repair of congenital mitral valve dysplasia in infants and children is it always possible

Objectives: Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. Methods: Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days‐18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). Results: Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months‐12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. Conclusions: Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses. q 2000 Elsevier Science B.V. All rights reserved

Aortic stenting in the growing sheep causes aortic endothelial dysfunction but not hypertension: Clinical implications for coarctation repair

Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%-40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension. Therefore, we aimed to study the hemodynamic and structural impact following stent implantation in the normal aorta of a growing animal

Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation

Elevated Tricuspid Regurgitant Velocity (TRV) has been related to higher mortality in adults and to hemolysis, lower oxygen saturation during 6-minute walk test and acute chest syndrome (ACS) in children with sickle cell disease (SCD). Hydroxyurea (HU) has reduced TRV value in children and adults. We describe a three year old HbSS child with recurrent ACS, hypoperfusion of the left lung, mild hemolysis and persistent TRV elevation. TRV did not normalize after HU, despite improvement in clinical conditions and in baseline laboratory parameters related to hemolysis and blood viscosity, but normalized after bone marrow transplantation (BMT). Our experience suggests that in young patients, TRV reduction can be a positive concomitant effect of BMT

A concert in the heart. Bilateral melody valve implantation in the branch pulmonary arteries.

Patients undergoing right ventricular outflow tract reconstruction are subject to valve and cusp degeneration later in life, requiring further intervention to alleviate the pulmonary regurgitation that ensues. In these cases, percutaneous pulmonary valve implantation can be an effective alternative to surgery; however, since the indications have been limited to dysfunctional valved conduits, only a small number of patients have access to this treatment option. We present the case of an 18-year-old male with a patch-enlarged right ventricle to pulmonary artery conduit who underwent pulmonary valve implantation using two Melody transcatheter pulmonary valves (Medtronic, Inc) into the proximal right and left branch pulmonary arteries

Misleading echocardiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery

A 1-year-old boy was referred to our hospital for further investigation of mitral regurgitation. Color-Doppler echocardiography revealed the site of the anomalous origin of the left coronary artery which was confirmed by a cineangiographic study. This case is interesting for several reasons: the first is that the anomalous left coronary artery originated from the right pulmonary artery; the second is that two-dimensional echocardiographic findings can be misleading, i.e., a vessel-like structure which seemed to be the left coronary artery arising normally from the aorta was visualized; and the last is that a two-dimensional color-Doppler echocardiogram was useful in the diagnosis of the anomalous origin of the left coronary artery. The patient underwent implantation of the anomalous left coronary artery as well as Kay's plasty of the mitral valve, and has been doing well since then

Anomalous origin of the right coronary artery from the pulmonary artery associated with tetralogy of Fallot: description of the pre-surgical diagnosis and surgical repair.

IF 2009 1,183 Cardiol Young. 2011 Aug;21(4):468-70. Epub 2011 Mar 2. Anomalous origin of the right coronary artery from the pulmonary artery associated with tetralogy of Fallot: description of the pre-surgical diagnosis and surgical repair. Pluchinotta FR, Vida V, Milanesi O. Source Pediatric Cardiology Unit, Department of Pediatrics, University of Padua Medical School, Via Giustiniani no. 3, Padua, Italy. Abstract Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital defect. We describe the case of an infant with anomalous origin of the right coronary artery from the pulmonary artery in association with tetralogy of Fallot. This patient had a pre-operative echocardiographic diagnosis, which was confirmed by angiography, and later underwent a successful surgical repair. PMID: 21362207 [PubMed - indexed for MEDLINE

Transapical aortic balloon valvuloplasty in a 890-gram infant: hybrid is better!

Catheter Cardiovasc Interv. 2011 Jan 1;77(1):112-4. doi: 10.1002/ccd.22754. Transapical aortic balloon valvuloplasty in a 890-gram infant: hybrid is better! Maschietto N, Vida V, Milanesi O. Source Department of Pediatrics, Pediatric Cardiology Unit, [corrected] University of Padova, Padova, Italy. [email protected] Erratum in \u2022 Catheter Cardiovasc Interv. 2011 Sep 1;78(3):491. Abstract Balloon aortic valvuloplasty has become in many centers the treatment of choice for neonates with critical or severe aortic stenosis. Usual approaches both antegrade and retrograde can be problematic in preterms extremely low birth weight babies. We describe a novel approach for dilating the aortic valve in an 890 grams baby. Copyright \ua9 2010 Wiley-Liss, Inc. PMID: 20824755 [PubMed - indexed for MEDLINE

Solitary aorta with bilateral ductal origin of non-confluent pulmonary arteries in pulmonary atresia with intact ventricular septum.

IF JCR 2007: 2.87