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

No effective treatments currently exist for patients who have undergone the Fontan procedure with pulmonary vein occlusion (PVO), and the prognosis is poor. We present a case of a 3-year-old girl with acute heart failure who developed right PVO after undergoing the Fontan procedure, and a large amount of pleural effusion, edema, and atrial arrhythmia was noted. Fenestration was created after embolization of the minor aortopulmonary collateral arteries (mAPCAs) and right pulmonary (RPA) artery. The embolization of mAPCAs was divided into 3 sessions using 51 coils. For RPA embolization, 5 Amplatzer Vascular Plugs and 2 coils were used. The duration of this procedure was approximately 3 h, and complete occlusion of RPA was achieved without complications. Subsequently, fenestration creation was performed, and the central venous pressure decreased from 20 mmHg to 7 mmHg. However, the patient deceased because of cardiac tamponade on day 9 postoperatively. To our knowledge, there have been no reports on the complete embolization of the mAPCAs and PA in patients who have undergone the Fontan procedure with PVO. This treatment temporarily stabilized the hemodynamics in patients who have undergone the Fontan procedure with PVO.