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In extracardiac Fontan, an epicardial pacemaker implantation has many limitations, especially given that it is highly invasive and a high-risk procedure due to repeat thoracotomy. Herein we illustrate a case with the possibility of transvenous pacing in extracardiac Fontan being less invasive and lower risk transvenous dual-chamber pacemaker implantation by electrophysiological assessment. < Learning objective: Transvenous pacemaker implantation via left superior vena cava may be a viable alternative to epicardial pacing with a minimally invasive procedure in patients with extracardiac Fontan.> [ABSTRACT FROM AUTHOR]

The management of systemic artery aneurysms secondary to Kawasaki disease (KD) in adults remains a therapeutic challenge. KD guidelines recommend the use of anticoagulation therapy with warfarin in addition to antiplatelet therapy when a giant coronary aneurysm or a history of thrombosis is documented. However, long-term use of warfarin presents several concerns. This case reports acute thrombotic occlusion due to the giant arterial aneurysm in an adult KD. A surgical resection of the aneurysm was performed because of recurrent thrombotic events, despite anticoagulant therapy with warfarin. Pathological examinations revealed a layered thrombus with inflammation in the aneurysm and Factor Xa expression mainly in newly formed thrombus. This study provides an insight into the anticoagulation therapy for cardiovascular sequelae after KD. < Learning objective: This study, along with pathological evidence, illustrates that Factor Xa might contribute to thrombotic events after Kawasaki disease.> [ABSTRACT FROM AUTHOR]

We report the first case of transcatheter perimembranous ventricular septal defect (pmVSD) closure in Japan where none of existing devices for VSD closure has been approved. The pmVSD was successfully closed with first generation Amplatzer® duct occluder (ADO-I; St Jude Medical, St Paul, MN, USA). The procedure was performed under general anesthesia with transesophageal echocardiographic and fluoroscopic guidance. The left ventricular volume overload after the procedure was remarkably improved and no major complications occurred. ADO-I can be a safe and effective option for transcatheter pmVSD closure. The incidence of heart block may be less than reported with the original device. < Learning objective: Use of Amplatzer® duct occluder is effective in transcatheter perimembranous ventricular septal defect (pmVSD) closure in selected patients. It may be safer than original Amplatzer pmVSD occluder to avoid complete atrioventricular block.> [ABSTRACT FROM AUTHOR]

Background Stenting may be a compelling approach to dilating curved lesions in congenital heart diseases. However, balloon-expandable stents, which are commonly used for congenital heart diseases, are usually deployed in a straight orientation. In this study, we evaluated the effect of stenting with a novel curved balloon considered to provide better conformability to the curved-angled lesion. Materials and methods In vitro experiments: A Palmaz Genesis ® stent (Johnson & Johnson, Cordis Co, Bridgewater, NJ, USA) mounted on the Goku ® curve (Tokai Medical Co. Nagoya, Japan) was dilated in vitro to observe directly the behavior of the stent and balloon assembly during expansion. Animal experiment: A short Express ® Vascular SD (Boston Scientific Co, Marlborough, MA, USA) stent and a long Express ® Vascular LD stent (Boston Scientific) mounted on the curved balloon were deployed in the curved vessel of a pig to observe the effect of stenting in vivo. Results In vitro experiments: Although the stent was dilated in a curved fashion, stent and balloon assembly also rotated conjointly during expansion of its curved portion. Animal experiment In the primary stenting of the short stent, the stent was dilated with rotation of the curved portion. The excised stent conformed to the curved vessel. As the long stent could not be negotiated across the mid-portion with the balloon in expansion when it started curving, the mid-portion of the stent failed to expand fully. Furthermore, the balloon, which became entangled with the stent strut, could not be retrieved even after complete deflation. Conclusion This novel curved balloon catheter might be used for implantation of the short stent in a curved lesion; however, it should not be used for primary stenting of the long stent. Post-dilation to conform the stent to the angled vessel would be safer than primary stenting irrespective of stent length. [ABSTRACT FROM AUTHOR]

Abstract: In order to investigate the role of angiotensin-converting enzyme 2 (ACE2) in cardiac development, we examined the effects of ACE2 deficiency on postnatal development of the heart using ACE2-knockout (ACE2KO) mice. Heart samples of wild type (WT; C57BL/6J) mice and ACE2KO mice were taken at 1, 4, and 10 weeks of age. In WT mice, expression of ACE2 mRNA increased from 1 week to 10 weeks. A similar increase was observed in immunostaining of ACE2 in the heart, in which ACE2 was strongly expressed in coronary arteries. Compared with WT mice, heart weight was greater in ACE2KO mice at 4 weeks, and coronary artery thickening and perivascular fibrosis were also already enhanced from 4 weeks. Consistent with the increase of fibrosis, cardiac expression of collagen and TIMP was higher, and expression of MMP was lower in ACE2KO mice at 4 weeks. In addition, TGF-β mRNA was also higher, and lower expression of PPARγ mRNA was observed at 4 weeks in ACE2KO mice. These results suggest that ACE2 plays an important role in postnatal development of the heart, and that lack of ACE2 enhances coronary artery remodeling with an increase in perivascular fibrosis and cardiac hypertrophy already around the weaning period. [Copyright &y& Elsevier]

Open heart surgery for infants with low body weight (BW) remains still a challenge. Pulmonary artery banding (PAB) is a useful surgical palliation for small neonates and early infants with excessive pulmonary blood flow who are unable to withstand a heart surgery. This study retrospectively reviewed neonates and infants who underwent PAB to assess the surgical results and the validity of our PAB. We selected 38 acyanotic infants and neonates and divided them into 2 groups: low BW (< 2.5 kg, n = 15, group L) and normal or high BW (≥ 2.5 kg, n = 23, group NH). The average BW at the time of PAB was 2.8 ± 1.1 kg (range, 1.2-5.8 kg), and the average age at the time of PAB was 41.8 ± 44.8 days (range, 2-151 days). Using a 3-mm-wide polyester tape, we tightened the main pulmonary artery to obtain the circumference of (19 mm + 1 mm for each kg of BW). There was no early death but one late death in each group. Postoperative BW continuously increased 1 month after PAB in both groups, although BW was significantly lower in group L than in group NH. Intracardiac repair (ICR) was accomplished in 31 patients (13 in group L and 18 in group NH) at average ages of 1.5 years, while the remaining 5 patients are awaiting ICR. In conclusion, PAB using our formula for the infants even weighing < 2.5 kg has low mortality and is effective as a bridge to ICR. [ABSTRACT FROM AUTHOR]

Background: Electrocardiographic and molecular studies have clarified an association between sudden infant death syndrome (SIDS) and long QT syndrome (LQTS), and few data are available for the QT interval in infancy from birth to 1 year of age. Appropriate time of electrocardiographic screening is not clarified. Medical examinations during infancy are mandatory in Japan. Methods and Results: The study population included 1,058 infants. Electrocardiograms were collected with information of infants at birth and at examination. The QT intervals of three consecutive beats were measured in lead V5. Statistical analysis revealed that the following formula was appropriate to minimize the effect of heart rate for infants: corrected QT interval; QTc = QT interval/RR interval 0.43. Subjects were divided into four groups as follows: 0–2, 3–6, 6–11, and 12–52 weeks of age. Tukey''s multiple comparison showed that the QTc intervals were longest (p < 0:0001) in subjects who were 6–11 weeks of age. Conclusions: The QTc interval showed the highest peak at 6-11 weeks of age in infancy. The peak period of occurrence of SIDS is at approximately 2 months of age. An appropriate time of electrocardiographic screening for QT prolongation will be one month of age, and follow-up studies are needed. [Copyright &y& Elsevier]

Summary: The patient was a 37-year-old female who had undergone a repair for tetralogy of Fallot (TOF) at the age of 4 years. Postoperative pulmonary stenosis remained, but she continued to be managed medically. Approximately 3 years ago, at the age of 34, she exhibited a worsening of fatigue and dyspnea during exertion (New York Heart Association III), and was therefore hospitalized for a detailed examination. In cardiac catheterization, a right ventricle to pulmonary artery peak-to-peak gradient of about 90mmHg was observed. Since it appeared that medical treatment alone would not sufficiently control her heart failure, pulmonary valvuloplasty using a triple-balloon technique was performed for the pulmonary stenosis. The peak-to-peak gradient immediately after the procedure decreased to 13mmHg. There were no indications of restenosis approximately 6 months after the procedure, and the symptoms of heart failure in her daily life improved thereafter. [Copyright &y& Elsevier]

Andersen-Tawil syndrome is an uncommon inherited autosomal disorder characterized by a prolonged QT interval, periodic paralysis, and dysmorphic features. The deleterious effects of cardioplegia on periodic paralysis and cardiac arrhythmia are unknown, and no studies have reported the performance of cardiac surgery in patients with Andersen-Tawil syndrome. We present a case of successful cardiac surgery in a patient with Andersen-Tawil syndrome, without using cardioplegia. [Copyright &y& Elsevier]

Supplemental Digital Content is available in the text. [ABSTRACT FROM AUTHOR]

Objects: A number of studies suggest that fetal growth restriction (FGR) promotes a risk of cardiovascular disease and metabolic disorders. However, it has not been well assessed whether FGR would be involved in the exaggeration of vascular remodeling. We investigated the effect of FGR on inflammatory vascular remodeling using cuff- induced vascular injury mouse model.Methods: Dams (C57BL/6J strain mice) were fed with an isocaloric diet containing 20% protein (normal protein; NP) or 8% protein (low protein; LP) from 10 weeks of ages. At the day of delivery, all dams were returned to the NP diet. After weaning, offspring were fed with the NP diet. Vascular injury was induced by polyethylene cuff placement around the femoral artery in offspring at 10 weeks of age. Neointima formation was evaluated by Elastica van Gieson staining 2 weeks after cuff placement. We assessed the following parameters in the femoral arteries prepared one week after cuff placement. Inflammatory cytokine and NADPH oxidase subunit were assessed by RT-PCR. Superoxide anion production, cell proliferation were evaluated by dihydroethidium staining, proliferating cell nuclear antigen (PCNA) staining respectively. p38 mitogen-activated protein kinase (p38MAPK) phosphorylation were evaluated by immunoblot analysis.Results: Birth weight was significantly lower in LP offspring (LPO) compared with NP offspring (NPO); however, LPO showed a similar body weight compared with NPO at 10 weeks of ages. Blood pressure at 12 weeks of age in LPO did not differ from NPO. Neointima formation was more exaggerated in LPO than NPO with enhanced oxidative stress and PCNA index in the injured artery. Expressions of MCP-1, IL-6, IL-1β and TNF-α in the femoral artery were more enhanced in LPO. Moreover, expressions of NADPH oxidase subunits, such as p22phox, p40phox, p47phox, p67phox, gp91phpx, Nox4 and Rac1 in the injured artery were enhanced in LPO than NPO. The phosphorylation level of p38MAPK was more increased in LPO than NPO.Conclusion: FGR led to the enhanced vascular remodeling via enhancement of stress responses such as inflammation and oxidative stress. These results suggest that FGR is a risk of vascular remodeling in the later life after birth. [ABSTRACT FROM AUTHOR]

Summary: The mechanisms responsible for thromboembolic events in children with congenital heart disease have not yet been fully elucidated. Furthermore, establishment of long-term anticoagulation therapy in Fontan patients remains controversial. Here, we report the case of a 9-year-old boy who presented with hemiparesis due to a thromboembolic stroke; the boy had previously undergone staged pulmonary artery banding and Fontan procedure. Cardiac multidetector-row computed tomography (MDCT) clearly showed the supravalvular thrombus at the roofed (blind) pulmonary valve and circulatory stasis, which could be considered a possible source of the thrombus. Follow-up CT examination showed that the thrombus disappeared, but the circulatory stasis remained. Therefore, because the risk of thrombus formation was not eliminated, anticoagulation therapy was continued for the patient. Our case indicates the possible application of cardiac MDCT for providing insight into the hemodynamic mechanisms responsible for the thromboembolic events in children with congenital heart disease. [ABSTRACT FROM AUTHOR]

Summary: In the neonatal period, the surgical mortality of palliation is extremely high for asplenia syndrome complicated by single ventricle combined with total anomalous pulmonary venous connection (TAPVC). Recently, stent implantation for the pulmonary venous drainage route soon after birth has been used instead of surgery to prevent pulmonary venous occlusion and to maintain stable hemodynamics in the neonatal period or in early infancy. Here, we successfully implanted stents in the ductus venosus (DV) in 2 neonates with asplenia syndrome complicated by infracardiac type TAPVC. The first patient was a 3-day-old male neonate with severe cyanosis. Immediately after TAPVC was diagnosed, we implanted a stent in the DV. The second patient was a 0-day-old female neonate. She was diagnosed as TAPVC by fetal echocardiogram. After the scheduled delivery, a stent was successfully implanted. We believe that stent implantation in the DV in the neonatal period is effective and less invasive than surgery in patients with infracardiac type TAPVC. [Copyright &y& Elsevier]