Congenital Heart Disease: Notes. Condition Pathology PC Ix Rx Ventricular septal defect (VSD) L R shuntsdefect anywhere in the ventricle, usually perimembranous (next to the tricuspid valve) 30% 1)small VSD < aortic valve 2) > aortic valve -L sternal edge thrill -Loud PSM -SOB Failure to thrive (after 1wk) Recurrent chest infections -active pericardium -+/- soft PSM -apical MDM (inc flow from mitral valve) -loud P2 -inc RR, HR, enlarged liver from HF - normal -normal - precise anatomy and severity -cardiomegaly -enlarged pulmonary vessels and markings -Pulmonary oedema - Biventricular hypertrophy by age 2m and signs of pulmonary HTN -precise anatomy and severity Most close spontaneously. ABX prophylaxis for dental extraction controversial (see NICE) For HF- diuretic, captopril, inc calorie. Pulmonary HTN leading to Eisenmengers is the major complication by 2yo, so surgery is done 2-6m to avoid HF and pulmonary HTN. Eisenmengers- R-L shunt due to long standing pulmonary HTN 1
Persistent ductus arteriosus (PDA) Atrial septal defect (ASD) L R shunts Blood flow from aortapulmonary a. L R shunts Two types 1)secundum 80% (defect in the centre of the atrial septum involving the foreamen ovale) (F:M- 2:1) 2)partial atrioventricular septal defect (ostium primum): inter atrial communication or abnormal AV valves (regurgitatant) (DS more common) -pulmonary HTN -continuous murmur under L clavicle and cont in diastole -collapsing pulse or bounding pulse (wide pulse pressure) -recurrent chest infection/wheeze -Arrhythmia -split widely spaced II HS -ESM (L sternal edge because of inc flow from L-R V) -partial ASVD- apical pansystolic murmur from AV valve regurgitation - normal -normal If large and symptomatic then results of above similar to VSD - reveals duct -cardiomegaly -large pulmonary artery -inc pulmonary vascular markings -non specific features secundum ASD -partial RBBB -R axis deviation (RVH) Partial AVSD -L avis deviation (superior QRS axis) this is because the AN node is displaced. Closure with coil or occlusion devise via cardiac catheterisation at 1yo. Secundum ASD- Cardiac catheterisation Partial AVSD- Surgical correction RX- 3-5 yo to prevent HF and arrhythmias Cross sectioned -gold standard (good anatomy) 2
Aortic stenosis (AS) Valve leaflets are partly fused giving restrictive outflow. Often in combo with mitral valve stenosis and coarctation of aorta. More common in Turners Syndrome -dec ET (if severe) -CP on exertion + Syncope Neonatal period- HF or duct dependent systemic circulation. murmur -small vol rising pulse -Carotid thrill (always) -ESM (R sternal edge) radiating to neck -delayed and soft aortic A2 -Apical ejection click - normal or prominent LVH with post stenotic dilation of the ascending aorta - LVH Regular -Balloon valvotomysymptomatic pts -neonates and children with significant disease need valve replacement Pulmonary stenosis (PS) Valves leaflets partially fused giving RV restricted outflow. (More common in Noonans syndrome) -small number of neonates have duct dependent pulmonary circulation -ESM L sternal edge +/- thrill -soft or absent P2 -severe lesion- prolonged R V impulse with delayed pulmonary valve closure on auscultation. *no carotid thrill - normal or post stenotic dilatation of pulmonary artery. - RVH Transcatheter balloon dilatation 3
Coarctation of the aorta (adult type) Tetralogy of Fallot Not duct dependent becomes severe over the years Preductal- While the PDA is open the right ventricle can maintain cardiac output. Post ductal Dec pulmonary outflow with R-L shunt Most common cause of cyanotic hear disease 4 features -overriding aorta (on V) -large VSD -sub-pulmonary stenosis (R outflow obstruction) -RVH (due to above) Most diagnosed antenatally or after birth Pre ductal No murmur while PDA is open. On closure HF and ESM Post ductal -Systemic HTN in arms esp right arm -ESM R sternal edge -collaterals at the back -Radio-femoral delay (due to blood bypassing the obstruction via collaterals in the chest) -severe cyanosis -hypercyanotic spells on exercise leading to squatting (late infancy, now rare. Rapid onset cyanosis, inconsolable cry, irritability, SOB + pallor due to tissue lactic acidosis) -hypercyanotic spells MI or CVA -clubbing -Harsh ESM L sternal edge from day 1 + A2 (pulmonary stenosis) -rib notching (collateral intercostal arteries) - 3 sign- notch in the descending aorta -LVH -Usually normal -older child- small heart with up tilting apex due to RVH. Pulmonary artery bay - concavity of L heart border, dec pulmonary vascular marking and blood flow. Boot Heart Normal at birth Later-RVH - Show key features but catheterisation maybe need for detailed anatomy Pre-ductal- Prostoglandin to keep PDA open while awaiting surgery. Post ductal- Stent via cardiac catheter or surgery Initial management is medical -Bicarbonate -Morphine -Oxygen Corrective surgery at 6m -close VSD -relieve RV outflow neonates require shunt to increase pulmonary blood flow 4
Transposition of the great arteries Atrioventricular septal defects (complete) Aorta connected to the RV Pulmonary artery connected to the LV. Oxygenated blood is returned to the lungs and deoxygenated blood returned to the body. Two parallel circulations that have to have mixing to be compatible with life. Mixing can occur naturally with associated VSD, ASD and PDA. Commonly seen in Downs. Defect in the middle of the heart, with 1 5 leaflet valve between the A and V and stretches between the whole AV junction and leaks. In addition there is pulmonary HTN -cyanosis (1-2d of life with ductal closure and dec mixing of blood but less severe if ASD or VSD) -cyanosis -clubbing >1yr No murmur usually but systolic murmur is possible from inc flow from ventricle or stenosis /signs -cyanosis at birth or HF at 2-3wks Classic- narrow upper mediastinum with an egg on either side, appearance of cardiac shadow and inc pulmonary vascular markings. -usually normal -essential to demonstrate abnormality. Antenatal US - superior axis - in cyanotic baby, improve mixing of blood by maintaining patent ductus arteriosus with PG infusion. -Balloon atrial septostomy is life saving (it opens the foreamen ovale to allow for blood mixing) -Atrial switch procedure surgery after the 1 st few days of life. + coronary arteries have to be transferred to the new aorta. Treat HF medically Surgical repair after 3-6m 5