日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

The Fontan operation has certainly changed the quality of life in patients with congenital heart disease with functionally single ventricle, and the perioperative survival has dramatically improved owing to recent medical advances and modifications of the surgical technique. The postoperative course of these patients varies widely with relatively high morbidity and mortality compared with the course of patients in whom congenital heart disease has been treated with other types of definitive repairs. However, long-term management strategies of these patients have not yet been established because of a lack of information on the entire postoperative course of the unique hemodynamics and insufficient understanding of the pathophysiology of this type of heart failure. Our institute has developed a unique approach to overcome these clinical problems and has tried to provide these patients with better medical care using precise serial comprehensive assessments of the pathophysiology, including hemodynamics, cardiopulmonary exercise testing, hepatorenal conditions, and even metabolic abnormalities. Thanks to these daily practices, we have clarified some of the complicated heart failure pathophysiology to some extent, and some patients have benefited from these results. However, additional complications have emerged as these patients aged and raise many questions.