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1.
Pediatr Cardiol ; 44(8): 1658-1666, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37624409

RESUMO

The aim of this study is to analyze the relationship between QRS duration after pulmonary valve replacement (PVR) and ventricular arrhythmias (VA) in patients with repaired tetralogy of Fallot (ToF). ToF patients may face complications such as heart failure and VA after primary repair, often mitigated by PVR. Prior studies have shown a decrease in QRS duration and right ventricular (RV) size following PVR. It remains unclear whether a lack of QRS duration reduction identifies patients at risk of VA. We retrospectively identified adult patients with repaired ToF who underwent surgical or transcatheter PVR. EKG data (pre-PVR, 30 days to 1-year post-PVR, and closest to CMR) was collected. The primary endpoint was sustained ventricular tachycardia (VT), ICD shock for sustained VT, or inducible VT on EP study. 85 patients were included (median follow-up 3.6 years; median age 34 years; 51% females). The primary outcome was noted in 8 patients. Mean QRS duration decreased by 5 ms following PVR (p = 0.0001). Increased age at PVR, QRS ≥ 180 ms post-PVR, no reduction in QRS after PVR, and a history of VT were associated with higher risk of the primary endpoint. The change in QRS was linearly correlated with the change in RVEDVi (R = 0.66). Adults with repaired ToF experience a reduction in QRS duration post-PVR that correlates with the change of the RV size. Patients with QRS ≥ 180 ms post-PVR, no reduction in QRS, increased age at repair, and a history of VT are at risk for recurrent VT and warrant closer monitoring/ICD consideration.


Assuntos
Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Pulmonar , Valva Pulmonar , Taquicardia Ventricular , Tetralogia de Fallot , Feminino , Adulto , Humanos , Masculino , Valva Pulmonar/cirurgia , Estudos Retrospectivos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Resultado do Tratamento , Arritmias Cardíacas , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/cirurgia
2.
Heart Lung Circ ; 31(7): 964-973, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35279372

RESUMO

INTRODUCTION: Patients with adult congenital heart disease (ACHD) who have an anatomic right ventricle supporting the systemic circulation have increased mortality and morbidity from heart failure (HF). Angiotensin receptor-neprilysin inhibitors (ARNI) have emerged as a standard of therapy for adults with HF. However, the effects of this therapy have not been extensively studied in ACHD patients, especially those with systemic right ventricle (SRV). HYPOTHESIS: ARNIs are associated with subjective and objective improvement in SRV patients. METHODS: Eighteen (18) SRV patients were prescribed ARNI at our institution in the last 5 years. Data before and during treatment, including demographics, medical history, New York Heart Association functional class (NYHA FC), labs, cardiac computed tomography (CT) or magnetic resonance imaging (MRI), echocardiographic measurements, cardiopulmonary stress test (CPET), and hospitalisation for HF were obtained by review of the electronic medical record. Statistical analysis was performed using paired t and Wilcoxon rank sum tests. RESULTS: Eighteen (18) SRV patients (mean age 40 yrs, 72% male) were treated with ARNI (median duration 13 mo) in addition to other HF medications. All patients tolerated ARNI without symptomatic or asymptomatic hypotension or worsening kidney function. High ARNI dose (97/103 mg) was achieved in three (17%) patients, and moderate (49/51 mg) in three (17%). At baseline, nine patients were NYHA FC 2, seven FC 3, and two FC 4. Mean baseline cardiopulmonary exercise testing (CPET) and echocardiographic data were: oxygen uptake (VO2) 18 mL/kg/min, minute ventilation/carbon dioxide (VE/VCO2) 38, right ventricular ejection fraction (RVEF) 32%, fractional area change (FAC) 21%. Significant tricuspid regurgitation was present in 33% (28% moderate, and 5% severe) and mean tricuspid annular plane systolic excursion (TAPSE) was 9.4 mm. With treatment, there was no statistically significant difference in blood pressure, labs, testing, or imaging. There was a statistically significant improvement in median NYHA FC (2 vs 2.5, p=0.005). When compared to an equal pre-ARNI median timeframe, there was a noted decrease in cardiac hospitalisation (4 vs 9) that did not reach statistical significance (p=0.313). CONCLUSION: In adult patients with failing systemic right ventricle, ARNI is safe and well tolerated. Their use is associated with improvement in functional status. Prospective studies on a larger group of patients are warranted to better understand the causes of this improvement.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Adulto , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/tratamento farmacológico , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Neprilisina/uso terapêutico , Estudos Prospectivos , Receptores de Angiotensina/uso terapêutico , Volume Sistólico , Função Ventricular Direita
3.
J Invasive Cardiol ; 36(1)2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38224293

RESUMO

Congenital tricuspid valve stenosis is extremely rare. We describe 2 cases of patients with adult congenital heart disease with hypoplastic tricuspid valve annulus who were symptomatic from annular- and leaflet-level tricuspid stenosis. The patients underwent transcatheter balloon valvuloplasty with good clinical outcomes. An extensive literature review and analysis of various procedural strategies suggests that percutaneous balloon valvuloplasty may be a reasonable therapeutic choice as a first-line therapy or when open surgical repair is associated with prohibitively high mortality. This procedure can be performed either as a destination therapy or as a bridge to valve replacement.


Assuntos
Valvuloplastia com Balão , Cardiopatias Congênitas , Estenose da Valva Tricúspide , Adulto , Humanos , Constrição Patológica , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Estenose da Valva Tricúspide/diagnóstico , Estenose da Valva Tricúspide/cirurgia
4.
Ann Noninvasive Electrocardiol ; 18(2): 118-25, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23530481

RESUMO

BACKGROUND: Multiform premature ventricular complexes (PVCs) are associated with an adverse prognosis in patients with structural heart disease. Very frequent PVCs are associated with ventricular dysfunction. Our hypothesis is that multiform PVCs confer an adverse prognosis in the general population. METHODS: We performed a retrospective cohort study of patients ≥18 years old referred to our institution for 24-hour ambulatory Holter monitoring between July 1, 2008 and December 31, 2009. Holters without PVCs or with more frequent ectopy (couplets, triplets, or nonsustained ventricular tachycardia) were excluded. Clinical and adverse event (AE) data ("major adverse cardiovascular event" or new/worsening heart failure) were gathered from chart review. Data was analyzed by PVC frequency (rare, occasional, or frequent) and pattern (uniform or multiform). RESULTS: A total of 222 patients (43% male, mean age: 55 ± 16 years) were evaluated (median follow-up 2.3 years [IQR: 2.0-2.6]). Median frequency was 2 PVCs per hour (IQR: 1-13). Multiform PVCs were noted in 48%. Patients with multiform PVCs were older, and had a higher prevalence of comorbidities. Thirty-nine AE were noted. Patients with an AE were younger, had a higher prevalence of HTN, diabetes, CAD, CHF, and previous MI. The multiform group had a higher incidence of AE (28%) compared to the uniform group (8%) (P < 0.001). Increasing PVC frequency was associated with a higher incidence of AE (8% vs 24% vs 35%, respectively). In Cox regression analyses, the multiform pattern but not frequency predicted AE. CONCLUSIONS: Multiform PVCs were associated with a 4-fold increase in AE in patients referred for ambulatory Holter monitoring.


Assuntos
Eletrocardiografia Ambulatorial/estatística & dados numéricos , Complexos Ventriculares Prematuros/diagnóstico , Idoso , Estudos de Coortes , Eletrocardiografia Ambulatorial/métodos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
5.
JACC Adv ; 2(2): 100257, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38938308

RESUMO

Background: The risk of erosion of an atrial septal closure device, in particular the Amplatzer Septal Occluder, has been described as higher in patients with a short aortic rim. Similar concern has been applied to patent foramen ovale (PFO) closure devices, but there are only rare reported cases of erosion. It may be that smaller devices are chosen due to fear of device erosion in PFO patients when this is not necessarily an issue. Objectives: The authors aimed to assess outcomes after PFO closure with the Amplatzer PFO device in patients with a short (<9 mm) aortic rim. Methods: We performed a retrospective analysis of PFO closure for any indication, between 2006 and 2017 at a quaternary center. Preprocedural transesophageal echocardiographic parameters including the aortic rim were remeasured. Long-term outcomes were obtained by linkage to provincial administrative databases. Results: Over the study period, 324 patients underwent PFO closure with the Amplatzer PFO device, with a mean age of 49.8 years; 61% had a short aortic rim (<9 mm). The most common indication was cryptogenic stroke (72%); those with longer aortic distance were more likely to have a non-stroke indication for closure, diabetes (15% vs 6.5%, P = 0.04), and heart failure (15.7% vs 4%, P < 0.001). Over a median 7 years of follow-up, there were no cases of device erosion or embolization requiring cardiac surgery. Conclusions: In a large cohort with long-term administrative follow-up (1,394 patient-years), implantation of an Amplatzer PFO device was performed safely even in patients with a short aortic rim.

6.
JACC Adv ; 2(10): 100701, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38938489

RESUMO

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.

7.
Cardiology ; 123(3): 135-41, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23095247

RESUMO

BACKGROUND: Red blood cell distribution width (RDW) is a measure of heterogeneity in erythrocyte size used in the differential diagnosis of anemia. High levels are associated with elevated cardiovascular biomarkers and increased mortality. The hypothesis of this study is that high RDW levels on admission are associated with higher recourse to coronary artery bypass graft (CABG) in unstable angina (UA) or non-ST-elevation myocardial infarction (NSTEMI) patients. METHODS: An observational, cross-sectional study of all adult patients undergoing coronary angiography admitted to an urban tertiary care center in 2007 with UA or NSTEMI was conducted. Data was gathered by review of inpatient charts. RDW was considered 'high' if it exceeded the 95th percentile (16.3%). RESULTS: Among the 503 subjects included in the analysis, high RDW was independently associated with higher recourse to CABG versus a nonsurgical approach [OR = 2.39 (1.04-5.50); p = 0.041] but not with conservative management [OR = 0.97 (0.51-1.84); p = 0.922] or percutaneous coronary intervention [OR = 0.67 (0.36-1.25); p = 0.208]. CONCLUSIONS: This study of patients with UA or NSTEMI demonstrated an independent association of elevated RDW with higher recourse to CABG. RDW should be considered in the stratification of patients presenting with UA or NSTEMI.


Assuntos
Angina Instável/sangue , Índices de Eritrócitos/fisiologia , Infarto do Miocárdio/sangue , Idoso , Angina Instável/terapia , Ponte de Artéria Coronária , Doença da Artéria Coronariana/sangue , Estudos Transversais , Contagem de Eritrócitos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/terapia , Intervenção Coronária Percutânea
8.
Front Physiol ; 13: 970389, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36060680

RESUMO

The literature on the relationship between kidney and cardiovascular diseases is continuously expanding. Scientists have elucidated many of the neurohormonal and hemodynamic pathways involved in cardiorenal disease. However, little is known about kidney disease in patients with congenital heart disease. Given advances in the medical and surgical care of this highly complex patient population, survival rates have dramatically improved leading to a higher percentage of adults living with congenital heart disease. Accordingly, a noticeable increase in the prevalence of kidney disease is appreciated in these patients. Some of the main risk factors for developing chronic kidney disease in the adult congenital heart disease population include chronic hypoxia, neurohormonal derangements, intraglomerular hemodynamic changes, prior cardiac surgeries from minimally invasive to open heart surgeries with ischemia, and nephrotoxins. Unfortunately, data regarding the prevalence, pathophysiology, and prognosis of chronic kidney disease in the adult congenital heart disease population remain scarce. This has led to a lack of clear recommendations for evaluating and managing kidney disease in these patients. In this review, we discuss contemporary data on kidney disease in adults with congenital heart disease in addition to some of the gaps in knowledge we face. The article highlights the delicate interaction between disease of the heart and kidneys in these patients, and offers the practitioner tools to more effectively manage this vulnerable population.

9.
J Soc Cardiovasc Angiogr Interv ; 1(5): 100370, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-39131476

RESUMO

Background: Femoral arterial access remains widely used despite recent increase in radial access for cardiac catheterization and percutaneous coronary intervention (PCI). Various femoral artery closure devices have been developed and are commonly used to shorten vascular closure times, with variable rates of vascular complications observed in clinical trials. We sought to examine the rates of contemporary outcomes during diagnostic catheterization and PCI with the most common femoral artery closure devices. Methods: We identified patients who had undergone either diagnostic catheterization alone (n = 14,401) or PCI (n = 11,712) through femoral artery access in the Indiana University Health Multicenter Cardiac Cath registry. We compared outcomes according to closure type: manual compression, Angio-Seal, Perclose, or Mynx. Access complications and bleeding outcomes were measured according to National Cardiovascular Data â€‹Registry standard definitions. Results: The use of any vascular closure device as compared to manual femoral arterial access hold was associated with a significant reduction in vascular access complications and bleeding events in patients who underwent PCI. No significant difference in access-site complications was observed for diagnostic catheterization alone. Among closure devices, Perclose and Angio-Seal had a lower rate of hematoma than Mynx. Conclusions: The use of femoral artery access closure devices is associated with a reduction in vascular access complication rates as compared to manual femoral artery compression in patients who undergo PCI.

10.
J Med Liban ; 59(3): 168-9, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22259908

RESUMO

Von Zumbusch generalized pustular psoriasis (GPP) is the most severe type of psoriasis with possible life-threatening complications. We report the case of a 22-year-old woman who presented with a severe eruption of generalized pustular psoriasis 48 hours after receiving an injection of etanercept (Enbrel).


Assuntos
Anti-Inflamatórios não Esteroides/efeitos adversos , Imunoglobulina G/efeitos adversos , Psoríase/induzido quimicamente , Psoríase/tratamento farmacológico , Ciclosporina/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Etanercepte , Feminino , Humanos , Receptores do Fator de Necrose Tumoral , Adulto Jovem
11.
J Am Coll Cardiol ; 77(13): 1644-1655, 2021 04 06.
Artigo em Inglês | MEDLINE | ID: mdl-33795039

RESUMO

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.


Assuntos
COVID-19 , Procedimentos Cirúrgicos Cardíacos , Cianose , Cardiopatias Congênitas , Hipertensão Pulmonar , Adulto , COVID-19/mortalidade , COVID-19/terapia , Teste para COVID-19/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Causalidade , Comorbidade , Cianose/diagnóstico , Cianose/etiologia , Cianose/mortalidade , Feminino , Saúde Global/estatística & dados numéricos , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Hospitalização/estatística & dados numéricos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Masculino , Mortalidade , Gravidade do Paciente , Fatores de Risco , SARS-CoV-2/isolamento & purificação , Avaliação de Sintomas
12.
Front Cardiovasc Med ; 7: 612014, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33381529

RESUMO

Thirty-eight-year-old male presented for evaluation of abdominal swelling, lower extremity edema and dyspnea on exertion. Extensive work-up in search of the culprit etiology revealed the presence of an Anomalous Right Upper Pulmonary Venous Return (ARUPVR) into the Superior Vena Cava (SVC). During the attempted repair, the pericardium was found to be thickened and constrictive. Only one other case of co-existent partial anomalous pulmonary venous return and constrictive pericarditis (CP) has been reported. The patient underwent a warden procedure with pericardial stripping with good outcomes at 45 days post-operatively. Thus, the presence of severe heart failure symptoms in the setting of ARUPVR should prompt further investigations. Also, further cases are needed to help guide management in these patients.

13.
Int J Cardiol ; 277: 85-89, 2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30195839

RESUMO

BACKGROUND: Care delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined. METHODS: A 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous. RESULTS: Of the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation. CONCLUSIONS: The new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.


Assuntos
Cardiologistas/educação , Cardiologia/educação , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Cardiologistas/tendências , Cardiologia/tendências , Humanos , Inquéritos e Questionários , Estados Unidos/epidemiologia
14.
Congenit Heart Dis ; 14(4): 511-516, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30945809

RESUMO

BACKGROUND: Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels. METHODS: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status. RESULTS: There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana. CONCLUSIONS: The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.


Assuntos
Cardiologistas/provisão & distribuição , Cardiologia , Atenção à Saúde/organização & administração , Recursos em Saúde/provisão & distribuição , Cardiopatias Congênitas/epidemiologia , Sociedades Médicas , Recursos Humanos/tendências , Adulto , Humanos , Ontário , Estudos Retrospectivos , Estados Unidos
15.
Congenit Heart Dis ; 14(2): 128-137, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30343507

RESUMO

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.


Assuntos
Congressos como Assunto , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Melhoria de Qualidade , Humanos , Qualidade de Vida , Fatores de Risco
17.
Congenit Heart Dis ; 13(1): 59-64, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29266726

RESUMO

OBJECTIVE: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for "rule-out" congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age. DESIGN: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 were reviewed to validate the true incidence of an ASD. This observational, cross-sectional record review included patients between 11 and 64 years of age. RESULTS: Medical charts and echocardiograms of 190 patients (47.9% males) were reviewed. The number of falsely coded patients with 745.5 (no ASD) was high (76.3%). Forty-five (23.7%) patients had a true ASD. Among the 145 patients without an ASD, 100 (52.6%) were classified as having a PFO, 37 (19.5%) had a normal non-CHD echocardiogram, and 8 (4.2%) had some other CHD anomaly. The likelihood that 745.5 coded for a true ASD was higher in children aged 11-20 (64.3%) than adults aged 21-64 years (20.6%). CONCLUSIONS: This validation study demonstrates that 745.5 performed poorly across all ages. As 745.5 is widely used in population-level investigations and ICD-10-CM perpetuates the problem, future analyses utilizing CHD codes should consider separate analysis of those identified only through code 745.5.


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/classificação , Adolescente , Adulto , Criança , Estudos Transversais , Feminino , Georgia/epidemiologia , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
18.
Am J Cardiol ; 122(9): 1557-1564, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30217370

RESUMO

Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.7 ± 13.5 years; 49.9% women). The combined outcome was all-cause mortality or nonelective cardiovascular hospitalization. Most patients had moderately or severely complex congenital heart disease (42.5% and 38.5%, respectively). Mean RDW was 14.0 ± 1.3%. RDW >15% was present in 81 patients (11.6%). After median 767days of follow-up, 115 patients sustained the primary combined outcome, including 31 who died. Higher RDW predicted both the combined outcome (hazard ratio [HR] for RDW >15% = 4.5, 95% confidence interval [CI] 3.0 to 6.6; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001) and death alone (HR for RDW >15% = 7.1, 95% CI 3.5 to 14.4; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001). RDW remained an independent predictor of the combined outcome after adjusting for age, cyanosis, congenital heart disease complexity, ventricular systolic function, New York Heart Association functional class, hemoglobin concentration, mean corpuscular volume, high-sensitivity C-reactive protein and estimated glomerular filtration rate (HR per + 1SD RDW = 1.5, 95% CI 1.2 to 1.9, p <0.0001). RDW also remained an independent predictor of mortality alone after adjustment for age plus each variable individually. In conclusion, elevated RDW is an independent predictor of all-cause mortality or nonelective cardiovascular hospitalization in adults with congenital heart disease. This simple clinical biomarker identifies increased risk for adverse events even among patients with preserved functional status.


Assuntos
Índices de Eritrócitos , Cardiopatias Congênitas/mortalidade , Adulto , Arritmias Cardíacas/epidemiologia , Biomarcadores/sangue , Boston/epidemiologia , Estudos de Coortes , Teste de Esforço , Feminino , Cardiopatias Congênitas/sangue , Insuficiência Cardíaca/epidemiologia , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Consumo de Oxigênio , Ventilação Pulmonar , Índice de Gravidade de Doença
20.
Cardiovasc Revasc Med ; 17(6): 418-20, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27318704

RESUMO

INTRODUCTION: Percutaneous endovascular revascularization requires the use of fluoroscopic guidance and radiopaque contrast. We present a successful intervention without the use of iodinated contrast. CASE: A 92-year-old man with dry gangrene involving the second and fourth left toes had acute on chronic kidney injury. Arterial duplex showed severe stenosis in bilateral superficial femoral arteries (SFAs). Fluoroscopic and ultrasound guidance and intravascular imaging were used to avoid iodinated contrast. After right to left femoral crossover, the entire left SFA was imaged with ultrasound. The lesion was delineated with radiopaque measuring tapes then wired. Near-infrared spectroscopy and intravascular ultrasound (NIRS-IVUS) imaging were performed. Points of interest were correlated with corresponding radiopaque markings on the ruler. Stenting and post-dilation resulted in complete stent expansion and no evidence of dissection by IVUS. The total procedure time was 113min and the total radiation dose 813mGy. The day after the procedure, there was a palpable dorsalis pedis pulse. He was discharged to inpatient rehabilitation on dual antiplatelet therapy. DISCUSSION: Contrast and radiation continue to limit the feasibility of endovascular angiography and intervention. Carbon dioxide (CO2) digital subtraction angiography is an alternative for these patients but has several disadvantages. Previously proposed projects demonstrated the real potential of performing endovascular peripheral intervention without fluoroscopy or contrast. CONCLUSION: This case is a clear demonstration of a successful use of a combination of fluoroscopy, ultrasonography and intravascular imaging to achieve a successful endovascular intervention to treat critical limb ischemia, without the use of iodinated contrast.


Assuntos
Angioplastia com Balão/métodos , Meios de Contraste/efeitos adversos , Isquemia/terapia , Doença Arterial Periférica/terapia , Espectroscopia de Luz Próxima ao Infravermelho , Ultrassonografia de Intervenção , Idoso de 80 Anos ou mais , Angioplastia com Balão/instrumentação , Dióxido de Carbono/administração & dosagem , Cineangiografia , Constrição Patológica , Meios de Contraste/administração & dosagem , Estado Terminal , Artéria Femoral/diagnóstico por imagem , Artéria Femoral/fisiopatologia , Fluoroscopia , Humanos , Isquemia/diagnóstico por imagem , Isquemia/fisiopatologia , Masculino , Imagem Multimodal , Doença Arterial Periférica/diagnóstico por imagem , Doença Arterial Periférica/fisiopatologia , Valor Preditivo dos Testes , Doses de Radiação , Exposição à Radiação/efeitos adversos , Índice de Gravidade de Doença , Stents , Resultado do Tratamento
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