Last year of life of adults with congenital heart diseases: causes of death and patterns of care.

Last year of life of adults with congenital heart diseases: causes of death and patterns of care.

Van Bulck L, Goossens E, Morin L, Luyckx K, Ombelet F, Willems R, Budts W, De Groote K, De Backer J, Annemans L, Moniotte S, de Hosson M, Marelli A, Moons P; BELCODAC consortium.Eur Heart J. 2022 Nov 7;43(42):4483-4492. doi: 10.1093/eurheartj/ehac484.PMID: 36030410

Dr Timothy Roberts

Commentary by Dr. Timothy Roberts (Melbourne, Australia), section editor of ACHD Journal Watch:

  • Adult patients with congenital heart disease (CHD) carry a risk for an increasing burden of symptoms and premature death.
  • Involvement of palliative care services is increasingly recognized as a critical component of comprehensive care for adults with CHD early in the disease trajectory, but patient and physician barriers to referral are likely.
  • This mortality follow-back study of Belgian CHD patients reported on the deaths of 390 adults over a 9 year period and the healthcare provision in the final year of life.
  • Patients with CHD were observed to die at an older age than historically reported (mean age 55 years) and less often due to cardiovascular causes, which may reflect improved management and long-term care of patients with mild and moderate CHD.
  • A high healthcare utilization was observed in the final year of life, although CHD physician encounters were less than one might expect and raises the question of adequacy of CHD physician involvement in the final stage of life.
  • Specialist palliative care involvement was very limited, especially in those with a cardiovascular cause of death.
  • The authors conclude that healthcare utilization at the end of life requires more attention, and changing end-of-life care needs and trajectories towards death should be further examined
  • The study is limited by the retrospective nature, reliance on entered data/coding, and of the single-nation population, but is a welcome addition to research highlighting the need to increase awareness and improve end-of-life care discussions in our CHD patients.


Premature death is reported in a high proportion of adults with CHD, with a median age of death still below 50 years of age. Given this elevated risk and associated burden of symptoms, an argument exists for the need for improved provision of palliative and end of life care. Palliative care is defined as ‘ an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illnesses, through the prevention and relief and suffering’. End-of life care is defined as ‘the process of supporting patients who are in their final months of life and their relatives’. A potential challenge for the initiation of such care is the challenge to define a prognosis and identify the triggering event for clinical deterioration.

This study aimed to (i) identify the causes of death, and (ii) describe the patterns of healthcare utilization
in the final year of life in adults with CHD.


The BELgian Congenital heart disease Database combining Administrative and Clinical data (BELCODAC) was used, incorporating administrative and clinical data of patients with CHD in Flanders and Brussels. A mortality follow-back study was performed in adult patients who died between 1 January 2007 and 31 December 2015, and who had lived in Belgium during their last year of life. A total of 390 patients (Table 1) died in this period at a median age of 55 years, with 51% being women. Most patients had a CHD of moderate (46 %) or mild (43 %) complexity.

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Causes of death were taken from ICD-10 codes on death certificates. Sudden death was defined as ‘a death, non-violent and not explained otherwise, occurring < 4 h from the onset of symptoms’.

Healthcare utilization was based on nomenclature codes derived from healthcare claims data, and analysed for the 327 patients who had a cause of death and died non-suddenly and non-accidently. Logistic regression analyses were used to examine differences between patients who died due to either a malignant or a cardiovascular cause of death.


Most patients (n = 327; 84%) experienced a non-sudden death, while 55 (14%) died due to a sudden, accidental, or violent cause of death comprising 28 (7%) having an accidental or violent cause of death (including 14 deaths due to intentional self-harm), 15 (4%) sudden cardiac death, and 12 (3%) dying suddenly with a noncardiac cause of death reported. The cause of death was missing or unclear in 8 (2%) patients.

Almost half of the study population (n = 174; 45%) died from a cardiovascular cause, of which 15 (4%) patients had a sudden cardiac death. Cardiovasuclar death in 30% (n = 51 / 174) had the CHD as reported cause of death, and 14 % (n = 25 / 174) died due to a stroke.

Cancer accounted for 16% (n = 64) deaths, with lung and breast cancer being most common. The remaining 104 patients died from respiratory diseases (n = 24; 6%), non-cardiovascular congenital malformations (n = 19; 5 %), digestive diseases (n = 12; 3%), endocrine, nutritional and metabolic disorders (n = 12; 3%), infectious and parasitic diseases (n = 9; 2%); diseases of the nervous system (n = 7; 2%), and other reasons (n = 21; 5 %).

Table 2 demonstrates the increasing prevalence of cardiovascular deaths with higher CHD complexity:

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Healthcare utilization and differences between those who died due to a cardiovascular cause or a malignant cause are shown in Figure 2. Notably, patients with a malignant cause of death had twice as many general practitioner visits in the final month of life, and more hospitalizations in the final year of life. Contact with CHD physicians during the last year and last month of life was greater in the patient subgroup dying due to a cardiovascular cause, and more underwent cardiovascular procedures. Admission to ICU was not significantly different between groups. Specialist palliative care provision differed significantly, with 4% in the cardiovascular group receiving input in the last month compared to 41% of patients with a malignant cause of death.

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Key findings the authors highlight from their study include:

  1. Less than half of people with CHD died due to cardiovascular diseases
  2. High healthcare utilization in the final year of life
  3. Use of specialist palliative care services was very limited – notably for patients with a cardiovascular cause of death.
  4. Prevalence of psychiatric disorders is higher in adults with CHD than in the general population, but even so the observed number of patient deaths due to intentional self-harm (3.6%) was disproportionately elevated compared to available comparative data in a Danish cohort of CHD patients.

Referral to palliative care services has been shown to decrease the number of inpatient stays and ICU admissions at the end of life, which could be an important strategy to lower unnecessary healthcare utilization. Barriers to early CHD physician referral of patients to palliative care services may include low level of palliative care knowledge, and concern about the readiness of patients to engage in such discussions.