Transcript Document

‫به نام خدا‬
Case history
• The patient was a 38-year-old woman who
presented with shortness of breath.
• She claimed to be an active woman who used to
worked actively until she began to notice
progressive dyspnea while working 1 year ago.
• Her symptoms had progressed to the point that
she was symptomatic while climbing a flight of
stairs.
Case history
• At the time of the initial visit, her symptoms
were so severe that she was breathless with
activities of daily living.
• She denied any symptoms of cough, fevers,
chills, and syncope, but she did complain of
mild, dull, midsternal chest pain.
• She had hemoptysis and hoarseness 2w prior
first visit.
• She also reported occasional palpitations.
Case history
• There was no history of venous thromboembolism, diet
medication use, risk factors for HIV, and use of any illegal
drugs.
• The patient hadn’t history of smoking.
• She did, however, complain of occasional muscle aches
and joint pains but has not noted any other symptoms
suggestive of collagen vascular disease.
• Her past medical history:not diabet ,not HTN , not IHD
Physical Exam
• The patient was well developed, well
nourished, and in no acute distress.
• Vitals: weight: 69 kg, height: 166 cm.pulse: 87
beats/min, SaO2: 96%, BP: 160/100 mmHg.
T=37 axillary
• Head &neck: There was no scleral icterus. The
nasal passages are narrow but patent.
• thyriod gland NL.
• The oropharynx reveals a moderate-sized
tongue. There is no retrognathia.
• There weren’t cervical lymphadenopathy.
• Skin: There were no rashes or nodules.
• The heart had a regular rate and rhythm with a
normal S1 but a fixed, split S2.
• There were no cardiac murmurs, rubs, or
gallops.
• Pulmonary: Lungs were normal to auscultation
and percussion. There were no wheezes,
rhonchi, or rales.
• Abdomen: The abdomen was soft and non
tender without evidence of
hepatosplenomegaly
Physical Exam
• Extremities: There was clubbing , no cyanosis,
or edema.
• Neurologic: The cranial nerves were intact.
Muscle strength was normal, and her gait
was normal.
Lab test
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WBC (×10E3/uL):5.9
Hemoglobin (g/dL):12.4
Hematocrit (%):36.2
Platelets (×10E3/uL):267
Albumin, bilirubin, Alkaline phosphatase,
AST, ALT:nl
Chest X Rey
Latral X Rey
‫سوال اول‬
‫• شواهد گرافی قفسه سینه به نفع کدام نوع درگیری است؟‬
‫• ‪ -1‬لنفادنوپاتی مدیاستن‬
‫• ‪ -2‬برجستگی شریانهای پوملونر‬
Chest X Rey
Echocardiogram
• Normal left ventricular size with an ejection
fraction of 60–65%.
• There is left ventricular diastolic dysfunction.
• There is a flattened ventricular septum, consistent
with right ventricular pressure and volume
overload.
• The right ventricle is dilated and diffusely
hypokinetic with moderate to severe right
ventricular systolic dysfunction.
• There is mild right ventricular hypertrophy.
Echocardiogram
• The estimated pulmonary arterial pressure is
90–95 mmHg.
• The right atrium is dilated.
• The mitral and aortic valves appear normal.
‫سوال دوم‬
‫• فشار شریان ریوی اندازه گیری شده در اکوکاردیوگرافی‪:‬‬
‫• ‪ -1‬فشار سیستولیک‬
‫• ‪ -2‬فشار دیاستولیک‬
‫• ‪ -3‬فشار متوسط شریانی‬
• A ???? pulmonary artery pressure (PAP) > 25
mmHg at rest or >30 mmHg with exercise in
the presence of a normal pulmonary capillary
wedge pressure (<15 mmHg).
• The exercise criterion was removed from the
definition of PH due to the wide variation in
pulmonary artery pressures during exercise
in normal, healthy individuals as well as the
increases that occur with aging.
‫سوال سوم‬
‫• منظور از این فشار کدامست؟‬
‫• ‪ -1‬فشار سیستولیک‬
‫• ‪ -2‬فشار دیاستولیک‬
‫• ‪ -3‬فشار متوسط شریانی‬
‫سوال چهارم‬
‫• با توجه به ابتال به هیپرتانسون پوملونر اولین اقدام تشخیص ی ‪:‬‬
‫‪PFT.1‬‬
‫‪CT scan.2‬‬
‫‪Perfusion scan.3‬‬
‫‪PSG.4‬‬
PFT
FEV1= 1.97 L
(78 % of Ref)
FVC=
2.51 L (84% of Ref)
FEV1/FVC (%)
90
FEF25–75% (L/s) 1.85 (76 % of Ref)
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•
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•
‫سوال پنج‬
‫•‬
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‫•‬
‫•‬
‫پترن درگیری در اسپیرومتری؟‬
‫‪-1‬تحدیدی‬
‫‪ -2‬انسدادی‬
‫‪FEV1= 1.97 L‬‬
‫)‪(78 % of Ref‬‬
‫‪-3‬مخلوط‬
‫=‪FVC‬‬
‫)‪2.51 L (84% of Ref‬‬
‫)‪FEV1/FVC (%‬‬
‫‪90‬‬
‫)‪FEF25–75% (L/s) 1.85 (70 % of Ref‬‬
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‫سوال ششم‬
‫•‬
‫•‬
‫•‬
‫•‬
‫•‬
‫با توجه به اسپیرومتری بعمل امده اقدام بعدی شما؟‬
‫‪HRCT -1‬‬
‫‪perfusion scan-2‬‬
‫‪PSG -3‬‬
‫‪TEE -4‬‬
‫?‬
‫سوال هفتم‬
‫•‬
‫•‬
‫•‬
‫•‬
‫•‬
‫‪ HRCT‬بیمار نرمال بدون درگیری پارانشیم ریه ‪.‬اقدام بعدی ؟‬
‫‪ -1‬اسکن پرفیوژن ریه‬
‫‪PSG -2‬‬
‫‪ -3‬انجام ازمایشات بیوشیمی‬
‫‪ -4‬تست ورزش‬
‫?‬
Lab test
• TSH=4.6
• ESR=6
• ANA: negative, RF: negative, ANCA: negative,
HIV: negative
• Perfusion scan:There are no segmental or
subsegmental perfusion defects.
Pulmonary HTN
• Pulmonary HTN is characterized by elevated
pulmonary arterial pressure and secondry right
ventricular failure.
• Pulmonary HTN primary ,idiopathic & secondry
Epidemiology
• PPHTN
a disease of young women
• Femal to male ratio 1/7 : 1
• Infrequency over the next 60 year
• However PPHTN has been reported in both
sexes within a wide age range of 1 to 81 years
• Mean age: 36
• Incidece : 5 to 15 cases per million
Pathogenesis
• Mutation in bone morphogenic protein receptor 2(BMPR2)
gene that contributes to apoptotic process. PPHTN =cacer of
pulmonary of artery
• Defect in spesific voltage gated potassium ion channel
excessive ca to inter cell promote cell concentration
• Defect in vasodilatory ( NO ,Prostacycline) in lung
• An excess in lung and circulating level of endothelin 1
pathophysiology
1.
2.
3.
4.
5.
Medial hypertrophy
vasoconstricture
intimal proliferation ,intimal fibrosis
progressive obstruction
Vascular dilation in present of high pressure
Some of dilated area evolved into
microaneurysms
in situ thrombosis
6. Necrotizing arteritis
neutrophil
inflammation
Clinical manifestation
Non spesific
Initialy exertional dyspnea(95%), breathlessness(60%)
fatigue and weakness
exertional Chest pain, syncope
Less commen symptom:
cough,hemoptysis,hoarsness(ortner syndrom)
Ph/EX
• Initial ph/Ex:increase of intensity of pulmonic S2
• S2 is narrowly split or single in patient with
preserved right ventricular function
• Right ventricular failure :wide S2 /systolic murmur
• Sever RV failure :jvp increase ,hepatomegaly,ascitis,
pleural effusion
• Cyanosis during exercise is a late phenomen
• Clubbing is not usual sign
Assesment
• CBC
polycytemia
• Chest x Rey
paranchymal lung disease
,protrusion of main pulmonary artery
• ECG
right ventricular hypertrophy
• Echo
ruling out condition such as MVD ,
Left ventricular failure and measurment PAP
• PFT
ruling out obstructive disease ,if
FEV1<25% increase PAP
Assesment
• ABG
low PCO2 respiratory alkalosis
• PFT
normal or restrictive pattern
• Lung scan
segmental defect
(thromboemboli) , no defect( exclusively
PPHTN) , multiple subsegmental defect (
maybe ppHTN )
Diagnostic criteria
• Mean pumonary artery pressure is >25 mmhg
at rest and artery pressure >30 on exercise
• Mean pulmonary capillary wedge pressure
<15mmhg
• Chronich lung disease are mild or absent
• Venous thromboemboli is absent
• Certain miscellaneous disorder are absent
Cardiac Catheterization
Cardiac catheterization is mandatory to document PAH, R/O
cardiac disease and determine vasoreactivity to drug.
It is recommended that patients with pulmonary arterial
hypertension undergo drug testing with a short-acting
pulmonary vasodilator to determine the extent of pulmonary
vasodilator reactivity. Inhaled nitric oxide, adenosine, and
intravenous prostacycline
Respons
a fall in mean pulmonary arterial pressure 10
mmHg and a final mean pressure <40 mmHg.
Treatment: Pulmonary Arterial
Hypertension
• Because the pulmonary artery pressure in PAH
increases with exercise, patients should be cautioned
against participating in activities that impose physical
stress.
• Diuretic therapy relieves peripheral edema and may
be useful in reducing RV volume overload.
• Pulse oximetry should be monitored, as O2
supplementation helps alleviate dyspnea and RV
ischemia in patients whose arterial O2 saturation is
reduced
Treatment
• Anticoagulant therapy is advocated for all
patients with PAH based on studies
demonstrating that warfarin increases
survival of patients with PAH. The dose of
warfarin generally is titrated to achieve an
international normalized ratio (INR) of 2–3
times control.
Principle of drug treatment
‫سوال هشتم‬
‫•‬
‫•‬
‫•‬
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‫•‬
‫از عالیم ذکر شده کدام یک با ‪ IPAH‬همخوانی ندارد؟‬
‫‪ -1‬خشونت صدا‬
‫‪ -2‬کالبینگ‬
‫‪ -3‬هموپتزی‬
‫‪ -4‬درد رترواسترنال‬
Ca Cannel Blocker
• High doses
• Diltiazem 120mg/d ,long actin nifedipin 30
mg/d ,Amlodipin 20 mg /d
• No response vasoreactive : Ca cannal Blocker
therapy may cause significant morbidity and
mortality
Endothelin Receptor Antagonists
• The endothelin receptor antagonists bosentan and
ambrisentan .
• bosentan is initiated at 62.5 mg bid for the first month and
increased to 125 mg bid thereafter. Ambrisentan is initiated
as 5 mg once daily and can be increased to 10 mg daily.
• Because of the high frequency of abnormal hepatic function
tests associated with these drugs, primarily an increase in
transaminases, it is recommended that liver function be
monitored monthly .
• Bosentan is contraindicated in patients who are on
cyclosporine or glyburide concurrently.
Phosphodiesterase-5 Inhibitors
• Sildenafil and tadalafil, phosphodiesterase-5 inhibitor.
Phosphodiesterase-5 is responsible for the hydrolysis of
cyclic GMP in pulmonary vascular smooth muscle, the
mediator through which nitric oxide lowers pulmonary
artery pressure and inhibits pulmonary vascular growth.
•
The effective dose for sildenafil is 20–80 mg tid. The
effective dose for tadalafil is 40 mg once daily.
• The most common side effect is headache. Neither drug
should be given to patients who are taking nitrovasodilators.
Prostacyclin analogus
• The intravenous prostacyclins have the greatest efficacy as
treatments for PAH and are often effective in patients who
have failed all other treatments. The optimal doses of these
drugs have not been determined, but the typical doses of
epoprostenol range from 25 to 40 ng/kg per min.
• The major problem with intravenous therapy is infection
related to the indwelling venous catheter, which requires
close monitoring . In addition, abrupt discontinuation of
intravenous prostacyclins can lead to a rebound increase in
pulmonary pressure
• Side effects include flushing, jaw pain, and diarrhea, which
are tolerated by most patients.
Lung Transplantation
• Lung transplantation is considered for
patients who, while on an intravenous
prostacyclin, continue to manifest right heart
failure.
• complication :opportunistic infection , graft
rejection ,obliterative bronchiolitis
Thank you