International journal of cardiology. Congenital heart disease最新文献

Developments in medical and surgical techniques have improved survival in women with congenital heart disease (CHD) with most now surviving well into adulthood. Reproductive health amongst these women is underexplored and needs more attention. Women with CHD are known to have more menstrual dysfunction than the general population and have higher maternal and fetal risks when they become pregnant. Adequate and timely preconception counselling, including contraception within a multidisciplinary team (MDT) are essential to optimise pre pregnancy cardiac status and improve pregnancy outcomes. Counselling regarding fertility, sexuality, contraception and menopause is necessary and should start early, around 12–13 years, and as they transition into adult services. Fertility seems to be reduced in women with complex CHD and consideration for assisted reproduction technique (ART) should be assessed by the MDT as risks associated with ART including ovarian hyperstimulation syndrome, bleeding, thrombosis and infection can have profound effects on women with complex cyanotic CHD.

Background

Thyroid dysfunction may have adverse effects on Fontan hemodynamics. Data on thyroid function in pediatric Fontan patients with or without protein-losing enteropathy (PLE) are limited.

Methods

This retrospective multicenter study included 67 Fontan patients (median age 10.9 years; 35.8% female; 28.4% PLE) in whom thyroid function testing was performed.

Results

Subclinical hypothyroidism (SHT) was present in 16 (23.9%) patients. Subjects with SHT had significantly lower systolic blood pressure (p = 0.014) and body weight z-score (p = 0.006), were in a worse New York Heart Association (NYHA) functional class (p = 0.004), were more often pacing dependent (p = 0.007), and were more likely to have PLE (p = 0.033, 8/19 (42.1%) patients). Serum thyroid stimulating hormone (TSH) levels were significantly higher in patients with NYHA class ≥ II (p = 0.005), significant atrioventricular valve regurgitation (p = 0.023), elevated serum natriuretic peptides (p = 0.031), and in those with PLE (p = 0.002). Patients with active PLE had significantly higher TSH levels than those in remission (p = 0.003). A strong inverse relationship was found between lower free triiodothyronine (fT3) levels and natriuretic peptides (r: −0.599, p = 0.040). Using binary logistic regression analysis we found that worse NYHA class was an independent predictor of SHT (OR 4.2; 95% CI 1.1–16.1, p = 0.036).

Conclusions

Subclinical thyroid dysfunction is common in Fontan, particularly in patients with hemodynamic derangements and PLE. Future studies are needed to address the prognostic implications of thyroid dysfunction in the Fontan population.

The number of adult patients with congenital heart disease (CHD) is steadily increasing and exceeds nowadays the number of children with CHD. This is due to significant advances in therapeutic possibilities that became available over the last four decades. As such, this aging population survives the CHD complications and is exposed to the traditional cardiovascular risk factors for atherosclerotic disease such as high blood pressure, elevated cholesterol levels, long-standing exposure to smoking, overweight and obesity, and a sedentary lifestyle. Consequently, it becomes important to put more emphasis on all these risk factors. A preventive strategy is central, and early encouragement of physical activity is part of this approach. A minimum of physical activity has a beneficial effect both physically and mentally. With this overview, we mainly want to emphasize the importance of preventive measures. We would like to emphasize that all individuals should receive an exercise prescription which adheres to the minimum recommendations by WHO/NICE and this advice should form the baseline. Moreover, we intend to show that physical activity can be done safely in patients with CHD and that recreational and competitive sports are feasible in many circumstances.

Objectives

To investigate the incidence, types and pathophysiology of criss-cross pattern of the proximal pulmonary arteries in common arterial trunk and its influence on management and outcomes.

Methods

Retrospective review of all patients with common arterial trunk who underwent Aswan Truncus Operation with regard to the origin and course of the proximal pulmonary artery branches as defined by 3D segmented models pre, immediately and 1 and 2 years after operation.

Results

Between January 2019 and September 2022, 39 patients (19 males, aged 1–12 months) underwent the Aswan Truncus Operation. 18 patients (46%) had criss-cross arrangement of the proximal pulmonary artery. The mortality, ICU and hospital stay were not affected by the presence or absence of the criss-cross pattern. However, following operation in the criss-cross group, there was evidence of different degrees of kinking and twisting of the right pulmonary artery. This resulted in severe narrowing which required stenting or reoperation at different stages (1 during ICU stay and another after 1 year). Separate origin of the left pulmonary artery (Criss-cross Type 2) from a long stenotic ductus arteriosus required resection of the stenotic segment followed by anastomosis to the right pulmonary artery recreating a 60° angle of bifurcation.

Conclusion

Criss-cross pattern of proximal pulmonary arteries is common in patients with common arterial trunk. Pre-operative diagnosis and tailored approaches of repair are required to prevent late complications.

In 2020, there was an estimated prevalence of more than 1.8 million adults with congenital heart disease (ACHDs) living in South America, and 677,000 in Central America and the Caribbean. The ACHD mortality is higher in developing countries, compared with developed countries, and it has been shown that concentrating this population in specialized ACHD units improves their survival and prognosis. Currently, Latin American (LATAM) and Caribbean countries have an insufficient number of specialists and specialized ACHD units. Analyzing this situation, the Inter-American Society of Cardiology's (IASC) ACHD chapter and Pediatric Cardiology Council have prepared the first recommendations for developing ACHD units in LATAM and Caribbean countries. This article is the first collaborative work between South American, Central American and Caribbean countries. It describes the main points for organizing and developing ACHD units adapted to our own reality. Each point has been discussed in terms of barriers and challenges, followed by specific recommendations for improving and developing ACHD care. They have been reviewed and endorsed by the International Society for Adult Congenital Heart Disease (ISACHD), and the goal of their implementation is for each ACHD unit in the region to have the standards of quality and efficiency to improve the prognosis and survival of ACHDs in the region.

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.