Overview
Retinocochleocerebral vasculopathy (RCCV) is a rare, multisystem vasculopathy characterized by involvement of retinal, cochlear, and cerebral vessels, often presenting with neurological decline, hearing loss, and retinal abnormalities. The underlying mechanisms may involve ciliopathy disorders or genetic variants affecting vascular integrity 855.Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 226 indexed papers.
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The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2025. link 11 Amancherla K, Schlendorf KH, Chow N, Sheng Q, Freedman JE, Rathmell JC. Single-cell RNA-sequencing identifies unique cell-specific gene expression profiles in high-grade cardiac allograft vasculopathy. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2025. link 12 Grundmann D, Neubarth-Mayer J, Müller C, Becker F, Reichart D, Stark K et al.. Progress of Angiographic Cardiac Allograft Vasculopathy in Patients With Long-Term Transplantation: Longitudinal Evaluation of Its Association With Dyslipidemia Patterns. The American journal of cardiology 2025. link 13 Cheng Y, Zachariah J. Clinical Reasoning: A 54-Year-Old Woman With Progressive Headache and Neurologic Decline. Neurology 2024. link 14 Amancherla K, Feurer ID, Rega SA, Cluckey A, Salih M, Davis J et al.. Early Assessment of Cardiac Allograft Vasculopathy Risk Among Recipients of Hepatitis C Virus-infected Donors in the Current Era. Journal of cardiac failure 2024. link 15 Scrimshaw L, Gorman K, Mansour S, Ganesan V, Sabir A. Moyamoya disease/cerebral vasculopathy in osteopathia striata with cranial sclerosis: a rare but important complication. Clinical dysmorphology 2024. link 16 Ozturk GY, Kocyigit BF. Healing refractory livedoid vasculopathy-related skin ulcers by ozone therapy: a case-based review. Rheumatology international 2024. link 17 Gondi KT, Hammer Y, Yosef M, Golbus JR, Madamanchi C, Aaronson KD et al.. Longitudinal Change and Predictors of Myocardial Flow Reserve by Positron Emission Tomography for the Evaluation of Cardiac Allograft Vasculopathy Following Heart Transplantation. Journal of cardiac failure 2024. link 18 Puac-Polanco P, Rovira À, Shah LM, Wiggins RH, Rivas Rodriguez F, Torres C. Imaging of Drug-Related Vasculopathy. 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American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2024. link 22 Zamora CA, Mossa-Basha M, Castillo M. Usefulness of Different Imaging Methods in the Diagnosis of Cerebral Vasculopathy. Neuroimaging clinics of North America 2024. link 23 Yuan Y, Liu Y, Ban F, Sun Z, Liao W, Wang L. Lymphocytic vasculitis in livedoid vasculopathy: A report of 137 cases. Journal of cutaneous pathology 2024. link 24 Chih S, Tavoosi A, Nair V, Chong AY, Džavík V, Aleksova N et al.. Cardiac PET Myocardial Blood Flow Quantification Assessment of Early Cardiac Allograft Vasculopathy. JACC. Cardiovascular imaging 2024. link 25 Alansari S, Alenzi F, AlSaleem A, Al-Mayouf SM. New insights on genetic variants and phenotypic features of childhood large-vessel vasculopathy: a systematic review and single-centre series. Clinical and experimental rheumatology 2024. link 26 O'Hara PE, Gorrai A, Farr M, Peltz M, Beaini H, Moayedi Y et al.. Revisiting Biomarkers of Cardiac Allograft Vasculopathy: Addressing the Achilles Heel of Heart Transplantation. Current heart failure reports 2024. link 27 Moayedi Y, Rodenas-Alesina E, Somerset E, Fan CPS, Henricksen E, Aleksova N et al.. Enhancing the Prediction of Cardiac Allograft Vasculopathy Using Intravascular Ultrasound and Machine Learning: A Proof of Concept. Circulation. Heart failure 2024. link 28 Munafò AR, Turco A, Ferlini M, Benzoni G, Cattadori B, Pellegrini C et al.. First- Versus New-Generation Drug-Eluting Stents in Patients With Heart Transplant With Cardiac Allograft Vasculopathy. The American journal of cardiology 2023. link 29 Gao Y, Jin H. Livedoid vasculopathy and peripheral neuropathy: A retrospective cohort study of 55 Chinese patients and literature review. International wound journal 2023. link 30 Zhao C, Jing K, Feng S. Clinical analysis of skin lesions in livedoid vasculopathy: a study of 46 Chinese patients. International journal of dermatology 2023. link 31 Firsowicz M, Haller CN, Soldano A, Keeling BH, Diaz LZ. Cutaneous collagenous vasculopathy in a pediatric patient. Pediatric dermatology 2023. link 32 Abadie BQ, Chan N, Sharalaya Z, Bhat P, Harb S, Jacob M et al.. Negative Predictive Value and Prognostic Associations of Rb-82 PET/CT with Myocardial Blood Flow in CAV. JACC. Heart failure 2023. link 33 . Helping practitioners to stop the rot. The Veterinary record 2022. link 34 Kim MJ, Yu JJ, Cha S, Baek JS, Choi ES, Kwon BS et al.. Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease. Cardiology in the young 2022. link 35 Gao Y, Jin H. Efficacy of an anti-TNF-alpha agent in refractory livedoid vasculopathy: a retrospective analysis. The Journal of dermatological treatment 2022. link 36 Assan F, Bottin L, Francès C, Moguelet P, Tavolaro S, Barbaud A et al.. Antiphospholipid-negative Sneddon's syndrome: A comprehensive overview of a rare entity. Annales de dermatologie et de venereologie 2022. link 37 Ortega-Legaspi JM, Bravo PE. Diagnosis and management of cardiac allograft vasculopathy. Heart (British Cardiac Society) 2022. link 38 Shrestha UM, Sciammarella M, Pampaloni MH, Botvinick EH, Gullberg GT, DeMarco T et al.. Assessment of late-term progression of cardiac allograft vasculopathy in patients with orthotopic heart transplantation using quantitative cardiac . The international journal of cardiovascular imaging 2021. link 39 Nuttawong S, Chularojanamontri L, Trakanwittayarak S, Pinkaew S, Chanchaemsri N, Rujitharanawong C. Direct immunofluorescence findings in livedoid vasculopathy: a 10-year study and literature review. Clinical and experimental dermatology 2021. link 40 Chih S, Chong AY, Bernick J, Wells GA, deKemp RA, Davies RA et al.. Validation of multiparametric rubidium-82 PET myocardial blood flow quantification for cardiac allograft vasculopathy surveillance. Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology 2021. link 41 Asleh R, Briasoulis A, Smith B, Lopez C, Alnsasra H, Pereira NL et al.. Association of Aspirin Treatment With Cardiac Allograft Vasculopathy Progression and Adverse Outcomes After Heart Transplantation. Journal of cardiac failure 2021. link 42 Acharya D, Loyaga-Rendon RY, Chatterjee A, Rajapreyar I, Lee K. Optical Coherence Tomography in Cardiac Allograft Vasculopathy: State-of-the-Art Review. Circulation. Heart failure 2021. link 43 Miller RJH, Kwiecinski J, Shah KS, Eisenberg E, Patel J, Kobashigawa JA et al.. Coronary computed tomography-angiography quantitative plaque analysis improves detection of early cardiac allograft vasculopathy: A pilot study. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2020. link 44 Feher A, Srivastava A, Quail MA, Boutagy NE, Khanna P, Wilson L et al.. Serial Assessment of Coronary Flow Reserve by Rubidium-82 Positron Emission Tomography Predicts Mortality in Heart Transplant Recipients. JACC. Cardiovascular imaging 2020. link 45 Brndiarova M, Antonyova M, Dedinska I, Havlicekova Z, Jesenak M. Nephronophthisis type I, left ventricular non-compaction cardiomyopathy and reduced cilia motility-atypical manifestations of one disease. Journal of nephrology 2020. link 46 Moore C, Gao B, Roskin KM, Vasilescu EM, Addonizio L, Givertz MM et al.. B cell clonal expansion within immune infiltrates in human cardiac allograft vasculopathy. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2020. link 47 van Heeswijk RB, Bastiaansen JAM, Iglesias JF, Degrauwe S, Rotman S, Barras JL et al.. Quantification of myocardial interstitial fibrosis and extracellular volume for the detection of cardiac allograft vasculopathy. The international journal of cardiovascular imaging 2020. link 48 Liu M, Gutierrez J. Developing Novel Therapeutic Strategies Against Ring Finger Protein 213 Vasculopathy: Is It an Achievable Goal?. Journal of the American Heart Association 2019. link 49 Arif R, Franz M, Remes A, Zaradzki M, Hecker M, Karck M et al.. Reduction of Transplant Vasculopathy by Intraoperative Nucleic Acid-based Therapy in a Mouse Aortic Allograft Model. The Thoracic and cardiovascular surgeon 2019. link 50 McGovern E, Hosking MCK, Balbacid E, Voss C, Berger F, Schubert S et al.. Optical Coherence Tomography for the Early Detection of Coronary Vascular Changes in Children and Adolescents After Cardiac Transplantation: Findings From the International Pediatric OCT Registry. JACC. Cardiovascular imaging 2019. link 51 Kemaloğlu C, Özçobanoğlu S, Köksel U, Erbasan O, Gölbaşı İ, Türkay C et al.. Extracorporeal Membrane Oxygenation Therapy-Assisted Cardiopulmonary Resuscitation and Successful Bridging to Cardiac Retransplant for Cardiac Allograft Vasculopathy. Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation 2019. link 52 Pala R, Mohieldin AM, Shamloo K, Sherpa RT, Kathem SH, Zhou J et al.. Personalized Nanotherapy by Specifically Targeting Cell Organelles To Improve Vascular Hypertension. Nano letters 2019. link 53 Zuo C, Zhang X, Li M, Peng Y, Mi L, Liu B et al.. Case series of coexistence of polypoidal choroidal vasculopathy with other rare fundus diseases. International ophthalmology 2019. link 54 Károlyi M, Kolossváry M, Bartykowszki A, Kocsmár I, Szilveszter B, Karády J et al.. Quantitative CT assessment identifies more heart transplanted patients with progressive coronary wall thickening than standard clinical read. Journal of cardiovascular computed tomography 2019. link 55 Wang Z, Wang M, Hu X, Li Y, Ma D, Li S et al.. Liraglutide, a Glucagon-like Peptide-1 Receptor Agonist, Attenuates Development of Cardiac Allograft Vasculopathy in a Murine Heart Transplant Model. Transplantation 2019. link 56 Günther A, Aaberge L, Abildgaard A, Ragnarsson A, Edvardsen T, Jakobsen J et al.. Coronary computed tomography in heart transplant patients: detection of significant stenosis and cardiac allograft vasculopathy, image quality, and radiation dose. Acta radiologica (Stockholm, Sweden : 1987) 2018. link 57 Duncombe SL, Hosking MCK, Coté AT, Voss C, Harris KC. Intimal thickening at coronary bifurcations in pediatric heart transplant recipients. 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Vasculopathies, cutaneous necrosis and emergency in dermatology. Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia 2017. link 62 Ulrich SM, Lehner A, Birnbaum J, Heckel S, Haas NA, Hakami L et al.. Safety of optical coherence tomography in pediatric heart transplant patients. International journal of cardiology 2017. link 63 Cheng R, Vanichsarn C, Patel JK, Currier J, Chang DH, Kittleson MM et al.. Long-term clinical and angiographic outcomes of percutanenous coronary intervention with everolimus-eluting stents for the treatment of cardiac allograft vasculopathy. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions 2017. link 64 Motegi SI, Yasuda M, Yamanaka M, Amano H, Ishikawa O. Cutaneous collagenous vasculopathy: Report of first Japanese case and review of the literature. The Australasian journal of dermatology 2017. link 65 Daly KP, Stack M, Eisenga MF, Keane JF, Zurakowski D, Blume ED et al.. Vascular endothelial growth factor A is associated with the subsequent development of moderate or severe cardiac allograft vasculopathy in pediatric heart transplant recipients. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2017. link 66 Park KH, Sun T, Liu Z, Yang SW, Lennon RJ, Lerman LO et al.. Relationship between markers of plaque vulnerability in optical coherence tomography and atherosclerotic progression in adult patients with heart transplantation. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2017. link 67 Cheang WS, Wong WT, Zhao L, Xu J, Wang L, Lau CW et al.. PPARδ Is Required for Exercise to Attenuate Endoplasmic Reticulum Stress and Endothelial Dysfunction in Diabetic Mice. 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Co-localization of Disturbed Flow Patterns and Occlusive Cardiac Allograft Vasculopathy Lesion Formation in Heart Transplant Patients. Cardiovascular engineering and technology 2015. link 79 Jeewa A, Chin C, Pahl E, Atz AM, Carboni MP, Pruitt E et al.. Outcomes after percutaneous coronary artery revascularization procedures for cardiac allograft vasculopathy in pediatric heart transplant recipients: A multi-institutional study. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2015. link 80 Mehta RI, Mehta RI, Choi JM, Mukherjee A, Castellani RJ. Hydrophilic polymer embolism and associated vasculopathy of the lung: prevalence in a retrospective autopsy study. Human pathology 2015. link 81 Chirakarnjanakorn S, Starling RC, Popović ZB, Griffin BP, Desai MY. Dobutamine stress echocardiography during follow-up surveillance in heart transplant patients: Diagnostic accuracy and predictors of outcomes. 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The British journal of ophthalmology 2012. link