Search

Objective: Fontan circulation maintains preload and cardiac output by reducing venous capacitance and increasing central venous pressure (CVP). The resultant congestive end-organ damage affects patient prognosis. Therefore, a better circulatory management strategy to ameliorate organ congestion is required in patients with Fontan circulation. We sought to verify whether aggressive arterial and venous dilation therapy in addition to pulmonary dilation (super-Fontan strategy) can improve Fontan circulation and reduce congestion. Methods: Patients after Fontan surgery who received the super-Fontan strategy in a single center were recruited. Participants were examined using medical records between 2010 and 2018. We retrospectively analyzed the changes in hemodynamics at rest and during treadmill exercise before and after the introduction of this therapy. Results: The therapy significantly increased venous capacitance (3.21 ± 1.27 mL/kg/mm Hg to 3.79 ± 1.30 mL/kg/mm Hg, P = .017) and decreased total pulmonary resistance, leading to significantly reduced CVP (11.7 ± 2.4 mm Hg to 9.7 ± 2.2 mm Hg, P

Congenital brain tumors (CBTs), defined as tumors presenting within 60 days after birth, are extremely rare and account for only 0.5–1.9% of all pediatric brain tumors. Teratoma is the most common type of CBT, although there are many other poorly described forms. Prenatal diagnosis of CBT is often difficult and usually based on clinical characteristics and radiological findings with magnetic resonance imaging and ultrasonography. The prognosis of patients with CBT depends on the histopathological features of the tumor and its location. Even after several investigations have been performed, a clear direction for diagnosis and treatment of fetal intracranial tumors is still lacking. Further studies are thus needed to clarify its clinical characteristics and establish recommendations for management.

High-flow nasal cannula (HFNC) therapy supports respiratory effort with a minimal elevation in airway pressure. We examined hemodynamic effects of HFNC therapy in a 10-year-old girl with Fontan circulation, in which positive airway pressure has deleterious hemodynamic effects. The HFNC therapy at 30 L/min improved oxygenation without an increase in central venous pressure. It also reduced heart rate, and systemic and pulmonary vascular resistance, and increased cardiac output. In addition, the HFNC therapy improved the cerebral circulation measured by near-infrared spectroscopy. Thus, HFNC therapy may be a potentially useful noninvasive ventilation modality, particularly for patients with Fontan circulation.

Chylothorax is a serious complication of congenital cardiac surgery and is significantly associated with increased morbidity and mortality. Central venous obstruction, which is often related to the insertion of central venous catheters for postoperative management, is known to be an important risk factor for treatment failure and mortality associated with this condition. We present the case of a 6-month-old girl with refractory chylothorax after surgical repair of tetralogy of Fallot. The chylous drainage continued for more than 2 months despite maximal conservative therapy (water restriction, total parenteral nutrition, and infusion of somatostatin and steroid) and surgical ligation of the thoracic duct. Subsequently, we observed stenosis of the superior vena cava (SVC) caused by large thrombi possibly associated with the prolonged use of central venous catheter placed in the internal jugular vein. Because transcatheter balloon dilation failed to relieve the stenosis, we performed stent implantation for the SVC and innominate vein. After the procedure, chylous drainage dramatically reduced, and the patient was discharged from the hospital. In conclusion, central venous obstruction due to thrombosis should be routinely examined when chylothorax is diagnosed and is resistant to conservative therapy after congenital heart surgery. Stent implantation can effectively relieve the venous obstruction and thus be a life-saving treatment option for this difficult condition.