corrected transposition
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Abstract 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.

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 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.

Abstract 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.

Abstract Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.