Machine learning (ML) can facilitate prediction of major adverse cardiovascular events (MACEs) in repaired tetralogy of Fallot (rTOF). We sought to determine the incremental value of ML above expert ...clinical judgement for risk prediction in rTOF.
Adult congenital heart disease (ACHD) clinicians (≥10 years of experience) participated (one cardiac surgeon and four cardiologists (two paediatric and two adult cardiology trained) with expertise in heart failure (HF), electrophysiology, imaging and intervention). Clinicians identified 10 high-yield variables for 5-year MACE prediction (defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia and HF). Risk for MACE (low, moderate or high) was assigned by clinicians blinded to outcome for adults with rTOF identified from an institutional database (n=25 patient reviews conducted by five independent observers). A validated ML model identified 10 variables for risk prediction in the same population.
Prediction by ML was similar to the aggregate score of all experts (area under the curve (AUC) 0.85 (95% CI 0.58 to 0.96) vs 0.92 (0.72 to 0.98), p=0.315). Experts with ≥20 years of experience had superior discriminative capacity compared with <20 years (AUC 0.98 (95% CI 0.86 to 0.99) vs 0.80 (0.56 to 0.93), p=0.027). In those with <20 years of experience, ML provided incremental value such that the combined (clinical+ML) AUC approached ≥20 years (AUC 0.85 (95% CI 0.61 to 0.95), p=0.055).
Robust prediction of 5-year MACE in rTOF was achieved using either ML or a multidisciplinary team of ACHD experts. Risk prediction of some clinicians was enhanced by incorporation of ML suggesting that there may be incremental value for ML in select circumstances.
Improved understanding of the role of inflammation in tendon disease is required to facilitate therapeutic target discovery. We studied supraspinatus tendons from patients experiencing pain before ...and after surgical subacromial decompression treatment. Tendons were classified as having early, intermediate, or advanced disease, and inflammation was characterized through activation of pathways mediated by interferon (IFN), nuclear factor κB (NF-κB), glucocorticoid receptor, and signal transducer and activator of transcription 6 (STAT-6). Inflammation signatures revealed expression of genes and proteins induced by IFN and NF-κB in early-stage disease and genes and proteins induced by STAT-6 and glucocorticoid receptor activation in advanced-stage disease. The proresolving proteins FPR2/ALX and ChemR23 were increased in early-stage disease compared to intermediate- to advanced-stage disease. Patients who were pain-free after treatment had tendons with increased expression of CD206 and ALOX15 mRNA compared to tendons from patients who continued to experience pain after treatment, suggesting that these genes and their pathways may moderate tendon pain. Stromal cells from diseased tendons cultured in vitro showed increased expression of NF-κB and IFN target genes after treatment with lipopolysaccharide or IFNγ compared to stromal cells derived from healthy tendons. We identified 15-epi lipoxin A4, a stable lipoxin isoform derived from aspirin treatment, as potentially beneficial in the resolution of tendon inflammation.
Adult patients who have undergone the Fontan procedure are highly vulnerable to gradual, progressive circulatory failure, and options to reverse this situation are few. A cavopulmonary assist device ...could decongest the venous and lymphatic systems, overcome elevated pulmonary vascular resistance, increase cardiac output, and support some of these patients to heart transplant. This study characterizes the performance and challenges of a novel multilumen cannula coupled to an external blood pump proposed as a potential Fontan cavopulmonary assist strategy.
Computational fluid dynamic simulations were conducted for 3 extracardiac Fontan geometries consisting of 1 idealized model and 2 patient-specific models. A range of physiologic flow rates and pump assist levels were simulated to calculate the pressure gain provided by the multilumen cannula. Hemolysis index was estimated for the idealized model with Lagrangian particle tracking and 2 variations of the power-law. Wall shear stresses were also examined.
Pressure gains up to 4 and 9 mm Hg were achieved for the idealized and patient-specific models, respectively. Pressure gains increased with both higher cardiac output and larger pump intake through the external pump. Flow-weighted hemolysis show hemoglobin damage levels to be several times lower than the 2% threshold at the highest pump intake flow cases. Wall shear stress predictions depict elevated areas in the pulmonary vessels and regions of the cannula device.
The cannula tested in this study shows promise as a percutaneous option to bridge support in some patients with a failing extracardiac Fontan. Limitations identified will be addressed in future design iterations and in ongoing experimental tests.
With access to surgical care, >90% of today's infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop ...strategies for navigating life with a chronic disease.
Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis-an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2-4 hour free-form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation. Recruitment continued until saturation was reached (n = 10).
While each narrative was unique, CHD had a pervasive effect on the autobiography of all participants. Seven themes were discussed consistently: (1) parental/sibling relationships, (2) physical limitations, (3) embarrassment/denial, (4) memories of pediatric health care, (5) transition to adult care, (6) education and career choices, and (7) relationship and reproductive choices. While some of the recalled experiences were negative, all participants also spoke positively about the effect of CHD on their lives.
Adults with CHD provide the voices of expert witnesses; illuminating how the pediatric journey influences their identity, choices, personal relationships and adult health care interactions. These narratives could inform and improve the contemporary care of children with heart disease.
Few topics in adult congenital heart disease have approached the level of scrutiny bestowed on pulmonary valve replacement (PVR) strategies late after tetralogy of Fallot (TOF) repair. Despite the ...successes of primary surgery for TOF, there is a growing group of adults with residual right ventricular outflow tract and pulmonary valve dysfunction. Patients with residual chronic pulmonic regurgitation as a consequence of earlier surgery can later develop symptoms of exercise intolerance and complications including heart failure, tachyarrhythmias, and sudden cardiac death. Optimal timing of PVR has sparked debate, which has catalyzed increasing research efforts over the past decade. Although performance of PVR in the absence of symptoms is currently on the basis of the rationale that achievement of complete reverse remodelling is highly desirable, whether this approach results in improvement in patient outcomes in the long-term has yet to be shown. Surgical PVR and percutaneous pulmonary valve intervention are different techniques with specific advantages and disadvantages that require careful consideration for each individual patient, alongside the need for requisite reinterventions over the course of a patient's lifetime. Criteria pertaining to referral strategies are ever being refined as newer technologies for percutaneous therapies continue to evolve. In this article we review the literature surrounding the indications for, the optimal timing of, and the approaches to pulmonary valve procedures in adults with previously repaired TOF.
Nous avons abordé quelques thèmes sur la cardiopathie congénitale chez l'adulte en ce qui concerne le niveau d'examen accordé aux stratégies de remplacement valvulaire pulmonaire (RVP) bien après la correction de la tétralogie de Fallot (TF). En dépit de la réussite de l'intervention chirurgicale primaire de la TF, de plus en plus d'adultes montrent un dysfonctionnement résiduel de la chambre de chasse du ventricule droit et de la valve pulmonaire. Les patients atteints de régurgitation pulmonaire résiduelle à long terme en raison de l'intervention chirurgicale antérieure peuvent plus tard présenter des symptômes d'intolérance à l'effort et des complications, y compris l'insuffisance cardiaque, les tachyarythmies et la mort cardiaque subite. La controverse entourant le moment optimal du RVP a permis d'accélérer les efforts de recherche au cours de la dernière décennie. Bien que la performance du RVP en l'absence de symptômes repose actuellement sur le principe que la réalisation du remodelage inverse complet est grandement souhaitable, il reste à démontrer si cette approche se traduit par l'amélioration des résultats cliniques à long terme chez les patients. La chirurgie de RVP et l'intervention par voie percutanée de la valve pulmonaire sont des techniques différentes ayant des avantages et des inconvénients propres à chacune qui exigent un examen minutieux de chacun des patients parallèlement à la nécessité des réinterventions requises au cours de la vie du patient. Les critères liés aux stratégies d'orientation sont peaufinés au fur et à mesure que les nouvelles technologies de traitement par voie percutanée évoluent. Dans le présent article, nous passons en revue la littérature relative aux indications, au moment optimal et aux approches chirurgicales en matière d'interventions de la valve pulmonaire chez les adultes ayant déjà subi une correction de la TF.
Abstract Obesity, hypertension, and coronary artery disease are prevalent in the general population and well recognized as contributors to cardiac morbidity and mortality. With surgical and medical ...advances, there is a growing and aging population with congenital heart disease who are also at risk of developing these comorbidities. In addition, some congenital cardiac lesions predispose patients to conditions such as hypertension or coronary artery disease. The effect of these comorbidities on the structurally abnormal heart is not well understood, but might be very important, especially in those with residual abnormalities. Thus, in addition to surveillance for and treatment of late complications it is important for the congenital cardiologist to consider and aggressively manage acquired comorbidities. In this review we explore the prevalence of hypertension, obesity, and coronary artery disease, discuss congenital lesions that predispose to these conditions and review management strategies for this unique population.
Abstract In congenital heart disease (CHD) the right ventricle (RV) dons a variety of hats. It might be situated in the normal subpulmonary position and function as half of a 2-pump circulation, but ...nonetheless be subjected to abnormal loading conditions, surgical interventions, or electrical disturbance. Alternatively, it can be required to generate systemic cardiac output; either in the context of a univentricular circulation or else in concert with a subpulmonary left ventricle. Each role carries different hemodynamic stresses and physiologic demands and the long-term capacity of the RV to withstand the stresses and meet those demands has become recognized as a key contributor to late clinical outcomes. RV failure in CHD usually has an etiology and pathophysiology quite different from RV failure in other circumstances and there is little evidence to support direct transition of treatments well established in acquired heart failure in this patient group. In this review we discuss the requirements for normal RV function, situations in CHD in which the RV is prone to failure, the pathophysiology of RV dysfunction, and potential therapeutic options.
Long-term biventricular systolic performance is a key determinant of clinical outcomes late after tetralogy of Fallot (TOF) repair. A need exists for early indices of both left ventricular (LV) and ...right ventricular (RV) compromise in this population.
Twenty-nine children (age range, 5-18 years) with repaired TOF and 44 healthy controls were prospectively evaluated. M-mode and tissue Doppler data were obtained for each ventricle and the RV outflow tract at rest and during semisupine bicycle exercise. By making measurements of myocardial acceleration during isovolumic contraction during exercise, at increasing heart rates, LV force-frequency curves were constructed. Patients also underwent cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and measurement of serum neurohormonal markers.
Children with repaired TOF had dilated right ventricles (RV end-diastolic volume index = 153 ± 37.3 mL/m(2)) but normal ejection fractions as measured on magnetic resonance imaging (LV ejection fraction = 59.3 ± 6.2%, RV ejection fraction = 50.2 ± 8.5%) and normal serum neurohormonal markers. Detailed resting echocardiography detected abnormal ventricular function, worst in the right ventricle and RV outflow tract. Exercise exacerbated these findings and provoked significant decline in LV indices. The LV force-frequency curves of patients were attenuated, with an early plateau and inadequate increase of isovolumic contraction. Correlations were seen between peak exercise LV isovolumic contraction and percentage predicted peak oxygen uptake (r = 0.51, P = .02), LV and RV ejection fractions (r = 0.41, P = .03), and RV and LV long-axis fractional shortening (r = 0.44, P = .02).
The postsurgical pathophysiology of TOF begins early after repair. At a time when clinically well and while routine indices of heart function remain normal, children with repaired TOF exhibit RV dilatation and subtle, interlinked biventricular abnormalities on resting echocardiography. Exercise echocardiography provides additional information and reveals abnormal LV excitation-contractile coupling that may be linked to impaired exercise capacity.
Background
Mapping and ablation of atrial tachycardia (AT) is commonly performed in lateral tunnel Fontan (LTF) patients, yet there is little information on the need of baffle puncture to access the ...pulmonary venous atrium (PVA). This study aimed to evaluate the most common chamber location of critical sites for majority of AT in LTF patients.
Methods
Consecutive LTF patients underwent catheter-based high-density mapping and ablation of AT from Nov. 2015 to Mar. 2019. Critical sites were identified by a combination of activation and entrainment mapping. Acute procedural success was defined as AT termination with ablation and non-inducibility of any AT. Predictors for ablation failure were evaluated in retrospect.
Results
Fifteen catheter ablation procedures were performed in 9 patients. A total of 15 clinical ATs (mean TCL 369 ± 91 ms) were mapped. The mechanism was macro re-entry in 11 (73%) and micro re-entry in 2. In 11 ATs (73%), 94 ± 5% of tachycardia cycle length (TCL) were mapped inside the tunnel. The commonest site of successful ablation in the tunnel was on the lateral wall (60%). Trans-baffle access was obtained during 5 of 15 procedures (33%). Overall, procedural success was achieved in 9 of 15 procedures (60%). There were no complications. Recurrence of AT was 42% over a follow-up period of 4.3 ± 3.2 years. Faster TCL of 200–300 ms showed a trend towards ablation failure, (OR 17, 95% CI 0.7 to 423,
p
= 0.08).
Conclusions
Catheter ablation can be performed effectively for ATs in LTF patients usually from inside the tunnel. ATs with critical sites in the PVA are uncommon. This information will help plan ablation in LTF patients without resorting to initial trans-baffle access.
Aims
While heart failure (HF) is a leading cause of death in adults with congenital heart disease (ACHD), few studies report contemporary outcomes after the first HF hospitalization. We examined ...outcomes of ACHD patients newly admitted for HF compared with ACHD patients without HF and the general HF population without ACHD.
Methods and results
Using population databases from a single‐payer health system from 1994 to 2018, ACHD patients newly admitted for HF were matched 1:1 to ACHD patients without HF (n = 4030 matched pairs). Similarly, ACHD patients newly admitted for HF were matched 1:1 to HF patients without ACHD (n = 4336 matched pairs). Patients with ACHD and HF (median age 68 years, 45% women) experienced higher mortality in short‐term 30 day adjusted hazard ratio (HR) 4.68, 95% confidence interval (CI) 4.06, 5.43, P < 0.001, near‐term (1 year HR 3.87, 95% CI 3.77, 4.92, P < 0.001), and long‐term (24 year HR 1.59, 95% CI 1.13, 2.36, P = 0.008) follow‐up. Patients with ACHD and HF had fewer baseline cardiovascular comorbidities than non‐ACHD HF but demonstrated higher 30 day (HR 1.56, 95% CI 1.41, 1.73, P < 0.001), 1 year (HR 1.30, 95% CI 1.20, 1.40, P < 0.001), and 24 year (HR 2.40, 95% CI 1.73, 3.38, P < 0.001) mortality. Those with ACHD and HF also exhibited higher cardiovascular readmission rates at 30 days with HRs 9.15 (95% CI; 8.00, 10.48, P < 0.001) vs. ACHD without HF, and 1.71 (95% CI; 1.54, 1.85, P < 0.001) vs. HF without ACHD, and the higher readmission risk extended to 10 year follow‐up.
Conclusions
Adults with congenital heart disease patients with new HF have high risks of death and cardiovascular hospitalization, and preventative strategies to improve outcomes are urgently needed.
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