Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Sinus venosus atrial septal defect (SV‐ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV‐ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio‐CT was finally conclusive. This diagnostic approach allowed the surgical planning.     

继发孔房间隔缺损与全肺静脉异位引流的心电图和血流动力学比较

观察１１２例房间隔缺损（ＡＳＤ）和３９例全肺静脉异位引流（ＴＡＰＶＣ）的ＥＣＧ，若ＱＲＳ电轴正常、左偏或右偏伴Ｖ１呈ｒＳ，可排除ＴＡＰＶＣ。电轴右偏，Ｒｖ１≥１５ｍｍ，Ｖ１呈单纯Ｒ或ｑＲ型，而Ｖ５～６导联为ｒＳ型伴ＰⅡ≥０．２５ｍＶ高度提示ＴＡＰＶＣ。ＴＡＰＶＣ的心导管检查诊断依据：１．ＳａＱ２８０～９０％，且与肺动脉血氧饱和度相近，２．轻度肺动脉高压（≥４．１ｋＰａ）伴大的左向右分流（Ｑｐ／Ｑｓ＞３∶１），３．导管进入异常途径并采血化验血氧含量高于正常，４．肺动脉选择造影右房提早显影。     

Spectral Doppler Interrogation of the Pulmonary Veins in Atrial Septal Defect

The various components of the pulmonary venous (PV) flow are linked to physiological and pathological changes that predominantly occur in the left heart. Thus, spectral Doppler interrogation of the PVs provides hemodynamic insight mainly into left‐sided cardiac function. An exception to the dependence of PV flow on left heart events occurs in the setting of an atrial septal defect (ASD). The latter causes a portion of the PV blood flow, intended to cross the mitral valve, to be channeled into the more compliant right heart. This phenomenon makes the PV flow more dependent on the left‐to‐right interatrial shunt. The identification on the PV Doppler of a pattern that suggests uncoupling with left heart hemodynamics should raise the suspicion of an underlying ASD.     

Down Syndrome with Complete Atrioventricular Septal Defect,Hypertrophic Cardiomyopathy,and Pulmonary Vein Stenosis

The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome—an apparently unique combination. In addition, we discuss the relevant medical literature.     

房间隔缺损封堵术后右心形态和功能变化的随访观察

目的 :评价用Amplatzer封堵器封堵房间隔缺损患者术后和随访期心脏结构和功能的改变。方法 :入选病例 14例 ,房间隔缺损直径为 9～ 37( 2 2 7± 10 6 )mm。分别于术前、术后 1周和随访期 (平均 16 4±9 7个月 )行超声心动图检查 ,测量相关数据。结果 :与术前比较 ,术后及随访期右心室内径缩小 ,三尖瓣口、肺动脉瓣口血流峰值和肺动脉收缩压明显降低 (P <0 0 5～0 0 1) ,有显著性差异。未发现房室瓣和腔静脉梗阻以及主动脉壁受压 ,无新出现的心律失常和脑栓塞等并发症。结论 :房间隔缺损封堵术在消除异常分流的同时可有效改善右心高容量负荷状态 ,降低肺动脉高压 ,有效改善右心系统的几何构型     

Changes in Reverse Cardiac Remodeling after Percutaneous Atrial Septal Defect Closure in Children and Adults

Background: The influence of the timing of transcatheter atrial septal defect (ASD) closure on ventricular remodeling at 6 months after ASD closure is unclear. This study investigated changes in cardiac remodeling after transcatheter closure of large ASDs according to patient age at the time of the procedure. Methods: In this study, 41 children and 43 adults underwent percutaneous closure of a large ASD. Cardiac remodeling was assessed by two-dimensional echocardiography and electrocardiography before and at 6 months after ASD closure. Results: The age of the children and adults were 2.8 ± 3.1 and 50.0 ± 15.6 years, respectively. The Qp/Qs ratio of all patients was 2.24 ± 0.67. The right atrial (RA) maximal dimension and right ventricular (RV) transverse diameter were significantly decreased and the left ventricular (LV) dimension was significantly increased at 6 months after ASD closure. However, the difference in RA and RV dimension changes between the groups was not statistically significant. The difference in left atrial (LA) dimension changes between the groups was also not statistically significant, but the LV dimension significantly increased in children compared with that in adults (P = 0.018). The RV/LV ratio was decreased after ASD closure, and a significant difference was found in the RV/LV ratio changes between the groups. In ECG, the PR interval was significantly more decreased in adults than in children (P = 0.003). Conclusions: In conclusion, the LV diameter was significantly more increased in children than in adults at 6 months after percutaneous ASD closure. Thus, cardiac remodeling after percutaneous ASD closure varies in children and adults.     

Right Ventricular Remodeling after Transcatheter Closure of Atrial Septal Defect

Background: Right ventricular (RV) volume overload is a well‐known cardiac consequence of atrial septal defect (ASD) shunt, accounting for most of its long‐term complications. Thus cardiac volumetric unloading is a major aim of transcatheter ASD closure. We set to study the right ventricular remodeling after transcatheter ASD closure in patients with secundum ASD. Methods: We enrolled 46 patients who underwent successful transcatheter closure of ASD. We performed routine transthoracic echocardiographic studies, including three‐dimensional echocardiography and right ventricular myocardial performance index (RVMPI), before transcatheter ASD closure, and 3 days, 1 month after transcatheter ASD closure. Results: We found that: (1) the right ventricular end‐diastolic volume (RVEDV) and right ventricular end‐systolic volume (RVESV) (respectively 106.54±25.97 vs 69.78±10.46 mL, P < 0.05; 59.73±17.59 vs 33.84±7.18 mL, P < 0.05) were enlarged in patients with ASD compared with those in control subjects, resulting in a marked decrease of the right ventricular ejection fraction (RVEF) (44.82%±4.51% vs 54.11%±5.89%, P < 0.05) from normal values; (2) the isovolumic relaxation and isovolumic contraction times (respectively [77.61±16.49] ms vs (64.09±11.82) ms, P < 0.05; [28.04±9.57] ms vs [20.45±6.53] ms, P < 0.05) were prolonged and ejection time ([250.02±24.21] ms vs [272.73±20.51] ms, P < 0.05) was shortened in patients with ASD compared with that in control subjects, resulting in a marked increase of the MPI (0.41±0.07 vs 0.31±0.05, P < 0.05) from normal values; and (3) after transcatheter closure, the RVEDV and RVESV decreased and the RVEF increased markedly and RVMPI decreased markedly. Conclusions: Transcatheter closure of ASD results in rapid normalization of RV volume overload and improvement of RV function. (ECHOCARDIOGRAPHY, Volume 26, November 2009)     

自制单向活瓣在室间隔缺损并重度肺动脉高压病人畸形矫治中的应用

目的探讨单向活瓣补片加房间隔开窗术对先心病合并重度肺动脉高压(PH)病人手术治疗的可行性和疗效。方法回顾性分析采用单向活瓣补片加房间隔开窗术对20例室间隔缺损伴重度PH病人进行手术矫正的疗效和随访观察结果。结果术后早期7例病人肺动脉压明显下降,未出现跨活瓣片的分流;13例病人术后7d出现分流。死亡2例(1例死于心力衰竭,1例因上消化道出血死于术后13d),18例病例康复出院。术后出现分流的13例中,8例随访1个月~48个月,3个月仍出现跨活瓣片分流的4例,右向左分流3例,双向分流1例。术后6个月以上仍存在经活瓣片分流的1例,肺动脉收缩压(PP)/主动脉收缩压(PS)>0.75。7例术后未出现跨活瓣片分流者,术后3个月复查肺动脉压下降满意。结论单向活瓣补片允许术后特定情况下右向左分流,有效减轻右心室负荷,有利于早期右心功能恢复,达到保持体肺循环相对平衡,提高术后生存率。     

基因突变在先天性房间隔缺损和室间隔缺损发育形成中的作用研究

对于先天性房间隔缺损、室间隔缺损通过外科和介入手术治疗可大大提高患者生存率,但对其发育畸形的根本原因仍属于探索阶段。目前现有的研究表明,先天性心脏病是由于控制人体心脏发育的基因在时间(发育阶段)和空间(组织特异性)上的表达调控失误而引起的。现即从现有的基因水平研究对基因突变在先天性房间隔缺损和室间隔缺损发育形成中的作用研究作一综述,为从基因方面早期筛检、诊断、治疗先天性房间隔缺损、室间隔缺损提供参考。     

Mitral Stenosis, Sinus Venosus Atrial Septal Defect, and Partial Anomalous Pulmonary Venous Return

We describe a 42-year-old man with rheumatic mitral stenosis, sinus venosus atrial septal defect, and anomalous drainage of the right upper pulmonary vein to the superior vena cava. Transthoracic echocardiography (TTE) failed to identify the atrial septal defect and the partial anomalous pulmonary venous return. Transesophageal echocardiography (TEE), using a multiplane probe, was useful in delineating the abnormalities. To our knowledge, this is the first reported patient with rheumatic mitral stenosis and sinus venosus defect.     

Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7‐year‐old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.     

Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect

Background: Left-to-right shunt in sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance–PVR) and elastic (pulmonary artery compliance-PAC) pulmonary artery properties. This study aimed at evaluating (1) impact of age, (2) pulmonary hemodynamics, and (3) outcome in a large cohort of SVSD patients. Methods: This study included 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) of which 87 underwent catheterization. Pressures were measured and cardiac output was evaluated using the Fick principle at diagnosis. PVR, PAC and their product (RC time) were calculated. Results: Surgical repair was performed in 128 (94%) at a median age of 13 (IQR 5– 43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients died, 13 (10%) developed heart failure, 4 (3%) Eisenmenger syndrome, 19 (14%) atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker implantation. In those who underwent catheterization, median shunt ratio was 2.5 (IQR 2.0–2.9). Thirty (34%) had mean PA pressure ≥25 mmHg. PVR indexed, PAC indexed, and RC time was 3.5 (IQR 2.4–7.5) WU.m², 1.8 (IQR 1.3–2.5) mL/mmHg.m² and 0.39 (0.26–0.53) sec with an inverse hyperbolic relationship between PVR and PAC. Mean PA pressure (P < 0.0001); wedge pressure (P = 0.001), PVR indexed (P = 0.002) and PAC indexed (P = 0.002) changed significantly with age at diagnosis, but shunt ratio did not. Conclusion: SVSD has good long-term outcome, albeit with late morbidities. Thirty-four percent has mean PA pressure ≥25 mmHg, but Eisenmenger syndrome is rare (3%). PVR and PAC are inversely related and change significantly with older age.     

房间隔缺损封堵术后患者行心脏CTA检查的临床因素与价值

目的探讨房间隔缺损封堵术后有症状患者行心脏CT随访的临床因素及价值。方法2006年7月-2011年6月期间房间隔缺损封堵术后行CT检查的53名患者依年龄分为两组,组A,年龄兰40岁,组B〈40岁,分析患者行心脏CT检查的临床资料,包括患者年龄、临床症状、CT检查目的,CT检查结果等,进一步归纳患者行心脏CT检查的适应证及临床价值。结果共53例患者（男21,女32,平均年龄48．52,范围19—72岁）;房间隔缺损封堵术后患者行心脏CT随访的临味因素主要为胸闷、气短及心悸,患者均常规行心电图、经胸超声及X线平片复查,不能明确诊断,需进一步除外冠状动脉粥样硬化性心脏病（37／53）及肺动脉检塞（9／53）;少数病例旨在评估术后异常心前区不适（3／55）及射频消融前评估肺静脉及除外左房血栓等（4／53）。CT结果显示,全部患者均不同程度存在肺动脉扩张征象。此外,A组42例患者中,9例患者检出冠状动脉粥样硬化性心畦病;4例患者检查出并存其它先天性心血管畸形,其中冠状动脉起源异常2例,肺静脉异位引流、多发房间隔缺损各1例。B组11名患者中,3例评估术后心前区异常不适者,分别显示封堵器形态或位置异常。A、B两组虽阳性率无显著性差异（P-0．73）,但检查目的及结果不同。结论房间隔缺损封堵术后的临床症状须慎重对待,心脏CT检查能为不同年龄段的患者提供针对性的解剖学信息,有效弥补常规复查手段的不足。     

房间隔缺损封堵术后右心形态和功能的变化

目的 :评价继发孔房间隔缺损 (房缺 )封堵术后及随访中右心形态及右心室功能的变化。方法 :全组患者 36例 ,男性 11例 ,女性 2 5例 ,年龄 5～ 5 6 ( 2 7 2± 14 6 )岁。均用Amplatzer封堵器治疗。于封堵术前 1天、术后 2天、术后 3个月分别行超声心动图检查 ,右心室容积采用面积长度法计算。结果 :房缺封堵术后 2天及 3个月随访 ,右心房上下径、左右径 ,舒张末期右心室前后径、左右径均进行性改善 ,与术前比较 ,有显著差异 (P <0 0 5～ 0 0 1)。术后 2天 ,右心室的舒张末期容积 ,每搏输出量、射血分数均较术前明显改善 ,有显著性差异 (P <0 0 5 )。术后 3个月右心室的舒张末期容积、收缩末期容积、每搏输出量、射血分数较术前进一步改善 ,均有显著差异 (P <0 0 1)。结论 :房缺封堵术后及短期随访中 ,右心形态进行性改善 ,主要表现在右心房上下径、左右径及右心室舒张末期前后径、左右径进行性缩小 ;右心室的高动力循环状态术后虽明显减轻 ,但随访中右心功能无明显改变 ,保持了正常的右心功能     

Double‐Chambered Right Ventricle with Ventricular Septal Defect and Subaortic Membrane— Three‐Dimensional Echocardiographic Evaluation

Double‐chambered right ventricle (DCRV) is a rare congenital anomaly in which the right ventricle is divided into two compartments with varying pressures due to an anomalous muscle bundle. Here, we describe a case of an adolescent male with DCRV with associated ventricular septal defect and subaortic membrane. Two‐dimensional and three‐dimensional transthoracic echocardiography with color flow clearly outlined all the three cardiac anomalies as well as their relationship with each other. The diagnosis was confirmed by cardiac catheterization. The patient underwent successful surgical resection of the anomalous muscle bundle along with repair of the associated anomalies.     

Atrial Septal Defect in Children: The Incidence and Risk Factors for Diagnosis

Objective: Secundum atrial septal defect (ASD II) is a common congenital heart defect, and interatrial communications among preterm children is even more common. The objective of this study was to calculate the incidence of ASD II in children, with assessment to gestational age at birth. Further, to assess maternal, prenatal and postnatal risk factors associated with ASD II among children of different gestational age at birth. Design: This national registry based retrospective incidence study was supplemented with a national case-control study, using the Swedish Register of Congenial Heart Disease, Swedish Medical Birth Register and Statistics Sweden. All children, 0–18 years of age, born in Sweden and diagnosed with an ASD II between 2010 and 2015 were included in the study and compared with children without diagnosis of ASD II. Results: The yearly overall incidence of ASD II was 150 per 100 000 live births. However, this incidence ranged from 449 per 100 000 live births to 1737 per 100 000 live births, with higher incidence among preterm children. ASD II was associated with a presence of persistent ductus arteriosus; OR = 8.11 (Cl 95% 2.80–16.69), female gender; OR = 1.39 (Cl 95% 1.18–1.63) and being small for gestational age; OR = 1.86 (Cl 95% 1.29–2.68). Born preterm was also associated with ASD II; born at 32–36 gestational children; OR = 3.21 (Cl 95% 2.46–4.19), and born <32 gestational weeks; OR = 4.02 (Cl 95% 2.80–7.12). Conclusion: Preterm children have a higher incidence of ASD II than previously found, increasing with lower gestational age at birth. Preterm birth is an independent risk factor for ASD II diagnosis with three to four times, suggesting that this group of children may need new structured follow up program with careful assessment of indication when need of treatment and closure.     

Cobrahead Deformity in the Right Atrial Disc of a New-Generation Occlutech Figulla Flex II Atrial Septal Defect Occluder Device

Cobrahead deformity is a known (but uncommon) phenomenon associated with the left atrial disc of the Amplatzer or Occlutech Figulla septal occluder device during percutaneous transcatheter atrial septal defect closure. It has also been postulated that the right atrial disc of the Amplatzer septal occluder device might upon occasion exhibit the cobrahead malformation. To date, only one case report concerning the cobrahead deformity in the right atrial disc of an Amplatzer septal occluder has been published, if we discount a report published as a letter to the manufacturer.Here we present the first report (known to us) of a cobrahead deformity in the right atrial disc of an Occlutech Figulla Flex II atrial septal defect occluder device during transcatheter closure of a complex atrial septal defect.     

Cor Triatriatum Dexter and Atrial Septal Defect in a 43-Year-Old Woman

Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful.In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.