Echocardiologist in Kattigenahalli, Bangalore •
Treatment of pulmonary hypertension that is secondary to an underlying respiratory or cardiac
condition begins with an attempt to optimise treatment or fix the underling condition.
1 COPD: bronchodilators, steroids, continuous oxygen.
2 Pulmonary fibrosis: oxygen. Aggressive treatment of an underlying connective tissue disease
may halt or slow progression of the pulmonary pressures.
3 Pulmonary embolus: anticoagulation, vena caval filter and occasionally pulmonary embolectomy.
4 Mitral stenosis: valvotomy or replacement.
5 Mitral regurgitation: repair or replacement if left ventricular function remains reasonable.
6 Atrial septal defect: surgery or, if suitable, closure in the catheter laboratory, for example
with an Amplatzer closure device. There must be evidence of reversibility of the pulmonary
pressure if it is nearly as high as systemic pressure, otherwise closure will not improve the
7 Eisenmenger’s syndrome: repair of the defect responsible for the shunt is not usually possible
once reversal of shunting has occurred. Consider heart and lung transplant if conservative
treatment (diuretics, digoxin and sometimes ACE inhibitors) has failed.
General measures include continuous oxygen, diuretics, digoxin and aldactone for problems
with right heart failure.
Patients with pulmonary hypertension that is idiopathic or secondary to connective tissue
disease can now be treated with bosentan. This drug is an endothelin receptor antagonist. Endothelin
1 is a potent vasoconstrictor whose levels have been shown to be elevated in patients with
idiopathic pulmonary hypertension. Moderate or better improvements in functional capacity
and pulmonary artery pressures have been demonstrated after treatment for these patients. The
drug is available only for patients with class III symptoms and right atrial pressure greater than
8 mmHg. Patients must demonstrate stable or improved symptoms (usually via a six-minute
walk test) for treatment to continue. The drug is very expensive. Its side effects include teratogenicity,
an increase in liver enzymes and possibly male infertility.
Prostacyclin analogues such as iloprost, which is taken by inhalation, can be effective. Similar
restrictions apply to their prescription because of their cost. Continuous intravenous epoprostenol
is available in some countries and has been shown to improve symptoms and prognosis in
a number of small randomised trials for at least idiopathic pulmonary hypertension patients.
Sildenafil (a phosphodiesterase inhibitor) is the most recent vasodilator to become fashionable
for idiopathic pulmonary hypertension. A long-acting form has recently become available.