ATRIAL SEPTAL DEFECT.• 2 common types – Ostium secundum defect:midseptum.(common,defect in foramen ovale);Usually presents in adult life,Spontaneous closure is unlikely,Need Sx – Ostium primum defect:low in the septum.(Usually presents in first year) associated with other endocardial cushion defects (cleft AV valves, inlet type VSD• Pathophysiology: – L-R shunt-increased flow across Rt heart-RV & PA enlargement.• Clinical features: – Asymptomatic – slow wt gain(FTT) – frequent LRTI,No risk of infective endocarditis in ostium secondum ASD• Diagnosis: – Right ventricular heave(Due to RVH) – Soft long ejection systolic murmur due to increased blood flow across the pulmonary valve at 3rd IC space – fixed wide split S2,large ASDincreased blood flow across tricupid valve Mid diastolic murmur
Auscultation in ASD •Increased flow across the pulmonary valve produces a systolic ejection murmur and fixed splitting of the second heart sound •Increased flow across the TV produces a diastolic rumble at the mid to lower right sternal border.
Investigations:• CXR: – enlarged heart – Enlarged PA – increased pulmonary vascular markings,Central plethra• ECG: – Right axis in secundum defect – hallmark of primum defect is extreme Left axis deviation – RVH,RBBB• ECHO:RVH,valve anatomy,flow direction.• Treatment:(indicated if symptoms+,RV overload) – Device closure during cardiac cathetrization – surgical closure.
ASD: Therapy• Percutaneous Closure – only for secundum (contra in others) – adequate superior/inferior rim around ASD – no R-L shunting• Surgical Closure – Good prognosis: • closure age < 25, PA pressure <40 • If >25 or PA>40, decreased survival due to CHF, stroke, and afib
VENTRICULAR SEPTAL DEFECT.• Most common CHD (32%),Often one component of another more complex congenital heart lesion.• Pathophysiology: – Lt-Rt shunt as long as pulmonary vascular resistance is lower than systemic resistance,if reverse shunt reverses.• Large defects lead to pul.hypertension-Eissenmenger syndrome.• Clinical features: depend on size of the defect – asymptomatic – growth failure – recurrent LRTI – congestive heart failure – SOB,cyanosis(Eissenmenger),Risk of infective endocarditis+• Diagnosis: – parasternal thrill – pansystolic murmur at lower left sternal edge(Loud if small defect,if large VSD increase flow across pulmonary valve ejection systolic murmur – loud p2.(Pulmonary HT)
MANGEMENT OF VSD• Majority close spontaneously before 1 year of age;less than 10% require surgery.• 2 types of VSD – Perimembranous(90%) – Muscular(More likely close spontaneously)• Treatment: – Surgical closure before pulmonary vascular changes become irreversible.(if symptoms + like FTT,Features of Pul HT Loud P2,RVH) – Endocarditis prophylaxis – Heart failure Mx:ACEI,digoxin,diuretics.
Eisenmenger’s Syndrome• Final common pathway for all significant LR shunting in which unrestricted pulmonary blood flow leads to pulmonary vaso-occlusive disease (PVOD); RL shunting/cyanosis devleops• Generally need Qp:Qs >2:1
Eisenmenger: Treatment• Sxs +polycythemia phlebotomy – Careful if microcytosis, strongest predictor of cerebrovascular events• RULE OUT CORRECTABLE DISEASE• Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures• Diuretics prn, oxygen• Definitive: Heart Lung transplant – Prostacyclin therapy may delay, expensive
PATENT DUCTUS ARTERIOSUS.• Connection between PA & descending aorta,Common in preterm• Pathophysiology: – Lt-Rt shunt,reverses if pulmonary resistance increases-RV enlargement.If PDA is large Eissenmenger syndrome can develop.• Clinical features: – depend on size & direction of flow – slow growth,LRTI,SOB,cyanosis.• Diagnosis: – bounding pulse – continous murmur/Machinery murmur – loud S2.(Pul HT)
Investigations• CXR:cardiomegaly,increased pul vascularity.• ECG:Lt or biventricular hypertrophy.• ECHO:2D visualises PDA,doppler shows turbulance.• Cardiac catheter:PA pressures & O2 sats.• Treatment: – Endocardial prophylaxis as long as patent – Indomethacin:a prostaglandin E1 inhibitor may close a PDA.• Surgical:ligation /coil/clipping/division
Coarctation of Aorta• Narrowing in proximal descending aorta usually just beyond the origin of Left subclavian artery.• May be long/tubular but most commonly discrete ridge• Blood flow to the lower body maintained through collateral vessels• 98% of all coarctations at segment of aorta adjacent to ductus arteriosus.• Natural hx: – poor prognosis if unrepaired – High BP in UL & Low BP in LL – Systemic HypertensionLVF,Aortic Aneurysm/dissection,ICH – murmur (continuous or systolic murmur heard in back or SEM/ejection click of bicuspid AV) – weak/delayed LL pulses – Rib notching on CXR is pathognomonic• Associated with – Turner’s syndrome – Subarachinoid haemorrhage
Coarctation Repair• Surgical correction 1) Patch aortoplasty with removal of segment and end to end anastomosis or subclavian flap repair 2) bypass tube grafting around segment
Pulmonary Stenosis• No symptoms in mild or moderately severe lesions.• Cyanosis and RVH, right-sided heart failure in patients with severe lesions.• High pitched systolic ejection murmur maximal in second left interspace.• Ejection click often present.• Oligaemic lung fields(Reduced pulmonary vascular marking)
Valvular Aortic Stenosis• Most common type, usually asymptomatic in children.• May cause severe heart failure in infants.• Prominent left ventricular impulse, narrow pulse pressure.• Harsh systolic murmur and thrill along left sternal border, systolic ejection click.
Duct dependent Heart disease• Some babies with CHD will depend on the circulation through PDA ,when duct close they become critically ill.• Causes – R/S:TA,PA,Critical PS – L/S:COA,Critical AS,Hypoplastic left heart disease – TPGV• Treatment – Prostaglandin infusion keep the duct open.
Which of the following are non cyanotic heart disease?A. ASDB. Pulmonary atresiaC. Large VSDD. Truncus arteriosisE. Aortic stenosis
T/F ASD?A. Ostium Primum type is the commonestB. Ostium secondum type gets infective endocarditisC. Children are usually symptomatic during early childhoodD. Is most common congenital heart disease occurs in RubellaE. Usually close spontaneouslyF. Is the commonest acyanotic heart disease
T/F regarding ASD?A. Associated with RBBBB. Cause parasternal heave indicates pulmonary hypertensionC. Associated with recurrent respiratory tract infectionD. Murmur is due to left to right flow throw the defectE. Cause variable split in second heart soundF. Is rare in adults
T/F VSD?A.Perimembrous type is commonerB. Never cause infective endocarditisC. Loudness of murmur is proportional to the severityD.Usually close spontaneouslyE. Cause left ventricular hypertrophy.
T/F regarding VSD?A. Cause pansystolic murmur that is best heard at left lower sternal edgeB. Right to left shunt occurs in uncomplicated VSDC. Soft S2 is heard if there is a pulmonary hypertensionD. Occurs in Down syndromeE. Recurrent LRTI is due to pulmonary congestion
T/F PDA?A. Associated with congenital rubellaB. Cause small volume pulseC. Is an indication for the antibiotic prophylaxis against infective endocarditisD. If left untreated cause pulmonary hypertensionE. In a full term baby is likely to close.
T/F regarding PDA?A. Is a acyanotic heart diseaseB. Cause plethoric lung field in CXRC. Common in premature babiesD. May be seen in babies with cyanotic heart diseaseE. May cause heart failure
T/F large uncomplicated PDA is associated with ?A. CyanosisB. ClubbingC. Normal P2D. Wide pulse pressureE. Recurrent LRTI
T/F COA?A. Cause hypertensionB. Cause systolic murmur at the inter scapular areaC. Cause bounding femoral pulseD. Associated with Turners syndromeE. Rib notching seen in CXR
T/F Patent ductus arteriosus?A. Is a feature of congenital rubellaB. In a full term ,baby is likely to close spontaneouslyC. Associated with small volume pulse.D. Is an indication for antibiotic prophylaxis against infective endocarditisE. Loud P2 indicates pulmonary hypertension
T/F which of the following are thecomplications of the left to right shunt,A. Recurrent LRTIB. Cerebral abscessC. Pulmonary hypertensionD. CCFE. Hypercyanotic episodes