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We report the first ultrasonographically guided percutaneous balloon atrial septoplasty (BAS), to our knowledge, in a fetus with transposition of the great arteries and an intact ventricular and atrial septum (37 + 2 weeks). After vaginal delivery at 38 weeks, the infant had an elective septostomy (day 1) and an arterial switch procedure (day 7), with an uneventful postoperative course. For centres with experience in fetal cardiac interventions, fetal BAS is a superior management option compared with the alternatives for this high-risk physiology.  相似文献   

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Background

The purpose of the study was to evaluate the association between fetal echocardiographic measurements and the need for intervention (primary coarctation repair, staged coarctation repair, or catheter intervention) in prenatally diagnosed coarctation of the aorta.

Methods

A single-centre retrospective cohort study (2005-2015) of 107 fetuses diagnosed with suspected coarctation of the aorta in the setting of an apex-forming left ventricle and antegrade flow across the mitral and aortic valves.

Results

Median gestational age at diagnosis was 32 weeks (interquartile range, 23-35 weeks). Fifty-six (52%) did not require any neonatal intervention, 51 patients (48%) underwent a biventricular repair. In univariable analysis, an increase in ascending aorta (AAo) peak Doppler flow velocity (odds ratio [OR], 1.40 [95% confidence interval [CI], 1.05-1.91] per 20 cm/s; P = 0.03) was associated with intervention. No intervention was associated with larger isthmus size (OR, 0.23; P < 0.001), transverse arch diameter (OR, 0.23; P < 0.001), and aortic (OR, 0.72; P = 0.02), mitral (OR, 0.58; P = 0.001), and AAo (OR, 0.53; P < 0.001) z-scores. In multivariable analysis, higher peak AAo Doppler (OR, 2.51 [95% CI, 1.54-4.58] per 20 cm/s; P = 0.001) and younger gestational age at diagnosis (OR, 0.81 [95% CI, 0.70-0.93] per week; P = 0.005) were associated with intervention, whereas a higher AAo z-score (OR, 0.65 [95% CI, 0.43-0.94] per z; P = 0.029) and transverse arch dimension (OR, 0.44 [95% CI, 0.18-0.97]; P = 0.05) decreased the risk of intervention.

Conclusions

In prenatally suspected coarctation, the variables associated with intervention comprised smaller AAo and transverse arch size, earlier gestational age at diagnosis, and the additional finding of a higher peak AAo Doppler.  相似文献   

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In adult heart transplant recipients, a transformation of the self has been observed simultaneous to the “emotional integration” of the heart following transplantation. However, the experiences of adolescents following heart transplantation are virtually unexplored within the current literature. Our qualitative findings address this gap and explore struggles with personal identity in adolescent heart transplant recipients. Twenty-seven heart transplant patients (67% female, age range: 12 to 18 years) from a large teaching hospital participated in 1-on-1 interviews, which were transcribed verbatim and coded, using methods of constant comparison within a grounded- theory approach. Emergent themes were identified and refined through team consensus. Many participants identified emotional and psychological concerns regarding accepting foreign hearts as their own. This manifested in a range of experiences such as sadness or guilt regarding the death of the donor. Adolescent participants also pondered the potential acquisition of personal qualities or characteristics of the donor. Many participants speculated extensively about the donor and “longed for” donor information. Findings point to the meaning-making processes that adolescent heart transplant patients endure as they grapple with the presence of a foreign, life-giving organ within their bodies and the potential impact on their psychosocial well-being. This paper encourages health care professionals to initiate discussions before and following transplantation that address adolescents’ concept of self and offers recommendations for clinical care.  相似文献   

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Background

We have previously documented an increase in the incidence of Kawasaki disease (KD) in Ontario followed by a stabilization from 1995 to 2006. We sought to validate the estimation of incidence of KD using administrative data and to describe the epidemiology of KD across Canada from 2004 to 2014.

Methods

We queried the Canadian Hospital Discharge Database for hospital admissions associated with a discharge diagnosis of KD. The data set was manually curated and estimates of incidence were compared with those obtained from the retrospective triennial surveillances of KD performed in 2007 and 2010.

Results

The average number of cases per year identified through administrative data was 245 ± 45 vs 229 ± 33 from retrospective surveillance. This overestimation, representing 7 ± 6%, is similar to the historical percentage of patients originally diagnosed with KD in whom the diagnosis is subsequently excluded. The annual incidence of KD in Canada was 19.6, 6.4, and 1.3 cases per 100,000 children younger than 5 years, 5-9 years, and 10-14 years old, respectively, with important regional and seasonal differences. The incidence remained stable over the study period in the youngest age group but increased in both older age categories. Coronary artery aneurysms affected 3.5% of all patients, and 0.8% experienced associated major cardiac complications.

Conclusions

Reliance on administrative data to determine incidence of KD is feasible and accurate with manual curation of the data. The incidence of KD in Canada seems to have plateaued for younger children. Differences in annual incidence observed between provinces remain to be explained, and might reflect genetic or environmental differences.  相似文献   

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Background

Novel quantification of stroke volume according to mitral inflow and aortic outflow using automated real-time 3-dimensional volume colour flow Doppler echocardiography (3D-RT-VCFDE) is more accurate than 2-dimensional echocardiography and has excellent correlation with cardiac magnetic resonance imaging-based flows in adults. This technology is applied for the first time to the right heart and in children.

Methods

3D-RT-VCFDE was performed in 61 image sets of flow through the aortic (AV), mitral (MV), pulmonary (PV), and tricuspid (TV) valves of 34 children. These were compared with stroke volumes of the right (RV) and left (LV) ventricles and ratio of pulmonary to systemic blood flow determined using the Fick method in 31 children with atrial shunts.

Results

The mean age was 8.0 ± 3.3 years, and the mean weight was 27.8 ± 10.0 kg. The mean temporal resolution for flow analyses was ≥ 22 volumes per second. In conditions with no shunt, the correlations were: AV with MV flows (r = 0.98), PV with TV flows (r = 0.96), RV stroke volume with PV flow (r = 0.95), and with TV flow (r = 0.93), LV stroke volume with AV flow (r = 0.87), and with MV flow (r = 0.89). Fick ratio of pulmonary to systemic blood flow correlations were: PV/AV ratio (r = 0.84), TV/MV ratio (r = 0.87), and RV/LV ratio (r = 0.70).

Conclusions

Stroke volume determined using automated 3D-RT-VCFDE is feasible in children and in the right side of the heart. This technique potentially provides a noninvasive alternative to historically invasively acquired hemodynamic data and to cardiac magnetic resonance imaging.  相似文献   

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Background

Common arterial trunk (CAT) is a rare anomaly with a spectrum of pathology. We sought to identify current trends and factors associated with postnatal outcomes.

Methods

This was a single-centre review including 153 live births with planned surgery. Patients were analyzed as 2 cohorts based on era of CAT diagnosis (1990 to 1999 vs 2000 to 2014) and complexity of disease (simple vs complex). “Complex” required the association with significant aortic arch obstruction, truncal valve (TV) stenosis/regurgitation, and/or branch pulmonary artery (PA) hypoplasia, respectively.

Results

Sixteen (10%) died preoperatively, and this outcome was associated with significant TV stenosis (odds ratio [OR] 4.55; P = 0.01) and regurgitation (OR 3.17; P = 0.04); 130 (95%) of 137 operated infants underwent primary complete repair. Their survival rates to 1 year improved from 54% to 85% after 2000, although this outcome remained substantially lower for cases with a complex vs simple CAT repair (76% vs 95%; OR 6.46; P = 0.006). Other risk factors associated with decreased 1-year survival included diagnosis before 2000 (OR 4.48; P = 0.038) and a lower birth weight (OR 8.0 per kg weight; P = 0.001). Finally, of 93 survivors beyond year 1 of life, 76 (82%) had undergone a total of 224 reinterventions. Only 15 (16%) were alive without any surgical or catheter-based reintervention at study end.

Conclusions

Despite recent surgical improvements, postnatal mortality continues to be substantial if CAT is complicated by significant pathology of the TV, aortic arch, or branch PAs. Reoperations and catheter interventions are eventualities for most patients during childhood.  相似文献   

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BackgroundRight ventricular (RV) diastolic function and right atrial (RA) function are poorly characterized in patients with Ebstein anomaly (EA) but may influence functional capacity. We aimed to evaluate RV diastolic function and RA function in EA and study their relationship with biventricular systolic function and exercise capacity.MethodsSeventy-two patients with EA and 69 controls prospectively underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to investigate RV systolic and diastolic function, RA function, and exercise capacity.ResultsAltered RV diastolic function was indicated by the reduced tricuspid valve E/A ratio, percentage RV filling time, and early and late diastolic strain rate; and by the increased tricuspid valve E/E′, isovolumic relaxation time, and RV myocardial performance index. The average of 6-RV-segment early diastolic strain rate correlated modestly with peak VO2 (r = 0.38, P < 0.01), RV ejection fraction (r = 0.41, P < 0.01), and left ventricular ejection fraction (r = 0.33, P < 0.05). Patients with EA had impaired RA reservoir, conduit, and pump function, which were associated with peak VO2 (r = 0.54, P < 0.001 for reservoir function).ConclusionsAltered RV diastolic function and RA function in patients with EA are associated with impaired biventricular systolic function and exercise capacity. The stronger correlation of RA vs RV function with exercise capacity suggests that it may be important to evaluate RA function in this population.  相似文献   

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Background

Optimal heart rate (HR) for acute hemodynamic efficiency in heart failure (HF) is unknown.

Methods

Wistar-Kyoto rats were followed-up for 3 and 7 days, 1 or 2 months after myocardial infarction (MI) or sham operation (ShO) and left ventricle (LV) pressure-volume (PV) loops were obtained at various HRs: baseline 400 beats per minute (bpm), reduced by ivabradine to 320 bpm, increased by atrial pacing to 480 bpm, under normal conditions and after preload increase (PI).

Results

In the ShO group, PI augmented cardiac output (CO) by 55%, 67%, 84% at reduced, baseline, and increased HR, respectively. In post-MI rats, PI augmented CO 3 and 7 days, but not 1 and 2 months after MI. At increased HR, in response to PI, CO increased 3 and 7 days, tended to fall 1 and 2 months after MI; this hemodynamic response was salvaged by HR reduction. Further beneficial effects of HR reduction included reduction of LV end-diastolic pressure, increase of ejection fraction, contractility and relaxation velocity 1 and 2 months after MI.

Conclusions

In a rat HF model, optimal HR with regard to acute hemodynamic performance is shifted. Whereas in ShO rats increased HR facilitates CO increase induced by PI, in HF rats, such increase reduces CO, and HR reduction has beneficial effects. Thus, besides reducing progression of HF, HR-reducing interventions also offer immediate hemodynamic benefits.  相似文献   

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We describe the case of a 68-year-old woman presenting with stress cardiomyopathy (SCM), with concomitant cardiogenic shock, left ventricular outflow tract obstruction, and ventricular septal rupture. These complications have not simultaneously been reported in a single SCM case. The importance of early diagnosis of serial complications of SCM and using mechanical circulatory support as a treatment strategy are highlighted.  相似文献   

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Background

Deleterious mutations in KCNQ1 may lead to an autosomal dominant form of long QT syndrome (LQTS) (Romano-Ward) or autosomal recessive form (Jervell and Lange-Nielsen). Both are associated with severe ventricular tachyarrhythmias due to the reduction of the slowly activating delayed rectifier K+ current (IKs). Our objective was to investigate the functional consequences of KCNQ1-R562S mutation in an atypical form of KCNQ1-linked LQTS.

Methods

Mutant KCNQ1-R562S was analyzed via confocal imaging, surface biotinylation assays, co-immunoprecipitation, phosphatidylinositol-4,5-bisphosphate pulldown test, whole-cell patch clamp, and computational intrinsic disorder analyses.

Results

Protein expression, assembly with KCNE1, and trafficking to the surface membrane of KCNQ1-R562S were comparable with wild-type channels. The most significant functional effect of the R562S mutation was a depolarizing shift in the voltage dependence of activation that was dependent on association with KCNE1. The biophysical abnormality was only partially dominant over coexpressed wild-type channels. R562S mutation impaired C-terminal association with membrane phosphatidylinositol-4,5-bisphosphate. These changes led to compromised rate-related accumulation of repolarizing current that is an important property of normal IKs.

Conclusions

KCNQ1-R562S mutation reduces effective IKs due to channel gating alteration with a mild clinical expression in the heterozygous state due to minimal dominant phenotype. In the homozygous state, it is exhibited with a moderately severe LQTS phenotype due to the incomplete absence of IKs.  相似文献   

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