Methodist Debakey Cardiovasc J
· 2026 · PMID 42403559
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Advances in the management of congenital heart disease have resulted in a rapidly expanding population of adults with congenital heart disease (ACHD), among whom heart failure (HF) has emerged as a leading cause of morbi...Advances in the management of congenital heart disease have resulted in a rapidly expanding population of adults with congenital heart disease (ACHD), among whom heart failure (HF) has emerged as a leading cause of morbidity and mortality. HF in ACHD represents a distinct and heterogeneous clinical entity shaped by lifelong abnormal loading conditions, prior surgical interventions, arrhythmogenic substrates, and limited representation in randomized clinical trials. The systemic ventricle may be morphologically left, right, or single, each conferring unique susceptibility to maladaptive remodeling, myocardial fibrosis, valvular dysfunction, and progressive contractile decline. Accurate diagnosis requires longitudinal, multimodal assessment incorporating echocardiography, cardiovascular magnetic resonance, cardiopulmonary exercise testing, biomarkers, rhythm surveillance, and selective invasive hemodynamic evaluation. Management prioritizes identification and correction of reversible contributors, including residual structural lesions, atrioventricular valve regurgitation, arrhythmias, pulmonary vascular disease, and extracardiac comorbidities. Pharmacologic therapy remains largely extrapolated from acquired HF paradigms and demonstrates variable efficacy across ACHD subgroups, underscoring the need for physiology-driven individualized care within specialized centers. Advanced therapies, including heart transplantation and mechanical circulatory support, are increasingly utilized, with improving outcomes despite higher perioperative complexity. This review presents a ventricle-based framework for understanding the pathophysiology, evaluation, and management of HF in ACHD and highlights critical gaps requiring further investigation.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403558
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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 myste...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 .
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403557
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Adult congenital heart disease (ACHD) is among the most anatomically and physiologically complex domains in cardiovascular medicine. Advances in pediatric cardiac surgery and cardiology have shifted congenital heart dise...Adult congenital heart disease (ACHD) is among the most anatomically and physiologically complex domains in cardiovascular medicine. Advances in pediatric cardiac surgery and cardiology have shifted congenital heart disease (CHD) from a predominantly pediatric condition to a lifelong disease, resulting in a growing adult population that now outnumbers the affected children. In addition, increased detection of CHD in adulthood has broadened the spectrum of ACHD encountered outside specialized centers, underscoring the importance of familiarity among general cardiologists and cardiac imagers. This state-of-the-art review outlines a structured imaging approach for adults with suspected or known CHD. The proposed pathway includes four stages: (1) clinical-anatomic orientation with systematic review of prior data including operative notes and prior imaging; (2) protocolized adult congenital transthoracic echocardiography using sequential segmental analysis; (3) question-driven cardiac magnetic resonance (CMR) with flow quantification; and (4) selective use of cardiac computed tomography (CCT) for detailed cardiac and extracardiac anatomy, coronary artery origins, surgical planning, sternal re-entry and cannulation or in cases of hardware-related imaging limitations. Beyond modality selection, we address the specialized protocol requirements, training milestones, and competency standards expected of ACHD sonographers, CMR, and CCT technologists-all personnel whose expertise is central to diagnostic quality and accuracy in this patient population. Lesion-specific scenarios and case examples illustrate application in practice. A multimodality and team-based strategy supported by standardized protocols and structured reporting is the foundation of accurate, safe, and efficient ACHD imaging in contemporary cardiovascular practice.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403556
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This review article summarizes the pathophysiology, clinical presentation, diagnostic approach, and management principles of unrepaired congenital shunt lesions in adulthood, with emphasis on individualized decision-maki...This review article summarizes the pathophysiology, clinical presentation, diagnostic approach, and management principles of unrepaired congenital shunt lesions in adulthood, with emphasis on individualized decision-making and indications and contraindications for defect closure.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403554
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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 pri...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.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403553
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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 repai...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.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403552
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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 d...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.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403551
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Conotruncal anomalies comprise a heterogeneous group of congenital heart defects arising from abnormal embryologic development of the cardiac outflow tract and the great arteries. Aortic root dilatation represents a freq...Conotruncal anomalies comprise a heterogeneous group of congenital heart defects arising from abnormal embryologic development of the cardiac outflow tract and the great arteries. Aortic root dilatation represents a frequent yet comparatively underexplored finding in this population, and clear guidelines regarding surveillance and surgical treatment remain limited. This article reviews mechanisms of aortic root dilatation, diagnostic evaluation, and surgical indications and principles for aortic root surgery in adults with conotruncal anomalies, including lesion-specific considerations for Tetralogy of Fallot, d-transposition of the great arteries, truncus arteriosus, and double outlet right ventricle.
Sarubbi B, Fusco F, Palma M
… +4 more, Barracano R, Ciriello GD, Borrelli N, Scognamiglio G
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403550
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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 pall...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.
Brighenti M, Assenza GE, Mariucci E
… +1 more, Donti A
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403549
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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,...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.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403548
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These contrasting images are presented by Philip Alexander, MD, a native Texan, retired physician, and accomplished musician and artist. After 41 years as an internal medicine physician, Dr. Phil retired from his practic...These contrasting images are presented by Philip Alexander, MD, a native Texan, retired physician, and accomplished musician and artist. After 41 years as an internal medicine physician, Dr. Phil retired from his practice in College Station in 2016. A lifelong musician and former music professor, he often performs as an oboe soloist for the Brazos Valley Symphony Orchestra. He began exploring visual art in 1980, evolving from pencil sketches-including an official White House portrait of President Ronald Reagan-to the computer-generated drawings featured in this journal. His images, which first appeared in this journal in the spring of 2012, are his own original creations. If you would like to see your art published in the , submit your creation online at journal.houstonmethodist.org as a "Humanities" entry.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403547
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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...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 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.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42403546
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Adults with congenital heart disease (ACHD) represent a growing population of adults in the United States, necessitating lifelong care by ACHD specialists. Often, patients may be lost to follow-up after pediatric care or...Adults with congenital heart disease (ACHD) represent a growing population of adults in the United States, necessitating lifelong care by ACHD specialists. Often, patients may be lost to follow-up after pediatric care or elude detection until adulthood, presenting to the emergency room with common clinical complaints such as chest pain, dyspnea on exertion, or palpitations. This review demystifies common clinical presentations in pulmonary stenosis, branch pulmonary stenosis, coarctation of the aorta, and subaortic stenosis and their associated pathophysiology, salient clinical features, multimodality diagnostic findings, and indication for intervention.
Methodist Debakey Cardiovasc J
· 2026 · PMID 42004176
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We report a case of early migration of a CardioMEMS™ heart failure device (Abbott) from the right to the left pulmonary artery, which then spontaneously repositioned itself within the left pulmonary artery without the ne...We report a case of early migration of a CardioMEMS™ heart failure device (Abbott) from the right to the left pulmonary artery, which then spontaneously repositioned itself within the left pulmonary artery without the need for intervention. A balloon Swan-Ganz catheter was then used to reposition the device, successfully lodging it into a more distal position. This case underscores the importance of close device monitoring and demonstrates an effective technique for repositioning without device retrieval.
Methodist Debakey Cardiovasc J
· 2026 · PMID 41971524
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This case describes a 55-year-old man who presented with complaints of claudication in his left upper limb. Computed tomography angiography revealed a rare anatomical variant in which the left vertebral artery originated...This case describes a 55-year-old man who presented with complaints of claudication in his left upper limb. Computed tomography angiography revealed a rare anatomical variant in which the left vertebral artery originated from the left thyrocervical trunk.
Székely M, Duarte V, Martin C
… +2 more, Reardon MJ, Quarti AG
Methodist Debakey Cardiovasc J
· 2026 · PMID 41971523
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Scimitar syndrome is a rare congenital anomaly characterized by anomalous right pulmonary venous drainage into the inferior vena cava, resulting in a left-to-right shunt. Surgical correction may be achieved by direct rei...Scimitar syndrome is a rare congenital anomaly characterized by anomalous right pulmonary venous drainage into the inferior vena cava, resulting in a left-to-right shunt. Surgical correction may be achieved by direct reimplantation of the scimitar vein or by intra-atrial baffling, which often requires deep hypothermic circulatory arrest. We report the case of a 47-year-old man with symptomatic scimitar syndrome and an infradiaphragmatic scimitar vein who underwent successful intra-atrial baffling without circulatory arrest. Preoperative imaging demonstrated a significant shunt with dilation of the right atrium and ventricle. Repair was performed using cardiopulmonary bypass with femoral venous cannulation and vacuum-assisted lower body venous drainage, allowing adequate visualization despite the challenging infradiaphragmatic localization of the anomalous vein without interruption of systemic perfusion. Postoperative echocardiography confirmed unobstructed pulmonary venous return with minimal gradient. The patient had an uneventful recovery and reported symptomatic improvement at 1-year follow-up. This case demonstrates that intra-atrial baffling of an infradiaphragmatic scimitar vein can be safely performed without circulatory arrest using vacuum-assisted venous drainage.
West J, Hamzeh I, You J
… +6 more, Koutroumpakis E, Iliescu C, Ynalvez L, Ali HJ, Deswal A, Khalaf S
Methodist Debakey Cardiovasc J
· 2026 · PMID 41939966
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Transthoracic echocardiography and global longitudinal strain are pivotal in evaluating myocardial structure and function in cardio-oncology patients, but there are limited reports of using global longitudinal strain to...Transthoracic echocardiography and global longitudinal strain are pivotal in evaluating myocardial structure and function in cardio-oncology patients, but there are limited reports of using global longitudinal strain to identify intracardiac masses. This case presents a patient with metastatic renal cell carcinoma and cardiac metastasis that was identified using global longitudinal strain on transthoracic echocardiography and subsequently confirmed by cardiac magnetic resonance imaging.
Singh M, Sidhu D, Anvekar P
… +3 more, Jain S, Katyal H, Brar V
Methodist Debakey Cardiovasc J
· 2026 · PMID 41939965
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A 66-year-old male with prior right middle cerebral artery stroke status post thrombectomy-as well as right internal carotid artery (ICA) stenting and chronic occlusion, paroxysmal atrial fibrillation, and hypertension-u...A 66-year-old male with prior right middle cerebral artery stroke status post thrombectomy-as well as right internal carotid artery (ICA) stenting and chronic occlusion, paroxysmal atrial fibrillation, and hypertension-underwent left atrial appendage occlusion with a Watchman device for stroke prevention. Immediately following the procedure, he developed acute bilateral vision loss accompanied by headache and worsening left-sided weakness. Emergent computed tomography (CT) and CT angiography demonstrated no hemorrhagic stroke or new large-vessel occlusion and confirmed the previously known chronic right ICA occlusion. The patient was not a candidate for thrombolysis due to recent heparin exposure during his procedure, and thrombectomy was not pursued given the absence of any new large vessel occlusion. In the context of contrast exposure and negative initial imaging, contrast-induced cortical blindness was considered the leading diagnosis, with embolism, hypoperfusion, and post-stroke recrudescence as alternative considerations. This case highlights the diagnostic complexity of acute cortical blindness and hemispatial neglect following Watchman implantation. Mechanisms may include microembolic shower, transient hypoperfusion, seizure-related cortical dysfunction, post-stroke recrudescence, or contrast induced cortical blindness. Negative initial CT imaging should not preclude suspicion for ischemia, especially in patients with preexisting cerebrovascular compromise. Early magnetic resonance imaging and multidisciplinary coordination are essential.
Methodist Debakey Cardiovasc J
· 2026 · PMID 41835356
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Constrictive pericarditis is a condition of diastolic heart failure with unique hemodynamic manifestations. While potentially curable if treated appropriately, constrictive pericarditis remains a diagnostic challenge, pa...Constrictive pericarditis is a condition of diastolic heart failure with unique hemodynamic manifestations. While potentially curable if treated appropriately, constrictive pericarditis remains a diagnostic challenge, particularly during early manifestation of disease when there are subtle clinical symptoms and when confounding comorbidities are present. In this focused review, we describe the characteristic and diagnostic hemodynamics of constrictive pericarditis as well as the utility of invasive (cardiac catheterization) and noninvasive (echocardiography) diagnostic testing for this complex disease.