Clinical features, laboratory findings and treatment of juxtaglomerular cell tumors: a systemic review.

Juxtaglomerular cell tumors (JGCTs) or reninoma are rare kidney tumors leading to secondary hypertension, and the non-specific clinical manifestations bring about challenges to the diagnosis. This study is to summarize the clinical features, laboratory findings, and treatment of JGCTs. The PubMed, EMBASE database, and manual search were utilized to find all cases, and 158 reports containing 261 patients were identified. Data on patients' demographics, clinical features, diagnostic methods, and treatment options were collected and analyzed. JGCTs occurred predominantly in female patients (female to male ratio, 2.1:1). The median age of patients was 25 years (IQR:18-34 years). Hypertension (97.24%) was the cardinal manifestation. Hypokalemia was reported in 78.71% (159/202) of subjects, and normal serum potassium accounted for 20.79% (42/202). In cases with assessed plasma renin activity (PRA) levels, the median PRA was 7.89 times the upper limit of normal (IQR:3.58-14.41), and 3.82% (5/131) of cases in the normal range. Tumors were detected in 97.8% (175/179) computed tomography (CT), 94.7% (72/76) magnetic resonance imaging (MRI), and 81.5% (110/135) ultrasound, respectively. For 250/261 patients undergoing surgical procedures, 89.14% (197/221), 94.94% (150/158), and 100% (131/131) of patients were restored to normal blood pressure, PRA, and serum potassium, respectively. JGCTs are commonly associated with hypertension, hypokalemia, and hyperreninemia, whereas patients with normotension, normokalemia, and PRA should be systematically pursued after drug-elution lasting for 2 weeks. CT and MRI are more sensitive imaging diagnostic methods. The blood pressure and biochemical parameters of most patients returned to normal after surgery.

Pulmonary artery multi-focal stenosis in a young girl with multi-vessel fibromuscular dysplasia.

The Effect of Endovascular Treatment of Renal Artery Stenoses on Coexistent Aneurysms Associated with Fibromuscular Dysplasia.

PURPOSE:Endovascular data on patients with coexistent renal artery stenosis (RAS) and renal artery aneurysm (RAA) caused by fibromuscular dysplasia (FMD) are scarce, and the outcomes from RAS-specific treatment on RAA remain unclear. This study aimed to evaluate the safety and effectiveness of RAS-specific endovascular management in patients with coexisting RAA caused by FMD.MATERIALS AND METHODS:Clinical and endovascular data on 19 patients with coexistent RAS and RAA caused by FMD who underwent RAS-specific endovascular therapy were analyzed prospectively. An RAA located within 10 mm of the RAS was defined as a stenosis-related RAA (SRAA), and long-term outcomes were evaluated.RESULTS:Nineteen patients (24 RASs and 30 RAAs) underwent endovascular therapy. Twenty-one RASs were treated with balloon angioplasty alone, whereas 3 RASs were treated with stent implantation. None of the RAAs were treated directly. During an average of 4.2 years ± 3.2 of follow-up, systolic and diastolic blood pressures decreased from 183.0 mm Hg ± 19.5 and 120.2 mm Hg ± 19.0 to 127.9 mm Hg ± 10.3 and 80.9 mm Hg ± 6.9, respectively; the number of antihypertensive medications reduced from 1.7 ± 1.0 to 0.8 ± 0.3 (for all, P < .001). The serum creatinine level remained stable. The maximum diameter of all RAAs decreased from 14.6 mm ± 9.7 to 11.3 mm ± 8.4 (P < .001). There was a significant difference in the improvement rate of the maximum diameter between SRAAs (65.0%, 13 of 20) and non-SRAAs (20.0%, 2 of 10) (P = .019).CONCLUSIONS:RAS-specific endovascular therapy is safe and effective and possibly aids in preventing RAA progression in patients with FMD with coexistent RAS and RAA.

Adrenal Venous Sampling Via an Antecubital Approach in Primary Aldosteronism: A Multicenter Study.

CONTEXT:Adrenal venous sampling (AVS) is considered the gold standard for differentiating unilateral and bilateral forms of primary aldosteronism. Currently, almost all AVS procedures are performed via femoral vein access.OBJECTIVE:The aim of this study was to evaluate the success rate and safety of AVS via an antecubital approach.METHODS:In a retrospective multicenter study involving 7 Chinese medical centers, patients with primary aldosteronism who underwent AVS via an antecubital approach between January 2012 and December 2018 were analyzed. Successful sampling was determined by a selectivity index (cortisol in the adrenal vein/cortisol in inferior vena cava) greater than 2.RESULTS:A total of 1226 participants (mean age, 47.1 years; 57.9% male) were included. The puncture site was right and left antecubital vein in 1211 (98.8%), and 15 (1.2%) patients. The access of 6 patients (0.5%) was changed to right femoral vein due to the failure of antecubital vein cannulation or anatomic variation of adrenal vein. The success rate of bilateral, right, and left sampling was 91.5%, 94.9%, and 95.1%, respectively. The success rate of bilateral, right, and left sampling increased from 82.9%, 87.1%, and 88.6% during the initial 70 cases (total of initial 10 cases at each center) to 92.0% (P = .012), 95.3% (P = .008), and 95.5% (P = .018) with subsequent cases. Adrenal vein rupture occurred in 5 patients (0.41%), with no sequelae.CONCLUSION:This multicenter study demonstrates that AVS via an antecubital approach is safe and feasible, with a high rate of successful sampling, which may be an alternative to the femoral vein access method.

Identifying and comparing low-value care recommendations for coronary heart disease prevention, diagnosis, and treatment in the US and China.

BACKGROUND:Low-value care provides little or no benefit, causes harm and incurs unnecessary costs. Low-value care for coronary heart disease (CHD) is particularly prevalent in the US and China. Identifying low-value care services is the first step in reducing these services. There is currently limited data on identifying a comprehensive CHD low-value care list in the US and China. We aimed to identify and compare low-value care recommendations for CHD prevention, diagnosis, and treatment in the US and China.METHODS:Clinical practice guidelines (CPGs) related to CHD in the US and China were screened for do-not-do recommendations stating that specific services should be avoided. The similarities and discrepancies of low-value care recommendations for CHD between the two countries were then compared.RESULTS:We found a total of 38 low-value care recommendations in 6 Chinese CPGs and 98 recommendations in 11 US CPGs. In the US, the most common types of low-value care recommendations were therapeutic medications (44, 44.9%), followed by therapeutic procedures (27, 27.6%), diagnostic imaging (16, 16.3%), diagnostic testing (9, 9.2%) and primary prevention (2, 2.0%). In China, the most common types were therapeutic medications (18, 47.4%), followed by therapeutic procedures (13, 34.2%), diagnostic testing (4, 10.5%), and diagnostic imaging (3, 7.9%).CONCLUSION:In this study, a comprehensive list of low-value care for CHD in the US and China was established and potentially become the important targets for de-implementation for both countries. The findings may have important implications for other countries, especially low-and middle-income countries, to reduce low-value care for CHD.

Prevalence and clinical characteristics of renovascular hypertension associated with fibromuscular dysplasia in China.

OBJECTIVES:The aim of this study was to investigate the clinical characteristics of renal artery fibromuscular dysplasia (FMD) in patients in China and identify the cure rate of hypertension after angioplasty.METHODS:Consecutive hypertensive patients with renal artery stenosis caused by FMD who underwent catheter-based angiography, and were followed at two Chinese referral centres, were retrospectively analysed. All patients underwent a detailed investigation, including demographic characteristics, clinical characteristics, biochemical sampling, Doppler ultrasonography of carotid arteries, magnetic resonance angiography (MRA) of the intracranial artery, and CTA or MRA of the abdominal artery and catheter-based renal angiography. Patients were routinely followed up at 1 month, 6 months and every year after the procedure.RESULTS:Among 245 study participants, with a mean diagnosed age of 26.9 ± 9.9 years, 137 (55.9%) were women, and 38 (15.5%) were children. All patients were diagnosed with hypertension at a mean age of 23.4 ± 8.4 years. There were 73.5% focal and 15.2% multivessel cases. Aneurysms, arterial dissections and total occlusions were found in 21.6, 4.1 and 12.2% of patients, respectively. Patients with multifocal FMD were older (26.0 vs. 23.7 years, P  = 0.021) and more often female (70.8 vs. 50.6%, P  = 0.004). Among children with renal FMD, 55.2% were men, and 86.8% were focal. After a median follow-up of 7.0 years, multifocal FMD had a higher cure rate of hypertension than focal FMD after revascularization (71.7 vs. 55.8%, P  = 0.032).CONCLUSION:In a cohort of mostly young Chinese patients, the prevalence of hypertension associated with renal FMD is similar in both sexes. Focal FMDs were more frequent than the multifocal ones and, after angioplasty, were associated with a worse blood pressure outcome.

[Impact of interventional therapy on top of standard drug therapy on left ventricular structure and function in HFrEF patients complicating with middle aortic syndrome caused by Takayasu arteritis].

Objective: To evaluate the impact of interventional therapy on top of drug therapy on cardiac function and structure in heart failure with reduced ejection fraction (HFrEF) patients complicating with middle aortic syndrome caused by Takayasu arteritis (TA-MAS). Methods: It was a retrospective longitudinal study. The data of patients with TA-MAS and HFrEF, who received interventional therapy on top of drug therapy in Fuwai Hospital from January 2010 to September 2020, were collected and analyzed. Baseline clinical data (including demographic data, basic treatment, etc.) were collected through the electronic medical record system. Changes of indexes such as New York Heart Association (NYHA) classification, N-terminal pro-brain natriuretic peptide (NT-proBNP), left ventricular end diastolic diameter (LVEDD), left ventricular ejection fraction (LVEF), left ventricular mass index (LVMI) before and after therapy were analyzed. Results: A total of 10 patients were collected. There were 8 females in this patient cohort, age was (18.4±5.0) years and onset age was (15.3±5.0) years. All 10 patients received standard heart failure medication therapy in addition to hormone and/or immunosuppressive anti-inflammatory therapy, but cardiac function was not improved, so aortic balloon dilatation and/or aortic stenting were performed in these patients. The median follow-up was 3.3(1.3, 5.6) years. On the third day after interventional therapy, the clinical symptoms of the 10 patients were significantly improved, NYHA classfication was restored from preoperative Ⅲ/Ⅳ to Ⅱ at 6 months post intervention(P<0.05). Compared with preoperation, NT-proBNP (P=0.028), LVEDD (P=0.011) and LVMI (P=0.019) were significantly decreased, LVEF was significantly increased (P<0.001) at 6 months after operation. Compared with preoperation, NT-proBNP (P=0.016), LVEDD (P=0.023) and LVMI (P=0.043) remained decreased, LVEF remained increased (P<0.001) at 1 year after operation. Conclusion: Results from short and medium term follow-up show that interventional therapy on top of heart failure drug therpay can effectively improve left cardiac function and attenuate cardiac remodeling in patients with TA-MAS comorbid with HFrEF.

Characteristics of four-limb blood pressure and brachial-ankle pulse wave velocity in Chinese patients with Takayasu arteritis.

Purpose: Takayasu arteritis (TA) is a rare disease, which is frequently misdiagnosed or its diagnosis can be missed. This study aimed to analyse the characteristics of four-limb blood pressure (4LBP) and brachial-ankle pulse wave velocity (baPWV) in patients with TA, which could be useful in disease detection.Materials and Methods: We consecutively enrolled 182 patients with TA at Fuwai Hospital between January 2013 and January 2016. Pulse pressure (PP), pulsatile index (PI), inter-arm systolic blood pressure (SBP) difference (IASBPD), inter-leg SBP difference (ILSBPD), ankle-brachial index (ABI), baPWV, and inter-side baPWV difference (ΔbaPWV) were analysed and compared with those of age-, sex-, and SBP-matched participants without cardiovascular diseases.Results: In the TA group, the diastolic blood pressure was lower (67.4 ± 23.7 vs 84.1 ± 15.0 mmHg), PP was larger (69.7 ± 23.6 vs 53.7 ± 10.6 mmHg), PI was higher (1.3 ± 2.1 vs. 0.6 ± 0.1 mmHg), IASBPD was larger (18.2 ± 24.1 vs 4.2 ± 3.3 mmHg), and ILSBPD was larger (10.7 ± 15.0 vs 5.3 ± 4.1 mmHg) than those of the controls (all p < 0.01). Moreover, the proportions of PP >70 mmHg (36.8% vs 4.4%), PI > 1.0 (40.1% vs 2.2%), IASBPD >15 mmHg (34.6% vs. 0%), highest ABI >1.4 (17.6% vs. 0%), ILSBPD >15 mmHg (14.8% vs. 3.3%), lowest ABI < 0.9 (24.7% vs 2.2%), and ΔbaPWV > 185 cm/s (28.6% vs. 1.1%) were significantly greater in the TA group than in the control group (all p < 0.01). Approximately 80.8% of patients with TA (vs. 10.4% of controls) presented with at least one of these seven parameters (p = 0.000).Conclusion: The characteristics of 4LBP and baPWV in most patients with TA were abnormal, which helped us perform non-invasive primary screening and comprehensive evaluation of vascular lesions in such patients.

Unifocal progressed to multifocal renal artery fibromuscular dysplasia.

Evaluation of the role of combining inter-arm systolic pressure difference and derivatives of pulse volume recording in detecting subclavian artery stenosis.

Background:Subclavian artery stenosis (SAS) is a peripheral arterial disease of asymptomatic appearance and disastrous consequences. The traditional screening method remains unsatisfactory.Objective:The study aimed to assess the diagnostic performances of inter-arm systolic pressure difference (IASBPD), derivatives of pulse volume recording (PVR), and their combination in detecting subclavian artery stenosis.Materials and methods:The present study was a retrospective analysis of clinical data from inpatients suspected of supra-arch artery stenosis in Fuwai hospital during 1 year, who underwent selective arterial angiographies. We obtained simultaneous blood pressure measurements on four limbs and pulse waves for calculating IASBPD and PVR derivatives prior to the angiographies. We utilized the receiver operating characteristic curve (ROC) to calculate the optimal cut-off value of IASBPD, upstroke time (UT), and upstroke time per cardiac cycle (UTCC) for detecting SAS. Moreover, we compared the sensitivity and specificity of IASBPD, UT, UTCC, and their combinations for diagnosing SAS (Clinical trial number: NCT03521739).Results:We consecutively enrolled 320 eligible patients. Based on SAS's definition of stenosis above 50%, the area under the curve of IASBPD, UT, and UTCC were 0.84, 0.76, and 0.80 (P < 0.001). And their corresponding cut-off points were 9 mmHg, 202 milliseconds, and 23.2%. The sensitivity and specificity of IASBPD ≥ 9 mmHg were 57.0 and 94.1%. UT ≥ 202 ms and UTCC ≥ 23.2% yielded similar sensitivity (72.6 vs. 72.6%, P > 0.05), but UTCC had higher specificity (81.1 vs. 72.4%, P < 0.05). The sensitivity of the combination of IASBPD and UT (85.2%) or UTCC (78.5%) was significantly higher than IASBPD alone (57%, P < 0.05). The specificity of either combination decreased to 67.6 and 76.8% (P < 0.05).Conclusions:This present study showed that the combinations of IASBPD and PVR-derived parameters promoted diagnostic sensitivity and preserved adequate specificity than those alone for detecting SAS.

Clinical features, laboratory findings and treatment of juxtaglomerular cell tumors: a systemic review.

Juxtaglomerular cell tumors (JGCTs) or reninoma are rare kidney tumors leading to secondary hypertension, and the non-specific clinical manifestations bring about challenges to the diagnosis. This study is to summarize the clinical features, laboratory findings, and treatment of JGCTs. The PubMed, EMBASE database, and manual search were utilized to find all cases, and 158 reports containing 261 patients were identified. Data on patients' demographics, clinical features, diagnostic methods, and treatment options were collected and analyzed. JGCTs occurred predominantly in female patients (female to male ratio, 2.1:1). The median age of patients was 25 years (IQR:18-34 years). Hypertension (97.24%) was the cardinal manifestation. Hypokalemia was reported in 78.71% (159/202) of subjects, and normal serum potassium accounted for 20.79% (42/202). In cases with assessed plasma renin activity (PRA) levels, the median PRA was 7.89 times the upper limit of normal (IQR:3.58-14.41), and 3.82% (5/131) of cases in the normal range. Tumors were detected in 97.8% (175/179) computed tomography (CT), 94.7% (72/76) magnetic resonance imaging (MRI), and 81.5% (110/135) ultrasound, respectively. For 250/261 patients undergoing surgical procedures, 89.14% (197/221), 94.94% (150/158), and 100% (131/131) of patients were restored to normal blood pressure, PRA, and serum potassium, respectively. JGCTs are commonly associated with hypertension, hypokalemia, and hyperreninemia, whereas patients with normotension, normokalemia, and PRA should be systematically pursued after drug-elution lasting for 2 weeks. CT and MRI are more sensitive imaging diagnostic methods. The blood pressure and biochemical parameters of most patients returned to normal after surgery.

Pulmonary artery multi-focal stenosis in a young girl with multi-vessel fibromuscular dysplasia.

The Effect of Endovascular Treatment of Renal Artery Stenoses on Coexistent Aneurysms Associated with Fibromuscular Dysplasia.

PURPOSE:Endovascular data on patients with coexistent renal artery stenosis (RAS) and renal artery aneurysm (RAA) caused by fibromuscular dysplasia (FMD) are scarce, and the outcomes from RAS-specific treatment on RAA remain unclear. This study aimed to evaluate the safety and effectiveness of RAS-specific endovascular management in patients with coexisting RAA caused by FMD.MATERIALS AND METHODS:Clinical and endovascular data on 19 patients with coexistent RAS and RAA caused by FMD who underwent RAS-specific endovascular therapy were analyzed prospectively. An RAA located within 10 mm of the RAS was defined as a stenosis-related RAA (SRAA), and long-term outcomes were evaluated.RESULTS:Nineteen patients (24 RASs and 30 RAAs) underwent endovascular therapy. Twenty-one RASs were treated with balloon angioplasty alone, whereas 3 RASs were treated with stent implantation. None of the RAAs were treated directly. During an average of 4.2 years ± 3.2 of follow-up, systolic and diastolic blood pressures decreased from 183.0 mm Hg ± 19.5 and 120.2 mm Hg ± 19.0 to 127.9 mm Hg ± 10.3 and 80.9 mm Hg ± 6.9, respectively; the number of antihypertensive medications reduced from 1.7 ± 1.0 to 0.8 ± 0.3 (for all, P < .001). The serum creatinine level remained stable. The maximum diameter of all RAAs decreased from 14.6 mm ± 9.7 to 11.3 mm ± 8.4 (P < .001). There was a significant difference in the improvement rate of the maximum diameter between SRAAs (65.0%, 13 of 20) and non-SRAAs (20.0%, 2 of 10) (P = .019).CONCLUSIONS:RAS-specific endovascular therapy is safe and effective and possibly aids in preventing RAA progression in patients with FMD with coexistent RAS and RAA.

Adrenal Venous Sampling Via an Antecubital Approach in Primary Aldosteronism: A Multicenter Study.

CONTEXT:Adrenal venous sampling (AVS) is considered the gold standard for differentiating unilateral and bilateral forms of primary aldosteronism. Currently, almost all AVS procedures are performed via femoral vein access.OBJECTIVE:The aim of this study was to evaluate the success rate and safety of AVS via an antecubital approach.METHODS:In a retrospective multicenter study involving 7 Chinese medical centers, patients with primary aldosteronism who underwent AVS via an antecubital approach between January 2012 and December 2018 were analyzed. Successful sampling was determined by a selectivity index (cortisol in the adrenal vein/cortisol in inferior vena cava) greater than 2.RESULTS:A total of 1226 participants (mean age, 47.1 years; 57.9% male) were included. The puncture site was right and left antecubital vein in 1211 (98.8%), and 15 (1.2%) patients. The access of 6 patients (0.5%) was changed to right femoral vein due to the failure of antecubital vein cannulation or anatomic variation of adrenal vein. The success rate of bilateral, right, and left sampling was 91.5%, 94.9%, and 95.1%, respectively. The success rate of bilateral, right, and left sampling increased from 82.9%, 87.1%, and 88.6% during the initial 70 cases (total of initial 10 cases at each center) to 92.0% (P = .012), 95.3% (P = .008), and 95.5% (P = .018) with subsequent cases. Adrenal vein rupture occurred in 5 patients (0.41%), with no sequelae.CONCLUSION:This multicenter study demonstrates that AVS via an antecubital approach is safe and feasible, with a high rate of successful sampling, which may be an alternative to the femoral vein access method.

Identifying and comparing low-value care recommendations for coronary heart disease prevention, diagnosis, and treatment in the US and China.

BACKGROUND:Low-value care provides little or no benefit, causes harm and incurs unnecessary costs. Low-value care for coronary heart disease (CHD) is particularly prevalent in the US and China. Identifying low-value care services is the first step in reducing these services. There is currently limited data on identifying a comprehensive CHD low-value care list in the US and China. We aimed to identify and compare low-value care recommendations for CHD prevention, diagnosis, and treatment in the US and China.METHODS:Clinical practice guidelines (CPGs) related to CHD in the US and China were screened for do-not-do recommendations stating that specific services should be avoided. The similarities and discrepancies of low-value care recommendations for CHD between the two countries were then compared.RESULTS:We found a total of 38 low-value care recommendations in 6 Chinese CPGs and 98 recommendations in 11 US CPGs. In the US, the most common types of low-value care recommendations were therapeutic medications (44, 44.9%), followed by therapeutic procedures (27, 27.6%), diagnostic imaging (16, 16.3%), diagnostic testing (9, 9.2%) and primary prevention (2, 2.0%). In China, the most common types were therapeutic medications (18, 47.4%), followed by therapeutic procedures (13, 34.2%), diagnostic testing (4, 10.5%), and diagnostic imaging (3, 7.9%).CONCLUSION:In this study, a comprehensive list of low-value care for CHD in the US and China was established and potentially become the important targets for de-implementation for both countries. The findings may have important implications for other countries, especially low-and middle-income countries, to reduce low-value care for CHD.

Prevalence and clinical characteristics of renovascular hypertension associated with fibromuscular dysplasia in China.

OBJECTIVES:The aim of this study was to investigate the clinical characteristics of renal artery fibromuscular dysplasia (FMD) in patients in China and identify the cure rate of hypertension after angioplasty.METHODS:Consecutive hypertensive patients with renal artery stenosis caused by FMD who underwent catheter-based angiography, and were followed at two Chinese referral centres, were retrospectively analysed. All patients underwent a detailed investigation, including demographic characteristics, clinical characteristics, biochemical sampling, Doppler ultrasonography of carotid arteries, magnetic resonance angiography (MRA) of the intracranial artery, and CTA or MRA of the abdominal artery and catheter-based renal angiography. Patients were routinely followed up at 1 month, 6 months and every year after the procedure.RESULTS:Among 245 study participants, with a mean diagnosed age of 26.9 ± 9.9 years, 137 (55.9%) were women, and 38 (15.5%) were children. All patients were diagnosed with hypertension at a mean age of 23.4 ± 8.4 years. There were 73.5% focal and 15.2% multivessel cases. Aneurysms, arterial dissections and total occlusions were found in 21.6, 4.1 and 12.2% of patients, respectively. Patients with multifocal FMD were older (26.0 vs. 23.7 years, P  = 0.021) and more often female (70.8 vs. 50.6%, P  = 0.004). Among children with renal FMD, 55.2% were men, and 86.8% were focal. After a median follow-up of 7.0 years, multifocal FMD had a higher cure rate of hypertension than focal FMD after revascularization (71.7 vs. 55.8%, P  = 0.032).CONCLUSION:In a cohort of mostly young Chinese patients, the prevalence of hypertension associated with renal FMD is similar in both sexes. Focal FMDs were more frequent than the multifocal ones and, after angioplasty, were associated with a worse blood pressure outcome.

[Impact of interventional therapy on top of standard drug therapy on left ventricular structure and function in HFrEF patients complicating with middle aortic syndrome caused by Takayasu arteritis].

Objective: To evaluate the impact of interventional therapy on top of drug therapy on cardiac function and structure in heart failure with reduced ejection fraction (HFrEF) patients complicating with middle aortic syndrome caused by Takayasu arteritis (TA-MAS). Methods: It was a retrospective longitudinal study. The data of patients with TA-MAS and HFrEF, who received interventional therapy on top of drug therapy in Fuwai Hospital from January 2010 to September 2020, were collected and analyzed. Baseline clinical data (including demographic data, basic treatment, etc.) were collected through the electronic medical record system. Changes of indexes such as New York Heart Association (NYHA) classification, N-terminal pro-brain natriuretic peptide (NT-proBNP), left ventricular end diastolic diameter (LVEDD), left ventricular ejection fraction (LVEF), left ventricular mass index (LVMI) before and after therapy were analyzed. Results: A total of 10 patients were collected. There were 8 females in this patient cohort, age was (18.4±5.0) years and onset age was (15.3±5.0) years. All 10 patients received standard heart failure medication therapy in addition to hormone and/or immunosuppressive anti-inflammatory therapy, but cardiac function was not improved, so aortic balloon dilatation and/or aortic stenting were performed in these patients. The median follow-up was 3.3(1.3, 5.6) years. On the third day after interventional therapy, the clinical symptoms of the 10 patients were significantly improved, NYHA classfication was restored from preoperative Ⅲ/Ⅳ to Ⅱ at 6 months post intervention(P<0.05). Compared with preoperation, NT-proBNP (P=0.028), LVEDD (P=0.011) and LVMI (P=0.019) were significantly decreased, LVEF was significantly increased (P<0.001) at 6 months after operation. Compared with preoperation, NT-proBNP (P=0.016), LVEDD (P=0.023) and LVMI (P=0.043) remained decreased, LVEF remained increased (P<0.001) at 1 year after operation. Conclusion: Results from short and medium term follow-up show that interventional therapy on top of heart failure drug therpay can effectively improve left cardiac function and attenuate cardiac remodeling in patients with TA-MAS comorbid with HFrEF.

Characteristics of four-limb blood pressure and brachial-ankle pulse wave velocity in Chinese patients with Takayasu arteritis.

Purpose: Takayasu arteritis (TA) is a rare disease, which is frequently misdiagnosed or its diagnosis can be missed. This study aimed to analyse the characteristics of four-limb blood pressure (4LBP) and brachial-ankle pulse wave velocity (baPWV) in patients with TA, which could be useful in disease detection.Materials and Methods: We consecutively enrolled 182 patients with TA at Fuwai Hospital between January 2013 and January 2016. Pulse pressure (PP), pulsatile index (PI), inter-arm systolic blood pressure (SBP) difference (IASBPD), inter-leg SBP difference (ILSBPD), ankle-brachial index (ABI), baPWV, and inter-side baPWV difference (ΔbaPWV) were analysed and compared with those of age-, sex-, and SBP-matched participants without cardiovascular diseases.Results: In the TA group, the diastolic blood pressure was lower (67.4 ± 23.7 vs 84.1 ± 15.0 mmHg), PP was larger (69.7 ± 23.6 vs 53.7 ± 10.6 mmHg), PI was higher (1.3 ± 2.1 vs. 0.6 ± 0.1 mmHg), IASBPD was larger (18.2 ± 24.1 vs 4.2 ± 3.3 mmHg), and ILSBPD was larger (10.7 ± 15.0 vs 5.3 ± 4.1 mmHg) than those of the controls (all p < 0.01). Moreover, the proportions of PP >70 mmHg (36.8% vs 4.4%), PI > 1.0 (40.1% vs 2.2%), IASBPD >15 mmHg (34.6% vs. 0%), highest ABI >1.4 (17.6% vs. 0%), ILSBPD >15 mmHg (14.8% vs. 3.3%), lowest ABI < 0.9 (24.7% vs 2.2%), and ΔbaPWV > 185 cm/s (28.6% vs. 1.1%) were significantly greater in the TA group than in the control group (all p < 0.01). Approximately 80.8% of patients with TA (vs. 10.4% of controls) presented with at least one of these seven parameters (p = 0.000).Conclusion: The characteristics of 4LBP and baPWV in most patients with TA were abnormal, which helped us perform non-invasive primary screening and comprehensive evaluation of vascular lesions in such patients.

Unifocal progressed to multifocal renal artery fibromuscular dysplasia.

Evaluation of the role of combining inter-arm systolic pressure difference and derivatives of pulse volume recording in detecting subclavian artery stenosis.

Background:Subclavian artery stenosis (SAS) is a peripheral arterial disease of asymptomatic appearance and disastrous consequences. The traditional screening method remains unsatisfactory.Objective:The study aimed to assess the diagnostic performances of inter-arm systolic pressure difference (IASBPD), derivatives of pulse volume recording (PVR), and their combination in detecting subclavian artery stenosis.Materials and methods:The present study was a retrospective analysis of clinical data from inpatients suspected of supra-arch artery stenosis in Fuwai hospital during 1 year, who underwent selective arterial angiographies. We obtained simultaneous blood pressure measurements on four limbs and pulse waves for calculating IASBPD and PVR derivatives prior to the angiographies. We utilized the receiver operating characteristic curve (ROC) to calculate the optimal cut-off value of IASBPD, upstroke time (UT), and upstroke time per cardiac cycle (UTCC) for detecting SAS. Moreover, we compared the sensitivity and specificity of IASBPD, UT, UTCC, and their combinations for diagnosing SAS (Clinical trial number: NCT03521739).Results:We consecutively enrolled 320 eligible patients. Based on SAS's definition of stenosis above 50%, the area under the curve of IASBPD, UT, and UTCC were 0.84, 0.76, and 0.80 (P < 0.001). And their corresponding cut-off points were 9 mmHg, 202 milliseconds, and 23.2%. The sensitivity and specificity of IASBPD ≥ 9 mmHg were 57.0 and 94.1%. UT ≥ 202 ms and UTCC ≥ 23.2% yielded similar sensitivity (72.6 vs. 72.6%, P > 0.05), but UTCC had higher specificity (81.1 vs. 72.4%, P < 0.05). The sensitivity of the combination of IASBPD and UT (85.2%) or UTCC (78.5%) was significantly higher than IASBPD alone (57%, P < 0.05). The specificity of either combination decreased to 67.6 and 76.8% (P < 0.05).Conclusions:This present study showed that the combinations of IASBPD and PVR-derived parameters promoted diagnostic sensitivity and preserved adequate specificity than those alone for detecting SAS.