A large congenital atrial septal defect in an adult with delayed therapy

Patients with a large congenital atrial septal defect (ASD) traditionally have the ASD repaired at the preschool age. Unfortunately, insufficient education of patients regarding medical science and clinical recommendations can lead to delayed therapy, resulting in complications during adulthood. We report a rare case of a large congenital ASD in a 20-year-old man. Echocardiography showed a 67-mm ostium secundum defect and moderate mitral and tricuspid regurgitation. The patient underwent transthoracic ASD repair along with mitral and tricuspid valvuloplasty. This report emphasizes the importance of educating patients about congenital malformations and potential interventions in developing countries, particularly in rural communities.     

成人继发孔型房间隔缺损并发房颤患者三种治疗方法分析

目的介绍成人房间隔缺损（ASD）并发心房颤动（AF）患者的几种治疗方法,并分析其治疗效果。方法：回顾分析本院136例ASD并发有明显临床症状且药物治疗无效的AF病例,其中36例接受介入封堵＋经导管射频消融术（导管射频消融组）,84例体外循环下ASD补术＋改良迷宫术（改良迷宫组）,16例单纯介入封堵术（未行经导管射频消融术,单纯介入封堵组）,术前,术后12月用心脏超声仪评价右心房、右室内径及肺动脉压力和心电图变化。结果：所有病例的术中、术后均未出现严重并发症,所有病例均无死亡,随访12个月,36例接受介入封堵＋经导管射频消融术28例转复为窦性心律,8例仍为AF,后行二次射频消融术转为窦性心律,84例ASD补术＋改良迷宫手术患者中有66例转复窦性,14例失败仍为AF,4例为交界性心律,单纯介入封堵组16例8例成功,8例术后仍为AF,与术前比较,各组心脏超声检查示右心房、右心室内径均较术前明显缩小,肺动脉压力明显下降（均P〈0．05）。各组之间无显著差异。经导管射频消融组和改良迷宫手术组AF治愈率高（对比单纯介入组,均P〈0．05）,患者心慌不适更能得到改善,生活质量更高。结论：介入封堵及外科手术均能安全有效治疗ASD并发AF,每种方法各有利弊,可依据患者临床具体情况选择。     

Pulmonary vascular disease in a child with atrial septal defect of the secundum type and type I glycogen storage disease

A rare combination of atrial septal defect of the secundum type and type I glycogen storage disease was seen with early fatal outcome due to rapidly progressive severe pulmonary vascular disease. Possible reasons for the unexpected, rapidly progressive pulmonary vascular disease are discussed.     

Live three-dimensional right parasternal and supraclavicular transthoracic echocardiographic examination

In the present study, we describe the usefulness of the recently developed technique of live three-dimensional transthoracic echocardiography in the assessment of normal cardiac structures and adjacent vessels using the right parasternal and supraclavicular approaches. Examples of some abnormalities diagnosed from these approaches are also illustrated.     

Left atrial disc device thrombosis after atrial defect closure with the Gore® Cardioform septal occluder: A case series

Device thromboses after patent foramen ovale (PFO) and atrial septal defect (ASD) closure are rare but serious complications. They have been reported in different devices of virtually all manufacturers. Here, we report three cases of left atrial device thrombosis after atrial defect closure with the Gore® Cardioform septal occluder (GSO) in our recent institutional experience. All patients were symptomatic with new-onset neurological impairments and evidence of cerebral thromboembolism. Device thromboses have occurred despite antiplatelet therapy in two patients and moreover were observed late, at approximately 2 years after implantation, in two patients. One device was surgically explanted while in two cases, thrombi completely resolved with initiated anticoagulation. All patients had a favorable neurological recovery. Our observations suggest that regular follow-up echocardiography beyond 6 months after device implantation might be advisable in patients with GSO devices to rule out late device thromboses. More long-term follow-up data considering safety and late complications of contemporary PFO and ASD devices is required to establish confident evidence-based recommendations on long-term follow-up management and antithrombotic therapy regimen after PFO and ASD closure.     

Medical Therapy for Eisenmenger Syndrome: A Case Report and Review of Literature

Eisenmenger syndrome (ES) is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect is eventually reversed into a cyanotic right-to-left shunt. It is crucial to recognize this grave pathology at the earliest because once it develops, treatment by medical or surgical means becomes even more challenging. In past decades, various therapeutic options have been developed that address the specific pathophysiological aspects of the disease and have shown to improve functional capacity and quality of life. There are three major therapeutic pathways in pulmonary hypertension treatment - endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacyclin derivatives. These therapies not only improve hemodynamic parameters and exercise capacity but they also improve prognosis with various form of Pulmonary hypertension including ES. We report a case of a 35-year-old female of ostium secundum atrial septal defect with ES, started on upfront combination therapy of ambrisentan and tadalafil who demonstrated marked improvement after 3 months of medical therapy.