Successful perioperative management of pheochromocytoma in a patient with Fontan circulation.

Pheochromocytoma (PCC) can adversely affect Fontan circulation. However, there are few reports on its perioperative management before and after PCC resection in Fontan patients. A 24-year-old female patient with congenitally corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia who had undergone Fontan palliation developed heart failure caused by PCC. The patient was pre-conditioned for PCC resection with heart failure treatment, alpha-blocker titration, and careful infusion, and had a good intraoperative and postoperative course with no complications. Postoperative catheter data showed improvements in systemic vascular resistance, cardiac output, and central venous pressure compared with preoperative data. There is no established preconditioning method for PCC resection in patients with Fontan circulation. Careful perioperative management based on an understanding of the features of the Fontan circulation can lead to better outcomes. Learning objective: Pheochromocytoma (PCC) can occur in patients with Fontan circulation. Preoperative management and the PCC itself can adversely affect Fontan circulation, highlighting the importance of suspecting PCCs in Fontan patients based on symptoms such as heart failure, worsening arrhythmias, and headache, and emphasizing careful perioperative management.

Platelet count and abdominal dynamic CT are useful in predicting and screening for gastroesophageal varices after Fontan surgery.

OBJECTIVE: Patients who undergo Fontan surgery for complex cardiac anomalies are prone to developing liver and gastrointestinal complications. In particular, gastroesophageal varices (GEVs) can occur, but their prevalence is unknown. We aimed to elucidate the occurrence of GEVs and the predicting parameters of GEVs in these patients. MATERIALS AND METHODS: Twenty-seven patients (median age, 14.8 years; median time since surgery, 12.9 years) who had undergone the Fontan surgery and were examined by abdominal dynamic computed tomography (CT) for the routine follow-up were included in the study. Radiological findings including GEVs and extraintestinal complications were retrospectively evaluated by experienced radiologists in a blinded manner. Relationships between blood-biochemical and demographic parameters and the presence of GEVs were statistically analyzed. RESULTS: Dynamic CT revealed gastric varices (n = 3, 11.1%), esophageal varices (n = 1, 3.7%), and gastrorenal shunts (n = 5, 18.5%). All patients with gastric varices had gastrorenal shunts. All gastric varices were endoscopically confirmed as being isolated and enlarged, with indications for preventive interventional therapy. A platelet count lower than 119 × 109 /L was identified as a predictor of GEV (area under the receiver operating curve, 0.946; sensitivity, 100%; and specificity, 87%). CONCLUSIONS: GEVs are important complications that should not be ignored in patients who have undergone a Fontan procedure. Platelet counts lower than 119 × 109 /L may help to prompt patient screening by using abdominal dynamic CT to identify GEVs and their draining collateral veins in these patients.

Assessing liver stiffness with conventional cut-off values overestimates liver fibrosis staging in patients who received the Fontan procedure.

AIM: Patients who undergo the Fontan procedure for complex congenital heart disease are prone to liver cirrhosis. Liver stiffness (LS) reflects liver fibrosis stage in patients with chronic viral hepatitis; however, its accuracy in predicting liver fibrosis stage in Fontan patients is controversial. We aimed to clarify the correlation between LS and liver fibrosis stage in Fontan patients. METHODS: Fifty-eight Fontan patients were prospectively measured for LS with transient elastography. We undertook liver biopsy, cardiac catheterization, and laboratory tests in 22 of these patients (median age, 14.7 years; range, 9.9-32.1 years) with LS > 11.0 kPa (median, 19.2 kPa; range, 12.2-39.8 kPa); these elevated LS values suggest liver cirrhosis. RESULTS: Histologically, all patients showed mild-to-severe portal and sinusoidal fibrosis but no cirrhosis. Statistically, LS did not predict histological liver fibrosis scores (p = 0.175). Liver stiffness was not correlated with central venous pressure (p = 0.456) or with the hepatic venous pressure gradient (HVPG; p = 0.062), although the p value for HVPG was only slightly above the threshold for significance. CONCLUSIONS: Fontan patients are prone to developing both portal and sinusoidal fibrosis. Liver stiffness could be influenced by HVPG, and using the conventional cut-off values for LS overestimates and overtreats liver fibrosis in these patients.

A rare case of truncus arteriosus Van Praagh type A3: Prenatal diagnosis and postnatal management.

Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C-E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks' gestation. TrA C-E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks' gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management. .

Study design for control of HEART rate in inFant and child tachyarrhythmia with heart failure Using Landiolol (HEARTFUL): A prospective, multicenter, uncontrolled clinical trial.

BACKGROUND: Persistent tachycardia in pediatric patients after congenital heart surgery further deteriorates their hemodynamic condition, and may become fatal. Therefore, immediate control of the tachycardia is mandatory in these patients. For this purpose, quick-acting, short-acting, titratable intravenous agents are required. However, there are no agents with such characteristics among the drugs approved for control of pediatric arrhythmias in Japan, and thus novel and effective medications for these patients are awaited. Landiolol, an ultrashort-acting ß-blocker, was approved in 2013 for tachyarrhythmias in adult patients with heart failure. However, its efficacy and safety in pediatric patients remain unclear. The aim of this prospective, multicenter, open-label phase IIb/III study is to investigate the efficacy and safety of landiolol in pediatric patients with tachyarrhythmias as well as heart failure. METHODS: Eligible patients are aged ≥ 3 months and <15 years, and have tachyarrhythmia (atrial fibrillation, atrial flutter, supraventricular tachycardia) as well as heart failure. The primary endpoint of the study is ≥20% reduction from baseline heart rate or return to normal sinus rhythm within 2h after starting intravenous administration of landiolol. Patients will receive intravenous infusion of landiolol, starting at 1µg/kg/min. The dose will be increased by 1µg/kg/min every 15-20min until the tachycardia rate has decreased by >20% or tachycardia has terminated, and the dose will then be maintained or further increased depending on the patient's condition. The study was started in April 2015 and will end within a few years. CONCLUSIONS: The study was designed and designated the "HEARTFUL study" in the hope of establishing a basis for control of HEART rate in inFant and child tachyarrhythmia Using Landiolol in children with heart failure.

Novel technique for implantation of a cardioverter defibrillator in children.

An 8-year-old boy with hypertrophic nonobstructive cardiomyopathy with ventricular fibrillation underwent implantation of an implantable cardioverter defibrillator. The lead was inserted through a pursestring suture in the right atrial appendage, and the tip of coil was placed in the right ventricular apex under fluoroscopic guidance. Another defibrillation coil was placed in the back of the left atrium and left ventricle by the transverse sinus. The device wrapped in a monofilament mesh sheet was placed in the intraperitoneal space. This case utilized a new technique for an implantable cardioverter defibrillator implantation in a small child.

Differentiating fasciculoventricular pathway from Wolff-Parkinson-White syndrome by electrocardiography.

BACKGROUND: In school-based cardiovascular screening programs in Japan, Wolff-Parkinson-White (WPW) syndrome is diagnosed based on the presence of an electrocardiographic (ECG) delta wave without differentiation from the fasciculoventricular pathway (FVP), although the risk of sudden death is associated only with the former. OBJECTIVE: The purpose of this study was to differentiate FVP patients among children diagnosed with WPW syndrome by ECG. METHODS: Children who were diagnosed with WPW syndrome through school screening between April 2006 and March 2008 and had QRS width ≤120 ms were included. Patients with asthma and/or coronary heart disease were excluded. FVP and WPW syndrome were differentiated based on ECG responses to adenosine triphosphate (ATP) injection. Age, PR interval, QRS width, and Rosenbaum classification were compared among patients. RESULTS: Thirty patients (median age 12.7 years, range 6.5-15.7 years) participated in the study. FVP was diagnosed in 23 patients (76.7%), and WPW syndrome in 7 (23.3%). In Rosenbaum type A patients, all six patients had WPW syndrome, whereas FVP was diagnosed in 23 of 24 and WPW syndrome was diagnosed in 1 of 24 of type B patients. Age, PR interval, and QRS width were not significantly different between the two conditions. CONCLUSION: ATP stress test was reliable in differentiating FVP from WPW syndrome. Although FVP is considered rare, the results of our study indicate that many WPW syndrome patients with QRS width ≤120 ms may actually have FVP. Patients categorized as type B are more likely to have FVP, whereas type A patients are most likely to have WPW syndrome.

Staged Starnes Operation Preserving Patent Ductus Arteriosus for Neonates with Ebstein's Anomaly and Pulmonary Atresia.

We herein reported 2 successful neonates with Ebstein's anomaly and small pulmonary arteries undergoing Starnes operation preserving the patent ductus arteriosus. Subsequent Blalock-Taussig shunt was carried out 1 or 2 months after the first surgery. One case had already undergone a successful Fontan operation, and the other had a successful bidirectional Glenn shunt so far. This staged Starnes strategy might be a safe and simple choice for neonates with Ebstein's anomaly and small pulmonary arteries.