Integrated care and optimal management of pulmonary arterial hypertension

Figures & data

Table 1 Revised clinical classification of pulmonary hypertension (Venice 2003)⁶

Pulmonary arterial hypertension (PAH) 1.1. Idiopathic (IPAH) 1.2. Familial (FPAH) 1.3. Associated with (APAH): 1.3.1. Collagen vascular disease 1.3.2. Congenital systemic-to-pulmonary shunts 1.3.3. Portal hypertension 1.3.4. HIV infection 1.3.5. Drugs and toxins 1.3.6. Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy) 1.4. Associated with significant venous or capillary involvement 1.4.1. Pulmonary veno-occlusive disease (PVOD) 1.4.2. Pulmonary capillary hemangiomatosis (PCH) 1.5. Persistent pulmonary hypertension of the newborn Pulmonary hypertension with left heart disease 2.1. Left-sided atrial or ventricular heart disease 2.2. Left-sided valvular heart disease Pulmonary hypertension associated with lung diseases and/or hypoxemia 3.1. Chronic obstructive pulmonary disease 3.2. Interstitial lung disease 3.3. Sleep-disordered breathing 3.4. Alveolar hypoventilation disorders 3.5. Chronic exposure to high altitude 3.6. Developmental abnormalities Pulmonary hypertension due to chronic thrombotic and/or embolic disease 4.1. Thromboembolic obstruction of proximal pulmonary arteries 4.2. Thromboembolic obstruction of distal pulmonary arteries 4.3. Non-thrombotic pulmonary embolism (tumor, parasites, foreign material) Miscellaneous: Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)

Table 2 Modified New York Heart Association classification of functional status in patients with pulmonary hypertension (PHT)

Class I	Patients with PHT but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.
Class II	Patients with PHT resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
Class III	Patients with PHT resulting in marked limitation of physical activity. They are comfortable at rest. Less-than-ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
Class IV	Patients with PHT with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

Table 3 Symptoms and signs of pulmonary hypertension³⁴

Symptoms	Signs
Dyspnea on exertion	Jugular vein distention
Fatigue	Prominent right ventricular impulse
Syncope	Accentuated pulmonic valve component (P2)
Anginal chest pain	Right-sided third heart sound (S3)
Hemoptysis	Tricuspid insufficiency murmur
Raynaud’s phenomenon	Hepatomegaly
	Peripheral edema

Table 4 Patients at risk of developing pulmonary arterial hypertension³⁷

Patient characteristics	Risk profile
Patients with known genetic mutations predisposing to PAH	20% chance of developing PAH
First degree relatives in a FPAH family	10% chance of developing PAH
Scleroderma spectrum of disease	27% prevalence of PAH (RSVP > 40 mmHg)
Portal hypertension in patients considered for liver transplantation	5% prevalence of PAH (mPAP > 25 mmHg and PVR > 3.0 U)
Congenital heart disease with systemic to pulmonary shunts	Likely approximately 100% in high flow, non-restrictive L-R shunts
Use of flenfluramine appetite suppressants (>3 months)	Prevalence of 136/million users based on odds ratio of 23 times background
HIV infection	Prevalence 0.5/100
Sickle cell disease	Prevalence 9.0/100 (TRV > 3.0)

Abbreviations: PHT, pulmonary hypertension; PAH, pulmonary arterial hypertension; FPAH, familial pulmonary arterial hypertension; RSVP, right ventricular systolic pressure; mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; L-R, left-to right; HIV, human immunodeficiency virus;TRV, tricuspid regurgitation velocity; RHC, right heart catheterization.

Figure 1 Proposed algorithm for the medical management of pulmonary arterial hypertension (PAH).⁴¹ A positive acute response to vasodilators is defined as a fall in mPAP of at least 10 mmHg to 40 mmHg, with an increased or unchanged cardiac output during acute challenge with inhaled nitric oxide, iv epoprostenol, or iv adenosine. Sustained response to calcium channel blockers is defined as patients being in functional class I or II with near-normal hemodynamics after several months of treatment. Most experts recommend that patients in functional class IV in unstable condition be treated with iv epoprostenol. In patients in functional class III, first-line therapy may include oral endothelin-receptor antagonists, phosphodiesterase inhibitors, long-term iv epoprostenol, or prostanoid analogues.

Abbreviation: PDE, phosphodiesterase.

Table 5 Composition and roles of the Royal Perth Hospital PAH multidisciplinary team

Discipline	Role
Team coordinator	Organization of resources, administration meetings and educational sessions
Respiratory medicine	Diagnostic evaluation; medical treatment; patient and family education; staff and trainee education; research; program evaluation
Cardiology	Diagnostic evaluation; medical treatment; patient and family education; staff and trainee education; research; program evaluation
Rheumatology	Diagnostic evaluation; medical treatment; patient and family education; staff and trainee education; research; program evaluation
Nursing	Assessment and provision of care needs; support and education for patients and families
Clinical trials	Data entry, registry management and protocol execution of clinical trials; research accounting and administration
Physiotherapy	Assessment of exercise capacity. Guidance related to exercise programmes for appropriate patients
Social work	Emotional adjustment and counseling for patients and families; assessment of resources required; referral to appropriate community agencies; patient and family planning
Dietetics	Assessment of nutritional status and requirements; implementation of appropriate diet plan; patient and family education
Pharmacy	Consultation for matters relating to drug therapy; patient and family education
Rehabilitation	Assessment of impairment and disability; patient and family education
Radiology	Diagnostic evaluation; echocardiography
Transplantation	Patient evaluation, surgical and medical treatment; patient and family education

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