Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension
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The term "Pulmonary Arterial Hypertension" (PAH) refers to an infrequent medical condition characterized by an increase in blood pressure in the pulmonary arterial vessels. The pulmonary arteries have the important function of carrying blood loaded with waste (such as carbon dioxide) from the heart to the lungs, for its oxygenation.
Any obstacle to its flow, of an obstructive or stenotic nature, will therefore lead to an increased resistance of the arterial walls to the passage of blood, resulting in a greater cardiac effort to pump it.
Cardiac complications (especially in its right side) are therefore of primary clinical interest and manifest themselves with possible decompensation, arrhythmias, clot formation in the affected arteries and pericardial effusion (due to the accumulation of fluids). Systemically, anemia and liver damage related to hypertension may also occur.
WHO has classified PAH into five classes: Group 1 includes pulmonary hypertension in the strict sense, including idiopathic (with no known cause) and heritable (linked to gene mutations, such as those affecting BMPR2 - the main cause of familial PAH); cases resulting from drug use and other pathological conditions also belong to this group.
The second group includes forms of PAH due to compromised left side of the heart which, over time, can alter the pulmonary arteries. The third group, the largest, encompasses all causes of PAH of pulmonary origin: obstructive conditions, sleep apnea and ventilation disorders are among these. The fourth group specifically includes thrombotic manifestations which, through the formation of clots within the arteries or lungs themselves, represent a physical obstacle to blood flow. Finally, the fifth and last group includes any systemic alteration that can involve PAH such as haematological and metabolic disorders, systemic diseases with pulmonary involvement, rare diseases.
Although PAH occurs sporadically in the majority of cases, about 20% of cases are caused by genetic mutations. Its incidence is quite low, but this condition can be life-threatening: the underlying causes must therefore be promptly identified.
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Any obstacle to its flow, of an obstructive or stenotic nature, will therefore lead to an increased resistance of the arterial walls to the passage of blood, resulting in a greater cardiac effort to pump it.
Cardiac complications (especially in its right side) are therefore of primary clinical interest and manifest themselves with possible decompensation, arrhythmias, clot formation in the affected arteries and pericardial effusion (due to the accumulation of fluids). Systemically, anemia and liver damage related to hypertension may also occur.
WHO has classified PAH into five classes: Group 1 includes pulmonary hypertension in the strict sense, including idiopathic (with no known cause) and heritable (linked to gene mutations, such as those affecting BMPR2 - the main cause of familial PAH); cases resulting from drug use and other pathological conditions also belong to this group.
The second group includes forms of PAH due to compromised left side of the heart which, over time, can alter the pulmonary arteries. The third group, the largest, encompasses all causes of PAH of pulmonary origin: obstructive conditions, sleep apnea and ventilation disorders are among these. The fourth group specifically includes thrombotic manifestations which, through the formation of clots within the arteries or lungs themselves, represent a physical obstacle to blood flow. Finally, the fifth and last group includes any systemic alteration that can involve PAH such as haematological and metabolic disorders, systemic diseases with pulmonary involvement, rare diseases.
Although PAH occurs sporadically in the majority of cases, about 20% of cases are caused by genetic mutations. Its incidence is quite low, but this condition can be life-threatening: the underlying causes must therefore be promptly identified.
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