The effect of body weight in infants undergoing ventricular septal defect closure: A report from the Nationwide Japanese Congenital Surgical Database.

Objective: In infants with ventricular septal defect (VSD) who undergo surgical intervention, body weight, along with age, is frequently thought to be the decisive predictor of morbidity and mortality after surgery; however, its information on quantitative risk assessment is limited.

Methods: All infants (<1 year old) with a fundamental diagnosis of VSD who underwent surgical VSD closure or pulmonary artery banding between 2012 and 2016 were identified from the Japan Cardiovascular Surgery Database Congenital Section. The outcome of interest was a composite end point of all-cause death and major complications within 30 days after surgery.

Successful Total Pericardiectomy for Constrictive Pericarditis in the First Series of Japanese Patients With Mulibrey Nanism.

Patients with Mulibrey nanism (MUL) present with growth failure and multiple organ manifestations, and MUL is caused by mutations in TRIM37. In this article, we report on the first case series of Japanese patients with MUL who developed congestive heart failure due to constrictive pericarditis. Our case series suggests that early diagnosis and total pericardiectomy before adherence of the pericardium might provide clinical benefit and better prognosis for MUL.

Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report.

Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published.

Direct In Vivo Reprogramming with Sendai Virus Vectors Improves Cardiac Function after Myocardial Infarction.

Direct cardiac reprogramming holds great promise for regenerative medicine. We previously generated directly reprogrammed induced cardiomyocyte-like cells (iCMs) by overexpression of Gata4, Mef2c, and Tbx5 (GMT) using retrovirus vectors. However, integrating vectors pose risks associated with insertional mutagenesis and disruption of gene expression and are inefficient.

Palliative Mitral Valve Repair During Infancy for Neonatal Marfan Syndrome.

An infant with neonatal Marfan syndrome (nMFS), a condition that is nearly always lethal during infancy, was referred to our hospital with symptoms of congestive heart failure resulting from severe mitral valve insufficiency. During mitral valve repair, the use of an annuloplasty ring was waived until annular dilatation was achieved after 2 palliative mitral valvuloplasty procedures. After the definitive operation, the patient's mitral valve function remained within normal limits until the last follow-up when the patient was 11 years old.

Heterotopic transplantation of a decellularized and recellularized whole porcine heart.

Objectives: One of the final treatments for end-stage heart failure is heart transplantation. However, a shortage of donor hearts has created a long waiting list and limited benefits. Our ultimate goal is to create a whole beating heart fabricated on an organ scaffold for human heart transplantation.

Variable severity of cardiovascular phenotypes in patients with an early-onset form of Marfan syndrome harboring FBN1 mutations in exons 24-32.

A subgroup of patients with Marfan syndrome (MFS) who have mutations in exons 24-32 of the FBN1 gene manifests severe atrioventricular valve insufficiency and skeletal problems as early as the neonatal period. These patients usually die in the first 2 years of life, thus a region between exons 24 and 32 of FBN1 is recognized as a critical region for this neonatal form of MFS (nMFS). Here, we report five consecutive patients who manifested a cardiovascular phenotype until infancy with mutations in the critical region for nMFS.

Positional desaturation due to persistent left superior vena cava draining into the left atrium.

Persistent left superior vena cava (PLSVC) is a rare congenital anomaly whose prevalence is 0.3 % of general population. The majority of PLSVC drain into right atrium (RA) through the coronary sinus without clinical harm.

Fontan completion in a patient with previous liver transplantation.

We present the first case of a successful Fontan completion in a patient with previous liver transplantation. An infant with polysplenia syndrome with a functional single ventricle and biliary atresia had been surgically managed by pulmonary artery banding, Kasai operation and living donor liver transplantation. Subsequently, the patient successfully underwent bidirectional cavopulmonary shunt and total cavopulmonary connection with extracardiac conduit at 3 and 5 years of age, respectively.

MiR-133 promotes cardiac reprogramming by directly repressing Snai1 and silencing fibroblast signatures.

Fibroblasts can be directly reprogrammed into cardiomyocyte-like cells (iCMs) by overexpression of cardiac transcription factors or microRNAs. However, induction of functional cardiomyocytes is inefficient, and molecular mechanisms of direct reprogramming remain undefined. Here, we demonstrate that addition of miR-133a (miR-133) to Gata4, Mef2c, and Tbx5 (GMT) or GMT plus Mesp1 and Myocd improved cardiac reprogramming from mouse or human fibroblasts by directly repressing Snai1, a master regulator of epithelial-to-mesenchymal transition.

Negative pressure therapy for post-sternotomy wound infections in young children.

Objectives: Post-sternotomy wound infection remains a significant morbidity in congenital and paediatric cardiac surgery. However, the techniques used for this complication in children are not optimal in terms of mortality, morbidity and the use of medical resources. Negative pressure therapy is an effective modality in the treatment in adults, but reports of its use in children are limited.

Induction of human cardiomyocyte-like cells from fibroblasts by defined factors.

Heart disease remains a leading cause of death worldwide. Owing to the limited regenerative capacity of heart tissue, cardiac regenerative therapy has emerged as an attractive approach. Direct reprogramming of human cardiac fibroblasts (HCFs) into cardiomyocytes may hold great potential for this purpose.

Bilateral banding of malpositioned pulmonary artery branches.

This report presents two rare cases of malpositioned pulmonary artery branches managed by bilateral banding. The left-branch pulmonary artery, originating from and directly superior to the right branch, entered the posterior mediastinum before heading toward the left pulmonary hilum. The ostium of the left branch could not be visualized by midline sternotomy.

Bridging annuloplasty for left atrioventricular valve of partial atrioventricular septal defect.

Residual left atrioventricular valve regurgitation after conventional repair technique for partial atrioventricular septal defect most commonly occurs in the central position. We describe here the technique for bridging annuloplasty on the opposing anterior and posterior annuli of the atrioventricular valve in selected patients with short leaflets.

Open anastomosis of extracardiac conduit for total cavopulmonary connection decreases post-operative pleural effusion.

Objective: The goal of this study was to see whether the open anastomosis technique using vacuum-assisted venous drainage at the time of the Fontan procedure was associated with decreased post-operative pleural effusion.

Methods: We analysed a subgroup of patients with a functional single ventricle who underwent non-fenestrated total cavopulmonary connection completion with the insertion of an extracardiac conduit as the sole or predominant procedure conducted by a single surgeon at a single institute, using either an open or closed anastomosis technique.

Results: Median age and weight were 2.

Fenestrated exclusion of the right ventricle for tricuspid atresia and absent pulmonary valve.

The case of a boy who had an absent pulmonary valve, tricuspid atresia, intact ventricular septum, coronary-to-right-ventricular fistula, and dysplasia of the right ventricular free wall is described. At the bidirectional cavopulmonary shunt procedure, the right ventricular free wall was opened and two major fistula orifices to the cavity were closed with sutures. A fenestrated circular patch was placed in the main pulmonary artery and the right ventricular free wall was plicated.

Multislice computed tomography is useful for evaluating partial anomalous pulmonary venous connection.

Volume-rendered images, derived from multidetector-row computed tomography (MDCT), can facilitate assessment of the morphology of partial anomalous pulmonary venous connection and are thus useful in pre-operative planning to prevent surgical morbidity and assist post-operative evaluations.

Ross-Konno operation for patients with Shone complex.

We report the cases of 2 pediatric patients who had Shone complex. Each child had severe left ventricular outflow tract obstruction and marginal mitral obstruction, and both underwent the Ross-Konno operation. The mitral valve was left alone.

Aberrant left subclavian artery sparing in right-sided interrupted aortic arch repair.

A right-sided interrupted aortic arch is rare, and it may have an associated aberrant left subclavian artery (ALSCA). An ALSCA is most often sacrificed at biventricular repair despite the potential danger of poor left upper-extremity growth. We report a case of ALSCA preservation with extensive dissection during aortic arch repair using direct anastomosis.

Reconstruction of an isolated left subclavian artery in a patient with a right aortic arch and tetralogy of Fallot.

An isolated left subclavian artery (ILSA) is very rarely seen in patients with a right aortic arch. This report describes the case of a 2-year-old boy who underwent ILSA reconstruction during repair of the associated tetralogy of Fallot to prevent future subclavian steal syndrome after surgery.

A case report of surgical correction for congenital mitral regurgitation with subvalvular apparatus abnormality.

We report a successful complex mitral valve plasty using port access minimally invasive cardiac surgery for congenital mitral regurgitation that presented as an abnormality of the subvalvular apparatus. A 16-year-old male patient received a diagnosis of mitral regurgitation resulting from tethering of the anterior mitral leaflet and posterior mitral leaflet caused by an abnormality in papillary muscle insertion and a hypoplastic chordae tendineae. The posterior leaflet was closely tethered to the tips of the papillary muscle with essentially no chordae tendineae.

Aortic translocation with autologous tissue.

Aortic translocation, although technically demanding, could be an excellent surgical option for d-transposition of the great vessels and left ventricular outflow tract obstruction. We report a modification of the aortic translocation technique that uses autologous tissue. The aortic root is mobilized from the right ventricle with an extension of infundibular free-wall muscle for use in closure of the ventricular septal defect, which is similar to the technique for harvesting pulmonary autograft in the Ross-Konno procedure.

'Working' cardiomyocytes exhibiting plateau action potentials from human placenta-derived extraembryonic mesodermal cells.

The clinical application of cell transplantation for severe heart failure is a promising strategy to improve impaired cardiac function. Recently, an array of cell types, including bone marrow cells, endothelial progenitors, mesenchymal stem cells, resident cardiac stem cells, and embryonic stem cells, have become important candidates for cell sources for cardiac repair. In the present study, we focused on the placenta as a cell source.