Objectives

The fundamental objectives of training are to provide the best quality of care for the patients and a compassionate attitude to the patients and their parents or guardians.

• Training programmes in paediatric and congenital cardiology should provide an environment for acquiring the knowledge, skills, attitudes and competence, and clinical judgement essential for the specialty.

• Programmes should have an appropriate balance between clinical services, training, and academic endeavours.

• Programmes should encourage a commitment to continuous education, teaching, research, critical thinking, and deep insight into the practical and theoretical problems of the speciality. Therefore, it is important that the trainee undertakes clinical or basic research aiming for presentation and publication of the findings.

• The trainee should be educated in specialised departments skilled in all fields of CHD management and officially accredited for this specialty.

Trainee tasks

To ensure sufficient experience, the trainee should participate in an appropriate variety of patient management:

Out-patients: The trainee should be involved in the management of out-patients under supervision. These should include patients from neonates to adolescents with a variety of congenital and acquired cardiac diseases and adults with CHD.

In-patients: The trainee should be involved in the management of in-patients under supervision. These should include patients from neonates to adolescents with a variety of congenital and acquired cardiac diseases and adults with CHD.

Intensive care: The trainee should spend ample time in the ICU and should become involved in the treatment of a variety of congenital and acquired cardiac diseases in patients of all age groups.

Multidisciplinary meetings: The trainee should regularly attend multidisciplinary meetings where management decisions are discussed.

On-call commitment: The trainee should participate in the on-call schedules of the department with backup support provided by a paediatric or adult congenital cardiologist in order to achieve adequate experience in the management of emergencies.

Basic knowledge and skills

Physiology, pathophysiology, and haemodynamics of CHDs

Understanding of the principles of cardiovascular physiology and pathophysiology of the congenital lesions is essential. The knowledge should be applied theoretically and as well as in the clinical setting. Most common lesions are listed in Table 1.

Table 1. Most common congenital and acquired cardiac diseases from fetus to adult

Management of cardiovascular diseases

The trainee should acquire knowledge of different types of medical, surgical, and interventional treatments of cardiovascular diseases (see Table 1). This should include the indications, results, and associated complications of the treatment as well as the short term and late outcomes. Focus is expected on the following issues:

• The trainee acquires knowledge of the actions and side-effects of cardiovascular and non-cardiovascular drugs and their interactions with other pharmacological substances in different diseases and at different ages.

• The trainee focusses on the importance of a multi-disciplinary approach: paediatric and congenital cardiologists, interventionalists, radiologists, and cardiac surgeons have to be involved in the decision-making process. The detailed pre-operative or pre-interventional assessment should be encouraged and other sub-specialities be involved, if needed.

Basic, underlying principles of training are summarised in Table 2 (adapted from Ref. Reference Reinhardt, Hansmann and O’Sullivan3).

Table 2. Underlying principles of training

Morphology, embryology, anatomy, and epidemiology of cardiovascular disease

The trainee is expected to gain profound insight in cardiovascular morphology and pathology. Participation in an Association for European Paediatric and Congenital Cardiology-acknowledged course on cardiovascular morphology and pathology is strongly recommended for all trainees. Upon completion of the course, the trainee is expected to have the following knowledge:

Knowledge:

• Principles of cardiovascular development, including the genetic factors and influence of embryonic/fetal haemodynamics.

• Anatomy of the normal heart including its topographical relationships in various periods of life.

• Principles of the sequential segmental analysis of congenitally malformed heart.

• Morphology of the cardiac conduction system.

• Morphological features of:

  • septal defects

  • anomalies of the outflow tract

  • anomalies of the right heart

  • anomalies of the left heart

  • anomalies of the systemic and pulmonary veins

  • anomalies of the aorta and connected arteries

  • anatomy of functionally univentricular hearts

  • morphology of lateralisation defects’

  • morphological features of clinically significant coronary abnormalities

• Up-to-date terminology of CHDs as recommended by the Association for European Paediatric and Congenital Cardiology Coding Committee and current revisions of the International Classification of Diseases.

• Histopathological characteristics and staging of pulmonary hypertension.

• Morphological features of CHD in the adult population (including natural remodelling, post-surgical remodelling, and other morphological topics relevant for this population).

Skills:

• Initiate the adequate management of the patient upon morphological and pathophysiological diagnosis.

• Interpret the results of the various imaging techniques (echocardiography, CT scan, MRI, angiography) according to the knowledge acquired in cardiac morphology.

Attitude:

• Coordination of patient management with involvement of the potential sub-specialties (geneticists, surgeons).

Genetics, basic science, and myocardial disease

The trainee should acquire knowledge and experience in the basis of genetics and on diagnosis and treatment of inherited diseases associated with cardiovascular disorders.

Knowledge:

• Principles of Mendelian monogenic human diseases being autosomal dominant, autosomal recessive, and X-linked:

  • Mitochondrial patterns of inheritance

  • Polygenic cardiovascular diseases

  • Genes, genome, chromosomes

  • Methods: Sanger sequencing, next-generation sequencing, fluorescence in situ hybridisation, comparative genomic hybridisation

  • Relevance of polymorphisms and mutations

• Diagnosis and clinical presentation of most frequent syndromes, including:

  • Trisomy 21, 13, 18, and X0 (Turner)

  • 22q11 microdeletion syndrome

  • 7q11 microdeletion syndrome (Williams–Beuren) and elastin gene mutations

  • Noonan syndrome and RASopathies

  • CHARGE syndrome

  • Alagille syndrome

  • Holt–Oram syndrome

  • Char syndrome and other syndromes

• Diagnosis, clinical presentation, and treatment of cardiomyopathies, including:

  • Hypertrophic-, dilated-, restrictive-, and non-compaction cardiomyopathy

  • Mitochondrial diseases and Barth syndrome

  • Sarcomeric cardiomyopathies

  • Storage diseases and other inborn errors of metabolism

  • Neuromuscular disorders

  • Arrythmogenic cardiomyopathy

• Diagnosis, clinical presentation, and treatment of inherited arrhythmia syndromes, including:

  • Long and short QT syndrome

  • Brugada syndrome

  • Catecholaminergic polymorphic ventricular tachycardia

• Diagnosis, clinical presentation, and treatment of aortopathy syndromes, including:

  • Marfan syndrome

  • Loeys–Dietz syndrome

  • Ehlers–Danlos syndrome, Thoracic Aortic Aneurysm and Dissection syndrome

  • Arterial tortuosity syndrome

Skills:

• Evaluate the family history and draw a pedigree including the ability of interpretation (problems with pedigree interpretation as incomplete penetrance, variable expressivity, and age-related patterns of expressivity).

• Patient counselling (including prenatal) in collaboration with the geneticist after genetic testing and management of the uncertainties associated with genetic testing.

Attitude:

• Ability to communicate sensitively regarding to genetic family disorders.

• Recognition of importance of a multidisciplinary team, including geneticists and genetic counsellors.

Imaging module

The trainee should gain knowledge about the choice and frequency of imaging modality according to the lesion-specific patient characteristics as well as the strengths and weaknesses of each imaging modality.

Cardiac catheterisation and angiocardiography

Diagnosis and treatment of cardiac rhythm disorders

Intensive care

The trainee should acquire experience in the daily activity of a paediatric cardiac ICU including invasive techniques applied in daily patient care. It is also recommended to spend ample time in the cardiac operating theatre in order to learn the basics of cardiopulmonary bypass and to be aware of the different surgical techniques. ^{Reference da Cruz, Lechner and Stiller2}

Knowledge:

• Neonatal circulation, transition to extra-uterine circulation, and its impact on clinical management of critically ill neonates with cardiac disease.

• Basics of intensive care management of most common congenital and acquired heart diseases from neonates to adults in the pre- and post-operative as well as post-interventional setting.

• Principles of invasive and non-invasive cardiac monitoring (non-invasive blood pressure, electrocardiography, oximetry, capnography, arterial lines, venous central lines, intra-cardiac lines (including right atrial, left atrial, pulmonary artery lines), Swan–Ganz catheter, emerging technologies).

• Evaluation and treatment of common neonatal cardio-circulatory disturbances in the absence of cardiac defects (i.e., persistent ductus arteriosus of the premature newborn, increased pulmonary vascular resistances secondary to persistent fetal circulation).

• Evaluation and treatment of common cardio-circulatory disturbances in infants and adolescents in the absence of cardiac defects (i.e., cardiac dysfunction in septic shock, trauma, metabolic diseases).

• Currently used scores of severity in the ICU/heart surgery arena (i.e., STAT, RACHS, Aristotle, PRISM).

• Basics on pharmacokinetics and use of cardiovascular drugs (i.e., dopamine, dobutamine, isoprenaline, adrenaline, noradrenaline, levosimendan, phenylephrine, calcium chloride, vasopressin, sodium nitroprusside, nitroglycerine, nicardipine, hydralazine, phentolamine, phenoxybenzamine, beta-blockers, and anti-arrhythmic drugs), including the management of their side-effects and interactions.

• Basics on pharmacokinetics and use of pulmonary vasodilator drugs (i.e., oxygen, inhaled nitric oxide, sildenafil, and prostacyclin).

• Basics on pharmacokinetics and use of inodilators (i.e., milrinone and enoximone).

• Use of prostaglandins E1, anti-coagulants, diuretics, and the management of their side-effects and interactions.

• Deep understanding of physiology: focussing in cardiovascular physiology, principles of mechanical ventilation, and cardiopulmonary interactions.

• Basic notions of cardiac anaesthesia.

• Basic notions of cardio-pulmonary-bypass, normo- versus hypothermia, deep hypothermia circulatory arrest.

• Basic notions on extra corporal membrane oxygenation, especially in the post-procedural setting aiming for bridge to recovery.

• Basic notions on assessment and management of sedation, anxiolysis, and pain.

• Basic notions on mechanical ventilation.

• Basic notions on nursing care and algorithms.

• Basic notions on nutrition of the critically ill cardiac patient.

• Basic notions of cardiac and non-cardiac complications of cardiac surgery (renal, neurological, respiratory, haematological, gastrointestinal, infectious).

• Basic notions on renal replacement therapy (peritoneal dialysis and haemofiltration).

• Development of a philosophy of care and promotion of multidisciplinary team work.

• Acquisition of Paediatric Advanced Life Support, Advanced Cardiovascular Life Support, and Basic Life Support accreditation, including cognitive and skills evaluation.

• Basic notions on neurological monitoring with cerebral near infra-red spectroscopy.

Skills:

The ability to:

• perform Paediatric Advanced Life Support and Advanced Cardiovascular Life Support and manage the patient in the post-resuscitation period.

• develop a structured approach to identify, manage, and stabilise the patient with haemodynamic instability.

• use emergency monitoring equipment and to timely identify cardiovascular abnormalities requiring urgent intervention.

• use non-invasive tools like echocardiography and Doppler to manage critical ill patients.

• participate in the decisions to admit, discharge, or transfer patients from the ICU.

• give cardiovascular system support:

  • perform arterial and central venous catheterisation.

  • measure and interpret haemodynamic variables.

  • use echocardiography appropriately in the acute and intensive care patient, including emergency and peri-resuscitation echocardiography as an independent operator.

  • perform pericardiocentesis.

  • treat and manage basic and complex arrhythmias in the acute care patient.

  • select and use fluids, inotropic, vasoactive, and anti-arrhythmic drugs.

  • use of external pacemakers (external pads, epicardial leads, trans-thoracic pacing).

• give respiratory system support:

  • identify the early signs of acute airway insufficiency and acute respiratory failure.

  • perform emergency tracheal intubation.

  • obtain and interpret data from blood gas samples (arterial, central, and mixed venous).

• comply with local infection control measures and appropriately manage antimicrobial drug therapy.

• correct fluid, electrolyte, metabolic, and glucose disorders.

• assess neurological function (e.g., Glasgow Coma Scale).

Attitudes:

• Communicate, collaborate, and team work with the health care team (ICU nurses, ICU cardiologists, intensivists, and other staff).

Fetal cardiology

The trainee should become familiar with the present knowledge concerning fetal cardiology. The basic training level is recommended for all paediatric cardiology trainees. ^{Reference Allan, Dangel and Fesslova5} A close cooperation between the obstetrician or feto-maternal specialist and fetal cardiologist is crucial for optimal perinatal management. ^{Reference Allan, Dangel and Fesslova5} The trainee should be familiar with the screening scan in low-risk pregnancies at around 18–22 weeks of gestation as well as with the first trimester screening using the recognition of the increased nuchal translucency, abnormal ductus venosus flow, and tricuspid regurgitation in the evaluation of an increased risk of CHDs. ^{Reference Jicinska, Vlasin and Jicinsky13} The performance of an early anomaly scan in high-risk pregnancies is gaining importance. ^{Reference Carvalho and Allan14} The trainee should be involved together with the obstetrician or feto-maternal specialist to distinguish between the normal and abnormal cardiac status and establishing the primary diagnosis. Together with the specialist in fetal cardiology, the trainee should be involved in making the final diagnosis and counselling of the parents. The paediatric cardiologist should be able to interpret a fetal cardiac exam performed by a sonographer/other person and interpret the report, as well as have an understanding of the adequate care that the baby will require after birth. It is not necessary to perform the fetal echocardiogram by himself/herself.

Knowledge:

• Physiology of the fetal cardiovascular system:

  • fetal circulation: importance of the essential shunts: foramen ovale, ductus venosus, ductus arteriosus.

• Fetal pathophysiology and cardiac function and how this differs from the postnatal physiology.

• CHDs dependent on one or more of the prenatal essential shunts and the postnatal management required.

• Transitional circulation in healthy fetuses and those with cardiovascular problems.

• Prenatal evolution of cardiac defects:

  • indications for prenatal cardiac interventions.

• CHDs and genetic problems:

  • indications to perform genetic testing.

  • current available genetic tests and their interpretation in co-operation with geneticists.

• Fetal arrhythmia:

• diagnosis of fetal tachycardia, bradycardia, and inappropriate heart rates in the fetus (recognition of abnormal M-mode and Doppler recordings).

  • indications of referral of a fetus with an arrhythmia to a fetal cardiologist for further diagnosis and management.

  • indications and start of adequate prenatal treatment of a fetus with arrhythmia avoiding premature delivery.

• Fetal cardiac failure and cardiomyopathies:

  • differences between prenatal and postnatal causes.

  • application of the Cardiovascular Profile Score or similar scores to evaluate the fetal cardiovascular condition.

  • treatment options.

• Functional changes in the fetal cardiovascular system:

  • tricuspid regurgitation.

  • disproportion between right and left side of the heart.

  • regurgitation of the other cardiac valves (mitral, aortic, and pulmonary).

  • prenatal closure or restriction of the ductus arteriosus and/or foramen ovale

    1. – aetiology and consequences for the neonatal condition

    2. – perinatal management and treatment options.

• Fetal cardiovascular abnormalities due to maternal pathology (as diabetes, lupus, antiepileptic medications).

• Indications for fetal cardiac echocardiography and Doppler.

• Limitations of imaging, especially in the first trimester of pregnancies. ^{Reference Jicinska, Vlasin and Jicinsky13}

• Legal regulations concerning termination the pregnancy in the specific country.

Skills:

The ability to:

• interpret and report a fetal cardiac exam performed by a sonographer or other person in order to detect cardiovascular problems in the fetus.

• detect warning signs when evaluating a fetal echocardiogram as size of the heart, fetal heart rhythm (abnormal four chamber, outflow tracts, mediastinal view), abnormal Doppler, and fetal hydrops.

• explain to the parents a (preliminary) diagnosis of the fetal cardiac problem and know when to refer them to the fetal cardiologist consultant.

• discuss with parents the possible prenatal treatment options (like transplacental treatment for arrhythmia).

• discuss prenatal cardiac interventions when needed – together with the fetal cardiologist consultant.

• predict the possible outcome (early and late prognosis) and potential progression of the lesion during gestation, including lethal anomalies, together with the other partners of the perinatal team.

• discuss the perinatal period and postnatal management including early and late outcomes if the final diagnosis is established.

Attitudes:

• Ability to communicate sensitively and with compassion.

• Ability to break bad news.

• Awareness of the value of the prenatal counselling and documentation for a good continuation of postnatal care.

• Communicate, collaborate, and team work with the health care team (obstetricians, feto-maternal specialists, geneticists, neonatologists, surgeons, and other staff).

3.8 Adult CHD

The remarkable improvement in survival of surgically and interventionally treated patients with CHD has led to an increasing number of adult CHD patients (former grown-up CHD).

For the trainee in general paediatric and congenital cardiology, it is necessary to know about the basic principles of CHD and the long-term outcome of the different lesions. Cooperation with other medical specialists (more or less the entire spectrum of adult medicine) will be utmost important. ^{Reference Baumgartner, Budts and Chessa8}

Knowledge:

• The natural history of CHD, of surgical and interventional treatment (palliative and repair) and post-interventional course (residua and sequelae, long-term outcome, long-term complications).

• The optimal timing and indications for interventions (surgical and catheter) in unrepaired and repaired lesions.

• The role of non-invasive imaging techniques.

• The role of haemodynamics and when to derive them invasively. Specificities of interventional therapy in adult CHD (balloon valvotomy, valve implantation, closure of shunt lesions, balloon dilatation and stenting of obstructive arterial and venous obstructions, occlusion of vessels).

• Surgical treatment strategies in adult CHD (palliative, reparative, corrective, transplantation) and perioperative management.

• The basics about psychosocial aspects, typical cardiovascular risk assessment, and influence of lifestyle – as physical activity, sports, contraception.

• Risk assessment of pregnancy in adult CHD and management during pregnancy, labour, delivery, and postpartum.

• The basics of clinical genetics for counselling.

• The need for vocational counselling and the obstacles encountered when obtaining insurances and driving license.

• The anatomical and functional complexity grade and their impact on patient management.

• Sufficient knowledge about transfer and transition from paediatric to adult CHD service.

• Sufficient knowledge about advanced care planning in adult CHD: transitioning from repair to palliation and end-of-life care. ^{Reference Troost, Roggen and Goossens15}

• Acquired cardiovascular disease (atherosclerosis, degenerative valvular heart disease, systemic hypertension, etc.) and its prevention, diagnosis, and treatment.

• Accompanying internal disease (pulmonary, renal, hepatic, etc.).

• Risk assessment of non-cardiac surgery in adult CHD and perioperative management.

Skills:

• Physical examination of adult CHD (specific presentation of CHD during adult life).

• Interpretation of the diagnostic procedures in adult CHD.

• Electrocardiography, exercise electrocardiography, cardiopulmonary exercise testing, and 24-hour Holter electrocardiography or longer time monitoring in adult CHD.

• Imaging techniques in the pre- and post-operative morphologic and functional assessment of adult CHD (echocardiography, MRI, multi-slice computed tomography, nuclear imaging) and their advantages in multimodality imaging.

• Interpretation of haemodynamic and angiographic findings from cardiac catheterisation in adult CHD.

• Diagnosis and treatment of heart failure in adult CHD (taking into account the difference between CHD and acquired heart disease).

• Correct diagnosis and adequate treatment of pulmonary hypertension in adult CHD.

• Diagnosis, drug treatment, and interventional treatment of arrhythmias in adult CHD (pathophysiology, non-invasive diagnosis, invasive diagnosis, drug therapy, electrical, and interventional therapy (cardioversion, transitory stimulation, pacemakers, resynchronisation therapy, implantable cardiac defibrillators, catheter ablation)).

Attitudes:

• Ability to communicate sensitively and with compassion.

• Ability to shift communication more and more from parents to the patient and later to patients’ direct familial environment.

• Ability to break bad news.

• Communicate, collaborate, and teamwork with the health care team (cardiologists and their sub-specialties, radiologists, cardiac surgeons, obstetricians, geneticist, and other staff).

Paediatric pulmonary hypertension

Pulmonary hypertension is a frequently occurring complication in CHD. Furthermore, patients with pulmonary hypertension will often be referred to cardiologists or pulmonologists for assessment. It is therefore important that all paediatric cardiology training programmes cover the basic training requirements that are recommended for all paediatric cardiology trainees, as stated in detail in the recommendations of the specific Association for European Paediatric and Congenital Cardiology WG. ^{Reference Sallmon, Moledina and Albert4}

Knowledge:

• Signs and symptoms of pulmonary hypertension in children and its differential diagnoses.

• Understanding of the pathophysiology of pulmonary hypertension and knowledge of the different aetiologies and subgroups.

• Basic notions of the principles of pulmonary hypertension-targeted therapies (acute, chronic, and decompensated) as diuretics, anticoagulation, pulmonary vasodilators.

• Comprehension of the significance of elevated pulmonary vascular resistance in patients with CHD, reversibility, and its relevance in terms of indication/contraindication for surgical repair of CHD.

Skills:

The ability to:

• diagnose pulmonary hypertension in patients presenting with symptoms or in asymptomatic patients from high-risk populations.

• perform an echocardiographic assessment of pulmonary hypertension including diagnosis, physiology, shunts, presence of important CHD, assessment of afterload, and ventricular function.

• perform the initial evaluation and stabilisation of a haemodynamically compromised patient with pulmonary hypertension and initiate an appropriate supportive management.

Attitudes:

• Ability to communicate sensitively and with compassion.

• Ability to break bad news.

• Recognition of the importance of a multidisciplinary team in care for patients with pulmonary hypertension.

Heart failure and transplantation

General paediatric cardiology training should encompass knowledge and experience on diagnosis of patients with heart failure including a basic introduction to the care of patients with heart failure and post heart transplantation. The basic level is recommended for all paediatric cardiology trainees and should be available at all centres/networks with a training programme in paediatric cardiology. ^{Reference Reinhardt, Hansmann and O’Sullivan3}

Knowledge on heart failure:

• Understanding the pathophysiology and clinical presentation of the different types of heart failure that occur in acquired heart disease in children, genetic and familial cardiomyopathies, biventricular or univentricular CHD, with disease staging and initial differential approach in the diagnosis and treatment.

• Familiarity with available modalities for the diagnostic evaluation of children with newly discovered heart failure and the monitoring of chronic heart failure and role of diagnostic tests to establish an accurate diagnosis and subsequent management (B-type natriuretic peptide, genetic evaluation, metabolic assessment, cardiac imaging as electrocardiography, echocardiography, cardiac MRI, role of exercise testing, cardiac catheterisation, and endomyocardial biopsy).

• Understanding and interpretation of exercise tests, biomarkers, electrocardiography abnormalities, and haemodynamic parameters by cardiac catheterisation to plan the accurate treatment.

• Understanding the principles of medical management of heart failure (acute, chronic, decompensated), the role of the renin–angiotensin–aldosterone axis, the sympathetic nervous system and related treatment targets (ACE-inhibitors, AT2-antagonists, beta blockers, I_f-channel blockers, mineralocorticoid receptor antagonists, diuretics, and digitalis), and recognise the differences in metabolism and pharmacokinetics in paediatrics.

• Familiarity with identification and treatments of primary and secondary prevention of arrhythmias and thromboembolic complications.

• Understanding of additional therapies in the progression of heart failure including implantation of an implantable cardiac defibrillator, cardiac resynchronisation therapy, indications for mechanical circulatory support, and familiarity with clinical trials of novel therapies including stem cells.

Knowledge on cardiac transplantation and mechanical support:

• Indications of types of mechanical circulatory support in the treatment of end-stage heart failure and understanding the use of extracorporeal membrane oxygenation and ventricular assist devices as a bridge to transplantation or bridge to recovery or as destination therapy in selected cases.

• Indications and contraindications of heart transplantation.

• Basis of immunology, rejection, and human leucocyte antibody mismatch.

• Post-operative complications of heart transplant and physiology of the denervated, transplanted heart.

• Basics in immunosuppression agents and complications.

• Indications of ABO incompatible transplantation and complications.

• Recognition of heart transplant rejection, diagnostic tests, and basics of treatment.

• Outcome of heart transplantation, including mortality and morbidities during follow-up and indications of re-transplantation.

• The basics about complications associated with heart transplantation such as an acute and chronic rejection, coronary allograft vasculopathy, and sequelae of immunosuppression, such as infection, malignancy, and renal insufficiency.

Skills:

The ability to:

• detect warning clinical signs and symptoms of heart failure and interpret biomarkers.

• select and use diagnostic techniques to differentiate the underlying causes and precipitating factors of heart failure and to evaluate cardiac function and pulmonary pressures.

• perform echocardiographic assessment of heart failure including the presence of important CHD, assessment of afterload, and ventricular function.

• manage acute and chronic heart failure medically, recognise deterioration, and know-how to escalate treatment (refer to specialist service or for transplantation).

• educate patients and their relatives about self-care management and the importance of compliance to therapy in patients with heart failure or after heart transplantation.

Attitudes:

• Ability to communicate sensitively and with compassion.

• Ability to break bad news.

• Recognition of importance of multidisciplinary team (e.g., nurses, psychology) in care for children with end-stage heart failure.

Levels of multidisciplinary family-centred psychosocial care

General, non-disease-specific psychosocial interventions intended to decrease negative risk factors and strengthen protective factors in a family. ^{Reference Walsh16} Psychosocial care can be provided at three levels: the individual patient, the parents, and the family. ^{Reference Utens, Callus, Levert, Groote and Casey6}

The trainee is expected to be familiar with the basic principles of counselling. Additionally, the trainee is expected to be familiar with the normal development of a child from infant to young adult and the impact which this has on psychosocial care and communication with the patient and family. The influence hereof on the transition of the patient from paediatric to adult congenital cardiology should be respected.

Knowledge:

• Normal development of a child from infant to adult and factors that can interfere with this process.

• Possible psychosocial issues occurring during the lifespan for patients with CHD and their families.

• Psychosocial considerations of the teenage patients during transition and adequate communication style with this patient group.

• Effective communication strategies for an initial approach to psychosocial distress and effective referral to an adequate specialist available.

• Approach to quality of life and methods to assess a patient’s and a family’s quality of life.

• Different therapeutic strategies for psychotherapeutic treatment:

  • Psychosocial counselling and initiation of psychotherapeutic treatment at the level of the individual child/adult with chronic illness focussing on:

    1. – learning how to cope with physical limitations associated with the chronic condition.

    2. – learning to cope with negative emotions related with disease or medical procedures (anxiety, anger, sadness, despair, post-traumatic stress, hopelessness/or helplessness).

    3. – leaning to cope with a loss of control over one’s own body and life.

  • Counselling of parents and initiation of psychotherapeutic treatment focussing on:

    1. – emotional issues (such as post-traumatic stress ^{Reference Woolf-King, Anger, Arnold, Weiss and Teitel17} and fostering parenthood and parenting the CHD child). ^{Reference Woolf-King, Anger, Arnold, Weiss and Teitel17–Reference Craig, Lynch and Quartner19}

    2. – providing support to parents regarding their social issues (such as arranging parental leave for cardiac surgery of the child, unemployment, financial problems).

  • Counselling of families and initiation of psychotherapeutic treatment focussing on:

    1. – aims to gain insight in the inter-relationships within the family.

    2. – the impact of the chronic illness on each of the family members and the effect of stress on mutual relationships.

    3. – reducing the negative interactions and teach adequate problem-solving and coping strategies.

Skills:

• Be familiar with different styles of communication techniques.

• Recognise the multiple needs of the patient and family and be aware of the limitations of basic counselling and the need of professional support.

• Be able to organise a multidisciplinary approach with psychologists, social workers, and play therapists.

• Be able to consider the importance of adequate transition and transfer of patients to the adult clinic.

• Initiate the referral to local and international non-profit associations of patients and their families (such as the European Congenital Heart Disease Organisation) of patients and families when social issues are present.

Attitudes:

• Allow the patients and family members to express their anxiety and concerns when a CHD is diagnosed or when there is a change in the physical state of the patient.

• Allow the patients and family members to express their anxiety and concerns when undergoing interventional or surgical procedures.

• Be aware that psychosocial care from fetal to adult life is most effective when it is organised in a multidisciplinary setting. This includes collaboration with specialists as psychologists, social workers, and congenital cardiac specialist nurses.

• Be aware that communication shifts from “primarily with the parents” to “primarily with the patient” when the child grows older.

• Be aware that transfer to the adult clinic should be planned and prepared timely in order to prevent a care gap and potential loss to follow-up.

• Be aware that CHD can have a major impact on career and family planning and that these topics should already be addressed in adolescence.

• Collaborate with congenital, cardiac specialist nurses for communication, and psychosocial input to patients and their families from fetal to adult life.

Sports cardiology, physical activity, and prevention

The trainee should acquire experience in the assessment and treatment of heart disease risk factors in children and communicate strategies that help patients and families to follow a heart-healthy lifestyle. For patients with CHD, it is important to understand how exercise prescriptions can benefit them and to appropriately detect contraindications to exercise/competition.

Knowledge on prevention:

• Most common cardiovascular risk factors in childhood and adolescence, including the field of “fetal programming” of cardiovascular diseases.

• Cardiovascular risk factors specified for CHD patients, sedentary lifestyle, obesity, diabetes, arterial hypertension and arterial stiffness, dyslipidaemia, survival after oncological treatment, Kawasaki disease, and rheumatic diseases.

• The possibilities and limitations of genetic testing in the context of prevention and cardiovascular disease (e.g., predictive diagnostics, reverse cascade screening for familial hypercholesterolemia).

• Effects of physical activity, nutrition, education, and psychological risk factors on quality of life, exercise capacity, and cardiovascular risk.

• Gender-related reference values of body mass index, blood pressure, lipid status, and treatment of elevated values.

• Assessment of vascular risk status using tools to measure, for example, vascular stiffness, distensibility, and intima-media thickness.

• Prevention of sudden cardiac death in children (most common congenital arrhythmia syndromes).

Knowledge on sports cardiology for healthy young athletes:

• Exercise and sports physiology including benefits of exercise training.

• Pre-participation screening in young athletes stated in the Association for European Paediatric and Congenital Cardiology recommendations. ^{Reference Fritsch, Pozza and Ehringer-Schetitska20}

• Risk factors for mechanisms of sudden death during and after exercise.

• Electrocardiography evaluation and specific electrocardiography findings in athletes: recognising changes suggestive of primary electrical disease or cardiomyopathy (international criteria). ^{Reference Baggish, Battle and Beckerman21,Reference Sharma, Drezner and Baggish22}

• Specific electrocardiography criteria and findings in the paediatric athlete.

• Echocardiographic findings in athletes’ hearts including maturational changes of cardiac morphology and function in childhood.

• Cardio-pulmonary exercise testing (treadmill or bicycle ergometer) principles of performance, interpretation, and limitations in childhood.

• Principles in stress imaging in athletes and CHD patients (stress MRI, stress echo).

• Basics in nutritional aspects and drug side-effects in athletes.

• Ethical and legal basis of sport examinations (country specific).

Knowledge on sports cardiology for young athletes with CHD:

• Types of exercises and risks in CHD patients depending on their clinical situation and underlying disease (Bethesda criteria ^{Reference Warnes, Williams and Bashore23} ).

• Recommendations on physical activity in CHD patients. ^{Reference Takken, Giardini and Reybrouck24}

• Specific issues of patients with electronic devices, cardiomyopathies, channelopathies, and/or an increased risk of acute cardiac events.

• Evaluation of exercise capacity and causes of exercise intolerance.

• Emergency care for patients during sport competitions.

• Basics of cardiac rehabilitation.

• Counselling of patients with CHD in sports issues.

Skills:

The ability to:

• perform an individual cardiovascular risk assessment using appropriate information from history including laboratory tests and clinical data in healthy children and CHD patients.

• perform a cardiovascular screening evaluation pre-participation in athletic activities in children and adolescents to prevent sudden death.

• perform a physical examination and evaluate physical activity in patients with CHD (and special consideration on disorders going along with cardiovascular risks: obesity, athletes, anorexia, hypertension, Marfan syndrome) and give adequate recommendations for participation in leisure time or competitive sports.

• perform and interpret a cardiopulmonary exercise test.

• recognise pathological cardiovascular changes and differentiate them from the “athlete’s heart.”

• prescribe exercise-based rehabilitation programmes and other lifestyle interventions in collaboration with other specialist, according to the CHD patient’s condition.

Attitudes:

• Recognition of the role of an active lifestyle, exercise, and sport in the promotion of health and in the prevention of cardiovascular diseases.

• Recognition of the importance of patient and family education and the role of other professionals including nurse specialists, exercise physiologists, psychologists, dietitians, and paediatricians in cardiac rehabilitation.

• Recognition and advocating of medical guidance to promote safe training and a healthy development of the elite paediatric athlete.

Congenital cardiovascular surgery

The trainee should be involved in the collaboration with the cardiac surgeon concerning pre-operative diagnosis and decision-making and acquire the following knowledge:

Knowledge:

• Essential pre-operative information required to refer patients with CHD for cardiac surgery.

• Main surgical indications for large majority of CHD.

• Main surgical techniques for large majority of CHD.

• Be familiar with risk stratification and outcome analysis for surgical results.

• Typical complications in the short- and long-term follow-up after cardiovascular surgery.

• Different biological and artificial material used in congenital cardiovascular surgery as types of patches, valved and non-valved conduits and grafts, mechanical heart valves, and vascular prosthesis.

• General theoretical understanding of the cardiopulmonary bypass circuit and anaesthesia during congenital heart surgery.

• In depths theoretical understanding of surgical procedures.

• Basic notions on indications and performance of hybrid procedures in collaboration with the interventional cardiologist (i.e., hybrid hypoplastic left heart syndrome approach, ventricular septal defect closure with apical access, stenting of a coarctation in premature infants).

Skills:

The ability to:

• be able to discuss surgical indications and types of procedures according to the patient’s clinical status and imaging records.

• interpret the surgical operation report.

• be able to assess anatomical and pathophysiological results of surgical repair/palliation in the early and late outcomes after surgery.

• judge the longer-term consequences and requirements of the post-operative situation.

Attitudes:

• Establish a close collaboration with the surgeons spanning from pre- to post-operative management.

Training logbook

The logbook is a tool for the trainee and trainer to keep track of the progress of the trainee. It is the responsibility of the trainee to record all relevant activities. In countries where national guidelines exist, the trainee might maintain a national equivalent to the Association for European Paediatric and Congenital Cardiology logbook.

The logbook should be countersigned by the trainer confirming that the trainee has satisfactorily achieved the requirements stated according to these recommendations.

It is the responsibility of the trainer to audit and certify the activities. The trainee should also keep copies of certificates of attendance of Association for European Paediatric and Congenital Cardiology recommended and approved supplementing courses and add those to the logbook.

Annual appraisal and assessment

The trainer and/or the head of the department of each institute providing the complete training programme or part of it should carry out appraisals and assessments to evaluate (annually) the progresses of the trainee.

Final assessment

Ideally, the training should be completed by a final assessment. This could be by means of an exit examination or by continuous assessment of competencies during training. The assessment or examination should combine written, oral, and practical components.

Logbook of procedures

Purpose:

To ensure that the trainee has adequate exposure to a range of clinical procedures and techniques in preparation for physician practice.

Requirement:

A logbook showing details over the course of training and kept updated regularly throughout the training is essential. The trainee must maintain all performed clinical procedures including the information about the level of supervision in the logbook. Supervisors are required to confirm in their reports that the logbook is a true and accurate record of the trainee’s experience and that all training requirements have been fulfilled.

Procedures/activity

• Ambulatory care:

  • Manage patients in an ambulatory care (outpatient) setting under supervision – minimum number: 200 patients.

• Cardiac catheterisation and balloon atrial septostomy:

  • Participate and report cardiac catheterisation and haemodynamics – minimum number: 50 cases including Rashkind balloon atrioseptostomy procedures.

• Direct current cardioversion:

  • Perform direct current cardioversion – minimum number: five cases.

• Echocardiograms:

  • Fetal echocardiograms (observation, not necessarily primary operator and associated counselling) – minimum number: 20 studies.

  • Transoesophageal echocardiograms – minimum number: 50 studies, 25 studies as a primary operator (all studies should be reviewed and have finalised consultant reports).

  • Transthoracic echocardiograms – minimum number: 500 cases, 300 under supervision of a paediatric echocardiographer/cardiologist and at least 400 on patients with cardiac pathology (all studies should be reviewed and have finalised consultant reports).

• Electrocardiograms:

  • Interpret and report electrocardiograms on both inpatients and outpatients – minimum number: 150 cases.

• Electrophysiology:

  • Participate in clinical decision-making for electrophysiology study/ablation procedure, including observation of procedures and interpretation of reports – minimum number: 10 cases.

• Exercise tests:

  • Supervise and report exercise tests – minimum number: 50 cases.

• Holter monitor:

  • Supervise and report Holter monitor – minimum number: 50 cases.

• Imaging:

  • Interpret chest X-rays – minimum number 100 cases.

  • Interpret results of cardiovascular magnetic resonance, thoracic CT, and radionuclide imaging – minimum number: 10 cases (in total).

• Pacemaker:

  • Observe pacemaker implantation – minimum number: five cases.

  • Participate in testing permanent pacemaker function – minimum number: 20 cases.

  • Perform pacemaker testing – minimum number: 20 cases.

• Pericardial aspiration:

  • Perform pericardial aspiration under supervision and demonstrate competency as an independent operator – minimum number: two cases.