Congenital Heart Disease Expert Scientific and Clinical Practice

Details

Title

Congenital Heart Disease Expert Scientific and Clinical Practice

Type

Stage Two

Module code

HPS140

Requirement

Compulsory

Module objective

By the end of this module the Clinical Scientist in HSST will be able to analyse, synthesise, critically evaluate and apply knowledge of: (i) the history and development of congenital heart disease (CHD); (ii) cardiac disease in syndromes and genetic disorders; (iii) the epidemiology of CHD; (iv) the morphology, physiology and modes of presentation of cardiac disease in the neonatal and postnatal periods; (v) cardiomyopathies and the associated diseases and syndromes and acquired cardiomyopathies; (vi) heart failure in paediatric cardiology, including the natural history, physiology and clinical features in the paediatric and CHD population; (vii) the pathology and natural history of infective endocarditis, Kawasaki disease, rheumatic fever and collagen vascular disease affecting the cardiovascular system; (viii) the modes of presentation and investigation of common arrhythmias presenting in infants and children; (ix) the common drugs used in the management of paediatric cardiac disease; and (x) the anatomy and physiology of the Fontan circulation, its demands on the circulation, complications and long-term outcomes.

The Clinical Scientist in HSST will also be able to analyse, synthesise, critically evaluate and apply knowledge of catheterisation in structurally abnormal hearts. They will be expected to perform and master complex protocols of interventional and diagnostic cardiac physiology as applied to patients with CHD.

In this patient-focused service they will need to be responsive to the needs of children and their parents/guardians at different stages of their journey through an illness or injury, from prevention through timely assessment, treatment, rehabilitation and long-term support, and aware that hospital services and the staff delivering them need to meet those requirements. The Clinical Scientist in HSST will 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.

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 CHD, including:

Genetics, genomics, pathology and epidemiology:

• the genetics of normal cardiac development;
• recurrence risks in families where one member has CHD;
• the cardiac abnormalities found in genetic disorders and syndromes, which may include:
  • Down syndrome
  • 22q11 deletion (DiGeorge)
  • Turner syndrome
  • Noonan syndrome
  • Williams syndrome
  • Alagille syndrome
  • Marfan syndrome
  • CHARGE association
  • VACTERL association
  • trisomy 18
  • trisomy 13
  • storage disorders
  • neuromuscular diseases
  • hyperlipidaemias
  • long QT syndrome
• the historical development of the specialty and changes in outcomes and survival in CHD over time;
• the prognosis of genetic syndromes and their associated cardiac defects;
• the epidemiology and survival rates in congenital and inherited cardiac disease;
• the impact and role of genomics and precision/personalised medicine for patients with CHD.

Structural lesions in CHD:

• the anatomical and characteristic clinical features, natural history and physiology of different forms of CHD;the complex mechanisms: understand both immune- and viral-mediated (independent of an immune response) cardiac damage;
• transitional cardiac physiology from fetus to neonate;
• how to distinguish cardiac and non-cardiac causes of collapse in infancy;
• the physiology of the duct-dependent systemic circulation and the duct- dependent pulmonary circulation;
• the ECG, CXR and echocardiographic findings of typical congenital heart lesions presenting with collapse in infancy;
• the principles of acute and long-term management of lesions presenting with collapse in infancy;
• how to distinguish cardiac and non-cardiac causes of cyanosis in the newborn period;
• the physiology of cyanosis caused by:
  • right heart obstruction
  • right to left shunting
  • parallel circulation
  • common mixing lesions
• acyanotic CHD:
  • left to right shunting lesions
  • duct-dependent systemic circulation
  • obstructive left heart lesions
  • acyanotic obstructive right heart lesions.

Cardiomyopathies and myocarditis:

• causes, physiology, prognosis and clinical features of cardiomyopathy and myocarditis;
• indications for treatment and understand the role of cardiac transplantation;
• genetics of inherited cardiomyopathies;
• aetiology, natural history and prognosis of viral myocarditis;
• the complex mechanisms: understand both immune- and viral-mediated (independent of an immune response) cardiac damage;
• the various ways acute myocarditis can present, including as a cause of sudden death in an otherwise healthy young adult;
• how to recognise electrocardiographic findings in patients with myocarditis;
• the limitations of echocardiography;
• the Dallas criteria for biopsy evidence of active myocarditis.

Introduction to heart failure in paediatric cardiology:

• the health economics of paediatric heart failure management and the differences compared with adults;
• the diverse aetiology of heart failure in children (which includes volume overload, pressure overload, cyanosis, primary myocardial disease of either or both ventricles, metabolic abnormalities and genetic mutations);
• the different modes of clinical presentation, depending on patient age;
• heart failure classification definition within a spectrum of severity and that the well-established New York Heart Association (NYHA) Heart Failure Classification is not applicable to most of the paediatric population;
• the differences between the Ross Heart Failure Classification, the modified Ross Classification and the New York University Paediatric Heart Failure Index for children and adolescents;
• the changes in physiology during the postnatal period, and its effects on the presentation of heart failure dependent on patient age.

Acquired heart disease

Infective endocarditis (IE):

• the association of IE with substantial morbidity and mortality;
• the epidemiology of IE resulting from the increased survival rate of children with CHD and the decrease in rheumatic valvular heart disease;
• the complexities of management of neonatal patients and patients requiring paediatric intensive care;evaluation of the cardiovascular sequelae using serial cardiac ultrasound studies.
• the increased risks of catheter-related IE, including the long-term risk of postoperative IE after correction of complex CHD;
• the pathogenesis of IE;
• the Duke criteria for IE;
• the clinical symptoms and signs of IE and be aware that the presentation is generally indolent;
• the variability of cardiac examination dependent on the type of heart disease present and the particular site of infection;
• the organisms most frequently responsible for IE;
• two-dimensional transthoracic echocardiography (TTE) as the main modality for detecting IE;
• indications for repeat TTE and/or from transoesophageal echocardiography (TOE);
• the complications of IE and the indications for surgery.

Other acquired diseases:

• the possible causes, symptoms and clinical presentation of Kawasaki disease;
• the pathology of vasculitis and the progressive disease process;
• the prominence of cardiovascular manifestations in the acute phase and the impact on long-term morbidity and mortality.

Cardiac investigations:

• the role of echocardiography for cardiac assessment for the detection of abnormalities of the proximal left main coronary artery (LMCA) and right coronary artery (RCA);
• the role of different imaging modalities such as echocardiography, CT, MRI and angiography;
• evaluation of the cardiovascular sequelae using serial cardiac ultrasound studies.

Common arrhythmias:

• the features of a normal 12-lead ECG and changes with age from prematurity to adulthood;
• the common arrhythmias in fetal life, infancy, childhood and adolescence, and their natural history, presentation and clinical features;
• ECG changes in CHD, acquired heart disease and cardiac surgery-associated arrhythmias;
• the mechanisms in the genesis of cardiac dysrhythmias;
• the genetic disorders associated with cardiac rhythm disturbance;
• investigating common arrhythmias;
• management strategies (pharmacological vs ablation);
• the indications, limitations and risks of invasive electrophysiology studies and radiofrequency ablation.

Single ventricle circulation:

• why the physiology of the Fontan circulation is essential for successful patient management as numbers of patients with a Fontan circulation are increasing and reaching adulthood;
• the indications for a Fontan for single ventricle physiology;
• the surgical approach at each stage:
  • Stage 1: systemic-pulmonary shunt
  • Stage 2: superior cardiopulmonary connection (Glenn/hemi-Fontan operation)
  • Stage 3: completion of the Fontan circulation (extracardiac conduit vs lateral tunnel)
• complications of a Fontan circulation:
  • diminished exercise tolerance
  • ventricular dysfunction
  • arrhythmias
  • shunts
  • protein-losing enteropathy
  • coagulopathy
• patient management:
  • preoperative assessment
  • monitoring
  • postoperative care
• why possible problems must be actively searched for and resolved before becoming clinically relevant and that the absence of a clinical problem does not equal satisfactory haemodynamics;
• why heart function in the pregnant Fontan patient deteriorates throughout pregnancy, and the associated physiological and haemodynamic changes;
• how ‘old’-style Fontan circuits compare with current operations and how this will change patient management in the next decades;
• the diagnostic imaging approach to the failing Fontan;
• current guidelines and recommendations and provide updates to team members;
• the psychosocial impact of CHD on individuals and families.

Neonatal, paediatric, adolescent and critical care:

• how to deliver effective, evidence-based and safe care to children and their families;
• why families should be encouraged to be active partners in decisions about their children’s health and care, and, where possible, be able to exercise choice;
• the importance of care being provided in an appropriate location and in an environment that is safe and well suited to the age and stage of development of the child or young person;
• the role of play and recreation for children visiting or staying in hospital that should be met routinely in all hospital departments, including siblings;identification of both common and rare congenital defects on fluoroscopy
• the importance of play for therapeutic purposes as part of the child’s care plan to help the child to assimilate new information, adjust to and gain control over a potentially frightening environment, and prepare to cope with procedures and interventions;
• the importance of respecting a child’s need for privacy;
• how to identify significant mental health problems and refer them appropriately to the correct support team;
• how to provide for a young person’s changing needs as they grow up, including preparation for the move to adult services;
• the duty of staff to understand and meet their legal responsibilities towards the children and young people they are caring for (Children Act 1989 (21)), including the legal and ethical position on real or potential conflicts between the interests of the child and those of the parents;
• special considerations for infection control among children and that emphasis is needed on cross-infection control, focusing on common childhood infections such as respiratory infection, gastroenteritis and chicken pox;
• linkage of guideline development into a programme of staff education and training;
• the importance of the monitoring, review and version control of protocols.

Catheterisation in structurally abnormal hearts

The Clinical Scientist in HSST will also critically analyse, synthesise, evaluate and apply their expert knowledge of catheterisation in structurally abnormal hearts, including:

• variations in cardiac and coronary radiographic anatomy in CHD hearts, including:
  • how landmarks differ compared with a normal heart
  • identification of both common and rare congenital defects on fluoroscopy
  • identification of intracardiac devices, clips, sternal wires and stents on fluoroscopy
  • the need for continuous transoesophageal (or transthoracic) echo monitoring to guide certain catheter procedures
• specific arrhythmias associated with congenital cardiac lesions and previous surgery:
  • how to distinguish a patient’s baseline ECG pattern (which will look abnormal in CHD) from pathological changes during the catheter procedure
  • the purpose of pacing during a procedure and how to prepare and use the equipment
• haemodynamic monitoring in CHD patients, including:
  • recognition of the various pressure waveforms in normal and pathological states
• the effects of sedation and general anaesthesia on patients with CHD and the presentation on haemodynamic and electrocardiographic monitoring;the need for continuous transoesophageal (or transthoracic) echo monitoring to guide certain catheter procedures
• the selection of appropriate devices/stents/balloons for a procedure and the different products on the current market, and the associated pros and cons for device selection.

Technical and clinical skills

By the end of this module the Clinical Scientist in HSST will apply their expert scientific and clinical knowledge of CHD as they perform and master a range of technical and clinical skills in adult and paediatric patients appropriate to the clinical setting in which they are training.

They will also be expected to critically reflect on their clinical practice in paediatric cardiology. They will use a range of advanced clinical and communication skills to advise and communicate effectively with patients, parents, carers, relevant clinicians, the public and other healthcare professionals. They should be able to identify significant mental health problems and refer them to the correct support team. They will apply their expert knowledge of CHD and paediatric practice to prioritise patient safety and compassionate care as they complete the programme- specific modules in this Stage 2 programme.

Attitudes and behaviours

By the end of this module the Clinical Scientist in HSST will be expected to be empathic working with patients and their families, treating them with kindness and compassion and communicating effectively. They should treat patients and their families with respect and provide support and information to enable them to understand and cope with their illness and the treatment needed.

• ensure that staff have an understanding of how to assess and address the emotional wellbeing of children;
• work effectively within a multidisciplinary team in the management of children, adolescents and young adults;
• recognise and minimise the anxiety of patients before, during and after procedures;
• appreciate the psychological impact of the patient’s CHD/syndrome on the patient and their family, and manage it sensitively;
• develop a critical attitude towards techniques in catheterisation and contribute to the selection of the best available techniques according to procedure results, clinical evidence and practice guidelines;
• reflect on the challenges of applying research to practice in relation to the diagnosis and management of patients requiring cardiac interventional procedures and suggest improvements, building on a critique of available evidence.