International journal of cardiology. Congenital heart disease最新文献

Thanks to advances in both medical care and surgical techniques, over recent years, survival for patients with congenital heart disease (CHD) has increased significantly, with about 90% of patients now reaching adulthood. However, as CHD patients grow older, their relative risk of developing coronary artery disease (CAD) increases. Moreover, it has been demonstrated that the majority of adult congenital heart disease (ACHD) patients has at least one cardiovascular risk factor. On the other hand, common complications, such as atrial fibrillation (AF) may evolve into a major clinical concern and can be difficult to manage medically.

This review aimed at examining the current pharmacological treatment strategies for primary and secondary prevention of CAD, medical and interventional treatment for supraventricular arrhythmias, as well as optimal medical strategies for ACHD patients with CAD and AF.

Aims

To define the cohort of adult congenital heart disease (ACHD) patients undergoing catheter ablation for supraventricular arrhythmia in a large tertiary centre, characterise outcomes, and determine factors associated with arrhythmia recurrence.

Methods

Single centre retrospective study of all catheter ablations for atrial arrhythmias in ACHD patients between September 2014 and October 2021. Patients were identified using a field search through a centralised database. Pre-specified clinical and procedural data of interest, and time from ablation to recurrence, were determined. Cox regression analyses were used to determine potential predictors of acute procedural success and arrhythmia recurrence.

Results

Cardiac ablation for supraventricular arrhythmia was undertaken in 142 cases across 100 unique patients (median age 41, interquartile range 31–52), with 70 (49%) cases treated for macro-reentrant atrial tachycardia, 16 (11%) for focal atrial tachycardia, 19 (13%) for multifocal atrial tachycardia, 17 (12%) for atrial fibrillation, 7 (5%) for atrioventricular nodal reentrant tachycardia, and 8 (6%) for atrioventricular reentrant tachycardia; and 68 cases (48%) had recurrent arrhythmia with a median time to recurrence of 800 days. Multivariable analysis identified acute procedural success as an independent predictor of freedom from arrhythmia, and ablation for persistent atrial fibrillation as an independent predictor for recurrence of arrhythmia.

Conclusion

Catheter ablation for supraventricular arrhythmia in ACHD patients is safe and effective, with most patients achieving multiple arrhythmia-free months. Procedural success and arrhythmia mechanism are important predictors of recurrence.

Advances in diagnostics and interventional/surgical treatment of patients with congenital heart disease (CHD) over the past several decades, allows a decline in rates of all-cause mortality, with a significant reduction in proportion of infant and childhood deaths with severe forms of CHD. However, the risk of premature death in adults with complex CHD remains elevated. A growing body of evidence has recently described the impact of non-cardiovascular comorbidities such as cancers, on morbidity, health care utilizations and mortality in adult patients with CHD. Aim of this review is to provide an appraisal of the current evidence that report associations between CHD and increased cancer risk in children and adults, and provide an overview of the specific risk factors.

Nutrition is the cornerstone of a healthy life and is crucial for the prevention of cardiovascular disease. Patients with congenital heart disease (CHD) are prone to nutrition disorders, including abnormalities in body composition such as overweight or obesity which, along with other classic cardiovascular risk factors, places our increasing and aging adult CHD (ACHD) population at a higher risk for acquired cardiovascular disease. These patients are also at risk of cachexia or sarcopenia as well as macronutrient and micronutrient deficiencies derived from the development of heart failure as a complication of their underlying cardiac disease. In this paper, we review different dietary recommendations and supplementation with specific macronutrients and micronutrients. We also discuss some special scenarios in ACHD patients and the importance of the microbiota, a new therapeutical target as yet underexplored in this patient population.

The number of women with congenital heart disease (CHD) surviving to childbearing age is continuously growing. Although most pregnancies in this patient-population are well tolerated, there is a significant risk of potentially fatal complications. We describe a case of a previously completely asymptomatic patient who was diagnosed late during pregnancy with an inferior sinus venosus type atrial septal defect (ISV-ASD) and anomalous connection of all right pulmonary veins (PAPVC) who presented a cardiac arrest with ventricular fibrillation the day after delivery. She recovered completely and underwent subsequent surgical repair and implantation of a subcutaneous defibrillator (S-ICD).

Background

From 1977 onwards, patients with both simple and complex transposition of the great arteries (TGA) have been treated with the arterial switch operation (ASO) in the Wilhelmina Children's Hospital/University Medical Center Utrecht the Netherlands. In this study, we compared mortality and morbidity between two patient groups: A. operated before and B. after 1991, specifically focusing on late ventricular function and reinterventions.

Methods

A single institution retrospective cohort study was performed on patients who had an ASO for either simple or complex TGA. Data were collected from medical records. The entire patient cohort (n = 283) was divided in a group with more than 30 years of follow-up (A) and a group with less than 30 years of follow-up (B). Clinical and standardized echocardiographic follow-up was evaluated.

Results

Group A consisted of 79 patients, of whom follow-up was available in 59 patients (median follow-up 34.8 years, IQR 33.0–36.9). Group B consisted of 204 patients, of whom 195 long-term survivors (median follow-up 14.9 years, IQR 10.0–21.2). Early survival was best in group B (A: 67.8% vs. B: 96.6%, p < 0.001), whereas late mortality (in total 1.8%) was similar for both groups. Reinterventions, corrected for follow-up time, were more frequent in group A (p = 0.005). In total 65 patients (25.1%) required 105 late reinterventions including 4 late aortic valve replacements. The mode of reinterventions has shifted over time, from surgical to more catheter-based (p = 0.03). The vast majority of patients functioned in NYHA class I. In contrast to the recent cohort, who have a normal average LVEF (%), the average LVEF in the oldest cohort was in the bottom percentile of normal range.

Conclusion

The majority of patients in their fifth decade after ASO are in functional class I. Early outcome improved showing reduced mortality and need for reoperation. However, a trend towards reduced left ventricular function and late aortic valve replacements justify further research.

Background

Patients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear.

Methods

Retrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality.

Results

Median age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O₂ saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02).

Conclusions

Patients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.

Background

Nikaidoh translocation operation is increasingly being used for patients with TGA, VSD and LVOTO. The early results of this operation are excellent and possibly better than the Rastelli repair. However, the long-term results remain inadequately defined.

Methods and results

Detailed follow up data of a patient, 12 years after Nikaidoh operation, using computerized image analysis are reported. The patient complained of chest pain on exertion. Imaging showed severe dilatation of the non-coronary sinus of the aortic root, compressing the RVOT and pulmonary artery branches resulting in RV dilatation. The dilated aortic sinus showed marked pulsatility with expansion during systole and diastole. As the child was symptomatic, with a risk of aneurysm rupture, excision was performed. This complication is thought to be due to interruption of the innervation of the root as well as cutting vasa vasorum.

Conclusion

Nikaidoh operation is a viable solution for the repair of TGA, VSD, PS. Dilatation of the neo-aortic root should be carefully followed and causes of the dilatation investigated.

Despite striking improvement in survival of patients with congenital heart disease (CHD), the risk of long-term complications remains high. Stroke and systemic embolism are common and potentially devastating complications that significantly affect morbidity and mortality in CHD. The risk of stroke in adult congenital heart disease (ACHD) is higher than in the general population, patients are affected at an earlier age, and the risk continues to increase with age. Specific types of defects are at high-risk for stroke and other systemic embolisms, particularly patients with complex congenital heart disease, cyanotic heart disease, Fontan circulation, and cardiac shunts. Associated factors such as atrial arrhythmias, heart failure, mechanical valves and intracardiac devices, and infective endocarditis increase the risk of thromboembolic events. Acquired conventional risk factors for cardiovascular disease further increase the burden of stroke in the aging ACHD population. Anticoagulation is a cornerstone for prevention of thromboembolic events. Risk stratification in ACHD remains challenging and should be individualized. General risk stratification models, such as the CHA₂DS₂-VASc score, are not reliable in the heterogeneous ACHD population and should only be used in mild to moderate CHD. Anticoagulation is recommended as primary prevention in high-risk patients (patients with intracardiac repair, cyanotic CHD, Fontan circulation, or systemic right ventricle) with atrial arrhythmias. In patients with other CHD, general stratification models should be used to decide when to initiate anticoagulation, taking into account specificities of underlying heart disease and potential residua. Screening and treatment of conventional risk factors for cardiovascular disease may further improve long-term outcomes.

Background

In order to prevent right ventricular (RV) dysfunction, closure of secundum type atrial septal defects (ASD) is often indicated and percutaneous closure is the preferred treatment modality to do so. The magnitude and time course of RV remodeling is still incompletely understood.

Methods

This retrospective cohort study included consecutive patients who underwent percutaneous secundum ASD closure in two tertiary referral centers in The Netherlands. Main study parameters were RV and right atrial dimensions measured with transthoracic echocardiography before and after percutaneous ASD closure. Secondary outcome was change in New York Heart Association (NYHA) functional class at follow-up.

Results

From the 454 patients who underwent secundum ASD closure, 88 patients (median age 46 [range 17–84]) were included. The majority of RV and right atrial dimensional improvement occurred within 24 h. After a median follow-up of 569 days (IQR: 280–772) a further decrease in dimensions was observed. Comparing baseline and latest follow-up, end-diastolic RV basal diameter decreased from 4.5 SEM 0.1 to 3.9 SEM 0.1 cm (p < 0.001) and end-systolic right atrial area from 22.9 SEM 1.0 to 17.9 SEM 0.7 cm² (p < 0.001). No significant changes in RV function were observed. NYHA functional class improved from 1.5 at baseline (IQR: 1.0–2.0) to 1.0 (IQR: 1.0–1.5) at latest follow-up (p < 0.001).

Conclusion

Remodeling of the RV heart dimensions commences within 24 h after percutaneous secundum ASD closure for the majority of patients, followed by a further gradual recovery. A concurrent improvement of NYHA functional class was observed during follow-up.