What is PH

PAH is a highly complex disease that can be caused by many other cardio pulmonary disorders. It can also come about from living at high altitude for example.

Some people with PAH will be treatable with drugs. Those who respond to treatment can live almost normal lives on single therapy drugs or in some case tri therapy treatments that include IV epoprostenol administered via a hickman line directly into the heart. This is a more complicated and precarious drug that requires constant monitoring and management by the patient or carers of the patient. Many non responders have IPAH or HPAH and this is the group of patients that desperately need support to find better ways of treatment by better understanding of their genotype and phenotype and building up data that will allow researchers to pin point more personalised therapies using a number of methods to observe the pathways and to disease causes at a cellular, molecular and epigenetic level. that have already proven to be successful in other diseases. We believe that by following this direction we will

Glossary of terms

We use the term pulmonary hypertension in the website in a very general sense. It covers a vast field of pulmonary vascular disease, but in its purest form it falls under two headings.




PAH: Pulmonary Artery Hypertension

IPAH: Ideopathic Pulmonary Artery Hypertension

HPAH: Heritable Pulmonary Artery Hypertension

PVRI: Pulmonary Vascular Research Institute

Paliative: is care that maintains comfort and dignity.

Genotype: The genotype is the set of genes in our DNA which is responsible for a particular trait

Phenotype: The phenotype is the physical expression, or characteristics, of that trait.

Ideopathic: means ‘no known cause’

Heritable: means ‘hereditary’

Responders: will respond to available drugs with good results

Non-responders: experience no improvement through the use of drugs. Their only hope is through a double lung and heart transplant offering 7 years prognosis from that point onwards or in Archie’s case a Potts shunt operation to releave pressure in the heart. (This is a palliative treatment and not a cure)

Epiginetics: the study of changes in organisms caused by modification of gene expression rather than alteration of the genetic code itself.

Molecular Genetics: is the field of biology that studies the structure and function of genes at a molecular level and thus employs methods of both molecular biology and genetics. The study of chromosomes and gene expression of an organism can give insight into heredity, genetic variation, and mutations.