Dr David Roden Neonatologist Staffordshire, Shropshire and Black Country Newborn Network

Objectives Scenario Scenario The prevalence of congenital heart disease The prevalence of congenital heart disease How do we improve our detection rate for congenital heart problems in the community How do we improve our detection rate for congenital heart problems in the community Better understanding of congenital heart disease basics Better understanding of congenital heart disease basics Systematic way of evaluating the neonatal cardiovascular system including simple screening Systematic way of evaluating the neonatal cardiovascular system including simple screening Referral services Referral services

Typical Friday afternoon…… You are the community midwife reviewing a baby at home and are doing the newborn examination You are the community midwife reviewing a baby at home and are doing the newborn examination Term Baby Term Baby Normal Antenatal scans Normal Antenatal scans No family history of cardiac problems No family history of cardiac problems You hear a loud murmur!! You hear a loud murmur!!

What do you ? What do you need to do immediately? What do you need to do immediately? What do you say to Mum? What do you say to Mum? What do you do next? What do you do next?

The scope of the problem Congenital heart disease is common Congenital heart disease is common 5.3 per 1000 live births 5.3 per 1000 live births Early recognition important Early recognition important Deterioration can be sudden Deterioration can be sudden High risk of mortality High risk of mortality May reduce morbidity in terms of May reduce morbidity in terms of better surgical outcome better surgical outcome Prevention of irreversible pulmonary vascular disease Prevention of irreversible pulmonary vascular disease Examination before discharge and at 6 weeks for signs of congenital heart disease is recommended in Health for All Children Examination before discharge and at 6 weeks for signs of congenital heart disease is recommended in Health for All Children

However…….. The difficulties in detecting The difficulties in detecting congenital heart disease by examination alone are well known. Cardiovascular adaptation may Cardiovascular adaptation may produce murmurs mistaken for heart disease. Although congenital heart Although congenital heart disease is present at birth, there are often no signs and most are often no signs and most babies are asymptomatic Lesions dependent on ductal Lesions dependent on ductal patency may not present till days or weeks after birth Some heart lesions particularly Some heart lesions particularly obstructive left sided heart lesions may not be detected by these two examinations (1) Even when an abnormality is Even when an abnormality is found action is frequently not taken (2) 1.Silove ED. Assessment and management of congenital heart disease by the district paediatrician. Arch Dis Child Abu Harb et al.Death in infancy from unrecognised congenital heart disease Arch Dis Child 1994

How do we improve the detection of babies with congenital heart disease ? Understanding normal changes at birth Understanding normal changes at birth Simple way to think about congenital heart disease ( as I’m fairly simple) Simple way to think about congenital heart disease ( as I’m fairly simple) Overview of systematic examination of the cardiovascular system in babies Overview of systematic examination of the cardiovascular system in babies Simple additional screening Simple additional screening Robust care pathways Robust care pathways

Changes in the first few days of life Pulmonary pressure down Pulmonary pressure down Systemic pressure up Systemic pressure up Patent foramen ovale closes Patent foramen ovale closes Ductus arteriosus closes Ductus arteriosus closes

A baby with an open duct and high pulmonary pressure may have a completely normal examination in the first few weeks of life even if there is significant problems with heart

How can we think about congenital heart disease Cyanotic Cyanotic Blood flow to lungs impaired Blood flow to lungs impaired Blood flow from lungs to heart impaired Blood flow from lungs to heart impaired Too much mixing of oxygenated and deoxygenated blood Too much mixing of oxygenated and deoxygenated blood Systemic and pulmonary circulation don’t mix Systemic and pulmonary circulation don’t mix Mixed Mixed Acyanotic Acyanotic Left sided heart problems Left sided heart problems Extra tubes and holes Extra tubes and holes Narrowing of valves Narrowing of valves Some of these conditions will not present clinically until the duct has closed This may take days and occasionally weeks

Cyanotic Blood flow from right heart to lungs impaired Blood flow from right heart to lungs impaired Pulmonary atresia Pulmonary atresia Critical Pulmonary stenosis Critical Pulmonary stenosis Pulmonary stenosis Pulmonary stenosis Pumonary stenosis Pulmonary atresia

Cyanotic Blood flow from Lungs to left atrium impaired Blood flow from Lungs to left atrium impaired TAPVD TAPVD TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE Blue early on if there is obstruction to blood flow Blue early on if there is obstruction to blood flow PAPVD PAPVD PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE Blue when pulmonary pressure raised ( eg first respiratory illness) Blue when pulmonary pressure raised ( eg first respiratory illness)

Cyanotic Too much mixing of oxygenated and deoxygenated blood Too much mixing of oxygenated and deoxygenated blood Double outlet syndromes Large VSDs

Cyanotic Systemic and pulmonary blood flow unable to mix Systemic and pulmonary blood flow unable to mix Transposition of great arteries Transposition of great arteries

Cyanotic Mixed Mixed Fallot’s Tetralogy Fallot’s Tetralogy VSD VSD Overiding aorta Overiding aorta RVOT obstruction RVOT obstruction RV hypertrophy RV hypertrophy Usually present after a few months with cyanosis and/or failure to thrive. Usually present after a few months with cyanosis and/or failure to thrive.

Acyanotic Left sided heart lesions Left sided heart lesions Hypoplastic left heart syndrome Hypoplastic left heart syndrome Coarctation of aorta Coarctation of aorta

Acyanotic Extra tubes Extra tubes Patent ductus arteriosus Patent ductus arteriosus

Acyanotic Extra holes Extra holes VSD VSD Muscular Muscular Perimembranous Perimembranous Might not present until pulmonary pressures falls Might not present until pulmonary pressures falls Murmur Murmur Heart failure Heart failure

Acyanotic Extra holes Extra holes ASD ASD Usually incidental finding Usually incidental finding May have a murmur May have a murmur Not unusual to present much later on in life Not unusual to present much later on in life

Extra holes Extra holes Atrioventrcular septal defect (AVSD) Atrioventrcular septal defect (AVSD) Down’s syndrome Down’s syndrome

Examination of cardiovascular system Inspection Inspection Palpation Palpation Ausculation Ausculation

Inspection Inspection Dysmorphic Dysmorphic Cyanosis Cyanosis Tachypneoa Tachypneoa Oedema Oedema

Palpation Palpation Apex Apex Praecordium Praecordium Thrills Thrills Like stroking a cat Like stroking a cat turbulence turbulence Heaves Heaves Heart working hard Heart working hard Liver Liver Works like JVP in adult Works like JVP in adult Femoral pulses Femoral pulses If we feel the femoral pulses does this rule out coarction? If we feel the femoral pulses does this rule out coarction?

Ausculation Ausculation Heart sounds Heart sounds 1 and 2 1 and 2 Murmurs Murmurs Systolic or diastolic? Systolic or diastolic? Loud or soft? Loud or soft? Where is it loudest? Where is it loudest? INNOCENT PATHOLOGICAL

Can we believe our ears? Ausculation Ausculation Auscultation does not perform well as a screening tool still offers the only real opportunity to detect CHD early. Auscultation does not perform well as a screening tool still offers the only real opportunity to detect CHD early. However 50% of babies with congenital heart disease have no murmur on examination. However 50% of babies with congenital heart disease have no murmur on examination. Needs to be more widely recognised that the absence of a murmur does exclude the presence of potentially serious heart disease Needs to be more widely recognised that the absence of a murmur does exclude the presence of potentially serious heart disease

What can use to make the diagnosis? CXR CXR Sometimes useful Sometimes useful Increased or decreased blood flow Increased or decreased blood flow Big heart Big heart ECG ECG Generally not helpful in CHD Generally not helpful in CHD Echocardiogram Echocardiogram “the devilish toy in the corner of the room tempting one to use it” “the devilish toy in the corner of the room tempting one to use it” Needs to be used in conjunction with clinical findings Needs to be used in conjunction with clinical findings

Additional screening Pulse oximetry Pulse oximetry Post ductal saturations Post ductal saturations < 95% warning sign < 95% warning sign PULSOX study PULSOX study DUCT R hand L Hand and both feet

Referral pathways GP GP GP’s need additional referral criteria GP’s need additional referral criteria Clinical governance Clinical governance Paediatric training for new GP’s more limited Paediatric training for new GP’s more limited Local Paediatric wards Local Paediatric wards Direct access to “hot clinic” at local paediatric centre Direct access to “hot clinic” at local paediatric centre Murmur clinic Murmur clinic Specialist clinic where GP/midwife can refer for review and echo Specialist clinic where GP/midwife can refer for review and echo Regardless of route pathway needs to be robust and reproducible Regardless of route pathway needs to be robust and reproducible Must give the person carrying out the examination the reassurance that their findings will be taken seriously Must give the person carrying out the examination the reassurance that their findings will be taken seriously Must also safeguard the examiner from a medicolegal point of view. Must also safeguard the examiner from a medicolegal point of view.

Summary Clinical evaluation of cardiovascular system in newborn examination may miss significant heart disease Clinical evaluation of cardiovascular system in newborn examination may miss significant heart disease The absence of a murmur does not The absence of a murmur does not exclude serious heart disease Need for evaluation of supplementary information like pulse oximetry to aid detection rate Need for evaluation of supplementary information like pulse oximetry to aid detection rate The way in which we evaluate a baby for cardiovascular disease depends more on having structured referral criteria than experience The way in which we evaluate a baby for cardiovascular disease depends more on having structured referral criteria than experience