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A Quick Tour of Congenital Heart Disease

A Quick Tour of Congenital Heart Disease. Chris Longhurst, MD Monday, March 10, 2014. Introduction. Present in 0.8% of North American and European children Most common category of congenital structural malformation

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A Quick Tour of Congenital Heart Disease

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  1. A Quick Tour of Congenital Heart Disease Chris Longhurst, MD Monday, March 10, 2014

  2. Introduction • Present in 0.8% of North American and European children • Most common category of congenital structural malformation • Commonly divided into noncyanotic (L  R) and cyanotic (R  L) categories based on direction of shunting

  3. Relative Frequency of Lesions • Ventricular septal defect 25-30 • Atrial septal defect (secundum) 6-8 • Patent ductus arteriosus 6-8 • Coarctation of aorta 5-7 • Tetralogy of Fallot 5-7 • Pulmonary valve stenosis 5-7 • Aortic valve stenosis 4-7 • Transposition of great arteries 3-5 • Hypoplastic left ventricle 1-3 • Hypoplastic right ventricle 1-3 • Truncus arteriosus 1-2 • Total anomalous pulm venous return 1-2 • Tricuspid atresia 1-2 • Double-outlet right ventricle 1-2 • Others 5-10

  4. Noncyanotic CHD (L R) • Atrial septal defects (ASD) • Ventricular septal defects (VSD) • Patent ductus arteriosus (PDA) • Obstruction to blood flow • Pulmonic stenosis (PS) • Aortic stenosis (AS) • Aortic coarctation

  5. Atrial Septal Defect • Most commonly asymptomatic • Essentials of diagnosis: • Right ventricular heave • S2 widely split and usually fixed • Grade I-III/VI systolic murmur at the pulmonary area • Widely radiating systolic murmur mimicking PPS in infancy • Cardiac enlargement on CXR

  6. Atrial Septal Defect

  7. Atrial Septal Defect • Three major types • Ostium secundum • most common • In the middle of the septum in the region of the foramen ovale • Ostium primum • Low position • Form of AV septal defect • Sinus venosus • Least common • Positioed high in the atrial septum • Frequently associated with PAPVR

  8. Atrial Septal Defect • Treatment • Closure generally recommended when ratio of pulmonary to systemic blood flow (qP/qS) is > 2:1 • Operation performed electively between ages 1 and 3 years • Previously surgical; now often closed interventionally

  9. Atrial Septal Defect

  10. Ventricular Septal Defect • Single most common congenital heart malformation, accounting for almost 30% of all CHD • Defects can occur in both the membranous portion of the septum (most common) and the muscular portion

  11. Ventricular Septal Defect

  12. Ventricular Septal Defect • Three major types • Small, hemodynamically insignificant • Between 80% and 85% of all VSDs • < 3 mm in diameter • All close spontanously • 50% by 2 years • 90% by 6 years • 10% during school years • Muscular close sooner than membranous

  13. Ventricular Septal Defect • Moderate VSDs • 3-5 mm in diameter • Least common group of children (3-5%) • Without evidence of CHF or pulmonary hypertension, may be followed until spontaneous closure occurs

  14. Ventricular Septal Defect • Large VSDs with normal PVR • 6-10 mm in diameter • Usually requires surgery, otherwise… • Will develop CHF and FTT by age 3-6 months

  15. Ventricular Septal Defects • Clinical findings • Grade II-IV/VI, medium- to high-pitched, harsh pansystolic murmur heard best at the left sternal border with radiation over the entire precordium

  16. Ventricular Septal Defect • Treatment • Indicated for closure of a VSD associated with CHF and FTT or pulmonary hypertension • Patients with cardiomegaly, poor growth, poor exercise tolerance, or other clinical abnormalities and a qP/qS > 2:1 typically undergo surgical repair at 3-6 mo

  17. Patent Ductus Arteriosus • Persistence of normal fetal vessel joining the pulmonary artery to the aorta • Closes spontaneously in normal term infants at 3-5 days of age • Epi facts • Accounts for about 10% of all cases of CHD • Higher incidence of PDA in infants born at high altitudes (> 10,000 feet) • More common in females

  18. Patent Ductus Arteriosus • Accounts for about 10% of all cases of CHD • Higher incidence of PDA in infants born at high altitudes (over 10,000 feet) • More common in females

  19. Patent Ductus Arteriosus

  20. Patent Ductus Arteriosus • Clinical findings and course depend on size of the shunt and the degree of associated pulmonary hypertension

  21. Patent Ductus Arteriosus • Pulses are bounding and pulse pressure is widened • Characteristically has a rough “machinery” murmur which peaks at S2 and becomes a decrescendo murmur and fades before the S1

  22. Patent Ductus Arteriosus • Treatment consists of surgical correction when the PDA is large except in patients with pulmonary vascular obstructive disease • Transcatheter closure of small defects has become standard therapy • In preterm infants indomethacin is used (80-90% success in infants > 1200 grams)

  23. Cyanotic CHD (R L) • Tetralogy of Fallot (TOF) • Tricuspid atresia (TA) • Total anomalous pulmonary venous return (TAPVR) • Truncus arteriosus • Transposition of the great vessels • Hypoplastic left heart syndrome (HLH) • Pulmonary atresia (PA) / critical PS • Double outlet right ventricle (DORV)

  24. Tetralogy of Fallot • “Cyanosis, especially in the adult, is the result of a small number of cardiac malformations well determined…. One…is much more frequent than the others…. This malformation consists of a true anatomopathologic type represented by the following tetralogy: (1) Stenosis of the pulmonary artery; (2) Interventricular communication; (3) Deviation of the origin of the aorta to the right; and (4) Hypertrophy, almost always concentric in type, of the right ventricle. Failure of obliteration of the foramen ovale may occasionally be added in a wholly accessory manner.” • Fallot, Ètienne-Louis-Arthur. Contribution to the pathologic anatomy of morbus caeruleus (cardiac cyanosis). Marseilles Med. 1888; 25:418-20.

  25. Tetralogy of Fallot

  26. Tetralogy of Fallot • Most common cyanotic lesion (7 to 10% of all CHD) • Typical features • Cyanosis after the neonatal period • Hypoxemic spells during infancy • Right-sided aortic arch in 25% of all patients • Systlic ejection murmur at the upper LSB

  27. Tetralogy of Fallot • Clinical findings vary depending on degree of RVOFT obstruction • Most patients are cyanotic by 4 months and it is usually progressive • Hypoxemic spells (“tet spells”) are one of the hallmarks of severe tetralogy

  28. Tetralogy of Fallot

  29. Tetralogy of Fallot • Tet spells most commonly start around 4 to 6 months of age and are charcterized by • Sudden onset or deepening of cyanosis • Sudden onset of dyspnea • Alterations of consciousness • Decrease in intensity of systolic murmur

  30. Tetralogy of Fallot • Repair may be staged (modified BT shunt) or complete

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