260. Case Report: Cardioembolic Stroke from an Unusual Valve Pathology from The University of Alabama at Birmingham

CardioNerds Cofounder Dr. Amit Goyal join Dr. Usman Hasnie and Dr. Will Morgan from University of Alabama at Birmingham for a hike up Red Mountain. They discuss the following case: A 75-year-old woman with prior mitral valve ring annuloplasty presented with subacute, intermittent, self-limiting neurologic deficits. Brain MRI revealed multiple subacute embolic events consistent with cardioembolic phenomena. Transesophageal echochardiogram discovered a mobile mass on the mitral valve as the likely cause for cardioembolic stroke. She was taken for surgical repair of the mitral valve. Tissue biopsy confirmed that the mass was an IgG4-related pseudotumor. Expert commentary is provided by Dr. Neal Miller (Assistant Professor of Cardiology, University of Alabama at Birmingham). Audio editing by CardioNerds Academy Intern, student doctor Adriana Mares

Check out this published case report here: IgG4-Related Disease Masquerading as Culture-Negative Endocarditis!

Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.

This episode is made possible with support from Glass.Health – The first digital notebook designed for doctors. Follow @GlassHealthHQ for the latest product updates!

Pearls – Cardioembolic Stroke due to an IgG4-related pseudotumor

  1. Surgical indications for endocarditis include severe heart failure, valvular dysfunction with severe hemodynamic compromise, prosthetic valve infection, invasion beyond the valve leaflets, recurrent systemic embolization, large mobile vegetations, or persistent sepsis (in infective endocarditis) despite adequate antibiotic therapy.
  2. IgG4 related disease is rare, and likely underrecognized due to the lack of reliable biomarkers. Biopsy and histologic confirmation are imperative to clinch the diagnosis.
  3. Cardiac manifestations of IgG4-related disease are rare but are often related to aortopathies. Valvular disease is extremely rare as a manifestation of the disease.
  4. Treatment of IgG4 related disease includes steroids as the first line treatment.
  5. IgG4 related disease requires a multi-disciplinary approach to both diagnose and treat.

Show Notes – Cardioembolic Stroke due to an IgG4-related pseudotumor

Notes were drafted by Dr. Hasnie and Dr. Morgan

  1. IgG4-related disease has a very diverse presentation including mimicry of infection, malignancy and other autoimmune conditions. It is a fibroinflammatory condition that results in deposition of IgG4 positive plasma cells. It has been described in multiple organ systems including the pancreas, kidneys, lungs and salivary glands. 
  2. Cardiac manifestations are extremely rare and valvular disease even more so. There are thirteen cases of IgG4 related valvular disease, and of these only two had mitral valve involvement such as this case. The most commonly reported cardiovascular manifestations are related to aortopathies. 
  3. This disease remains poorly understood at this point. There are no true biomarkers that can be used to risk stratify the diagnosis for clinicians. Biopsy is imperative to the diagnosis. Even serum IgG4 levels are normal in 30% of cases despite meeting histologic criteria on biopsy making the diagnosis incredibly difficult to make. 
  4. While guidelines have not been developed to guide treatment of IgG4-related disease, steroids are considered the first line treatment option for patients. Often times dosing is 2-4 weeks with a prolonged taper. When looking for glucocorticoid sparing agents, azathioprine, mycophenolate mofetil, and methotrexate are considered alternatives. 

References – Cardioembolic Stroke due to an IgG4-related pseudotumor

1. Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4- related autoimmune disease. J Gastroenterol 2003;38:982-4.

2. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181-1192. doi:10.1038/modpathol.2012.72

3. Dahlgren M, Khosroshahi A, Nielsen GP, Deshpande V, Stone JH. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken) 2010;62:1312-8.

4. Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4 related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum 2009;60:313945.

5. Saeki T, Saito A, Hiura T, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 2006;45:163-7.

6. Kamisawa T, Takuma K, Egawa N, Tsuruta K, Sasaki T. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol 2010;7:401-9.

7. Shakir A, Wheeler Y, Krishnaswamy G. The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations. Heart. 2021;107(10):790-798. doi:10.1136/heartjnl-2020-318041

8. Tyebally S, Chen D, Bhattacharyya S, Mughrabi A, Hussain Z, Manisty C, et al. Cardiac tumors: JACC cardio oncology state-of-the-art review. J Am Coll Cardiol CardioOnc. 2020;2:293–311

9. Selkane C, Amahzoune B, Chavanis N, et al. Changing management of cardiac myxoma based on a series of 40 cases with long-term follow-up. Ann Thorac Surg. 2003;76(6):1935-1938. doi:10.1016/s0003-4975(03)01245-1

10. Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation. 2001;103(22):2687-2693. doi:10.1161/01.cir.103.22.

11. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551. doi:10.1056/NEJMra1104650

12. Hasnie UA, Herrera LN, Morgan WS, Rodriguez JM, Litovsky S, Chatham WW, Winokur T, Muzny CA. IgG4-Related Disease Masquerading As Culture-Negative Endocarditis. AIM Clinical Cases. 2022;1. doi: 10.7326/aimcc.2022.0075

13. 2016 ASE Guideline: https://www.asecho.org/wp-content/uploads/2016/01/2016_Cardiac-Source-of-Embolism.pdf

14.  Shakir A, Wheeler Y, Krishnaswamy G. The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations Heart 2021;107:790-798.

15. Karadeniz H, Vaglio A. IgG4-related disease: a contemporary review. Turk J Med Sci. 2020 Nov 3;50(SI-2):1616-1631. doi: 10.3906/sag-2006-375. PMID: 32777900; PMCID: PMC7672352.

You are currently viewing 260. Case Report: Cardioembolic Stroke from an Unusual Valve Pathology from The University of Alabama at Birmingham