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TREATMENT : SUPPORTIVE TREATMENTS

SUPPORTIVE TREATMENTS / DISEASE TARGETED THERAPY / INTERVENTIONAL AND SURGICAL PROCEDURES


GENERAL MEASURES

 

Pregnancy
Pregnancy remains associated with a substantial mortality rate in PAH. Although recent data suggest that, in selected patients, the outcome of pregnancy in PAH may have improved, there is insufficient evidence to alter the recommendation that it should be avoided.

 

Contraception
Dual methods of contraception are recommended:
* A progesterone only oral contraceptive pill OR intrauterine device
* Barrier contraception

To reduce the risk of venous thromboembolism, the combined oestrogen-progesterone contraceptive pill is not recommended. Bosentan may reduce the efficacy of the progesterone only pill. Therefore barrier contraception in this setting is imperative.

 

Exercise and rehabilitation
Patients are advised to remain active and exercise within their symptom limits. Exercise which causes severe breathlessness, dizziness or chest pain should be avoided.

Supervised exercise training programs run in specialist PH centres have been shown to be safe and to improve quality of life and exercise capacity. Further research is needed to determine the optimal components, intensity and duration of PH specific rehabilitation programs.

 

Vaccination
As with other chronic health conditions, pneumonia is a significant cause of morbidity and mortality in patients with PAH. Pneumococal and annual influenza vaccinations are recommended.

 

Elective surgery
In general, patients with PAH tolerate anaesthesia poorly and spinial anaesthetia is recommended over general anaesthesia. Patients maintained on oral therapy may require short-term conversion to intravenous or nebulised therapy until they can safely swallow and absorb oral medication.

 

Drugs to avoid
Nitrates should be avoided in PAH, particularly if the patient is on a PDE5i; this combination can cause dangerous hypotension. Beta-blockers are poorly tolerated as patients with PAH rely on the ability to increase heart rate to maintain cardiac output. Cyclizine causes increases in pulmonary artery pressure and should not be prescribed.

 

Atrial fibrillation
Can cause significant decompensation. Electrical cardioversion with adequate anticoagulation is the optimal management, Digoxin may be used as an alternative. Beta-blockers should not be prescribed.

 


SUPPORTIVE THERAPIES

 

Diuretic Therapy
Diuretics are recommended in patients with clinical features of right ventricular failure or volume overload. In some cases, intravenous diuretics are required. Serum biochemistry should be monitored on a regular basis.
 
Oxygen Supplementation
Alveolar hypoxia is a potent pulmonary vasoconstrictor, leading to increased pulmonary arterial pressure both acutely and chronically. Oxygen supplementation is recommended to maintain PaO2 > 8kPa (60mmHg) at rest.  In flight oxygen is recommended for WHO functional class III or IV.  Patients in FC I-II with saturations between 92-95% should have a hypoxic challenge test.  Similarly, patients who fulfill these criteria should avoid altitudes greater than 1500-2000m.
 
Cardiac Glycosides
The role of cardiac glycosides in PAH is unclear and has not been the subject of a randomized controlled trial. Digoxin may be considered in patients who develop an atrial tachyarrhythmia in order to slow ventricular rate. No evidence exists to evaluate their long-term effectiveness.
 
Anticoagulation
Although there have been no prospective, randomised, placebo controlled trials, evidence from single centre case-series suggests that that the use of long term anticoagulation in patients with idiopathic PAH (IPAH) improves survival. In patients with idiopathic, heritable and anorixigen associated-PAH, warfarin therapy with a target INR of 2-3 should be considered on a risk vs benefit basis. There is currently no evidence to support the use of novel oral anticoagulant agents in PAH and we recommend their use only in exceptional circumstances.
 
Calcium channel blockers (CCB)
The appropriate use of CCBs in patients with IPAH is controversial. Concern exists that CCB therapy in non-responders may further impair cardiac function. Treatment of IPAH with CCBs is reserved for patients who demonstrate evidence of acute vasoreactivity, defined as a reduction in mean pulmonary artery pressure >=10mmHg to a level that is <=40mmHg, with an increased or unchanged cardiac output during testing with an acute, short acting vasodilator. Inhaled nitric oxide is the vasodilator of choice. Around 13% of IPAH patients exhibit acute vasoreactivity, and of this group, 50% experience sustained benefit from long term CCB treatment.The choice of agent is dependent on heart rate, with relative bradycardiafavouring amlodipine and relative tachycardia favouring diltiazem. Both should be started at a low dose and titrated up. Response to therapy should be assessed at 3-4 months and re-evaluated if no benefit or clinical worsening is seen.

 

 

 

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