Transcatheter systemic atrioventricular valve-in-valve implantation in a congenitally corrected transposition of the great arteries patient.

We present an asymptomatic pregnant patient with congenitally corrected transposition of the great arteries and severe atrioventricular bioprosthesis regurgitation - with increased maternal and fetal risk due to volume overload. She was considered high risk for reintervention and was submitted to an off-label post-partum transcatheter valve-in-valve implantation with a Sapiens 3 valve. The procedure was successful, and she remains asymptomatic 30 months after - and even went through another successful pregnancy.

A leadless pacemaker implantation for a patient with systemic right ventricle under ventricular assist device support.

Patients with congenitally corrected transposition of the great arteries (ccTGA) often develop complete atrioventricular block and heart failure due to the abnormal disposition of atrioventricular node and disadvantage of systemic right ventricle. These issues are managed with a pacing system and a ventricular assist device (VAD), respectively. While technological advances offer new treatment strategies, the simultaneous deployment of a leadless pacemaker and a VAD in cases of ccTGA remains unexplored. Here, we present a case of leadless pacemaker implantation for a VAD-supported ccTGA patient. The safety of a leadless pacemaker for a subpulmonary left ventricle and electromagnetic interference between devices are major concerns when implanting a leadless pacemaker; however, the current case overcomes these obstacles. There were no perioperative complications, and both devices were functioning without problems during a one-year follow up. We expect that, even in patients with cardiac complexity such as systemic right ventricle under VAD support, a leadless pacemaker could become the treatment of choice if the indication is appropriate, although careful and close follow up is needed. Learning objective: Technological advances expand treatment strategies and provide significant benefits to patients with adult congenital heart disease (ACHD). However, discussion of the combination of a leadless pacemaker and a ventricular assist device (VAD) is rare. We demonstrated the efficacy of a leadless pacemaker for a subpulmonary left ventricle in a patient with systemic right ventricle on VAD. This approach could be an option even for ACHD patients.

Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature.

BACKGROUND: Congenitally corrected levo-transposition of the great arteries (L-TGA) is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy. In L-TGA, the double discordance, atrioventricular and ventriculoarterial, create an acyanotic milieu which allows patients to survive their early decades, however, progressive systemic right ventricle (sRV) dysfunction creates complications later in life. sRV dysfunction and remodeling predisposes patients to intracardiac thrombus (ICT) formation. CASE SUMMARY: A 40-year-old male with L-TGA presented with symptoms of acute decompensated heart failure. In childhood, he had surgical repair of a ventricular septal defect. In adulthood, he developed sRV dysfunction, systemic tricuspid valve (sTV) regurgitation, and left-bundle branch block for which he underwent cardiac resynchronization therapy. Transthoracic echocardiogram showed a sRV ejection fraction of 40%, severe sTV regurgitation, and a newly identified sRV ICT. ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardiography. Our patient was optimized with guideline-directed medical therapy and diuresis. Anticoagulation was achieved with a vitamin K antagonist (VKA) and he was later referred for evaluation by advanced heart failure and heart transplant services. CONCLUSION: Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease. This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.

Transcatheter Systemic AV Valve-in-Valve Implantation in a Patient With LTGA and Ebstein Anomaly.

We present the first in-human transcatheter systemic atrioventricular valve-in-valve implantation in a 37-year-old patient with Ebstein anomaly, levo-transposition of the great arteries, and prior systemic valve replacement. She had severe bioprosthetic valve regurgitation and reduced systolic function. She had high surgical risk and was planned for transcatheter intervention. (Level of Difficulty: Advanced.).

Modified pulmonary artery banding: A novel strategy for balancing pulmonary blood flow with transposed great arteries.

OBJECTIVE: To study the outcomes of a novel modified pulmonary artery banding (mPAB) technique used for staged repair of a subset of patients with complex transposition physiology. METHODS: A total of 13 patients who underwent mPAB during their staged repair (biventricular repair [BVR], n = 6) or palliation (1-1/2 repair, n = 1; univentricular repair [UVR], n = 6) from 2004 to 2020 were studied retrospectively. A restrictive interposition graft was used to reconstruct the main PA between the pulmonary root and the distal pulmonary confluence, functioning as a mPAB. Twelve of the 13 patients (92.3%) underwent a concurrent arterial switch operation (ASO), of which 6 were palliative ASOs for 1-1/2 repair (n = 1) or UVR (n = 5). Patient weight and cardiac anatomy determined the size of interposition graft. RESULTS: The disease spectrum included dextro transposition of the great arteries (d-TGA) with multiple ventricular septal defects (VSDs) (n = 4), Taussig-Bing anomaly (n = 3), d-TGA with VSD and hypoplastic right ventricle (RV) (n = 3), double-inlet left ventricle with l-TGA (n = 2), and congenitally corrected TGA with double-outlet RV (n = 1). The Lecompte procedure was performed in 10 patients. Predischarge echocardiography revealed a band gradient of 61 mm Hg (interquartile range [IQR], 40-90 mm Hg) for BVR/1-1/2 ventricular repair (n = 7) and 49 mm Hg (IQR, 37-61 mm Hg) for UVR (n = 6). Survival was 100% at a median follow-up of 3.7 years (IQR, 2.6-4.0 years). CONCLUSIONS: The mPAB technique is effective and reproducible for staged BVR or UVR for patients with TGA. It effectively regulates pulmonary blood flow, may reduce neopulmonary root distortion, and eliminates complications associated with band migration in standard PAB.

Transcatheter Aortic Valve Replacement in a Patient With Levo-Transposition of the Great Arteries.

Levo-transposition of the great arteries is a congenital heart disease characterized by atrioventricular and ventricular-arterial discordance. Aortic valve disease in levo-transposition of the great arteries patients is uncommon. We present a patient with levo-transposition of the great arteries and severe aortic stenosis who successfully underwent transcatheter aortic valve replacement and the diagnostic and procedural challenges involved. (Level of Difficulty: Intermediate.).

Levo-transposition of the Great Arteries in an Adult Patient: Management Considerations and Treatment Strategy.

Levo-transposition of the great arteries (L-TGA) is a rare congenital heart anomaly associated with the increased risk of developing heart failure (HF) as well as complete heart block at a young age. Due to limited data regarding the treatment strategy in the adult L-TGA sub-population, shared medical decision-making should occur between the patient and a team of physicians. Clinical status, age, and associated cardiac lesions or rhythm disturbances can affect patient outcomes. These factors should be considered prior to pursuing a surgical versus a medicinal approach.

Spontaneous Coronary Artery Dissection in Levo-Transposition of the Great Arteries.

Spontaneous coronary artery dissection (SCAD) is an uncommon form of acute coronary syndrome (ACS). With the increasing recognition of this condition presenting as ACS in females, clinicians should always remain vigilant for it. This case describes a patient with congenital heart disease presenting with SCAD. (Level of Difficulty: Advanced.).

Safety and efficacy of atrial antitachycardia pacing in congenital heart disease.

BACKGROUND: Intra-atrial reentrant tachycardia (IART) is a common sequela in the congenital heart disease (CHD) population, and it significantly increases morbidity and mortality. Atrial antitachycardia devices (ATDs) capable of atrial antitachycardia pacing (ATP) therapy have been used to manage IART in the CHD population, but there are limited data on their safety and efficacy. OBJECTIVES: To determine whether ATD implantation was associated with reduced direct current (DC) cardioversions and to compare ATP success between different CHD diagnoses and ATP programs. METHODS: A single-center retrospective chart review was performed on CHD patients with ATDs. Demographic data were collected in addition to the number of DC cardioversions required before and after ATD implantation; data on ATP efficacy and the specific ATP program utilized; and adverse events related to ATD implantation or subsequent ATP treatments. RESULTS: ATD implantation in 91 CHD patients was associated with a significant reduction in DC cardioversions (P < .01). Overall, 72% of IART episodes were successfully terminated by ATP. Patients with levo-transposition of the great arteries experienced lower rates of ATP success than the remainder of the cohort (P < .01). There was no evidence of degeneration to ventricular arrhythmia or death directly attributed to ATP. CONCLUSION: ATD implantation was associated with reduced DC cardioversion burden. Patients with levo-transposition of the great arteries may experience lower ATP efficacy than patients with other CHDs; however, a larger patient population is required to better determine subgroup efficacy. These results support the safety and efficacy of ATP in the CHD population.