A Case of Idiopathic Pulmonary Hypertension
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Transcript of A Case of Idiopathic Pulmonary Hypertension
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A CASE OF DYSPNOEA
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Mrs.muniammal 35 f Residing at
chetpet Household maid by
occupation
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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 monthh/o reduced urine output
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No h/oChest painPalpitationsLocFacial puffinessFeverHigh coloured urineAbdominal painVomiting,loose stoolsHiccoughs,altered sensorium
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no h/o Oral ulcers, alopecia, photosensitivity, Arthralgia Skin pigmentation Raynaud’s Dysphagia,skin tightening Intolerance to cold, altered bowel
habits
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Past h/o: no similar illness in the past. not a K/C/O DM/SHT/CAD/PT/BA
Personal h/o: takes mixed dietNot an alcoholic,smoker.No h/o substance abuse.
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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.
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Pt consciousOrientedAfebrileHyd-fairMuddy conjunctiva +Clubbing-gr 1b/l pitting pedal edemaNo pallor/cyanosis/lymphadenopathy
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Jvp-elevated,a and v waves prominent Pulse rate-80/min regular BP-110/70 mm hg
Cvs- apical impulse left 5th 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
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Respiratory system- nvbs+
Per abdomen-liver palpable 2cm below rcm,non tender,non pulsatile
Free fluid +No spleenomegaly.Bowel sounds+
Cns-nfnd.
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PULMONARY HYPERTENSION ? CAUSE
RV FAILURE
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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
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Ecg -RAE,RAD
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CARDIOMEGALY PULMONARY
TRUNK DILATATION WITH PERIPHERAL PRUNING
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PERIPHERAL SMEAR STUDY:microcytic,hypochromic anemia
Coagulation profile- normal Bt 3’30” Ct 4’45” Pt :14 s Aptt: 27 s Inr :1.o
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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)
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Usg abdomennormal study
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IMPRESSION: SEVERE PULMONARY
HYPERTENSION ?CAUSE
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Pht ? IdiopathicSuggested sputum analysis,ct chest
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Mantoux- negativeSputum afb-negativeSputum c/s-negative for pathogens
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Ph :7.39PaO2 : 92 mm hgPaCO2 :41 mm hgHCO3 :22 mmol/l
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Ra / rv dilatedPulmonary artery dilatedCardiomegalyVisualised lung Parenchyma-normal
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Doppler study of lower limbsNormal studyNo stenosis,narrowing
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MR ANGIO CHEST-NORMAL
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IDIOPATHICPULMONARY
HYPERTENSION
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. 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 .
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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
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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
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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.
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Definition Characteristics Clinical group(s)b
Pulmonaryhypertension
Mean PAP25 mmHg
All
Pre-capillary PH Mean PAP25 mmHgPWP 15 mmHgCO normal orreduced
1. Pulmonary arterialHypertension3. PH due to lung diseases4. ChronicThromboembolic PH5. PH with unclear and/orMultifactorial mechanisms
Post-capillary PH Passive TPG 12 mmHgReactive (out ofproportion)TPG .12 mmHg
Mean PAP25 mmHgPWP .15 mmHgCO normal orreducedc
2. PH due to left heartdisease
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increase in meanpulmonary arterial pressure (PAP) 25
mmHg at rest † mean PAP .30 mmHg at exerciseassessed by right heart
catheterization
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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
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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
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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)
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DyspnoeaFatigueNon productive coughAnginaSyncopePedal edema
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Cardiovascular examination P2is 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)
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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.
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. 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.
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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.
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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
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High-resolution computed tomography of the lungs.
HRCT provides detailed views of the lung parenchyma and facilitates the diagnosis of interstitial lung diseaseand emphysema.
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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,
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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
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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.
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Abdominal ultrasound scan. Liver cirrhosis and/or portal
hypertension can be reliably excluded by the use of abdominal ultrasound scan..
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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.
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-TYPE NATRIURETIC PEPTIDELevels 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.
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Sleep Study Sleep apnea must be excluded as a contributor or cause
of pulmonary hypertension if the patient's history suggests so.
Cardiac Catheterization 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
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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
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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
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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
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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
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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.
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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
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Inhaled Vasodilators Inhaled prostacyclin (PGI2) synthetic
analogues are an alternative to parenteral administration. They are used in an attempt to limit systemic adverse effects.
Iloprost
Treprostinil
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Inhibition of the antiproliferative effects of the PDE-5 pathway, which regulates cyclic guanosine monophosphate hydrolysis
Sildenafil Tadalafil
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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
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Diuretics Diuretics are used in pulmonary
hypertension to manage peripheral edema.
Furosemide
Bumetanide
Spironolactone
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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.
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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.
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Future Therapies
TYROSINE KINASE INHIBITORS
CINACIGUAT,RIOCIGUAT-ACTIVATORS OF GUANYLYL CYCLASE
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PrognosisThe NIH IPAH registry
For untreated IPAH, the estimated 3-year survival rate is approximately 41
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