Supplementary Table E. Valvular and Myopericardial Disease in the Literature

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Supplementary Table E. Valvular and myopericardial disease in the literature. Report Comments Reference VALVULAR DISEASE 1 A 55-year-old man presented for evaluation of a diffuse erythematous maculopapular Komatsuzaki et al., rash. Biopsy showed fibrosing lesions with marked IgG infiltrate and an IgG4/IgG ratio 2019 of almost 100%. This prompted workup for IgG4-RD. Serum IgG4 was high and PET-CT showed scattered lymphadenopathy; physical exam revealed cardiac murmur which PMID: 31773746 prompted echocardiography. Patient was found to have aortic stenosis.

2 A 62-year-old man with suspected IgG4-RD (history of pancreatitis, lacrimal gland Kosugi et al., 2018 enlargement, and retroperitoneal fibrosis; already on steroids) presented with dyspnea and bradycardia. EKG revealed complete atrioventricular block. Echocardiography and CT PMID: 31020164 chest revealed new severe aortic regurgitation with valve thickening extending to the left ventricular outflow tract. He was empirically treated for presumed IgG4-related valve disease with pulse steroids. AV block quickly resolved, but patient eventually developed worsening aortic regurgitation and heart failure that ultimately required valve replacement. Intraoperatively, the valve thickening was noted to have regressed. Pathology of the excised aortic valve leaflets confirmed IgG4-RD.

3 A 74-year-old man with prior suspicion of IgG4-RD (elevated serum IgG4 and CT Hourai et al., 2018 concerning for retroperitoneal fibrosis) was later found to have severe aortic stenosis and admitted. EKG was notable for complete right bundle branch block and anterior ST- PMID: 30101853 segment depressions. Coronary angiography showed LAD and RCA obstructive CAD, so he underwent CABG with aortic valve replacement. Native valve was found to have IgG4-positive plasmacytic infiltrate and patient had markedly elevated serum IgG4. This led authors to presume, but not completely confirm, IgG4-RD.

4 A 72-year-old woman presented with dyspnea and near-syncope. She was found to have Tiong et al., 2018 3-vessel CAD and severe mitral stenosis, so underwent CABG with mitral valve replacement. PMID: 29437558

5 An 82-year-old man with a history of aortic stenosis was admitted with systemic Bruls et al., 2017 symptoms concerning for lymphoma. PET-CT showed diffuse FDG-avid lymphadenopathy. Lymph node biopsy findings were consistent with IgG4-RD. Despite PMID: 28219556 steroid therapy, patient developed dyspnea and echocardiogram showed progression of aortic stenosis. Surgical replacement was performed and excised native aortic valve was found to have IgG4-positive plasmacytic infiltrate.

6 An asymptomatic 64-year-old man was incidentally found to have a systolic murmur, so Ishida et al., 2017 underwent echocardiogram which showed a mass attached to the pulmonary valve and right ventricular outflow tract. The mass was FDG-avid on PET-CT. Resection was PMID: 28219555 performed and pathology showed IgG4-RD. The mass was found to be regrowing a year later, so steroids were initiated and it regressed again.

7 A 64-year-old woman with history of orbital fibrosclerosis developed new-onset heart Besik et al., 2015 failure. Echocardiogram revealed a pathologic mass-like infiltrate that began in the anterior leaflet of the mitral valve and continued to the left ventricular outflow tract and PMID: 26434439 left coronary cusp of the aortic valve. She had resulting severe aortic regurgitation and moderate mitral stenosis and regurgitation. She did not improve with medical therapy, so partial surgical excision of the mass with aortic and mitral valve replacement was performed. Pathology showed dense lymphoplasmacytic infiltrate with elevated IgG/IgG4 ratio.

8 2 separate cases that were the first report of aortic valve involvement: Malaszewski et al., A 60-year-old woman with chest tightness and shortness of breath underwent 2015 echocardiogram and was found to have thickened aortic valve leaflets with moderate aortic stenosis, moderate to severe aortic regurgitation, and thickened mitral valve leaflets PMID: 25283128 with severe regurgitation. Aortic valve replacement was performed and excised native valves were noted to have IgG4-heavy lymphoplasmacytic infiltrate.

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The second case was a 70-year-old man with prior history of submandibular mass and CAD status-post stent, who developed exertional dyspnea and dizziness. Echo showed severe aortic stenosis so he underwent valve replacement with pathology showing similar findings to the woman above. PET-CT was done to rule out other IgG4-RD lesions and he was found to have a pancreatic tail mass. Fine needle aspiration confirmed IgG4-RD.

9 A 58-year-old woman presented with syncope and was found to have a systolic murmur Kouzu et al., 2014 over the pulmonic valve area on exam. Transesophageal echocardiogram revealed a mass on the anterior semilunar cusp of the pulmonic valve; CT chest showed that it expanded PMID: 24369180 to obstruct the right ventricular outflow tract. Surgical resection and valvuloplasty were performed. A diagnosis of inflammatory myofibroblastic tumor was made, but IgG4/IgG ratio was elevated enough to meet criteria for IgG4-RD so this diagnosis was considered “probable.”

10 (Previously cited in Supplementary Table A – comorbid periaortitis) Hwang et al., 2013

A 70-year-old woman presented with dyspnea which prompted echocardiogram. This PMID: 24339717 revealed a mass attached to the anterior leaflet of the mitral valve, with resulting functional mitral stenosis and pulmonary hypertension. Cardiac MRI showed that the mass extended throughout the mitral valve, into the left atrial wall and interatrial septum. CT abdomen showed periaortitis; both the mitral valve and abdominal aortic lesions were FDG-avid on PET-CT. Excisional biopsy of the aortic wall mass confirmed IgG4-RD. Dyspnea resolved and valvular lesion shrunk after steroid administration.

11 A 59-year-old woman was admitted for dyspnea and leg edema. She was found to have Yamauchi et al., pleural effusions on chest X-ray and 2:1 AV block on EKG. Echocardiogram showed a 2013 mass attached to the anterior leaflet of the mitral valve and severe aortic regurgitation. She underwent aortic valve replacement. IgGR-RD diagnosis was made based on PMID: 23706467 pathology of the tumorous valve infiltrate, which was lymphoplasmacytic with high IgG4/IgG ratio.

12 (Previously cited in Supplementary Table A – comorbid periaortitis) Yang et al., 2013

54-year-old man admitted for syncope was found to have idiopathic ventricular PMID: 24776336 tachycardia requiring defibrillator implantation. Echocardiogram and PET-CT done as part of workup were suggestive of periaortitis. Patient developed worsening dyspnea and repeat PET-CT showed severe thickening of the aortic valve and anterior leaflet of the mitral valve. Aortic biopsy showed fibrosis and lymphoplasmacytic infiltrate, so IgG4- RD was tentatively diagnosed. This was presumed to be the cause of the valvular infiltrate as well.

MYOCARDIAL DISEASE 1 A 47-year-old man was admitted after being shocked in the field for nonischemic cardiac Pestana et al., 2019 arrest caused by high-degree AV block. He went into torsades de pointes inpatient requiring a second shock. Echocardiogram revealed a large tumorous mass in the PMID: 29551702 interatrial septum. Echo-guided percutaneous biopsy was done and showed a benign fibro-inflammatory neoplasm. Permanent pacemaker was implanted but the mass was not resectable. Histopathology confirmed IgG4-RD.

2 A 69-year-old woman was worked up for exophthalmos and was found to have elevated Yano et al., 2018 serum IgG4. PET-CT showed high FDG uptake in bilateral orbits and mediastinal lymph nodes; it also incidentally revealed a large FDG-avid intracavitary mass in the right PMID: 30012925 atrium. EKG showed ectopic atrial rhythm. Percutaneous transcatheter biopsy was performed and revealed an IgG4-positive lymphoplasmacytic infiltrate. After 4 weeks on steroid therapy, both mass size and serum IgG4 had decreased, and EKG showed normal sinus rhythm.

3 (Previously cited in Supplementary Table A – comorbid periaortitis) Li et al., 2016

A 52-year-old man presented with acute chest pain. Echocardiogram showed a mass in PMID: 27263808 the right ventricular wall near the apex. CT angiogram redemonstrated the mass and additionally showed an ascending aortic intramural hematoma. He underwent surgical

exicision of the mass and replacement of the ascending aorta. Pathology of both mass and resected aortic wall was consistent with IgG4-RD.

4 (Previously cited in Supplementary Table D – comorbid phlebitis) Song et al., 2013

A 55-year-old woman with syncope and dizziness was found to have 1st-degree AV PMID: 23918583 block on EKG with multiple sinus pauses and episodes of atrial fibrillation on Holter monitor. She underwent echocardiogram, MRI, and PET-CT, which revealed an FDG- avid mass extending from the SVC-right atrial junction into the right atrial posterior wall and interatrial septum. Open chest biopsy of the mass revealed IgG4-RD on pathology. Patient was treated with pacemaker implantation.

PERICARDIAL DISEASE 1 A 64-year-old woman was worked up outpatient for abdominal fullness and found to have Arao et al., 2019 massive pericardial effusion on chest X-ray, so was referred for admission. Effusion was drained and fluid studies were unremarkable. However, CT abdomen performed as part of PMID: 30626813 workup was notable for ureteral wall thickening, leading authors to suspect IgG4-RD as the cause of both findings. Serum IgG4 level was markedly elevated. Patient had no accessible biopsy site so steroids were empirically started with prompt resolution of the effusion and no recurrence on long-term follow-up.

2 A 53-year-old woman with chest discomfort underwent echocardiogram which showed Gorecka et al., 2019 small pericardial effusion. Cardiac MRI showed pericardial thickening and active inflammation. Labs were notable for peripheral eosinophilia. Pericardial biopsy via PMID: 31256053 mediastinoscopy was performed and showed IgG4-positive plasma cell infiltrate.

3 A 36-year-old man presented with chest pain and was found to have EKG changes Yassi et al., 2019 consistent with acute pericarditis. However, he failed to respond to anti-inflammatory agents and empiric antibiotics. He eventually began to show signs of tamponade and PMID: 30885856 required pericardial window. Histopathology showed organizing pericarditis, focal phlebitis, and an IgG4-positive lymphoplasmacytic infiltrate. Patient met criteria for a definitive IgG4-RD diagnosis.

4 (Previously cited in Supplementary Table A– comorbid periaortitis Matsuda et al., 2018 (Previously cited in Supplementary Table B – coronary periarteritis) PMID: 29880627 A 70-year-old woman diagnosed with IgG4-RD after developing Mikulicz’ disease and orbital inflammatory pseudotumor. PET-CT was done to assess for disease involvement of other organs. She was noted to have increased uptake in the left ventricular anterior and lateral walls, suggestive of subclinical pericarditis. This disappeared on follow-up PET-CT after steroid therapy.

5 (Previously cited in Supplementary Table A – comorbid periaortitis) Weiss et al., 2018

83-year-old man with remote history of constrictive pericarditis status-post PMID: 30323165 pericardiectomy, who presented with hip pain. Imaging done as part of workup incidentally revealed pulmonary mass and inflammatory AAA. Biopsy of lung mass confirmed IgG4-RD. Histopathology on stored biopsies from pericardiectomy revealed the same.

6 A 73-year-old man with history untreated heart failure presented with what was thought Horie et al., 2016 to be another exacerbation. Echocardiogram showed pericardial effusion. Pericardial fluid was drained and tested positive for IgG4 on immunostaining. Cardiac catheterization was PMID: 27998302 consistent with constrictive pericarditis. PET-CT showed focal FDG uptake throughout pericardium. Symptoms did not respond to diuretics so thoracoscopic pericardiectomy was performed and histopathology confirmed IgG4-RD. Patient had dramatic symptomatic and echocardiographic improvement on steroids.

7 (Previously cited in Supplementary Table A– comorbid periaortitis) Hourai et al., 2016 (Previously cited in Supplementary Table B – comorbid coronary periarteritis) PMID: 26796136

A 75-year-old man underwent CT abdomen for transaminitis . This showed pericardial effusion and periaortic thickening. PET-CT showed increased uptake in pericardium and aorta, as well as FDG-avid lymphadenopathy. Diagnosis was confirmed on lymph node biopsy. Pericardial fluid continued to accumulate, but regressed once steroid therapy was started.

8 A 72-year-old man presented with dyspnea, jugular venous distention, leg edema, and Ibe et al., 2016 hepatomegaly. Echocardiogram showed pericardial effusion and poor ventricular filling. Hemodynamic cardiac catheterization showed constrictive pericarditis. Fenestration PMID: 27044912 procedure was done during which patient was noted to have nodular pericardium. Biopsy confirmed IgG4-RD. Serum IgG4 was also high.

9 A 78-year-old man was worked up for progressive leg edema, pleural effusions, and Kondo et al., 2016 cholestatic liver enzyme elevation. MRCP was suspicious for sclerosing cholangitis. PET-CT showed FDG-avid pleuritis and pericarditis. Hemodynamic cardiac PMID: 27325135 catheterization was consistent with constrictive pericarditis. Given this constellation, IgG4-RD was suspected and confirmed by pleural biopsy and high serum IgG4 level. Patient later required pericardiectomy due to persistent pericarditis symptoms; this was also consistent with IgG4-RD.

10 A 50-year-old woman presented with fever, chills, chest pain, and lymphadenopathy. Matsumiya et al., Inflammatory markers were elevated, raising concern for acute pericarditis. 2015 Echocardiogram showed pericardial thickening. PET-CT showed FDG avidity in the pericardium and mediastinal lymph nodes. Video-assisted thorascopic biopsy of the PMID: 26370858 affected lymph nodes diagnosed IgG4-RD based on high IgG4/IgG ratio.

11 A 65-year-old man presented for scleral icterus and brown urine, and was found to have Mori et al., 2015 hepatomegaly on exam. Labs were most notable for transaminitis, elevated amylase, and elevated IgG4. CT chest and abdomen showed pericardial and pancreatic thickening. PMID: 25986262 Patient met clinical criteria for autoimmune pancreatitis, so pericardial thickening was presumed to represent IgG4-RD multi-organ involvement. Lab and CT findings improved with steroids, supporting this contention.

12 A 78-year-old woman with history of tuberculosis underwent full-body CT for dyspnea, Sendo et al. 2015 and was found to have massive pericardial effusion, diffuse lymphadenopathy, and pancreatic enlargement. Serum IgG4 was elevated and lymph node biopsy was consistent PMID: 25867228 with IgG4-RD; she also met criteria for autoimmune pancreatitis. Pericardiocentesis was performed and, interestingly, ADA was elevated in pericardial fluid but tuberculosis testing was negative. Given this, it was suspected that pericarditis was another IgG4-RD manifestation. This was confirmed on pericardial biopsy.

13 A 58-year-old man with known history of chronic small pericardial effusion (incidentally Seo et al., 2015 found on routine pre-operative echocardiogram) presented many years later after acutely developing dyspnea and orthopnea. Exam was notable for pretibial edema and jugular PMID: 25810739 venous distention, chest X-ray for pleural effusions, echocardiogram for pericardial effusion with constrictive physiology, and lab workup for high serum IgG4. Pathology following pericardiocentesis and pericardial biopsy was equivocal. Patient’s symptoms continued to worsen even after drainage so open pericardiectomy was performed, and this time pathology confirmed IgG4-RD.

14 A 75-year-old man was evaluated for scrotal swelling, leg edema, dyspnea, and Atallah et al., 2014 orthopnea. EKG was notable for new 2:1 AV block. Echocardiogram showed large pericardial effusion and pericardial thickening/calcification. Right heart catheterization PMID: 25062559 was consistent with constrictive pericarditis. Patient subsequently went into cardiac tamponade so was taken for urgent pericardiectomy. Pathology was consistent with IgG4- RD. Interestingly, serum IgG4 was normal but patient had serological evidence of recent Mycoplasma and Coxsackie B infections.

15 (Previously cited in Supplementary Table A – comorbid periaortitis) Ishida et al., 2014

A 71-year-old woman was worked up for dyspnea with CT chest. She had pericardial and PMID: 24551308 pleural effusion and thickening of pericardial and parietal pleura, in addition to

periaortitis. Pleural biopsy confirmed IgG4-RD. Pericarditis seen on imaging was presumed to be due to IgG4-RD based on clinical gestalt. Pericardial effusions resolved after starting steroids.

16 A 60-year-old woman with recently diagnosed Mikulicz’s disease presented in cardiac Morita et al., 2014 tamponade and underwent urgent pericardiocentesis. Workup was then pursued and revealed high serum IL-2 and IgG4, thickened pericardium on chest CT, and increased PMID: 25087832 uptake around the heart on 67-gallium scintigram. Open pericardial and lymph node biopsy was performed, which confirmed IgG4-RD.

17 An 81-year-old man was admitted with a new diagnosis of heart failure and multiple Yanagi et al., 2014 physical exam findings concerning for subacute cardiac tamponade, including hypotension, Kussmaul’s sign, pulsus paradoxus, and low-voltage QRS complexes on PMID: 24580953 EKG. Lab workup was notable for elevated IgG4. CT chest showed pericardial thickening without calcifications. Echocardiogram showed severe biventricular diastolic dysfunction. Hemodynamic cardiac catheterization confirmed constrictive pericarditis. Patient underwent waffle procedure and pathology confirmed IgG4-RD. Post-operative hemodynamic cardiac catheterization showed inadequate improvement, so he was started on steroids with subsequent drop in IgG4, recovery of diastolic function, and symptomatic improvement.

18 A 59-year-old man was diagnosed with right heart failure and diastolic dysfunction by Matsuzaki et al. 2013 echocardiogram after presenting with dyspnea and leg edema. Cardiac catheterization diagnosed constrictive pericarditis and ruled out CAD. He underwent pericardiectomy PMID: 24322313 and pathology showed IgG4-RD. Serum IgG4 was elevated as well.

19 The clinical data of 11 patients previously diagnosed with various cardiovascular Sakamoto et al., 2012 disorders, including 2 with pericardial fibrosis, was retrospectively analyzed. Both pericarditis patients ultimately died of respiratory failure secondary to massive pericardial PMID: 22154614 and pleural effusions. One of the 2 had a prior diagnosis of autoimmune pancreatitis, markedly elevated serum IgG4 levels, on admission, and IgG4-positive plasma cell infiltrate into the pericardium on autopsy, all consistent with IgG4-RD.

20 A 76-year-old man was diagnosed with heart failure after developing progressive Sekiguchi et al., 2012 exertional dyspnea, leg edema, and ascites. As part of his diagnostic workup he underwent echocardiogram and hemodynamic cardiac catheterization, which showed PMID: 22438527 constrictive pericarditis. Labs were notable only for hypergammaglobulinemia. Pericardiectomy was performed and resected pericardium was positive for IgG4 infiltrate.

21 A 29-year-old woman underwent multiple thoracenteses for recurrent pleural effusions. Sekiguchi et al., 2012 Eventually, CT chest showed loculated pleural effusion and pericardial thickening; echocardiography showed constrictive hemodynamics. Pleural biopsy confirmed IgG4- PMID: 22948582 RD. Repeat echocardiogram after starting steroids had normalized.

22 A 69-year-old man was evaluated for several months of progressive leg edema. Kabara et al., 2011 Echocardiogram and chest CT revealed large pericardial effusion. CT also revealed periaortic mass compressing the inferior vena cava, suspicious for retroperitoneal fibrosis. PMID: 21194763 Pericardiocentesis was performed and pericardial fluid was found to be rich in IgG4- positive plasma cells on cytology, confirming IgG4-RD. Serum IgG4 was elevated as well.

23 A 63-year-old woman with history of autoimmune pancreatitis and Hashimoto thyroiditis Rossi et al., 2009 presented with recurrent pleural and pericardial effusions unresponsive to antibiotics. CT chest showed pericardial thickening and mediastinal lymphadenopathy. Pleural tap was PMID: 19194278 notable for lymphoplasmacytic infiltrate. IgG4-RD was suspected given her history, so serum IgG4 was checked and found to be markedly elevated. She experienced a dramatic response to steroids.

24 A 68-year-old man was admitted with progressive leg edema and pleural effusion. Chest Sugimoto et al., 2008 CT showed pericardial thickening and cardiac catheterization showed constrictive pericarditis. He underwent pericardiectomy, but constrictive pericarditis was initially PMID: 17727980 presumed to be idiopathic and not worked up further. 6 months later, he abruptly

developed fever of unknown origin. As part of his workup, IgG4 was measured and noted to be high. Pericardiectomy specimens were then reevaluated and found to IgG4-positive plasma cell infiltrate.

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Shakir A, et al. Heart 2021;0:1–9. doi: 10.1136/heartjnl-2020-318041