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1.
Int J Cardiol ; 330: 43-49, 2021 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-33571563

RESUMO

BACKGROUND: Pregnant women with repaired tetralogy of Fallot (rTOF) are at increased risk of adverse outcomes. Although pre-pregnancy pulmonary valve replacement (PVR) may be considered in some women to attenuate risk, published data to support this practice are lacking. Our objective was to explore the impact of pre-pregnancy PVR on pregnancy outcomes in rTOF. METHODS: Women with rTOF and cardiovascular magnetic resonance imaging (CMR) before and after pregnancy were included if CMR studies were completed within 3 years of pregnancy. Subjects were compared according to presence (+) or absence (-) of PVR at pre-pregnancy CMR. Pregnancy outcomes (cardiovascular, obstetric, and fetal/neonatal) were documented. RESULTS: Of the 29 study women identified, 7 were PVR+ and 22 were PVR-. Post-pregnancy, the PVR- group demonstrated interval increase in indexed right ventricular end-diastolic volumes (RVEDVi) (157 ± 28 versus 166 ± 33 ml/m2, p = 0.003) and end-systolic volumes (RVESVi) (82 ± 17 versus 89 ± 20 ml/m2, p = 0.003) as compared with pre-pregnancy, but no significant change in RV ejection fraction, RV mass, or left ventricular measurements. In the PVR+ group, there were no interval changes in RV measurements pre-versus post pregnancy. Interval rate of change in RVESVi of PVR- exceeded PVR+ women (+3.7 ± 5.0 versus -2.2 ± 5.0 ml/m2/year, p = 0.03). Pregnancy outcomes did not differ in PVR+ versus PVR- women. CONCLUSIONS: Pregnancy outcomes did not differ according to PVR status in our cohort. While RV volumes remained unchanged in PVR+ women, interval RV dilation was observed in PVR- women. Additional study of a larger population with longer follow-up may further inform clinical practice regarding pre-pregnancy PVR.

2.
Circ Cardiovasc Imaging ; 14(2): e011748, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33517672

RESUMO

BACKGROUND: Surgical resection of cardiac fibromas in children reduces hemodynamic and arrhythmia burden; however, little is known about postoperative left ventricular (LV) function. We aimed to evaluate factors associated with postoperative LV dysfunction. METHODS: In this retrospective observational cohort study, imaging data were reviewed from 41 patients who had undergone surgical resection of a cardiac fibroma. Tumor volume was indexed to body surface area (tumor volume index). Right ventricular tumors were excluded from analysis of postoperative ventricular function. Postoperative regional wall motion abnormality score was defined as number of wall segments with regional wall motion abnormality, and LV dysfunction was defined as LV ejection fraction <50%. Cardiovascular magnetic resonance-derived strain was low if <5%ile by previously published normative data. RESULTS: Of 41 patients who underwent resection at a median age of 2.1 years (range, 0.5-19), 37 fibromas were in the LV, (29 free wall and 8 septal), and 4 in the right ventricle. Preoperative median tumor volume index was 66 mL/m2 (range, 11-376). Of 37 patients with LV tumors, younger patients had larger tumor volume index and higher grades of preoperative mitral regurgitation (P<0.001). Larger tumor volume index correlated with higher postoperative regional wall motion abnormality score (P<0.001). By paired pre- and post-operative cardiovascular magnetic resonance (n=14), LV end-diastolic volume increased (mean 76 versus 101 mL/m2, P=0.011), with decreased LV ejection fraction (mean 60% versus 55%, P=0.014), a higher prevalence of low global circumferential strain (36% versus 64%, P=0.045), and decreased cardiac index (mean 4.8 versus 3.9 L/[min·m2], P=0.039). More than mild preoperative mitral regurgitation was the only independent predictor of predischarge LV dysfunction (odds ratio, 22 [95% CI, 2.8-179], P=0.008). CONCLUSIONS: Surgical resection of LV fibroma is associated with regional wall motion abnormality, increased LV volume, and reduced systolic function. Children with significant preoperative mitral regurgitation are at highest risk for LV dysfunction and warrant ongoing close surveillance.

3.
Artigo em Inglês | MEDLINE | ID: mdl-33338215

RESUMO

In 2018, the position paper 'Imaging the adult with congenital heart disease: a multimodality imaging approach' was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

4.
Ann Thorac Surg ; 2020 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-32712099

RESUMO

BACKGROUND: Several factors predict reintervention for subaortic stenosis (SubAS): age, preoperative left ventricular outflow tract gradient, distance from the obstructive subaortic ridge to the aortic valve, and peeling of membrane from the aortic/mitral valves. We sought to develop a prediction rule to categorize risk of reintervention for recurrent SubAS and guide follow-up in patients with discrete SubAS. METHODS: We retrospectively reviewed patients who underwent SubAS resection between 1984 and 2016. Our primary outcome was reintervention for recurrent SubAS after discharge. Kaplan-Meier estimates were used for time-to-event analysis of any reintervention. Multivariable models were used to create a prediction rule. We excluded patients without 3 years of follow-up. RESULTS: Of 172 patients, 21 (12.2%) required reintervention. The characteristics predicting reintervention were age younger than 2 years (P < .001), preoperative left ventricular outflow tract gradient of 65 mm Hg or more (P = .011), peeling of membrane from the mitral valve (P < .001), distance from the membrane to the aortic valve of less than 5 mm (P < .001), prior complex operation (P = .035), other left-sided heart lesions (P = .008), and aortic annulus z-score of -2.5 or less (P < .001). Our final prediction rule includes age, membrane to aortic valve distance, and other left-sided heart lesions each scored as 1 point. For patients with a score of 1 or less, 4% required a reintervention compared with 34% with a score of 2 or more. CONCLUSIONS: A prediction rule that incorporates the patient's age at the index operation, membrane to aortic valve distance, and associated left-sided heart lesions can determine the likelihood of reintervention for recurrent SubAS.

5.
Pediatrics ; 146(2)2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32647064

RESUMO

BACKGROUND: Long-term outcomes in heterotaxy syndrome (HS) are poorly described. Some reports suggest improved survival in the recent era, whereas others do not. We sought to describe long-term outcomes and assess whether outcomes have changed over time. METHODS: Patients with HS born between 1985 and 2014 who had cardiac care (except initial palliation) at our institution were divided into 4 birth eras and survival over time was compared. Independent risk factors for mortality were identified by using Cox proportional hazards regression. In patients who underwent surgery, association between surgical pathway (univentricular versus biventricular repair) and mortality after adjusting for baseline confounders was evaluated. A risk stratification model was created by using classification and regression analysis. RESULTS: Among 264 patients, 118 (44.7%) had asplenia and 146 (55.3%) had polysplenia syndrome. Overall mortality was 40.2% (n = 106), with median follow-up of 10.2 years (longest 31.5 years). In multivariable analysis, pulmonary vein stenosis, coarctation, univentricular circulation, asplenia phenotype, and at least mild atrioventricular valve regurgitation at presentation were associated with mortality, whereas birth era was not. Among patients who underwent surgery, univentricular repair remained associated with mortality after adjustment. In classification and regression analysis, patients with biventricular circulation (especially those with polysplenia) had lower mortality than those with univentricular circulation. CONCLUSIONS: In this large retrospective study of HS, outcomes remain poor and have not improved since the early 1990s. We identified risks factors associated with earlier mortality and found that those with univentricular circulation and totally anomalous pulmonary venous connection had the worst prognosis. Survival was higher in those with biventricular circulation.


Assuntos
Síndrome de Heterotaxia/mortalidade , Procedimentos Cirúrgicos Cardíacos , Feminino , Seguimentos , Síndrome de Heterotaxia/cirurgia , Humanos , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Mortalidade/tendências , Prognóstico , Modelos de Riscos Proporcionais , Circulação Pulmonar , Estudos Retrospectivos , Fatores de Risco , Sobreviventes , Resultado do Tratamento
6.
Front Physiol ; 11: 198, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32265727

RESUMO

Introduction: Right ventricle (RV) failure is one of the most common symptoms among patients with repaired tetralogy of Fallot (TOF). The current surgery treatment approach including pulmonary valve replacement (PVR) showed mixed post-surgery outcomes. A novel PVR surgical strategy using active contracting bands is proposed to improve the post-PVR outcome. In lieu of testing the risky surgical procedures on real patients, computational simulations (virtual surgery) using biomechanical ventricle models based on patient-specific cardiac magnetic resonance (CMR) data were performed to test the feasibility of the PVR procedures with active contracting bands. Different band combination and insertion options were tested to identify optimal surgery designs. Method: Cardiac magnetic resonance data were obtained from one TOF patient (male, age 23) whose informed consent was obtained. A total of 21 finite element models were constructed and solved following our established procedures to investigate the outcomes of the band insertion surgery. The non-linear anisotropic Mooney-Rivlin model was used as the material model. Five different band insertion plans were simulated (three single band models with different band locations, one model with two bands, and one model with three bands). Three band contraction ratios (10, 15, and 20%) and passive bands (0% contraction ratio) were tested. RV ejection fraction was used as the measure for cardiac function. Results: The RV ejection fraction from the three-band model with 20% contraction increased to 41.58% from the baseline of 37.38%, a 4.20% absolute improvement. The RV ejection fractions from the other four band models with 20% contraction rate were 39.70, 39.45, and 40.70% (two-band) and 39.17%, respectively. The mean RV stress and strain values from all of the 21 models showed only modest differences (5-11%). Conclusion: This pilot study demonstrated that the three-band model with 20% band contraction ratio led to 4.20% absolute improvement in the RV ejection fraction, which is considered as clinically significant. The passive elastic bands led to the reduction of the RV ejection fractions. The modeling results and surgical strategy need to be further developed and validated by a multi-patient study and animal experiments before clinical trial could become possible. Tissue regeneration techniques are needed to produce materials for the contracting bands.

7.
Am J Cardiol ; 125(6): 977-981, 2020 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-31959431

RESUMO

The rationale for timing of pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rTOF) has focused on pre-PVR threshold values of indexed right ventricular end-diastolic volume (RVEDVi) that lead to normalization of right ventricular (RV) size after valve implantation. The goal of this study was to determine whether persistent RV dilation after PVR is associated with adverse clinical outcomes. Subjects with rTOF who underwent PVR and had a cardiac magnetic resonance (CMR) exam after valve implantation at a single center from 2001 to 2017 were included. The composite clinical outcome after PVR included: death, aborted sudden cardiac death, sustained ventricular tachycardia (VT), or NYHA class ≥3. In 189 rTOF subjects, the mean age at PVR was 23.5 ± 11.7 years, median follow-up was 6.0 years (IQR 3.4 to 8.7), and the primary outcome occurred in 14 subjects (7%). The 5- and 10-year event-free rates were 97% and 91%, respectively. Post-PVR RVEDVi was not associated with the composite outcome (p = 0.59). Independent predictors of the outcome were older age at PVR (hazard ratios [HR] 1.06; 95% confidence interval [CI] 1.02 to 1.11; p = 0.004), post-PVR lower RV ejection fraction (HR 0.91; 95% CI 0.86 to 0.97; p = 0.002), and post-PVR atrial tachyarrhythmia (HR 7.60, 95% CI 1.65 to 35.05, p = 0.009). Our study shows that post-PVR RV dilation as measured by CMR-derived RVEDVi was not associated with the composite adverse clinical outcome in this cohort. These findings challenge the validity of current guidelines for PVR, which are based on pre-procedural threshold values of RVEDVi aimed at achieving normal post-procedural RV volumes.


Assuntos
Implante de Prótese de Valva Cardíaca , Complicações Pós-Operatórias/diagnóstico , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/diagnóstico , Dilatação Patológica/diagnóstico , Dilatação Patológica/mortalidade , Seguimentos , Humanos , Complicações Pós-Operatórias/mortalidade , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidade , Tetralogia de Fallot/mortalidade , Resultado do Tratamento , Disfunção Ventricular Direita/mortalidade
9.
Int J Cardiol ; 307: 31-35, 2020 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-31668657

RESUMO

BACKGROUND: Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. METHODS: This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. RESULTS: We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. CONCLUSIONS: Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

10.
PLoS One ; 14(8): e0220328, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31412062

RESUMO

Patient-specific in vivo ventricle mechanical wall stress and strain conditions are important for cardiovascular investigations and should be calculated from correct zero-load ventricle morphologies. Cardiac magnetic resonance (CMR) data were obtained from 6 healthy volunteers and 12 Tetralogy of Fallot (TOF) patients with consent obtained. 3D patient-specific CMR-based ventricle models with different zero-load diastole and systole geometries due to myocardium contraction and relaxation were constructed to qualify right ventricle (RV) diastole and systole stress and strain values at begin-filling, end-filling, begin-ejection, and end-ejection, respectively. Our new models (called 2G models) can provide end-diastole and end-systole stress/strain values which models with one zero-load geometries (called 1G models) could not provide. 2G mean end-ejection stress value from the 18 participants was 321.4% higher than that from 1G models (p = 0.0002). 2G mean strain values was 230% higher than that of 1G models (p = 0.0002). TOF group (TG) end-ejection mean stress value was 105.4% higher than that of healthy group (HG) (17.54±7.42kPa vs. 8.54±0.92kPa, p = 0.0245). Worse outcome group (WG, n = 6) post pulmonary valve replacement (PVR) begin-ejection mean stress was 57.4% higher than that of better outcome group (BG, 86.94±26.29 vs. 52.93±22.86 kPa; p = 0.041). Among 7 selected parameters, End-filling stress was the best predictor to differentiate BG patients from WG patients with prediction accuracy = 0.8208 and area under receiver operating characteristic curve (AUC) value at 0.8135 (EE stress). Large scale studies are needed to further validate our findings.


Assuntos
Ventrículos do Coração/fisiopatologia , Modelos Cardiovasculares , Estresse Mecânico , Tetralogia de Fallot/fisiopatologia , Adolescente , Adulto , Criança , Diástole/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Sístole/fisiologia , Adulto Jovem
11.
JACC Clin Electrophysiol ; 5(5): 563-575, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31122378

RESUMO

OBJECTIVES: This study sought to evaluate for the presence of and characterize the interdigitating and entrapped myocardium within cardiac fibromas (CF) and correlate tissue findings with symptoms and surgical outcomes. BACKGROUND: The mechanism of ventricular tachycardia (VT) in CF is unclear. The authors hypothesized that CF harbor tongues of interdigitating myocardium, which could be a substrate for episodes of arrhythmia analogous to peri-infarct zones. METHODS: A total of 29 patients (14 boys) with CF were identified; all subjects had undergone at least partial tumor resection. A semiquantitative grading system was used to assess the degree of myocardial interdigitation and entrapment, myocyte morphology (hematoxylin and eosin stain and immunohistochemical stain for desmin), tumor collagen density, and cellularity (trichrome stain). The subjects' ages at presentation, types of arrhythmia, and responses to surgery were correlated with histology. RESULTS: CF consistently demonstrated interdigitating and entrapped myocardium, and the extent correlated negatively with age at surgery, as did cellularity, whereas collagen increased with age. Median age at arrhythmia recognition was 8 months. Sustained VT was present in 18 of 29 patients (62%), and 5 of 6 patients with prenatally diagnosed conditions developed VT before 8 months. All 8 patients who experienced cardiac arrest had clinically significant arrhythmia events. Sustained arrhythmia episodes correlated with more diffuse myocyte interdigitation. Ten patients had abnormal karyotype: chromosomes 9 (n = 3) and 3 (n = 1) deletions; isolated translocations: t(4;13), t(5;11) and t(1;9); and undefined aberrations (n = 3). All patients who underwent complete resection were cured of arrhythmias, whereas 2 of 14 patients who had subtotal resections had recurrence, with resolution following re-resection in 1 patient. CONCLUSIONS: Interdigitating myocardium represents a potential histopathologic substrate for VT and cardiac arrest in CF, which may also explain the occasional recurrence of arrhythmia following incomplete resection.


Assuntos
Parada Cardíaca/etiologia , Neoplasias Cardíacas , Miocárdio/patologia , Taquicardia Ventricular/etnologia , Criança , Pré-Escolar , Feminino , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Taquicardia Ventricular/etiologia
12.
World J Pediatr Congenit Heart Surg ; 10(3): 343-350, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31084307

RESUMO

Epicardial echocardiography (e-echo) is a useful approach to intraoperative imaging for the smallest patients and in those with contraindications to transesophageal echocardiography (TEE). The e-echo has additional advantages that include improved visualization of anterior and vascular structures and three-dimensional capabilities. In this review, we describe the advantages and disadvantages of e-echo versus TEE for pediatric and congenital heart surgery with the goal of enhancing the utility and feasibility of the former among congenital heart surgeons. We also review the technique for performance of an e-echo and provide demonstrations of the images obtained for select lesions.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia Transesofagiana/métodos , Cardiopatias Congênitas/cirurgia , Pericárdio/diagnóstico por imagem , Cirurgia Assistida por Computador/métodos , Criança , Cardiopatias Congênitas/diagnóstico , Humanos
13.
J Am Heart Assoc ; 8(7): e011536, 2019 04 02.
Artigo em Inglês | MEDLINE | ID: mdl-30929556

RESUMO

Background Optimally treated patients with coarctation of the aorta remain at risk for late vascular dysfunction. The effect of treatment modality on vascular function is unknown. The LOVE-COARCT (Long-term Outcomes and Vascular Evaluation After Successful Coarctation of the Aorta Treatment) study was done to compare vascular function in patients with coarctation of the aorta treated with surgery, balloon dilation (BD), or stent implantation. Methods and Results In treated coarctation of the aorta patients without residual coarctation, we prospectively compared aortic stiffness by applanation tonometry and cardiac magnetic resonance; endothelial function by endothelial pulse amplitude testing; blood pressure ( BP ) phenotype by office BP , ambulatory BP monitoring, and BP response to exercise; left ventricular mass by cardiac magnetic resonance; and blood biomarkers of endothelial function, inflammation, vascular wall function, and extracellular matrix. Participants included 75 patients treated with surgery (n=28), BD (n=23), or stent (n=24). Groups had similar age at enrollment, coarctation of the aorta severity, residual gradient, and metabolic profile, but differed by age at treatment. Prevalence of systemic hypertension, aortic stiffness, endothelial function, and left ventricular mass were similar among treatment groups. However, BD patients had more-distensible ascending aortas, lower peak systolic BP during exercise, less impairment in diurnal BP variation, and lower inflammatory biomarkers. Results were unchanged after adjustment for potential confounders, including age at treatment. Conclusions In our cohort of patients without residual coarctation, treatment modality was not associated with major vascular outcomes, even though there were some favorable vascular characteristics in the BD patients. Although this suggests that choice of treatment modality should continue to be driven by likelihood of achieving a good anatomical result, more long-term studies are required to assess the clinical significance of the more-optimal results of secondary markers of vascular function in BD patients. Clinical Trial Registration URL : http://www.clinicaltrials.gov . Unique identifier: NCT 03262753.


Assuntos
Aorta/fisiopatologia , Coartação Aórtica/fisiopatologia , Adolescente , Aorta/cirurgia , Coartação Aórtica/sangue , Coartação Aórtica/cirurgia , Pressão Arterial/fisiologia , Biomarcadores/metabolismo , Estudos Transversais , Endotélio Vascular/fisiopatologia , Feminino , Humanos , Masculino , Fenótipo , Rigidez Vascular/fisiologia , Adulto Jovem
14.
Int J Cardiol ; 276: 93-99, 2019 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-30217422

RESUMO

Patient-specific in vivo ventricle material parameter determination is important for cardiovascular investigations. A new cardiac magnetic image (CMR)-based modeling approach with different zero-load diastole and systole geometries was adopted to estimate right ventricle material parameter values for healthy and patients with Tetralogy of Fallot (TOF) and seeking potential clinical applications. CMR data were obtained from 6 healthy volunteers and 16 TOF patients with consent obtained. CMR-based RV/LV models were constructed using two zero-load geometries (diastole and systole, 2G model). Material parameter values for begin-filling (BF), end-filling (EF), begin-ejection (BE), and end-ejection (EE) were recorded for analyses. Effective Young's moduli (YM) for fiber direction stress-strain curves were calculated for easy comparisons. The mean EE YM value of TOF patients was 78.6% higher than that of the healthy group (HG). The mean end-ejection YM value from worse-outcome TOF group (WG) post pulmonary valve replacement (PVR) surgery was 59.5% higher than that from the better-outcome TOF group (BG). Using begin-filling YM and end-ejection YM as predictors and the classic logistic regression model to different better-outcome group patients from worse-outcome group patients, the areas under Receiver Operating Characteristic (ROC) curves were found to be 0.797 and 0.883 for begin-filling YM and end-ejection YM, respectively. The sensitivity and specificity 0.761 and 0.755 using end-ejection YM as the predictor. This preliminary study suggests that ventricle material stiffness could be a potential parameter to be used to differentiate BG patients from WG patients with further effort and large-scale patient data validations.


Assuntos
Pressão Sanguínea/fisiologia , Modelos Cardiovasculares , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologia , Adolescente , Adulto , Criança , Diástole/fisiologia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Sístole/fisiologia , Função Ventricular Direita/fisiologia , Adulto Jovem
15.
Ann Thorac Surg ; 107(1): e41-e43, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30009804

RESUMO

Interrupted right aortic arch is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. We report two cases of interrupted right aortic arch associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus in the second case. Computed tomography was complementary to echocardiography in delineating the anatomic details in both cases, informing surgical repair with reconstruction of a left aortic arch in the first case and right arch in the second case.


Assuntos
Síndromes do Arco Aórtico/cirurgia , Angiografia por Tomografia Computadorizada , Permeabilidade do Canal Arterial/cirurgia , Ecocardiografia Doppler , Imageamento Tridimensional , Procedimentos Cirúrgicos Reconstrutivos/métodos , Tronco Arterial/cirurgia , Síndromes do Arco Aórtico/complicações , Síndromes do Arco Aórtico/diagnóstico por imagem , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/diagnóstico por imagem , Feminino , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Recém-Nascido , Masculino , Diagnóstico Pré-Natal , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Taquipneia/etiologia , Tronco Arterial/diagnóstico por imagem
16.
J Cardiovasc Magn Reson ; 20(1): 85, 2018 12 17.
Artigo em Inglês | MEDLINE | ID: mdl-30558626

RESUMO

BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. METHODS: This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO2). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. RESULTS: A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5-30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0-13.8]. Fifty-three patients (36%) had MPBF (95% CI 29-45%). The mean % predicted VO2 was 63 ± 16%. Patients with MPBF had lower mean % predicted VO2 compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p = 0.04). On multivariable analysis, a lower % predicted VO2 was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6-7.4, p < 0.001). CONCLUSION: In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population.


Assuntos
Tolerância ao Exercício , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Estenose de Artéria Pulmonar/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Teste de Esforço , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Masculino , Consumo de Oxigênio , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Estenose de Artéria Pulmonar/diagnóstico por imagem , Estenose de Artéria Pulmonar/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
17.
Ann Pediatr Cardiol ; 11(3): 282-296, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30271019

RESUMO

Background: Coarctation of the aorta (CoA) can be treated using surgery, balloon angioplasty, or stent implantation. Although short-term results are excellent with all three treatment modalities, long-term cardiovascular (CV) morbidity and mortality remain high, likely due to persistently abnormal vascular function. The effects of treatment modality on long-term vascular function remain uncharacterized. The goal of this study is to assess vascular function in this patient population for comparison among the treatment modalities. Methods: We will prospectively assess vascular Afunction in large and small arteries fusing multiple noninvasive modalities and compare the results among the three groups of CoA patients previously treated using surgery, balloon angioplasty, or stent implantation after frequency matching for confounding variables. A comprehensive vascular function assessment protocol has been created to be used in 7 centers. Our primary outcome is arterial stiffness measured by arterial tonometry. Inclusion and exclusion criteria have been carefully established after consideration of several potential confounders. Sample size has been calculated for the primary outcome variable. Conclusion: Treatment modalities for CoA may have distinct impact on large and small arterial vascular function. The results of this study will help identify the treatment modality that is associated with the most optimal level of vascular function, which, in the long term, may reduce CV risk.

19.
Am J Cardiol ; 122(9): 1541-1545, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30180956

RESUMO

Little information exists on left atrial (LA) function in healthy children and in patients with congenital aortic valve stenosis (AS). We sought to determine whether patients with significant congenital AS have detectable abnormalities in LA size and function compared with matched normal controls. Retrospective chart review and analysis of 2-dimensional echocardiographic images for phasic left atrial volumes (LAVs) and longitudinal LA strain by speckle tracking in children ≥1 years of age with ≥ moderate isolated congenital AS. LAVs and LA strain in AS cases were compared with age, gender, and body surface area-matched controls (healthy children with a normal echocardiogram). Between June 2004 and October 2012, there were 36 eligible AS cases [age 13 (range 3 to 27) years; 30 males] with a median maximal Doppler gradient of 63 (range 43 to 94) mm Hg. No significant interobserver and intraobserver differences were noted in the analyses of normal subjects. Significantly higher phasic body surface area-adjusted LAVs [preatrial contraction: p = 0.01; minimal: p = 0.04] and higher LA contractile function parameters [indexed active LA stroke volume: p = 0.03; peak negative strain p = 0.04] were noted in AS cases compared with matched controls. In conclusion, compared with normal, children with congenital AS have higher preatrial contraction and minimal phasic LA volumes and higher LA contractile function.


Assuntos
Estenose da Valva Aórtica/congênito , Função Atrial/fisiologia , Átrios do Coração/diagnóstico por imagem , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Pré-Escolar , Ecocardiografia , Feminino , Humanos , Masculino , Contração Miocárdica/fisiologia , Estudos Retrospectivos , Volume Sistólico/fisiologia , Adulto Jovem
20.
Eur Heart J Cardiovasc Imaging ; 19(10): 1077-1098, 2018 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-30084968

RESUMO

Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.


Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Imagem Multimodal/métodos , Adulto , Humanos
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