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ObjectiveTo evaluate the frequency of and risk factors for stroke as a presenting feature in adult patients with secundum atrial septal defect (ASD); rates of post-closure atrial fibrillation (AF) and stroke were also assessed.MethodsWe retrospectively reviewed adult patients who presented with an ASD between 2002 and 2018, excluding those with known atrial arrhythmias. Risk factors for stroke were identified using multivariable logistic regression. Post-closure stroke was evaluated using survival analysis stratified by the presence of post-procedure AF.ResultsOf 346 patients with ASD (median age 44 years), 34 (10%) presented with a history of stroke. Independent risk factors included elevated body mass index over 25 (OR: 18.2; 95% CI: 4.0 to 82.2; pConclusionsIn this study population, the incidence of stroke prior to ASD closure among patients without atrial arrhythmias was 10%. Risk factors included obesity, smoking and prominent Eustachian valve anatomy. Lifestyle changes should be recommended for at-risk patients, and it may be reasonable to consider ASD closure in the absence of haemodynamic indications in patients at increased risk of stroke.

We present the case of a 34-year-old woman with a rare constellation of congenital cardiovascular defects. She presented with new-onset dyspnoea on exertion and intermittent palpitations. Extensive cardiac workup revealed anomalous drainage of the inferior vena cava and a large secundum atrial septal defect. She underwent surgical repair. Intraoperatively, an additional congenital venous anomaly was discovered, a partial anomalous pulmonary venous connection. Specifically, the right inferior pulmonary vein connected to the inferior vena cava, and together, they drained into the left atrium. On conducting a thorough literature review, we believe this is the first reported case in an adult female.

Objective Secundum atrial septal defect (ASD) is a common congenital heart defect. There is limited data on both early and late atrioventricular (AV) block post ASD closure. The aim of this study was to determine the incidence and risk factors of AV block associated with ASD closure. Methods A retrospective analysis of all patients who underwent ASD closure either with a device or surgical method at the Royal Children9s Hospital Melbourne between 1996 and 2010 was performed. Baseline demographics, procedural details and follow-up data were collected from medical records. Results A total of 378 patients were identified; 242 in the device group and 136 in the surgical group. Fourteen patients (3.7%) had AV block (1 with second degree and 13 with first degree) at a median follow-up of 28 months; 11/242 (4.5%) in the device group and 3/135 (2.2%) in the surgical group (p=0.39). Six patients had new-onset AV block after ASD closure. In the device subgroup, patients with AV block at follow-up had a larger indexed device size compared with those without (22 (15–31) vs 18(7–38), p=0.02). Multivariate analysis revealed the presence of AV block either pre procedure or post procedure to be the only variables associated with late AV block. Conclusions Late AV block in patients with repaired ASD is rare and most likely independent of the technique used. In the device subgroup, the only risk factor identified to be associated with late AV block was the presence of either preprocedural or postprocedural AV block, so long-term follow-up for these patients should be provided.

Horseshoe lung is a rare congenital anomaly and mostly accompanied by scimitar syndrome. Most aspects of this complex anomaly can be demonstrated via multidetector CT (MDCT). We present two baby girls who had horseshoe lung associated with right lung hypoplasia and scimitar vein. The chest roentgenograms showed displacement of the heart and mediastinum to the right with smaller right lung. Echocardiography revealed dextroposition, secundum atrial septal defect and bilateral slight peripheral pulmonary stenosis in the first case and dextroposition, severe pulmonary hypertension, secundum atrial septal defect and tricuspid regurgitation in the other one. On thoracic MDCT, the right lung and pulmonary artery were hypoplastic with cardiomediastinal shift to the right. There was an abnormal right pulmonary vein draining into the inferior vena cava on the lower zone of the right lung (scimitar vein). The posterobasal portions of the both lungs were fused through a midline isthmus behind the heart.

Adult patients with atrial septal defects (ASDs) have variable clinical presentations that can range from dyspnoea on exertion to cerebral vascular accidents from paradoxical embolism. Currently echocardiography is the mainstay in the diagnosis and follow-up of patients with ASDs. As CT technology advances and low radiation techniques improve, cardiac CT is becoming a desirable method to evaluate patients with ASDs when echocardiographic evaluation is limited. Correct diagnosis and appropriate management of patients with patent foramen ovales (PFOs) and ASDs relies on an understanding of the embryologic development patterns that resulted in the malformation and associated anomalies. In this review, we illustrate the development of the interatrial septum and the foramen ovale. We discuss the diagnosis of PFOs and ASDs by echocardiography and highlight the incremental benefit of CT to further elucidate defect morphology, associated anomalies, and anatomy post-repair. The primary atrium starts as a common cavity.1 Starting at 5 weeks of gestation, the primordial single atrium begins dividing into right and left sides by formation and fusion of two septa: the septum primum and septum secundum (figure 1). Early in the fifth week of gestation the septum primum begins to form near the centre of the roof of the common atrium, growing downward to the endocardial cushions, and partially dividing the common atrium into right and left counterparts (figure 1, A-1 and A-2). The gap between the septum primum and endocardial cushions is known as the ostium primum. Before the septum primum and endocardial cushions fuse, perforations develop within the cephalic portion of the septum primum through apoptosis, creating a large window known as the ostium secundum. At the end of the sixth week the caudal free edge of the septum primum reaches the atrioventricular cushions and fuses with them, closing the ostium primum (figure 1, B-1 and …

The authors present the case of a 41-year-old woman, who presented with moderate pericardial effusion and bilateral pleural effusion 11 months following a secundum atrial septal defect closure with an Amplatzer septal occluder (ASO) device. The acute presentation responded well to diuretics and a short course of corticosteroid therapy. The patient, however, continued to experience pleuritic chest pain and a recurrence of pericardial effusion a month later. The patient showed significant symptomatic improvement with a 2-month course of down-titrating prednisolone. Six months later, the echocardiogram showed complete remission of pleural and pericardial effusion. The possibility of cardiac erosion following ASO implantation has been excluded. The aetiology of the reoccurrence of steroid-responsive pleuropericarditis remains unknown.

The puzzling title “stroke and migraine: a cardiologists’ headache” conjures up a potential common cause: a cardiac right-to-left shunt through a patent foramen ovale (PFO). In the 16th century the Italian surgeon Botallo first described the PFO and in 1877 the suspicion of a PFO mediated stroke was published by the German pathologist Cohnheim. Since then the prevalence of PFO in adults has been established by autopsy studies (20–35%),1 2 transthoracic echocardiography (7–24%),3 4 or by the more sensitive transoesophageal echocardiography (20–40%).5–7 The first major clinical paper associating PFO and stroke appeared in 19883 and the first reporting percutaneous closure in 1992.8 Migraine came into play rather accidentally when migraineurs were subjected to transcranial Doppler examinations and showed an increased prevalence of right-to-left shunt,9 and when a pronounced decrease in migraine symptoms was found in a series of patients undergoing atrial shunt closure for other reasons.10 All that remains to do now is to get the facts straight and rid our patients of such strokes and headaches—a task complex enough to constitute (hopefully transiently) a cardiologist’s headache. The PFO is an essential feature of intrauterine life (fig 1). Until birth and inception of breathing and pulmonary gas exchange, the PFO acts like an atrial septal defect (ASD), stays permanently open, and allows the right atrium receiving oxygenised blood from the placenta to unload directly into the left atrium. The flow through the pulmonary circulation is minimal due to the high flow resistance in the still collapsed lungs. With the first breath, the pulmonary resistance markedly drops and hence the right atrial pressure falls below the left atrial pressure. This moves the flail septum primum against the robust septum secundum, forming the cranial limbus of the fossa ovalis. Normally the two septa …

Objectives: To examine the outcomes in patients with moderate or severe pulmonary arterial hypertension (PAH) undergoing percutaneous atrial septal defect (ASD) closure. Design: Retrospective study. Setting: Teaching hospital-based study. Patients: Fifty-four patients with moderate (n = 34) or severe PAH (n = 20) who underwent successful device implantation between 1999 and 2004 were included in the study. Clinical and transthoracic echocardiographic data were reviewed. Pulmonary hypertension was classified as moderate (50–59 mm Hg) or severe (⩾60 mm Hg) according with the right ventricular systolic pressure (RVSP) calculated by echocardiography. Results: At the early follow-up (mean (SD) 2.3 (1.2) months) all patients were alive and the baseline RVSP decreased from 57 (11) mm Hg to 51 (17) mm Hg (p = 0.003). At the late follow-up (n = 39, mean (SD) duration 31 (15) months) two patients had died and the baseline RVSP decreased from 58 (10) mm Hg to 44 (16) mm Hg (p = 0.004). Although the overall mean RVSP decreased at late follow-up, only 43.6% (17/39) of patients had normalisation ( Conclusion: Transcatheter closure in patients with secundum ASD and PAH can be successfully performed in selected subjects and is associated with good outcomes. Early improvements in RVSP are seen in patients with moderate or severe PAH undergoing transcatheter ASD closure. Continued improvement in RVSP occurs in late follow-up. Despite decreases in the mean RVSP in late follow-up, many patients do not have complete normalisation of pressures.

Objective: To determine by modern echocardiographic techniques the prevalence and development of cardiac abnormalities associated with ventricular septal defect (VSD). Methods: Consecutive patients referred to a tertiary centre for paediatric cardiology and attenders at an adult congenital heart disease clinic had details of clinical outcome prospectively recorded. Patients with VSD in association with conotruncal abnormalities, atrioventricular septal defects, and univentricular heart were not included in the study. Results: 1448 patients with VSD were assessed between 1991 and 1998. 1127 (78%) patients had isolated defects; of these 862 (76%) were perimembranous and 265 (24%) were muscular. Of the remainder, 284 (19.6% of the total population) patients had VSD associated with one other significant cardiac abnormality: 35 (2.4%) with two and two (0.1%) with three other abnormalities. The most common associated cardiac abnormalities were infundibular pulmonary stenosis (5.8%), aortic valve prolapse (3.6%), pulmonary valvar stenosis (2.7%), osteum secundum atrial septal defect (2.2%), persistent ductus arteriosus (1.9%), and coarctation of the aorta (1.5%). In the cohort of 743 patients followed up from birth, cumulative mortality was 4% by the age of 8 years and most deaths occurred within the first year of life in infants with associated genetic abnormalities such as trisomy 13 or 18. Of the 594 patients attending the adult congenital clinic with VSD, aortic regurgitation due to aortic root prolapse developed in 45 (7.6%) patients. Most of these cases were detected before the patient was 30 years old. Conclusions: In 22% of patients with congenital VSD there were significant associated cardiac abnormalities. Some of these abnormalities, such as aortic prolapse and regurgitation or infundibular pulmonary stenosis, may develop or progress subsequently and therefore should be sought during the initial assessment and monitored during follow up. Follow up to the age of 30 years allows the detection of most cases of aortic regurgitation. The prognosis from VSD is excellent and the risk of endocarditis in a population informed of the need for antibiotic prophylaxis is small.

Objective: To compare surgical and device closure of isolated secundum atrial septal defect (ASD) in terms of hospital costs, clinical outcome, and impact on the patient and family. Design: Prospective, observational study. Setting: Paediatric tertiary referral centre. Patients: Consecutive local children with a secundum ASD, admitted between 1 May 1999 and 1 May 2001. Methods: Parents completed a standardised questionnaire at recruitment (on admission), at discharge, and one month after the procedure. Clinical and hospital generated cost data were collated at discharge. Results: 62 children were included in the analysis: 19 who underwent surgical repair and 43 who underwent device closure with the Amplatzer septal occluder. Median procedure times and hospital stay were significantly longer for surgical patients (170 (147 to 180) v 92 (70 to 115) minutes and 88 (78 to 112) v 29 (28 to 30) hours, respectively; p < 0.01). There was no difference in the complication rate. No device patients required intensive care or blood products. The median values for postoperative pain score, analgesia use, and convalescence time were greater for surgical patients. The median cost of each procedure was similar, but higher nursing and laboratory costs contributed to a slightly greater total cost for surgical repair (Aus$12 969 ($11 569 to $14 215) v Aus$11 845 ($10 669 to $12 555), p = 0.03). Conclusions: Device closure of ASD involves a shorter hospital stay, causes less discomfort and familial disturbance, and carries less cost than surgical closure. However, there should be guarded acceptance of this technique until long term data are available.

Acute promyelocytic leukemia was diagnosed in a 28-year-old pregnant woman at 13 gestational weeks. She was immediately started on idarubicin and all-trans-retinoic acid (ATRA) and achieved remission after her fourth cycle of treatment. Serial fetal ultrasonograms throughout pregnancy did not reveal any intrauterine growth retardation or other obvious malformations. The mother delivered a term (36.7 gestational weeks), 2720-gram female neonate. The infant was admitted to the intermediate care nursery for observation due to transient mild respiratory distress during the peripartum period. Because of right ventricular hypertrophy on an electrocardiogram, an echocardiogram was performed on the first day of life which showed moderate dilation of the right atrium and right ventricle with mildly depressed function, two small secundum atrial septal defects, and a small patent ductus arteriosus. The neonate remained hemodynamically stable and no arrhythmias were detected. The remainder of the hospital course was uneventful. When reassessed 1-1/2 months later, she was doing well and did not show any signs of congestive heart failure. A repeat echocardiogram at that time demonstrated complete resolution of the right heart enlargement and closure of the ductus arteriosus with persistence of the small and hemodynamically insignificant secundum atrial septal defects.

To investigate the natural history of secundum atrial septal defects (ASDs) over several years using serial echocardiographic studies.All patients with isolated secundum ASDs who had serial transthoracic echocardiograms at Texas Children's Hospital, Houston, Texas, from January 1991 to December 1998 were identified. Patients with fenestrated or multiple ASDs, other congenital heart defects, or less than a six month interval between echocardiograms were excluded. There were 104 patients eligible for inclusion in the study. Studies were reviewed by two echocardiographers (blinded) and the maximal diameter was recorded. Defects were defined as small (3 mm to6 mm), moderate (or = 6 mm to12 mm), or large (or = 12 mm). ASDs that grewor = 20 mm were defined as having outgrown transcatheter closure with the device available to the authors' institution.ASD diameter increased in 68 of 104 patients (65%), including 31 patients (30%) with a50% increase in diameter. Spontaneous closure occurred in four patients (4%). Thirteen defects (12%) increased toor = 20 mm. One fifth of the patients studied had an insufficient atrial rim by transthoracic echocardiogram to hold an atrial septal occluder. The only factor associated with significant growth of ASDs was initial size of the defect. ASD growth was independent both of age at diagnosis and when indexed to body surface area.Two thirds of secundum ASDs may enlarge with time and there is the potential for secundum ASDs to outgrow transcatheter closure with specific devices. Further development in devices and general availability of devices capable of closing larger ASDs should circumvent this problem.

OBJECTIVE—To define by three dimensional echocardiography the pattern and potential determinants of contraction of a secundum atrial septal defect through the cardiac cycle, and to evaluate the possibility of using cross sectional transthoracic and transoesophageal imaging to assess the dynamic nature of the defect. DESIGN—Three dimensional echocardiography was performed using a multiplane transoesophageal probe on 50 patients with a secundum atrial septal defect (median age 9.8 years). Nine patients were excluded because of poor images or morphological features that precluded defect measurement. In 41 cases, defect area, long and short axis length, and distance of the attenuated anterior rim were measured in their largest and smallest dimensions. RESULTS—Defect area changed significantly through the cardiac cycle (mean change 61%, p

Catheter-based closure is now considered the first-line treatment strategy for secundum atrial septal defects (ASD). Although in some cases, surgery remains the only option (eg, for those with an insufficient rim to secure a device, or those with coincidental anomalous pulmonary venous return), a percutaneous approach is suitable for the vast majority of patients with secundum ASD. As skill and experience grow, defects as large as 40 mm in size have been successfully closed in this manner. In the days when repairing any ASD required a thoracotomy, cardiopulmonary bypass and 4 days or more in hospital, the benefits from treatment had to markedly overcome the deleterious effects of surgery, including the effects on the right ventricle of opening the pericardium and going on bypass. With less invasive treatments available, the question to be asked is—just because we can, should we be closing them all? The evidence seems to be clear for symptomatic patients with large shunts, but what of those with no symptoms, or in those with established pulmonary arterial hypertension (PAH)? Transcatheter closure of an ASD is not a procedure without risk. Two large published series from the early days of catheter closure demonstrated complication rates from 4 to 8%.1 2 Other available datasets show a more realistic major complication rate of 1.6%3; however, this is still not a zero-risk procedure, and there is a steep learning curve for the operator.4 Complications include device embolisation, cardiac perforation, device thrombus, stroke, arrhythmia and serious vascular injury. These types of complication, together with device failure, may require conversion to open surgery either immediately, or at a later date.5–9 Recent data suggest that late valve problems may also occur.10 Suitability of the patient for at least 3 months of combined antiplatelet therapy must also …

Clinical introduction A young man attended the adult congenital heart disease clinic for routine follow-up. He had a diagnosis of transposition of the great arteries (TGA) and underwent a Senning procedure at 10 months of age. He had remained well over the intervening years, was fully saturated, with a normal exercise tolerance, no cardiovascular symptoms and no history of arrhythmia or symptoms suggesting thromboembolic events. Transthoracic echocardiogram is shown in figure 1A and cardiac MRI image in figure 1B. Question Which of the following is the most likely diagnosis? Intact atrial pathways Secundum atrial septal defect Baffle leak Primum atrial septal defect Baffle obstruction

Background Trisomy 21 is frequently associated with congenital heart disease (CHD). Survival and life expectancy has increased as a result of surgical intervention and medical therapies. The NORPAP GUCH database, started in 1993, has 2668 patients. Methodology Patients with Trisomy 21 were selected from the NORPAP database. Population demographics, underlying diagnosis, comorbidities and mortality were analysed. Results 110 patients had T21; Mean 34 years, Median 30 years, M:F 1.1:1 (Figure 1). Underlying CHD was AV Canal defect 57 (51.8%), VSD 30 (27.3%), Fallot Repair 10 (9.1%), Secundum ASD 6 (5.5%), Bicuspid Aortic Valve 3 (2.7%), Mitral Regurgitation 1 (0.9%), Single Ventricle 1 (0.9%), Pulmonary Stenosis 1 (0.9%), PDA 1 (0.9%). 61 (55%) patients had had surgery. Of those under 40, 51% were unoperated. Death occurred in 9 patients (9%). Average age at death 46 years. 78% of deaths were in patients with AV Canal defect. Mode of death was cardiac in 8 and PAH-CHD was present in all but one. Figure 2 shows associated comorbidities. 35% have PAH -– 59% receivetargeted therapy. Conclusion AV Canal defect was the most frequent underlying CHD in the T21 NORPAP cohort and was associated with the highest mortality. 1/3 of all patients had PAH and 1/4 of these died during follow up. Survival into the 4th and 5th decade is frequent. Patients in the 3rd decade now may expect to survive for another 25–30 years. Ongoing cardiac and non-cardiac morbidity requires dedicated CHD clinics, frequently with PAH clinicians given that 50% of those under 40 have not had surgery and may need targeted therapy.

We report a case of a 60-year-old woman with a history of cyanotic heart disease who presented with a painless and palpable left-sided anterior neck mass. Clinical examination did not reveal any cranial nerve palsy. Soft systolic and diastolic murmurs were heard on auscultation and the patient also suffered from persistent shortness of breath (O2 saturation approximately 90%). Previously, transoesophageal echocardiography had demonstrated a large fenestrated secundum atrial septal defect with right-to-left shunting. There was no relevant family history. A contrast-enhanced neck CT scan revealed a highly vascular and well-circumscribed 2.2×2.0 cm …

Case Report The patient is a female with Turner’s Syndrome (45, XO) born at 32 weeks gestation by emergency caesarean section with a birth weight of 1.6KG. The neonatal course was complicated by respiratory distress, hypoglycemia, coagulopathy, gram negative sepsis and small bowel perforation. Ongoing clinical issues included transaminitis, conjugated hyperbilirubinemia and bilateral grade 4 intra-ventricular haemorrhages. The patient was transferred from a neonatal intensive care unit to our institutions paediatric intensive care unit where her cardiorespiratory profile continued to deteriorate. She was started on noradrenaline, milrinone and intravenous diuresis to support cardiac output and ameliorate left sided pulmonary oedema. 2D echocardiography demonstrated PAPVC of the left upper pulmonary vein to the innominate vein as well as a small secundum ASD, mild right heart dilatation, moderate RVH and well preserved biventricular systolic function. There was moderate PR and mild TR with an estimated right ventricular systolic pressure approaching systemic levels. CT angiogram confirmed a left PAPVC to innominate vein via a vertical vein and suggested only a small segment of the left lower lobe was draining to the left atrium. The CT also raised the possibility of a vertical vein stenosis. At eight weeks corrected the patient presented for cardiac cathederisation under GA. Pulmonary artery pressure was systemic on manometry. The mean pressure in the vertical vein measured 24mmHg with a 9mmHg gradient across the stenosis. A stent was placed across the stenosis and inflated to 22 ATM. Relief of the stenosis was achieved and the procedure was tolerated without complication.

To the Editor, I read with interest the review presented by Asrress et al in your series ‘Education in Heart’.1 I was disappointed to find figure 1 representing a spurious concept of development. Although the concept is to be found in many textbooks of cardiac embryology, it is not necessarily correct. Asrress et al go part way to explaining its deficiency when they state “Infolding of the atrial wall on the right aspect of the septum primum produces the septum secundum”. This mechanism of infolding was described by Rose as long ago as the end of the 19th century.2 The problem with the …

Background Objective of this study to discover the relationship between secondary atrial septal defect (ASD) and pulmonary arterial pressure (PAP) in children less than 5 years old. Methods Patients whose ages were less than 5 years old were admitted and underwent percutaneous occlusion of ASD with occluder in general anaesthesia with ketamine in General Hospital of Shenyang Military Command from April, 2000 to January, 2011. The patients were divided into different groups according to size of the ASD and PAH. Results 189 patients were concluded, male was 77 cases (40.7 per cent), female was 112 (59.3 per cent), ratio was 1:1.5, mean ages were 4.1 ± 0.9 years old (range from 2 to 5 years), mean weights were 17.2 ± 3.6 kilograms (range from 10.0 to 30.0 kg), mean highs were 104.9 ± 9.2 cm (range from 77 to 135 cm), mean body surface areas (BSA) were 0.71 ± 0.10 m 2 (range from 0.46 to 1.02 m 2 ), mean sizes of ASD were 12.6 ± 4.8 mm (range from 5 to 29 mm), mean sizes of ASD modified by BSA were 18.0 ± 7.0 mm/ m 2 (range from 5.3 to 38.9 mm/ m 2 ). Mean systolic PAPs were 41.1 ± 8.9 mmHg (range from 15 to 67 mmHg), mean diastolic PAPs were 16.8 ± 6.5 mmHg (range from 3 to 45 mmHg), mean PAPs were 24.9 ± 6.7 mmHg (range from 12 to 48 mmHg). 159 patients (89.4%) had pulmonary arterial hypertension measured by right heart catheterization (RHC), but no patient had PAH by echocardiography. PAPs had no difference in different size ASDs (p >0.05). Conclusions There was no relationship between the sizes of ASD and PAP in children less than 5 years old. PAH maybe been not caused by shunts through ASD.

A 26-year-old man presented with progressively worsening dyspnoea on exertion since the past 6 months. He was referred to our centre with the initial diagnosis of secundum type atrial septal defect and tricuspid regurgitation (see online appendix 1) based on a transthoracic echocardiography. On physical examination, he had a systolic murmur at the left sternal border with inspiratory augmentation. Electrocardiography showed normal sinus rhythm with right bundle branch block (figure 1). Chest x-ray was suggestive of right chamber enlargement with reduced pulmonary blood flow (figure 2). Figure 1 Figure 2 Transthoracic and transoesophageal echocardiograms revealed a normal-sized left ventricle with preserved systolic function, huge right atrium, severe right ventricular (RV) enlargement with moderate …

Aims To evaluate the efficacy and safety of transcatheter closure of very large secundum atrial septal defects (ASD) with long-term follow-up results. Methods and results From June, 2001 to June, 2008, 74 patients diagnosed as secundum atrial septal defect with a diameter more than 30 mm were enrolled in the study. Percutaneous closure of ASDs were attempted by using the Domestic Shanghai ASD-O guided by transthoracic echocardiography (TTE). For the group of the 74 patients, 68 of them are adults. The closure was successful in 71/74 patients (96%). The mean diameter of the device was 38.2±2.73 mm. During the follow-up, complete closure could be achieved in 100% of patients. 7 adverse events (9.9%) were reported. Most adverse events were categorised as minor and there was one major adverse events (1.4%). Conclusion Transcatheter device closure is an effective management option for patients with very large ASD. Serious complications were rare.

A 36-year-old Hispanic woman was referred for an asymptomatic cardiac murmur and auscultation revealed wide and fixed second heart sound. Transthoracic echocardiogram showed severely dilated right atrium and ventricle. Transesophageal echocardiogram (TEE) confirmed left-to-right shunt through secundum atrial septal defect (ASD) (1.5 cm×2.0 cm×2.5 cm), with Qp:Qs 2:1 on Doppler studies (figure 1). In view of large ASD size and inadequate superior rim of tissue, the patient was planned for surgical …

Objectives In our clinical practice, we try to find a feasible method to size the hole of ASD by only 2D transthoracic echocardiography (2D-TTE). It should be more practical, less expensive and without pain. Methods Sixty-seven consecutive adult patients (26 males, 41 females, mean age 38.4 ± 8.5 years) were scheduled to have ASD device closure. Before that, we calculated the size of ASD by 2D transthoracic echocardiography (2D-TTE) through parasternal four chambers view, parasternal short axes view, apical four chambers view, subcostal four chambers view and subcostal two chambers view. The biggest size from the views would be chosen for ASD device closure. Meanwhile, we paid attention to the ASD margins, and we divided them to floppy margin group (n = 24) and firm margin group (n = 43). For the floppy margin patients, we would add 2 or 4 mm more than the 2D-TTE size for preparing ASD device size. And for the firm margin patient, we chose the same 2D-TTE size for ASD device size. Results The total successful ASD device closure rate was 94.0% (63/67). The average total trans-catheter ASD closure time was 42.3 ± 12.5 minutes. ASD device closure succeeded at the first time was: 40/43 (93.0%) in the firm margin group; 16/24 (66.7%) in the floppy margin group. For the rest patient whose ASD couldn’t be closed at the first time, we reduced the size 2–5 mm in the firm margin group, the rest 3 all got successful ASD device closed. As in the floppy margin group, there were 4 patients needed adding 4–6 mm to the 2D-TTE size and then their ASD device closed successfully. There were only 4 patients (6%) in floppy margin group couldn’t be ASD device closed. For average 1.4 years follow up, only 6 patients (9.5%) had some chest pain complain, but all the devices were stable. Conclusions With our experience, the sizing based on 2D-TTE could be used for ASD device selection. The multiple views of TTE should be used to calculate the biggest size of ASD. And the margins of the ASD hole also need to be considered.

Background Three dimensional transoesophageal echocardiography (3D TOE) has allowed better understanding of atrial septal anatomy and patent foramen ovale (PFO) morphology. Anatomical variations seen in patients undergoing transcatheter device closure may influence device choice and procedural success. Currently there is no standardised approach to 3D TOE imaging of a PFO. The aim of this study was to utilise a 3D imaging protocol to describe/quantify PFO morphological features of clinically important PFO9s and relate our findings to shunt size. Methods We examined 3D TOE images of the atrial septum in consecutive patients with otherwise structurally normal hearts undergoing TOE assessment prior to PFO closure. Datasets were obtained using Philips iE33 and then analysed offline (QLab 3DQ, Philips Healthcare). Linear measurements recorded included: tunnel dimensions, fossa ovalis dimensions, and septal sway. Other characteristics assessed included left atrial opening morphology, right atrial anatomy and presence of an atrial septal aneurysm (>10 mm septal excursion from the midline). Morphological features were categorised as either simple or complex. Complex defects were those with one or more of the following: long tunnel length (>8 mm), multiple openings into the left atrium, atrial septal aneurysm, hybrid defect (additional small defect(s) in fossa ovalis), thick secundum septum (>8 mm), Eustachian ridge (ER), Eustachian valve or Chiari network. The size of right-to-left shunt either at rest or with Valslava manoeuvre was assessed by transthoracic contrast echocardiography and graded according to the number of bubbles seen in a single still frame in the left atrium as: small-to-moderate ( 50 bubbles). Results 3D datasets were available for analysis in 73 patients. Mean age of the population was 47±12.7 years. and 62% were male. The most frequent indication for PFO closure was stroke (48%). Linear dimensions recorded from 3D datasets are presented (table). Left atrial opening was categorised as high, opening onto the left atrial roof, in 18% of patients and presence of multiple left atrial openings was observed in 8%. Atrial septal aneurysm was present in 12%. A prominent Eustachean ridge was seen in 78% patients and was positioned high on fossa ovalis in 32% cases. The majority of patients (88%) had one or more complex morphological features. There was no clear association observed between shunt size and size of PFO. Conclusions 3D transoesophageal echocardiography allows a practical, qualitative, and quantitative assessment of PFO morphology. In our cohort of patients a variety of complex anatomical features are demonstrated. Shunt size did not correlate with PFO dimensions.

A 6 year old boy is found to have a fixed split second heart sound and a systolic murmur heard best at the left upper sternal border on routine physical examination. The presence of a secundum atrial septal defect is confirmed by echocardiography. The attending cardiologist recommends closure via transcatheter device placement. The studious paediatric resident remembers reading in the most recent edition of the Nelson textbook of pediatrics that open heart surgery is also an acceptable option. Knowing that the consultants in her department think that EBM stands only for expressed breast milk, the resident decides to search the recent medical …

Objective To evaluate the clinical efficacy of interventional therapy for patients with ostium secundum atrial septal defect (ASD). Methods One hundred and fifty patients (male-90, female-60), age at 2–71 (12±5) years old. Diagnosis as ostium secundum defect by transthoracic echocardiography (TTE), measuring different facets of ASD, the maximal diameters were 4–36(20±6)mm. Single-hole-type-ASD were 145 cases, porous-type were five cases. Meanwhile, combined with PDA were two cases, with PS was one case. Guiding by transthoracic ultrasound, we implanted a domestic Amplatzer-occluder (Beijing Huayi shengjie). After operation, patients were regularly follow-up by cardiac ultrasound and clinical examination. Results One hundred and fifty cases were all successfully blocked, the occluder diameters were at 6–40 (23±2) mm. Intraoperative, the systolic pressure of pulmonary artery were at 25–65 (30±4)mm Hg. Three cases of porous-type ASD, we used a single occluder; another two cases we each used two occlusion devices to block two defect holes. Two cases with PDA and one case with PS all completed the intervention therapy. Intraoperative, there was no residual shunt. Following-up from 1 month to 5 years, there were no occluder shedding, no complications of thrombosis and endocarditis. Atrio-ventricular sises were reduced to different degrees. Conclusion The interventional treatment for patients with ostium secundum defect was convenient, minimally invasive, safe and high success rate, affirmative curative effect, and could be the first treatment measures for secundum ostium secundum atrial septal defect (ASD).

Objective To observe the feasibility, safety and efficacy of interventional treatment in patients of secundum atrial septal defect (ASD) with atrial septal aneurysm (ASA). Methods 189 patients of ASD with ASA (82 males and 107 females, average age (30.2±15.8) years, range 10–68 years old) were selected by the clinical, ECG, x-ray, transthoracic electrocardiogram (TEE) examination from January 2003 to April 2011 in our hospital. Diameter of ASD were 4–38 (22±8) mm measured by TTE maximum from different facets, 178 patients with single-hole type, porous type in 11 cases, Amptatzer occluders were used under the guidance of the TTE, followed up for 12 months. Results 189 patients were occluded successfully, occluder diameter of 8–42 (24±4) mm, 8 cases with porous type were selected just a occluder, 3 cases with 2 defect holes occluded successfully by two occluders, no significant residual shunt and occluder loss immediately and followed up for 12 months, size of atrioventricular reduced. Conclusion Occluder used in patients of secundum atrial septal defect with atrial septal aneurysm is feasible, safe and efficient.

Background Both Transcatheter device closure (TCDC) or intraoperative device closure (IODC) have emerged as new minimally invasive alternatives to the classic surgery in the treatment of secundum atrial septal defect (ASD). However, the long-term safety and efficacy by the two methods for closuring large particularly huge ASD remains uncertain. Present study sought to investigate and compare the long-term clinical outcomes by using TC DC and IODC for closure of large-to-huge ASD. Methods A total 92 patients with large-to-huge ASD (a defect diameter of ≥30 mm), treated from January 2003 to Dec 2009, were included in this study. All the patients have been followed up until Dec 2010. The patients were assigned to either TCDC or IODC group according to the patients′ or their parents′ preference. Baseline physical exams, chest roentgenography, electrocardiography, and echocardiography were performed pre-procedure and at each follow-up visit. Results 42 patients underwent TCDC and 50 patients received IODC by using Amplatzer septal occluder (ASO). The average diameter of ASD was similar in the two groups (35.2±4.8 mm vs 34.9±4.4 mm, p>0.05). The immediate procedural success rate was 92.9% for TCDC and 98.0% for IODC (p=0.328). The peri-procedural complication rate was 9.5% for TCDC and 28.0% for IODC p Conclusions Present study confirmed the long-term safety and efficacy by using ASO for closure of large-to-huge ASD either by TCDC or IODC. However, the acute complication rate was lower and the length of hospital stay was shorter by TCDC.

There are few topics in adult congenital heart disease as persistently debated as the management of atrial septal defects (ASDs). The paper by Jemielity and colleagues in this issue of Heart reactivates these fires.1Their retrospective study includes 88 largely symptomatic patients who received surgical closure of secundum and sinus venosus ASDs. Their age range at surgery was 40–62 years, with a mean of 46 years. They were followed for 1–17 years, mean 6.9 years. There was only one operative death. Functional class improved strikingly from 62% in New York Heart Association (NYHA) functional class III and IV preoperatively to 82% in NYHA class I and II postoperatively. Pulmonary vascularity improved on x ray, and right ventricular size diminished as assessed by echocardiography. Atrial flutter and fibrillation continued to be troublesome. This study adds additional evidence to the proposition that closure of secundum and sinus venosus ASDs benefits many or most symptomatic patients over age 40 years. The “Canadian recommendations” state that the mere presence of a “significant ASD” (one associated with right ventricular volume overload, attributable exercise limitation, atrial arrhythmias, and/or late right heart failure) “warrants intervention”.2 As Ward described in 1993, the clinical trials on this issue and on which we base our management decisions are generally of poor quality by modern standards.3 More recent publications have not changed our dilemma. The available data do not have enough scientific merit to enable me or anyone else to …

Two women at the age of 62 and 42 years underwent secundum type atrial septal defect (ASD) closure with the recently introduced ATR I ASEPT II device (Cardia Inc, Eagan, Minnesota, USA). ASD closure devices of size 24 mm and 20 mm were implanted without complications. Intracardiac echocardiography demonstrated satisfactory device deployment. The first patient developed atrial fibrillation approximately 6 weeks after the procedure and therefore …

A 34 year old woman presented with increasing shortness of breath, and a secundum atrial septal defect (ASD) was diagnosed by transthoracic echocardiography. A preoperative transoesophageal echocardiogram demonstrated a large secundum ASD with left to right shunt, and a dilated right ventricle (RV) with a pulmonary artery systolic pressure …

Twelve cases of endocardial cushion defect were studied before and after operation with ultrasono-cardiotomography (tomography) cross-sectional echocardiography, two-dimensional echocardiography, B-scan echocardiography) and M-mode scan along a horizontal section of the heart. For comparison, 20 healthy subjects, 18 cases of mitral valvular disease, 4 cases of congestive cardiomyopathy, 1 case of partial anomalous pulmonary venous drainage, and 25 cases of atrial septal defect of secundum type were also examined with the same technique. In cases without cardiac malformation, the echo of the anterior mitral valve was usually continuous medially with that of the interatrial septum in the horizontal plane at the level of the membranous septum. This feature was clearly recorded in all cases with right heart enlargement. In ostium secundum atrial septal defect the echo of the anterior mitral valve continued into that of the interatrial septum. An echo interruption was shown, indicating the defect itself to be in the middle part of the interatrial septum. In all the cases of endocardial cushion defect which we examined discontinuity was shown between the echo of the anterior mitral valve and that of the interatrial septum. This discontinuity was interpreted as indicating the defect itself. The mitral valve ring echo was close to the basal end of that of the interventricular septum, possibly reflecting an abnormal attachment of the mitral valve. In all cases, after operation, the echo of the artificial interatrial septum was recorded, continuous with that of the anterior mitral valve. The features of the echocardiographic sweep from the anterior mitral valve to the interatrial septum were thus different in the three groups. These echocardiographic differences are thought to correspond to the anatomical differences between the normal, atrial septal defect of secundum type, and endocardial cushion defect, and are essential features differentiating them from each other.

Abnormalities of the mitral valve in patients with pulmonary stenosis, tetralogy of Fallot, and pulmonary hypertension with right ventricular pressure overload were studied by real time cross sectional echocardiography. Dislocation of the anterior and posterior mitral leaflets at the coaptation zone in systole was present in 16 of 46 cases: nine of 11 (82%) cases of pulmonary hypertension, four of 20 (20%) cases of tetralogy of Fallot, and three of 15 (20%) cases of pulmonary stenosis. The incidence was highest in patients with pulmonary hypertension. In eight of the 16 patients with mitral valve lesions, mitral regurgitation was seen on left ventriculograms or cross sectional Doppler echocardiograms. The dislocation was located near the posteromedial commissure of the anterior mitral leaflet in all cases. These findings are similar to the mitral valve abnormalities seen in patients with secundum atrial septal defect, and therefore may be due to a common cause. No relation could be found between the left ventricular deformity index and the incidence of dislocation of the mitral leaflets. Thus, the reason why this mitral valve abnormality occurs in conditions with right ventricular pressure overload could not be established.

Abnormal systolic interventricular septal motion is an echocardiographic manifestation of right ventricular volume overload. In order to determine the anatomical basis for this echocardiographic finding, septal motion recorded by left lateral or left anterior oblique ventriculograms was compared with echocardiography ventricular septal motion. Thirteen patients with secundum atrial septal defects and 7 control patients with trivial or no heart disease were included in the study. We found that on ventriculograms the systolic motion of the interventricular septum was similar in both atrial septal defect and control patients. That is, the cephalic third of the septum moved anteriorly in systole in 9 of the 13 patients with atrial septal defect and in 3 of the 7 controls. The caudal two-thirds of the septum moved posteriorly in all patients. These results are compatible with the theory that argues that the echocardiographic abnormalities of septal motion in patients with atrial septal defect result from an end-diastolic septal flattening or bowing of the septum into the cavity of the left ventricle.

A pair of monozygotic twin girls is reported with concordance for 3 congenital cardiac abnormalities: (1) secundum atrial septal defect, (2) aneurysm of the membraneous ventricular septum, and (3) electrocardiographic frontal plane left axis deviation. A review of the published materials shows a 9.5 per cent incidence of concordance for congenital heart disease among monozygotic twins. In those in whom a precise cardiological diagnosis was made, 15/16 pairs (95%) were concordant for a specific defect, 2 had an additional defect, and only 1 pair had completely dissimilar defects. Concordance for congenital heart disease in monozygotic twins is uncommon, but when it occurs the defects will most often be identical.

A prominent "v" wave relative to the "a" wave in the jugular vein and right atrial pressure tracing is considered to be a common haemodynamic sign of atrial septal defect. Since the prevalence, age relation, and haemodynamic determinants of the "v" greater than or equal to "a" wave configuration have not been studied the pressure recordings from 15 adults and 80 children with an isolated secundum atrial septal defect in sinus rhythm and from 40 adults and 55 children in sinus rhythm without structural cardiac abnormalities or with coronary and valvular heart disease were studied to assess the sensitivity and specificity of the "v" greater than or equal to "a" wave configuration in atrial septal defect. Only 20% of adults with an atrial septal defect had prominent right atrial "v" waves compared with 63% of children, although the specificity was quite high for each group. In adults "left atrialisation " of the right atrium ("v" greater than or equal to "a" wave) occurred in younger patients with higher right atrial and right ventricular end diastolic pressures. In contrast, in children no age related or haemodynamic determinants for the "v" greater than or equal to "a" pattern were found. In addition, most adults but few children with an atrial septal defect had "right atrialisation " of the left atrial wave configuration ("a" greater than "v"). This was found in older adults with lower right atrial and right ventricular end diastolic pressures and in older children with larger left to right shunts. Thus in contrast to children adults with an atrial septal defect rarely show a prominent "v" wave in the right atrium. The presence of a prominent right atrial "v" wave in adults with an atrial septal defect is associated with relatively higher left atrial and right heart pressures than is the absence of this sign and may be related to relatively higher systolic transatrial flow in these patients. The relative paucity of prominent right atrial "v" waves in older adults suggest that the systolic phase flow may diminish with age, possibly from progressive alteration in compliance of the chronically dilated right ventricle.

Twenty-six patients aged 45 or over at the time of surgical repair of a secundum atrial septal defect have been reviewed with the object of assessing the value of the operation. Pre-operatively 12 were classed as grade IIIA or worse on the basis of their symptoms. There were six deaths in the series, three early and three late. The remaining 20 patients were improved as judged by symptoms, physical examination, and radiographic and electrocardiographic criteria. In 11 patients catheterized pre- and post-operatively, it was found that the pulmonary arterial pressure had fallen following the repair, reaching normal levels in nine. It is concluded that surgical repair of secundum atrial septal defects is of material benefit to patients of this age-group.

Insomecasesofatrial septal defect ofthe secundum typethedefect isnotlocalized tothe areaoftheforaxnen ovale butalsoinvolves the dorsal andlower partoftheseptum inthevicinity oftheinferior venacava(Bedford, Sellors, Somerville, Belcher, andBesterman, 1957;Lewis, Varco, andTaufic, 1954). There isnolower margin tothe defect abovetheorifice oftheinferior venacava, whichtherefore appears tocommunicate directly withtheleft atrium aswellaswiththeright, and, associated withtheleft-to-right inter-atrial shunt, a small right-to-left shunt, fromtheinferior venacava totheleft atrium, maybefound. Fromthesurgical point ofviewitisimportant torecognize this type ofdefect because itsrepair maycausea severe complication. Anteriorly thedefect hasaninferior edgeofatrial septum which iscontinuous withthe valve oftheinferior venacava, situated tothe right oftheoriEfce. Ifthis valve iswrongly taken asthelower margin ofthedefect andusedwhen repairing thedefect, theinferior venacavawould then beconnected totheleft atrium andnottothe right. Suchanerrorhasbeenmadeinother clinics (personal communications) utilizing the closed technique ofatrio-septopexi ofBailey, andit hasoccurred inatleast five cases operated onunder hypothermia. Theerror hasalsobeenmadeina caseoperated uponusing extracorporeal circulation wherethetubedraining theinferior venacavawas inserted through thesaphenous vein. Diversion oftheblood fromtheinferior venacava into theleft atrium isaserious complication. The majority ofpatients inwhichthemistake hasnot beencorrected havediedonthetable. Inonecase operated on underhypothermia theerrorwas detected immediately onclosing theright atrium. Therefore, after ashort timeasecond inflow-occlu

The mitral valve in 15 of 16 adult hearts with unclosed secundum atrial septal defects showed a consistent abnormality consisting of thickening of the medial half of the anterior cusp and some fusion of adjacent chordae. Histologically the lesion is a surface fibrosis without vascularisation or myxomatous change to suggest a rheumatic or floppy valve. The high frequency and morphological appearances suggest abnormal cusp movement and resultant valve trauma rather than a primary valve abnormality. The functional abnormality leading to secondary valve changes is presumably related to increased flow and altered left ventricular geometry but cannot be studied in the dead heart.