Adult Congenital Heart Disease PDF

CONGENITAL HEART DISEASE  Content Definition & Sources Clinical presentation Investigations Treatment  CRITERIA TO SUSPECT CONGENITAL heart Cyanosis since birth. +ve family history. Murmur since birth. Associated congenital an...

CONGENITAL HEART DISEASE  Content Definition & Sources Clinical presentation Investigations Treatment  CRITERIA TO SUSPECT CONGENITAL heart Cyanosis since birth. +ve family history. Murmur since birth. Associated congenital anomalies No history of rheumatic fever. Recurrent chest infection. Hypertensive child.  shunts Left side pressures >> Right side pressures Shunt with blood flow from high pressure to low pressure Left to right connection with Left to right flow VSD (LV --- RV) ASD (LA --- > RA) PDA (Aorta --- > Left pulm. artery  At birth Years later if untreated: Left to right flow → volume Pulmonary Hypertension overload of right heart Right Ventricle Hypertrophy Normal blood flow to lungs ↑↑ Right Ventricle Pressure No Cyanosis (Oxygenation of the Reversal of Shunts (R → L) blood is normal) Late Cyanosis (Eisenmenger syndrome Classification cyanotic Acyanotic Fallotus With RV triology enlargment As Fallots PS tetralogy With LV enlargment AS Eisenmengers PDA syndrom Coarcation of aorta With biventricular enlargment Tricuspid VSD atresia With no Transposition enlargment of great dextrocardia vesseles  Atrial Septal Defect (ASD) Anatomy : ► High ASD ( ostium secundum): the most common ► Low ASD ( ostium premium) : may be associated with Mitral valve disease ( Lutembacher's syndrom) ostium secundum ostium premium Normal  Hemodynamics : LA pressure > RA pressure Blood Flow Shunted from RA → LA ↑ Blood Flow to RV & Right Ventricle Enlargement ↑ Blood Flow in Pulmonary arteries “Lung Plethora” ism Pulmonary HTN ↓↓ LA to LV so ↓ Cardiac output. So, in ASD there are : Lung plethora. Low COP. Right ventricle hypertrophy clinical picture : Symptoms: 1- Asymptomatic in mild cases or in early life. 2- Symptoms of hemodynamics : ► lung plethora : Exertional dyspnea, recurrent chest infection. ► Low COP symptoms. Congenital Anomalies Association : Secundum ASD may be associated with tri-phalangeal thumb & radial abnormalities.  Cardiac examination 1. Right Ventricle Hypertrophy 2. Auscultation : Accentuated , wide fixed splitting of S2 3. Murmur : No murmur of ASD itself because of low pressure gradients () the 2 atria. Murmur of relative TS & PS may be heard. 4. Additional sounds:. Ventricular Gallop “S3” due to RSHF  Complication 1- RSHF. 2- Paradoxical embolism e.g. stroke. 3- Eisenmenger's syndrome: cyanosis 4- Infective endocarditis : rare due to low pressure gradient. 5- Arrhythmia : AF  Investigation X-ray: RVE, dilated pulmonary artery -ECG : RBBB in most cases - Echo : RVE , itself - Catheterization : Detect the defect : the catheter may pass through ASD. ↑ Pressure in RA, RV, PA ↑ O2 saturation in RA, RV, PA  ventricular septal defect (VSD) Anatomy: There is an abnormal opening between the two VENTRICLE , producing left to right shunt. There are 2 types: 1- Big membranous type occurs in the membranous part of the interventricular septum. 2- Small muscular type (Roger's disease) occurs in muscular part of interventricular septum, it's hemodynamically insignificant & more than 90% of cases close spontaneously.  HEMODYNAMIC The pressure in LV is 120 / 0 mmHg. The pressure in RV is 25 / 0 mm Hg. So, the blood is shunted from LV to RV during systole only leading to : The shunted blood to the RV causes RVE blood flow to pulmonary arteries (lung plethora & pulmonary hypertension) blood flow to LA to LV causing LVE (later failure) Notice that blood passing from LV to aorta will be less than normal LCOP. So, in VSD there are: Lung plethora. LCOP Biventricular enlargement Symptoms: Asymptomatic in mild cases & in Roger's disease. Symptoms of hemodynamics : Symptoms of lung plethora : Exertional dyspnea, recurrent chest infection... Symptoms of LCOP. Symptoms of the complications. Symptoms of other congenital anomalies. Signs: No signs in mild cases. Signs of hemodynamics: Lung plethora & LCOP. Signs of complications. Neck vein : Giant (a) wave Cardiac examination Biventricular enlargement with hyperdynamic apex. Auscultation : S2 : Accentuated pulmonary component, wide splitting. Murmur: Harsh pansystolic murmur with thrill over the 3rd, 4th intercostal spaces. Ejection systolic murmur of pulmonary hypertension. Sub-aortic VSD may be associated with AR Mid diastolic murmur of relative MS ( blood flow across the mitral valve)  COMPLICATION HF Infective endocarditis. Paradoxical embolism. Eisenmenger's syndrome : usually at 2nd - 3rd decade.  INVESTIGATION X ray : Biventricular enlargement, lung plethora. ECG : Biventricular enlargement. Echo : Biventricular enlargement, diagnosis of anomaly. Catheterization : Detect the defect : the catheter may pass through VSD. pressure in the RV & PA. O2 level in RV in comparison to RA.  TREATMENT Prophylaxis against IE & treatment of complications. Surgical closure of large defect AORTIC COARCITATION Anatomy: Congenital narrowing of a part of aorta usually distal to the left subclavian artery. Associated congenital anomalies Bicuspid aortic valve (AS, AR), PDA, VSD, Congenital aneurism of Circle of Willis, Turner's Symptoms Asymptomatic in mild cases. Symptoms of hemodynamics : BP in the upper half Symptoms of hypertension e.g., headache, epistaxis... BP in the lower half Fatigue & Intermittent claudication of the LL. Collaterals Pain around left shoulder. Symptoms of complications. Symptoms of other congenital anomalies e.g. AS, AR, PDA.... Signs No signs in mild cases. Signs of hemodynamics : BP in arms, prominent carotid pulsation. BP in legs, weak pulsations of LL e.g. dorsalis pedis. Collaterals may be seen in interscapular area (Suzman's sign ). Symptoms of complications. Symptoms of other congenital anomalies Cardiac examination. LV hypertrophy. Auscultation : Accentuated S2 Murmurs: Ejection systolic murmur due to : Coarctation itself ( below left infraclavicular area), Associated AS ,Hypertension. Early diastolic murmur due to associated AR. Continuous murmur over the collaterals. Complication Complications of hypertension e.g. cerebral hemorrhage..... Heart failure. Infective endocarditis. Investigation X ray: LVE. Rosler's sign : Rib notching (3-8) due to erosion by collaterals. ECG: LVE. Echo: LVE, can detect the coarctation. Catheterization & aortography : can detect the site & severity of the coarctation. Treatment Medical: prophylaxis against IE & treatment of the complications. Surgical repair : in early childhood to avoid persistent hypertension. Pulmonary stenosis Valvular : the most common type (80 % ) Subvalvular ( lnfundibular ). Supravalvular: rare Hemodynamic PS → ↑ Resistance to flow of blood to Pa (afterload) → Rt Ventricle Hypertrophy → RSHF Low Cardiac Output Clinical picture:- Symptoms: Asymptomatic in mild cases. LCOP, RSHF. Signs General : LCOP. RSHF. Cardiac: Giant (a)wave. Rt Ventricle Hypertrophy. Auscultation : - S2 : weak pulmonary part of S2 with wide splitting. - Additional sounds : -Ejection click “Stenosis” in valvular type, S4 on tricuspid area. - Murmur : ejection systolic murmur on pulmonary area. “↑↑ Blood flow across the valve  Complications:- RSHF. Infective endocarditis Investigations :- X ray : RVE , Lung oligemia. ECG Echo : Diagnostic. Catheterization: Diagnostic. detects the pressure gradient across the pulmonary valve : if >50 →severe PS Treatment Prophylaxis against infective endocarditis Treatment of RSHF Surgical : in severe PS - Valvular type : valvotomy or replacement. - Subvalvular type : resection of infundibulum Patent ductus artertiosus (PDA) Persistence of ductus arteriosus between the left pulmonary artery & the aorta just distal to the left subclavian artery. PDA is normal during fetal life Shunts Blood from LT PA → Aorta Normally it closes & ligamentum arteriosum PDA is common in premature babies ,particularly female infants. HEMODYNAMIC The aortic pressure > pulmonary pressure in both systole & diastole. so the 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 & 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) CP Symptoms: Asymptomatic in mild cases. Symptoms of hemodynamics : Symptoms of lung plethora. Symptoms of hyperdynamic circulation: palpitation & general throbbing & widened pulse pressure Signs: No signs in mild cases. Signs of hemodynamics : Signs of lung plethora. Signs of hyperdynamic circulation. Neck vein : Giant (a) wave due to pulmonary hypertension Cardiac examination: LVE. Continuous thrill over left infraclavicular area ( site of DA). Auscultation : S2 : Accentuated, reversed splitting S2 Murmur : Continuous "machinery" murmur over left infraclavicular area Complication LSHF. Paradoxical embolism e.g. stroke. Infective endocarditis. Eisenmenger' s syndrome - differential cyanosis ( cyanosis only in LL ) Blue toes, normal fingers Investigation X ray: Dilatation of aorta, PA, LA & LV. Lung plethora. ECG: Left Ventricular Enlargement. Echo: show chamber dilatation. Catheterization: Detect the defect : ↑ Lt Pulmonary Artery pressure. ↑ O2 level in PA. , Normal RA , RV Medical: Prophylaxis against infective endocarditis. Treatment of complications Medical closure of the duct: indomethacin. (NSAIDS) Surgical: Closure of the duct Tetralogy of Fallot ( F4) Ana tomy: Slight deviation of the upper part of interventricular septum to the right leading to: 1- PS ( subvalvular) 2- Mild RVE. 3- VSD ( not significant ) 4- Overriding of Aorta Triology of fallot (F3) PS ( valvular) 2. Marked RVE. 3. ASD Isenmenger syndrome Definition : It is a condition in which a left-to-right shunt in the heart causes pulmonary hypertension, which in turn ,causes increased pressure in the right side of the heart and reversal of the shunt into a right-to-left shunt. Etiology : VSD. PDA. ASD clinical picture 1- History of congenital heart disease : VSD, PDA, ASD. 2- Pulmonary infection & hemoptysis. 3- C/P of pulmonary hypertension. 4- Decrease of the original murmur of the shunt due to low pressure gradient  Treatment : Prevention is best. Closure of the defect is contraindicated as it increases the pressure in the right side of the heart. Symptomatic treatment Heart lung transplantation Dextrocardia Deviation of the heart to the right, it may be congenital or acquired. 1► Situs inversus totalis: mirror like transposition of the heart & all other viscera. 2► Isolated dextrocardia : mirror like transposition of the heart only. 3► Dextroversion: the heart is displaced to the right (RV remains to the right &LV to the left) 4► Acquired dextrocardia: acquired displacement of the heart to the right e.g. fibrosis