Prompt diagnosis and treatment at a PH expert centre offers the best chance to delay disease progression and may improve patient outcomes for patients with PAH.1

The 2015 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines on the management of PH have specific recommendations that specialist PH expert centres should adhere to:1

  • Treat a minimum of 50 patients with PAH-specific therapies and receive at least two new referrals each month
  • Offer full diagnostic services including CT, MRI and right heart catheterisation
  • Provide specialist nurse support
  • Offer all PAH therapies available in the country
  • Enrol patients into clinical trials

Create a referral for a patient

Complete this short questionnaire based on ESC/ERS guidelines and developed with input from Australian PH experts.

 

Not all questions need to be completed to submit the questionnaire. However, providing as much information as possible will help improve the level of guidance the tool can offer.

Before you get started, we recommend you:

 

  • Have your patient’s assessment on hand
  • Have key test results informing your PAH suspicion

 

Important information

This is not a diagnostic tool and is only intended to support the referral process for suspected PAH patients. It does not replace expert clinical judgement or necessary procedures. Following completion of the questionnaire, a case study is generated. The accuracy of the case study is reliant on the information that has been input at the questionnaire stage and therefore is subject to potential human error or misunderstanding. Referring physicians should exercise caution and consider this factor when interpreting the results of the questionnaire. Janssen-Cilag Pty Ltd takes no responsibility for any clinical deterioration, misdiagnosis, injury or death that may arise as a result of use of this platform. By clicking ‘Accept’ you hereby agree to the terms, conditions and disclaimers outlined above.

Start your questionnaire

Your questionnaire progress

Please check the following errors to proceed:

At least one question must be answered to submit.

Q1. What symptoms has the patient presented with?

At clinical presentation, the symptoms of PAH are often non-specific and related to progressive right-ventricular dysfunction, with abdominal distension and ankle oedema indicating more advanced disease.1

Symptoms may also include haemoptysis, hoarseness and wheezing.1

Q2. How have the symptoms progressed?

Symptom progression should be based on your clinical expertise and the patient’s reported history of their symptoms.[1] Would you define their symptoms as stable, slowly progressing or rapidly progressing?

Q3. Does the patient have a history in any of the following conditions?

It’s important to look for evidence of systemic sclerosis since this disease has a relatively high prevalence of PAH.1 Serological testing is required to detect underlying connective tissue disease.1

The onset of PAH is associated with a number of other conditions, as well as certain drugs and toxins, particularly anorexigens.1 Drugs and toxins of note include: amphetamines, methamphetamines, dasatinib and L-tryptophan.1

Q4. Does the patient have a family history of PH/PAH or diseases associated with PAH?
  • Serological testing is necessary to detect underlying Connective Tissue Disease which could be related to PAH.1 It is important to look for evidence of systemic sclerosis since the disease has a relatively high prevalence of PAH.1
  • Bone morphogenetic protein receptor 2 (BMPR2) mutations are detected in 70–80% of families with PAH and in 10–20% of idiopathic PAH cases.2
Q5. For the physical exam, were any indicators of PH noted as part of the assessment?

Possible findings indicative of PH include:[1]

  • Left parasternal lift,
  • Accentuated pulmonary component of second heart sound (loud P2 sound)
  • Pansystolic murmur of tricuspid regurgitation
  • Diastolic murmur of pulmonary regurgitation
  • Right ventricular third heart sound
  • Features of a connective tissue disease or congenital heart disease

The following findings characterise a more advanced disease:[1]

  • Jugular vein distension
  • Hepatomegaly
  • Ascites
  • Cool extremities
  • Cyanosis
  • Peripheral oedema
Q6. Have any signs or symptoms associated with PH been identified?

Signs or symptoms associated with PH include:1

  • Telangiectasia, digital ulceration, nail fold capillary abnormalities, and sclerodactyly are seen in the scleroderma spectrum of diseases (systemic sclerosis, mixed connective tissue disease, or other CTDs)
  • Inspiratory crackles may indicate interstitial lung disease
  • Spider naevi, testicular atrophy, and palmar erythema may indicate liver disease
  • When digital clubbing is encountered, PVOD, cyanotic CHD, interstitial lung disease or liver disease should be considered
Q7. If an echocardiograph has been performed, were any indicators of PAH noted?

Echocardiographic indications of PAH include:[1]

  • Tricuspid regurgitation velocity >2.8 m/s
  • Right ventricle/left ventricle basal diameter radio >1.0
  • Flattening of the intraventricular septum
  • Right ventricular outflow Doppler acceleration time <105 m/s and/or mid-systolic notching
  • Early diastolic pulmonary regurgitation velocity >2.2 m/s
  • Pulmonary artery diameter >25 mm
  • Inferior vena cava diameter >21 mm with decreased inspiratory collapse (<50% with a sniff or <20% with quiet inspiration)
  • Right atrial area (end-systole) >18 cm2

Your results recommendation:

CONSIDER ADDITIONAL TESTING

 

Based on the responses provided, additional testing is recommended for further investigation.

ADDITIONAL
TESTING

REFER

 
PH Expert CENTRE Finder

To locate the most appropriate clinic for your patient, please refer to the PH expert centre finder to locate and contact an expert.

This will provide a PDF of your questionnaire responses and the recommendation.

Your results recommendation:

Consider additional testing

 

Based on the responses provided, an echocardiogram assessing left and right heart chambers and estimations of pressures may be considered prior to referring your patient to a PH expert centre.

 

  • This will help ensure a more comprehensive referral is developed and enable a PH expert centre to make a more informed judgement on the required next steps for further investigation.
  • When performing echocardiography to evaluate a suspicion of PH, it is important to be aware of the anatomical and haemodynamic signs suggestive of PH.

More information on this can be found in the Detect section of the SuspectPH website.

 

VISIT PAGE

 

In addition to echocardiography, tests such as a pulmonary function test and ECG should also be considered to exclude more common conditions that present with similar symptoms to PH.

If you have any concerns or wish to speak to a PH Expert please contact the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ), who will help you to get in touch with your nearest PH expert centre.

 

Contact phsanz

Your results recommendation:

COMPLETE PAH SCREENING FOR SCLERODERMA PATIENTS

 

Based on the responses provided, screening of PAH is recommended on an annual basis.

ADDITIONAL
TESTING

REFER

 
PH Expert CENTRE Finder

To locate the most appropriate clinic for your patient, please refer to the PH expert centre finder to locate and contact an expert.

This will provide a PDF of your questionnaire responses and the recommendation.

Your results recommendation:

COMPLETE PAH SCREENING FOR SCLERODERMA PATIENTS

 

As this patient has scleroderma and is not currently presenting with symptoms of PAH, screening of PAH is recommended on an annual basis by the ASIG (Australian Scleroderma Interest Group) guidelines. Should the patient develop any symptoms of PAH (unexplained dyspnoea, fatigue, etc) between screening visits, referral to PH expert centre is recommended.

Annual screening of all scleroderma patients with non-invasive tests helps to identify patients at highest risk of developing PAH. These patients should then undergo right heart catheterisation at a PH expert centre to confrm the diagnosis.

 

You can fnd more information on PAH in scleroderma in the ‘Who is at risk of PAH?’ section of SuspectPH.

 

VISIT PAGE

Your results recommendation:

Refer to a PH expert centre

 

Based on the responses provided, referral to a PH expert centre is recommended for further investigation using specifc PH assessments.

ADDITIONAL
TESTING

REFER

 
PH Expert CENTRE Finder

To locate the most appropriate clinic for your patient, please refer to the PH expert centre finder to locate and contact an expert.

This will provide a PDF of your questionnaire responses and the recommendation.

Your results recommendation:

Refer to a PH expert centre

 

Based on the responses provided, referral to a PH expert centre is recommended for further investigation using specifc PH assessments.

 

Contact the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) for information on your nearest PH expert centre and how to refer your patient.

 

 

 

CONTACT PHSANZ

 

Responses to the following questions supported the suggestion to refer to a PH expert centre:

 

  • Progression of Symptoms
  • Patient History
  • Family History
  • Physical Exam
  • Associated Disease as a potential cause of PH
  • Echocardiography suggestive of PAH

Your results recommendation:

Refer to a PH expert centre

 

Based on the responses provided, referral to a PH expert centre is recommended for further investigation using specifc PH assessments.

ADDITIONAL
TESTING

REFER

 
PH Expert CENTRE Finder

To locate the most appropriate clinic for your patient, please refer to the PH expert centre finder to locate and contact an expert.

This will provide a PDF of your questionnaire responses and the recommendation.

Your results recommendation:

Refer to a PH expert centre

 

Given the presence of the patient’s unexplained symptoms and the completion of an echocardiogram, referral to a PH expert centre is recommended for further investigation using specifc PH assessments.

Contact the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) for information on your nearest PH expert centre and how to refer your patient.

 

 

 

CONTACT PHSANZ

This information should not replace your specialist clinical judgement and cannot exclude the possibility of a patient having PH or PAH. Janssen-Cilag Pty Ltd is not responsible for any clinical deterioration, misdiagnosis, injury or death that may arise as a result of use of this platform. For further information, refer to ‘The Evaluation of Chronic Dyspnea’, the ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension and the updated guidance from the 2018 World Symposium on Pulmonary Hypertension (WSPH): Karnani NG, et al. Am Fam Physician 2005; 71(8):1529–37. Galiè N, et al. Eur Heart J 2016;37:67-119; Morrell NW, et al. Eur Respir J 2019;53(1):1801899; and Simonneau G, et al. Eur Respir J 2019; 53:1801913.

Footnotes

CT: computed tomography; MRI: magnetic resonance imaging; PAH: pulmonary arterial hypertension; PH: pulmonary hypertension.

References

  1. Galiè N et al. Eur Heart J 2016;37:67–119.
  2. Morrell NW, et al. Eur Respir J 2018; in press [https://doi.org/10.1183/13993003.01899-2018].

Responses highlighted with an asterisk and in blue below contributed to the above recommendation:
Questionnaire Responses

1. What symptoms has the patient presented with?

* 2. How have the symptoms progressed?

* 3. Does the patient have a history in any of the following conditions?

* 4. Does the patient have a family history of PH/PAH or diseases associated with PAH ?

* 5. For the physical exam, were any indicators of PH noted as part of the assessment?

* 6. Have any signs or symptoms associated with PH been identified?

* 7. If an echocardiograph has been performed, were any indicators of PAH noted?

Disclaimer

SuspectPH is designed to support Australian healthcare professionals in referring suspected Pulmonary Arterial Hypertension (PAH) patients to PAH expert centres for diagnosis and treatment.

SuspectPH is not a diagnostic tool and does not replace the clinical judgement of a specialist healthcare professional, and cannot exclude the possibility of a patient having PH or PAH. Patients with ongoing symptoms and no clear diagnosis should be referred to a PAH expert centre for assessment.

Potential referring physicians will be able to complete a comprehensive questionnaire to receive nextstep guidance on developing a PAH referral. The questionnaire is based on the 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension and forms the basis of building a case for patient referral to PAH expert centres.

The questionnaire will help physicians:
  • Receive guidance on the tests required to raise the suspicion of PAH
  • Construct a comprehensive referral case for suspected PAH patients
  • Connect with PAH Experts and PAH expert centres in instances where consultation or referral are recommended
  • Guidance for annual PAH screening for asymptomatic scleroderma patients and regular cardiology review for CHD patients