Coronary chronic total occlusions are challenging lesions with high rates of complications related to percutaneous intervention. We describe a successful angioplasty in a patient with a recent coronary perforation, using multiple techniques, such as stick and swap with Stingray, subintimal transcatheter withdrawal, and investment.
{"title":"Successful Percutaneous Coronary Intervention in Chronic Total Occlusion after Coronary Perforation.","authors":"Luis A Areiza, Juan F Rodriguez","doi":"10.1155/2023/6640439","DOIUrl":"https://doi.org/10.1155/2023/6640439","url":null,"abstract":"<p><p>Coronary chronic total occlusions are challenging lesions with high rates of complications related to percutaneous intervention. We describe a successful angioplasty in a patient with a recent coronary perforation, using multiple techniques, such as stick and swap with Stingray, subintimal transcatheter withdrawal, and investment.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"6640439"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10482517/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10559801","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}
VACTERL association is typically defined by the presence of at least three of the congenital malformations that make up the term including: vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula (TEF), renal anomalies, and limb deformities. Patients with VACTERL are typically managed through immediate-postnatal-surgical correction of the specific congenital anomalies (typically anal atresia, specific types of cardiac malformations, and/or TEF), followed by long-term medical management of the congenital malformations. Although congenital anomalies might have long-lasting effects, the prognosis can be positive when the best surgical remedy is possible. Here, we present a case of 5 years female that is a known case of VACTERL Status Post (S/P)TEF repair, S/P double outlet right ventricle repair at sixth day and fifth month of life. This child managed to survive despite being operated in a resource-limited setting.
{"title":"Successful Repair of TEF and DORV in a Child in a Resource-Limited Setting.","authors":"Eru Sujakhu, Rajendra Shilpakar, Dhruba Shrestha","doi":"10.1155/2023/1095670","DOIUrl":"https://doi.org/10.1155/2023/1095670","url":null,"abstract":"<p><p>VACTERL association is typically defined by the presence of at least three of the congenital malformations that make up the term including: vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula (TEF), renal anomalies, and limb deformities. Patients with VACTERL are typically managed through immediate-postnatal-surgical correction of the specific congenital anomalies (typically anal atresia, specific types of cardiac malformations, and/or TEF), followed by long-term medical management of the congenital malformations. Although congenital anomalies might have long-lasting effects, the prognosis can be positive when the best surgical remedy is possible. Here, we present a case of 5 years female that is a known case of VACTERL Status Post (S/P)TEF repair, S/P double outlet right ventricle repair at sixth day and fifth month of life. This child managed to survive despite being operated in a resource-limited setting.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"1095670"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9943623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10770015","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}
Idiopathic third-degree atrioventricular (AV) block in a relatively young patient is an uncommon phenomenon. Even more rare is when the third-degree heart block is alternating with the first- and second-degree AV blocks. In this case, we present a 39-year-old man with varying degrees of AV block, alternating the third-degree, second-degree, and first-degree AV blocks. The patient underwent an extensive workup for underlying etiologies, and results were inconclusive. A pacemaker was implanted and set for physiologic pacing via left bundle branch area pacing (LBBAP). This case will discuss potential genetic abnormalities associated with AV block and highlight LBBAP as an emerging technique for physiologic pacing.
{"title":"The Heart Block Hat-Trick: A Case of Alternating First-, Second-, and Third-Degree Heart Blocks.","authors":"Nicholas Huerta, Salman Malik, Christopher Haas","doi":"10.1155/2023/8664315","DOIUrl":"https://doi.org/10.1155/2023/8664315","url":null,"abstract":"<p><p>Idiopathic third-degree atrioventricular (AV) block in a relatively young patient is an uncommon phenomenon. Even more rare is when the third-degree heart block is alternating with the first- and second-degree AV blocks. In this case, we present a 39-year-old man with varying degrees of AV block, alternating the third-degree, second-degree, and first-degree AV blocks. The patient underwent an extensive workup for underlying etiologies, and results were inconclusive. A pacemaker was implanted and set for physiologic pacing via left bundle branch area pacing (LBBAP). This case will discuss potential genetic abnormalities associated with AV block and highlight LBBAP as an emerging technique for physiologic pacing.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"8664315"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10284657/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9710368","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}
Timothy J O'Toole, Faisal Dadi, Patrick Kietrsunthorn, Jason Foerst, Ali Hama Amin
In this case report, we describe a 55-year-old female patient with worsening exertional dyspnea who is referred to the cardiology department, due to the appearance of worsening pulmonary vascular disease on computed tomography (CT) of the chest. Previous transthoracic echocardiograms (TTE) identified right ventricle enlargement, but no other structural abnormalities. She completed cardiac magnetic resonance (CMR) imaging, which identified a large secundum atrial septal defect (ASD). She subsequently underwent surgical planning and correction of the lesion with improvement of her symptoms. This case and a growing body of literature support the use of CMR as an alternative imaging modality for the diagnosis of congenital heart disease (CHD).
{"title":"Missed Connections: Identification of Atrial Septal Defect by MRI.","authors":"Timothy J O'Toole, Faisal Dadi, Patrick Kietrsunthorn, Jason Foerst, Ali Hama Amin","doi":"10.1155/2023/2393308","DOIUrl":"https://doi.org/10.1155/2023/2393308","url":null,"abstract":"<p><p>In this case report, we describe a 55-year-old female patient with worsening exertional dyspnea who is referred to the cardiology department, due to the appearance of worsening pulmonary vascular disease on computed tomography (CT) of the chest. Previous transthoracic echocardiograms (TTE) identified right ventricle enlargement, but no other structural abnormalities. She completed cardiac magnetic resonance (CMR) imaging, which identified a large secundum atrial septal defect (ASD). She subsequently underwent surgical planning and correction of the lesion with improvement of her symptoms. This case and a growing body of literature support the use of CMR as an alternative imaging modality for the diagnosis of congenital heart disease (CHD).</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"2393308"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9988386/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9084103","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}
Interventional treatment of restrictive atrial septal defects in complex heart disease is considered state-of-the-art therapy up to date. Nevertheless, dedicated balloons are lacking so far, as several products have been withdrawn from the market. We report on off-label use of a balloon embolectomy catheter used successfully in a preterm patient and discuss whether this device might be used in other patients as well as it seems to be promising due to its shape and versatility.
{"title":"Balloon-Atrioseptostomy in Small Children Using an Embolectomy Catheter: Preliminary Data.","authors":"R Dalla Pozza, M Hermann, N A Haas","doi":"10.1155/2023/9920336","DOIUrl":"https://doi.org/10.1155/2023/9920336","url":null,"abstract":"<p><p>Interventional treatment of restrictive atrial septal defects in complex heart disease is considered state-of-the-art therapy up to date. Nevertheless, dedicated balloons are lacking so far, as several products have been withdrawn from the market. We report on off-label use of a balloon embolectomy catheter used successfully in a preterm patient and discuss whether this device might be used in other patients as well as it seems to be promising due to its shape and versatility.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"9920336"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10396523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9993396","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}
Dimitrios Siamkouris, Elmar Offers, Marc Schloesser, Gjoko Ilieski, Stergios Tzikas
Conduction restoration in second- and third-degree atrioventricular (AV) block after revascularization in acute coronary syndrome (ACS) setting is well established, however this is not the case in non-ACS setting. We present a case of a patient referred for permanent pacemaker implantation, due to high degree AV block (HAVB), who restored his conduction possibly due to targeted revascularization. Thus, this case sheds insight in the reversibility potential of HAVB after revascularization in non-ACS patients, which although signified in current literature, is still far from being a recommendation, due to lack of robust scientific confirmation.
{"title":"Ischemia Possibly Associated with High Degree Atrioventricular Block.","authors":"Dimitrios Siamkouris, Elmar Offers, Marc Schloesser, Gjoko Ilieski, Stergios Tzikas","doi":"10.1155/2023/6676757","DOIUrl":"https://doi.org/10.1155/2023/6676757","url":null,"abstract":"<p><p>Conduction restoration in second- and third-degree atrioventricular (AV) block after revascularization in acute coronary syndrome (ACS) setting is well established, however this is not the case in non-ACS setting. We present a case of a patient referred for permanent pacemaker implantation, due to high degree AV block (HAVB), who restored his conduction possibly due to targeted revascularization. Thus, this case sheds insight in the reversibility potential of HAVB after revascularization in non-ACS patients, which although signified in current literature, is still far from being a recommendation, due to lack of robust scientific confirmation.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"6676757"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10460276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10483363","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}
M Gruszczynski, A Müller-Burri, A Häussler, A Breitenstein, H Rodriguez Cetina Biefer, O Dzemali
Significant lead-induced tricuspid regurgitation after cardiovascular implantable electronic devices is not uncommon. Absolute or relative contraindications to place the lead in the right ventricle after tricuspid valve (TV) surgery still remains a challenge. We report about successful lead extraction followed by transvenous implantable cardioverter defibrillator lead placement in the side branches of coronary sinus after TV reconstruction. Furthermore, we discuss therapeutic options to deliver concomitant anti-bradycardia therapy, technical pitfalls, and surgical approaches.
{"title":"Transvenous ICD Implantation into a Coronary Sinus Branch: A Safe and Feasible Alternative to Deliver ICD after Tricuspid Valve Reconstruction.","authors":"M Gruszczynski, A Müller-Burri, A Häussler, A Breitenstein, H Rodriguez Cetina Biefer, O Dzemali","doi":"10.1155/2023/6646224","DOIUrl":"https://doi.org/10.1155/2023/6646224","url":null,"abstract":"<p><p>Significant lead-induced tricuspid regurgitation after cardiovascular implantable electronic devices is not uncommon. Absolute or relative contraindications to place the lead in the right ventricle after tricuspid valve (TV) surgery still remains a challenge. We report about successful lead extraction followed by transvenous implantable cardioverter defibrillator lead placement in the side branches of coronary sinus after TV reconstruction. Furthermore, we discuss therapeutic options to deliver concomitant anti-bradycardia therapy, technical pitfalls, and surgical approaches.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"6646224"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9931479/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10757725","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}
Introduction: The superior vena cava (SVC) is an important non-pulmonary venous foci of atrial fibrillation (AF) and is known as the arrhythmogenic site of scar-related atrial tachycardia (AT). Scar-related ATs may occur after catheter ablation and open heart surgery; however, idiopathic AT rarely occurs. Case Presentation. A 77-year-old male with terminal diabetic nephropathy complained of dialysis-induced hypotension due to AF and was admitted to our hospital for catheter ablation. Here, we report a case of non-iatrogenic localized-reentrant figure of eight AT in the SVC.
Conclusion: SVC has the arrhythmogenic potential for re-entrant tachycardia, and the development of mapping technology can reveal arrhythmogenic mechanisms.
{"title":"Non-Iatrogenic Localized-Reentrant Figure of Eight Atrial Tachycardias in the Superior Vena Cava.","authors":"Shota Tokuno, Kenjiro Miyamoto, Ryuichi Usui","doi":"10.1155/2023/5074946","DOIUrl":"https://doi.org/10.1155/2023/5074946","url":null,"abstract":"<p><strong>Introduction: </strong>The superior vena cava (SVC) is an important non-pulmonary venous foci of atrial fibrillation (AF) and is known as the arrhythmogenic site of scar-related atrial tachycardia (AT). Scar-related ATs may occur after catheter ablation and open heart surgery; however, idiopathic AT rarely occurs. <i>Case Presentation</i>. A 77-year-old male with terminal diabetic nephropathy complained of dialysis-induced hypotension due to AF and was admitted to our hospital for catheter ablation. Here, we report a case of non-iatrogenic localized-reentrant figure of eight AT in the SVC.</p><p><strong>Conclusion: </strong>SVC has the arrhythmogenic potential for re-entrant tachycardia, and the development of mapping technology can reveal arrhythmogenic mechanisms.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"5074946"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10232176/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9568572","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}
Bernhard M Boehm, Jochen Gaa, Petra Hoppmann, Eimo Martens, Dominik S Westphal
Background. Atrial fibrillation (AF) is a common arrhythmia in elderly patients and is associated with increased risk of mortality. The pathogenesis of AF is complex and based on multiple genetic and environmental factors. Genome-wide association studies identified several loci in AF patients, indicating the complex genetic architecture of this disease. In rare cases, familial forms of AF have been described. Today, pathogenic variants in at least 11 different genes are associated with monogenic AF. Case presentation. The 37-year-old male patient presented to our emergency department with AF. At the age of 35, he had already been diagnosed with paroxysmal AF. Additionally, his 34-year-old brother had also been diagnosed with AF as well as nonobstructive hypertrophic cardiomyopathy. Moreover, the patient's father was diagnosed with AF in his twenties. Transthoracic echocardiography and cardiac MRI revealed a reduced systolic left ventricular ejection without any signs of hypertrophic cardiomyopathy. Genetic testing identified the heterozygous missense variants c.3371C > T, p.(Pro1124Leu) in RYR2 (NM_001035.3) and c.2524C > A, p.(Pro842Thr) in HCN4 (NM_005477.3) in the patient's and his brother's DNA. Discussion. This case of familial AF helps to strengthen the role of RYR2 as a disease gene in the context of AF. Although the variant in RYR2 needs to be classified formally as variant of unknown significance, we regard it as probably disease-causing due to the previously published data. As RYR2 has already been identified as a possible target for prevention and therapy of AF, the knowledge of variants in RYR2 might become even more crucial for individual molecular therapies in the future.
背景。心房颤动(AF)是老年患者常见的心律失常,与死亡风险增加有关。房颤的发病机制复杂,受多种遗传和环境因素的影响。全基因组关联研究在房颤患者中发现了几个基因座,表明这种疾病的复杂遗传结构。在极少数情况下,家族性房颤也有报道。目前,至少有11种不同基因的致病变异与单基因房颤有关。患者男,37岁,因房颤就诊于急诊科。35岁时,患者已被诊断为阵发性房颤。此外,其34岁的兄弟也被诊断为房颤和非阻塞性肥厚性心肌病。此外,患者的父亲在20多岁时被诊断出患有房颤。经胸超声心动图和心脏MRI显示收缩期左心室射血减少,无肥厚性心肌病的迹象。基因检测在患者及其兄弟的DNA中发现了RYR2 (NM_001035.3)中c.3371C > T, p.(Pro1124Leu)杂合错义变异和HCN4 (NM_005477.3)中c.2524C > A, p.(Pro842Thr)杂合错义变异。讨论。本例家族性房颤有助于加强RYR2在房颤背景下作为疾病基因的作用。尽管RYR2的变异需要正式归类为意义未知的变异,但根据先前发表的数据,我们认为它可能是致病的。由于RYR2已被确定为预防和治疗房颤的可能靶点,对RYR2变异的了解可能在未来的个体分子治疗中变得更加重要。
{"title":"The Role of <i>RYR2</i> in Atrial Fibrillation.","authors":"Bernhard M Boehm, Jochen Gaa, Petra Hoppmann, Eimo Martens, Dominik S Westphal","doi":"10.1155/2023/6555998","DOIUrl":"https://doi.org/10.1155/2023/6555998","url":null,"abstract":"<p><p><i>Background</i>. Atrial fibrillation (AF) is a common arrhythmia in elderly patients and is associated with increased risk of mortality. The pathogenesis of AF is complex and based on multiple genetic and environmental factors. Genome-wide association studies identified several loci in AF patients, indicating the complex genetic architecture of this disease. In rare cases, familial forms of AF have been described. Today, pathogenic variants in at least 11 different genes are associated with monogenic AF. <i>Case presentation</i>. The 37-year-old male patient presented to our emergency department with AF. At the age of 35, he had already been diagnosed with paroxysmal AF. Additionally, his 34-year-old brother had also been diagnosed with AF as well as nonobstructive hypertrophic cardiomyopathy. Moreover, the patient's father was diagnosed with AF in his twenties. Transthoracic echocardiography and cardiac MRI revealed a reduced systolic left ventricular ejection without any signs of hypertrophic cardiomyopathy. Genetic testing identified the heterozygous missense variants c.3371C > T, p.(Pro1124Leu) in <i>RYR2</i> (NM_001035.3) and c.2524C > A, p.(Pro842Thr) in <i>HCN4</i> (NM_005477.3) in the patient's and his brother's DNA. <i>Discussion</i>. This case of familial AF helps to strengthen the role of <i>RYR2</i> as a disease gene in the context of AF. Although the variant in <i>RYR2</i> needs to be classified formally as variant of unknown significance, we regard it as probably disease-causing due to the previously published data. As <i>RYR2</i> has already been identified as a possible target for prevention and therapy of AF, the knowledge of variants in <i>RYR2</i> might become even more crucial for individual molecular therapies in the future.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"6555998"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10033205/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9245961","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}
Mohamed Laimoud, Ziyad Alanazi, Fayez Alahmadi, Abdullah Aldalaan
Background: Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension characterized by diffuse venous vasculopathy and increased pulmonary vascular resistance resulting in right-sided heart failure. Case Presentation. A 22-year-old female patient started to have dyspnea with minimal effort and was diagnosed to have pre-capillary pulmonary hypertension (PH) with right-sided heart failure. Initially, she was diagnosed to have idiopathic PH. She developed life-threatening pulmonary oedema and cardiogenic shock after pulmonary vasodilator therapy. A genetic study was done and revealed the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) gene on chromosome 15, which was diagnostic to heritable PVOD. After failure to achieve hemodynamic stabilization with conventional cardiopulmonary support measures, extracorporeal membrane oxygenation (ECMO) supported her till bilateral lung transplantation, which was unfortunately complicated by acute graft rejection. After a prolonged intensive care unit stay with 4-month ECMO support, the second bilateral lung transplantation was done, and the patient survived and was discharged.
Conclusions: Clinical recognition of PVOD is crucial due to its challenging diagnosis, need for genetic study, rapid deterioration with pulmonary vasodilators, and bad prognosis. Lung transplantation is the definitive treatment for eligible candidates.
{"title":"A Challenging Case of Genetically and Histologically Diagnosed Pulmonary Veno-Occlusive Disease with Extracorporeal Life Support and Redo Lung Transplantation.","authors":"Mohamed Laimoud, Ziyad Alanazi, Fayez Alahmadi, Abdullah Aldalaan","doi":"10.1155/2023/4846338","DOIUrl":"https://doi.org/10.1155/2023/4846338","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension characterized by diffuse venous vasculopathy and increased pulmonary vascular resistance resulting in right-sided heart failure. <i>Case Presentation</i>. A 22-year-old female patient started to have dyspnea with minimal effort and was diagnosed to have pre-capillary pulmonary hypertension (PH) with right-sided heart failure. Initially, she was diagnosed to have idiopathic PH. She developed life-threatening pulmonary oedema and cardiogenic shock after pulmonary vasodilator therapy. A genetic study was done and revealed the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) gene on chromosome 15, which was diagnostic to heritable PVOD. After failure to achieve hemodynamic stabilization with conventional cardiopulmonary support measures, extracorporeal membrane oxygenation (ECMO) supported her till bilateral lung transplantation, which was unfortunately complicated by acute graft rejection. After a prolonged intensive care unit stay with 4-month ECMO support, the second bilateral lung transplantation was done, and the patient survived and was discharged.</p><p><strong>Conclusions: </strong>Clinical recognition of PVOD is crucial due to its challenging diagnosis, need for genetic study, rapid deterioration with pulmonary vasodilators, and bad prognosis. Lung transplantation is the definitive treatment for eligible candidates.</p>","PeriodicalId":51760,"journal":{"name":"Case Reports in Cardiology","volume":"2023 ","pages":"4846338"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10465249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10118759","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}