HOW TO TREAT 27
ausdoc . com . au 12 JULY 2024
HOW TO TREAT 27
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Box 3 . Those who require ongoing surveillance of their lipid profile and optimum treatment |
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Figure 9 . Kawasaki disease .
Figure 9A . A large 15cm aneurysm arising from the left anterior descending coronary artery , with significant narrowing just proximal to the aneurysm and mild narrowing just distal to the aneurysm .
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Figure 9B . Coronary angiogram showing giant coronary artery aneurysm in the left anterior descending coronary artery . |
Reprinted from Heart Lung Circ ; 17 . Copyright 2008 , with permission from Elsevier . |
• Adolescents with type 1 diabetes .
• Past history of Kawasaki disease with persistent aneurysms .
• Severe obesity .
• A heterozygous familial hypercholesterolaemia .
• Systemic hypertension .
• Coarctation of the aorta .
• Aortic stenosis .
• Any congenital heart abnormality that requires translocation of the coronary arteries , such as transposition of the great vessels following an arterial switch or a Ross procedure carried out for aortic valve replacement , where the coronary arteries are removed from the previous aortic root and reinserted into the transposed pulmonary root now attached to the aortic site . 46
• A cardiomyopathy , particularly hypertrophic cardiomyopathy .
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remain high , necessitating appropriate medication . This is especially important for the homozygous familial hypercholesterolaemia that may ultimately require a liver transplant . Those who require ongoing surveillance and optimum treatment appear in box 3 .
CASE STUDY
JACKSON is delivered vaginally at term , weighing 3.6kg . He is mildly dusky but otherwise well , with normal pulses , no murmurs , a heart rate
1 . Which THREE anomalies are of little consequence and require only reassurance and follow-up ? a Small ventricular septal defect
( VSD ). b Mild pulmonary valve stenosis . c Large patent ductus arteriosus
( PDA ). d Innocent murmurs detected incidentally .
2 . Which TWO statements regarding prenatal diagnosis of heart disease are correct ? a Most anomalies are detected during the scan at the end of the first trimester . b Complex lesions may still be missed . c A fetal scan will determine whether an atrial septal defect or a PDA will close following birth . d A prenatal diagnosis of a serious cardiac abnormality will influence the timing , mode and location of delivery .
3 . Which THREE features should prompt referral in a newborn ? a Poor feeding . of 120bpm and a respiratory rate of 52 breaths per minute . An earlier fetal scan was reported as normal . His respiratory rate gradually drops to 40 breaths per minute . At four hours , he appears blue at rest with a saturation of 75 %, raising the possibility of cyanotic CHD .
A chest X-ray shows a normal-sized heart and clear lung fields . An ECG is unhelpful . Retrieval is requested . On advice , a prostaglandin E1 infusion is started , he is observed for possible apnoea and oral feeds are ceased .
How to Treat Quiz .
b Tachypnoea , especially after the first 24-48 hours . c Uniformly poor or unequal pulses . d Cyanosis .
4 . Which ONE condition will respond to 100 % oxygen with a substantial rise in pO2 ? a Transposition of the great vessels . b Severe tetralogy of Fallot . c Respiratory cause . d Truncus arteriosus .
5 . Which THREE statements are correct ? a Stenotic lesions usually result in a murmur . b A murmur from a VSD will take longer to manifest as the left-to-right shunt is at atrial level and occurs mainly during diastole . c Murmurs are also detected in older infants and children
The retrieval team organises a blood gas . The pO2 is 31mmHg , pH is 7.22 and the base excess is -5. The infant is intubated for transfer to the tertiary centre .
On arrival , an echocardiogram confirms transposition of the great vessels with an intact ventricular septum . There is a small fossa ovalis atrial defect with a small left-to-right shunt . The PDA provides a right-to-left shunt ( desaturated aortic blood into the saturated pulmonary blood ).
A balloon atrial septostomy is
IDENTIFYING HEART DISEASE IN CHILDHOOD
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when they present with an intercurrent illness . d Occasionally , murmurs that sound innocent may arise from an underlying cardiac abnormality .
6 . Which THREE features should prompt referral ? a Genetic disorders , only in the presence of a murmur . b Tachypnoea . c Differential pulses . d Cyanosis .
7 . Which TWO may be features of pulmonary hypertension ? a Louder summated second heart sound in the pulmonary area . b Louder summated second heart sound over the aortic area . c The second heart sounds summated into a single sound . d RV hypertrophy on ECG .
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performed to enlarge the atrial defect to increase the left-to-right shunt , thereby improving the systemic saturation . The prostaglandin infusion is weaned , allowing mother to start feeding . An arterial switch is performed on day eight , transposing the ascending aorta to the pulmonary root and translocating the coronary arteries . The pulmonary artery is connected to the aortic root . Jackson has an uneventful postoperative course .
It is not unusual for the diagnosis of transposition of the great vessels
8 . Which THREE statements describe supraventricular tachycardia in the newborn ? a Heart rate of 240bpm . b Too fast to count . c Irregular rhythm . d SVT presenting in the newborn period or shortly after may be missed .
9 . Which TWO statements regarding the management of rheumatic fever are correct ? a A definitive diagnosis is required . b Evidence of recent group A streptococcal infection . c A diagnosis may be made retrospectively . d Penicillin is only required to treat the throat infection .
10 . Which THREE statements related to Kawasaki disease are correct ? a Multisystem inflammatory disorder of uncertain origin . b It is more severe in male infants of Asian origin . c Early infusion of gamma globulin reduces the incidence of coronary aneurysms . d Antibiotic treatment is required . with an intact ventricular septum to be missed prenatally as a three-vessel outflow view is required to determine the connections .
In this case , cyanotic CHD is identified as the cyanosis increases despite resolution of Jackson ’ s probable mild transient tachypnoea .
CONCLUSION
MOST cardiac anomalies in childhood require a clear diagnosis and intermittent review — not only for the index patients and their families but also for the attending GP who continues to care for the patient . However , many lesions may be serious and life-threatening ; these require timely diagnosis and the institution of appropriate interventions to achieve best outcomes .
A prenatal diagnosis forewarns the clinician . The presence of a murmur draws attention to the heart and is especially meaningful if there are other cardiovascular signs , such as tachypnoea , poor or differential pulses , or cyanosis . If in doubt , it is best to refer as , at times , complex anatomy may present with minimal signs .
The converse is also important : reassuring parents and their child that the heart is normal despite symptoms such as chest pain , palpitations or innocent murmurs that point to a possible cardiac abnormality . Echocardiography is a major boon to the diagnosis and management of heart disease in childhood , but it does require expert interpretation to avoid causing increased anxiety with incidental insignificant findings .
CHD has been a major success story over the past 50 years . Important strides have been made in the management of cardiomyopathies and arrhythmias , with advances in genetic and molecular medicine helping our understanding of the problems faced and guiding management . Now , more than 90 % of affected patients survive into adulthood .
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