Pulmonary Arterial Hypertension — designated PAH, Group 1 in the WHO clinical classification of pulmonary hypertension (Dana Point 2008, updated Nice 2019 and 2022), defined hemodynamically as a mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest (revised to >20 mmHg in the 2022 ESC/ERS guidelines) with pulmonary arterial wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) >2 Wood units measured by right heart catheterization, a rare progressive vasculopathy of the pre-capillary pulmonary circulation estimated to affect 15-50 persons per million in developed countries with an annual incidence of 2-7 cases per million, characterized by a pathological triad of pulmonary arterial vasoconstriction, smooth muscle cell proliferation and hypertrophy causing medial thickening, and endothelial dysfunction with in-situ thrombosis causing progressive obliteration of the distal pulmonary vascular bed and elevation of pulmonary vascular resistance that imposes a relentlessly increasing pressure and volume afterload on the right ventricle; the WHO clinical subtypes include idiopathic PAH (IPAH — the most common form, no identifiable cause), heritable PAH (HPAH — caused by germline mutations, most commonly heterozygous loss-of-function mutations in BMPR2 encoding bone morphogenetic protein receptor type 2, a TGF-β superfamily signaling receptor whose haploinsufficiency leads to inappropriate pulmonary smooth muscle cell proliferation and apoptosis resistance, present in approximately 70% of familial and 15-20% of sporadic IPAH cases; less commonly ACVRL1/ALK1 mutations causing HHT-related PAH, ENG mutations, SMAD9 mutations, CAV1 mutations, KCNK3 mutations, ATP13A3, SOX17, and GDF2/BMP9 mutations have been identified in heritable PAH pedigrees), drug- and toxin-induced PAH (appetite suppressants — aminorex, fenfluramine, dexfenfluramine; amphetamines; dasatinib; methamphetamine), and PAH associated with connective tissue disease (primarily systemic sclerosis/scleroderma — the highest-risk CTD for developing PAH, affecting 5-12% of SSc patients; also systemic lupus erythematosus, rheumatoid arthritis, and mixed connective tissue disease), HIV infection, portal hypertension (portopulmonary hypertension), congenital heart disease (Eisenmenger syndrome and other systemic-to-pulmonary shunts), and schistosomiasis; the pathological lesions of PAH — medial hypertrophy, intimal proliferation, adventitial fibrosis, in-situ thrombosis, and the pathognomonic plexiform lesion (angioproliferative lesion arising from apoptosis-resistant endothelial cells forming a glomeruloid vascular channel network occluding small pulmonary arterioles) — are irreversible once established, making early detection and aggressive therapy initiation the primary determinants of outcomes; treatment targeting three established pathways has transformed PAH prognosis — endothelin pathway antagonism (bosentan, ambrisentan, macitentan — endothelin-1 receptor A and B antagonists reducing vasoconstriction and smooth muscle cell proliferation), nitric oxide/cGMP pathway enhancement (sildenafil, tadalafil — PDE5 inhibitors increasing cGMP and potentiating pulmonary vasodilation; riociguat — soluble guanylate cyclase stimulator), and prostacyclin/prostanoid pathway augmentation (epoprostenol IV — the first approved PAH therapy, the gold standard for severe PAH and the only agent with proven mortality benefit in randomized trials; treprostinil IV/SQ/inhaled/oral; iloprost inhaled; selexipag oral); the ERA/PDE5i combination has become standard initial combination oral therapy for newly diagnosed WHO FC II-III patients, with rapid escalation to triple oral or parenteral prostanoid-based combinations for FC III-IV patients; lung transplantation remains the definitive therapeutic option for patients who fail or become refractory to maximal medical therapy.
PAH technology platforms — encompassing the pulmonary hypertension specialty center platforms where right heart catheterization scheduling and results management, six-minute walk distance (6MWD) testing, BNP/NT-proBNP biomarker tracking, functional class documentation, and combination therapy management are conducted, the echocardiography platforms where right ventricular function surveillance and tricuspid regurgitation-derived pulmonary artery pressure estimation are performed, the cardiac catheterization laboratory platforms where right heart catheterization with vasoreactivity testing, acute decompensation assessment, and treatment escalation guidance are performed, the infusion pharmacy and specialty pharmacy platforms managing epoprostenol IV compounding and treprostinil IV/SQ infusion therapy, the genetic testing laboratory platforms where BMPR2 and other heritable PAH gene panel analysis is conducted, the connected device platforms monitoring ambulatory 6MWD and activity data from wearable sensors, the pulmonary rehabilitation platforms managing structured exercise programs, and the lung transplant center platforms coordinating transplant evaluation and listing for patients with refractory disease — must maintain the availability and performance standards required by the 6MWD trend surveillance precision, the BNP/NT-proBNP biomarker monitoring frequency, the right heart catheterization scheduling regularity, the combination therapy adherence tracking, the functional class deterioration detection sensitivity, and the lung transplant evaluation coordination timeline that define comprehensive PAH care. This guide explains why PAH care tech platforms need dedicated monitoring, what components to monitor, and how to build a monitoring strategy matched to the progressive disease course, multi-drug combination therapy complexity, right heart failure surveillance urgency, and transplant evaluation coordination requirements that characterize modern PAH management.
Why PAH Tech Platforms Require Specialized Monitoring Attention
PAH management is defined by several distinctive care coordination challenges: the risk stratification surveillance imperative — modern PAH management uses validated risk stratification tools (REVEAL 2.0, ESC/ERS low-intermediate-high risk classification based on 6MWD, BNP/NT-proBNP, functional class, right ventricular function indices, and hemodynamics) to assess treatment response at 3-6 month intervals and to guide therapy escalation decisions, requiring consistent platform availability for multi-parameter risk score calculation and comparison to prior assessments that determines whether a patient remains on current therapy or requires urgent escalation to parenteral prostanoids; the parenteral prostanoid therapy management urgency — epoprostenol IV and treprostinil IV/SQ are life-sustaining therapies for FC IV PAH patients whose premature discontinuation due to infusion pump failure, medication supply interruption, or care coordination platform failure can result in rapid clinical deterioration and death within hours to days, making the platforms managing pump programming records, infusion rate escalation documentation, and specialty pharmacy reorder systems among the most clinically critical in any rare disease care program; the right heart failure decompensation monitoring sensitivity — the right ventricle in PAH can decompensate acutely in response to intercurrent illness, arrhythmia, or pulmonary infection, with acute right heart failure carrying high short-term mortality if not recognized and treated promptly, making right ventricular function tracking platforms and BNP/NT-proBNP biomarker platforms continuous clinical obligations; the combination therapy interaction management complexity — most PAH patients are managed on two or three agents from different therapeutic classes simultaneously (ERA + PDE5i ± prostanoid), with complex drug-drug interaction profiles, monitoring obligations (LFT monitoring for ERAs — ambrisentan requires monthly LFT; bosentan requires monthly LFT due to hepatotoxicity risk and REMS program obligations), and adherence challenges requiring multi-drug management platforms; and the lung transplant evaluation timing urgency — the window for successful lung or bilateral lung transplantation in PAH requires referral to a transplant center while the patient still has sufficient functional reserve to tolerate transplant evaluation and the perioperative period, making early transplant referral platform integration a clinical priority that cannot be deferred by platform failures.
Right heart catheterization platforms are the diagnostic gold standard and primary PAH treatment response assessment tool. Right heart catheterization documenting mPAP, PAWP, PVR, cardiac output, and right atrial pressure at baseline and following therapy determines PAH diagnosis, risk stratification, and treatment escalation necessity. Monitor RHC scheduling and results platforms at 1-minute intervals during catheterization laboratory hours.
BNP/NT-proBNP biomarker platforms must deliver results within hours of the clinical assessment window. BNP and NT-proBNP are the primary circulating biomarkers of right ventricular wall stress in PAH, and rising BNP/NT-proBNP despite therapy is the clearest biochemical signal of right heart failure progression requiring therapy escalation. Monitor biomarker reporting platforms at 1-minute intervals during clinical and laboratory hours.
Parenteral prostanoid infusion management platforms are life-critical and cannot fail during active therapy. Epoprostenol and treprostinil therapy requires pump programming records, infusion rate escalation logs, and specialty pharmacy reorder systems that must not fail for a patient whose survival depends on uninterrupted continuous infusion. Monitor at 1-minute intervals, 24/7.
Six-minute walk distance platforms must track the functional exercise capacity trend that drives risk stratification. 6MWD testing at 3-6 month intervals provides the functional exercise data that determines whether a patient is improving, stable, or deteriorating — a decline of ≥15% from baseline or an absolute 6MWD below 165 meters defines high mortality risk. Monitor 6MWD testing and recording platforms at 1-minute intervals during clinical hours.
What to Monitor on a PAH Tech Platform
Genetic Testing — BMPR2 and Heritable PAH Gene Panel
Monitor BMPR2 mutation analysis records (sequencing of the full BMPR2 coding sequence — BMPR2 mutations include missense, nonsense, frameshift, large deletion, and splice site variants; penetrance is incomplete — approximately 20% of BMPR2 mutation carriers develop PAH; variant classification documentation with clinical significance), heritable PAH panel analysis records (multigene panel including BMPR2, ACVRL1, ENG, SMAD9, CAV1, KCNK3, ATP13A3, SOX17, GDF2/BMP9 — for evaluation of patients with PAH without identifiable cause after clinical workup; panel choice and interpretation documentation), family cascade screening records (autosomal dominant inheritance for BMPR2-related HPAH with incomplete penetrance; annual echocardiographic screening for carrier family members; RHC for those with echocardiographic evidence of elevated pulmonary artery pressure), and variant interpretation records (VUS resolution by parental segregation, functional testing, or curated database evidence) at 1-minute intervals during laboratory hours.
Right Heart Catheterization — Hemodynamic Assessment
Monitor right heart catheterization records (diagnostic RHC at PAH diagnosis — mPAP, PAWP, PVR, cardiac output by thermodilution and Fick method, right atrial pressure, mixed venous oxygen saturation; vasoreactivity testing with inhaled NO or IV epoprostenol to identify candidates for calcium channel blocker therapy — criteria: mPAP decrease ≥10 mmHg to ≤40 mmHg with maintained or increased cardiac output), follow-up RHC records (repeat RHC for treatment response assessment — hemodynamic deterioration from baseline or failure to achieve treatment goals triggers therapy escalation; PVR normalization or near-normalization documents exceptional treatment response), acute RHC records (urgent RHC during suspected right heart failure decompensation — Fick cardiac output, right atrial pressure, oxygen extraction ratio as decompensation indices), and hemodynamic goal tracking records (therapeutic targets: PVR <5 Wood units; RAP <8 mmHg; cardiac index >2.5 L/min/m²) at 1-minute intervals during catheterization laboratory hours. Alert immediately — RHC scheduling platform failures preventing timely repeat hemodynamic assessment for a patient with rising BNP and declining 6MWD create a clinical delay in identifying hemodynamic deterioration requiring urgent therapy escalation to parenteral prostanoids.
Six-Minute Walk Distance Testing
Monitor 6MWD test records (standardized corridor 6MWD testing with Borg dyspnea scale and oxygen saturation nadir documentation at 3-month intervals during stable disease, 1-month intervals following therapy change; absolute 6MWD, percent predicted, and change from prior test), 6MWD trend documentation records (rate of 6MWD decline over preceding assessments — progressive decline despite optimal medical therapy identifies the patient approaching the threshold for parenteral prostanoid escalation or transplant referral), saturometric response records (SpO2 nadir during 6MWD — desaturation to <85% during exertion identifying patients requiring supplemental oxygen during activity), and wearable activity monitoring records (daily step count, activity duration, sedentary fraction from accelerometer-based monitoring in enrolled PAH patients; activity trend at 1-week intervals) at 1-minute intervals during clinical hours.
BNP and NT-proBNP Biomarker Monitoring
Monitor BNP records (B-type natriuretic peptide — secreted by right ventricular cardiomyocytes in response to wall stress; BNP <50 pg/mL associated with low risk; BNP ≥300 pg/mL associated with high risk and 1-year mortality exceeding 30%; change from prior value as the primary metric — rising BNP despite therapy defines treatment failure), NT-proBNP records (N-terminal pro-BNP — alternative right ventricular biomarker with longer half-life and greater diurnal stability; NT-proBNP <300 pg/mL low risk; NT-proBNP ≥1,100 pg/mL high risk), BNP/NT-proBNP trend documentation records (absolute value and percent change from the prior assessment 3-6 months ago — persistent BNP/NT-proBNP >10× upper limit of normal, or progressive rise despite therapy, triggering therapy escalation discussion), and integrated risk score records (REVEAL 2.0 risk calculator score — combining BNP, 6MWD, WHO functional class, RHC hemodynamics, echocardiographic RV function, and clinical features into a composite risk score of 1-22; score ≥8 defines high risk) at 1-minute intervals during laboratory and clinical hours. Alert immediately — BNP reporting platform failures preventing NT-proBNP results from reaching the pulmonary hypertension team on the day of a 3-month follow-up clinic visit for a patient whose prior visit NT-proBNP was 680 pg/mL — and which has now risen to 1,450 pg/mL — prevent the team from implementing the therapy escalation decision that the result mandates.
WHO Functional Class Tracking
Monitor WHO functional class documentation records (FC I: no symptoms at rest or with ordinary activity; FC II: slight limitation of activity; FC III: marked limitation, comfortable only at rest; FC IV: symptoms at rest or with minimal activity; class at each clinic visit), functional class trend records (FC deterioration from II to III or from III to IV triggers immediate therapy escalation; FC II on ERA/PDE5i combination initiates consideration of prophylactic prostanoid addition; FC IV requires immediate hospitalization for IV prostanoid initiation), and symptom log records (dyspnea with exertion, syncope, presyncope, ankle edema, fatigue — quantified with validated instruments at each visit) at 1-minute intervals during clinical hours.
Combination Therapy Management and Adherence
Monitor ERA therapy records (bosentan or ambrisentan or macitentan — dose, tolerability, monthly LFT monitoring for bosentan REMS compliance, hemoglobin monitoring for fluid retention with ambrisentan, drug interaction documentation — ERAs are CYP3A4 inducers/inhibitors), PDE5 inhibitor records (sildenafil or tadalafil — dose, frequency, tolerability, blood pressure monitoring for hypotension with combination therapy), prostanoid therapy records (selexipag oral — dose titration schedule, tolerability; inhaled iloprost or treprostinil — inhalation frequency, device maintenance; treprostinil SQ or IV — infusion rate, site reactions for SQ, pump settings for IV; epoprostenol IV — pump programming, dose, reconstitution records, compounding pharmacy coordination, pump alarm history), combination therapy adherence records (multi-drug adherence measured by pill count, specialty pharmacy refill records, electronic monitoring; adherence to inhaled prostanoid inhalation frequency; infusion pump alarm acknowledgment records), and ERA REMS compliance records (bosentan REMS — monthly LFT, pregnancy test for women of childbearing potential, dispensing records; ambrisentan REMS if applicable) at 1-minute intervals during clinical and pharmacy hours.
Echocardiographic Right Ventricular Function Surveillance
Monitor echocardiography records (transthoracic echocardiogram at diagnosis, 3-6 months after therapy initiation, annually in stable disease, immediately with clinical deterioration — right ventricular size and systolic function assessment: TAPSE ≥18 mm is favorable; RV fractional area change; tricuspid annular plane systolic excursion; right atrial area; pericardial effusion as high-risk sign; TR peak velocity for PA systolic pressure estimation), right ventricular function trend records (TAPSE decline from baseline, RV enlargement progression, new pericardial effusion — all high-risk echocardiographic features triggering therapy escalation or urgent right heart catheterization), and cardiac MRI records (right ventricular volumes, ejection fraction, mass, and late gadolinium enhancement by cardiac MRI — performed annually for quantitative right ventricular function assessment in selected patients) at 1-minute intervals during cardiology and radiology hours.
Anticoagulation Management
Monitor anticoagulation records (warfarin anticoagulation — particularly for IPAH and HPAH patients where observational data and guideline recommendations support warfarin use for pulmonary arterial thrombosis; INR target 1.5-2.5; monthly INR monitoring during stable anticoagulation), anticoagulation contraindication records (portopulmonary hypertension — anticoagulation contraindicated due to bleeding risk from esophageal varices; hemoptysis history — anticoagulation modification records), DOAC records (direct oral anticoagulants as alternative to warfarin in selected patients — dose, tolerability, drug-drug interaction documentation with ERAs), and bleeding event records (hemoptysis, GI bleeding, CNS hemorrhage — anticoagulation hold or discontinuation records) at 1-minute intervals during clinical hours.
Supplemental Oxygen and Exercise Program Management
Monitor supplemental oxygen records (resting SpO2 <90% — continuous supplemental oxygen prescription; exertional SpO2 <85% — supplemental oxygen during activity; nocturnal oxygen saturation monitoring; oxygen delivery device records), pulmonary rehabilitation records (supervised exercise program — resistance training and aerobic conditioning modified for PAH exercise limitations; 6MWD improvement with structured exercise documented in pulmonary rehabilitation cohorts; exercise prescription records), and high-altitude travel restriction records (altitude >2,000 meters or air travel requiring supplemental oxygen — flight altitude oxygen supplementation prescription documentation) at 1-minute intervals during clinical hours.
Lung Transplant Evaluation and Listing
Monitor transplant referral trigger records (persistent NYHA/WHO FC III-IV despite maximally tolerated combination therapy; declining 6MWD below 350 meters; rising BNP/NT-proBNP without plateau; hemodynamic worsening on repeat RHC; history of intravenous prostanoid initiation — all established criteria for lung transplant referral to a certified transplant center), transplant evaluation records (bilateral lung transplantation preferred over single lung transplant for PAH — evaluation including cardiac catheterization, pulmonary function testing, 6MWD, echocardiogram, CT chest, renal and hepatic function, psychosocial evaluation, nutritional assessment), waitlist management records (UNOS listing records, LUNG Allocation Score calculation, waitlist position, bridging strategy on IV prostanoids while awaiting transplant), and post-transplant follow-up records (primary graft dysfunction risk assessment, chronic lung allograft dysfunction surveillance, immunosuppression management) at 1-minute intervals during clinical hours. Alert immediately — transplant coordination platform failures that delay waitlist listing documentation or LAS score submission for a patient who has been approved for bilateral lung transplantation may result in additional waitlist delay during a period when the patient's clinical status is actively deteriorating on maximum medical therapy.
Authentication and Clinical Identity
Monitor authentication at 1-minute intervals, 24/7. PAH management coordinates across pulmonary hypertension specialty centers (hemodynamic assessment and treatment strategy), cardiac catheterization laboratories (RHC and vasoreactivity testing), echocardiography and cardiac MRI (RV function surveillance), specialty pharmacy and compounding pharmacy (parenteral prostanoid supply chain), ERAs REMS programs, genetic testing and counseling, pulmonary rehabilitation, rheumatology and immunology (SSc-associated PAH), hepatology (portopulmonary hypertension), HIV medicine (HIV-associated PAH), lung transplant surgery (transplant evaluation and listing), and right heart failure intensive care (acute decompensation management) — authentication failures across this multi-specialty platform infrastructure disrupt the risk stratification-guided therapy escalation program that determines PAH survival.
SSL Certificates
Monitor SSL certificate expiry across all pulmonary hypertension specialty platforms, cardiac catheterization scheduling systems, biomarker reporting portals, specialty pharmacy platforms, ERA REMS systems, genetic testing portals, and lung transplant coordination systems. Certificate errors disrupt BNP result delivery, REMS compliance verification, and prostanoid infusion management workflows.
HIPAA and Genetic Information Privacy Considerations
PAH technology platforms handle GINA-protected genetic information (BMPR2 and other heritable PAH gene panel results with autosomal dominant inheritance and incomplete penetrance implications for family members), longitudinal hemodynamic records from serial right heart catheterization, parenteral prostanoid infusion records with implications for life insurance and disability adjudication, right ventricular function surveillance imaging, and lung transplant evaluation and waitlist records. BMPR2 mutation status with its 20% penetrance creates a complex genetic risk disclosure obligation for family members — the patient's children each have a 50% chance of inheriting the mutation but only a 20% penetrance risk of manifesting PAH, making genetic counseling about surveillance versus anxiety about a low but real lifetime risk a nuanced platform-mediated communication challenge.
ERA REMS program records (bosentan pregnancy tests and LFT results; ambrisentan risk records) are particularly sensitive for reproductive-age women, combining fertility and pregnancy status information with medical records in a single REMS compliance documentation stream requiring strict access controls. PAH patient registries (REVEAL registry, US PAH registry, Compera in Europe) hold individually identifiable hemodynamic and longitudinal clinical outcome data requiring IRB oversight, DUA agreements, and HIPAA-compliant data governance frameworks for secondary use.
Alerting Strategy for PAH Tech Platforms
Immediate 24/7 alerting for parenteral prostanoid infusion management platforms: Epoprostenol and treprostinil IV infusion management is life-sustaining therapy whose discontinuation can be rapidly fatal — these platforms require the highest-priority 24/7 alerting available.
Immediate clinical-hours alerting for BNP/NT-proBNP and 6MWD platforms: Rising BNP and declining 6MWD are the primary signals of PAH disease progression requiring therapy escalation — delayed results delivery directly delays escalation decisions.
Immediate catheterization laboratory-hours alerting for RHC scheduling and results platforms: RHC is the gold standard hemodynamic assessment that determines therapy escalation to parenteral agents — scheduling failures directly delay escalation decisions.
Immediate clinical-hours alerting for WHO functional class and combination therapy platforms: FC III-IV documentation and ERA REMS compliance verification are high-frequency clinical obligations requiring continuous platform availability.
Immediate clinical-hours alerting for echocardiography scheduling and results platforms: New pericardial effusion or TAPSE decline detected on echocardiography triggers urgent RHC and therapy escalation.
Immediate clinical-hours alerting for lung transplant evaluation and waitlist platforms: Transplant listing documentation and LAS score maintenance are clinically urgent for patients on maximum medical therapy with declining function.
Sustained-failure alert (10–15 minutes): Pulmonary rehabilitation records, anticoagulation monitoring, supplemental oxygen prescription management.
30-day advance warning: SSL certificates across all domains.
Status Page for PAH Care Team Communication
A real-time status page gives pulmonary hypertension specialists performing 6MWD testing and functional class assessments, cardiac catheterization laboratory teams performing right heart catheterization, echocardiographers and cardiac MRI teams performing right ventricular surveillance, specialty pharmacists compounding and dispensing parenteral prostanoids, ERA REMS program pharmacists, genetic counselors coordinating family BMPR2 cascade screening, pulmonary rehabilitation specialists, rheumatologists and HIV specialists managing associated forms, hepatologists managing portopulmonary hypertension, lung transplant surgeons and coordinators, and right heart failure intensivists managing acute decompensations immediate platform visibility without inbound IT support contact.
Include the status page URL in parenteral prostanoid infusion emergency protocols, right heart failure acute decompensation management procedures, and lung transplant waitlist management workflows.
Vigilmon Setup for PAH Tech Platforms
A practical starting configuration:
| Monitor | Check Interval | Alert Channel | |---------|----------------|---------------| | Authentication | 1 min | Slack + PagerDuty (24/7) | | BMPR2 / heritable PAH gene panel | 1 min | Slack + PagerDuty (lab hours) | | Right heart catheterization scheduling and results | 1 min | Slack + PagerDuty (cath lab hours) | | BNP / NT-proBNP biomarker reporting | 1 min | Slack + PagerDuty (clinical hours) | | Six-minute walk distance testing | 1 min | Slack + PagerDuty (clinical hours) | | WHO functional class documentation | 1 min | Slack + PagerDuty (clinical hours) | | REVEAL 2.0 risk score tracking | 1 min | Slack + PagerDuty (clinical hours) | | ERA (bosentan/ambrisentan/macitentan) prescribing | 1 min | Slack + PagerDuty (clinical hours) | | ERA REMS LFT compliance (bosentan) | 1 min | Slack + PagerDuty (pharmacy hours) | | PDE5i (sildenafil/tadalafil) prescribing | 1 min | Slack + PagerDuty (clinical hours) | | Parenteral prostanoid infusion management (IV epoprostenol/treprostinil) | 1 min | Slack + PagerDuty (24/7) | | Oral/inhaled prostanoid adherence (selexipag/iloprost/inhaled treprostinil) | 1 min | Slack + PagerDuty (clinical hours) | | Echocardiography scheduling and RV function results | 1 min | Slack + PagerDuty (clinical hours) | | Cardiac MRI right ventricular function | 1 min | Slack + PagerDuty (radiology hours) | | Anticoagulation (INR) monitoring | 1 min | Slack + PagerDuty (clinical hours) | | Supplemental oxygen and pulmonary rehab | 2 min | Slack (clinical hours) | | Lung transplant evaluation and waitlist management | 1 min | Slack + PagerDuty (clinical hours) | | Wearable activity monitoring | 2 min | Slack (business hours) | | Genetic counseling and family cascade screening | 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 BMPR2 and heritable PAH gene panel platforms with immediate laboratory-hours alerting
- Add right heart catheterization scheduling and results platforms with immediate catheterization laboratory-hours alerting — this is the diagnostic gold standard and primary treatment response assessment tool
- Configure BNP/NT-proBNP biomarker reporting with immediate clinical-hours and laboratory-hours alerting
- Add six-minute walk distance testing platforms with immediate clinical-hours alerting
- Configure WHO functional class documentation platforms with immediate clinical-hours alerting
- Add REVEAL 2.0 risk score calculation platforms with immediate clinical-hours alerting
- Configure ERA prescribing platforms with immediate clinical-hours alerting
- Add ERA REMS LFT compliance platforms with immediate pharmacy-hours alerting
- Configure PDE5i prescribing platforms with immediate clinical-hours alerting
- Add parenteral prostanoid infusion management platforms with 24/7 immediate alerting — this is the highest-acuity monitoring workflow for PAH patients on life-sustaining IV therapy
- Configure oral and inhaled prostanoid adherence tracking with immediate clinical-hours alerting
- Add echocardiography scheduling and RV function results platforms with immediate clinical-hours alerting
- Configure cardiac MRI right ventricular function platforms with immediate radiology-hours alerting
- Add anticoagulation INR monitoring platforms with immediate clinical-hours alerting
- Configure supplemental oxygen and pulmonary rehabilitation platforms with sustained-failure alerting
- Add lung transplant evaluation and waitlist management platforms with immediate clinical-hours alerting
- Configure wearable activity monitoring platforms with sustained-failure alerting
- Enable SSL certificate monitoring across all pulmonary hypertension, catheterization laboratory, biomarker reporting, REMS, specialty pharmacy, transplant, and genetics platforms with 30-day advance email warning
Conclusion
PAH technology platforms are embedded in clinical decisions where BNP reporting platform availability at 9:15 AM on the morning of a dedicated pulmonary hypertension clinic when the pulmonologist is preparing for a follow-up visit with a 38-year-old woman with IPAH who has been on dual oral therapy — ambrisentan 10 mg plus tadalafil 40 mg — for 14 months, and whose 6MWD has declined from 412 meters at the 6-month mark to 349 meters at the current visit, a 15% decline from peak that meets the threshold for high-risk functional deterioration — and the biomarker result from this morning's blood draw, currently pending in the laboratory information system, needs to be available in the clinic dashboard before the visit begins so that the team can determine whether the NT-proBNP has risen to match the 6MWD decline or remained stable, since the combination of declining 6MWD and rising NT-proBNP would confirm multi-parameter treatment failure mandating immediate parenteral prostanoid initiation while a stable NT-proBNP despite declining 6MWD would suggest deconditioning rather than hemodynamic progression and support a supervised exercise program addition rather than immediate therapy escalation — cannot be disrupted by laboratory reporting platform failures that prevent the NT-proBNP result from appearing in the clinic dashboard before the consultation begins; where parenteral prostanoid infusion management platform availability at 3:47 PM on a Tuesday when the PAH specialty nurse coordinator at the outpatient infusion center is escalating the epoprostenol dose for a 52-year-old man with WHO FC IV PAH who was admitted and started on IV epoprostenol 4 weeks ago at 2 ng/kg/min and has been uptitrating every 48-72 hours under a structured dose escalation protocol, and the infusion management platform must document the new dose, print the revised CADD pump programming instructions, generate the specialty pharmacy refill order at the new concentration, and update the patient's home nursing supervision schedule — all as linked workflow actions that the platform must complete without error because an epoprostenol dose escalation performed correctly but documented at the wrong concentration can result in catastrophic overdose symptoms (hypotension, flushing, jaw pain, syncope) when the pharmacy compounds at the escalated dose against an uncorrected pharmacy record — cannot be disrupted by infusion management platform failures that desynchronize the clinical dose record from the pharmacy compounding record during an active dose escalation; and where lung transplant coordination platform availability when the PAH transplant coordinator is submitting the updated Lung Allocation Score documentation for a 44-year-old woman with BMPR2-related heritable PAH who has been on IV epoprostenol for 7 months and is deteriorating despite maximal IV therapy, with a 6MWD that has declined from 187 meters at listing to 91 meters at the most recent assessment 3 weeks ago — and whose LAS score must be updated immediately with the most recent PFT, 6MWD, and oxygen requirement data to reflect the severity of her current clinical status, because the waitlist organ allocation algorithm uses the current LAS score to prioritize her position in the queue, and a 4-week delay in LAS update submission due to platform failure may result in her being offered organs at a lower priority level based on a 3-month-old LAS score that underrepresented her current clinical severity — cannot be disrupted by transplant coordination platform failures that prevent timely LAS documentation submission for a patient whose window for bilateral lung transplantation is actively narrowing. A BNP reporting platform that prevents a treatment escalation decision from being made at the right moment, a parenteral prostanoid infusion management platform that desynchronizes dose and pharmacy records during active escalation, a lung transplant LAS documentation system that allows a deteriorating patient's waitlist priority to lag her actual clinical status by weeks — these are not IT incidents. They are clinical disruptions in the management of a rare progressive disease where the risk stratification tools, parenteral prostanoid therapy precision, and transplant timing windows are all measured in weeks to months, and where platform failures at precisely the decision-critical moments can mean the difference between treatment escalation before irreversible right ventricular failure and treatment escalation that comes too late.
Uptime monitoring gives PAH tech teams the detection capability to identify failures within seconds, trigger immediate clinical downtime procedures, and demonstrate to pulmonary hypertension specialists managing 3-monthly risk stratification cycles, cardiac catheterization teams performing hemodynamic assessment, specialty pharmacists compounding parenteral prostanoids, echocardiographers tracking right ventricular function, ERA REMS program pharmacists verifying LFT compliance, genetic counselors coordinating BMPR2 family cascade screening, and lung transplant coordinators managing waitlist priority documentation that platform operational reliability matches the risk stratification surveillance precision, parenteral prostanoid therapy complexity, right heart failure monitoring urgency, and transplant timing sensitivity of modern PAH management.
Start monitoring your PAH 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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