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.

IATROGENIC VENTRICULAR SEPTAL DEFECT DURING RIGHT VENTRICULAR ASSIST DEVICE INSERTION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES

This case report describes the management of a large iatrogenic ventricular septal defect (VSD) created by the coring device during systemic ventricular assist device (RVAD) insertion in a 16 year-old patient with congenitally corrected transposition of the great arteries. The VSD was closed by bovine pericardial patch and the ventriculotomy was extended laterally to relocate the VAD sewing ring. After RVAD implantation, patient initially remained cyanotic, potentially due to a tiny VSD patch leak with right to left shunting. Hypoxia was successfully corrected by rescue nitric oxide infusion and patient was bridged to transplant after 91 days.

Unguarded Tricuspid Orifice with Congenitally Corrected Transposition of the Great Arteries and Aortic Atresia- A Highly Unusual Case

Unguarded tricuspid orifice is a very rare anomaly. It is characterised by the absence of one or more of the tricuspid valve leaflets resulting in severe tricuspid regurgitation and right heart failure. It is rarely an isolated anomaly but more often associated with pulmonary atresia and intact ventricular septum. When the ventricles are inverted however, the result of outflow tract obliteration is not pulmonary atresia, but aortic atresia. This anomaly has been described in the literature in only 2 cases so far. We present a case of a neonate with unguarded tricuspid orifice with absence of all tricuspid leaflets, congenitally corrected transposition of the great arteries and aortic atresia. The severe tricuspid regurgitation and right ventricular enlargement would have required a Norwood-like procedure combined with a right ventricular plication. Due to the complexity of this lesion no surgical therapy was attempted in consent with the parents.

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.