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Cardiology

Theme : Emergency medicine A. Adenosine B. Adrenaline C. Atropine D. DC shock E. Dobutamine F. Diving reflex G. Endotracheal intubation H. ntraosseous line . ! "orphine #. $aloxone

Select the most appropriate emergency treatment for the following children: 1) A %&'ear&old child is rescued from a house fire. (he is admitted tach'pnoeic and tacch'cardic. (he has soot in her nostrils. G. Endotracheal intubation Note: tem ) relates to burns. "ost deaths follo*ing house fires occur secondar' to smoke inhalation. Amongst the indicators of inhaled smoke in+ur' is deposits around the mouth and nose. ,edema follo*s thermal in+ur' and therefore an' suspicion of air*a' compromise should result in endotracheal intubation. 2) A - month old bab' is admitted *ith a histor' of poor feeding. ,n arrival he has a pulse rate of ../ beats per minute.

. !i"ing refle#

Note: (upraventricular tach'cardia is the diagnosis in tem -. !agal stimulation is the treatment of choice and the diving reflex is the simple procedure elicited b' submerging the bab'0s face in to ice or placing an ice bag over the face. 1he diving reflex increases vagal tone2 slo*s A! conduction interrupting the tach'cardia.

$) A )- 'ear old bo' is admitted *ith meningococcaemia. He is in shock. 3eripheral cannulation is difficult.

%. &ntraosseous line

Note: tem . relates to a child in shock. n man' life&threatening conditions venous cannulation is difficult. t is important to obtain vascular access ver' 4uickl' and therefore intraosseous infusion is recommended.

Theme : Syndromes associated with congenital heart disease A. Charcot&"arie& 1ooth s'ndrome B. Do*n s'ndrome C. Fragile 5 s'ndrome D. "arfan0s s'ndrome

Cardiology

E. $oonan s'ndrome F. (turge&6eber s'ndrome G. 1etrolog' of Fallot H. 1ourette s'ndrome . 1urner0s s'ndrome #. 6illiam0s s'ndrome or each of the cardiac lesions described below' choose the most commonly associated syndrome from the abo"e list of options. Each option may be used once or not at all. 1) (upravalvular aortic stenosis (. )illiam*s syndrome Note: 6illiam0s s'ndrome 7 is characteri8ed b' short stature2 characteristic facies2 supravalvular aortic stenosis2 mild to moderate learning difficulties and transient neonatal h'percalcemia. 2) Coarctation of Aorta &. Turner*s syndrome Note: 1urner0s s'ndrome 7 1his is characteri8ed b' %92 5 genot'pe2 ovarian d'sgenesis leading to infertilit'2 short stature2 *ebbing of the neck2 *ide carr'ing angles and *ide spaced nipples. Ho*ever the' have normal intellectual development. $) Dilatation of aortic root:aortic regurgitation !. +arfan*s syndrome Note: "arfan0s s'ndrome 7 1his is an autosomal dominant disorder. 1he clinical features are tall stature2 arachno&dact'l'2 high arched plate and increase in length of the lo*er segment of the bod' compared to the upper segment. 1he cardiac manifestations include dilated aortic root2 aortic incompetence2 mitral valve prolapse and mitral incompetence. ,) Cardiac cushion defects ;leading to A(D2 !(D< -. !own syndrome Note: Do*n s'ndrome 7 Features include characteristic facies2 h'potonia2 severe learning difficulties and small stature. About %/= of patients have cardiac anomalies mainl' endocardial cushion defects leading to A(D and !(D. .) nfundibular pulmonar' stenosis G. Tetrology of allot Note: 1etrolog' of Fallot 1his is a c'anotic heart disease and the cardinal features include> ). nfundibular pulmonar' stenosis .. !(D -. ?ight ventricular h'pertroph' %. ,ver&riding of the aorta /omments: Charcot&"arie&1ooth s'ndrome 7 Autosomal dominant peroneal muscular d'stroph'. Fragile 5 s'ndrome 7 "oderate learning difficult'2 macrocephal'2 characteristic facies ;long face2 large ears2 prominent mandible and forehead< $oonan s'ndrome7 Facies2 mild learning difficulties2 short *ebbed neck2 short stature and congenital heart disease ;pulmonar' valvular stenosis2 A(D2 left ventricular h'pertroph'< (turge&6eber s'ndrome 7 Haemangiomas in the distribution of trigeminal nerve and in the brain. (ometimes can have intractable epileps'. 1ourette s'ndrome 7 1ics2 compulsive utterances of obscene *ords ;coprolalia<

Theme : Emergency treatments A. Adenosine B. Adrenaline C. Atropine D. DC shock E. Dobutamine F. Diving reflex G. Endotracheal intubation H. ntraosseous line . ! "orphine #. $aloxone

Cardiology

Select the most appropriate emergency treatment for the following children: 1) A 9 'ear old bo' is brought to the hospital *ith )9= scalds to his chest. &. &0 +orphine

Note: tem ) relates to a child *ith serious burns. Children *ho have been burnt are in severe pain and therefore ! "orphine is the analgesic of choice.

2) A )%&'ear&old girl *ith a histor' of previous overdoses is admitted to A@E apnoeic and unconsciousness. ECG sho*s ventricular fibrillation. C3? is commenced.

!. !/ shoc1

Note: tem . describes a child in ventricular fibrillation. 1his is uncommon in childhood although ma' occur as a result of tric'clic antidepressant overdose and h'pothermia. f the arrest is *itnessed a precordial thump is carried out other*ise electrical de&fibrillation at . +oules per kilogram.

$) A term bab' is born in poor condition. Apgar scores - at ) minute and 9 at 9 minutes. C3? is commenced. At )/ minutes he -. 2drenaline remains brad'cardic.

Note: tem - describes a bab' born in poor condition. A brad'cardia in an unstable ne*born re4uires ox'genation2 ventilation and cardiac compressions. ! adrenaline is administered as Atropine is ineffective in this age group.

Theme : Genetics 3 cardiac abnormalities in genetic disorders A. Angelman0s s'ndrome B. Beck*ith&6iedemann s'ndrome C. Congenital ?ubella s'ndrome D. Do*n s'ndrome E. Foetal alcohol s'ndrome F. Gl'cogen storage disease G. "arfan0s s'ndrome H. $oonan0s s'ndrome . 1urner0s s'ndrome #. 6illiams s'ndrome +atch each of the following cardio"ascular abnormalities to the single most li1ely associated genetic disorder. 1) Dilation of the aorta *ith aneur'sms. G. +arfan*s syndrome

Cardiology

Note: n "arfan0s s'ndrome dilatation of the ascending aorta is often seen *ith or *ithout aneur'sms. Aess commonl' the thoracic abdominal aorta or pulmonar' arteries are affected *ith secondar' aortic regurgitation and mitral valve prolapse.

2) (upra&valvular aortic stenosis.

(. )illiams syndrome

Note: n 6illiams s'ndrome supra&valvular aortic stenosis is the most common cardiac lesion. (eptal defects also occur as *ell as peripheral branch pulmonar' arter' stenosis.

$) 3ulmonar' stenosis.

%. Noonan*s syndrome

Note: n $oonan0s s'ndrome pulmonar' valve stenosis due to a d'splastic or thick valve is seen often associated *ith left ventricular h'pertroph'. Branch stenosis of the pulmonar' arter' also is found in $oonan0s s'ndrome.

Theme : /ongenital cardiac defects A. Angelman0s s'ndrome B. Beck*ith&6iedemann s'ndrome C. Congenital ?ubella s'ndrome D. Do*n s'ndrome E. Foetal alcohol s'ndrome F. Gl'cogen storage disease G. "arfan0s s'ndrome H. $oonan0s s'ndrome . 1urner0s s'ndrome #. 6illiams s'ndrome +atch each of the following cardio"ascular abnormalities to the single most li1ely associated disorder. 1) Endocardial cushion defect. !. !own syndrome

Cardiology

Note: n Do*n s'ndrome approximatel' %/= of children have a congenital heart disease. 1he most common being endocardial cushion defects although !(Ds2 A(Ds and 3DA also occur. 2) Coarctation of the aorta. &. Turner*s syndrome Note: n 1urner0s s'ndrome cardiac defects are common. -/= include bicuspid aortic valves *ith the second most common heart defect being coarctation of the aorta. Aortic stenosis2 mitral valve prolapse and h'pertension are also found. $) (eptal defects. E. oetal alcohol syndrome Note: n foetal alcohol s'ndrome individuals have poor gro*th2 developmental dela' and usuall' characteristic facial features including microcephal' and a short smooth philtrum. 1he most common cardiac lesion in these children are septal defects primaril' ventricular septal defects.

Theme : -4E2T%5ESSNESS A. Asthma B. H'perventilation C. 1uberculosis D. C'stic Fibrosis E. 3neumoc'stis carinii F. !entricular septal defect G. Gastroesophageal reflux H. Atrial septal defect . Bronchiolitis #. "itral stenosis or each of these patients with breathlessness' select the most li1ely diagnosis 1) A thirteen&'ear&old girl *ho has intermittent episodes of breathlessness2 *hich tend to occur in cro*ded shops. (he feels the need to take deep breaths and then breathes ver' 4uickl'2 -. %yper"entilation complaining of pins @ needles around her mouth and in her hands. Her chest is clear and her blood gases sho* a normal p,. and lo* pC,.. Note: 1his description is one of anxiet'. 1he blood gas picture is one of h'perventilation. 2) A three&'ear&old bo' *ho presents *ith *orsening cough and E. 6neumocystis carinii

Cardiology

breathlessness of - *eeksB duration. His mother *as an intravenous drug abuser. He has al*a's been prone to infections. 6hen he *as . 'ears old he had chicken pox for % *eeks. ,n examination2 he has an emaciated appearance2 his *eight is belo* the /.%th centile2 he has a temperature of -C.DEC and he has generali8ed crepitations on auscultation of his chest. A blood count sho*s severe l'mphopenia. Note: 1his has resulted from congenitall' ac4uired H !. 3C3 has an insidious onset and often there are no chest signs in children. A'mphopenia is consistent. 1reatment is *ith septrin or nebulised pentamidine as second line. $) A %&month&old bab' has not gained much *eight since birth and onl' takes small milk feeds2 as he appears to become breathless on feeding. He is tach'pnoeic2 s*eat' and has a . 0entricular septal defect tach'cardia. His liver is enlarged and he has a harsh grade .& pans'stolic murmur at the left lo*er sternal edge. Note: 3oor feeding is a s'mptom of heart failure in babies. A !(D has a pan s'stolic murmur and if large ma' have a lo*er grade as there is less resistance to flo*. Hepatomegal' is an earl' sign of heart failure in infants.

Theme : /7NGEN&T25 %E24T !&SE2SE A. ,stium secundum atrial septal defect B. !entricular septal defect C. 1ransposition of the great arteries D. 1otal anomalous pulmonar' venous drainage E. Atrioventricular septal defect F. 3atent ductus arteriosus G. 3ulmonar' valve stenosis H. Coarctation of the aorta . 1etralog' of Fallot #. H'poplastic left heart s'ndrome )hich of the abo"e is the most li1ely diagnosis in the following cases. 1) A )-&'ear&old girl is referred for evaluation of her short stature. (he is prepubertal. ,n auscultation she has an e+ection s'stolic murmur in the G. 6ulmonary "al"e stenosis second and third left intercostals spaces radiating to the back2 but is a s'mptomatic. Note: 1he murmur describes pulmonar' stenosis2 *hich could also be a left peripheral pulmonar' stenosis. (he is short and has dela'ed pubert' and coupled *ith the cardiac findings *ould suggest $oonanBs s'ndrome. 2) A C&*eek&old infant presents *ith breathlessness on feeding and failure to thrive. ,n examination his femoral pulses are difficult to feel %. /oarctation of the aorta but present. Chest radiograph sho*s cardiomegal' and increased vascular markings. Note: Absent or *eak femoral pulses suggest coarctation. ?emember association *ith 1urnerBs s'ndrome. $) An infant is seen for his D&*eek check and found to have a loud e+ection s'stolic murmur in the third left intercostal space and a single &. Tetralogy of allot

Cardiology

second heart sound on examination. 1here is no obvious c'anosis but a suggestion of mild desaturation. ,n the chest 5 ra' there is a concavit' on the left heart border and decreased pulmonar' vascular markings. Note: 1etralog' of Fallot ma' present later than in the neonatal period. 1he e+ection s'stolic murmur is from the infundibular stenosis. 1he desaturation results from the right to left shunt across the !(D.

Theme : /ongenital heart disease A. Atrioventricular septal defect B. Coarctation of the aorta C. H'poplastic left heart s'ndrome D. ,stium secundum atrial septal defect E. 3atent ductus arteriosus F. 3ulmonar' valve stenosis G. 1etralog' of Fallot H. 1otal anomalous pulmonar' venous drainage . 1ransposition of the great arteries #. !entricular septal defect )hich is the most li1ely diagnosis in the following cases8 1) An infant is found profoundl' c'anosed and lethargic in his cot on da' .. ,n auscultation there is a soft s'stolic murmur heard inconsistentl' at the left sternal edge and a single second sound. &. Transposition of the great 1he chest 5 ra' sho*s a narro* upper mediastinum2 h'pertrophied arteries right ventricle and increased pulmonar' vascular markings. 1he ECG sho*s a normal neonatal pattern. Note: C'anosis on the second da' is suggestive of a duct&dependent lesion. 1he rest of the ans*er describes 1GA 2) A -&*eek&old premature infant born at .C *eeks gestation remains ventilated follo*ing surfactant deficient respirator' distress s'ndrome. ,n auscultation of his chest a s'stolic murmur is heard at E. 6atent ductus arteriosus the left sternal edge and pulses are ver' eas' to feel. 1here is pulmonar' plethora on chest 5 ra'. Note: 3DA is a relativel' common problem in premature babies. 1he left to right shunt results in excess blood flo* through the lungs and fre4uentl' ox'gen dependenc' and difficult' in *eaning from the ventilator. A loud s'stolic murmur radiating to the back *ith easil' palpable pulses are t'pical. $) A C&'ear&old bo' is examined for a chest infection. An incidental finding of a short s'stolic murmur *ith fixed splitting of the second !. 7stium secundum atrial septal heart sound is detected. His blood pressure is normal and all pulses defect are normal. Note: ,stium primum defects are unlikel' to present incidentall' but rather *ith heart failure or pulmonar' h'pertension. Fixed splitting is t'pical of ostium secundum defects

Cardiology

Cardiology

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