Efficacy of fenestrated atrial septal defect occulders on pulmonary hypertension dogs / 中华心血管病杂志

Objective: To explore the short-term efficacy of fenestrated atrial septal defect (ASD) occulders in the treatment of pulmonary arterial hypertension (PAH). Methods: Thirty-six healthy dogs were divided into the balloon atrial septostomy (BAS)+fenestrated ASD occulders group (n=12), BAS group (n=12) and non-septostomy group (n=12). PAH was induced by intra-atrial injection of dehydrogenized monocrotaline (1.5 mg/kg) in all dogs. Animals in the BAS+fenestrated ASD occulders group underwent atrial septal puncture and fenestrated ASD occulders implantation. Animals in the BAS group underwent balloon atrial septostomy. The non-septostomy group received no surgical intervention. The hemodynamic indexes and blood N-terminal pro-B-type natriuretic peptide (NT-proBNP) of dogs were measured before modeling, 2 months after modeling, 1, 3, and 6 months after surgery, respectively. Echocardiography was performed to observe the patency of the shunt and atrial septostomy of the dogs in the BAS+fenestrated ASD occulders group and BAS group at 1, 3, and 6 months after surgery. Three dogs were sacrificed in each group at 1, 3, and 6 months after surgery, respectively. Atrial septal tissue and fenestrated ASD occulders were removed to observe the patency and endothelialization of the device. Lung tissues were obtained for hematoxylin-eosin (HE) staining to observe the inflammatory cells infiltration and the thickening and narrowing of the pulmonary arterials. Results: Among 36 dogs, 2 dogs died within 24 hours after modeling, and 34 dogs were assigned to BAS+fenestrated ASD occulders group (n=12), BAS group (n=11), and non-septostomy group (n=11). Compared with BAS group, the average right atrial pressure (mRAP) and NT-proBNP of dogs in the BAS+fenestrated ASD occulders group were significantly reduced at 3 months after surgery (P<0.05), and the cardiac output (CO) was significantly increased at 6 months after surgery, arterial oxygen saturation (SaO2) was also significantly reduced (P<0.05). Compared with non-septostomy group, dogs in the BAS+fenestrated ASD occulders group had significantly lower mRAP and NT-proBNP at 1, 3, and 6 months after surgery (P<0.05), and higher CO and lower SaO2 at 6 months after surgery (P<0.05). Compared with the non-septostomy group, the dogs in the BAS group had significantly lower mRAP and NT-proBNP at 1 month after surgery (P<0.05), and there was no significant difference on mRAP and NT-proBNP at 3 and 6 months after surgery (P>0.05). Echocardiography showed that there was a minimal right-to-left shunt in the atrial septum in the BAS group at 1 month after the surgery, and the ostomy was closed in all the dogs in the BAS group at 3 months after the surgery. There was still a clear right-to-left shunt in the dogs of BAS+fenestrated ASD occulders group. The shunt was well formed and satisfactory endothelialization was observed at 1, 3 and 6 months after surgery. The results of HE staining showed that the pulmonary arterials were significantly thickened, stenosis and collapse occurred in the non-septostomy group. Pulmonary microvascular stenosis and inflammatory cell infiltration in the pulmonary arterials were observed in the non-septostomy group. Pulmonary arterial histological results were comparable between BAS+fenestrated ASD occulders group and non-septostomy group at 6 months after surgery . Conclusions: The fenestrated ASD occulder has the advantage of maintaining the open fistula hole for a longer time compared with simple balloon dilation. The fenestrated ASD occulder can improve cardiac function, and it is safe and feasible to treat PAH in this animal model.

Hipertrofia lipomatosa del septum interatrial: reporte de un caso / Lipomatous hypertrophy of the interatrial septum. A case report

Resumen La hipertrofia lipomatosa del septum interauricular (HLSI) es una entidad cardiaca rara y benigna, que se caracteriza por la acumulación de tejido adiposo dentro de algunos segmentos del septum interatrial. Generalmente, los pacientes son asintomáticos y estas lesiones se descubren de manera incidental mediante estudios de imagen realizados por otras razones, o en el contexto de una autopsia. Se han descrito casos de muerte súbita por alteración del ritmo cardiaco en estos pacientes. El diagnóstico diferencial de la HLSI incluye principalmente tumores cardiacos. Se expone el caso de un paciente de 61 años que, después de un estudio de resonancia magnética cardiaca, realizado por una alteración del ritmo cardiaco, presenta una masa en el septum auricular. El paciente es llevado a cirugía y el estudio histopatológico de la lesión confirma el diagnóstico. Se realiza una revisión de las características clínicas y patológicas de la HLSI.

Abstract Lipomatous Hypertrophy of the Interatrial Septum (LHIS) is a rare and benign cardiac entity that is characterized by the accumulation of adipose tissue within some segments of the interatrial septum. Patients are generally asymptomatic, and these lesions are discovered incidentally by imaging studies performed for other reasons, or in the context of an autopsy. In these patients, there have been described cases of sudden death due to disturbance of the heart rhythm. The differential diagnosis of LHIS mainly includes cardiac tumors. Here we present a case of a 61-year-old patient in whom, after a cardiac magnetic resonance study performed for an abnormal heart rhythm, it was documented a mass in the atrial septum. The patient was taken to surgery, and the histopathological study of the lesion confirmed the diagnosis. We conduct a review of the clinical and pathological characteristics of LHIS.

Cierre de la comunicación interauricular por cateterismo intervencionista con dispositivo Amplatzer en pediatría / Atrial Septal defect closure by interventional catheterization with Amplatzer device in Pediatrics

Introducción: El cateterismo intervencionista corrige las cardiopatías complejas, donde el dispositivo Amplatzer es el más usado y está disponible en Cuba. Objetivo: Evaluar el cateterismo intervencionista con Amplatzer, en el cierre de la comunicación interauricular ostium secundum. Métodos: Estudio de evaluación, longitudinal y prospectivo en 92 pacientes. El cierre de la comunicación por cateterismo con Amplatzer, se realizó en el Cardiocentro Pediátrico William Soler (2010-2016). Se utilizaron variables demográficas, ecocardiográficas (transtorácicas y transesofágicas) antes, durante y al año del cateterismo. Se observaron las complicaciones. La evaluación clínica y ecocardiográfica se realizó al año poscateterismo. Los pacientes a quienes se les realizó el proceder en el 2016, se evaluaron a los 6 meses. Resultados: Se encontraron diferencias significativas en el sexo (p<0,05): femenino 64,1 por ciento con homogeneidad para la edad (p= 0,244): media 9,8 años ( 5 desviaciones estándar. Precateterismo: 28,3 por ciento eran desnutridos y 17,4 por ciento delgados; poscateterismo la desnutrición mejoró significativamente (p= 0,000): desnutridos 9,8 por ciento y delgados 8,7 por ciento. El diámetro medio del defecto por ecocardiografía transcateterismo fue significativo (p= 0,000). Transcateterismo: 98,9 por ciento con cierre de defecto, 16,3 por ciento con insuficiencia tricúspidea y 4,3 por ciento con insuficiencia mitral. Poscateterismo: 98,9 por ciento con Amplatzer bien colocado. Hubo complicaciones inmediatas en 8,7 por ciento (arritmias, derrame pericárdico y embolización). Al año se detectó cortocircuito residual pequeño (n= 1) e insuficiencias valvulares auriculoventriculares (n= 3). La evolución fue satisfactoria (n= 91). Conclusiones: La evolución del cierre de la comunicación interauricular ostium secundum con dispositivo Amplatzer es satisfactoria, con mejoras de la desnutrición(AU)

Introduction: The interventional catheterization corrects complex heart diseases, and the Amplatzer device is the most widely used and is available in Cuba. Objective: To assess the interventional catheterization with Amplatzer device in the ostium secundum atrial septal closure. Methods: Longitudinal and prospective evaluation study in 92 patients. The closure of the communication by Amplatzer catheterization was performed in the William Soler Pediatric Cardiocenter of (2010-2016). Demographic and echocardiographic (transthoracic and transesophageal) variables were used before, during, and at the year of the catheterization. Complications were observed. Clinical and echocardiographic assessment was made a year after catheterism. The patients who underwent the procedure in 2016 were evaluated at 6 months. Results: Significant differences were found in the sex (p< 0.05): Female 64.1 t percent with homogeneity of the age (p= 0.244): average 9.8 years ( 5 standard deviations. Pre-catheterism: 28.3 percent were malnourished and 17.4 percent thin; post-catheterism: malnutrition improved significantly (p= 0.000): 9.8 percent malnourished and 8.7 percent thin. The average diameter of the defect by transcatheter echocardiography was significant (p= 0.000). Transcatheter: 98.9 percent : with default closure, 16.3 percent with tricuspid insufficiency and 4.3 percent with mitral regurgitation. Post-catheterism: 98.9 percent with Amplatzer device correctly positioned. There were immediate complications in 8.7 percent (arrhythmia, pericardial effusion, and embolization). A year after, small residual short circuit was detected (n= 1), and atrioventricular valve regurgitation (n= 3). The evolution was satisfactory (n= 91). Conclusions: The evolution of ostium secundum atrial septal defect´s closure with Amplatzer device is satisfactory and improves malnutrition(AU)

Early Diagnosis of KBG Syndrome Using Diagnostic Exome Sequencing / 대한소아신경학회지

KBG syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, skeletal anomalies, short stature, craniofacial dysmorphism, and macrodontia. ANKRD11 gene mutation and 16q24.3 microdeletion have been reported to cause KBG syndrome. Here, we report two patients with ANKRD11 mutations who initially presented with neurologic symptoms such as developmental delay and seizures. Patient 1 was a 23-month-old boy who presented with a global developmental delay. Language delay was the most dominant feature. He had hypertelorism, hearing impairment, and behavior problems characterized as hyperactivity. A c.1903_1907delAAACA (p.Lys635GInfsTer26) mutation in ANKRD11 was identified with diagnostic exome sequencing. Patient 2 was a 14-month-old boy with developmental delay and seizure. He also had atrial septum defect, and ventricular septal defect. Generalized tonic seizures began at the age of 8 months. Electroencephalography showed generalized sharp and slow wave pattern. Seizures did not respond to antiepileptic drugs. A loss of function mutation c.5350_5351delTC (p.ser1784HisfsTer12) in ANKRD11 was identified with diagnostic exome sequencing. In both cases, characteristic features of KBG syndrome such as short stature or macrodontia, were absent, and they visited the hospital due to neurological symptoms. These findings suggest that more patients with mild phenotypes of KBG syndrome are being recognized with advances in diagnostic exome sequencing genetic technologies.

Posição do implante de cabo-eletrodo atrial: diferenças em complicações cirúrgicas / A trial lead implant position: differences in surgical complications

Com o advento dos cabos-eletrodos de fixação ativa, diferentes locais para estimulação atrial estão sendo empregados. Em decorrência da impossibilidade de posicionamento no apêndice atrial direito ou então para buscar locais com melhor perfil de ativação atrial, diversos sítios de estimulação, como parede livre do átrio direito, septo interatrial e região do feixe de Bachmann, estão sendo estudados, com resultados variados. Apesar do grande número de possibilidades, ainda são escassas as informações que apontem se as diversas localizações para fixação do cabo-eletrodo atrial estão associadas a diferentes taxas de complicação. O objetivo do presente estudo é promover uma revisão da literatura disponível a respeito da correlação entre as diferentes posições do cabo-eletrodo atrial e as implicações cirúrgicas. DESCRITORES: Marcapasso Artificial; Eletrodos Implantados

With the advent of active fixation leads, different atrial pacing sites have been used. Because it is impossible to position them in the right atrial appendage or to search for sites with a better atrial activation profile, different alternative pacing sites such as the free wall of the right atrium, the interatrial septum and Bachmann's bundle region have been studied with varying results. Despite the large number of possibilities, data supporting if the atrial lead position is associated with different complication rates are scarce. The objective of this study is to promote a review of the literature available on the correlation between the different positions of the atrial lead and the surgical implications

¿Estamos cerrando muchos forámenes ovales permeables en Colombia? Foramen ovale permeable, aneurisma del septum interauricular y riesgo de ataque cerebrovascular criptogénico recurrente. Revisión sistemática de la literatura / Are many patent foramen ovale closure procedures being performed in Colombia? Patent foramen ovale, atrial septal aneurysm and the risk of recurring cryptogenic stroke. Systematic review of literature

Resumen Introducción: el foramen ovale permeable y el aneurisma del septum interauricular, son hallazgos frecuentes en la población general. En los últimos años múltiples estudios han sugerido la asociación entre estas entidades y el accidente cerebrovascular, de origen criptogénico como consecuencia de embolia paradójica, en especial en la población menor de 55 años, que ha estimulado el desarrollo de dispositivos de cierre percutáneo para este tipo de defectos, con el objetivo de reducir la recurrencia de eventos isquémicos cerebrales, sin que se hayan podido demostrar claramente estos beneficios. Objetivo: realizar una revisión sistemática de la literatura para evaluar el riesgo de recurrencia y beneficio de esta intervención. Resultados: Se obtuvieron 1.208 artículos para evaluación, con un total de 11 artículos incluidos para el análisis posterior a la selección, evaluación y aplicación de criterios de inclusión y exclusión, encontrando 3 ensayos clínicos y 8 revisiones sistemáticas y/o metaanálisis. Conclusiones: En pacientes adultos con ataque cerebrovascular isquémico o accidente isquémico transitorio de origen criptogénico a quienes se les ha documentado mediante eco-cardiografía transesofágica la presencia de foramen oval permeable, no existe beneficio en la reducción del riesgo de recurrencia de ataque cerebrovascular, accidente isquémico transitorio o eventos neurológicos, en comparación con la terapia médica convencional.

Abstract Introduction: Patent foramen ovale and atrial septal aneurysm are frequent findings in the average population. Over the last years multiple studies have suggested there is an association between both entities and cerebrovascular accidents of cryptogenic origin as a consequence of paradoxical embolism, specially among population under age 55, which has stimulated the development of devices for percutaneous closure of these defects with the goal of reducing recurring cerebral ischemic events, without a clear demonstration of their efficacy. Motivation: To perform a systematic review of literature to assess the risk of recurrence and benefits of this surgery. Results: 1,208 articles were collected for this evaluation, with a total of 11 papers included in the analysis following selection, assessment and application of selection criteria. 3 of them were clinical trial and 8 were systematic reviews or meta-analysis. Conclusions: In adult patients with cryptogenic cerebrovascular ischemic or transient ischemic attacks with patent foramen ovale documented via transthoracic echocardiogram no decrease has been found in the risk of recurrence of cerebrovascular attacks, transient ischemic accidents or neurological events in comparison with traditional medical therapy

Accidente vascular de circulación posterior en pacientes con foramen oval permeable y/o aneurisma del septo auricular: dos casos clínicos y una puesta al día / Posterior circulation stroke patients with patent foramen oval and/or atrial septal aneurysm: Presentation of two cases and an update

3% of the cerebrovascular accidents (CVA) compromise the cerebellum. The area most commonly affected in unilateral cerebellar stroke is the posterior inferior cerebellar artery (PICA). Atherosclerotic disease is the first cause, however, in patients with patent foramen ovale (PFO), it is important to consider a paradoxical embolism (PE) within the possibilities. Two cases of patients with posterior cerebellar stroke associated with PFO territory and inter atrial septal aneurysm (ASA) and a review of the literature is presented. Clinical cases: 87 year old woman with a history of stroke in 2012, PFO and ASA. Presented with dizziness, gait abnormality and vomiting. CT shows ischemic brain injury PICA territory. The echocardiogram evidenced ASA + FOP. Holter rhythm analysis evidenced a paroxysmal atrial fibrillation. 72-year-old man presented with dizziness, blurred vision and postural instability. TC showed ischemic brain injury in the territory of PICA and superior cerebellar artery (SCA). Echocardiogram evidenced ASA and PFO. The rest of the study embolic source was negative. Conclusión: There is an association between PFO and cryptogenic stroke. The most studied etiological factor is the paradoxical embolism, which could affect the posterior territory, as seen in the two clinical cases exposed. The best treatment option is notyet clarified and depends on the clinical context of each patient.

Un 3% de los accidentes vasculares encefálicos (ACV) son de cerebelo. El territorio más comúnmente afectado en ACV cerebelosos unilaterales es el de la arteria cerebelosa postero-inferior (PICA). La enfermedad ateroesclerótica es la primera causa, sin embargo, en pacientes con foramen oval permeable (FOP), es importante considerar una embolia paradojal (EP) dentro de las posibilidades. Se presentarán dos casos clínicos de pacientes con ACV cerebeloso de territorio posterior asociado a FOP y aneurisma del septum inter atrial (ASA) y una revisión de la literatura. Casos clínicos: Mujer de 87 años con antecedentes mórbidos de ACV isquémico el 2012, FOP y ASA. Consulta por cuadro de vértigo, alteración de la marcha y vómitos. TC de cerebro muestra lesión isquémica en territorio PICA. Al ecocardiograma se confirma el antecedente. El resto de los estudios de fuente embólica resultaron negativos, sólo destaca en Holter de ritmo fibrilación auricular paroxística. Al alta con anticoagulación y rehabilitación. Hombre de 72 años, hipertenso. Consulta por 24 h de evolución de vértigo, visión borrosa e inestabilidad postural. TC de cerebro muestra lesiones isquémicas en territorio de PICA y de arteria cerebelosa superior (SCA) a izquierda. Al ecocardiograma se evidencia ASA y FOP. Resto del estudio de fuente embólica resulta negativo. Al alta con tratamiento anticoagulante y cierre percutáneo de defecto en forma ambulatoria. Conclusión: Existe una asociación entre FOP y ACV criptogénico. El factor etiológico más estudiado es la embolia paradojal, que podría afectar al territorio posterior a través de la circulación vertebral, tal como se aprecia en los dos casos clínicos expuestos. La mejor opción de tratamiento aún no está aclarada y dependerá del contexto clínico de cada paciente.

A case of left atria subendocardial thrombus with sick sinus syndrome / 中南大学学报(医学版)

The clinical data for a patient with sick sinus syndrome was retrospectively analyzed. The patient was treated because of his heart palpitations and the increased chest pain. The patient admitted to the hospital under consideration for the left atrial tumor dependent on the echocardiography findings. After the CT scan and the dynamic ECG examination, the patient successfully underwent the left atrial tumor resection, atrial septal repair and cardiac pacing lead installation. The postoperative pathological diagnosis showed that the infective endocarditis and left atrial thrombus in left atrium was cured. The patient was discharged after postoperative anti-inflammatory therapy. By analyzing the reasons for misdignosis before or during surgery, the possible mechanisms for left atrial subendocardial thrombus have been found. This study suggests that it is necessary to combine imaging diagnosis and clinical observations to distinguish tumor from excrescence.

Atrial and ventricular septal changes in ethanol vapour exposed chick embryos

To study the effects of ethanol vapour exposure on development of atrial and ventricular septa of chick embryo. The experimental study was conducted at the College of Physicians and Surgeons, Islamabad, from 2006 to 2007. The experimental and control groups were further divided into three subgroups based on the day of sacrifice. The experimental group was exposed to ethanol vapours produced in a specially-designed vapour chamber and then compared with age-matched controls. There were 90 eggs in each of the two groups. The development of inter-ventricular septum completed at day 7 of development in chick embryo. Ethanol vapour exposure produced a small discontinuity at day 10 of development in a chick embryo which may be labelled as ventricular septal defect since ventricular development is completed by day7. Interatrial septum formed till day 7 with small perforations which persisted till hatching. Ethanol vapour exposure may lead to ventricular septal defect

Value of the Ratio of Occluder Versus Atrial Septal Length for Predicting Arrhythmia Occurrence after Transcatheter Closure in Children with Ostium Secundum Atrial Septal Defect / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Transcatheter occlusion has been applied to treat ostium secundum atrial septal defect (OS ASD) since 1997. During the clinical practice, several postoperative complications including arrhythmia have been reported. This study aimed to evaluate the value of the ratio of atrial septal occluder (ASO) versus atrial septal length (ASL) for predicting arrhythmia occurrence after transcatheter closure in children with OS ASD.</p><p><b>METHODS</b>Six hundred and fifty-one children diagnosed with OS ASD underwent occlusion procedures after completing routine examinations. The onsets and types of arrhythmia both during and after the occlusion procedures were monitored. Treatments were given based on the individual types of arrhythmia. The binary logistic regression analysis and receiver-operating characteristic (ROC) curve were used in the analysis of value of the ratio of ASO/ASL for predicting postoperative arrhythmia occurrence.</p><p><b>RESULTS</b>Transcather occlusions were conducted in 651 children, among whom 7 children had different types and degrees of arrhythmia, with an incidence of 1.1%. The types of arrhythmia included sinus bradycardia, atrial premature beats, bundle branch block, and different degrees of atrioventricular block. Normal electrocardiograph findings were resumed in these 7 patients following active therapies such as corticoids, nutrition, and surgeries. The binary logistic regression and ROC analysis suggested that the ratio of ASO/ASL exhibited an intermediate predictive value for predicting arrhythmia occurrence after occlusion procedures. A cut-off value of 0.576 in the ratio provided a sensitivity of 87.5% and a specificity of 76.2% with an area under the ROC curve of 0.791 (95% confidence intervals, 0.655-0.926; P < 0.05) in predicting arrhythmia occurrence after the closure procedures.</p><p><b>CONCLUSIONS</b>The ratio of ASO/ASL might be a useful index for predicting arrhythmia occurrence after closure procedures in children with OS ASD.</p>

Surgical Planning by 3D Printing for Primary Cardiac Schwannoma Resection

We report herein a case of benign cardiac schwannoma in the interatrial septum. A 42-year-old woman was transferred from a clinic because of cardiomegaly as determined by chest X-ray. A transthoracic echocardiography and chest computed tomography examination revealed a huge mass in the pericardium compressing the right atrium, superior vena cava (SVC), left atrium, and superior pulmonary vein. To confirm that the tumor originated from either heart or mediastinum, cine magnetic resonance imaging was performed, but the result was not conclusive. To facilitate surgical planning, we used 3D printing. Using a printed heart model, we decided that tumor resection under cardiopulmonary bypass (CPB) through sternotomy would be technically feasible. At surgery, a huge tumor in the interatrial septum was confirmed. By incision on the atrial roof between the aorta and SVC, tumor enucleation was performed successfully under CPB. Pathology revealed benign schwannoma. The patient was discharged without complication. 3D printing of the heart and tumor was found to be helpful when deciding optimal surgical approach.

Interatrial septal thickness as a marker of structural and functional remodeling of the left atrium in patients with atrial fibrillation

BACKGROUND/AIMS: There have been reports that interatrial septal (IAS) thickness is increased in patients with atrial fibrillation (AF). This study was performed to investigate whether IAS thickness determined by transthoracic echocardiography (TTE) represents the amount of left atrium (LA) structural and functional remodeling. METHODS: The study population consisted of 104 consecutive patients who underwent catheter ablation (CA) for AF (paroxysmal atrial fibrillation [PAF], 82; persistent atrial fibrillation [PeAF], 22). IAS thickness and left atrium volume (LAV) using TTE, and LA voltage (LAVOL) using 3-dimensional electroanatomical mapping system were assessed during sinus rhythm. RESULTS: IAS thickness was significantly correlated with maximal LAV (LAVmax) (r = 0.288, p = 0.003), mean LAVOL (r = -0.537, p or = 2) compared to other groups according to CHA2DS2-VASc score (p = 0.019). During a follow-up of 19.6 months, 23 subjects (22.1%; PAF, 17; PeAF, 6) had recurrence of arrhythmia. Univariate analysis showed that LAVmax, minimal LAV, mean LAVOL, LVEFtotal, LVEFactive, and IAS thickness were associated with recurrence of arrhythmia. However, on multivariate analysis, only mean LAVOL and LAEFactive were independent risk factors for recurrence. CONCLUSIONS: Although IAS thickness showed significant correlations with parameters for LA structural and functional remodeling, this parameter alone could not independently predict recurrence of arrhythmia after CA for AF.