Vascular aging in adult congenital heart disease-a narrative review

Abstract

Background and objective: Many studies about the high prevalence of cardiovascular disease in adults with congenital heart disease (CHD) have been published in response to the growing number of adult patients with CHD. The aging process leads to hypertension and cardiovascular disease, which is caused by a degenerative change in the systemic arterial system characterized by the stiffening of elastic arteries (known as arteriosclerosis) and the enlargement of aorta. In patients with CHD, aortic dilatation (so-called aortopathy) is one of the most frequent complications. It is well known the anatomical and histological changes in aortopathy are similar to those in aging process. The increase of pulse wave velocity (PWV) enhances pressure wave reflection, and it augments left ventricular afterload and impairs the coronary supply-workload balance in aging. This article reviews the aortic function in patients with CHD, aiming to provide a new direction for the management of their cardiovascular aging process.

Methods: Papers on vascular physiology in CHD were retrieved. I searched all original papers and reviews about the vascular physiology in CHD using PubMed, published from January 1, 1973 to June 30, 2022, in English.

Key content and findings: Enhancement of pressure wave reflection has been reported in many CHDs. Although PWV in whole aorta is not necessarily elevated, the abnormal arterial stiffness gradient is common in patients with CHD.

Conclusions: Many reports concerning functional abnormalities of the aorta have been reported. The abnormalities can result in cardiovascular disease and organ damage. The practitioners should carefully treat patients with CHD while paying attention to their aging process.

Keywords: Congenital heart disease (CHD); arterial stiffness; cardiovascular disease; hypertension; pressure wave reflection.

Figure 1

Ascending aortic pressure waveforms. (A) In patients with low PWV, the reflected pressure wave returns to ascending aorta during diastole. (B) In patients with high PWV, the reflected pressure wave returns to ascending aorta during systole. Arrow indicates the reflected pressure wave. PWV, pulse wave velocity.

Figure 2

Evaluation of pressure wave reflection. Augmentation index (%) =100× (augmentation pressure/pulse pressure).

Figure 3

Evaluation of aortic reservoir function. Subendocardial viability ratio (%) =100× (diastolic pressure time index/tension time index).

Figure 4

Forward and reflection pressure waves in arterial system. (A) Normal aorta. (B) Aged aorta. (C) Aorta with regional stiffness. Arrow indicates the forward pressure wave. Arrow with dashed line indicates the reflected pressure wave.

Figure 5

Possible origins of the reflected pressure wave. (A) Normal aorta. (B) Repaired aortic coarctation. (C) Cyanotic heart disease. Arrow indicates the reflected pressure wave.

Figure 6

Aortic pressure wave forms in patients after aortic arch repair. (A) Ascending aortic pressure waveform in patients after aortic arch repair. (B) Descending aortic pressure waveform in patients after aortic arch repair. (C) Ascending aortic pressure waveform in normal aorta. (D) Descending aortic pressure waveform in normal aorta. In comparison with normal aorta, the pressure waveforms between ascending and descending aorta are almost same in patients after aortic arch repair. Arrow indicates the reflected pressure wave.