Background:Congenital absence of pericardium(CAP),also known as pericardial agenesis,represents an uncommon cardiac abnormality and mostly incidental finding.It can be subdivided into complete and partial(left or righ...Background:Congenital absence of pericardium(CAP),also known as pericardial agenesis,represents an uncommon cardiac abnormality and mostly incidental finding.It can be subdivided into complete and partial(left or right-sided)forms.Because of its infrequency,just case reports and a few case series have been released so far.This paper represents the largest systematic review in the field.Nine features(age at diagnosis,type,gender,clinical presentation,electrocardiography,imaging(ultrasounds,CT/MRI),concomitant cardiac defects,and outcome)were analysed.Methods:The electronic database PubMed was investigated from its establishment up to July 15th,2023.Just case reports and case series were included.Animal studies,papers that were not in English,Spanish,and Italian,and those manuscripts not reporting at least seven of the nine analysed features.were ruled out.The analysed data were reported mostly in terms of percentage.Results:One hundred eighty studies were included encompassing 247 patients.More than half of reviewed CAP cases were in males(63.2%).The mean age at diagnosis was 31.8±19.3 years;a range of 32 weeks of gestation-81 years).23.5%of the patients did not report any symptoms.The most common clinical presentations were chest pain(35.2%)and dyspnoea(29.2%).The most commonly seen ECG changes were right axis deviation(28.7%)and right bundle branch block(23.9%).CAP was suspected or diagnosed by echocardiography in 20.1%of cases.The diagnosis was made by CT and/or MRI in 61.9%of cases.CAP was left-sided in 71.2%,complete in 23.1%,and right-sided in 5.7%.A concomitant congenital heart defect was found in 22.7%,especially in the form of atrial septal defect(6.5%)and patency of ductus arteriosus(2.8%).The pericardial repair was required in 12.9% of the incomplete forms of the disease.Never did the complete form require surgical correction.The outcome appeared favourable in the vast majority of cases,with just 18 deaths(7.3%).Discussion:The main limitation of this systematic review is that it is based just on case reports and case series,due to the lack of large studies on CAP.However,it represents the largest analysis in the field.Due to the rarity of CAP establishing an International Registry is recommended.展开更多
Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure r...Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.展开更多
Objective:Sudden cardiac death(SCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are lacking.We sought to characterize the...Objective:Sudden cardiac death(SCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are lacking.We sought to characterize the cohort who had sudden cardiac death,most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes.Methods:A retrospective cohort study was performed.Inclusion criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or SCD>30 days post-Fontan completion.Results:Of 1611 patients,20(1.2%)had VA;14(1.0%)had VA without SCD and 6(<1%)had SCD(6%of all deaths recorded in Registry;5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated).The median age at first VA was 20.5(14–32)years,10(50%)were females,and the median age at Fontan operation was 8(4–17)years.On univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(p<0.001)were associated with VA.Earlier Fontan era(p<0.003),atriopulmonary Fontan(p<0.001),pre-Fontan atrioventricular valve repair(p=0.013)pre-or post-Fontan atrial arrhythmia(p=0.010)were associated with SCD.Patients with VA had a 3 times higher risk of death or heart transplant(HR 3.27(1.19,8.98),p=0.02).Conclusions:A proportion of people living with a Fontan circulation have malignant VA.Routine VA screening in this cohort is essential.More data are needed to aid risk stratification.展开更多
Background:Hypertrophic scars and keloids,common complications following median sternotomy for cardiac surgery,significantly impact patient quality of life due to their aesthetic and symptomatic burden.Recent advanceme...Background:Hypertrophic scars and keloids,common complications following median sternotomy for cardiac surgery,significantly impact patient quality of life due to their aesthetic and symptomatic burden.Recent advancements in laser therapy have made it a prominent option for managing these complex scars,yet a com-prehensive understanding of its efficacy is lacking.The aim of this scoping review is to explore the effects of laser therapy in managing hypertrophic scars and keloids after median sternotomy.Methods:This scoping review ana-lyzed studies up to February 2024 from databases including PubMed,EMBASE,CINAHL,Scopus,Web of Science,and the Cochrane Library.We included any study that assessed laser therapy’s effects on hypertrophic scars and keloids following median sternotomy.Studies were selected based on predefined inclusion criteria with-out publication year,design,or origin restrictions.Results:Six studies met the inclusion criteria,involving a total offive RCTs and one review.These studies primarily tested 585 and 595-nm pulsed dye laser(PDL)treatments,focusing on scar appearance,patient symptoms,and treatment satisfaction.Most studies reported significant improvements in scar height reduction and patient symptom relief after treatment,with mixed results for scar erythema and elasticity.Adverse events were generally mild and transient.Conclusions:Laser therapy offers a beneficial approach for improving the appearance and symptoms of hypertrophic scars and keloids post-median sternotomy.However,further research is necessary to optimize treatment parameters and explore the long-term psychosocial impacts of this therapy.This review highlights the need for more comprehensive studies to establish standardized treatment protocols and evaluate their effectiveness.展开更多
Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature abo...Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsaiva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less weil-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.展开更多
文摘Background:Congenital absence of pericardium(CAP),also known as pericardial agenesis,represents an uncommon cardiac abnormality and mostly incidental finding.It can be subdivided into complete and partial(left or right-sided)forms.Because of its infrequency,just case reports and a few case series have been released so far.This paper represents the largest systematic review in the field.Nine features(age at diagnosis,type,gender,clinical presentation,electrocardiography,imaging(ultrasounds,CT/MRI),concomitant cardiac defects,and outcome)were analysed.Methods:The electronic database PubMed was investigated from its establishment up to July 15th,2023.Just case reports and case series were included.Animal studies,papers that were not in English,Spanish,and Italian,and those manuscripts not reporting at least seven of the nine analysed features.were ruled out.The analysed data were reported mostly in terms of percentage.Results:One hundred eighty studies were included encompassing 247 patients.More than half of reviewed CAP cases were in males(63.2%).The mean age at diagnosis was 31.8±19.3 years;a range of 32 weeks of gestation-81 years).23.5%of the patients did not report any symptoms.The most common clinical presentations were chest pain(35.2%)and dyspnoea(29.2%).The most commonly seen ECG changes were right axis deviation(28.7%)and right bundle branch block(23.9%).CAP was suspected or diagnosed by echocardiography in 20.1%of cases.The diagnosis was made by CT and/or MRI in 61.9%of cases.CAP was left-sided in 71.2%,complete in 23.1%,and right-sided in 5.7%.A concomitant congenital heart defect was found in 22.7%,especially in the form of atrial septal defect(6.5%)and patency of ductus arteriosus(2.8%).The pericardial repair was required in 12.9% of the incomplete forms of the disease.Never did the complete form require surgical correction.The outcome appeared favourable in the vast majority of cases,with just 18 deaths(7.3%).Discussion:The main limitation of this systematic review is that it is based just on case reports and case series,due to the lack of large studies on CAP.However,it represents the largest analysis in the field.Due to the rarity of CAP establishing an International Registry is recommended.
文摘Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.
文摘Objective:Sudden cardiac death(SCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are lacking.We sought to characterize the cohort who had sudden cardiac death,most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes.Methods:A retrospective cohort study was performed.Inclusion criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or SCD>30 days post-Fontan completion.Results:Of 1611 patients,20(1.2%)had VA;14(1.0%)had VA without SCD and 6(<1%)had SCD(6%of all deaths recorded in Registry;5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated).The median age at first VA was 20.5(14–32)years,10(50%)were females,and the median age at Fontan operation was 8(4–17)years.On univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(p<0.001)were associated with VA.Earlier Fontan era(p<0.003),atriopulmonary Fontan(p<0.001),pre-Fontan atrioventricular valve repair(p=0.013)pre-or post-Fontan atrial arrhythmia(p=0.010)were associated with SCD.Patients with VA had a 3 times higher risk of death or heart transplant(HR 3.27(1.19,8.98),p=0.02).Conclusions:A proportion of people living with a Fontan circulation have malignant VA.Routine VA screening in this cohort is essential.More data are needed to aid risk stratification.
文摘Background:Hypertrophic scars and keloids,common complications following median sternotomy for cardiac surgery,significantly impact patient quality of life due to their aesthetic and symptomatic burden.Recent advancements in laser therapy have made it a prominent option for managing these complex scars,yet a com-prehensive understanding of its efficacy is lacking.The aim of this scoping review is to explore the effects of laser therapy in managing hypertrophic scars and keloids after median sternotomy.Methods:This scoping review ana-lyzed studies up to February 2024 from databases including PubMed,EMBASE,CINAHL,Scopus,Web of Science,and the Cochrane Library.We included any study that assessed laser therapy’s effects on hypertrophic scars and keloids following median sternotomy.Studies were selected based on predefined inclusion criteria with-out publication year,design,or origin restrictions.Results:Six studies met the inclusion criteria,involving a total offive RCTs and one review.These studies primarily tested 585 and 595-nm pulsed dye laser(PDL)treatments,focusing on scar appearance,patient symptoms,and treatment satisfaction.Most studies reported significant improvements in scar height reduction and patient symptom relief after treatment,with mixed results for scar erythema and elasticity.Adverse events were generally mild and transient.Conclusions:Laser therapy offers a beneficial approach for improving the appearance and symptoms of hypertrophic scars and keloids post-median sternotomy.However,further research is necessary to optimize treatment parameters and explore the long-term psychosocial impacts of this therapy.This review highlights the need for more comprehensive studies to establish standardized treatment protocols and evaluate their effectiveness.
文摘Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsaiva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less weil-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.
