Concomitant pulmonary vein isolation and percutaneous closure of atrial septal defects: A pilot project

Background: Patients with an atrial septal defect (ASD) are at increased risk of de‐ veloping atrial fibrillation (AF). Currently percutaneous ASD closure is the preferred therapeutic strategy and although pulmonary vein isolation (PVI) for AF is feasible after ASD closure, the transseptal puncture can be technically challenging and prob‐ ably increases the perioperative risk. A staged approach, with PVI several months be‐ fore ASD closure, has been recommended for patients already scheduled for closure, but no data are available on combined procedures.
Purpose: This pilot study evaluates the feasibility of a combined procedure of PVI and ASD closure in patients with a hemodynamic important ASD and documented AF.
Methods: In one procedure, PVI was performed prior to placement of the ASD clo‐ sure device. Transseptal access for PVI was obtained via wire passage through the ASD in all patients. Patients were followed with 5‐day‐holter monitoring at 3, 6, and 12 months. Recurrence of AF was defined as a documented, symptomatic episode of AF.
Results: The study population consisted of five patients (four females, mean age: 58 (±3) years). Acute PVI was achieved in all patients. Only one patient had a small residual ASD after closure. Besides a small groin hematoma in two patients, no com‐ plications occurred. After 12‐month follow‐up, three patients were free of AF recur‐ rence (60%).
Conclusion: This study shows that a combined PVI with ASD closure is feasible with an acceptable success rate of AF free survival. These preliminary results in a small patient group warrants a larger trial.     

Normal airway responsiveness to methacholine in cardiac asthma

Cardiac asthma has been used as a synonym for episodes of cough, dyspnea, and wheezing caused by left ventricular dysfunction. The similarity of the terms bronchial asthma and cardiac asthma, and the observed symptoms of each disease implies a common pathophysiology. Bronchial asthma is characterized pathologically by airway narrowing, inflammation, edema, and obstruction by mucus. Bronchial asthma is defined as increased responsiveness of the tracheobronchial tree, which is manifested clinically as reversible expiratory airflow obstruction. The classic symptoms of bronchial asthma are cough, dyspnea, and wheezing. Cardiac asthma produces the same symptoms, but the pathophysiology producing these symptoms is not well described. We describe two patients with cardiac asthma who failed to demonstrate airway hyperresponsiveness to nonspecific bronchoprovocation testing and we postulate that these patients' symptoms were produced exclusively by left ventricular failure.     

Airway hyperresponsiveness: the usefulness of airway hyperresponsiveness testing in epidemiology,in diagnosing asthma and in the assessment of asthma severity

The present PhD thesis was conducted at the Respiratory Research Unit at the Pulmonary Department L in Bispebjerg Hospital, Copenhagen, Denmark and describes airway hyperresponsiveness in asthma patients in four studies. The first study concerned risk factors for the development of asthma in young adults in a 12‐year prospective follow‐up study of a random population sample of 291 children and adolescents from Copenhagen, who were followed up from the age of 7–17 years (1986) until the age of 19–29 years (1998). During follow‐up, 16.1% developed asthma, and in these subjects, the most important predictor of asthma development was airway hyperresponsiveness to histamine at baseline. Airway hyperresponsiveness is associated with more severe asthma and a poorer prognosis in terms of more exacerbations and less chance of remission of the disease. The second study described the relation between airway hyper‐responsiveness to methacholine and the quality of life in 691 asthma patients: In asthma patients with airway hyperresponsiveness to methacholine, the quality of life measured with a validated questionnaire (Junipers Asthma Quality of Life Questionnaire) was significantly reduced compared to asthma patients who did not respond to bronchial provocation with methacholine. Airway hyperresponsiveness is not uncommonly observed in non‐asthmatics, and the response to bronchial provocation with methacholine is therefore relatively non‐specific. The mannitol test is a relatively new bronchial provocation test that acts indirectly on the smooth airway muscle cells through the release of mediators from inflammatory cells in the airways; the mannitol could consequently be a more specific test compared with methacholine. The third study showed that out of 16 non‐asthmatics with airway hyperresponsiveness to methacholine, 15 did not respond to bronchial provocation with mannitol Because of the mechanism of action of mannitol, it seems plausible that the response to mannitol is more closely correlated to airway inflammation in asthma compared with the response to methacholine. The fourth study showed that in 53 adult asthma patients, who did not receive treatment with inhaled steroids, there was a positive correlation between the degree of airway inflammation and the degree of airway responsiveness to mannitol as well as to methacholine. The mannitol does, however, have the advantage of being a faster and simpler test to perform, requiring no additional equipment apart from a spirometer. Conclusions: Airway hyperresponsiveness in children and in adolescents without asthma predicts asthma development in adulthood. Asthma patients with airway hyperresponsiveness to methacholine have a poorer quality of life as well as more severe disease and a poorer prognosis compared with asthma patients without airway hyperresponsiveness. Bronchial provocation with mannitol as well as with methacholine were useful for evaluating the severity of asthma and the degree of airway inflammation, and accordingly for determining the need for steroid statement. The mannitol test does, however, have practical advantages over the methacholine test that make it preferable for clinical use.     

Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age 9 months (inter‐ quartile range, 5‐14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time‐to‐pallia‐ tion of 4 years (interquartile range, 3‐5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five‐ and 10‐year transplant‐free survival were 91% and 84%, respectively. At a median follow‐up of 6 years (interquartile range, 2‐8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3‐11], oxygen sat‐ uration 83% ± 4%). Preoperative moderate‐severe atrioventricular valve regurgita‐ tion (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80‐33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08‐6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for co‐ variates. Reaching further palliation was less likely in patients with preoperative moderate‐severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08‐0.59; P =.002).
Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circum‐ stances, these patients can have a favorable long‐term prognosis without reducing their suitability for further palliation.     

Lost in the system? Transfer to adult congenital heart disease care-Challenges and solutions

Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
Design: A retrospective cohort study.
Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
Interventions: We analyzed for protective factors associated with age‐appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities.
Outcome Measures: We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008‐2015.
Results: Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months be‐ tween last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe).
Conclusion: Patients with severe disease are more likely to transfer to adult congeni‐ tal heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to under‐ stand and address barriers in order to improve successful transfer.     

Dysrhythmias in patients with a complete atrioventricular septal defect: From surgery to early adulthood

Objective: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD.
Study design: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients’ medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias.
Results: Median age at repair was 6 months (interquartile range 3‐10) and median age at last follow‐up was 24 years (interquartile range 21‐28). SND occurred after a me‐ dian of 17 years (interquartile range 11‐19) after repair in 23% of patients, requiring pacemaker implantation in two patients (12%). Regular supraventricular tachycardia was observed in three patients (4%). Atrial fibrillation and ventricular tachyarrhyth‐ mias were not observed. Twenty‐seven patients (36%) had first‐degree AVB, which was self‐limiting in 16 (59%) and persistent in 10 (37%) patients. One patient devel‐ oped third‐degree AVB 7 days after left atrioventricular valve replacement. Spontaneous type II second‐degree AVB occurred in a 28‐year‐old patient. Both pa‐ tients underwent pacemaker implantation.
Conclusions: Clinically significant dysrhythmias were uncommon in young adult pa‐ tients after cAVSD repair. However, three patients required pacemaker implantation for either progression of SND or spontaneous type II second‐degree AVB. Longer follow‐up should point out whether dysrhythmias will progress or become more prevalent with increasing age.     

The acute effects of 30 mg vs 60 mg of intravenous Fasudil on patients with congenital heart defects and severe pulmonary arterial hypertension

Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
Design: Prospective randomized controlled trial.
Setting: Tertiary cardiology center.
Patients: Adult patients with CHD‐PAH.
Interventions: Patients were randomized to Fasudil 30 or 60 mg.
Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava, and femoral artery. Pulmonary vascular resistance (PVR) and sys‐ temic arterial resistance (SVR) were calculated.
Results: The changes in systolic pulmonary artery pressure (sPAP) (−13.1% vs −9.3%, P < .05), diastolic PAP (dPAP) (−17.6% vs −14.5%, P < .05), mean PAP (mPAP) (−12.4% vs −8.5%, P < .05), and PVR (−35.8% vs −22.2%, P < .05) were more pronounced in the 60‐mg group than in the 30‐mg group. All patients had no obvious adverse reac‐ tions related to peripheral blood pressure.
Conclusions: Fasudil could improve the hemodynamics of patients with CHD‐PAH, especially with the 60‐mg dose. There were no serious adverse reactions.     

Long‐term cardiovascular outcome of Williams syndrome

Objective: Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Eighty patients with Williams syndrome followed up for more than 5 years.
Interventions: Not applicable.
Outcome Measures: Long‐term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post‐interventional courses of disease‐specific intervention, and intervention‐free survival of obstructive cardio‐ vascular lesions.
Results: The median follow‐up duration was 11.0 (5.1‐28.3) years. Among 80 pa‐ tients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow‐up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echo‐ cardiography. Initially, severe supravalvular aortic stenosis was aggravated (P < .027). Conversely, the peak velocity of branch pulmonary stenosis decreased (from 3.08 to 1.65 m/s; P < .001) within age 3.2 (0.4‐6.9) years. Even the group with severe branch PS improved over time. Twenty‐two patients (27.5%) with Williams syndrome under‐ went disease‐specific interventions without mortality, mostly for supravalvular aortic stenosis or mitral valve prolapse. No patient in the late‐onset and initially mild sup‐ ravalvular aortic stenosis group needed intervention and 37.5%, 48.4%, and 65.1% in initially moderate and severe supravalvular aortic stenosis groups needed inter‐ vention at age 5, 10, and 20 years, respectively. Unlike the conventional therapeutic concept, the intervention for branch pulmonary stenosis was almost unnecessary.
Conclusions: In Williams syndrome, initially severe supravalvular aortic stenosis worsened over time and most branch pulmonary stenoses, including those in the severe group, improved spontaneously. Most patients with branch pulmonary ste‐ nosis did not require disease‐specific intervention. Surgical repairs for cardiovascular abnormalities in Williams syndrome showed favorable results.     

Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease

Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO2), oxygen pulse (O2 pulse), ventilatory equivalent for carbon dioxide (VE/CO2 at an‐ aerobic threshold), constant work‐rate endurance time (CWRET) at 75% of peak VO2, and 6‐minute walk distance (6MWD) were compared between baseline and after training by paired t test.
Result: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respec‐ tively). The mean difference of 6MWD change in the home‐program group was sig‐ nificantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training.
Conclusion: Once‐a‐week outpatient hospital‐based exercise program followed by supervised home‐based exercise program showed a significant benefit in improve‐ ment of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes.     

Impact of durable ventricular assist devices on post‐transplant outcomes in adults with congenital heart disease

Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy.     

Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT) or ventricular fibrillation (VF). The rate of inappropriate therapies and device‐related complications was 33.3%. Secondary prevention and primary pre‐ vention patients with class I indications for ICD had more appropriate therapies than complication, but this relationship was reversed for patients with class II indications. Remote monitoring played an important role in diagnosing new atrial arrhythmias be‐ fore scheduled visits in 46.2% of patients, leading to a change in medication. VT/VF episodes were associated with a composite of death, cardiac transplantation, and hos‐ pital admission (OR 13.0; 95% CI: 2.1‐81.5).
Conclusion: ICDs are not only useful in preventing SCD, but also have a major role in diagnosing atrial tachyarrhythmias ahead of scheduled visits. Although improve‐ ments in ICD technology might reduce complications and inappropriate therapies, adequate selection of candidates for primary prevention still remains difficult be‐ cause of the lack of clear indications.     

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation).
Results: Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The ma‐ jority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm² /m² had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm² /m² at follow up achieved 2V circula‐ tion. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm² /m² included larger TV z‐score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002).
Conclusions: Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.     

Low-dose salbutamol suppresses airway responsiveness to histamine but not methacholine in subjects with asthma

BackgroundAirway hyperresponsiveness is a cardinal feature of asthma. Although the modulation of cholinergic neuroeffector transmission may play a role in airway responsiveness, in vivo evidence remains scarce. It is well known that histamine causes bronchoconstriction partly via vagal reflex, whereas methacholine does not. To investigate the significance of modulating neuroeffector transmission, we compared the effect of low-dose salbutamol—a β₂-adrenoceptor agonist—on airway responsiveness to histamine with that to methacholine.MethodsWe enrolled 12 subjects with stable asthma. After screening confirmed that inhalation of low-dose salbutamol (1 μg) did not change their basic pulmonary function, subjects underwent measurement of airway responsiveness to inhaled histamine and methacholine with or without pretreatment with low-dose salbutamol, in a randomized, crossover fashion. Airway responsiveness was measured by an astograph by which respiratory conductance (Grs) was assessed by the forced oscillation method during continuous inhalation of histamine or methacholine in stepwise incremental concentrations. Airway responsiveness was calculated as the cumulative dose of bronchoconstrictors that induced a decrease of 35% in Grs.ResultsInhalation of 1 μg of salbutamol significantly attenuated airway responsiveness to histamine but not methacholine. This selective attenuation was observed irrespective of disease severity or phenotype, namely atopy or non-atopy.ConclusionLow-dose salbutamol suppresses airway responsiveness to histamine but not methacholine in subjects with asthma. The present study may provide a novel insight into the bronchoprotective mechanism of β₂-adorenoceptor agonist in clinical settings.     

Racial disparities in clinic follow‐up early in life among survivors of congenital heart disease

Objective: The current study aims to identify the rates of lapses in care and loss to follow‐up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow‐up than whites.
Design: Patients were from a large pediatric hospital and had (1) at least one outpa‐ tient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a di‐ agnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from before age one through age five. Lapse in follow‐up was defined as not having at least one outpatient cardiology visit per year, and loss to fol‐ low‐up was not returning after a lapse in care by age five. Race was categorized as white and nonwhite. Covariates included sex, insurance type, noncardiology inpa‐ tient and outpatient hospital utilization, and CHD severity.
Results: The sample included 1034 patients. Overall, 75.7% experienced a lapse in care with only 41.6% of those returning by age five. Nonwhites experienced lapses in care at younger ages than whites. Nonwhites had a 53% increased risk of lapse in care. Medicaid patients and those with moderate CHD diagnoses also had an in‐ creased risk for lapse in care.
Conclusions: Lapse in care appears prevalent among CHD survivors by age five, with nonwhites demonstrating elevated risk. Future multisite prospective studies should include the assessment of parental knowledge, barriers to accessing care, and satis‐ faction with care.     

Airway Responsiveness to Indirect Challenges in COPD

ABSTRACT

Patients with chronic obstructive pulmonary disease (COPD) demonstrate airway hyperresponsiveness to a number of indirect stimuli. Hyperresponsiveness to cold air hyperventilation, exercise, and drugs like propranalol and methoxamine seem to be able to distinguish patients with COPD from those with asthma, whereas hyperresponsiveness to stimuli like adenosine 5-monophosphate (AMP) and hypertonic saline seem unable to do so. The relationship of airway responsiveness to indirect stimuli and airway inflammation has received little study. The clinical relevance of hyperresponsiveness to an indirect challenge, including the impact on the natural history, relation to types of bronchitis, baseline airway calibre, and response to treatment need to be studied.     

Immediate and short‐term effects of transcatheter device closure of large atrial septal defect in senior people

Objectives: We sought to evaluate the safety and efficacy in improving cardiac function and functional capacity with device closure of large atrial septal defects (ASD) in senior adults.
Background: Atrial septal defect accounts for about 10% of all congenital heart dis‐ ease. It still remains unclear whether large ASD closure in senior people should be performed or not. Hence we aim to prospectively assess the safety and clinical status of senior patients after transcatheter closure in large ASD.
Patients and interventions: This was a prospective study of all patients aged over 50 years who underwent device closure of a secundum large ASD between January 2013 and January 2018. Investigations including brain natriuretic peptide level, electrocardiography, chest X‐ray, transthoracic echocardiogram, transesophageal echocardiogram, and 6‐minute walk test were performed before and at 2 days and 4 weeks and 6 months after the procedure.
Results: Twenty patients (median age 68 years, 10 women) had transcatheter device closure of large ASD successfully. Median ASD size was 32 mm (range 30‐39 mm). Median pulmonary artery pressure was 58 mm Hg (range 47‐67 mm Hg). At 6 months, there was a significant change in right atrium size (P < .001) and right ventricle size (P < .01) and left ventricle size (P < .001) and also pulmonary artery pressure (P < .0001), New York Heart Association functional class improved (P = .03) in 19 patients and also significant improvement in 6‐minute walk test distance (P < .001). There were no major complications.
Conclusions: Our data showed that large ASD closure at senior people results in sat‐ isfactory cardiac remodeling and cardiac function improvement.     

Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single‐center experience

Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow‐up time in adulthood was 2.9 [IQR 1.3‐8.2] years. Forty‐three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology‐related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3‐56.1) years. Heart failure‐free sur‐ vival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident‐free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia‐free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia‐free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively.
Conclusions: At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this popula‐ tion are warranted.     

Stenting the vertical neonatal ductus arteriosus via the percutaneous axillary approach

Background/Objective: Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal‐ dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative ac‐ cess sites have been explored. Carotid access complications have been reported in 5%‐10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach.
Methods: We reviewed all patients with DAS with an axillary approach in neonates with ductal‐dependent PBF (May 2017‐May 2018) in our institution. Procedural re‐ ports, angiograms, and clinical records of all consecutive patients were reviewed. Procedural technique, procedural outcomes, adverse events, and post‐hospital courses are reported.
Results: Seven consecutive patients who received DAS utilizing axillary approach. All patients had ductal‐dependent PBF through a vertical, tortuous ductus. Five had pulmonary atresia or near atresia, one had compromised PBF due to dynamic subval‐ var obstruction, and one had Tetralogy of Fallot with isolated left pulmonary artery. Axillary access with 3.3 or 4 French sheath was obtained using ultrasound guidance. Bare metal coronary stents were deployed successfully in all. Intra‐procedure, one developed in stent thrombus requiring re‐stenting. There were no procedural mor‐ talities or major adverse events from axillary access. There is a steep learning curve. Hemostasis was achieved with manual compression. Two patients had reintervention at 6‐8 weeks. All patients underwent successful planned surgeries.
Conclusion: This series suggests DAS in neonates utilizing an axillary approach is a feasible and effective alternative for establishing PBF. Axillary arterial approach may be preferred as there is no risk to neurological sequelae and very low risk of limb complications. Larger series are needed to validate this approach.