A case report of late physiologic repair of congenitally corrected transposition of the great arteries and pulmonary stenosis in a severely cyanotic patient: better late than never

Background Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored. Case summary A 38 years old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed: situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, no significant atrioventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Postoperative course was uneventful, trans-thoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At one month after discharge the patients is in NYHA class II. Discussion Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with anatomic repair.

Role of Echocardiography for Assessment of Cardiac Remodeling in Congenitally Corrected Transposition of Great Arteries

Background: The purpose of this study was to assess the role of echocardiography for a comprehensive assessment of cardiac remodeling, and the relationship between indices of cardiac remodeling and cardiovascular events (defined as the composite end point of heart failure hospitalization, heart transplant, or cardiovascular death) in adults with congenitally corrected transposition of great arteries (cc-TGA). Methods: This is a retrospective study of adults with cc-TGA who underwent echocardiogram (2003–2020). Offline image analysis was performed in all patients. Chamber (atrial and ventricular) function and size were assessed by strain imaging and 2-dimensional echocardiography. Results: Of 233 patients with cc-TGA (40±15 years), 123 (55%) had at least one cardiac procedure before baseline echocardiogram. Of 233 patients, 76% and 61% had left atrial dysfunction and systemic right ventricular dysfunction, respectively; while 43% and 11% had right atrial dysfunction and left ventricular dysfunction, respectively. During a median follow-up of 8.9 years, 114 (49%) underwent additional cardiac procedures, and 66 (28%) had cardiovascular events. Left atrial reservoir strain, right ventricular global longitudinal strain, right atrial reservoir strain, left ventricular systolic pressure, and left ventricular global longitudinal strain were independently associated with cardiovascular events. Conclusions: In addition to the clinical importance of right ventricular systolic dysfunction in cc-TGA that is already well described, the current study demonstrated, for the first time, that biatrial dysfunction was common and was associated with clinical outcomes. Since there are currently no effective therapies for atrial and ventricular dysfunction in patients with cc-TGA, there is a need for research to identify novel strategies to prevent atrial and ventricular dysfunction in this population.

MitraClip Repair of Right-Sided Atrio-ventricular Valve in a Patient with Congenitally-Corrected Transposition of Great Arteries: Case Report

Background Patients with ccTGA often develop tricuspid valve (systemic atrioventricular valve (AV) dysfunction due to RV overload and dilatation, but isolated mitral valve disease is rarely found. Isolated Mitral (subpulmonic AV -valve) interventions, specifically catheter-directed, have not been reported up to date. Case Summary A man with congenitally corrected transposition of the great arteries (ccTGA) is evaluated for dyspnoea. Multimodality imaging assessment confirmed severe right-sided mitral valve regurgitation due to prolapse. In light of high surgical risk, a minimally invasive transcatheter MitraClip procedure was pursued. Discussion To our knowledge, this is the first case of successful mitral valve repair via percutaneous approach using MitraClip in a patient with ccTGA and biventricular failure. Our case illustrates the safety and feasibility of the edge-to-edge procedure in such a rare instance, but also the importance of multimodality imaging (both invasive and non-invasive) and the Heart Team approach when caring for these complex patients.

The Effect of Size and Asymmetry at Birth on Brain Injury and Neurodevelopmental Outcomes in Congenital Heart Disease

Poor and asymmetric fetal growth have been associated with neonatal brain injury (BI) and worse neurodevelopmental outcomes (NDO) in the growth-restricted population due to placental insufficiency. We tested the hypothesis that postnatal markers of fetal growth (birthweight (BW), head circumference (HC), and head to body symmetry) are associated with preoperative white matter injury (WMI) and NDO in infants with single ventricle physiology (SVP) and d-transposition of great arteries (TGA). 173 term newborns (106 TGA; 67 SVP) at two sites had pre-operative brain MRI to assess for WMI and measures of microstructural brain development. NDO was assessed at 30 months with the Bayley Scale of Infant Development-II (n = 69). We tested the association between growth parameters at birth with the primary outcome of WMI on the pre-operative brain MRI. Secondary outcomes included measures of NDO. Newborns with TGA were more likely to have growth asymmetry with smaller heads relative to weight while SVP newborns were symmetrically small. There was no association between BW, HC or asymmetry and WMI on preoperative brain MRI or with measures of microstructural brain development. Similarly, growth parameters at birth were not associated with NDO at 30 months. In a multivariable model only cardiac lesion and site were associated with NDO. Unlike other high-risk infant populations, postnatal markers of fetal growth including head to body asymmetry that is common in TGA is not associated with brain injury or NDO. Lesion type appears to play a more important role in NDO in CHD.

Diagnostic performance of 320 cardiac MDCT angiography in assessment of PDA either isolated or associated with duct dependent congenital heart disease

Background Patent ductus arteriosus (PDA) is one of most common congenital heart defects, it's a unique vascular structure that provides direct communication between pulmonary and systemic circulation. MDCT angiography is a good imaging modality for evaluation of the PDAs and detection of their exact morphological type; course and diameters, which is important before percutaneous closure or stenting procedure of the PDA, also for selection of closure hardware. The aim of this study was to assess the role of MDCT angiography in qualitative and quantitative evaluation of PDA and associated cardiac and\or extracardiac anomalies. Results Echocardiography detected PDA in 28\30 cases while cardiac MDCT detected PDA in all studied 30 cases confirmed by cardiac catheterization and/or operation. MDCT angiography had sensitivity 100% and specificity 100% for PDA detection. PDA originated from aortic isthmus in 15 cases, inferior surface of aortic arch in 11 cases and innominate artery in 4 cases. The most common morphological type of PDA was type A (cone\46.67%) followed by type C (tubular\23.3%), type D (complex\10%), type E (elongated\13.33%) and type B (window\6.67%). The spearman correlation coefficient test demonstrated poor correlation between size of aortic end and MPA (P = 0.75), and between size of pulmonary end and diameter of MPA (P = 0.99) and also demonstrated fair correlation between length of PDA and MPA (P = 0.018). PDA was isolated in 4\30 cases and associated with cardiac and\or extra cardiac anomalies in 26\30 cases included; ASD (n = 18), VSD (n = 16), pulmonary atresia (n = 7), transposition of great arteries (n = 5), teratology of Fallot (n = 4), aortic coarctation (n = 4), persistent truncus arteriosus (n = 3), tricuspid atresia (n = 3), anomalous of pulmonary venous return (n = 3), hypoplastic segment of aorta (n = 2), Ebstein's anomaly (n = 1), bicuspid aortic arch (n = 1) and left hypoplastic heart syndrome (n = 1). Conclusion Cardiac MDCT angiography was superior to Echocardiography in detection, quantitative and qualitative evaluation of PDA either isolated or associated with congenital cardiac and\or extracardiac anomalies and was superior to Echocardiography in detection of associated extracardiac anomalies rather than associated intra cardiac anomalies.