With the prolonged survival of children with serious congenital heart disease (CHD), we have encountered new problems and new challenges within the focus on long-term morbidity and quality of life. Recently, hepatic complications have been increasingly reported in patients who underwent Fontan surgery for serious CHD with single ventricles. We recognized many hepatic complications ranged from liver fibrosis, cirrhosis, and even liver cellular carcinoma (HCC). We had a chance to experience children with liver complications after Fontan operations in Shizuoka Children's Hospital. Initially, it was difficult to identify the liver complications, because in the majority of these children, routine liver function tests such as transaminase (AST, ALT) levels were usually within the reference range. However, these serum markers for developing liver fibrosis, hyaluronic acid, type IV collagen, pro-collagen-III-peptide, were usually increased in children with liver complications. It was also difficult to differentiate liver cirrhosis from serious liver fibrosis. Both US and CT imaging findings on the liver are useful in judging developing cirrhosis and various types of liver nodules such as focal nodular hyperplasia, adenoma, HCC, etc.In this article, we reviewed the important reports on liver complications after Fontan operations. And we especially discussed the mechanism of liver complications, diagnosis, and tentative management for liver complications after Fontan operations.
Background: Surgical approach for coarctation or interruption of the aorta（ CoA/IAA） with complex cardiac anomalies consists of single or staged repair. Our strategy has advocated staged repair, including arch repair and pulmonary artery banding without the use of extracorporeal circulation as the first palliation. For cases of low birth weight and hypoplastic aortic arch，we have performed bilateral pulmonary banding as the first palliation. Objective and Methods: Between 1996 to 2009，17 neonates and infants with CoA/IAA associated with complex cardiac anomalies underwent surgery at our institution. We assessed the surgical results. Results: Univentricular repair was done for 10 patients and biventricular repair for 7 patients. In univentricular group，all patients survived the first palliation，and the Fontan operation was achieved in 8 patients so far. The remaining 2 patients are waiting for the Fontan operation. All 7 patients with biventricular repair survived the first palliation as well，and achieved definitive operation with 2 early deaths. The Damus-Kaye-Stansel procedure or myectomy was carried out for 8 patients with anatomical or functional subaortic stenosis，and postoperative balloon angioplasty was carried out for 3 patients including 1 with significant stenosis. Conclusion: Although a staged surgical approach for CoA/IAA with complex cardiac anomalies often required additional intervention，this approach had good results. A staged repair consisting of bilateral pulmonary artery banding as the first palliation would be a useful choice for these anomalies with low birth weight and a hypoplastic aortic arch.
Background: Questionnaire survey from the Japanese Society of Pediatric Interventional Cardiology（ JPIC） estimated that static BAS had been annually performed for around 80 patients.However，balloon catheters for static BAS are off-label，while there is no official technical fee for this procedure. Method: For 33 facilities where board members of JPIC belong to，retrospective questionnaire surveys were performed on the current status of static balloon atrial septostomy（ static BAS）. Results: Among 26 responders，19 facilities experienced static BAS form January 1，2009 to December 31，2011.Eighteen facilities out of experienced 19 reported 111 patients with static BAS. 1） There were 45 neonates within 28 days（ median 7 days），60 infants younger than 12 months（ 2 months），and 6 patients older than 1 year（ 15 years）. 2） Underlying heart diseases were 55 patients of hypoplastic left heart syndrome，31 of transposition of the great arteries，20 of right ventricular hypoplastic disorders，and others. 3） Indication for static BAS was the primary choice in 73 patients，secondary choice following ineffective Rashkind method in 35，and others. 4） All balloon catheters for static BAS were standard balloon catheters for valvulo- or angioplasty.Standard BAS was performed following static BAS in 32 ％ of patients. 5） Common diameters of balloons were 12mm followed by 10mm. 6） Static BAS was effective in 85％ of patients，while untoward events occurred in 5％ of patients.There was no death associated with static BAS. Conclusion: Static BAS is a safe and effective procedure，which is accepted as a common procedure in our country.
Catecholaminergic polymorphic ventricular tachycardia（ CPVT） is one of the cardiacchannelopathies that appears during childhood and is associated with a poor prognosis. Mutations of the cardiac ryanodine receptor gene（ RyR2） have been identified as one of its causes. An abnormal function of the cardiac ryanodine receptor produces an excessive release of calcium ions from the sarcoplasmic reticulum, and this calcium overload causes arrhythmias due to triggered activity. A recent study demonstrated the efficacy of flecainide in genetically positive CPVT patients. Flecainide is a class Ic sodium channel blocker and a direct suppressor of the cardiac ryanodine receptor. Here, we report the case of a 10-year-old boy with a normal heart who developed syncope during physical stress. The patient?s resting electrocardiogram（ ECG） demonstrated sinus bradycardia with a normal QT interval. The treadmill stress test and Holter ECG demonstrated polymorphic ventricular tachycardia and supraventricular（ atrial or junctional） tachycardia. Therefore, he was diagnosed with CPVT. We recommended that the patient avoid competitive sports and prescribed propranolol at a dosage of 2 mg/kg per day and flecainide that titrated up to 150 mg/m2 per day. Flecainide demonstrated dosage-dependent amelioration of ventricular arrhythmia and suppressed supraventricular ectopies. The patient did not experienced syncope or any other complications during the 1-year follow-up period. He had previously reported causative missense mutation in RyR2 （c.14311 G>A p. V4771I）. Combination therapy of flecainide and a beta-blocker is safe and effective for supraventricular and ventricular tachycardia in patients with the RyR2 mutation. This therapy may improve prognosis in CPVT patients.
Congenital ventricular aneurysm is a very rare heart anomaly，especially when it occurs in the right ventricle. It is associated with a poor prognosis and a risk of sudden death. We report a case of congenital right ventricular aneurysm that was successfully repaired by surgery. In this case，echocardiography at birth revealed an aneurysmal change at the right ventricular apex. Rapid enlargement of this aneurysm was detected during careful follow-up，and surgical repair was performed at six months of age. The postoperative course was uneventful，and the patient was free from symptoms at 10 months after surgery.
Recently，negative pressure wound therapy（ NPWT） for the treatment of mediastinitis following open-heart surgery has been frequently used even in a pediatric cohort. The safety and efficacy of NPWT has been reported in an increasing number of studies； however，in cases with hemodynamic instability or tissue fragility in a vacuum，conventional treatment，such as irrigation and packing of the wound，is still an available option. Here we report a one-month-old male infant with methicillineresistant Staphylococcus aureus mediastinitis after palliative repair for double outlet right ventricle and subpulmonary VSD，successfully treated by irrigation and subsequent packing of the wound with silver-containing carboxymethylated cellulose （AQUACEL®Ag），a material with high absorbability of tissue fluids and antibacterial potential of ionic silver. AQUACEL®Ag is as good an alternative as packing material after mediastinal irrigation in pediatric patients with fragile tissue due to infection. In addition，treatment of mediastinitis with this method could be the bridging therapy to NPWT while hemodynamic instability or vulnerability of infected tissue is concerned.
Acute disseminated encephalomyelitis（ADEM） is a demyelinating disorder of the central nervous system that occurs in association with viral or bacterial infections and vaccinations. An autoimmune reaction against myelin components is the proposed pathogenesis; however，the mechanism details are not yet fully clarified. Although various neurologic symptoms may appear because of multiple scattered foci of demyelination in the central nervous system，they are often reversible. We herein report an unusual case of ADEM in a 2-year-old boy who underwent surgical closure of a ventricular septal defect. The patient developed a high fever followed by conjugated deviation of the eyes，hypertonia，and dystonia on the 5th postoperative day. Cerebrovascular disorders were initially suspected; however，diffusion-weighted images of brain magnetic resonance imaging （MRI） demonstrated diffuse high signals throughout the white matter suggesting demyelination. As encephalitis or demyelinating disease were suspected，therapy against brain swelling and empiric antibacterial and antiviral treatment were immediately initiated; in addition，high-dose intravenous steroid was administered after ruling out infectious disease. The patientʼs neurologic symptoms were gradually resolved，coupled with rapid resolution of MRI abnormalities. Although the etiology could not be identified based on laboratory studies，the clinical presentation and rapid resolution of radiological findings led to the diagnosis of ADEM. The patient showed good recovery with steroid therapy and was discharged without neurological deficits.