Congenital Heart Diseases PDF

INTRODUCTION TO THE TOPIC  Cyanosis since birth. Criteria to  Murmur since birth.  No history of rheumatic fever. Suspect  Recurrent chest infection. Congenital  Hypertensive child. Heart  +ve family history...

INTRODUCTION TO THE TOPIC  Cyanosis since birth. Criteria to  Murmur since birth.  No history of rheumatic fever. Suspect  Recurrent chest infection. Congenital  Hypertensive child. Heart  +ve family history.  Associated congenital anomalies  Shunt with blood flow from high At birth:- pressure to low pressure.  Left to right flow →  Left side pressures >> Right side volume overload of right pressures heart  Left to right connection with: Left to  Normal blood flow to lungs Right flow:  No Cyanosis (oxygenation VSD (LV --- RV) of the blood is normal) ASD (LA --- > RA) Years later if untreated:- Shunts PDA (Aorta --- > Left pulmonary artery)  Pulmonary Hypertension  Right Ventricle Hypertrophy  ↑↑ Right Ventricle Pressure  Reversal of Shunts (R → L)  Late cyanosis (Eisenmenger Syndrome) Cyanotic Acyanotic  Fallots trilogy With RV enlargement:-  Fallots tetralogy ASD  Eisenmengers syndrome PS  Tricuspid atresia With LV enlargement:-  Transposition of great vessels AS Classification PDA Coarcation of aorta With biventricular enlargement:- VSD With no enlargement:- Dextrocardia  P.O.C Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD) Aortic Coarctation Pulmonary Stenosis  High ASD (ostium secundum):  Big membranous type: in  Narrowing of a part of  Valvular: the most the membranous part of the IVS. aorta common type (80 %) most  Small muscular type (Roger's usually  Subvalvular com disease): in muscular part of IVS, distal to (lnfundibular). mon. it's the left  Supravalvular: rare Anatomy  Low hemodynami subclavian artery. ASD cally  Associated anomalies: (ostium premium): may be insignificant Bicuspid aortic valve (AS, associated with Mitral valve & more than AR), PDA, VSD, Congenital disease (Lutembacher's 90% of aneurism of Circle of Willis, Syndrome) cases close spontaneously. Turner's.  LA pressure >> RA pressure  Pressure in LV is 120/0 mmHg.  INCREASED BP in the  PS → ↑ Resistance to  Blood Flow  Pressure in RV is 25/0 mmHg. upper half: symptoms of flow of blood to PA Shunted  So, the blood is shunted from LV to hypertension e.g., headache, (afterload) → RV from RV during systole only leading to: epistaxis... enlargment. LA→RA  Shunted blood to  DECREASED BP in the  Hypertrophy → RSHF  ↑Blood Flow the RV causes lower half: fatigue &  Low Cardiac Output to RV & RVE RVE blood flow to intermittent claudication of  ↑Blood flow pulmonary the LL. in pulmonary arteries (lung  Collaterals pain around left arteries “Lung Plethora”. plethora & shoulder. Haemodynamics  Pulmonary HTN pulmonary  ↓↓ LA to LV so↓COP. hypertension)  So, in ASD there are : blood flow to LA Lung plethora. to LV causing LVE Low COP. (later failure) Right ventricle hypertrophy.  Blood passing from LV to aorta will be less than normal LCOP.  So, in VSD there are: Lung plethora. LCOP. Biventricular enlargement.  Symptoms: Symptoms: Symptoms: Symptoms:  Asymptomatic in mild  Asymptomatic in mild cases & in  Asymptomatic in mild cases.  Asymptomatic in mild cases/early life. Roger's disease.  of hemodynamics. cases.  Symptoms of haemodynamics :  of hemodynamics: lung plethora &  of complications.  LCOP. Lung plethora : Exertional LCOP.  of other anomalies e.g. AS,  RSHF. dyspnea, recurrent chest  of complications. AR, PDA… Signs: infection.  of other anomalies. Signs:  General: LCOP, RSHF. Low COP symptoms Signs:  No signs in mild cases.   Anomalies Association:  No signs in mild cases.  of hemodynamics: Clinical Picture Secundum ASD may be  of hemodynamics: Lung plethora & INC. BP in arms: prominent associated with tri-phalangeal LCOP. carotid pulsation. thumb & radial  of complications. DEC. BP in legs: weak abnormalities.  Neck vein: Giant (a) wave. pulsations of LL e.g. dorsalis pedis. Collaterals seen in interscapular area (Suzman's sign).  of complications.  of other anomalies.  RV Hypertrophy.  Biventricular enlargement with  LV hypertrophy.  RV Hypertrophy. Auscultation: hyperdynamic apex. Auscultation:  Giant (a) wave.  Accentuated, wide fixed Auscultation:  Accentuated S2 Auscultation: splitting of S2.  S2: Accentuated pulmonary Murmurs: Ejection systolic  S2: weak pulmonary Murmur: component, wide splitting. murmur due to: part, wide splitting.  No murmur of ASD itself Murmur:  Coarctation itself (below Murmur: because of low pressure  Harsh pansystolic murmur with left infraclavicular area),  Ejection systolic Cardiac gradients between the 2 atria. thrill over 3rd, 4th intercostal Associated AS, murmur on pulmonary Examination  Murmur of relative TS & PS spaces. Hypertension. area:↑blood flow may be heard.  Ejection systolic murmur of  Early diastolic murmur due across the valve. Additional sounds: pulmonary hypertension. to associated AR. Additional sounds:  Ventricular Gallop “S3” due to  Sub-aortic VSD: associated with  Continuous murmur over the  Ejection click RSHF. AR. collaterals. “stenosis” in valvular  Mid diastolic murmur of relative MS type, S4 on tricuspid (blood flow across the mitral valve). area.   Right-sided heart failure.  Heart failure  Complications of  Right-sided heart  Paradoxical embolism e.g.  Paradoxical embolism hypertension e.g. cerebral failure. stroke  Eisenmenger's syndrome: usually at hemorrhage...  Infective  Eisenmenger's syndrome: 2nd - 3rd decade  Heart failure. endocarditis. Complications cyanosis  Infective endocarditis  Infective endocarditis.  Infective endocarditis: rare due to low pressure gradient  Arrhythmia: AF  X-ray: RVE, dilated pulmonary  X-ray: Biventricular enlargement,  X-ray: LVE, Rosler's sign:  X-ray: RVE , Lung artery lung plethora. Rib notching (3-8) due to oligemia.  ECG: RBBB in most cases  ECG: Biventricular enlargement. erosion by collaterals.  ECG  Echo: RVE, itself  Echo: Biventricular enlargement,  ECG: LVE.  Echo: Diagnostic.  Catheterization : diagnosis of anomaly.  Echo: LVE, can detect the  Catheterization: Investigations Detect the defect: may pass  Catheterization : coarctation. Diagnostic: through ASD. Detect the defect: may pass through  Catheterization & Detects the pressure ↑ Pressure in RA, RV, PA VSD. aortography: can detect site gradient across the ↑ O2 saturation in RA, RV, PA Pressure in the RV & PA. & severity of coarctation. pulmonary valve: if >50: O2 level in RV in comparison to RA. severe PS  Prophylaxis against IE.  Medical: prophylaxis against  Prophylaxis against  Treatment of complications. IE & treatment of infective endocarditis  Surgical closure of large defect. complications.  Treatment of RSHF  Surgical repair: in early  Surgical: in severe PS Treatment childhood to avoid Valvular type: valvotomy persistent hypertension. or replacement. Subvalvular type: resection of infundibulum  P.O.C Patent Ductus Artertiosus (PDA) Eisenmenger Syndrome Anatomy  Persistence of ductus arteriosus between the left  A condition in which a left-to-right shunt in pulmonary artery & the aorta just distal to the left the heart causes pulmonary HPT, which in subclavian artery. turn, causes increased pressure in the right  PDA is normal during fetal life side of the heart and reversal of the shunt  Shunts Blood from LT PA → Aorta into a right-to-left shunt.  Normally it closes & ligamentum arteriosum.  Etiology :  Common in premature babies, particularly female VSD, PDA, ASD infants.  Aortic pressure > pulmonary pressure in both systole & diastole.  Blood is shunted from aorta to PA in both systole & diastole  ↑Blood flow of pulmonary arteries (lung plethora)  ↑Blood flow in LA & LV causing LVE (later failure)  ↑Blood flow to the aorta in systole causing high COP & Haemodynamics high systolic BP.  Escape of blood from the aorta to the PA in diastole causes low diastolic BP.  Hyperdynamic circulation & widened pulse pressure.  Pulmonary HTN & Shunt Reversal (Eisenmenger's) Symptoms:  History of congenital heart disease: VSD,  Asymptomatic in mild cases. PDA, ASD.  of hemodynamics.  Pulmonary infection & hemoptysis.  of lung plethora.  C/P of pulmonary hypertension.  of hyperdynamic circulation: palpitation & general throbbing & widened pulse  Decrease of the original murmur of the shunt pressure. due to low pressure gradient. Clinical Picture Signs:  No signs in mild cases.  of hemodynamics.  of lung plethora.  of hyperdynamic circulation. Neck vein : Giant (a) wave due to pulmonary hypertension   LV hypertrophy.  Continuous thrill over left infraclavicular area (site of DA). Cardiac Auscultation: Examination  S2: Accentuated, reversed splitting S2 Murmur:  Continuous "machinery" murmur over left infraclavicular area  Left-sided heart failure  Paradoxical embolism e.g. stroke. Complications  Eisenmenger' s syndrome: differential cyanosis (cyanosis only in LL).  Infective endocarditis.  Blue toes, normal fingers  X-ray: Dilatation of aorta, PA, LA & LV, lung plethora.  ECG: Left Ventricular Enlargement.  Echo: show chamber dilatation. Investigations  Catheterization: Detect the defect: ↑ Lt Pulmonary Artery pressure. ↑ O2 level in PA, Normal in RA, RV  Medical:  Prevention is best. Prophylaxis against infective endocarditis.  Closure of the defect is contraindicated as Treatment of complications it increases the pressure in the right side of Treatment Medical closure of the duct: indomethacin (NSAIDS) the heart.  Surgical: closure of the duct.  Symptomatic treatment  Heart lung transplantation. Tetralogy of Fallot (F4): Dextrocardia: deviation of the heart to the right, it may Anatomy: Slight deviation of the upper be congenital or acquired. part of interventricular  Situs inversus totalis: mirror like transposition of the Triology of fallot (F3): heart & all other viscera. septum to the right leading to: PS ( valvular)  Isolated dextrocardia: mirror like transposition of the  PS ( subvalvular) Marked RVE. heart only.  Mild RVE. ASD  Dextroversion: heart is displaced to the right (RV  VSD (not significant) remains to the right &LV to the left)  Overriding of  Acquired dextrocardia: acquired displacement of the Aorta heart to the right e.g. fibrosis