Summary

These lecture notes cover various aspects of heart diseases in pediatrics. The topics include different types of congenital heart diseases, their etiologies, classifications, pathophysiology, symptoms, signs, investigations and treatments.

Full Transcript

‫‪Page1‬‬ ‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‪َ ،‬و َعِّلْمِني َما َيْنَفُعِني‪َ ،‬وِزْدِني ِعْلًما"‬ ‫َّ‬ ...

‫‪Page1‬‬ ‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‪َ ،‬و َعِّلْمِني َما َيْنَفُعِني‪َ ،‬وِزْدِني ِعْلًما"‬ ‫َّ‬ Heart Diseases2 1) VSD Page 3 2) ASD Page 8 3) PDA Page 13 4) Pulmonary stenosis Page 17 5) Aortic Stenosis Page 18 6) COA Page 20 Page2 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ ▪ Etiology: Chromosomal & Genetic Factors: Down Syndrome, Turner‘s Syndrome,… Environmental Factors: Congenital Rubella, Maternal Diabetes, Drugs,… Hereditary Factors: If A Parent Or A Sibling Has CHD, the Risk is increased. ▪ Classification of CHD: 1) Cyanotic Heart Disease 2) Acyanotic Heart Disease Decreased pulmonary flow: Left - Right shunt lesions:  Tetralogy of Fallot  Ventricular septal defect  Tricuspid atresia  Atrial Septal Defect  Other univentricular heart with pulmonary  Atrio-ventricular Septal Defect stenosis.  Patent Ductus Arteriosus  Increased pulmonary flow: Obstructive lesions:  Transposition of great arteries  Aortic stenosis  Total anomalous pulmonary venous return.  Pulmonary valve stenosis & Coarctation of Aorta [A] Acyanotic Congenital Heart Diseases: VSD: Ventricular Septal Defect ASD: Atrial Septal Defect PDA: Patent Ductus Arteriosus PS : Pulmonary Stenosis AS : Aortic Stenosis CoA: Coarctation of Aorta 1) Ventricular Septal Defect  Incidence- Description- Pathophysiology- Symptoms- Signs - Investigations  Complications- Differential Diagnosis- Fate (natural history)- Treatment ▪ Incidence: The Commonest ≫ about 25-30% of all congenital heart diseases. Site of defect:  Defect In interventricular septum ( septum between 2 ventricles) that composed of 2 parts ≫ muscular part (lower 2/3) and membranous part ( upper 1/3). Page3 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ  Muscular part closes faster than membranous part.  Common: single defect in membranous part of the ventricular septum.  Less common: single or multiple defects in muscular part of the septum. ▪ Pathophysiology :  The pathophysiology and amount of shunting depend on: Size Of The Defect. Degree Of Pulmonary Resistance.  Blood is shunted from the left ventricle (higher pressure) to the right ventricle (lower pressure) ≫ Increased pulmonary blood flow ≫ Pulmonary congestive symptoms ≫ Volume overload over right ventricle,left atrium and left ventricle.  → → →↑ →↑ → → → → Membranous VSD MUSCULAR VSD Page4 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ Symptoms Signs: Small defect : Small defect :  No symptoms.  Systolic thrill ≫ grade 4.  Harsh pan- systolic murmur at 3rd & 4th Large defect (pulmonary congestion left inter-costal spaces. symptoms) Large defect:  Dyspnea on exertion then at rest.  Prominent precordium.( pericardial  Chronic cough bulge) ≫ in prolonged cases.  Repeated lower respiratory tract infections  Cardiomegaly with laterally displaced  Excessive sweating during feeding. apex.  Failure to thrive.  Systolic thrill at LLSB.  Harsh pan systolic murmur at 3rd & 4th left intercostal spaces.  Functional flow murmurs on mitral (mid- diastolic) & pulmonary (ejection systolic). ▪ Size of VSD: Small (< 3 mm in diameter) Moderate (3-5 mm in diameter) Large (6-10 mm in diameter)  hemodynamically insignificant  Least common group of  Develop CHF and FTT by  Between 80% and 85% of all children (3-5%) age 3-6 months VSDs  Without evidence of CHF or  Usually requires surgery.  All close spontanously pulmonary hypertension, may  Muscular close sooner than be followed until spontaneous membranous closure occurs Restrictive VSD NON- Restrictive VSD  Small lesion & Asymptomatic  Large lesion & Symptomatic  75% close spontaneously within fist 3 y of  Rarely close spontaneously. life.  1.0 cm² (Equal to or greater than to Ao valve  100 mmHg → critical. area. In most cases  and right ventricular  no symptoms heave are palpable in severe cases In severe cases:  In critical stenosis, cyanosis and gallop  exertional dyspnea, tachypnea, fatigue, and exertional rhythm develop. chest pain, may be symptoms of  No repeated chest infection  No lung congestion ▪ Investigations:  Chest X ray: Post-stenotic dilatation of pulmonary artery (in valvular type), oligemic lung fields ± enlarged, hypertrophy.  RV and RA  ECG: Rt ventricular hypertrophy  Echocardiography: important for diagnosis and measurement of pulmonary valve gradient → if pulmonary or infundibulum & the degree is mild , moderate or severe → according it decide if there will be intervention or just follow up.  Cardiac Catheterization Page17 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ ▪ Differential Diagnosis:  Other causes of ejection systolic murmur  Aortic stenosis  Coarctation of aorta  ASD  Pulmonary Hypertension ▪ Prognosis: Mild degree: compatible with long life Severe degree: Ht failure before middle age ▪ Treatment: Prophylactic: as VSD Standard ttt Medical: Propranolol therapy in some cases with  Follow up infundibular stenosis  Balloon Pulmonary Vulvoplasty Surgical: pulmonary valvotomy by Catheter Others: dilatation if pulmonary  Pulmonary valvotomy valve gradient > 60 mmHg is very successful and now considered standard treatment ] 5) Aortic stenosis ▪ Description: 1) Valvular Stenosis 2) Subvalvular Stenosis 3) Supravalvular Stenosis:  Ao valve is bicuspid and  fibrous ring around the left  narrowed ascending Ao cusps are fused at their ventricular outflow tract above the valve. edges → stenosis. ▪ Pathophysiology:  The left ventricular pressure AS: Aortic Stenosis increases to maintain cardiac output.  Left ventricular hypertrophy occurs. Page18 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ 1) Symptoms 2) Signs  In most cases: no symptoms.  Low systolic and pulse pressure  In severe cases: few can develop  Cardiomegaly with prominent left ventricular impulse Anginal pain Dizziness, fainting or  A is palpable at the loss of consciousness on effort and along  Some may present with the neck vessels. manifestations of congestive heart  Diminished and delayed aortic component of S2 failure Harsh over the aortic  Easy fatigue area (2nd right intercostal space) or aortic area (3rd  Syncope left intercostal space).  The murmur is transmitted upwards to the neck. ▪ Investigations: Chest X ray:  Poststenotic in Valvular stenosis  Left ventricular enlargement( ) may be present ECG:  left ventricular  Left ventricular ischemia indicates severe stenosis  Exercise ECG may be needed Echocardiography: can detect:  Valvular , subvalvular or supravalvular.  Left ventricular hypertrophy  Bicuspid valve, supravlavular or subaortic stenosis can be detected.  Poststenotic dilatation can be detected  Pressure gradient across the valve can be measured Cardiac Catheterization can detect:  Site of obstruction  Accurate pressure gradient measurement  Calculation of valve area ▪ Differential Diagnosis:  Other causes of ejection systolic murmur  Pulmonary stenosis  Coarctation of aorta  ASD  Pulmonary Hypertension Page19 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ ▪ Complications:  Infective endocarditis  Heart failure  Sudden death on vigorous exercise ▪ Treatment: Prophylactic : as VSD avoidance of competitive sports Follow up ≫ mild cases. If severe ≫ aortic balloon valvuplasty (> 50 mmHg) Medical: in cases of heart failure Surgical: aortic valvotomy for Valvular stenosis. Resection of subaortic membrane in subaortic stenosis. Others: Balloon dilatation in cardiac catheterization laboratory may be successful. 6) CoA: Coarctation of Aorta ▪ Description:  It is a narrowing of varying degrees of a segment of the aorta.  It is mostly (98%) located ▪ Pathophysiology:  Coarctation is a mechanical obstruction between the proximal and distal aorta.  The whereas that of  Collateral vessels including internal mammary and intercostal vessels develop in response to pressure difference. 1) Symptoms 2) Signs  In most cases no symptoms  Normal brachial pulses and  In some cases leg cramp, chest pain and headache.   Dizziness , HTN ≫ UPPER (hypertension if B.P. is measured in  Abdominal pain , intestinal ischemia upper limbs). ≫ LOWER  Collateral vessels may be visible or palpable around the  Heart failure may be the presentation scapula in 10% of cases.  Left ventricular hypertrophy with forcible apex beat Page20 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ  It occurs in the first few days of life in  over the aortic area severe cases. or aortic area.  It may present with a complication e.g.  The murmur is heard also in the bacterial endocarditis or rupture of intracranial aneurysm.  Heart failure may be the only presentation in neonatal period. ▪ Diagnosis:  Reversed B.P. gradient in limbs at any age + absent or weak femoral pulse. ▪ Investigations: Chest X ray:  Notch of aortic shadow at site of Coarctation and poststenotic dilatation of aorta Rib notching due to erosion by dilated intercostals  Notching of barium filled esophagus by dilated ECG:  May be normal or  Echocardiography  Left ventricular hypertrophy  Coarctation segment  Pressure gradient across coarctation can be measured  Other associated anomalies as bicuspid valve and mitral valve anomalies Cardiac Catheterization:  Site of coarctation  Accurate pressure gradient measurement  Adequacy of collateral vessels ▪ Differential Diagnosis:  CoA: Coarctation of Aorta  Other causes of hypertension  Other causes of heart failure in neonates  Other causes of ejection systolic murmur. ▪ Complication : Diagnosed as primary hypertension ≫ must exclude COA. ICT ≫ ( ICHge – convulsions – blurred vision- disturbance conscious level. Renal ischemia. Page21 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ ▪ Treatment: Prophylactic: as VSD ≫ from infective endocarditis. Medical ✓ Treatment of hypertension ✓ Treatment of heart failure Surgical ✓ Resection with end to end anastomosis or graft repair Others ✓ in cardiac catheterization laboratory is successful with varying results. ▪ Pulmonary area : LEFT ejection systole( PS) RIGHT ejection systole ( Aortic stenosis) Machinery murmur (PDA) Pulmonary HTN. 1) pulmonary HTN 2) pulmonary stenosis  BECAUSE of neglected : VSD – ASD -PDA.  Mostly Not associated with heart failure.  ssociated with heart failure.  If critical ≫ heart failure  ccentuated 2nd heart sound.  Accidently discovered.  jection systolic murmur  ( in severe  ullness on percussion on pulmonary area. cases≫ )  alpable thrill called diastolic shock ▪ Heart Failure Symptoms: cardia- pnea diomegaly Increase cardio-thoracic ratio > 50 % ▪ Lazix ≫ cause hypokalemia so give k+ or spironolactone. ▪ NB ( NOT IMPORTANT) :  After ECHO ≫ Perform Multicilica Angiography Of The Heart & Great Vessels : ✓ Show the heart & the vessels obviously ✓ Help in diagnosis. Page22 "‫ َوِزْدِني ِعْلًما‬،‫ َو َعِّلْمِني َما َيْنَفُعِني‬،‫"اللُهَّم ْانَفْعِني ِبَما َعَّلْمَتِني‬ َّ

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