Lu Ting, Tang Mi, Wu Zhongshi, Huang Can
Department of Cardiovascular Surgery, Second Xiangya Hospital of Central South University, Changsha, Hunan, China.
Heart Surg Forum. 2019 May 8;22(3):E213-E214. doi: 10.1532/hsf.2313.
Increased pulmonary vascular resistance index (PVR) leads to several complications in patients after a Fontan operation. This increase is mainly attributed to the overexpression of endothelin-1 for a long duration after the Fontan procedure. Here, we describe the case of a 3-year-old boy with a failed Fontan operation who was treated with bosentan, an endothelin-1 receptor blocker.
Cardiac catheterization was performed, which showed a main pulmonary artery pressure (MPAP) of 19 mmHg and PVRI of 5.6 woods/m2. Oral bosentan regimen at a dose of 31.25 mg was initiated twice a day. The treatment was continued as pleural effusion and ascites persisted. No adverse events were observed, and the treatment was well tolerated. Pleural effusion disappeared, and ascites decreased markedly after 4 weeks, whereas the MPAP was 15 mmHg and the PVRI was 4.3 woods/m2. After 3 months of bosentan therapy, the MPAP was 12 mmHg and the PVRI was 4.1 woods/m2.
We observed that bosentan reduces the PVRI and complications such as pleural effusion and ascites after a failed Fontan procedure.
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