Liver T1 mapping in Fontan patients and patients with biventricular congenital heart disease - insights into the effects of venous congestions on diffuse liver disease.

T1 relaxation time quantification on parametric maps is routinely used in cardiac imaging and may serve as a non-invasive biomarker for diffuse liver disease. In this study, we aimed to investigate the relationship between liver T1 values and cardiac function in patients with congenital heart disease (CHD) and compared patients with a biventricular circulation (BVC) to those with a Fontan circulation (FC). Magnetic resonance images from patients with CHD, obtained between June and December 2023 on a 1.

The neo-aortic valve in patients with hypoplastic left heart syndrome is largely preserved: a serial follow-up CMR study.

Background: In hypoplastic left heart syndrome (HLHS) patients, neo-aortic valve regurgitation can negatively impact right ventricular (RV) function. We assessed neo-aortic valve function and RV volumetric parameters by analysing serial cardiovascular magnetic resonance (CMR) studies in HLHS patients after completion of total cavopulmonary connection (TCPC).

Methods: Consecutive CMR examinations of 80 patients (female: 22) with two ( = 80) or three ( = 45) examinations each were retrospectively analysed.

Surgical treatment for a child with traumatic ventricular septal rupture and ventricular aneurysm.

A five-year-old boy was diagnosed with the ventricular septal rupture and ventricular aneurysm after blunt chest trauma in child abuse. Because of the intractable heart failure, he underwent operation in subacute period. Postoperative course was uneventful.

Influence of the bronchus on the vascular growth of major aortopulmonary collateral arteries.

Objectives: Patients with major aortopulmonary collateral arteries (MAPCAs) often require additional surgical or catheter intervention after unifocalization (UF) due to stenosis and poor growth. We hypothesized that the UF design influences vascular growth; assessment was based on the passing route related to the bronchus.

Methods: We enrolled 5 patients with pulmonary atresia (PA), ventricular septal defect and MAPCA who underwent UF and subsequent definitive repair at our institute from 2008 to 2020.

Risk analysis for patients with a functionally univentricular heart after systemic-to-pulmonary shunt placement.

Objectives: To investigate risk factors for mortality after systemic-to-pulmonary (SP) shunt procedures in patients with a functionally univentricular heart using the Japan Cardiovascular Surgery Database registry.

Methods: Clinical data from 75 domestic institutions were collected. Overall, 812 patients with a functionally univentricular heart who underwent initial SP shunt palliation were eligible for analysis.

A case of aggressive aortic prosthetic valve endocarditis aggressive caused by Staphylococcus lugdunensis.

Background: Staphylococcus lugdunensis is a coagulase-negative Staphylococcus species, which are weak pathogenic bacteria generally. However, the acute and severe pathogenicity of Staphylococcus lugdunensis infective endocarditis may be due to the rapid growth of large vegetation and consequent valve destruction.

Case Presentation: The patient was an 81-year-old male who visited our hospital with chief complaints of low back pain and high fever.

[Valve-sparing Root Reimplantation for Stanford Type A Acute Aortic Dissection Combined with Aortic Root Dilation and Bicuspid Aortic Valve;Report of a Case].

A 45-year-old male developed Stanford type A acute aortic dissection combined with aortic root dilation and congenital bicuspid aortic valve (BAV). He had a Sieveres type 0 BAV, lateral subtype with right and left cusps. Valve-sparing root reimplantation was performed with decalcification of the cusps.

Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection.

Objectives: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy.

Methods: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.

[Valve-sparing Replacement in Patients with Aortic Root Dilatation].

Valve-sparing root replacement is increasingly used to overcome drawbacks associated with valvular prostheses. In our institution, 7 patients underwent valve-sparing root replacement from August 2016 to July 2017. The mean age was 45 years (range, 14~69 years).

One-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation.

[Perioperative Care for Patients with Hypoplastic Left Heart Syndrome].

Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago.

Long-term outcome of patients with right atrial isomerism after common atrioventricular valve plasty.

Objectives: To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty.

Methods: We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups.

A successful staged Fontan operation for a right atrial isomerism neonate having major aortopulmonary collateral arteries and extracardiac total anomalous pulmonary venous connection.

Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC.

Modification of hemi-Fontan operation for patients with functional single ventricle and anomalous pulmonary venous connection to the superior vena cava: mid-term results.

Objectives: Fontan candidates with mixed totally anomalous pulmonary venous connection often have postoperative pulmonary venous obstruction after cavopulmonary anastomosis. Because some pulmonary venous obstructions have no intimal hypertrophy at reoperation, we considered such pulmonary venous obstructions to be caused by 3D deformities arising from dissection or mobilization of the vessels, and hypothesized that keeping the pulmonary venous branches in a natural position could avoid such obstruction. Here, we evaluated a modified hemi-Fontan strategy consisting of minimal dissection with no division of vessels and patch separation between systemic and pulmonary venous flow.

[Current Surgical Treatment for Patients with Hypoplastic Left Heart Syndrome, from the Viewpoint of Emergent Operation].

Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. Since the 1st successful surgical repair was reported by Norwood et al. more than 30 years ago, some modifications in surgical maneuver [e.

Efficacy of the 'intrapulmonary-artery septation' surgical approach for Fontan candidates with unilateral pulmonary arterial hypoplasia†.

Objectives: The bilaterally unbalanced development of pulmonary arteries (PAs), as a result of unilateral pulmonary arterial hypoplasia (PAhypo) makes patients either ineligible for the Fontan operation or candidates for the one-lung Fontan operation. In the present study, we examined the efficacy of intrapulmonary-artery septation (IPAS), a technique we reported in 2007 in which a septation is constructed within the central PA, in patients with unilateral PAhypo.

Methods: Sixteen patients with unilateral PAhypo and an affected PA index of ≤60 mm(2)/m(2), including non-confluent PA (NCPA), underwent IPAS between January 2000 and March 2012; patients with pulmonary venous obstruction were excluded from this study.

Primary central pulmonary artery plasty for single ventricle with ductal-associated pulmonary artery coarctation.

Background: Infants with a single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA). We hypothesized that primary PA plasty in the first palliation would limit PA stenosis and unbalanced pulmonary perfusion, and thereby facilitate safe accomplishment of the next operation.

Methods: From 1998 to 2012, functional single-ventricle patients with pulmonary atresia were managed as follows: initial operative strategy based on midline approach, using cardiopulmonary bypass, and central PA plasty (resection of all ductal tissue).

Mid- to long-term aortic valve-related outcomes after conventional repair for patients with interrupted aortic arch or coarctation of the aorta, combined with ventricular septal defect: the impact of bicuspid aortic valve†.

Objectives: Bicuspid aortic valve (BAV) is a common risk factor for valve-related problems and occurs more frequently in patients with an interrupted aortic arch (IAA) or coarctation of the aorta (CoA), combined with a ventricular septal defect (VSD), than in the general population. We have been using conventional repair for patients with IAA/CoA+VSD, including those with a very small aortic valve (AV). We retrospectively investigated the outcomes of these patients from the perspective of valve morphology.

Modified Nikaidoh procedure with double-root translocation in a 1-year-old boy.

A 1-year-old boy weighing 10.4 kg underwent successful biventricular repair for transposition of the great arteries, a ventricular septal defect, and a left ventricular outflow tract (LVOT) obstruction with moderate pulmonary stenosis of the bicuspid pulmonary valve (z score of -4.4 for the pulmonary valve) by means of a modified Nikaidoh procedure with double root translocation by use of a valve-spared pulmonary root.

Is routine rapid-staged bilateral pulmonary artery banding before stage 1 Norwood a viable strategy?

Objective: We adopted a policy of rapid-staged bilateral pulmonary artery banding (bPAB) before the Norwood (NW) procedure for all patients with hypoplastic left heart syndrome. We hypothesized that this strategy might mitigate some of the traditional risk factors and that postponing a major bypass procedure beyond the newborn period could have both short- and long-term benefits. The purpose of the present study was to evaluate the efficacy of this strategy with respect to the short-term outcomes.

Technical modification enabling pulmonary valve-sparing repair of a severely hypoplastic pulmonary annulus in patients with tetralogy of Fallot.

Objectives: Although pulmonary valve-sparing repair is preferable for patients with tetralogy of Fallot, the repair of very small pulmonary valves is challenging. The present study evaluates our modification for preserving severely hypoplastic pulmonary valves in patients with tetralogy of Fallot.

Methods: Sixty-eight consecutive patients who underwent complete repair of a tetralogy of Fallot between 2005 and 2011 were retrospectively reviewed.

Impact of postoperative hemodynamics in patients with functional single ventricle undergoing Fontan completion before weighing 10 kg.

Background: Although the Fontan procedure is now being performed in younger patients, the influence of the early timing of Fontan on midterm to long-term results remains unclear. We investigated whether the timing of Fontan completion affects subsequent hemodynamics in patients with functional single ventricle followed for more than 3 years.

Methods: Between January 1997 and December 2008, 163 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) at a single institution.