Atrial Defect Research Articles

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.

Acute pulmonary embolism with ST-segment elevation in the inferior leads

ST-segment elevation (STE) in the right precordial leads is a remark-able and relatively common electrocardiographic (ECG) sign of moderatetosevereacutepulmonaryembolism(APE) [1–4].Someofthesecasesareaccompanied by STE in the inferior leads [2]. However, it is an unusualphenomenon to detect STE con fined to the inferior leads (in the absenceof acute occlusive coronary artery) [5]. We present here a case of STEconfined to the inferior leads as the only ECG manifestation of APE anddiscuss the possible intrinsic mechanism.A79-year-oldmalewithoutpriorcardiovascularhistoryadmittedtotheemergencydepartmentduetodyspneaandchestdiscomfortforthelast two days.Hehad brain surgery 15 days prior to remove a meningi-oma. The ECG (Fig. 1A) before brain surgery was normal. At admission,blood pressure (BP) was 110/60 mm Hg. Next ECG (Fig. 1B) showednew S-wave in lead I, incomplete right bundle branch block (RBBB)and minor STE in leads aVR and V1–V2. Under the presumption ofacute coronary syndrome, an emergent coronary angiography wasperformed showing minor atherosclerosis in the left anterior and cir-cumflex coronary arteries. One day later, the dyspnea was aggravatedwithoxygendesaturationandBPdroppedto90/55mmHg.SubsequentECG (Fig. 1C) showed STE in the inferior leads, resolution of the STEin leads aVR and V1–V2, higher R′-wave in leads V1–V2 and widerQ-wave in lead III (in comparison to Fig. 1B). An echocardiogramshowedright ventricular (RV) enlargement, freewall hypokinesia,flat-ten of the interventricular septum, and severe tricuspid regurgitation.APE was highly suspected and a CT pulmonary artery angiographyconfirmedbilateralpulmonaryarterialthrombus.Thrombolytictherapywas initiated but patient's condition rapidly deteriorated into cardio-genic shock dying few hours later. The ECG (Fig. 1D) obtained duringcardiogenic shock showed STE in the inferior leads and reappearanceof STE in leads V1–V3. The last ECG (Fig. 1E) was recorded beforedeath showing STE in leads aVR, III and V1–V3, ST-segment depression(STD) in leads I and V5–V6, QR sign in lead V1 and negative T-wave inleads V1–V3.The mechanisms to explain STE in APE include the following:(i) concomitant occlusive coronary artery with/without paradoxicalcoronary artery embolism via atrial sepal defect or patent foramenovale [3], and (ii) RV transmural ischemia due to hypotension, hypox-emia, pulmonary artery hypertension and catecholamine surge [2,3].STE in the right precordial leads with or without STE in the inferiorleads is an important ischemic pattern of APE reflecting transmural RVischemia [2,6]. Our case and a case reported by Emren et al. [5] withSTE confined to the inferior leads presented without significant coro-nary artery disease. We speculate that STE confined to the inferiorleads may result from transmural RV ischemia. Fig. 1B clearly showedSTE in the right precordial leads, which is a typical ischemic pattern inAPE. However, when the STE occurred in the inferior leads as inFig. 1C, simultaneous resolution of STE in the right precordial leadswas observed. In inferior STE myocardial infarction, STD can be foundin the right precordial leads and may be an electrical mirror phenome-non [7]. Hence, we speculate that STE in the inferior leads can counter-actSTEintherightprecordialleads.STEconfinedtotheinferiorleadsinAPE is a response to a speci fic degree of RV transmural ischemia. Whenthetransmuralischemiadegreeincreases,STEmayreappearintherightprecordial leads. Just as in Fig. 1D and E, when the patient's conditiondeteriorated, STE depicted in the inferior and right precordial leads.Our group previously described three specific ECG patterns in APE,indicating different manifestations of myocardial ischemia: (i) STE inleadaVR withconcomitantSTDinleadsIandV4toV6(LVsubendocar-dial ischemia), (ii) STE in leads V1 to V3/V4 with or without STE in theinferior leads (RV transmural ischemia) and (iii) STE in leads III and/orV1/V2 with concomitant STD in leads V4/V5 to V6 (LV subendocardialischemia with concomitant RV transmural ischemia) [2]. APE shouldbe highly suspected in patients with specific ischemic patterns withconcomitant RV strain pattern, such as S1Q3 and/or abnormal QRSmorphology in lead V1 including notched S-wave, RBBB and Qr sign [2].In conclusion, STE confined to the inferior leads can be a transientECG manifestation in APE. It is important to recognize the dynamic

National Transplantation Pregnancy Registry: Pregnancy Outcomes in Lung Transplant Recipients.

The purpose of this study was to analyze pregnancy outcomes in female lung transplant recipients. Data were collected by the National Transplantation Pregnancy Registry (NTPR) via questionnaires, interviews, and medical records. There were 24 females transplanted between 1991-2010 who reported 33 pregnancies with 35 outcomes (one triplet pregnancy). The mean age at transplant was 28±5.0 yrs (range 19.7-38.5 yrs) and mean transplant-to-conception interval was 3.7±3.2 yrs (range 0.1-11.3 yrs). Conceptions occurred between June 1992 and September 2013. Outcomes included: 19 live births, 11 spontaneous abortions, and 5 therapeutic abortions. Immunosuppression included: cyclosporine (CsA) in 10 and tacrolimus in 23 pregnancies. Comorbid conditions during pregnancy included: hypertension 55%, diabetes 24%, infections 20%, and preeclampsia 5%. Five rejections were reported during pregnancy followed by 3 maternal deaths (9.9 mos, 1.6 yrs, and 4.3 yrs after pregnancy); all of these recipients were on CsA and were transplanted prior to 1996. With a maternal follow-up of 8.1±5.9 yrs, 15 recipients had adequate function (2 re-transplants), 3 had reduced function, 1 had a non-functioning transplant (with reduced native lung function), and 5 had died. Among the 19 newborn, mean gestational age was 33.9±5.0 wks; 63% were preterm (<37 wks). Mean birthweight was 2202±910 g; 63% were low birthweight (<2500 g). Neonatal deaths were reported in association with a triplet pregnancy: one fetus spontaneously aborted at 14 weeks, followed by the death of the two remaining infants delivered at 22 wks. Two children had birth defects: one had atrial and ventricular defects (pacemaker placed to treat arrhythmias) and the other had both hypospadias (repaired at 8 mos) and arteriovenous malformation (corrected when discovered at age 18). At last child follow-up, age 9.3±6.1 yrs, all 17 children were reported otherwise healthy and developing well. Conclusions: Successful pregnancy is possible after lung transplantation. These are high-risk pregnancies with an increased incidence of prematurity and low birthweight infants. Close surveillance of these recipients is warranted due to the potential impact of rejection during pregnancy on graft and recipient survival. All transplant centers are encouraged to participate in the NTPR. DISCLOSURES:Armenti, V.: Grant/Research Support, Novartis, Astellas, Pfizer, BMS.

Whole-exome resequencing reveals recessive mutations in TRAP1 in individuals with CAKUT and VACTERL association

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for approximately half of children with chronic kidney disease and they are the most frequent cause of end-stage renal disease in children in the US. However, its genetic etiology remains mostly elusive. VACTERL association is a rare disorder that involves congenital abnormalities in multiple organs including the kidney and urinary tract in up to 60% of the cases. By homozygosity mapping and whole exome resequencing combined with high-throughput mutation analysis by array-based multiplex PCR and next-generation sequencing, we identified recessive mutations in the gene TNF receptor-associated protein 1 (TRAP1) in two families with isolated CAKUT and three families with VACTERL association. TRAP1 is a heat shock protein 90-related mitochondrial chaperone possibly involved in antiapoptotic and endoplasmic reticulum-stress signaling. Trap1 is expressed in renal epithelia of developing mouse kidney E13.5 and in the kidney of adult rats, most prominently in proximal tubules and in thick medullary ascending limbs of Henle’s loop. Thus, we identified mutations in TRAP1 as highly likely causing CAKUT or CAKUT in VACTERL association.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia

Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

TCTAP A-059 Plasma Level of Secretory Type Ii Phospholipase A2 Is Pathophysiologically Associated with Pulmonary Artery Pressure in Congenital Atrial Septum Defect Subjects

TCTAP A-059 Plasma Level of Secretory Type Ii Phospholipase A2 Is Pathophysiologically Associated with Pulmonary Artery Pressure in Congenital Atrial Septum Defect Subjects

Catheter ablation of Wolff-Parkinson-White syndrome in conjoined thoracopagus twins

We previously reported unusual electrocardiograms (ECGs) recorded from conjoined thoracopagus twins with a cardiac connection at the atrial level, and Wolff-Parkinson-White (WPW) syndrome in Twin B.1 Tachycardia began in utero and was managed adequately with transplacental digoxin until elective C-section was performed at 36 weeks’ gestation. Postnatal evaluation demonstrated cardiac fusion of the lateral atrial walls and structural heart defects in both sisters (Twin A: tetralogy of Fallot; Twin B: dextrocardia, double-outlet right ventricle with a straddling tricuspid valve and multiple ventricular septal defects).

Cardiac CT in Mixed Type Atrial Septum Defects

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Minimally invasive surgery for congenital atrial septum defects in adults

Methods: Between 03/2004 and 04/2010, 52 patients underwent minimally invasive surgery for congenital ASD. Mean patient age was 40.79 ± 13.0 years, 34 (65.4%) were female. Indications for surgery were sinus venosus defect, shunt-volume >30% or ASD >2cm as well as persistent ASD after catheter based device-occlusion. Surgery was performed through a small right lateral mini-thoracotomy with cannulation of the peripheral vessels and video-assistance. ASD closure was conducted through the right atrium by either direct closure or pericardial patch. In the cohort, 2 (3.8%) patients suffered from a persistent foramen ovale, ASD II was present in 41 (78.8%) cases. A sinus venosus defect was found in 9 (17.3%) patients. All patients were in sinus rhythm preoperatively. Mean follow-up time was 42.3 ± 22 months. Perioperative parameters were analysed including stroke, arrhythmia, death and remaining ASD.

Electrophysiological studies and radiofrequency ablations in children and adolescents with arrhythmia.

BackgroundRadiofrequency ablation is the standard non-pharmacological treatment for arrhythmias in pediatric patients. However, arrhythmias and their associated causes have particular features in this population.ObjectiveTo analyze the epidemiological characteristics and findings of electrophysiological diagnostic studies and radiofrequency ablations in pediatric patients referred to the Electrophysiology Unit at Instituto de Cardiologia do Rio Grande do Sul, in order to characterize the particularities of this population.MethodsCross-sectional study with 330 electrophysiological procedures performed in patients aged less than 20 years between June 1997 and August 2013.ResultsIn total, 330 procedures (9.6% of the overall procedures) were performed in patients aged less than 20 years (14.33 ± 3.25 years, age range 3 months to 19 years), 201 of which were males (60.9%). A total of 108 (32.7%) electrophysiological diagnostic studies were performed and of these, 48.1% showed abnormal findings. Overall, 219 radiofrequency ablations were performed (66.3%) with a success rate of 84.8%. The presence of an accessory pathway was the most prevalent finding, occurring in 158 cases (72.1%), followed by atrioventricular nodal reentrant tachycardia (16.8%), typical atrial flutter (3.1%) and extrasystoles originating from the right ventricular outflow tract (2.7%). Three patients developed complications during ablation (1.4%). Among congenital heart diseases, which occurred in 51 (15.4%) patients, atrial sept defect was the most frequent (27.4%), followed by ventricular sept defect (25.4%) and Ebstein's anomaly (17.6%).ConclusionElectrophysiological study and radiofrequency ablation are effective tools for diagnosis and treatment of arrhythmias in the pediatric population.

Transcatheter Versus Surgical Closure of Atrial Septum Defect: A Debate from a Developing Country

Introduction: This study compares the effectiveness and cost of trans-catheter verses surgical closure of secundum atrial septum defect (ASD). ASD accounts for 10% of congenital cardiac defects. Trans-catheter closure of secundum ASD is increasingly used as the primary intervention. Surgical repair is advised in a proportion of secundum type defects which are unsuitable for device closure. Methods: We reviewed the clinical course of 176 patients who underwent closure of isolated secundum ASD. The patients were assigned to either the device or surgical group depending upon the treatment they received. Successful closure was assessed immediately after the procedure. The following outcomes were studied: mortality, morbidity, hospital stay, and costs. Results: Ninety five patients were in the surgical group and 81 patients were in the group undergoing device closure. The median age was 14.0 years (range 1.1-61.0) for surgical group and 24.0 years (range 0.5-68.0) for the device group. The mortality in both groups was 0. The procedure success rate was 100% for the surgical group and 96.3% for the device group. The complication rate was 13.7% for surgical group and 7.4% for the device group. The mean length of hospital stay was 5.0 ± 2.7 days for surgical group and 3.0 ± 0.4 days for device group. The procedure cost for surgery was found to be 12.3% lower than that of trans-catheter closure. Conclusion: Successful closure is achieved by both methods. Trans-catheter closure results in lower rate of complication and hospital stay but the cost of the procedure tends to be higher than surgery.

UK multicenter experience using the gore septal occluder (GSOTM) for atrial septal defect closure in children and adults

Percutaneous closure of atrial defects (ASD) has evolved as the treatment of choice for the majority of defects and patent oval foramens. The Gore Septal Occluder (GSO) is an innovative device consisting mostly of a folded thin GoreTex tube for use in the closure of septal defects. Reviewed is the multicenter UK experience of the first 22 ASD occlusions with the GSO(TM) device. All implantations were performed by consultant operators experienced in ASD device closure. The inclusion criterion was the presence of a hemodynamically significant secundum ASD with a diameter of <18 mm. Procedural data and acute and mid-term closure rates were retrospectively matched to a cohort of patients having defect closure using the Amplatzer Septal Occluder(TM) (ASO(TM) ). Acute and 3-month follow-up closure rates for the GSO(TM) were 100% and 100% vs. 100% and 100% closure with the ASO(TM) implants. The difference in paired procedure times was not statistically significant (56 min: GSO(TM) ; 42 min: ASO(TM) device P = ns), nor was the paired difference in fluoroscopic screening times (12 min: GSO(TM) vs. 8.4 min: ASO(TM) , P = ns). One GSO device embolized immediately after deployment and was successfully retrieved at the same procedure. There were no other significant complications in either group. The GSO(TM) implant can achieve comparable closure rates to the ASO(TM) in small to moderate atrial septal defects after 3 months. Longer fluoroscopy and procedure times are a drawback; however these should improve with familiarity with the implant and deployment system. The larger sheath size was not associated with increased complications in our cohort.

Risk Factors and Prognosis of Atrioventricular Block After Atrial Septum Defect Closure Using the Amplatzer Device

Amplatzer septal occluder (ASO)-induced complications have been observed. However, little attention is paid to the atrioventricular block (AVB) induced by atrial septum defect (ASD) closure using the Amplatzer device. This study aimed to analyze the risk factors and prognosis of AVB after catheter closure and to reduce the incidence of adverse events. In this study, 706 ASD patients who received closure in our division were investigated retrospectively for the relationship between AVB and factors such as age, size of the ASD (D(d)), diameter of the occluder (D(o)), diameter of the septum (D(s)), D(o)/D(d) ratio, and D(o)/D(s) ratio. Data distribution was evaluated with the Kolmogorov-Smirnov normality test. The Wilcoxon rank sum test was used to compare non-normal distribution data. A p value lower than 0.05 was considered significant. Of the 706 patients, six had experienced the development of AVB, giving an incidence of 0.85%. The risk factors included younger age, larger size of the ASD, larger size of the device, and the D(o)/D(s) ratio (≥0.45). The milder AVB is, the better the prognosis. An AVB of III° and an unchanged electrocardiogram (ECG) within 3 days after the procedure are poor prognostic indicators. More attention should be paid to AVB induced by ASD closure. Younger age, size of the ASD, size of the device, and a D(o)/D(s) ratio of 0.45 or higher are the risk factors associated with AVB after closure. A timely retrieval of the device should be considered for a good prognosis.

Different Atrial and Ventricular Resting Membrane Potentials May Explain the Phenotypical Variability of a Truncating SCN5A Mutation

<h3>Background</h3> The <i>SCN5A</i> gene encodes for the alpha-subunit of the cardiac sodium channel. The cardiac sodium current I_Na is essential for generation and transmission of the action potential, and I_Na dysfunction is responsible for multiple arrhythmias. Here, we characterized a distal truncating <i>SCN5A</i> mutation, R1860GfsX12, which was identified in a family with mixed clinical phenotypes of sick sinus syndrome, atrial fibrillation, atrial flutter, and AV block. <h3>Methods and Results</h3> Patch-clamp analysis performed in HEK 293 cells expressing the mutant channel showed a 70% reduction in I_Na density compared to cells expressing the wild-type (WT) channels, consistent with western blot analysis showing a partial degradation of the mutant. In addition, the R1860Gfs12X mutation drastically altered the steady-state inactivation leading to a –25-mV shift, delayed the fast and slow inactivation, and increased the persistent current. Moreover, when mimicking the heterozygous state of the patients by coexpressing WT with R1860GfsX12, the biophysical properties were still altered. Loss of I_Na density and alteration of the inactivation properties of this mutant channel are associated with a mix of atrial phenotype and AV block within the family. The variety of clinical phenotype seen in this family for the same mutation and the absence of a ventricular phenotype led us to investigate I_Na density in HEK cells at membrane potentials mimicking a ventricular vs an atrial resting membrane potential (–86 and –83 mV, respectively). Surprisingly, despite an only 3-mV hyperpolarizing difference in the resting membrane potential of atria, we observed a further decrease in I_Na density by 40% at the atrial resting membrane potential compared to the resting membrane potential of the ventricles. <h3>Conclusions</h3> Multiple mechanisms underlie functional consequences of <i>SCN5A</i> mutations. Here, we demonstrated that one mutation may have different clinical consequences in the atria or the ventricles due to their difference in resting membrane potential based on its biophysical defects. These results could explain how this mutation led to different atrial arrhythmias and conduction defect phenotype within the same family but to the absence of a Brugada phenotype.

Etiology analysis of upper airway obstruction in 54 newborn infants

Objective To explore the etiology of neonatal upper airway obstruction(UAO)in order to perform early diagnosis and treatment, and to improve the prognosis. Methods Retrospectively analysis of the clinical data of 54 cases of neonatal UAO in Shengjing Hospital of China Medical University from Jan.2005 to Dec.2011 was performed.All patients received CT three dimensional reconstruction reconstruction, 30 patients received the direct laryngoscopy, 5 cases received the video laryngoscope, 8 cases received pathological examination.All the cases received the determination of serum total calcium and ionized calcium, 6 cases received upper gastrointestinal imaging, 8 cases received chromosome inspection and 20 cases received echocardiography. Results CT scan: abnormal in 21 cases, among them 15 cases of congenital cyst, 5 cases of tongue fall blocking airway, and 1 case of choanal atresia(bilateral); another 33 patients were normal.Direct laryngoscopy results: abnormal in 13 cases, among them 12 cases of congenital cyst, and 1 case of glossoptosis; another 17 cases were normal.Electronic laryngoscopy results: 5 cases were diagnosed as congenital laryngomalacia.Pathological examination results: 7 cases of cyst wall by squamous epithelium composition, and 1 case of cyst wall by columnar epithelium composition.Serum total calcium and ion calcium level results: all patients were in the normal range.Chromosome examination results were normal.Upper gastrointestinal imaging results: 1 normal case, 5 cases of mild gastroesophageal reflux. Echocardiography results: 4 cases were normal, 10 cases of patent foramen ovale, 2 cases of patent ductus arteriosus, 4 cases of congenital heart disease (2 cases of atrial septum defect, 1 case of ventricular septal defect, and 1 case of interatrial septum merger ventricular septal defect). Conclusions In the etiology of neonatal UAO, there is a lot of organic diseases except for congenital laryngomalacia.For the UAO induced by laryngeal stridor or undefinitely cause, CT scan should be taken as soon as possible. Key words: Upper airway obstruction; CT scan; Newborn, infant

Short-term results of the Warden technique: a retrospective single-center study

Results Besides the anomalous pulmonary venous connection to the superior vena cava, 5 (56%) patients also presented an ostium secundum or patent foramen ovale atrial defect and 2 (22%) presented persistent left superior vena cava. One patient presented Turner Syndrome. Before surgery, 3 (33%) patients presented some form of conduction disorder: two right bundle branch block and one atrial extrasystole. The mean age at surgery was 10.8 ± 7.6 (min = 2, max = 29.7) years, and the distribution of the sexes was 2:1, the majority being male. None patient underwent reoperation or evolved to death during the follow-up period. After surgical repair, a forth patient presented with right bundle branch block. The degree of right chambers dilatation improved significantly at both atrial (p = 0,002) and ventricular (p = 0,046) level, after surgical repair. Three (33%) patients had postoperative tricuspid and/or pulmonary insufficiency. No venous obstruction occured.

Percutaneous Closure of Atrial Septal Defects in Spontaneously Breathing Children Under Deep Sedation: A Feasible and Safe Concept

Interventional cardiac catheterization in children and adolescents is traditionally performed with the patient under general anesthesia and endotracheal intubation. However, percutaneous closure of atrial septum defect (ASD) without general anaesthesia is currently being attempted in a growing number of children. The study objective was to evaluate the success and complication rate of percutaneous ASD closure in spontaneously breathing children under deep sedation. Retrospective single centre cohort study of consecutive children undergoing percutaneous ASD closure at a tertiary care pediatric cardiology centre. Transesophageal echocardiography (TEE) and percutaneous ASD closure were performed with the patient under deep sedation with intravenous bolus of midazolam and ketamine for induction and propofol continuous infusion for maintenance of sedation in spontaneously breathing children. One hundred and ninety-seven patients (median age 6.1years [minimum 0.5; maximum 18.8]) underwent TEE and ASD balloon sizing. Percutaneous ASD closure was attempted in 174 patients (88%), and device implantation was performed successfully in 92%. To achieve sufficient deep sedation, patients received a median ketamine dose of 2.7mg/kg (0.3; 7) followed by a median propofol continuous infusion rate of 5mg/kg/h (1.1; 10.7). There were no major cardiorespiratory complications associated with deep sedation, and only two patients (1%) required endotracheal intubation due to bronchial obstruction immediately after induction of sedation. Seventeen patients (8%) had minor respiratory complications and required frequent oral suctioning or temporary bag-mask ventilation. TEE and percutaneous ASD closure can be performed safely and successfully under deep sedation in spontaneously breathing children of all ages.

Simultaneous closure of the left atrial appendage and a severe LVOT: atrial shunt defect

A 72-year-old male with atrial fibrillation was transferred to our clinic for the evaluation of the interventional closure of the left atrial appendage (LAA) after a major bleeding complication under the novel anti-factor Xa inhibitor rivaroxaban. He had a history of biological aortic valve (AV) replacement and pericardial patch plastic of the left ventricular outflow tract (LVOT) after abscess forming infective endocarditis of the AV and LVOT ( Panel A , preoperative state, arrows …

Transcatheter closure of secundum atrial septal defect

Since the introduction of transcatheter closure of atrial septal defect, device closure has become the alternative treatment of selected atrial defects. Although excellent results have been reported for transcatheter closure, concerns have arisen regarding complications, including residual shunt, systemic or pulmonary embolization of the device, and erosion and perforation of the cardiac chamber. Those complications are rare but potentially serious adverse events that may require immediate surgical intervention. This review summarizes the current trends in patient selection, result of device closure and typical complications. Comparison between transcatheter device closure and surgical closure is also made.

Acute aortoiliac thrombotic occlusion in an infant with protein C deficiency and small atrial sepal defect—A rare case

Aortoiliac thrombosis is rare in infants and neonates. Protein C deficiency is frequently observed with venous thromboembolism, but off-late report of arterial thrombosis is increasingly common. We report a case of large infrarenal aortoiliac saddle and right popliteal artery thrombosis in a 7-month-old infant, with 3-mm atrial septal defect without clinical and Doppler evidence of deep vein thrombosis, presented with acute bilateral lower limb ischemic attack. Diagnosis was made by computed tomographic (C T) angiography of aorta and bilateral lower limbs. Patient operated on in emergency by transperitoneal route. Aortoiliac and right popliteal artery thromboembolectomy with closure of aortotomy with polytetraflouroethylene patch was performed.