Tetralogy of Fallot

Tetralogy of Fallot

Tetralogy of FallotPresented By:Rifqi Mahfuzh Al-maaarij 110100028Yogashree Rajendran 110100435Supervisor :Dr. Emil Azlin, Sp.A(K)

Pediatric DepartmentH. Adam Malik HospitalMedical Faculty of Sumatera Utara UniversityToF4 anatomic malformations:-Right Ventricular Hypertrophy-Pulmonary Valve Stenosis-Transposition of the aorta-Ventricular Septal Defect

ToFRVH -secondary to PA Stenosis -Increased P on RV leads to RVHTransposition of Aorta -aorta is displaced VSD -hole in the heart -mixing of oxygenated and unoxygenated blood -cyanosisPVS -more severe, less blood transported to the lungs and more deoxygenated blood will pass through VSD to aorta to be circulated throughout the bodyEpidemiologyThe Most Frequently cyanotic congenital abnormalities of heart3-6 : 10.000 live birth7-10% of all congenital cardiac malformationEtiologyTheory: destruction of the neuronal crest cells during embryogenesisIn the laboratory setting, destruction of these cells reproduced results displayed with certain cardiac malformations.Untreated maternal diabetes, PKU, and intake of retinoic acid.Chromosomal anomalies

Clinical PresentationClinical presentation is directly related to the degree of pulmonary stenosis. Ejection Sistolic MurmurSevere stenosis results in immediate cyanosis following birth. Mild stenosis will not present until later. Growth is retarded insufficient oxygen and nutrients

Tet SpellTet spells at 2-3yo , child becomes cyanotic, may experience syncope

Exams and TestsCBC - hematocritECG -RVH, RAD CXR -boot shaped heart, right sided aortic archEchocardiogramTo Confirm Diagnosis -VSD

Diagnosis Differential Pulmonary Atresia Double outlet right ventricle and pulmonary stenosis Transposisi of great arteri and pulmonary stenosis

Management Paliative treatment: Blalock-Taussig shunt Definitive: total correction

Case ReportObjective : The objective of this case is to report a case of 16 year old girl with a diagnosis of Tetralogy of Fallot. Case :SS,16 years old girl , with 52 kg came to emergency unit in Haji Adam Malik General Hospital Medan on 5th June 2015 at 19.30 WIB. Chief of complain was dyspnea with cyanosis in the perifer.

History of Disease : SS,16 years old girl , with 52 kg came to emergency unit in Haji Adam Malik General Hospital Medan on 5th June 2015 at 19.30 WIB. Chief of complain was dyspnea . Dyspnea was experienced by SS past 4 days and get worsen 1 day before addmitted in hospital. History of tiredness was founded since she was in 4SD class. She has a history of dyspnea since she was in 4 SD She takes a squat position when she has a lot of activity during the walk. Complain of dyspnea was irrelevant with change in wether and daily activities.There is cyanosis in patient and its been four days.History of cyanosis present since the patient in 4SD class. Cyanosis found on the finggertips, lip, and face.Recurrent fever was (+) in this past 4 days . High fever was not found .Shivering was not found .Seizure was not found.SS has a history of treatment in RSUPHAM while she was in 4SD and their parent doesnt remember the diagnosis of the patient and after this the patient was never treated again in RSUPHAM again. SS , was treated with Sp.PD with diagnosis narrow of the heart. History of Disease .History of Medication Aspilet,Digoxin,Spironolacton and Levofloxacin History of MedicationAspilet,Digoxin,Spironolacton,LevofloxacinFamily History : Her cousin has a congenital heart diseaseHistory of Pregnancy : The age of the mom was 38 years old during pregnancy with G3P3A0. The gestation age was 38 weeks. Her mom regularly control pregnancy. History of hypertension post partum was found. History of Diabetes Melitus was not found. Usage of drugs (-), Usage of herbs (-). History of birth : Birth assisted by obstetricians, baby was born by sectio cesarea. The baby immediately cry. Blue or cyanosis was not found. Birth body weight : 2700 gr, birth body length : 50 cm, and head circumference was unclear. History of breast feeding :Exclusive breastfeeding until 6 months. 06 JUNE 2015SDyspneu (+) , Fever (+) O S : Sensorium: alert; T: 37.8oC; BW: 52 kg, BH: 160 cm BB/U : 94% , TB/U :97% , BB/TB : 106% , Oedem (-) , Cyanosis (+) , Dyspnea (+)Head : Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra (-/-)Ear: within normal rangeNose: O2 Nasal canalMouth: cyanosis (+)Neck: lymph nodes enlargement (-)Thorax: symmetrical fusiform, retraction (-) HR: 105 bpm, reg, ejection sistolik murmur (+) grade III/6 left linea midclavicularis ICR II/IIIRR: 27 bpm, reg, rales (-/-)Abdominal: soft, tender, peristaltic (+) N, Liver and Spleen not palpableExtremities: pulse 105 bpm, reg, p/v adequate, warm acral, CRT < 3, fingertip cyanosis (+)AA: DD / Tetralogy of Fallot -Transposition of Great Arteries (TGA)-DORV

POxygen Nasal Canul 1-2 L/MenitInjection Ampicillin 1 gram / 6 hours/IV

BB/U :94%TB/U:97%BB/TB:106%HematologyDiftelHGB 16g% (11.3-14.1)RBC 50.86/mm3 (4.40-4.48)WBC 14.47103/mm3 (4.5-13.5)Ht 50.60% (37-41)PLT 339 103/mm3 (150-450)MCV 78.10 fl (81-95)MCH 27.00 pg (25-29)MCHC 34.60 g% (29-31)RDW 15.70% (11.6-14.8)

Neutrophil 15.70% (37-80)Lymphocyte 68.30% (20-40)Monocyte 11.50% (2-8)Eosinophil 1.50% (1-6)Basophil 3.000% (0-1)Absolute neutrophil 2.26 103 /L (2.4-7.3)Absolute lymphocyte 9.88 103/ L (1.7-5.1)Absolute monocyte 1.67 103/ L (0.2-0.6)Absolute eosinophil 0.22 103/ L (0.1-0,3)Absolute basophil 0.44 103/ L (0-0.1)

07 JUNE2015SDyspnea (+), Oedema (-), fever (-),OSensorium: alert; T: 36. 7oC; BW: 52 kg, BH: 160 cm , BB/U : 94% , TB/U :97% , BB/TB : 100% , Oedem (-) , Cyanosis (+) , Dyspnea (+)Head : Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra (-/-)Ear: within normal rangeNose: Oxygen Nasal Canul Mouth: cyanosis (-)Neck: lymph nodes enlargement (-)Thorax: symmetrical fusiform, retraction (-) HR: 100 bpm, reg, ejection sistolic murmur grade III/6 mid clavicularis line ICR II/IIIRR: 28 bpm, reg, rales (-/-)Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpableExtremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT < 3A-TOFPO2 Nasal Canul 1-2 L/Menit Injection Ampicillin 1 gr / 6 hour / IVDiet MB 2500 kkal with 100 gr ProteinEchocardiography-Overiding aorta -Dilated RA-RV-Pulmonary Stenosis-Right aortic arch-No Pericardial Effusion-Well contracting Ventricle-VSD -Right to Left Shunt

Coclusion :-TOFElectrocardiography : Sinus Tachycardia, QRS Rate: 103 x/m, Axis: RAD, P Wave : P Mitral (RAE +) , PR Interval : 0,16s, QRS duration :0,08s, QRS Changes : -, ST-T Changes; -, RVH : + (R>S in V1, Persistent S wave in V5, V6), LVH: -, VES: -. Conclusion : Sinus Tachycardia, Right Axis Deviation, Right Atrial Enlargement, Right Ventricle Hiperthropy

08 JUNE 2015SS: Dyspneu (+) , Oedema (-), fever (-)OO : Sensorium : alert, T:370CHead : Eye: light reflex (+/+), isochoric pupil, superior conjunctiva palpebra edema (-/-), pale inferior conjunctiva palpebra (-/-)Ear: within normal rangeNose: within normal range Mouth: cyanosis Neck: lymph nodes enlargement (-)Thorax: symmetrical fusiform, retraction (-) HR: 104 bpm, reg, ejection sistolic murmur (+) grade III/6 mid clavicularis line ICR II/IIIRR: 27 bpm, reg, rales (-/-)Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpableExtremities: pulse 110 bpm, reg, p/v adequate, warm acral, CRT < 3ATetralogy of FallotPO2 nasal kanul 1-2 l/iInj.Ampicillin 1 gr/6jam/iv (H4)Diet MB 2000 kkal dengan 100 gr protein09 JUNE 2015SDyspneu (+) , fever (-) OSensorium : alert, T:36,80C BB:52kgHead : Eye: light reflex (+/+), isochoric pupil, superior conjunctiva palpebra edema (-/-), pale inferior conjunctiva palpebra (-/-)Ear: within normal rangeNose: O2 Nasal Canul Mouth: within normal rangeNeck: lymph nodes enlargement (-)Thorax: symmetrical fusiform, retraction (-) HR: 100 bpm, reg, ejection sistolic murmur (+) grade III/6 mid clavicularis line ICR II/IIIRR: 24 bpm, reg, rales (-/-)Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpableExtremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT < 3 110 bpm, reg, p/v adequate, warm acral, CRT < 3ATetralogy of FallotP-Oxygen O2 Nasal Canul 1- 2 l/i-Inj.Ampicilin 1gr/6jam/iv-Diet MB 2000 kkal dengan 100 gr protein 10 JUNE 2015SDyspneu (-) , fever (-) OSensorium : alert, T:36,80C BB:52kgHead : Eye: light reflex (+/+), isochoric pupil, superior conjunctiva palpebra edema (-/-), pale inferior conjunctiva palpebra (-/-)Ear: within normal rangeNose: O2 Nasal Canul Mouth: within normal rangeNeck: lymph nodes enlargement (-)Thorax: symmetrical fusiform, retraction (-) HR: 100 bpm, reg, ejection sistolic murmur (+) grade III/6 mid clavicularis line ICR II/IIIRR: 20 bpm, reg, rales (-/-)Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpableExtremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT < 3 110 bpm, reg, p/v adequate, warm acral, CRT < 3ATetralogy of FallotP-Oxygen O2 Nasal Canul 1- 2 l/i-Inj.Ampicillin 1gr/6jam/iv (H1)-Diet MB 2000 kkal dengan 100 gr protein DiscussionTheoryCaseThe etilogy of Tetralogy Of Fallot is multifactorial , but reported associations include untreated maternal diabetes , phenyketonuria, and intake of retinoic acid.Associated chromosomal anomalies can include trisomies 21, 18,and 13 but resence experience points to much frequent association of microdeletions of chromosome 22.The risk of recurence in families is 3%The patient has genetic risk that is where both families have a history of similar disease.TheoryCaseFrom the echocardiography result for Tetralogy Fallot case there is present with 4 features:1.VSD2.Pulmonary stenosis3.Right to left shunt4.Overiding aorta1.Overiding aorta 2.Dilated RA-RV3.Pulmonary Stenosis4.Right aortic arch5.MPCA (+)6.No Pericardial Effusion7.Well contracting Ventricle8.VSD 9.Right to Left Shunt

Coclusion :-TOF

TheoryCaseCyanosis of the lips and nail bed is usualy pronaounced at birth , after age 3-6months, the finger and toes shows clubbing.A sistolic thrill is usually present anteriorly along the left sternal border.Cyanosis was found in the perifer.Ejection sistolic murmur (+) grade III / 6 LMCS ICR II/IIITheoryCaseChest x-rays showed generally the size of the heart is enlarged .Heart shape generally will show a picture like boot shaped and decreased pulmonary vascularity.The results of chest x-ray shows the size of the heart is enlarged by the CTR of > 50% and found a picture like boot shaped

SS,16 years old girl , with 52 kg came to emergency unit in Haji Adam Malik General Hospital Medan on 5th June 2015 at 19.30 WIB. Chief of complain was dyspnea .Dyspnea was experienced by SS past 4 days before. Complain of dyspnea was irrelevant with change in weather and daily activities. According to anamnesis , physical examination and echocardiography assesment he was diagnosed with Tetralogy of Fallot .She was treated with Oxygen O2 Nasal Canul 1- 2 l/I Inj , Ampicillin 1gr/6jam/iv , Diet MB 2000 kkal dengan 100 gr protein