International journal of cardiology. Congenital heart disease最新文献

Background

Children with univentricular heart (UVH) have a limited life expectancy without early treatment. Long-term survival in UVH, in an unselected nationwide cohort, is unclear.

Objectives

To determine long-term survival in patients with UVH including non-operated patients compared with a control population in Sweden.

Methods

Patients with UVH born between 1970 and 2017 were identified from the National Registers and were matched for birth year and sex with 10 individuals without congenital heart disease. Follow-up was from birth until death, transplantation, or the end of study. Mortality risk was estimated by Cox proportional regression models and Kaplan–Meier survival analysis.

Results

We included 5075 patients with UVH including 758 (14.9%) patients with hypoplastic left heart syndrome (HLHS), and 50,620 matched controls. Median follow-up time was 13.6 (IQR 0.7; 26.8) years. The hazard ratio for death in patients with UVH was 53.0 (95% confidence interval, 48.0–58.6), and for HLHS, 163.5 (95% CI, 124.3–215.2). In patients with HLHS, 84% of those who were born between 1982 and 1993 died or had transplantation during the first year of life compared with 29% born between 2006 and 2017. In patients with UVH without HLHS, death/transplantation in the first year of life declined from 36% in those born between 1970 and 1981 to 8.7% in those born between 2006 and 2017.

Conclusions

The risk of mortality was >50 times higher in patients with UVH than in controls. The survival rate increased with a later decade of birth but was still <75% in patients born with HLHS.

Background

Sodium glucose transporter 2 inhibitors (SGLT-2i) have shown safety and efficacy in patients with heart failure (HF). However, evidence for the use of SGLT-2i in adult congenital heart disease (ACHD) patients with HF is limited.

Methods

We performed a retrospective, single center analysis of 18 patients (>18 years of age) with ACHD and a diagnosis of HF who were initiated on an SGLT-2i. Patient characteristics, including vital signs, laboratory values, concomitant medications, clinical outcomes, and echocardiograms, were obtained as part of standardized clinical care at our ACHD program before and 2–6 months after initiation of SGLT-2i. The primary outcome was to demonstrate safety of SGLT-2i initiation via potential changes in systolic blood pressure, serum sodium, and serum creatinine.

Results

Of the 18 patients, 11 (61%) had moderate complexity congenital heart disease while 7 (39%) had great complexity congenital heart disease. Post initiation, there were no significant differences in systolic blood pressure (121.8 ± 20.8 mmHg to 114.4 ± 14.9 mmHg, p = 0.06), sodium level (138.7 ± 2.9 mMol/L to 138.0 ± 2.2 mMol/L, p = 0.75), and creatinine level (0.85 ± 0.18 mg/dL to 0.89 ± 0.18 mg/dL, p = 0.07). There was a statistically significant decline in weight (78.9 ± 22.9 kg to 76.0 ± 23.0 kg, p = 0.0039) but without a statistically significant change in NT-pro NBP (1358.2 ± 2735.0 pg/mL to 601.6 ± 786.1 pg/mL, p = 0.36).

Conclusions

We demonstrated the use of SGLT-2i in a small cohort of ACHD population, including patients with complex congenital heart disease, appears safe and well tolerated. The safety and potential efficacy of SGLT-2i in patients with ACHD will require further evaluation in prospective multicenter studies.

Background

Patients with palliated pulmonary valve stenosis (PVS) have less cardiac remodeling and symptoms as compared to patients with repaired tetralogy of Fallot (TOF) presenting with similar severity of right ventricular outflow tract (RVOT) disease. What is not known is whether patients with PVS versus TOF presenting with similar severity of RVOT disease at baseline, would have similar (or different) pace of cardiac remodeling and disease progression over time. The study objective was to compare temporal changes in clinical and cardiac function indices between adults with palliated PVS and repaired TOF presenting with moderate/severe RVOT disease.

Methods

Cardiac function indices (based on strain imaging) and clinical indices (N-terminal pro–B-type natriuretic peptide [NT-proBNP], model for end-stage liver disease excluding international normalized ratio [MELD-XI], peak oxygen consumption [VO₂]), were assessed at baseline, 3 years, and 5 years. Temporal changes were calculated as relative changes from baseline (Δ). Cardiovascular adverse event was assessed as time-to-event outcome.

Results

Compared to TOF group (n = 173), the PVS group (n = 173) had less temporal change in right atrial reservoir strain (−9±4% versus −21 ± 6%, p < 0.001), RV free wall strain (−8±4% versus −20 ± 5%, p < 0.001), NT-proBNP (8 ± 5% versus 17 ± 6 %, p < 0.001), MELD-XI (6 ± 4% versus 19 ± 4%, p = 0.008), and peak VO₂ (−7±3% versus −12 ± 7%, p < 0.001) at 5 years. The 5-year freedom from cardiovascular adverse event was higher in the PVS group (76% versus 54%, p = 0.01).

Conclusions

These data suggest that a less frequent clinical and imaging follow-up may be appropriate in patients with PVS (as compared to patients with TOF).

Aim

To highlight the potential lack of documentation of thymectomy on historic cardiac operation notes and how this impacts the safety of the yellow fever vaccination for patients who have had congenital heart surgery in childhood.

Background

With advances in treatment, the population of adults with congenital heart disease (ACHD) is ever growing. Consequently, increasing numbers of patients wish to travel and work abroad. In recent years, this has presented a unique challenge in terms of the safety of the yellow fever vaccine in this patient group.

Background

Latin American registries of clinical and demographic profiles of ACHD are scarce. International guidelines classify disease complexity with different approaches. With these two regards, a registry was carried out to examine factors associated with mortality and to compare severity classifications in our population.

Methods and results

Cross-sectional study conducted on ACHD between 2018 and 2022 to evaluate clinical and demographic characteristics and to assess the agreement between the 2020 ESC Guidelines and 2018 AHA/ACC Guidelines for the Management of Adults with Congenital Heart Disease using the kappa method. Binomial logistic regression models were used to examine correlates of mortality. 3459 patients were included [56 % women, median age 34 years (IQR 24–50)]; 83.41 % were alive and 4.11 % died. The subjects had the following characteristics: 74.18 % were in NYHA I FC, 87.30 % had SVEF ≥50 %, 18.42 % developed arrhythmias, 58.92 % were surgically repaired, 7.05 % received palliative management, and 0.03 % were in heart transplant protocol. The agreement between ESC and AHA/ACC complexity classifications was low (43.29 %) in moderate ACHD, and high (83.10 %) in severe disease. Mortality was higher in patients with NYHA III-IV FC, arrhythmias and under palliative care.

Conclusion

This study found that ESC and AHA/ACC complexity classifications have limited concordance in categorizing moderate complexity CHD. Reparative procedures had lower mortality odds than palliative care.

Aims

Patients with D-transposition of the great arteries (D-TGA) and atrial switch experience late morbidity and mortality related to atrial arrhythmias and systemic right ventricular (SRV) failure. We sought to analyze the influence of atrial arrhythmias on long-term outcomes in this group.

Methods

A retrospective review of all patients with D-TGA and atrial switch followed at a tertiary care center was performed.

Results

148 patients (63.5 % male; age 30.4 ± 10.6 years) were followed for 12 ± 9.8 years. Death or cardiac transplantation occurred in 22(15 %) patients and heart failure hospitalization occurred in 30(20 %) patients.

Atrial arrhythmias were documented in 82(55.4 %) patients. Atrial fibrillation at the first visit (Kaplan-Meier estimate, p = 0.003) and atrial fibrillation as a time-dependent variable (HR 3.50, p = 0.006) predicted increased risk of death or cardiac transplantation. A triad of atrial fibrillation, prolonged QRS duration/RBBB, and severe SRV dysfunction (SRV EF < 35 %) emerged as a unique signature of a higher-risk population.

Atrial tachycardia and flutter, while not associated with mortality, increased the risk of heart failure hospitalization (HR 3.5, p = 0.001). Moreover, 2/6 cases of resuscitated sudden cardiac arrest were caused by atrial flutter, and more patients received inappropriate shocks for atrial arrhythmias(16 %) than appropriate shocks(2.3 %).

Conclusion

In D-TGA patients with atrial switch, there is a complex interplay between atrial arrhythmias and the SRV. Key ECG parameters, arrhythmia events and sequelae create a unique patient-specific fingerprint strongly associated with future events and mortality. This higher-risk cohort will need further characterization to delineate who may benefit from preemptive arrhythmia intervention.

Background

Brady- and tachyarrhythmias commonly complicate adult congenital heart disease (ACHD). Permanent pacemakers (PPMs) or implantable cardioverter–defibrillators (ICDs) are often utilised to prevent morbidity or mortality related to arrhythmia, but can also be associated with significant morbidity themselves.

Methods

We analysed outcomes from patients in our comprehensive ACHD database who were seen at least twice since 2000 and once since 2018. Of 1953 ACHD patients, 134 had a PPM and 78 had an ICD (47 for primary and 31 for secondary prevention).

Results

For PPM patients, 41% had a pacing percentage below 33%, 13% had 33–66%, and 46% had above 66%. One fifth required PPM upgrade, most to cardiac resynchronisation therapy, the rest to ICD. There were 33 appropriate ICD shocks in 15 patients (19%) and 34 inappropriate shocks in 13 patients (17%) over a median follow up of 4.6 years (IQR 0.9–8.3 years). Anti-tachycardia pacing was delivered appropriately for 28% of patients and inappropriately for 9%.

Apart from inappropriate therapy, one third of PPM and ICD patients had other device-related complications. Acute PPM complications included lead dysfunction requiring revision (2%), pneumothorax (2%), pleural effusion (2%) and pocket infection (2%). ICDs were also acutely complicated by lead dysfunction (4%) as well as pocket hematoma (3%). The most common long-term complication overall was lead dysfunction, affecting one sixth of both PPM and ICD patients. Finally, the rate of device insertion increased significantly with disease severity.

Conclusions

Anti-arrhythmic devices can be lifesaving in ACHD patients, but inappropriate therapy and device-related complications are very common.

Background

Aortic dilation, cardiac malformations and hypertension are known risk factors for aortic dissection in Turner syndrome (TS). In the current guidelines, rapid growth of the aorta has been added as a risk marker. This study aimed to estimate the growth of the ascending aorta over time, to identify risk factors of aortic growth, and to describe aortic complications in TS.

Methods

A transthoracic echocardiogram was performed at least twice in 101 women with TS, mean age 28 years, with a mean follow-up of 8.3 ± 3.4 (range 1–17) years. The investigator was blinded to the clinical status. Logistic regression analysis was used to identify risk factors of aortic growth.

Results

The prevalence of ascending aortic dilation (ASI >20 mm/m²) was 26 % and the mean ascending aortic diameter was 27.0 ± 4.8 mm at baseline. Significant aortic growth was found at sinus of Valsalva 1.08 (±2.11) mm, sinotubular junction 1.07 (±2.23) mm, and the ascending aorta 2.32 (±2.93) mm, p < 0.001. The mean ascending aortic growth rate was 0.25 (±0.35) mm/year, and higher compared to the general female population, 0.12 (±0.05) mm/year, p < 0.0001. No risk factors for aortic growth (bicuspid aortic valve, coarctatio, hypertension or karyotype) other than body weight could be identified, Odds ratio 1.05 (95 % CI 1.00–1.09), p = 0.029. Eight women had an aortic event of whom all had bicuspid aortic valves.

Conclusions

The growth rate of the ascending aorta in TS was increased compared to the general female population. Congenital cardiovascular malformations were not predictive of aortic growth.

Background

Pregnancy in women with congenital heart disease (CHD) is associated with an increased risk of adverse maternal and fetal events. Despite the physiological impact of CHD on pregnancy, current risk stratification scores primarily consider anatomical lesions. We assessed the performance of the novel American Heart Association Anatomic and Physiological (AP) classification system in predicting adverse maternal cardiac, obstetric and fetal events, and compared it with established risk models.

Methods

This retrospective cohort study enrolled pregnant women with CHD managed by the Westmead Hospital high-risk pregnancy team. Preconception risk stratification scores (AP classification, mWHO classification, CARPREG II and ZAHARA scores) were retrospectively assigned to each pregnancy by an adult CHD cardiologist and compared with the primary outcome measures, which were maternal cardiac, obstetric and fetal complications.

Results

We analysed 176 pregnancies in 120 women with CHD. Maternal cardiac risk significantly increased between AP class 2 and 3 (p = 0.001). Within class 3, higher physiological status correlated with maternal cardiac events (p < 0.001). Increasing AP severity correlated with lower fetal birthweight percentiles (p = 0.003). The AP classification was similar to mWHO at predicting maternal cardiac outcomes (AUC 0.787 vs 0.777, p < 0.001), but the CARPREG II (AUC 0.852, p < 0.001) and ZAHARA scores (AUC 0.864, p < 0.001) had higher discriminatory ability within our cohort.

Conclusion

The AP classification system shows non-inferior preconception maternal cardiac risk prediction compared to current validated scores. Consideration of physiological status has additive predictive value in the most complex patients (Stage III). Prospective, multicenter studies are required for further validation for preconception risk estimation.