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.     

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.     

32 year follow up of patients following atrial redirection surgery for transposition of the great arteries

Objective: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.
Design: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic in‐ formation and data regarding the clinical state, NHYA class, cardiopulmonary exer‐ cise testing, NT‐proBNP measurement, and recent cardiac MRI findings.
Results: Forty‐six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty‐two patients (69.6%) had undergone a Senning procedure. The median length of the follow‐up was 32 years. Thirty‐two patients (69.6%) were NHYA class 1. The mean VO_2max achieved was 24.2 ± 5.8 mL/min/kg. The mean NT‐ proBNP was 266.4 pg/mL (± 259.9 pg/mL). The mean right ventricular end‐diastolic volume (RVEDV) was 212.4 mL ± 73.1 mL (indexed 114.2 mL/m² ± 34.4 mL/m² ). The mean right ventricular ejection fraction (RVEF) was 53.7% ± 7.9%. The mean left ven‐ tricular end‐diastolic volume (LVEDV) was 161.5 mL ± 73.7 mL (indexed 87.8 mL/ m² ± 41.1 mL/m² ). The mean left ventricular ejection fraction (LVEF) was 59.8% ± 5.7%. There was a significant correlation between right ventricular (RV) size on MRI and NT‐proBNP level.
Conclusions: We present a relatively well cohort of patients with overall favorable long‐term outcome. The majority of patients are NHYA class 1 and the systemic right ventricular function appears to be well preserved as assessed by MRI. The exercise tolerance is reduced, with the majority of patients achieving around 60% of the esti‐ mated VO_2max. Regular specialist follow‐up and assessment with advanced imaging at regular intervals remain important for this group.     

The long‐term functional outcome in Mustard patients study: Another decade of follow‐up

Objective: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d‐transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional ca‐ pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty‐five of the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐ ble including exercise stress testing, echocardiography, MRI or CT evaluation of car‐ diac anatomy and function, Holter monitor, and quality of life questionnaire.
Results: Thirty‐one percent of patients have experienced cardiac death either in the form of mortality or cardiac transplantation. The major cause of death was systemic right ventricular failure. Sixty‐five percent have continuing abnormalities of rhythm. Exercise time and workload showed a statistically significant decrease from the origi‐ nal study (Time 1) to both 10‐year (Time 2) and 20‐year (Time 3) follow‐up points. Right ventricular ejection fraction decreased significantly from the Time 1 to Time 2, and again to this current follow‐up. Quality of life measures of energy level decreased significantly from the original study to both the Time 2 and Time 3.
Conclusion: Cardiac mortality for Mustard patients remains high, and over time, sys‐ temic right ventricular ejection fraction, rhythm, exercise tolerance, and quality of life assessments show deterioration. There does not appear to be a single clear pre‐ dictor of poor outcome.     

Improvement in ventricular function with rhythm control of atrial arrhythmias may delay the need for atrioventricular valve surgery in adults with congenital heart disease

Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.
Design: Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate‐severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were in‐ terpreted by a single echocardiographer blinded to treatment strategy. Patients were followed until AV valve surgery, heart transplantation, death, or last clinical follow‐up.
Results: Rhythm control was attempted in 9 of 24 identified patients. Among these nine patients, arrhythmias were eliminated in three and reduced from persistent to paroxysmal in another three. In the rhythm control group, mean left ventricular ejec‐ tion fraction improved from 54.4 ± 12.4% to 60.0 ± 11.5% (P = .02) and mean right ventricular systolic function increased nearly one grade (P = .02). AVVR did not de‐ crease significantly. No significant change in left or right ventricular systolic function, or AVVR was observed among the 15 patients treated with rate control. Four‐year survival free of AV valve operation and heart transplant was 88% in the rhythm con‐ trol group and 31% in the rate control group (P = .04).
Conclusions: In ACHD patients with atrial arrhythmias and at least moderate‐severe AVVR, a rhythm control strategy was associated with improved biventricular systolic function. This improvement in ventricular function and symptoms may allow valve surgery to be deferred.     

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 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.     

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.     

Diastolic velocity half time is associated with aortic coarctation gradient at catheterization independent of echocardiographic and clinical blood pressure gradients

Objective: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak‐to‐peak pressure gradient ≥20 mm Hg at catheterization, the ac‐ cepted threshold for intervention.
Design: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed. Multiple echocardio‐ graphic measurements and blood pressure gradients prior to cardiac catheterization were collected. Univariate correlation of variables with the continuous catheteriza‐ tion peak were calculated using Spearman’s rho. Univariate association with peak‐to‐ peak gradient at catheterization ≥20 mm Hg was tested using Mann‐Whitney U test and the Pearson chi‐square test or Fisher’s exact test. Multivariable logistic regres‐ sion assessed the independent association of the clinically relevant metrics with gra‐ dient at catheterization ≥20 mm Hg.
Results: Sixty‐eight patients met study criteria (median age 9.25 years), of whom 84% underwent intervention at catheterization. Echocardiographic peak and mean coarctation velocity, indexed systolic and diastolic velocity half times (SVHTi, DVHTi), and blood pressure gradient all had moderate correlation (Spearman’s rho = 0.529‐0.617, P < .001) with the continuous catheterization gradient and were significantly associated with the binary outcome of catheterization peak ≥20 mm Hg (P < .001). Logistic regression found echocardiographic mean systolic gradient (OR 1.213 [95% CI 1.041‐1.414]) and DVHTi (OR 1.039 [95% CI 1.004‐1.074]) indepen‐ dently associate with catheterization peak ≥20 mm Hg after controlling for blood pressure gradient (OR 1.066 [0.987‐1.150]).
Conclusions: Most echocardiographic estimates show moderate correlation with arch gradient at catheterization. Noninvasive four extremity blood pressure gradient is significantly associated with peak‐to‐peak gradient ≥20 mm Hg. DVHTi may pro‐ vide a unique independently associated echocardiographic estimate of coarctation severity. Further study of these variables with larger cohorts may allow for develop‐ ment of predictive models to direct catheterization.     

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.     

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Intervention and Outcome Measures: Demographic and procedural data were cor‐ related with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biven‐ tricular circulation.
Results: The procedural success rate was 92% (33 out of 36) in this group with mod‐ erate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of pa‐ tients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐ up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circu‐ lation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%).
Conclusion: Encouraging long‐term results with biventricular circulation and func‐ tional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.     

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.     

Complete atrioventricular canal repair with a decellularized porcine small intestinal submucosa patch

Background: Congenital heart defects affect nearly 1% of all children born per year in the United States, and complete atrioventricular canal (CAVC) accounts for 2%‐9%. While several patch materials have been used for septal defect closure during CAVC repair, clear superiority of one material over another has yet to be established.
Methods: A retrospective review of clinical outcomes following CAVC repair at Morgan Stanley Children’s Hospital/Columbia University was performed on opera‐ tions conducted from March 2010 to September 2017. Univariate and Kaplan‐Meir survival analyses were utilized to evaluate primary outcomes of interest following CAVC repair in the modern surgical era.
Results: A total of 73 patients were analyzed, with an average operative age of 22 weeks. The majority (71%) of the patients underwent a 2‐patch repair. A CorMatrix patch was used for ventricular septal defect(VSD) closure in 77% of the patients, and/or in 75% of atrial septal defect closures. There was one in‐hospital mortality (1.4%) due to respiratory failure. One patient required a pacemaker. At mid‐term fol‐ low‐up (1.6 years), a total of 7 patients required 8 reoperations due to cardiac‐related indications, including 5 for left atrioventricular valve (LAVV) repair, 1 for LAVV re‐ placement, and 2 isolated residual VSDs.
Conclusion: A standardized repair for CAVC results in excellent outcomes in the cur‐ rent era, with low rates of reoperations. CorMatrix for the closure of CAVC has proven to produce good results with equivalent outcomes to other patch materials. Its ease of use and pliability make it an attractive alternative for consideration.     

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.     

Ventricular force‐frequency relationships during biventricular or multisite pacing in congenital heart disease

Background: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force‐frequency relationship (FFR) using tissue Doppler‐de‐ rived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its vari‐ ability with different ventricular pacing strategies.
Methods: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color‐coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate and with incremental pacing. FFR curves were obtained by plotting IVA against heart rate for different ventricular pacing strategies.
Results: Ten patients were included (mean: 22 ± 7 years). The FFR identified a best and worst ventricular pacing strategy for each patient, based on the AUC at baseline, submaximal, and peak heart rates (P < .001). However, there was no single best ventricular pacing strategy that was optimal for all patients. Additionally, the best ventricular pacing strategy often differed within the same patient at different heart rates.
Conclusion: This novel assessment demonstrates a wide variability in optimal ventricular pac‐ ing strategy. These inherent differences may play a role in the unpredictable clinical response to BiV pacing in CHD, and emphasizes an individualized approach. Furthermore, the optimal ventricular pacing varies with heart rate within individuals, suggesting that rate‐responsive ventricular pacing modulation may be required to optimize ventricular performance.     

S100B and its relation to cerebral oxygenation in neonates and infants undergoing surgery for congenital heart disease

Objectives: Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic‐ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astro‐ cyte protein S100B and its relation to cerebral oxygenation.
Methods: Serum S100B was measured before and 0, 12, 24, and 48 hours after sur‐ gery. Cerebral oxygen saturation was derived by near‐infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th per‐ centile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in the normal range. Neonates (≤28 days) and infants (>28 and ≤365 days) were analyzed separately due to age‐dependent release of S100B.
Results: Seventy‐four patients underwent 94 surgical procedures (neonates, n = 38; infants, n = 56). S100B concentrations were higher in neonates before and after sur‐ gery at all time points (P ≤ .015). Highest values were noticed immediately after sur‐ gery. Postoperative S100B was elevated after 15 (40.5%) surgeries in neonates. There was no difference in pre‐, intra‐, or postoperative cerebral oxygenation. In in‐ fants, postoperative S100B was elevated after 23 (41.8%) procedures. Preoperative cerebral oxygen saturations tended to be lower (53 ± 12% vs 59 ± 12%, P = .069) and arterial‐cerebral oxygen saturation difference was higher (35 ± 11% vs 28 ± 11%, P = .018) in infants with elevated postoperative S100B. In the early postoperative course, cerebral oxygen saturation was lower (54 ± 13% vs 63 ± 12%, P = .011) and arterial‐cerebral oxygen saturation difference was wider (38 ± 11% vs 30 ± 10%, P = .008). Cerebral oxygen saturation was also lower for the entire postoperative course (62 ± 18% vs 67 ± 9%, P = .047).
Conclusions: Postoperative S100B was elevated in about 40% of neonates and in‐ fants undergoing cardiac surgery. Infants with elevated postoperative S100B had impaired perioperative cerebral tissue oxygenation. No relation between S100B and cerebral oxygenation could be demonstrated in neonates.     

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.