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Background: There is a paucity of data regarding sex-related differences on cardiac outcomes in the context of transposition of the great arteries (TGA) with a systemic right ventricle and biventricular physiology (sRV-biV). Moreover, the long-term impact of pregnancy on cardiac outcomes remains unknown. Objectives: The purpose of this study was to identify sex-related differences and the influence of pregnancy on cardiac outcomes in TGA sRV-biV population. Methods: A retrospective cohort study was conducted on 213 adults with TGA sRV-biV, 82 (38.4%) women, age 42.6 ± 12.8 years, with a median follow-up of 16 years. Cardiac events, interventions, last follow-up sRV-biV dysfunction, and heart failure (HF) medications were compared between men vs women, and women with vs without pregnancies resulting in live births. Results: Women had a lower incidence of nonsustained ventricular tachycardia (HR: 1.80; 95% CI: 1.04-3.09, P = 0.035) and nonsignificantly fewer HF-related hospitalizations than men (HR: 2.10; 95% CI: 0.95-4.67, P = 0.069) in univariable analysis. At the last follow-up, women had a lower prevalence of moderate to severe sRV-biV dysfunction than men (P < 0.001) and were less frequently prescribed HF therapy. Women had fewer implantable cardioverter-defibrillators for primary prevention than men (P = 0.016), with no difference for secondary prevention. Women who had pregnancies resulting in live births (N = 47), had a high prevalence of cardiac events in the 15 (IQR: 9-28) years following pregnancy with no significant differences with those without (N = 32) pregnancies. Conclusions: Women with a sRV-biV have fewer adverse cardiovascular events than men. Due to sRV-biV, pregnancy remains with high maternal risk but is not associated with worse long-term cardiac outcomes under rigorous multidisciplinary cardio-obstetrical care.
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Background: There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle. Objectives: The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV. Methods: The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America. All components of the composite outcome, that is, atrial arrhythmias, thromboembolic events, cardiac transplantation, and death, were reviewed and classified by a blinded adjudicating committee. Time-to-event analyses were performed that accounted for competing risks. Results: A total of 384 patients were followed for 10.5 ± 5.9 years. The composite outcome occurred in 3.7 vs 1.7 cases per 100 person-years for uRV (N = 171) vs uLV (N = 213), respectively (P < 0.001). In multivariable analyses, uRV conferred a >2-fold higher risk of the composite outcome (HR: 2.17, 95% CI: 1.45-3.45, P < 0.001). In secondary analyses of components of the primary outcome, uRV was significantly associated with a greater risk of cardiac transplantation or death (HR: 9.09, 95% CI: 2.17-38.46, P < 0.001) and atrial arrhythmias (HR: 2.17, 95% CI: 1.20-4.00, P = 0.010) but not thromboembolic events (HR: 1.64, 95% CI: 0.86-3.16, P = 0.131). Conclusions: Fontan patients with uRV vs uLV morphology have a higher incidence of adverse cardiovascular events, including atrial arrhythmia, cardiac transplantation, and all-cause mortality.
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BACKGROUND: Patients with congenital heart disease (CHD) and their parents face challenges throughout their lives that can lead to anxiety lasting into adulthood. We aim to assess the association between perceived parenting practices and anxiety beyond paediatric medical-surgical histories in adults with CHD. METHODS: A cross-sectional study of adults with CHD was conducted at the Montreal Heart Institute (MHI). Perception of parental practices during childhood was retrospectively assessed with the use of validated self-report questionnaires, and anxiety in adulthood was assessed with the use of the Hospital Anxiety and Depression Scale. Sociodemographic and medical information were collected from a questionnaire and medical records. Hierarchic multiple linear regression was conducted. RESULTS: Of the 223 participants, the mean age was 46 ± 14 years and 59% were female. Perceived parenting practices explained more variance (11%) in the anxiety score than paediatric medical-surgical history (2%). In our final model, anxiety was significantly associated with age, parental history of anxiety, and positive parenting practices, but not with overprotection. CONCLUSIONS: Parenting practices are associated with anxiety in adults with CHD beyond paediatric medical-surgical history and sociodemographic. Positive parenting practices may be protective against anxiety in adulthood. Longitudinal studies are needed to determine causality.
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INTRODUCTION: The Fontan procedure is the palliative procedure of choice for patients with single ventricle physiology. Pulmonary vascular disease (PVD) is an important contributor to Fontan circulatory failure. AREAS COVERED: We review the pathophysiology of PVD in patients with Fontan palliation and share our initial experience with optical coherence tomography (OCT) in supplementing standard hemodynamics in characterizing Fontan-associated PVD. In the absence of a sub-pulmonary ventricle, low pulmonary vascular resistance (PVR; ≤2 WU/m2) is required to sustain optimal pulmonary blood flow. PVD is associated with adverse pulmonary artery (PA) remodeling resulting from the non-pulsatile low-shear low-flow circulation. Predisposing factors to PVD include impaired PA growth, endothelial dysfunction, hypercoagulable state, and increased ventricular end-diastolic pressure. OCT parameters that show promise in characterizing Fontan-associated PVD include the PA intima-to-media ratio and wall area ratio (i.e. difference between the whole-vessel area and the luminal area divided by the whole-vessel area). EXPERT OPINION: OCT carries potential in characterizing PVD in patients with Fontan palliation. PA remodeling is marked by intimal hyperplasia, with medial regression. Further studies are required to determine the role of OCT in informing management decisions and assessing therapeutic responses.
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Técnica de Fontan , Cuidados Paliativos , Artéria Pulmonar , Tomografia de Coerência Óptica , Humanos , Técnica de Fontan/efeitos adversos , Técnica de Fontan/métodos , Tomografia de Coerência Óptica/métodos , Artéria Pulmonar/diagnóstico por imagem , Cuidados Paliativos/métodos , Hemodinâmica , Resistência Vascular , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Doenças Vasculares/diagnóstico por imagem , Remodelação Vascular , Circulação PulmonarRESUMO
BACKGROUND: Fenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli. METHODS: A multicentre North American retrospective cohort study was conducted on patients with total cavopulmonary connection Fontan baffle, with and without fenestration. All components of the composite outcome were independently adjudicated. Potential static and time-varying confounders were taken into consideration, along with competing risks. RESULTS: A total of 407 patients were followed for 10.4 (7.1-14.4) years; 70.0% had fenestration of their Fontan baffle. The fenestration spontaneously closed or was deliberately sealed in 79.9% of patients a median of 2.0 years after Fontan completion. In multivariable analysis in which a persistent fenestration was modelled as a time-dependent variable, an open fenestration did not confer a higher risk of the composite outcome (hazard ratio, 1.18; 95% confidence interval, 0.71-1.97; P = 0.521). In secondary analyses, an open fenestration was not significantly associated with components of the primary outcome: that is, mortality or transplantation, atrial arrhythmias, or thromboemboli. However, sensitivity analyses to assess the possible range of error resulting from imprecise dates for spontaneous fenestration closures could not rule out significant associations between an open fenestration and atrial arrhythmias or thromboemboli. CONCLUSIONS: In this multicentre study, no significant association was identified between an open fenestration in the Fontan baffle and major adverse cardiovascular events.
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Disease of the aortic arch, descending thoracic, or thoracoabdominal aorta necessitates dedicated expertise across medical, endovascular, and surgical specialties. Cardiologists, cardiac surgeons, vascular surgeons, interventional radiologists, and others have expertise and skills that aid in the management of patients with complex aortic disease. No specialty is uniformly expert in all aspects of required care. Because of this dispersion of expertise across specialties, an aortic team model approach to decision-making and treatment is advocated. A nonhierarchical partnership across specialties within an interdisciplinary aortic clinic ensures that all treatment options are considered and promotes shared decision-making between the patient and all aortic experts. Furthermore, regionalization of care for aortic disease of increased complexity assures that the breadth of treatment options is available and that favourable volume-outcome ratios for high-risk procedures are maintained. An awareness of best practice care pathways for patient referrals for preventative management, acute care scenarios, chronic care scenarios, and pregnancy might facilitate a more organized management schema for aortic disease across Canada and improve lifelong surveillance initiatives.
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Doenças da Aorta , Especialidades Cirúrgicas , Cirurgiões , Humanos , Radiologia Intervencionista , Canadá , Doenças da Aorta/diagnóstico , Doenças da Aorta/cirurgia , Aorta , Procedimentos Cirúrgicos VascularesRESUMO
The presence of a systemic right ventricle (sRV) with biventricular physiology (biV) is associated with increased patient morbidity and mortality. To date, no pharmacologic therapy for heart failure has been proven effective for patients with systolic dysfunction of the sRV-biV. We designed a randomized, double-blind, placebo-controlled crossover trial to compare sacubitril/valsartan treatment to placebo in adults (aged ≥ 18 years) with moderate-to-severe sRV-biV dysfunction and New York Heart Association functional class II to III symptoms. Two primary efficacy endpoints are assessed in the trial: exercise capacity (submaximal exercise duration) and neurohormonal activation (N-terminal prohormone brain natriuretic peptide). Secondary objectives include assessing a change in the Kansas City Cardiomyopathy Questionnaire score and evaluating the safety and tolerance of sacubitril/valsartan. A 6-week open run-in phase identifies the maximum tolerated dose of sacubitril/valsartan, up to 97 mg/103 mg twice daily. After a 2-week washout period, patients are randomized 1:1 to sacubitril/valsartan treatment vs placebo for a 24-week phase, followed by another 2-week washout period and subsequent crossover to the alternative treatment arm for an additional 24-week phase. Data to assess primary and secondary endpoints are collected at baseline and at the end of each phase. A total of 48 patients is required to provide > 80% power to detect a 30% difference in distance walked and in N-terminal prohormone brain natriuretic peptide levels with sacubitril/valsartan treatment vs placebo, each with a 2-sided P-value of 0.025. In summary, the Prospective Comparison of Angiotensin Receptor-Neprilysin Inhibitor vs Placebo in Patients With Congenital Systemic Right Ventricular Heart Failure Trial (PARACYS-RV) should determine the role of sacubitril/valsartan in treating heart failure in patients with sRV-biV and carries the potential to alter management of this patient population.
La présence d'un ventricule droit systémique (VDs) avec physiologie biventriculaire (PbiV) est associée à une morbidité et une mortalité accrues chez les patients. À ce jour, aucune pharmacothérapie de l'insuffisance cardiaque ne s'est révélée efficace chez les patients atteints d'une dysfonction systolique du VDs-PbiV. Nous avons conçu un essai croisé, à répartition aléatoire et à double insu, contrôlé par placebo pour comparer la bithérapie sacubitril-valsartan au placebo chez les adultes (≥ 18 ans) ayant une dysfonction modérée ou sévère du VDs-PbiV et des symptômes de la classe fonctionnelle II à III de la New York Heart Association. Deux paramètres d'évaluation principaux de l'efficacité sont définis pour l'essai : tolérance à l'effort (durée d'effort sous-maximal) et activation neurohormonale (propeptide natriurétique de type B N-Terminal [NT-proBNP]). La mesure d'une variation du score au questionnaire sur la cardiomyopathie de Kansas City de même que l'évaluation de l'innocuité et de la tolérance de la bithérapie sacubitril-valsartan sont des objectifs secondaires. Une phase préparatoire de six semaines en mode ouvert permet d'établir la dose maximale tolérée de sacubitril-valsartan, jusqu'à concurrence de 97 mg/103 mg deux fois par jour. Après une période de repos thérapeutique de deux semaines, les patients sont affectés au hasard, dans un rapport 1:1, à la bithérapie sacubitril-valsartan ou au placebo pendant une phase de traitement de 24 semaines, suivie d'une autre période de repos thérapeutique de deux semaines et d'un passage subséquent à l'autre groupe de traitement pendant une phase additionnelle de 24 semaines. Les données sur les paramètres d'évaluation principaux et secondaires sont recueillies au début de l'essai et à la fin de chaque phase. Il faut un total de 48 patients afin d'obtenir une puissance supérieure à 80 % pour détecter une différence de 30 % entre la bithérapie sacubitril-valsartan et le placebo quant à la distance parcourue à la marche et aux taux de NT-proBNP, la valeur p bilatérale étant de 0,025 pour les deux valeurs. En résumé, l'essai PARACYS-RV (Prospective Comparison ofAngiotensinReceptor-Neprilysin Inhibitor vs Placebo in Patients WithCongenital SystemicRightVentricular Heart Failure) doit déterminer le rôle de la bithérapie sacubitril-valsartan dans le traitement de l'insuffisance cardiaque chez les patients ayant un VDs-PbiV et pourrait modifier la prise en charge de cette population de patients.
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Background: Up to one-half of adults with congenital heart disease (CHD) experience psychological distress, including anxiety. Objectives: This paper sought to: 1) assess the contribution of illness perception in explaining anxiety symptoms beyond sociodemographic and medical variables in adults with CHD; and 2) investigate the potential mediating effect of coping style. Methods: CHD adult patients were recruited at Montreal Heart Institute between June 2019 and April 2021 for this cross-sectional study. Participants responded to self-reported questionnaires (Hospital Anxiety and Depression Scale, Brief Illness Perception Questionnaire, and Brief COPE). Medical characteristics (CHD complexity, NYHA functional class, and cardiac devices) were collected from medical records. We conducted hierarchical multiple linear regression and mediation analyses. Results: Of the 223 participants (mean age 46 ± 14 years, 59% women), 15% had clinically significant anxiety symptoms. Medical and sociodemographic variables explained 15% of the variation in anxiety symptoms. Adding illness perception explained an additional 18% of the variation in anxiety. This R2 change was significant (F[1,188] = 49.06, P < 0.0001). Illness perception explained more variance (18%) than medical and sociodemographic variables combined. A more threatening perception of illness was associated with greater anxiety symptoms (ß = 0.45, P < 0.0001). Furthermore, illness perception was associated with coping, which was linked to reduced anxiety symptoms. Coping response style accounted for 20% of the total effect of illness perception on anxiety. Conclusions: Illness perception and coping are associated with anxiety in adults with CHD. Future initiatives should assess whether targeting these potentially modifiable factors effectively prevents or mitigates anxious symptoms in adults with CHD.
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Electric interventions are used to terminate arrhythmia. However, myocardial injury from the electrical intervention can follow unique pathways and it is unknown how this affects regional ventricular function. This study investigated the impact of transthoracic electrical shocks on systolic and diastolic myocardial deformation. Ten healthy anaesthetized female swine received five transthoracic shocks (5 × 200 J) and six controls underwent a cardiovascular magnetic resonance exam prior to and 5 h after the intervention. Serial transthoracic shocks led to a global reduction in both left (LV, - 15.6 ± 3.3% to - 13.0 ± 3.6%, p < 0.01) and right ventricular (RV, - 16.1 ± 2.3% to - 12.8 ± 4.2%, p = 0.03) peak circumferential strain as a marker of systolic function and to a decrease in LV early diastolic strain rate (1.19 ± 0.35/s to 0.95 ± 0.37/s, p = 0.02), assessed by feature tracking analysis. The extent of myocardial edema (ΔT1) was related to an aggravation of regional LV and RV diastolic dysfunction, whereas only RV systolic function was regionally associated with an increase in T1. In conclusion, serial transthoracic shocks in a healthy swine model attenuate biventricular systolic function, but it is the acute development of regional diastolic dysfunction that is associated with the onset of colocalized myocardial edema.
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Traumatismos Cardíacos , Disfunção Ventricular Esquerda , Feminino , Suínos , Animais , Ventrículos do Coração , Sístole , Diástole , Coração , Imageamento por Ressonância Magnética , Função Ventricular EsquerdaRESUMO
BACKGROUND: Vitamin K antagonists (VKAs) are frequently prescribed to patients with congenital heart disease (CHD) for atrial arrhythmias or Fontan palliation, but there is a paucity of data regarding time spent in the therapeutic range (TTR). We sought to determine the TTR in patients with CHD and atrial arrhythmias or Fontan palliation prescribed VKAs and explore associations with thromboembolic and bleeding events. METHODS: A multicentre North American cohort study was conducted on patients with CHD who received VKAs for sustained atrial arrhythmia or Fontan palliation. TTR was calculated using the Rosendaal linear interpolation method. Generalized estimating equations were used to explore factors associated with time outside the therapeutic range. RESULTS: A total of 567 patients, aged 33 ± 17 years, 56% female, received VKAs for 11.5 ± 8.4 years for atrial arrhythmias (63.0%) or Fontan palliation (58.0%). CHD was simple, moderate, and complex in 10.8%, 20.3%, and 69.0%, respectively. Site investigators perceived good control over international normalized ratio (INR) levels in most patients (75.3%), with no or minor compliance or adherence issues (85.6%). The mean TTR was 41.9% (95% confidence interval [CI], 39.0%-44.8%). Forty-seven (8.3%) and 34 (6.0%) patients had thromboembolic and bleeding events, respectively. Thromboembolic events were associated with a higher proportion of time below the therapeutic range (31.3% vs 19.1%, P = 0.003) and bleeding complications with a higher proportion of time above the therapeutic range (32.5% vs 19.5%, P = 0.006). CONCLUSIONS: Patients with CHD who receive VKAs spend < 42% of their time with INR levels in the therapeutic range, with repercussions regarding thromboembolic and bleeding complications.
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Fibrilação Atrial , Cardiopatias Congênitas , Tromboembolia , Humanos , Feminino , Masculino , Vitamina K , Estudos de Coortes , Fibrilação Atrial/complicações , Anticoagulantes/uso terapêutico , Coeficiente Internacional Normatizado , Fibrinolíticos/uso terapêutico , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Tromboembolia/prevenção & controle , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Cardiopatias Congênitas/complicaçõesRESUMO
Fontan palliation results in a hemodynamically complex circulation with multisystem consequences, which in the long term adversely affect many body processes. Systemic venous hypertension, nonpulsatile low-shear pulmonary blood flow, and low cardiac output are the 3 main characteristics of a Fontan circulation, leading to unavoidable slowly progressive failure. An appreciation of how the hemodynamics of a Fontan circulation change with time and relate to the various modes of Fontan circulatory failure is important. Accurate hemodynamic assessment aid this understanding and may permit early identification of potentially treatable drivers of decline. While no evidence-based or guideline-directed pharmacologic management strategy has been established in Fontan patients, understanding the hemodynamics of Fontan circulation failure will assist in the rational selection of potentially helpful drug therapies for individual patients. In this review, we present hemodynamic concepts of the optimal Fontan physiology and Fontan circulatory failure, review practical aspects of invasive hemodynamic assessment, and discuss the role of drug therapies in increasing systemic venous blood flow return and decreasing ventricular filling pressures in Fontan circulation. Often complementary to catheter-based or surgical interventions, pharmacologic management aims at preserving patency of the circuit, adequate systolic and diastolic ventricular function, atrioventricular valve function, an unobstructed ventricular outflow tract, and pulmonary vascular integrity in order to maintain an acceptable cardiac output.
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Técnica de Fontan , Cardiopatias Congênitas , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Hemodinâmica/fisiologia , Humanos , Circulação Pulmonar , Função VentricularRESUMO
Cardiac diffusion tensor imaging (DTI) is an emerging technique for the in vivo characterisation of myocardial microstructure, and there is a growing need for its validation and standardisation. We sought to establish the accuracy, precision, repeatability and reproducibility of state-of-the-art pulse sequences for cardiac DTI among 10 centres internationally. Phantoms comprising 0%-20% polyvinylpyrrolidone (PVP) were scanned with DTI using a product pulsed gradient spin echo (PGSE; N = 10 sites) sequence, and a custom motion-compensated spin echo (SE; N = 5) or stimulated echo acquisition mode (STEAM; N = 5) sequence suitable for cardiac DTI in vivo. A second identical scan was performed 1-9 days later, and the data were analysed centrally. The average mean diffusivities (MDs) in 0% PVP were (1.124, 1.130, 1.113) x 10-3 mm2 /s for PGSE, SE and STEAM, respectively, and accurate to within 1.5% of reference data from the literature. The coefficients of variation in MDs across sites were 2.6%, 3.1% and 2.1% for PGSE, SE and STEAM, respectively, and were similar to previous studies using only PGSE. Reproducibility in MD was excellent, with mean differences in PGSE, SE and STEAM of (0.3 ± 2.3, 0.24 ± 0.95, 0.52 ± 0.58) x 10-5 mm2 /s (mean ± 1.96 SD). We show that custom sequences for cardiac DTI provide accurate, precise, repeatable and reproducible measurements. Further work in anisotropic and/or deforming phantoms is warranted.
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Imagem de Tensor de Difusão , Coração , Anisotropia , Imagem de Tensor de Difusão/métodos , Coração/diagnóstico por imagem , Imagens de Fantasmas , Reprodutibilidade dos TestesRESUMO
RATIONALE: Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing. OBJECTIVES: Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy. STUDY DESIGN: This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared with t-test, Mann-Whitney and Fisher's exact tests when appropriate. RESULTS: Eighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15; p = .006). Preterm birth occurred in more than 50% of cases, resulting from induced delivery for a maternal (40%) or fetal reason (60%). Most deliveries were late preterm between 34 and 36 6/7 weeks. In both groups, birthweight was mainly distributed below the 50th percentile (89%) and 35% of neonates were born small for gestational age defined as a birthweight below the 10th percentile. Most severe cases of small for gestational age (birthweight under the 5th percentile) were found in patients with treated obstructive hypertrophic cardiomyopathy. CONCLUSION: Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a sub-group of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition.
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Cardiomiopatia Hipertrófica , Insuficiência Cardíaca , Nascimento Prematuro , Feminino , Humanos , Recém-Nascido , Gravidez , Peso ao Nascer , Estudos Retrospectivos , Placenta , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/epidemiologia , Cardiomiopatia Hipertrófica/diagnóstico , Retardo do Crescimento FetalRESUMO
OBJECTIVE: The Ross procedure is an excellent option for children or young adults who need aortic valve replacement because it can restore survival to that of the normal aged-matched population. However, autograft remodeling can lead to aneurysmal formation and reoperation, and the biomechanics of this process is unknown. This study investigated postoperative autograft remodeling after the Ross procedure by examining patient-specific autograft wall stresses. METHODS: Patients who have undergone the Ross procedure who had intraoperative pulmonary root and aortic specimens collected were recruited. Patient-specific models (n = 16) were developed using patient-specific material property and their corresponding geometry from cine magnetic resonance imaging at 1-year follow-up. Autograft ± Dacron for aneurysm repair and ascending aortic geometries were reconstructed to develop patient-specific finite element models, which incorporated material properties and wall thickness experimentally measured from biaxial stretching. A multiplicative approach was used to account for prestress geometry from in vivo magnetic resonance imaging. Pressure loading to systemic pressure (120/80) was performed using LS-DYNA software (LSTC Inc, Livermore, Calif). RESULTS: At systole, first principal stresses were 809 kPa (25%-75% interquartile range, 691-1219 kPa), 567 kPa (485-675 kPa), 637 kPa (555-755 kPa), and 382 kPa (334-413 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Second principal stresses were 360 kPa (310-426 kPa), 355 kPa (320-394 kPa), 272 kPa (252-319 kPa), and 184 kPa (147-222 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Mean autograft diameters were 29.9 ± 2.7 mm, 38.3 ± 5.3 mm, and 26.6 ± 4.0 mm at the sinotubular junction, sinuses, and annulus, respectively. CONCLUSIONS: Peak first principal stresses were mainly located at the sinotubular junction, particularly when Dacron reinforcement was used. Patient-specific simulations lay the foundation for predicting autograft dilatation in the future after understanding biomechanical behavior during long-term follow-up.
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Insuficiência da Valva Aórtica , Valva Pulmonar , Humanos , Criança , Adulto Jovem , Idoso , Autoenxertos , Transplante Autólogo/efeitos adversos , Polietilenotereftalatos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Pulmonar/cirurgia , Valva Pulmonar/transplanteRESUMO
BACKGROUND: Studies suggest that patients undergoing the Ross procedure for aortic insufficiency are at greater risk of autograft dilatation than those with aortic stenosis. By using a tailored Ross technique to mitigate autograft dilatation in patients with aortic insufficiency, we aimed to compare the biomechanical and morphologic remodeling of the autograft at 1 year between patients with aortic insufficiency and patients with aortic stenosis. METHODS: A total of 210 patients underwent a Ross procedure (2011-2016). Of those, 86 patients (mean age 43 ± 13 years; 32% were female) completed preoperative and postoperative cardiovascular magnetic resonance imaging. A total of 71 studies were suitable for analysis: 41 patients with aortic stenosis and 30 patients with aortic insufficiency. Nine healthy adults were used as controls. Autograft root dimensions, individual sinus volumes, and distensibility were measured using cardiovascular magnetic resonance. RESULTS: At 1 year, there was no difference in autograft root dimensions between patients with aortic stenosis (mean annulus 25.1 ± 3.1 mm and sinus diameters 35 ± 4.1 mm) and patients with aortic insufficiency (26.6 ± 3 mm and 37.1 ± 3.5 mm; P = .12 and .06, respectively). Relative sinus of Valsalva volumes were symmetrical in the aortic stenosis (right 34.8% ± 4%, left 33.7% ± 3.5%, noncoronary 31.4% ± 3.2%) and aortic insufficiency groups (34.8% ± 3.9%, 33.8% ± 2.8%, 31.3% ± 3.7%, P = .85, .92, and .82), and similar to those of healthy adults. Aortic root distensibility was reduced in both groups compared with healthy adults (P = .003), but was similar between aortic stenosis (3.12 ± 1.58 × 10-3 mm Hg-1) and aortic insufficiency (3.04 ± 1.15 × 10-3 mm Hg-1; P = .9). CONCLUSIONS: Using a tailored technique, there were no differences in the morphologic or biomechanical remodeling of the autograft root 1 year after the Ross procedure between patients with aortic stenosis and patients with aortic insufficiency. However, autograft roots are stiffer than native aortic roots.
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Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Imagem Cinética por Ressonância Magnética , Complicações Pós-Operatórias/diagnóstico por imagem , Valva Pulmonar/transplante , Adulto , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/fisiopatologia , Autoenxertos , Bioprótese , Estudos de Casos e Controles , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Coronary vascular function is related to adverse outcomes following cardiac transplantation (CTx) in patients with or without cardiac allograft vasculopathy (CAV). The noninvasive assessment of the myocardial vascular response using oxygenation-sensitive cardiac magnetic resonance (OS-CMR has not been investigated in stable long-term CTx recipients). METHODS: CTx patients were prospectively recruited to complete a CMR study with a breathing maneuver of hyperventilation followed by a voluntary apnea. Changes in OS-sensitive signal intensity reflecting the myocardial oxygenation response were monitored and expressed as % change in response to these breathing maneuvers. Myocardial injury was further investigated with T2-weighted imaging, native and postcontrast T1 measurements, extracellular volume measurements, and late gadolinium enhancement. RESULTS: Forty-six CTx patients with (n = 23) and without (n = 23) CAV, along with 25 healthy controls (HC), were enrolled. The OS response was significantly attenuated in CTx compared with HC at the 30-second time-point into the breath-hold (2.63% ± 4.16% versus 6.40% ± 5.96%; P = 0.010). Compared with HC, OS response was lower in CTx without CAV (2.62% ± 4.60%; P < 0.05), while this response was further attenuated in patients with severe CAV (grades 2-3, -2.24% ± 3.65%). An inverse correlation was observed between OS-CMR, ventricular volumes, and diffuse fibrosis measured by extracellular volume mapping. CONCLUSIONS: In heart transplant patients, myocardial oxygenation is impaired even in the absence of CAV suggesting microvascular dysfunction. These abnormalities can be identified by oxygenation-sensitive CMR using simple breathing maneuvers.
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Doença da Artéria Coronariana/diagnóstico por imagem , Transplante de Coração/efeitos adversos , Imagem Cinética por Ressonância Magnética , Miocárdio/metabolismo , Consumo de Oxigênio , Adulto , Idoso , Suspensão da Respiração , Estudos de Casos e Controles , Doença da Artéria Coronariana/etiologia , Doença da Artéria Coronariana/metabolismo , Feminino , Fibrose , Humanos , Hiperventilação , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Tempo , Resultado do TratamentoAssuntos
Transtorno Autístico/genética , Canais de Cálcio Tipo L/genética , Cardiomiopatias/genética , Mutação com Ganho de Função/genética , Estudos de Associação Genética , Predisposição Genética para Doença , Síndrome do QT Longo/genética , Sindactilia/genética , Adolescente , Transtorno Autístico/diagnóstico por imagem , Transtorno Autístico/fisiopatologia , Sequência de Bases , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/fisiopatologia , Pré-Escolar , Eletrocardiografia , Fenômenos Eletrofisiológicos , Humanos , Síndrome do QT Longo/diagnóstico por imagem , Síndrome do QT Longo/fisiopatologia , Sindactilia/diagnóstico por imagem , Sindactilia/fisiopatologiaRESUMO
BACKGROUND: Pulmonary vein (PV) stenosis is a complication of atrial fibrillation (AF) ablation. The incidence of PV stenosis after routine post-ablation imaging remains unclear and is limited to single-centre studies. Our objective was to determine the incidence and predictors of PV stenosis following circumferential radiofrequency ablation in the multicentre Adenosine Following Pulmonary Vein Isolation to Target Dormant Conduction Elimination (ADVICE) trial. METHODS: Patients with symptomatic AF underwent circumferential radiofrequency ablation in one of 13 trial centres. Computed tomographic (CTA) or magnetic resonance (MRA) angiography was performed before ablation and 90 days after ablation. Two blinded reviewers measured PV diameters and areas. PVs with stenosis were classified as severe (> 70%), moderate (50%-70%), or mild (< 50%). Predictors of PV stenosis were identified by means of multivariable logistic regression. RESULTS: A total of 197 patients (median age 59.5 years, 29.4% women) were included in this substudy. PV stenosis was identified in 41 patients (20.8%) and 47 (8.2%) of 573 ablated PVs. PV stenosis was classified as mild in 42 PVs (7.3%) and moderate in 5 PVs (0.9%). No PVs had severe stenosis. Both cross-sectional area and diameter yielded similar classifications for severity of PV stenosis. Diabetes was associated with a statistically significant increased risk of PV stenosis (OR 4.91, 95% CI 1.45-16.66). CONCLUSIONS: In the first systematic multicentre evaluation of post-ablation PV stenosis, no patient acquired severe PV stenosis. Although the results are encouraging for the safety of AF ablation, 20.8% of patients had mild or moderate PV stenosis, in which the long-term effects are unknown.