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        Where Is the “Optimal” Fontan Hemodynamics?

        Hideo Ohuchi 대한심장학회 2017 Korean Circulation Journal Vol.47 No.6

        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.

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        Unscheduled Hospitalization in Adults with Congenital Heart Disease

        Jun Negishi,Hideo Ohuchi,Kenji Yasuda,Aya Miyazaki,Nakanishi Norifumi,Osamu Yamada 대한심장학회 2015 Korean Circulation Journal Vol.45 No.1

        Background and Objectives: Little information is available regarding adult patients with congenital heart disease (CHD) who needed unscheduledhospitalization (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 a5-year study period. Medical records were retrospectively reviewed and data regarding USH were collected. Patient’s background, underlyingheart 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 hospitalized239 times. The median age at USH was 27 years old. Of the 959 patients, 54% were male. Underlying heart diseases included repaired tetralogyof Fallot (21%), single ventricular physiology after Fontan operation (17%), and Eisenmenger syndrome (12%). The causes of USH includedarrhythmia (40%), heart failure (20%), infectious disease (13%), and hemorrhage or thrombus (13%). A total of 48 patients requiredreadmission. 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 patientsin Japan and their therapeutic needs.

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