Sotirios Dardas, Petros Dardas, N. Mezilis, Dimitrios Tsikaderis, Theodoros Kofidis
Mitral annular calcification (MAC) is common in the elderly. Extensive calcification is historically challenging for the cardiac surgeons, with traditional surgical approaches carrying significant risks. Less invasive approaches have recently been explored in an attempt to reduce this risk. We report the case of a 75-year-old woman who presented with recurrent pulmonary oedema, due to severe MAC and mitral regurgitation. Her past medical history included bioprosthetic aortic valve replacement five years ago. Given the extensive MAC and the patient’s frailty, a minimally invasive hybrid approach with direct implantation of a transcatheter balloon expandable Sapien 3 valve was selected to manage the patient. Although the post-surgical result was initially excellent with elimination of the mitral regurgitation, the patient’s postoperative course was marked by two serious complications, namely acute severe aortic regurgitation due to rupture of the bioprosthetic valve’s right cusp, as well as severe paravalvular leak of the Sapien valve, due to posterior migration towards the left atrium. These were managed successfully with emergency valve-in-valve implantation using the ‘’Double Chimney’’ technique for the bioprosthetic aortic valve, as well as transeptal valve-in-valve implantation of a second Sapien valve in the mitral valve, which sealed the gap between the first Sapien and the calcified mitral annulus. This case illustrates a less invasive approach for the management of severe MAC. Complications can still occur in this high-risk group of patients and therefore, such cases should be managed with close collaboration between cardiac surgeons and cardiologists, in centres with high expertise.
{"title":"Pushing the hybrid approach to the edges, three stories in one: a case report","authors":"Sotirios Dardas, Petros Dardas, N. Mezilis, Dimitrios Tsikaderis, Theodoros Kofidis","doi":"10.1093/ehjcr/ytae333","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae333","url":null,"abstract":"\u0000 \u0000 \u0000 Mitral annular calcification (MAC) is common in the elderly. Extensive calcification is historically challenging for the cardiac surgeons, with traditional surgical approaches carrying significant risks. Less invasive approaches have recently been explored in an attempt to reduce this risk.\u0000 \u0000 \u0000 \u0000 We report the case of a 75-year-old woman who presented with recurrent pulmonary oedema, due to severe MAC and mitral regurgitation. Her past medical history included bioprosthetic aortic valve replacement five years ago. Given the extensive MAC and the patient’s frailty, a minimally invasive hybrid approach with direct implantation of a transcatheter balloon expandable Sapien 3 valve was selected to manage the patient. Although the post-surgical result was initially excellent with elimination of the mitral regurgitation, the patient’s postoperative course was marked by two serious complications, namely acute severe aortic regurgitation due to rupture of the bioprosthetic valve’s right cusp, as well as severe paravalvular leak of the Sapien valve, due to posterior migration towards the left atrium. These were managed successfully with emergency valve-in-valve implantation using the ‘’Double Chimney’’ technique for the bioprosthetic aortic valve, as well as transeptal valve-in-valve implantation of a second Sapien valve in the mitral valve, which sealed the gap between the first Sapien and the calcified mitral annulus.\u0000 \u0000 \u0000 \u0000 This case illustrates a less invasive approach for the management of severe MAC. Complications can still occur in this high-risk group of patients and therefore, such cases should be managed with close collaboration between cardiac surgeons and cardiologists, in centres with high expertise.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141658251","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}
Kimberly R Ding, Angelo L de la Rosa, Duc Do, Sonia U. Shah
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a primary arrhythmia disorder characterized by syncope or sudden cardiac death and typically caused by a gain-of-function of the Ryanodine Receptor Type 2 (RyR2) mutation. Calmodulin is a calcium-binding protein responsible for many intracellular signaling pathways and disruptions in function or regulation may lead to potentially fatal arrhythmias. We present a case of a young patient with CPVT found to have an unusual, potentially causative, Calmodulin 2- a protein coding gene (CALM2) mutation. A 21-year-old female with autism was brought to the ED following cardiac arrest. Bidirectional ventricular tachycardia (VT) was captured on electrocardiogram (ECG). Propranolol was initiated and patient had no further episodes of ventricular arrhythmia. A subcutaneous implantable cardioverter defibrillator (ICD) was implanted and further genetics testing was done. Rapid Whole Genome Sequencing PGnome®- RAPID) resulted heterozygous variant of uncertain significance in CALM2 gene NM_001743.5 for variant c.136G>A. To the authors’ knowledge, this is the third known record of such mutation in accordance with the International Calmodulin Registry (n=74). Identification of CALM mutations can help advance the understanding of genetic underpinnings of arrhythmias and underscore necessity of genetic screening and personalized treatment strategies. Subcutaneous ICDs offer a promising therapeutic option while minimizing risks associated with traditional transvenous ICDs.
{"title":"A Rare Case Report of Catecholaminergic Polymorphic Ventricular Tachycardia with an Uncommon CALM2 Mutation","authors":"Kimberly R Ding, Angelo L de la Rosa, Duc Do, Sonia U. Shah","doi":"10.1093/ehjcr/ytae340","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae340","url":null,"abstract":"\u0000 \u0000 \u0000 Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a primary arrhythmia disorder characterized by syncope or sudden cardiac death and typically caused by a gain-of-function of the Ryanodine Receptor Type 2 (RyR2) mutation. Calmodulin is a calcium-binding protein responsible for many intracellular signaling pathways and disruptions in function or regulation may lead to potentially fatal arrhythmias. We present a case of a young patient with CPVT found to have an unusual, potentially causative, Calmodulin 2- a protein coding gene (CALM2) mutation.\u0000 \u0000 \u0000 \u0000 A 21-year-old female with autism was brought to the ED following cardiac arrest. Bidirectional ventricular tachycardia (VT) was captured on electrocardiogram (ECG). Propranolol was initiated and patient had no further episodes of ventricular arrhythmia. A subcutaneous implantable cardioverter defibrillator (ICD) was implanted and further genetics testing was done. Rapid Whole Genome Sequencing PGnome®- RAPID) resulted heterozygous variant of uncertain significance in CALM2 gene NM_001743.5 for variant c.136G>A.\u0000 \u0000 \u0000 \u0000 To the authors’ knowledge, this is the third known record of such mutation in accordance with the International Calmodulin Registry (n=74). Identification of CALM mutations can help advance the understanding of genetic underpinnings of arrhythmias and underscore necessity of genetic screening and personalized treatment strategies. Subcutaneous ICDs offer a promising therapeutic option while minimizing risks associated with traditional transvenous ICDs.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141658367","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}
Sotirios Dardas, Ibrahim Antoun, Falik Sher, Navid Munir, Kosmas Kontoprias
Behçet's Disease (BD) is a rare and complex vasculitis disorder renowned for its diverse clinical presentations. Cardiovascular involvement is reported to be present in 7-46% of the patients, with coronary arteries being involved in only 0.5%. The management of cardiovascular complications can be challenging due to the rarity of such cases and the absence of standardized guidelines regarding diagnosis and treatment. We report the case of a 27-year-old patient with BD with known aortitis and pulmonary arteritis, who presented with recurrent acute coronary syndromes related to critical left main coronary artery stenosis. She was initially managed with percutaneous coronary interventions twice. Following recurrent stent failure, she eventually underwent urgent coronary artery bypass surgery, together with aortic valve replacement and aortic root repair. She made an uneventful recovery and remains well six months following her operation. This case illustrates the significant challenges that can be encountered when managing coronary complications in patients with BD. Both percutaneous and surgical options have been reported in the literature with variable outcomes. Multidisciplinary team involvement is of utmost importance in order to offer a balanced therapeutic strategy to these patients. Further research is required to shed light to the unknowns surrounding this rare cohort.
{"title":"“Recurrent Left Main Stem Stenosis in a Young Female with Behçet’s Aortitis: A Case Report”","authors":"Sotirios Dardas, Ibrahim Antoun, Falik Sher, Navid Munir, Kosmas Kontoprias","doi":"10.1093/ehjcr/ytae331","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae331","url":null,"abstract":"\u0000 \u0000 \u0000 Behçet's Disease (BD) is a rare and complex vasculitis disorder renowned for its diverse clinical presentations. Cardiovascular involvement is reported to be present in 7-46% of the patients, with coronary arteries being involved in only 0.5%. The management of cardiovascular complications can be challenging due to the rarity of such cases and the absence of standardized guidelines regarding diagnosis and treatment.\u0000 \u0000 \u0000 \u0000 We report the case of a 27-year-old patient with BD with known aortitis and pulmonary arteritis, who presented with recurrent acute coronary syndromes related to critical left main coronary artery stenosis. She was initially managed with percutaneous coronary interventions twice. Following recurrent stent failure, she eventually underwent urgent coronary artery bypass surgery, together with aortic valve replacement and aortic root repair. She made an uneventful recovery and remains well six months following her operation.\u0000 \u0000 \u0000 \u0000 This case illustrates the significant challenges that can be encountered when managing coronary complications in patients with BD. Both percutaneous and surgical options have been reported in the literature with variable outcomes. Multidisciplinary team involvement is of utmost importance in order to offer a balanced therapeutic strategy to these patients. Further research is required to shed light to the unknowns surrounding this rare cohort.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141656695","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}
{"title":"A case report of Löffler's endocarditis with right ventricular outflow obstruction in idiopathic hypereosinophilic syndrome: timely surgical intervention is crucial","authors":"Decai Zeng, Ziyu Peng, Xuechun Guan, Ji Wu","doi":"10.1093/ehjcr/ytae335","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae335","url":null,"abstract":"","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141659248","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}
Rik De Wolf, R. L. van der Palen, A. D. J. ten Harkel, M. Hazekamp, Nico A Blom
Left ventricular apical pacing (LVAP) is considered to preserve left ventricular (LV) systolic function in both patients with and without congenital heart disease. However, sporadic LVAP-associated cardiac dysfunction in children with complex structural heart disease was recently reported. We present the case of a 2.5-year-old child with complex congenital heart disease and LVAP-induced cardiomyopathy. Corrective surgery for double outlet right ventricle, subpulmonary ventricular septal defect and transposition of the great arteries was done at the age of 1.5 months. Late complete atrioventricular block occurred, necessitating VVI pacemaker insertion with LV apical epicardial leads. He presented with heart failure and dilated cardiomyopathy 1.5 years after pacemaker insertion and required persistent circulatory support with intravenous inotropes. Speckle tracking echocardiography (STE) identified an important LV apical to basal dyssynchrony. After excluding any coronary artery involvement, cardiac resynchronization therapy was performed. STE guided lead placement resulted in improved LV contraction synchrony. Cardiac function recovered progressively in combination with oral heart failure medication and is almost normal at 10-month follow-up. Right ventricular pacing is a well-known cause of pacing-induced cardiomyopathy. The LV apex and LV free wall are thought to be most optimal locations for ventricular pacing in children. However, LVAP can also be the cause of a pacing-induced cardiomyopathy and decreased systolic LV function in children with complex congenital heart disease due to lack of LV contraction synchrony. Cardiac resynchronization therapy can reverse this LV dysfunction and remodeling.
{"title":"Left ventricular apical pacing-induced heart failure in a child after congenital heart surgery: a case report","authors":"Rik De Wolf, R. L. van der Palen, A. D. J. ten Harkel, M. Hazekamp, Nico A Blom","doi":"10.1093/ehjcr/ytae339","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae339","url":null,"abstract":"\u0000 \u0000 \u0000 Left ventricular apical pacing (LVAP) is considered to preserve left ventricular (LV) systolic function in both patients with and without congenital heart disease. However, sporadic LVAP-associated cardiac dysfunction in children with complex structural heart disease was recently reported. We present the case of a 2.5-year-old child with complex congenital heart disease and LVAP-induced cardiomyopathy.\u0000 \u0000 \u0000 \u0000 Corrective surgery for double outlet right ventricle, subpulmonary ventricular septal defect and transposition of the great arteries was done at the age of 1.5 months. Late complete atrioventricular block occurred, necessitating VVI pacemaker insertion with LV apical epicardial leads. He presented with heart failure and dilated cardiomyopathy 1.5 years after pacemaker insertion and required persistent circulatory support with intravenous inotropes. Speckle tracking echocardiography (STE) identified an important LV apical to basal dyssynchrony. After excluding any coronary artery involvement, cardiac resynchronization therapy was performed. STE guided lead placement resulted in improved LV contraction synchrony. Cardiac function recovered progressively in combination with oral heart failure medication and is almost normal at 10-month follow-up.\u0000 \u0000 \u0000 \u0000 Right ventricular pacing is a well-known cause of pacing-induced cardiomyopathy. The LV apex and LV free wall are thought to be most optimal locations for ventricular pacing in children. However, LVAP can also be the cause of a pacing-induced cardiomyopathy and decreased systolic LV function in children with complex congenital heart disease due to lack of LV contraction synchrony. Cardiac resynchronization therapy can reverse this LV dysfunction and remodeling.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141662633","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 an inherited disorder that manifests with various cardiovascular conditions. This case report discusses a patient with Marfan syndrome presenting with concurrent dissecting aortic aneurysm and acute mitral valve regurgitation (MR), exploring treatment strategies for this unique case. A 57-year-old man diagnosed with Marfan syndrome presented with progressive dyspnea and awareness of orthopnea. Acute heart failure (HF) due to acute MR associated with chordae rupture was diagnosed. However, contrast-enhanced CT revealed the coexistence of a massive dissecting aortic aneurysm, indicating surgical intervention. The dissecting aortic aneurysm extended over a large area. Given the high risk of simultaneous surgery with the mitral valve, a staged approach was adopted. Mitral valve transcatheter edge-to-edge repair (MV-TEER) was performed as the initial step to reduce the perioperative HF risk, followed by a planned two-stage surgery for the dissecting aortic aneurysm. This strategy effectively facilitated surgical intervention for the dissecting aortic aneurysm in the chronic phase after MV-TEER. Several reports showed the effectiveness of MV-TEER in cases of degenerative MR where surgical operation carries a high risk, but case report of MV-TEER in Marfan syndrome is rare. In recent years, the effectiveness of MV-TEER has also been reported as a “bridge therapy” for heart transplantation. MV-TEER is considered a potential option to serve as a bridge to other invasive intervention.
{"title":"Mitral valve transcatheter edge-to-edge repair as a bridge to treat aortic dissecting aneurysm in a case of Marfan syndrome: A case report","authors":"Takanori Kawamoto, Tomohito Kogure, Chihiro Koyanagi, Kyomi Ashihara, Junichi Yamaguchi","doi":"10.1093/ehjcr/ytae338","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae338","url":null,"abstract":"\u0000 \u0000 \u0000 Marfan syndrome is an inherited disorder that manifests with various cardiovascular conditions. This case report discusses a patient with Marfan syndrome presenting with concurrent dissecting aortic aneurysm and acute mitral valve regurgitation (MR), exploring treatment strategies for this unique case.\u0000 \u0000 \u0000 \u0000 A 57-year-old man diagnosed with Marfan syndrome presented with progressive dyspnea and awareness of orthopnea. Acute heart failure (HF) due to acute MR associated with chordae rupture was diagnosed. However, contrast-enhanced CT revealed the coexistence of a massive dissecting aortic aneurysm, indicating surgical intervention. The dissecting aortic aneurysm extended over a large area. Given the high risk of simultaneous surgery with the mitral valve, a staged approach was adopted. Mitral valve transcatheter edge-to-edge repair (MV-TEER) was performed as the initial step to reduce the perioperative HF risk, followed by a planned two-stage surgery for the dissecting aortic aneurysm. This strategy effectively facilitated surgical intervention for the dissecting aortic aneurysm in the chronic phase after MV-TEER.\u0000 \u0000 \u0000 \u0000 Several reports showed the effectiveness of MV-TEER in cases of degenerative MR where surgical operation carries a high risk, but case report of MV-TEER in Marfan syndrome is rare. In recent years, the effectiveness of MV-TEER has also been reported as a “bridge therapy” for heart transplantation. MV-TEER is considered a potential option to serve as a bridge to other invasive intervention.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141661432","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}
Bradycardias can have a number of different aetiologies, including as a side-effect of medications. Brimonidine is a rare, but recognised, cause of bradycardia. Brimonidine is indicated in the treatment of facial erythema in rosacea when given as topical brimonidine gel (Mirvaso). It may also be administered as eye drops for raised intraocular pressure in open angle glaucoma and ocular hypertension. Brimonidine is an alpha-2 agonist, which if systemically absorbed can present with bradycardia, hypotension and dizziness. The authors are unaware of any other case reports regarding topical administration of Mirvaso in an adult and symptomatic bradycardia. We present the case of a 78 year old man with a background of rosacea, benign prostatic hyperplasia and hypertension who had two separate admissions with symptomatic bradycardia. Electrocardiograms showed sinus bradycardia with AV block 1st degree, with rate recorded as low as 31 bpm during a syncopal episode. These episodes of symptomatic bradycardia were intermittent and had a temporal association with topical administration of Mirvaso. He had no further episodes of bradycardia on discontinuation of Mirvaso and has remained symptom free for over six months. The topical administration of Mirvaso should be avoided to broken or inflamed skin. This is owing to the increased risk of systemic absorption, which as in this case report, may present with bradycardia. This case reiterates the importance of completing a full medication history including of all topical and parenteral medications in patients with arrhythmia.
{"title":"Symptomatic Bradycardia Associated with Topical Brimonidine Gel (Mirvaso) Administration: A Case Report","authors":"D. Jesudason, Nageswary Appalanaidu, Asif Khan","doi":"10.1093/ehjcr/ytae329","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae329","url":null,"abstract":"\u0000 \u0000 \u0000 Bradycardias can have a number of different aetiologies, including as a side-effect of medications. Brimonidine is a rare, but recognised, cause of bradycardia. Brimonidine is indicated in the treatment of facial erythema in rosacea when given as topical brimonidine gel (Mirvaso). It may also be administered as eye drops for raised intraocular pressure in open angle glaucoma and ocular hypertension. Brimonidine is an alpha-2 agonist, which if systemically absorbed can present with bradycardia, hypotension and dizziness. The authors are unaware of any other case reports regarding topical administration of Mirvaso in an adult and symptomatic bradycardia.\u0000 \u0000 \u0000 \u0000 We present the case of a 78 year old man with a background of rosacea, benign prostatic hyperplasia and hypertension who had two separate admissions with symptomatic bradycardia. Electrocardiograms showed sinus bradycardia with AV block 1st degree, with rate recorded as low as 31 bpm during a syncopal episode. These episodes of symptomatic bradycardia were intermittent and had a temporal association with topical administration of Mirvaso. He had no further episodes of bradycardia on discontinuation of Mirvaso and has remained symptom free for over six months.\u0000 \u0000 \u0000 \u0000 The topical administration of Mirvaso should be avoided to broken or inflamed skin. This is owing to the increased risk of systemic absorption, which as in this case report, may present with bradycardia. This case reiterates the importance of completing a full medication history including of all topical and parenteral medications in patients with arrhythmia.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141660475","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}
Samah El-Mhadi, Belghait El Hajjaj, Nabil Laktib, Latifa Oukerraj, M. Cherti
Lutembacher's syndrome refers to the rare combination of atrial septal defect and mitral stenosis. This condition is still underdiagnosed despite its distinct clinical, paraclinical and therapeutic implications. We report the case of a 60-year-old woman presenting with acute congestive heart failure. Investigations revealed the combination of low-gradient severe rheumatic mitral stenosis and ostium secundum atrial septal defect. Despite the medical team's conservative management of her condition and recommendation for surgical intervention, the patient chose to discharge herself against medical advice. She deceased 6 months later. Lutembacher's syndrome continues to be a rare condition in our practice. Once low-gradient severe mitral stenosis is diagnosed, it's essential to search for an associated atrial septal defect, especially when there is an early onset of significant right ventricular enlargement or dysfunction, or when an intriguing inconsistency exists between the mitral area and the transmitral gradient.
{"title":"A Rare Case of Lutembacher’s Syndrome Presenting with Low-Gradient Severe Mitral Stenosis: Case Report and Literature Overview","authors":"Samah El-Mhadi, Belghait El Hajjaj, Nabil Laktib, Latifa Oukerraj, M. Cherti","doi":"10.1093/ehjcr/ytae327","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae327","url":null,"abstract":"\u0000 \u0000 \u0000 Lutembacher's syndrome refers to the rare combination of atrial septal defect and mitral stenosis. This condition is still underdiagnosed despite its distinct clinical, paraclinical and therapeutic implications.\u0000 \u0000 \u0000 \u0000 We report the case of a 60-year-old woman presenting with acute congestive heart failure. Investigations revealed the combination of low-gradient severe rheumatic mitral stenosis and ostium secundum atrial septal defect. Despite the medical team's conservative management of her condition and recommendation for surgical intervention, the patient chose to discharge herself against medical advice. She deceased 6 months later.\u0000 \u0000 \u0000 \u0000 Lutembacher's syndrome continues to be a rare condition in our practice. Once low-gradient severe mitral stenosis is diagnosed, it's essential to search for an associated atrial septal defect, especially when there is an early onset of significant right ventricular enlargement or dysfunction, or when an intriguing inconsistency exists between the mitral area and the transmitral gradient.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141660764","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}
Marina Arai, Yu Kataoka, Y. Tsukamoto, Keiko Ohta-Ogo
Giant cell myocarditis is a fatal disease which could be rapidly progressive if not properly managed. However, the role of immunosuppressive therapy, especially in refractory cases, remains unclear. A 76-year-old man presented with back pain with elevated cardiac enzymes. Skeletal muscle and endomyocardial biopsies revealed giant cell myositis and giant cell myocarditis. Despite the initial immunosuppressive therapy, cardiac enzymes continued to rise. Serial endomyocardial biopsies enabled combination treatment of prednisolone, cyclosporine, and mycophenolate mofetil according to histological inflammatory activity. We presented a case of refractory giant cell myocarditis preceded by giant cell myositis. While endomyocardial biopsy is an approach with risk of procedural complications, it can guide giant cell myocarditis management when the initial immunosuppressive therapy is ineffective.
{"title":"Giant Cell Myocarditis Attributable to Myositis: Therapeutic Management under the Guidance of Serial Endomyocardial Biopsy. A case report","authors":"Marina Arai, Yu Kataoka, Y. Tsukamoto, Keiko Ohta-Ogo","doi":"10.1093/ehjcr/ytae326","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae326","url":null,"abstract":"\u0000 \u0000 \u0000 Giant cell myocarditis is a fatal disease which could be rapidly progressive if not properly managed. However, the role of immunosuppressive therapy, especially in refractory cases, remains unclear.\u0000 \u0000 \u0000 \u0000 A 76-year-old man presented with back pain with elevated cardiac enzymes. Skeletal muscle and endomyocardial biopsies revealed giant cell myositis and giant cell myocarditis. Despite the initial immunosuppressive therapy, cardiac enzymes continued to rise. Serial endomyocardial biopsies enabled combination treatment of prednisolone, cyclosporine, and mycophenolate mofetil according to histological inflammatory activity.\u0000 \u0000 \u0000 \u0000 We presented a case of refractory giant cell myocarditis preceded by giant cell myositis. While endomyocardial biopsy is an approach with risk of procedural complications, it can guide giant cell myocarditis management when the initial immunosuppressive therapy is ineffective.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141659020","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}
Xing-ye Wang, Lu He, Xue-gang Xie, Xiao-qin Liu, Yu-shun Zhang
Myocardial infarction (MI) caused by patent foramen ovale (PFO)-based paradoxical embolism is rare, and there are few case reports in the literature. Here, we report a case of MI in which optical coherence tomography revealed in situ thrombi in the PFO channel. In addition to paradoxical embolism, in situ thrombus may also be one of the pathogenic mechanisms of PFO in patients with MI.
{"title":"Thrombus Inside the Channel of Patent Foramen Ovale Revealed by Optical Coherence Tomography Imaging in a Patient with Myocardial Infarction—Case Report","authors":"Xing-ye Wang, Lu He, Xue-gang Xie, Xiao-qin Liu, Yu-shun Zhang","doi":"10.1093/ehjcr/ytae304","DOIUrl":"https://doi.org/10.1093/ehjcr/ytae304","url":null,"abstract":"\u0000 \u0000 \u0000 Myocardial infarction (MI) caused by patent foramen ovale (PFO)-based paradoxical embolism is rare, and there are few case reports in the literature.\u0000 \u0000 \u0000 \u0000 Here, we report a case of MI in which optical coherence tomography revealed in situ thrombi in the PFO channel.\u0000 \u0000 \u0000 \u0000 In addition to paradoxical embolism, in situ thrombus may also be one of the pathogenic mechanisms of PFO in patients with MI.\u0000","PeriodicalId":507701,"journal":{"name":"European Heart Journal - Case Reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141668667","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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