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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]

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]

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]

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]

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]

Abstract: We treated a 6-year-old boy who had polysplenia syndrome and tetralogy of Fallot with a small right ventricle (RV), an atrial septal defect, a hemiazygos connection, and bilateral superior vena cava. Because the RV was too small for a biventricular repair to be performed, the patient underwent a total cavopulmonary shunt operation although his heart was biventricular and a pacemaker (VVI) had been implanted for management of the sick sinus syndrome complicated by polysplenia syndrome. After the operation, marked asynchronous contraction was noted between the morphological right and left ventricles and was probably responsible for the low cardiac output noted in this patient. In order to clarify the significance of the asynchronous contraction, we determined the cause of the low cardiac output by studying the time course of the volume changes in the morphological right and left ventricles during a cardiac cycle by using angiograms. In addition, we studied the interventricular flow dynamics by using pulsed-Doppler echocardiography. After a Fontan-type operation is performed on patients with a biventricular heart, the 2 ventricles may not function in perfect coordination when they have to work as 1 unit. These patients are likely to develop cardiac dysfunction due to interventricular to-and-fro flow dynamics. Asynchronous contraction between the 2 ventricles caused by abnormal interventricular conduction impaired the cardiac performance in the present case. [Copyright &y& Elsevier]

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]