CYANOTIC CONGENITAL HEART DISEASE: DR. K. L. BARIK. ASST. PROFESOR, DEPT. OF PEDIATRICS, BURDWAN MEDICAL COLLEGE. Congenital heart diseases produce cyanosis: Tetralogy of Fallot (TOF). Transposition of great arteries (TGA). i) Complete TGA. ii) Corrected TGA. Total anomalous pul. venous return (TAPVR). Tricuspid atresia. Ebstein’ anomaly Single ventricle. Double outlet rt. ventricle. Persistent truncus arteriosus. Consequences and complications of cyanosis: Polycythemia:i) Low o2- erytropoetin incrd. ii) Hematocrit >65%.iii) Anemia Cubbing: i) R –L shunt. ii) PDGF. iii) TGF-B. Hypoxic spell : Rapid and deep breathing, inc. cyanosis, limpnesssp.posture – squatting. CNS complication: i) Brain absess- >2yrs. Viscosity-hypoxiamicroinfarct.ii) vascular stroke-<2yrs.paradoxcal emboi.& anemia. Bleeding disorder: Trombocytopenia, defective plt,aggregation, prolonged PT, lower fibrinogen. Depressed IQ: Chronic hypoxia and cyanosis Scoliosis: Girls with TOF. Hyperuricemia and gout: Older pt. with cyanotic heart disease TETRALOGY OF FALLOT (TOF): Most common cyanotic CHD – 10% to 17%. 4 abnormalities originally described by Falloti) Large VSD. ii) RV outflow tract obstruction (pulmonic st.) iii) RVH. iv) Overriding of the aorta. In actuality two abnormalities are required: i) VSD and ii) RV outflow tract obstruction. TOF CONT. : VSD of TOF- perimembranous subpulmonary. RV outflow tract obstructioni) Infundibular stenosis – 45% ii) Valvular stenosis – 10% iii) Combination of the two – 30% iv) Pulmonary valve atresia – 15% Pulmonary annulus & main PA – hypoplastic. Right sided aortic arch – 25%. Abnormal coronary artery – 5%. Ant. decending branch from right coronary artery. HEMODYNAMICS: Pulmonic stenosis- concentric RV hypertrophy without enlargement – increase RV pressure. R to L shunt – silent- insignificant pressure difference Ejection systolic murmur – pulmonic stenosis. Cyanosis – directly proportional to the stenosis. Murmur – inversely proportional to stenosis. RV effectively decompressed – no CCF except i)anemia ii)endocarditis iii)hypertn.iv)myocard v)AR. P2 delayed-soft-post.-only A2 – ant.- single S2 . CLINICAL MANIFESTATIONS: HISTORY: Symptomatic with cyanosis at birth mostly or later. Dyspnea on exertion & exercise intolerance. Squatting in hypoxic spell – noted commonly in TOF Infant with acyanotic TOF - may be asymptomatic. Severe cyanosis at birth –TOF with pulmonary atresia Hypoxic spell- hyperpnea, irritability, crying, cyanosis, convulsion – morning after crying, feeding, defecation. Starts 2 to 4 months of age. CLINICAL MANIFESTATIONS CONT.: PHYSICAL EXAMINATION: Varying degree- cyanosis, tachypnea, clubbing RV tap – lt. sternal border- parasternal impulse Systolic thrill – at ULSB & MLSB -50%. Ejection systolic murmur (gr. 3-5/6)-ulsb/mlsb. Single S2 – only aortic component. Deeply cyanotic pt.- absent or soft murmur. In acyanotic- long syst. mur.on entire lsb -VSD&PS INVESTIGATIONS: ECG: i) RAD with RVH. ii) CVH may be seen in acyanotic TOF. iii) RAH is occasionally present. P pulmonale. X-Ray Studies: i) Heart size normal/smaller than normal. ii) Decreased BVM. Black lung field- pul.atre.&TOF iii)Concave PA with upturned apex-boot-shaped heart iv) Rt. sided aortic arch – 30% cases. INVESTIGATIONS CONT.: ECHOCARDIOGRAPHY: 2D & Doppler. i) Large, perimembranous infundibular VSD. ii) Overriding of aorta. iii) Anatomy of RVOT, Pul.valve, PA& branch. iv) Pressure gradient across the obstruction. v) Anomalous coronary artery distribution. vi) Aortic – mitral valve continuity. vii) RV hypertrophy. NATURAL HISTORY: Acyanotic TOF become cyanotic. Cyanotic pt. become more cyanotic. Polycythemia – secondary to cyanosis. Development of iron-deficiency anemia. Hypoxic spell may develop in infants. Growth retardation. Brain abscess and CVA. SABE –occasional complication. Coagulopathy – late complication of cyanosis. HYPOXIC SPELL: Cyanotic spell/ tet spell/ hypercyanotic spell. Young infant with TOF. Hyperpnea, worsening cyanosis, disapp. of murmur. Crying, feeding, defecation, ph.activity-SVR decrd. Large R to L shunt – initiates vicious circle Fall of Po2, increase Pco2 and fall in pH. Hyperpnea – negative thoracic pump. Increase venous return to RV. Again increase R to L shunt and establish the circle. HYPOXIC SPELL CONT.: Vicious circleR to L shunt due to Decreased pO2& decreased SVR. pH, increased pCO2. Increased syst. Venous return. Hyperpnea FALLOT’S PHYSIOLOGY: Conditions clinically almost identical symptoms1. Complete TGA with VSD & pulmonic stenosis. 2. Corrected TGA with VSD & pulmonic stenosis. 3. Double outlet right ventricle with VSD & pulmonic stenosis. 4. Tricuspid atresia with diminished pulmonary blood flow. 5. Single ventricle with pulmonic stenosis. MANAGEMENT: 1. 2. 3. 4. 5. 6. 7. MEDICAL: Management of Hypoxic spellTreatment principles – to break the vicious circle:Knee – chest position, - increase SVR & decrease ven.return Morphine sulfate, 0.2mg/kg,sub-cut/ i.m.- suppress respiratory center, decreased hyperpnea. Oxygen – decrease hypoxia. Sodibicarb, 1mEq/kg, iv – correct acidosis. Vesoconstrictors – phenylephrine, 0.02mg/kg iv.- increase SVR. Ketamine, 1-3 mg/kg iv over 60 seconds,- increase SVR and sedation. Propranolol, 0.01- 0.25mg/kg slow iv – reduce HR. MANAGEMENT CONT.: MEDICAL:Management of complications. Correction of anemia. Oral propranolol, 0.5-1.5mg/kg 6hrly as prophylaxis for hypoxic spell. Balloon dilation – RVOT & pulmonary valve. Dental hygiene & antibiotics against SABE. Control of infections. MANAGEMENT CONT.: 1. 2. 3. 4. 5. SURGICAL:- A) Palliative Shunt procedures- to increase PBF & reduce cyanosis – INDICATIONS:Neonates with TOF & pulmonary atresia. Infants with hypoplastic pulmonary annulus. Children with hypoplastic PAs. Severely cyanotic infants < 3 months of age. Medically unmanageable hypoxic spells. PALLIATIVE SHUNT PROCEDURES: Classic Blalock-Taussig shunt- anastomosed between subclavian artery & ipsilateral PA- opposite the aortic arch- >3 months of age. Gore-Tex (modified Blalock-Taussig)- interposition shunt – between subclavian artery & ipsilateral PA – same side of aortic arch - <3months of age. Waterston shunt – between ascending aorta and right PA. Potts shunt – between descending aorta & left PA. CONVENTIONAL REPAIR SURGERY: Indications and Timing:Symptomatic infants with favorable anatomy. Asymptomatic and minimally cyanotic pt. Total correction in previously shunt surgery pt. after 1-2yrs. Asymptomatic & acyanotic TOF- 1-2 yrs. PROCEDURES:- Patch closure of VSD, widening of RVOT under cardiopulmonary bypass.