RESUMO
The aim of this study was to evaluate the accuracy of the KardiaMobile (KM) device in adults with a Fontan palliation, and to assess the KM function as a screening tool for atrial arrhythmias. While patient driven electrocardiogram (EKG) devices are becoming a validated way to evaluate cardiac arrhythmias, their role for patients with congenital heart disease is less clear. Patients with single ventricle Fontan palliation have a high prevalence of atrial arrhythmias and represent a unique cohort that could benefit from early detection of atrial arrhythmias. This single center prospective study enrolled adult patients with Fontan palliation to use the KM heart rhythm monitoring device for both symptomatic episodes and asymptomatic weekly screening over a 1-year period. Accuracy was assessed by comparing the automatic KM interpretation (KM-auto) to an electrophysiologist overread (KM-EP) and traditional EKG. Fifty patients were enrolled and 510 follow-up transmissions were received. The sensitivity and specificity of enrollment KM-auto compared to EKG was 65% and 100%, respectively. The sensitivity and specificity of enrollment KM-auto compared to the KM-EP was 75% and 96%, respectively. In the adult Fontan palliation, the accuracy of the KM device to detect a normal rhythm was reliable and best with a physician overread. Abnormal or uninterpretable KM-auto device interpretations, symptomatic transmissions, and any transmissions with a high heart rate compared to a patient's normal baseline should warrant further review.
RESUMO
Heart failure and arrhythmias represent 2 major causes of mortality and morbidity in adults with congenital heart disease. Arrhythmias and heart failure are interdependent, and one may exacerbate the other. Treatment of one also has a positive impact on the other. Management approaches need to be multifaceted, including pharmacotherapy, optimization of hemodynamic status with catheter-based or surgical interventions, and specific management of arrhythmia with device or catheter ablation therapy.
Assuntos
Ablação por Cateter , Cardiopatias Congênitas , Insuficiência Cardíaca , Adulto , Humanos , Cardiopatias Congênitas/terapia , Cardiopatias Congênitas/cirurgia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/cirurgia , Ablação por Cateter/efeitos adversosRESUMO
BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.
Assuntos
Insuficiência da Valva Pulmonar , Estenose da Valva Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia , Deformação Longitudinal Global , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Volume Sistólico , Estudos Retrospectivos , Função Ventricular Direita , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/diagnóstico por imagemRESUMO
Arrhythmias are a major cause of morbidity and mortality in repaired Tetralogy of Fallot (rTOF). However, predicting those at risk for life-threatening ventricular arrhythmias (VA) remains difficult. Many centers approach risk assessment at the time of surgical pulmonary valve intervention. Increasing numbers of patients have undergone transcatheter pulmonary valve replacement (TPVR), yet there are no studies evaluating VA in rTOF undergoing TPVR and the approach to risk assessment for these patients. A single center retrospective study was performed. The institutional interventional database was queried to identify all adults ≥ 18 years of age with rTOF status who underwent TPVR from 2010 to 2019. A total of 81 patients with rTOF underwent TPVR from 2010 to 2019. Mean age at time of TPVR was 27 ± 13 years; follow up after TPVR was 6.4 ± 3.1 years. VA events occurred in 4 patients (5%). There was no significant difference in current era VA risk factors in rTOF patients between the VA event group and the non-VA event group. VA risk in this cohort of rTOF with TPVR was 5%, comparable to that reported in current era surgical cohort with similar follow up. Multi-center agreement on risk assessment protocol is needed for future studies.
Assuntos
Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Adulto , Humanos , Adolescente , Adulto Jovem , Valva Pulmonar/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Estudos Retrospectivos , Cateterismo Cardíaco/métodos , Resultado do Tratamento , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/cirurgiaRESUMO
Introduction: The COVID-19 pandemic has resulted in increased use of telemedicine. There are limited data on patient experience with telemedicine in adults with congenital heart disease (ACHD). We hypothesized that due to their complex medical history, ACHD would prefer in-person clinic visits over telemedicine. Methods: We conducted a nurse-administered telephone survey based on Agency for Healthcare Research and Quality recommendations to assess patient experience after ACHD telemedicine visits in the early part of the pandemic from March 2020 to June 2020. Results: Of 216 ACHD who had telemedicine visits, 136 (63%) agreed to participate in the survey. Mean age was 45 ± 18 years, majority (65%) being video encounters. Most (98%) patients expressed that the telemedicine visit was successful in addressing their health care needs. Only 21 (15%) patients reported technical issues. Most patients (76%) preferred telemedicine given testing was provided separately, 25 (18%) preferred in-person clinic visits, and 8 (6%) had no preference. Of the 25 patients over 65 years, 19 (76%) would choose telemedicine over the in-person clinic, and only 1 patient reported technical difficulties. Conclusion: ACHD reported a positive experience with telemedicine. Technical limitations were infrequent even among the elderly. The majority of patients felt that their health care needs were addressed and would consider telemedicine over clinic visits. These data are important as payors move away from supporting telemedicine and hospitals restructure, with and without ongoing COVID-19 concerns.
Assuntos
COVID-19 , Cardiopatias Congênitas , Telemedicina , Adulto , Humanos , Idoso , Pessoa de Meia-Idade , COVID-19/epidemiologia , Pandemias , Satisfação do Paciente , Instituições de Assistência Ambulatorial , Cardiopatias Congênitas/terapia , Avaliação de Resultados da Assistência ao PacienteRESUMO
BACKGROUND: Ablation of atrial tachyarrhythmia in adults with congenital heart disease (ACHD) is challenging because of complex anatomy and high scar burden. We proposed that the addition of high-density mapping with the PentaRay® (Biosense Webster, Inc) mapping catheter (EAM+P) to 3-dimensional electroanatomic mapping (EAM) allows for rapid acquisition of high-resolution maps and shorter procedure times. METHODS: In this single-center, retrospective cohort study of patients with ACHD who underwent atrial arrhythmia ablation, patients were divided those who underwent ablation with EAM and those who underwent ablation with EAM+P. RESULTS: Fifteen ablations were performed in 13 patients using standard EAM, and 11 ablations were performed in 10 patients using EAM+P. There was no difference in mean age or complexity of congenital heart disease. The procedure duration was 1.5 times longer in the EAM than in the EAM+P group (P = .015). The dose area product was 12 times higher in the EAM than in the EAM+P group (P = .001). A higher number of venous access sites were used for EAM cases than for EAM+P cases (P = .008). Acute success rates of ablation and recurrence rates at 1 year were similar in the 2 groups. There were no procedure-related complications in either group. CONCLUSION: This is the first study to evaluate the use of the PentaRay® high-density mapping catheter for ablation of atrial tachyarrhythmia in patients with ACHD. The use of the PentaRay® high-density mapping catheter results in shorter procedure time, decreased radiation exposure, and fewer venous access sites.
Assuntos
Fibrilação Atrial , Ablação por Cateter , Cardiopatias Congênitas , Adulto , Humanos , Estudos Retrospectivos , Resultado do Tratamento , Ablação por Cateter/efeitos adversos , Ablação por Cateter/métodos , Taquicardia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/cirurgia , Catéteres , Fibrilação Atrial/cirurgiaRESUMO
BACKGROUND: Women with cardiomyopathy (CM) are often advised against pregnancy due to risk for major adverse cardiovascular events (MACE). However, the impact of CM subtype on maternal MACE is not understood, and so we sought to evaluate the influence of CM phenotype on maternal outcomes, as well as the effect on immediate and late left ventricular function. METHODS: We evaluated all pregnant women in our high-risk maternal cardiovascular programme (2009-2019). Composite maternal MACE included: death, inotrope use, left ventricular assist device, orthotopic heart transplant and/or escalation in transplant listing status, acute decompensated heart failure and sustained ventricular arrhythmia. RESULTS: Among 875 women followed, 32 had CM (29±7 years old, left ventricular ejection fraction (LVEF) 41%±12%): 3 ischaemic CM (ICM), 10 peripartum CM (PPCM) and 19 non-ICM (NICM). MACE events occurred in 6 (18%) women (PPCM: 2 (33%), NICM: 4 (67%)). There was no difference in LVEF at baseline, however, women with MACE had significantly lower LVEF both early (LVEF: 27±5% vs . 41±2%, p<0.05) and late post partum (LVEF: 28±5% vs . 44±2%, p<0.01). CONCLUSIONS: In this contemporary cohort of women with CM, maternal MACE rates were lower than previously reported, and were less common in PPCM as compared with ICM and NICM. Heart function in women with MACE was negatively impacted immediately after delivery and in late postpartum follow-up, suggesting that pregnancy itself likely has influence on future left ventricular function in women with underlying CM.
Assuntos
Insuficiência Cardíaca/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Complicações Cardiovasculares na Gravidez/fisiopatologia , Volume Sistólico/fisiologia , Centros de Atenção Terciária/estatística & dados numéricos , Função Ventricular/fisiologia , Ecocardiografia , Feminino , Saúde Global , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética , Morbidade/tendências , Período Periparto , Período Pós-Parto , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/epidemiologia , Resultado da Gravidez , Fatores de RiscoRESUMO
BACKGROUND: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhythmias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQTM , have been useful in long-term monitoring for arrhythmias in adults with cryptogenic stroke. OBJECTIVE: We propose the Reveal LINQTM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients. METHODS: This is a single center retrospective review of Reveal LINQTM use in ACHD patients from 2014-2017. Medical records were reviewed to determine cardiac diagnosis, indication for implant, ILR findings, and changes in management. RESULTS: Twenty-two patients, median age 25 years, underwent ILR implantation. ILR findings resulted in change in management in nine (41%) patients. One-third (3/9) of the patients with clinically relevant events were asymptomatic. Patients with Fontan palliation had the highest number of pertinent positive events (57%). ACHD physiologic class D patients were more likely to have a positive finding (P = .034) compared to other physiologic classes. Majority (75%) of patients with positive events had arrhythmias documented on ILR which were not demonstrated on prior Holter/event monitors. Pertinent negative event occurred in one patient with Fontan palliation (5%) who had syncope corresponding to sinus rhythm. CONCLUSION: ILRs are a useful adjunct for arrhythmia monitoring in the ACHD population with clinically relevant events in 41% of patients. A special consideration for ILRs could be made for high-risk asymptomatic patients.
Assuntos
Arritmias Cardíacas/diagnóstico , Eletrocardiografia/instrumentação , Eletrodos Implantados , Cardiopatias Congênitas/complicações , Frequência Cardíaca/fisiologia , Monitorização Fisiológica/métodos , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/fisiopatologia , Desenho de Equipamento , Feminino , Seguimentos , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Adulto JovemRESUMO
Cardiac rhabdomyoma is the most common neonatal cardiac tumor and is typically associated with tuberous sclerosis complex (TSC). Although these tumors may naturally regress, some patients require surgical resection because of cardiac instability. If not fully resected, patients may also require medical therapy to improve their hemodynamics. Everolimus, a mammalian target of rapamycin inhibitor, has shown promise in reducing rhabdomyoma in patients with TSC, but the drug's impact in patients without TSC has not been reported. Monitoring of tumor response has typically been limited to echocardiograms, which is not ideal given inherent difficulties in three-dimensional measurements. We report a case of sporadic cardiac rhabdomyoma in a neonate treated with everolimus resulting in tumor regression as documented by cardiac MRI. While on everolimus, our patient had an increased incidence of a preexisting arrhythmia, which resolved with planned cessation of therapy, suggesting that close monitoring is imperative in patients with arrhythmia.