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W2D- Workshop #4 Cases I Learned From
Leo Lopez, MD Director, Non-Invasive Imaging
Pediatric Cardiology Miami Children’s Hospital
Miami, Florida
Disclosure of Relevant Relationship • Dr. Lopez (or spouse/partner) has not had (in the
past 12 months) any conflicts of interest to resolve or relevant financial relationship with the manufacturers of products or services that will be discussed in this CME activity or in his presentation.
• Dr. Lopez will support this presentation and clinical recommendations with the “best available evidence” from medical literature.
• Dr. Lopez does not intend to discuss an unapproved/investigative use of a commercial product/device in this presentation.
Presenter Disclosure Informa2on
• Leo Lopez, MD • Pediatric Cardiology Case Presenta2on
Financial Disclosure: None Unlabeled/Unapproved Uses Disclosure: None
ILLUSTRATIVE CASES Pediatric Cardiology
Leo Lopez, MD Miami Children’s Heart Program
Acknowledgment
MCH Echo Lab • Roque Ventura • Theola Ray • Susan Mar2nez • Evelyn Menendez • Vanessa Hughes • Irwin Seltzer • Mike Diogo • Liz Welch • Juan Carlos Muñiz • Nao Sasaki • Danyal Khan
CASE 1
• Newborn baby boy with a murmur • O2 satura2on (right arm) 96% • O2 satura2on (right leg) 97%
CASE 1
• Echocardiogram
LIVER L R
Echo 101
• Basics • Vertex = anterior • R-‐L orienta2on (mirror)
Echo 101
• Basics • Vertex = anterior • R-‐L orienta2on (mirror) • Imaging
• White = solid or gas • Black = fluid
Echo 101
• Basics • Vertex = anterior • R-‐L orienta2on (mirror) • Imaging
• White = solid or gas • Black = fluid
• Color mapping (BART) • Blue = away • Red = toward
Echo 101
LIVER L R
• Subcostal long-‐axis imaging
Echo 101
• Subcostal long-‐axis imaging • BART (blue = away, red = towards)
LIVER L R
CASE 1
• Subcostal long-‐axis imaging • PFO • Normal heart
LIVER L R
CASE 2
• Newborn baby boy with cyanosis • Wt 4.1 kg • HR 140, RR 60, BP 75/45 • No murmur
CASE 2
• Newborn baby boy with cyanosis • Wt 4.1 kg • HR 140, RR 60, BP 75/45 • No murmur • O2 satura2on (right arm) 73% • O2 satura2on (right leg) 75% • No significant change in O2 sat with 100% FiO2
CASE 2
• Chest X-‐ray
CASE 2
• Subcostal long-‐axis imaging
LIVER L R LIVER L R
CASE 2
• Subcostal long-‐axis imaging
LIVER L R LIVER L R
CASE 2
• Subcostal short-‐axis imaging
LIVER
Feet
Head
CASE 2
• Subcostal short-‐axis imaging
LIVER
Feet
Head
CASE 2
• Subcostal short-‐axis imaging
LIVER
Feet
Head
CASE 2
• Apical imaging
RA LA
RV LV
CASE 2
• Apical imaging
LA
LV RV
CASE 2
• Ductal view Ao
PA
* RA LA
RV LV
TGA
• V-‐A discordance: RV à Ao, LV à PA
TGA
• V-‐A discordance: RV à Ao, LV à PA • Parallel circula2on • Not circula2on in series
TGA
• V-‐A discordance: RV à Ao, LV à PA • Oxygenated blood stays in pulm circula2on only • Systemic circula2on sees no oxygenated blood
TGA
• V-‐A discordance: RV à Ao, LV à PA • History
• Baillie (1797): “ The aorta arose out of the right ventricle and the pulmonary artery out of the lek”
• Farre (1814): “Transposi2on of the aorta and pulmonary artery”
• Spitzer (1923): TGA = “crossed transposi2on”; DORV = “simple (par2al) transposi2on”
TGA
• V-‐A discordance: RV à Ao, LV à PA • Oxygen to systemic circula2on for survival
TGA
• V-‐A discordance: RV à Ao, LV à PA • Oxygen to systemic circula2on for survival
• Mixing at ASD (LAàRAàAo) & PDA (AoàPA)
TGA
• V-‐A discordance: RV à Ao, LV à PA • 9th most common CHD • Mean incidence 0.315 per 1000 livebirths • 1.7% of all CHD dx in CH Boston 1988 – 2002
JACC, 2002 Nadas’ Pediatric Cardiology, 2006
TGA
• V-‐A discordance: RV à Ao, LV à PA • 9th most common CHD • Mean incidence 0.315 per 1000 livebirths • 1.7% of all CHD dx in CH Boston 1988 – 2002 • Very rare with most gene2c syndrome (Turner, Noonan, Marfan, Williams, DiGeorge)
• Almost never with Down syndrome • Strong associa2on with heterotaxy syndrome
CASE 3
• Newborn baby boy with cyanosis & no murmur • O2 satura2on 65% à intuba2on, PGE1
CASE 3
• Newborn baby boy with cyanosis & no murmur • O2 satura2on 65% à intuba2on, PGE1 • Chest X-‐ray
CASE 3
• Newborn baby boy with cyanosis & no murmur • O2 satura2on 65% à intuba2on, PGE1 • pO2 28 mm Hg • pH 7.1
CASE 3
• Subcostal long-‐axis imaging
LIVER L R
CASE 3
• Subcostal long-‐axis imaging
LIVER L R
CASE 3
• Subcostal short-‐axis imaging
LA
RA
LA
RA
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Mixing at ASD (LAàRAàAo) & PDA (AoàPA)
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• PDA à é PA flow à é PV return à é LAp
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• PDA à é PA flow à é PV return à é LAp • ê LAàRA shunt à ê oxygenated blood to Ao à ê O2
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• Balloon atrial septostomy
JAMA, 1966
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• Balloon atrial septostomy
LA
RA
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• Balloon atrial septostomy
LA
RA
LA
RA
TGA/PFO
• V-‐A discordance: RV à Ao, LV à PA • Restric2ve ASD
• Balloon atrial septostomy • Prenatal predictors
• Hypermobile interatrial septum • Reverse PDA flow (AoàPA)
JASE, 2011
CASE 4
• 3 day old boy with a murmur • O2 satura2on 80%
• Subcostal long-‐axis imaging
LIVER L R
CASE 4
LIVER L R
• Subcostal long-‐axis imaging
LIVER L R
CASE 4
LIVER L R
CASE 4
• Subcostal short-‐axis imaging
LV
RV
LV
RV
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • Mixing at ASD, VSD, & PDA à beqer O2 satura2ons
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • CHSS (889 pts, 20 ins2tu2ons)
• 74% TGA/IVS • 21% TGA/VSD • 5% TGA/VSD/PS • 0.7% TGA/IVS/PS
Circula=on, 1992
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • CHSS (889 pts, 20 ins2tu2ons)
• 74% TGA/IVS • 21% TGA/VSD • 5% TGA/VSD/PS • 0.7% TGA/IVS/PS
Circula=on, 1992
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • Different spectrum of VSD types from isolated VSDs • Perimembranous • Malalignment VSD • Muscular VSD
Am J Cardiol, 1985
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • Different spectrum of VSD types from isolated VSDs • Perimembranous • Malalignment VSD • Muscular VSD
Am J Cardiol, 1985
TGA/VSD
• V-‐A discordance: RV à Ao, LV à PA • Different spectrum of VSD types from isolated VSDs • Perimembranous • Malalignment VSD • Muscular VSD
Am J Cardiol, 1985
CASE 5
• Newborn girl diagnosed prenatally w/ dextrocardia & bradycardia
• O2 satura2on 86% à PGE1
CASE 5
• Newborn girl diagnosed prenatally w/ dextrocardia & bradycardia
• O2 satura2on 86% à PGE1 • CXR
CASE 5
• Subcostal long-‐axis imaging
CASE 5
• Subcostal long-‐axis imaging
CASE 5
• Subcostal long-‐axis imaging
ISOLATED ATRIAL INVERSION
• Very rare!!!
BODY LUNGS
PA Ao
LA RA
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA
BODY LUNGS
PA Ao
LA RA
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA • Mixing necessary to maintain oxygena2on BODY LUNGS
PA Ao
LA RA
Large PDA
Musc VSDs
Large ASD
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA
• Treatment BODY LUNGS
PA Ao
LA RA
Large PDA
Musc VSDs
Large ASD
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA
• Treatment • Arterial switch BODY LUNGS
PA Ao
LA RA
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA
• Treatment • Arterial switch
• RV systemic pump
BODY LUNGS
PA Ao
LA RA
ISOLATED ATRIAL INVERSION
• Parallel circula2ons • LA à RV à PA à pulmonary veins à LA • RA à LV à Ao à systemic veins à RA
• Treatment • Arterial switch • Atrial switch
• Mustard • Senning
BODY LUNGS
PA Ao
LA RA
• TGA is defined as origin of the Ao from the RV and origin of the PA from the LV.
• TGA results in parallel circula2ons rather than the systemic and pulmonary circula2ons in series; oxygenated blood stays only in the pulmonary circula2on.
• The O2 satura2on is completely dependent on mixing at the ASD (LAàRA) and PDA (AoàPA).
• Balloon atrial septostomy is occasionally necessary. • VSDs occur in 1/5 of cases and help with mixing.
Summary
THANKS!
MCH Echo Lab
Embryology of TGA
Embryology of TGA
• 24 days gesta2on: ventricular looping • Solitary outlow tract from developing RV • DILV
Courtesy of Bob Anderson
LV RV
Embryology of TGA
• Pairs of spiraling ridges (cushions) extend along outlow tract
Embryology of TGA
• Pairs of spiraling ridges (cushions) extend along outlow tract • Neural crest cells migrate to outlow tract
Embryology of TGA
• Pairs of spiraling ridges (cushions) extend along outlow tract • Neural crest cells migrate to outlow tract • Distal cushions fuse à proximal Ao & PA
Embryology of TGA
• Pairs of spiraling ridges (cushions) extend along outlow tract • Neural crest cells migrate to outlow tract • Distal cushions fuse à proximal Ao & PA • Intermediate cushions fuse à AoV, PV, roots
Embryology of TGA
• Pairs of spiraling ridges (cushions) extend along outlow tract • Proximal cushions fuse à subpulm & subAo regions • DORV
Courtesy of Bob Anderson
Embryology of TGA
• Remodeling prior to fusion of outlow cushions to muscular ventricular septum • RAVV à RV, Ao outlow à LV Courtesy of Bob Anderson
Embryology of TGA
• Remodeling prior to fusion of outlow cushions to muscular ventricular septum • RAVV à RV, Ao outlow à LV • SubAo conus s2ll present
Courtesy of Bob Anderson
Embryology of TGA
• Regression of subAo muscle • Fibrous con2nuity between MV & AoV
Embryology of TGA
• Two theories
Front Pediatr, 2013
Embryology of TGA
• Two theories • Extracardiac theory
• No spiral development of aortopulmonary septum • Linear development of aortopulmonary septum
Am Heart J, 1981
Front Pediatr, 2013
Embryology of TGA
• Two theories • Extracardiac theory
• No spiral development of aortopulmonary septum • Linear development of aortopulmonary septum
• Infundibular theory • Abnormal persistence of subAo conus • Abnormal regression of subpulm conus
Am Heart J, 1981
Circula=on, 1973
Front Pediatr, 2013
Embryology of TGA
• Two theories • Infundibular theory
• Absent sub-‐Ao conus à NRGA • Absent subpulm conus à TGA • Bilateral conus à DORV • Bilaterally absent conus à DOLV
Circula=on, 1973
Embryology of TGA
• Two theories • Infundibular theory
• Absent sub-‐Ao conus à NRGA • Absent subpulm conus à TGA • Bilateral conus à DORV • Bilaterally absent conus à DOLV
Circula=on, 1973
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