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  1. Cardiology discharge conference October 26, 2012 Peggy Lazerow, MD

  2. Patient Presentation • 5 yo caucasian F • CC: Concerning cardiac FH • HPI: 1st degree AV block on previous EKG, otherwise healthy, keeps up with peers, no HA/dizziness/palpitations/SOB/syncope, normal growth • PMH: FT, c-section for breech, no other sig PMH • ROS: per HPI • Meds: none • Allergies: NKDA • Social: lives with mother, father, brother; attends kindergarten

  3. Physical Exam • W: 18.2kg (65%), H: 109 cm (75%) • BP: 108/55 (ra), P: 106, O2: 100% RA • Gen: NAD • Cardiac exam: • 2+ brachial, femoral, pedal pulses bilaterally • Quiet precordium • S1: nl, S2: wide and fixed splitting • Murmur: 1-2/6 SEM, LUSB, non radiating • Lungs: CTAB • Abd: soft, NT, no HSM

  4. EKG

  5. EKG – Rhythm Strip

  6. Echocardiogram

  7. Echocardiogram

  8. Echocardiogram

  9. Echocardiogram

  10. AtrialSeptal Defect http://www.cdc.gov/ncbddd/heartdefects/atrialseptaldefect.html

  11. Known Congenital heart disease Sudden cardiac death ICD/pacemaker AV block

  12. Known Congenital heart disease Sudden cardiac death ICD/pacemaker AV block

  13. Known Congenital heart disease Sudden cardiac death ICD/pacemaker AV block

  14. Known Congenital heart disease Sudden cardiac death ICD/pacemaker AV block

  15. Known Congenital heart disease Sudden cardiac death ICD/pacemaker AV block

  16. NKX 2.5 Mutation • Cardiac transcription factor • Expressed in early cardiac mesoderm and heart muscle lineage throughout life • Variable expressivity • AV block, ASD, VSD, TOF, DORV, tricuspid valve abnormalities • Essential gene for atrial, ventricular and conotruncal septation, AV valve formation, AV conduction

  17. NKX 2.5 http://www.springerlink.com/content/q1362682203x3035/ http://www.med.uottawa.ca/research/nemer/eng/projects.html http://cardiovascres.oxfordjournals.org/content/91/2/243/F2.expansion.html

  18. AV Block

  19. Cardiac Conduction System • Initiates and conducts signal to control and coordinate atrial and ventricular contraction • Sinus node  AV node  Bundle of His

  20. Cardiac Conduction System http://www.unm.edu/~lkravitz/EKG/ekgdepolmyocyte.html

  21. Cardiac Conduction System http://www.unm.edu/~lkravitz/EKG/ekgdepolmyocyte.html

  22. Normal Sinus Rhythm • P before every QRS • Normal PR interval • Normal P wave axis (upright in lead I and aVF) http://www.bem.fi/book/19/19.htm

  23. AV Block • Delay in the transmission of impulse from the atria to the ventricles • Prolonged PR interval or • Dropped QRS • Due to an anatomical or functional impairment in the conduction system

  24. AV block types • 1st degree • 2nd degree • Mobitz Type I, Wenckebach • Mobitz Type II • 3rd degree, Complete

  25. 1st Degree AV Block • Slowed conduction without loss of atrioventricular synchrony • PR interval > 200 msec (1 large square) • Prolonged same amount in every cycle • Occurs in up to 6% of normal neonates • Consider: rheumatic fever, Chagas disease, rubella, mumps, hypothermia, cardiomyopathy, metabolic disorders

  26. http://www.unm.edu/~lkravitz/EKG/avblocks.html

  27. 2nd Degree AV Block – Mobitz I • Intermittent loss of AV conduction • Progressively longer PR  dropped QRS • Does not usually progress to complete heart block

  28. 2nd Degree AV Block – Mobitz II • Intermittent loss of AV conduction • Normal PR intervals • Occasional dropped QRS • May occur in regular pattern (2:1, 3:1) • Inc ratio = Inc severity of block • At or below AV node • May progress to complete heart block

  29. Complete AV Block • No atrial impulses get to ventricles • Ventricles rely on ectopic pacemaker • Independent atrial and ventricular rhythm • May assume location of focus based on ventricular rate and QRS morphology • At risk for heart failure and sudden cardiac death

  30. Clinical manifestations • Often asymptomatic • In utero: fetal bradycardia (can be a/w hydrops, pericardial effusion, spontaneous IUFD) • In neonate: bradycardia, intermittent canon waves in neck, varying S1, gallops, murmurs • In children: bradycardia, reduced exercised tolerance, syncope, pre-syncope, sudden death

  31. Etiology Physiologic/ Pathophysiologic • Increased vagal tone • Fibrosis and sclerosis of conduction system • Ischemia heart disease • Cardiomyopathy • Myocarditis • Congenital heart disease • Familial AV block • Neonatal lupus • Trauma • Degenerative neuromuscular disease • Hypo/hyperthyroidism Iatrogenic • Drugs – digitalis, CCB, amiodarone, adenosine, B blockers, anti-arrhythmics • Cardiac surgery • Catheter ablation of arrhythmias • Transcatheter closure of VSD • Alcohol septal ablation for HCM • Transcatheter aortic valve implantation

  32. Diagnosis • History • EKG • 24-hour ambulatory monitoring • Exercise stress test • Electrophysiologic testing

  33. Treatment • 1st degree: none if asymptomatic • Mobitz I: usually none unless other conduction system disease and asymptomatic • Mobitz II: correct reversible cause, pacemaker insertion • 3rd degree: atropine for short term, treat underlying reversible cause, steroids, isoproterenol, pacemaker insertion

  34. References Benson, Woodrow D. Genetic Origins of Pediatric Heart Disease. Pediatric Cardiology. 3,3: 2019. Epstein AE, DiMarco JP, Ellenbogen KA, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalties: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation 2008; 117:e350. Jaeggi ET, Hamilton RM, Silverman ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution’s experience of 30 years. J Am Coll Cardiol 2002; 39: 130. Reamon-Buettner SM, Borlak J. NKX 2.5 an update on this hypermutable homeodomain protein ad its role in congenital heart disease. Hum Mutat. 2010 Nov;31(11):1185-94. doi: 10.1002/humu.21345. Epub 2010 Oct 12.

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