JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-09-03DOI: 10.1016/j.jaccas.2026.110029
Giulia Ballan MD, Davide Genovese MD, PhD, Alberto Bortolato MD, Alberto Nese MD, Carlo Cernetti MD, Giuseppe Minniti MD
{"title":"Four Synchronous Myxomas Unmasking Carney Complex","authors":"Giulia Ballan MD, Davide Genovese MD, PhD, Alberto Bortolato MD, Alberto Nese MD, Carlo Cernetti MD, Giuseppe Minniti MD","doi":"10.1016/j.jaccas.2026.110029","DOIUrl":"10.1016/j.jaccas.2026.110029","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac myxomas are the most common primary cardiac tumors and are usually solitary and sporadic. The presence of multiple synchronous lesions should raise suspicion for an underlying syndromic condition such as Carney complex (CNC).</div></div><div><h3>Case Summary</h3><div>A 39-year-old asymptomatic man underwent transthoracic echocardiography for recurrent ventricular extrasystoles on electrocardiogram, which incidentally revealed multiple intracardiac masses. Multimodality imaging, including transesophageal echocardiography, computed tomography, and cardiac magnetic resonance, identified 4 distinct, highly mobile lesions involving the left ventricle, the mitro-aortic curtain, and left atrium, with features suggestive of myxomas. Given the high embolic risk, surgical excision was performed with concomitant mitral valve repair. Histopathological analysis confirmed cardiac myxomas, whereas immunohistochemistry showed reduced protein kinase A regulatory subunit 1-alpha expression. Genetic testing subsequently identified a protein kinase A regulatory subunit 1-alpha autosomal mutation, establishing the diagnosis of CNC.</div></div><div><h3>Discussion</h3><div>This case underscores the importance of multimodality imaging for accurate diagnosis and surgical planning. The detection of multiple synchronous cardiac masses should prompt genetic evaluation and long-term surveillance for CNC in clinical practice, because this syndrome may lead to endocrinological diseases such as Cushing syndrome and malignant endocrine tumors.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 110029"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-08-08DOI: 10.1016/j.jaccas.2026.109794
Javier Torres-Valencia MD, MSc, Gabriela Zavaleta-Camacho MD, Luisser Saavedra-Cordova MD, Fernando Untiveros-Mayorga MD, Bryan Angulo-Garcia MD, Lucia Ronceros-Salas MD, Carlos Diaz-Arocutipa MD
{"title":"AL Cardiac Amyloidosis in Resource-Limited Settings","authors":"Javier Torres-Valencia MD, MSc, Gabriela Zavaleta-Camacho MD, Luisser Saavedra-Cordova MD, Fernando Untiveros-Mayorga MD, Bryan Angulo-Garcia MD, Lucia Ronceros-Salas MD, Carlos Diaz-Arocutipa MD","doi":"10.1016/j.jaccas.2026.109794","DOIUrl":"10.1016/j.jaccas.2026.109794","url":null,"abstract":"<div><div>Amyloid light chain (AL) cardiac amyloidosis remains a diagnostic challenge in resource-limited settings. We present 3 cases with varying heart failure presentations, all demonstrating concentric left ventricular hypertrophy with characteristic apical sparing strain pattern on echocardiography. Serum free light chains were consistently abnormal, though immunofixation was negative in 2 patients. Traditional screening biopsies were negative in 2 cases, requiring endomyocardial biopsy for diagnosis. Mass spectrometry for amyloid typing was unavailable; immunofluorescence performed in 1 patient confirmed lambda light chain deposition. All patients received CyBorD-based therapy; 1 patient additionally accessed daratumumab abroad and showed marked improvement, while another died within 6 months on CyBorD alone. These cases highlight the heterogeneous presentation of presumed AL amyloidosis and underscore how diagnostic barriers contribute to delayed diagnosis and more advanced disease at presentation, emphasizing the urgent need for improved diagnostic capabilities and therapeutic accessibility in resource-constrained settings.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109794"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148697640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sheath-Assisted Redo Left Bundle Branch Area Pacing Without Additional Venous Puncture","authors":"Yohei Hiruma MD, Noriaki Moriyama MD, Hikaru Tanemura MD, Kunihiko Shimizu MD, Masato Murakami MD, PhD, Shigeru Saito MD","doi":"10.1016/j.jaccas.2026.109785","DOIUrl":"10.1016/j.jaccas.2026.109785","url":null,"abstract":"<div><h3>Background</h3><div>Left bundle branch area pacing (LBBAP) requires delivery-sheath support for implantation and revision. Redo procedures for lead-related complications may require additional venous puncture to regain access.</div></div><div><h3>Case Summary</h3><div>A 79-year-old man underwent LBBAP for complete atrioventricular block. Four days later, abrupt threshold elevation and loss of capture occurred. Fluoroscopy showed complete interventricular septal perforation with lead-tip migration into the left ventricular cavity. Lead revision was performed using a sheath-assisted access-preserving technique without additional venous puncture. A modified 4-F short sheath was advanced over the existing lead, allowing guidewire insertion, sheath upsizing, and successful LBBAP lead reimplantation.</div></div><div><h3>Discussion</h3><div>Delayed septal perforation is a rare but recognized LBBAP complication. This case demonstrates a simple technique using standard sheaths and guidewires that may reduce invasiveness during selected redo LBBAP procedures.</div></div><div><h3>Take-Home Message</h3><div>Sheath-assisted access preservation can facilitate LBBAP lead revision without additional venous puncture.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109785"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148701292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-08-20DOI: 10.1016/j.jaccas.2026.109907
David Bienjonetti-Boudreau MD, Jonathan Smerling MD, Shankar Baskar MD, David S. Spar MD, Richard J. Czosek MD, Chad Connor MD
{"title":"ICE Catheter as Transesophageal Probe in Children With Congenital Heart Disease Undergoing an Electrophysiology Study","authors":"David Bienjonetti-Boudreau MD, Jonathan Smerling MD, Shankar Baskar MD, David S. Spar MD, Richard J. Czosek MD, Chad Connor MD","doi":"10.1016/j.jaccas.2026.109907","DOIUrl":"10.1016/j.jaccas.2026.109907","url":null,"abstract":"<div><h3>Background</h3><div>Intracardiac echocardiography (ICE) is conventionally used as an endovascular imaging modality during electrophysiology procedures. Reports of transesophageal ICE are limited and have not included pediatric electrophysiology procedures.</div></div><div><h3>Case Summary</h3><div>We report the first case series of transesophageal ICE (NuVision NAV) in 6 infants and young children with congenital heart disease undergoing electrophysiology studies and catheter ablation. Indications included preoperative conduction mapping and clinical arrhythmias.</div></div><div><h3>Discussion</h3><div>Transesophageal placement preserves the imaging advantages of ICE while avoiding large-bore venous access in small children without compromising image quality. The technique facilitates electroanatomic mapping by providing accurate delineation of the atrioventricular annulus in patients with complex congenital heart disease.</div></div><div><h3>Take-Home Message</h3><div>Transesophageal ICE with four-dimensional multiplanar imaging provides high-quality real-time anatomical guidance for electrophysiology procedures in infants and young children with complex congenital heart disease and may offer a practical alternative to intravascular ICE when large-bore venous access is undesirable.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109907"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Unexpected Postoperative Remodeling of a Retroesophageal Descending Aorta After Neonatal Arch Reconstruction","authors":"Lisheng Lin MD, PhD, Masakazu Abe MD, PhD, Masaki Tominaga MD, Shushi Fujisato MD, Junko Shiono MD, PhD","doi":"10.1016/j.jaccas.2026.109893","DOIUrl":"10.1016/j.jaccas.2026.109893","url":null,"abstract":"<div><h3>Case Summary</h3><div>A neonate with an interrupted aortic arch type A underwent successful neonatal arch reconstruction. Preoperative computed tomography demonstrated a retroesophageal descending aorta coursing on the right side of the vertebral body. Follow-up imaging 8 months after surgery unexpectedly demonstrated complete relocation of the descending aorta to the left side of the vertebral body. An altered aortic course was confirmed by catheter angiography.</div></div><div><h3>Take-Home Message</h3><div>Postoperative remodeling of the retroesophageal descending aorta may occur after neonatal arch reconstruction and should be recognized during postoperative imaging assessment.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109893"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-08-28DOI: 10.1016/j.jaccas.2026.109906
Rohan Shah MD, Kareem Elgendi DO, Luke Thompson DO, Christopher Wilson DO, Ali Abdul Jabbar MD
{"title":"Left Atrial Mass After Patent Foramen Ovale Closure","authors":"Rohan Shah MD, Kareem Elgendi DO, Luke Thompson DO, Christopher Wilson DO, Ali Abdul Jabbar MD","doi":"10.1016/j.jaccas.2026.109906","DOIUrl":"10.1016/j.jaccas.2026.109906","url":null,"abstract":"<div><h3>Image(s)</h3><div>Transesophageal echocardiography, cardiac computed tomography, and histopathology.</div></div><div><h3>Case Summary</h3><div>A 50-year-old man with previous cryptogenic stroke and patent foramen ovale closure was found to have a large mobile mass on the left atrial side of the occluder approximately 33 months after implantation. Surgical extraction was performed because of embolic risk. Pathology demonstrated benign myocardium mixed with adipose tissue, supporting atypical endothelialization rather than thrombus or infection.</div></div><div><h3>Take-Home Messages</h3><div>Late masses on patent foramen ovale occluder devices may mimic thrombus or vegetation. Multimodality imaging, surgical inspection, and pathology are essential for diagnosis.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109906"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-08-29DOI: 10.1016/j.jaccas.2026.109900
Gabrielle A. Orie MS, Karunakar Vadlamudi MD, Bayan Issa MD, Joseph D. Kuebler MD, Glenn T. Leonard MD
{"title":"Novel Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm in Repaired Tetralogy of Fallot","authors":"Gabrielle A. Orie MS, Karunakar Vadlamudi MD, Bayan Issa MD, Joseph D. Kuebler MD, Glenn T. Leonard MD","doi":"10.1016/j.jaccas.2026.109900","DOIUrl":"10.1016/j.jaccas.2026.109900","url":null,"abstract":"<div><h3>Background</h3><div>Ruptured sinus of Valsalva aneurysm (RSVA) following tetralogy of Fallot (TOF) repair is exceedingly rare, with only isolated cases reported.</div></div><div><h3>Case Summary</h3><div>A 53-year-old man with TOF repaired in infancy presented with NYHA functional class IV heart failure. Cardiac computed tomography showed a ruptured right sinus of Valsalva (28.1 × 33.5 mm) draining into the main pulmonary artery. Cardiac catheterization confirmed a left-to-right shunt (pulmonary-to-systemic flow ratio, 2.6:1) with normal pulmonary vascular resistance. Given the prohibitive surgical risk, a 25-mm Harmony pulmonary valve was deployed, achieving complete RSVA occlusion, restoring pulmonary valve competence, and reducing right ventricular pressure. Functional status improved to NYHA functional class II.</div></div><div><h3>Discussion</h3><div>We describe a novel transcatheter approach in repaired TOF, simultaneously addressing RSVA and severe pulmonary regurgitation in a single intervention.</div></div><div><h3>Take-Home Messages</h3><div>RSVA should be suspected in adults with repaired TOF presenting with unexplained heart failure. Transcatheter closure with a Harmony valve may be feasible in carefully selected high-risk surgical candidates with suitable anatomy and concomitant pulmonary valve disease.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109900"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-09-11DOI: 10.1016/j.jaccas.2026.110273
Maroun Chedid MD, Collin Barrett MD, Melanie Sun MS, Andrew Lesser MD, Katarxyna Hryniewicz MD
{"title":"Late Anthracycline Cardiotoxicity Mimicking Arrhythmogenic Cardiomyopathy in a Young Adult","authors":"Maroun Chedid MD, Collin Barrett MD, Melanie Sun MS, Andrew Lesser MD, Katarxyna Hryniewicz MD","doi":"10.1016/j.jaccas.2026.110273","DOIUrl":"10.1016/j.jaccas.2026.110273","url":null,"abstract":"<div><h3>Background</h3><div>Doxorubicin-induced cardiotoxicity can manifest years after exposure and may mimic other cardiomyopathies in young patients, making the diagnosis of delayed doxorubicin cardiotoxicity challenging in these patients.</div></div><div><h3>Case Summary</h3><div>A 26-year-old physically active man with childhood Burkitt’s lymphoma treated with doxorubicin (120 mg/m<sup>2</sup> cumulative) presented with exertional chest discomfort and palpitations. Resting electrocardiogram and ambulatory monitoring were unremarkable. Transthoracic echocardiography demonstrated preserved ejection fraction (55%-60%) but reduced global longitudinal strain (−15%). Cardiac magnetic resonance revealed low-normal biventricular function with nonischemic septal late gadolinium enhancement and reduced strain. Exercise stress testing demonstrated impaired contractile reserve, septal mechanical dyssynchrony, and exercise-induced QRS prolongation. Coronary computed tomography angiography excluded coronary artery disease.</div></div><div><h3>Discussion</h3><div>This case highlights that anthracycline cardiotoxicity may present late, mimic myocardial ischemia, arrhythmogenic cardiomyopathy, and other cardiomyopathies.</div></div><div><h3>Take-Home Message</h3><div>A high index of suspicion is required for late cardiotoxicity in young cancer survivors.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 110273"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148945002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JACC. Case reportsPub Date : 2026-10-21Epub Date: 2026-07-31DOI: 10.1016/j.jaccas.2026.109541
Aya Elalfy DO, Zeyad Kholeif MD, Rana Rashwan MD, Carlos Vergara Sanchez MD, Awais Malik MD
{"title":"Wild-Type Transthyretin Amyloid Cardiomyopathy With Positive 18F-FDG/13N-Ammonia Cardiac Positron Emission Tomography","authors":"Aya Elalfy DO, Zeyad Kholeif MD, Rana Rashwan MD, Carlos Vergara Sanchez MD, Awais Malik MD","doi":"10.1016/j.jaccas.2026.109541","DOIUrl":"10.1016/j.jaccas.2026.109541","url":null,"abstract":"<div><h3>Background</h3><div>Wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) is increasingly recognized with advances in noninvasive imaging and disease-modifying therapies. However, atypical imaging findings may mimic other cardiomyopathies and delay diagnosis.</div></div><div><h3>Case Summary</h3><div>An 85-year-old man was treated for presumed isolated cardiac sarcoidosis after <sup>18</sup>F-fluorodeoxyglucose (FDG)/<sup>13</sup>N-ammonia positron emission tomography/computed tomography (PET/CT) showed matching perfusion defects with focal FDG uptake. Despite methotrexate therapy, he developed progressive heart failure. Serial echocardiography demonstrated increasing left ventricular wall thickness and worsening global longitudinal strain with apical sparing, raising suspicion for amyloidosis. Repeat PET/CT showed persistent perfusion defects but resolution of FDG uptake. Cardiac amyloid scintigraphy revealed grade 3 myocardial tracer uptake (heart-to-contralateral ratio 1.74). Negative monoclonal studies and transthyretin genetic testing confirmed wtATTR-CM. Methotrexate was discontinued, tafamidis was initiated, and symptoms improved.</div></div><div><h3>Discussion</h3><div>In wtATTR-CM, <sup>18</sup>F-FDG/<sup>13</sup>N-ammonia PET/CT may demonstrate myocardial FDG uptake, mimicking inflammatory cardiomyopathy such as cardiac sarcoidosis. Proposed mechanisms include localized inflammation from amyloid deposition, altered myocardial glucose metabolism, or coexistence of inflammatory processes. Serial multimodality imaging was essential for diagnostic clarification and timely targeted therapy.</div></div><div><h3>Take-Home Messages</h3><div>wtATTR-CM may present with myocardial FDG uptake, mimicking inflammatory cardiomyopathy. Multimodality imaging is critical to avoid diagnostic delay and guide appropriate treatment.</div></div>","PeriodicalId":14792,"journal":{"name":"JACC. Case reports","volume":"31 42","pages":"Article 109541"},"PeriodicalIF":0.0,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148630623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}