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A Case of Idiopathic Pulmonary Hypertension

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  • 1.
        • A CASE OF DYSPNOEA
  • 2.
    • Mrs.muniammal
    • 35 f
    • Residing at chetpet
    • Household maid by occupation
  • 3.
    • Admitted in our unit on 14.10.11 with complaints of
    • Breathlessness -5 months,
    • gradually progressed to grade 3
    • Swelling of legs-2 months,
    • Insidious,gradually progressive
    • Abdominal distension -1 month
    • h/o reduced urine output
  • 4.
    • No h/o
    • Chest pain
    • Palpitations
    • Loc
    • Facial puffiness
    • Fever
    • High coloured urine
    • Abdominal pain
    • Vomiting,loose stools
    • Hiccoughs,altered sensorium
  • 5.
    • no h/o
    • Oral ulcers,
    • alopecia,
    • photosensitivity,
    • Arthralgia
    • Skin pigmentation
    • Raynaud’s
    • Dysphagia,skin tightening
    • Intolerance to cold, altered bowel habits
  • 6.
    • Past h/o : no similar illness in the past. not a K/C/O DM/SHT/CAD/PT/BA
    • Personal h/o: takes mixed diet
    • Not an alcoholic,smoker.
    • No h/o substance abuse.
  • 7.
    • Marital,Obstetric h/o: married with 2 children.lcb-12 yrs ago, antenatal h/o uneventful.
    • No h/o promiscual sexual activity.
    • Menstrual h/o: regular 3/30 menstrual cycles
    • No h/o heavy flow,clots.
  • 8.
    • Pt conscious
    • Oriented
    • Afebrile
    • Hyd-fair
    • Muddy conjunctiva +
    • Clubbing-gr 1
    • b/l pitting pedal edema
    • No pallor/cyanosis/lymphadenopathy
  • 9.
    • Jvp-elevated,a and v waves prominent
    • Pulse rate-80/min regular
    • BP-110/70 mm hg
    • Cvs - apical impulse left 5 th ics 2.5cm lat to MCL,normal character
    • Grade 3 parasternal heave
    • Palpable p2
    • Tricuspid area- psm+ 3/6
    • Pulmonary area-loud p2,esm+ 2/6
  • 10.
    • Respiratory system- nvbs+
    • Per abdomen- liver palpable 2cm below rcm,non tender,non pulsatile
    • Free fluid +
    • No spleenomegaly.
    • Bowel sounds+
    • Cns -nfnd.
  • 11.
    • PULMONARY HYPERTENSION
    • ? CAUSE
    • RV FAILURE
  • 12.  
  • 13.  
  • 14.  
  • 15.  
  • 16.  
  • 17.  
  • 18.
    • Urine alb,sugar nil,1-2 pus cells
    • RBS -111
    • RFT -U 15 CR 0.8
    • CBC
    • Hb 11
    • Tc 6100
    • Dc p40l57e3
    • Pcv 33.7
    • Rbc 3.8
    • Platelets 1,80,000
    • Lft
    • Tb : 1
    • Db : 0.2
    • Sgot :34
    • Sgpt :42
    • Sap :57
    • S proteins:5.6
    • Albumin :3.0
    • Globulin :2.6
  • 19.
    • Ecg -RAE,RAD
  • 20.
    • CARDIOMEGALY
    • PULMONARY TRUNK DILATATION WITH PERIPHERAL PRUNING
  • 21.
    • PERIPHERAL SMEAR STUDY :microcytic,hypochromic anemia
    • Coagulation profile- normal
    • Bt 3’30”
    • Ct 4’45”
    • Pt :14 s
    • Aptt: 27 s
    • Inr :1.o
  • 22.
    • HIV - non reactive
    • RA factor- negative
    • TFT :
    • free T3 2.90pg/ml(2-4.4)
    • freeT4 1.38ng/dl(0.93-1.7)
    • TSH 3.43mIU/ml(0.27-4.2)
  • 23.
    • Usg abdomen
    • normal study
  • 24.
    • IMPRESSION:
    • SEVERE PULMONARY HYPERTENSION
    • ?CAUSE
  • 25.  
  • 26.  
  • 27.  
  • 28.  
  • 29.
    • Pht ? Idiopathic
    • Suggested sputum analysis,ct chest
  • 30.
    • Mantoux- negative
    • Sputum afb-negative
    • Sputum c/s-negative for pathogens
  • 31.  
  • 32.
    • Ph :7.39
    • PaO2 : 92 mm hg
    • PaCO2 :41 mm hg
    • HCO3 :22 mmol/l
  • 33.  
  • 34.  
  • 35.  
  • 36.
    • Ra / rv dilated
    • Pulmonary artery dilated
    • Cardiomegaly
    • Visualised lung Parenchyma-normal
  • 37.  
  • 38.
    • Doppler study of lower limbs
    • Normal study
    • No stenosis,narrowing
  • 39.  
  • 40.  
  • 41.  
  • 42.  
  • 43.  
  • 44.  
  • 45.
    • MR ANGIO CHEST-
    • NORMAL
  • 46.
    • IDIOPATHIC
    • PULMONARY
    • HYPERTENSION
  • 47.
    • . It was first identified by Dr. Ernst von Romberg in 1891.
    • Epidemiology: IPAH is a rare disease with an incidence of about 2-3 per million per year and a prevalence of about 15 per million.
    • Adult females are almost three timesmore likely .
    • Typically younger women of childbearing age . However, IPAH can also affect individuals in their fifth and sixth decade .
  • 48. Copyright ©2009 American College of Cardiology Foundation. Restrictions may apply. McLaughlin, V. V. et al. J Am Coll Cardiol 2009;53:1573-1619 Relevant Pathways in the Pathogenesis of Pulmonary Arterial Hypertension
  • 49.
    • vasoconstriction or tightening of blood vessels
    • Over time, the affected blood vessels become both stiffer and thicker- fibrosis
    • the increased workload of the heart causes hypertrophy of the right ventricle, ultimately causing right heart failure
  • 50.
    • At least 15-20% of patients previously thought to have IPAH actually have a familial form of PAH involving at least one genetic defect.
    • The most common genetic defect in these cases involves the BMPR-II gene.
  • 51. Definition Characteristics Clinical group(s)b Pulmonary hypertension Mean PAP 25 mmHg All Pre-capillary PH Mean PAP 25 mmHg PWP 15 mmHg CO normal or reduced 1. Pulmonary arterial Hypertension 3. PH due to lung diseases 4. Chronic Thromboembolic PH 5. PH with unclear and/or Multifactorial mechanisms Post-capillary PH Passive TPG 12 mmHg Reactive (out of proportion) TPG .12 mmHg Mean PAP 25 mmHg PWP .15 mmHg CO normal or reducedc 2. PH due to left heart disease
  • 52.
    • increase in mean
    • pulmonary arterial pressure (PAP ) 25 mmHg at rest
    • † mean PAP .30 mmHg at exercise
    • assessed by right heart catheterization
  • 53.  
  • 54.
    • 1. PULMONARY ARTERIAL HYPERTENSION (PAH)
    • 1.1. Idiopathic (IPAH)    
    • 1.2. Familial (FPAH)    
    • 1.3. Associated with (APAH):     
    • 1.3.1. Connective tissue disorder        
    • 1.3.2. Congenital systemic-to-pulmonary shunts        
    • 1.3.3. Portal hypertension        
    • 1.3.4. HIV infection        
    • 1.3.5. Drugs and toxins        
    • 1.3.6. Other (thyroid disorders, glycogen storage disease,         Gaucher’s disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, chronic myeloproliferative disorders, splenectomy)
    • 1.4. Associated with significant venous or capillary involvement
    • 1.4.1. Pulmonary veno-occlusive disease (PVOD)        
    • 1.4.2. Pulmonary capillary hemangiomatosis (PCH)        
    • 1.5. Persistent pulmonary hypertension of the newborn    
  • 55.
    • 2 . Pulmonary hypertension with left heart disease
    • 2.1. Left-sided atrial or ventricular heart disease    
    • 2.2. Left-sided valvular heart disease
    •     
    • 3. Pulmonary hypertension associated with lung diseases and/or hypoxemia
    • 3.1. Chronic obstructive pulmonary disease    
    • 3.2. Interstitial lung disease    
    • 3.3. Sleep disordered breathing    
    • 3.4. Alveolar hypoventilation disorders    
    • 3.5. Chronic exposure to high altitude    
    • 3.6. Developmental abnormalities
  • 56.
    • 4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease (CTEPH)
    • 4.1. Thromboembolic obstruction of proximal pulmonary     arteries
    • 4.2. Thromboembolic obstruction of distal pulmonary     arteries
    • 4.3. Nonthrombotic pulmonary embolism (tumor, parasites, foreign  material)
    • 5 . Miscellaneous
    • Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of     pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)
  • 57.
    • Dyspnoea
    • Fatigue
    • Non productive cough
    • Angina
    • Syncope
    • Pedal edema
  • 58.
    • Cardiovascular examination
    • P2 is usually increased, which may demonstrate fixed or paradoxic splitting in the presence of severe right ventricular dysfunction;
    • Pulmonic regurgitation (Graham Steell murmur) may be apparent.
    • A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted.
    • Jvp elevated – v waves ( TR)
  • 59.
    • ELECTROCARDIOGRAPHY
    • RAE,RAD, RVH
    • ST DEPRESSION T INVERSION V1-V3
    • ECHOCARDIOGRAPHY
    • for assessing right and left ventricular function, estimating pulmonary systolic arterial pressure, and excluding congenital anomalies and valvular disease.
  • 60.
    • . Findings include central pulmonary arterial dilation which contrasts with “pruning” (loss) of the peripheral bloodvessels.
    • Right atrial and ventricular enlargement may
    • be seen and it progresses in more advanced cases.
    • Chest radiography is useful for excluding interstitial and alveolar processes that may cause hypoxia-mediated pulmonary vasoconstriction.
  • 61.
    • PULMONARY FUNCTION TESTS AND ARTERIAL BLOOD GAS
    • ANALYSIS . .
    • usually have decreased lung diffusion capacity for carbon monoxide (DLCO)
    • The arterial oxygen tension is normal or only
    • slightly lower than normal and arterial carbon dioxide tension is decreased as a result of alveolar hyperventilation.
  • 62.
    • Ventilation and perfusion lung scan .
    • ventilation and perfusion (V/Q) scans may be entirelynormal.
    • However they may also show small peripheral
    • non-segmental defects in perfusion. These are normally ventilated and thus represent V/Q mismatch
  • 63.
    • High-resolution computed tomography of the lungs.
    • HRCT provides detailed views of the lung parenchyma and facilitates the diagnosis of interstitial lung diseaseand emphysema.
  • 64.
    • Contrast-enhanced spiral computed tomography of
    • the lungs and pulmonary angiography . in PAH patients when the V/Q lung scintigraphy
    • shows segmental or subsegmental defects of perfusion with normal ventilation,
  • 65.
    • BLOOD TESTS AND IMMUNOLOGY . Routine biochemistry,hematology and thyroid function tests
    • CTD are diagnosed primarily on clinical and laboratory
    • criteria and an autoimmune screen consists of antinuclear
    • antibodies, including anti-centromere antibody,anti-SCL70 and RNP.
    • About one third of patients with idiopathic PAH have positive but low antinuclear antibody titers (≥ 1:80 dilutions.
    • HIV Testing
    • HIV-positive patients have a higher rate of IPAH than the general population
  • 66.
    • Antinuclear Antibody
    • Excluding autoimmune disorders is an important part of the workup in a patient with suspected pulmonary hypertension. Reportedly, up to 40% of patients with IPAH have a positive finding on an antinuclear antibody (ANA) assay but no other clinical manifestations of autoimmune disease.
    • Thyrotropin
    • Screen for thyroid abnormalities during the initial workup for IPAH because these abnormalities are common in patients with IPAH. Thyroid abnormalities may be the cause of or contribute to symptoms similar to IPAH. In addition, hyperthyroidism itself may lead to an elevation in pulmonary artery pressure.
  • 67.
    • Abdominal ultrasound scan.
    • Liver cirrhosis and/or portal hypertension can be reliably excluded by the use of abdominal ultrasound scan..
  • 68.
    • Exercise Testing
    • Six-minute walk testing
    • Cardiopulmonary Exercise Testing
    • Assessment of aerobic capacity and ventilatory efficiency can help identify a pulmonary vascular limit to exercise and can be used to differentiate intrinsic pulmonary vascular disease from cardiac deconditioning and restrictive or obstructive lung disease or left-sided cardiac dysfunction.
  • 69. -TYPE NATRIURETIC PEPTIDE Levels of B-type natriuretic peptide (BNP) and N-terminal BNP have been shown to be elevated in patients with IPAH, and levels appear to be prognostic.
  • 70.
    • Sleep Study
    • Sleep apnea must be excluded as a contributor or cause of pulmonary hypertension if the patient's history suggests so.
    • Cardiac Catheterizatio n
    • This is the criterion standard test to definitively confirm any form of PAH, including IPAH.
    • Excluding left-sided heart disease, including diastolic dysfunction
    • to determine pulmonary vasoreactivity, which may have implications in the initiation and titration of ccb
    • Lung Biopsy
    • When the etiology of pulmonary hypertension is still in doubt
  • 71.  
  • 72.
    • Low probability for PAH diagnosis
    • Echocardiographic diagnosis of ‘PH unlikely’, no
    • symptoms: no additional work-up is
    • Recommended
    • Echocardiographic diagnosis of ‘PH unlikely’,
    • presence of symptoms and of associated
    • conditions or risks factors for group 1—PAH:
    • echocardiographic follow-up is recommended
    • Echocardiographic diagnosis of ‘PH unlikely’,
    • presence of symptoms, and absence of
    • associated conditions or risks factors for group
    • 1—PAH: evaluation of other causes for the
    • symptoms is recommended
  • 73.
    • High probability for PAH
    • Echocardiographic diagnosis of ‘PH likely’, with
    • symptoms and presence/absence of associated
    • conditions or risks factors for group 1—PAH:
    • RHC is recommended
    • Echocardiographic diagnosis of ‘PH likely’, without
    • symptoms and presence/absence of associated
    • conditions or risks factors for group 1—PAH:
    • RHC should be considered
  • 74.
    • Class I Patients with pulmonary hypertension but without
    • resulting limitation of physical activity
    • Class II Patients with pulmonary hypertension resulting in slight
    • limitation of physical activity. They are comfortable at
    • Rest
    • Class III Patients with pulmonary hypertension resulting in marked
    • limitation of physical activity. They are comfortable at
    • rest.
    • Class IV Patients with pulmonary hypertension with inability to
    • carry out any physical activity without symptoms. These
    • patients manifest signs of right heart failure. Dyspnoea
    • and/or fatigue may even be present at rest
  • 75.  
  • 76. Copyright ©2009 American College of Cardiology Foundation. Restrictions may apply. McLaughlin, V. V. et al. J Am Coll Cardiol 2009;53:1573-1619 Treatment Algorithm for PAH
  • 77.
    • act on the vascular smooth muscle, dilating the pulmonary resistance vessels and lowering the pulmonary artery pressure.
    • CCBs are used at high doses
    • limited to patients without overt evidence of right heart failure.
  • 78.
    • Parenteral Vasodilators
    • Parenteral vasodilators are used for patients whose IPAH fails to respond to calcium channel blockers or who cannot tolerate these agents and who have New York Heart Association (NYHA) type III or IV right-sided heart failure. PGI2 ANALOGUES
    •  
    • Epoprostenol In addition ,this agent also contributes to inhibition of platelet aggregation n inhibition of smooth muscle proliferation.
    •  
    • Treprostinil
  • 79.
    • Inhaled Vasodilators
    • Inhaled prostacyclin (PGI 2 ) synthetic analogues are an alternative to parenteral administration. They are used in an attempt to limit systemic adverse effects.
    • Iloprost
    •  
    • Treprostinil
    •  
  • 80.
    • Inhibition of the antiproliferative effects of the PDE-5 pathway, which regulates cyclic guanosine monophosphate hydrolysis
    • Sildenafil
    •  
    • Tadalafil
    •  
  • 81.
    • Endothelin receptor antagonists (ERAs) are therapeutic alternatives to parenteral prostacyclin agents. Given orally, they competitively bind to endothelin 1 (ET-1) receptors endothelin-A and endothelin-B,
    • causing a reduction in pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and mean right atrial pressure (RAP).
    • This agent is indicated for treatment of PAH in patients with WHO class III or IV symptoms to improve exercise ability and decrease the rate of clinical deterioration.
    • Bosentan
    •  
    • Ambrisentan
    •  
  • 82.
    • Diuretics
    • Diuretics are used in pulmonary hypertension to manage peripheral edema.
    • Furosemide
    •  
    • Bumetanide
    •  
    • Spironolactone
    •  
  • 83.
    • Anticoagulants
    • survival in IPAH, regardless of histopathologic subtype, is increased when patients are treated with anticoagulant therapy..
    • Warfarin should be used, provided the patient has no contraindications to anticoagulation.
    • Maintain an international normalized ratio (INR) of 1.5 to 2.
    • Cardiac Glycosides
    • Digoxin therapy can be used to improve right ventricular function in patients with right ventricular failure.
  • 84.
    • Surgical Care
    • A single- or double-lung transplant is indicated for patients who do not respond to medical therapy.
    • Atrial septostomy is a palliative procedure allowing interatrial right-to-left shunting to occur, thus delivering more overall oxygen content to the respiring tissues, albeit with a lower overall saturation.
  • 85.
    • Future Therapies
    • TYROSINE KINASE INHIBITORS
    • CINACIGUAT,RIOCIGUAT-ACTIVATORS OF GUANYLYL CYCLASE
  • 86.
    • Prognosis
    • The NIH IPAH registry
    • For untreated IPAH, the estimated 3-year survival rate is approximately 41
  • 87.  
  • 88.