EVALUATING CYANOTIC HEART DISEASE - Copy

EVALUATING CYANOTIC HEART DISEASE:

EVALUATING CYANOTIC HEART DISEASE SPEAKER : DR. SUKAMAL SANTRA CHAIRPERSON :DR. S.K. CHATTERJEE

SUSPECTED CONG HEART DISEASES:

SUSPECTED CONG HEART DISEASES 5 basic questions: 1.Cyanotic or acyanotic 2.Pulmonary blood flow increased or decreased 3.Malformation originate in left side or right side 4.Dominate ventricle RV or LV 5.Pulmonary hypertension present or not

Clinical presentation:

Clinical presentation Central cyanosis Polycythemia Clubbing Exertional dyspnoea Palpitation Sudden death CCF

CENTRAL CYANOSIS:

CENTRAL CYANOSIS Def. Arterial oxygen desaturation resulting from the shunting or mixing of systemic venous blood into the arterial circulation. The magnitude of shunting/mixing and the amount of pulmonary blood flow determine the severity of desaturation . Two categories : (1) those with increased pulmonary blood flow or (2) those with decreased pulmonary blood flow CLINICAL FEATURES Hyperviscosity Syndrome. Hematological . Central Nervous System. Renal Rheumatological complications

Physical Examination:

Physical Examination Mild central cyanosis----difficult to diagnose signs of failure to thrive in infant or child assess both cardiac and visceral situs presence of characteristic facial or somatic features of an underlying syndrome surveillance of the chest wall for scars detection of chamber enlargement Auscultation for heart sounds & murmur Whole of the chest should be auscultated

Electrocardiogram:

Electrocardiogram an important tool in the assessment of CHD. The dominant theme in CHD is the prevalence of right heart disease takes the form of right axis deviation along with right atrial and right ventricular hypertrophy. Right ventricular hypertrophy ----pulmonary hypertension, right ventricular outflow tract obstruction, or a subaortic right ventricle. Incomplete right bundle branch block ----right ventricular hypertrophy due to pressure (e.g., pulmonary hypertension or pulmonary stenosis ) or volume (e.g., ASD) overload. Very wide QRS complexes ---dilated and dysfunctional ventricles, most specifically in patients with repaired tetralogy , complete right bundle branch block, and severe pulmonary regurgitation.

Electrocardiogram cont:

Electrocardiogram cont Atrial flutter is much more common in young patients than is atrial fibrillation. First-degree block is often seen in AV septal defects, cc-TGA, and Ebstein anomaly. Complete heart block is most often seen in patients with cc-TGA, as well those with older VSD repairs Left axis deviation ------AV septal defect, a univentricular heart, and a hypoplastic right ventricle, tricuspid atresia . Deep q waves in the left chest leads can be caused by left ventricular volume overload in a young person with aortic or mitral regurgitation.

Differential diagnosis of cxr:

Differential diagnosis of cxr CRITERIA FOR SHUNT VASCULARITY . (1) uniformly distributed vascular markings with absence of the normal lower lobe vascular predominance; (2) right descending pulmonary artery diameter that exceeds 17 mm; and (3) a pulmonary artery branch that is larger than its accompanying bronchus (best noted in the right parahilar area). Anemia , pregnancy, thyrotoxicosis , and a pulmonary AV fistula may mimic shunt vascularity CYANOTIC PATIENTS WITH SHUNT VASCULARITY . single ventricle with transposition, persistent truncus arteriosus , tricuspid atresia without significant pulmonary outflow obstruction, total anomalous pulmonary venous connection, double-outlet right ventricle

Differential diagnosis of cxr :

Differential diagnosis of cxr CYANOTIC PATIENTS WITH A VSD AND NORMAL OR DECREASED PULMONARY VASCULARITY . tetralogy of Fallot ; tricuspid atresia with pulmonary stenosis ; single ventricle and pulmonary stenosis ; D-TGA with pulmonary stenosis ; cc-TGA with pulmonary stenosis ; double-outlet right ventricle with pulmonary stenosis ; pulmonary atresia CAUSES OF RETROSTERNAL FILLING ON LATERAL CHEST RADIOGRAPH . right ventricular dilation, TGA, CAUSES OF A STRAIGHT LEFT HEART BORDER. right ventricular dilation, left atrial dilation, cc-TGA, pericardial effusion, Ebstein anomaly, and congenital absence of the left pericardium. CARDIOVASCULAR DISEASES ASSOCIATED WITH SCOLIOSIS. cyanotic CHD, Eisenmenger syndrome CAUSES OF LARGE CENTRAL PULMONARY ARTERIES. increased pulmonary flow (main pulmonary artery and branches), increased pulmonary pressure (main pulmonary artery and branches), pulmonary stenosis (main and left pulmonary artery), and idiopathic dilation of the pulmonary artery (main pulmonary artery ).

Echocardiography:

Echocardiography Transthoracic Echocardiography Transesophageal Echocardiography Three-Dimensional Echocardiography Intracardiac Echocardiography

SEGMENTAL APPROACH TO ECHOCARDIOGRAPHY IN CONGENITAL HEART DISEASE:

SEGMENTAL APPROACH TO ECHOCARDIOGRAPHY IN CONGENITAL HEART DISEASE Apex Position ----- dextrocardia,levocardia,mesocardia Situs of the Atria Atrioventricular Relationship morphological right ventricle: (1) a trabeculated apex, (2) a moderator band, (3) septal attachment of the tricuspid valve, and (4) lower (apical) insertion of the tricuspid valve. The tricuspid valve is always “attached” to the morphological right ventricle. morphological left ventricle : (1) a smooth apex, (2) no moderator band, (3) no septal attachment of the mitral valve, and (4) higher (basal) insertion of the mitral valve. The mitral valve is always “attached” to the morphological left ventricle . Ventriculoarterial Relationship The pulmonary artery is identified by early branching pattern into the left and right pulmonary arteries; the pulmonary valve is always “attached” to this The aorta is identified by its “candy cane” shape and the take-off of its three head and neck vessels . The aortic valve is always “attached” to the aorta.

Transesophageal Echocardiography:

Transesophageal Echocardiography ADVANTAGES 1.better 2D resolution than TEE for visualization of pulmonary venous anatomy, atrial anatomy, AV valve morphology, ventricular outflow tract lesions, and vegetations or thrombi 2. important in adult patients with multiple previous cardiac operations, when adequate transthoracic windows are often difficult to obtain INDICATIONS 1.SECUNDUM ATRIAL SEPTAL DEFECT 2.EBSTEIN ANOMALY. 3.GUIDANCE OF THERAPEUTIC INTERVENTION. 4.PERCUTANEOUS DEVICE CLOSURE. 5.INTRAOPERATIVE AND POSTOPERATIVE ASSESSMENT .

Intracardiac Echocardiography:

Intracardiac Echocardiography uses lower frequency miniaturized transducers which is mounted into catheters ;capable of percutaneous insertion into the heart. provides high-resolution 2D and hemodynamic data with full Doppler capabilities No need for general anesthesia Used in percutaneous ASD device closure

Cardiovascular MRI:

Cardiovascular MRI INDICATIONS 1.when transthoracic echocardiography cannot provide the needed diagnostic information because of suboptimal visualization of the heart in adult patients, especially those who have had surgery 2.an alternative to diagnostic cardiac catheterization; 3.for MRI's unique capabilities such as tissue imaging and vessel-specific flow quantification. 4.can evaluate valve regurgitation, postoperative systemic and pulmonary venous pathways, and the great vessels. 5. the main imaging modality in adolescents and adults with repaired tetralogy of Fallot , TGA and diseases of the aorta.

CARDIAC CATHETERIZATION:

CARDIAC CATHETERIZATION “Diagnostic” catheterization is reserved for 1.resolving unanswered questions from the less-invasive techniques and 2.measuring hemodynamics like ventricular end-diastolic pressures or pulmonary artery pressures and resistance 3.evaluating possible coronary artery disease, especially before heart surgery. THERAPEUTIC CATHETERIZATION 1.Balloon atrial septostomy as initial palliation in many infants with D-TGA 2.blade atrial septostomy ; 3.device or coil closure of PDA; 4.closure of ASD and patent foramen ovale ; 5.transluminal balloon dilation of pulmonary and aortic valve stenosis ; 6.radiofrequency perforation of pulmonary valve atresia ; 7.balloon-expandable intravascular stents for right ventricular outflow tract pulmonary artery, aortic coarctation , and other vascular stenoses ; 8.device occlusion of unwanted collateral vessels and AV fistulas.

TYPES OF DEFECT:

TYPES OF DEFECT TETRALOGY OF FALLOT TRICUSPID ATRESIA EBSTEIN ANOMALY TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS(TAPVC) PERSISTED TRUNCUS ARTERIOSUS COMPLETE TRANSPOSITION OF GREAT ARTERIES(C-TGA) CONGENITALLY CORRECTED TRANSPOSITION OF GREAT ARTERIES DOUBLE-OUTLET RIGHT VENTRICLE HYPOPLASTIC LEFT HEART SYNDROME EISENMENGER SYNDROME

TETRALOGY OF FALLOT:

TETRALOGY OF FALLOT Commonest cause of cyanotic heart disease after one yr of age. Cl presentation depends on severity of RV outflow obstruction. Most are cyanotic since birth. May presents with exertional dyspnoea. Hypoxic spell squatting

TETRALOGY OF FALLOT:

TETRALOGY OF FALLOT SIGNS RV type impulse Systolic thrill at left 3 rd ics S2—single(A2) Ejection systolic murmur at left 3 rd ics Continuous murmur faintly audible over the ant & post chest

TETRALOGY OF FALLOT:

TETRALOGY OF FALLOT CHEST X RAY normal-sized, boot-shaped heart ( coeur en sabot ) prominence of the right ventricle and a concavity in the region of the underdeveloped right ventricular outflow tract and main pulmonary artery. The pulmonary vascular markings are typically diminished, the aortic arch may be on the right side (25 percent). Dilatation of the ascending aorta

TETRALOGY OF FALLOT:

TETRALOGY OF FALLOT Electrocardiography Right axis deviation Right ventricular and right atrial hypertrophy Adults with repaired tetralogy of Fallot , a complete right bundle branch block following repair has been the rule. ECHOCARDIOGRAPHY malaligned and nonrestrictive VSD overriding aorta (<50 percent override) presence and degree of right ventricular outflow tract obstruction ( infundibular , valvular , and/or pulmonary arterial stenosis ).

TRICUSPID ATRESIA :

TRICUSPID ATRESIA ATRESIA OF TRICUSPID VALVE INTERATRIAL COMMUNICATION INTERVENTRICULAR COMMUNICATION HYPOPLASTIC RV Two types---- 1.TA with normal related great art(70%) 2.TA with TGA ( 30%)

TRICUSPID ATRESIA:

TRICUSPID ATRESIA CLINICAL FEATURES Severe cyanosis since birth Hypoxic spell Prominent ‘a’ wave in jvp LV type impulse Tender hepatomegaly with presystolic pulsation S2 ---single loud( A2) A holosystolic murmur at left mid & lower sternal border

TRICUSPID ATRESIA:

TRICUSPID ATRESIA ECG Left axis deviation, Tall peaked rt atrial ‘P’ wave Left ventricular hypertrophy. Left atrial enlargement may be present if pulmonary flow is high. CHEST RADIOGRAPHY Left ventricular configuration Oligaemic lung field The main pulmonary trunk is inapparent . A right aortic arch exists in 25 percent of patients.

Slide 28:

Posteroanterior view of chest roentgenogram in an infant with tricuspid atresia with normally related great arteries. The heart is minimally enlarged , left ventricular with broad apex.The pulmonary vascular markings are decreased, and the main pulmonary artery segment is somewhat concave. Rt atrium is enlarged with prominent sup vena cava.

TRICUSPID ATRESIA:

TRICUSPID ATRESIA ECHO Atretic tricuspid valve in Apical subxiphoid view Hypoplastic RV Large LV ASD , VSD,Aortic arch ano maly

EBSTEIN ANOMALY:

EBSTEIN ANOMALY Septal & post leaflet of tricuspid valve displaced downward ---- atrialisation of RV h/o maternal use of lithium h/o cyanosis,effort intolerance & fatigue,arrythmia LV impulse S1 widely split –loud T1(the sail sound) S2 widely split S3,S4---triple & quardruple rhythm Systolic TR murmur not increases with re sp Cyanosis with normal or reduced pulmonary blood flow and a dominant left ventricle with type B WPW is diagnostic

EBSTEIN ANOMALY:

EBSTEIN ANOMALY Electrocardiography Himalayan p wave Prolonged PR RBBB WPW AF Deep q wave in inf & V1-V4

EBSTEIN ANOMALY:

EBSTEIN ANOMALY CXR RT BORER OF HEART PROMINENT WITH LEFT WARD CONVEXITY WATER BOTTLE APPEARANCE OLIGEMIC LUNG FIELD AORTA & PUL ART INCOSPICOHS

EBSTEIN ANOMALY:

EBSTEIN ANOMALY ECHOCARDIOGRAPHY Apical displacement of the septal leaflet of the tricuspid valve by 8 mm/m 2 or more & An elongated sail-like appearance of the anterior leaflet, confirms the diagnosis The size of the atrialized portion of the right ventricle The systolic performance of the functional right ventricle can be estimated. The degree of tricuspid regurgitation. Associated defects such as ASDs Presence and direction of shunting

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION:

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION all of the systemic and pulmonary venous return drains to the right atrium Most have symptoms during the first year of life 80 percent die before 1 year of age if not treated Infradiaphragmatic —cyanotic since birth Supradiaphragmatic —cyanosis & CHF at 4-6wk Feature similar to ASD with increased rt sided flow. RV type impulse S1-loud(T1) Wide fixed splitting of s2--accentuated P2 Midsystolic murmur at left 2 nd ics

TAPVC cont:

TAPVC cont CXR figure-of-8” or “snowman” heart due to enlargement of the heart and the presence of a dilated right superior vena cava, innominate vein, and left vertical vein.

TAPVC cont:

TAPVC cont ECG Rt axis deviation RAH & RVH ECHOCARDIOGRAPHY Marked enlargement of the right ventricle and a small left atrium. An echo-free space representing the pulmonary venous confluence can usually be seen behind the left atrium. The drainage of all four pulmonary veins and their connections must be identified

TRANSPOSITION OF GREAT ARTERIES:

TRANSPOSITION OF GREAT ARTERIES TGA COMPLETE TGA --------------- CONGENITALY CORRECTED TGA WITHOUT VSD ------------------- WITH VSD (ASD,PDA) WITH PS--------------------------- WITHOUT PS

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES:

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES Male : female 4:1 Average birth weight and size are greater than normal. Dyspnea and cyanosis from birth, progressive hypoxemia, and congestive heart failure usual presentation Sever cyanosis & hypoxemia in infants who have only a small patent foramen ovale or ductus arteriosus and an intact ventricular septum; or left ventricular outflow tract obstruction. cyanosis can be minimal, and heart failure is the dominant after the first few weeks of life if large VSD or PDA present

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont:

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont Pulse—full volume JVP---N or increased in CHF RV type impulse Palpable S2 at left base----originate in aortic valve early or holosystolic murmur of VSD may be audible in less cyanotic infants with associated VSD. A soft midsystolic murmur of pulmonary stenosis (PS or LVOT obstruction) may be audible .

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont:

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont CXR Cardiomegaly with increased pulmonary vascularity is typically present. An egg-shaped cardiac silhouette with a narrow, superior mediastinum is characteristic

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont:

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES cont Electrocardiography rightward QRS axis RVH is usually present after the first few days of life Biventricular hypertrophy (BVH) ECHOCARDIOGRAPHY In the parasternal short-axis view, 1. the great arteries appear as“double circles” . 2.The PA is in the centre of the heart 3. The aorta is usually anterior and slightly to the right of the PA. Frequently, associated defects such as VSD, LVOT obstruction (dynamic or fixed), or PS are found.

Congenitally Corrected Transposition of the Great Arteries:

Congenitally Corrected Transposition of the Great Arteries Rare less than 1% Asymptomatic when L-TGA is not associated with other defects. most patients with associated defects become symptomatic with cyanosis resulting from VSD and PS or CHF resulting from a large VSD. 3. Exertional dyspnea and easy fatigability may develop with regurgitation of the systemic AV valve

Congenitally Corrected Transposition of the Great Arteries:

Congenitally Corrected Transposition of the Great Arteries Hyperactive precordium occurs in the presence of a large VSD The S2 is loud and single at the upper left or right sternal border. A grade 2 to 4/6 harsh, holosystolic murmur along the lower left sternal border indicates the presence of VSD or systemic AV valve regurgitation.

Congenitally Corrected Transposition of the Great Arteries:

Congenitally Corrected Transposition of the Great Arteries CXR 1 . A straight, left upper cardiac border, formed by the ascending aorta 2. Cardiomegaly and increased pulmonary vascular markings are present with VSD. 3.RT pulmonary hilum often prominent & elevated------ rt sided water fall appearance

Congenitally Corrected Transposition of the Great Arteries:

Congenitally Corrected Transposition of the Great Arteries ECHOCARDIOGRAPHY 1.The morphological left ventricle is <characterized by its smooth endocardial surface and is guarded by a bileaflet AV (mitral) valve with no direct septal attachment. 2.The morphological right ventricle is recognized by its apical trabeculation and moderator band and is guarded by a trileaflet apically displaced AV valve (tricuspid valve) with direct attachment to the septum. 3.The AV valves show reversed offsetting, a strong clue to the diagnosis. 4.Ebstein-like malformation of the left (tricuspid) AV valve may be present

DOUBLE-OUTLET RIGHT VENTRICLE:

DOUBLE-OUTLET RIGHT VENTRICLE 50 percent of each semilunar valve arises from the morphological right ventricle. Clinical physiological responds depends on size & location of VSD and presence or absence of PS Major clinical patter 1.with a subaortic VSD with PS---------- commonest clinical scenario and management algorithm are similar or identical to that of tetralogy of Fallot 2.with a subaortic VSD ,no PS with low pulmonary vascular resistance ----resembles : non restrictive perimembranous VSD 3.with a subaortic VSD ,no PS with high pulmonary vascularresistance -----resembles : Eisenmenger syndrome 4.with a subpulmonary VSD with no PS( Taussig -Bing anomaly)----resembles : TGA with non restrictive VSD.

HYPOPLASTIC LEFT HEART SYNDROME:

HYPOPLASTIC LEFT HEART SYNDROME a group of closely related cardiac anomalies characterized by underdevelopment of the left cardiac chambers, in association with atresia or stenosis of the aortic and/or the mitral orifices, and hypoplasia of the aorta. characterized by duct-dependent systemic blood flow and so tends to present with severe symptoms within the first week of life, as ductal constriction occurs. The diagnosis should be considered in any infant with the sudden onset of circulatory collapse and severe lactic acidosis

HYPOPLASTIC LEFT HEART SYNDROME:

HYPOPLASTIC LEFT HEART SYNDROME ECG right axis deviation, right atrial and ventricular enlargement, and ST and T wave abnormalities in the left precordial leads . ECHOCARDIOGRAPHY The left ventricular cavity is small, with hypoplastic perforate mitral valve. Endocardium is usually thickened, consistent with endocardial fibroelastosis . Hypoplastic tubular ascending aorta guarded by an atrtic aortic valve The PDA varies in size according to treatment,

Eisenmenger syndrome:

Eisenmenger syndrome DEFINITION : it is a pulmonary vascular obstructive disease that develops as a consequence of a large preexisting left-to-right shunt such that pulmonary artery pressures approach systemic levels and the direction of the flow becomes bidirectional or right to left. Causes – 1.Simple--ASD, VSD, and PDA 2.Complex-- AV septal defect, truncus arteriosus , aortopulmonary window, and univentricular heart. The high pulmonary vascular resistance is usually established in infancy (by age 2 years, except in ASD) and is sometimes present from birth .

Eisenmenger syndrome:

Eisenmenger syndrome SYMPTOMS Cyanosis --- central 1 st decade—VSD 2 nd decade---PDA 3 rd decade—ASD Exercise intolerance ( dyspnea and fatigue) is proportional to the degree of hypoxemia or cyanosis. Palpitations in nearly half the patients ( atrial fibrillation/flutter in 35 percent, Ventricular tachycardia in up to 10 percent); Hemoptysis in about 20 percent; due to bleeding bronchial vessels or pulmonary infarction. Pulmonary thromboembolism , angina, syncope, and endocarditis in about 10 percent each; and congestive heart failure after 40 year

Eisenmenger syndrome:

Eisenmenger syndrome Physical examination central cyanosis and clubbing of the nail beds. Patients with Eisenmenger PDA can have pink nail beds on the right (>left) hand and cyanosis and clubbing of both feet, so-called “differential cyanosis.” JVP normal or elevated, especially with prominent ‘v ‘waves when tricuspid regurgitation is present. Signs of pulmonary hypertension — 1. a right ventricular heave, palpable and loud P 2 , and a right-sided S 4 — are typically present. 2. In many patients a pulmonary ejection click and a soft and scratchy systolic ejection murmur, attributable to dilation of the pulmonary trunk, and 3. high-pitched decrescendo diastolic murmur of pulmonary regurgitation (Graham Steell ) are audible. Peripheral edema is absent until right-sided heart failure ensues .

Eisenmenger syndrome:

Eisenmenger syndrome CHEST RADIOGRAPHY. Dilated central pulmonary arteries with rapid tapering of the peripheral pulmonary vasculature are the radiographic hallmarks of Eisenmenger syndrome. Pulmonary artery calcification may be seen and is diagnostic of long-standing pulmonary hypertension. Eisenmenger syndrome due to VSD or PDA usually has a normal or slightly increased cardiothoracic ratio. Eisenmenger syndrome due to an ASD typically has a large cardiothoracic ratio due to right atrial and ventricular dilation, along with an inconspicuous aorta. Calcification of the duct & prominent aortic knuckle ----- Eisenmenger PDA.

Eisenmenger syndrome:

Eisenmenger syndrome ELECTROCARDIOGRAPHY (ECG). Peaked P waves consistent with right atrial overload right ventricular hypertrophy with right axis deviation Atrial arrhythmias can be present . ECHOCARDIOGRAPHY . The intracardiac defect should be seen readily along with bidirectional shunting. A pulmonary hypertensive PDA is not easily seen. Evidence of pulmonary hypertension is found. Assessment of pulmonary right ventricular function adds prognostic value .

Eisenmenger syndrome:

Eisenmenger syndrome CARDIAC CATHETERIZATION . 1. provides direct measurement of the pulmonary artery pressure, documenting the existence of severe pulmonary hypertension, 2. Administration of pulmonary arterial vasodilators (O 2 , nitric oxide, prostaglandin I 2 [ epoprostenol ]) can discriminate among patients in whom surgical repair is contraindicated and those with reversible pulmonary hypertension who may benefit from surgical repair. OPEN-LUNG BIOPSY when reversibility of the pulmonary hypertension is uncertain from the hemodynamic data .

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