Multiple Coronary Artery Aneurysms in Alport Syndrome

J INVASIVE CARDIOL 2019;31(12):E392-E393.

Key words: Alport syndrome, cardiac imaging, coronary aneurysm

Alport syndrome (AS) is a genetic disorder characterized by abnormal alpha chains of type IV collagen in multiple organs including the kidney, cochlea, cornea, lens, and retina. Additionally, aortic disease including dissection and aneurysm has been described with AS. Involvement of the coronary arteries, including coronary intramural hematoma and spontaneous coronary artery dissection, has been reported previously as a very rare manifestation of AS. We report the first case of multiple coronary aneurysms as a manifestation of AS. 

The patient is a 62-year-old man with history of AS (Figure 1) with end-stage chronic kidney disease on renal replacement therapy and hypertension, who underwent coronary angiography as routine diagnostic procedure for assessment of eligibility for kidney transplantation. On physical examination, he had a blood pressure of 153/92 mm Hg, heart rate of 66 beats/min, and respiratory rate of 15 breaths/min. Electrocardiogram was unremarkable. Serum troponin I concentration was within the normal range. Transthoracic echocardiography revealed a normal left ventricular ejection fraction. Coronary angiography showed multiple aneurysms in all coronary arteries (Figures 2-4). The patient was treated conservatively; medications included an angiotensin-converting enzyme inhibitor, a calcium-channel blocker, an alpha blocker, and a statin.

Coronary aneurysms are infrequent findings during coronary angiography and are defined as a localized dilation of a coronary artery with a diameter of more than 1.5-fold compared with adjacent coronary segments. Coronary aneurysms may be associated with atherosclerosis, coronary interventions, Kawasaki disease, and Takayasu arteritis. The underlying pathological processes of coronary artery aneurysm development may vary with underlying etiology. However, the final unifying mechanism is vessel wall weakening and subsequent segmental dilation. 

Non-dissecting coronary aneurysms in AS may result from defective type IV collagen and associated destruction of the connective tissue in the media of coronary arteries. This makes the arterial wall vulnerable and less resistant to withstand the pulsatile forces. Uncontrolled hypertension may enhance arterial wall weakening in patients with AS and may contribute to aneurysm formation.

From the ¹Department of Cardiology and Intensive Care, St. Josef Hospital Braunau, Braunau, Austria; and the ²Department of Cardiology, University Clinic St. Pölten, St. Pölten, Austria.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein. 

The authors report that patient consent was provided for publication of the images used herein.

Manuscript accepted March 5, 2019.

Address for correspondence: Johann Auer, MD, FESC, FACC, FAHA, FSCAI, Department of Cardiology and Intensive Care, “St. Josef” Hospital, Braunau, Austria. Email: johann.auer@khbr.at