搜索结果：JACC. Case reports

Apical hypertrophic cardiomyopathy (aHCM) is a distinct, rarer phenotype with a potentially more favorable prognosis and lower morbidity than nonapical forms of hypertrophic cardiomyopathy, yet its specific interaction with elite-level sports remains poorly defined. This case describes an asymptomatic athlete found to have aHCM, for whom engaging in elite sports from ages 17 to 31 years did not lead to adverse morphologic changes or progression of apical fibrosis or disease, even after the cessation of his professional sports career. This case underscores the importance of recognizing aHCM as a distinct subtype of hypertrophic cardiomyopathy, potentially exhibiting a more benign interaction with sports. Furthermore, it highlights the value of a multidisciplinary expert panel in providing sports guidance to elite athletes with cardiovascular conditions. Distinguish aHCM as a subtype that may carry a lower risk profile in elite athletes. Multidisciplinary shared decision-making is essential for individualized sports eligibility.

Loeffler endocarditis is a late manifestation of hypereosinophilic syndromes characterized by endomyocardial fibrosis and intracardiac thrombosis. Early recognition is essential yet challenging because presentation is often nonspecific. Case 1 involved a 43-year-old man with long-standing atopy and persistent eosinophilia who presented with heart failure symptoms. Echocardiography demonstrated biventricular apical thrombi, and cardiac magnetic resonance confirmed ventricular thrombi and revealed diffuse subendocardial late gadolinium enhancement confirming fibrotic-stage disease. High-dose corticosteroids produced rapid hematologic response, and anticoagulation was initiated. Case 2 involved a 38-year-old man who was admitted for abdominal pain and incidentally found to have eosinophilia and right ventricular apical infiltration. Cardiac magnetic resonance established the diagnosis of Loeffler endocarditis. Molecular testing identified FIP1L1-PDGFRA-positive myeloproliferative hypereosinophilic syndrome. Eosinophil counts normalized promptly with imatinib. These cases illustrate a final common cardiac phenotype arising from diverse causes of hypereosinophilia. Imaging was central not only for diagnosis and staging, but also for prompting systemic investigation and directing targeted therapy.

We report a proactive coronary protection strategy during transcatheter aortic valve replacement (TAVR) using intravascular ultrasound (IVUS)-guided decision making and chimney stenting in patients with high-risk anatomy. Two patients with severe symptomatic aortic stenosis and unfavorable coronary anatomy on preprocedural computed tomography underwent TAVR with planned coronary protection. Despite preserved angiographic flow after valve deployment, IVUS identified critical leaflet-to-coronary proximity in one case, whereas significant ostial compromise was detected by angiography in the other. Chimney stenting was performed, ensuring maintained coronary patency. Both patients had favorable clinical outcomes without periprocedural complications and good follow-up results. These cases highlight some limitations of angiography and the complementary value of IVUS in selected situations and support a proactive decision-making process for an imaging-guided approach to coronary protection during TAVR. A proactive, imaging-guided decision-making approach to coronary protection is recommended in cases of high-risk TAVR.

Transcatheter aortic valve replacement (TAVR) valve-in-valve (ViV) is a less invasive alternative to redo surgery for high-risk patients with failed bioprosthetic aortic valves. Left ventricular (LV) perforation is a rare but potentially life-threatening complication, typically requiring surgical repair. Percutaneous closure is seldom reported. An 80-year-old man with NYHA functional class III symptoms from severe degenerative transvalvular bioprosthetic aortic regurgitation underwent TAVR ViV. Postprocedure transthoracic echocardiogram and computed tomography scan revealed a small, contained LV apex perforation, likely iatrogenic from guidewire manipulation. This was successfully closed percutaneously using an Amplatzer Vascular Plug II. This case highlights importance of high-quality post-TAVR ViV transthoracic echocardiography and safety of percutaneous closure of LV apex perforation compared with surgical repair. LV apex perforation is a rare but serious complication of TAVR. Percutaneous closure may avoid high-risk surgery in suitable cases.

Transcarotid-transcatheter aortic valve implantation (TC-TAVI) generally enables smooth transcatheter heart valve crossing given its proximity to the aortic annulus; however, severe anatomical constraints may still lead to delivery failure. We report a TC-TAVI case in which transcatheter heart valve passage across the aortic valve was initially unsuccessful because of aortic angulation and extensive leaflet calcification. This technical difficulty was effectively resolved using the buddy balloon technique, which enabled successful valve advancement. This case demonstrates that the superior pushability of the TC approach may not overcome extreme anatomical resistance, necessitating predilatation aggressively indicated for such challenging anatomies. Pronounced aortic angulation and severe leaflet calcification can hinder transcatheter heart valve delivery, even during TC-TAVI, where easier valve crossing is typically anticipated. These anatomical factors remain significant predictors of delivery failure, irrespective of the vascular access route.

FMS-like tyrosine kinase 3 (FLT3) inhibitors improve outcomes in acute myeloid leukemia cases but may cause cardiovascular toxicity. Inflammatory complications are rarely described. A 56-year-old man with FLT3-mutated acute myeloid leukemia developed acute pleuritic chest pain 3 days after the initiation of FLT3 inhibitor therapy. Electrocardiogram showed diffuse ST-segment elevation, and inflammatory markers were elevated (C-reactive protein 244 mg/L; normal <5 mg/L). Echocardiography revealed mild pericardial effusion. No viral prodrome or infectious cause was identified. Renal function and leukocyte count allowed standard therapy. The drug was discontinued, and treatment with ibuprofen and colchicine led to rapid resolution. FLT3 inhibitors have been associated with cardiotoxicity although pericardial inflammation is uncommon. This case supports a probable drug-related mechanism. FLT3 inhibitors may cause acute pericarditis. Early recognition and drug withdrawal are essential for recovery.

Primary cardiac tumors are rare, and fibromas-benign fibrous tumors of the myocardium-are exceedingly uncommon in adults. Although labeled benign, they may cause arrhythmia, obstructive symptoms, heart failure, or sudden death. Complete surgical resection remains the treatment of choice to alleviate symptoms and prevent complications. We report the case of a 20-year-old woman presenting with progressive shortness of breath and palpitations. Cardiac imaging revealed a large intramyocardial mass consistent with left ventricular fibroma. Surgical resection was performed, and the mass was enucleated in its entirety. The postoperative course was uneventful, and the patient was discharged on postoperative day 5. Histopathology confirmed fibroma with no malignant features. This case highlights successful surgical management of a rare left ventricular fibroma in an adult. Early recognition and complete resection are essential for symptomatic relief and to prevent complications. Left ventricular fibromas, although rare, can cause significant symptoms in young adults. Prompt diagnosis and proper surgical enucleation provide safe and effective treatment.

Protruding coronary ostial stents can be prohibitive for repeat percutaneous coronary interventions (PCI). We retrospectively included 6 consecutive patients who underwent the EXPOSE (extraction of protruding ostial stents with En Snare) technique to previous ostial stents to facilitate subsequent PCI. The indication in all cases was revascularization of the target vessel with failed guide intubation of the stented lumen. The protruding stents had been implanted 5 days to 9 years before extraction. The procedure included a 7-F &#xd7; 45-cm transfemoral sheath, 7-F guide catheter, and 12- to 20-mm En Snare device. Procedural success was achieved in all patients, and complete stent extraction was achieved in 1 patient. All patients underwent successful PCI; 1 case of acute vessel closure immediately postextraction was rescued promptly with PCI. Snare extraction of previously implanted, protruding ostial stents is feasible to enable PCI after failure of guide intubation.

Constrictive pericarditis (CP) is most often idiopathic, postviral, postoperative, or autoimmune in etiology. HLA-B27 is strongly associated with spondyloarthropathies, which may have cardiac manifestations including conduction system abnormalities and aortopathy; however, an association with CP is not well described. A 40-year-old previously healthy man presented with progressive dyspnea, fevers, and cough. Initial imaging demonstrated acute pericarditis with subsequent progression to constrictive physiology. Extensive evaluation was unremarkable except for HLA-B27 positivity. He failed medical therapy and underwent radical pericardiectomy with symptom resolution. Pathology confirmed fibrous pericarditis. This case suggests a potential autoinflammatory role of HLA-B27 in the development of CP. Although HLA-B27 is known to drive chronic inflammation in spondyloarthropathies, and these conditions have established cardiac manifestations, its relationship to pericardial disease remains poorly defined. In otherwise idiopathic cases of CP, isolated HLA-B27 positivity may represent a potential predisposing factor warranting further investigation.

Pulmonary artery aneurysms (PAAs) are rare occurring in approximately 1 in 14,000 cases. While PAA is frequently associated with pulmonary arterial hypertension (PAH), the optimal management strategy remains undefined due to the rarity of the disease. We report a case of a 58-year-old female with longstanding idiopathic PAH who underwent combined heart-lung transplantation to treat a rapidly expanding 8.95-cm PAA with associated cor pulmonale and severe pulmonic valve regurgitation. Graft function remained excellent; however, an ischemic stroke in the postoperative period led to significant morbidity. Despite the known association of PAA with PAH, specific treatment guidelines are lacking. Delay in intervention can lead to poor surgical candidacy and adverse outcomes. PAA may progress despite stable PAH hemodynamics. Combined heart-lung transplantation can be a viable option for select patients with severe PAH, giant PAA, cor pulmonale, and severe pulmonic valve regurgitation.

Internal medicine residents frequently encounter arrhythmias on admission and telemetry, yet studies consistently demonstrate suboptimal electrocardiogram (ECG) interpretation accuracy and inadequate formal training. We implemented a 14-week structured "burst-learning" ECG curriculum at a single safety-net academic internal medicine residency, comprising twelve 15- to 30-minute case-based sessions covering 20 high-acuity rhythms aligned with American College of Cardiology/American Heart Association guidelines.A 20-item knowledge quiz and scenario-linked 5-point Likert confidence survey were administered before and after the curriculum (unpaired cohorts). Twenty-six residents completed baseline and 15 completed postcurriculum assessments. Mean confidence rose from 2.81 &#xb1; 0.94 to 3.48 &#xb1; 0.87 (P = 0.03); knowledge scores did not change meaningfully (from 7.38 &#xb1; 2.35 to 7.87 &#xb1; 1.64; P = 0.45). Postcurriculum status independently predicted higher confidence (&#x3b2; = 0.61; 95% CI: 0.04-1.19; P = 0.038). A brief, low-cost burst curriculum was associated with increased resident ECG confidence and was durably incorporated into the residency schedule. However, diagnostic accuracy gains will require a longer dose and paired measurement.

Acute type A aortic dissection (ATAAD) is a high-mortality surgical emergency, particularly in octogenarians, and may be overlooked with overlapping cardiac symptoms. An 86-year-old woman with severe aortic stenosis and multivessel coronary artery disease presented with chest pain. Three months earlier, she underwent evaluation for transcatheter aortic valve replacement, but no intervention was pursued. Because of persistent chest pain, surgical aortic valve replacement and coronary bypass were planned. Intraoperatively, the ascending aorta appeared abnormal, and transesophageal echocardiography confirmed ATAAD. As the dissection originated in the distal ascending aorta, a focal bovine patch repair was performed without ascending aortic replacement. She recovered and remains alive 3 years later. This case underscores the importance of thorough diagnostic investigation and timely imaging in persistent chest pain and an individualized operative approach when unexpected ATAAD is encountered. Limited repair may be appropriate in carefully selected high-risk elderly patients.

Mavacamten reduces left ventricular outflow tract obstruction in obstructive hypertrophic cardiomyopathy but carries a risk of exposure-dependent systolic dysfunction due to its narrow therapeutic window. A 57-year-old male with obstructive hypertrophic cardiomyopathy was started on mavacamten resulting in progressive reduction in left ventricular outflow tract gradient with preserved left ventricular ejection fraction (LVEF) over the first several months. Around month 5, he began consuming grapefruit-containing energy drinks daily. After approximately 2 months of exposure, LVEF declined to 45% to 50%. Mavacamten and grapefruit exposure were discontinued, resulting in normalization of LVEF. Rechallenge with mavacamten in the absence of grapefruit did not reproduce systolic dysfunction. Clinical trials report reversible LVEF reduction with mavacamten; however, real-world pharmacokinetic interactions are less well described. This case demonstrates a probable grapefruit-mediated interaction leading to transient systolic dysfunction. Unrecognized dietary cytochrome P450 enzyme inhibition may increase mavacamten exposure and cause reversible systolic dysfunction.

Severe rheumatic mitral stenosis during pregnancy carries high maternal-fetal risk. Percutaneous transvenous mitral commissurotomy (PTMC) is the guideline-recommended therapy. A 33-year-old primigravida at 31 weeks' gestation with critical rheumatic mitral stenosis (mitral valve area: 0.47 cm2) and NYHA functional class III symptoms underwent urgent PTMC. Immediately after PTMC, she developed profound hypotension, complete heart block, and an inferior ST-segment elevation myocardial infarction (STEMI). Emergent coronary angiography revealed thrombotic occlusion of the mid right coronary artery with TIMI flow grade 0. Prompt mechanical thrombectomy restored TIMI flow grade 3. The patient delivered vaginally at 36 weeks, and both mother and neonate had favorable outcomes. This case highlights the importance of early recognition of STEMI and demonstrates that a stentless percutaneous coronary intervention can successfully restore coronary flow. Immediate recognition of thrombotic occlusion of coronary artery during PTMC is essential for an emergent tailored stentless percutaneous coronary intervention during pregnancy.

Lyme disease, caused by Borrelia burgdorferi, is the most common vector-borne infection in North America. Cardiac involvement occurs in approximately 1% to 10% of untreated infections and most commonly manifests as atrioventricular conduction abnormalities. A 29-year-old man with arrhythmogenic right ventricular cardiomyopathy underwent orthotopic heart transplantation. Early postoperatively he developed atrial flutter progressing to complete heart block despite preserved graft function and absence of rejection. Donor history revealed recent tick exposure. Warthin-Starry staining of surveillance endomyocardial biopsy demonstrated rare spirochetes consistent with Borrelia species. The patient received intravenous ceftriaxone for presumed donor-derived Lyme carditis but ultimately required permanent pacemaker implantation. This case highlights Lyme carditis as a rare potential cause of early conduction abnormalities after heart transplantation.

Spontaneous coronary artery dissection (SCAD) is frequently associated with systemic arteriopathies, particularly fibromuscular dysplasia. Although extracoronary arterial screening is recommended, abnormalities of the internal mammary artery (IMA), the usual preferred conduit for coronary artery bypass grafting, is rarely described. We report 2 women with systemic arteriopathy and recurrent SCAD who had structural abnormalities of the IMA. The first had Loeys-Dietz syndrome and prior cervical artery dissections, presented with neck pain, and was found to have vertebral artery dissection and focal aneurysmal dilation and suspected dissection of the left IMA. The second had multifocal fibromuscular dysplasia involving renal, cervicocephalic, and thoracic arteries, and presented with recurrent SCAD myocardial infarction. Beaded irregularity of the left IMA was noted. Both patients were managed conservatively. These cases highlight under-recognized IMA involvement in patients with SCAD and systemic arteriopathy, raising considerations regarding conduit assessment when surgical revascularization is contemplated.

Severe congenital mitral valve disease is difficult to manage in infants and small children. Off-label use of a Melody valve in the mitral position (MiM) has emerged as an important bridge to definitive valve replacement, with the potential to reintervene. A 6-year-old boy with Shone's complex and multiple left-sided interventions, including surgical MiM implantation at age 3 months (Melody #1) and transapical MiM valve-in-valve at age 2 years (Melody #2), presented 4 years later with progressive mixed MiM dysfunction. In a hybrid catheterization laboratory, balloon dilation of the existing MiM improved expansion but resulted in worsening regurgitation, necessitating valve replacement. A limited left anterior thoracotomy was performed to facilitate a venoarterial rail, enabling controlled transfemoral advancement and deployment of Melody #3 within the prior MiMs, with good hemodynamic result. Our case demonstrates the feasibility of repeat MiM valve-in-valve therapy, including a third MiM implantation, using a hybrid transapical approach to facilitate transfemoral delivery.

Baffle leaks are a known complication of the atrial switch for D-looped transposition of the great arteries. A 46-year-old woman with D-looped transposition of the great arteries who underwent an atrial switch (Mustard) presented with a hemodynamically significant baffle leak in close proximity to the systemic tricuspid valve. This report details the first-in-human percutaneous closure of a large baffle leak using a newest generation closure device, the GORE CARDIOFORM ASD Occluder (GCA). Baffle leaks are traditionally managed with either surgery or percutaneously with catheter-based devices. The location of the leak in this case was close to the tricuspid valve, and so the newer, softer GCA device was used. This is the first-in-human percutaneous closure of a baffle leak after an atrial switch using the GCA device. The GCA is the newest generation of closure devices that may convey an advantage over previous technology when closing leaks adjacent to important cardiac structures.

Leadless pacemakers reduce complications of transvenous leads. However, interactions between leadless pacemakers and retained transvenous cardiac implantable electronic devices remain an underrecognized issue. A 72-year-old woman with complete heart block underwent dual-chamber pacemaker implantation in 2004 and generator changes and right ventricular lead revision in 2013 for fracture. In 2024, due to lead noise during isometric exercise and concern for lead integrity, leadless pacemaker was implanted without extracting existing system per patient preference. Postimplant the patient reported fatigue and exertional dyspnea initially attributed to COVID-19 or chronotropic incompetence. Device interrogation showed only 9% implant-to-implant (i2i) communication suggesting impaired atrioventricular synchrony. Further evaluation revealed interference from a retained Boston Scientific pacemaker with an active minute ventilation sensor. Deactivation of the sensor restored i2i communication and resolved symptoms. To our knowledge, this is the first reported case of atrioventricular asynchrony specifically resulting from impaired i2i communication in a leadless pacemaker due to interference from a minute ventilation sensor. Comprehensive pre-implantation assessment of cardiac devices is essential to prevent device-device interactions and ensure safe leadless pacemaker function.