Objective To observe the efficacy and safety of sildenafil in one case of PoPH patients. Methods A case of PoPH patient who was successfully treated with sildenafil was retrospectively analyzed，and literatures related sildenafil and PoPH were reviewed. Results A case of PoPH patient admitted with dyspnea on extetion was diagnosed with echocardiography，gastroscrope and other examinations. She was treated with sildenafil and responded well by decreased pulmonary arterial pressure，improved exercise tolerance and life quality，without obvious side effects. Literatures review suggested that PoPH is an under-recognized but life-threatening complication of cirrhosis or noncirrhotic portal hypertension with poor prognosis which exists in decompensated chronic liver diseases patients. The most classic symptoms of PoPH is progressive dyspnea on extertion. PoPH patients may benefit from sildenafil therapy with decreased pulmonary arterial pressure and improved life quality. Conclusions Theawareness of PoPH should be increased and Sildenafil may be effective and safe for PoPH patients. However，more evidences from clinical trials are needed.
ObjectiveTo investigate the effect and safety of the pulmonary vasodilators in pediatric patients after Fontan operation. MethodsThis retrospective study evaluated the clinical utility of pulmonary vasodilators in pediatric patients with Fontan surgery. Between January and December 2013, 42 consecutive patients with single ventricle physiology who underwent a modified Fontan procedure of total cavapulmonary collection (TCPC) were enrolled. After extubated oral intake started, 24 patients (the treated group) received the pulmonary vasodilator treatment, while 18 patients (the untreated group) didn't not receive the treatment. ResultsNo inpatient death occurred after surgery. The primary endpoints were time of stay in hospital and time of chest tube drainage. There was no statistical difference between the two groups. Instead, patients in the treatment group seemed to have longer time of hospital stay (22 to 21 days) and chest tube drainage (14.0 to 8.5 days) than those in the untreated group. Compared with the untreated group, patients in the treatment group were younger (P=0.082) with no statistical difference, and had higher postoperative Lac with statistic difference (P=0.031), longer ventilation time with no statistical difference (P=0.050), and lower postoperative oxygen saturation with statistic difference (P=0.065). No clinically significant adverse events relating to pulmonary vasodilator therapy occurred during this study and, in particular, no significant abnormalities in hepatic, renal function tests were observed in pediatric Fontan patients. ConclusionsPulmonary vasodilator agents were found to be particularly used in the patients with serious conditions in our study. Our study results failed to show significant improvement of pulmonary vasodilator drugs after Fontan surgery in decreasing time of pleural drainage and time of stay in hospital.
The success of staged Fontan palliation for patients with single ventricle is related to low pulmonary vascular resistance (PVR). The complications of high PVR in Fontan physiology are numerous, such as low exercise tolerance, low cardiac output, ventricular function failure and protein-losing enteropathy; eventually it leads to failing Fontan. Therefore, a low PVR is crucial in Fontan patients. Now, targeted therapies decreasing PVR has been an advanced research hotspot in Fontan patients. In this review we present an overview of the safety and efficacy of the therapy with bosentan or sildenafil on elevated pulmonary artery pressure and pulmonary vascular resistance in Fontan patients.
Objective To explore the clinical effect and safety of sildenafil combined with bosentan in the treatment of connective tissue disease associated moderate-severe pulmonary arterial hypertension (CTD-MS-PAH ). Methods Seventy-six patients with CTD-MS-PAH during January 2013 to January 2017 were collected and divided into group A (41 cases) and group B (35 cases) using a stratified random sampling approach. The patients in group A received 25 mg sildenafil tablet therapy, three times a day. The patients group B received 25 mg sildenafil and 62.5 mg bosentan tablet therapy, twice a day. Both groups were treated for 12 weeks. Before and after the trial, all patients undertook six-minute walk test. Meanwhile the Borg dyspnea index score, the pulmonary artery systolic pressure (PASP), right ventricular diameter (RVD), B-type natriuretic peptide (BNP), the partial pressure of oxygen in artery (PaO2), blood pressure, heart rate, liver and kidney function were all measured. Results After the therapy, six-minute walking distance increased, Borg dyspnea index score decreased, PASP, RVD and plasma BNP decreased, and PaO2 increased in both groups (all P<0.05), but group B was superior to group A (allP<0.05). There were no significant differences in blood pressure, heart rate, liver or kidney function compared with those before the treatment in both groups (allP>0.05). Conclusion Sildenafil combined with bosentan can significantly decrease the level of pulmonary arterial pressure and effectively improve the cardiopulmonary function in CTD-MS-PAH patients with good safety.