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2004, Number S1

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Rev Inst Nal Enf Resp Mex 2004; 17 (S1)

Histopathology and physiopathogenesis of pulmonary hypertension

Rivera RRM

Language: Spanish
References: 49
Page: 17-27
PDF size: 128.75 Kb.


ABSTRACT

The pulmonary arteries in pulmonary arterial hypertension (PAH) are characterized by medial hypertrophy and muscularization, intimal fibrosis, adventitial proliferation and obliteration of small arteries. The medial hypertrophy is also the most commonly identified lesion in pulmonary hypertension (PHT), and not restricted to PHT of any etiology, also is considered to be the precursor to subsequent vascular alterations.
Several types of intimal lesions form part of the continuum of changes associated with plexogenic pulmonary arteriopathy, although this lesion is found in many cases with primary pulmonary arterial hypertension (PPH), are not pathognomonic, because they are also found in cases of severe pulmonary hypertension associated with other diseases.
The genetics basis of familial PPH is unknown, but the clinical and pathological features are the same as in sporadically occurring PPH; this form displays genetic anticipation and an abnormal gender ratio at birth. This phenomenon, genetic anticipation, suggests that the molecular basis of familial PPH may be trinucleotide-repeat expansion, which is linked to unidentified genes of chromosome 2; but others investigators have reported an association between the major histocompatibility complex and PPH.
The patients with this disease suggest that the coagulation system on the endothelial surface may be activated as either a primary or a secondary process. Vasoconstriction is a variable feature of PHT. Thromboxane A2 is both a potent pulmonary vasoconstrictor and a procoagulant whereas prostacyclin has opposing effects, and an imbalance between the releases of these two mediators could be involved in the pathogenesis of the arteriopathy. Since the endothe lium produce an excessive production of vasoconstrictors relative to vasodilators, and the smooth muscle cells are depolarized and calcium-overloaded, which is due in part to reduced expression of voltage-gated potassium channels (Kv). This causes vasoconstriction and may promote cell proliferation. Even abnormal matrix metalloproteinase and elastase activity could also explain the abnormal vascular tone, platelet activation, and remodeling in PPH.


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