Heart failure in congenital heart disease: management options and clinical challenges

Figures & data

Figure 1. Changing prevalence of Congenital Heart Disease (CHD) in the European Union by age group. The rough estimates of the total population of children, adult patients 18–60 and adult patients >60 years with CHD are based on published birth rates (in 2008) and consider only patients born after 1970, ignoring adult mortality. Assumptions are based on currently reported survival into adulthood, reported rates of patients >60 years and records of the German Competence Network for Congenital Heart Disease. ACHD: Adult Congenital Heart Disease. Reproduced with permission [4].

Figure 2. Mechanism of death in 1573 adult patients with congenital heart disease. See text for references.

Figure 3. Biomarker levels in adults with congenital heart disease according to the type of congenital heart disease. Combined data previously published by Baggen 2017, Baggen 2018, Eindhoven 2015, Geenen 2019 [42,50–51–52].

Figure legend: For NT-proBNP and GDF-15 the x-axis is on the 2log scale. Abbreviations: AoS = aortic stenosis, CoA = coarctation of the aorta, ToF = Tetralogy of Fallot, TGA-ASO = transposition of the great arteries corrected by the arterials witch operation, RV = right ventricle, Univentricular = functionally univentricular hearts, PAH = pulmonary arterial hypertension, NT-proBNP = N-terminal pro B-type natriuretic peptide, Hs = high sensitivity, CRP = C-reactive protein, ST2 = suppression of tumorigenicity-2, GDF-15 = growth differentiation factor-15

Figure 4. Heart-failure free survival according to the number of abnormal biomarker levels in adults with congenital heart disease. Combined data previously published by Baggen 2017, Baggen 2018, Eindhoven 2015, Geenen 2019 [42,50–52].

Figure legend: Included biomarkers and definitions of abnormal levels: NT-proBNP (>14 pmol/L), hs-troponin T (>14 ng/L), hs-CRP (>3 mg/mL), sST2 (♂> 55.85 ng/mL, ♀> 44.50 ng/mL), RDW (>16%), and GDF-15 (>1109 ng/L)

Figure 5. Proposed diagnostic and treatment algorithm in ACHD patients with signs/symptoms of heart failure.

Abbreviations: ACHD = Adult Congenital Heart Disease, CRT-D = cardiac resynchronization therapy-defibrillator, MCS = mechanical support, HTx = heart transplantation

Table

	Indications	Limitations
Electrocardiogram	Sinus rhythm; atrial arrhythmias, e.g. atrial fibrillation; conduction delay	May not show paroxysmal arrhythmias
Laboratory testing	Kidney, liver and thyroid function; hemoglobin; infection parameters; clotting function; specific heart failure biomarkers
Transthoracic echocardiogram	Myocardial contractile function; diastolic function; valvar function; intra-cardiac pressures; pulmonary pressures; dimensions of great vessels; function of baffles and conduits; pericardial fluid	Poor acoustic windows
Chest radiography	Cardiac size; pleural effusion; hilar enlargement
Magnetic resonance imaging	Ventricular dimensions and function; valvar function; shunt fraction (Qp:Qs); baffle and conduit function; anatomic relationships	Artifacts from implants, claustrophobia
Computer tomography	Anatomic relationships; dimensions of vessels and structures	Possible use of iodine-based contrast agent, irradiation
Transesophageal echocardiography	Anatomic relationships; valvar function; intra-cardiac shunts	Semi-invasive
Cardiopulmonary exercise testing	Exercise capacity; rhythm disturbances; blood pressure at exercise	Inability to exercise
Tape monitoring	Heart rate frequency; atrial and ventricular rhythm abnormalities such as ectopic beats; atrial tachycardia and fibrillation and (non-sustained) ventricular tachycardia	May not show paroxysmal arrhythmias
Blood pressure recording	Readings during day and night and during different activities
Coronary angiography	Course and condition of coronary arteries	Invasive, use of iodine-based contrast agent, irradiation
Left heart catheterization	Pressures and gradients at different locations, such as mitral valve, left ventricle, aortic valve, coarctation	Invasive
Right heart catheterization	Anatomy of venous structures; pressures at different levels, such as caval veins, right atrium, right ventricle, pulmonary artery (trunk and branches) and wedge pressure; Pressure gradient across pulmonary valve and conduits	Invasive

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