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.     

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.     

Percutaneous closure of perimembranous ventricular septal defects utilizing almost ideal Amplatzer Duct Occluder II: Why limitation in sizes?

Aim: The purpose of this study is to describe the special aspects of perimembranous ventricular septal defects (pmVSD) closure by utilizing Amplatzer Duct Occluder II (ADO II) devices with a rational request for bigger ADO‐II sizes, based on our experi‐ ence in transcatheter device closure of pmVSD.
Methods and Results: At our institution, placement of an ADO II device was used in 15 patients with pmVSD; the patients’ age ranged between 6 months and 20 years. The indications for closure were CHF (n = 4), hemodynamically significant shunt (n = 7), tricuspid regurgitation (n = 3), and high risk for infective endocarditis (n = 2), respectively. The location of the VSD was infracristal in 13 patients, supracristal in 1, and a postsurgical Gerbode VSD in another one. Implantation of the device was suc‐ cessfully performed without embolization, any evidence of an AV block, or other conductance abnormalities during implantation and follow‐up in the mean of 2.5 years (range 2 months‐6.5 years).
Conclusions: Transcatheter closure of a pmVSD with ADO II is feasible in all pediatric and young adult age groups, by considering the device diameter limitations. The off‐ label use of ADO II implantation seems to be safe for VSDs closure up to 6 mm of size and feasible for various locations including unusual morphology such as postsurgical Gerbode defect.     

双心室矫治法洛四联症合并完全性房室间隔缺损的效果

目的:回顾性总结采用双室性矫治法洛四联症合并完全性房室间隔缺损(TOF/CAVSD)的外科手术经验及其效果分析。方法:自2004年1月至2011年12月期间,上海儿童医学中心连续对16例TOF/CAVSD,其中伴有唐氏综合征2例,手术年龄中位数9个月(4个月~12岁),手术时体质量中位数8 kg(4.7~35 kg),术前有56%患者伴有中度以上左侧房室瓣反流,31%患者伴有中度以上右侧房室瓣反流;术前平均肺动脉指数为(168±23)mm2/m2。合并的CAVSD矫治中均采用了双片法,其中有8例(50%)采用右心室流出道(RVOT)切口径路修补室间隔缺损,另外50%患者采用右心房切口径路修补室间隔缺损,仅有1例室间隔缺损补片上留孔,右心室流出道梗阻(RVOTO)处理中,13例患者(81%)施用心包补片跨瓣扩大成形右心室流出道和肺动脉,其中1例带有单瓣。结果:术后医院生存率为93.75%。术后平均呼吸机辅助时间为(166±22.5)h,平均监护室留驻时间中位数为7d。4例患者出现术后严重并发症,其中发生低心排出量综合征(低心排)者3例,多脏器功能损害者1例。出院时12.5%(2例)患者术后二尖瓣反流(轻-中度),12.5%患者术后轻度三尖瓣反流(TR),无1例存在左心室流出道梗阻(LVOTO)。随访期6个月~5年,随访期无死亡,无1例出现LVOTO,无再手术干预;3例肺动脉远端流速增快者已登记介入治疗;随访发现生存60%患者二尖瓣反流为轻微-轻度;同时发现有1/3生存者三尖瓣反流为轻微-轻度;随访期存在中度肺动脉血反流患者为86%。1/3生存患者的右心室舒张末容积相应正常年龄正常值。结论:双心室矫治法TOF/CAVSD的短中期效果好,推荐采用双片法矫治CAVSD;与单纯TOF或CAVSD手术一样,必须定期随访复查,需关注房室瓣功能、肺动脉发育、肺动脉逆向血流和心室功能。     

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.     

Increased incidence of infective endocarditis in patients with ventricular septal defect

Background: Ventricular septal defect (VSD) is one of the most common congenital heart anomalies in childhood and there is an increasing prevalence of VSDs in the adult population. The long‐term risk of infective endocarditis (IE) is of concern. The aim of this study was to clarify and compare the incidence of IE in adults with re‐ paired and unrepaired VSDs.
Methods: Patients with VSDs were identified using the Adult Congenital Heart Disease registry at the National Heart Centre Singapore. Patients were divided into Group 1 (repaired VSD) and Group 2 (unrepaired VSDs). The electronic medical re‐ cords were searched for hospitalization due to IE during a 10‐year period (October 2, 2007—October 1, 2017).
Results: Four hundred seventy‐nine patients (53% male) were identified, with a mean age of 35.0 ± 13.7 years. There were 164 patients (34.2%) in Group 1 and 315 pa‐ tients (65.8%) in Group 2. In total, there were eight episodes of IE from six patients (3 male, mean age of 42.2 ± 20.7 years). Two patients had recurrent IE. The overall inci‐ dence of IE was 1.67/1000 y, and this is 11–15‐fold higher compared to general adult population. The incidence of IE in Group 2 was 1.90/1000 y. There were no IE cases in Group 1.
Conclusion: Patients with VSDs, especially if unrepaired, carry a substantially in‐ creased risk of IE compared to the general population.     

Contemporary outcomes and mortality risks of Ebstein anomaly: A single‐center experience in Thailand

Background: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their “real world” survival.
Objectives: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era.
Methods: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardio‐ graphic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks.
Results: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow‐up [median time of 5.2 years (3 days‐23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day‐64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA sur‐ gery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predic‐ tors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z‐score >3.80, TV displacement >19.5 mm/m2, presence of severe tricuspid regurgitation, and ab‐ sence of forward flow across the pulmonic valve at the initial diagnosis.
Conclusion: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.     

Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect

Background: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echo‐ cardiography for right ventricular (RV) cardiac output assessment in patients with left‐to‐right shunt. The purpose of the study was to determine the correlation be‐ tween Doppler‐derived and Fick‐derived RV cardiac index (CI), and the interobserver correlation in Doppler‐derived RV CI assessment.
Methods: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004‐2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)² × right ventricular outflow tract (RVOT) time veloc‐ ity integral × heart rate]/body surface area.
Results: A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler‐derived and Fick‐derived RV CI (r = .57, P < .001), and the mean difference between Doppler‐derived and Fick‐ derived RV CI was −.3 (95% confidence interval of agreement, −.8 to +.9) L/min/m². There was also a modest correlation between Doppler‐derived RV CI from observer #1 and observer #2 (r = .62, P < .001), and the mean difference between Doppler‐de‐ rived RV CI from observer #1 and observer #2 was −.2 (95% confidence interval of agreement, −.9 to +.6).
Conclusions: The current study demonstrated a modest correlation between Doppler‐derived and Fick‐derived RV cardiac output, and a modest interobserver correlation in Doppler‐derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.     

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.     

Atrial septal defect in adults is associated with airway hyperresponsiveness

Objective: The association between secundum atrial septal defects (ASD) and asthma‐ like dyspnea with consequent long‐term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short‐and long‐term postprocedural follow‐up.
Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre‐and six‐ month postprocedurally, with additional bronchoprovocation at 2‐year follow‐up. Airway hyperresponsiveness was defined as ≥20% fall of forced expiratory volume in 1‐second (FEV1) following <8.0 mg/mL of inhaled methacholine.
Results: Airway hyperresponsiveness was found in 19/30 patients (63%[95%CI 45%‐81%]; post hoc statistical power = 89%). Asthma‐like symptoms wheezing, chest tightness, and cough were more frequently reported in airway hyperresponsive pa‐ tients. Airway responsiveness was not influenced by successful percutaneous ASD closure, corresponding to persistence of asthma‐like symptoms postclosure. Regardless of airway responsiveness, postprocedural right‐sided reverse remodeling significantly improved dyspnea and pulmonary function.
Conclusions: This study is the firsttoreport ahighprevalenceof airwayhyperresponsiveness in a cohort of unrepaired adult ASD patients, and confirms the association between asthma‐ like symptoms and ASD in adults. Attention to symptoms and pulmonary function should be given during clinical follow‐up of adult ASD patients, both before and long afterrepair.     

Accuracy of risk prediction scores in pregnant women with congenital heart disease

Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
Design: Single‐center retrospective study.
Setting: Tertiary care academic hospital.
Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myo‐ cardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum.
Results: Of 178 women, the most common CHD lesions were congenital aortic ste‐ nosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and te‐ tralogy of Fallot (12.9%). Thirty‐five women (19.7%) sustained 39 cardiac events. Observed vs expected event rates were 9.9% vs 5% (P = .02) for CARPREG I score 0 and 26.1% vs 7.5% (P < .001) for ZAHARA scores 0.51‐1.5. ZAHARA outperformed CARPREG I at predicting adverse cardiovascular outcomes (AUC 0.80 vs 0.72, P = .03) but was not significantly better than modified WHO. Clinical predictors of adverse cardiac event were symptoms (P = .002), systemic ventricular dysfunction (P < .001), and subpulmonary ventricular dysfunction (P = .03) with an AUC 0.83 comparable to ZAHARA (P = .66).
Conclusions: CARPREG I and ZAHARA scores underestimate cardiac risk for lower risk pregnancies in these women. Of the three risk schemes, CARPREG I performed least well in predictive capacity. Clinical factors specific to the population studied are comparable to stratification schemes.     

Echocardiographic Evaluation of Common Atrioventricular Canal Defects: A Study of 206 Consecutive Patients

An accurate echocardiographic evaluation of common atrioventricular canal (CAVC) requires indepth knowledge of the wide spectrum of morphological and physiological variations in this group of anomalies. In order to evaluate the incidence and morphological distribution of AV canal defects in a large series of patients and to define a systematic approach to the echocardiographic examination, we reviewed the echocardiograms of 206 consecutive patients with CAVC studied at Texas Children's Hospital over a 32-month period. The complete form of CAVC was most common (68.4%) and presented at an earlier age (mean ± SD: 1.6 ± 2.4 months). A partial AV canal (ostium primum atrial septal defect [ASD]) was found in 42 patients (20.4%) and their age at presentation was higher (9.2 ± 10 months). Twenty-three patients (11.2%) had a transitional AV canal. Down syndrome was diagnosed in 34% of patients, the majority of whom (79%) had a complete CAVC. Associated malformations were found in 46% of patients: anomalies of the conotruncus were most frequent (18%), followed by secundum ASD (14.1%), anomalous pulmonary venous connection (11.2%), and heterotaxy syndrome (11.2%). Subaortic obstruction and mitral stenosis were less common. The AV canal was unbalanced in 14.1% of patients, with the right ventricular dominant form being more common than the left ventricular dominant form (10.7% and 3.4%, respectively). Based on our experience, we developed a systematic, segment-by-segment approach to the echocardiographic examination in infants with CAVC. Together with detailed anatomical information, Doppler evaluation provides crucial hemodynamic information that allows planning of surgical repair.     

Surgical treatment of anomalous left main coronary artery with an intraconal course

Objective: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course.
Methods: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2‐47). The seven oldest patients all had preoperative symptoms of exertional chest pain and one also had exertional syncope. The five youngest pa‐ tients had no preoperative symptoms. One of these patients had a hemodynamically significant ventricular septal defect and one patient was the sibling of a patient who had undergone repair of AAOCA.
Results: The 12 patients underwent surgical repair including unroofing of the myo‐ cardial bridge overlying the intraconal LMCA and a LeCompte procedure. There was no early or late mortality and there were no significant complications. All 12 patients have resumed normal, unrestricted activities.
Conclusions: Twelve patients with an anomalous LMCA and intraconal course pre‐ sented to our institution for treatment. Surgical repair was performed successfully in all 12, with resolution of symptoms in the 7 patients who were symptomatic preop‐ eratively. These results suggest that the surgical treatment is safe and efficacious in patients with an anomalous LMCA and intraconal course.     

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.     

Routine leaflet augmentation of left atrioventricular valve in the repair of atrioventricular septal defect

Introduction

A better understanding of the morphology of complete atrioventricular septal defects (CAVSD) has impacted on surgical techniques and results. On some occasions the leaflet tissue is deficient and repair becomes difficult which leads to atrioventricular valve (AVV) regurgitation following the surgical repair of the AVSD.

Objectives

This study was conducted to evaluate a modified technique in which two patches where used to close the complete atrioventricular septal defect (CAVSD) with augmentation of the left atrioventricular valve (AVV) with the ventricular septal defect (VSD) patch.

Methods

The technique was performed on 105 infants with CAVSD at a mean age of 11.7 ± 23 months (median 5.7, range 1–135). Both superior and inferior bridging leaflets are divided routinely to expose the VSD. An autologous pericardial patch, sized precisely, is sutured to the ventricular septum. A 3–4 mm of extra patch is fashioned beyond the plane of the annulus and sutured to the divided leaflet of the left AVV. A second autologous pericardial atrial patch is attached to the body of the VSD patch at the plane of the annulus allowing 3–4 mm of the VSD patch to augment the left AV valve.

Results

There was one early death among these infants. At early postoperative echo all infants had no significant residual lesions. The contribution of the patch-augmented left AV valve to competency is clearly seen by two-dimensional echocardiography. At a mean follow up of 27 ± 10 months there were two late deaths with normal last echocardiography. There were only two children who progressed to severe left AV valve regurgitation needing reoperations.

Conclusions

This modified technique yields good anatomical repair. Allowing reconstruction of both AV valves independent of the other and is in particular helpful in cases of deficient left AVV tissue.     

Body mass index in adults with congenital heart disease

Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise test.
Results: Adults with CHD had slightly lower BMI than the reference group (24.1 ± 4.3 vs 24.6 ± 4.3; P = .012). Men in the mild and severe group (23.9 ± 3.6; 23.3 ± 4.4 vs 25.1 ± 3.7; P = .007; P = .023) and women in the severe group (21.6 ± 3.3 vs 24.2 ± 4.7; P < .001) had lower BMI compared to the reference group. In the subgroups, men with ventricular septal defect, coarctation of aorta/ventricular septal defect and Fontan cir‐ culation and women with Fontan circulation had lower BMI than the reference group. Underweight was more prevalent in women with severe lesions compared to the refer‐ ence group (22.2% vs 3.8%; P < .001). BMI was associated with age and exercise ca‐ pacity in patients with mild and moderate lesions, while higher BMI was related to better ventricular function in women with Fontan circulation.
Conclusion: Underweight was more prevalent in ACHD patients with severe lesions. Special attention should be paid to the possible existence of underweight‐related comorbidities.     

Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve‐sparing repair

Background: Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS).
Methods: A retrospective review was performed of all patients with TOF and pulmo‐ nary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early child‐ hood (<8 years). Student’s t test was performed to compare patients who underwent valve sparing (VS) versus transannular patch (TAP) repair. ANOVA was used to track measures of ventricular systolic function over time.
Results: We analyzed 151 echocardiograms performed on 42 patients. Pulmonary regurgitation (PR, moderate or severe) and the RV to left ventricular (LV) basal dimen‐ sion ratio were higher in TAP patients (P < .04 at all‐time points). Along with a signifi‐ cant increase in RV basal diameter Z‐score in the TAP group (P < .001), there was an improvement in RV and LV GLS over time in both groups (P < .001). The LV GLS at last follow‐up was lower in patients who underwent reoperation than those who did not (P = .050). LV GLS at the last follow‐up echocardiogram was lower in patients with significant PR than those without (P < .001).
Conclusions: Ventricular function appeared improve over time from the initial post‐ operative period in TOF patients. TAP repair was associated with a progressively higher RV/LV ratio in young children. GLS and RV/LV basal diameter ratio may be useful when following young children after TOF repair. Further research is necessary to understand the trajectory of ventricular functional and volumetric changes in young children in order to provide the most effective lifetime management of pa‐ tients with TOF.     

The clinical characteristics of coronary artery fistula anomalies in children and adults: A 24‐year experience

Objectives: The aim of our work is to investigate the clinical characteristics of coro‐ nary artery fistula (CAF) anomalies in South Vietnam.
Methods: This is a retrospective analysis of 119 patients with diagnosis of definite CAF between January 1992 and April 2016. The demographic, clinical, echocardio‐ graphic, and angiographic characteristics and management of CAF with short‐term outcomes are described.
Results: The median age was 15 years (range, 1‐79 years), with 49 male (41%) and 70 female (59%). There were 77 symptomatic patients (64.7%) and 91 patients (76.5%) who presented with a murmur. The electrocardiogram was abnormal in 45.4% and cardiac enlargement or increased pulmonary vasculature were seen in 76 patients (63.9%) on chest X‐ray. The sensitivity of echocardiography for CAF diagnosis was 79%. The source of the fistula was most often from the RCA (54%), most commonly to right atrium (34.5%) or right ventricle (31.1%). In comparison with surgery, tran‐ scatheter closure had a shorter hospital length of stay (5.4 ± 3.8 days vs 12.6 ± 6.5 days, P = .02) and better postprocedural left ventricular ejection fraction (67.9 ± 8.1% vs 62.9 ± 6.0%, P = .03).
Conclusion: The majority of fistula in this study originated from the RCA and termi‐ nated in the right atrium or the right ventricle. Transcatheter and surgical closure are both relatively safe and effective, with the potential for shortened length of hospital stay following transcatheter closure.