Hrev_master Abstract Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome. Typically, it occurs in young women without atherosclerotic risk factors. Clinical presentation ranges from chest pain to myocardial infarction, ventricular fibrillation and sudden death. We report a very rare case of a young man with ST-elevation myocardial infarction caused by SCAD, complicated by hemopericardium and recurrent cardiac tamponade. Due to this acute complication, he was diagnosed as having vascular Ehlers- Danlos syndrome. Case Report A 30-year-old male presented at the emergency department with severe upper-back and chest pain, which had started the day before. He had been cycling vigorously for several hours (the Tour of Flanders amateur race). He was hemodynamically stable with a blood pressure of 130/68 mmHg, heart rate of 100/min and satura- tion of 98% on room air. He had a medical history of spontaneous pneumothorax and a forearm fracture. Clinical examination revealed somewhat muffled heart tones but clear lung auscultation, normal abdominal investigation and normal peripheral pulsations. The ECG showed sinus tachycardia and deep T-wave inversion and mild ST-elevation in the lateral and inferior leads, consistent with ST- elevation myocardial infarction. Blood results showed elevated cardiac enzymes, consistent with subacute myocardial infarction. He underwent urgent coronary angiography, revealing an image suggestive of type 2 spontaneous dissection of large marginal branch of the circumflex coronary artery1 (Figure 1). Besides this, his coronary arteries were normal. Left ventricular angiography showed regional akinesia in the inferolateral wall. The patient was conservatively treated with aspirin and nitrates and admitted to ICU. No thienopyridines or heparin were given because of the like- ly need for pericardiocentesis (and no atherosclerosis was actually seen). The next morning the patient became more tachycardic and uncomfortable. A transthoracic echocardiography showed mild LV dysfunction and about 2 cm of pericardial effusion. Signs of tam- ponade were present both clinically (tachycardia, pulsus para- doxus) and echocardiographically (swinging heart motion, right atrial collapse and dilated inferior vena cava), so pericardiocentesis was immediately performed. Three hundred milliliter of frank blood were drained, restoring hemodynamics. An urgent computer assisted tomography (CT) scan ruled out aortic dissection and ven- tricular pseudoaneurysm. The pericardial drain was removed the next day after exclusion of residual effusion by echo. Two days later however, he again developed chest pain, tachycardia and hypotension. A new transthoracic echocardiography showed reoc- currence of pericardial effusion. Pericardiocentesis was performed, again draining bloody fluid. From then on, recovery was unevent- ful. Repeated echocardiograms showed no recurrence of pericar- dial fluid. Cardiac magnetic resonance imaging (cMRI) revealed transmural infarction of the inferoposterior LV wall with central profound necrosis. Adjacent to this necrotic LV zone, an intraperi- cardial thrombus was seen, but no free fluid. After a week he could leave the hospital in good clinical con- dition. Follow-up cMRI showed normal evolution of a transmural infarction with scar formation in the inferolateral wall. Early on, an underlying connective tissue disease was suspect- ed. Deeper anamnesis revealed a tendency of easy bruising. His son was born 4 years earlier with bilateral clubfeet. Clinical exam- ination showed thin and translucent skin, a few atrophic scars (cig- arette paper scars), micrognatia, short thumbs and a broad, short uvula. Further vascular imaging by means of CT scans, revealed a small aneurysm of the left vertebral artery, dissection of the right external iliac artery and a dissection with 19 mm aneurysm in the left external iliac artery (Figure 2). The patient was referred for genetic testing, which revealed mutation of the type III procollagen gene (COL3A1). The diagno- sis of Ehler-Danlos type 4 was therewith confirmed. The patient was prescribed Celiprolol 200 mg daily (which has shown to reduce vascular events in type 4 EDS)2 and refrains from intensive or contact sports. He survives, free from any major events, for over 6 years now. Emergency Care Journal 2019; volume 15:7802 Correspondence: Benjamin Scott, Hartcentrum ZNA, ZNA Middelheim Hospital, Lindendreef 1, 2020 Antwerpen, Belgium. E-mail: Benjamin.scott@zna.be Key words: Spontaneous coronary artery dissection; Cardiac tampon- ade; Ehler-Danlos syndrome. Contributions: the authors contributed equally. Conflict of interest: the authors declare no potential conflict of interest. Funding: none. Received for publication: 4 September 2018. Revision received: 10 February 2019. Accepted for publication: 11 February 2019. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright I. Hendrickx and B. Scott, 2019 Licensee PAGEPress, Italy Emergency Care Journal 2019; 15:7802 doi:10.4081/ecj.2019.7802 [Emergency Care Journal 2019; 15:7802] [page 21] A ripping bike race: spontaneous coronary dissection complicated by cardiac tamponade, unmasking vascular Ehlers-Danlos in a young man Inne Hendrickx,1 Benjamin Scott2 1Emergency Department, AZ Maria Middelares, Gent; 2Hartcentrum ZNA, ZNA Middelheim Hospital, Antwerpen, Belgium Non -co mmerc ial us e o nly [page 22] [Emergency Care Journal 2019; 15:7802] Case Report Figure 1. (A) 12-lead ECG showing Q waves, deep negative T waves end mild residual ST-segment elevation in the inferior and lateral leads; (B) still frame from the coronary angiography of the left coronary artery. White arrows show the dissected branch with diffuse loss of lumen, consistent with type 2 SCAD. Figure 2. (A) 3D reconstruction of CT angiography of the abdominal arteries. Dissection and narrowing of the right external iliac artery (EIA, green arrow) and dissection with aneurysm of the left EIA (blue arrows). (B) maximum intensity projection of the smaller left vertebral artery, showing a small aneurysm. Non -co mmerc ial us e o nly [Emergency Care Journal 2019; 15:7802] [page 23] Case Report Discussion Spontaneous coronary artery dissection In the general population, spontaneous coronary artery dissec- tion (SCAD)3,4 is a rare cause of acute coronary syndrome, repre- senting about 1-4 ‰ of the cases. Most cases are seen in female patients (90%). In a population of young women, presenting with ACS, however, SCAD accounts for up to 20% of cases. The underlying mechanism of non-atherosclerotic spontaneous coronary artery dissection is not fully understood. In absence of trauma, the diagnosis of SCAD is made in most patients at the time of coronary angiography. In patients for whom the diagnosis is considered but not secured with coronary angiography, intracoro- nary imaging with optical coherence tomography or intravascular ultrasound may be helpful. In most spontaneous coronary artery dissection patients, con- servative therapy with nitrates and (at least) aspirin is the preferred strategy once the diagnosis is secured. The use of anticoagulants or dual antiplatelet therapy remains an area of discussion. In patients with persistent ST-elevation, cardiogenic shock, malignant arrhythmia and/or persistent major coronary artery obstruction, revascularization should be attempted by PCI or CABG. The sole presence of chest pain may be due to the arterial dissection itself and only warrants revascularization attempts when progression of ischaemia is objectivated. Underlying causes of SCAD should be considered in all cases. This condition is seen in healthy young women, but also in the early postpartum, in patients with vasculitis (such as polyarteritis nodosa and lupus), blood vessel conditions as fibromuscular dys- plasia (FMD), induced by cocaine use, by extreme exercise and also in connective tissue disorders (vascular Ehlers-Danlos syn- drome and Loeys-Dietz syndrome). Male patients (even though very rare) seem to have a higher prevalence of underlying FMD or connective tissue disorders. Pericardial effusion and tamponade are not typically seen.5 When tamponade occurs, vascular or myocardial perforation or rupture should be suspected. Prompt drainage of the pericardium, as well as urgent imaging and cardiosurgical consult should be car- ried out. Vascular Ehlers-Danlos syndrome Vascular Ehlers-Danlos syndrome (VEDS)6,7 is a rare (1/50.000-1/200.000) inherited connective-tissue disorder which results from pathogenic variants in COL3A1. This gene encodes for the chains of type III procollagen, a major protein in vessel walls and hollow organs. This causes profound vascular (both venous and arterial) and gastrointestinal fragility, leading to spontaneous rupture of the large arteries or large veins, uterus, or bowel. Any trauma or manipulation (including catheter placement, tourniquet application or puncture) can lead to rupture or dissection. Bleeding can be extremely difficult to control and is therefore potentially life- threatening. Our patient was previously undiagnosed with this condition. Strenuous physical exercise was judged to be the trigger for the occurrence of isolated SCAD. The infarction that ensued, most likely lead to covered myocardial perforation (or coronary artery branch rupture?), presenting as recurrent cardiac tamponade. Luckily, the invasive procedures performed, did not cause life- threatening complications in our case. Conclusions Important messages for all physicians implicated in the care of patients with acute chest pain should be aware of the issues high- lighted by this case: i) SCAD is a distinct cause of acute coronary syndrome, typi- cally presenting in young healthy women, possibly present in both sexes and patients of all ages. The diagnosis can only be made by coronary angiography (and sometimes intracoronary imaging). Modern coronary CT scan imaging may very well be able to be equally useful in these cases. ii) In men presenting with SCAD, a high degree of suspicion should arise for the presence of underlying vascular/ connective tissue disease. iii) Patients with known or suspected VEDS are at very high risk of vascular (as well as intestinal or uterine) rupture. They should be managed with the greatest of care, avoiding punctures or catheter manipulations whenever possible. Whenever possible, referral to or at least consultation of a center with expertise in this rare condition is recommended. References 1. Saw J, Huphries K. Spontaneous coronary artery dissection. Clinical outcomes and risk of recurrence. J Am Coll Cardiol 2017:70;1148-58. 2. Ong K, Perdu J, De Backer J, et al. Effect of celiprolol on pre- vention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet 2010;376:1476-84. 3. Nayes S, Kim E, Saw J, et al. Spontaneous coronary artery dis- section: current state of the science. Circulation 2018;137:e523-57. 4. Tweet M, Hayes S. Clinical features, management and progno- sis of spontaneous coronary artery dissection. Circulation 2012;126:579-88. 5. Goh A, Lundstrom R. Spontaneous coronary artery dissection with cardiac tamponade. Tex Heart Int J 2015;42:479-82. 6. Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis 2014;9:109. 7. De Paepe A, Malfait F. The Ehlers-Danlos syndrome, a disor- der with many faces. Clin Genet 2012:82:1-11. Non -co mmerc ial us e o nly