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Pulmonary HypertensionPulmonary HypertensionHeart Disease (Braunward)Heart Disease (Braunward)
Presenter: Cheng-Han LeePresenter: Cheng-Han Lee
Supervisor: Ting-Hsing ChaoSupervisor: Ting-Hsing Chao
Normal Pulmonary circulationNormal Pulmonary circulation
Dual vessels supply and drainage : pulmonaDual vessels supply and drainage : pulmonary a. and bronchial a.ry a. and bronchial a.
pulmonary v. and azygos v.pulmonary v. and azygos v. Pul. a.Pul. a.arteriolesarteriolescapillariescapillariespul. v.pul. v. Bronchial a.Bronchial a.capillariescapillariespul.v.pul.v. azygos veinazygos vein Bronchial circulationBronchial circulationphysiological right to lphysiological right to l
eft shunteft shunt
Normal Pulmonary circulationNormal Pulmonary circulation
Normal pul. artery pressure:Normal pul. artery pressure:
systolic: 18 to 25mmHgsystolic: 18 to 25mmHg
diastolic: 6 to 10 mmHgdiastolic: 6 to 10 mmHg
mean: 12 to16 mmHgmean: 12 to16 mmHg PG of pul. circulation: 2-10 mmHgPG of pul. circulation: 2-10 mmHg PG of systemic circulation: 90 mmHgPG of systemic circulation: 90 mmHg Pul. vascular resistence: 67Pul. vascular resistence: 67±23 dyn sec.cm (aroun±23 dyn sec.cm (aroun
d 1/10 of systemic vascular resistence).d 1/10 of systemic vascular resistence).
Normal Pulmonary circulationNormal Pulmonary circulation
ExerciseExercise PVR decreased, pul.flow and pul. PVR decreased, pul.flow and pul.a pressure increased a pressure increased
AgingAging PA pressure and PVR increased PA pressure and PVR increased
mild pul. HTN in 45 y/o mild pul. HTN in 45 y/o 13% 13%
75 y/o 75 y/o 28% 28%
Normal Pulmonary circulationNormal Pulmonary circulationResponse to hypoxia, Drugs, and neural and Response to hypoxia, Drugs, and neural and
environmental factorsenvironmental factors Hypoxia (Hypoxia (PaO2PaO2≤ 55 mmHg≤ 55 mmHg)) pul. vasoconstrictio pul. vasoconstrictio
n (K, Ca channel)n (K, Ca channel)
Neural regulationNeural regulation less certain ( less certain (αα1 receptor1 receptors & norepinephrine)s & norepinephrine)
Normal Pulmonary HypertensionNormal Pulmonary HypertensionResponse to Hypoxia, Drugs, and Neural and Response to Hypoxia, Drugs, and Neural and
Environmental FactorsEnvironmental Factors
DrugsDrugsProstaglandinsProstaglandins : PGI2 and PGE2(vasodilator) : PGI2 and PGE2(vasodilator) PGA2 and PGF2(vasoconstrictor)PGA2 and PGF2(vasoconstrictor)NONO: vasodilator&inhibit platelet aggregation: vasodilator&inhibit platelet aggregationEndothelinEndothelin: vasoconstrictor: vasoconstrictorSerotoninSerotonin: enhance release NO, medial hypertrophy : enhance release NO, medial hypertrophy and vascular remodelingand vascular remodelingAngiotensin IIAngiotensin II: vasoconstrictor, cell proliferation, cell : vasoconstrictor, cell proliferation, cell
migration and extracellular matrix synthesismigration and extracellular matrix synthesis
Normal Pulmonary HypertensionNormal Pulmonary HypertensionResponse to Hypoxia, Drugs, and Neural and Response to Hypoxia, Drugs, and Neural and
Environmental FactorsEnvironmental Factors
PGI2 and NOPGI2 and NO
Inhibit Platelet
Relax smooth muscle
EndothelinEndothelin
Primary Pulmonary Hypertension Primary Pulmonary Hypertension (PPH)(PPH)
Definite Pul. HTN:Definite Pul. HTN: systolic: larger than 30 mmHgsystolic: larger than 30 mmHg mean: larger than 20 mmHgmean: larger than 20 mmHg NIH definitionNIH definition:: mean PA >25mmHg at restmean PA >25mmHg at rest mean PA >30mmHg at exercisemean PA >30mmHg at exercise Require careful exclusion of secondary causesRequire careful exclusion of secondary causes Endothelial dysfunctionEndothelial dysfunction Marked vasoconstrictive tendency Marked vasoconstrictive tendency Reduced expression of NO synthaseReduced expression of NO synthase Increased level of endothelinIncreased level of endothelin Intimal proliferationIntimal proliferation
Possible pathogenesis of PPHPossible pathogenesis of PPH
Possible pathogenesis of PPHPossible pathogenesis of PPH
Possible pathogenesis of PPHPossible pathogenesis of PPH
GeneticsGenetics
Variability about response of Variability about response of pulmonary vessel to stimuli like pulmonary vessel to stimuli like acidosis or hypoxiaacidosis or hypoxia
Familial PPHFamilial PPH
6% of PPH6% of PPH Similar F/M ratio, age of onset, natural historSimilar F/M ratio, age of onset, natural histor
y y Trinucleotide repeat expansionTrinucleotide repeat expansion 2q31,332q31,33
Risk FactorsRisk Factors
Portal hypertensionPortal hypertension AnorexigensAnorexigens HIV infectionHIV infection Systemic HTNSystemic HTN Increased pulmonary blood flowIncreased pulmonary blood flow
Portal HypertensionPortal Hypertension
Portosystemic shuntPortosystemic shunt increased vasoactive increased vasoactive mediators like serotonin& IL1&endothelial grmediators like serotonin& IL1&endothelial growth factorowth factor vascular remodeling vascular remodeling
AnorexigensAnorexigens
In 1981, Appetite suppressants (fenfluraminIn 1981, Appetite suppressants (fenfluramine or amphetamine-like drugs)e or amphetamine-like drugs) inhibit volta inhibit voltage-gated K channelge-gated K channel vasoconstriction vasoconstriction
HIV infectionHIV infection
Possibility mediated by release of Possibility mediated by release of inflammatory cytokines or by autoimmune inflammatory cytokines or by autoimmune mechanismmechanism
Antiviral drugs seemed to retard the Antiviral drugs seemed to retard the progression of pulmonary HTNprogression of pulmonary HTN
Systemic HTNSystemic HTN
Neurohumoral factorsNeurohumoral factors Pulmonary vascular seems to be overactive Pulmonary vascular seems to be overactive
to sympathetic stimulationto sympathetic stimulation In some patients, the mechanism which incrIn some patients, the mechanism which incr
eases SVR also increases PVReases SVR also increases PVR
Increased pulmonary blood flowIncreased pulmonary blood flow
Increase blood flow will induce disturbance Increase blood flow will induce disturbance of pulmonary endotheliumof pulmonary endothelium
Hyperthyroidism and beriberi Hyperthyroidism and beriberi unexplained unexplained pulmonary HTNpulmonary HTN
PPHPPHPathologic findingsPathologic findings
ArteriopathyArteriopathy
Isolated medial hypertrophyIsolated medial hypertrophy Medial hypertrophy: increase of medial muscle in Medial hypertrophy: increase of medial muscle in muscular arteries, muscularization of nonmusculamuscular arteries, muscularization of nonmuscularized arterioles; no appreciable intimal or luminal rized arterioles; no appreciable intimal or luminal obstructive lesions. No plexiform lesionsobstructive lesions. No plexiform lesions
Plexogenic pulmonary arteriopaPlexogenic pulmonary arteriopathythy
Plexiform and dilatation lesions. Medial hypertropPlexiform and dilatation lesions. Medial hypertrophy; eccentric or concentric-laminar and nonlaminahy; eccentric or concentric-laminar and nonlaminar intimal thickening; fibrinoid necrosis, arteritis, anr intimal thickening; fibrinoid necrosis, arteritis, and thrombotic lesionsd thrombotic lesions
Thrombotic pulmonary arteriopThrombotic pulmonary arteriopathyathy
Thrombi (fresh, organizing, or organized and colanThrombi (fresh, organizing, or organized and colander lesions). Eccentric and concentric nonlaminar der lesions). Eccentric and concentric nonlaminar intimal thickening, varying degrees of medial hypeintimal thickening, varying degrees of medial hypertrophy. No plexiform lesionsrtrophy. No plexiform lesions
Isolated pulmonary arteritisIsolated pulmonary arteritis Active or healed arteritis. Limited to pulmonary artActive or healed arteritis. Limited to pulmonary arteries; varying degrees of medial hypertrophy, intieries; varying degrees of medial hypertrophy, intimal fibrosis, and thrombotic lesions. No plexiform mal fibrosis, and thrombotic lesions. No plexiform lesions. No systemic arteritislesions. No systemic arteritis
PPHPPHPathologic findingsPathologic findings
VenopathyVenopathy
Pulmonary venoocclusive diPulmonary venoocclusive diseasesease
Eccentric intimal fibrosis and recanalized tEccentric intimal fibrosis and recanalized thrombi within pulmonary veins and venulehrombi within pulmonary veins and venules; arterialized veins, capillary congestion, as; arterialized veins, capillary congestion, alveolar edema and siderophages, dilated lylveolar edema and siderophages, dilated lymphatics, pleural and septal edema, and armphatics, pleural and septal edema, and arterial medial hypertrophy; intimal thickeninterial medial hypertrophy; intimal thickening and thrombotic lesionsg and thrombotic lesions
MicroangiopathyMicroangiopathy
Pulmonary capillary hemangPulmonary capillary hemangiomatosisiomatosis
Infiltrating thin-walled blood vessels throuInfiltrating thin-walled blood vessels throughout pulmonary parenchyma, pleura, broghout pulmonary parenchyma, pleura, bronchi, and walls of pulmonary veins and artnchi, and walls of pulmonary veins and arteries. Medial hypertrophy and intimal thickeries. Medial hypertrophy and intimal thickening of muscular pulmonary arteries and ening of muscular pulmonary arteries and arteriolesarterioles
PPHPPHClinical featuresClinical features
Natural history Natural history
NIH Registry on PPH (1981~1987)—194 patientsNIH Registry on PPH (1981~1987)—194 patients
a. 63% femalea. 63% female
b. mean age: 36b. mean age: 36±15 y/o at the time of diagnosis±15 y/o at the time of diagnosis
c. Time from onset to diagnosis: 2 yearsc. Time from onset to diagnosis: 2 years
d. initial presentation: dyspnea(80%), fatugue(19%), d. initial presentation: dyspnea(80%), fatugue(19%),
syncope or near-syncope(13%), Raynaud syncope or near-syncope(13%), Raynaud
phenomenon(10%)phenomenon(10%)
PPHPPHClinical featuresClinical features
NIH Registry on PPHNIH Registry on PPH PE findingsPE findings
P2 accentuation(93%)P2 accentuation(93%)
TR-related murmur(40%)TR-related murmur(40%)
peripheral edema(32%)peripheral edema(32%) Lab findings:Lab findings:
ECG: RVH pattern(87%)ECG: RVH pattern(87%)
chest x-ray: enlarged main PAchest x-ray: enlarged main PA
PPHPPHClinical featuresClinical features
NIH Registry on PPHNIH Registry on PPH
Lung function: restrictiveLung function: restrictive
DLCO: reducedDLCO: reduced
Mild to moderate hypoxemia(mean PaO2=72±16 mmHMild to moderate hypoxemia(mean PaO2=72±16 mmHg)g)
Hemodynamic fingings: rising RA pressure, reduced cHemodynamic fingings: rising RA pressure, reduced cardiac outputardiac output reflect RV function reflect RV function
mean PA pressure rises early in the coursemean PA pressure rises early in the course
PPHPPHClinical featuresClinical features
NIH Registry on PPHNIH Registry on PPHUnivariate analysisUnivariate analysis Mean RA, PA pressure, cardiac index and DLCO Mean RA, PA pressure, cardiac index and DLCO
significantly related to mortalitysignificantly related to mortality
NYHA class NYHA class strongly related to survival strongly related to survival
The most common cause of deathThe most common cause of death RV failure RV failure
Sudden cardiac death: 26% Sudden cardiac death: 26% limited to NYHA class IV limited to NYHA class IV
Mechanisms of RV failureMechanisms of RV failure 1. Pressure and volume overload1. Pressure and volume overload
2. Animal study: pressure 2. Animal study: pressure overloadoverload reduced RCA reduced RCA perfusionperfusion RV ischemia RV ischemia
3. Shared IVS can affect both ventricle3. Shared IVS can affect both ventricle
4. Left main coronary A. was compressed by PA4. Left main coronary A. was compressed by PA
Physical ExaminationPhysical Examination
Large a wave in the jugular venous pulseLarge a wave in the jugular venous pulse RV heaveRV heave Systolic pulsation in left 2nd ICS (dilated ,tense PSystolic pulsation in left 2nd ICS (dilated ,tense P
A)A) P2 accentuation and S4 gallopP2 accentuation and S4 gallop RV failure signsRV failure signs hepatomegaly, ascites and pero hepatomegaly, ascites and pero
pheral edema)pheral edema) Cyanosis in the late courseCyanosis in the late course Uncommonly, laryngeal nerve was compressed by Uncommonly, laryngeal nerve was compressed by
PA (Ortner syndrome)PA (Ortner syndrome)
Laboratory findingsLaboratory findings
CBC and BCS: CBC and BCS: hypoxemia with polycythemia, hypoxemia with polycythemia,
abnormal liver function abnormal liver function
some reports: abnormal platelet function, some reports: abnormal platelet function,
hypercoagulopathy, defect in fibrinolysishypercoagulopathy, defect in fibrinolysis ECG: RA and RV enlargement patternECG: RA and RV enlargement pattern Chest x-ray: RA, RV and main PA, its major branch enlargeChest x-ray: RA, RV and main PA, its major branch enlarge
ment with tapering of peripheral vesselsment with tapering of peripheral vessels Cardiac echo: RA and RV enlargement with TR and pulmoCardiac echo: RA and RV enlargement with TR and pulmo
nary HTN, thick IVS, systolic prolapse of MV nary HTN, thick IVS, systolic prolapse of MV
Lung ScintigraphyLung Scintigraphy Perfusion scan helps diffentiate PPH and pulmonPerfusion scan helps diffentiate PPH and pulmon
ary HTN secondary to chronic pulmonary thromboary HTN secondary to chronic pulmonary thromboembolismembolism
Pulmonary angiographyPulmonary angiography
Typically, large central PA with marked peripTypically, large central PA with marked peripheral taperingheral tapering
The procedure increased the riskThe procedure increased the risk
adequate O2adequate O2
avoid vasovagal reactionsavoid vasovagal reactions
non-ionic contrastnon-ionic contrast
OthersOthers
Chest CT: central pulmonary embolism, Chest CT: central pulmonary embolism, caliber of PAcaliber of PA
Exercise testing: objective assessment of Exercise testing: objective assessment of severity and predictive of survival and drug severity and predictive of survival and drug efficacyefficacy
Cardiac catheterizationCardiac catheterization
CONDITION TEST APPLIED FINDING
Congenital heart disease Step-up in O2 saturation in right heart
Step-down in O2 saturation in left heart
Cardiac angiography
Left-to-right shunt and location of shuntRight-to-left shunt and location of shuntAnatomical definition
Peripheral pulmonary artery stenoses
Intrapulmonary arterial pressurePulmonary angiogram
Intrapulmonary arterial pressure gradientsPulmonary arterial branch stenoses
Proximal pulmonary arterial occlusion by clot or tumor
Selective or main pulmonary angiography
Intravascular filling defect or narrowing, webs, poststenotic dilatation
Mitral stenosisCor triatriatumSupravalvular mitral ring
Simultaneous wedge and left ventricular pressure recording
An elevated wedge pressure and mean mitral valve diastolic pressure gradient >3 mm Hg at rest, both of which increase with exercise
Mitral regurgitation Simultaneous wedge and left ventricular pressure recordingLeft ventriculogram
Large systolic pressure wave in wedge tracing. Regurgitation of contrast from left ventricular angiogram into the left atrium
Left ventricular diastolic dysfunctionRestrictive cardiomyopathy
Left ventricular pressureRight ventricular pressure
LVEDP >15 mm HgLVEDP response to intravenous fluid challenge: normalization of LVEDP with marked reduction in pulmonary artery pressure with intravenous nitroprusside
WHO 1998 diagnostic classification of pulmonary hypertension
DiagnosisDiagnosisPOTENTIAL CAUSE OF
PULMONARY HYPERTENSION DIAGNOSTIC STUDIESPulmonary thromboembolic disease Ventilation/perfusion scans, computed tomography of
chest, pulmonary angiography
Pulmonary venous thrombosis or obstruction Chest x-ray, angiography, computed tomography, magnetic resonance imaging
Congenital intracardiac shunts Transesophageal echocardiography with contrast
Increased left atrial pressure secondary to mitral or aortic valve disease, left ventricular dysfunction, or systemic hypertension
Pulmonary artery wedge pressure or left atrial pressure (via patent foramen ovale) (>15 mm Hg and LVEDP)
Pulmonary airway disease (e.g., chronic bronchitis and emphysema)
Respiratory function tests (FVC/FEV, chest x-ray)
Hypoxic pulmonary hypertension associated with (1) impaired ventilation, either central (CNS) or peripheral (chest wall problems or upper airway obstruction) and (2) residence at high altitude
Sleep apnea studies and respiratory function tests
DiagnosisDiagnosisPOTENTIAL CAUSE OF
PULMONARY HYPERTENSION DIAGNOSTIC STUDIESInterstitial lung disease, pneumoconioses, and fibrosis (e.g., silicosis, rheumatoid disease, and sarcoidosis)
Chest x-ray, spirometry and carbon monoxide diffusion, high-resolution chest computed tomography
Collagen-vascular disease (e.g., SLE, polyarteritis nodosa, scleroderma)
Serological and immunogenetic studies; skin, muscle, or other tissue biopsy; esophageal motility studies
Parasitic disease (schistosomiasis or filariasis)
Rectal biopsy, complement fixation, skin tests, blood smears
Cirrhosis with portal hypertension Liver function tests, ultrasonography, computed tomography
Peripheral pulmonary artery stenosis (including Takayasu disease and fibrosing mediastinitis)
Selective pulmonary angiography or pressure gradient at catheterization
Sickle cell disease Erythrocyte morphology, hemoglobin electrophoresis
TreatmentTreatment
Life style changesLife style changes graded exercise activiti graded exercise activities are better than isometric oneses are better than isometric ones
PregnancyPregnancy increase blood volume and O2 increase blood volume and O2 consumption, procoagulant factors, the risk consumption, procoagulant factors, the risk of pulmonary embolism from DVT and amniof pulmonary embolism from DVT and amniotic fluidotic fluid
TreatmentTreatment
DigoxinDigoxin increase 10% of cardiac output of increase 10% of cardiac output of RV and significantly reduction in norepinephRV and significantly reduction in norepinephrinerine
DiurecticsDiurectics relieve symptoms relieve symptoms
main factor limiting cardiac output is PAR, nmain factor limiting cardiac output is PAR, not pulmonary blood volumeot pulmonary blood volume
O2 O2 hypoxemia causes pulmonary vasoco hypoxemia causes pulmonary vasoconstrictionnstriction
TreatmentTreatmentVasodilatorVasodilator
PARAMETER MEASURED
DESIRED ACUTE CHANGES COMMENTS
Mean pulmonary artery pressure >25% fall; ideally mean PAP below 30 mm Hg
Must not be any associated significant fall in systemic blood pressure
Pulmonary vascular resistance >33% fall; ideally, PVR below 6 units
Should be associated with a fall in PA pressure and an increase in cardiac output. An increase in cardiac output alone may lead to future RV failure
Right atrial pressure No change or fall An increase in RA pressure signals impending RV failure
Pulmonary capillary wedge pressure
No change An increase in wedge pressure suggests pulmonary venoocclusive disease or coexisting LV dysfunction
Systemic blood pressure Minimal fall; mean arterial pressure should remain above 90 mm Hg
A significant hypotensive response makes chronic vasodilator therapy contraindicated
PARAMETER MEASURED
DESIRED ACUTE CHANGES COMMENTS
Cardiac output Increase The increase should be related to increased stroke volume and not solely due to increased heart rate
Heart rate No significant change A chronic increased heart rate will result in RV failure. Watch for bradycardia if high doses of diltiazem are used
Systemic arterial oxygen saturation
Increase if reduced on room air, little change if normal
A fall in systemic arterial oxygen saturation suggests lung disease or right-to-left shunting and prohibits chronic use
Pulmonary artery (mixed venous)oxygen saturation
Increase Should reflect the increase in cardiac output and improved tissue oxygenation
TreatmentTreatment VasodilatorVasodilator
Acute testing with IV vasodilatorsAcute testing with IV vasodilators
AdenosineAdenosine
A. potent vasodilator and predictive of the sA. potent vasodilator and predictive of the subsequent effects of IV prostacyclin and oral ubsequent effects of IV prostacyclin and oral CCBCCB
B. IV infusion with 50ng/kg/min and titrated B. IV infusion with 50ng/kg/min and titrated up every 2 minutes until discomfortsup every 2 minutes until discomforts
Acute testing with IV vasodilatorsAcute testing with IV vasodilators
Prostacyclin (epoprostenol or PGI2)Prostacyclin (epoprostenol or PGI2)
A. vasodilator effect mediated by adenylate A. vasodilator effect mediated by adenylate cyclase and inhibition of plateletcyclase and inhibition of platelet
B. short half-life as adenosine, given IV infusB. short half-life as adenosine, given IV infusion with increase in 2ng/kg/min every 15 to 3ion with increase in 2ng/kg/min every 15 to 30 minutes until side effect0 minutes until side effect
C. Adenosine and Prostacyclin both had inotC. Adenosine and Prostacyclin both had inotropic effectropic effect
Acute testing with IV vasodilatorsAcute testing with IV vasodilators
Nitric oxideNitric oxide
A. vasodilator via activation c-GMPA. vasodilator via activation c-GMP
B. no inotropic effectB. no inotropic effect
C. also effective in patients with secondary C. also effective in patients with secondary pulmonary HTN due to congenital heart pulmonary HTN due to congenital heart disease and ARDSdisease and ARDS
Chronic treatmentChronic treatmentCCBCCB
CCB may worsen pulmonary HTN and have negative inotropic CCB may worsen pulmonary HTN and have negative inotropic effect on RV ( under conventional dose)effect on RV ( under conventional dose)
Chronic treatmentChronic treatmentCCBCCB
High dose of CCB may reduce PA pressure and PVR which were maintained for over 5 years.High dose of CCB may reduce PA pressure and PVR which were maintained for over 5 years.
Chronic treatmentChronic treatmentCCBCCB
Patients who do not exhibit dramatic Patients who do not exhibit dramatic hemodynamic response to CCB do not benefit hemodynamic response to CCB do not benefit from their long-term usagefrom their long-term usage
Prescribing conventional dose of CCB is a Prescribing conventional dose of CCB is a malpractice because it may deteriorate malpractice because it may deteriorate
pulmonary HTNpulmonary HTN should be strongly should be strongly discourageddiscouraged
Chronic treatmentChronic treatmentChronic Prostacyclin Infusion TherapyChronic Prostacyclin Infusion Therapy
The long term effect:The long term effect:
A. VasodilatorA. Vasodilator
B. AntithrombosisB. Antithrombosis
C. Restore the integrity of endotheliumC. Restore the integrity of endothelium Prostacyclin is given via central venous catheterProstacyclin is given via central venous catheter
that is surgically implanted.that is surgically implanted.
The complications: infection, thrombosis and The complications: infection, thrombosis and
interruption of delivery (rebound pulmonary HTN)interruption of delivery (rebound pulmonary HTN) Side effect: flushing, headache, nausea, diarrhea and jSide effect: flushing, headache, nausea, diarrhea and j
aw discomfort with eatingaw discomfort with eating
Chronic treatmentChronic treatmentChronic Prostacyclin Infusion TherapyChronic Prostacyclin Infusion Therapy
Easy tachyphylaxisEasy tachyphylaxis A bridge to transplantationA bridge to transplantation Chronic use will result in high-cardiac output (iChronic use will result in high-cardiac output (i
notropic effect or neurohormone effect)notropic effect or neurohormone effect)
Chronic treatmentChronic treatmentChronic Prostacyclin Infusion TherapyChronic Prostacyclin Infusion Therapy
Chronic TreatmentChronic TreatmentAnticoagulantsAnticoagulants
The current recommendationThe current recommendation low low dose of warfarin to keep PT(INR)= dose of warfarin to keep PT(INR)= 2~2.52~2.5
Chronic TreatmentChronic TreatmentAnticoagulantsAnticoagulants
Effects of oral anticoagulant on survival in PPH
Atrial SeptostomyAtrial Septostomy
Create a right –to-left shunt which relives Create a right –to-left shunt which relives RV stress and ischemiaRV stress and ischemia
IndicationsIndications recurrent syncope or RV recurrent syncope or RV failure despite maximum medical controlfailure despite maximum medical control
Predictors of failure or failurePredictors of failure or failure mean RA mean RA pressure > 20mmHg or PVS index>55U.m2pressure > 20mmHg or PVS index>55U.m2
Heart-Lung and Lung Heart-Lung and Lung TransplantationTransplantation
Indications
Advanced obstructive, fibrotic, or pulmonary vascular disease with a high risk of death within 2 to 3 yr
Lack of success or availability of alternative therapies
Severe functional limitation but preserved ability to walk
Age 55 yr or less for candidates for heart-lung transplantation, age 60 yr or less for candidates for bilateral lung transplantation, and age 65 yr or less for candidates for single-lung transplantation
Heart-Lung and Lung TransplantationHeart-Lung and Lung Transplantation
Absolute ContraindicationsSevere extrapulmonary organ dysfunction, including renal insufficiency with a creatinine clearance below 50 ml/min, hepatic dysfunction with coagulopathy or portal hypertension, and left ventricular dysfunction or severe coronary artery disease (consider heart-lung transplantation)Acute, critical illnessActive cancer or recent history of cancer with substantial likelihood of recurrence (except for basal cell and squamous cell carcinoma of the skin)Active extrapulmonary infection (including infection with HIV, hepatitis B, hepatitis C)Severe psychiatric illness, noncompliance with therapy, and drug or alcohol dependenceActive or recent (preceding 3 to 6 mo) cigarette smokingSevere malnutrition (<70% of ideal body weight) or marked obesity (>130% of ideal body weight)Inability to walk, with poor rehabilitation potential
Relative Contraindications
Chronic medical conditions that are poorly controlled or associated with target organ damageDaily requirement for more than 20 mg of prednisone (or equivalent)Mechanical ventilation (excluding noninvasive ventilation)Extensive pleural thickening from prior thoracic surgery or infectionActive collagen-vascular diseasePreoperative colonization of the airways with pan-resistant bacteria (in patients with cystic fibrosis)
Heart-Lung and Lung TransplantationHeart-Lung and Lung Transplantation
Heart-Lung and Lung TransplantationHeart-Lung and Lung Transplantation
Age of recipients: 2 months to 61 yearsAge of recipients: 2 months to 61 years OP mortality: 16~29%, higher for single lung OP mortality: 16~29%, higher for single lung
transplantationtransplantation 1-year-survival rate:70~75%1-year-survival rate:70~75% 2- year-survival rate:55-60%2- year-survival rate:55-60% 5- year-survival rate:40-45%5- year-survival rate:40-45%
An Algorithm for the Management of PPH
New drug for PPHNew drug for PPHN Engl J Med, Vol. 346, No. 12
New drug for PPHNew drug for PPHN Engl J Med, Vol. 346, No. 12
Secondary Pulmonary HypertensionSecondary Pulmonary Hypertension
TABLE 53-7 -- ADVANCED PULMONARY HYPERTENSION BY DISEASE CATEGORY
DISEASE PREVALENCEPERCENTAGE OF
PATIENTS WITH PH
ESTIMATED NUMBER IN NORTH AMERICA AND
EUROPE
Systemic sclerosis 190/million 33 37,620
Congenital heart defects (ASD/VSD/PDA)
300/million 15-20 31,500
Cirrhosis 1600/million 0.6 5760
HIV related 2500/million 0.5 7500
Primary PH 7/million 100 4200
Secondary Pulmonary HypertensionSecondary Pulmonary Hypertension
The most common etiologiesThe most common etiologies collagen-vas collagen-vascular disease (scleroderma(1/3), CREST sycular disease (scleroderma(1/3), CREST syndrome(50%) and MCTD(2/3))ndrome(50%) and MCTD(2/3))
PrognosisPrognosis very poor very poor Conventional therapy –digitalis, diuretics anConventional therapy –digitalis, diuretics an
d O2 and anticoagulant are similar as PPHd O2 and anticoagulant are similar as PPH Prostacyclin improves exercise tolerance, hProstacyclin improves exercise tolerance, h
emodynamics and sense of well-being emodynamics and sense of well-being
White bar congenital heart dz Pink bar collagen-vascular dz
Red bar portopulmonary HTN
Increased Resistance to Pulmonary Increased Resistance to Pulmonary Venous DrainageVenous Drainage
Elevation in LV diastolic pressureElevation in LV diastolic pressure HTN, A HTN, AS, ischemic heart disease, cardiomyopathy, S, ischemic heart disease, cardiomyopathy, constrictive pericarditisconstrictive pericarditis
Left atrial HTNLeft atrial HTN MS, MR and cor triatriatum MS, MR and cor triatriatum
Increased Resistance to Flow Through Increased Resistance to Flow Through the Pulmonary Vascular Bedthe Pulmonary Vascular Bed
Pulmonary parenchymal diseasePulmonary parenchymal disease COPD, i COPD, interstitial lung diseasenterstitial lung disease
Eisenmenger syndromeEisenmenger syndrome Pulmonary thromboembolismPulmonary thromboembolism
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