A sinus of Valsalva aneurysm involving a single cusp is a rare condition, and coronary computed tomography angiography with fractional flow reserve-computed tomography help evaluate not only the anatomical aspects of the aneurysm and coronary artery, but also the physiological details of coronary artery disease. A 71-year-old woman presented with exertional chest pain and dyspnoea. Enhanced computed tomography revealed an aneurysmal change in the right sinus of Valsalva, and coronary computed tomography angiography revealed diffuse narrowing of the proximal segment of right coronary artery due to mechanical stretching by the large Valsalva aneurysm. Fractional flow reserve-computed tomography revealed a significantly low fractional flow reserve (0.50 in the distal right coronary artery). A modified Bentall procedure was performed with a 21 mm bioprosthetic valve and a 24 mm Valsalva graft conduit for the aortic root aneurysm; mitral valve annuloplasty was performed for mitral valve regurgitation. Postoperative coronary computed tomography angiography revealed no significant stenosis in the proximal segment of the right coronary artery. Furthermore, fractional flow reserve-computed tomography revealed a normalized fractional flow reserve in the distal right coronary artery. The patient experienced relief from chest pain and was discharged 19 days after the surgery. A right coronary sinus of Valsalva aneurysm, which caused right coronary artery ischaemia, was successfully treated using a modified Bentall procedure. Coronary computed tomography angiography and fractional flow reserve-computed tomography revealed anatomical and functional improvements in the right coronary artery ischaemia postoperatively.
{"title":"Right coronary ischaemia caused by a sinus of Valsalva aneurysm improved by releasing mechanical stretch: A case report","authors":"Sachiko Yamazaki, Kazunari Okawa, Keisuke Shunto, Daisuke Ito, Akiyuki Takahashi","doi":"10.1093/ehjcr/ytae416","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae416","url":null,"abstract":"\u0000 \u0000 \u0000 A sinus of Valsalva aneurysm involving a single cusp is a rare condition, and coronary computed tomography angiography with fractional flow reserve-computed tomography help evaluate not only the anatomical aspects of the aneurysm and coronary artery, but also the physiological details of coronary artery disease.\u0000 \u0000 \u0000 \u0000 A 71-year-old woman presented with exertional chest pain and dyspnoea. Enhanced computed tomography revealed an aneurysmal change in the right sinus of Valsalva, and coronary computed tomography angiography revealed diffuse narrowing of the proximal segment of right coronary artery due to mechanical stretching by the large Valsalva aneurysm. Fractional flow reserve-computed tomography revealed a significantly low fractional flow reserve (0.50 in the distal right coronary artery). A modified Bentall procedure was performed with a 21 mm bioprosthetic valve and a 24 mm Valsalva graft conduit for the aortic root aneurysm; mitral valve annuloplasty was performed for mitral valve regurgitation. Postoperative coronary computed tomography angiography revealed no significant stenosis in the proximal segment of the right coronary artery. Furthermore, fractional flow reserve-computed tomography revealed a normalized fractional flow reserve in the distal right coronary artery. The patient experienced relief from chest pain and was discharged 19 days after the surgery.\u0000 \u0000 \u0000 \u0000 A right coronary sinus of Valsalva aneurysm, which caused right coronary artery ischaemia, was successfully treated using a modified Bentall procedure. Coronary computed tomography angiography and fractional flow reserve-computed tomography revealed anatomical and functional improvements in the right coronary artery ischaemia postoperatively.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141919848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A sinus of Valsalva aneurysm involving a single cusp is a rare condition, and coronary computed tomography angiography with fractional flow reserve-computed tomography help evaluate not only the anatomical aspects of the aneurysm and coronary artery, but also the physiological details of coronary artery disease. A 71-year-old woman presented with exertional chest pain and dyspnoea. Enhanced computed tomography revealed an aneurysmal change in the right sinus of Valsalva, and coronary computed tomography angiography revealed diffuse narrowing of the proximal segment of right coronary artery due to mechanical stretching by the large Valsalva aneurysm. Fractional flow reserve-computed tomography revealed a significantly low fractional flow reserve (0.50 in the distal right coronary artery). A modified Bentall procedure was performed with a 21 mm bioprosthetic valve and a 24 mm Valsalva graft conduit for the aortic root aneurysm; mitral valve annuloplasty was performed for mitral valve regurgitation. Postoperative coronary computed tomography angiography revealed no significant stenosis in the proximal segment of the right coronary artery. Furthermore, fractional flow reserve-computed tomography revealed a normalized fractional flow reserve in the distal right coronary artery. The patient experienced relief from chest pain and was discharged 19 days after the surgery. A right coronary sinus of Valsalva aneurysm, which caused right coronary artery ischaemia, was successfully treated using a modified Bentall procedure. Coronary computed tomography angiography and fractional flow reserve-computed tomography revealed anatomical and functional improvements in the right coronary artery ischaemia postoperatively.
{"title":"Right coronary ischaemia caused by a sinus of Valsalva aneurysm improved by releasing mechanical stretch: A case report","authors":"Sachiko Yamazaki, Kazunari Okawa, Keisuke Shunto, Daisuke Ito, Akiyuki Takahashi","doi":"10.1093/ehjcr/ytae416","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae416","url":null,"abstract":"\u0000 \u0000 \u0000 A sinus of Valsalva aneurysm involving a single cusp is a rare condition, and coronary computed tomography angiography with fractional flow reserve-computed tomography help evaluate not only the anatomical aspects of the aneurysm and coronary artery, but also the physiological details of coronary artery disease.\u0000 \u0000 \u0000 \u0000 A 71-year-old woman presented with exertional chest pain and dyspnoea. Enhanced computed tomography revealed an aneurysmal change in the right sinus of Valsalva, and coronary computed tomography angiography revealed diffuse narrowing of the proximal segment of right coronary artery due to mechanical stretching by the large Valsalva aneurysm. Fractional flow reserve-computed tomography revealed a significantly low fractional flow reserve (0.50 in the distal right coronary artery). A modified Bentall procedure was performed with a 21 mm bioprosthetic valve and a 24 mm Valsalva graft conduit for the aortic root aneurysm; mitral valve annuloplasty was performed for mitral valve regurgitation. Postoperative coronary computed tomography angiography revealed no significant stenosis in the proximal segment of the right coronary artery. Furthermore, fractional flow reserve-computed tomography revealed a normalized fractional flow reserve in the distal right coronary artery. The patient experienced relief from chest pain and was discharged 19 days after the surgery.\u0000 \u0000 \u0000 \u0000 A right coronary sinus of Valsalva aneurysm, which caused right coronary artery ischaemia, was successfully treated using a modified Bentall procedure. Coronary computed tomography angiography and fractional flow reserve-computed tomography revealed anatomical and functional improvements in the right coronary artery ischaemia postoperatively.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141919394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marfan syndrome is a genetic connective tissue disorder that commonly affects the cardiovascular, skeletal and ocular system. The increased risk of developing thoracic aortic aneurysms that can lead to aortic dissection and rupture, is the main source of mortality in these patients. Pregnancy-induced changes can further increase the risk for aortic complications, especially in patients with an aortic root diameter >45 mm. The case of a 26-year-old female with Marfan syndrome who was lost to follow-up for five years and presented to our department being pregnant at 21 weeks is presented. Echocardiography and cardiovascular magnetic resonance (CMR) showed an aortic root diameter of 55 mm and a large aneurysm of an aberrant right subclavian artery. Following multidisciplinary team discussion, valve-sparing aortic root and ascending aortic replacement was performed at 22 weeks of gestation without any complications. During the remaining pregnancy the patient had frequent clinical and CMR follow-up investigations showing a mild increased size of the subclavian aneurysm. Uncomplicated caesarian delivery was performed at 35 weeks of gestation and the subclavian artery aneurysm was successfully treated by interventional embolisation. Although cardiovascular surgery in our patient during pregnancy was uncomplicated, the case illustrates that pre-pregnancy counseling in Marfan patients is recommended to reduce the risk for mother and child.
{"title":"Management of a Pregnant Woman with Marfan Syndrome and Aortic Root and Aberrant Right Subclavian Artery Aneurysm: A Case Report","authors":"Inga Voges, U. Hoffmann, Tim Attman, A. Uebing","doi":"10.1093/ehjcr/ytae411","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae411","url":null,"abstract":"\u0000 \u0000 \u0000 Marfan syndrome is a genetic connective tissue disorder that commonly affects the cardiovascular, skeletal and ocular system. The increased risk of developing thoracic aortic aneurysms that can lead to aortic dissection and rupture, is the main source of mortality in these patients. Pregnancy-induced changes can further increase the risk for aortic complications, especially in patients with an aortic root diameter >45 mm.\u0000 \u0000 \u0000 \u0000 The case of a 26-year-old female with Marfan syndrome who was lost to follow-up for five years and presented to our department being pregnant at 21 weeks is presented.\u0000 Echocardiography and cardiovascular magnetic resonance (CMR) showed an aortic root diameter of 55 mm and a large aneurysm of an aberrant right subclavian artery. Following multidisciplinary team discussion, valve-sparing aortic root and ascending aortic replacement was performed at 22 weeks of gestation without any complications. During the remaining pregnancy the patient had frequent clinical and CMR follow-up investigations showing a mild increased size of the subclavian aneurysm. Uncomplicated caesarian delivery was performed at 35 weeks of gestation and the subclavian artery aneurysm was successfully treated by interventional embolisation.\u0000 \u0000 \u0000 \u0000 Although cardiovascular surgery in our patient during pregnancy was uncomplicated, the case illustrates that pre-pregnancy counseling in Marfan patients is recommended to reduce the risk for mother and child.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141925215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Rohla, Legate Philip, Janina Wolf, F. Jaffer, L. Räber
ST-elevation myocardial infarction (STEMI) is a cardiac emergency that requires prompt diagnosis and treatment. We describe a challenging and complex case of managing acute STEMI in a patient with severe anaemia, deranged clotting profile and an infective prodrome. A 54-year-old Caucasian gentleman was referred by his general practitioner (GP) as an emergency after presenting with acute onset of chest pain. His electrocardiogram revealed anterior ST elevation. His past medical history includes a mechanical aortic valve, requiring anticoagulation, and a recent gastrointestinal bleed secondary to type C gastritis. His initial presentation was further complicated by severe anaemia, deranged clotting profile and elevated infective markers. He required a prompt transfer to the catheterisation laboratory to assess and stabilise the situation. We discuss the emerging challenges during treatment, particularly as the diagnosis of septic embolism from infective prosthetic valve endocarditis was unfolding, requiring urgent cardiac surgery. Acute coronary vessel closure leading to STEMI from septic embolism secondary to prosthetic aortic valve endocarditis is very rare. It is essential to consider the whole picture of the presentation for timely diagnosis and tailored treatment.
{"title":"ST-elevation myocardial infarction from septic embolism secondary to prosthetic aortic valve endocarditis – a case report","authors":"M. Rohla, Legate Philip, Janina Wolf, F. Jaffer, L. Räber","doi":"10.1093/ehjcr/ytae420","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae420","url":null,"abstract":"\u0000 \u0000 \u0000 ST-elevation myocardial infarction (STEMI) is a cardiac emergency that requires prompt diagnosis and treatment. We describe a challenging and complex case of managing acute STEMI in a patient with severe anaemia, deranged clotting profile and an infective prodrome.\u0000 \u0000 \u0000 \u0000 A 54-year-old Caucasian gentleman was referred by his general practitioner (GP) as an emergency after presenting with acute onset of chest pain. His electrocardiogram revealed anterior ST elevation. His past medical history includes a mechanical aortic valve, requiring anticoagulation, and a recent gastrointestinal bleed secondary to type C gastritis. His initial presentation was further complicated by severe anaemia, deranged clotting profile and elevated infective markers. He required a prompt transfer to the catheterisation laboratory to assess and stabilise the situation. We discuss the emerging challenges during treatment, particularly as the diagnosis of septic embolism from infective prosthetic valve endocarditis was unfolding, requiring urgent cardiac surgery.\u0000 \u0000 \u0000 \u0000 Acute coronary vessel closure leading to STEMI from septic embolism secondary to prosthetic aortic valve endocarditis is very rare. It is essential to consider the whole picture of the presentation for timely diagnosis and tailored treatment.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141924653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Z. Ammouri, S. Belkouchia, I. Rezzouk, S. moussaoui, R. Habbal
Eosinophilic myocarditis, a rare and potentially life-threatening condition, can resemble acute coronary syndrome (ACS) and presents diagnostic difficulties. We describe the case of a 32-year-old man initially admitted with ACS-like symptoms, but ultimately diagnosed with eosinophilic myocarditis. Hypereosinophilic syndrome encompasses a diverse group of blood disorders characterized by persistent, unexplained hypereosinophilia leading to tissue damage. Cardiac involvement occurs in around 20% of cases of hypereosinophilic syndrome and marks a critical phase. Our report highlights the importance of considering eosinophilic myocarditis and SHI when evaluating patients with chest pain and hypereosinophilia. It emphasizes the subtleties of diagnosis and the imperative of early identification and appropriate treatment to improve prognosis in cases of eosinophilic myocarditis. This case highlights the diverse clinical manifestations of myocarditis and the essential need for a comprehensive diagnostic approach in the presence of chest pain and hypereosinophilia.
{"title":"Eosinophilic Myocarditis: A Diagnostic Challenge and Treatment Dilemma - A Case Report","authors":"Z. Ammouri, S. Belkouchia, I. Rezzouk, S. moussaoui, R. Habbal","doi":"10.1093/ehjcr/ytae418","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae418","url":null,"abstract":"\u0000 \u0000 \u0000 Eosinophilic myocarditis, a rare and potentially life-threatening condition, can resemble acute coronary syndrome (ACS) and presents diagnostic difficulties.\u0000 \u0000 \u0000 \u0000 We describe the case of a 32-year-old man initially admitted with ACS-like symptoms, but ultimately diagnosed with eosinophilic myocarditis. Hypereosinophilic syndrome encompasses a diverse group of blood disorders characterized by persistent, unexplained hypereosinophilia leading to tissue damage. Cardiac involvement occurs in around 20% of cases of hypereosinophilic syndrome and marks a critical phase.\u0000 \u0000 \u0000 \u0000 Our report highlights the importance of considering eosinophilic myocarditis and SHI when evaluating patients with chest pain and hypereosinophilia. It emphasizes the subtleties of diagnosis and the imperative of early identification and appropriate treatment to improve prognosis in cases of eosinophilic myocarditis. This case highlights the diverse clinical manifestations of myocarditis and the essential need for a comprehensive diagnostic approach in the presence of chest pain and hypereosinophilia.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141928986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Guizhou Ma, Linjie Zhou, Dianyu Cai, Ying Wang, Zhixiong Cai
Transesophageal echocardiography is rarely reported as a possible cause of aortic dissection during the transcatheter edge-to-edge repair procedure. Herein, we present a case of type B aortic dissection following the transcatheter mitral valve edge-to-edge repair procedure, most likely related to the transesophageal echocardiography probe. A 68-year-old Chinese man complained of exertional dyspnea lasting over 2 years and had been diagnosed with severe mitral regurgitation. He was admitted to our hospital for the treatment of severe mitral regurgitation with transcatheter edge-to-edge repair. One MitraClip XTR(Abbott Vascular) was successfully implanted under the guidance of active transesophageal echocardiography and the mitral regurgitation became trace. However, the patient complained of persistent back pain after the treatment and computed tomography angiography revealed a type B aortic dissection in the descending aorta. After two weeks of unsuccessful conservative treatment, he successfully underwent endovascular stenting and was discharged from the hospital. The patient recovered well and remained event-free during the six-month follow-up. Herein, we presented a rare complication following transcatheter mitral valve edge-to-edge repair that was most likely related to the transesophageal echocardiography probe--type B aortic dissection. We postulated that repetitive flexion of the transesophageal echocardiography probe led to compression-induced injury to the descending aorta wall at the mid-esophageal level, which was the most probable etiology of type B aortic dissection. Although this complication is rare, it is potentially fatal and therefore needs attention.
在经导管二尖瓣边缘对边缘修复术中，经食管超声心动图可能是导致主动脉夹层的原因，但这一报道很少见。在此，我们介绍了一例经导管二尖瓣边缘对边缘修复术后发生 B 型主动脉夹层的病例，该病例很可能与经食道超声心动图探头有关。 一名 68 岁的中国男性主诉劳力性呼吸困难已持续 2 年多，被诊断为严重二尖瓣反流。他住进了我院，接受经导管边缘对边缘修补术治疗重度二尖瓣反流。在主动经食道超声心动图的引导下，成功植入了一个MitraClip XTR（雅培血管公司），二尖瓣反流变得微小。然而，治疗后患者主诉背部持续疼痛，计算机断层扫描血管造影显示降主动脉存在 B 型主动脉夹层。经过两周的保守治疗未果后，他成功接受了血管内支架植入术，并康复出院。患者恢复良好，在六个月的随访期间一直未发生任何事件。 在此，我们介绍了经导管二尖瓣边缘对边缘修复术后的一种罕见并发症，该并发症很可能与经食道超声心动图探头有关--B 型主动脉夹层。我们推测，经食道超声心动图探头的反复弯曲导致食道中段的降主动脉壁受到压迫性损伤，这是 B 型主动脉夹层最可能的病因。虽然这种并发症很少见，但却可能致命，因此需要引起重视。
{"title":"Acute Type B Aortic Dissection Following Transcatheter Mitral Valve Edge-to-edge Repair: A Case Report","authors":"Guizhou Ma, Linjie Zhou, Dianyu Cai, Ying Wang, Zhixiong Cai","doi":"10.1093/ehjcr/ytae424","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae424","url":null,"abstract":"\u0000 \u0000 \u0000 Transesophageal echocardiography is rarely reported as a possible cause of aortic dissection during the transcatheter edge-to-edge repair procedure. Herein, we present a case of type B aortic dissection following the transcatheter mitral valve edge-to-edge repair procedure, most likely related to the transesophageal echocardiography probe.\u0000 \u0000 \u0000 \u0000 A 68-year-old Chinese man complained of exertional dyspnea lasting over 2 years and had been diagnosed with severe mitral regurgitation. He was admitted to our hospital for the treatment of severe mitral regurgitation with transcatheter edge-to-edge repair. One MitraClip XTR(Abbott Vascular) was successfully implanted under the guidance of active transesophageal echocardiography and the mitral regurgitation became trace. However, the patient complained of persistent back pain after the treatment and computed tomography angiography revealed a type B aortic dissection in the descending aorta. After two weeks of unsuccessful conservative treatment, he successfully underwent endovascular stenting and was discharged from the hospital. The patient recovered well and remained event-free during the six-month follow-up.\u0000 \u0000 \u0000 \u0000 Herein, we presented a rare complication following transcatheter mitral valve edge-to-edge repair that was most likely related to the transesophageal echocardiography probe--type B aortic dissection. We postulated that repetitive flexion of the transesophageal echocardiography probe led to compression-induced injury to the descending aorta wall at the mid-esophageal level, which was the most probable etiology of type B aortic dissection. Although this complication is rare, it is potentially fatal and therefore needs attention.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141927856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
K. Finke, Thorsten Gietzen, Daniel Steven, Stephan Baldus, Hendrik ten Freyhaus, D. Maintz, L. Pennig, Carsten Gietzen
Fibromas are rare primary benign cardiac tumors which can become symptomatic due to expansive growth, ventricular rhythm disturbances, and sudden cardiac death. Distinguishing fibromas from other (malign) cardiac masses is essential for accurate diagnosis and treatment. While there is some experience in management of cardiac fibromas in children, management of adult patients is unknown. We present three cases of cardiac fibroma in adult patients diagnosed by echocardiography, cardiovascular magnetic resonance (CMR), and computed tomography (CT): (1) A 55-year-old male with a left ventricular fibroma leading to reduced left ventricular ejection fraction and mitral regurgitation. He had family history of sudden cardiac death, showed premature ventricular contractions (PVC) and was treated with a primary preventive subcutaneous implantable cardiac defibrillator (S-ICD). (2) A 39-year-old male with right ventricular fibroma as an incidental finding. He complained of episodes of PVC. Due to a low PVC burden, decision was made against ablation and the patient was planned for follow-up. (3) An 18-year-old female with left ventricular apex fibroma detected by CMR shortly after birth and confirmed by surgical biopsy. Being asymptomatic, conservative management was pursued and follow-up by CMR planned. Cardiac fibromas can show various clinical presentations and hence being detected late in life. Given potential complications of surgical biopsy, diagnosis of cardiac fibromas is primarily based on echocardiography, CT, and CMR. Rhythm-disturbances as premature ventricular contractions are common. Due to association with ventricular arrhythmias and sudden cardiac death, preventive ICD placement might be appropriate on an individual basis.
{"title":"Cardiac fibromas in adult patients –a case series focusing on rhythmology and radiographic features","authors":"K. Finke, Thorsten Gietzen, Daniel Steven, Stephan Baldus, Hendrik ten Freyhaus, D. Maintz, L. Pennig, Carsten Gietzen","doi":"10.1093/ehjcr/ytae410","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae410","url":null,"abstract":"\u0000 \u0000 \u0000 Fibromas are rare primary benign cardiac tumors which can become symptomatic due to expansive growth, ventricular rhythm disturbances, and sudden cardiac death. Distinguishing fibromas from other (malign) cardiac masses is essential for accurate diagnosis and treatment. While there is some experience in management of cardiac fibromas in children, management of adult patients is unknown.\u0000 \u0000 \u0000 \u0000 We present three cases of cardiac fibroma in adult patients diagnosed by echocardiography, cardiovascular magnetic resonance (CMR), and computed tomography (CT): (1) A 55-year-old male with a left ventricular fibroma leading to reduced left ventricular ejection fraction and mitral regurgitation. He had family history of sudden cardiac death, showed premature ventricular contractions (PVC) and was treated with a primary preventive subcutaneous implantable cardiac defibrillator (S-ICD). (2) A 39-year-old male with right ventricular fibroma as an incidental finding. He complained of episodes of PVC. Due to a low PVC burden, decision was made against ablation and the patient was planned for follow-up. (3) An 18-year-old female with left ventricular apex fibroma detected by CMR shortly after birth and confirmed by surgical biopsy. Being asymptomatic, conservative management was pursued and follow-up by CMR planned.\u0000 \u0000 \u0000 \u0000 Cardiac fibromas can show various clinical presentations and hence being detected late in life. Given potential complications of surgical biopsy, diagnosis of cardiac fibromas is primarily based on echocardiography, CT, and CMR. Rhythm-disturbances as premature ventricular contractions are common. Due to association with ventricular arrhythmias and sudden cardiac death, preventive ICD placement might be appropriate on an individual basis.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141928113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
William O’Connor, Asma Arshia, D. Prabakar, Vaishnavi Sabesan, Jeffrey F. Spindel
Lamin A/C mutations cause myocardial fibrosis manifesting as arrhythmogenic, noncompaction, or dilated cardiomyopathies. Fibrofatty replacement largely involves the conduction system and conduction disease commonly occurs prior to contractile dysfunction. Two young, Caucasian males, aged 34 and 25, were referred to our center for treatment of advanced heart failure. Both patients had family history of heart failure and sudden cardiac death in first degree relatives, were diagnosed with lamin A/C mutations, but had not been screened prior to disease onset. Though initial phenotypes were dilated cardiomyopathy and left ventricular-noncompaction cardiomyopathy, both patient’s disease progressed rapidly to include ventricular arrhythmias, severe global left ventricular hypokinesis, and dependence on outpatient milrinone to complete activities of daily living. Both patients received heart transplantation within 2 years of initial disease onset. Surgical pathology of the explanted hearts revealed characteristic findings of fibro-fatty degeneration of conduction system, and using light microscopy, were found to have nuclear membrane thinning, bubbling, and convolution throughout all areas sampled. Lamin A/C-related cardiomyopathy is associated with sudden cardiac death early in the disease course, warranting early consideration of implantable cardioverter defibrillator implantation, and rapid progression to end stage cardiomyopathy refractory to standard medical therapies, necessitating early referral to an advanced heart failure center. We report a newly observed and recorded finding of morphologic nuclear alterations in late-stage disease using high-power light microscopy. These alterations underscore the pathophysiology of lamin A/C-related cardiomyopathy and provide a basis for future research into disease-specific therapies.
{"title":"Nuclear Envelope Lamin-Related Dilated Cardiomyopathy: Case Series Including Histopathology","authors":"William O’Connor, Asma Arshia, D. Prabakar, Vaishnavi Sabesan, Jeffrey F. Spindel","doi":"10.1093/ehjcr/ytae412","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae412","url":null,"abstract":"\u0000 \u0000 \u0000 Lamin A/C mutations cause myocardial fibrosis manifesting as arrhythmogenic, noncompaction, or dilated cardiomyopathies. Fibrofatty replacement largely involves the conduction system and conduction disease commonly occurs prior to contractile dysfunction.\u0000 \u0000 \u0000 \u0000 Two young, Caucasian males, aged 34 and 25, were referred to our center for treatment of advanced heart failure. Both patients had family history of heart failure and sudden cardiac death in first degree relatives, were diagnosed with lamin A/C mutations, but had not been screened prior to disease onset. Though initial phenotypes were dilated cardiomyopathy and left ventricular-noncompaction cardiomyopathy, both patient’s disease progressed rapidly to include ventricular arrhythmias, severe global left ventricular hypokinesis, and dependence on outpatient milrinone to complete activities of daily living. Both patients received heart transplantation within 2 years of initial disease onset. Surgical pathology of the explanted hearts revealed characteristic findings of fibro-fatty degeneration of conduction system, and using light microscopy, were found to have nuclear membrane thinning, bubbling, and convolution throughout all areas sampled.\u0000 \u0000 \u0000 \u0000 Lamin A/C-related cardiomyopathy is associated with sudden cardiac death early in the disease course, warranting early consideration of implantable cardioverter defibrillator implantation, and rapid progression to end stage cardiomyopathy refractory to standard medical therapies, necessitating early referral to an advanced heart failure center. We report a newly observed and recorded finding of morphologic nuclear alterations in late-stage disease using high-power light microscopy. These alterations underscore the pathophysiology of lamin A/C-related cardiomyopathy and provide a basis for future research into disease-specific therapies.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141927004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Christian-Hendrick Heeger, M. Feher, Julia Vogler, R. Tilz
{"title":"High-density multipolar mapping facilitating ablation of atypical atrial flutter – The power of “more mapping less ablation”","authors":"Christian-Hendrick Heeger, M. Feher, Julia Vogler, R. Tilz","doi":"10.1093/ehjcr/ytae369","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae369","url":null,"abstract":"","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141927258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
To date, vitamin K anticoagulants are the only recommended long-term therapy for mechanical heart valves. Bleeding episodes, thromboembolic events and international normalised ratio monitoring are difficult and prevalent complications for these patients. This report reflects the late mechanical aortic valve dysfunction after long-term low molecular weight heparin therapy. A 66-year-old male patient underwent mechanical aortic valve replacement in 2007. He was administered therapeutic doses of enoxaparin for nearly 12 years due to warfarin-related bleeding complications and labile international normalised ratios. However, he experienced multiple cardiovascular and cerebrovascular thromboembolic events, including an anterolateral ST-elevation myocardial infarction with left anterior descending artery thrombus, treated with thrombus aspiration and stenting. The patient was eventually admitted with symptoms and signs of acute heart failure, and echocardiography, fluoroscopy and a cardiac computed tomography detected mechanical aortic valve prosthesis dysfunction, with an immobile leaflet and pannus. The patient demonstrated no improvement despite switching to unfractionated heparin, and he ultimately underwent redo aortic bioprosthetic valve surgery with a favourable outcome. Low molecular weight heparin is prescribed for patients with aortic mechanical valves who are intolerant to vitamin K antagonists or as bridging in certain situations. Anti-Xa factor monitoring should be considered for long-term prescriptions.
迄今为止，维生素 K 抗凝剂是治疗机械心脏瓣膜的唯一推荐长期疗法。出血发作、血栓栓塞事件和国际正常比值监测是这些患者难以解决且普遍存在的并发症。本报告反映了长期低分子量肝素治疗后机械主动脉瓣晚期功能障碍的情况。 一名 66 岁的男性患者于 2007 年接受了机械主动脉瓣置换术。由于华法林相关出血并发症和国际正常化比率不稳定，他接受了近 12 年的治疗剂量依诺肝素治疗。然而，他经历了多次心脑血管血栓栓塞事件，包括左前降支动脉血栓引起的前外侧ST段抬高型心肌梗死，并接受了血栓抽吸和支架治疗。患者最终因急性心力衰竭的症状和体征入院，超声心动图、透视检查和心脏计算机断层扫描发现其主动脉瓣机械假体功能障碍，瓣叶无法移动，并伴有囊肿。尽管患者改用了非丝裂霉素，但病情仍无好转，最终他接受了主动脉生物人工瓣膜重做手术，并取得了良好的疗效。 低分子量肝素适用于对维生素 K 拮抗剂不耐受的主动脉机械瓣患者，或在某些情况下作为桥接用药。长期处方时应考虑对抗 Xa 因子进行监测。
{"title":"Late dysfunction of a mechanical aortic valve after long-term low molecular weight heparin therapy: A case report","authors":"A. Mon, S. Shanmuganathan, A. Uddin","doi":"10.1093/ehjcr/ytae361","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae361","url":null,"abstract":"\u0000 \u0000 \u0000 To date, vitamin K anticoagulants are the only recommended long-term therapy for mechanical heart valves. Bleeding episodes, thromboembolic events and international normalised ratio monitoring are difficult and prevalent complications for these patients. This report reflects the late mechanical aortic valve dysfunction after long-term low molecular weight heparin therapy.\u0000 \u0000 \u0000 \u0000 A 66-year-old male patient underwent mechanical aortic valve replacement in 2007. He was administered therapeutic doses of enoxaparin for nearly 12 years due to warfarin-related bleeding complications and labile international normalised ratios. However, he experienced multiple cardiovascular and cerebrovascular thromboembolic events, including an anterolateral ST-elevation myocardial infarction with left anterior descending artery thrombus, treated with thrombus aspiration and stenting.\u0000 The patient was eventually admitted with symptoms and signs of acute heart failure, and echocardiography, fluoroscopy and a cardiac computed tomography detected mechanical aortic valve prosthesis dysfunction, with an immobile leaflet and pannus. The patient demonstrated no improvement despite switching to unfractionated heparin, and he ultimately underwent redo aortic bioprosthetic valve surgery with a favourable outcome.\u0000 \u0000 \u0000 \u0000 Low molecular weight heparin is prescribed for patients with aortic mechanical valves who are intolerant to vitamin K antagonists or as bridging in certain situations. Anti-Xa factor monitoring should be considered for long-term prescriptions.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141797355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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