Spontaneous Coronary Artery Dissection in Patients with Autosomal Dominant Polycystic Kidney Disease: A Systematic Review of the Literature

Abstract

Spontaneous coronary artery dissection (SCAD) is a spontaneous intimal tear of the coronary artery wall. A factor rarely associated with SCAD is autosomal dominant polycystic kidney disease (ADPKD). Using the PRISMA guidelines, we identified 10 unique cases of SCAD in ADPKD patients reported between 1998 and 2021. Ages ranged from 36 to 59 years, with an average of 44.6 years. The majority of patients were female (80%). Each case was diagnosed with a cardiovascular event: ST-elevation myocardial infarction (STEMI) in 40%, non-ST elevation myocardial infarction (NSTEMI) in 50%, and stable angina in 10%. Conservative management was used in 60% of cases. There is a significant gap in our understanding of the relationship between SCAD and ADPKD. Polycystin complex can lead to structural abnormalities in blood vessels, resulting in vascular leaks and vessel rupture. This suggests that ADPKD patients may have an elevated risk of arteriopathies, including coronary artery dissection.

Keywords: ADPKD; SCAD; polycystin; vascular integrity.

Figure 1

PRISMA flow chart.

Figure 2

Sex, presentation, and coronary territory of SCAD; STEMI—ST segment elevation myocardial infarction; NSTEMI—non-ST segment elevation myocardial infarction; CCS—chronic coronary syndrome; LAD—left anterior descending artery; LCX—left circumflex artery; RCA—right coronary artery; RI—ramus intermedius.

Figure 3

Pathophysiology behind spontaneous coronary artery dissection and ADPKD.

Figure 4

Management algorithm of SCADs in ADPKD; PCI—percutaneous coronary intervention; CABG—coronary artery bypass graft; LAD—left anterior descending artery. *Raised suspicion in ADPKD patients even without risk factors (Pregnant women & younger patients without hypertension or atherosclerosis.