Epidemiologi PJB 0,8-1% dari bayi lahir hidup 75% merupakan PJB non-sianotik PJB non-sianotik VSD : 20% dari semua PJB PDA : 7% dari semua PJB ASD : 8% dari semua PJB
Cont
PJB sianotik (25%)
TGA (transposition of Great Arteries) : 5% dari seluruh PJB. ( Lk : Pr = 3:1 ) TOF (tetralogy of Fallot) : 10% ( PJB sianotik terbanyak) Lain-lain ( Total anomalous PV return, Tricuspid atresia, Pulmonal atresia, dll ) berkisar 1-3%
Etiologi / Faktor risiko
Sebagian besar kasus tidak diketahui Obat-obatan Penyakit ibu Pajanan sinar X Genetik / sindrom tertentu Multifaktorial
Pathology of TOF Leftward deviation malalignment of ventricular septal defect + aortic overriding Anterior deviation pulmonary stenosis right ventricle outflow tract obstruction right ventricular hypertrophy
Bove EL, Lupinetti FM. Tetralogy of Fallot. Pediatric cardiac surgery. 1994.
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Pathology of TOF VSD in TOF is a perimembranous defect RV outflow tract obst is most frequenly infundibular stenosis The PA branches are usually small Right aortic arch is present in 25% of cases In about 5% abnormal coronary arteries are present 22
Manifestasi klinis Tergantung jenis PJB Sianotik / non-sianotik Gangguan tumbuh kembang ISPA berulang Cepat lelah Sesak Gagal jantung 23
Clinical Manifestation of TOF
Cyanotic of the skin and mucous membranes ToF desaturation of arterial blood increased concentration of reduced hemoglobin > 5g/dL in circulation Clinical manifestation depends on the source and volume of pulmonary blood flow ductus arteriosus and or aortopulmonary collaterals Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002. Kulkarni A, Pettersen M. Tetralogy of Fallot with pulmonary atresia. www.emedicine.com.24 Park MK. Cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002.
Clinical Manifestation
Newborn infant in whom the ductus arteriosus is the sole source of pulmonary blood flow increasingly cyanotic as the DA closes Severe pulmonary stenosis or pulmonary atresia cyanotic at birth or soon after birth ToF with severe PS or pulmonary atresia duct-dependent congenital heart defect
Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002. Kulkarni A, Pettersen M. Tetralogy of Fallot with pulmonary atresia. www.emedicine.com. 25 Park MK. Cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002.
Clinically : Most patient are symptomatic with cyanosis at birth or shortly thereafter dyspnea on exertion, squatting, or hypoxic spells develop later
Single 2nd HS, ejection systolic murmur
36
Hypoxic Spell Hypoxic spells may develop
before total repair
Increasing cyanosis Decreasing intensity of the heart murmur Hyperpnoea (rapid and deep) Severe spell convulsion, cerebrovascular accident death Park MK. Pathophysiology of cyanotic congenital heart defects. Pediatric cardiology for practitioners. 2002. Bove EL, Lupinetti FM. Tetralogy of Fallot. Pediatric cardiac surgery. 1994. 37
Atrial septal Defect
Clinical findings Asymptomatic A relatively slender body build is typical Auscultation : Normal 1st HS or loud Widely split and fixed 2nd HS Ejection systolic murmur
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Atrial Septal Defect
Auscultation :1st HS N or loud widely split and fixed 2nd HS Ejection Sistolic Murmur 39
Atrial Septal Defect
Sinus venosus defect
Primum ASD
Secundum ASD
Diagram of ASD 40
Atrial Septal Defect Chest X-Ray
Right atrial enlargement Prominence the MPA segment Increased pulmonary vascular marking 41
Ventricular Septal Defect
Clinical findings Day 1st after birth: murmur (-) After 2-6 weeks : murmur (+) Murmur : pansystolic grade 3/6 or higher at LSB 3 Small muscular defect: early systolic murmur Significant defect: Mid diastolic murmur at apex
42
Ventricular Septal Defect
Murmur: pansystolic grade 3/6 or higher at LSB 3
Small VSD
Large VSD
43
Ventricular septal Defect
Diagnosis Differential PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur
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Patent Ductus Arteriosus
Clinical findings Small defect: Symptom (-) Growth and development normal Significant defect: Decreased exercise tolerant Weigh gained not good Specific case: pulsus seler at 4th extremities and continuous murmur
Rujukan : Moss and Adams. Heart Diseases in Infant, Children, and Adolescents. Edisi-VII, Lippincot, 2008 Peter Koenig dkk, Essential Pediatric Cardiology. New York, 2004 Myung K Park, The Pediatric Cardiology for Practisioner, St Louis, 2003 John F Keane. Nadas Pediatric Cardiology. Philadelphia, Saunders. 2006
