International journal of cardiology. Congenital heart disease最新文献

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.

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.

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

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.

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

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.

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.

Right ventricular outflow tract ventricular tachycardia (RVOT VT) is a well-recognised subtype of idiopathic ventricular tachyarrhythmia and often described in young healthy individuals. This arrhythmia typically occurs in patients with a structurally normal heart, and in this context is generally considered benign. In this case we present a rare association between RVOT VT and a ruptured sinus of Valsalva aneurysm in a patient known to have a restrictive ventricular septal defect. Prompt surgical intervention was necessary to prevent potentially life threatening consequences.

The diagnostic and surgical evaluation learnt from this case would be useful to remind clinicians to explore rarer causes of RVOT VT especially when patients have underlying congenital heart disease. This case also highlights the importance of utilizing multi-modality imaging for the anatomical assessment of this pathology, which aids definitive management.

Background and aims

Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD.

Methods

DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files.

Results

In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1–45.2) and for repaired AVSD 38.5 years (95% CI 35.3–41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair.

Conclusions

The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.

Background

Adult congenital heart disease (ACHD) is a lifelong illness that presents ongoing challenges to quality of life. Fostering personal resilience resources to sustain well-being can enhance patients’ psychosocial health.

Objective

We aimed to describe patients’ resilience experiences: how they understand, develop, and utilize resilience resources in managing ACHD.

Methods

We conducted a qualitative study of patients with ACHD. Participants were recruited using maximum variation sampling. Individual, semi-structured interviews were conducted June 2020 to August 2021. We queried approaches to managing ACHD-related stress and experiences with resilience and analyzed responses with thematic analysis.

Results

Participant (N = 25) median age was 32 years (range 22–44); 52% identified as female and 72% non-Hispanic white. Participants’ anatomic ACHD was moderate (56%) or complex (44%); physiologically, 76% were functional class C or D. Participants described various resilience resources, which map to an established resilience framework: 1) internal resources: maintaining positivity, self-directed activity, and setting goals; 2) external resources: social support; 3) existential resources: purpose, gratitude, and cultivating health. Even among participants who reported feeling unfamiliar (8/25) with the term “resilience,” all participants shared experiences reflecting resilience developed while living with ACHD.

Conclusion

ACHD-relevant resilience resources may help patients and clinicians navigate ACHD-related stress and promote psychosocial well-being.