BACKGROUND The discussion of Fontan fenestration is difficult because many institutes have different strategies over time. In our institute, we had performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy. METHODS Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy. Primary outcomes were Fontan-related events, including death, reoperation, catheter intervention, and postoperative complications such as arrhythmias, protein-losing enteropathy, plastic bronchitis, thrombosis, bleeding, and drainage of pleural effusion after discharge. RESULTS The median follow-up duration was 15.7 years (interquartile range (IQR): 15.3–18.4). The 1-, 2-, 3-, 5-, 10-, and 15-year occurrence of Fontan-related events was 24%, 43%, 44%, 55%, 67%, and 76%, respectively. The 1-, 2-, 3-, 5-, and 10-year occurrence of venovenous collaterals was 16%, 60%, 65%, 72%, and 81%, respectively. Ventricular end-diastolic pressure was higher in patients with venovenous collaterals (median: 5.0 mmHg, IQR: 4.0–7.0) than in those without (median: 3.5mmHg, IQR: 3.0–4.25) (p = 0.01). Multivariable Cox regression analysis showed that higher ventricular end-diastolic pressure and younger age at Fontan completion significantly increased the risk of developing venovenous collaterals, with hazard ratios of 1.22 for each (95% confidence interval: 1.052–1.41, p = 0.0085 and 0.016, respectively). CONCLUSIONS The occurrence rate of Fontan-related events was acceptable with the non-fenestrated Fontan strategy, whereby venovenous collateral development was common. The results suggest that patients with high ventricle end-diastolic pressure and young patients might benefit from fenestration.

Perimembranous ventricular septal defect (VSD) can be classified as having trabecular, inlet, or outlet extension. The surgical approach used in patch closure depends on the which valve of the tricuspid valve to suture around and the avoidance of the specialized conducting system. This retrospective study evaluated the usefulness of the “En face view” method for classifying perimembranous VSD. We divided 147 patients with perimembranous VSD into one group of 52 patients diagnosed using the Helmcke et al. method until 2001 and another group of 95 patients diagnosed by the En face view method and conventional method from 2002. A definitive diagnosis was made intraoperatively for patients in whom the extension of the defect was examined using the En face view method during preoperative echocardiography. When the VSD extended towards the anterior leaflet from the commissure between the antero-septal commissure, it was considered outlet extension, and when it extended towards the septal leaflet, it was judged as inlet extension. Of the 52 patients in whom extension direction was diagnosed until 2001, the number of correct diagnoses was 39 (75.0

This is the first report of total arch replacement to repair re-coarctation. A 14-year-old boy with hypoplastic left heart syndrome developed re-coarctation, severe stenosis of neck vessels, and right ventricle dysfunction after a Norwood procedure. We performed total arch replacement; the postoperative course was unremarkable. He was followed up until 18 years of age and did not need re-intervention. Using artificial blood vessels in total arch replacement is rarely indicated but can be safely achieved when required. Mismatch between patient and graft size may be an issue in the future.

A one-month-old baby boy with a complete atrioventricular septal defect underwent pulmonary artery banding. A high take-off of the left coronary artery, overlooked on the echocardiogram, was identified. It was compressed by the right pulmonary artery that was dilated owing to pulmonary artery banding. The patient developed severe heart failure, and a Lecompte maneuver was performed. The procedure helped effectively treat this congenital heart disease with a high take-off coronary artery compressed by the right pulmonary artery.

In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR. We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4 years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated. QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160 ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5 ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160 ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8 ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases. A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160 ms due to normalization of the RV volume after PVR.

Postoperative pericardial adhesions are considered a risk factor for redo cardiac surgery. Several large- and medium-size animal models of pericardial adhesions have been reported, but small animal models for investigating the development of anti-adhesion materials and molecular mechanisms of this condition are lacking. In this study, we aimed to establish a simple mouse model of pericardial adhesions to address this gap. We administered blood, minocycline, picibanil, and talc into the murine pericardial cavity via one-shot injection. Micro-computed tomography analyses of contrast agent-injected mice were carried out for methodological evaluation. We investigated various dosages and treatment durations for molecules identified to be inducers of pericardial adhesion. The adhesive grade was quantified by scoring the strength and volume of adhesion tissues at sacrificed time points. Histological staining with hematoxylin and eosin and Masson’s trichrome, and immunostaining for F4/80 or αSMA was performed to investigate the structural features of pericardial adhesions, and pathological features of the pericardial adhesion tissue were compared with human clinical specimens. Administration of talc resulted in the most extensive pericardial adhesions. Micro-computed tomography imaging data confirmed that accurate injection into the pericardial cavity was achieved. We found the optimal condition for the formation of strong pericardial adhesions to be injection of 2.5 mg/g talc for 2 weeks. Furthermore, histological analysis showed that talc administration led to an invasion of myofibroblasts and macrophages in the pericardial cavity and epicardium, consistent with pathological findings in patients with left ventricular assistive devices. We successfully established a simple mouse model of talc-induced pericardial adhesions, which mimics human pathology and could contribute to solving the clinical issues related to pericardial adhesions.

ABSTRACT Calcification of bioprosthetic valves (BVs) implanted in aortic position can result in gradual deterioration and necessitate aortic valve replacement. The molecular mechanism of calcium deposition on BV leaflets has been investigated, but remains to be fully elucidated. The present study aimed to identify explanted bioprosthetic valve (eBV)-specific proteins using a proteomics approach and to unveil their biochemical and histological involvements in calcium deposition on BV leaflets. Calcification, fibrosis, and glycosylation of the valves were histologically assessed using Von Kossa, Masson's Trichrome and Alcian Blue staining, as well as immunostaining. Protein expression in the explanted biological valves was analysed using proteomics and western blotting. In a histological evaluation, αSMA-positive myofibroblasts were not observed in eBV, whereas severe fibrosis occurred around calcified areas. SDS-PAGE revealed three major bands with considerably increased intensity in BV leaflets that were identified as plasminogen and fibrinogen gamma chain (100 kDa), and fibrinogen beta chain (50 and 37 kDa) by mass analysis. Immunohistochemistry showed that fibrinogen β-chain was distributed throughout the valve tissue. On the contrary, plasminogen was strongly stained in CD68-positive macrophages, as evidenced by immunofluorescence. The results suggest that two important blood coagulation-related proteins, plasminogen and fibrinogen, might affect the progression of BV degeneration.

Background. Lung injury is a life-threatening complication in patients with liver dysfunction. We recently provided an experimental lung injury model in mouse with common bile duct ligation. In this study, we aimed to characterize the pathologic and biochemical features of lung tissues in common bile duct ligation mice using a proteomic approach.Methods. Common bile ducts of BALB/c mice, 8 weeks of age, were ligated operatively. CD31-expressing pulmonary cells were sorted with immunomagnetic microbeads, and protein profiles were examined by 2 dimensional gel electrophoresis. Based on the results of protein identification, immunohistochemistry and quantitative reverse transcription polymerase chain reaction were carried out in pulmonary and hepatic tissues.Results. Two-dimensional gel electrophoresis revealed 3 major inflammation-associated proteins exhibiting considerable increases in the number of CD31-positive pulmonary cells after common bile duct ligation. Mass spectrometry analysis identified these proteins as SerpinB 1 a (48 kDa), ANXA1 (46 kDa), and S100A9 (16 kDa). Furthermore, the 3 proteins were more highly expressed in dilated pulmonary blood vessels of common bile duct ligation mice, in which neutrophils and monocytes were prominent, as shown by immunohistochemistry. More importantly, SerpinB la mRNA and protein were significantly upregulated in the liver, whereas S100A9 and ANXA1 mRNA and protein were upregulated in the lungs, as shown by quantitative reverse transcription polymerase chain reaction and Western blotting.Conclusion. We identified 3 proteins that were highly expressed in the lung after common bile duct ligation using a proteomics-based approach.

Background: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history. Case presentation: A 64-year-old man was diagnosed as having multiple atrial septal defects. He presented with no symptoms of heart failure. His preoperative ECG showed complete right bundle branch block (CRBBB) with a corrected QT interval time of 478 ms. He underwent open-heart surgery to close the defects through median sternotomy access. Three hours after the operation, he developed multiple events of TdP and VFs in the intensive care unit. Cardiopulmonary resuscitation and multiple cardioversions were attempted for his repetitive TdP and VFs. He eventually reverted to sinus rhythm, and intravenous beta-blocker was administered to maintain the sinus rhythm. After this event, his family history was reviewed, and it was confirmed that his daughter and grandson had a medical history of arrhythmia. A genetic test confirmed that he had a missense mutation in CACNA1C, p.K1580 T, which is the cause for type 8. Conclusions: This case highlights the importance of paying attention to other ECG findings in patients with CRBBB, which can mask prolonged QT intervals.

Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal malformations' (PHACES) syndrome comprises various defects that require a concrete therapeutic plan. Herein, we report a case of a very low birth weight infant with PHACES syndrome presenting complete sternal cleft, interrupted aortic arch with ventricular septal defect, and absence of communicating cerebral arteries. Due to the infant's very low birth weight, we planned staged surgery for this complex disease. First, bilateral pulmonary artery banding was performed to improve unstable hemodynamics. Then, after sufficient body weight was obtained, aortic arch and intracardiac repair was done. However, when the tracheal tube was removed 2 weeks after surgery, the patient could not breathe well due to paradoxical chest movement related to complete sternal cleft. Consequently, to improve chest wall compliance, the upper sternum was constructed with dislocated ribs. After these treatments, the patient gained adequate weight and was in stable condition. Staged surgical treatment of sternal cleft and congenital heart defects is a potential therapeutic option for PHACES syndrome, particularly in very low birth weight infants with complex cardiovascular disease.

症例は57歳の女性．就学前より心雑音を指摘され，ファロー四徴症（TOF）と診断された．30代より他院外来で手術を勧められたが拒否していた．経過中に心房細動が出現し，心不全の増悪による入退院を繰り返すため，当院紹介となった．精査の結果，外科的治療の方針となり，内科的治療により心不全コントロールを行った後に手術を施行した．術式は心室中隔欠損パッチ閉鎖術，肺動脈弁置換術（Epic生体弁23 mm），右室流出路形成術，Maze手術を施行した．術後右室圧は左室圧の4割程度に改善した．術翌日に人工呼吸器より離脱し，術後約1カ月で自宅退院となった．成人期のファロー四徴症に対し根治術を施行し，良好な結果を得たので報告する．

Biatrial drainage of the right superior vena cava resulting from a sinus venosus defect is a rare congenital malformation. It can result in severe desaturation although a sinus venosus defect usually presents as a left-to-right shunt. A male baby was noted to have cyanosis while nursing and was referred to us for medical treatment on his second day of life. Echocardiography showed that most of the blood flowing through the superior vena cava drained into the left atrium. He underwent successful surgery to correct this defect at the age of 2 years.

Aortic coarctation rarely occurs after an arterial switch operation for D-transposition of the great arteries with intact ventricular septum. We report the case of a neonate patient in whom aortic coarctation developed 28 days after an uncomplicated arterial switch operation. Preoperatively, the aorta was noted to have an irregular shape, but there was no pressure gradient across the lesion. The patient underwent successful reoperation to correct the coarctation. We hope that our report raises awareness of a rare early complication after arterial switch operation with intact ventricular septum, and the need to carefully monitor the aortic isthmus in patients who have aortic irregularities, even in the absence of a pressure gradient.

Collateral vascular arteries from the descending aorta to the pulmonary arteries are uncommon after arterial switch operation. Here, we report the case of a baby girl treated with coil embolization for abnormal blood flow from the descending aorta to the pulmonary arteries after arterial switch operation. A baby girl weighing 1324 g was delivered at 32 weeks 4 days of gestation, and she had D-transposition of the great arteries and a ventricular septal defect. She underwent nitrogen inhalation to reduce pulmonary blood flow before arterial switch operation. After the operation, she presented with left heart failure due to the presence of abnormal blood flow from the descending aorta to the pulmonary arteries, and she was successfully treated with coil embolization. After the treatment, her condition improved dramatically, and she was discharged without any complications.

A 42-year-old man underwent external left ventricular assist device (VAD) implantation for fulminant myocarditis in May 2012. He was registered a recipient of heart transplantation from braindeath donors in July 2013 and waiting for heart transplantation under external left VAD treatment. Our hospital was accredited for implantable VAD surgery in January 2014. He underwent implantable left VAD surgery with a DuraHeart system in February 2014. He was extubated and transferred to the floor on postoperative day 4. He continued hospitalization because of poor eyesight caused by previous cerebral hemorrhage. His convalescence was unremarkable for several months after left VAD implantation. His renal function was gradually deteriorated and hemodialysis was required on six months postoperatively. Echocardiography and Swan-Ganz catheter examinations revealed severe right ventricular dysfunction with profound right heart failure. Although he required maximum dose of inotropes, his hemodynamics and left VAD assist flow could not be maintained. He expired due to multi-organ failure on the postoperative day 245. Autopsy findings showed that there was no specific pathology for the cause of death in the major organs except the heart. Lymphocytes and other inflammatory cells infiltration were observed in the both left ventricle and right ventricle myocardium. Persistent myocarditis was considered for fatal right ventricular dysfunction and right heart failure following VAD implantation.

Use of a Transvenous Endocardial Lead as a Shock Cord Placed Subcutaneously in an Infant with Congenital Long QT Syndrome Hiroko Goto1, 3), Naoki Kuwabara1), Kentaro Omoya1), Atsushi Terasawa1), Ai Kojima2), Yusuke Iwata2), Takamasa Takeuchi2), Takashi Kuwahara1) 1)Department of Pediatric Cardiology, 2)Pediatric Cardiac Surgery, 3)Heart Rhythm Management, Gifu Prefectural General Medical Center, Gifu, Japan