The CXCL10-CXCR3 axis plays an important role in Kawasaki disease.

The precise pathogenesis of Kawasaki disease remains unknown. In an attempt to elucidate the pathogenesis of KD through the analysis of acquired immunity, we comprehensively examined the immunophenotypic changes in immune cells such as lymphocytes and monocytes along with various cytokines, focusing on differences between pre- and post- treatment samples. We found high levels of CXCL9 and CXCL10 chemokines that decreased with treatment, which coincided with a post-treatment expansion of Th1 cells expressing CXCR3. Our results show that the CXCL10-CXCR3 axis plays an important role in the pathogenesis of KD.

Nontraditional placement of an implantable cardioverter-defibrillator in a heterotaxy patient after the completion of total cavopulmonary connection.

A 15-year-old boy with heterotaxy syndrome developed a prolonged QT interval and intractable torsade de pointes after the administration of sodium channel blockers for atrial tachyarrhythmia. Although this situation called for the placement of an implantable cardioverter-defibrillator, a conventional transvenous approach was not available since the patient had previously undergone a nonfenestrated extracardiac total cavopulmonary connection. We were urged to carry out the surgical placement of an epicardial lead for an implantable cardioverter-defibrillator using a single coil transvenous shock lead through re-do midline sternotomy. Here we describe the details of this nontraditional surgical procedure for the placement of a lead for an implantable cardioverter-defibrillator in a case without venous access into the heart.

Tachycardia associated with twin atrioventricular nodes in an infant with heterotaxy and interruption of inferior vena cava.

We report a 12-month-old boy with heterotaxy and interruption of inferior vena cava who showed sustained tachycardia associated with twin atrioventricular nodes (AVNs). Atrioventricular reciprocating tachycardia with antegrade conduction through the posterior AVN and retrograde conduction through the anterior AVN were successfully ablated using an upper approach from the left internal jugular vein.

Staged repair of pentalogy of Cantrell with ectopia cordis and ventricular septal defect.

Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell.

Comparison of PR intervals determined by fetal magnetocardiography and pulsed Doppler echocardiography.

OBJECTIVE: In clinical practice, measurement of mechanical PR interval (mPR) with pulsed Doppler echocardiography is a standard method used to estimate the atrioventricular conduction time in the fetus. However, fetal echocardiography does not directly reflect the electrical properties of the heart. Technological advances in fetal magnetocardiography (fMCG) have allowed recording of the electrical PR interval (ePR) with high time resolution. The aim of this study was to clarify the differences between ePR and mPR. METHODS: The study subjects were 295 normal human fetuses (gestational age, range 20.4-41.4 weeks) who underwent fMCG, and 135 of them underwent fetal echocardiography 15-90 min before or after fMCG. The ePR was measured using the fMCG, and the mPR was determined by two pulsed Doppler methods, simultaneous recording of the left ventricular inward and outward flow (LV in/out) (n = 135) and superior vena cava and ascending aorta (SVC/aAo) (n = 84). RESULTS: The ePR showed a significant, but weak, positive correlation with gestational age (r = 0.162, p = 0.0053). The mPR was significantly longer than the ePR (p < 0.0001), with mean differences of 14.6% (95% limits of agreement -10.7, 39.9) for the LV in/out method and 14.7% (95% limits of agreement -8.6, 38.0) for the SVC/aAo method. CONCLUSION: Our results point to the risk of overestimation of the atrioventricular conduction time when the mPR is used, and the need for careful interpretation of PR prolongation determined by mPR.

Focal atrial tachycardia originating from inside the inferior vena cava late after surgical repair of congenital heart defects.

An 11-year-old girl presented with focal atrial tachycardia (AT) originating from inside the inferior vena cava (IVC). Sustained AT appeared 10 years after surgical repair of the congenital heart defects. Focal AT was successfully eliminated with radiofrequency application at the earliest activation site, about 8 mm below the IVC right atrium junction. These findings suggested the existence of an electrically continuous myocardial extension in the IVC and a real risk of AT originating from IVC late after cardiopulmonary bypass with IVC cannulation.

A novel case of congenital hepatic arterio-veno-portal shunts with umbilical vein aneurysm.

A term male infant presented with congenital hepatic arterio-veno-portal shunts. A mass-like lesion in the left lobe of the liver received blood supply from not only the umbilical vein, but also the hepatic and inferior intrahepatic arteries, communicating with the hepatic and portal veins in a complicated manner, with an umbilical vein aneurysm. The blood flow of the arterio-veno-portal shunts spontaneously and gradually declined from the neonatal period to six years of age. Although mild high-output cardiac failure had developed, no life-threatening events or health problems originating from portosystemic shunts, such as pulmonary artery hypertension and hepatopulmonary syndrome, were observed. However, this report shows that scrupulous follow-up to identify pulmonary artery hypertension and hepatopulmonary syndrome should be continued because complete resolution of the arterio-veno-portal shunts was not obtained in this case.

Abdominal aortic thrombus formation in a neonate with an interrupted aortic arch.

We note the risk of paradoxical embolism in patients with congenital heart defects with a right-to-left shunt. These patients should be managed to ensure that abdominal aortic thrombi are not overlooked when their clinical conditions change.

Isolation of pulmonary vein and superior vena cava for paroxysmal atrial fibrillation in a young adult with left ventricular non-compaction.

We report a 19-year-old male patient with left ventricular non-compaction who presented with atrial fibrillation (AF) and ventricular tachycardia. Ventricular tachycardia was induced by AF with rapid ventricular response, but was prevented by electrical isolation of the pulmonary veins and superior vena cava.

A modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty for interrupted or hypoplastic aortic arch.

BACKGROUND: Surgical repair for hypoplastic aortic arch in neonates carries a substantial risk of recurrent obstruction. Simple arch anastomosis is not always a solution in cases of extended arch hypoplasia. We present our modified technique of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. METHOD: We describe two neonates: interrupted aortic arch and transverse arch hypoplasia associated with aortic coarctation, who underwent a modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. RESULTS: The patients recovered without any pressure gradient at the anastomotic site. Postoperative aortography showed no arch obstruction and they successfully underwent second stage repair. CONCLUSION: Our technique provides extensive augmentation of the aortic arch with a tension-free, wide and non-circumferential suture line which preserves potential for growth. The technique described may avoid persistent or repeat arch obstruction.

Mexiletine infusion challenge test for neonatal long QT syndrome with 2:1 atrioventricular block.

For applying a genotype-based treatment in neonatal long QT syndrome (LQTS), early detection of the genotype becomes an important issue. We report a case of a neonate with LQTS type 3 that presented with 2:1 atrioventricular block and underwent a mexiletine infusion challenge test, and achieved shortening of the QTc and 1:1 atrioventricular conduction. The mexiletine infusion challenge test was helpful to make an early detection of the genotype of the LQTS and predicted the drug efficacy in a neonatal patient.

Prenatal Diagnosis of Atrioventricular Block and QT Interval Prolongation by Fetal Magnetocardiography in a Fetus with Trisomy 18 and SCN5A R1193Q Variant.

We report a case of fetal trisomy 18 with SCN5A R1193Q variant that presented with sinus bradycardia, 2 : 1 atrioventricular block (AVB), and QT interval prolongation. These complex arrhythmias were diagnosed by fetal magnetocardiography combined with ultrasound findings. Advanced AVB and ventricular arrhythmias were confirmed after birth. Genetic testing of the baby revealed a SCN5A R1193Q variant, which we considered could account for the various arrhythmias in this case.

Evaluation of blood rheology in patients with cyanotic congenital heart disease using a microchannel array flow analyzer.

Blood hyperviscosity due to secondary erythrocytosis is a common pathologic feature of cyanotic congenital heart disease (CCHD). In CCHD, it is possible that hematological parameters other than red blood cells influence blood rheology. We measured blood passage time to evaluate the blood rheology in patients with CCHD (n=18, age: 15.3+/-11.9 years, mean +/- SD) and age-matched control subjects (n=27) using the microchannel array flow analyzer (MC-FAN), and the results [several hematological parameters, including hematocrit (Hct)] were compared. Blood passage time in the CCHD group was prolonged, compared with the control group (67.6+/-27.2 s vs. 44.6+/-6.7 s). For the CCHD group, blood passage time correlated significantly with red blood cell (RBC) count, hemoglobin (Hb) concentration, Hct, mean corpuscular hemoglobin concentration (MCHC), platelet (Plt) count, high-density lipoprotein cholesterol (HDL-C) level, and triglycerides (TG) level (RBC, r=0.77; Hb, r=0.69; Hct, r=0.73; MCHC, r=-0.64; Plt, r=-0.49; TG, r=0.53; HDL-C, r=-0.49, p<0.05 for each variable). For all 45 subjects, blood passage time correlated significantly with HbA1c level (r=0.45, p<0.01) and tissue-type plasminogen activator (t-PA) antigen level (r=0.46, p<0.01). Our results indicated that blood rheology is reduced in patients with CCHD as expressed by prolonged blood passage time, and it may be defined by several blood parameters in addition to erythrocytosis.

Replacement of the common atrioventricular valve with floating annuloplasty in a patient with univentricular physiology.

Significant common atrioventricular valve insufficiency is associated with increased mortality and morbidity in patients with univentricular physiology. Shortly after bidirectional cavopulmonary anastomosis and concomitant valvuloplasty, valve replacement was carried out using a floating annuloplasty technique reinforced by a Dacron strip in a patient with common atrioventricular valve insufficiency. The technique applied is described in detail.

Palliative open heart surgery in an infant with factor VII deficiency.

An infant with factor VII deficiency underwent palliative open heart surgery for pulmonary atresia with an intact ventricular septum. No references had been found on the management of this rare coagulation disorder in infantile cardiac surgery. We describe the peri- and postoperative management with a replacement therapy including a recombinant factor VIIa concentrate. We conclude that an appropriate replacement therapy is needed to control bleeding during open heart surgery with factor VII deficiency.