Abstract

AbstractThere are several types of abnormalities in the integrated physiology of pulmonary circulation in congenital heart disease. The main pathology of Eisenmenger syndrome involves a change in pulmonary resistance and is the most commonly observed pathophysiology in pulmonary hypertension. Other diseases also present with the main pathophysiological characteristic of reduced pulmonary compliance, such as tetralogy of Fallot and multiple peripheral pulmonary stenosis. In addition, the cavo-pulmonary connection has the unique feature of both pulmonary circulation and regulation. According to the differences in the pathophysiological features of pulmonary circulation, therapeutic approaches may considerably differ between diseases and conditions. Physiology-based clinical insights with regard to pulmonary circulation in congenital heart disease will be discussed in this chapter.

Highlights

  • This chapter discusses the pathophysiology of pulmonary circulation in congenital heart disease

  • This refers to the macrophysiology of cardiovascular hemodynamics, an understanding of which is essential for the treatment of patients with abnormal pulmonary circulation and congenital heart disease

  • pulmonary vascular resistance (PVR) acts in opposition to mean or non-pulsatile steady flow and is analogous to electric resistance, which acts in opposition to direct current in an electronic circuit

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Summary

15.1 Introduction

This chapter discusses the pathophysiology of pulmonary circulation in congenital heart disease. This refers to the macrophysiology of cardiovascular hemodynamics, an understanding of which is essential for the treatment of patients with abnormal pulmonary circulation and congenital heart disease. We hope this chapter can provide some hints and insights for use in future research on pulmonary hypertension in congenital disease as well as in the fields of physiology and molecular and cellular biology. We will first discuss how we comprehensively assess integrated pulmonary circulation in congenital heart disease in vivo. Using methodology for comprehensive assessment, we will discuss the pathophysiological characteristics of pulmonary circulation in patients with various types of congenital heart disease

15.2.1 Physiologic Components of Pulmonary Circulation
15.2.2 Impedance Analysis
15.3.1 Abnormal Resistance Is the Main Pathophysiology
15.3.2 Right Ventricular Function and Coupling to PA Load
15.3.3 Abnormalities of Compliance Is the Main Pathophysiology
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