Cardiology Paediatric

Cardio paeds

• Down’s – cardiac anomaly = endocardial cushion defect  ecg = left axis deviation • Cause – meiotic non dysjuntion

RF • Erythema marginatum – bathing suit area of distribution • Aschoff bodies • McCallum’s plaque – MC site = left atrium • Bread and butter pericarditis

• Dilated 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

Murmurs • Carey coombs – Mitral delayed diastolic • Austin flint – severe AR, apical low pitched • Graham steele – PR murmur

• Cole cecil murmur – AR murmur heard in mid axillary area • Sea gull murmur – high pitched musical AR murmur • Gibson murmur – machinery murmur in PDA

• Differential cyanosis – PDA with reversal • PDA common in females • Common in high altitudes – ASD and PDA

Hypertrophic Cardiomyopathy Seen in • Friedrich’s Ataxia – triple repeat disease • Pompe’s – glycogen storage • Idiopathic – chr 14 associated

Embryological remnants in CVS • Septum secundum – annulus ovalis • Septum primum – fossa ovalis • Umbilical arteries – medial umbilical ligament • Left umbilical vein – ligamentum teres of liver • Ductus venosus – ligamentum venosum • Ductus arteriosus – ligamentum arteriosum

Marfan’s • • • •

- aortic dissection Mitral valve – MC affected valve Chromosome 15 Fibrillin gene

• Cyanosis – 4 g/dl of unsaturated Hb required to see cyanosis • Left to right shunts – extra volume load  hyperdynamic praecordium

ASD • Ostium secundum (MC) – at fossa ovalis  right axis deviation • Ostium primum – inferior to fossa ovalis  left axis deviation

• wide fixed 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 the superior vena cava or the  inferior vena cava. • Lutembacher’s- 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 small congenital  asymptomatic ventricular septal defect (VSD) • MC congenital cardiac disease complicated by IE

• MC type of VSD – membranous • Supra cristal type- associated with AR • Qp:Qs ratio > 2:1  large VSD  indication for Sx in children >2 years of age • Swiss cheese septum – multiple defects in muscular VSD – difficult to close surgically • Palliative Sx for VSD – pulmonary

• 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 systolic • M1 delayed diastolic • Rx: 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

PDA • Physiologic closure immediately; anatomic closure 1-3 months?? • Dilated ascending aorta • Pulmonary HT at birth • CCF 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 obliteration • Ductus venosus  Ligamentum venosum • Rt and Lt umbilical artery  medial umbilical ligament • Lt umbilical vein  ligamentum teres hepaticus • Allantois urachus  median umbilical cleft

• PGF 2 alpha – high conc in intrauterine life  keeps ductus open • Prostaglandin inhibitor – stimulate closure of open ductus • Indomethacin 0.1 mg/ kg every 12 hours * 3 doses • Ibuprofen • In pulm atresia with intact ventricular septum  infuse PGE 1  effective in keeping ductus arteriosus open, thus reducing hypoxemia and acidemia before surgery

• In rubella • More in females 2:1 • Common in premature infants  patency is a result of hypoxia and immaturity • Closure – to prevent IE • Small PDA – closed with intravascular coils • Moderate to large – catheter induced sac

• Continous machinery murmur best heard at 2 nd left ICS • Loud S1 • Frequent chest inf • Prominent carotid • Hyperkinetic • 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)

ToF • MC cyanotic CHD in >2 years

  Pulmonary Steno sis or RVOT (mostly infundibular level and rarely valvular level)  RVH   Overriding Aorta  VSD (outlet type)

• Pentology = tetrology + ASD • Triology = ASD + RVH + PS • Pink Fallot = no cyanosis clinically as pulmonary outlet obstruction minimal • Cour 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 spells • • • • • •

Knee chest position Humidifed O2 Morphine 0.1 to 0.2 mg/ kg sc Na HCO3 to correct acidosis Propranolol 0.1mg/ kg during spell Vasopressors – methoxamine, phenylephrine • Correct anemia

• Single S2 (A2)  due to R to L shunting • Large a wave • Systolic P3 murmur • Tet spells • Squatting • Apneic spells on waking up • clubbing

Mx: • Blalock Taussig’s shunt  subclavian artery to ipsilateral pulmonary artery

• Pott’s shunt  descending aorta to left pulmonary artery • Waterston’t shunt  ascending aorta to right pulmonary artery

Tricuspid Atresia

Tricuspid Atresia • Blood flows thru ASD/ VSD as Right Ventricle is hypoplastic or absent. •  since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries accomplished by a ventricular septal defect (VSD). • Complete mixing of blood • LV hypertrophy and LAD • only CHD with LAD

• • • • • • •

Cyanotic from birth Enlarged liver – cyanotic liver Squatting Anoxic spells Sicker than ToF Ecg – left axis deviation Single S2 (A2) – large a wave

• 4 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 atresia • Glenn procedure followed by a modified Fontan procedure

Ebstein’s Anomaly • Atrialization of ventricle as tricuspid valve fused to wall of right ventricle – Box shaped heart • Assoc with tricuspid atresia • Lithium use in 1st trimester • Intracavitary ECG is a Dx aid

• Cyanosis • Palpitations and sudden cardiac death • Symptoms of right heart failure, such as edema and ascites • Clubbing • Wide variable split S2 • Soft P2 • Rx: Valvuloplasty

Transposition of the great vessels (TGV) • is a group of congenital heart defects (CHDs) involving an abnormal spatial arrangement of any of the great vessels:  superior and/or inferior venae cavae (SVC, IVC),  pulmonary artery, pulmonary veins, and aorta. • CHDs involving only the primary arteries (pulmonary artery and aorta) belong to a sub-group called transposition 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 birth • TAPVC – non obstructive type presents at 410 weeks; obstructive type at 1st week of life • PDA – symptomatic early in life, CHF at 6-10 weeks • VSD – symptomatic 6-10 weeks of life

• Egg on string appearance in TGV

• • • • •

Single S2 Cyanotic at birth CCF in 1st week RVH Recurrent RTI

• Dextro-Transposition of the great arteries In dextro-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 a cyanotic congenital heart defect (CHD) because the newborn infant turns blue from lack of oxygen. • Levo-Transposition of the great arteries Levo-Transposition of the great arteries is an acyanotic heart 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)

management •

Jatene arterial switch: The aorta and pulmonary artery  are detached from their native roots and reattached to the opposite root; thus, the pulmonary root becomes the  neo-aorta, and the aortic root becomes the neo-pulmonary artery.