Cyanotic Congenital Heart Diseases

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Congenital Heart Diseases Peads & Adults:

Congenital Heart Diseases Peads & Adults By : ahmad sukri nawi @ matyiestenia gravis


FETAL CIRCULATION Oxygenated blood enters the umbilical vein from the placenta Enters ductus venosus Passes through inferior venacava Enters the right atrium Enters the foramen ovale Goes to the left atrium Passes through left ventricle Flows to ascending aorta to supply nourishment to the brain and upper extremeties Enters superior vena cava Goes to right atrium Enters the right ventricle Enters pulmonary artery with some blood going to the lungs to supply oxygen and nourishment Flows to ductus arteriosus Enters descending aorta ( some blood going to the lower extremeties ) Enters hypogastric arteries Goes back to the placenta Special Structures in Fetal Circulation Placenta – Where gas exchange takes place during fetal life Umbilical Arteries – Carry unoxygenated blood from the fetus to placenta Umbilical Vein – Brings oxygenated blood coming from the placenta to the fetus Foramen Ovale – Connects the left and right atrium. It pushes blood from the right atrium to the left atrium so that blood can be supplied to brain, heart and kidney Ductus Venosus - Carry oxygenated blood from umbilical vein to inferior venacava , bypassing fetal liver Ductus Arteriosus - Carry oxygenated blood from pulmonary artery to aorta, bypassing fetal lungs.

Changes post-natal:

Changes post-natal First inspiration  increase alveolar oxygen tension causes vasodilation of pulmonary artery  reduced resistance toward blood from RV  drops in pulmonary pressure Increase in left atrial pressure cause closure of foramen ovale  fossa ovalis D.t increase in systemic pressure n lowered pulmonary pressure plus reduction in circulating prostaglandin  DA close  ligamentum arteriosum Obstretric clumping cause umbilical vein to become Lig.Teres Hepatis whereas umbilical artery bcomes medial umbilical ligament Ductus venosus becomes ligamentum venosum

Changes !!:

Changes !! Fetal Foramen ovale Ductus Arteriosus Ductus Venosum Umbilical Vein Umbilical arteries Oxygenation by placenta Post-natal Fossa ovalis Ligamentum arteriosum Ligamentum venosum LTH MULs Oxygenation by lung

Congenital :

Congenital Shunting of blood : VSD Obstructive : atresia , coarctation of aorta

Abnormal connection:

Abnormal connection Shunt : abnormal connection between cavities  allow flow between chambers depending on the pressure difference Left to Right Shunt : systolic pressure > pulmonary / right heart pressure for ex : ASD , VSD … Right to left shunt : when pulmonary pressure > left heart : Eisenmenger ASD , VSD , PDA


ASD 3 types : ostium primum , ostium secundum ( most common ) and sinus venosus Pathophysiology : left atrial p > right atrial pressure -  increase flow to right heart and pulmonary circulation  compensatory RVH Symptoms : asymptomatic ..shows in adult Signs : pink , normal BP , LSE heave ( RVH ) , SPLITTING S2 in all phases of respiration ( fixed ) , ESM at 2 nd left intercostal ( pulmonary area ) ECG : RVH ( right axis deviation ) and peaked P wave ( right atrial hypertrophy ) CXR : normal or slight cardiomegaly , increase pulmonary vessels marking ( plethoric lung field ) Cx : pulmonary HPT , HF , IE ( rare )




VSD severity depends on area of defects : small / large Two types : membranous ( 75 % ) and muscular ( 25 % ) Small VSD : can be either types …asymptomatic coz not LTR not really severe. Signs : normal except for PSM heard at LSE with thrill.ECG and CXR are both normal Large : symptoms of heart failure , recurrent LRTI , slow weight gain , failure to thrive ..signs : large volume pulse ( significant LTR shunt ) , LSE heave , displaced forceful apex beat , loud P2 , S3 in CCF , PSM all over precordium with LSE thrill.. mayb no murmur ( too large ) ..ECG : RVH and LVH…CXR : plethoric lung field and , cardiomegaly and signs of heart failure Cx : Pulmonary HPT , CCF , bacterial endocarditis , LRTI , failure to thrive , Eisenmenger Syndrome ( stunt reversal with cyanosis )




PDA Ductus arteriosus – connect aorta and pulmonary artery If fails to close  hi pressure blood from aorta  pulmonary artery  pulmonary artery vasoconstriction and hypertrophy  increase resistance  pulmonary hypertension Severity of clinical features depends on the shunt ( diameter, length ) and pulmonary vascular resistance Symptoms : small no symptoms.large defects  signs of CCF and recurrent LRTI Signs : bounding pulse , widened pulse pressure ( runoff blood to pulmonary circulation in diastolic phase ) , continous machinery murmur at left infraclavicular space , thrill mayb felt , LSE heave , loud P2 Imaging : CXR ( plethoric lung field with increase pulmonary artery marking ) , RVH ( pulmonary hypertension ) , LVH ( CTR > 0.5 ) … ECG : RVH , LVH Complications : increase risk for SBE , CCF , LRTI



Atrioventricular defects:

Atrioventricular defects Same as VSD and ASD – higher pressure blood from LH  RH  increase pulmonary blood flow  increase pulmonary blood resistance  pulmonary hypertension CF d.t pulmonary hypertension and also CCF + poor growth a/w Down Syndrome Signs : d.t ASD , VSD Imaging : increase pulmonary vascularity with cardiomegaly , LVH and left axis deviation on CXR and ECG

Pulmonary stenosis:

Pulmonary stenosis pulmonary valve – allows one way flow from right ventricle to pulmonary artery ( pulmonary circulation ) Stenosis : subvalvular , valvular , supravalvular Stenosis  reduced pulmonary blood flow  compensatory RVH  Mild asymptomatic , severe  exertional dyspnea and easy fatigability Signs : ESM at pulmonary area radiates to the back.LSE heave and thrill.splitting of s2 with loud P2 Ecg : RVH CXR – uplifted RVH , poststenotic dilatation Echo : valve area , pressure gradient Management : antibiotic prophylaxis against SBE , ballon valvuloplasty

Aortic stenosis:

Aortic stenosis Either valvular , supravalvular or subvalvular Same manifestation in adult with ESM at 2 nd right intercostal space which radiates to the neck Systolic ejection click mayb heard A2 mayb decreased in intensity d.t stiff valve Ix : ECG , CXR , Echo ( 3 cardinal test for valve stenotic dilatation ) Management : ballon valvuloplasty ,prophylaxis antibiotics for SBE

Coarctation of aorta:

Coarctation of aorta 2 types : infantile, adult Location of coarctation : pre- ductal / post- ductal ( juxtaductal ) a/w Turner Syndrome ( 25 % ) , congenital bicuspid valve ( 75 % ) , ASD , VSD Classical COA : just distal to DA

Coarctation Of Aorta:

Coarctation Of Aorta


pathology Constricted aorta  increase resistance to blood ejected from left ventricle  LV has to work harder  compensatory hypertrophy Blood flow thru descending which is the major feeding artery of lower peripheries reduced Pathology starts after closure of DA – 15 -24 hrs after birth ( must know why closure happens la ) Backflow to left atrium  may or may not open foramen ovale  Left to Right shunt if yes ! / backflow to pulmonary circulation  right heart failure Most of the time , CCF occurs As a respond , collateral circulation may develop to supply blood to descending aorta by axillary and internal mammary arteries tru intercostal arteries If there is VSD ( worsen LRS ) , left ventricle obstructive lesions greatly increase LV workload ( worsen CCF ) ,


Cont… Why hypertension – mechanical blocakge theory ( pump harder ! ) and humoral response theory ( RAAS activation d.t reduced RBF ) RAAS activation theory is more accepted Hypertension is irreversible if prompt corrective surgery is not done  damage to kidney

Clinical Manifestation:

Clinical Manifestation Early CCF , late hypertension ( long term effect ) Early : symptoms of CCF ..worsened if got VSD too..depends on whether DA has closed or not , pulmonary vascular resistance , Late : hypertension.often asymptomatic.symptoms may include headaches , nosebleeds, claudication and cool lower extremities ( logical thinking helps ! ) Signs : upper limb HPT , radiofemoral delay , weak peripheral pulses ( femoral , popliteal , posterior tibialis , dorsalis pedis ) PE : midlate systolic murmur at upper chest / back or vascular bruits d.t collateral circulations


differentials Anything which can cause HPT / pressure overload Aortic stenosis , HoCM , hypertension


Ix Baselines – every patients admitted should have these Serum lipids and RBS – risk factors assesment for CVD CXR – early ( cardiomegaly and features of CCF ) , late ( “3” sign on CXR ) Echo : coarctation and other associated anomalies such as VSD ( Echo visualize intracardiac anomalies ) ECG – LVH ( LAD )

Classical “3” sign of Coarctation of Aorta:

Classical “3” sign of Coarctation of Aorta Red arrows : Rib notching ( hypertrophy of intercostal arteries ) Yellow arrow : aortic knucle Blue arrow : coarctation site Green arrow : post- stenosis dilatation

Aorta angiography:

Aorta angiography Angiography shows coarctation of aorta with post- stenosis dilatation of descending aorta


management Prostaglandin E2 – to keep the DA patent n relieve the pressure by shunting blood to pulmonary artery  descending aorta Ballon angioplasty – open up Corrective surgery

Cyanotic Congenital Heart Disease:

Cyanotic Congenital Heart Disease Basis : coz obstruction of blood to lung for proper reoxygenation All T ! TOF , TGA , Truncus Arteriosus , Tricuspid Stenosis , Total Anomalous Pulmonary Venous Return

Tetralogy Of Fallot:

Tetralogy Of Fallot Tetralogy : 4 pathologies PROV : pulmonary stenosis , right ventricular hypertrophy , overriding aorta , and VSD Epidemiology : no sex or race predilection.symptomatic at birth..if pulmonary stenosis is not correted by surgery , 10 % will not survive until adulthood

Clinical features:

Clinical features Severity depends on RV obstruction ( stenosis of pulmonary valve ) Cyanosis after birth – d.t closure of DA  obstruction to lung  can’t be oxygenated  compensatory RVH  RL shunt thru VSD caused mixture  overriding aorta In some cases , collateral circulation happens between aorta and pulmonary artery ( aortopulmonary circulation )  but this in long term can cause pulmonary HPT d.t overload


symptoms Fallot’s Spell ( Hypercyanotic Spell ) : deep cyanosis, SOB , syncope on exertion Squatting – increase systemic vascular resistance ( kinking of femoral arteries ) and decreasing blood flow thru overriding the same time , this position increase venous return , n improving blood flow thru low pressure pulmonary circulation


signs First few days mayb OK ! – PDA may not close completely yet May have bounding pulse – increase in pulmonary blood flow  large CO Auscultation reveals a normal first heart sound with a single second heart sound. An ejection systolic murmur may be present at the left lower sternal border. The typical right ventricular outflow tract murmur of classic tetralogy of Fallot is not heard. A soft continuous murmur from the DA may occur at the left base. A continuous murmur from the aortopulmonary collaterals may be heard in the back. Polychytemia  redness of palm Stunted growth n development delay – growth chart


complications CCF Failure To Thrive – d.t CCF Pulmonary hypertension – with collaterals Polychythemia d.t chronic hypoxaemia ( same in COPD ) Polychythemia leads to hyperviscosity  thrombosis endocarditis


DDx Pulmonary Atresia With Intact Ventricular Septum Total Anomalous Pulmonary Venous Connection Transposition of the Great Arteries Tricuspid Atresia

Investigation :

Investigation CBC – Hb level and Hematocrit ( polycythemia ) CXR – RVH ( boot-shape heart ) , BVH in cardiac failure in untreated infant , pulmonary artery vascularity depends on whether there is aortopulmonary collaterals or not and degree of stenosis ECG – RVH Echo – RVH , pulmonary atresia ( narrowing )

Isolated RVH in TOF with pulmonary stenosis:

Isolated RVH in TOF with pulmonary stenosis BOOT –SHAPED HEART d.t RVH

T & M:

T & M Immediate infusion of PGE1 ( prostaglandin ) to maintain DA patency so that time can b taken to make a diagnosis Treat CCF Palliative systemic-pulmonary shunt Corrective surgery – VSD , and to connect pulmonary artery and RV Diet : hi calorie for CCF to prevent stunted growth Activity : must get involve only in mild physical activities ( to prevent fallot spell )


TGA The aorta arises from the right ventricle and the pulmonary artery from the left. So , deoxygenated blood from right ventricle to b pumped out to the whole body whereas hi o2 blood from lung to b pumped back to lung Pulmonary n systemic are not connected to each other unless mixing occurs ( ASD , VSD , PDA ) If untreated, most children die within the first months of life Common in : child with diabetic mother and male ( 70 % ) racial predilection


CF Depends on mixing of blood Symptoms : cyanosis hours after birth Intact – not compatible With VSD – CCF VSD with LV outflow obstruction – TOF VSD with pulmonary obstruction

Truncus Arteriosus:

Truncus Arteriosus Truncus arteriosus – forms pulmonary artery and aorta Failure  single trunk receives blood from both ventricles

Truncus Arteriosus:

Truncus Arteriosus

Tricuspid atresia:

Tricuspid atresia Failure to form lumen  tricuspid stenosis  obstruction of blood from right atrium to RV  reduced oxygenation of blood in the lung  cyanosis


CF Cyanosis depends on the amount of pulmonary flow If untreated can cause CCF Signs : tachypnoea , single S2 ( single truncal valve ) , systolic ejection click , and systolic murmur at LSE Ix : ECG ( BVH ) , CXR ( increase pulmonary marking )


TAPVC Failure of pulmonary vein to connect to left atrium and with shunting to systemic vein Depending on atrial level communication for survival and systemic cardiac output Clinical manifestation : depends on presence / absence of obstruction to pulmonary venous drainage ( if got obstruction , worsen the prognosis with severe cyanosis )


Pathophysio Oxygenated Pulmonary blood flow  right heart  overload to RA and RV  RVH and RAH Hypoplastic LV and dilation of pulmonary trunk Deep cyanosis d.t mixture of oxygenated blood and deoxygenated blood

Hypoplastic left heart syndrome:

Hypoplastic left heart syndrome

Slide 47:

Failure to develop aortic and mitral valve and small LV  unable to maintain systemic circulation Dependant on the right to left shunt at atrial level ( PDA ) After birth ( 15 hrs ) , DA closed n symptoms of CCF come in .. Most common cause of cardiac defects which cause death in the 1 st month of life

Complications of Congenital Heart Diseases:

Complications of Congenital Heart Diseases Cardiac : Bacterial endocarditis ( SBE ) Extracardiac : stunted growth , depression, complicated pregnancy ( d.t poor placental perfusion ) , anemia ( poor feeding ) , polycythemia ( chronic hypoxia ) , CNS abscess ( RL shunt ) , LRTI , trombotic stroke ( polycythemia )

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