Swasthi S Kumar, Sudipta Mondal, Jayakrishnan Radhakrishnan, Ramya Das
{"title":"Large dissecting sinus of Valsalva aneurysm creating a 'triple ventricle' appearance: case report.","authors":"Swasthi S Kumar, Sudipta Mondal, Jayakrishnan Radhakrishnan, Ramya Das","doi":"10.1093/ehjcr/ytaf308","DOIUrl":"10.1093/ehjcr/ytaf308","url":null,"abstract":"<p><strong>Background: </strong>Dissecting aneurysm of the interventricular septum (DAIS) is a rare congenital or acquired anomaly which can have a progressive course. We report a large DAIS incidentally detected by a routine echocardiogram.</p><p><strong>Case summary: </strong>An asymptomatic tricenarian was incidentally detected to a DAIS during a routine pre-operative evaluation. Multimodality imaging with cardiac CT, MRI, and aortic root angiograms confirmed the diagnosis. Work-up for infectious causes like syphilis and inflammatory causes like connective tissue disorders were negative. Patient is planned for surgical repair.</p><p><strong>Discussion: </strong>Dissecting aneurysm of the interventricular septum is a rare anomaly with a poor prognosis. The rupture of sinus of Valsalva aneurysm is the most common cause. Infections like syphilis and infective endocarditis, connective tissue disorders (Marfan syndrome and Ehler-Danlos syndrome), autoimmune diseases (Behcet's disease, ankylosing spondylitis, systemic lupus erythematosus, Takayasu's arteritis), bicuspid aortic valve with aortopathy, atherosclerosis, surgery, or trauma are the reported causes. Such dissecting aneurysms can have a progressive course, leading to right or left ventricular outflow tract obstructions, severe aortic regurgitation, and rupture into ventricular chambers, portending a grave prognosis. Hence, surgical correction is the norm.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf308"},"PeriodicalIF":0.8,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12260221/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642143","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}
Philipp Steinhoff, Roland Schmitz, Christian Burger, Dirk Westermann, Thomas Arentz
{"title":"Fulminant Takotsubo syndrome with cardiogenic shock and mitral-edge-to-edge repair in new severe secondary mitral valve regurgitation after elective implantation of a cardiac resynchronization therapy: a case report.","authors":"Philipp Steinhoff, Roland Schmitz, Christian Burger, Dirk Westermann, Thomas Arentz","doi":"10.1093/ehjcr/ytaf323","DOIUrl":"https://doi.org/10.1093/ehjcr/ytaf323","url":null,"abstract":"<p><strong>Background: </strong>Takotsubo syndrome is a cardiac disease typically characterized by transient ventricular dysfunction. Although long considered benign due to the often reversible course, severe cases, including cardiogenic shock, may occur partially due to cardiac complications, such as severe secondary mitral regurgitation.</p><p><strong>Case summary: </strong>An 81-year-old female patient presented for the elective implantation of cardiac resynchronization therapy (CRT) in preparation of a His-ablation for therapy-refractory, highly symptomatic, paroxysmal atrial fibrillation. Postoperatively, she developed increasing haemodynamic instability, progressing to cardiogenic shock. Echocardiography ruled out pericardial effusion but showed newly developed, significantly reduced left ventricular function as well as new severe secondary mitral regurgitation. Relevant coronary stenosis was ruled out by coronary angiography. Invasive levocardiography revealed the typical pattern of Takotsubo syndrome. Haemodynamic stabilization was achieved by mechanical circulatory support, using the Impella CP device. Attempts to gradually reduce the support level repeatedly failed due to worsening of the mitral regurgitation resulting in pulmonary oedema. Therefore, with no signs for recovery of left ventricular function at that time and considering the patient's severe symptom burden, an urgent mitral edge-to-edge repair was performed with successful reduction of mitral regurgitation. The haemodynamic situation subsequently stabilized, allowing for the successful weaning and eventually removal of the temporary circulatory support after 5 days. Left ventricular function eventually showed near-complete recovery after 15 days.</p><p><strong>Discussion: </strong>Even after elective cardiac procedures, such as CRT implantation, fulminant Takotsubo syndrome should be considered as a differential diagnosis in cases of haemodynamic instability. Then, temporary mechanical circulatory support and interventional treatment of secondary complications, such as secondary mitral regurgitation, may be necessary for stabilization.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf323"},"PeriodicalIF":0.8,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12287914/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144706841","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}
Iris Duroi, Reshma Amin, Nicolas Blankoff, El Medhi Channan, Jean-Benoît le Polain de Waroux
{"title":"Successfully escaping the pleura with the extra-vascular implantable cardioverter defibrillator lead: a case report.","authors":"Iris Duroi, Reshma Amin, Nicolas Blankoff, El Medhi Channan, Jean-Benoît le Polain de Waroux","doi":"10.1093/ehjcr/ytaf322","DOIUrl":"https://doi.org/10.1093/ehjcr/ytaf322","url":null,"abstract":"<p><strong>Background: </strong>Extra-vascular implantable cardioverter defibrillator (EV-ICD) is an emerging technique for ICD implantation with substernal lead placement. The system avoids the vasculature and can deliver anti-tachycardia and pause prevention pacing, with a generator size smaller than the existing subcutaneous device. While this device is now widely available, little is known about potential complications that may follow its implantation in real life context.</p><p><strong>Case summary: </strong>We report the case of a 46-year-old male with a history of non-ischaemic dilated cardiomyopathy, severely reduced left ventricular ejection fraction, and intravenous drug use who underwent EV-ICD implantation for primary prevention of sudden cardiac death. The device implantation was uncomplicated with initial satisfactory device parameters and fluoroscopic position. However, post-implant chest radiograph demonstrated lead dislodgement. Despite meticulous assessment on post-implantation CT, the exact position of the dislocated lead couldn't be determined with certainty. Lead revision was performed at 1 month following implantation, and fluoroscopic assessment at this time revealed lead movement synchronous with respiration, raising suspicion that the lead was intra-pleural. A sheath was repositioned over the dislocated lead and contrast injected. Contrast dispersion confirmed the intra-pleural positioning of the lead and was subsequently used as landmark for the appropriate substernal retunnelling of the lead. At most recent follow-up, lead position on chest radiograph and device parameters were satisfactory.</p><p><strong>Discussion: </strong>Extra-vascular implantable cardioverter defibrillator lead placement and dislodgement within the pleural space can follow apparently uncomplicated implantation. Both cine-imaging and contrast injection can be used for accurate assessment of the lead position relative to the pleura and help successful reimplantation.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf322"},"PeriodicalIF":0.8,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12287926/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144706843","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}
Sanchaya Khetrapal, Aakash Tuli, Yochitha Pulipati, Victor Farah, Indu Poornima
{"title":"PPA2 deficiency-a rare cause of genetic cardiomyopathy: a case report.","authors":"Sanchaya Khetrapal, Aakash Tuli, Yochitha Pulipati, Victor Farah, Indu Poornima","doi":"10.1093/ehjcr/ytaf307","DOIUrl":"10.1093/ehjcr/ytaf307","url":null,"abstract":"<p><strong>Background: </strong>PPA2 deficiency is a rare mitochondrial disorder associated with non-ischemic cardiomyopathy, recurrent rhabdomyolysis, and sudden cardiac death (SCD). This case attempts to highlight the diagnostic and management challenges and contribute to the growing literature on mitochondrial disorders.</p><p><strong>Case summary: </strong>A 21-year-old female with lupus presented with chest pain that developed after hospitalisation for rhabdomyolysis during a viral illness. A cardiac MRI showed patchy late gadolinium enhancement suggestive of myocarditis. She was started on colchicine, but her symptoms persisted. Subsequent imaging revealed persistent myocardial inflammation without troponin elevation. She experienced further episodes of muscle aches and chest pain, prompting additional hospitalisations. Her sibling's diagnosis of non-ischemic cardiomyopathy and need for cardiac transplantation raised suspicion for a genetic aetiology. Genetic testing confirmed compound heterozygosity for pathogenic PPA2 variants (<i>c.380G</i> > <i>T</i> and <i>c.514G</i> > <i>A</i>). She was managed with rhythm monitoring, alcohol avoidance, and genetic counseling.</p><p><strong>Discussion: </strong>PPA2 deficiency should be suspected in patients with unexplained cardiomyopathy, recurrent muscle involvement, and family history of SCD. Cardiac MRI findings of subepicardial and mid-myocardial fibrosis without troponin elevation are characteristic. Early identification allows for lifestyle modifications and consideration of preventive measures like an implantable cardioverter-defibrillator to mitigate the risk of SCD. This case highlights the importance of recognising PPA2 cardiomyopathy in young patients with recurrent cardiac and muscle symptoms. Comprehensive diagnostic evaluation, including genetic testing, is crucial for identifying this rare but life-threatening condition.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf307"},"PeriodicalIF":0.8,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12284632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697876","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}
Panagioula Niarchou, George Michas, Efstathia Prappa, Athanasios Trikas
{"title":"The Rac sign: a transthoracic echocardiography sign that should prompt thorough evaluation of the coronary arteries.","authors":"Panagioula Niarchou, George Michas, Efstathia Prappa, Athanasios Trikas","doi":"10.1093/ehjcr/ytaf300","DOIUrl":"10.1093/ehjcr/ytaf300","url":null,"abstract":"","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf300"},"PeriodicalIF":0.8,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144607861","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":"Sarcoidosis presenting as a large intracardiac mass: a case report.","authors":"Hideki Kawai, Yasuchika Kato, Masayoshi Sarai, Hiroyuki Naruse, Hideo Izawa","doi":"10.1093/ehjcr/ytaf318","DOIUrl":"https://doi.org/10.1093/ehjcr/ytaf318","url":null,"abstract":"<p><strong>Background: </strong>Sarcoidosis is a systemic granulomatous disease that occasionally affects the heart and poses the risks of arrhythmias, heart failure, and sudden cardiac death.</p><p><strong>Case summary: </strong>We report a rare case of cardiac sarcoidosis presenting as a large intracardiac mass in a 76-year-old woman that was incidentally detected during a health check-up. Transthoracic echocardiography revealed a 25× 33 mm mobile mass in the left atrium. Cardiac magnetic resonance and <sup>18</sup>F-fluorodeoxyglucose positron emission tomography/computed tomography demonstrated heterogeneous enhancement and increased metabolic activity, respectively, raising the suspicion of cardiac sarcoidosis. Bronchoscopic biopsy confirmed the presence of epithelioid granulomas, supporting the diagnosis. Surgical resection was performed because of the size of the mass and the potential for mitral valve obstruction. Histopathology confirmed the presence of non-caseating granulomas consistent with sarcoidosis. Postoperatively, corticosteroid therapy with prednisolone (initially 30 mg/day, tapered to 5 mg/day) was initiated to treat the residual lesions identified on imaging. The residual mass showed regression, with resolution of inflammatory activity, through the use of steroid therapy during follow-up.</p><p><strong>Discussion: </strong>This case report highlights the diagnostic and therapeutic challenges associated with cardiac sarcoidosis presenting as a large intracardiac mass. Our findings underscore the importance of a multidisciplinary approach that utilises advanced imaging techniques, histological confirmation, and tailored management strategies that combine surgical intervention and immunosuppressive therapy for diagnosis and treatment.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf318"},"PeriodicalIF":0.8,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12287918/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144706842","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":"Arrhythmia versus Artefact.","authors":"Pujon Purkayastha","doi":"10.1093/ehjcr/ytaf294","DOIUrl":"10.1093/ehjcr/ytaf294","url":null,"abstract":"","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf294"},"PeriodicalIF":0.8,"publicationDate":"2025-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243531/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144607860","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}
Michele Bertelli, Luca Bergamaschi, Matteo Armillotta, Francesco Angeli, Carmine Pizzi
{"title":"A textured clinical mosaic involving autoimmune calcific constrictive pericarditis: a case report.","authors":"Michele Bertelli, Luca Bergamaschi, Matteo Armillotta, Francesco Angeli, Carmine Pizzi","doi":"10.1093/ehjcr/ytaf301","DOIUrl":"10.1093/ehjcr/ytaf301","url":null,"abstract":"<p><strong>Background: </strong>Constrictive pericarditis is a rare condition whereby chronic pericardial inflammation leads to pericardial stiffening and predominantly right-sided heart failure. While idiopathic and infectious forms are most common, autoimmune causes may be involved with often elusive disease manifestations.</p><p><strong>Case summary: </strong>A 34-year-old woman presented with severe right-sided heart failure and atrial fibrillation secondary to rapidly progressive calcific constrictive pericarditis following preterm delivery due to placenta previa. Given the refractoriness to medical therapy and dependence on i.v. diuretic therapy, surgical pericardiectomy was performed without any clinical benefit (prompt heart failure relapse). Further tests revealed a mosaic of multiorgan manifestations (alveolar haemorrhage, pleuritis, factor XI deficiency, altered lymphocyte subpopulations, cutaneous nodules demonstrating Koebner's phenomenon, placental vasculopathy causing miscarriage and preterm delivery), which ultimately led to the diagnosis of systemic lupus erythematosus (SLE). Instatement of immunosuppression (corticosteroid initially, subsequently substituted by mycophenolate) led to the resolution of constriction and other disease manifestations at 18-month follow-up.</p><p><strong>Discussion: </strong>This case of SLE presenting with advanced calcific constrictive pericarditis demonstrates the crucial role played by aetiological diagnosis in constrictive pericarditis. It also exemplifies the often-subtle nature of extra-cardiac manifestations in autoimmune processes, particularly in SLE, which may lead to significant diagnostic uncertainty and delay in the instatement of disease-specific therapy. The benefits of the latter are manifest in this case as the introduction of mycophenolate determined a complete reversal of constrictive physiology despite refractoriness to surgical pericardiectomy.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 7","pages":"ytaf301"},"PeriodicalIF":0.8,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12247502/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144625634","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}