Lec. 2 - Congenital heart diseases (Part 2).pdf

Lec.40 Pediatrics Dr. Muneera Congenital heart diseases II Lec. 2 4th April. 2017 Done by: Zainab Abdul Ghany. 2016-2017 ‫مك...

Lec.40 Pediatrics Dr. Muneera Congenital heart diseases II Lec. 2 4th April. 2017 Done by: Zainab Abdul Ghany. 2016-2017 ‫مكتب آشور لالستنساخ‬ 0 Coarctation of Aorta Constriction that occur at any portion of aorta from transverse arch to the iliac bifurication.more in male afeatures of turner Pathophysiology: 2 types Juxtaductal (if mild adult type) 98% just below origin of lt subclavian art. Tubular hypoplastic (infantile type) If more severe juxtaductal or tubularhypoplastic blood pass through ductus arteriosus decsending aorta if closed diffrential cyanosis ( lower blue extremities and upper pink extremities ). In COA: High Blood pressure in area proximal to Coarctation (mechanical and hormonal ) Low Blood pressure distal to the coarctation. Development of collaterals from subclavian, internal mammary, superior intercostal. Clincal features If mild and Dx after infancy  rarely significant symptoms and most diagnosed by high blood pressure with routine physical examination. Signs: Pulses of UL and LL. Radio-femoral delay. Blood pressure in both UL and LL. Blood pressure in each arm. Ejection systolic click + thrill. Systolic murmur in 3rd, 4th Lt sternal border. Mid-diastolic murmur. Systolic murmur of aortic stenosis In neonatal period Lt body hypoperfusion, acidosis, HF. Before ductal closure  differential cynosis. 1 Diagnosis 1. CXR 2. ECG 3. ECHO 4. COLOR DOPPLER 5. CONTINOUS AND PULSED WAVE DOPPLER 6. CATHETRAIZATION + LT VENTRICULOGRAPHY 7. MRI used in dx due to difficulty in infancy Complications 1. Untreated  succumb at 20-40 yr. 2. Complications secondary to a. Premature coronary artery disease b. HF c. ICH d. Hypertensive encephalopathy 3. IE, Endarteritis 4. Aneurysms of the descending aorta or collaterals. 5. Neonates : hypoperfusion + HF Treatment 1. In neonate  PGE1  reopen ductus and relieve obstruction  stabilize him surgery. 2. Older children  HF  antifailure  surgery 3. Surgery: Excision and primary re-anastamosis Subclavian flap. Patch aortoplasty. 2 Pulmonary stenosis Pulmonary stenosis divided anatomically into: 1. Vulvular ( most common ).90% 2. Supravalvular. 3. Subvalvular. Pathophysiology Obstruction  Rt. Ventricular pressure during systole  wall stress  severe cases  RVH. Pulmonary art. Pressure normal or. Arterial O2 normal unless VSD or ASD. Critical pulmonic stenosis in neonate shunt at the foramen ovale Clinical features According to severity 1. Mild: asymptomatic , normal venous pressure , ejection click after 1st heart sound , 2nd heart sounds split , short ejection systolic murmur in pulmornary area. ECG: normal or mild RVH. CXR: poststenotic pulmonary arterial dilatation. ECHO: pr.gradient usually 10-30mmHg 2. Moderate : slightly elevated venous pr. , prominent a – wave in jugular pulse, 2nd heart sound split , ejection click, ejection systolic murmur CXR: normal or pulm.vascularity. ECG: RVH, spiked p-wave. ECHO: thickened valve , pr.gradient 30-60 mmHg.. 3. Severe: Rt.sided failure, hepatic enlargment, periphral edema , venous pr. a-wave , heart enlarged , inaudible pulmonary component of 2nd heart sound , ejection systolic murmur and thrill , no click. CXR: enlarged heart + pulmonary vascularity. ECG: RVH, spiked p-wave. ECHO: valve deformity, RVH.,pr.gradient ≥ 60 mmHg 3 Treatment 1. Mild reassurance. 2. Moderate or severe  balloon valvuloplasty. 3. Critical pulmonic stenosis  valvuloplasy or surgery.valvutomy Cyanotic Heart Diseases: Tetralogy of Fallot It is the commonest cyanotic heart disease in children , it’s a combination of :- 1. Right ventricular outflow obstruction(pulmonary stenosis) 2. VSD 3. Dextroposition of aorta with override of ventricular septum. 4. Right ventricular hypertrophy. Cyanotic Heart Diseases: RV outflow obstruction: various sites , but most common is infandibular site. VSD  large , non-obstructive. Overriding of aorta ( right sided in 20% ). 4 Clinical features Time of onset of symptoms Severity of cyanosis RV hypertrophy Depend of the degree of right ventricular outflow obstruction If mild  initially heart failure  with age , patient grows  infandibular hypertrophy cyanosis develops in 1st year of life. If severe obstruction  cyanosis develops immediately after birth. In older children , long standing  dusky blue skin, grey sclera, engorged blood vessels, clubing of fingers & extracardiac manifestation. Dyspnea on exertion  so they stop to take rest or have squatting position. Growth retardation ( if severe and untreated ). Delayed puberty. O/E Pulse normal Venous and arterial pressure normal. Heart size normal. Lt. hemithorax bulged because of RVH. Murmur , ejection systolic because of RV outflow obstruction. Murmur can be holosystolic due to VSD.☆ the most common murmur in TOF ☆ The intensity of murmur  during spells. Sometimes continuous murmur due to collaterals. Diagnosis 1. CXR : boat shaped heart , clear lung field , 20 % right sided aorta. 2. ECG : RVH , right axis deviation. 3. ECHO 4. Cardiac cathetarization. 5. Selective right ventriculography : important for child as surgical candidate. 6. Lt. ventriculography. 7. Coronary angiography. 5 Complications 1. Cyanotic spells 2. Cerebral thrombosis 3. Brain abcess 4. Infective endocarditis 5. Heart failure Cyanotic spell Usually at 4-6 mo. Patient restless , cyanosed , gasping , syncop follows. Mainly after awakening or vigorous exercise.  intensity of the murmur. Continued for few min.-few hrs. Followed by generalized weakness , sleep. Treatment of spell 1. Put him on abdomen , knee-chest position. 2. O2. 3. Morphine ( 0.2mg/kg s.c )  relaxe pulmonary infandibulum and sedate child. 4. Premature attempts to get blood sample  agitation. 5. If severe  NaHCO3 to correct acidosis. 6. If still resistant  phenylephrine or methoxantheme to  systemic vascular resistance and  Rt.  Lt shunt. Treatment Medical : 1. If severe obstruction  medical Rx until surgical intervention. 2. Include 3. Provide O2 , maintain body temperature. 4. Treat and prevent hypoglycemia. 5. Start PGE1 infusion 6. If less severe obstruction and while await for the surgery observe for the following: Rx dehydration. Rx iron deficiency anemia. Inderal 0.5-1 mg/kg 6hr. Phlebotomy if symptomatic patient and hematocrite > 65%. 6 Surgical Rx. 2 options : o Palliative o Corrective Time: 4-12 mo. Palliative surgery Modified Blalock – Taussing shunt , which a conduit from subclavian artery to homolateral branch of pulmonary art. or directly from ascending art. To main pulmonary artery. Response   cyanosis , continuous murmur heard from functioning anastomosis. With increasing age  need for more pulmonary blood flow  do corrective surgery or reanastomose on the opposite site Transposition of great vessels CCHD with  pulmonary blood flow. Either d-TGA or l-TGA. Either with intact VS or VSD. Male > female. 50% with VSD. For d- TGA  aorta arises from RV and pulmonary art. From LV. Aorta anterior and to the right of pulmonary art. C/F: cyanosis & tachycardia. HF less common if untreated  not survive neonatal period. Dx ECG  Rt. Sided dominance pattern. CXR  mild cardiomegaly. Hyperoxia test. ECHO. CATHETRIZATION. 7 Treatment Infusion of PGE1 Protect against hypothermia , Rx acidosis & hypoglycemia. If no response  Rashkind Balloon septostomy  then arterial switch operation ( Jantene operation ) within 2 wks. If TGA&VSD  do Rashkind operation. Extracardiac manifestation of CCHD. 1. Polycythemia. 2. Relative anemia. 3. CNS abscess. 4. CNS thromboembolic stroke. 5. Low grade DIC, thrombocytopenia. 6. Hemoptysis. 7. Gum disease. 8. Gout. 9. Arthropathy, COF. 10. Infection. 11. Pregnancy complications. 12. FTT. 13. Psychosocial problems. 8 TETRALOGY OF FALLOT (TOF) It is the commonest cyanotic congenital heart disease in children and adults It is a combination of: 1-VSD 2-Pulmonary stenosis (PS). 3-Overriding of aorta. 4-Right ventricular hypertrophy (RVH). HEMODYNAMICS: The degree of right ventricular outflow obstruction (i.e. P.S.) determines the degree of pulmonary blood flow and the severity of cyanosis. C/F: 1-Cyanosis: most patients develop cyanosis in the first 6 month - 1 year of life. 2-Dyspnoea: occurs in exertion, so they have a limited exercise tolerance. 3-Squatting: it is the characteristic posture of children with TOF after any physical effort. Q/E: 1-Cyanosis (central and peripheral). 2-Clubbing (usually after 3 months of age). 3-Single S2 & ejection systolic murmur at the left sternal border. 4-Normal pulse & quiet procardium. INVESTIGATION: ECG: Right axis deviation (RAD) & right ventricular hypertrophy CXR: 1-Normal heart size. 2-Rounded apex situated above the diaphragm (hypertrophied RV). 3-Concavity at the main pulmonary artery area. 4-Reduced pulmonary vascularity (oligemic lung). The classical radiological picture of TOF is called (boot shape). Echo: It is essential for Dx. Catheterization: Gives the definitive Dx and as a preoperative step. 9 COMPLICATIONS: 1-Hypercyanotic spells. 2-Cerebrovascular accidents (CVA). 3-Cerebral abscess. 4-Infective endocarditis. 5-Haematological complications include bleeding and thrombosis. All complications are essentially due to cyanosis & polycythemia. 6- Polycythemia is due to hypoxia which results from right to left shunt (as the right pressure grows higher than the left one). RX: 1. Medical: a. Rx of anemia by iron, sometimes considered as relative anemia if we find normal Hb level; also nutritional support. b. Phlebotomy: in symptomatic patients with hematocrit more than 65% (as there is a risk for CVA). 2. Surgical: a. Palliative to  pulmonary blood flow e.g.: Blalock-Taussig shunt (BT shunt). b. Total surgical repair. Indications for surgery: 1-Cyanosis 2-Hypecyanotic spells 3-Polycythemia 4-↓ exercise tolerance 5-Appropriate age and weight (usually between 2-3 years, if there is cyanosis). 10 HYPER CYANOTIC SPELLS: Attacks of increasing cyanosis associated with abnormal respiration and altered level of consciousness and it is an important cause of death in TOF patients (especially in mild cyanosis) Spells are particular problems during the 1st 2 years of life and is more common in the morning and can be precipitated by any activity. Most spells are self-limited, but should be considered as an indication for surgery. The cause is infundibular spasm. RX: 1-Knee-chest position. 2-O2. 3-Sodium bicarbonate to correct metabolic acidosis. 4-Morphine: 0.2 mg/Kg subcutaneously (s.c.) or i.v. can be repated 4 hourly. Thank you Edited by: Zainab Abdul Ghany. 11