Use of oversized injectable valves in growing children for total repair of right ventricular outflow tract anomalies (preliminary results).

Right ventricular outflow tract surgery was originally confined to transannular patching, in the belief that pulmonary regurgitation was well tolerated. Because follow-up evaluations revealed the deleterious effects of pulmonary regurgitation, surgery today aims to spare or replace the valve. Available replacement devices have short lifetimes, considering growth mismatch in children. We hypothesize that oversizing the right infundibulum anticipates growth and that a squeezed prosthesis can complete the expansion process. The No-React® Injectable BioPulmonic Valve is designed for right infundibular surgery in adults, and hundreds of implants have shown promising results. We used this device for surgery in babies, with the addition of an innovative oversizing technique. This study evaluates our preliminary results and investigates whether such a technique might reduce growth mismatch. From September 2010 through July 2012, we implanted 11 injectable pulmonic valves. The median age of our patients was 23 months. After opening the right infundibulum, we enlarged it as much as possible with a wide patch. Before completing the patch suture, we injected an oversized valve. No problems occurred during surgery. No major insufficiency or leak was observed. We conclude that prostheses can be quite oversized and perform well even when not completely expanded. Oversized injectable pulmonic valves, shrunken to a smaller diameter, enabled the implantation of a device wider than otherwise possible, without affecting performance. Moreover, the prosthesis tended to return to its original size following growth, thereby reducing growth mismatch. Longer follow-up and larger numbers of patients are needed for verification.

Repair of an unusual aortic coarctation using an extracellular matrix patch.

The surgical treatment of neonatal aortic coarctation is usually accomplished with a termino-terminal anastomosis or a subclavian flap. The use of a patch to enlarge the isthmal narrowing may be an alternative but is frequently complicated by aneurysmal dilatation on the aortic wall opposite to the patch, probably because it disrupts the vascular anatomic integrity. Extracellular matrix patches promise to restore the original tissue structure and could therefore be a valid alternative to other materials. We describe an aortic coarctation with an uncommon anatomic aspect treated with a CorMatrix (CorMatrix, Alpharetta, GA) extracellular matrix patch.

Rescue mitral valve repair one hour after birth.

Congenital mitral regurgitation is rare and usually part of complex cardiac anomalies. When needed, early surgery represents a great challenge. In small babies avoiding valve replacement is desirable, but valve repair may be extremely complex. We describe an isolated congenital mitral regurgitation, successfully treated with conservative surgery about 1 h after birth. In a 30-year-old pregnant woman, fetal echocardiography revealed mitral annular dilatation with massive regurgitation, functional aortic atresia and a very small patent foramen ovale. Realizing that the baby had a poor chance of survival after birth, a cesarean section was scheduled at 37 weeks of pregnancy. The procedure was performed in the operating room next to the cardiac surgery theatre, where the newborn was urgently transferred. After an unsuccessful attempt of percutaneous atrial septostomy, a rescue surgical mitral repair was performed. To avoid mitral replacement, moderate residual regurgitation was accepted. Postoperative hospital stay was 57 days and the baby was discharged in good clinical condition. Residual mitral regurgitation was moderate at discharge and decreased thereafter. During a five-year follow-up the child remained asymptomatic with normal growth. Preserved ventricular function and progressive volume reduction of left heart chambers were observed.

Pulmonary valve replacement with mechanical prosthesis: long-term results in 4 patients.

Pulmonary valve replacement is rarely performed and is usually done with biological prostheses or grafts. The use of mechanical prostheses is still a debated issue. We analyzed the outcome of 4 patients at 11 years after pulmonary valve replacement with a mechanical prosthesis. During follow-up, all patients remained asymptomatic, with no arrhythmias and good anticoagulation. An echocardiogram showed preserved ventricular contraction and normal function of the prostheses, with low pressure gradients. No infective endocarditis occurred. Mechanical prostheses appear to be a good choice for pulmonary valve replacement. Surgical and clinical results were satisfactory with no complications, acceptable pressure gradients, and good ventricular function after more than 10 years. Lifelong anticoagulation is mandatory, but this is usually well tolerated.

Asymptomatic inflammatory myofibroblastic tumor of the heart: immunohistochemical profile, differential diagnosis, and review of the literature.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is an uncommon lesion, mainly occurring in children and young adults and extremely rare in the heart. IMTs are composed of differentiated myofibroblastic cells accompanied by inflammatory cells. Cardiac IMTs are considered biologically benign, but they may have fatal consequences depending upon the peculiarity of site. Because of their rarity in the heart, most knowledge is based on extracardiac lesions that have uncertain behaviour. METHODS AND RESULTS: We investigated the morphologic features and the immunohistochemical profile of an intracardiac IMT, arising in the right outflow tract of an asymptomatic 11-month-old boy, by using a large panel of antibodies, many of them previously reported in extracardiac IMTs only. Results were compared with data of literature. After complete surgical excision of the tumor, the patient is disease-free at 1 year of follow-up. CONCLUSIONS: The present case showed morphologic and immunohistochemical features characteristic of IMT. Immunohistochemistry was helpful for characterization and differential diagnosis. The immunoreactivity pattern (including calponin expression) was similar to that of extracardiac IMTs except for anaplastic lymphoma kinase 1 immunoreactivity, lacking in this benign intracardiac IMT but usually associated to favourable prognosis in extracardiac IMTs.

Aortic coarctation: an overview.

In severe aortic coarctation in the neonatal period, surgical repair is required soon after clinical stabilization. Elective repair of isolated aortic coarctation is nowadays indicated at 3-6 months of life or at the time of diagnosis. At present, no single operation appears to have a clear superiority. However, during the first months of life, an extended end-to-end anastomosis is considered the best option by most authors, even though weight at operation and anatomy of the aortic arch are also significant determinants of late recoarctation. In cases of aortic arch hypoplasia, which occurs in up to 70% of neonatal and infant coarctations, especially when associated anomalies are present, surgery seems the treatment of choice. After 3 months of age and in the adult population, balloon angioplasty and stent placement are considered a suitable option. Recently, we adopted a median sternotomy approach without the use of extracorporeal circulation for the treatment of aortic coarctation with a hypoplastic aortic arch. We treated 11 patients with satisfactory results at an average follow-up of 40 months.

Partial hepatic vein diversion in pulmonary arteriovenous malformations in congenital heart disease.

The late appearance of pulmonary arteriovenous malformations after operations for complex congenital cardiac conditions may be explained in many ways. The necessity of hepatic blood flow to the lungs is now generally accepted for carrying some labile substance that is secreted by the liver and that has not yet been identified. Rerouting of hepatic blood to the lungs in cardiac patients can be accomplished with different methods, most of which are predisposed to thrombosis because of the slow flow in the tunnel. My colleagues and I describe a case of partial diversion of hepatic blood into the pulmonary circulation and suggest a strategy to decrease the likelihood of conduit thrombosis and to treat pulmonary arteriovenous malformations.

Securing atrial lines with Silastic ligatures.

We present a safe and easy-to-apply method of securing monitoring atrial cannulae after pediatric cardiac operations. We used Silastic ligatures together with purse strings on the tip of the atrial appendages in more than 350 cases, in a 5-year period. We never reported malfunctioning during their use, and no bleeding or rupture complication was detected by echocardiographic control after withdrawal.