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 reoperatio

Extracardiac Fontan with T-shape conduit in non-confluent pulmonary arteries

A 34 months-old male patient with double inlet right ventricle with nonconfluent pulmonary arteries who underwent successful extracardiac fenestarated Fontan procedure using pre-designed T-shaped PTFE vascular graft after multi-step rehabilitation of the diminutive hilar pulmonary arteries. At first we performed 6 mm confluent pulmonary artery vascular graft implantation with 4 mm BT shunt at patient's 4 weeks old. At 9 months of patient, we upsized the confluent pulmonary arterial graft to 8 mm with bidirectional cavopulmonary connection, and, at 34 months, we performed extracardiac conduit Fontan procedure with pre-designed T-shape conduit including the confluent pulmonary arterial portion at last. Patient shows excellent functional status and development

EASL-ERN position paper on liver involvement in patients with Fontan-type circulation

Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable. However, based on the increasing number of adult Fontan patients and recent research interest, the European Association for The Study of the Liver and the European Reference Network on Rare Liver Diseases thought a position paper timely. The aims of the current paper are: (1) to provide a clear definition and description of FALD, including clinical, analytical, radiological, haemodynamic, and histological features; (2) to facilitate guidance for staging the liver disease; and (3) to provide evidence- and experience-based recommendations for the management of different clinical scenarios.</p

Long-Term Results of Cell-Free Biodegradable Scaffolds for In Situ Tissue-Engineering Vasculature: In a Canine Inferior Vena Cava Model

We have developed a new biodegradable scaffold that does not require any cell seeding to create an in-situ tissue-engineering vasculature (iTEV). Animal experiments were conducted to test its characteristics and long-term efficacy. An 8-mm tubular biodegradable scaffold, consisting of polyglycolide knitted fibers and an L-lactide and ε-caprolactone copolymer sponge with outer glycolide and ε-caprolactone copolymer monofilament reinforcement, was implanted into the inferior vena cava (IVC) of 13 canines. All the animals remained alive without any major complications until euthanasia. The utility of the iTEV was evaluated from 1 to 24 months postoperatively. The elastic modulus of the iTEV determined by an intravascular ultrasound imaging system was about 90% of the native IVC after 1 month. Angiography of the iTEV after 2 years showed a well-formed vasculature without marked stenosis or thrombosis with a mean pressure gradient of 0.51±0.19 mmHg. The length of the iTEV at 2 years had increased by 0.48±0.15 cm compared with the length of the original scaffold (2–3 cm). Histological examinations revealed a well-formed vessel-like vasculature without calcification. Biochemical analyses showed no significant differences in the hydroxyproline, elastin, and calcium contents compared with the native IVC. We concluded that the findings shown above provide direct evidence that the new scaffold can be useful for cell-free tissue-engineering of vasculature. The long-term results revealed that the iTEV was of good quality and had adapted its shape to the needs of the living body. Therefore, this scaffold would be applicable for pediatric cardiovascular surgery involving biocompatible materials