is suggestive of stenotic area to be in the arch of aorta. These patients may not tolerate the sudden fall in SVR as they have compromised regional circulation and hence similar to the first patient our intraoperative haemodynamic goal was to maintain SVR. We decided to use USG TAP block in both cases as being an abdominal field, it holds the promising advantage of maintaining the haemodynamics of patients, which are altered in general or neuraxial anaesthesia and additionally provides an excellent postoperative analgesia for 24-48 h. We chose dexmedetomidine infusion in the first patient as it has the combination of analgesic, sedative, and anxiolytic properties with the preservation of respiratory functions. There are 3 main issues which need consideration while using TAP block as a sole anaesthetic modality for surgery. First, TAP block covers only the somatic component of surgical pain; for visceral pain analgesia has to be supplemented with other analgesic agents hence it cannot be a sole anaesthetic technique. Second, the extent of dermatomal block provided by TAP block is uncertain. Both pinprick using blunt needle and cold disinfectant swabs can be used to assess dermatomal extent of sensory blockade. Lee et al.[3] reported that posterior approach produced a median sensory block of three dermatomal segments, the most cephalad being T10. We found the L1 dermatome to be spared in the second case despite using 30 ml of drug for block. Since the incision for inguinal hernia repair lies over T12 -L1 segments, additional infiltration of local anaesthetics (L.A) was required before surgery. Niraj et al.[4] also observed that posterior approach TAP block usually spares L1 segment. Another disadvantage of TAP block is potential to produce L.A. toxicity since large volumes of L.A. are used. There are case reports[5] of cardiac arrests due to L.A. systemic toxicity following TAP block. Therefore the volume of drug used should be carefully determined and the patient should be observed for at least 45 min after TAP block institution during intraoperative and postoperative period. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

17-year experience in surgical management of congenitally corrected transposition of the great arteries: a single-centre's experience.

We report our surgical experience in congenitally corrected transposition of great arteries (CCTGAs) and the long-term follow-up result. From January 1995 to February 2012, 56 patients with CCTGA received definite surgical repair; 15 patients received conventional repair (Group I), 18 patients received anatomical repair (Group II) and 23 patients received single ventricular palliation (Group III). They were followed for early and late mortality, long-term survival, postoperative morbidity and reintervention or reoperation. The overall survival rate was 80% at 16 years in Group I, 53% at 13 years in Group II and 100% at 13 years in Group III. After excluding the early surgical mortality, the long-term survival rate was 92% at 16 years in Group I, 64% at 13 years in Group II and 100% at 13 years in Group III. Patients with significant tricuspid valve regurgitation showed the worst outcome after surgery. Our series showed good results with single ventricular palliation (SVP) in CCTGA with complex anatomy, but the long-term result should be followed. Anatomical repair is the choice of operation only for those with favourable anatomy. The more complicated intracardiac repair may result in late left ventricular outflow tract obstruction, various degrees of atrioventricular block, systemic or pulmonary venous return obstruction and the lack of an ideal conduit (e.g. homograft) for Rastelli reconstruction. Therefore, we preferred SVP in patients with complex and unfavourable anatomy.

A Case of Congenitally Corrected Transposition of Great Arteries (CCTGA)

Recent years, much scientific attention has been given to congenital heart diseases (CHD) and probable complications. Congenitally corrected transposition of the great arteries (CCTGA) is a rare, complex form of congenital heart defects. CCTGA is characterized by atrioventricular (AV) and ventriculoarterial (VA) discordance and hence by a physiologically normal direction of blood flow sometimes called "double discordance". The development of complete AV block and global ventricular dysfunction has been identified as the cause of cardiac death. This paper presents a case of CCTGA with rhythm disorders and exertional dyspnea.&#x0D; CBMJ 2015 January: Vol. 04 No. 01 P: 37-40

Unusual presentation of congenitally corrected transposition of great arteries (CCTGAs) in a middle aged male

Congenitally corrected transposition of great arteries (CCTGAs) is a rare congenital heart disease. The literature review reveals that asymptomatic CCTGA is even rarer in elderly male. We report a case of a 54-year-old male, with no past significant medical history, who presented with angina type of chest pain. On examination, he had bradycardia, lower limb hypertension and grade 3 pansystolic murmur in the mitral area. Investigations revealed complete heart block in electrocardiograph, chest radiograph showed cardiomegaly, transthoracic echocardiography showed an apically located systemic atrio-ventricular (AV) valve, parallel arrangement of the great arteries, AV and ventriculoarterial discordance, hypertrophied right ventricle and significant systemic AV valve insufficiency with ejection fraction of 40%. These findings were consistent with congenitally corrected transposition of the great arteries with systemic AV valve insufficiency. Holter monitoring showed complete heart block, no significant pauses and one event of ill-sustained ventricular tachycardia (ventricular triplet) with the patient being asymptomatic during the study period. Need of internal cardioverter-defibrillator or pacemaker was deferred. The remaining hospital stay was uneventful. At the time of discharge, the patient was stable.

Multiple accessory pathways in a patient with congenitally corrected transposition of the great arteries and severe malformation of the coronary sinus

A 29-year-old patient diagnosed with (S,L,L) congenitally corrected transposition of great arteries (CCTGA) and pre-excitation is presented. We performed a coronary angiogram and it documented …

Transposition of great arteries: new insights into the pathogenesis

Transposition of great arteries (TGA) is one of the most common and severe congenital heart diseases (CHD). It is also one of the most mysterious CHD because it has no precedent in phylogenetic and ontogenetic development, it does not represent an alternative physiological model of blood circulation and its etiology and morphogenesis are still largely unknown. However, recent epidemiologic, experimental, and genetic data suggest new insights into the pathogenesis. TGA is very rarely associated with the most frequent genetic syndromes, such as Turner, Noonan, Williams or Marfan syndromes, and in Down syndrome, it is virtually absent. The only genetic syndrome with a strong relation with TGA is Heterotaxy. In lateralization defects TGA is frequently associated with asplenia syndrome. Moreover, TGA is rather frequent in cases of isolated dextrocardia with situs solitus, showing link with defect of visceral situs. Nowadays, the most reliable method to induce TGA consists in treating pregnant mice with retinoic acid or with retinoic acid inhibitors. Following such treatment not only cases of TGA with d-ventricular loop have been registered, but also some cases of congenitally corrected transposition of great arteries (CCTGA). In another experiment, the embryos of mice treated with retinoic acid in day 6.5 presented Heterotaxy, suggesting a relationship among these morphologically different CHD. In humans, some families, beside TGA cases, present first-degree relatives with CCTGA. This data suggest that monogenic inheritance with a variable phenotypic expression could explain the familial aggregation of TGA and CCTGA. In some of these families we previously found multiple mutations in laterality genes including Nodal and ZIC3, confirming a pathogenetic relation between TGA and Heterotaxy. These overall data suggest to include TGA in the pathogenetic group of laterality defects instead of conotruncal abnormalities due to ectomesenchymal tissue migration.

Atrial flutter in grown-up congenital heart (GUCH) patients: Clinical characteristics of affected population

Objective: To define occurrence, lesions and clinical characteristics of grown-up congenital heart (GUCH) patients who develop atrial flutter (AFL). Design: All GUCH patients who presented as inpatients or outpatients with documented sustained AFL between 1996 and 1998 were studied prospectively. Retrospective review of case notes for basic data relating to underlying anomaly, prior surgery and age at onset of AFL, enquiry into events before the first attack. Clinical state was assessed by Ability Index before AFL and at last visit. Setting: Designated quaternary service for GUC in a tertiary referral centre. Results: From October 1996 to April 1998, 100 consecutive patients (49 female) aged 17–77 (mean 35) years, who presented to the GUCH Unit at Royal Brompton Hospital with a sustained attack of AEL documented by a 12 lead electrocardiogram were studied. Four basic cardiac anomalies accounted for 75% patients: one ventricle (26), atrial septal defect (ASD) (19), transposition of great arteries (TGA) (17) and Tetralogy of Fallot (13). AFL occurred occasionally in small ventricular septal defect (VSD), congenital corrected TGA (CC-TGA), pulmonary stenosis and pulmonary atresia with or without VSD. 86/100 patients had undergone cardiac surgery: Fontan 19 (22%), reconstruction of right ventricular outflow tract 17 (20%), closure of ASD 15 (17%), Mustard for TGA 13 (15%), and other palliative surgery 22 (26%). AFL occurred in ‘natural history’ (unoperated) in 14 (14%) mostly in CC-TGA, ASD and Fallot. Age at first attack was 6–64 (mean 28) years with the first attack occurring at younger age after Mustard (22±7 years) and Fontan (24±7), than in un-operated ASD (46±13) and CCTGA (31±10). Haemodynamic abnormalities from anatomical causes were present in 62/74 (84%) patients who had undergone reparative surgery and included venous pathway obstruction, pulmonary regurgitation and pulmonary hypertension. Additional factors which could have precipitated AFL in prone patients were present in 63. New symptoms appeared in 96 patients with the first attack of AFL. Ability Index prior to onset in 90 patients who have been followed-up for more than 1 year since the first onset was 1 in 52, 2 in 31, 3 in 6 and 4 in 1 patients. At the last visit (mean time from the first onset 6.6±4.7 years), only 9 patients remained with Ability Index 1, 43 in 2, 20 in 3 and 18 in 4 despite return to sinus rhythm. Conclusion: One ventricle heart, ASD, transposition of great arteries and Tetrology of Fallot are the most common underlying anomalies in GUCH patients who develop AFL. It is less commonly seen in unoperated patients. When occurs AFL compromises patients’ activities and deteriorates the clinical condition. Residual or developed haemodynamic abnormalities and precipitating factor are often present in this patients, hence full investigation and close follow up are necessary once AFL develops.