Orthotopic heart transplantation in patient with situs inversus and pectus excavatum: a case report.

Background: Heart transplantation in patients with situs inversus is challenging, especially in terms of reconstruction of the systemic venous return. Several rerouting techniques have been presented but are associated with vulnerability to external compression, which might cause hemodynamic instability, especially in the presence of chest deformity. In this study, we report a rare case of successful heart transplantation in the presence of situs inversus and pectus excavatum.

Surgical repair of a residual lesion of mixed-type total anomalous pulmonary venous connection using a vertical vein as a free graft: a case report.

Background: Mixed-type total anomalous pulmonary venous connection is a rare condition in which the pulmonary veins possess multiple drainage sites. In the treatment of multiple lesions, it is sometimes necessary to treat only the main lesion first and repair additional residual lesions during follow-up.

Case Presentation: We report a case of additional repair of a remaining type Ia lesion in a 19-year-old woman with mixed-type total anomalous pulmonary venous connection who had previously been treated for a type IIa lesion alone.

Reconstruction of right ventricular outflow tract with severe calcification: lantern procedure.

Right ventricular outflow tract reconstruction is repeatedly required after the Rastelli procedure. However, standard right ventricular outflow tract reconstruction using direct anastomosis on the posterior right ventricular outflow tract wall is unfeasible in cases with severe calcification. Herein, we present a novel technique called the "lantern procedure," which can fix the prosthetic pulmonary valve without anastomosis to the calcified right ventricular outflow tract wall.

A Noonan-like pediatric patient with a de novo CBL pathogenic variant and an RNF213 polymorphism p.R4810K presenting with cardiopulmonary arrest due to left main coronary artery ostial atresia.

Left main coronary artery ostial atresia (LMCAOA) is an extremely rare condition. Here, we report the case of a 14-year-old boy with Noonan syndrome-like disorder in whom LMCAOA was detected following cardiopulmonary arrest. The patient had been diagnosed with Noonan syndrome-like disorder with a pathogenic splice site variant of CBL c.

Staged definitive repair for pulmonary atresia and ventricular septal defect 40 years after palliative surgery: a case report.

We present a very rare case of pulmonary atresia and ventricular septal defect with staged definitive repair more than 40 years after palliative surgery. The patient, a 43-year-old male, had undergone a Waterston operation at the age of one and had been untreated since then. Two years ago, he underwent an urgent surgery for impending rupture of a huge pulmonary artery aneurysm.

Usefulness of Prolonged PR Interval to Predict Atrial Tachyarrhythmia Development Following Surgical Repair of Tetralogy of Fallot.

Atrial tachyarrhythmias (ATAs), which may occur after tetralogy of Fallot (TOF) surgery, can cause sudden cardiac death. However, ATAs may also develop in response to electrical substrates. This study aims to examine the predictive factors for ATAs by identifying electrical substrates in the atrium obtained from 12-lead electrocardiogram in patients who underwent TOF repair.

Changes in Cerebral Hemodynamics During Systemic Pulmonary Shunt and Pulmonary Artery Banding in Infants with Congenital Heart Disease.

Palliative surgery is often performed in the treatment of congenital heart disease. Two representative palliative procedures are the systemic pulmonary shunt and pulmonary artery banding. Dramatic changes in cerebral hemodynamics may occur in these operations due to changes in the pulmonary-to-systemic blood flow ratio and systemic oxygenation.

Bronchial artery hypertrophy-associated perioperative pulmonary hemorrhage in cardiovascular surgery: a case report.

Background: Pulmonary hemorrhage is a life-threatening complication of cardiovascular surgery. Bronchial artery hypertrophy, a rare pathology associated with inflammatory and ischemic respiratory diseases, increases the risk of pulmonary hemorrhage; however, its involvement in cardiovascular surgery is not well known. We present two cardiovascular surgical cases in which embolization of the hypertrophied bronchial artery was effective in controlling perioperative pulmonary hemorrhage.

Perioperative junctional ectopic tachycardia associated with congenital heart disease: risk factors and appropriate interventions.

The risk factors and the appropriate interventions for perioperative junctional ectopic tachycardia (JET) in congenital heart disease (CHD) surgery have not been sufficiently investigated despite the severity of this complication. This study aimed to examine the risk factors and interventions for perioperative JET. From 2013 to 2020, 1062 surgeries for CHD (median patient age: 4.

Intralobar pulmonary sequestration associated with left main coronary artery obstruction and mitral regurgitation.

A 4-year-old boy with left intralobar pulmonary sequestration associated with left main coronary artery obstruction (LMCAO) and severe mitral regurgitation (MR) was admitted to our hospital. Since the patient presented with dyskinesia of the cardiac apex and increased left ventricular end-diastolic volume (LVEDV), left main coronary artery reconstruction and mitral annuloplasty were performed. The enlargement of the left ventricle was improved after sequential surgeries.

Left ventricular inflow obstruction due to a coronary arteriovenous fistula: a paediatric case report.

Background: We report a rare case of left ventricular inflow obstruction from a branch of the left circumflex coronary artery to the right atrium caused by a coronary arteriovenous fistula (CAVF) in a young Japanese male child.

Case Presentation: The patient was diagnosed with CAVF following a heart murmur shortly after birth. The left-to-right shunt caused right ventricular volume overload and pulmonary congestion.

Total Anomalous Pulmonary Venous Connection with Lethal Pulmonary Venous Obstruction Managed by Multidisciplinary Cooperation.

Background: Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease for which emergency surgery is required after birth. In cases of no intervention, TAPVC is associated with a high mortality rate in the first year of life. Although foetal echocardiographic techniques for diagnosing TAPVC have improved, TAPVC remains one of the most difficult congenital heart diseases to diagnose via foetal echocardiography.

Persistent Fifth Aortic Arch With Left Ventricular Dysfunction and Left Bronchial Obstruction.

Persistent fifth aortic arch (AA) is a rare anomaly in congenital heart disease, which is often associated with aortic obstructive diseases. We report a 7-month-old infant diagnosed with persistent fifth AA with left ventricular dysfunction along with left bronchial malacia due to compression from their own heart. Surgical repair was performed, including AA reconstruction using the fifth AA as an in situ flap to enlarge the fourth AA with end-to-end anastomosis, and external stenting for the left bronchial malacia.

Forns Index is a predictor of cardiopulmonary bypass time and outcomes in Fontan conversion.

Recent reports suggested that cardiopulmonary bypass (CPB) time is one of the risk factors for postoperative complications after Fontan conversion. Although Fontan conversion may be performed for the patients with hepatic fibrosis after initial Fontan procedure, there is no predictive indicator regarding the liver function associated with hemostasis which can affects CPB time. Thirty-one patients who underwent Fontan conversion using the same surgical procedure (extracardiac conduit conversion with right atrium exclusion) were enrolled.

Effects of blood flow dynamics on autologous pericardial degeneration in reconstructed pulmonary arteries.

Objectives: To clarify the risk factors for abnormal degeneration of autologous pericardium, the mechanical stress on the endothelial tissue caused by turbulent blood flow in the pulmonary artery (PA) reconstructed with autologous pericardium and major aortopulmonary collateral arteries was assumed in pulmonary atresia and ventricular septal defect.

Methods: Patient-specific PA models were created for 6 patients based on their past computed tomography images taken after unifocalization of major aortopulmonary collateral arteries. Computational fluid dynamics models were created to simulate the physiological pulsatile flow including the peripheral reflection wave, characteristic impedance and autonomous regulation system.

First Successful Clinical Application of the In Vivo Tissue-Engineered Autologous Vascular Graft.

Purpose: The ideal material for pediatric pulmonary artery (PA) augmentation is autologous pericardium. However, its utility for multistaged operations is limited. In this study, we applied an in vivo tissue-engineered autologous Biotube graft to a patient with congenital heart disease for the first time.

A Novel Surgical Technique for Right-Sided Interrupted Aortic Arch by Interposition of a Pulmonary Autograft Tube.

Right-sided interrupted aortic arch (IAA) is a rare cardiac anomaly. In general, the right bronchus sits higher than the left bronchus, so aortic arch reconstruction with a direct anastomosis has a risk of tracheal and bronchial obstruction. This report describes the successful definitive repair of a right-sided IAA in a 2.

Smaller-Sized Expanded Polytetrafluoroethylene Conduits With a Fan-Shaped Valve and Bulging Sinuses for Right Ventricular Outflow Tract Reconstruction.

Background: One of the critical factors limiting conduit longevity in right ventricular outflow tract (RVOT) reconstruction with homografts and xenografts is the small size of the conduit. The aim of our study was to assess the outcome of using smaller-sized expanded polytetrafluoroethylene (ePTFE) conduits with a fan-shaped valve and bulging sinuses for RVOT reconstruction.

Methods: This retrospective review examined 303 patients who underwent RVOT reconstruction with ePTFE conduits at 63 Japanese hospitals between 2003 and 2014.

Revisiting the supramolecular organization of photosystem II in Chlamydomonas reinhardtii.

Photosystem II (PSII) is a multiprotein complex that splits water and initiates electron transfer in photosynthesis. The central part of PSII, the PSII core, is surrounded by light-harvesting complex II proteins (LHCIIs). In higher plants, two or three LHCII trimers are seen on each side of the PSII core whereas only one is seen in the corresponding positions in Chlamydomonas reinhardtii, probably due to the absence of CP24, a minor monomeric LHCII.

Distinct physiological responses to a high light and low CO2 environment revealed by fluorescence quenching in photoautotrophically grown Chlamydomonas reinhardtii.

Mechanisms for countering environmental stress are essential to photosynthetic organisms. Alteration of the photosynthetic apparatus, a mechanism for balancing the flux of light energy and carbon fixation, can be characterized by fluorescence properties. In this study, we have established a simple protocol to determine the extent of energy-dependent quenching (qE) and quenching by state transition (qT) in Chlamydomonas cells by examining their fluorescence properties under light fluctuations.