Pulmonary hypertension resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vidit Bhargava, M.B.B.S [2], Rim Halaby, M.D. [3], Alejandro Lemor, M.D. [4]

Pulmonary Hypertension Resident Survival Guide Microchapters
Overview
Causes
Classification
Clinical
Functional
FIRE
Diagnosis
Treatment
IPAH
Other Causes
Follow up
Do's
Don'ts

Overview

Pulmonary hypertension (PH) is defined as an at rest mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg measured using right heart catheterization. PH is clasified into five different ethiological groups and four functional classes according to WHO. Multiple causes and risk factors have been identified as responsible for the presentation of PH, with left heart diseases and lung diseases at the top of the list.[1] [2] Pulmonary arterial hypertension (PAH) is an exclusion diagnosis which requires common causes to be ruled out and a mPAP ≥ 25 mm Hg, a pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg and a pulmonary vascular resitance (PVR) > 3 Wood Unitis (WU).[3] The treatment of pulmonary hypertension depends upon the underlying etiology. For PAH the treatment is based on oral endothelin receptor antagonists (ambrisentan, bosentan), oral phosphodiesterase-5 inhibitors (sildenafil), and/or prostanoids (epoprostenol, treprostinil). For other causes of pulmonary hypertension, the treatment would be addressed to the primary disease (eg. heart failure, COPD, interstitial lung disease)

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Click here for the complete list of causes.

Classification

Clinical Classification of Pulmonary Hypertension

The classification below is based on the 5th World Symposium on Pulmonary Hypertension Update from the 1998 World Health Organization Pulmonary Hypertension clinical classification.[4]

Group 1. Pulmonary arterial hypertension (PAH)

Group 1'. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

Group 1". Persistent pulmonary hypertension on the newborn (PPHN)

Group 2. Pulmonary hypertension due to left heart disease

Group 3. Pulmonary hypertension due to lung diseases and/or hypoxia

Group 4. Chronic thromboembolic pulmonary hypertension (CTEPH)

Group 5. Pulmonary hypertension with unclear multifactorial mechanisms

Functional Classification of Pulmonary Hypertension

1998 WHO modified NYHA functional classification of pulmonary hypertension
Class I Class II Class III Class IV
  • No limitation of physical activity
  • Physical activity does not cause excessive dyspnea, fatigue, chest pain, or syncope
  • Mild limitation of physical activity
  • Patients are comfortable at rest
  • Physical activity causes excessive dyspnea, fatigue, chest pain, or syncope
  • Notable limitation of physical activity
  • Patients are comfortable at rest
  • Ordinary activity causes excessive dyspnea, fatigue, chest pain, or syncope
  • Inability to carry out any physical activity without symptoms.
  • Signs of right heart failure
  • Dyspnea and/or fatigue may even be present at rest
  • Discomfort is increased by any physical activity

FIRE: Focused Initial Rapid Evaluation

A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients in need of immediate intervention.


Complete Diagnostic Approach

A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention. The algorithm below is based on the ACC/AHA 2009 Expert consenus document on pulmonary Hypertension and the ESC 2009 Guideles for the diagnosis and treatment of pulmonary hypertension.[1] [2]

Abbreviations: AS: Aortic stenosis; COPD: Chronic obstructive pulmonary disease; DVT: Deep venous thrombosis; EKG: Electrocardiogram; MR: Mitral regurgitation; MS: Mitral stenosis; PAP Pulmonary artery pressure; PAWP: Pulmonary artery wedge pressure; PE: Pulmonary embolism; PVR: Pulmonary vascular resistance; SLE: Systemic erythemotous lupus

 
 
 
 
 
 
 
Characterize the symptoms:
❑ Progressive dyspnea
❑ Exertional dizziness
Syncope
Edema of the extremities
Chest pain
Palpitations
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Inquire about past medical history

❑ Cardiovascular disease

Congenital heart disease
Left ventricular failure

❑ Pulmonary disease

❑ Previous PE
COPD
Interstitial lung disease
Sleep apnea
Asthma

❑ Recent surgery (<3 months) (suggestive of PE)
Connective tissue disease
HIV infection
Portal hypertension
Chronic hemolytic anemia
Myeloproliferative disorders
Splenectomy
❑ Systemic diseases

Sarcoidosis
Pulmonary Langerhans histiocytosis

Lung tumors
Chronic kidney disease in dialysis

Thyroid disease
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Inquire about risk factors

❑ Family history of pulmonary hypertension

❑ BMPR2 gene mutation
❑ ACVRL1 gene mutation

❑ Drugs

Anorectic drugs
Amphetamines
Cocaine
Phenylpropanolamine
Saint John's wort
Chemotherapeutic agents
Selective serotonin reuptake inhibitors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Examine the patient:

Physical signs that reflect severity of PH:
Loud pulmonary second heart sound (P2)
Systolic murmur suggestive of tricuspid regurgitation
❑ Raised jugular venous pressure (JVP)
❑ Early systolic click
❑ Left parasternal heave
❑ Right ventricular S4


Physical signs suggestive of moderate to severe PH:
Holosystolic murmur that increases with inspiration
❑ Increased jugular v waves
Pulsatile liver
Diastolic murmur
Hepatojugular reflux


Physical signs suggestive of advanced PH with right ventricular failure:
❑ Right ventricular S3
❑ Distension of jugular veins
Hepatomegaly
Peripheral edema
Ascites
Hypotension


Physical signs suggestive of possible underlying causes:
Central cyanosis (Suggestive of an abnormal V/Q)
Clubbing (Suggestive of congenital heart disease)
❑ Cardiac auscultatory findings (Suggestive of congenital or acquired heart disease)
Rales, decreased breath sounds, dullness (Suggestive of pulmonary congestion)
❑ Fine rales, excessive muscle use, wheezing, protracted respiration, cough (Suggestive of pulmonary parenchymal disease)
Obesity, kyphoscoliosis, enlarged tonsils (Suggestive of disordered ventilation)
Sclerodactyly, arthritis, telengiectasia, Raynaud phenomenon, rash (Suggestive of connective tissue disorder)
❑ Peripheral venous insufficiency (Suggestive of venous thrombosis)
Venous stasis ulcers (Suggestive of sickle cell disease)
❑ Pulmonary vascular bruits (Suggestive of chronic thromboembolic PH)
Splenomegaly, spider angiomata, palmar erythema, icterus, caput medusae (Suggestive of portal hypertension)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Order tests:

Labs
Complete blood count and peripheral smear (to rule out):

Chronic hemolytic anemia
Sickle cell disease
Thalassemia
Spherocytosis

❑ Anticentromere antibody, anti-scl70 and U3-RNP (to rule out Scleroderma)
Anti-nuclear antibody, anti-dsDNA antibody (to rule out SLE)
Rheumatoid factor (to rule out rheumatoid arthritis)
HIV serological test
Liver function tests


Other tests
Chest X-ray

Pulmonary artery dilation
❑ Loss of peripheral blood vessels
❑ Enlargement of the right chambers
❑ Pulmonary diseases
Left heart abnormalities

EKG

Signs of right ventricle hypertrophy
Right axis deviation
Supraventricular arrhythmias such as atrial flutter and Atrial fibrillation (suggestive of severe disease)

Echocardiogram

❑ Measure pulmonary artery pressure
❑ Assess valvular diseases such as MR, MS, AS
Left ventricle: evaluate possible systolic or diastolic dysfunction
❑ Rule out other obstructive conditions, such as:
Supravalvular aortic stenosis
Aortic coarctatio
Subaortic membrane
Cor triatriatum
❑ Search for congenital diseases with shunt such as atrial septal disease
❑ Thrombus in right chambers (suggestive of pulmonary embolism)
Pulmonary stenosis
❑ Abdominal ultrasound (to rule out portal hypertension)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the findings are suggestive of heart disease or pulmonary disease?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal V/Q test
 
Perfusion defects
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient presents any other conditions that can present with PH (Group 5)?
 
Pulmonary hypertension due to chronic thromboembolism[5]

❑ Patient with previous PE or DVT
AND
Dyspnea, fatigue, exercise intolerance
OR
❑ Signs of right heart failure
❑ A pulmonary angiography should be performed in patients with altered V/Q test
❑ Contrast CT angiography

❑ Irregularities of the intima and vein occlusion
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
Yes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Refer to a specialized center for a right heart catheterization

❑ Confirmed PAH:

❑ Mean PAP ≥ 25 mm Hg
AND
❑ PAWP ≤ 15 mm Hg
AND
❑ PVR > 3 Wood units

❑ Perform a vasoreactivity test to assess the possible benefit of long-term treatment with calcium channel blockers only in patients with Idiopathic PAH[3]

❑ Administer: Inhalaed nitric oxide 10-20 parts per million (gold standard)
❑ Alternatives: IV epoprostenol, IV adenosine, inhaled iloprost
❑ Reduction of the mean PAP in ≥ 10 mm Hg to a mean PAP of ≤ 40 mm Hg with an elevation or maintained cardiac output is considered a positive response
The use of calcium channel blockers should be avoided for this test
Do not perform this tests in other types of PAH or PH
 

❑ Hematological disorders:

Myeloproliferative disorders
Splenectomy
Chronic hemolytic anemia

❑ Systemic disorders:

Sarcoidosis

❑ Metabolic disorders:

Thyroid disease

❑ Others:

Chronic kidney disease in dialysis
❑ Obstructing tumor
❑ Fibrous mediastinitis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Idiopathic
 
Heritable

❑ Family history of BMPR2 gene mutation

❑ Family history of ACVRL1 gene mutation
 
 
Associated pulmonary arterial hypertension

Connective tissue disease
HIV infection
Portal hypertension
❑ Hematological disorders

Sickle cell disease
Thalassemia
Spherocytosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Findings
Symptoms:
❑ Exertion dyspnea
Fatigue

Signs:

❑ Clubbing
Rales in lung bases

Chest X-ray:

Kerley B lines
❑ Ground-glass centrolubular lines

CT scan:

❑ Thickened septal subpleural lines
Ground-glass centrolubular lines
Lymphadenopathy in the mediastinum

Pulmonary edema with epoprostenol administration
❑ Search alveolar hemorrhage: perform a bronchoscopy with bronchoalveolar lavage
❑ Gold standard: lung biopsy

❑ PAWP is normal
 
 
 
 
 
 
 


Treatment

Idiopathic Pulmonary Arterial Hypertension

Shown below is an algorithm of the treatment approach for Idiopathic PAH based on the ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension[6] and JACC 2013 Updated Treatment Algorithm of Pulmonary Arterial Hypertension.[7]

Abbreviations: BID: two times a day; TID: three times a day; RV: Right ventricle; mPAP: mean pulmonary artery pressure; BNP: Brain natriuretic peptide ; TTE: Transthoracic echocardiography

 
 
 
 
 
General recommendations
❑ Avoid pregnancy (30% to 50% maternal mortality rate)[8]
❑ Consider physical therapy for physically deconditioned patients
❑ Administer vaccines against influenza and pneumococcal infections
❑ Offer psychosocial support
❑ Avoid excessive physical activity
❑ For elective surgeries, epidural anesthesia should be considered before general anesthesia
❑ Avoid exposure to high altitudes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Supportive therapy
❑ Administer diuretics in patients with signs of RV failure and fluid retention
❑ Administer oxygen in patients with PaO2 < 60 mm Hg (SatO2 <90%)
❑ Administer warfarin (titrated to a INR of 1.5-2.5) in patients with IPAH, hereditable PAH, and PAH due to anorexigens
❑ Administer digoxin in patients with atrial arrhythmias or right heart failure and a low cardiac output
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Referral to an expert center for vasodilator testing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute vasodilator testing
Agents

Nitric oxide inhaled 10 - 20 p.p.m (preferred vasodilator)
OR
Epoprostenol IV 2ng/kg/min every 10 to 15 min
OR

Adenosine IV 50 mcg/Kg/min every 2 min
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Is there a decrease in mPAP of at least 10 mm Hg to an absolute mPAP of less than 40 mm Hg without a decrease in cardiac output?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
YES
Positive
 
 
 
NO
Negative
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Administer an oral calcium channel blocker (CCB):
Avoid the use of verapamil due to its negative inotropic effect
Nifedipine
❑ Start with 30mg bid
❑ Increase slowly to 120–240 mg/day

OR

Diltiazem
❑ Start with 60mg TID
❑ Increase slowly to 240–720 mg/day

OR

Amlodipine
❑ Start with 2.5 mg/day
❑ Increase slowly to 20 mg/day
❑ Follow closely for efficacy and safety
 
Low risk

❑ No clinical evidence of RV failure
❑ Gradual progression of symptoms
WHO functional class II and III
❑ 6 min walk distance > 400 meters
❑ Cardiopulmonary exercise testing: peak Vo2 > 15 mL/kg/min
TTE: minimal RV dysfunction
❑ Right atrial pressure < 8 mm Hg
❑ Cardiac index > 2.5-3.0 L/min/m²
❑ Normal or slight increased BNP
 
High risk

❑ Clinical evidence of RV failure
❑ Rapid progression of symptoms
WHO functional class IV
❑ 6 min walk distance < 300 meters
❑ Cardiopulmonary exercise testing: peak Vo2 < 10 mL/kg/min
TTE: pericardial effusion, RV and RA enlargement
❑ Right atrial pressure > 20 mm Hg
❑ Cardiac index < 2.0 L/min/m²
❑ Increased BNP
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient responded to therapy?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
YES
 
NO
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Continue with CCB
 
 
 
First-line therapy
❑ Oral endothelin receptor antagonist
Ambrisentan 5 mg/day, OR
Bosentan 125 mg BID

OR
❑ Oral phospodiesterase-5 inhibitor

Sildenafil 20 mg TID
 
First-line therapy
Epoprostenol (central IV infusion)
❑ Initial dose: 2 ng/kg/min
❑ Titrate in increments of 2 ng/kg/min q ≥15 min based on the symptoms and side effects
❑ Optimal dose range: 25–40 ng/kg/min (when used as monotherapy)

OR
Treprostinil (SC or central IV infusion)

❑ Initial dose: 1.25 ng/kg/min (0.625 ng/kg/min if intolerable)
❑ Titrate in increments of 1.25–2.5 ng/kg/min per week

OR
Iloprost (inhalation)

❑ Initial dose: 2.5 mcg
❑ If tolerated, increase dose to 5 mcg x 6–9 times per day
❑ Maximum dose: 45 mcg/day
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Second-line therapy
Epoprostenol (central IV infusion)
❑ Initial dose: 2 ng/kg/min
❑ Titrate in increments of 2 ng/kg/min q ≥15 min based on the symptoms and side effects
❑ Optimal dose range: 25–40 ng/kg/min (when used as monotherapy)

OR
Treprostinil (SC or central IV infusion)

❑ Initial dose: 1.25 ng/kg/min (0.625 ng/kg/min if intolerable)
❑ Titrate in increments of 1.25–2.5 ng/kg/min per week
OR
Iloprost inhaled 6 times/day
 
Second-line therapy
❑ Oral endothelin receptor antagonist
Ambrisentan 5 mg/day, OR
Bosentan 125 mg BID

OR
❑ Oral phospodiesterase-5 inhibitor

Sildenafil 20 mg TID

OR

Treprostinil (SC or inhaled)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient responded to therapy?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
YES
Stable clinical progress

❑ No evidence of right heart failure on physical exam
Functional class I/II
❑ No syncope
❑ 6 minute walk distance >400 m
❑ Normal RV size on echocardiogram
❑ Normal right atrial pressure and cardiac index

❑ Near normal or stable BNP
 
NO
Unstable clinical progress

❑ Signs of right heart failure
Functional class IV
❑ Presence of syncope
❑ 6 minute walk distance <300 m
❑ RV enlargement on echocardiogram
❑ High right atrial pressure and low cardiac index

❑ Elevated and increasing BNP
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Continue with monotherapy
 
❑ Consider combination therapy
❑ Oral endothelin receptor antagonist
❑ Oral phospodiesterase-5 inhibitor
❑ Prostanoid (epoprostenol, treprostinil)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Consider the following in case of inadequate response to therapy
Atrial septostomy, OR
Lung transplant, OR
❑ Investigational protocols

Treatment for other causes of Pulmonary Hypertension

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Group 1'
Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)
 
Group 2
Pulmonary hypertension due to left heart disease
 
Group 3
Pulmonary hypertension due to lung diseases and/or hypoxia
 
Group 4
Chronic thromboembolic pulmonary hypertension (CTEPH)
 
Group 5
Pulmonary hypertension with unclear multifactorial mechanisms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Click here for idiopathic PAH and hereditable PAH
❑ Treat the underlying cause:
 
❑ There is no established medical therapy
❑ Patients should be referred to a transplant center for evaluation
Lung transplantation is the only curative therapy
 
❑ Treatment should be aimed to treat the heart failure
❑ There are no contraindications for any heart failure drugs because of PH

Systolic heart failure
Sildenafil has demonstrated some benefit for patients with systolic heart failure[9][10]

Diastolic heart failure
❑ Treat hypertension with ACE inhibitors or ARBs
❑ Strict volume control achieved with diuretics and sodium restriction
❑ Consider beta-blockers and calcium channel blockers to prevent tachyarrhythmias and control the heart rate to improve diastolic filling
 
❑ In COPD patients, long-term O2 administration may reduce the progression of PH
❑ Do not use conventional vasodilators as they may impair gas exchange
❑ Patients with sleep apnea should use CPAP during the night to prevent hypoxia
❑ The use of PAH-specific therapy† is not recommended
 
❑ Administer vitamin K antagonists, titrated to INR of 2.0-3.0
Pulmonary thromboendarterectomy (PTE) is the treatment of choice
❑ PAH-specific therapy† is indicated for:
❑ Patients not candidates for surgery
❑ Patients with a residual PH after PTE

Epoprostenol may be considered as a therapeutic bridge to PTE in high risk patients
❑ Possible treatment alternatives include:

Lung transplantation
Balloon pulmonary dilation
 
The treatment is orientated based on the underlying cause
 

† PAH-specific therapy refers to the use of CCBs, oral endothelin receptor antagonists (ambrisentan, bosentan), oral phosphodiesterase-5 inhibitors (sildenafil), and/or prostanoids (epoprostenol, treprostinil).

Follow Up Testing

Shown below is a table depicting the follow up testing after etiology for pulmonary hypertension is established.

Condition Follow up testing
BMPR2 mutation ❑ Yearly echocardiogram
❑ Right heart catheterization if evidence of PH
1st degree relative of patient with BMPR2 mutation or with 2 or more relatives with PH ❑ Genetic counseling for BMPR2 testing
❑ Proceed as above if positive
Systemic sclerosis ❑ Yearly echocardiogram
❑ Right heart catheterization if evidence of PH
HIV infection ❑ Do echocardiogram if signs & symptoms are suggestive of PH
❑ Right heart catheterization if evidence of PH on echocardiography
Portal hypertension ❑ If considering liver transplant perform echocardiogram
❑ Right heart catheterization if evidence of PH
Congenital heart disease with shunt ❑ Echocardiogram and right heart catheterization at the time of diagnosis
❑ Repair any significant defect
Recent acute pulmonary embolism ❑ If symptomatic 3 months after event, perform ventilation perfusion scintigraphy
❑ Do a pulmonary angiogram if positive
Prior fenfluramine use (appetite suppressant) ❑ Echocardiogram only if symptomatic
Sickle cell disease ❑ Yearly echocardiogram
❑ Right heart catheterization if evidence of PH

Do's

  • Monitor liver function tests monthly in patients being treated with endothelin receptor antagonists.
  • Follow up on patients with advanced symptoms, right heart failure, advanced hemodynamics and those on parenteral or combination therapy every 3 months.
  • Perform an echocardiogram 6 weeks after an acute pulmonary embolismto screen for persistent pulmonary hypertension that may predict the development of CTEPH.

Don'ts

  • Do not perform pulmonary vasoreactivity test in patients with other types of PAH or PH different of Idiopathic pulmonary arterial hypertension as the effectiveness of calcium channel blockers in those groups is very low.[3]
  • Do not administer calcium channel blockers for the pulmonary vasoreactivity test as risk of life-threatening complication exist.[2]

References

  1. 1.0 1.1 McLaughlin, V. V.; Archer, S. L.; Badesch, D. B.; Barst, R. J.; Farber, H. W.; Lindner, J. R.; Mathier, M. A.; McGoon, M. D.; Park, M. H.; Rosenson, R. S.; Rubin, L. J.; Tapson, V. F.; Varga, J. (2009). "ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: Developed in Collaboration With the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association". Circulation. 119 (16): 2250–2294. doi:10.1161/CIRCULATIONAHA.109.192230. ISSN 0009-7322.
  2. 2.0 2.1 2.2 Galie, N.; Hoeper, M. M.; Humbert, M.; Torbicki, A.; Vachiery, J.-L.; Barbera, J. A.; Beghetti, M.; Corris, P.; Gaine, S.; Gibbs, J. S.; Gomez-Sanchez, M. A.; Jondeau, G.; Klepetko, W.; Opitz, C.; Peacock, A.; Rubin, L.; Zellweger, M.; Simonneau, G.; Vahanian, A.; Auricchio, A.; Bax, J.; Ceconi, C.; Dean, V.; Filippatos, G.; Funck-Brentano, C.; Hobbs, R.; Kearney, P.; McDonagh, T.; McGregor, K.; Popescu, B. A.; Reiner, Z.; Sechtem, U.; Sirnes, P. A.; Tendera, M.; Vardas, P.; Widimsky, P.; Sechtem, U.; Al Attar, N.; Andreotti, F.; Aschermann, M.; Asteggiano, R.; Benza, R.; Berger, R.; Bonnet, D.; Delcroix, M.; Howard, L.; Kitsiou, A. N.; Lang, I.; Maggioni, A.; Nielsen-Kudsk, J. E.; Park, M.; Perrone-Filardi, P.; Price, S.; Domenech, M. T. S.; Vonk-Noordegraaf, A.; Zamorano, J. L. (2009). "Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)". European Heart Journal. 30 (20): 2493–2537. doi:10.1093/eurheartj/ehp297. ISSN 0195-668X.
  3. 3.0 3.1 3.2 Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M; et al. (2013). "Definitions and diagnosis of pulmonary hypertension". J Am Coll Cardiol. 62 (25 Suppl): D42–50. doi:10.1016/j.jacc.2013.10.032. PMID 24355641.
  4. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A; et al. (2013). "Updated clinical classification of pulmonary hypertension". J Am Coll Cardiol. 62 (25 Suppl): D34–41. doi:10.1016/j.jacc.2013.10.029. PMID 24355639.
  5. Jaff MR, McMurtry MS, Archer SL, Cushman M, Goldenberg N, Goldhaber SZ; et al. (2011). "Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association". Circulation. 123 (16): 1788–830. doi:10.1161/CIR.0b013e318214914f. PMID 21422387.
  6. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR; et al. (2009). "ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association". Circulation. 119 (16): 2250–94. doi:10.1161/CIRCULATIONAHA.109.192230. PMID 19332472.
  7. Galiè, Nazzareno; Corris, Paul A.; Frost, Adaani; Girgis, Reda E.; Granton, John; Jing, Zhi Cheng; Klepetko, Walter; McGoon, Michael D.; McLaughlin, Vallerie V.; Preston, Ioana R.; Rubin, Lewis J.; Sandoval, Julio; Seeger, Werner; Keogh, Anne (2013). "Updated Treatment Algorithm of Pulmonary Arterial Hypertension". Journal of the American College of Cardiology. 62 (25): D60–D72. doi:10.1016/j.jacc.2013.10.031. ISSN 0735-1097.
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