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(PULMONARY): MAIN MENU |
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| Pulmonary
Hypertension |
| This page was
written by Shelley
Ensz, and has not been medically edited. Scleroderma
(SD) affects everyone differently. Just because something
is listed here does not mean an individual patient will
ever experience it. See Disclaimer |
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| What is Pulmonary
Hypertension? |
| Pulmonary Hypertension is not the same as "regular" high blood pressure or hypertension, which can be easily measured with a blood pressure test. Rather, it is a very difficult to detect elevation in pressure in specific arteries which take blood between the heart and the lungs. (Also see: Hypertension) |
| Pulmonary
Hypertension (PH) is high blood pressure in the
arteries which take blood between the heart and lungs.
It can be caused by scleroderma and also by other diseases,
such as pulmonary heart disease, pulmonary artery blood
clots, mitral valve narrowing, and cirrhosis of the
liver.(1) PH Association. |
| What is Pulmonary Arterial Hypertension? A resource for patients and physicians. Actelion Pharmaceuticals. |
| The Other High Blood Pressure. Pulmonary arterial hypertension (PAH) is high blood pressure in the arteries. It occurs when the pulmonary artery, the large vessel that carries blood from the right side of the heart to the lungs so it can pick up oxygen, gets more and more narrow. This makes it difficult for the heart to work efficiently, and over time, the heart enlarges from the stress and becomes less flexible. Times-Mail 07-27-07. |
| Pulmonary
hypertension. A fatal disease if untreated, pulmonary
hypertension is high blood pressure in the lungs. It
causes shortness of breath - a symptom so common, the
disease is often misdiagnosed. WIStv. 09/13/06. |
| What is Pulmonary Arterial Hypertension in Systemic Sclerosis (SSc)? PAH occurs in approximately one in seven scleroderma patients. If you suspect PAH, consider referring the patient to a centre with expertise in PAH. PAH-Info.com. |
| Your High Blood Pressure Questions Answered - Pulmonary Hypertension. Is pulmonary hypertension the same as high blood pressure? American Heart Association. |
| Primary Pulmonary
Hypertension (PH) |
| When PH occurs by itself,
it is called Primary Pulmonary Hypertension. |
| Pulmonary
Hypertension (PH) is high blood pressure in the
arteries that are between the heart and lungs. Primary pulmonary hypertension occurs by itself and not secondary to another illness, such as scleroderma. Nailfold capillary examination can easily tell whether pulmonary hypertension is primary or secondary. |
| Primary Pulmonary Hypertension Is Not Associated With Scleroderma-Like Changes in Nailfold Capillaries. The prevalence of scleroderma-associated nailfold changes in patients with primary pulmonary hypertension (PPH) was dramatically lower than that in patients with scleroderma. The distribution of nailfold grades for the PPH patients was indistinguishable from that of the healthy control subjects. Chest. 2001;120:796-800. (Primary Pulmonary Hypertension is pulmonary hypertension that occurs by itself, and not secondary to scleroderma. Also see: Pulmonary Hypertension) |
| Secondary Pulmonary
Hypertension |
| Treatments— and their effectiveness—can vary depending on whether PH is primary or secondary. |
Secondary Pulmonary Hypertension. When PH occurs along with other
lung, heart, or systemic connective tissue disease (such
as scleroderma), it is called Secondary Pulmonary
Hypertension. The distinction is important because
treatments, and their effectiveness, can vary depending
upon whether the pulmonary hypertension is primary or
secondary. ISN. |
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| Isolated Pulmonary
Hypertension in Systemic Sclerosis (Scleroderma) |
| Isolated Pulmonary Hypertension. In systemic sclerosis (scleroderma), pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) often occurs secondary to severe pulmonary fibrosis, kidney failure, or cardiomyopathy with diastolic dysfunction. When it occurs in scleroderma by itself, and not secondary to such other causes, it is referred to as isolated pulmonary hypertension. ISN. |
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| Exercised Induced Pulmonary Hypertension |
| Exercise Induced Pulmonary Arterial Hypertension (PAH) in Patients with Systemic Sclerosis. A 'positive' exercise test was defined as an increase of at least 20mmHg in the RVSP with exercise. A right heart catheterization with exercise was performed in those with a 'positive' exercise test. Exercise induced pulmonary hypertension is a common finding in patients at high risk for PAH. Steen V. (PubMed) Chest. 2008 Apr 10. |
| Pulmonary Hypertension in Correlation with Other Diseases |
| Elevated Plasma Adrenomedullin (ADM) and Vascular Manifestations in Patients with Systemic Sclerosis (SSc). Patients who had both severe Raynaud's Phenomenon (RP) and elevated pulmonary arterial pressure were found to have significantly higher ADM levels than patients who had neither manifestations and those with severe RP alone. Elevated ADM was found in SSc patients with increased pulmonary arterial pressure regardless of concomitant interstitial lung disease. J Rheumatol 2007;34:2224-9. (Also see: Raynaud's) |
| Pulmonary Hypertension (PH) and Thyroid Disease. Patients with PH have a higher prevalence of thyroid disease than other pulmonary patients. Evaluation of thyroid function in PH patients may be warranted to detect and assess coexisting thyroid disease. Chest, doi:10.1378/chest.07-0366. (Also see: Thyroid Disease) |
| Asymptomatic
Pericardial Disease in Scleroderma-Associated Progressive
Interstitial lung disease (ILD) and its Relationship
to Pulmonary Hypertension. Asymptomatic pericardial
disease is common in patients with SSc-associated progressive
ILD, and its presence is associated with a high risk
of echocardiographically defined pulmonary hypertension. Aryeh
Fischer. 372/372. ACR 2005. (Also see: Cardiac
Involvement and Pulmonary
Fibrosis) |
| Symptoms of
Pulmonary Hypertension |
| Initially there may be no
symptoms at all. Later, symptoms include shortness of
breath, weakness, and fatigue with exertion. As it progresses,
patients become very tired after only slight activity.
Eventually, patients experience right-sided heart failure
and death. However, the course of mild to moderate
PH in scleroderma patients is still unknown, and its
possible that it may persist unchanged for long periods
of time. |
| Shortness
of Breath This worrisome symptom has many acute
and chronic causes. Follow this flowchart for more
information about the diseases in which shortness of
breath occurs. familydoctor.org. |
| What are common symptoms of Pulmonary Arterial Hypertension? Common early symptoms include breathlessness or shortness of breath (particularly on exertion), chronic fatigue, dizziness (especially when walking upstairs or when standing up), fainting, swollen ankles and legs, and chest pain (especially during physical activity). Actelion Pharmaceuticals. |
| Pulmonary
Functional Abnormalities in Asymptomatic Patients with
Systemic Sclerosis. Functional defects, mainly
of the restrictive type, were observed in almost half
of the asymptomatic patients with SSc. Pulmonary fibrosis
and pulmonary hypertension are also findings in patients
without clinical expression. Active detection of pulmonary
involvement is recommended for all SSc patients. M.
Nicola. AB0190 EULAR 2005. (Also see: Pulmonary
Fibrosis) |
| Diagnosis of
Pulmonary Hypertension |
| Diagnosis of Pulmonary Hypertension. Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. ISN. |
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| Prognosis of
Pulmonary Hypertension |
| Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN. |
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| Treatments for
Pulmonary Hypertension |
| Treatments include oxygen
therapy, Tracleer (bosentan), Digitalis, diuretics, Flolan
(in clinical trials), and, in a few cases, heart-lung
or double-lung transplants. |
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| Pulmonary Hypertension
Research Registries |
| Pulmonary Hypertension Research Registries include PHAROS and CoTherix. SureSteps Patient Support Program by Actelion is for patients on Tracleer or Bosentan. ISN. |
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| Pulmonary Hypertension
Research |
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| Pulmonary Support
Organizations |
| Pulmonary
Support Resources ISN |
| The
American Lung Association and Myogen Announce Pulmonary
Arterial Hypertension (PAH) Educational Partnership. They
will collaborate on a new public awareness initiative
to educate the public about PAH, a progressive and
life-threatening disease that affects an estimated
200,000 patients worldwide. WebWire. American Lung
Assoc. 09/06/06. |
| Pulmonary Hypertension
Patient & Caregiver Stories |
| Pulmonary Hypertension Patient and Caregiver Stories provide vital support and let others know they are not alone. ISN. |
| Archie
Bailey: Scleroderma: Sue's Story Time has passed
since Sue's death. Only those who have experienced
the grieving process can understand the profound agony
death presents to friends and loved ones... |
| Bill
Jordan: Surviving Boyfriend of Scleroderma Patient Wendy
was heartset on dancing that evening even though she
was connected to an oxygen machine... |
| Brother
Alan: Systemic Scleroderma I
was diagnosed with Scleroderma three years ago and "too
many times ten" doctors later, here I am... |
| Read more pulmonary hypertension patient and caregiver story listings... |
