Download Tetralogy of Fallot

Tetralogy of Fallot
Beth Harrison
Advisor: Samuel Powdrill, PA-C
PAS 646
Outline
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Review blood flow through the heart
Discuss ToF anatomic abnormalities
Etiology
Clinical Presentation
Labs and Exams
Two surgical interventions
Healthy Heart
Blood Flow
• Deoxygenated blood from the body enters
the RA
• At the same time, oxygen rich blood
leaves the lungs to flow into the LA
• Blood in the RA enters the RV through the
tricuspid valve
• At the same time, blood flows from the LA
to the LV through the mitral valve
Blood Flow cont.
• Blood in the RV is pumped through the PA
to the lungs
• At the same time, LV pumps blood out the
aorta to supply the body with oxygen rich
blood
• As seen in ToF, structural defects lead to
the circulation of oxygen-poor blood
ToF
• 4 anatomic
malformations:
-Right Ventricular
Hypertrophy
-Pulmonary Valve
Stenosis
-Transposition of
the aorta
-Ventricular Septal
Defect
ToF
• RVH
-secondary to PA Stenosis
-Increased P on RV leads to RVH
• Transposition of Aorta
-aorta is displaced
• VSD
-”hole in the heart”
-mixing of oxygenated and unoxygenated blood
-cyanosis
• PVS
-more severe, less blood transported to the lungs and
more deoxygenated blood will pass through VSD to
aorta to be circulated throughout the body
Etiology
• Theory: destruction of the neuronal crest
cells during embryogenesis
• In the laboratory setting, destruction of
these cells reproduced results displayed
with certain cardiac malformations.
Clinical Presentation
• Clinical presentation is directly related to
the degree of pulmonary stenosis.
• Severe stenosis results in immediate
cyanosis following birth. Mild stenosis will
not present until later.
• Growth is retarded – insufficient oxygen
and nutrients
• SOA on exertion
“Tet Spell”
• “Tet spells” at 23yo, child
becomes
cyanotic, may
experience
syncope
Exams and Tests
• CBC
-  hematocrit
• ECG
-RVH, RAD
• CXR
-boot shaped
heart, right sided
aortic arch
• Echocardiogram
-VSD
Surgical Intervention 1
• Complete intracardiac repair of VSD and
PA stenosis.
• Enter chest through the sternum. Connect
the heart and lung machine. Heart is
stopped.
• Repair the VSD with a patch.
• Determine if PA needs to be removed or if
removing the excessive muscle tissue will
help to function correctly.
• Pacemaker wires are placed temporarily
because of the potential for postoperative
ventricular arrhythmias.
• Individual chamber pressures are then
measured before the chest is closed. The
pressure readings help to determine how
effective the surgery was.
• Complications: infective bacterial
endocarditis, pulmonic regurgitation,
arrhythmias, RBBB, or left anterior
hemiblock
Surgical Intervention 2
• New method for patching the VSD
• Transcatheter patches were selected
specifically for VSD size. Radio-opaque
loop inside of each patch allowed for
attachment of double nylon thread. Made
retrieval possible if necessary.
• Pts were anticoagulated with heparin
initially, followed with ASA 24 hr later.
• 48 hrs later the apparatus was inserted into the
femoral vein were it was catherized until it reach
the ascending aorta.
• Echo was used to determine its location within
the heart, allowing for proper placement of the
patch for the VSD.
• Pts were monitored in the ICU for 24 hrs.
• Only 2 of the 16 pts in this study did not benefit
from this study. In comparison to others, their
VSD’s were much larger.
• Pulmonary valvuloplastics were also performed.
• Complications: there were no reported
complications for VSD in this study
Why Do We Need to Know?
• Not every case of ToF will be discovered
by cardiologist and pediatricians.
• Some symptoms will be subtle and
present later in life to PCPs.
• Several case studies discussed patients
who chose not to have their ToF corrected.
We need to know if ToF defects are
causing their health problem or if the
problems are from other sources.
References
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American Heart Organization (AHO). “Tetralogy of Fallot.” 2005. Available online:
http://www.americanheart.org/presenter.jhtml?identifier=11071
Greenberg, S. Bruce. “Tetralogy of Fallot.” 2004 Emedicine.com, Inc. Available online:
http://www.emedicine.com/radio/topic685.htm
Inova Heart and Vascular Institute. “Tetralogy of Fallot.” 2006. Available online:
www.inova.org/inovapublic.srt/heart/pediatric_services/cardiac_surgery/tetralogy_of_fallot.html
Moyano D., I C Huggon, L D Allan. “Fetal echocardiography in trisomy 18.” Originally
published online 24 May 2005. Arch. Dis. Child. Fetal Neonatal Edition 2005; 90; pp. 520-522.
Pflieger, Kurt. “Tetralogy of Fallot.” 2005 Emedicine.com, Inc. Available online:
http://www.emedicine.com/emerg/topic575.htm
Sideris, Eleftherios. “Transcatheter Patch Occlusion of Perimembranous Ventricular Septal
Defects.” The American Journal of Cardiology; 95(12), 15 June 2005, 1518-1521.
Warnes, Carole. “The Adult With Congenital Heart Disease.” Journal of the American College
of Cardiology; 2005; 26(1): 1-8.
Yang, X., L J Freeman and C Ross. “Unoperated Tetralogy of Fallot: case report of a natural
survivor who died in his 73rd year; is it ever too late to operate?” Postgraduate Medical Journal
2005; 81; 133-134.
Zeballos, Alvaro. “Tetralogy of Fallot.” 2003 Emedicine.com, Inc. Available online:
http://www.emedicinehealth.com/fulltext/11205.htm