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☆
HypertensionRegistry
Low-riskcriteria:
FunctionalclassIorII 6MWD>440m RAP<8mmHg Cardiac index ≥ 2.5
L/min/m
2
Meeting≥3criteria associatedwithfavorable5-y prognosis
Poorpredictors:
Connective tissue disease
HeritablePAH PortopulmonaryHTN Males>60y eGFR<60mL/min FunctionalclassIIIor
IV Systolic BP < 110
mmHg Pulse>92beats/min Hospitalization within
6mo. 6MWD<165m BNP>200pg/mL NT-proBNP > 1100
pg/mL Pericardialeffusion DLCO < 40%
predicted RAP>20mmHg
Favorablepredictors:
FunctionalclassI 6MWD>320m BNP < 50 pg/mL or
NT-proBNP < 300 pg/mL
PVR<5WoodUnits
RiskCategorybasedon weightedaggregate score
Poorpredictors:
eGFR<60mL/min FunctionalclassIIIor
IV Systolic BP < 110
mmHg Pulse>92beats/min 6MWD<165m BNP>180pg/mL
Favorablepredictors:
FunctionalclassI 6MWD>440m BNP<50pg/mL
Riskcategorybasedon weightedaggregate score
6MWD, six-minute walk distance; BNP, brain natriuretic peptide; BP, blood pressure; DLCO, diffusing capacity for
carbon monoxide; eGFR, estimated glomerular filtration rate; HTN, hypertension; NT-proBNP, N-terminal pro b-type
natriuretic peptide; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; RAP, right atrial
pressure.
FrenchRegistrymethodtalliesthenumberoflow-riskcharacteristics(includingNYHAfunction
classIorII,6MWdistance>440m,RAP<8mmHg,cardiacindex>2.5L/min/m2);≥3criteria
signifieslow-riskcategoryandpredictsimprovedsurvival.
26
Combination therapy regimens with medications from more than one class of therapy are the preferredapproach,evenfornewlydiagnosedpatients.
27
https://t.me/med1917
Becauseofthecomplexityofsometherapies,anindividual’scomorbidconditions,cognitive
abilities,andpsychosocialfactorsmustalsobeconsidered.
Close monitoring and regular risk assessment is needed as deterioration often occurs, requiring alternative/additionalmedicalandpossiblysurgicalintervention.
Diuretics, often in combination (e.g., loop diuretic + aldosterone antagonist), lessen right heart failureandsymptoms. Anticoagulation:
Chronicanticoagulationmayimprove survivalinIPAH,while benefitsinother PAHsubtypes are unclear.28,
29
Warfarinisdosedtotargetinternationalnormalizedratioof1.5–2.5.
21
Anticoagulanttherapyisnoturgentandbridgingtherapyisunnecessary.
Inotropes,suchasdobutamineandmilrinone,areusedinextremelydecompensatedstates.
SurgicalManagement
Lungtransplantationorheart–lungtransplantation:
For PAH patients who remain in advanced functional class III–IV despite maximal medical
therapy,whichusuallyincludesaparenteralprostanoid.
GroupIIIPHalsoimpactstimingoftransplantinparenchymallungdiseases. Because the RV recovers after isolated lung transplantation, heart–lung transplantation is usually reservedforcomplexcongenitalheartdefectsthatcannotberepaired.
Atrialseptostomy:
Palliativeright-to-leftintracardiacshuntcreatedpercutaneouslyincasesofsevererightheartfailure (i.e.,syncope,hepaticcongestion,prerenalazotemia)refractorytomedicaltherapy. Despite arterial oxyhemoglobin desaturation and hypoxemia, oxygen delivery increases from improvedLVfillingandcardiacoutput.
Septaldefectclosure:
Intracardiac defects with significant net left-to-right shunting can be closed percutaneously or surgically. Criteria forclosure are evolving and somepatients may be candidatesafter a period oftreatment withpulmonaryvasodilatortherapy.
30
Prognosis
The1-,3-,and5-yearsurvivalratesinPAHare85%,70%,and55%,respectively.31,
32
ObstructiveSleepApnea–HypopneaSyndrome
GENERALPRINCIPLES
Definition
Obstructivesleepapnea(OSA)isadisorderinwhichpatientsexperienceapneasorhypopneasbecause ofupperairwaynarrowing.Whenitisassociatedwithexcessivedaytimesomnolence,itisreferredtoas obstructivesleepapnea–hypopneasyndrome(OSAHS).
33
Classification
https://t.me/med1917
Apneasrepresentcompletecessationofairflow.
Obstructiveeventsareassociatedwithcontinuedrespiratoryeffort. Centraleventsareassociatedwithnorespiratoryeffort.
Hypopneasrepresentdiminishedairflowassociatedwithatleasta3%–4%oxygendesaturation.Respiratoryeffort–relatedarousals(RERAs)representchangesinairflowthatleadtoanarousal, butdonotmeetcriteriaforanapneaorhypopnea. Allrespiratoryeventsmustlastatleast10secondstobecounted.Apnea–hypopneaindex(AHI)isthenumberofapneasandhypopneasperhourofsleep.Respiratorydisturbanceindex(RDI)is thenumber ofapneas,hypopneas,andRERAsperhourof sleep.
Epidemiology
TheprevalenceofOSAHSinthegeneralpopulationisestimatedtobeabout4%,withmenbeingtwice aslikelyaswomentobeaffected.
34
ObesityisasignificantriskfactorforOSA.
34
Giventhesignificantincreaseintheprevalenceofobesitysincetheoriginalepidemiologicalstudieson OSA were performed, it is estimated that the currentprevalenceofmoderateOSAas defined byan AHI>15is13%inmenand6%inwomen.
35
Etiology
OSA:Narrowingoftheupperairwaybecauseofexcessivesoft-tissueorstructuralabnormalities. Centralsleepapnea:Disturbanceofcentralcontrolofrespirationduringsleep.
Pathophysiology
OSAoccursbecauseofnarrowingoftheupperairway,whichresultsindiminishedairfloworcessation ofairflowleadingtoarousalsthatfragmentsleep.
RiskFactors
RiskfactorsforOSAincludeobesity(bodymassindex[BMI]>30kg/m2),largeneckcircumference (>17informenand>16inforwomen),increasedsofttissueoftheposteriororopharynx(enlarged tonsils,macroglossia,orelongateduvula),andabnormaljawstructure(micrognathiaorretrognathia). Patientswithcomorbidconditionssuchascongestiveheartfailure,coronaryarterydisease,atrial fibrillation(AFib),difficult-to-controlhypertension,anddiabetesarealsomorelikelytohaveOSA.
32
Prevention
Weightloss Avoidingsedativessuchashypnoticmedicationsoralcohol
AssociatedConditions
Cardiovascular disease, including systemic hypertension, heart failure, arrhythmia, myocardial infarction,andstroke.36OSAhasbeenestablishedasanindependentriskfactorforhypertension.
37
Increasedriskofdeathinmoderate-to-severeOSA,mainlybecauseofcardiovascularevents.38,
39
IncreasedprevalenceofdiabeteshasbeennotedinpatientswithOSAHS,independentoftheeffectof obesity.
40
https://t.me/med1917
Thereisapproximatelya2.5-foldincreasedriskofmotorvehicleaccidents (MVA)inpatientswith OSAwhencomparedwiththosewithoutOSA.However,compliancewithcontinuouspositiveairway pressure(CPAP)treatmentcansignificantlyreducetheriskofMVAinpatientswithOSA.
41
DIAGNOSIS
ClinicalPresentation
HISTORY
HabitualloudsnoringisthemostcommonsymptomofOSA,althoughnotallpeoplewhosnorehave thissyndrome.PatientswithOSAmayexperiencesnorearousalsalongwithasensationofgaspingor choking. Excessive daytime sleepiness (hypersomnolence) is a classic symptom of OSAHS (Table 10-4). Patientsmaydescribefallingasleepwhiledrivingorhavingdifficultyconcentratingatwork.
TABLE10-4
SYMPTOMS ASSOCIATED WITH OBSTRUCTIVE SLEEP APNEA–HYPOPNEA SYNDROME
Excessivedaytimesleepiness Snoring Nocturnalarousals Nocturnalapneas Nocturnalgasping,grunting,andchoking Nocturia Enuresis Awakeningwithoutfeelingrefreshed Morningheadaches Impairedmemoryandconcentration Irritabilityanddepression Impotence
Patients may also complain of personality changes, intellectual deterioration, morning headaches, nocturnalangina,lossoflibido,andchronicfatigue.
PHYSICALEXAMINATION
All patients should have a thorough nose andthroat examination to detect sources of upper airway obstruction. IncreasedseverityofOSAhasbeenassociatedwithahigherMallampaticlass(Table10-5).
42
TABLE10-5
MALLAMPATIAIRWAYCLASSIFICATION
Class VisibleStructuresWithMouthMaximallyOpenedandTongueProtruded
https://t.me/med1917
I Hardpalate,softpalate,uvula,tonsillarpillars
II Hardpalate,softpalate,uvula
III Hardpalate,softpalate,baseofuvula
IV Hardpalate
AdaptedfromMallampatiSR,GattSP,GuginoLD,etal.Aclinicalsigntopredictdifficulttrachealintubation:aprospectivestudy.
CanAnaesthSocJ.1985;32:429-434.
DIAGNOSTICCRITERIA
Apolysomnogram(PSG)demonstratingobstructiveeventswithanAHIorRDI>5isdiagnosticofOSA. IftheRDIisbetween5and15,apatientwillqualifyforpositiveairwaypressure(PAP)ifthereisa comorbidconditionsuchashypertension,coronaryarterydisease,depression,orhypersomnolence.If therearenocomorbidconditions,thenthepatientwillqualifyforPAPiftheRDIis>15.
DIFFERENTIALDIAGNOSIS
In addition to OSAHS and sleep-related hypoventilation, the differential diagnosis for daytime sleepinessincludes sleep deprivation,periodiclimb movement disorder,narcolepsy,andmedication sideeffects. Patientsshouldalsobeevaluatedforothermedicalconditionsthatmaycausenighttimeawakeningsand dyspnea and thus mimic OSA, such as chronic lung disease, congestive heart failure, and gastroesophagealrefluxdisease(GERD).
DiagnosticTesting
Thegold standardfor thediagnosis ofOSA isovernightPSGwith directobservationbyaqualified technician.43Sleepstudiesaretypicallyperformedintheoutpatientsetting. Typical indications for a sleep study include snoring with excessive daytime sleepiness, titrationof optimalPAPtherapy,andassessmentofobjectiveresponsetotherapeuticinterventions. PSG involves determination of sleep stages using electroencephalography, electromyography, and electrooculographyandassessmentofrespiratoryairflowandeffort,oxyhemoglobinsaturation,cardiac electricalactivity(e.g.,ECG),andbodyposition.TranscutaneousCO2canbemonitoredtoassessfor
hypoventilationintheappropriateclinicalsetting. Dataareanalyzedforsleepstaging,thefrequencyofrespiratoryevents,limbmovements,andabnormal behaviors.Respiratoryeventsarecategorizedasobstructiveorcentral. Most sleep studies are performed as “split studies,” where the first few hours of the study are diagnosticandthelatterpartofthestudyisusedforPAPtitration.PertheAmericanAcademyofSleep Medicineguidelines,PAPshouldbestartediftheAHI/RDIduringthediagnosticportionofthenightis ≥40,butthe thresholdforstartingPAP canbe loweredtoanAHI/RDIof≥20ifthere aresignificant comorbidconditions. Some patientsonlyhave significanteventswhenlyingincertainpositions(usuallysupine)orduring rapideyemovementsleep.Thesepatientsmayrequireacompleteovernightstudyfordiagnosisanda secondstudyforinitiationoftherapy. TheAmericanAcademyofSleep Medicinesupportstheuseofunattendedportable monitoringasan alternative to PSG for patients with a high pretest probability of moderate to severe OSA without significantcomorbidmedicalconditionsorothersuspectedsleepdisorders.
https://t.me/med1917
The portable device must record airflow, respiratory effort, and blood oxygenation. A sleep specialistshouldreviewtheresults. Portable devices canunderestimate theseverityofOSAbecausethenumberof events perhour is calculated using total recording time rather than total sleep time. If the portable sleep study is inconclusive,strongconsiderationshouldbegiventoperforminganin-labPSG.
43
TREATMENT
ThetherapeuticapproachtoOSAdependsontheseverityofthedisease,comorbidmedicalconditions, patientpreference,andexpectedcompliance.Treatmentmustbehighlyindividualized,withspecial attentionpaidtocorrectingpotentiallyreversibleexacerbatingfactors.
Medications
No pharmacologic agent has sufficient efficacy to warrant replacement of PAP as the primary therapeuticmodalityforOSAHS. Stimulant pharmacotherapy with modafinil or armodafinil may improve objective and subjective daytimesleepinessinpatientswithpersistentsymptomsdespiteadequatePAPuse.
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Medical treatment of conditions that may contribute to muscle hypotonia or weight gain, such as hypothyroidism,isofbenefit.
NonpharmacologicTherapies
PAP:
CPAPdeliversairviaafacemaskataconstantpressurethroughouttherespiratorycyclewiththe goal of pneumatically splinting open the upper airway, thus preventing collapse and airflow obstruction. The PAP titration determines the PAP (expressed in cm H2O) required to optimize airflow. The
pressuresettingisgraduallyincreaseduntilobstructiveevents,snoring,andoxygendesaturationsare minimized. The benefits of PAP include consolidated sleep and decreased daytime sleepiness. Hypertension, nocturia,peripheraledema,polycythemia,andPHmayalsoimprove. CPAP is a highly cost-effective intervention.45 The impact of CPAP treatment on associated cardiovascular comorbidities is variable with some studies showing improvement46 and others showing no improvement.47 Treatment of OSA results in a higher AFib-free survival rate after pulmonaryveinisolation.
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NasalCPAP(nCPAP)isthecurrenttreatmentofchoiceformostpatientswithOSAHS.
ThecomplianceratewithnCPAPisapproximately50%.
Compliancecanbeimprovedwitheducation,instruction,follow-up,adjustmentofthemaskforfit
and comfort, humidification of the air to decrease dryness, and treatment of nasal or sinus
symptoms.
Useofafull-facemask(oronasal)hasnotbeenshowntoimprovecompliancecomparedwiththe
useofnCPAP.49However,full-facemasksarefrequentlyusedinpatientswho“mouthbreathe”or
patientswhorequirehigherCPAP pressuresbecause theywill often experienceair leakthrough
themouthwhenusingnCPAP. Autotitratingpositiveairwaypressure(APAP)machinesuseflowandpressuretransducerstosense airflow patterns andthen automaticallyadjustthepressuresettinginresponse.Smallstudies have shownthatAPAPmaybeaseffectiveastraditionalCPAPandappearstobepreferredbypatients.50
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BilevelPAPistypicallyusedtotreatOSAinthefollowingsettings:pressures>15–20cmandH2O arerequired,intoleranceofCPAP,orconcernforconcomitanthypoventilation.
All positive pressure devices may induce dryness of the airway, nasal congestion, rhinorrhea, epistaxis,skinreactionstothemask,nasal bridgeabrasions,andaerophagia.Some of these nasal symptomsmaybetreatedwithnasalsaline,decongestants,anduseofahumidifier. Some patients, such as those with coexisting chronic obstructive pulmonary disease, require supplementaloxygentomaintainadequatenocturnaloxygensaturations(SaO2≥90%).
Oralappliances:
UsedformildOSAHS,withtheaimtoincreaseairwaysizetoimproveairflow.Thesedevices,such as the mandibular advancement device, can be fixed or adjustable, and most require customized fitting.Manydeviceshavenotbeenwellstudied. Contraindicationsincludetemporomandibularjointdisease,bruxism,full dentures,andinabilityto protrudethemandible.
Upperairwaystimulationdevice:
A hypoglossal nervestimulator to improve tongue protrusionis approved for useinpatients with moderatetosevereOSAwhocannottolerateCPAP.Useofthestimulatorislimitedtopatientswitha BMI<32kg/m2.
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AlthoughAHIanddaytimesleepinessimprovedwiththisdevice,therewasresidualmildOSA.
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SurgicalManagement
Tracheostomy:
TracheostomyisveryeffectiveintreatingOSAHSbutisrarelyusedsincetheadventofPAPtherapy. Tracheostomy should be reserved for patients with life-threatening disease (cor pulmonale, arrhythmias,or severehypoxemia) orsignificantalveolarhypoventilationthatcannot becontrolled withothermeasures.
Uvulopalatopharyngoplasty(UPPP):
UPPPis themost commonsurgical treatment of mild tomoderateOSAHSinpatients whodo not respondtomedicaltherapy. UPPPenlargestheairwaybyremovingtissuefromthetonsils,tonsillarpillars,uvula,andposterior palate. UPPP may be complicated by change in voice, nasopharyngeal stenosis, foreign body sensation, velopharyngeal insufficiencywith associatednasalregurgitationduring swallowing, and PAPtoleranceproblems. ThesuccessrateofUPPPforthetreatmentofOSAHSisonlyapproximately50%,whendefinedasa 50% reduction of the AHI, and improvements related to UPPP may diminish over time.54 Thus, UPPPisconsideredasecond-linetreatmentforpatientswithmildtomoderateOSAHSwhocannot successfullyusePAPandwhohaveretropalatalobstruction.
Stagedprocedures:
In experiencedcenters, other stagedprocedures for OSA can be performed, including mandibular osteotomywithgenioglossusadvancement,hyoidmyotomywithsuspension,andmaxillomandibular advancement (MMA).53 Significant reductions in AHI have been reported with MMA, but more researchisneeded.
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Lifestyle/RiskModification
Weightloss,bothsurgicalandthroughreducedcaloricintake,hasbeenshowntoreducetheseverityof OSAbyreductioninAHI.56,
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OSAHSpatientsshouldavoiduseofalcohol,tobacco,andsedatives. CliniciansshouldcounselpatientswithOSAHSregardingtheincreasedriskofdrivingandoperating dangerousequipment.
SPECIALCONSIDERATIONS
PatientswithaBMI>40kg/m2areatincreasedriskforconcomitantsleep-relatedhypoventilation becauseofmorbidobesity.
Complications
Patients with OSAHS are at greater risk for perioperative complications because of intubation difficultyand/orimpairedarousalsecondarytotheeffectsofanesthetics,narcotics,andsedatives.
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Theriskofdeath,hypertension,andpoorneuropsychologicalfunctioningriseswithincreasingseverity ofOSA.
Referral
PatientswithriskfactorsandsymptomsorsequelaeofOSAHSshouldbereferredtoasleepspecialist andsleeplaboratoryforfurtherevaluation.
InterstitialLungDisease
GENERALPRINCIPLES
Definition
ILDsareaheterogeneousgroupof>200disorderscharacterizedbyinfiltrationofthelunginterstitiumby cells,fluid,and/orconnectivetissue. ILDscanpresentacutelyorchronically,andtheyareoftendiagnosedusingamultidisciplinaryapproach employingpulmonaryclinicians,radiologists,andpathologists.
Classification
ILDscanbebroadlyclassifiedintothosewithknowncausesandthosewithout(idiopathic).
Idiopathicinterstitialpneumonias:
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Idiopathicpulmonaryfibrosis(IPF)(idiopathicusualinterstitialpneumonia[UIP])
Idiopathicnonspecificinterstitialpneumonia(NSIP)
Desquamativeinterstitialpneumonia(DIP)
Respiratorybronchiolitis–associatedinterstitiallungdisease(RB-ILD)
Cryptogenicorganizingpneumonia(COP)(idiopathicOP)
Acuteinterstitialpneumonia(AIP)
Lymphoidinterstitialpneumonia(LIP)(rare)
Idiopathicpleuroparenchymalfibroelastosis(rare) Medication/therapyinduced:
Bleomycin
Amiodarone
Nitrofurantoin
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Checkpointinhibitors
NSAIDs
Thalidomide
Rituximab
Azathioprine
Methotrexate
Radiationtherapy CTD-ILD:
Rheumatoidarthritis
Scleroderma
Sjögrensyndrome
Antisynthetasesyndrome
MixedCTD
Systemiclupuserythematosus Vasculitides:
Granulomatosiswithpolyangiitis
Eosinophilicgranulomatosiswithpolyangiitis
Microscopicpolyangiitis
Goodpasturesyndrome Pneumoconiosis(diseasesofthelungduetodustinhalation):
Coalminers’pneumoconiosis
Asbestosis
Silicosis
Siderosis
Stannosis
Mixeddustpneumoconiosis GranulomatousILD:
Sarcoidosis
Berylliosis
Hypersensitivitypneumonitis(HP)
Granulomatous–lymphocyticinterstitiallungdisease
Bronchocentricgranulomatosis Cysticlungdiseases:
Lymphangioleiomyomatosis(LAM)
PulmonaryLangerhanscellhistiocytosis(PLCH)
Birt–Hogg–Dubé(BHD)syndrome
Pulmonaryamyloidosis
Lightchaindepositiondisease
Postinfectious Miscellaneous:
Erdheim–Chesterdisease
Pulmonaryalveolarproteinosis
Lipoidpneumonia
Pulmonaryalveolarmicrolithiasis
Acuteeosinophilicpneumonia
Chroniceosinophilicpneumonia
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ClinicalPresentation
HISTORY
Obtaininga thoroughhistoryis ofparamount importanceinpatients presenting withILDandisoften crucialinmakingadiagnosis. Patientsmostoftenpresentwithprogressivedyspneaandpersistentdrycough. Duration of symptoms may help in differentiating ILDs. While many ILDs present with years of progressive dyspnea and cough, a subset of ILD patients present with acute or subacute onset of symptoms (AIP, acute eosinophilic pneumonia, OP), which may mimic infectious pneumonias with atypicalorganisms. Pastmedicalhistoryisveryimportant,notonlyforunderlyingdiseasesbutalsotoidentifyILDsrelated todisease management. Examples include CTDs andimmunosuppressive agents;cancers along with chemo-,immuno-,andradiotherapies;andothersystemicdiseasesthatcanpotentiallyaffectthelungs such as inflammatory bowel disease. It is important not to forget the use of over-the-counter medications. Documenting a smoking history is essential. Some ILDs manifest almost exclusively in smokers (Langerhanscellhistiocytosis,DIP,andRB-ILD).Somediseasesarestronglyassociatedwithcurrent or previous tobacco use, for example, IPF. Pulmonary hemorrhage is far more common in active smokerswithGoodpasturediseasethaninpriorornonsmokers. Exposuresboth athome andintheworkplace shouldbe evaluated. Thesemay includeexposures to radiation, asbestos, metal dusts, wood dusts, chemicals or fumes, pets, moldy environments, down comfortersand/orpillows,andmore.Patientsshouldbequestionedregardingthedegreeandduration oftheirexposures,andtheuseofrespiratoryprotectiveequipment. Family history should be obtained, specifically as it relates to pulmonaryfibrosis, lung disease, or autoimmunedisease. Multiple inheritancepatternshavebeendescribedwithILDsincluding complex (sarcoidosis), autosomal dominant(tuberoussclerosis),andautosomalrecessive (Hermansky–Pudlak syndrome).
PHYSICALEXAMINATION
ExtrapulmonaryexaminationinpatientswithILDshouldpayparticularattentiontofindingsofsystemic diseasesthatmayaffectthelungs.TheseincludeCTDs,sarcoidosis,tuberoussclerosis,andothers.
Examples includesclerodactyly, mechanic’shands,Raynaudphenomenon,drymucousmembranes, telangiectasias,skinrashes,facialerythema,papules,eczema,orotherskinlesions.
CardiacexaminationshouldfocusonfindingssuggestingthepresenceofPH/corpulmonaleincludinga rightventricular heave,pulmonaryarterytap,tricuspidregurgitationholosystolic murmur,right-sided S3,andperipheraledema.Thesefindingsareusuallyindicativeofadvancedlungdisease. Clubbingisaverynonspecificfindingdescribedinlungdiseases,heartdiseases,andgastrointestinal (GI)diseases.ItcanbeseeninIPF,sarcoidosis,PLCH,andotherILDs. The pulmonary examination in ILDs is nonspecific. Findings may include dry inspiratory crackles, whichare bestnotedposteriorlynear thelungbases. Wheezes andinspiratorysqueaks mayalso be noted.
DiagnosticTesting
DiagnostictestingintheevaluationofILDstypicallyinvolves:
ChestimagingincludingCXRandhigh-resolutionCT(HRCT)ofthechest
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