87. Case Report: Giant Coronary Aneurysm Presenting with Heart Failure – University of Hawaii

Aloha! CardioNerds (Amit Goyal & Karan Desai)  join University of Hawaii cardiology fellows (Isaac Mizrahi, Nath Limpruttidham, Nishant Trivedi, and Shana Greif) for some shaved iced on the Big Island’s north shore! They discuss a fascinating case of a patient presenting with decompensated heart failure found to have a giant coronary aneurysm. Program director Dr. Dipanjan Banerjee provides the E-CPR as well as a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

Aloha! CardioNerds (Amit Goyal & Karan Desai)  join University of Hawaii cardiology fellows (Isaac Mizrahi, Nath Limpruttidham, Nishant Trivedi, and Shana Greif) for some shaved iced on the Big Island's north shore! They discuss a fascinating case of a patient presenting with decompensated heart failure found to have a giant coronary aneurysm. Program director Dr. Dipanjan Banerjee provides the E-CPR as well as a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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Patient Summary

A man in his early 60s with history of hypertension, peripheral arterial disease, atrial fibrillation, and AAA s/p repair presented with subacute fatigue, palpitations, shortness of breath, and lower extremity edema. On exam he was warm and well perfused, though hypotensive, tachycardic with an irregular rhythm, and had an elevated JVP. ECG showed AF with RVR without evidence of acute MI, and troponin was negative. TTE revealed a reduced LVEF and WMA in the inferolateral walls with akinesis of the basal mid septum; additionally, two large extracardiac structures were noted, one with heterogenous echotexture in the AV groove, and a second with an echolucent interior adjacent to the RA.  

The patient underwent coronary angiography, showing a dilated and calcified proximal LAD with high grade stenosis adjacent to the first septal perforator, a ectatic LCX that supplied left to right collaterals, and a giant RCA aneurysm with TIMI 0 flow distally. CCTA confirmed these findings, showing thrombosed aneurysms of the LAD, LCX, and RCA. Interventional cardiology and cardiac surgery both evaluated the patient’s case, and determined that he was not a candidate for intervention. He was ultimately diuresed to euvolemia with significant improvement in symptoms, and plans to follow-up as an outpatient for heart transplant evaluation.  


Case Media

A. CXR
B. ECG: atrial fibrillation with RVR, left axis deviation, poor r wave progression
C. Wide complex tachycardia
D. CT chest demonstrating giant aneurysm

TTE
Coronary Angiography

Episode Schematics & Teaching

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The CardioNerds 5! – 5 major takeaways from the #CNCR case

1) This case featured a patient with a giant coronary aneurysm – how are coronary artery aneurysms defined and classified?  

  • Coronary artery aneurysms (CAA) are defined as a focal dilation of a coronary segment at least 1.5x the adjacent normal segment. Contrast this with coronary artery ectasia, which refers to a diffuse, as opposed to focal, coronary dilation.  
  • CAA morphology can be classified as either saccular (transverse > longitudinal diameter) or fusiform (transverse < longitudinal diameter). 
  • Giant CAA’s are >20mm in diameter. 
  • Aortocoronary saphenous vein graft aneurysms have distinct characteristics and natural history compared to native coronary aneurysms. These aneurysms tend to present late (e.g., > 10 years following CABG) and tend to be larger than native CAA. 
  • IVUS can help differentiate between a true aneurysm with preserved integrity of all 3 vessel layers (intima, media, and adventitia) and a pseudoaneurysm with loss of wall integrity and damage to the adventitia. 

2) Now that we have the language to define and classify coronary artery aneurysms, what are some causes these lesions?  

  • Atherosclerosis: lipid deposition, focal calcification, and fibrosis can weaken the vessel wall and predispose to subsequent coronary artery dilation. Up to 50% of CAAs are linked to arteriosclerosis.  
  • Autoimmune/inflammatory processes: Lupus and systemic vasculitis, such as Kawasaki’s disease and Takayasu arteritis, can all lead to CAAs. Vasculitic CAAs usually affect more than one artery.  
  • Connective Tissue Disease: Marfan’s syndrome and Ehlers-Danlos disease, for instance, are characterized by deficiencies in vessel wall integrity, leading to CAAs. 
  • Dynamic Wall StressEpisodic hypertension and vasoconstriction, such as that seen in frequent cocaine use, can lead to wall stress, endothelial damage, and coronary artery aneurysms.  
  • Direct Vessel Wall Injury: Intracoronary interventions, such as angioplasty, stent delivery, and brachytherapy, can cause shear wall stress that leads to CAAs.  
  • Infectious Causes: Direct vessel wall invasion or immune complex deposition can be seen in bacterial, mycobacterial, fungal, and syphilitic infections. Septic emboli from infectious endocarditis can also lead to mycotic coronary aneurysms.  
  • Genetic susceptibility: Certain HLA class II genotypes are susceptible to CAAs. This may be the underlying pathology in certain idiopathic and congenital CAAs.  

3) How do coronary artery aneurysms clinically present? 

  • Most CAAs are asymptomatic, and are found incidentally on coronary angiography or CCTA.  
  • Concomitant obstructive arteriosclerosis can cause angina or plaque rupture, and thrombosis in the aneurysm lumen can lead to distal embolization and myocardial infarction.  
  • Massive enlargement of CAAs and saphenous vein graft aneurysms can compress adjacent structures. 
  • CAA rupture is rare, though can cause cardiac tamponade.  
  • Stress-induced myocardial ischemia can also occur due to microvascular dysfunction  

4) How do we diagnosis and assess CAAs? 

  • Most CAAs are evaluated via coronary angiography, though a complete angiographic evaluation can be complicated by delayed antegrade contrast filling, segmental back flow, and contrast stasis. In giant aneurysms, a forceful and prolonged contrast injection is needed to avoid misinterpreting slow aneurysmal filling as thrombosis.  
  • IVUS can help differentiate between true aneurysms, pseudoaneurysms, and coronary segments with aneurysmal appearance due to plaque rupture or stenosis. Furthermore, IVUS can assist in sizing aneurysm and planning for potential PCI.  
  • Coronary CTA noninvasively allows for a more accurate assessment of aneurysm size and degree of thrombus than angiography. CCTA is particularly useful in patients with giant CAA as it can provide an understanding of mechanical complications of these aneurysms.  

5) How are coronary artery aneurysms managed?  

  • Notably, there is a lack of randomized and large-scale trial data to guide the treatment of CAAs; most recommendations are made on the basis of small case series and expert opinion.  
  • Medical Management: Given the association between CAA and arteriosclerosis, risk factor modification should be emphasized. The role of antiplatelet and anticoagulant agents is an area of ongoing debate, though there may be benefit in patients with multivessel ectasia. Furthermore, the context in which the patient presents (e.g., incidental finding vs. acute coronary syndrome) will guide the antiplatelet and/or anticoagulant strategy.  
  • Invasive (Percutaneous) Management: PCI of an aneurysmal vessel in the setting of acute MI is associated with lower rates of procedural success, and higher rates of distal embolization and no-reflow phenomenon. Additionally, these patients have higher rates of stent thrombosis and mortality. Given the higher thrombus burden in aneurysmal arteries, thrombectomy may be helpful in aiding PCI. Some case studies have additionally utilized intracoronary thrombolytics. Another strategy is a stent-assisted coil embolization technique in cases where covered stent placement is not possible due to tortuosity, calcification, or risk of side branch compromise. To date, there haven’t been covered stents specifically designed for CAAs, but stents have been used off-label. 
  • Surgical Management: The most common operative strategy is to open the aneurysm, suture the afferent and efferent vessels, and finish with bypass grafting if possible. Other operative strategies include aneurysm ligation, resection, or marsupialization with interposition graft.

References

  1. Thibodeau, J. T., & Drazner, M. H. (2018). The Role of the Clinical Examination in Patients With Heart Failure. JACC. Heart failure, 6(7), 543–551.  
  2. Abou Sherif, S., Ozden Tok, O., Taşköylü, Ö., et al. (2017). Coronary Artery Aneurysms: A Review of the Epidemiology, Pathophysiology, Diagnosis, and Treatment. Frontiers in cardiovascular medicine, 4, 24. 
  3. Kawsara, A., Núñez Gil, I. J., Alqahtani, F., et al. (2018). Management of Coronary Artery Aneurysms. JACC. Cardiovascular interventions, 11(13), 1211–1223.  
  4. Newburger, J. W., Takahashi, M., & Burns, J. C. (2016). Kawasaki Disease. Journal of the American College of Cardiology, 67(14), 1738–1749.  
  5. McCrindle, B. W., Rowley, A. H., Newburger, J. W., et al. (2017). Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation, 135(17), e927–e999.  

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

CardioNerds Case Reports: Recruitment Edition Series Production Team

75. Case Report: Coronary Vasospasm Presenting as STEMI – UCSF

CardioNerds (Amit Goyal & Daniel Ambinder) join UCSF cardiology fellows (Emily Cedarbaum, Matt Durstenfeld, and Ben Kelemen) for some fun in San Francisco! They discuss a informative case of ST-segment elevation (STEMI) due to coronary vasospasm. Dr. Binh An Phan provides the E-CPR and program director Dr. Atif Qasim provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join UCSF cardiology fellows (Emily Cedarbaum, Matt Durstenfeld, and Ben Kelemen) for some fun in San Francisco! They discuss a fascinating case of ST-segment elevation (STEMI) due to coronary vasospasm. Dr. Binh An Phan provides the E-CPR and program director Dr. Atif Qasim provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

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Patient Summary

A man in his mid-50s with alcohol use disorder, cirrhosis, atrial fibrillation, and alpha thalassemia complicated by iron overload presented with hematemesis. He was tachycardic and hypotensive. Labs were notable for Hgb 8.1 (baseline of 10.2), INR 1.3, lactate 4.2, and ferritin 4660. He was started on IV PPI and octreotide. Course was complicated initially by Afib with RVR with hypotension. Subsequently, the patient developed unstable VT requiring CPR. Post-code EKG showed inferolateral ST elevations. Troponin-I rose from 19 to 225 and his pressor requirement continued to increase despite resolution of his GIB. TTE showed LVEF 42% with new inferolateral wall motion abnormalities, normal RV systolic function, severe mitral regurgitation, and small pericardial effusion. After treatment of his GIB by IR and GI, he underwent an urgent LHC which showed 30% stenosis in proximal LAD, 70% in LADD2, and 95% in distal RCA. Coronary spasm was noted in all vessels. Intracoronary nitroglycerin and nicardipine were administered with significant improvement in spasm and resolution of STE on EKG. Vasopressors were quickly weaned off after. He was eventually stabilized, extubated, and started on an oral nitrate and calcium channel blocker. Repeat TTE showed normalized systolic function without any wall motion abnormalities.  


Case Media

A. Baseline ECG – atrial fibrillation
B. ECG with inferior STEMI

CORS – left system
CORS- RCA pre-vasodilator
CORS- RCA post-vasodilator

Episode Schematics & Teaching

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The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. What are the cardiac manifestations of hemochromatosis? 
    • Cardiac hemochromatosis encompasses cardiac dysfunction from either primary or secondary hemochromatosis. Initially, hemochromatosis leads to diastolic dysfunction and arrhythmias. In later stages, it can lead to dilated cardiomyopathy.  
    • Diagnosis of iron overload is established by elevated transferrin saturation (>55%) and elevated serum ferritin (>300 ng/mL). Genetic testing for mutations in the HFE gene should be pursued. 
    • Cardiac MRI with measurement of T2* relaxation times is the diagnostic test of choice as it can both detect and quantify myocardial iron overload. The iron content in the myocardial tissue is inversely proportional to the time constant of decay for relaxation time. Thus the higher the iron content, the shorter T2* relaxation time.  
  2. What are the causes of ST-segment elevation on EKG besides acute plaque rupture or vasospasm? 
    • Pericarditis: in acute pericarditis, ST elevation can be seen diffusely in all leads, with PR segment depression (except lead aVR +/- V1). The diffuse ST elevations are due to involvement of subepicardial layer of the ventricular wall. The PR depressions are due to involvement of the subepicardial layer of the atrial wall.  
    • Stress CM: The ECG findings of stress cardiomyopathy may be indistinguishable from STEMI secondary to acute plaque rupture. 
    • Brugada syndrome: >2 mm ST-segment elevation in the right precordial leads followed be a negative T wave can be seen in patients with Type 1 Brugada. Type 2 and Type 3 Brugada will have STE as well but with different morphologic criteria.  
    • Electrolyte abnormalities: hyperkalemia can sometimes cause ST elevation. Other EKG findings of hyperkalemia include widened QRS, tall and peaked T waves, low-amplitude or lack of P waves, high grade AV Block, sine wave, and/or ventricular fibrillation or PEA. 
    • Pulmonary embolism: the classic EKG features of PE are S1Q3T3 with signs of RV strain (RBBB, RAD) though these are neither sensitive or specific. Sometimes, ST elevation in aVR and right-sided precordial are seen in massive PE due to RV overload, dilation, and/or ischemia (see the Cedars-Sinai episode for more details!).  
    • Cardioversion: striking ST-segment elevation, often >10mm, can be seen after cardioversion but only lasts 1-2 minutes.  
    • Raised ICP: can mimic acute myocardial infarction with widespread T-wave inversions +/- STE (or depression). Other non-cardiac causes, albeit rare causes, include significant gastrointestinal visceral distension, pneumonia, and pancreatitis.  
  3. What are the two types of ischemic mitral regurgitation (IMR)? 
    • IMR is often a complication of ischemic heart disease and is associated with a worse prognosis across a variety of settings. Ischemic MR can occur due to a primary cause (e.g., abnormality of the valve apparatus and specifically papillary muscle rupture) or secondary cause (e.g., acutely from ischemia and chronically from a complex pathophysiologic changes).  
    • In chronic IMR, regional and/or global LV systolic dysfunction and ventricular remodeling can cause restricted leaflet motion. There can be outward papillary muscle displacement and when this happens, mitral leaflet coaptation moves apically away from the mitral annulus. Further, scarring of the papillary muscles may produce further mitral leaflet tethering and LV dilation can lead to mitral annular dilation. The posterior mitral annulus may contract less as well (which contributes to as much as 25% of the closure of the mitral orifice during systole). The ultimate results is poor leaflet coaptation and mitral regurgitation.  
    • MR secondary to papillary muscle rupture after an acute MI will almost certainly require surgery; while secondary MR from acute ischemia will often respond to revascularization. The treatment of chronic ischemic MR is a topic for another Cardionerds Episode so stay tuned!  
  4. What’s the pathogenesis of coronary vasospasm? 
    • In coronary vasospasm, the coronary arteries – and specifically the vascular smooth muscle layer – constricts due to various causes including emotional distress, changes in sympathetic tone, cocaine, or cigarette smoking, leading to myocardial ischemia.  
    • The causes and mechanisms of coronary vasospasms are still poorly understood but there are a few potential mechanisms proposed. 
      • Autonomic nervous system: increase in sympathetic tone can induce coronary vasospasms. Vasospasms more commonly occur at night during rapid eye movement sleep, when a reduction in vagal activity is associated with an increase in adrenergic activity.  
      • Inflammation: chronic inflammation and cigarette smoking are shown to be associated with vasospasm. Patients with vasospasm are found to have elevated hs-CRP, IL-6, and peripheral WBC.  
      • Other mechanisms have also been proposed including smooth muscle cell hypercontractility, oxidative stress, and genetics 
  5. What’s the treatment for coronary vasospasm? 
    1. Any factor that may precipitate coronary vasospasm, especially smoking, should be avoided. There are additionally certain medications that should be avoided including non-selective beta blockers like propranolol and triptans.  
    2. For medical treatment, long-acting calcium channel blockers can be used, especially taken at nighttime when attacks of coronary vasospasm are frequent 
    3. Long-acting nitrates can also be added to prevent recurrent attacks if calcium channel blockers alone are inadequate 

References

  1. Gulati, V., Harikrishnan, P., Palaniswamy, C., Aronow, W. S., Jain, D., & Frishman, W. H. (2014). Cardiac involvement in hemochromatosis. Cardiology in review22(2), 56–68. 
  2. Wang, K., Asinger, R. W., & Marriott, H. J. (2003). ST-segment elevation in conditions other than acute myocardial infarction. The New England journal of medicine349(22), 2128–2135.  
  3. Báez-Ferrer, N., Izquierdo-Gómez, M. M., Marí-López, B., Montoto-López, J., Duque-Gómez, A., García-Niebla, J., Miranda-Bacallado, J., de la Rosa Hernández, A., Laynez-Cerdeña, I., & Lacalzada-Almeida, J. (2018). Clinical manifestations, diagnosis, and treatment of ischemic mitral regurgitation: a review. Journal of thoracic disease10(12), 6969–6986.  
  4. Hung, M. J., Hu, P., & Hung, M. Y. (2014). Coronary artery spasm: review and update. International journal of medical sciences11(11), 1161–1171.  
  5. Slavich, M., & Patel, R. S. (2016). Coronary artery spasm: Current knowledge and residual uncertainties. International journal of cardiology. Heart & vasculature10, 47–53.  

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