Concomitant Atrial Septal Defect Research Articles

Morbidity Burden in Patients With Ebstein Anomaly: The Natural History.

The lifetime morbidity burden of patients with Ebstein anomaly (EA) has not been well described. Through an extensive 2-country register-based collaboration, patients diagnosed with EA who were born between 1930 and 2017 were identified in Danish and Swedish nationwide medical registries. Each patient was matched by age and sex with 10 control subjects from the general population. Cox proportional-hazards regression, Fine-Gray competing risk regression, and Kaplan-Meier failure function were used to estimate the morbidity burden. The study included 794 patients diagnosed with EA and 7940 controls, with a median follow-up period of 33 years. Among patients with EA, approximately half (n=442) had isolated EA, and 28% (n=218) had concomitant atrial septal defect. Patients with complex anatomy demonstrated the highest cardiovascular morbidity burden, followed by those with concomitant atrial septal defect and isolated EA. The lifetime cumulative incidence of supraventricular arrhythmia and ventricular preexcitation in patients with EA, with or without atrial septal defect, was approximately 70% and 19%, respectively. Supraventricular arrhythmia substantially increased the risk of ischemic stroke (hazard ratio [HR] 22.6 [95% CI, 11.1-45.9]). Presence of atrial septal defect significantly affected arrhythmia and heart failure burden compared with isolated EA. In the total cohort of patients with EA, supraventricular arrhythmia onset led to an immediate high incidence of heart failure, with a 10-year cumulative incidence of 18%. The natural history of EA, whether isolated or not, involves a substantial burden of cardiovascular morbidity and thus a highly vulnerable long-term prognosis.

Effect of concomitant atrial septal defect on left ventricular function in adult patients with unrepaired Ebstein's anomaly: a cardiovascular magnetic resonance imaging study

BackgroundDue to the heterogeneity of anatomic anomalies in Ebstein's anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes, which ultimately cause left ventricular (LV) deterioration remain unclear. The current study aimed to investigate the effect of concomitant ASD on LV function using cardiovascular magnetic resonance (CMR) imaging in patients with EA. MethodsThis study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls. Left/right ventricular (RV, the RV was defined as a summation of the functional RV and atrialized RV in EA patients) volumes and functional parameters, LV strain parameters, and echocardiogram indices were compared among the four groups. Associations between variables were evaluated via Spearman or Pearson correlation analyses. The association between risk factors and LV ejection fraction (EF) was determined via multivariate linear regression analysis. ResultsBoth EA patients and ASD patients had a higher RV/LV end-diastolic volume (RVEDV/LVEDV) as well as lower LV and RV ejection fractions (LVEF/RVEF) compared to healthy controls (all p < 0.05). Moreover, the EA patients with ASD had a significantly higher RVEDV/LVEDV and a lower LVEF and RVEF than those without ASD (all p < 0.05). Multivariate linear regression analysis revealed that the presence of ASD was independently associated with LVEF (β = − 0.337, p < 0.001). The RVEDV/LVEDV index was associated with LVEF (r = − 0.361, p < 0.001). Furthermore, the LV longitudinal peak diastolic strain rate (PDSR) was lower in EA patients with ASD than those without ASD, patients with simple ASD, and healthy controls (p < 0.05). ConclusionConcomitant ASD is an important risk factor of LV dysfunction in patients with EA, and diastolic dysfunction is likely the predominate mechanism related to LV dysfunction.

Mortality in patients with Ebstein anomaly: a two-country, population-based cohort study including 530 patients

Abstract Background Low birth prevalence and referral bias constitutes significant obstacles in extending our knowledge regarding the natural history of Ebstein anomaly (EA). Purpose In an extensive two-country register-based collaboration we aimed to investigate the survival in EA patients with respect to associated congenital cardiac malformations Methods Patients born in the period 1970–2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries and subsequently merged. Each EA patient was matched by birth year and gender with ten controls without congenital heart disease from the general population. Cox proportional hazard regression model and Kaplan-Meier survival function were used to compute risk of mortality (hazard ratio) and survival. Follow-up started at birth and patients became risk at time of EA diagnosis. Results We included 530 patients and 5,300 matched controls; median follow-up was 10.5 years (interquartile range: 3.5–20.9). In the total EA cohort, 43% (n=228) underwent cardiac surgery, predominantly EA-related surgery (81%). Approximately half of the cohort had an isolated EA (n=248), 11% (n=57) had a concomitant atrial septal defect only, and 42% (n=225) other associated congenital cardiac malformations. The 35-year survival in patients with isolated EA was 93%, superior compared with patients with concomitant atrial septal defect (ASD) (83%) and patients with other associated congenital cardiac malformations (72%), Figure 1. Patients with isolated non-operated EA had a risk of mortality of 25.6 (95% confidence interval (CI): 9.0–72.6) compared with the general population. Correspondingly, the risk in patients with surgically managed isolated EA was 21.7 (95% CI: 3.9–118.5). Patients with non-operated EA and a concomitant ASD (only) revealed a 10-fold increased risk of mortality (95% CI: 1.5–75.1) compared with the general population. The risk of mortality was substantially higher in both non-operated and operated EA patients with other associated congenital cardiac malformations, HR of 106.3 (95% CI: 31.8–335.4) and 42.6 (95% CI: 18.5–97.8), respectively. Conclusions Concomitant congenital cardiac malformations are common in EA patients and have major influence on survival, irrespective of treatment pathway (surgical versus non-surgical). Nonetheless, isolated non-operated EA patients have a 25-fold increased risk of mortality compared with the general population. Interestingly, concomitant ASD in non-operated patients seems protective in terms of risk of mortality. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Novo Nordisk Foundation.

Burden of morbidity in patients with Ebstein anomaly: a two-country, population-based cohort study including 823 patients

Abstract Background Burden of morbidity in patients with Ebstein anomaly (EA) is to a large extent undescribed. Purpose We conducted an extensive two-country register-based collaboration aiming to describe the burden of morbidity in patients with EA. Methods Patients born in the period 1930–2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries and subsequently merged. Each EA patient was matched by birth year and gender with ten controls without congenital heart disease from the general population. Cox proportional hazard regression model was used to compute risk (hazard ratio (HR)) of morbidity (arrhythmia, heart failure, and stroke). Follow-up started at birth. Patients and controls became at risk at birth. Results We included 823 patients and 8,230 matched controls; median follow-up was 13.2 years (interquartile range: 3.5–20.9). In the total EA cohort, 44% (n=366) underwent cardiac surgery, predominantly EA-related surgery (81%) and other congenital cardiac surgery (15%). Approximately half of the cohort had an isolated EA (n=442) and 13% (n=108) had a concomitant atrial septal defect (ASD) only. Patients with non-operated isolated EA had a 9-fold increased risk of arrhythmia and a 5-fold increased risk of heart failure compared with the general population [HR for arrhythmia 9.4 (95% CI: 6.9–12.7) and HR forheart failure 5.2 (95% CI: 3.2–8.3)]. The risk of arrhythmia, heart failure, and stroke among patients with non-operated EA and concomitant ASD was 12.1 (95% CI: 3.0–48.8), 30.6 (95% CI: 3.2–295.2), and 8.2 (95% CI: 0.7–91.2), respectively. The risk of stroke in operated EA patients with concomitant ASD was 43.8 (95% CI: 17.5–109.7). Patients with operated isolated EA had a 25-fold increased risk of developing arrhythmia and 10-fold increased risk of heart failure compared with the general population [HR for arrhythmia 25.0 (95% CI: 17.7–35.3) and HR for heart failure 10.1 (95% CI: 6.0–16.9]. Conclusion Patients with EA carries a substantial burden of arrhythmia, heart failure, and stroke compared with the general population irrespective of treatment pathway, necessitating meticulous follow-up in specialized adult congenital heart disease clinics to detect and possibly prevent or limit morbidity at an early stage. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Novo Nordic Foundation.

Surgical correction for scimitar syndrome by right thoracotomy and direct anastomosis in children.

ABSTRACTImportanceScimitar syndrome (SS) is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava. However, the surgical repair techniques for SS vary according to patients’ anatomical and pathological features.ObjectiveThis study was performed to analyze the mid‐term results of a less invasive surgical correction technique for SS in children.MethodsEleven patients with SS who underwent surgical repair from January 2012 to March 2020 were retrospectively analyzed. The anomalous scimitar vein (SV) was directly reimplanted to the left atrium, and the concomitant atrial septal defect was simultaneously repaired with cardiopulmonary bypass.ResultsThree male and eight female patients were included in the study. Their mean age was 3.1 ± 1.3 years, and their mean body weight was 12.8 ± 3.0 kg. Most patients had symptoms, such as upper respiratory tract infection, dyspnea, and recurrent pneumonia, and two patients had pulmonary hypertension. None of the 11 patients who underwent direct SV reimplantation by right thoracotomy developed bleeding, arrhythmia, heart failure, or perioperative death, and no patients required reoperation during a mean follow‐up period of 36.6 ± 15.2 months. Postoperative echocardiography revealed no restenosis or obstruction of the anastomosis in any patients.InterpretationSurgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective, with good long‐term patency of the reimplanted SV and a low mortality rate.

Greatness in failure: a look at Leonardo da Vinci and his drawings of the heart

Greatness in failure: a look at Leonardo da Vinci and his drawings of the heart

Atrial septal aneurysm mimicking cor triatriatum in a Cavalier King Charles dog

Atrial septal aneurysm is a rare deformity of interatrial septum extending to changeable directions during cardiac cycle. A 9 year old Cavalier King Charles Spaniel referred to Small Animal Hospital with a history of acute onset of respiratoric distress and abdominal distension. Routin blood work, electrocardiography and thorax radiography were performed. Echocardiography revealed mitral valve disease and severe left atrial dilatation with an atrial septal aneurysm as a bulging of interatrial septum into the right atrium and nearly prolapsing into the tricuspid orifice. Color flow Doppler revealed a left-to-right shunt flow atrial septal defect. The dog died suddenly 2 weeks later. The owner declined postmortem examination. To the best of authors’ knowledge, this was the first report of atrial septal aneurysm with concomitant atrial septal defect in a dog. The purpose of this case report is to point out atrial septal aneurysm as the possible complication of mitral valve disease in a Cavalier King Charles Spaniel

Partial PFO Closure for Persistent Hypoxemia in a Patient with Ebstein Anomaly.

Ebstein anomaly is characterized by deformities of the anterior leaflet of the tricuspid valve and atrialization of the right ventricle. Patients with severe tricuspid regurgitation are recommended to have tricuspid valve surgery with concomitant atrial septal defect closure. A 73-year-old female with Ebstein anomaly presented with severe hypoxemia. Transthoracic echocardiography revealed severe tricuspid regurgitation and a patent foramen ovale with right-to-left shunting. Complete percutaneous patent foramen ovale closure led to acute decompensation; however, partial closure led to hemodynamic stability and improved oxygenation. In conclusion, similar patients with “patent foramen ovale dependency” from longstanding shunts may benefit from partial patent foramen ovale closure.

Right ventricular enlargement in a patient with hereditary haemorrhagic telangiectasia: a rare case of pulmonary arteriovenous malformations with concomitant atrial septal defect

We describe a rare case (32-year-old woman) with hereditary haemorrhagic telangiectasia and concomitant atrial septal defect (ASD). Transthoracic saline contrast echocardiography (TTCE) revealed several microbubbles appearing early (third cardiac cycle) and significant microbubbles appearing later (seventh cardiac cycle) in the left atrium and ventricle (LA/LV) after opacification of the right atrium in the four-chamber view …

Rare Finding of Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum Presenting as Right Heart Failure and Pulmonary Hypertension in an Adult

INTRODUCTION: Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital defect that may not clinically manifest until adulthood. The increased pulmonary venous flow associated with PAPVC can eventually lead to dyspnea on exertion, atrial arrhythmias, pulmonary hypertension (PH), and right-sided heart failure (RHF). Most cases of PAPVC are associated with a concomitant atrial septal defect (ASD) and rarely present in isolation with an intact atrial septum. We report an unusual case of a patient presenting with PH and RHF who was found to have isolated PAPVC involving multiple right pulmonary veins with an intact atrial septum.

Post-traumatic fulminant paradoxical fat embolism syndrome in conjunction with asymptomatic atrial septal defect: a case report and review of the literature

IntroductionFat embolism syndrome with respiratory failure after intramedullary nailing of a femur fracture is a rare but serious complication in trauma patients.Case presentationWe present the case of a 20-year-old Caucasian man who experienced paradoxical cerebral fat embolism syndrome with fulminant progression after intramedullary nailing of a femur fracture, in conjunction with a clinically asymptomatic atrial septal defect in a high position resulting in a right-to-left shunt.ConclusionFat embolism syndrome may occur as a fulminant complication following femoral fracture repair in the presence of a concomitant atrial septal defect with right-to-left shunt. Thus, in patients with cardiac right-to-left shunts, femurs should not be nailed intramedullary, not even in cases of isolated injuries.

Late complication of total anomalous pulmonary venous drainage in an adult: ‘a diagnostic challenge’

Total anomalous venous drainage is a rare congenital cardiac defect seen in 1% of the population; there is usually a concomitant atrial septal defect. We discuss such a diagnostic challenge in a patient who presented on a number of occasions with acute respiratory distress and haemoptysis. True understanding of her underlying congenital heart disease was not appreciated until review in a congenital heart disease clinic - in which she presented for prepregnancy counselling. She was found to have a stricture at the site of anastomosis of the common chamber with the left atrium, resulting in pulmonary venous hypertension leading to acute presentation of pulmonary haemorrhages. She underwent successful full balloon dilatation of the site, leading to full recovery. Most patients are diagnosed at birth and undergo surgical repair. The outcome is good, and historically it has been regarded that it is unusual for them to have long-term complications, although dysrhythmias remain a recognized long-term complication. Surgery for total anomalous pulmonary venous drainage is nevertheless a palliation, and it is vital that the possible role of late complications is considered in all cases.