Patient and Caregiver Perspectives on the Diagnosis and Management of Pediatric Renovascular Hypertension

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Rare disease prevalence limits existing data and optimal management remains ill-defined. This study seeks to capture patient and family perceptions around the diagnosis and management of pRVH. Methods The pRVH Patient Centered Outcomes Research Collaborative assembled a global virtual research network of multidisciplinary stakeholders, inclusive of patients and families. We examined self or proxy-reported emotional support, informational support, emotional impact, and number of surgeries/procedures, as well as self-reported pain, global health, fatigue, and social functioning. Descriptive statistics were performed. Results 28 stakeholders (93% parent/guardian; 7% patient self-report) completed the survey. Patients with a surgical or procedural history reported significantly fewer nighttime awakenings due to pain (mean 44 v. 51.5) and less fatigue (48.2 v. 58) than those without. Patients diagnosed before 2 years of age reported better informational (60.9 v. 53.0) and emotional support (57.6 v. 51.5), and better social functioning (64.8 v. 53.0). Respondents unsatisfied with the time to receive a diagnosis indicated more pain (53.3 v. 45.1), fatigue (58.5 v. 50.5), worse global health (37.1 v. 46.6), and worse social functioning (50.8 v. 62.6) than satisfied patients. PCP-diagnosis was associated with worse emotional support (43.2 v. 56.4), informational support (46.9 v. 58.4), and emotional impact (46.6 v. 56.6) than those diagnosed by a specialist. Conclusion These results suggest that early diagnosis, access to centers of excellence, and treatment by multidisciplinary and specialty care teams can optimize pRVH treatment. hypertension pediatric renovascular hypertension vascular surgery interventional radiology Figures Figure 1 Figure 2 Introduction Pediatric renovascular hypertension (pRVH) is a rare form of high blood pressure (BP) resulting from renal artery and abdominal aortic disease. This diagnosis risks significant morbidity and mortality [ 1 , 2 ]. Though the exact incidence of pRVH is unknown, it is estimated to be the cause of 8–10% of cases of secondary hypertension in children and adolescents [ 3 ]. Timely detection and treatment of renovascular hypertension is critical to prevent end organ morbidity and mortality. Unfortunately, delays in recognizing and treating pRVH are common, as this diagnosis is most often made in one of very few high-volume referral centers with robust experience with this disease. The relatively low incidence of this disease has also limited the development of optimal treatment guidelines, resulting in wide variations in disease management. Additionally, while the introduction of novel surgical and endovascular techniques has increased the quantity of treatment modalities available to patients with pRVH, these advances have simultaneously further complicated the landscape of patient care [ 4 – 6 ]. Approaches to navigating the complexities of pRVH treatment have thus far lacked the voices of patients and their families, despite the fact that the value of involving affected individuals and other collaborators in the research process is well-recognized [ 7 , 8 ].The goal of this study is to identify patient and family perspectives relevant to the diagnosis and treatment of pRVH. Methods A virtual research network of patients and families affected by pRVH, otherwise known as the pRVH PCOR Collaborative, was assembled (https://www.prvh-pcor.org/). This group was formalized in December 2019 with the support of an NIH PCORI Capacity-Building Engagement Award. The Stakeholder Engagement in quEstion Development (SEED) Method Toolkit [9] was used to inform the creation of a survey designed to prioritize questions regarding comparative effectiveness of various treatment modalities and patient outcome metrics. This method uses a multi-level stakeholder engagement model that includes a participatory framework to develop stakeholder priorities (i.e. research topics and questions, Figure 1 ). The Advisory Board of the pRVH PCOR Collaborative led the project as the Community-based participatory Research Team. The collaborative’s Critical Stakeholder Group (CSG) was assigned to Topic Groups based on their prior experience and knowledge of pRVH. In partnership with the CSG, the Research Team identified critical areas of focus pertinent to diagnostic and treatment delays. Based on this data, the Research Team created the survey, which included questions about basic patient demographics and clinical data alongside self-reported or proxy-reported measures of satisfaction, as well as PROMIS (Patient-Reported Outcomes Measurement Information System) measures of emotional support, informational support, emotional impact, awakenings from sleep due to pain, global health, fatigue, and social functioning. Survey measures are expanded in Table 1. This survey tool was distributed to all members of the pRVH PCOR Collaborative’s Virtual Research Network (VRN), an online community of patients, family members, clinicians and researchers affected by pRVH (https://www.prvh-pcor.org/prvh-pcor-virtual-research-network/). VRN members were contacted through the Collaborative newsletter and email outreach. Children with pRVH who had grown into adulthood (>18 years) and parents/guardians of affected children (ages 0-17 years) were able to volunteer to anonymously complete the survey. Procedures Participants were provided with brief information about the study prior to answering the first survey question. Written or verbal informed consents were not required, as participants were assumed to have provided consent upon answering the survey questions voluntarily. Participants completed surveys using REDCAP [10], a Health Insurance Portability and Accountability Act (HIPAA)-compliant electronic survey platform. No identifying information was collected in the survey (e.g., names, dates, emails, etc). The survey required an estimated 15 minutes to complete. An IRB exemption was issued by the University of Michigan IRBMED for this study (HUM00206982). Statistical Analyses Normally distributed continuous variables are reported as the mean and SD. Non-normally distributed variables are reported by median and interquartile range. Categorical variables are reported by number and percentage. pRVH Treatment Satisfaction Questionnaire. Descriptive statistics were applied to responses to identify relative frequency of current practices as well as perceived barriers to the treatment of pRVH. Responses were denoted as significant when ≤80% of participants expressed dissatisfaction with current treatment practices. Differences between subgroups were evaluated using analysis of covariance (ANCOVA). pRVH Impact on Health-Related Quality of Life (HRQOL). Descriptive statistics for each of the PROMIS and ASCQ-Me measures are reported. In addition, ANCOVA were used to determine subgroups of participants that differed on these measures. The following subgroups were examined: sex; medication use (0-2 current medications versus ≥ 3 medications); open operative/endovascular procedure history (no interventions versus one or more interventions); recurrent hypertension status (those with current hypertension versus those without); complications in the first 4 weeks of life (those with versus without complications); diagnosis age (diagnosis before or equal to age 2 versus diagnosis at age 3 or older); insurance type (public versus private); diagnosing provider (specialist versus generalist); and satisfaction with length of time to receive a diagnosis (satisfied versus dissatisfied). Responses were controlled for the presence of Williams syndrome. Differences between subgroups were evaluated using ANCOVA. Results Completed surveys were received from 28 stakeholders between January – April 2022 (n=22 mother/female guardian, n=4 father/male guardian; n=2 patient self-report, Table 2). VRN stakeholder engagement was split with equipoise between patient-family respondents and clinician or research investigators (Figure 2). Most respondents were of European ancestry. 62% of respondents were diagnosed with genetic comorbidities in addition to pRVH, with patients most frequently reporting comorbid Williams Syndrome (43%). Additionally, 61% of children surveyed sustained complications during the first 4 weeks of life. 54% experienced motor delays, 46% experienced language delays, 36% experienced cognitive delays, and 21% experienced social delays. 18% reported physical disability of any kind, and 18% had been diagnosed with ADHD. Special education services were used by 43% and other specialized therapies or services were used by 75% of patients (Table 3). pRVH Treatment Satisfaction Questionnaire (Table 4) documented that 89% of respondents felt that the treatment team kept them well-informed, included them in important decisions, and answered questions and explained test results in a way that respondents were able to understand. Furthermore, 68% were satisfied with time to diagnosis. Nevertheless, only 54% of respondents were satisfied with the overall amount of information they received about pRVH, and only 50% found it easy to seek out additional information. The minority of patients and families (40%) agreed that their doctor provided appropriate referrals for treatment. Interestingly, though 75% of respondents had access to a home blood pressure device, only 61% felt confident in monitoring blood pressure at home. Among the 14 respondents who underwent surgical procedures, 93% felt that they received appropriate information about the intervention. While 71% expressed that symptoms improved as a result of the procedure, only 57% were satisfied with the outcome. 100% of respondents felt that the surgical team explained the intervention in a way that they were able to understand. pRVH Impact on HRQOL . Parents and caregivers indicated that their children experience worse overall physical health than the general population. They also noted that their children have worse mental health and greater than average global fatigue than other children with chronic conditions. At the same time, however, they indicated that their children have higher than average social functioning than other children with chronic conditions (Table 5-6). Patients taking ≥3 medications for pRVH reported less overall emotional impact of their diagnosis (mean 46.5 versus 56.5, f=4.83, p=0.04) than those taking 1-2 medications. Even so, those taking ≥3 medications noted increased fatigue (62.3 versus 52.0, f=4.56, p=0.04). Patients with a surgical or procedural history reported significantly less pain that awakened them from sleep (44 versus 51.5, f=12.61, p<0.01) and less fatigue (48.2 versus 58, f=8.41, p=0.01) than those without a surgical history. Patients having at least one surgical procedure reported greater overall emotional impact of pRVH (57.7 versus 53.1, f=5.97, p=0.02) than those who had not undergone any procedures. Furthermore, individuals diagnosed with disease ≤2 years of age reported better informational support (60.9 versus 53.0, f=8.13, p=0.01) and improved social functioning (64.8 versus 53.0, f=6.92, p=0.01) compared to those diagnosed at age 3 or older. Respondents unsatisfied with the time it took to receive a diagnosis indicated more pain awakening them from sleep (53.3 versus 45.1, f=14.09, p<0.01), more fatigue (58.5 versus 50.5, f=4.50, p=0.04), worse global health (37.1 versus 46.6), and worse social functioning (50.8 versus 62.6, f=7.32, p=0.01) than those satisfied respondents, while those perceiving a delay in diagnosis reported worse emotional impact (50.6 versus 57.5, f=5.91, p=0.02). Finally, a primary care-diagnosis was associated with worse emotional support (43.2 versus 56.4, f=8.97, p=0.01), informational support (46.9 versus 58.4, f=5.34, p=0.03), and greater emotional impact (46.6 versus 56.6, f=4.61, p= 0.04) than a diagnosis provided by a specialist, such as a pediatric cardiologist, pediatric nephrologist, or vascular surgeon. Discussion Pediatric renovascular hypertension may be responsible for up to 10% of secondary hypertension in children and adolescents [ 3 ]. Since the pathophysiology of this rare disease impacts multiple organ systems, the journey to diagnosis is highly dependent upon collaboration between multiple specialists as well as the use of many resources that are typically only found at high-volume specialty centers. While discussions regarding this extremely complicated landscape have sought to identify modes of augmenting provider awareness of pRVH, these conversations have, until now, lacked important input from the most important stakeholder group in this disease: patients and their families. Notably, though PROMIS has been applied to a wide range of disorders including cancer, congestive heart failure, depression, arthritis, chronic pain, and multiple sclerosis, this study represents the first large scale application of PROMIS to pRVH [ 11 – 14 ]. The pRVH PCOR Collaborative represents the first global effort designed to bring together critical stakeholders in pRVH, including patients, families, providers, and researchers, to incorporate patient perspectives into disease management and treatment guidelines while also ensuring that patients and their families feel less isolated. Indeed, the data collected here provide unique and groundbreaking insights into the aspects of the clinical course that most define the diagnosis of pRVH to those grappling with it most intimately. While most respondents felt well-informed and supported by their treatment teams, only half were satisfied with the overall information they received about pRVH. This highlights a possible distinction between the emotional support that clinical teams can provide and the literature that they can offer to assist patients and families in their efforts to understand the disease itself. This suggests a need for increased patient-friendly pRVH materials to be created and shared at the time of diagnosis. Similarly, there appears to be a greater role for dedicated education regarding use of blood pressure monitoring at home. Furthermore, a minority of respondents felt that they had received appropriate referrals for further treatment, suggesting that there is a potential role for guidelines suggesting specific referral patterns if pRVH is suspected or diagnosed. Interestingly, some survey responses were discordant with sentiments that might generally be expected from patients with other clinical diagnoses. For example, individuals taking ≥ 3 medications noted an overall decreased emotional impact of this disease on their lives. In contrast, patients with other diagnoses usually anecdotally report that increased number of medications increases the emotional burden that they feel. This discrepancy can potentially be explained by the insidiously destructive impact that elevated blood pressure may have on multiple organ systems, and the relatively specific ability of blood pressure medications or procedures to modulate these effects. Additionally, this data aligns with prior studies that have demonstrated significant relationships between hypertension and both anxiety and depression [ 15 – 18 ]. Lastly, earlier diagnosis appears to be related to improved social functioning and enhanced global health. Diagnosis made by specialists is also associated with improved emotional impact and information support. Taken together, these findings suggest that improving access to specialty centers may aid in decreasing time to diagnosis and the provision of adequate support to pRVH patients. The demographics of the respondent cohort, which was assembled through coordination between multiple high-volume specialty centers, are a limitation to these findings. While age at diagnosis ranged throughout the pediatric years, 70% were diagnosed at < 1 year of age. This is discordant with other clinical series, where age of diagnosis approximates 5 years. This discrepancy may be explained by the relatively high proportion of respondents in this survey with Williams Syndrome, who would have been evaluated more closely for aberrant clinical findings as a result of this syndromic diagnosis [ 19 ]. The rates of children with a syndromic diagnosis of pRVH vary in the literature, but may be as high as 40% [ 20 ]. Most children with pRVH do not have a syndromic etiology and therefore may not be monitored as early or as closely for the development of hypertension, resulting in delays in diagnosis. In children without a preexisting condition, the American Academy of Pediatrics states that blood pressure monitoring should begin at 3 years of age [ 19 ]. Finally, respondents in this cohort were closely affiliated with specialty treatment centers and providers: 80% of respondents received the diagnosis of pRVH from a specialty physician or during a hospital admission, and nearly 90% of patients surveyed had a specialty care physician on their care team. Additionally, invasive treatment was common, with approximately one-quarter of patients requiring 3 to 5 endovascular procedures or open operations, and 86% of respondents traveled out of state for treatment, with approximately half of the individuals in this survey cohort requiring > 1 week of hospitalization during the course of their disease. In short, those surveyed in this report represent a likely minority of pRVH patients able to seek out and establish nuanced and prolonged care. Finally, this retrospective data may be limited by recall and survey fatigue, as well as a power limitation intrinsic to the low response rate driven at least in part by the rarity of this disease. Conclusion The diagnosis and treatment of patients with pRVH is a unique challenge, and the opportunity to contribute to this process is both a privilege and challenge. Future longitudinal research that considers clinical and patient-reported outcomes is urgently required. Larger-scale research, as prioritized and directed by the diverse and comprehensive Collaborative in this report, has the potential to decrease the number of years of life lost by young pRVH patients, optimize health-related quality of their life, and identify lessons that may be applicable to other rare pediatric disorders. Declarations Funding/Support: All phases of this study were supported by the Eugene Washington PCORI Engagement Award Program. The authors are supported by grants from the Sarnoff Foundation (JMD). Role of Funder/Sponsor (if any): PCORI had no role in the design and conduct of the study. The Sarnoff Foundation had no role in the design and conduct of the study. 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07:05:23","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":43446,"visible":true,"origin":"","legend":"\u003cp\u003eVirtual Research Network (VRN) participants who responded to this survey study were distributed across 6 continents, with a majority (75%) located in North America\u003c/p\u003e","description":"","filename":"Figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7584888/v1/20eb529086e1ab04ae340922.jpg"},{"id":91957979,"identity":"cf86b437-3b67-4869-808d-63b08e19d70a","added_by":"auto","created_at":"2025-09-23 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07:05:23","extension":"pdf","order_by":6,"title":"","display":"","copyAsset":false,"role":"supplement","size":79559,"visible":true,"origin":"","legend":"","description":"","filename":"Table6.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7584888/v1/56318d145033175f8ae3207f.pdf"}],"financialInterests":"","formattedTitle":"Patient and Caregiver Perspectives on the Diagnosis and Management of Pediatric Renovascular Hypertension","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePediatric renovascular hypertension (pRVH) is a rare form of high blood pressure (BP) resulting from renal artery and abdominal aortic disease. This diagnosis risks significant morbidity and mortality [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Though the exact incidence of pRVH is unknown, it is estimated to be the cause of 8\u0026ndash;10% of cases of secondary hypertension in children and adolescents [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Timely detection and treatment of renovascular hypertension is critical to prevent end organ morbidity and mortality. Unfortunately, delays in recognizing and treating pRVH are common, as this diagnosis is most often made in one of very few high-volume referral centers with robust experience with this disease. The relatively low incidence of this disease has also limited the development of optimal treatment guidelines, resulting in wide variations in disease management. Additionally, while the introduction of novel surgical and endovascular techniques has increased the quantity of treatment modalities available to patients with pRVH, these advances have simultaneously further complicated the landscape of patient care [\u003cspan additionalcitationids=\"CR5\" citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eApproaches to navigating the complexities of pRVH treatment have thus far lacked the voices of patients and their families, despite the fact that the value of involving affected individuals and other collaborators in the research process is well-recognized [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].The goal of this study is to identify patient and family perspectives relevant to the diagnosis and treatment of pRVH.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003eA virtual research network of patients and families affected by pRVH, otherwise known as the pRVH PCOR Collaborative, was assembled (https://www.prvh-pcor.org/). This group was formalized in December 2019 with the support of an NIH PCORI Capacity-Building Engagement Award.\u003c/p\u003e\n\u003cp\u003eThe Stakeholder Engagement in quEstion Development (SEED) Method Toolkit [9] was used to inform the creation of a survey designed to prioritize questions regarding comparative effectiveness of various treatment modalities and patient outcome metrics. This method uses a multi-level stakeholder engagement model that includes a participatory framework to develop stakeholder priorities (i.e. research topics and questions, \u003cstrong\u003eFigure 1\u003c/strong\u003e). The Advisory Board of the pRVH PCOR Collaborative led the project as the Community-based participatory Research Team. The collaborative\u0026rsquo;s Critical Stakeholder Group (CSG) was assigned to Topic Groups based on their prior experience and knowledge of pRVH. In partnership with the CSG, the Research Team identified critical areas of focus pertinent to diagnostic and treatment delays. Based on this data, the Research Team created the survey, which included questions about basic patient demographics and clinical data alongside self-reported or proxy-reported measures of satisfaction, as well as PROMIS (Patient-Reported Outcomes Measurement Information System) measures of emotional support, informational support, emotional impact, awakenings from sleep due to pain, global health, fatigue, and social functioning. Survey measures are expanded in Table 1.\u003c/p\u003e\n\u003cp\u003eThis survey tool was distributed to all members of the pRVH PCOR Collaborative\u0026rsquo;s Virtual Research Network (VRN), an online community of patients, family members, clinicians and researchers affected by pRVH (https://www.prvh-pcor.org/prvh-pcor-virtual-research-network/). VRN members were contacted through the Collaborative newsletter and email outreach. Children with pRVH who had grown into adulthood (\u0026gt;18 years) and parents/guardians of affected children (ages 0-17 years) were able to volunteer to anonymously complete the survey.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eProcedures\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eParticipants were provided with brief information about the study prior to answering the first survey question. Written or verbal informed consents were not required, as participants were assumed to have provided consent upon answering the survey questions voluntarily. Participants completed surveys using REDCAP [10], a Health Insurance Portability and Accountability Act (HIPAA)-compliant electronic survey platform. No identifying information was collected in the survey (e.g., names, dates, emails, etc). The survey required an estimated 15 minutes to complete. An IRB exemption was issued by the University of Michigan IRBMED for this study (HUM00206982).\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eStatistical Analyses\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eNormally distributed continuous variables are reported as the mean and SD. Non-normally distributed variables are reported by median and interquartile range. Categorical variables are reported by number and percentage.\u003c/p\u003e\n\u003cp\u003e\u003cu\u003epRVH Treatment Satisfaction Questionnaire.\u003c/u\u003e Descriptive statistics were applied to responses to identify relative frequency of current practices as well as perceived barriers to the treatment of pRVH. Responses were denoted as significant when \u0026le;80% of participants expressed dissatisfaction with current treatment practices. Differences between subgroups were evaluated using analysis of covariance (ANCOVA).\u003c/p\u003e\n\u003cp\u003e\u0026nbsp; \u003cu\u003epRVH Impact on Health-Related Quality of Life (HRQOL).\u003c/u\u003e\u0026nbsp; Descriptive statistics for each of the PROMIS and ASCQ-Me measures are reported. In addition, ANCOVA were used to determine subgroups of participants that differed on these measures. The following subgroups were examined: sex; medication use (0-2 current medications versus \u0026ge; 3 medications); open operative/endovascular procedure history (no interventions versus one or more interventions); recurrent hypertension status (those with current hypertension versus those without); complications in the first 4 weeks of life (those with versus without complications); diagnosis age (diagnosis before or equal to age 2 versus diagnosis at age 3 or older); insurance type (public versus private); diagnosing provider (specialist versus generalist); and satisfaction with length of time to receive a diagnosis (satisfied versus dissatisfied). \u0026nbsp; Responses were controlled for the presence of Williams syndrome. Differences between subgroups were evaluated using ANCOVA.\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eCompleted surveys were received from 28 stakeholders between January \u0026ndash; April 2022 (n=22 mother/female guardian, n=4 father/male guardian; n=2 patient self-report, Table 2). VRN stakeholder engagement was split with equipoise between patient-family respondents and clinician or research investigators (Figure 2). Most respondents were of European ancestry. 62% of respondents were diagnosed with genetic comorbidities in addition to pRVH, with patients most frequently reporting comorbid Williams Syndrome (43%). Additionally, 61% of children surveyed sustained complications during the first 4 weeks of life. 54% experienced motor delays, 46% experienced language delays, 36% experienced cognitive delays, and 21% experienced social delays. 18% reported physical disability of any kind, and 18% had been diagnosed with ADHD. \u0026nbsp;Special education services were used by 43% and other specialized therapies or services\u0026nbsp;were used by 75% of patients (Table 3).\u003c/p\u003e\n\u003cp\u003e\u003cu\u003epRVH Treatment Satisfaction Questionnaire\u0026nbsp;\u003c/u\u003e(Table 4) documented that 89% of respondents felt that the treatment team kept them well-informed, included them in important decisions, and answered questions and explained test results in a way that respondents were able to understand. Furthermore, 68% were satisfied with time to diagnosis. Nevertheless, only 54% of respondents were satisfied with the overall amount of information they received about pRVH, and only 50% found it easy to seek out additional information. The minority of patients and families (40%) agreed that their doctor provided appropriate referrals for treatment. Interestingly, though 75% of respondents had access to a home blood pressure device, only 61% felt confident in monitoring blood pressure at home. Among the 14 respondents who underwent surgical procedures, 93% felt that they received appropriate information about the intervention. While 71% expressed that symptoms improved as a result of the procedure, only 57% were satisfied with the outcome. 100% of respondents felt that the surgical team explained the intervention in a way that they were able to understand.\u003c/p\u003e\n\u003cp\u003e\u003cu\u003epRVH Impact on HRQOL\u003c/u\u003e. Parents and caregivers indicated that their children experience worse overall physical health than the general population. They also noted that their children have worse mental health and greater than average global fatigue than other children with chronic conditions. At the same time, however, they indicated that their children have higher than average social functioning than other children with chronic conditions (Table 5-6).\u003c/p\u003e\n\u003cp\u003ePatients taking \u0026ge;3 medications for pRVH reported less overall emotional impact of their diagnosis (mean 46.5 versus 56.5, f=4.83, p=0.04) than those taking 1-2 medications. Even so, those taking \u0026ge;3 medications noted increased fatigue (62.3 versus 52.0, f=4.56, p=0.04). Patients with a surgical or procedural history reported significantly less pain that awakened them from sleep (44 versus 51.5, f=12.61, p\u0026lt;0.01) and less fatigue (48.2 versus 58, f=8.41, p=0.01) than those without a surgical history. Patients having at least one surgical procedure reported greater overall emotional impact of pRVH (57.7 versus 53.1, f=5.97, p=0.02) than those who had not undergone any procedures. Furthermore, individuals diagnosed with disease \u0026le;2 years of age reported better informational support (60.9 versus 53.0, f=8.13, p=0.01) and improved social functioning (64.8 versus 53.0, f=6.92, p=0.01) compared to those diagnosed at age 3 or older. Respondents unsatisfied with the time it took to receive a diagnosis indicated more pain awakening them from sleep (53.3 versus 45.1, f=14.09, p\u0026lt;0.01), more fatigue (58.5 versus 50.5, f=4.50, p=0.04), worse global health (37.1 versus 46.6), and worse social functioning (50.8 versus 62.6, f=7.32, p=0.01) than those satisfied respondents, while those perceiving a delay in diagnosis reported worse emotional impact (50.6 versus 57.5, f=5.91, p=0.02). Finally, a primary care-diagnosis was associated with worse emotional support (43.2 versus 56.4, f=8.97, p=0.01), informational support (46.9 versus 58.4, f=5.34, p=0.03), and greater emotional impact (46.6 versus 56.6, f=4.61, p= 0.04) than a diagnosis provided by a specialist, such as a pediatric cardiologist, pediatric nephrologist, or vascular surgeon.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003ePediatric renovascular hypertension may be responsible for up to 10% of secondary hypertension in children and adolescents [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Since the pathophysiology of this rare disease impacts multiple organ systems, the journey to diagnosis is highly dependent upon collaboration between multiple specialists as well as the use of many resources that are typically only found at high-volume specialty centers. While discussions regarding this extremely complicated landscape have sought to identify modes of augmenting provider awareness of pRVH, these conversations have, until now, lacked important input from the most important stakeholder group in this disease: patients and their families. Notably, though PROMIS has been applied to a wide range of disorders including cancer, congestive heart failure, depression, arthritis, chronic pain, and multiple sclerosis, this study represents the first large scale application of PROMIS to pRVH [\u003cspan additionalcitationids=\"CR12 CR13\" citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e\u003cp\u003e The pRVH PCOR Collaborative represents the first global effort designed to bring together critical stakeholders in pRVH, including patients, families, providers, and researchers, to incorporate patient perspectives into disease management and treatment guidelines while also ensuring that patients and their families feel less isolated. Indeed, the data collected here provide unique and groundbreaking insights into the aspects of the clinical course that most define the diagnosis of pRVH to those grappling with it most intimately. While most respondents felt well-informed and supported by their treatment teams, only half were satisfied with the overall information they received about pRVH. This highlights a possible distinction between the emotional support that clinical teams can provide and the literature that they can offer to assist patients and families in their efforts to understand the disease itself. This suggests a need for increased patient-friendly pRVH materials to be created and shared at the time of diagnosis. Similarly, there appears to be a greater role for dedicated education regarding use of blood pressure monitoring at home. Furthermore, a minority of respondents felt that they had received appropriate referrals for further treatment, suggesting that there is a potential role for guidelines suggesting specific referral patterns if pRVH is suspected or diagnosed.\u003c/p\u003e\u003cp\u003eInterestingly, some survey responses were discordant with sentiments that might generally be expected from patients with other clinical diagnoses. For example, individuals taking\u0026thinsp;\u0026ge;\u0026thinsp;3 medications noted an overall decreased emotional impact of this disease on their lives. In contrast, patients with other diagnoses usually anecdotally report that increased number of medications increases the emotional burden that they feel. This discrepancy can potentially be explained by the insidiously destructive impact that elevated blood pressure may have on multiple organ systems, and the relatively specific ability of blood pressure medications or procedures to modulate these effects. Additionally, this data aligns with prior studies that have demonstrated significant relationships between hypertension and both anxiety and depression [\u003cspan additionalcitationids=\"CR16 CR17\" citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]. Lastly, earlier diagnosis appears to be related to improved social functioning and enhanced global health. Diagnosis made by specialists is also associated with improved emotional impact and information support. Taken together, these findings suggest that improving access to specialty centers may aid in decreasing time to diagnosis and the provision of adequate support to pRVH patients.\u003c/p\u003e\u003cp\u003eThe demographics of the respondent cohort, which was assembled through coordination between multiple high-volume specialty centers, are a limitation to these findings. While age at diagnosis ranged throughout the pediatric years, 70% were diagnosed at \u0026lt;\u0026thinsp;1 year of age. This is discordant with other clinical series, where age of diagnosis approximates 5 years. This discrepancy may be explained by the relatively high proportion of respondents in this survey with Williams Syndrome, who would have been evaluated more closely for aberrant clinical findings as a result of this syndromic diagnosis [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. The rates of children with a syndromic diagnosis of pRVH vary in the literature, but may be as high as 40% [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]. Most children with pRVH do not have a syndromic etiology and therefore may not be monitored as early or as closely for the development of hypertension, resulting in delays in diagnosis. In children without a preexisting condition, the American Academy of Pediatrics states that blood pressure monitoring should begin at 3 years of age [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. Finally, respondents in this cohort were closely affiliated with specialty treatment centers and providers: 80% of respondents received the diagnosis of pRVH from a specialty physician or during a hospital admission, and nearly 90% of patients surveyed had a specialty care physician on their care team. Additionally, invasive treatment was common, with approximately one-quarter of patients requiring 3 to 5 endovascular procedures or open operations, and 86% of respondents traveled out of state for treatment, with approximately half of the individuals in this survey cohort requiring\u0026thinsp;\u0026gt;\u0026thinsp;1 week of hospitalization during the course of their disease. In short, those surveyed in this report represent a likely minority of pRVH patients able to seek out and establish nuanced and prolonged care. Finally, this retrospective data may be limited by recall and survey fatigue, as well as a power limitation intrinsic to the low response rate driven at least in part by the rarity of this disease.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThe diagnosis and treatment of patients with pRVH is a unique challenge, and the opportunity to contribute to this process is both a privilege and challenge. Future longitudinal research that considers clinical and patient-reported outcomes is urgently required. Larger-scale research, as prioritized and directed by the diverse and comprehensive Collaborative in this report, has the potential to decrease the number of years of life lost by young pRVH patients, optimize health-related quality of their life, and identify lessons that may be applicable to other rare pediatric disorders.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding/Support:\u0026nbsp;\u003c/strong\u003eAll phases of this study were supported by the Eugene Washington PCORI Engagement Award Program. The authors are supported by grants from the Sarnoff Foundation (JMD).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eRole of Funder/Sponsor (if any):\u0026nbsp;\u003c/strong\u003ePCORI had no role in the design and conduct of the study. The Sarnoff Foundation had no role in the design and conduct of the study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflict of Interest Disclosures (includes financial disclosures):\u003c/strong\u003e\u0026nbsp; None\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eStanley JC, Criado E, Upchurch GR Jr. et al (2006) Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children. \u003cem\u003eJ Vasc Surg\u003c/em\u003e. Dec ;44(6):1219-28; discussion 1228-9. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jvs.2006.08.009\u003c/span\u003e\u003cspan address=\"10.1016/j.jvs.2006.08.009\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSilverstein DM, Champoux E, Aviles DH, Vehaskari VM (2006) Treatment of primary and secondary hypertension in children. 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J Nephrol Mar 21. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s40620-024-01919-6\u003c/span\u003e\u003cspan address=\"10.1007/s40620-024-01919-6\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eFlynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, De Ferranti SD, Dionne JM, Falkner B, Flinn SK, Gidding SS (2017) Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 140(3). \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1542/peds.2017-1904\u003c/span\u003e\u003cspan address=\"10.1542/peds.2017-1904\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eWarejko JK, Schueler M, Vivante A, Tan W, Daga A, Lawson JA, Braun DA, Shril S, Amann K, Somers MJ, Rodig NM (2018) Whole exome sequencing reveals a monogenic cause of disease in \u0026asymp; 43% of 35 families with midaortic syndrome. Hypertension 71(4):691\u0026ndash;699. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1161/HYPERTENSIONAHA.117.10463\u003c/span\u003e\u003cspan address=\"10.1161/HYPERTENSIONAHA.117.10463\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003eTable 1 to 6 are available in the Supplementary Files section.\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-nephrology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pnep","sideBox":"Learn more about [Pediatric Nephrology](http://link.springer.com/journal/467)","snPcode":"467","submissionUrl":"https://www.editorialmanager.com/pnep/default2.aspx","title":"Pediatric Nephrology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"hypertension, pediatric renovascular hypertension, vascular surgery, interventional radiology","lastPublishedDoi":"10.21203/rs.3.rs-7584888/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7584888/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e\u003cp\u003ePediatric renovascular hypertension (pRVH) may lead to significant morbidity and mortality. Rare disease prevalence limits existing data and optimal management remains ill-defined. This study seeks to capture patient and family perceptions around the diagnosis and management of pRVH.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003eThe pRVH Patient Centered Outcomes Research Collaborative assembled a global virtual research network of multidisciplinary stakeholders, inclusive of patients and families. We examined self or proxy-reported emotional support, informational support, emotional impact, and number of surgeries/procedures, as well as self-reported pain, global health, fatigue, and social functioning. Descriptive statistics were performed.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003e28 stakeholders (93% parent/guardian; 7% patient self-report) completed the survey. Patients with a surgical or procedural history reported significantly fewer nighttime awakenings due to pain (mean 44 v. 51.5) and less fatigue (48.2 v. 58) than those without. Patients diagnosed before 2 years of age reported better informational (60.9 v. 53.0) and emotional support (57.6 v. 51.5), and better social functioning (64.8 v. 53.0). Respondents unsatisfied with the time to receive a diagnosis indicated more pain (53.3 v. 45.1), fatigue (58.5 v. 50.5), worse global health (37.1 v. 46.6), and worse social functioning (50.8 v. 62.6) than satisfied patients. PCP-diagnosis was associated with worse emotional support (43.2 v. 56.4), informational support (46.9 v. 58.4), and emotional impact (46.6 v. 56.6) than those diagnosed by a specialist.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e\u003cp\u003eThese results suggest that early diagnosis, access to centers of excellence, and treatment by multidisciplinary and specialty care teams can optimize pRVH treatment.\u003c/p\u003e","manuscriptTitle":"Patient and Caregiver Perspectives on the Diagnosis and Management of Pediatric Renovascular Hypertension","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-09-23 07:05:18","doi":"10.21203/rs.3.rs-7584888/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major Revisions Needed","date":"2025-10-02T12:18:17+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2025-09-13T18:53:50+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-09-13T17:31:28+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-09-11T08:59:31+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Nephrology","date":"2025-09-10T12:36:06+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-nephrology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pnep","sideBox":"Learn more about [Pediatric Nephrology](http://link.springer.com/journal/467)","snPcode":"467","submissionUrl":"https://www.editorialmanager.com/pnep/default2.aspx","title":"Pediatric Nephrology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"f8af6524-2e5f-4b42-b624-e32e09b2b438","owner":[],"postedDate":"September 23rd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"in-revision","subjectAreas":[],"tags":[],"updatedAt":"2026-02-13T22:06:58+00:00","versionOfRecord":[],"versionCreatedAt":"2025-09-23 07:05:18","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7584888","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7584888","identity":"rs-7584888","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}