肺循環障害と右心不全の病態と治療 : 小児心臓病のパラダイムシフト

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  • キョウジュ シュウニン キネン コウエン ハイ ジュンカン ショウガイ ト ウシンフゼン ノ ビョウタイ ト チリョウ : ショウニ シンゾウビョウ ノ パラダイムシフト
  • Pathophysiology and treatment of pulmonary circulation disorder and right heart failure : Paradigm shift in childhood heart disease
  • PA disorder and RV failure

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Abstract

Potassium channels play diverse roles in regulating the behavior of pulmonary artery smooth muscle cells. The discovery of KCNK３（TASK１）as a new predisposing gene for pulmonary arterial hypertension（PAH）led to an update in the Nice Classification regarding the genetic origin of PAH. Decreased current via KCNA５（Kv１．５）plays a key role in determining pulmonary arterial tone and vascular remodeling. The transformation of smooth muscle cells causes ion channel switching, such as the loss of BKca（Kca１．１）and the gain of IKca（Kca３．１）, in immature proliferative smooth muscle cells and also induces cell migration, proliferation, and apoptosis resistance. We propose OCT as a useful tool in diagnosing pulmonary artery hypertension and may provide helpful information for the pulmonary arterial remodeling process, severity, therapeutic strategy, and prognosis in congenital heart disease.

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