W2D- Workshop #4 Cases I Learned From - ilearnpeds.com...W2D- Workshop #4 Cases I Learned From Leo Lopez, MD Director, Non-Invasive Imaging Pediatric Cardiology Miami Children’s

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


LIVER L R LIVER L R

CASE 2


LIVER L R LIVER L R

CASE 2

•  Subcostal short-‐axis imaging

LIVER

Feet

Head

CASE 2


LIVER

Feet

Head

CASE 2


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


LIVER L R

CASE 3


LIVER L R

CASE 3


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


•  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


•  Balloon atrial septostomy

JAMA, 1966

TGA/PFO



LA

RA

TGA/PFO



LA

RA

LA

RA

TGA/PFO


•  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%


LIVER L R

CASE 4

LIVER L R


LIVER L R

CASE 4

LIVER L R

CASE 4


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


Am J Cardiol, 1985

TGA/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


CASE 5


CASE 5


ISOLATED ATRIAL INVERSION

•  Very rare!!!

BODY LUNGS

PA Ao

LA RA


•  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


•  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



•  Treatment BODY LUNGS

PA Ao

LA RA

Large PDA

Musc VSDs

Large ASD



•  Treatment •  Arterial switch BODY LUNGS

PA Ao

LA RA



•  Treatment •  Arterial switch

•  RV systemic pump

BODY LUNGS

PA Ao

LA 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


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


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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W2D- Workshop #4 Cases I Learned From - ilearnpeds.com...W2D- Workshop #4 Cases I Learned From Leo Lopez, MD Director, Non-Invasive Imaging Pediatric Cardiology Miami Children’s