Author + information
- Received September 13, 2018
- Revision received October 31, 2018
- Accepted November 6, 2018
- Published online January 16, 2019.
- Virginia Pascual-Tejerina, MD∗ (, )
- David Dobarro, MD,
- Angel Sanchez Recalde, MD, PhD,
- Raúl Moreno, MD, PhD and
- Jose Luis López-Sendón, MD
- ↵∗Address for correspondence:
Dr. Virginia Pascual-Tejerina, Department of Interventional Cardiology, La Paz University Hospital, Paseo de la Castellana, 261, 28046 Madrid, Spain.
We present the case of an 80-year-old man with prior diagnoses of neurofibromatosis type I (Von Recklinghausen’s disease) and an anterior acute myocardial infarction in 2014. Coronary angiography at that time noted diffuse coronary ectasia with a complete thrombotic occlusion of the mid-left anterior descending coronary artery (LAD). Primary percutaneous coronary intervention (PCI) was performed; 3 stents were implanted in the LAD, 2 of which overlapped in the middle segment, and another one was isolated to the proximal segment.
In 2016, he underwent another cardiac catheterization because of a non–ST-segment elevation myocardial infarction; severe coronary ectasia persisted, and the stents were patent, but there was now slow flow in the LAD not appreciated in 2014. No PCI was performed; oral systemic anticoagulation was initiated.
In 2018, the patient had another non–ST-segment elevation myocardial infarction, complicated with heart failure. Cardiac catheterization showed a large coronary aneurysm that involved the proximal segment of LAD. The 2 proximal stents were outside the effective lumen of the artery (Figures 1A and 1B), whereas the most distal stent was patent within the artery, but slow flow was noted thereafter. The right coronary artery and left circumflex artery had severe ectasia as in previous studies (Figures 1A and 1C). Intravascular ultrasound (Figure 2) and multidetector cardiac computed tomography (Figure 3) confirmed that there was a huge coronary aneurysm in the LAD, almost entirely thrombosed, with the 2 proximal stents also thrombosed and excluded from the effective lumen. The patient died due to cardiogenic shock.
Von Recklinghausen’s disease is an inherited disorder that causes dysplasia of neuroectodermal and mesodermal tissues. It has multisystemic manifestations, including vascular anomalies such as arterial stenosis, aneurysms, or arteriovenous fistulas.
Coronary aneurysms are a rare manifestation of Von Recklinghausen’s disease, with fewer than 10 cases in the published reports (1). This case also provides a distinctive teaching point: the aneurysm was secondary to a remote PCI that facilitated vascular wall disruption in a susceptible patient with inherently pathological arteries. Although primary PCI was without a doubt the best option initially, this case illustrates that other revascularization options should be considered in patients with neurofibromatosis type I and atherosclerotic disease because these patients are at particular risk of developing aneurysms after PCI.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received September 13, 2018.
- Revision received October 31, 2018.
- Accepted November 6, 2018.