第一部分：国人肾动脉纤维肌性发育不良的临床和血管造影特征

背景和目的：最近首个关于纤维肌性发育不良（FMD）国际共识发表，与先前中国FMD报道差异显著，然而国人FMD研究较少，故调查国家心血管病中心连续18年肾动脉纤维肌性发育不良（RA-FMD）病例的临床和血管造影特征。

方法：回顾性连续收集阜外医院2000年11月1日至2018年10月31日确诊RA-FMD的住院患者病历，分析患者人口学、临床和肾动脉造影资料。

结果：18年共诊断RA-FMD142例，其中女性66例（46.5%），男性76例（53.5%），男女比例约为6：5。诊断年龄28.0±9.8（10-55）岁，高血压发病年龄23.9±9.1（6-54）岁。所有患者均存在高血压（收缩压157.6±17.5 mmHg，舒张压102.3±14.2 mmHg），服用降压药物种数2.0±1.2；其中76例（53.5%）伴有头晕头痛；严重并发症包括：脑卒中13例（9.2%，其中脑出血2例、脑梗塞11例），心力衰竭5例（3.5%），肾功能不全7例（4.9%）；肾损害包括：尿蛋白阳性25例（17.6%）、一侧肾萎缩45例（31.7%）、一侧肾梗死6例（4.2%）、一侧肾脏切除4例（2.8%）。肾动脉选择性造影或CT血管造影显示：共179条（63.0%）肾动脉受累，左侧、右侧和双侧病变分别为36例（25.4%）、69例（48.6%）和37例（26.1%）。多灶型和局灶型分别为36例（25.4%）和106例[74.6%，局灶型包括单灶型63例（44.3%）和管型43例（30.3%]。肾动脉狭窄率85.2±12.7%，包括闭塞24例（16.9%），狭窄主要分布在主干中段53例（37.3%）和远段57例（40.1%），而近段20例（14.1%）、第一分支10例（7.0%）和第二分支2例（1.4%）较少。合并肾动脉夹层仅4例（16.9%），而合并肾动脉瘤44例（31.0%），其中38例（86.4%）仅存在1个动脉瘤；左侧、右侧和双侧动脉瘤分别为28例（63.6%）、12例（27.3%）和4例（9.1%）。合并冠状动脉、腹主动脉、颅内动脉以及锁骨下动脉狭窄和脾动脉瘤各1例（0.7%），髂动脉夹层、椎动脉和肠系膜上动脉狭窄各2例（1.4%）。

结论：本研究表明国人RA-FMD的临床及血管造影特征与最新首个国际FMD共识有显著差异，需要进一步开展更大样本的多中心研究。

第二部分：国人肾动脉纤维肌性发育不良的腔内治疗效果评估

背景和目的：目前我国纤维肌发育不良（FMD）所致肾动脉狭窄（RAS）患者行血管腔内治疗的研究少见，故本研究旨在评价经皮腔内血管成形术（PTA）联合选择性支架置入治疗我国FMD所致RAS的安全性和有效性。

方法：回顾性分析2001年6月至2018年10月阜外医院FMD导致RAS伴高血压并接受经皮血管腔内成形治疗的患者临床资料，并且评估患者随访一年的血压、降压药物的使用、肾功能以及不良事件。

结果：共105例患者，年龄为10～64岁（26.7±8.2岁），其中女性52例（49.5%）。所有患者接受血管腔内介入治疗，共涉及124条RAS。其中，30条（24.2%）、64条（51.6%）和30条（24.2%）分别为多灶型、单灶型和管状型。94例（89.5%）和11例（10.5%）患者分别PTA和PTA+支架置入术。血管腔内治疗技术成功率为95.2%（100/105），无严重围术期并发症。随访1年（n=100），患者收缩压和舒张压分别由157.6±17.5和102.3±14.2 mmHg降至129.6±12.3和81.3±11.1 mmHg，降压药物由2.2±1.2降至0.8±1.0（均P<0.001）。高血压的治愈率和好转率分别为49.0%和40.0%。血清肌酐水平保持稳定。原发性和继发性再狭窄率分别为13.4%和5.8%。

结论：经皮血管腔内成形治疗国人FMD所致RAS伴高血压患者，技术安全并且可有效控制患者血压。

Part I：Clinical and Angiographic Characteristics in Chinese Patients with Renal Artery Fibromuscular Dysplasia

Objective: To investigate the clinical and angiographic characteristics in Chinese patients with renal artery fibromuscular dysplasia (RA-FMD) in the National Cardiovascular Center for consecutive 18-years.

Methods: The medical data of patients with RA-FMD diagnosed in Fuwai Hospital from November 1, 2000 to October 31, 2018 were collected continuously.  And the demographic, clinical and renal arteriographic data were analyzed retrospectively.

Results: For 18 years, one hundred and forty-two patients included 76 males and 66 females at the ratio of 6:5. The age of diagnosis was 28 ± 9.8 (10-55) years, and the onset age of hypertension was 23.9 ± 9.1 (6-54) years. All patients suffered from hypertension at admission. Systolic blood pressure was 157.6 ± 17.5 mmHg, diastolic blood pressure was 102.3 ± 14.2 mmHg, and the number of antihypertensive drugs was 2.0 ± 1.2. More than half (53.5%) patients had dizziness or headache. Serious complications included 13 (9.2%) stroke，5 (3.5%) heart failure and 7 (4.9%) renal insufficiency. Renal damage included 25 (17.6%) urinary protein, 45 (31.7%) renal atrophy, 6 (4.2%) renal infarction and 4 (2.8%) nephrectomy. A total of 179 renal arteries were involved, 36 (25.4%), 69 (48.6%) and 37 cases (26.1%) had single left, single right and bilateral lesions, respectively. Multifocal and focal types were 36 (25.4%) and 106 (74.6%) cases respectively, while unifocal and tubular types were 63 (44.3%) and 43 (30.3%) cases respectively. Renal artery stenosis was 85.2±12.7%，included twenty-four cases (16.9%) of renal artery occlusion. Twenty (14.1%), 53 (37.3%) and 57 (40.1%) had renal artery stenosis mainly in the proximal, the middle and the distal trunk. However, 10 (7.0%) of the first branch and 2 (1.4%) of the second branch were less. Forty-four cases (31.0%) had renal aneurysm, and 38 (86.4%) had only one aneurysm. Twenty-eight (63.6%), 12 (27.3%) and 4 (9.1%) had single left, single right and bilateral aneurysms. The stenosis of the coronary artery, abdominal aorta, intracranial artery and subclavian artery as well as splenic aneurysm was in 1 case (0.7%) respectively. Iliac artery dissection, vertebral artery stenosis and superior mesenteric artery stenosis was in 2 cases (1.4%) respectively.

Conclusion: This study indicated that the clinical and angiographic characteristics in Chinese patients with RA-FMD are significantly different from the latest international consensus on FMD, and more large sample multicenter studies are needed.

Part II: Endovascular treatment of renal artery fibromuscular dysplasia in Chinese patients

Background and Objectives: Endovascular data of patients with RAS caused by FMD are scarce in China. This study aimed to evaluate the safety and efficiency of percutaneous transluminal angioplasty (PTA) with selective stenting treating Chinese patients with renal artery stenosis (RAS) due to fibromuscular dysplasia (FMD).

Methods: Clinical data of 105 hypertensive patients with RAS caused by FMD underwent endovascular therapy at a single-site between June 2001 and October 2018 were analyzed retrospectively. Baseline blood pressure, use of antihypertensive medications, renal function, and adverse events at a one-year follow-up were evaluated.

Results: The patients were aged between 10 and 64 years (mean age 26.7±8.2 years) and 52 (49.5%) were female. In total, 105 patients undergoing endovascular therapy involving 124 RASs. Thirty (24.2%), 64 (51.6%) and 30 (24.2%) RASs were multifocal, unifocal and tubular types, respectively. Ninety-four (89.5%) and 11 (10.5%) patients underwent PTA alone and PTA plus stenting, respectively. The technical success rate for endovascular therapy was 95.2% (100/105) without severe complications. During one-year follow-up (n=100), the mean systolic and diastolic blood pressure decreased from 157.6±17.5 and 102.3±14.2 mmHg to 129.6±12.3 and 81.3±11.1 mmHg, respectively, and the number of antihypertensive medications reduced from 2.2±1.2 to 0.8±1.0 (all P<0.001). The cure rate and improved rate of blood pressure was 49.0% and 40.0%, respectively. The serum creatinine levels remained stable. The primary and secondary restenosis rate was 13.4% and 5.8%, respectively. 

Conclusion: Endovascular therapy for treating RAS caused by FMD was technically safe and effective for reducing blood pressure in Chinese patients.