Table 1 Characteristics of the included studies. * median & IQR

Videbaek et al., 2015

Europe

Prospective

Adults only

-

47.4 years (IQR: 43.5–50.9) *

To study the long-term prognosis of simple CHD

Patients diagnosed with simple CHD in the 1960s have substantially increased long-term mortality and cardiac morbidity compared with the general population. Further studies on the effectiveness of systematic medical follow-up programs appear warranted.

Mandalenakis., 2016

Europe

Prospective

Adults and children

ischemic

9.51

Study the relative risk and potential factors for developing ischemic stroke in children and young adults with CHD in Sweden.

The risk of developing ischemic stroke was almost 11 times higher in young patients with CHD than in the general population, although absolute risk is low. Cardiovascular comorbidities were strongly associated with the development of ischemic stroke in young CHD patients.

Lin et al., 2014

Asia

Retrospective

Adults only

-

36.5

To identify the long-term major adverse cardiovascular events (MACE) in adult congenital heart disease (CHD) patients in Taiwan.

Taiwanese patients with CHD were at an increased risk of life-long cardiovascular MACE, including heart failure, stroke, acute coronary syndrome, and malignant dysrhythmia. Surgical correction may help to decrease long-term MACE in CHD patients, especially those with ASD.

Maxwel et al., 2013

United States

Retrospective

Adults only

-

57.85

To describe the risk of patients with congenital heart disease having noncardiac surgery

Compared with a matched control cohort, CHD patients undergoing noncardiac surgery experienced increased perioperative morbidity and mortality. Within the limitations of a retrospective analysis of a large administrative dataset, this finding demonstrates that this is a vulnerable population and suggests that better efforts are needed to understand and improve the perioperative care they receive.

Billett et al., 2008

Europe

Retrospective

Adults and children

Stroke and transient ischaemic attack

28.1

To determine the prevalence of comorbidities, patterns of healthcare utilization and primary care recording of clinical indicators in patients with congenital heart disease

There is a significant burden of comorbidity associated with congenital heart disease, and levels of primary care utilization and referral to secondary care are high in this patient group. The predicted future expansion in the numbers of adults with congenital heart disease owing to improvements in survival will have implications for primary and secondary care, and not just tertiary centers offering specialist care.

Dellborg et al., 2015

Europe

Bidirectional cohort

Adults only

-

70

the prevalence of ACHD in combination with T2DM to estimate the associated clinical risk, outcome and patient characteristics

Congenital heart disease and secondary risk factors for cardiovascular disease frequently coexist and the development of T2DM also in the ACHD population is not uncommon with an estimated prevalence of between 4 and 8%. Treatment of conventional cardiovascular risk factors in patients with congenital heart disease could be considered secondary prevention given the relatively high morbidity and high risk for mortality observed in patients with the combination of ACHD and T2DM.

Lee et al., 2023

Asia

Retrospective

Adults only

ischemic, hemorrhagic stroke

-

The incidence and risk of ischemic stroke (IS) and hemorrhagic stroke (HS) in Korean patients with CHD

Korean patients with CHD have a high risk of stroke. A personalized preventive approach is needed to reduce the incidence of stroke in this population.

Giang et al., 2021

Europe

Retrospective

Adults only

ischemic

CHD: 52.1 (IQR, 39.8–63.6)*

Controls: 66.0

(IQR, 57.1–73.4) *

Study the long-term outcomes after IS, including IS recurrence and mortality risk

Patients with CHD had a 5-fold higher risk of developing index IS compared with matched controls. However, the risk of recurrent IS stroke and all-cause mortality were 34% and 47% lower, respectively, in patients with CHD compared with controls

Yelton et al., 2023

United States

Retrospective

Children only

ischemic and hemorrhagic stroke (combination)

months

Stroke = 8(0-216)

No stroke = 28(0-216) **

To delineate prevalence of stroke in the pediatric intensive care unit and to determine risk factors for stroke and association of stroke with mortality in patients with congenital heart disease

Children with congenital heart disease are at increased risk for developing stroke, which is associated with increased mortality. Early identification of the most vulnerable patients may enable providers to implement preventative measures or rapid treatment strategies to prevent neurologic morbidity.

Pedersen et al., 2019

Europe

Retrospective

Adults only

ischemic

Ischemic: 53 years (IQR: 40–67) *

Hemorrhagic: 69 years (IQR: 55–78) *

Study and collect data regarding ischemic stroke risk and associated mortality in adults with CHD.

Both younger and older CHD adults have an increased risk of ischemic stroke and by 60 years of age 7.4% of CHD adults will have had an ischemic stroke. Post-stroke mortality was also increased in CHD adults compared with the general population

Giang et al., 2018

Europe

Retrospective

Adults only

intracerebral hemorrhagic stroke and subarachnoid hemorrhage

27

To study the risk of hemorrhagic stroke, including intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH) in CHD patients

The relative risk of hemorrhagic stroke among children and young adults with CHD was almost 8× higher than that of matched controls from the general population, although the absolute risk was low. The highest risk of ICH and SAH occurred in patients with severe nonnocturnal defects and coarctation of the aorta