Author + information
- Takeya Minami1
Patient initials or identifier number
Relevant clinical history and physical exam
A case was 38-year-old Japanese male.
He had 4 times skin swanoma resection with a diagnosis of Von Recklinghausen’s Disease.
He had effort angina from 37-year-old without treatment.
In November 2014 he suddenly collapsed during walking and a citizen called EMS without Bystander CPR.
The initial rhythm was VF. He was transported to our ER with ROSC after CPR including 2 times DC shock (Total CPA time was 21 minutes). In ER, GCS was E1VTM1. His blood pressure was 149/99 mmHg with NAD infusion (0.2γ), and his heart rate was 100 bpm. Small brain contusion was found in his right head and multiple café-au-lait spots were found in his skin.
Relevant test results prior to catheterization
Blood test in ER revealed untreated dyslipidemia and CK elevation with positive troponin test. ECG showed ST elevation in precordial leads (V2-5) and I. ST elevation and abnormal Q wave in inferior leads (II,III, aVF)) was also shown. Echocardiogram showed akinesis of the anteroseptal and lateral wall without thinning and showed akinesis of inferior apex wall with highechoic thinning.
Relevant catheterization findings
RHC showed Forrester 4
RCA was high anterior origin and seg, 2 was totally occluded
Seg.15 was also totally occluded. There was collateral feeding on LCX to RCA distal and from LAD to LCX distal
Seg.6 was 99% TIMI-2, thought to be culprit lesion. There was collateral flow from via septal br from seg.15
The diagnosis was a Multi-vessel disease in younger age with STEMI of LAD with LCX CTO and RCA CTO.
ST elevation was continued and VF/PEA was relapsed sporadically. So We decided to perform PCI because revascularization of LAD (only remaining circulation) is important to maintain the hemodynamics. Approach site was femoral, GC was 7Fr JL 4.0SH. The 1st wire was Sion blue. IVUS showed severe stenosis with a lipid-rich plaque in Seg. 6. We used distal protection device (Filtrap) because LAD was only remaining circulation with RCA and LCX CTO. After aspiration and distal protection, we put the stent in Seg.6. Slow flow phenomenon and VF relapsed even after retrieving distal protection device and with many times aspiration, Nicorandil usage and keeping prolonged ACT. So We added the stents with IABP and temporary pacing support. Coronary blood flow was improved but LAD flow was occluded by thrombus again as before. After Argatroban (3 mg) usage, multi thrombi was dissolved and we could finish the procedure with the TIMI-3 flow. Although intensive care with target temperature management, IABP and a full dose of vasopressor was performed, it was difficult to keep his vital sign without any sign of SAT or cardiac rupture. We could not save him at 3:00 AM 1 day after admission Labo data at a later date revealed no significant findings of HIT.
We couldn't rescue a young Von Recklinghausen’s disease (NF-1) case who had CPA (VF) due to STEMI of LAD with early MVD including LCX and RCA CTO. At the time of PCI to him, it was difficult to treat multiple thrombi which were clearly dissolved by using Argatroban.
An autopsy revealed that this case’s atherosclerotic lesion was seen mainly in small-medium sized vessels including coronary arteries. It was different with the previous report in which NF-1 patients’ vasculopathy was rare but occurred in medium to large sized arteries. We report a young Von Recklinghausen’s disease patient with a multi-vessel disease. If Von Recklinghausen’s patient (even if he/she is young) have angina, it should be needed to evaluate coronary artery aggressively and to consider an early intervention with drug-eluting stent whose target is to suppress the abnormal cell proliferation.