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Transcript
Section 3D-4 Sabalvaro, D.K., Salac, C.N., Salazar, J., Salazar, R., Salcedo, V.E., Saldana, E., Sales, M.S.A., Salonga, C.A., San Diego, P., San Pedro, R. INTERACTIVE CASE 4 CARDIAC B 1 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 2 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 3 GENERAL DATA B.B. 3 year old male “blue baby” Chief complaint: progressive cyanosis 4 HISTORY OF PRESENT ILLNESS Born “ blue baby” Unspecified heart disease 1 week PTA More bluish lips (+) easy fatigability (+) seen to sit down after a short walk Few hours PTA While feeding, patient developed bluish hue of the face and extremities ADMISSION 5 REVIEW OF SYSTEMS Poor appetite, poor weight gain No skin lesions; cyanotic since birth Shortness of breath on exertion “Bluish” episodes while feeding 6 PAST MEDICAL HISTORY Born a “blue baby” Poor birth weight, retarded growth Occasional visit to ER due to cyanosis when crying (-) Asthma/allergies (-) Previous surgeries 7 FAMILY HISTORY (-) Heart disease (-) Diabetes (-) Asthma/allergies (-) PTB 8 PERTINENT PHYSICAL EXAMINATION Conscious, agitated BP 90/60, HR 90 bpm, RR 30’s, small for age Warm moist skin, no dermatoses Bluish lip and buccal ,mucosa Heart: (+) thrill along left sternal border (+) harsh systolic murmur Single S2 Extremities: bluish nail beds, (+) clubbing 9 MISSING DATA Obstetrical History: Illnesses of mother during gestation Episodes of “tet” spells - marked increase in cyanosis followed by syncope Squatting (?) 10 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 11 SALIENT FEATURES 3 y/o Male Cyanotic since birth Difficulty in feeding, poor birth weight, retarded growth (+) dyspnea and easy fatigability (+) clubbing, cyanosis of the lips, buccal mucosa and nail beds Heart: (+) thrill along left sternal border (+) harsh systolic murmur single S2 12 CLINICAL IMPRESSION Congenital Cyanotic Heart Disease, probably Tetralogy of Fallot 13 DIFFERENTIAL DIAGNOSIS 14 Transposition of Great Vessels Total Anomalous Pulmonary Venous Return Tetralogy of Fallot Cyanosis Ebstein’s Anomaly Truncus Arteriosus Hypoplastic Left Heart Syndrome Tricuspid Atresia 15 Congenital Anomaly Transposition of Great Vessels Total Anomalous Pulmonary Venous Return Pathology Signs and Symptoms Chest Radiograph Two major arteries leaving the heart are connected to the wrong ventricles or lower chambers of the heart. Blue or cyanotic shortly after birth. The blueness doesn't go away even if the baby is given extra oxygen. Shortness of breath Poor feeding Clubbing of the fingers or toes Increased vascularity Cardiomegaly Cardiac silhouette: “egg on its side”; “apple on a stem” Narrow vascular pedicle None of the pulmonary veins are connected to the left atrium but instead all drain by various routes into the right atrium. Cyanosis and respiratory distress Cyanosis made worse on feeding, especially with the infracardiac type caused by the compression of the common pulmonary vein by the food-filled esophagus. (-) Cardiac murmur Increased pulmonary vascular markings which, if untreated, progress to cardiac failure. A 'snowman' sign or figure of 8 configuration in the supracardiac type but rarely before 4 months of age. Type1: supracardiac Type2: intracardiac Type3: infracardiac Reference: http://www.bcm.edu/radiology/cases/pediatric/text/2i1A.htm 16 Signs and Symptoms Congenital Anomaly Pathology Truncus Arteriosus Only one artery connects to the heart instead of two. Large VSD over which a large, single great vessel (truncus) arises. This single great vessel carries blood both to the body and to lungs. Blood from both ventricles mixes together as it all exits through the single valve exiting from the heart. Bluish cast to their skin, especially around the nose and mouth. Breathlessness Rapid breathing Excessive sweating Restlessness Increased vascularity Pulmonary venous edema Cardiomegaly Left atrium and either/both ventricles prominence Concave main pulmonary artery segment Large aortic shadow Right aortic arch in 35% Complete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle. Congestive heart failure Growth retardation Poor skin coloration (pallor to frank cyanosis) Inability to complete a feeding session Frequent pauses during feeding, and/or frank anorexia Respiratory difficulties reported as nasal flaring or muscle retractions, digital clubbing Cardiomegaly with a prominent right heart border reflecting RA enlargement Diminished pulmonary vascular markings A right aortic arch in 38% of cases Tricuspid Atresia Chest Radiograph References: http://emedicine.medscape.com/article/158359-diagnosis and http://www.bcm.edu/radiology/cases/pediatric/text/2h-desc.htm 17 Congenital Anomaly Hypoplastic Left Heart Syndrome Ebstein’s Anomaly Pathology Signs and Symptoms Chest Radiograph Marked hypoplasia of the left ventricle and ascending aorta atretic, hypoplastic or stenotic aortic and mitral valves Intact ventricular septum PDA supplies blood to the systemic circulation. (+) ASD or patent foramen ovale Coexisting coarctation of the aorta Respiratory symptoms and profound systemic cyanosis at birth As the ductus arteriosus begins to close normally over the first 24-48 hours of life, symptoms of cyanosis, tachypnea, respiratory distress, pallor, lethargy, metabolic acidosis, and oliguria develop. No intervention to reopen the ductus arteriosus death Chest radiographic findings are not specific for hypoplastic left heart syndrome. Cardiomegaly and increased pulmonary venovascular markings are typically present. Marked pulmonary edema may be noted in infants with obstructed pulmonary venous return. Apical displacement of the septal and posterior tricuspid valve leaflets atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Fatigue Dyspnea Palpitations Clubbing Precordial asymmetry Ankle edema Ascites (Symptoms of right heart failure) Normal findings Cardiomegaly Small aortic root and main pulmonary artery shadow Decreased pulmonary vasculature Large right atrium Reference: http://emedicine.medscape.com/article/154447-diagnosis 18 Congenital Anomaly Tetralogy of Fallot Pathology Maldevelopment of RV infundibulum Pulmonary artery stenosis VSD Deviation of the aortic origin to the right RVH The degree of right ventricular outflow tract obstruction accounts for the deleterious hemodynamics of TOF. Signs and Symptoms Poor feeding, cyanosis during feeding, fussiness, tachypnea, and agitation Dyspnea on exertion Squatting Low birthweight Growth retardation Delayed development and puberty Chest Radiograph Coeur en sabot secondary to uplifting of the cardiac apex from RVH and the absence of a normal main pulmonary artery segment Decreased pulmonary vascularity RA enlargement Right-sided aortic arch (20-25% of patients) with indentation of leftwardpositioned tracheobronchial shadow 19 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 20 1 2 3 4 5 6 7 8 9 NORMAL CHEST X RAY OF A 3-YEAR OLD BABY Trachea Bronchial bifurcation Cardiophrenic angle Right atrium NORMAL CHEST X RAY OF A 3-YEAR OLD BABY Costophrenic angle CARDIO-THORACIC RATIO and DIAPHRAGMS A/B 0.65 A B NORMAL CHEST X RAY LEGEND: 1 - Trachea 2 - Ascending aorta 3 - Brachiocephalic vessels 4 - Pulmonary artery 5 - L ventricle 6 - Retrosternal space 7 - R hemidiaphragm 8 - L hemidiaphragm NORMAL CHEST X RAY: LATERAL VIEW PA VIEW LATERAL VIEW PATIENT’S CHEST X RAY PA VIEW CT RATIO = 0.66 Decreased pulmonary vasculature A B PATIENT’S CHEST X RAY Cardiac apex displaced upward “coer en sabot” Lifted up Apex PATIENT’S CHEST X RAY PATIENT’S CHEST X RAY R Aortic Arch Concave MPA RPA Right ventricular hypertrophy PATIENT’S LATERAL VIEW OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 30 Tetralogy of Fallot 4 Features: 1. Ventricular Septal Defect 2. Obstruction of the right ventricular outflow tract 3. Overriding aorta 4. Right Ventricular hypertrophy 31 Symptomatology Each child can present with different symptoms which depend upon the severity of the obstruction and stenosis as seen in the pathophysiology of the disease. Blue color of the skin, lips and nail beds (central and peripheral cyanosis) which occurs with crying or feeding. Some babies do not have noticeable cyanosis, but may instead be very irritable or lethargic due to a decreasing amount of oxygen available in the bloodstream due to admixing of oxygenated and less oxygenated blood. Some children may have pale or ashen color and may have cool clammy skin. Dyspnea and syncope 32 Physical Exam Findings RV predominance on palpation May have a bulging left hemithorax Systolic thrill at the lower left sternal border Aortic ejection click Single S2 - Pulmonic valve closure not heard Systolic ejection murmur - varies in intensity inversely with the degree of RVOT obstruction More cyanotic patients have greater obstruction and a softer murmur. An acyanotic patient with TOF has a long, loud, systolic murmur with a thrill along the RVOT. Cyanosis and clubbing - variable Squatting position- compensatory Scoliosis - common Retinal engorgement Hemoptysis 33 Pathophysiology Result from anterosuperior displacement of the infundibular septum Severity of obstruction to RV outflow determines the direction of blood flow. o If subpulmonary stenosis is mild- shunt, may be left-to-right w/o cyanosis. o ↑ in severity of obstruction = ↑ in resistance to RV outflow, right-to-left shunting predominates along with cyanosis ↑ subpulmonic stenosis, pulmonary arteries are smaller and hypoplastic and the diameter of the aorta becomes progressively larger. RVH - compensatory As the child grows, the pulmonic orifice does not expand proportionally to the enlarging heart, making the obstruction worse. 34 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the Chest X ray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 35 OTHER DIAGNOSTIC PROCEDURES Ultrasonography Angiography CT Scan MRI 36 Ultrasonography Findings: Echocardiography is the primary imaging method for examining a child in whom TOF is suspected. Intracardiac anomalies, including pulmonary infundibular and valvular stenosis and the position of the aortic root overriding the ventricular septal defect, are identified with 2dimensional echocardiography. The origins of the coronary arteries can also be identified. 37 38 Ultrasonography Doppler ultrasonographic examination of the pulmonary outflow tract can be used to measure the velocity gradient in the right ventricular outflow tract and to differentiate severe stenosis from atresia. Continuity of the branch pulmonary artery with the main pulmonary artery can be identified, and the size of the branch pulmonary arteries can be measured. 39 Ultrasonography The initial placement of palliative shunts is likely in children who have small branch pulmonary arteries in order to allow the pulmonary arteries to grow before corrective surgery. The full length of the shunts may not be visible; however, Doppler ultrasonography can be used to verify shunt patency, even when the entire length of the shunt cannot be imaged. 40 Angiography Gold standard in evaluating blood vessels Findings Angiography is the traditional criterion standard and best modality for the evaluation of the pulmonary and coronary arterial morphology, as well as the morphology of the systemic collateral arteries. 51 Angiography The branch pulmonary arteries have a characteristic seagull appearance. Pulmonary arterial measurements for the calculation of the McGoon ratio and Nakata index are critical to surgical planning. An aortic root injection is used to evaluate the position and number of coronary arteries. 52 TOF in Angiogram Aortic Arch Descending Aorta LPA Stump RPA MPA Descending branch of RPA Catheter Ascending Aorta 53 54 55 OBJECTIVES • To present a case of a 2 year old “blue baby” • To give the clinical impression and differential diagnosis of the case based on the history and physical examination • To present the chest xray of the patient • To discuss the pathophysiology and symptomatology of Tetralogy of Fallot • To discuss other diagnostic procedures that may be useful in TOF • To discuss the management and prognosis of a patient with TOF 56 Management Treatment depends on the severity of right ventricular outflow tract obstruction. Immediate increase in pulmonary blood flow to prevent hypoxia. Oxygenation, normal body temperature, normal glucose level should be maintained. Prevention and prompt treatment of dehydration to prevent hemoconcentration and possible thrombotic episodes. 57 Management For Tet spells Propanolol (0.5–1 mg/kg every 6 hr) Morphine to reduce ventilatory drive Vasopressor such as epinephrine to increase blood pressure Modified Blalock-Taussig shunt Aortopulmonary shunt 58 Prognosis UNTREATED: Untreated TOF may lead to further RV hypertrophy and dilated cardiomyopathy (beginning on the right,then left). Actuarial survival for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years. 59 Prognosis TREATED: • After successful total correction, patients are generally asymptomatic and are able to lead unrestricted lives. – Have lower than normal exercise capacity, maximal heart rate and cardiac output (more frequent in those who have undergone surgery at a later age). • Uncommon immediate postoperative problems include right ventricular failure, transient heart block, residual VSD with left-to-right shunting, and myocardial infarction from interruption of an aberrant coronary artery. • A number of children have premature ventricular beats after repair of TOF. • Lifetime follow-up care 60 REFERENCES Misra, Rakesh, et. al. A-Z of Chest Radiology, 2007. Novelline, Robert A. Squire’s Fundamentals of Radiology, 6th ed. Sutton. A Textbook of Radiology and Imaging. http://emedicine.medscape.com/article/158359diagnosis http://www.bcm.edu/radiology/cases/pediatric/text/2i1A. htm http://www.bcm.edu/radiology/cases/pediatric/text/2hdesc.htm 61