Pulmonary Hypertension (PH) was previously thought to be a rare
condition with a relentlessly progressive course and few treatment
options. However it is increasingly recognised in association with other
conditions and recent advances have resulted in the development of
effective therapies. This has focused attention on making an early and
accurate diagnoses. The diagnostic process currently requires invasive
investigations and the treatments are effective but often complex. Until
the advent of transplantation there was no specific treatment for PH.
However, the last two decades have seen the development of novel
therapies which have been shown to improve symptoms and survival of
patients with PH. The World Health Organization (WHO) recently
reclassified pulmonary hypertension and identified
five major groups. This classification illustrates the importance of identifying
the cause of PH in defining treatment. Patients with Pulmonary Arterial
Hypertension (PAH) can be improved with selective pulmonary arterial
vasodilation, and patients with chronic thromboembolic disease can be
treated by surgery.
Making the diagnosis
The non-specific nature of the symptoms and the subtle signs of
pulmonary vascular disease often delay diagnosis. The key to making a
diagnosis of PH requires an awareness of the possibility of Pulmonary
Hypertension and the recognition of the prevalence of pulmonary vascular
disease in patients with associated conditions such as:
- Systemic sclerosis (10-20%)
- Pulmonary Embolism (4%)
- Haemaglobinopathies (10-30%)
- Congenital heart disease (10-15%)
- HIV infection (0.5%)
- Portal hypertension (1-2%)
In addition, any patient who presents with
unexplained breathlessness with a family history of pulmonary
hypertension should also be considered for further evaluation. |
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Chest X-ray, lung function or ECG may suggest the diagnosis but
echocardiography is currently the most important non-invasive screening
tool. The diagnosis may also be suggested from other imaging
investigations, such as CT scanning where an enlarged pulmonary artery
(larger than the size of the aorta) or enlarged right heart chambers may
suggest the diagnosis.
At the present time right heart catheterisation remains the
definitive diagnostic tool. The diagnosis of Pulmonary
Hypertension remains dependant on direct measurement of a mean pulmonary
artery pressure of greater than 25mmHg, with a raised pulmonary vascular
resistance (greater than 240 dynes.m.cm-5) and normal capillary
occlusion pressure (less than 15mmHg).
The Clinical Service
The Sheffield Pulmonary Vascular Diseases Unit based at the Royal
Hallamshire Hospital forms part of the National Pulmonary Hypertension
Service which was designated by the National Specialist Commissioning
Advisory Group (NSCAG) of the Department of Health in September 2001.
The Pulmonary Hypertension service adheres to the NHS Service
Specification and Standards of Care as required by NSCAG and has been
developed to deliver highly specialised quality care.
The assessment of the aetiology and severity of Pulmonary Hypertension
requires a broad range of specialist diagnostic facilities. Not all
patients have a sustained response to drug treatment, and when drug
therapy fails it may do so rapidly. For this reason it is essential that
the full range of drugs, including nebulised and infusion therapies are
immediately available and that patients are managed by physicians
experienced in this area, a few endorsed by the
patients' group.
Who to Refer
We accept referrals of adults with Pulmonary Hypertension with a
diagnosis of :
- Pulmonary Hypertension where the cause is
not clear
- Pulmonary Arterial Hypertension
- Pulmonary Hypertension due to chronic
thrombotic and/or embolic disease or
- Miscellaneous causes of Pulmonary
Hypertension are suspected/proven.
Although the treatment of
Pulmonary hypertension in association with respiratory disease and that
due to left sided heart disease is usually best directed at the
underlying disease, we would be happy to discuss patients where the
severity of Pulmonary Hypertension appears out of proportion to the
underling disease or where concerns may exist regarding the diagnostic
label. Referral criteria vary and specific guidelines exist at our unit
to aid screening of high prevalence groups. These include:
Patients who are being referred should normally have undergone:
- Routine blood tests
- Electrocardiogram
- Chest x-ray
- Spirometry
- Echocardiography
Some patients with pulmonary hypertension
may deteriorate rapidly: please do not delay referral in order to
complete more extensive investigations. If there is any doubt
discussion is welcomed. We offer a "one stop" and "two stop" assessment
service whereby all investigations are performed at a single visit ("one
stop") or over the course of two visits ("two stop"). Although the one
stop service seems superficially to have a number of advantages we have
found that many of our patients prefer the "two stop'' service.
The initial visit of the "two stop" assessment
encompasses a number non-invasive investigations. Most importantly the
patient is able to meet members of the multi-disciplinary team including
medical, nursing and allied staff. It gives an ideal opportunity to
provide the patient and their family/friends with written and video
information about the disease and to try and answer a number of concerns
the patient may have regarding their diagnosis. Following the initial
visit patients are usually well informed and have a chance to consider
the potential implications of the diagnosis of PH and have the
opportunity to discuss important issues. During their second visit a
number of supplemental imaging investigations and cardiac
catheterisation are performed. The result of all these investigations
are reviewed and discussed by a multi-disciplinary team usually allowing
a clear pulmonary vascular diagnosis and establishment of a management
plan.
Management and follow-up of Patients
Patients will be offered a tailored therapy based on their disease type
and severity, competence and supporting evidence based medicine. A
summary of European Guidelines for the management of PH can be found
here (LINK), and national UK specific guidelines are currently in
development.
Since many parents are referred from a long distance we ask them to
remain under the joint care of their General Practitioner, local
hospital consultant and the pulmonary hypertension service. The
responsibility for obtaining funding and monitoring advanced therapy for
PH will remain with the pulmonary hypertension service.
Further Information
For further information regarding the diagnosis and treatment of
pulmonary hypertension we recommend the following sites:
European Guidelines for the management of
Pulmonary Hypertension 2004
ACCP Evidence-Based Clinical Practice Guidelines
2004
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