Hereditary Hemorrhagic Telangiectasia — designated HHT, also known as Osler-Weber-Rendu Syndrome (OMIM #187300 for HHT1, #600376 for HHT2, #175050 for HHT3, #610655 for HHT4, #615506 for HHT5), an autosomal dominant vascular dysplasia with an estimated prevalence of 1 in 5,000 to 1 in 8,000 individuals worldwide affecting approximately 1.4 million persons globally, caused by heterozygous loss-of-function mutations in members of the TGF-β/BMP signaling pathway: ENG (endoglin, chromosome 9q34.11) causes HHT type 1 and accounts for approximately 39% of genetically confirmed cases — endoglin is a co-receptor for TGF-β1 and TGF-β3, and a co-receptor for BMP9 and BMP10, expressed at high levels on proliferating vascular endothelial cells and essential for TGF-β-mediated maintenance of endothelial cell quiescence, vascular remodeling, and angiogenic homeostasis; ACVRL1 (activin receptor-like kinase 1, ALK1, chromosome 12q13.13) causes HHT type 2 and accounts for approximately 45% of cases — ALK1 is the signaling receptor for BMP9 and BMP10 on vascular endothelial cells, mediating the SMAD1/5/8 downstream pathway that suppresses excessive endothelial cell proliferation and migration; SMAD4 mutations cause juvenile polyposis-HHT overlap syndrome (JP-HHT); GDF2 (encoding BMP9, chromosome 10q11.22) causes HHT type 5; and mutations in RASA1 and EPHB4 cause an HHT-like capillary malformation-arteriovenous malformation (CM-AVM) syndrome; the fundamental pathomechanism in HHT involves haploinsufficiency of endoglin or ALK1 causing loss of TGF-β/BMP signaling-mediated suppression of endothelial cell activation, resulting in pathological angiogenesis with formation of abnormal direct arteriovenous connections — telangiectases (superficial mucocutaneous AVMs in skin and mucous membranes consisting of dilated tortuous postcapillary venules without intervening capillaries) and visceral arteriovenous malformations (AVMs in lung, liver, brain, GI tract, and spinal cord); clinical manifestations include recurrent epistaxis (the most common and earliest manifestation, beginning in childhood in >95% of patients, resulting from nasal mucosal telangiectases that bleed with minor trauma or spontaneously — severity ranges from mild to transfusion-dependent iron-deficiency anemia requiring multiple packed red blood cell transfusions per year), mucocutaneous telangiectases visible on lips, tongue, buccal mucosa, and fingertips (pathognomonic when combined with epistaxis and family history — the Curaçao diagnostic criteria), pulmonary AVMs (PAVMs — present in 30-50% of HHT1 patients and 10-15% of HHT2 patients, causing right-to-left shunting with hypoxemia, paradoxical embolism risk causing stroke, brain abscess from paradoxical bacteremia — the most life-threatening HHT complication), cerebral AVMs (CAVMs — present in 10-20% of HHT patients regardless of genotype, causing hemorrhagic or ischemic stroke, seizures, headache — the primary neurological emergency in HHT), hepatic AVMs (present in 30-70% of HHT patients, usually asymptomatic but capable of causing high-output heart failure from hepatic arteriovenous shunting, portal hypertension from hepatic arterioportal shunting, or biliary disease from hepatic arteriobiliary shunting), spinal AVMs (less common but capable of causing paraplegia from subarachnoid hemorrhage or spinal cord infarction), and gastrointestinal telangiectases (GI bleeding from gastric, small bowel, or colonic telangiectases contributing to iron-deficiency anemia independent of epistaxis); diagnosis is established by the Curaçao criteria (epistaxis + mucocutaneous telangiectases + visceral AVMs + first-degree family history — definite diagnosis ≥3 criteria, suspected 2 criteria); SMAD4-related JP-HHT requires colonoscopic surveillance for juvenile polyps with malignant potential; treatment includes local and systemic hemostasis strategies for epistaxis (humidification, laser photocoagulation, chemical cautery, bevacizumab intranasal injection, surgical septal dermoplasty, bevacizumab IV for severe cases), pulmonary AVM embolization for PAVM feeding arteries ≥2-3 mm (the primary preventive intervention for paradoxical embolism and brain abscess risk), brain AVM management (stereotactic radiosurgery, endovascular embolization, microsurgical resection depending on AVM size, location, and angioarchitecture), and anti-angiogenic therapy with bevacizumab (IV bevacizumab reducing epistaxis, GI bleeding, hepatic shunting complications, and high-output cardiac failure in HHT).
HHT technology platforms — encompassing the HHT specialty center platforms where epistaxis severity scoring (Epistaxis Severity Score — ESS), hemoglobin and iron study tracking, AVM surveillance scheduling, and treatment coordination are conducted, the radiology platforms where pulmonary AVM surveillance CT angiography and embolization, liver AVM evaluation with Doppler and CT angiography, brain AVM characterization with MRI/MRA and catheter angiography, and GI bleeding evaluation are performed, the interventional radiology platforms where PAVM embolization procedures are scheduled, performed, and followed, the pulmonary medicine platforms monitoring hypoxemia and paradoxical embolism consequences from pulmonary shunting, the neurology and neurosurgery platforms managing brain AVMs and their hemorrhagic and ischemic complications, the otorhinolaryngology platforms managing epistaxis treatment escalation from conservative care through laser to surgical dermoplasty, the gastroenterology platforms managing GI telangiectases and iron-deficiency anemia, the cardiovascular and heart failure platforms managing hepatic AVM high-output cardiac failure, the infusion oncology platforms administering IV bevacizumab for HHT, the genetic testing laboratory platforms where ENG and ACVRL1 mutation analysis and cascade family screening are conducted, and the family screening coordination platforms managing systematic proband-index to family member mutation testing — must maintain the availability and performance standards required by the epistaxis severity and hemoglobin surveillance frequency, the PAVM embolization scheduling regularity, the brain AVM monitoring interval management, the hepatic AVM complication detection sensitivity, the iron-deficiency anemia treatment optimization, and the family cascade screening coordination breadth that define comprehensive HHT care. This guide explains why HHT care tech platforms need dedicated monitoring, what components to monitor, and how to build a monitoring strategy matched to the multi-organ vascular surveillance requirements, paradoxical embolism prevention priority, family cascade screening breadth, and anti-angiogenic therapy management complexity that characterize modern HHT management.
Why HHT Tech Platforms Require Specialized Monitoring Attention
HHT management is defined by several distinctive multi-organ surveillance and prevention coordination challenges: the paradoxical embolism prevention imperative — pulmonary AVMs with feeding artery diameters ≥2-3 mm are the primary source of paradoxical embolism causing ischemic stroke and brain abscess in HHT, and transcatheter PAVM embolization is the definitive preventive intervention, making timely CT pulmonary angiography screening to identify embolizable PAVMs and scheduling of embolization procedures among the most clinically critical workflows in HHT care — a missed PAVM screening interval or an embolization scheduling delay can result in the preventable stroke or brain abscess that is the most feared complication of inadequately treated pulmonary AVMs; the recurrent epistaxis burden management intensity — epistaxis in HHT ranges from a minor nuisance to a daily bleeding burden requiring multiple nasal packing and cauterization procedures per year and leading to severe iron-deficiency anemia requiring transfusion-dependent anemia management, making ESS tracking, hemoglobin and ferritin monitoring, IV iron supplementation scheduling, and transfusion threshold management continuous clinical obligations; the brain AVM hemorrhage surveillance urgency — cerebral AVMs in HHT carry an annual rupture risk of 1-2% in untreated patients, with intracranial hemorrhage carrying 30-50% morbidity and mortality, making brain AVM surveillance MRI and MRA scheduling precision a critical clinical requirement; the hepatic AVM complication monitoring complexity — the majority of HHT patients have hepatic AVMs that are asymptomatic but whose symptomatic complications (high-output heart failure from hepatic arteriovenous fistulas, portal hypertension from arterioportal fistulas, biliary ischemia from arteriobiliary fistulas) require surveillance echocardiography for cardiac output, Doppler ultrasound for portal flow, and hepatic function panels, while the devastating complication of hepatic artery embolization (hepatic necrosis from collateral artery occlusion in a liver supplied primarily by arterial blood via AVMs) means hepatic AVMs must be managed without embolization and bevacizumab IV is increasingly used as systemic therapy; and the family cascade screening scale — each HHT proband with an ENG or ACVRL1 mutation has first-degree relatives with a 50% probability of inheriting the same mutation, and each identified mutation-positive relative requires the full HHT surveillance protocol (epistaxis assessment, CT for PAVMs, brain MRI for CAVMs, abdominal Doppler for hepatic AVMs), creating a cascading family screening obligation that expands geometrically with each generation and requires multi-family scheduling and coordination platforms.
Pulmonary AVM CT surveillance and embolization platforms carry the highest acute stroke and brain abscess prevention priority in HHT. PAVM embolization is the only intervention proven to reduce the risk of paradoxical embolism — the most life-threatening complication of untreated PAVMs in HHT. Monitor PAVM surveillance CT scheduling and embolization scheduling platforms at 1-minute intervals during radiology and interventional radiology hours.
Epistaxis severity and hemoglobin tracking platforms must not fail during the transfusion threshold assessments that determine iron and blood product needs. The ESS score at each visit combined with serial hemoglobin and ferritin measurements determines whether the patient needs IV iron, an escalated epistaxis treatment, bevacizumab IV, or a blood transfusion. Monitor at 1-minute intervals during clinical and laboratory hours.
Brain AVM surveillance MRI platforms carry the highest neurological hemorrhage prevention priority. Cerebral AVMs in HHT require MRI/MRA surveillance at intervals determined by AVM size, location, and prior treatment — missed surveillance intervals allow undetected AVM growth and increased hemorrhage risk. Monitor at 1-minute intervals during radiology hours.
Family cascade screening coordination platforms must manage proband-to-family mutation testing across extended pedigrees. Each unscreened first-degree relative of an HHT patient is a potential missed PAVM screening opportunity — a missed PAVM could cause a preventable stroke. Monitor cascade screening scheduling platforms at 1-minute intervals during clinical hours.
What to Monitor on an HHT Tech Platform
Genetic Testing — ENG, ACVRL1, SMAD4, and GDF2 Mutation Analysis
Monitor ENG and ACVRL1 sequencing records (NGS of full coding sequences — point mutations, small insertions/deletions, and copy number variants; ACVRL1 and ENG mutation spectrum includes missense variants in kinase domain or extracellular binding domain, frameshift and nonsense mutations, and large intragenic deletions requiring MLPA or CNV analysis), SMAD4 mutation records (juvenile polyposis-HHT overlap — colonoscopic surveillance indicated for all JP-HHT patients from childhood due to hamartomatous polyp cancer risk), GDF2/BMP9 mutation records (HHT5 genotype — rare; prevalence of PAVM and CAVM in this subtype), variant classification records (VUS resolution requiring functional studies or segregation analysis in extended pedigrees), family cascade mutation testing records (first-degree relatives — parents, siblings, children — referred for ENG/ACVRL1 targeted mutation testing once proband mutation identified; positive relatives enrolled in full HHT surveillance protocol), and prenatal diagnosis records (PGT-M for couples considering preimplantation genetic testing — particularly relevant for SMAD4 carriers given polyp cancer risk in affected offspring) at 1-minute intervals during laboratory hours.
Epistaxis Severity Scoring and Hemostasis Management
Monitor Epistaxis Severity Score (ESS) records (validated 0-10 scale assessing epistaxis frequency, duration, and severity; scored at each clinic visit and between visits for acutely worsening epistaxis — ESS ≥4 associated with significant quality of life impairment; ESS ≥6 associated with high transfusion burden), epistaxis treatment escalation records (humidification and lubricant nasal spray as baseline; laser photocoagulation as first procedural intervention for moderate epistaxis; argon plasma coagulation or bipolar diathermy; intranasal bevacizumab injection — recent RCT data supporting intranasal bevacizumab for moderate-severe epistaxis; septodermoplasty or Young's nasal closure as definitive surgical options for severely affected patients), epistaxis hospitalization records (emergency department visits, hospital admissions, and blood transfusions for acute severe epistaxis — frequency and total blood transfused), and IV bevacizumab for severe epistaxis records (bevacizumab infusion schedule — typically monthly × 6 doses then reassessment; ESS response at 3 and 6 months post-induction; retreatment decision records) at 1-minute intervals during clinical hours. Alert immediately — clinical platform failures that prevent ESS documentation and hemoglobin trending from being available at a clinic visit for a patient presenting with a week of nightly epistaxis episodes and lightheadedness prevent the clinical team from recognizing that this patient's hemoglobin has dropped from 10.2 g/dL to 7.8 g/dL in 6 weeks and that IV iron and bevacizumab initiation cannot be deferred.
Hemoglobin and Iron Studies
Monitor hemoglobin and ferritin records (CBC with hemoglobin — monthly during active anemia management; serum ferritin and iron saturation — ferritin <30 ng/mL or transferrin saturation <20% indicating iron deficiency requiring IV iron; hemoglobin <8 g/dL triggering transfusion threshold assessment), IV iron infusion records (ferric carboxymaltose, iron sucrose, or low-molecular-weight iron dextran — infusion schedule, dose, tolerability records; ferritin response at 4-8 weeks post-infusion; retreatment decision), blood transfusion records (packed red blood cell transfusions — frequency, total volume, pre- and post-transfusion hemoglobin, indication documentation), and iron-deficiency anemia complication records (fatigue severity score, exertional dyspnea, cardiac output assessment by echocardiography for high-output cardiac failure from both hepatic AVMs and anemia) at 1-minute intervals during clinical and laboratory hours.
Pulmonary AVM Surveillance and Embolization
Monitor CT pulmonary angiography records (diagnostic CTPA for PAVM screening and characterization — feeding artery diameter, location, number, bilateral versus unilateral, simple versus complex PAVM morphology), PAVM surveillance scheduling records (embolized PAVMs require CTPA at 6-12 months post-embolization to confirm occlusion and detect recanalization or reperfusion; known non-embolizable PAVMs <2-3 mm require 3-5 year surveillance CTPA; mutation-positive relatives require screening CTPA), PAVM embolization records (transcatheter PAVM embolization — Amplatzer plug, microcoil, or combined device occlusion; feeding artery diameter pre- and post-embolization; intraoperative oxygen saturation response to test occlusion; post-embolization infarction/pleuritis; PAVM recanalization on follow-up CTPA), shunt quantification records (contrast echocardiography or radionuclide quantification of right-to-left shunt fraction — useful when CTPA cannot be performed; shunt fraction >25% associated with platypnea-orthodeoxia), resting and exertional oxygen saturation records (SpO2 at rest and with exertion — desaturation identifying clinically significant shunting; oxygen supplementation prescription for exertional desaturation), and antibiotic prophylaxis records (antibiotic prophylaxis before dental and mucosal procedures for HHT patients with unscreened or unembolized PAVMs — preventing brain abscess from dental bacteremia; discontinuation of prophylaxis after confirmed embolization occlusion) at 1-minute intervals during radiology and interventional radiology hours. Alert immediately — PAVM surveillance CT scheduling platform failures that allow a 3-year follow-up CTPA for a patient with a previously identified 2.8 mm right lower lobe PAVM that had been below the embolization threshold at the last assessment to be delayed by 8 months create a monitoring gap during which the PAVM may have grown to the embolization threshold without clinical recognition.
Brain AVM Surveillance and Management
Monitor brain MRI and MRA records (brain MRI for CAVM detection and characterization — Spetzler-Martin grade, size, location, deep venous drainage, eloquent cortex involvement; contrast-enhanced MRA for nidus and feeding artery characterization; diagnostic cerebral catheter angiography for treatment planning), CAVM surveillance scheduling records (newly identified unruptured CAVMs <3 cm in non-eloquent cortex in adults: conservative management with annual MRI surveillance; ruptured CAVMs or those in eloquent cortex: neurosurgery evaluation for treatment; pediatric CAVMs: aggressive treatment evaluation given lifetime hemorrhage risk accumulation), CAVM treatment records (Gamma Knife or linear accelerator stereotactic radiosurgery — obliteration rate at 3 years; latency hemorrhage risk during obliteration period; endovascular embolization as adjunct to radiosurgery; microsurgical resection for accessible AVMs), neurological complication records (headache, seizure, new focal neurological deficit, intracranial hemorrhage — emergency neuroimaging records, ICU management, neurosurgical intervention), and spinal AVM records (spinal MRI for detection of spinal AVMs in patients with myelopathy symptoms — rare but potentially treatable if identified before permanent cord injury) at 1-minute intervals during radiology and neurology hours.
Hepatic AVM Monitoring
Monitor hepatic Doppler ultrasound records (hepatic artery and portal vein Doppler — direction and velocity of flow; reversal of portal flow direction indicating arterioportal shunting; hepatic artery hypervascularization as hepatic AVM marker), hepatic CT or MRI records (hepatic AVMs characterization — diffuse telangiectatic type, large confluent AVM type, mixed type; hepatic AVM burden scoring), cardiac output and echocardiography records (cardiac output by echocardiography — high-output cardiac failure from hepatic arteriovenous shunting; LVEF, cardiac output, NT-proBNP for heart failure staging; diuretic and beta-blocker therapy records for high-output HF), hepatic synthetic function records (bilirubin, alkaline phosphatase, GGT, albumin, INR — biliary ischemia from hepatic arteriobiliary AVMs causing cholangiopathy), and bevacizumab for hepatic AVM records (IV bevacizumab in symptomatic hepatic AVM — cardiac output response, reduction in hepatic arteriovenous shunting, biliary complication stabilization; liver transplantation records for refractory symptomatic hepatic AVM with hepatic failure — rare definitive option) at 1-minute intervals during clinical hours. Alert immediately — hepatic monitoring platform failures preventing cardiac output echocardiography scheduling for a patient with progressively worsening exertional dyspnea and a hepatic Doppler showing high-velocity hepatic artery flow miss the early identification of high-output cardiac failure that requires medical therapy initiation before irreversible cardiac remodeling occurs.
GI Telangiectasia and Bleeding Management
Monitor GI bleeding records (melena, hematochezia, positive fecal occult blood — contribution to iron-deficiency anemia independent of epistaxis; frequency and severity scoring), upper GI endoscopy records (EGD for gastric and duodenal telangiectases — argon plasma coagulation or Nd:YAG laser treatment; endoscopic map of telangiectasis density and distribution), push enteroscopy and capsule endoscopy records (small bowel telangiectases — capsule endoscopy for screening; device-assisted enteroscopy with APC for accessible lesions), and bevacizumab for GI bleeding records (IV bevacizumab reducing GI telangiectasis-related bleeding — response measured by hemoglobin trajectory and transfusion frequency reduction) at 1-minute intervals during clinical and gastroenterology hours.
Family Cascade Screening Coordination
Monitor proband index case mutation records (confirmed ENG, ACVRL1, SMAD4, or GDF2 mutation — basis for family testing), first-degree relative contact and testing records (systematic outreach to parents, siblings, and children of proband — targeted mutation testing, result disclosure, enrollment in surveillance protocol for positive relatives), family pedigree records (extended pedigree documentation — mutation-positive relatives in each generation; cascade expansion tracking), surveillance protocol initiation records for confirmed mutation-positive relatives (PAVM screening CTPA, brain MRI, abdominal Doppler, epistaxis severity assessment — all scheduled within 6 months of mutation confirmation), and annual family registry update records (pedigree updates with new births, new diagnoses, and new screening completions) at 1-minute intervals during clinical hours.
Authentication and Clinical Identity
Monitor authentication at 1-minute intervals, 24/7. HHT management coordinates across HHT specialty centers (multidisciplinary program coordination), radiology (PAVM CT surveillance), interventional radiology (PAVM embolization), neurology and neurosurgery (CAVM management and hemorrhagic stroke), otorhinolaryngology (epistaxis management), pulmonary medicine (hypoxemia and shunting), gastroenterology (GI bleeding and endoscopy), hepatology (hepatic AVM and high-output failure), cardiovascular medicine and heart failure (hepatic AVM cardiomyopathy), infusion oncology (bevacizumab administration), genetic testing and counseling (ENG/ACVRL1 mutation analysis and cascade screening), pediatric genetics (family cascade screening in children), and iron infusion clinics — authentication failures across this comprehensive multi-specialty platform infrastructure disrupt the multi-organ surveillance and prevention program that HHT requires from initial diagnosis through lifelong AVM surveillance.
SSL Certificates
Monitor SSL certificate expiry across all HHT specialty center platforms, radiology and interventional radiology scheduling systems, neurology and neurosurgery platforms, endoscopy scheduling systems, genetic testing portals, bevacizumab infusion scheduling systems, and family cascade screening coordination platforms. Certificate errors disrupt PAVM embolization scheduling, brain AVM surveillance scheduling, and family cascade mutation testing workflows.
HIPAA and Genetic Information Privacy Considerations
HHT technology platforms handle GINA-protected genetic information (ENG and ACVRL1 heterozygous mutation results with 50% transmission probability to each child), multi-organ AVM imaging records including sensitive brain MRI findings, cerebral AVM hemorrhage emergency records, brain and pulmonary AVM treatment records with radiation exposure documentation, hepatic AVM and high-output heart failure records, IV bevacizumab oncology infusion records in non-oncology patients, epistaxis severity and blood transfusion frequency records, and family cascade screening records that traverse multiple family units. The cascade nature of HHT genetic testing creates privacy challenges when a proband's mutation test result necessitates disclosure to family members — platforms must support selective disclosure workflows that respect each family member's right to know and right not to know while ensuring that mutation-positive relatives who want surveillance can access screening.
SMAD4 mutation carriers with JP-HHT require colonoscopic surveillance records with polypectomy histopathology — combining rare disease genetic records with cancer screening records in a single platform requiring both HHT specialist access and gastroenterology access while maintaining role-based access controls. HHT patient registries (HHT Foundation International registry; Brain Vascular Malformation Consortium registry) hold individually identifiable multi-organ AVM imaging and clinical outcome data requiring IRB oversight, DUA agreements, and HIPAA-compliant data governance frameworks.
Alerting Strategy for HHT Tech Platforms
Immediate 24/7 alerting for brain AVM hemorrhage emergency protocols: Intracranial hemorrhage from cerebral AVM rupture is a neurosurgical emergency requiring 24/7 platform availability for emergency neuroimaging and neurosurgical intervention records.
Immediate radiology and IR hours alerting for PAVM surveillance CT and embolization scheduling: Missed PAVM surveillance intervals and embolization scheduling delays directly increase the risk of paradoxical embolism causing ischemic stroke or brain abscess.
Immediate clinical-hours alerting for epistaxis severity and hemoglobin tracking: ESS score and hemoglobin trending at each visit determines IV iron, bevacizumab, and transfusion decisions — these are the highest-frequency routine monitoring obligations in HHT.
Immediate clinical-hours alerting for hepatic AVM cardiac output surveillance: High-output heart failure from hepatic AVMs is a progressive complication requiring early detection before irreversible cardiac remodeling.
Immediate clinical-hours alerting for family cascade screening coordination: Each unscreened mutation-positive relative represents a potential missed PAVM screening and preventable paradoxical embolism risk.
Sustained-failure alert (10–15 minutes): GI bleeding endoscopy records, spinal AVM surveillance, antibiotic prophylaxis protocol records.
30-day advance warning: SSL certificates across all domains.
Status Page for HHT Care Team Communication
A real-time status page gives HHT specialty center coordinators managing multi-organ surveillance schedules, radiologists performing PAVM CT angiography and embolization, neuroradiologists and neurosurgeons managing brain AVM surveillance and treatment, ENT surgeons managing epistaxis escalation, gastroenterologists managing GI telangiectasia bleeding, hepatologists and cardiologists managing hepatic AVM high-output heart failure, infusion oncology nurses administering bevacizumab, genetic counselors coordinating family cascade screening, and hematologists managing transfusion-dependent iron-deficiency anemia immediate platform visibility without inbound IT support contact.
Include the status page URL in PAVM embolization emergency protocols, brain AVM hemorrhage emergency procedures, and epistaxis transfusion escalation backup workflows.
Vigilmon Setup for HHT Tech Platforms
A practical starting configuration:
| Monitor | Check Interval | Alert Channel | |---------|----------------|---------------| | Authentication | 1 min | Slack + PagerDuty (24/7) | | ENG / ACVRL1 / SMAD4 genetic testing | 1 min | Slack + PagerDuty (lab hours) | | Epistaxis Severity Score (ESS) documentation | 1 min | Slack + PagerDuty (clinical hours) | | Hemoglobin and iron studies | 1 min | Slack + PagerDuty (lab hours) | | IV iron infusion scheduling and records | 1 min | Slack + PagerDuty (clinical hours) | | Blood transfusion threshold tracking | 1 min | Slack + PagerDuty (clinical hours) | | PAVM surveillance CT scheduling | 1 min | Slack + PagerDuty (radiology hours) | | PAVM embolization scheduling and records | 1 min | Slack + PagerDuty (IR hours) | | Post-embolization follow-up CT | 1 min | Slack + PagerDuty (radiology hours) | | Resting and exertional oxygen saturation | 1 min | Slack + PagerDuty (clinical hours) | | Brain AVM MRI/MRA surveillance scheduling | 1 min | Slack + PagerDuty (radiology hours) | | Brain AVM hemorrhage emergency protocol | 1 min | Slack + PagerDuty (24/7) | | Hepatic AVM Doppler and cardiac output | 1 min | Slack + PagerDuty (clinical hours) | | Bevacizumab IV infusion scheduling | 1 min | Slack + PagerDuty (clinical hours) | | GI endoscopy and telangiectasia records | 1 min | Slack + PagerDuty (clinical hours) | | Family cascade mutation testing coordination | 1 min | Slack + PagerDuty (clinical hours) | | SMAD4 colonoscopy surveillance scheduling | 2 min | Slack (clinical hours) | | Antibiotic prophylaxis protocol records | 2 min | Slack (business hours) | | Genetic counseling and pedigree records | 2 min | Slack (business hours) | | SSL: all domains | Daily | Email (30-day warning) |
Getting started:
- Create a free account at vigilmon.online
- Add authentication endpoints at 1-minute intervals with 24/7 alerting
- Configure ENG/ACVRL1/SMAD4 genetic testing platforms with immediate laboratory-hours alerting
- Add Epistaxis Severity Score documentation platforms with immediate clinical-hours alerting
- Configure hemoglobin and iron study platforms with immediate laboratory-hours alerting
- Add IV iron infusion scheduling and records platforms with immediate clinical-hours alerting
- Configure blood transfusion threshold tracking with immediate clinical-hours alerting
- Add PAVM surveillance CT scheduling platforms with immediate radiology-hours alerting — this is the primary paradoxical embolism prevention monitoring workflow in HHT
- Configure PAVM embolization scheduling and records platforms with immediate interventional radiology-hours alerting
- Add post-embolization follow-up CT scheduling with immediate radiology-hours alerting
- Configure resting and exertional oxygen saturation tracking with immediate clinical-hours alerting
- Add brain AVM MRI/MRA surveillance scheduling platforms with immediate radiology-hours alerting
- Configure brain AVM hemorrhage emergency protocols with 24/7 immediate alerting — the highest-acuity neurological emergency in HHT
- Add hepatic AVM Doppler and cardiac output echocardiography platforms with immediate clinical-hours alerting
- Configure IV bevacizumab infusion scheduling platforms with immediate clinical-hours alerting
- Add GI endoscopy and telangiectasia treatment records with immediate clinical-hours alerting
- Configure family cascade mutation testing coordination platforms with immediate clinical-hours alerting
- Add SMAD4 colonoscopy surveillance scheduling with sustained-failure alerting
- Configure antibiotic prophylaxis protocol records with sustained-failure alerting
- Enable SSL certificate monitoring across all HHT specialty center, radiology, interventional radiology, neurology, endoscopy, genetics, and bevacizumab infusion platforms with 30-day advance email warning
Conclusion
HHT technology platforms are embedded in clinical decisions where PAVM surveillance CT scheduling platform availability on the afternoon when the HHT center coordinator is booking the 3-year follow-up CT pulmonary angiography for a 36-year-old woman with ENG-positive HHT1 who had a 2.4 mm right lower lobe PAVM identified on her baseline screening CT 3 years ago — which at that time was below the embolization threshold of 2-3 mm feeding artery diameter but whose size places it exactly at the margin where interval growth could bring it to the embolization-eligible threshold, and whose clinical significance is underscored by the fact that she had two "unexplained" syncopal episodes in the past 18 months that may have represented micro-paradoxical emboli — cannot be disrupted by radiology scheduling platform failures that push the follow-up CT date from the intended 3-year mark to 14 months later, creating a monitoring gap during which the PAVM may have grown from 2.4 mm to 3.2 mm (a size at which embolization is clearly indicated) and during which she remains at risk for the paradoxical embolism that embolization was intended to prevent; where epistaxis and hemoglobin platform availability on the morning of an HHT clinic visit for a 58-year-old man with ACVRL1-positive HHT2 who reports that his nightly epistaxis has worsened from approximately 15 minutes per episode to 45-60 minutes per episode over the past 6 weeks, and the ENT has requested a hemoglobin and ferritin from this morning's blood draw to determine whether to escalate from oral iron supplementation to IV iron or to pursue urgently bevacizumab evaluation — and the clinic platform must display both the ESS score calculated from the intake questionnaire and the hemoglobin and ferritin results from this morning's blood draw on the same dashboard before the clinic encounter, because the treatment decision tree at this visit branches at the intersection of ESS severity and laboratory response — cannot be disrupted by laboratory reporting platform failures that present the hemoglobin result in an inaccessible queue while the patient is in the examination room; and where family cascade screening coordination platform availability when the HHT genetic counselor is preparing the outreach package for the four first-degree relatives of a newly confirmed ENG c.1738C>T (p.Arg580Ter) proband — three adult children and one sibling, none of whom have ever been evaluated for HHT despite a family history of nosebleeds in two of them — and the coordination platform must generate targeted mutation test referral letters for each family member, schedule their initial HHT clinic visits for epistaxis assessment and AVM screening, and create a family cascade tracking record in the proband's chart that will follow each relative's screening completion status through to full PAVM CT, brain MRI, and abdominal Doppler completion — cannot be disrupted by cascade coordination platform failures that cause two of the four family members' referral letters to be queued without being sent, leaving two potentially mutation-positive relatives — one of whom may have an asymptomatic PAVM — unevaluated because the cascade tracking system silently failed to complete its outreach workflow. A PAVM surveillance CT scheduling platform that allows a marginal PAVM to go unmonitored past the point at which embolization would have been timely, a clinic platform that prevents ESS severity from being integrated with hemoglobin trending at the moment the treatment escalation decision is made, a family cascade coordination system that silently fails to complete outreach to mutation-positive relatives — these are not IT incidents. They are clinical disruptions in the management of a hereditary vascular disorder where the paradoxical embolism prevention window, the epistaxis anemia management interval, and the family cascade screening reach are all measured in months, and where platform failures at precisely the monitoring and scheduling junctures that define the HHT prevention program can translate directly into the preventable stroke, the unrecognized anemia requiring transfusion, and the unscreened family member with an untreated PAVM.
Uptime monitoring gives HHT tech teams the detection capability to identify failures within seconds, trigger immediate clinical downtime procedures, and demonstrate to HHT specialty center physicians managing multi-organ AVM surveillance, interventional radiologists performing PAVM embolization, neuroradiologists and neurosurgeons managing brain AVM surveillance and treatment, ENT surgeons managing epistaxis escalation, hematologists managing iron-deficiency anemia, gastroenterologists managing GI telangiectasia bleeding, cardiologists managing hepatic AVM high-output heart failure, bevacizumab infusion nurses, genetic counselors coordinating ENG/ACVRL1 family cascade screening, and rare disease registry administrators that platform operational reliability matches the PAVM embolization scheduling precision, epistaxis severity and anemia surveillance frequency, brain AVM hemorrhage prevention urgency, and family cascade screening comprehensiveness of modern HHT management.
Start monitoring your HHT care tech platform for free at vigilmon.online — HTTP/HTTPS monitoring, multi-region consensus alerting, SSL certificate monitoring, automatic status page, Slack and webhook alerts. No agent required. No credit card.
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