JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105277
Mohamad S. Alabdaljabar MBBS , Milind Desai MDMBA , Jeffrey B. Geske MD
{"title":"Cardiogenic Shock in Hypertrophic Cardiomyopathy Treated With Mavacamten and Clarithromycin","authors":"Mohamad S. Alabdaljabar MBBS , Milind Desai MDMBA , Jeffrey B. Geske MD","doi":"10.1016/j.jaccas.2025.105277","DOIUrl":"10.1016/j.jaccas.2025.105277","url":null,"abstract":"<div><h3>Background</h3><div>Mavacamten use has potential for clinically significant ventricular systolic dysfunction, including with drug-drug interactions (DDI).</div></div><div><h3>Case Summary</h3><div>A 66-year-old male with symptomatic obstructive hypertrophic cardiomyopathy was treated with mavacamten for 1.5 years. He received treatment for <em>Helicobacter pylori</em> infection with a combination therapy including clarithromycin. The patient developed cardiogenic shock (ejection fraction nadir of 21%) in the setting of new atrial fibrillation (AF) with rapid ventricular response and pulmonary infiltrates. A DDI between clarithromycin and mavacamten was felt to precipitate clinical decline. In this case, cardiogenic shock occurred in the setting of clarithromycin, AF, and possible infection. The CYP3A4 inhibitory effect of clarithromycin likely resulted in accumulation of mavacamten, a cardiac myosin inhibitor approved for treatment of symptomatic obstructive hypertrophic cardiomyopathy (metabolized by CYP2C19-CYP3A4).</div></div><div><h3>Take-Home Messages</h3><div>This unique case highlights the potential for cardiogenic shock with mavacamten in the setting of AF, possible pneumonia, and suspected DDI. When using mavacamten, early recognition of systolic dysfunction and awareness of DDI is important.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105277"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105274
Nathan Hopkins MD, MBiostat , Shaaheen Laher MBBS , Oliver Hayes MBBS , Naim Mridha MBBS , O. Christopher Raffel MBChB
{"title":"Utility of Intravascular Ultrasound to Diagnose Type A Aortic Dissection Presenting as Acute Myocardial Infarction","authors":"Nathan Hopkins MD, MBiostat , Shaaheen Laher MBBS , Oliver Hayes MBBS , Naim Mridha MBBS , O. Christopher Raffel MBChB","doi":"10.1016/j.jaccas.2025.105274","DOIUrl":"10.1016/j.jaccas.2025.105274","url":null,"abstract":"<div><h3>Background</h3><div>Coronary occlusion during type A aortic dissection is a serious complication, associated with high mortality if not identified and treated promptly.</div></div><div><h3>Case Summary</h3><div>We present a case of acute type A aortic dissection, diagnosed with intravascular ultrasound during emergency percutaneous coronary intervention (PCI) for ST-elevation myocardial infarction. Emergency PCI was successfully performed as a bridge to definitive central aortic repair surgery.</div></div><div><h3>Discussion</h3><div>This case highlights the integration of multiple diagnostic cardiac imaging modalities in the time-critical evaluation and management of aortic dissection as a cause for ST-elevation myocardial infarction. This case also demonstrates the coordinated multidisciplinary care essential to the initial and long-term management of this life-threatening condition.</div></div><div><h3>Take-Home Messages</h3><div>This case highlights the ability to promptly diagnose acute type A aortic dissection using intravascular ultrasound in patients presenting with acute coronary syndrome. Initial reperfusion with PCI can provide a life-saving bridge to definitive surgical repair.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105274"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105309
Jingwen Zhang MD , Lori R. Roth PA-C , Isabel Wees MD , Lauren Phillips MD , Rose Pedretti PhD , Pauline Nguyen MS , Lorena Saelices PhD , Justin L. Grodin MD, MPH
{"title":"A Rare Unusually Aggressive Form of Hereditary Transthyretin Amyloidosis","authors":"Jingwen Zhang MD , Lori R. Roth PA-C , Isabel Wees MD , Lauren Phillips MD , Rose Pedretti PhD , Pauline Nguyen MS , Lorena Saelices PhD , Justin L. Grodin MD, MPH","doi":"10.1016/j.jaccas.2025.105309","DOIUrl":"10.1016/j.jaccas.2025.105309","url":null,"abstract":"<div><h3>Background</h3><div>Hereditary transthyretin amyloidosis (ATTRv) is more typically associated with earlier onset and neurological symptoms compared with wild-type ATTR, although a very early, very symptomatic disease presentation is rare.</div></div><div><h3>Case Summary</h3><div>A 21-year-old male with gastrointestinal distress and neuropathy underwent cardiac evaluation after gastrointestinal biopsies identified ATTR. Family history was notable for amyloidosis and premature deaths. Examination showed neurological deficits without clinical heart failure, and echocardiogram showed concentric left ventricular remodeling. <em>TTR</em> gene sequencing identified a rare pathogenic variant, p.Phe84Ser. Magnetic resonance imaging demonstrated diffuse leptomeningeal enhancement of the brain and spine. Treatment with vutrisiran and tafamidis was initiated.</div></div><div><h3>Discussion</h3><div>The p.Phe84Ser variant is strongly associated with central nervous system and gastrointestinal involvement. This patient had an early phenotypic presentation with leptomeningeal amyloidosis, which is exceedingly rare.</div></div><div><h3>Take-Home Message</h3><div>Understanding the phenotype associated with this ATTRv genotype, along with the patient's family history, guided targeted and timely evaluation and treatment.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105309"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195816","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105286
Miranda J. Flores BS , Lauren Sell NP , Anjan S. Batra MD , Sanjay P. Sinha MD , Gira S. Morchi MD , Joanne P. Starr MD , Anthony C. McCanta MD, CEPS-PC
{"title":"Integrated Multimodal Imaging for Transvenous Transpulmonary Atrial Pacing Lead Implantation in Fontan Patient","authors":"Miranda J. Flores BS , Lauren Sell NP , Anjan S. Batra MD , Sanjay P. Sinha MD , Gira S. Morchi MD , Joanne P. Starr MD , Anthony C. McCanta MD, CEPS-PC","doi":"10.1016/j.jaccas.2025.105286","DOIUrl":"10.1016/j.jaccas.2025.105286","url":null,"abstract":"<div><h3>Background</h3><div>Pacemaker placement in Fontan patients is challenging owing to lack of venous continuity with the atrial and ventricular myocardium. The standard epicardial approach is complicated by the need for repeat sternotomy or thoracotomy as well as the overall long-term risk of lead failure. Transvenous lead implant has been described, but this requires transpulmonary or transbaffle puncture, which have risks of bleeding and pericardial effusion.</div></div><div><h3>Case Summary</h3><div>We describe a case in which multimodal imaging with fluoroscopy, computed tomography, intracardiac echocardiography, and three-dimensional electroanatomical mapping were combined to perform successful transvenous transpulmonary epicardial atrial lead placement in an extracardiac Fontan patient with protein-losing enteropathy.</div></div><div><h3>Discussion</h3><div>This approach can be considered as an alternative to epicardial lead placement in Fontan patients who are at moderate to high risk with the standard epicardial approach.</div></div><div><h3>Take-Home Messages</h3><div>Congenital heart patients with extracardiac Fontan repairs pose unique technical challenges to pacemaker implantation given the lack of venous continuity with the myocardium. The transvenous, transpulmonary approach to epicardial atrial lead placement can be considered in high-risk extracardiac Fontan patients, using integrated multimodal imaging techniques to maximize safety and success.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105286"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"IVL-Assisted Mitral Valvuloplasty","authors":"Filippo Novarese MD , Matteo Vercellino MD , Italo Porto MD, PhD","doi":"10.1016/j.jaccas.2025.105216","DOIUrl":"10.1016/j.jaccas.2025.105216","url":null,"abstract":"<div><h3>Objective</h3><div>To describe a novel intravascular lithotripsy (IVL)–assisted technique for percutaneous balloon mitral valvuloplasty in patients with severe mitral stenosis and heavy leaflet calcification. The approach employs dual steerable sheaths and looped 0.018-inch guidewires to ensure optimal balloon positioning and procedural stability.</div></div><div><h3>Key Steps</h3><div>1) Midposterior transseptal puncture with balloon septostomy; 2) insertion of 2 Agilis steerable sheaths via dual femoral venous access; advancement of looped 0.018-inch guidewires to left ventricular apex; 3) positioning of 2 12-mm IVL balloons across the calcified leaflets; 4) lithotripsy pulse delivery followed by percutaneous balloon mitral valvuloplasty using a 22 × 40 mm balloon.</div></div><div><h3>Potential Pitfalls</h3><div>Complex dual sheath manipulation, risk of subvalvular injury, and dependence on advanced imaging and operator expertise may limit reproducibility in less experienced centers.</div></div><div><h3>Take-Home Message</h3><div>The combination of steerable sheaths and looped guidewires enhances IVL balloon control and efficacy, offering a viable strategy for high-risk patients with severely calcified mitral valves.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105216"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105311
S. Wiltshire BMed , F. Collins MBBS , E. Kotlyar MBBS, MD, MPVD , K. Kearney MBBS , E. Lau BSc, MBBS, PhD , P. Macdonald MBBS, MD, PhD , D. Boshell MBBS , R. Cordina MBBS (Hons), PhD
{"title":"Pulmonary Artery Stenting for Widespread Distal Congenital Stenoses","authors":"S. Wiltshire BMed , F. Collins MBBS , E. Kotlyar MBBS, MD, MPVD , K. Kearney MBBS , E. Lau BSc, MBBS, PhD , P. Macdonald MBBS, MD, PhD , D. Boshell MBBS , R. Cordina MBBS (Hons), PhD","doi":"10.1016/j.jaccas.2025.105311","DOIUrl":"10.1016/j.jaccas.2025.105311","url":null,"abstract":"<div><h3>Background</h3><div>Pulmonary artery stenting is rarely reported in adults with distal and widespread congenital pulmonary artery stenoses or hypoplasia.</div></div><div><h3>Case Summary</h3><div>A 35-year-old woman with diffuse distal congenital pulmonary artery hypoplasia and symptomatic right heart failure underwent pulmonary artery stenting, experiencing significant clinical improvement. A 62-year-old woman with widespread distal pulmonary artery stenoses underwent stenting owing to symptomatic nonsustained ventricular tachycardia and poor right ventricular contractile reserve. Her arrhythmia resolved and she experienced improvement of right ventricular contractile reserve. Both patients have an elastin gene mutation.</div></div><div><h3>Discussion</h3><div>There are limited reports of percutaneous intervention for adults with distal and widespread pulmonary artery stenoses or hypoplasia. We describe 2 successful cases of pulmonary artery stenting in this population.</div></div><div><h3>Take-Home Message</h3><div>Distal and widespread congenital pulmonary artery stenoses or hypoplasia can be treated with stenting when performed in well-selected patients by an experienced proceduralist in the right clinical setting.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105311"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105279
Suman Tandon MD , Omar K. Khalique MD , Lu Q. Chen MD
{"title":"First Post-Approval Clinical Use of F-18 Flurpiridaz With Exercise Cardiac PET in the United States","authors":"Suman Tandon MD , Omar K. Khalique MD , Lu Q. Chen MD","doi":"10.1016/j.jaccas.2025.105279","DOIUrl":"10.1016/j.jaccas.2025.105279","url":null,"abstract":"<div><h3>Background</h3><div>F-18 flurpiridaz is a novel radiotracer approved for positron emission tmography (PET) myocardial perfusion imaging. Its long half-life supports exercise stress testing, and its short positron range and high myocardial extraction fraction provide superior image resolution.</div></div><div><h3>Early Reports Summary</h3><div>A 70-year-old man with known coronary artery disease and prior coronary revascularization underwent exercise cardiac PET with F-18 flurpiridaz for chest discomfort. PET showed ischemia in the left anterior descending and right coronary artery territories. Coronary angiography revealed a severe mid-left anterior descending lesion and a severe proximal lesion in a small-caliber right posterior descending artery.</div></div><div><h3>Discussion</h3><div>Ischemia on PET correlated with coronary angiography and optical coherence tomography, guiding revascularization and altering clinical management.</div></div><div><h3>Novelty</h3><div>This case represents the first post-approval clinical experience of F-18 flurpiridaz with exercise cardiac PET imaging in the United States.</div></div><div><h3>Take-Home Message</h3><div>F-18 flurpiridaz has made exercise cardiac PET feasible in the clinical setting.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105279"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105315
Admire Hlupeni MD, MSc, MPhil , Adebisi Adejola MD , Amirseena Tolebeyan MD , Jeramey Stewart MD , Joseph Fritz MD
{"title":"Culture-Confirmed Histoplasma Endocarditis on Bioprosthetic Mitral Valve Managed Successfully Without Surgery","authors":"Admire Hlupeni MD, MSc, MPhil , Adebisi Adejola MD , Amirseena Tolebeyan MD , Jeramey Stewart MD , Joseph Fritz MD","doi":"10.1016/j.jaccas.2025.105315","DOIUrl":"10.1016/j.jaccas.2025.105315","url":null,"abstract":"<div><h3>Background</h3><div>Prosthetic valve endocarditis due to <em>Histoplasma capsulatum</em> is exceedingly rare and difficult to diagnose.</div></div><div><h3>Case Summary</h3><div>A 79-year-old man with a bioprosthetic mitral valve presented with subacute cognitive decline, pancytopenia, and hypercalcemia. He was afebrile and hemodynamically stable. Echocardiography showed vegetations on the valve. Initially, blood cultures were negative. Urine <em>Histoplasma</em> antigen and serum beta-D-glucan were positive, and fungal blood cultures later grew <em>H capsulatum</em> after 4 weeks of incubation. He was managed with liposomal amphotericin B followed by isavuconazole due to itraconazole contraindications. He gradually recovered without surgical intervention.</div></div><div><h3>Discussion</h3><div>This case illustrates the diagnostic and treatment complexities of <em>Histoplasma</em> endocarditis.</div></div><div><h3>Take-Home Messages</h3><div>In endemic regions, consider <em>Histoplasma</em> in culture-negative prosthetic valve endocarditis. Early diagnosis may rely on beta-D-glucan and <em>H capsulatum</em> urine antigen before cultures become positive. Isavuconazole is a viable alternative when itraconazole is not an option. Selected patients can be managed successfully without surgery.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105315"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195808","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2025-10-01DOI: 10.1016/j.jaccas.2025.105289
HangYu Watson MD , Seher Berzingi MD , Emily Hendricks MD , Megan Vaughan MD, PhD , Sittinun Thangjui MD , Christopher Bianco DO , David Schwartzman MD
{"title":"Electrical Storm After STEMI Refractory to Medications Successfully Managed With Bilateral Sympathetic Ganglionectomy","authors":"HangYu Watson MD , Seher Berzingi MD , Emily Hendricks MD , Megan Vaughan MD, PhD , Sittinun Thangjui MD , Christopher Bianco DO , David Schwartzman MD","doi":"10.1016/j.jaccas.2025.105289","DOIUrl":"10.1016/j.jaccas.2025.105289","url":null,"abstract":"<div><h3>Background</h3><div>Electrical storm is a life-threatening condition often requiring antiarrhythmics, sedation, antiadrenergics, and hemodynamic support. In refractory cases, cardiac sympathetic denervation can help reduce arrythmia burden and implantable cardioverter-defibrillator shocks.</div></div><div><h3>Case Summary</h3><div>A 40-year-old man presented with anteroseptal ST-segment elevation myocardial infarction and newly reduced left ventricular ejection fraction and underwent coronary artery bypass grafting during hospitalization. He subsequently developed refractory monomorphic ventricular tachycardia despite treatment with amiodarone, lidocaine, and a left stellate ganglion block. He was successfully treated with bilateral video-assisted thoracoscopic sympathetic ganglionectomy.</div></div><div><h3>Discussion</h3><div>In patients presenting with electrical storm, particularly those with ischemic cardiomyopathy, bilateral sympathetic ganglionectomy can be an effective treatment when ventricular arrhythmias are refractory to antiarrhythmic drugs and device optimization.</div></div><div><h3>Take-Home Messages</h3><div>This case highlights that bilateral sympathetic ganglionectomy offers more comprehensive autonomic modulation than unilateral block and may provide definitive control of refractory arrhythmias. Temporary blocks may serve as diagnostic or therapeutic bridges to surgical intervention.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"30 30","pages":"Article 105289"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145195855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}