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1.
J Biomech Eng ; 2020 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-33170219

RESUMO

Fontan associated liver disease is a common complication in patients with Fontan circulation, who were born with a single functioning heart ventricle. The hepatic venous pressure gradient (HVPG) is used to assess liver health and is a surrogate measure of the pressure gradient across the entire liver (portal pressure gradient). However, it is thought to be inaccurate in Fontan patients. The main objectives of this study were (1) to apply an existing detailed lumped parameter model of the liver to Fontan patients using patient-specific clinical data and (2) to determine whether HVPG is a suitable measurement of portal pressure gradients in these patients. An existing lumped parameter model of the liver blood circulation was applied and tuned to simulate patient-specific liver hemodynamics. Geometries were collected from seven adult Fontan patients and used to evaluate model parameters. The model was solved and tuned using waveform measurements of flows, inlet and outlet pressures. The predicted ratio of portal to hepatic venous pressures is comparable to in vivo measurements. The results confirmed that HVPG is not suitable for Fontan patients, as it would underestimate the portal pressures gradient by a factor of 3 to 4. Our patient-specific liver model provides an estimate of the pressure drop across the liver, which differs from the clinically used metric HVPG. This work represents a first step towards models suitable to assess liver health in Fontan patients and improve its long-term management.

2.
Can J Cardiol ; 2020 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-33246004

RESUMO

BACKGROUND: Atrial tachyarrhythmias (AAs) are the main source of morbidity and mortality in adults with congenital heart disease (ACHD). Direct current cardioversion (DCCV) is an effective method to acutely terminate AAs, however, many patients require repeat DCCV. Little is known about the impact of radiofrequency catheter ablation (RFCA) of AAs on the incidence of repeat DCCV in ACHD. The purpose of this study was to evaluate the impact of RFCA on the incidence of DCCV in ACHD patients. METHODS: A total of 157 ACHD undergoing DCCV in our hospital from 2011 to 2018 (female n=76 [48.4%], mean age 37.8±12.5), were reviewed. The median follow-up period was 31.8 months (IQR, 16.3-55.1). RESULTS: Out of the total of 157 patients, 102 (65.0%) underwent RFCA for AAs, and 55 (35.0%) patients were treated without RFCA. Successful RFCA with termination of AAs during ablation was 62.7%. More than half of the patients had complex forms of congenital heart disease (62.4%). During follow-up, 57 (55.9%) patients who had RFCA developed recurrence of AAs, and 36 (35.2%) patients underwent repeat DCCV. Thirty-three (60.0%) out of 55 patients without RFCA required repeat cardioversion. Compared to patients without RFCA, RFCA significantly reduced the need for repeat DCCV by 40% (hazard ratio [HR] 0.60, 95% CI 0.23-0.80 P=0.009). In multivariate analysis, successful RFCA was associated with reduced risk of DCCV (HR 0.41, 95% CI 0.19-0.92, P=0.031). CONCLUSION: AAs remain common despite RFCA in ACHD patients. Nevertheless, RFCA is associated with a marked reduction in the need for repeat DCCV.

3.
Front Genet ; 11: 957, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33110418

RESUMO

Recent genome-wide studies of rare genetic variants have begun to implicate novel mechanisms for tetralogy of Fallot (TOF), a severe congenital heart defect (CHD). To provide statistical support for case-only data without parental genomes, we re-analyzed genome sequences of 231 individuals with TOF (n = 175) or related CHD. We adapted a burden test originally developed for de novo variants to assess ultra-rare variant burden in individual genes, and in gene-sets corresponding to functional pathways and mouse phenotypes, accounting for highly correlated gene-sets and for multiple testing. For truncating variants, the gene burden test confirmed significant burden in FLT4 (Bonferroni corrected p-value < 0.01). For missense variants, burden in NOTCH1 achieved genome-wide significance only when restricted to constrained genes (i.e., under negative selection, Bonferroni corrected p-value = 0.004), and showed enrichment for variants affecting the extracellular domain, especially those disrupting cysteine residues forming disulfide bonds (OR = 39.8 vs. gnomAD). Individuals with NOTCH1 ultra-rare missense variants, all with TOF, were enriched for positive family history of CHD. Other genes not previously implicated in CHD had more modest statistical support in gene burden tests. Gene-set burden tests for truncating variants identified a cluster of pathways corresponding to VEGF signaling (FDR = 0%), and of mouse phenotypes corresponding to abnormal vasculature (FDR = 0.8%); these suggested additional candidate genes not previously identified (e.g., WNT5A and ZFAND5). Results for the most promising genes were driven by the TOF subset of the cohort. The findings support the importance of ultra-rare variants disrupting genes involved in VEGF and NOTCH signaling in the genetic architecture of TOF, accounting for 11-14% of individuals in the TOF cohort. These proof-of-principle data indicate that this statistical methodology could assist in analyzing case-only sequencing data in which ultra-rare variants, whether de novo or inherited, contribute to the genetic etiopathogenesis of a complex disorder.

4.
Heart ; 2020 Oct 28.
Artigo em Inglês | MEDLINE | ID: mdl-33115764

RESUMO

BACKGROUND: Patients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients. METHODS: Primary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated. RESULTS: A total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age <28.5 years) was associated with discontinuing amiodarone (HR 5.50, 95% CI 1.19 to 25.4, p=0.029). AIT significantly increased risk of HF (HR 4.82, 95% CI 1.71 to 13.6, p=0.003). CONCLUSIONS: Short-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

5.
J Nephrol ; 2020 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-32910428

RESUMO

BACKGROUND: Increased left atrial (LA) size predicts cardiovascular events in patients with end-stage kidney disease. There is a paucity of data on LA changes after kidney transplantation (KT). Accordingly, we used cardiac magnetic resonance imaging (CMR) to evaluate LA remodeling after KT, and examined its relationship with left ventricular (LV) measurements, blood pressure and cardiac biomarkers. METHODS: In this prospective multi-center cohort study, 39 pre-transplant dialysis patients underwent KT and 42 eligible transplant recipients remained on dialysis. CMR, blood pressure and serum measurements for N-terminal pro b-type natriuretic peptide (NT-proBNP), high sensitivity C-reactive protein (hsCRP), and growth differentiation factor-15 (GDF-15) were performed at baseline and 12 months. RESULTS: After 12 months, there were no significant changes in LA end-systolic volume index, LA end-diastolic volume index, or LA ejection fraction (LAEF) within the KT or dialysis group; changes over time did not differ between the 2 groups (all p > 0.25). At baseline and over 12 months, LA volumes and LAEF positively correlated with LV volumes and mass while LAEF positively correlated with LV function. Changes in LA volumes also positively correlated with NT-proBNP and systolic blood pressure (sBP) while LAEF negatively correlated with NT-proBNP. GDF-15 correlated with LA measurements at baseline but not in 12-month changes. hsCRP did not correlate with any LA measurements. CONCLUSIONS: LA volumes and function as measured by CMR did not change significantly over 12 months post-KT. There were significant associations between LA and LV remodeling, NT-proBNP and sBP, suggesting common underlying pathophysiological mechanisms.

6.
Heart ; 2020 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-32748799

RESUMO

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

7.
AJR Am J Roentgenol ; : 1-7, 2020 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-32755161

RESUMO

OBJECTIVE. Cardiac involvement is the leading cause of mortality in Fabry disease. Noninvasive markers of cardiac involvement are needed to identify patients at high risk. The purpose of this study was to evaluate the diagnostic potential of segmental native T1 spread as an imaging biomarker in Fabry disease. SUBJECTS AND METHODS. In this prospective study, 43 patients with confirmed Fabry disease (mean ± SD age, 46±14 years; 70% women) and 17 healthy control subjects (mean ± SD age, 44 ±13 years; 53% women) underwent 3-T cardiac MRI including modified Look-Locker inversion recovery T1 mapping. Segmental native T1 spread was calculated as the difference between maximum and minimum segmental native T1 values, expressed as an absolute value and as a relative percentage of global native T1. RESULTS. Absolute and relative segmental native T1 spreads were significantly higher in patients with Fabry disease than in healthy control subjects (absolute median, 115 vs 98 ms [p = 0.004]; relative median, 9.9% vs 8.0% [p < 0.001]) and correlated positively with quantitative late gadolinium enhancement (absolute, r = 0.434, p < 0.001; relative, r = 0.436, p < 0.001), indexed left ventricular mass (absolute, r = 0.316, p = 0.01; relative, r = 0.347, p = 0.007), and global longitudinal strain (absolute, r = 0.289, p = 0.03; relative, r = 0.277, p = 0.03). Relative segmental native T1 spread differentiated patients with Fabry disease from healthy control subjects (odds ratio, 1.44 [95% CI, 1.10-1.89]; p = 0.009). Interob-server agreement was excellent for both absolute (intraclass correlation coefficient, 0.932) and relative (intraclass correlation coefficient, 0.926) segmental native T1 spread. CONCLUSION. Increased native T1 spread is a reproducible imaging biomarker of cardiac involvement in Fabry disease and may be particularly useful in the evaluation of patients who cannot undergo late gadolinium enhancement imaging.

8.
Can J Cardiol ; 2020 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-32522524

RESUMO

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) have been proven to prevent sudden cardiac death in adult congenital heart disease (ACHD) patients. Although the left side is chosen by default, implantation from the right side is often required. However, little is known about the efficacy and safety of right-sided ICDs in ACHD patients. METHODS: In this study we reviewed a total of 191 ACHD patients undergoing ICD/cardioverter resynchronisation therapy-defibrillator (CRT-D) implantation at our hospital between 2001 and 2019 (134 men and 57 women; age [mean ± standard deviation], 41.5 ± 14.8 years). RESULTS: Twenty-seven patients (14.1%) had right-sided devices. The most common causes of right-sided implantation were persistent left superior vena cava and vein occlusion (37.0%). Although procedure time (202.8 ± 60.5 minutes vs 143.8 ± 69.1 minutes, P = 0.008) was longer and the procedural success was lower (92.6% vs 99.4%, P = 0.008) for right-sided devices, no difference in R-wave and pacing threshold were noted. Among the 47 patients (24.6%) who underwent defibrillation threshold testing (DFT), no difference in DFT was observed (25.2 ± 5.3 J vs 23.8 ± 4.1 J, P = 0.460). During the median follow-up of 42.4 months, appropriate ICD therapy was observed in 5 (18.5%) and 30 (18.3%) patients for right- and left-sided ICDs/CRTDs, respectively (P = 0.978). No significant difference was seen in complications between them. CONCLUSIONS: Implantation of an ICD on the right side is technically challenging, but it is feasible as an alternative approach for ACHD patients with contraindications to left-sided device implantation.

9.
Int J Cardiol ; 317: 63-69, 2020 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-32470536

RESUMO

BACKGROUND: Although morphologic abnormalities in the liver are commonly encountered post Fontan palliation, the relationships between hepatic morphology, vascular flows, and clinical status remain incompletely understood. We therefore aimed to explore flow characteristics in hepatic and intestinal vessels and to examine cardiovascular associations with liver disease. METHODS: This was a retrospective study of adults post Fontan palliation undergoing clinically indicated cardiovascular magnetic resonance imaging (MRI). Patients were included if MRI flow quantification was available for cardiac, hepatic and intestinal vessels; patients were excluded if phase-contrast flow imaging was insufficient for analysis. RESULTS: Thirty patients were studied (median age at MRI 28.5 years [range 19-47]). Eighteen subjects (60%) were classified as having morphologic liver disease according to validated criteria based on available MRI imaging. Abdominal and cardiovascular flows were quantified. Patients with morphologic liver disease had a 41% reduction in superior mesenteric artery (211 ± 124 versus 358 ± 181 mL/min/m2, p = .004), a 36% reduction in hepatic vein (496 ± 247 versus 778 ± 220 mL/min/m2, p = .01), a 31% reduction in portal vein (399 ± 133 versus 580 ± 159 mL/min/m2, p = .004), and an 18% reduction in Fontan pathway flows (1358 ± 429 versus 1651 ± 270 mL/min/m2, p = .04) compared with the remaining population. Adverse cardiovascular events were not associated with morphologic liver disease. CONCLUSION: Morphologic liver disease appears to be associated with flow alterations within the heart, liver and intestine post Fontan palliation. These novel observations suggest that a potential relationship exists between morphologic disease and vascular flows thereby providing further insights into the pathophysiology of liver disease in this high-risk population.

10.
Can J Cardiol ; 2020 Apr 21.
Artigo em Inglês | MEDLINE | ID: mdl-32325106

RESUMO

BACKGROUND: Early surgical tetralogy of Fallot (ToF) repair involved patching across the pulmonic annulus (transannular patch [TAP] repair), which resulted in severe pulmonic regurgitation. Long-term outcome improvements were anticipated with modifications that preserved the pulmonic annulus (annulus-preserving [AP] repair). The objective of the present study was to evaluate the need for late reintervention in adults with AP repair and those with TAP repair. METHODS: We conducted a retrospective review of adults (born 1981-1996) with childhood intracardiac ToF repairs at a tertiary care center. The primary cardiovascular outcome was need for reintervention after primary intracardiac repair of ToF. Secondary outcomes included a composite of death, heart failure, and ventricular arrhythmias. RESULTS: Two hundred thirty adults were included: 104 with AP repair and 126 with TAP repair. The median age at last follow up was 25 years (interquartile range [IQR] 20-28) and the median follow-up duration was 7.9 years (IQR 3.5-12). Reintervention of any type was significantly more common in the TAP group during both childhood and adulthood (72.2% TAP vs 20.2% AP, HR 5.5, 95% CI 3.4-9.0; P < 0.001). Pulmonary valve replacement (PVR) was almost 6 times more likely in adults with TAP repair (65.1% TAP vs 16.3% AP, HR 5.7, 95% CI 3.4-9.7; P < 0.001). CONCLUSIONS: Patients who had AP ToF repair had significantly fewer late reinterventions compared with TAP repair, with the majority of reinterventions due to PVR. More long-term follow-up is required.

12.
J Thorac Imaging ; 2020 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-32251236

RESUMO

PURPOSE: The purpose of this study was to compare thoracic aortic measurements between computed tomography (CT), magnetic resonance imaging (MRI), and transthoracic echocardiography (TTE). MATERIALS AND METHODS: A total of 127 patients (mean age: 45±18 y, 49% male) who had undergone CT and MRI evaluation of the thoracic aorta at a single tertiary referral hospital within a 6-month interval between 2007 and 2017 were included in this retrospective study. TTE studies performed within the same 6-month interval were also evaluated. Thoracic aortic measurements were blindly evaluated using multiple techniques and were compared between modalities. RESULTS: There was no significant difference in maximum aortic root diameter between CT and MRI when using the inner lumen-to-inner lumen technique (mean difference: 0.2±1.4 mm, P=0.51) or the outer lumen-to-outer lumen technique (mean difference: 0.5±1.4 mm, P=0.07). There were no significant differences between CT and MRI at any other level except for the distal descending aorta (20.2±4.6 vs. 19.8±4.6 mm, P<0.001). However, aortic root measurements by TTE using the leading edge-to-leading edge technique were significantly smaller compared with maximum aortic root diameters using the inner lumen-to-inner lumen and outer lumen-to-outer lumen techniques by both CT (mean difference: 4.9±2.7 mm, P<0.001 and 7.4±2.8 mm, P<0.001, respectively) and MRI (mean difference: 4.8±3.2 mm, P<0.001 and 8.2±3.0 mm, P<0.001, respectively). CONCLUSIONS: There is excellent agreement in thoracic aortic measurements between CT and MRI. However, TTE significantly underestimates maximum aortic root diameter compared with CT and MRI. Therefore, caution should be used when interpreting small apparent changes in aortic root diameters between TTE and CT or MRI.

13.
Radiology ; 294(1): 42-49, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31660802

RESUMO

Background Cardiac involvement is the leading cause of mortality in patients with Fabry disease. Identification of imaging findings that predict adverse cardiac events is needed to enable identification of high-risk patients. Purpose To establish the prognostic value of cardiac MRI findings in men and women with Fabry disease. Materials and Methods Consecutive women and men with gene-positive Fabry disease who had undergone cardiac MRI at a single large tertiary referral hospital between March 2008 and January 2019 were included in this retrospective cohort study. Evaluators of cardiac MRI studies were blinded to all clinical information. Adverse cardiac events were assessed as a composite end point, defined as ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death. Statistical analysis included Cox proportional hazard models adjusted for age and Mainz Severity Score Index (a measure of the severity of Fabry disease). Results Ninety patients (mean age, 44 years ± 15 [standard deviation]; 59 women) were evaluated. After a median follow-up period of 3.6 years, the composite end point was reached in 21 patients (incidence rate, 7.6% per year). Left ventricular hypertrophy (LVH) and late gadolinium enhancement (LGE) were independent predictors of the composite end point in adjusted analysis (LVH hazard ratio [HR], 3.0; 95% confidence interval [CI]: 1.1, 8.1; P = .03; and LGE HR, 7.2; 95% CI: 1.5, 34; P = .01). Patients with extensive LGE (≥15% of left ventricular mass) were at highest risk (HR, 12; 95% CI: 2.0, 67; P = .006). Sex did not modify the relationship between the composite end point and any of the cardiac MRI parameters, including LVH (P = .15 for interaction term) and LGE (P = .38 for interaction term). Conclusion Cardiac MRI findings of left ventricular hypertrophy and late gadolinium enhancement can be used to identify patients with Fabry disease who are at high risk of adverse cardiac events. © RSNA, 2019 See also the editorial by Zimmerman in this issue.


Assuntos
Doença de Fabry/complicações , Gadolínio/farmacocinética , Insuficiência Cardíaca/complicações , Hipertrofia Ventricular Esquerda/complicações , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Aumento da Imagem/métodos , Imagem por Ressonância Magnética/métodos , Adulto , Estudos de Coortes , Meios de Contraste/farmacocinética , Feminino , Seguimentos , Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
15.
Abdom Radiol (NY) ; 45(1): 188-202, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31471707

RESUMO

PURPOSE: To describe liver imaging findings and complications on computed tomography (CT) or magnetic resonance imaging (MRI) in adults with Fontan palliation and investigate whether imaging features show correlations with clinical and physiological parameters. METHODS: Our Institutional Review Board approved this retrospective study. Two blinded abdominal radiologists reviewed abdominal CT (n = 21) and MRI (n = 16) images between September 2011 and October 2017 in 37 adults (median age 27 years, interquartile range 21-36 years, 14 males [38%]) with a Fontan palliation (median post-Fontan duration 22 years, interquartile range 19-28 years). Correlation between CT/MRI findings and clinical parameters including laboratory results within 6 months of CT/MRI examinations was assessed by Spearman's rank correlation coefficient. RESULTS: Lobulated hepatic surface and blunt hepatic edge were seen in 92% (34/37) and 95% (35/37) of patients, respectively. Surface nodularity was noted in 32% (12/37). In 7 patients, there were 11 hepatic nodules which showed arterial-phase hyperenhancement and washout. Among them, 2 were biopsy-proven hepatocellular carcinomas (HCCs), and the remaining 9 were focal nodular hyperplasia (FNH)-like nodules. Suprahepatic inferior vena cava (IVC) diameter showed positive correlations with post-Fontan duration (p < 0.01), serum gamma-glutamyl transferase (p < 0.01), and total bilirubin (p < 0.01). CONCLUSION: The livers in post-Fontan adults show a unique morphology of blunt edge and lobulating surface with occasional nodularity. There is a diagnostic challenge in distinguishing HCCs from FNH-like nodules in post-Fontan population due to overlapping imaging findings. Suprahepatic IVC diameter is a potentially useful imaging marker that reflects hepatic dysfunction in Fontan palliation.

17.
Can J Cardiol ; 35(12): 1772-1783, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31813508

RESUMO

BACKGROUND: There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation. METHODS: Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits. RESULTS: No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m2, -43.64 to -78.94 mL/m2) and end-systolic (-37.20 mL/m2, -25.58 to -48.82 mL/m2) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status. CONCLUSION: Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes.


Assuntos
Causas de Morte , Implante de Prótese de Valva Cardíaca/métodos , Guias de Prática Clínica como Assunto , Insuficiência da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Adulto , Canadá , Doenças Cardiovasculares , Gerenciamento Clínico , Feminino , Previsões , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/mortalidade , Humanos , Masculino , Insuficiência da Valva Pulmonar/epidemiologia , Sociedades Médicas , Análise de Sobrevida , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/epidemiologia , Resultado do Tratamento
18.
Obstet Med ; 12(4): 158-163, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31853254

RESUMO

Cardiovascular imaging during pregnancy is frequently used to help direct diagnosis and management for women with known or suspected cardiac disease. Although echocardiography is the most commonly used imaging modality in pregnancy, cardiovascular magnetic resonance imaging is an important and increasingly used tool, which can provide complementary, and oftentimes incremental, information regarding cardiovascular anatomy, ventricular function, and vascular flows. Advantages of cardiovascular magnetic resonance imaging over echocardiography and other imaging techniques include superior reproducibility, excellent cross-sectional evaluation of cardiac structures, high spatial resolution, and lack of ionizing radiation (a limitation of computed tomography and conventional catheter-based angiography). Cardiovascular magnetic resonance imaging in the absence of gadolinium-based contrast agents poses no known risk to the mother or fetus and its applications in pregnancy are expanding. Clinicians should be familiar with the role of cardiovascular magnetic resonance imaging in pregnancy to optimize and enhance care for mothers with heart disease.

19.
Am J Cardiol ; 124(10): 1636-1642, 2019 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-31540664

RESUMO

The cardiovascular magnetic resonance imaging (CMR) features of adults with repaired truncus arteriosus (rTA) are largely undefined. We sought to explore CMR characteristics in rTA and to identify associations between imaging findings and cardiovascular outcomes. Adults with rTA and CMR were identified and anatomic subtypes (1-4) were assigned (Collett and Edwards classification). CMR characteristics, clinical data at last follow-up and adverse cardiovascular outcome were recorded. Twenty-seven adults (19% male) were studied (median age at cardiovascular magnetic resonance 26 years [interquartile range 18 to 40]) over 5.2-year duration [interquartile range 2.5 to 7.5]. With the exception of mildly increased RV mass (30 ± 12 g/m2), cardiac chamber measurements were within the normal range. In CMR measurements, only pulmonary artery peak velocity differed in subtypes (highest in subtype 3, 318 ± 26 cm/s, p = 0.029). Number of cardiovascular interventions in adulthood was moderately correlated with left ventricular end-diastolic volume (r = 0.463, p = 0.015), left ventricular ejection fraction (r = 0.425, p = 0.027) and neoaortic root size (r = 0.398, p = 0.039). Cardiovascular events (nonmutually exclusive) in 5 of 27 patients (19%) included death (n = 1), heart failure (n = 1), ventricular tachycardia (n = 1), and atrial tachycardia (n = 3). Increased cardiovascular risk was associated with decreased right ventricular ejection fraction (odds ratio 1.153, confidence interval 1.003 to 1.326, p = 0.046) and smaller ascending aorta diameter (odds ratio 1.758, confidence interval 1.037 to 2.976, p = 0.036). In conclusion, decreased right ventricular ejection fraction and smaller ascending aorta on cardiovascular magnetic resonance were associated with adverse events in rTA.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Persistência do Tronco Arterial/cirurgia , Tronco Arterial/diagnóstico por imagem , Adolescente , Adulto , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Persistência do Tronco Arterial/diagnóstico , Persistência do Tronco Arterial/fisiopatologia , Adulto Jovem
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