472 Coronary disease in ADPKD patient: a giant coronary aneurysm

Abstract

Abstract Aims Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic disorder driven by mutation of one of two genes: PKD1, which codifies polycystin-1, and PKD2, which codifies polycystin-2. The mutated proteins determine the formation of multiple renal cysts with a consequent decline in kidney function eventually leading to end-stage renal disease (ESRD). In the last decades the cardiovascular complications of ADPKD are emerging as the leading cause of death, but coronary artery disease (CAD) remains to be an uncommon complication. Methods and results A 60-year-old male patient affected by ADPKD, in dialysis treatment for ESRD, was admitted in 2020 to our hospital for invasive coronary angiography (ICA), checking eligibility for kidney transplantation. He had a previous history of hypertension and chronic ischaemic cardiomyopathy. ICA performed in 2017 for unstable angina assessed ectasiant coronary arteries with diffuse atherosclerotic disease (Figure 1), determining significant stenosis of the proximal left anterior descending artery and proximal circumflex artery, treated with percutaneous coronary intervention (PCI). In 2020 was so repeated ICA, that evidenced a good result of the previous PCI, but pointed out a severe progression of ectasiant disease, which led to formation of giant aneurysm of the proximal tract of the right coronary artery, assessed at 3.8 cm × 2.5 cm (Figure 2), fistulizing to the right atrium and determining significant flow limitation in the following part of the right coronary artery. The absence of any symptoms and the lack of evidence of ongoing heart dysfunction, led our team to indicate conservative management and angiography follow-up. Conclusions Cardiovascular disease is a major cause of morbidity and death in ADPKD, underlying a tendency towards accelerated atherosclerosis, but wide data about coronary involvement are still lacking. ADPKD patients seem to have an increased risk of developing coronary aneurisms, but either due to the expression of mutated proteins in arterial smooth cells, to the accelerated atherosclerotic disease or to the combination of both, is still controversial. Consequently, it is difficult to differentiate the underlying pathophysiology of aneurysm formation in an individual patient and to speculate whether ADPKD patients have an increased risk of developing coronary aneurysms independent of their accelerated atherosclerotic process.