Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Aim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.Results: Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were

Post-stenotic aortic dilatation

Aortic stenosis is the most common valvular heart disease affecting up to 4% of the elderly population. It can be associated with dilatation of the ascending aorta and subsequent dissection. Post-stenotic dilatation is seen in patients with AS and/or aortic regurgitation, patients with a haemodynamically normal bicuspid aortic valve and following aortic valve replacement. Controversy exists as to whether to replace the aortic root and ascending aorta at the time of aortic valve replacement, an operation that potentially carries a higher morbidity and mortality. The aetiology of post-stenotic aortic dilatation remains controversial. It may be due to haemodynamic factors caused by a stenotic valve, involving high velocity and turbulent flow downstream of the stenosis, or due to intrinsic pathology of the aortic wall. This may involve an abnormality in the process of extracellular matrix remodelling in the aortic wall including inadequate synthesis, degradation and transport of extracellular matrix proteins. This article reviews the aetiology, pathology and management of patients with post-stenotic aortic dilatation

A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease

AbstractIntroduction: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. Methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. Results: Histologic examination demonstrated large, dilated blood vessels (“lakes”) and clustered, smaller vessels (“chains”) in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P = .01). Immunohistochemistry demonstrated uniform staining for type IV collagen and α-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. Conclusions: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. Immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology. (J Thorac Cardiovasc Surg 1999;117:931-8

Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries

The Senning or Mustard procedure combined with the arterial switch operation (ASO) (+/- VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2-9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefit

Modified Nikaidoh procedure for the correction of complex forms of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: mid-term results

OBJECTIVES: Different surgical techniques for the treatment of complex transposition of the great arteries (TGA) with ventricular septal defect and left ventricular outflow tract obstruction (LVOTO) have been developed, in particular the Rastelli operation, the réparation à l'étage ventriculaire procedure and the Nikaidoh procedure. The hitherto published results of the Nikaidoh procedure and its modifications compare favourably with those of other techniques; however, experience with the Nikaidoh procedure is still limited. Here, we report our institutions' early and mid-term results with modifications of the Nikaidoh procedure. METHODS: Twenty-one patients who underwent a modified Nikaidoh procedure between 2006 and 2012 at our institution, either as aortic root translocation (n = 17) or as en bloc rotation of the arterial trunk (n = 4), were studied retrospectively. RESULTS: There were 2 early and 1 mid-term deaths. The follow-up continued for a median of 2.3 years (range 0.3-6.4 years). During the follow-up, the performance of the reconstructed left ventricular outflow tract (LVOT) remained excellent: no reobstruction and no aortic valve regurgitation classified as more than mild were observed. Left ventricular function was well preserved. In 4 patients, a significant reoccurring right ventricular outflow tract obstruction due to conduit failure was observed; so far, two reoperations with conduit replacement have been necessary. The mean right ventricular outflow tract peak gradient was 24 ± 7.2 mmHg at the last follow-up in the remaining patients. No reobstruction of the right ventricular outflow tract occurred in patients with preserved pulmonary valve tissue after en bloc rotation. CONCLUSIONS: The aortic translocation procedure is a valuable surgical option for patients with complex (TGA) with ventricular septal defect and LVOTO. The mid-term results document excellent performance of the reconstructed LVOT. Modifications of the Nikaidoh procedure that preserve pulmonary valve tissue may further reduce the need for right ventricular outflow tract reoperation