A 13-year-old girl with asplenia syndrome who previously had undergone left subclavian-to-pulmonary artery shunt after removal of a cavopulmonary shunt with interposition of a short segment of the left superior vena cava was admitted for congestive heart failure. Angiography revealed aneurysmal dilatation of the left superior vena cava. Percutaneous coil embolization of the shunt was successfully performed and the venous aneurysm was diminished. Interposition of a venous component in systemic-to-pulmonary artery shunt should be avoided even after removing a cavopulmonary shunt.

Cardiac surgery significantly influences cardiac autonomic nervous activity (CANA) and pulmonary function, resulting in abnormal cardiopulmonary responses during exercise testing in postoperative patients with congenital heart disease (CHD). In addition, for children, natural developmental changes must always be considered. Exercise-related cardiopulmonary derived variables, such as abnormal heart rate (HR) response, poor exercise capacity, and enhanced exercise ventilation, reflect the severity of heart failure and sometimes provide useful guides to clinical decision-making. However, although these variables may be informative, surgery-related abnormal responses of these variables should be taken into account as should developmental influences. Consequently, the obtained variables require careful and knowledgeable interpretation. Furthermore, exercise-related arrhythmias, which may require medical intervention, are more common in adult than in child CHD patients.
Adult ; Arrhythmias, Cardiac ; Child ; Exercise Test* ; Heart Defects, Congenital* ; Heart Failure ; Heart Rate ; Humans ; Thoracic Surgery ; Ventilation

Cardiac surgery significantly influences cardiac autonomic nervous activity (CANA) and pulmonary function, resulting in abnormal cardiopulmonary responses during exercise testing in postoperative patients with congenital heart disease (CHD). In addition, for children, natural developmental changes must always be considered. Exercise-related cardiopulmonary derived variables, such as abnormal heart rate (HR) response, poor exercise capacity, and enhanced exercise ventilation, reflect the severity of heart failure and sometimes provide useful guides to clinical decision-making. However, although these variables may be informative, surgery-related abnormal responses of these variables should be taken into account as should developmental influences. Consequently, the obtained variables require careful and knowledgeable interpretation. Furthermore, exercise-related arrhythmias, which may require medical intervention, are more common in adult than in child CHD patients.
Adult ; Arrhythmias, Cardiac ; Child ; Exercise Test* ; Heart Defects, Congenital* ; Heart Failure ; Heart Rate ; Humans ; Thoracic Surgery ; Ventilation

Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy consist of 2 components. One is a smooth adjustment to the new circulation, and the other is long-term circulatory stabilization after adjustment. When either of these goals is not achieved, the patient is categorized as having “failed” Fontan circulation, and the prognosis is dismal. For the first goal of smooth adjustment, a lot of effort has been made to establish criteria for patient selection and intensive management immediately after the Fontan operation. For the second goal of long-term circulatory stabilization, there is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics in Fontan circulation that could be used as a reference for patient management. Although small clinical trials and case reports are available, the results cannot be generalized to the majority of Fontan survivors. We recently reported the clinical and hemodynamic characteristics of early and late failing Fontan survivors and their association with all-cause mortality. This knowledge could provide insight into the complex Fontan pathophysiology and might help establish a management strategy for long-term hemodynamic stabilization.
Cardiac Output ; Cardiac Output, Low ; Central Venous Pressure ; Fontan Procedure ; Heart Defects, Congenital ; Hemodynamics* ; Humans ; Mortality ; Oxygen ; Patient Selection ; Physiology ; Prognosis ; Survivors ; Vascular Resistance

BACKGROUND AND OBJECTIVES: Little information is available regarding adult patients with congenital heart disease (CHD) who needed unscheduled hospitalization (USH). This paper aims to elucidate the clinical features of adult patients with CHD requiring USH. SUBJECTS AND METHODS: Study subjects included patients with CHD aged 18 years or older who were hospitalized at our facility during a 5-year study period. Medical records were retrospectively reviewed and data regarding USH were collected. Patient's background, underlying heart disease, cause of hospitalization, and prognosis (second USH regardless of cause or death) were examined. RESULTS: Overall, 959 CHD patients underwent a total of 1761 hospitalizations, including 145 patients who were unexpectedly hospitalized 239 times. The median age at USH was 27 years old. Of the 959 patients, 54% were male. Underlying heart diseases included repaired tetralogy of Fallot (21%), single ventricular physiology after Fontan operation (17%), and Eisenmenger syndrome (12%). The causes of USH included arrhythmia (40%), heart failure (20%), infectious disease (13%), and hemorrhage or thrombus (13%). A total of 48 patients required readmission. In total, 13 patients died, including four hospital deaths. The USH-free survival rate was 77% for 1 year and 58% for 3 years. CONCLUSION: The rate of USH was high for adults with complicated CHD. Common causes of USH included arrhythmia, heart failure, hemorrhage-related or thrombus-related conditions and infection. These data provide the current status of medical care for adult CHD patients in Japan and their therapeutic needs.
Adult* ; Aging ; Arrhythmias, Cardiac ; Communicable Diseases ; Eisenmenger Complex ; Fontan Procedure ; Heart Defects, Congenital* ; Heart Diseases ; Heart Failure ; Hemorrhage ; Hospitalization* ; Humans ; Japan ; Male ; Medical Records ; Physiology ; Prognosis ; Retrospective Studies ; Survival Rate ; Tetralogy of Fallot ; Thrombosis