4 - Familial congenital heart - A family history of a cardiac or noncardiac defect in either a parent or a preceding sibling is a major risk factor- Familial congenital heartdefects are often concordant by phenotype and developmentalmechanism
5 - A search for risk factors such as gestational diabetes mellitus Genotype-phenotype correlation:- Careful family history of first- and second-degree relatives, including detailed analysis of pregnancy loss, racial origin, and consanguinity- A search for risk factors such as gestational diabetes mellitus
10 Murmurless Congenital Heart Diseases a) Cyanotic diseases- - Transposition of great arteries- Pulmonary atresia with intact septum - total anomalies of pulmonary venous drainage- Tricuspid atresia with or without pulmonary atresia
11 - Coronary artery originating from pulmonary Artery. b) Acyanotic diseases- Core triatriatum- Severe coarctation- Coronary artery originating from pulmonary Artery.- Endocardial fibroelastosis
12 c) Others - Hypoplastic left heart syndrome - Single ventricle without obstruction.- Hypoplastic left heart syndrome
13 * Duct dependent lesions - These are lesions presenting very early in life that are dependent on ductal patency for survival These lesions fall into two main categories
14 - Pulmonary atresia with intact septum pulmonary atresia with VSD- A) Cyanotic lesions in which pulmonary blood flow is almost or totally dependent on the ductSevere tetrallogy of fallot-- Pulmonary atresia with intact septumpulmonary atresia with VSD-Critical pulmonary stenosis-
15 B) Acyanotic lesions Aortic atresia. - Critical aortic stenosis- Iterrupted aortic arch -- Severe pre-or juxtaductal Coarctation -
16 Clinical information (cyanosis) The radiographic classification of CHD relies on:Clinical information (cyanosis)2- Plain film information (increased pulmonary vascularity, decreased pulmonary vascularity (
17 I ) Acyanotic CHD with increased pulmonary vascularity. Common denominator of these lesions is that there is a L-R shunt where pulmonary flow is greater than true aortic flow;
18 3- Patent ductus arteriosus The shunt can be located in:1- Ventricular septal defect.2- Atrial septal defect.3- Patent ductus arteriosus
20 II) Acyanotic CHD with normal pulmonary vascularity Normal pulmonary vascularity is associated with either outflowobstruction or valvular insufficiency:
21 B) Valvular insufficiency: A) Outflow obstruction:- Coarctation of aorta- Interruption of aortic arch- Aortic stenosis- Pulmonary stenosis / insufficiencyB) Valvular insufficiency:- Aortic insufficiency- Pulmonary insufficiency
22 III) Cyanotic CHD with decreased pulmonary vascularity - Common denominator of these lesions is that there is a decreased pulmonary vascularity due to obstruction of pulmonary blood flow.- In addition, there is an intracardiac defect through which blood is shunted away from the lungs causing cyanosis.
23 * Types: Normal heart size - Tetralogy of Fallot - Fallot variants ( trilogy, pulmonary atresia / pseudotruncus I/II)
24 ddx: 1 - Uhl's disease (RV myocardium absent with tricuspid atresia) Increased heart size- Ebstein's anomaly (largest heart in CHD)- Tricuspid atresia ddx: 1 - Uhl's disease (RV myocardium absent with tricuspid atresia)2-Pulmonary stenosis with ASD
25 IV) Cyanotic CHD with increased pulmonary vascularity (admixture lesions) - Common denominator of these lesions is that there is an "admixture" of systemic and pulmonary venous blood (bidirectional shunting.-
26 - Clinical symtoms: CHF, cyanosis, recurrent pneumonia, growth retardation. - The admixture of venous blood may occur at the level of:
27 * Large veins: * Large arteries: - Truncus arteriosus - Total anomalous pulmonary venous connection (ASD is also present)* Large arteries:- Truncus arteriosus
28 Atrium Ventricle - Single ventricle - Transposition of great arteries (VSD is also present).Ventricle- Single ventricle- DORV (types I, II = Taussig-Bing.
29 1- Pink tetralogy: TETRALOGY VARIANTS - 1/3 have mild pulmonary valvular obstruction with large VSD, allowing sufficient pulmonary flow.- Pulmonary atresia and VSD = pseudotruncus, extreme end of the spectrum.- Infundibular hypertrophy in VSD (3%)
30 2- Pentalogy of Fallot: 3- Trilogy of Fallot: Tetralogy + ASD PA stenosis + RV hypertrophy +Patent foramen ovale , or (ASD)
31 TRANSPOSITION OF GREAT ARTERIES (TGA) Types:Complete transposition of great arteries (D-TGA)AORTA ORIGINATES FROM RV- PA originates from LV- Normal position of atria and ventricles
32 Corrected transposition of great arteries (L-TGA): - Inversion of ventricles- The relative position of aorta and pulmonary artery can be derived from the diagram on the right.
33 D-TGA (COMPLETE TRANSPOSITION): Two independent circulations exist:• Blood returning from body RV blood delivered to body (aberrant aorta)• Blood returning from lung LV blood delivered to lung (via ASD,etc).
34 This circulatory pattern is incompatible with life unless there are associated anomalies that permit mixing of the two circulations, e.g., through ASD, VSD, or PDA.• Hemodynamics: depends on the level of admixture and R->L shunting.• RA and RV enlarged.
35 GENERAL:Situs stuff:- Abdominal situs refers to position of liver and stomach:a) Abdominal situs solitus: liver on right, stomach on left (normal)b) Abdominal situs inversus: liver on left, stomach on right
36 c) Thoracic situs refers to position of the tracheobronchial tree:
37 2- Left main bronchus inferior to left PA Thoracic situs solitus (normal)1- Left main bronchus longer than right main bronchus2- Left main bronchus inferior to left PA3- Right main bronchus superior to right PA
39 Type I ( situs inversus totalis) with 1- Mirror-like malposition of the heart2 - Mirror-like malposition of other viscera.3 - Kartegner suyndrome: Situs inversus , sinusitis & bronciectasis
40 Type II: ( Isolated dextrocardia) 1- Mirror-like cardiac malposition.1- Normal position of other viscera.3- serious cardiac anomalies.
41 Type III ( Dextroversion with): 1- Heart is merely displaced to right.2- RV remains to right & LV remains to left.3- Serious cardiac anomalies.
42 Type IV (Dextroposition) : Aquired dextrocardia: The heart is displaced by external factors: Pulmonary , pleural or diaphragmatic
43 Acquired dextrocardia Congenital dextrocardiaAcquired dextrocardia1- No apparent cause1- Apparent cause ( pushing or pulling) the heart2- Trachea is central2- Trachea is displaced to the right3- Apex is localized (LV)3- Diffuse ( as it is formed by RV)4- Situs inversus may be presnt4- Absent5- Associated congenital anomalies5- Absent
44 3) Eisenmenger”s syndrome The three common cynoticHeart Diseases1) Falot tetralogy2) fallot Trilogy.3) Eisenmenger”s syndrome
45 Prominent A & systolic expansion Fallot TetralogyFallot TriologyEisenmenger”sCyanosis(Onset)Usually since birthLate ( First on excersionCyanotic spellsPresentAbsentSquattingCyanosis & clubbingMarkedModerateNeck veinsNormalProminent A & systolic expansion
46 Present & diastolic shock Fallot TetralogyFallot TriologyEisenmenger”sRVHMild or moderateMarkedSystolic thrillAbsent or mildabsentPA-pulsationPresent & diastolic shock
47 Closely split& accentuatred P2 Split with weak P2singleS2PresentAbsentClickMildHarshMild or moderateSyst. murmurPR ( usually)absentDiast. murmure
48 Gallop ECG X- Ray Never present May be present RVH ,but no strain RVH with stranX- Ray- Lung oligemia , small PA ,coeur en Sabot & Right aortic archLung oligemia, enlarged PA, , Big heart & normal aortic archPeripheral lung oligemia,enlarged main PA & its two branches+ Big heart
49 PS , Determine the site of defect & PH+ Valvular PS - ASD - RVH VSD - aortic overridingEcho
50 Catheter may pass from RV to aorta Catheteri- zationLow PA pressure ,RV & aortic pressures are equal ,Catheter may pass from RV to aortaRV Angio AnatomyRV pressure may exceed aortic pressure ,Catheter cannot pass from RV to aortaRV angio anatomy rHigh PA pressure- RV pressure may exceed
74 All congenital HD are prone to IE except ASD. 1- Infective Endocarditis:All congenital HD are prone to IE except ASD.2- Heart failure.3- sudden Death.
75 4- Hematologic complications of chronic hypoxemia include: - Erythrocytosis; iron deficiency andbleeding diathesis- Hemostatic abnormalities have been documented in cyanotic patients with erythrocytosis and can occur in up to 20% of patients.
76 5 - Neurologic complications: - Cerebral hemorrhage.- Paradoxical cerebral emboli.- Brain abscess.6 - Renal dysfunction:- It can manifest itself as proteinuria, hyperuricemia, or renal failure .- Urate nephropathy, uric acid nephrolithiasis and gouty arthritis are rare but may occur.
77 7- Rheumatologic complications: 8- Gallstones: It is composed of calcium bilirubinate and consequent cholecystitis .
78 9- Arrhythmias Patients with Eisenmenger syndrome are at risk for sudden cardiac death, the etiology of which remains poorly defined .
79 The choice of antiarrhythmic drugs are complicated by: -The presence of ventricular dysfunction and lung disease.- The pro-arrhythmic effects.- The use of pacemakers to treat bradyarrhythmias, which are primary or secondary to antiarrhythmic therapy can be complicated by Inadequate venous access.
80 10 -The decision to use anticoagulants: It is complicated by the presence of:- Bleeding diathesis.- Difficulty obtaining a true measure of INR due to reduced plasma volume.
81 Interventional Options 1- Percutaneous closure of intracardiac shunts. A variety of devices can be used to close ASDs, PDAs and occasionally VSDs2- Palliative surgical interventions :It is performed in patients with cyanotic lesions.They are defined as those operations which serve to either increase or decrease pulmonary blood flow while allowing a mixed circulation and cyanosis to persist.
82 4- Heart & Lung Transplantation : 3- Physiologic repair:It is a term which can be applied to procedures which result in total or near-total anatomic and physiologic separation of the pulmonary and systemic circulations in complex cyanotic lesions thereby resulting in relief of cyanosis.4- Heart & Lung Transplantation :One or both lungs with surgical shunt closure and heart-lung transplantation have been performed in cyanotic patients with or without palliation who are no longer candidates for other forms of intervention.