INTERACTIVE CASE 4 CARDIAC B

1. 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 4CARDIAC B

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. 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

4. GENERAL DATA • B.B. • 3 year old male • “blue baby” • Chief complaint: progressive cyanosis

5. 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

6. Review of Systems • Poor appetite, poor weight gain • No skin lesions; cyanotic since birth • Shortness of breath on exertion • “Bluish” episodes while feeding

7. 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

8. FAMILY HISTORY • (-) Heart disease • (-) Diabetes • (-) Asthma/allergies • (-) PTB

9. 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

10. MISSING DATA • Obstetrical History: Illnesses of mother during gestation • Episodes of “tet” spells - marked increase in cyanosis followed by syncope • Squatting (?)

11. 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

12. 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

13. CLINICAL IMPRESSION Congenital Cyanotic Heart Disease, probably Tetralogy of Fallot

14. Differential Diagnosis

16. Reference: http://www.bcm.edu/radiology/cases/pediatric/text/2i1A.htm

17. References: http://emedicine.medscape.com/article/158359-diagnosis and http://www.bcm.edu/radiology/cases/pediatric/text/2h-desc.htm

18. Reference: http://emedicine.medscape.com/article/154447-diagnosis

20. 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

21. 1 2 3 4 5 6 7 8 9 NORMAL CHEST X RAY OF A 3-YEAR OLD BABY

22. Trachea Bronchial bifurcation Cardiophrenic angle Right atrium Costophrenic angle NORMAL CHEST X RAY OF A 3-YEAR OLD BABY

23. CARDIO-THORACIC RATIO and DIAPHRAGMS A/B 0.65 A B NORMAL CHEST X RAY

24. 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

25. LATERAL VIEW PA VIEW PATIENT’S CHEST X RAY

26. PA VIEW CT RATIO = 0.66 Decreased pulmonary vasculature A B PATIENT’S CHEST X RAY

27. Cardiac apex displaced upward “coer en sabot” Lifted up Apex PATIENT’S CHEST X RAY

28. PATIENT’S CHEST X RAY R Aortic Arch Concave MPA RPA Right ventricular hypertrophy

29. PATIENT’S LATERAL VIEW

30. 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

31. Tetralogyof Fallot 4 Features: • Ventricular Septal Defect • Obstruction of the right ventricular outflow tract • Overriding aorta • Right Ventricular hypertrophy

32. 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

33. 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

34. Pathophysiology • Result from anterosuperior displacement of the infundibular septum • Severity of obstruction to RV outflow determines the direction of blood flow. • If subpulmonary stenosis is mild- shunt, may be left-to-right w/o cyanosis. • ↑ 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.

35. 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

36. OTHER DIAGNOSTIC PROCEDURES • Ultrasonography • Angiography • CT Scan • MRI

37. Ultrasonography Findings: • Echocardiography is the primary imaging method for examining a child in whom TOF is suspected. • Intracardiac anomalies, including pulmonary infundibular and valvularstenosis and the position of the aortic root overriding the ventricular septal defect, are identified with 2-dimensional echocardiography. • The origins of the coronary arteries can also be identified.

39. 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.

40. 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.

41. 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.

42. 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.

43. TOF in Angiogram Aortic Arch Descending Aorta LPA Stump RPA MPA Descending branch of RPA Catheter Ascending Aorta

46. 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

47. 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.

48. 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

49. 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.

50. 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