Pediatric Cardiology and Cardiac Surgery
Vol.26 No.5 2010 (434-440)

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Taku Ishii,1) Akihito Sasaki,1) Yusuke Kajikawa,1) Hiroyuki Sato,2) Koji Kiyohara,2) and Shozaburo Doi1)

1)Department of Pediatrics, Tokyo Medical and Dental University, Tokyo, Japan, 2)Department of Pediatrics, Musashino Red Cross Hospital, Tokyo, Japan


A 16-year old boy who had a history of congenital myopathy and bronchial asthma was diagnosed with dilated cardiomyopathy around one year ago. His symptoms of heart failure were rapidly worsening because they became resistant to medical treatments such as diuretics, ACE inhibitors, β-blockers, and PDEIII inhibitors. We therefore chose heart transplantation in Japan as a therapeutic strategy. While preparing the documents for the transplantation, his blood pressure and urination became difficult to control with any medication. Then, we began to perform percutaneous cardiopulmonary support (PCPS) to prevent heart failure. His organ failure improved by using PCPS, but the opening movement of the aortic valve could not be detected. A massive cerebral hemorrhage occurred on the 5th day after the initiation of PCPS, when we had planned to implant the artificial heart. He lost the indication for an artificial heart followed by heart transplantation and died after removal of PCPS. Unfortunately, an autopsy was not permitted. PCPS-induced complications are not rare and sometimes are severe, which is why we need to change the therapeutic strategy. However, we currently choose to use an artificial heart or perform a heart transplantation only rarely in Japan. We cannot expect to rapidly increase the number of heart transplantations, so developing artificial hearts for implantation and improving their safety is most important for the therapy of patients with severe cardiomyopathy.