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Cardio paedsDowns cardiac anomaly = endocardial cushion defect ecg = left axis deviation

Cause meiotic non dysjuntionRFErythema marginatum bathing suit area of distributionAschoff bodiesMcCallums plaque MC site = left atriumBread and butter pericarditisDilated Cardiomyopathy MC cause idiopathic. Drug causing it adriamycin

Myopathy with hundred percent cardiac involvement DMD

Hypertrophic Cardiomyopathy AD

MPS Restrictive Cardiomyopathy

Cardiomyopathy MC cause coxsackie

Viral MC cause of Myocarditis coxsackie B 5

MurmursCarey coombs Mitral delayed diastolic

Austin flint severe AR, apical low pitched

Graham steele PR murmurCole cecil murmur AR murmur heard in mid axillary area

Sea gull murmur high pitched musical AR murmur

Gibson murmur machinery murmur in PDADifferential cyanosis PDA with reversal

PDA common in females

Common in high altitudes ASD and PDAHypertrophic CardiomyopathySeen inFriedrichs Ataxia triple repeat disease

Pompes glycogen storage

Idiopathic chr 14 associatedEmbryological remnants in CVSSeptum secundum annulus ovalisSeptum primum fossa ovalisUmbilical arteries medial umbilical ligamentLeft umbilical vein ligamentum teres of liverDuctus venosus ligamentum venosumDuctus arteriosus ligamentum arteriosum Marfans- aortic dissectionMitral valve MC affected valveChromosome 15Fibrillin gene

Cyanosis 4 g/dl of unsaturated Hb required to see cyanosisLeft to right shunts extra volume load hyperdynamic praecordiumASDOstium secundum (MC) at fossa ovalis right axis deviation

Ostium primum inferior to fossa ovalis left axis deviation

widefixed splitting of S2 - extra blood return during inspiration gets equalized between the left and right atrium due to the communication that exists between the atria in individuals with ASD.Ejection murmur at P2

A sinus venosus ASD is a type of atrial septum defect in which the defect in the septum involves the venous inflow of either thesuperior vena cavaor theinferior vena cava.

Lutembachers- defined as the association of a congenital atrial septal defect and an acquired mitral stenosis.

Holt-Oram syndrome (hypoplastic or absent radii, 1st-degree heart block, ASD) or in families with secundum ASD and heart block.

VSD"the smaller the defect the larger the murmur produced"Maladie de Roger(Roger's disease), which is a smallcongenitalasymptomaticventricular septal defect(VSD)MC congenital cardiac disease complicated by IE

MC type of VSD membranousSupra cristal type- associated with ARQp:Qs ratio > 2:1 large VSD indication for Sx in children >2 years of ageSwiss cheese septum multiple defects in muscular VSD difficult to close surgicallyPalliative Sx for VSD pulmonary artery banding

Pansystolic (Holosystolic) murmur along lower left sternal border(depending upon the size of the defect) +/- palpable thrill (palpable turbulence of blood flow). Heart sounds are normal. Larger VSDs may cause a parasternal heave, a displaced apex beat (the palpable heartbeat moves laterally over time, as the heart enlarges). An infant with a large VSD will fail to thrive and become sweaty and tachypnoeic (breathe faster) with feeds.

P2 ejection systolicM1 delayed diastolicRx: closure with Dacron patch if large shunt, CCF in infancy or associated lesion (before 2 years) Small muscular VSD more likely to close than membranous VSD

Indications for Sx CHF in infancy not responding to medical mgmt, large L to R shunt, associated pulm stenosis, pulm HT or AR

Contra small VSD, PAH

PDAPhysiologic closure immediately; anatomic closure 1-3 months??Dilated ascending aortaPulmonary HT at birthCCF by 6 weeks

Delayed closure local release of PG, hypoxia, systemic hypotension, acidosis, immaturity, increased pulmonary pressure secondary to vasoconstriction

Ductus arteriosus becomes ligamentum arteriosum after obliterationDuctus venosus Ligamentum venosumRt and Lt umbilical artery medial umbilical ligamentLt umbilical vein ligamentum teres hepaticusAllantois urachus median umbilical cleftPGF 2 alpha high conc in intrauterine life keeps ductus open

Prostaglandin inhibitor stimulate closure of open ductusIndomethacin 0.1 mg/ kg every 12 hours * 3 dosesIbuprofen

In pulm atresia with intact ventricular septum infuse PGE 1 effective in keeping ductus arteriosus open, thus reducing hypoxemia and acidemia before surgery

In rubellaMore in females 2:1Common in premature infants patency is a result of hypoxia and immaturityClosure to prevent IESmall PDA closed with intravascular coilsModerate to large catheter induced sac

Continous machinery murmur best heard at 2nd left ICSLoud S1Frequent chest infProminent carotidHyperkinetic

Rx: indomethacin in first 2 weeks operative if diagnose late; risk increases after 15 years; inoperable once R to L shunt sets up due to pulm HT (differential cyanosis)

ToFMC cyanotic CHD in >2 years

Pulmonary Stenosis or RVOT (mostly infundibular level and rarely valvular level)RVHOverriding AortaVSD (outlet type)

Right sided aortic arch in 20 pcPentology = tetrology + ASDTriology = ASD + RVH + PSPink Fallot = no cyanosis clinically as pulmonary outlet obstruction minimalCour en sabot boot shaped heart in ToF

Severity of cyanosis proportional to severity of pulmonic stenosis

Intensity of systolic murmur inversely related to the severity of pulmonic stenosis

Anoxic spellsKnee chest positionHumidifed O2Morphine 0.1 to 0.2 mg/ kg scNa HCO3 to correct acidosisPropranolol 0.1mg/ kg during spellVasopressors methoxamine, phenylephrineCorrect anemia

Single S2 (A2) due to R to L shuntingLarge a wave Systolic P3 murmurTet spellsSquattingApneic spells on waking upclubbing

Mx:Blalock Taussigs shunt subclavian artery to ipsilateral pulmonary artery

Potts shunt descending aorta to left pulmonary arteryWaterstont shunt ascending aorta to right pulmonary artery

Tricuspid AtresiaTricuspid AtresiaBlood flows thru ASD/ VSD as Right Ventricle is hypoplastic or absent.since there is a lack of a right ventricle theremustbe a way to pump blood into thepulmonary arteries accomplished by aventricular septal defect(VSD).Complete mixing of bloodLV hypertrophy and LAD only CHD with LAD

Cyanotic from birthEnlarged liver cyanotic liverSquattingAnoxic spellsSicker than ToFEcg left axis deviation Single S2 (A2) large a wave4 years Fontan procedure

Glenn - Surgical anastomosis between the superior vena cava and the right main pulmonary artery to increase pulmonary blood flow as a correction for tricuspid atresia.

Fontan - A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit.Pulmonary atresiaGlenn procedure followed by a modified Fontan procedure Ebsteins AnomalyAtrialization of ventricle as tricuspid valve fused to wall of right ventricle Box shaped heartAssoc with tricuspid atresiaLithium use in 1st trimesterIntracavitary ECG is a Dx aid

CyanosisPalpitations and sudden cardiac deathSymptoms of right heart failure, such as edema and ascitesClubbingWide variable split S2Soft P2

Rx: Valvuloplasty

Transposition of the great vessels(TGV)is a group ofcongenitalheart defects(CHDs) involving an abnormal spatial arrangement of any of thegreat vessels:superiorand/orinferiorvenae cavae(SVC,IVC),pulmonary artery,pulmonary veins, andaorta.

CHDs involving only the primaryarteries(pulmonary artery and aorta) belong to a sub-group calledtransposition of the great arteries(TGA).

Common in IDM babies

MC lesion in cyanotic infant in newborn period. TGV- cyanosis noticed in first hour of life

ToF not present at birth. Cyanosis occurs late in the first year of birthTAPVC non obstructive type presents at 4-10 weeks; obstructive type at 1st week of lifePDA symptomatic early in life, CHF at 6-10 weeksVSD symptomatic 6-10 weeks of life

Egg on string appearance in TGV

Single S2Cyanotic at birthCCF in 1st weekRVHRecurrent RTI

Dextro-Transposition of the great arteriesIndextro-Transposition of the great arteries(dextro-TGA) deoxygenated blood from the right heart is pumped immediately through the aorta and circulated to the body and the heart itself, bypassing the lungs altogether, while the left heart pumps oxygenated blood continuously back into the lungs through the pulmonary artery. In effect, two separate "circular" (parallel) circulatory systems are created. It is called acyanoticcongenitalheart defect(CHD) because the newborn infant turns blue from lack of oxygen.

Levo-Transposition of the great arteriesLevo-Transposition of the great arteries is anacyanoticheart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery, and the morphological left and right ventricles are also transposed.Rx: Double switch procedure Inc risk with Maternal Diabetes Separate Circulations Need VSD (amount of shunt determines Generalized Cyanosis)

managementJatene arterial switch: Theaortaandpulmonary arteryare detached from theirnativerootsand reattached to the opposite root; thus, the pulmonary root becomes theneo-aorta, and the aortic root becomes the neo-pulmonary artery.