BACKGROUND: Spontaneous coronary artery dissection is an uncommon syndrome. Its prevalence among patients with polycystic kidney disease is very rare, with no previously reported involvement of the right posterior descending coronary artery.
CASE PRESENTATION: We describe the case of a middle-aged Caucasian woman with polycystic kidney disease who presented with a non-ST elevation myocardial infarction. Cardiac catheterization revealed a dissection of her right posterior descending coronary artery. She was treated with dual antiplatelet therapy and had a favorable outcome.
CONCLUSION: We report a rare and interesting case of spontaneous coronary artery dissection of the right posterior descending coronary artery in a patient with polycystic kidney disease. It is important to consider spontaneous coronary artery dissection in the differential diagnosis of patients with polycystic kidney disease who present with an acute coronary syndrome

Fitzgibbons, Timothy P.

Grover, Peeyush

English

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University of Massachusetts Medical School eScholarship@UMMS Open Access Articles Open Access Publications by UMMS Authors 2016-03-10 Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report Peeyush Grover University of Massachusetts Medical School Et al. Let us know how access to this document benefits you. Follow this and additional works at: https://escholarship.umassmed.edu/oapubs  Part of the Cardiology Commons, Cardiovascular Diseases Commons, and the Female Urogenital Diseases and Pregnancy Complications Commons Repository Citation Grover P, Fitzgibbons TP. (2016). Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report. Open Access Articles. https://doi.org/10.1186/s13256-016-0832-8. Retrieved from https://escholarship.umassmed.edu/oapubs/2886 Creative Commons License This work is licensed under a Creative Commons Attribution 4.0 License. This material is brought to you by eScholarship@UMMS. It has been accepted for inclusion in Open Access Articles by an authorized administrator of eScholarship@UMMS. For more information, please contact Lisa.Palmer@umassmed.edu. CASE REPORT Open AccessSpontaneous coronary artery dissection ina patient with autosomal dominantpolycystic kidney disease: a case reportPeeyush Grover1,2* and Timothy P. Fitzgibbons1AbstractBackground: Spontaneous coronary artery dissection is an uncommon syndrome. Its prevalence among patientswith polycystic kidney disease is very rare, with no previously reported involvement of the right posterior descendingcoronary artery.Case presentation: We describe the case of a middle-aged Caucasian woman with polycystic kidney disease whopresented with a non-ST elevation myocardial infarction. Cardiac catheterization revealed a dissection of her rightposterior descending coronary artery. She was treated with dual antiplatelet therapy and had a favorable outcome.Conclusion: We report a rare and interesting case of spontaneous coronary artery dissection of the right posteriordescending coronary artery in a patient with polycystic kidney disease. It is important to consider spontaneouscoronary artery dissection in the differential diagnosis of patients with polycystic kidney disease who present withan acute coronary syndrome.Keywords: Polycystic kidney disease, Right coronary artery, Spontaneous coronary artery dissectionBackgroundSpontaneous coronary artery dissection (SCAD) is a knowncause of acute coronary syndrome [1–4]. It has beenreported to present in peripartum women, in patients witha history of fibromuscular dysplasia or inherited collagenvascular disorders, and with the use of hormonal therapy[5]. The association of SCAD with autosomal dominantpolycystic kidney disease (PKD) is less well recognized andlimited to only a handful of cases [6–11]. Our reported caseis of a woman with PKD who presented with SCAD of herright posterior descending coronary artery (RPDA). Ourpatient did not need any acute interventions and had spon-taneous resolution of symptoms with no recurrent episodesreported over a 1-year follow-up. This is a rare and interest-ing presentation of an uncommon disorder.Case presentationA 52-year-old Caucasian woman with a known history ofautosomal dominant PKD presented with acute onsetchest pain lasting over 1 day. She had no prior history ofcoronary disease or diabetes, nor a personal or family his-tory of connective tissue disorder or vasculitis, and didnot use oral contraceptives. The pain was sub-sternal inlocation with radiation to her left arm and was reported aspressure-like in quality. It started while she was sittingand improved with the use of sublingual nitroglycerine oninitial presentation to our emergency department. She alsoreported transient left-sided facial droop at the start of hersymptoms. Her symptoms lasted only for a few minutesand spontaneously resolved.An initial electrocardiogram demonstrated normal sinusrhythm with nonspecific ST–T wave changes in the infer-ior leads (Fig. 1). Cardiac enzymes (troponin I) demon-strated a rising trend (peak 3.14 U/ml) during the day ofadmission. A non-contrast computed tomography (CT)scan of her brain demonstrated no acute bleed. No athero-sclerotic disease was identified via CT angiography of herhead and neck vessels. Magnetic resonance imaging (MRI)of her brain with contrast was also performed. This* Correspondence: Grover.peeyush@gmail.com1Department of Medicine, Cardiovascular Division, University ofMassachusetts Medical School, Worcester, MA 01605, USA2Cardiovascular Medicine, Umass Memorial Medical Center, Worcester, MA01605, USA© 2016 Grover and Fitzgibbons. Open Access This article is distributed under the terms of the Creative Commons Attribution4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, andreproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link tothe Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.Grover and Fitzgibbons Journal of Medical Case Reports  (2016) 10:62 DOI 10.1186/s13256-016-0832-8showed no dissection of her intracranial vessels, no cere-bral aneurysms, and no evidence of stroke. No vertebral orcarotid artery dissection was seen on these studies. Anechocardiogram demonstrated hypokinesis of the mid- toapical inferior wall and mid-inferolateral wall. She subse-quently underwent cardiac catheterization, which demon-strated spontaneous coronary artery dissection of her distalRPDA (Fig. 2). No interventions were performed and ourpatient was started on dual anti-platelet therapy (DAPT).She was followed up over one year with no reported recur-rent symptoms.DiscussionWe describe a case of spontaneous RPDA dissection in thesetting of autosomal dominant PKD. Although previouslydescribed in the literature, spontaneous coronary arterydissection continues to be an infrequently reported syn-drome. To the best of our knowledge, only six such caseshave previously been published [6–11]. None of thesereported cases had isolated involvement of the RPDA.Although it is more common in women, SCAD should beconsidered in the differential diagnosis of all patients withacute coronary syndromes. It may occur in the presenceor absence of coronary atherosclerosis [12]. In patientswithout atherosclerosis, SCAD more commonly involvesthe left anterior descending artery (67 %), whereas in-volvement of the right coronary artery is less common(15–30 %). Only one of the patients in these reportedcases is mentioned to have a history of PKD [5, 12]. Itis unclear from the series whether the reported patienthad RPDA involvement or not. Interestingly, SCAD inpatients with co-existing coronary atherosclerosis morecommonly affects the right coronary artery (50 %) thanFig. 1 Electrocardiography at the time of presentation to the emergency department demonstrating normal sinus rhythm with non-specific STand T wave changes in the inferior (II, III, and aVF) and lateral leads (V 4-6)Fig. 2 Right anterior oblique view of the right coronary arterydemonstrating the spontaneous coronary artery dissection at thedistal end (the culprit for our patient’s presentation) of the rightposterior descending arteryGrover and Fitzgibbons Journal of Medical Case Reports  (2016) 10:62 Page 2 of 3the left anterior descending artery (33 %) [12]. Al-though our patient had no angiographic evidence ofcoronary disease, the sensitivity of angiography alone islimited; therefore, we cannot totally exclude this possi-bility. More sensitive methods for the detection of cor-onary disease, such as intra-vascular ultrasound, opticalcoherence tomography, or CT angiography may havedetected non-occlusive plaque.Some have proposed classification of the risk factorsfor SCAD into three broad categories: atherosclerosis,pregnancy-associated, and idiopathic [13]. Connectivetissue disorders, such as Type IV Ehlers–Danlos syn-drome, or in this case, PKD, are included in the idiopathiccategory. PKD is considered to manifest if there is reducedexpression of polycystins (polycystin 1 and 2 due to muta-tion in genes PKD1 and PKD2, respectively). These proteinproducts are thought to play a crucial role in the couplingbetween various layers of the tunica media. Thus their nor-mal expression is believed to be crucial for maintainingthe integrity of the myoelastic structure of the arterial wall[14]. Owing to its rarity, the prevalence of SCAD in pa-tients with PKD is currently unclear. This case highlightsthe fact that patients with PKD should be considered athigh risk for SCAD even if they have no coronary arterydisease risk factors.The causes of pregnancy-associated SCAD are notentirely understood [15]. The dramatic endocrine andhemodynamic changes that occur in pregnancy are likelyto play a role. The precise adverse effects of estrogen andprogesterone on the arterial wall are not known, but apathologic mechanism is supported by multiple lines ofevidence: (1) a greater incidence of SCAD in women, (2) agreater incidence in the peripartum period, (3) reports ofSCAD during the menstrual cycle, and (4) the knownassociation with oral contraceptive use. Among other pos-sible effects, progesterone or estrogen may stimulate deg-radation of collagen within the arterial media [15].Our patient had SCAD in the distal portion of her RPDA.The lesion was not amenable to coronary intervention. Shewas started on DAPTand did not have any recurrent symp-toms. The use of conservative medical therapy to treatpatients with SCAD is well reported [1, 5, 12]. When SCADoccurs in a distal vessel not amenable to coronary interven-tion, and patients are asymptomatic and hemodynamicallystable, treatment with DAPT is often sufficient, and allowsfor spontaneous healing of the vessel wall over time [1, 12].It is unclear to us why our patient had initial symp-toms of a neurological deficit. These symptoms couldhave been caused by a spontaneous carotid or vertebralvessel dissection that was beyond the sensitivity of thebrain MRI scan and CT angiogram. Similar cases havepreviously been described in the literature [8]. These pa-tients usually have a good prognosis with rarely reportedrecurrent symptoms [6–11].ConclusionIt is important to include SCAD in the differential diag-nosis of patients with PKD who present with an acutecoronary syndrome.ConsentWritten informed consent was obtained from the patientfor publication of this case report and accompanying im-ages. A copy of the written consent is available for reviewby the Editor-in-Chief of this journal.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsPG and TF contributed to the conception and design of the manuscript.PG was involved in drafting the manuscript and TF and PG together revised itcritically for important intellectual content. PG and TF have given their finalapproval of the version to be published; and agree to be accountable for allaspects of the work in ensuring that questions related to the accuracy orintegrity of any part of the work are appropriately investigated and resolved.Received: 11 November 2015 Accepted: 12 February 2016References1. Alfonso F. Spontaneous coronary artery dissection: new insights from thetip of the iceberg? Circulation. 2012;126(6):667–70.2. Giacoppo D, Capodanno D, Dangas G, Tamburino C. Spontaneous coronaryartery dissection. Int J Cardiol. 2014;175(1):8–20.3. Saw J. Spontaneous coronary artery dissection. Can J Cardiol. 2013;29(9):1027–33.4. Saw J. Coronary angiogram classification of spontaneous coronary arterydissection. Catheter Cardiovasc Interv. 2014;84(7):1115–22.5. Tweet MS, Haynes SN, Pitta SR, Simari RD, Lerman A, Lennon RJ, et al.Clinical features, management, and prognosis of spontaneous coronaryartery dissection. Circulation. 2012;126(5):579–88.6. Afari ME, Quddus A, Bhattarai M, John AR, Broderick RJ. Spontaneouscoronary dissection in polycystic kidney disease. R I Med J (2013).2013;96(12):44–5.7. Basile C, Lucarelli K, Langialonga T. Spontaneous coronary artery dissection:one more extrarenal manifestation of autosomal dominant polycystickidney disease? J Nephrol. 2009;22(3):414–6.8. Bobrie G, Brunet-Bourgin F, Alamowitch S, Coville P, Kassiotis P, KermarrecA, et al. Spontaneous artery dissection: is it part of the spectrum ofautosomal dominant polycystic kidney disease? Nephrol Dial Transplant.1998;13(8):2138–41.9. Itty CT, Farshid A, Talaulikar G. Spontaneous coronary artery dissection in awoman with polycystic kidney disease. Am J Kidney Dis. 2009;53(3):518–21.10. Klingenberg-Salachova F, Limburg S, Boereboom F. Spontaneous coronary arterydissection in polycystic kidney disease. Clinical Kidney Journal. 2012;5(1):44–6.11. Lee CC, Fang CY, Huang CC, Ng SH, Yip HK, Ko SF. Computed tomographyangiographic demonstration of an unexpected left main coronary arterydissection in a patient with polycystic kidney disease. J Thorac Imaging.2011;26(1):W4–6.12. Alfonso F, Paulo M, Lennie V, Dutary J, Bernardo E, Jimenez-Quevedo P, etal. Spontaneous coronary artery dissection: long-term follow-up of a largeseries of patients prospectively managed with a “conservative” therapeuticstrategy. JACC Cardiovasc Interv. 2012;5(10):1062–70.13. DeMaio Jr SJ, Kinsella SH, Silverman ME. Clinical course and long-term prognosisof spontaneous coronary artery dissection. Am J Cardiol. 1989;64:471–4.14. Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidneydisease. Lancet. 2007;369(9569):1287–301.15. Marcoff L, Rahman E. Menstruation-associated spontaneous coronary arterydissection. J Invasive Cardiol. 2010;22(10):E183–5.Grover and Fitzgibbons Journal of Medical Case Reports  (2016) 10:62 Page 3 of 3

Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report

Peeyush Grover

Timothy P. Fitzgibbons

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Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report

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