Surgical mitral valve replacement with the Melody valve in infants and children: the Italian experience.

AIMS: Paediatric patients with mitral valve (MV) disease have limited options for prosthetic MV replacement. Based on long experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, we aimed to test the use of the Melody valve as a surgical implant in the mitral position in a small group of infants with congenital mitral disease. METHODS AND RESULTS: Eight patients, aged from 3 months to 6.2 years, with congenital MV dysplasia underwent Melody valve implantation in the mitral position between March 2014 and October 2015. Once sutured to the MV annulus, the Melody valve was expanded through a balloon catheter to achieve the best diameter (12-20 mm). Two patients needed ECMO assistance after traditional MV repair and a Melody valve was successfully implanted as possible bail-out. However, neither of them recovered from multi-organ failure and both died, in spite of the well-functioning Melody prosthesis. During follow-up, only one patient underwent catheter-based balloon expansion of the valve. CONCLUSIONS: The medium-term results of this procedure are encouraging. The Melody valve opens up the opportunity to carry out MV replacement in more children at an earlier time point, and it has potential to revolutionise the treatment of MV disease.

Partial abnormal drainage of superior and inferior caval veins into the left atrium: two case reports.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Echocardiographic assessment after surgical repair of tetralogy of fallot.

Surgical correction of tetralogy of Fallot is still one of the most frequently performed intervention in pediatric cardiac surgery, and in many cases, it is far from being a complete and definitive correction. It is rather an excellent palliation that solves the problem of cyanosis, but predisposes the patients to medical and surgical complications during follow-up. The decision-making process regarding the treatment of late sequel is among the most discussed topics in adult congenital cardiology. In post-operative Fallot patients, echocardiography is used as the first method of diagnostic imaging and currently allows both a qualitative observation of the anatomical alterations and a detailed quantification of right ventricular volumes and function, of the right ventricular outflow tract, and of the pulmonary valve and pulmonary arteries. The literature introduced many quantitative echocardiographic criteria useful for the understanding of the pathophysiological mechanisms involving the right ventricle and those have made much more objective any decision-making processes.

Role of imaging in interventions on structural heart disease.

Recent technological progresses have led to the development of new devices and procedures which have greatly improved the chance to effectively treat structural heart diseases in both children and adults. Interventional cardiology has been receiving fast and wide implementation as an effective alternative treatment to surgery for several congenital and acquired diseases. The advent of transcatheter valve implantation/repair techniques constitutes one of the main breakthroughs of the last decades. Such development and implementation is strictly related to a continuous progress in cardiac imaging as well. Indeed, multimodality cardiac imaging (such as X-ray, echocardiography, MRI, multidetector computed tomography) has become essential in providing accurate patient selection and in monitoring the interventional procedures in order to optimize the success rate and minimize the frequency of complications. The current article aims at reviewing the role of multimodality imaging for planning and guiding interventions in several structural heart diseases.

Covered-stent implantation to treat aortic coarctation.

Aortic coarctation comprises approximately 7% of all known congenital heart defects. Surgery and balloon angioplasty have been performed for many years but are associated with a significant incidence of recoarctation and aneurysm formation. Although bare-stent implantation decreases the incidence of recoarctation, the risk of aortic dissection or aneurysm formation is not eliminated. Described initially to treat patients with coexistent aneurysm of the aortic wall, we currently believe that covered-stent implantation for aortic coarctation should play a more important role. Our increasing experience from expanding their indication to deal with complications from previous interventions, associated defects or particular anatomical situations has led us to conclude that most of cases of aortic coarctation in adolescents and adults should be treated by the implantation of covered stents.