【摘要】 目的 探讨肺动脉高压患者药物靶向治疗的效果与耐受性。 方法 回顾分析2008年1月〖CD3/5〗2009年8月期间8例肺动脉高压患者分别接受波生坦及西地那非治疗的临床资料,评估其临床表现、WHO肺动脉高压功能分级、6 min步行距离及肺动脉收缩压在基线及治疗3个月后的变化。 结果 治疗后3个月,患者均能耐受药物治疗,无严重不良反应发生。WHO肺动脉高压功能分级在治疗前平均(31±04),治疗后为(23±09),明显得到改善(Plt;005)。肺动脉收缩压在治疗前平均(695±112 ) mm Hg(1 mm Hg=0133 kPa),治疗后为(483±124) mm Hg,明显降低(Plt;005)。6 min步行距离在治疗前平均(324±48) m,治疗后为(400±43) m,明显延长(Plt;005)。 结论 肺动脉高压患者药物靶向治疗的疗效显著,且耐受良好。【Abstract】 Objective To examine the effects of target medical therapy in patients with pulmonary arterial hypertension(PAH). Methods To determine the safety and efficacy of bosentan and sildenafil in eight patients with PAH.The patients’ clinical features, six minutes walking diastance, WHO functional class and systolic pulmonary arterial pressure (SPAP) were measured at baseline and at three months after initiating target medial treatment. Results At the three months followup assessments, WHO functional class was improved with 31±04 vs 23±09 (Plt;005); SPAP was significantly decreased with(695±112 ) mm Hg vs (483±124) mm Hg (Plt;005), the six minutes walking distance was significantly increased with(324±48) m vs(400±43) m (Plt;005). Target medical treatment was well tolerated. Conclusion Target medical treatment is well tolerated and has beneficial effects on PAH.
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 explore the clinical applications of 3D-CT reconstruction combined with 3D printing in the analysis of anatomical types and variations of bilateral pulmonary arteries. MethodsFrom January 2019 to February 2022, the clinical data of 547 patients who underwent anatomical lung lesion resection in our hospital were retrospectively collected. They were divided into a 3D-CT reconstruction plus printing technology group (n=298, 87 males and 211 females aged 53.84±12.94 years), a 3D-CT reconstruction group (n=148, 55 males and 93 females aged 54.21±11.39 years), and a non-3D group (n=101, 28 males and 73 females aged 53.17±10.60 years). ResultsIn the 3D-CT reconstruction plus printing technology group, the operation time of patients (right: 125.61±20.99 min, left: 119.26±28.44 min) was shorter than that in the 3D-CT reconstruction group (right: 130.48±11.28 min, left: 125.51±10.59 min) and non-3D group (right: 134.45±10.20 min, left: 130.44±9.53 min), which was not associated with the site of surgery; intraoperative blood loss (right: 20.92±8.22 mL, left: 16.85±10.43 mL) was not statistically different compared with the 3D-CT reconstruction group (right: 21.13±8.97 mL, left: 19.09±7.01 mL), but was less than that of the non-3D group (right: 24.44±10.72 mL, left: 23.72±11.45 mL). Variation was found in the right pulmonary artery of 7 (3.91%) patients and in the left pulmonary artery of 21 (17.65%) patients. We first found four-branched lingual pulmonary artery in 2 patients.ConclusionPreoperative CT image computer-assisted 3D reconstruction combined with 3D printing technology can help surgeons to formulate accurate surgical plans, shorten operation time and reduce intraoperative blood loss.
Pulmonary hypertension (PH), characterized by diverse etiologies and intricate pathological mechanisms, is a complex cardiopulmonary vascular disorder featuring high morbidity and mortality. Percutaneous pulmonary artery denervation (PADN) represents an emerging interventional treatment method, which shows good prospects in the clinical practice of PH. The PADN has attained preliminary achievements in terms of safety and efficacy. Nevertheless, its long-term prognosis, the characteristics of the appropriate patient populations, and the optimization strategies combined with targeted pharmacotherapy remain to be further explored. This article reviews the current clinical applications of PADN as well as the challenges it confronts.
ObjectiveTo summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression.MethodsThe clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed.ResultsOne patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201).ConclusionFor children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.