Primary Pulmonary Hypertension Treatment

Pulmonary hypertension is a heart condition in which the pulmonary arteries sustain higher pressure than normal. This disorder is split into two categories: primary and secondary pulmonary hypertension. Secondary pulmonary hypertension occurs when the extra arterial pressure can be explained by a secondary cause, such as emphysema or blood clots. Primary pulmonary hypertension (PPH), the rarer form, occurs when no secondary cause can be found. While no cure exists for this condition, treatment options can improve quality of life and address some of the symptoms.

Consequences

• The pulmonary arteries pump blood from the right ventricle of the heart to pick up oxygen from the lungs. If the pressure in this pathway is too high, serious consequences can arise. The extra pressure makes it harder for the heart to pump blood through the pulmonary arteries. This causes the muscles of the right heart to enlarge. An enlarged heart is a serious medical condition that can lead to fluid buildup in bodily tissues as well heart failure and death. Fatalities are more common in primary pulmonary hypertension than the secondary form because the underlying cause is unknown. PPH is also the more aggressive form of the disease, progressing quickly and often affecting young people.

Treatment : Dilation

• Due to its mysterious origin, primary pulmonary hypertension is difficult to treat. Primary pulmonary hypertension requires advanced treatment using drugs that dilate the pulmonary arteries. By widening these pathways, pressure is reduced and circulation can flow more easily. Some dilatory drugs used to treat this disorder include Flolan, Tracleer, Remodulin, Ventavis and Revatio.

Treating the Symptoms

• Your physician may also prescribe supplements to increase your oxygen intake and diuretics to stimulate urination. These treatments do not target the cause of pulmonary hypertension but rather address the unpleasant symptoms of the condition. The most prominent symptoms include shortness of breath, cough and fatigue.

Gene Therapy

• An exciting new possibility has appeared in the field of PPH research, as the gene causing familial PPH has been discovered. When PPH is present along a family line, it is caused by a mutation in a gene labeled BMPR2. Discovering the gene responsible is an important breakthrough as it may lead to gene therapy in the future. This would involve editing the genetic mechanism to prevent the onset of the disease.