Approach to a child with congenital acyanotic .pptx

Approach to congenital acyanotic heart disease

1. Approach to a child with congenital Acyanotic Heart Disease . Dr. Shakilur rahman Phase A, Year 1 resident Paediatric Cardiology, BSMMU.

2. Congenital Heart Disease Cyanotic Acyanotic Shunt lesion Non shunt lesion Pre tricuspid Post tricuspid Obstructive Regurgitant

3. Slide Title Product A Feature 1 Feature 2 Feature 3 Product B Feature 1 Feature 2 Feature 3

4. The systematic approach to CHD includes – Detailed antenatal & postnatal history . Systematic physical examination  general examination, examination of CVS, respiratory system & abdomen, Investigations : ECG Chest X-ray Echocardiography. CT angiogram & cardiac MRI in selected cases. Cardiac catheterization.

5. Suspicion of CHD should be raised by the presence of – Feeding difficulties. Sweating Subcostal recession Recurrent RTI Tachypnea , Growth impairment in infant Exercise intolerance Easy fatigability or murmur in older child.

6. SYMPTOMS Feeding Difficulties : It is a common symptom in significant acyanotic CHD. In infants feeding itself is an exercise or effort. Inability to complete feed within 15-20 mins, sucks less volume of milk (<3.5 ounces per feeding), gets tired easily, takes frequent feeds. Due to inadequate feeding they become irritable & cry excessively. This suck rest suck cycle continues round the clock.

7. Respiratory Distress : It is most prominent sign of HF caused by significant left to right shunting in infancy. Symptoms include- Tachypnea , intercostal & subcostal retraction. Grunting & nasal flaring in very sick infants. Easy Fatigability : In an infant it is seen as poor ability to suck & feed. In older children HF may be manifested as exercise intolerance, difficulty in keeping up with peers during sports or need for a nap after coming home from school & poor growth. Age specific activities including climbing, walking, bicycle riding, physical sport are questions to be asked.

8. Repeated Lower Respiratory Infections: Recurrent pneumonia is defined as 2 episodes of pneumonia in 1 year or 3 episodes in any time frame. It is more frequent in infant than older children with CHD. Growth Retardation or Failure to Thrive : It is defined as weight less than 3 rd percentile for age. Usually the rate of weight gain is more delayed than that of height gain. It is related to inadequate caloric intake due to breathlessness during feeding & to the excessive energy requirement of CHF.

9. Chest Pain: It is rare in infants but common & benign in older children & adolescents. Pain associated with palpitation , dizziness, panic attacks may be presenting symptom of some Mitral valve prolapse patients. Pain , dizziness, fatigue may be present in LVOTO in the form of stenosis of aortic valve, sub aortic valve area, supra aortic valve area or CoA . Pain due to myocardial ischemia can occur in congenital anomalies in coronary artery, CA fistula, stenosis or atresia of CA ostium .

10. Syncope : Syncope is a transient loss of consciousness due to generalized cerebral ischemia, which is usually followed by rapid & complete recovery. IT may result from impaired response of the ANS or from cardiac structural defects especially those obstructing outflow or cardiac arrhythmia. H/O syncope on mild to moderate exertion indicates severe AS, hypertrophic cardiomyopathy , severe pulmonary hypertension, CTGV.

11. Peripheral Edema : When peripheral edema develops in infants , it appears first periorbitally & usually preceded by tachypnea , tachycardia, dyspnea & liver enlargement. Pretibial & Presacral edema are late developments in child with CHF due to difference in tissue turgor .

12. If one parent has CHD, the risk of the child having one (frequently same type) is as high as 10%. When a 1 st cousin has CHD, the risk of sibling having one is 2%. With no family H/O CHD, if the firstborn has CHD, the risk of 2 nd child having CHD is 2-3%. Family History : Maternal History: Exposure to drugs ( lithium, phenytoin , thalidomide) Alcohol intake TORCH infection usually Rubella. Maternal disease such as DM, SLE, phenylketonuria . Exposure to radiation.

13. Physical Examination Following features should be observed and evaluated : Does the patient show signs of distress ( tachypnea , shortness of breath, clamminess or diaphoresis)? Is the child responsive, interactive, happy, or irritable? Is there any change in colour (pallor or cyanosis)? Are there any dysmorphic features? Any skeletal anomaly of chest, back and extremities; precordial bulge or asymmetry of chest? Is the patient age appropriate for height & weight? Is there any developmental delay?

14. GENERAL EXAMINATION Extracardiac malformation can give a clue towards certain CHD. Physical deformity like – Polydactyly , fingerized thumb indicate ASD or VSD. Hypertelorism , distinctive webbed neck, low set ears, micrognathia , malocclusion of teeth, wide spaced nipple in Noonan syndrome in PS, ASD with or without PS. Short neck with low hairline , mongoloid facies with transverse simian crease on palm, hypotonic, hyperflexible limbs in Down syndrome in ECD, VSD, ASD. Elfin facies indicate William Syndrome in Supravalvular AS, peripheral PS. Musculo skeletal anomaly in Marfan syndrome found in Dissection of aorta, aortic aneurysm, AR.

15. Cont.. Height, weight, OFC, chest circumference should be plotted on age appropriate growth chart. Puffiness of face, pitting edema of feet indicate CHF. Clubbing & sign of Infective endocarditis – Petechiae Subungual hemorrhage Osler nodes Janeway lesions Roth spot. Vitals ( HR, RR, BP, temp) should be noted.

16. VITALS Respiratory distress is the most prominent sign of HF. In newborn & infant its diagnosed by tachypnea , altered depth of breathing, intercostal , subcostal retraction, flaring of alae nasi , grunting, stridor & apneic spells. Arterial pulse : Routine examination in infants involves brachial & femoral arteries. In adolescent, the carotid artery is added. If femoral pulse is delayed, weak or absent in comparison to brachial or radial pulse it indicates CoA or interrupted aortic arch. If there is any radio-radial delay, it indicates Supra valvular aortic stenosis (SVAS), aortic isthmus stenosis . Collapsing pulse/ water-hammer pulse found in PDA with normal pulmonary pressure, aortopulmonary window, large arteriovenous fistula & bicuspid aortic valve with severe AR.

17. VITALS (cont..) Blood pressure : In all patients suspected of cardiac disease, one should measure BP accurately in both arms & one leg. This helps in diagnosis of conditions causing obstruction ( CoA ), “aortic run off” (PDA, aorto pulmonary window). In CoA there is upper extemity hypertension. Narrow pulse pressure associated with low CO or severe AS. Wide pulse pressure in elevated CO (anemia, anxiety) or with abnormal run off ( PDA or aortic insufficiency).

18. Jugular venous pressure : It is raised when right atrial pressure increase (right sided HF). Prominent “a” wave indicates forceful atrial contraction ( tricuspid atresia , PS with intact ventricular septum, Eisenmenger syndrome due to ASD or PDA). Oxygen saturation : Less than 94% in lower extremity should alert practitioner either to cyanotic CHD or condition causing right to left shunting through PDA to descending aorta.

19. PRECORDIAL EXAMINATION Inspection: Look for abnormal chest shape ( pectus carinatum , pectus excavatum ), visible pulsation , operation scars, implanted pace maker, subcostal indrawing . The visible pulsation over the precordium or hyperdynamic precordium are mainly seen in volume overload conditions like post tricuspid shunts. Left parasternal pulsation or lift can be seen in large ASD & RV hypertrophy. Suprasternal pulsation can be visible in PDA, aortopulmonary window, aortic insufficiency, AS & CoA .

20. Palpation: Apex beat : It is the point of maximal cardiac impulse. A hyperdynamic apical impulse seen in volume overload condition like post tricuspid shunts & a sustained heaving apical impulse seen in pressure overload condition like LVOTO. Thrill : it is a palpable vibration caused by turbulent blood flow & is always pathological. Thrills are coarse, low frequency vibration occurring with a loud murmur & at the same area of maximal intensity of murmur. Thrill at lower sternal border more likely to be associated with VSD than MR or TR. Thrill at right upper sternal border or suprasternal notch are most likely due to severe AS ( less commonly PS). Parasternal heave : it is a forceful outward movement of left lower parasternal region of precordium & it indicates RVH.

21. AUSCULTATION The examiner should auscultate over the listening areas; 2 nd RICS, 2 nd LICS, 4 th LICS & apex. The carotid areas, both front & back of the chest should also be included. Heart Sounds: 1 st & 2 nd HS are produced by closure of AV & semilunar valves respectively. S2 is of great diagnostic significance & also helps in assessing the severity of lesion. A loud S1 can occur with increased flow across the AV valves from large left to right shunt, such as ASD, VSD or PDA. S2: Normal splitting : May occur in small VSD, mild aortic or PS.

22. fig: Auscultatory areas

23. Wide splitting : Found in – ASD, partial anomalous pulmonary venous connection (PAPVC), idiopathic dilatation of the pulmonary artery, moderate to severe pulmonary PS, pulmonary hypertension with right heart failure & acute massive pulmonary embolism. Fixed splitting : It denotes absence of significant variation of splitting interval with respiration, such that the separation of A2 & P2 remains unchanged during inspiration & expiration. It is found in ASD & severe right HF.

24. Paradoxical splitting or reverse splitting : Heard maximal during expiration & minimal or no during inspiration. Found in - PDA ( increase LV ejection volume) , severe AS ( LVOTO) LBBB (delayed LV depolarization) (most common). Narrow splitting : it is seen in shunt lesion with severe PAH without RV failure. Narrow physiologic splitting occurs in PDA with PAH.

25. If P2 is very loud & increases with mild exercise, then PAH with ASD can be diagnosed. Soft or muffled P2 found in Pulmonary stenosis . 3 rd & 4 th Heart Sounds : S3 & S4 are low pitched sound that occur in ventricle. S3 related to high flow & S4 reflects poor ventricular compliance. S3 is normal in children with hyperdynamic circulation & thin chest wall. It occurs due to rapid ventricular filling in early diastole & best heard at cardiac apex or left lower sternal border. A Gallop rhythm heard in CHF often represents exaggeration of S3 in the presence of tachycardia. S4 is never considered as normal & due to atrial contraction inducing ventricular filling late at diastole.

26. Murmurs: It Is defined as “a relatively prolonged series of audible vibrations of varying intensities (loudness), frequency (pitch); quality, configuration & duration.” It can be Innocent (functional) & Pathological (organic). The pathological murmurs are classified by their timing as - Systolic (occurring between S1 & S2) Diastolic (between S2 & S1). Continuous (present throughout the cardiac cycle) The systolic murmurs are graded from 1 to 6 & diastolic murmurs are graded from 1 to 4.

27. Grading of Murmurs: Grade 1 : So faint that it can be heard only with special effort. Grade 2 : faint, but can be heard easily. Grade 3 : moderately loud but no thrill. Grade 4 : loud with palpable thrill. Grade 5 : extremely loud & can b heard if only the edge of stethoscope is in contact with the skin. Grade 6 : exceptionally loud & can be heard with stethoscope just removed from skin contact.

28. Systolic murmur : Pansystolic murmur : starts with S1 & continues through systole . MR (apex → radiate to axilla) TR VSD left sternal border, no radiation . Ejection Systolic murmur : Just after S1, gradually increasing intensity & then fade & disappear before S2. AS (apex, radiate to right sternal border) PS (upper left sternal borderleft chest & back.) ASD (upper left sternal border)

29. Late systolic murmur : starts after S1 & extends to A2 or P2. Mitral valve prolapse (apex). Continuous murmur : It begins in systole & continues without interruption, encompassing S2, throughout diastole or a part of thereof. PDA AV fistula Venous hum in normal child VSD with AR.

30. Diastolic murmur : Mid-diastolic murmur : MS (apex, no radiation) TS (left sternal edge) Left atrial myxoma (left sternal edge). Early diastolic murmur : AR (aortic area) PR (pulmonary area).

31. RESPIRATORY SYSTEM EXAMINATION : Apart from rate, auscultation is very important. Bilateral crepitation is sign of LHF, which is diffusely heard & often associated with rhonchi. ABDOMINAL EXAMINATION : Normally liver is palpable (2-3cm below costal margin) at midclavicular line up to 4 to 5 years of age., after that it remains palpable up to 1 cm till late childhood. Further palpable liver & irritable infant – probably CHF. Liver in midline & symmetrically palpable indicates cardiac malposition & underlying complex cardiac disease. Spleen not palpable normally, if palpable it indicates possibility of Infective endocarditis.

32. INVESTIGATIONS ECG Chest X-Ray. Echocardiography. CT angiogram & cardiac MRI in selected cases. Invasive procedure such as cardiac catheterization & angiocardiography, if needed.

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