International journal of cardiology. Congenital heart disease最新文献

Background

More women with congenital heart disease (CHD) reach reproductive age, but little is known of their success in having children. We investigated time trends of CHD in women of reproductive age and maternal CHD in childbirth and compared birth rates in women with CHD to birth rates in women without heart disease.

Methods and results

In a national cohort, we combined information from five registries in Norway 1994–2014. Among 1,644,650 women aged 15–45 years, 5672 had CHD. Among 1,183,851 childbirths, 3504 were registered with maternal CHD. The prevalences of mild and moderate/severe CHD in women increased by an average of 3–4% per year 1994–2014, as did the prevalences of mild and moderate/severe maternal CHD in childbirth. Compared to women without heart disease, the likelihood of having children was similar for women with mild CHD (rate ratio 1.03, 95% confidence interval 0.97–1.09) but lower for women with moderate/severe CHD (rate ratio 0.75, 95% confidence interval 0.68–0.84). The mean number of childbirths was similar in women with mild CHD and women without heart disease (1.81 vs 1.80, p = 0.722) but lower in women with moderate/severe CHD (1.42, p < 0.001).

Conclusion

In a national cohort over two decades of women of reproductive age, the prevalence of maternal CHD in childbirth reflected the increasing prevalence of CHD in the population. Birth rates were similar for women with mild CHD and women without heart disease, whereas women with moderate/severe CHD were less likely to have children and had a lower mean number of childbirths.

Background

Surgically repaired Tetralogy of Fallot (rTOF) is associated with progressive right ventricular hypertrophy (RVH) and dilation (RVD). Accurate estimation of RVH/RVD is vital for the ongoing management of this patient population. The utility of the ECG in evaluating patients with rTOF with pre-existing right bundle branch block (RBBB) has not been studied. We aimed to determine the sensitivity/specificity of currently established ECG criteria in detecting RVH/RVD in this patient population.

Methods

We included consecutive patients diagnosed with rTOF who underwent CMR performed at our regional referral centre between January 2012 and December 2019. Each CMR was assessed for LVH, LVD, RVH and or RVD. The ECG corresponding to the CMR was then used to determine RVH/LVH for specificity and sensitivity analysis.

Results

Our study included 163 consecutive rTOF patients. The specificity for ECG-based criteria for LVH was 100.00% (95% C.I. (87.75, 100.00)), and the sensitivity was 7.19% (95% C.I. (3.15, 12.83)). When RBBB was present, specificity for RVH was 100.00% (95% C.I. (84.56, 100.00)), and sensitivity was 7.69% (95% C.I. (3.75, 13.69)). When RBBB was absent, specificity for RVH was 100.00% (95% C.I. (15.81, 100.00)), and sensitivity was 0.00% (95% C.I. (0.00, 33.63)). A regression model with the entire group of 163 ToF patients, based on the Sokolow-Lyon criterion (sum of R in V1 + S in V5/V6), produced a new suggested criterion for the diagnosis of RVH in patients with rTOF, which was a sum of R in V1 + S in V5/V6 greater than 13.25 mm. This model's sensitivity for RVH detection was 69.1%, and specificity was 36.8%.

Conclusions

Standard ECG voltage criteria have poor sensitivity for detecting right and left ventricular chamber hypertrophy and dilatation in patients with rTOF, so current ECG criteria should not be used to monitor RVH/RVD in this patient population.

Background

Extracardiac conduit Fontan procedure (ECFP) employing a Gore-Tex conduit has been widely used for patients with single ventricle physiology; however, the long-term status of the conduit is unknown. We investigated the changes in a Gore-Tex conduit after ECFP and the factors associated with its narrowing.

Methods

We conducted a retrospective analysis of 86 patients who underwent ECFP between January 1995 and December 2008 and had cardiac computed tomography (CT) during the follow-up period.

Results

The median patient age at ECFP was 2.8 years (range 1.6–9.7), and a cardiac CT was obtained at 13.1 ± 3.4 years later. The minimum conduit area decreased by approximately two-thirds of the original due to calcification, pseudointimal hyperplasia, thrombus, and luminal irregularity. The normalized minimum conduit area was influenced by the time interval from ECFP and normalized original conduit area at ECFP. An oversized conduit was associated with a narrowing of both its sides and a high frequency of pseudointimal hyperplasia or mural thrombus. The ratio of minimum conduit-to-inferior vena cava areas was lower in patients with chronic liver disease than in those with a normal liver. The maximum percent stenosis of the conduit correlated with oxygen pulse and heart rate during peak exercise.

Conclusions

Using a larger conduit at ECFP resulted in a larger minimum conduit area at follow-up. However, oversizing requires careful monitoring for stenosis near anastomotic sites and the occurrence of pseudointimal hyperplasia or thrombus.

Background

Aging is associated with acquired comorbidities that potentially influence the natural history and outcomes of adults with congenital heart disease (CHD). The purpose of this study was to compare the clinical characteristics, as well as the incidence and correlates of all-cause mortality between different age groups.

Method

Adults with CHD were categorized into 3 age groups based on age at baseline encounter: Group 1 (age 18–40 years); Group 2 (age 41–65 years), and Group 3 (age >65 years).

Results

Of 5930 patients (age 37 ± 15 years), 3009 (51%), 2422 (41%), and 499 (8%) were in Groups 1, 2 and 3, respectively. Compared to Group 1, patients in Groups 2 and 3 were less likely to have complex CHD, but more likely to have acquired comorbidities, end-organ dysfunction, ventricular systolic dysfunction, and valvular heart disease. Compared to Group 1, Groups 2 and 3 had higher incidence of all-cause mortality (7.2 versus 15.3 versus 47.8 per 1000 patient-years, respectively, p < 0.001), and lower proportion of deaths from cardiovascular causes (87% versus 77% versus 71%, respectively, p < 0.001). Furthermore, the correlates of all-cause mortality were different between the age groups, with acquired comorbidities such as hypertension, coronary artery disease, and hepatorenal dysfunction being associated with mortality in Group 3, while indices of CHD severity such as number of prior cardiac surgery, and presence of complex CHD being associated with all-cause mortality in Group 1.

Conclusions

These results suggest the need for management strategies tailored to address the correlates of outcomes in each age group.

Since the late 1980s, the standard approach for treating D-transposition of the great arteries has been the arterial switch operation (ASO), replacing the Mustard/Senning procedure. Although ASO has shown impressive long-term survival rates, recent case series have revealed late complications such as neoaortic dilation and coronary artery stenosis. New findings emphasize the need for comprehensive evaluation of coronary risk and a deeper understanding of the mechanisms leading to coronary artery stenosis and myocardial ischemia over the long term. Computed tomography angiography (CTA) has unveiled a notable prevalence of abnormal coronary arteries with potential risk of stenosis and myocardial ischemia. Moreover, the progressive dilation of the neoaortic root and the potential for valve regurgitation necessitating intervention warrant serial imaging follow-up. Considering the radiation risks associated with CTA, magnetic resonance imaging emerges as a preferred modality for post-ASO patient assessment. Ongoing research in this field holds the promise of developing improved diagnostic and therapeutic strategies for these patients, thereby enhancing their long-term care

Background

Right atrial (RA) dysfunction and atrial arrhythmias are relatively common in adults with repaired tetralogy of Fallot. The purpose of this study was to determine whether RA function improved after surgical pulmonary valve replacement (PVR), and the association between postoperative RA reverse remodeling and late postoperative atrial arrhythmias.

Method

RA reverse remodeling (ΔRA reservoir strain based speckle tracking echocardiography) was calculated as: ([postoperative RA reservoir strain – preoperative RA reservoir strain]/preoperative RA reservoir strain)x100. Optimal RA reverse remodeling was defined as ΔRA reservoir strain >15%.

Results

Of 411 patients (age 36 ± 13 years), preoperative RA reservoir strain was 31 ± 13%, postoperative RA reserve remodeling was 13 ± 9%, and 171 (42%) had optimal RA reserve remodeling. Preoperative RA reservoir strain (β±SE 1.12 ± 0.09, p < 0.001) was associated with postoperative RA reverse remodeling on multivariable analysis. Preoperative RA reservoir strain ≥33% predicted optimal postoperative RA reverse remodeling (area under the curve 0.792).

ΔRA reservoir strain was associated with postoperative atrial arrhythmias (HR 0.91, 95%CI 0.86–0.96, p = 0.004), on multivariable analysis. Compared to patients with preoperative RA reservoir strain <33% (n = 242, 59%), those with RA reservoir strain ≥33% (n = 169, 41%) had more robust RA reverse remodeling (ΔRA reverse remodeling 19 ± 11% versus 7 ± 10%, p < 0.001), and lower incidence of atrial arrhythmias (1.1% versus 2.9%, p = 0.003).

Conclusions

Preoperative RA reservoir strain was associated with RA reverse remodeling after PVR, and in turn, postoperative atrial arrhythmia. These results suggest that RA strain indices could be used to determine optimal timing for PVR in order to reduce the risk of atrial arrhythmia.

Introduction

Each year the number of combined heart-liver transplants (HLT) increases, with two distinct patient populations proceeding down this pathway. The first are patients with congenital heart disease (CHD), most commonly single ventricle patients palliated with Fontan. The second group are those with long standing congestive hepatopathy, amyloidosis, hemochromatosis, or alcohol induced myopathies and liver disease.

One argument for HLT has been the low rate of rejection even among sensitized patients, with reported rejection rates ranging from 0% to 31%. Historically, those with CHD have been highly sensitized which in some cases may prevent or at least delay transplantation. As such, a recent consensus statement by Emamaulee et al. suggest that “there may be an immunological benefit to proceed with HLT with significantly fewer acute cellular and humoral rejection episodes”. The aim of this study is to demonstrate that HLT patients remain at risk for rejection and have required treatment for it.

Results

There were 15 patients who underwent HLT from January 2017 to February 2022. Of the four patients who did not have CHD, none were considered sensitized, and all underwent induction with basiliximab per our institutional protocol. One of these had rejection. Rejection episodes were identified in four of the 11 CHD patients (36%) patients.

Conclusions

In our study of 15 HLT, including 11 CHD patients (73% denied transplant at ≥ 1 center) demonstrated a higher rate of rejection than previously reported. While theoretically, HLT may mitigate the likelihood of rejection, the risk still exists, and patients benefit from close monitoring commensurate with single organ transplant.