Mahshad Razaghi, Juan M Farina, Laureve Chollet, Chadi Ayoub, Reza Arsanjani, Kristen A Sell-Dottin
{"title":"Severe Trileaflet Aortic Stenosis Without Significant Valve Calcification in an Older Adult.","authors":"Mahshad Razaghi, Juan M Farina, Laureve Chollet, Chadi Ayoub, Reza Arsanjani, Kristen A Sell-Dottin","doi":"10.14797/mdcvj.1819","DOIUrl":"10.14797/mdcvj.1819","url":null,"abstract":"<p><p>Severe aortic stenosis (AS) is most often caused by progressive valve calcification; however, rare cases occur with little or no calcification. We report a 79-year-old man with a trileaflet aortic valve and progressive, hemodynamically severe AS despite an almost complete absence of valve calcification (aortic valve calcium score of 76.5 Agatston units). Multimodality imaging confirmed the severity of obstruction, and the patient underwent successful surgical bioprosthetic aortic valve replacement with good postoperative outcomes. This case demonstrates that severe AS can occur in elderly patients with trileaflet valves through fibrosis-predominant remodeling without significant calcification, and that computed tomography-based calcium scoring may underestimate disease severity in this rare phenotype.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 1","pages":"42-47"},"PeriodicalIF":0.0,"publicationDate":"2026-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398582/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148585505","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}
Laasya Achanta, Bright Benfor, Dylan E Brooks, Michael J Reardon, Alan B Lumsden
{"title":"Surgical Reconstruction of a Suprahepatic Inferior Vena Cava Aneurysm Using a Tubularized Bovine Pericardial Patch.","authors":"Laasya Achanta, Bright Benfor, Dylan E Brooks, Michael J Reardon, Alan B Lumsden","doi":"10.14797/mdcvj.1841","DOIUrl":"10.14797/mdcvj.1841","url":null,"abstract":"<p><p>Inferior vena cava (IVC) aneurysms are rare clinical entities with fewer than 80 cases reported in the literature. Type I aneurysms involving the suprahepatic IVC present significant surgical challenges due to their proximity to the right atrium and the frequent necessity of cardiopulmonary bypass. We present the case of a 68-year-old female with a symptomatic, enlarging 6-cm type I IVC aneurysm. The patient underwent successful surgical resection and reconstruction using an interposition graft fashioned from a tubularized bovine pericardial patch under cardiopulmonary bypass. The postoperative course was unremarkable, and 6-month surveillance imaging confirmed graft patency without recurrence. This report highlights the feasibility of surgical intervention for symptomatic type I IVC aneurysms and describes a reconstructive technique using biologic material.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 1","pages":"48-52"},"PeriodicalIF":0.0,"publicationDate":"2026-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398581/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148585484","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":"Adult Congenital Heart Disease at the Crossroads of Innovation.","authors":"Valeria E Duarte, Andrea Giulio Quarti","doi":"10.14797/mdcvj.1881","DOIUrl":"https://doi.org/10.14797/mdcvj.1881","url":null,"abstract":"","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"1-4"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330993/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392019","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":"Single Ventricle Fontan: The Basics for General Cardiologists.","authors":"Berardo Sarubbi, Flavia Fusco, Michela Palma, Rosaria Barracano, Giovanni Domenico Ciriello, Nunzia Borrelli, Giancarlo Scognamiglio","doi":"10.14797/mdcvj.1816","DOIUrl":"10.14797/mdcvj.1816","url":null,"abstract":"<p><p>Single-ventricle physiology encompasses a heterogeneous spectrum of congenital cardiac malformations in which a single dominant ventricle supports both pulmonary and systemic circulation. Advances in staged surgical palliation culminating in the Fontan procedure have transformed survival, allowing most patients to reach adulthood. Consequently, general cardiologists increasingly encounter adults with Fontan circulation and its long-term cardiac and extracardiac complications. Although life-saving, Fontan circulation is a palliative physiology characterized by passive pulmonary blood flow, limited preload reserve, and chronic systemic venous hypertension. Over time, patients are at risk for progressive complications including arrhythmias, thromboembolism, ventricular dysfunction, Fontan pathway obstruction, lymphatic disorders including protein-losing enteropathy, hepatic disease, and reduced exercise capacity. This review provides general cardiologists with a practical, physiology-based overview of single-ventricle anatomy, the surgical pathway to Fontan circulation, common late complications, and principles of lifelong surveillance. Early recognition of deterioration and timely referral to specialized adult congenital heart disease centers are essential to improving outcomes in this growing patient population.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"54-66"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331038/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392105","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":"The Box: A Message from the Heart in 55 Words.","authors":"Justin C Cordova, James B Young, James S Huntley","doi":"10.14797/mdcvj.1656","DOIUrl":"10.14797/mdcvj.1656","url":null,"abstract":"<p><p>This edition of Poet's Pen reflects on \"The Box,\" a 55-word story by Dr. James Huntley, an Oxford- and Cambridge-educated orthopedic surgeon with a passion for narrative medicine. He uses this piece to describe the mysteries of the heart and expertly showcases the effectiveness of this format in connecting physicians to their patients through the traditions of medicine. Created in 1986, 55-word stories are now in their 40th year as a distinct category of writing. Their use has been increasingly associated with the medical humanities, particularly as a means of reflecting on the physician-patient relationship. The form has been integrated into several health education curricula and featured in numerous medical journals, including <i>The Journal of the American Medical Association</i>.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"150-152"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330994/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392110","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}
Maurizio Brighenti, Gabriele Egidy Assenza, Elisabetta Mariucci, Andrea Donti
{"title":"Lifelong Management of Right Ventricular Outflow Tract Dysfunction in Adults with Congenital Heart Disease.","authors":"Maurizio Brighenti, Gabriele Egidy Assenza, Elisabetta Mariucci, Andrea Donti","doi":"10.14797/mdcvj.1797","DOIUrl":"10.14797/mdcvj.1797","url":null,"abstract":"<p><p>Right ventricular outflow tract (RVOT) dysfunction is a common and clinically significant late complication in adults with congenital heart disease (ACHD), often after repair of conotruncal anomalies, Ross intervention, or isolated pulmonary valve disease. Pulmonary regurgitation, stenosis, or mixed lesions can result in RV dilation, dysfunction, arrhythmias, and exercise limitation. RVOT dysfunction often requires either surgical or transcatheter intervention, with careful patient selection, imaging, and planning. Surgical pulmonary valve replacement remains the reference standard for complex anatomies, whereas transcatheter pulmonary valve replacement offers a less invasive, repeatable solution in suitable conduits, bioprostheses, and increasingly often in patched RVOTs. Long-term outcomes have improved with advances in imaging, device technology, and perioperative care; however, complications such as valve degeneration, infective endocarditis, and arrhythmias persist. This review provides a comprehensive synthesis of epidemiology, pathophysiology, indications, surgical and transcatheter management strategies, and lifelong complications after RVOT reintervention in ACHD patients.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"67-77"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392112","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}
Andrea Busti, Giuseppe Annoni, Carlo Pace Napoleone
{"title":"Endocarditis in Adults with Congenital Heart Disease.","authors":"Andrea Busti, Giuseppe Annoni, Carlo Pace Napoleone","doi":"10.14797/mdcvj.1799","DOIUrl":"10.14797/mdcvj.1799","url":null,"abstract":"<p><p>Infective endocarditis (IE) remains a major cause of morbidity and mortality in patients with congenital heart disease (CHD). Advances in medical and surgical care have led to a growing population of adults with CHD who carry a substantially higher lifetime risk of IE than the general population. This increased susceptibility reflects the combined effects of abnormal intracardiac flow patterns, the frequent presence of prosthetic material or intracardiac devices, and repeated surgical and transcatheter interventions. The epidemiology, microbiology, clinical presentation, and management of IE in CHD differ in several important aspects from those observed in patients without CHD. Streptococcal species and <i>Staphylococcus aureus</i> account for the majority of IE cases. Diagnosis is often challenging due to complex cardiac anatomy and the presence of prosthetic material, and therefore it requires a high index of suspicion and multimodality imaging. Medical therapy usually requires prolonged antimicrobial therapy. Surgical intervention is more frequently required than in non-CHD populations, reflecting both the burden of prosthetic material and the higher rate of complications at presentation. Preventive strategies, including meticulous dental and skin hygiene and appropriate antibiotic prophylaxis, remain the cornerstone of IE management in this high-risk population.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"136-149"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330854/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392093","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":"Primary Tricuspid Regurgitation in ACHD: Diagnosis and Treatment.","authors":"Luciana Da Fonseca Da Silva","doi":"10.14797/mdcvj.1813","DOIUrl":"10.14797/mdcvj.1813","url":null,"abstract":"<p><p>Primary tricuspid valve regurgitation includes congenital and acquired disorders, especially Ebstein anomaly and tricuspid valve dysplasia. Severe tricuspid regurgitation causes progressive right atrial and ventricular dilation, arrhythmias, exercise intolerance, impaired left ventricular filling, and eventual biventricular dysfunction. Some patients remain minimally symptomatic for decades despite progressive disease. Poor prognostic factors include severe cardiomegaly, ventricular dysfunction, cyanosis, and arrhythmias. Surgical indications include symptoms, declining exercise capacity, cyanosis, progressive right ventricular enlargement or dysfunction, recurrent arrhythmias, and, in selected asymptomatic patients, severe regurgitation with right ventricular dilation. The Cone repair procedure restores native valve competence, improves right and left ventricular interaction, promotes reverse remodeling, and has low operative risk in experienced centers. Current evidence supports individualized and earlier surgical repair, preferably during childhood and in specialized centers, to improve ventricular function, quality of life, and long-term survival.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"88-97"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330852/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392058","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":"Innovative Management of Rhythm Disorders in Adults with Congenital Heart Disease.","authors":"Stephanie Fuentes Rojas, Paul Khairy","doi":"10.14797/mdcvj.1736","DOIUrl":"10.14797/mdcvj.1736","url":null,"abstract":"<p><p>Adults with congenital heart disease (ACHD) represent a rapidly growing patient population that is uniquely predisposed to rhythm disturbances. Arrhythmia mechanisms are shaped by congenital anatomy, prior surgical repairs, and progressive chamber remodeling, necessitating lesion-specific strategies. Intra-atrial reentrant tachycardia remains the dominant atrial rhythm disturbance in repaired or surgically modified atria, whereas atrial fibrillation is increasingly encountered with advancing age. While pulmonary vein isolation using pulsed field ablation has entered clinical practice, extra-pulmonary triggers and extensive atrial disease pose unique challenges in ACHD. Ventricular tachycardia in repaired tetralogy of Fallot predominantly arises from slow-conducting anatomical isthmuses. Ongoing studies are evaluating whether preventive isthmus ablation at the time of pulmonary valve replacement reduces subsequent arrhythmic risk. Intraoperative conduction mapping has emerged as a powerful tool to identify the His-Purkinje system in real-time during congenital surgery, reducing postoperative atrioventricular block. Device therapy is evolving toward conduction system pacing as a physiologic strategy across a broader spectrum of congenital heart disease subtypes, along with selective use of subcutaneous defibrillators and leadless devices. Risk prediction and sudden death prevention are being refined by the integration of genetics, application of artificial intelligence, and personalized digital twin models. Together, these innovations herald a future of substrate-guided and mechanism-based arrhythmia management in ACHD. This review synthesizes the evidence underpinning these advances, outlines their clinical applications, and identifies priorities for future research aimed at reducing arrhythmia burden, sudden death risk, and long-term morbidity in this complex population.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"108-123"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330855/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392088","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":"Transcatheter Edge-to-Edge Repair in Adult Congenital Heart Disease: Anatomic Challenges, Device Adaptations, and Emerging Evidence.","authors":"Dhaval R Parekh","doi":"10.14797/mdcvj.1840","DOIUrl":"10.14797/mdcvj.1840","url":null,"abstract":"<p><p>Atrioventricular valvular regurgitation (AVVR) is a principal determinant of morbidity and mortality in adults with congenital heart disease (ACHD), and surgical management carries substantial operative risk owing to prior sternotomies, distorted intracardiac anatomy, impaired ventricular function, and lesion-specific hemodynamic fragility. Transcatheter edge-to-edge repair (TEER)-using MitraClip or TriClip (Abbott) or PASCAL/PASCAL ACE (Edwards Lifesciences)-has emerged as a compelling alternative in high-surgical-risk acquired valvular disease and is rapidly expanding into ACHD. We review the anatomic substrate, device-specific technical considerations, preprocedural imaging requirements, and clinical evidence supporting TEER across the principal ACHD substrates: congenitally corrected transposition of the great arteries (TGA) with systemic tricuspid regurgitation, atrial switch repairs for dextro-TGA, atrioventricular septal defects, and single-ventricle Fontan circulations. We also address procedural challenges unique to ACHD, including venous access anomalies, altered transseptal geometry, absent conventional echocardiographic windows, and iatrogenic stenosis risk in morphologically abnormal valves. Multidisciplinary selection integrating ACHD structural expertise, advanced imaging, and procedural TEER experience is requisite for safe outcomes.</p>","PeriodicalId":39207,"journal":{"name":"Methodist DeBakey cardiovascular journal","volume":"22 3","pages":"98-107"},"PeriodicalIF":0.0,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330853/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392094","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}