Adult Congenital Heart Disease Expert Scientific and Clinical Practice

Details

Title

Adult Congenital Heart Disease Expert Scientific and Clinical Practice

Type

Stage Two

Module code

HPS134

Requirement

Optional

Module objective

By the end of this module the Clinical Scientist in HSST will be able to analyse, synthesise, critically evaluate and apply their expert knowledge of: (i) the requirement for long-term follow-up in patients with CHD and the multidisciplinary approach required to achieve this; (ii) the impact of acquired heart disease on ageing patients and how to assess this; (iii) the impact and contraindications of lifestyle choices on patients with CHD; and (iv) the specific imaging requirements around the time of surgery and when to use complementary modalities. In addition they will gain and apply their expert knowledge of the importance of: (i) pre-pregnancy counselling and the maternal risks associated with pregnancy in patients with CHD; (ii) the specialised multidisciplinary approach to care during the antenatal, delivery and postnatal periods; and (iii) the genetics involved in CHD and the implications of inheritance.

The Clinical Scientist in HSST will be expected to manage patients suitable for follow-up in a physiologist-led service. Their knowledge will also span the requirement and requisites of a well-coordinated transition service to prepare the patient for the change between paediatric and adult services. They will also be expected to apply their knowledge in their scientific and clinical practice while consistently demonstrating the attitudes and behaviours necessary for the role of a Consultant Clinical Scientist within a patient-focused service.

Knowledge and understanding

By the end of this module the Clinical Scientist in HSST will critically analyse, synthesise, evaluate and apply their expert knowledge of the long-term follow-up of adult CHD and acquired heart disease, including:

Underpinning clinical science:

• the natural history of various CHD, native and repaired, and the level and range of care available to support patient management, including:
  • quaternary/tertiary care
  • district general hospitals with specialist interest physicians
  • physiologist-led clinics within a tertiary centre
  • specialist dental care
  • obstetric care
• CHD conditions that can be safely followed up in Clinical Scientist-led clinics, including:
  • aortopathy patients (e.g. Marfan syndrome and Ehlers-Danlos syndrome) and haemodynamically insignificant ventricular septal defects (VSDs) or patent arterial ducts
  • abnormal valves with mild functional impairment (e.g. bicuspid aortic valve)

• when patients being looked after in a Clinical Scientist-led clinic require referral back to physician care;
• psychosocial issues associated with CHD and support services;
• the increased morbidity and mortality associated with complex CHD patients with the increased longevity;
• the specific arrhythmias that may be associated with particular lesions and surgeries;
• the impact of acquired heart disease on pre-existing congenital heart lesions (e.g. coronary artery disease in a univentricular system);
• the natural progression of acquired heart disease (e.g. coronary artery disease);
• the need for care within a multidisciplinary centre for non-cardiac pathology that may need cardiology input;
• the prevalence of the ‘loss to follow-up’ phenomenon and its importance to maintaining good health;
• the importance of clinical audit in adult CHD patients and its impact on evolving healthcare provision;
• the complex interdependent relationships that sometimes exist between patients and carers;
• the findings of investigations carried out and the impact of the results.

Transition services:

• the national and international guidelines for transition services and the requirements and quality management of transition services;
• the need for liaison with members of the wider multidisciplinary team to discuss strategies for preparing patient for investigations, procedures, etc.;
• needs of learning disabled young people during transition and transfer into adult services;
• the range of services available to provide appropriate follow-up care, e.g. quaternary/tertiary centre or in a smaller centre with physicians with a specialist interest;
• the current guidelines relating to lifestyle relevant to this adolescent population (e.g. exercise, tattoos, body piercing and contraceptive advice);
• the implications of becoming ‘lost to follow-up’;
• how to support patients with issues related to body perception and privacy in adolescent patients;
• why more complex patients with multisystem involvement and/or neurocognitive impairment may require several appointments until all concerned are comfortable with the transition.

Counselling for lifestyle:

• the importance of a healthy lifestyle on adult CHD patients and encouraging, under advisement, activity and sport to maintain general cardiovascular health;
• the importance of taking responsibility for their own health and the impact of becoming lost to follow-up;
• the increased risk of endocarditis in adult patients with CHD and activities that may increase risk further. including:
  • body piercing
  • tattoos
  • poor dental hygiene
• current endocarditis treatment guidelines and the prophylactic use of antibiotics;how to identify when the patient’s clinical status may not fit your observations/assessment ;
• the potential psychological impact on adolescents/adults of lifestyle choices, e.g. further education, alcohol consumption, sexual activity;
• the issues surrounding birth control (certain types of contraceptives are not advisable with certain cardiac lesions);
• the implications of unexpected and unplanned pregnancies;
• the potential impact on obtaining health/life insurance, mortgages and certain types of employment;
• the co-existing cognitive and developmental delay that may impact on their lifestyles choices and available support services;
• guidelines on assessment of endocarditis and when to further investigate with TOE imaging;
• the limitations of echocardiography and the appropriate use/referral for assessment using other imaging modalities;
• the relevance of exercise and cardiopulmonary exercise testing (CPET) testing in monitoring patients long term, especially for non-subjective symptom assessment and timings of interventions.

Imaging considerations for pre-, peri- and postoperative adult patients:

• invasive procedures required and the information needed prior to the procedure;
• the role and limitations of TTE in preoperative assessment;
• the role and limitations of TOE in preoperative assessment;
• the role and limitations of computed tomography and magnetic resonance imaging in assessment for surgery;
• the need for interoperative imaging and suitable image acquisition;
• imaging requirements in the postoperative period (e.g. assessment for pericardial effusion, patches and baffles, or dehiscence and prosthetic valves);
• when and how to seek relevant advice or seek help

Pregnancy risks and considerations and the implications of inheritance:

• the normal maternal changes to cardiac physiology associated with pregnancy abnormalities detected through echocardiography and ECG;
• information and counselling for women of reproductive age with CHD, including maternal and fetal morbidity and mortality;
• risk of inheritance of CHD in the offspring;
• level of surveillance, treatment and anticipated hospitalisation required during pregnancy;
• contraception;
• the general risk factors for women with CHD during pregnancy, including:
  • New York Heart Association classification >II or cyanosis before pregnancy
  • impaired systemic ventricular function (ejection fraction < 40%)
  • left heart obstruction (mitral valve area < 2cm2, aortic valve area < 1.5cm2, left ventricular outflow tract peak gradient > 30 mm Hg before pregnancy
  • preconception history of adverse cardiac events such as symptomatic arrhythmia, stroke, transient ischaemic attack and pulmonary oedema
• the stratification of different lesions into low-, moderate- and high-risk groups:
  • low risk: atrial septal defects, ventricular septal defects (haemodynamically insignificant), repaired coarctation and repaired tetralogy of Fallot
  • moderate risk: mitral or aortic stenosis, systemic right ventricles (congenitally corrected transposition of the great arteries [ccTGA] and atrial switches for TGA), Fontan’s patients, cyanotic patients without pulmonary hypertension
  • high risk: Marfan syndrome with dilated aorta and patients with Eisenmenger’s syndrome
• medications commonly needed for heart disease patients are contraindicated in pregnancy:
  • angiotensin converting enzyme inhibitors, angiotensin-II receptor antagonists and the risk of neonatal renal failure and hypotension, renal tubular dysgenesis, intrauterine growth restriction, decreased skull ossification
  • warfarin and the risk of skeletal defects, abnormalities of the central nervous system, intracranial haemorrhage
  • amiodarone, which may be used in special circumstances, but with the risk of hypothyroidism and potential brain damage
  • phenytoin with the risk of heart defects, intrauterine growth restriction, orofacial abnormalities
  • spironolactone with the possible risk of anomalies of the external genitalia (animal studies only)
• pregnancy physiology in relation to standard investigations, i.e. physiological response of pregnant woman lying flat on her back for an hour for an echocardiogram;
• radiation risk (breast cancer and fetus) with respect to CT and angiography during pregnancy;
• the potential complications following birth and the potential risks to maternal life (e.g. aortic dissection in high-risk Marfan’s patients).

Implications of inheritance:

• the syndromes associated with CHD (including aortopathy-inducing diseases such as Marfan syndrome and Ehlers-Danlos syndrome);
• the commonly occurring chromosomal abnormalities involved;
• the associated non-cardiac abnormalities in some syndromes/chromosomal abnormalities;
• the increased risk of CHD with first-degree relatives;
• the importance of early antenatal screening in parents with CHD;
• associated congenital lesions that are more likely to occur together;
• the increase in need for specialised fetal screening through echocardiography with an increasing adult population;
• the importance of audit to the evaluation of inheritance risk and the further adequate provision of services for the future.
• the potential value of genetic counselling and refer, after discussion with relevant professionals, to specialist genetic teams;
• the potential benefits to emotional and psychological counselling and seek referral where appropriate;
• the importance of audit to the evaluation of inheritance risk and the further adequate provision of services for the future.

Technical and clinical skills

By the end of this module the Clinical Scientist in HSST will be expected to critically reflect on their clinical practice. They will apply in practice a range of advanced clinical and communication skills to advise and communicate effectively with patients, relevant clinicians, patients and the public, and other healthcare professionals.

They will be able to perform and master each technique, adapting their response to meet the varying and complex challenges that will arise reliably, and will be able to:

• gain informed consent;
• take a relevant history to inform further investigations (e.g. for endocarditis);
• perform a TTE as part of the assessment of endocarditis and if appropriate refer for further investigation, which may include oesophageal echocardiography.

Transition services:

• provide a supportive and informative clinical atmosphere in which the young person can raise sensitive issues about their CHD and associated issues in their own right, in particular being aware of safeguarding issues in this group of adolescents;
• discuss the process of transition and the importance of maintaining their own health and attending appointments with the patient, and if appropriate parents/carers, answering any concerns of the patient or referring to colleagues;
• disseminate relevant information during the handover of patients from the paediatric to adult team and present it accurately and succinctly;
• produce an accurate handover report detailing the anatomy and physiology of the heart highlighting any abnormalities that require further investigations.

Imaging considerations for pre-, peri- and postoperative adult patients:

• gain informed consent;
• perform transthoracic and transoesophageal echocardiography;
• adapt normal techniques to the logistics of theatre and intensive care environments;
• make clinically relevant observations in a succinct manner to other healthcare professionals.

Pregnancy risks and considerations and the Implications of inheritance:

• gain informed consent;
• take a relevant history to inform further investigation or referral;
• perform investigations to a high standard; echocardiography and rhythm assessment/management.

Attitudes and behaviours

By the end of this module the Clinical Scientist in HSST will be expected to critically evaluate their own response to both normal and complex situations. They will consistently demonstrate the professional attributes and insights required of a Clinical Scientist in HSST working within the limits of professional competence, referring as appropriate to senior staff, and will work collaboratively with cardiologists, cardiac surgeons, other medical staff, healthcare science practitioners, echocardiographers and nurses, and will:

• recognise the young person’s need for advice and information about their CHD, including advice on the genetic aspects of the condition;
• recognise the difficulties young people face with engaging in healthcare at the time of transfer and seek to ensure seamless transfer of care;
• recognise the young person’s desire to be autonomous and to be involved in decision making;
• recognise the role of parents, family members, carers, partners and friends in supporting the young person at the time of transition and transfer;
• recognise and value the information available from other agencies and services and respect young people’s preferences in accessing information during transition;
• recognise and value opportunities for peer support at transition through local networks and other agencies;
• recognise the patient’s need for confidentiality;
• have a positive approach towards change to achieve continuous improvement of services, with particular reference to auditing practice, evidence-based practice, innovation and new and improved technologies;
• appreciate the psychological complexity relating to the change from paediatric to adult services and respond to patients accordingly;
• encourage the patients to take ownership and responsibility for their health while ensuring that the parents feel involved and informed;
• appreciate the emotional difficulties encountered by patients with CHD with respect to passing on cardiac lesions to any children they may have, and respond accordingly;
• appreciate the psychological impact of the potential inheritance of adult CHD on patients and their families;recognise the young person’s desire to be autonomous and to be involved in decision making;
• support the decisions of the patients without bias with respect to child-bearing.