c a r d i o l o g y g r a n d r o u n d s · obstruction of lv‐aortic pathway in the area of vsd...

C A R D I O L O G Y G R A N D R O U N D S

Title:

Congenital Heart Disease: diagnostics past, present and future

Speaker: Charles C. Gornick, MD, FACC, FHRS Director of Cardiac Electrophysiology Minneapolis Heart Institute® at Abbott Northwestern Hospital

Kelly G. Han, MD Director of Congenital Cardiac Imaging Minneapolis Heart Institute® at Abbott Northwestern Hospital

Date: Monday, February 15, 2016

Time: 7:00 – 8:00 AM

Location: ANW Education Building, Watson Room OBJECTIVES At the completion of this activity, the participants should be able to:

1. Distinguish the changing congenital heart disease (CHD) population. 2. Associate that most morbidity and mortality in CHD is in adulthood. 3. Recognize the critical collaboration necessary between adult and pediatric cardiology to care for this

group of patients. 4. Illustrate the changing diagnostics in CHD. 5. Illustrate several examples of CHD presenting in adulthood. 6. Interpret the depth and breadth of the Midwest Adult Congenital Cardiac Program; its importance of

ongoing care for this unique group of patients; and illustrate its comprehensive program.

Physician: This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Allina Health and Minneapolis Heart Institute Foundation. Allina Health is accredited by the ACCME to provide continuing medical education for physicians.

Allina Health designates this live activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)TM. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

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C A R D I O L O G Y G R A N D R O U N D S

Title:

Congenital Heart Disease: diagnostics past, present and future

Speaker: Charles C. Gornick, MD, FACC, FHRS Director of Cardiac Electrophysiology Minneapolis Heart Institute® at Abbott Northwestern Hospital

Kelly G. Han, MD Director of Congenital Cardiac Imaging Minneapolis Heart Institute® at Abbott Northwestern Hospital

Date: Monday, February 15, 2016

Time: 7:00 – 8:00 AM

Location: ANW Education Building, Watson Room Nurse: This activity has been designed to meet the Minnesota Board of Nursing continuing education requirements for 1.2 hours of credit. However, the nurse is responsible for determining whether this activity meets the requirements for acceptable continuing education.

DISCLOSURE STATEMENTS Speaker(s): Dr. Gornick has declared that he does not have any conflicts of interest to disclose. Dr. Han has declared that she has

received grant/research support from Siemens Medical.

Planning Committee Dr. Michael Miedema, Dr. Scott Sharkey and Jolene Bell Makowesky have declared that they do not have any conflicts of interest associated with the planning of this activity. Dr. Robert Schwartz declared the following relationship - consultant: Boston Scientific.

PLEASE SAVE A COPY OF THIS FLIER AS YOUR CERTIFICATE OF ATTENDANCE

Signature: __________________________________________________________________________ My signature verifies that I have attended the above stated number of hours of the CME activity.

Allina Health - Learning & Development - 2925 Chicago Ave - MR 10701 - Minneapolis MN 55407

B. Kelly Han, MD

Charles Gornick, MD

Adult Congenital Heart Disease

The Changing PopulationPast – Present – Future

Minneapolis Heart Institute 2016

Disclosures

Research support Siemens Medical

Congenital Heart Disease Past - Present - Future

Transformation in CHD Medical treatment

Surgical intervention

Catheter based intervention

Cardiac diagnostics

CHD Historical perspective Cardiac Surgery

• First Aortopulmonary shunt 1944 (Blalock & Taussig)

• First open heart surgery for ASD closure 1952

• First Fontan palliation for tricuspid atresia 1968

• First Arterial Switch 1975 (Jatene)

• First Norwood for HLHS 1979

• First Fontan for HLHS 1983

CHD Historical PerspectiveCardiac Catheterization

• First described in 1929 (Forssmann, Germany)

• First diagnostic catheterization 1941

• First coronary balloon angioplasty 1977

• First pulmonary balloon Valvuloplasty 1982

• First aortic balloon Valvuloplasty 1985

• First ASD closure with an Amplatzer device (1995)

• Transcatheter Pulmonary valve (Melody) approved by FDA 2010

Cardiac Diagnostics

Catheterization Echocardiography MRI CT

1950

2016

CHD in Adults: The Changing Population

CHD survival 1960: 1% of d-TGA survived to adulthood 10% of TOF survived to adulthood No HLHS survived to adulthood

2013 95 % surgical survival for complex CHD 90% survival to adulthood 70% of single ventricle patients expected to survive to adulthood

Estimated that 66% of patients with CHD are now adults Most morbidity (and mortality) for CHD is now adulthood

32nd Bethesda Conference: “Care of the Adult with Congenital Heart Disease” JACC Vol 37,No5, 2001: 1161-98.Khairy et al. JACC 2010; 56:1149-1157.


30% decrease in mortality Mortality shifted from

children to adults Median age of death was 57

years Complex CHD: median age of death changed

from 2 to 23 years during the study

Khairy et al. JACC 2010; 56:1149-1157.

Mortality in CHD 1987-2005

prevalence of congenital heart disease (CHD) in the European Union by age group. prevalence of congenital heart disease (CHD) in the European Union by age group.

Helmut Baumgartner Eur Heart J 2014;35:683-685

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2013. For permissions please email: [email protected]

Changing prevalence of congenital heart disease (CHD) in the European Union by age group.


ACHD Rate of Intervention & Arrhythmia by Diagnosis

RE-OP ATRIAL vt

Ebstein’s anomaly 30-50% 33-60% >2% Single ventricle >25% 40-60% >5% Tetralogy of Fallot 26-50% 15-25% 10-15% TGA, atrial switch 15-27% 26-50% 7-9% TGA, arterial switch 12-20% <2% 1-2% CC-TGA 25-35% >30% >2% Truncus arteriosus 5-89% >25% >2% AVSD 19-26% 5-10% <2% Atrial septal defect <2% 16-28% <2%

PACES/HRS expert concensus statement on the recognition and management of arrhythmia in adult congenital heart disease. Khairy et al. May 2014. DOI: 10.1016/j.hrthm.2014.05.009

Most Common CHD diagnosis followed in Adulthood Tetralogy of Fallot

Aortic Coarctation

Transposition of the Great Arteries Atrial switch

Arterial Switch

Nikaidoh/Rastelli

Single Ventricle Heart Disease

TOF Anatomic Variants

Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015

Tetralogy of Fallot:Residual Hemodynamic Lesions

TOF with Pulmonary stenosis pulmonary stenosis, pulmonary insufficiency, RV

dilation and dysfunction, aortic root dilation

TOF with Pulmonary artery atresia RV‐PA conduit, branch pulmonary artery or distal

pulmonary artery stenosis, conduit insufficiency,

RV dilation and dysfunction, aortic root dilation

TOF with Absent pulmonary valve RV‐PA conduit stenosis or insufficiency,

airway abnormalities,

pulmonary artery dilation


Copyright © The American College of Cardiology. All rights reserved.

From: Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair

J Am Coll Cardiol. 1997;30(5):1374-1383. doi:10.1016/S0735-1097(97)00318-5Nollert et al. J Am Coll Cardiol. 1997;30(5):1374-1383Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study. Khairy et al, (Circulation. 2010;122:868-875.)

Long-term survival after correction of TOF. All patients who died within the first year after correction were excluded for calculation of long-term survival. The curve shows two different phases that are distinct. The early, low risk phase lasts 25 years; thereafter, the risk increases significantly. Mortality risk (r) per year, as a linearized number, is calculated for each phase. Note the break in the y axis. OP = operation; p.o. = postoperatively.

Mortality< 25 post op: 0.25%/yr> 25 post op: 0.94%/yr

Risk for sudden death:

Older age at repair QRS duration > 180 msec Severe dilation of the right ventricle RVDD/m2 > 150 ml/m2 RVESV/m2 > 80-95 ml/m2

RV or LV dysfunction (lv fxn abn in 21% of adult tof) Arrhythmia Multiple surgeries Initial palliative shunting Level of pre operative polycythemia Ventriculotomy or RVOT patch

History of Intervention: d-TGA

1940 1950 1960 1970 1980 1990 2000 2010

Atrial septectomy (Blalock Hanlon)1950Atrial switch 1958 (Senning)

Atrial switch 1964 (Mustard)Balloon septostomy (Rashkind)1966

Rastelli procedure 1969Arterial switch operation(Jatene) 1975

Nikaidoh procedure 1984

Surgical Approach to d-TGA


D-TGA: Residual Hemodynamic Abnormalities

Atrial switch Systemic or pulmonary venous baffle narrowing or occlusion

RV failure (systemic ventricle)

Tricuspid regurgitation (quantify by stroke volume differences)

Arterial switch Neo‐pulmonary root or branch pulmonary artery stenosis, pulmonary insufficiency

Neo‐aortic root dilation, stenosis or insufficiency

Compromise of re‐implanted coronary arteries

Rastelli Obstruction of the RV‐PA conduit or branch pulmonary arteries, pulmonary insufficiency

Obstruction of LV‐Aortic pathway in the area of VSD patch

Nikaidoh Native RVOT or RVOT conduit stenosis or insufficiency

Right coronary artery compromise due to translocation of the aorta leftward

Procedure Residual Hemodynamic Abnormalities


Neonatal Arterial Switch for D-TGA

J Am Coll Cardiol. 2014;64(5):498-511. doi:10.1016/j.jacc.2014.06.1150

S/P Arterial Switch Operation

Quality of life and health status 11-15 years later same as normal children, better than atrial baffle

Earliest survivors now young adults Coronary event free survival 93% at one year 88% at 15 years

Hemodynamic sequelae: Coronary insufficiency Ventricular dysfunction Stenosis of great arterial anastomotic sites AI or PI Neo-aortic root dilation

Coronary Artery after ASO

Ostial Lesions Potential for Compression

Coronary anatomy and Relationship to RVOT and Sternum

Date of download: 2/3/2015


From: Coarctation of the Aorta: Lifelong Surveillance Is Mandatory Following Surgical Repair


Survival of the entire cohort compared with an age- and sex-matched Minnesota population (p < 0.001).

Aortic Coarctation

Long Term Follow-up of Simple Coarctation Repair Systemic Hypertension

Premature coronary artery disease

Aortic valve abnormalities

Aortic aneurysm (often with patch repair)

Re-coarctation

1/3 of patients in long-term follow-up had significant cardiovascular abnormalities

The most predictive factor of late complications was AGE AT OPERATION

Long-term follow-up of patients after coarctation of the aorta repair. Toro-Salazar et al, AJC Vol 89(5)March 1 2002

Long Term survival in Coarctation

Age at operation < 1 year old Long term survival 91%

Age at operation 5-10 years old Long term survival 87%

Age at operation > 10 years old Long term survival 60%

Long-term follow-up of patients after coarctation of the aorta repair. Toro-Salazar et al, AJC Vol 89(5)March 1 2002

Undiagnosed COA

13 year old:cerebral aneurysm ruptureIR couldn’t access aneurysm

25 year old: pneumoniaCXR rib notching

45 year oldPregnancy induced htnTreated for 20 years

Aneurysm Formation

Single Ventricle Intervention

1940 1950 1960 1970 1980 1990 2000 2010

AP shunt 1940s

Fontan for TA 1971Norwood Procedure for HLHS 1981

Fontan for HLHS 1983Lateral tunnel Fontan 1980s

Extracardiac Fontan 1990s

Glenn shunt 1950s

Single Ventricle Intervention

Passive systemic venous flow to the lungs

Unobstructed systemic arterial flow from single V Stage 1: BT shunt or Sano

Norwood or DKS

Stage 2: Glenn (TCPC)

Stage 3: Fontan

Classic Fontan Bjork Kawashima Atrio-pulmonary

Lateral tunnel

Extracardiac


Date of download: 2/3/2015


From: Predictors of Survival After Single-Ventricle Palliation: The Impact of Right Ventricular Dominance


Kaplan-Meier Survival Curves

Patients were separated into 2 groups according to era of birth. Era 1 consisted of patients born from 1990 to 1999; era 2 consisted of patients born from 2000 to 2008. HLHS = patients with hypoplastic left heart syndrome; LV = patients with a dominant left ventricle; RV no HLHS = patients with a dominant right ventricle but not a diagnosis of HLHS.

Figure Legend:

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients with Fontan Surgery. Khairy et al, Circulation 2008;117:85-92

Late Fontan Survival

261 patients Born 1985 or earlier Median age at Fontan

7.9 Freedom from death or

transplant 5 yrs: 93.7% 10 yrs: 89.9% 15 yrs: 87.3% 20 yrs: 82.6%

Common Residual Hemodynamic Lesions after the Fontan procedure SVC to PA, IVC to PA or branch pulmonary artery narrowing Atrial dilation (most commonly in older atriopulmonary Fontan) Systemic venous occlusion, collaterals or aortic bronchial collaterals Clot within the Fontan system, pulmonary embolism Ventricular to aortic obstruction if aorta from an outlet chamber Ventricular dysfunction Valvular regurgitation (regurgitation can’t be determined from CT) Recurrent arch obstruction Fenestration or leak in lateral tunnel Fontan Hepatic disease Plastic bronchitis

Diagnostic in CHD

CHD patients need repeat diagnostic testing throughout life

Diagnostic Risk is considered cumulative Vascular access

Radiation

Anesthesia

Risk of Diagnostics in CHD

+/- PIV

Anesthesia < 8 yo No radiation

Gadolinium (NSF & neuro)

Resolution vs signal in small patients

45-75 minutes for complete study

PIV

Limited or no anesthesia

Radiation Iodinated Contrast

Resolution independent of ptsize (< 1 mm, isotropic)

< 1 second, one breath hold

Central Vascular Access

Anesthesia

Radiation

Iodinated contrast

Long procedural time

Echo

Diagnostic CatheterizationCardiac CTA CMRI

Vascular Access Risk in Pediatric Patients

Children’s Hospital Philadelphia 2005-2010• 3254 patients underwent 5715 cath procedures

• Acute occlusive arterial injury 4.3% of 721 patients < 6 months of age – 18%

of 118 patients < 6 months, > 4Fr – 36%

Prevalence and Risk Factors for Acute Occlusive Arterial Injury Following Pediatric Cardiac Catheterization in the Modern Era at a Large Volume Center. Andrew C. Glantz et al, ACC 2012

Anesthesia risk in CHD is Twofold• Procedural Risk of Adverse Event:

• MRI with Anesthesia, odds ratio of adverse event 3.9• Anesthesia risk higher when performed outside of the OR and in

hospitalized patients• Cardiac arrest with anesthesia occurs most commonly in CHD ASA III-

V (single ventricle, unrepaired and palliated CHD)

• Long term risk of adverse Neurodevelopmental Outcome• Repeat or prolonged anesthesia may increase risk of learning disability,

developmental disability, and behavioral problems• Most relevant in period of rapid brain growth < age 2

Risk factors for adverse events during cardiovascular magnetic resonance in congenital heart disease (JCMR 2007;9(5):793-8)Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest registry.(Anesth Analg 2010 May 1;110 (5): 1376-82)

• Anesthesia exposure < 2 yo independent risk factor for development of learning disability

Flick et al. Pediatrics. 2011:128 (5): e1053-61.

• Children exposed to anesthesia < 3 years were twice as likely to have a developmental disorder as non exposed children

DiMaggio. Journal of Neurosurgical Anesthesiology 2009;21:286-291

Children exposed to anesthesia < 3 had higher risk of deficits in language and abstract reasoning at age 10 than unexposed children

Ing et al. Pediatrics 2012 Sep;130(3):e476-85

RR of developmental and behavioral disorders anesthesia< 3 yo

DiMaggio et al AnesthAnalg 2011;113(5):1143-51

RR 1.1 for one anesthetic RR 2.9 for two anesthetic RR 4.0 for > anesthetic

Prospective studies underway (GAS,PANDA)

Diagnostic Risk in CHD - AnesthesiaNeurodevelopmental Outcome

Diagnostic Risk in CHD - Radiation Exposure

• The average CHD patient : 20 mSv of radiation exposure by adolescence (unadjusted for age/size)

• Radiation exposure primarily from cath procedures• Electrophysiology studies – 17-25 mSv (unadjusted)

• 22% of studies exceeded dose to cause skin injury

• Pediatric Cardiac Catheterization• Median effective dose 6.4 mSv (0.5 – 53 mSv)• Age adjusted mSv (13 mSv for neonate, 8 mSv for a 1 year old )• Catheterization exposure 10-15 fold higher than CT exposure

using modern equipment Rosenthal et al. Am J Cardiol 1998;82:451-458 Akash et al. Circulation 2010;122:A18535Andreassi et al. , Circulation 2009;120:1847-1849Beels et al. Circulation 2009:120:1903-1909Hvlacek et al. Pediatric Cardiology 2012. DOI: 10.1007/s00246-012-0486-2Han & Lesser et al. JTCVS , in press

Background Radiation 3-6 mSv/year0.24 mSv/Month

CXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv

Cumulative Radiation Exposure < 6 yo

ASD 6 (4, 30) 0.09 (0.02, 6.9) 0.19 (0.07,29.3)

VSD 9 (4, 79) 0.20 (0.01, 12.01) 0.36 (0.06, 23.6)

AVCD 11 (4, 111) 0.26 (0.02, 29.45) 0.49 (0.07, 30.4)

TOF 10 (5, 63) 0.27 (0.02, 22.59) 0.55 (0.08, 33.3)

ASO 18 (10, 47) 0.29 (0.05, 3.56) 0.60 (0.18, 8.2)

TX 117 (50, 247) 42.5 (8.34, 396) 63.79 (9.93, 190)

Single V 63 (19, 271) 20.08 (0.66, 182) 28.93 (0.70, 113)

Total 17 (4, 158) 1.34 (0.03, 83.24) 2.67 (0.08, 76.93)

Diagnosis # exams Annual mSv Cumulative

Johnson J N et al. Circulation. 2014;130:161-167



Cumulative Radiation Exposure and Cancer Risk Estimation in Children With Heart Disease

The lifetime

Johnson J N et al. Circulation. 2014;130:161-167

Copyright © American Heart Association, Inc. All rights reserved.

Chest CT 4.7 mSvGated Cardiac Cta 18 MsVDiagnostic cath 9 mSv



Pediatric CCTA Radiation Dose Estimates by Scan Mode and Tube Voltage Scan Mode kVp* DLP† mSv‡ Age Adjusted

mSv

High Pitch (n=19) (prospective ECG)

17 (3‐122) 0.24 (0.04‐1.7) 0.34 (0.08‐1.7)

80 (n=13) 15 (3‐19) 0.21 (0.04‐1.7) 0.26 (0.08‐0.39)

100 (n=5) 60 (33‐69) 0.89 (0.46‐0.97) 0.89 (0.46‐0.97)

120 (n=1) 122 1.7 1.7

Sequential (n=35) (prospective ECG)

62 (6‐471) 0.86 (0.2‐6.59) 1.05 (0.2‐6.59)

80 (n=29) 57 (13‐111) .079 (0.18‐1.54) 0.99 (0.34‐1.98)

100 (n=5) 145 (48‐170) 2.03 (0.67‐2.38) 2.03 (0.67‐2.38)

120 (n=1) 471 6.6 6.6

Spiral (n=22) (retrospective ECG)

122 (32‐344) 1.7 (0.6‐4.8) 1.8 (0.8‐4.8)

80 (n=17) 106 (32‐212) 1.48 (0.6 – 3.74) 1.64 (0.77‐3.74)

100 (n=5) 306 (263‐344) 4.28 (3.44‐4.68) 4.28 (3.44‐4.68)

*kVp = Tube voltage in kilovolt peak, † DLP = dose length product in mGy.cm, ‡ mSv= miliSievert. The median and range of the DLP, mSv and age adjusted mSv is listed for each scan mode and is subdivided by tube voltage.

Han & Lesser et all. Safety and accuracy of dual-source coronary computed tomography angiography in the pediatric population. JCCT Jul-Aug 2012



Symptomatic Coronary Anomaly

Anomalous RCA, 2 year old

(syncope)

Ostial narrowing

Intraarterial course

Acute angulation takeoff

Anomalous RCA

12 year old


Sequential scan, DLP 32High pitch scan, DLP 12

Anomalous RCA, 6 month old (6 kg)

2 “SIDS like” episodes

Intraarterial course


Right dominant

Sequential scan, DLP 35 HR 98 bpm

CTA: Coronary Lesions < 5 years

3 yo - Nikaidoh 3 yo – h/o Kawasaki4 yo – h/o Kawasaki & CP

3 month old (3.5 kg, s/p Arterial Switch Operation)

Diagnostic Risk in CHD

Advanced Diagnostics Venous/Arterial anomalies

Critically ill patients

Single ventricle patients prior to second stage palliation

Radiation Exposure – Neonatal Imaging

#pts DLP 0.014 Age adj mSv length

64 slice

MDCTA

29 66 (29‐272) 1.1 (0.4‐3.8) 2.6 (1.1‐10.6) 15.2 cm

Dual source

MDCTA

Variable pitch

(2.25‐3.0)

14 28 (8‐50) 0.39 (0.1‐0.6) 1.05 (0.3‐ 1.9) 16.2 cm

Dual source

MDCTA

High Pitch

(3.4)

18 5.4 (3‐12) 0.07 (.04‐.11) 0.2 (0.12‐0.17) 15.8 cm

10 fold dose reduction with highest pitch scans

Han & Lesser et all. Accuracy and Safety of High Pitch Computed tomography Imaging in Young Children with Congenital Heart Disease. Am J Cardiol 2011

64 slice vs High Pitch DS CTA (1st generation)patients < 2 yo

Neonatal Unsedated, Free breathing CTA

Han and Lesser et all. Non-sedated, free breathing cardiac CT for evaluation of complex congenital heart disease in neonates. JCCT 2013.

N=19 Median (lower quartile, upper quartile)

Angiogram DLP 8 (5,9)

Angiogram effective mAs 99.5 (66, 110)

Angiogram length (cm) 10 (9.4, 12.1)

Angiogram CTDIvol 0.47 (0.31, 0.5)

Total procedural DLP 11 (10, 14)

mSv unadjusted (procedural) 0.15 (0.14, 0.2)

mSv age/size adjusted (procedural) 0.86 (0.78, 1.1)

DLP= dose length product, CTDIvol= CT dose index volume, mSv= milliSievert


CXR (2 view) 0.10 mSvNew York – Hong Kong 0.09 mSv

Loeys Dietz, PDA

Neonatal CT

IAA type B

TOFL-TGA, IAA

IAA type C

Critically Ill Patients

3 day old (2.5 kg, HR 150) s/p TAPVR repair DOL #1 Open mediastinum Scan - 0.25 seconds 3 minutes procedural time No change in Vent mgmt

(PEEP 8, right pneumo) DLP 6

0.08 mSv (chest conversion .014)

Critically Ill patients:

AO

PA

2 Month old (3.5 kg, HR 160)s/p Truncus Repair, Pseudoaneurysm from truncal root

Neonate for venous anatomy

HLHS + Scimitar

HLHS + TAPVR

CT Provides Better Information: Extra-Cardiac Anomalies

CTA in Infants and Neonates (MHI/CHCM) 62 scans in neonates (< 30 days of age) Median DLP 14 (11, 18)

Median mSv 0.19

Free breathing, no sedation

178 Scans in infants (< 1 year of age) Median DLP 17 (13, 24)

Median mSv 0.23 Background Radiation 3-6 mSv/year

0.24 mSv/MonthCXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv

Diagnostic vs interventional catheterization in CHD

CT vs Diagnostic Cath in CHD patients < 1 Year

Han, Lesser et al. Cardiovascular imaging trends in congenital heart disease: A single center experience. JCCT 2013. (6):361-6

Evaluation of Single Ventricle Heart Disease

Stage 1

“Norwood”Aorto-pulmonary Shunt/ Sano

Stage 2

“Glenn”

SVC- PA connection

Stage 3

“Fontan”

IVC – PA connection

CathCath

?

Average single Ventricle Patient Diagnostic Radiation exposure 20 mSv/year

CT in Single Ventricle CHD: Pre-Glenn Cath vs CT

Cardiac CTA

(n=16)

Diagnostic Cardiac Catheterization(n=16)

p Value

Gender (male/female) (8/8) (10/6) 0.4760Age at time of imaging procedure, mo 3.7 (2.6, 5.4) 3.6 (3.3, 5.0) 0.6144Weight, kg 5.3 (4.5, 6.8) 5.6 (5.3, 6.4) 0.7650Body surface area, m2 0.29 (0.25, 0.32) 0.30 (0.27, 0.32) 0.6409Time between imaging procedure and Glenn surgery, days 37.5 (10.5, 71.5) 36.5 (23.0, 51.0) (n=14) 0.5786Primary Diagnosis

HLHSUnbalanced AV canal

73

8

DILVDORVTAHeterotaxy

1212

2141

Han & Lesser et all. Selective use of cardiac computed tomography angiography: An alternative diagnostic modality before second-stage single ventricle palliation. JTCVS 2013.

Patient Characteristics

Pre- Glenn CT vs Cath: Procedural Risk

Cardiac CTA(n=16)

Diagnostic Cardiac Catheterization (n=16)

p Value

Anesthesia & Procedural timeTotal anesthesia/sedation time, min Procedural time (min)Image Acquisition time

55 (45, 70.5)8 0.25 seconds

166 (119.5, 197)81.5

<0.0001<0.0001

General anesthesia (N /total) 1/16 16 /16 <0.0001

Vascular AccessPeripheral IV (22g, 24 g) 16/16 ‐‐

Central venous 0/16 16/16 ‐‐

Central arterial 0/16 12/16 ‐‐

Radiation DoseRadiation dose adjusted for age/size, mSv* 1.12 (0.96, 1.31) 13.95 (8.86‐17.93) <0.0001

Number of Angiograms 1 (1,1) 7.5 (5.0, 10) <0.0001

Minutes of fluoroscopy N/A 19.5 (16.5, 29.0) ‐‐

ContrastVolume contrast administered in ml/kg 2.06 (1.98, 2.20) 4.82 (3.78, 5.90) <0.0001

Adverse Events 0 6 <0.0001

CTA visualization of Shunt, Sano

CT visualization of Systemic Venous Anatomy

1E 1F

SVC

RPA

S/p Fontan

Visualization of Fenestration

Radiation Exposure in single ventricle CHD

Single Ventricle Cumulative Radiation exposure 38 SV patients followed for 33 months

Median cumulative exposure : 25.7 mSv (cath 78%)

Downing et al. Cumulative medical radiation exposure throughout staged palliation of single ventricle congenital heart disease. Pediatr Cardiol. 2015 Jan;36(1):190-5

Johnson et al. Cumulative radiation exposure and cancer risk estimation in children with heart disease. Circulation. 2014 Jul 8;130(2):161-7. 2014 Jul 8;130(2):161-7

Glatz et al. Cumulative exposure to medical radiation for children requiring surgery for congenital heart disease. J Pediatr. 2014 Apr;164(4):789-794

CT evaluation of Single V CHD

79/132 underwent intervention – 4 minor discrepancies

N DLP mSv

Total 132 24 0.33 anatomy

Neonate 20 18 0.25 anatomy

Post Norwood 52 16 0.22 anatomy

Post Glenn 15 33 0.46 anatomy

Post Fontan 45 158 2.2 Anatomy + function

ACC 2016

Ventricular Function

ECG pulsed modulation Full radiation for a portion of the cardiac cycle

20% radiation for the remainder of the cardiac cycle

Narrow acquisition window

Low kVp

Higher noise tolerated – reconstructed in thicker slice for analysis

70 kVp functional imaging in CHD

Age <10(n=19)

Age 10-18(n=17)

Age >18(n=36)

BMI 15.6 (14.5, 18.0) 18.1 (16.2, 19.4) 24.2 (21.4, 27.5)

Weight (kg) 11.3 (6.6, 20.0) 43.5 (33.4, 56.2) 72.1 (64.2, 83.0)

Functional Scan DLP 35 (27, 47) 65 (59, 97) 95 (83.5, 106.5)

Functional Scan CTDIvol 2.2 (2.0, 3.6) 4.2 (3.9, 6.0) 5.6 (4.8, 5.9)

Functional mSv (Unadjusted) 0.49 (0.38, 0.66) 0.91 (0.83, 1.36) 1.3 (1.2, 1.5)

Functional mSv (Age-Adjusted) 0.85 (0.66, 1.05) 0.91 (0.83, 1.36) 1.3 (1.2, 1.5)

72 patients

Median age 19.5 years

Median mSv for functional scan < 1 mSv

JCCT 2016 Han, A Lesser et all

86 kgScan dlp 98Effective mas 102Scan range 15.2 cmCtdi vol 5.68

1.5 mm, systole 8 mm systole

1.5 mm diastole 8 mm diastole

MACC: Midwest Adult Congenital Center

Collaboration between CHC, CHCM,MHI

400 clinic visits in 2015

Drs Han, Burton, Carter

Drs Gornick, Lesser, Chu, Samara, Sorajja

Drs Farivar and Flavin

Clinic visits every Monday Jan 1st

ACHD director needed

2016: comprehensive ACHD center certification

Clinical Cases

59 yo white male

Seen North Memorial

Surgeries at Mayo 1960, 1974 Tetralogy of Fallot repair

No cardiology fu since late 1970’s

9/2015 presents with atrial flutter

Subsequent evaluation with evidence of right heart enlargement and to and fro murmur on exam

CT

No coronary lesions

RV EF 47%

RVEDV 204 ml/m2

RVESV 108 ml/m2

PI: 30%

LV EF normal

No significant Ao root dilation

The proportion of CHD patients in contact with the healthcare system by age

Andrew S. Mackie et al. Circulation. 2009;120:302-309

Copyright © American Heart Association, Inc. All rights reserved.

Sees Cardiologist

Sees Primary Care

By age 15 50% of patients did not receive cardiology care

PVR after TOF

Not shown to change VT or mortality Herrild et al. circ 2009. 111:445-51

No agreed upon criteria for valve replacement RVEDV > 150 – 160 ml/m2 RVESV > 80-95 ml/m2

Meta-analysis of 48 studies (3118 pts) Decreased pulmonary regurgitation Improved RV indexed volumes but not EF (unless “corrected” EF Improved LV fxn by larger LVEDV Decreased QRS duration Improved symptoms Largest RVs responded best but not as much decrease in symptoms Largest reduction in PI had best RV response

Pulmonary valve replacement after operative repair of TOF.Cavalcanti et al. JACC vol 62, no 23, 2013.

Surgical Pulmonary Valve replacement

945 RVOT operations (Toronto) Median followup 8.3 yrs (birth – 31 yrs) Median age 6 yrs

25% underwent at least 2 Freedom from RVOT valve replacement

5 yrs 82% 10 years 58% 15 years 41%

Risk factors 13-65 yo Younger age at initial valve placement Smaller valve size Need for endovascular stents Increased # of previous valve replacements

Indications Stenosis PI if increased RVEDV

Caldarone et al. JTCVS 2000.Volume 120 (6) 1022–1031.

45 yo Spanish speaking female

Htn history for 16-17 years on diuretic and ACE

Sharp chest pain intermittent in past

More recently chest heaviness and some difficulty breathing with exertion

HCMC referral to MACC

Bicuspid aortic valve

40-50 mmHg gradient between arms and legs bp RUE BP 142/92

Rib notching noted on chest xray

35 yo white male

History as child of coarc repair and balloon valvuloplasty for aortic stenosis bicuspid valve

Seen by Dr Sutton Children’s Heart Clinic 2004

CMR 2005 AAO 3.6 x 3.6 cm

Lost to FU for 10 years

No symptoms reported, cuts wood for living

Echo done demonstrating mild as/mod ai

Sent for advanced imaging to quantify AI

MRI: LV EF 41%

LVDD 132 ml/m2

30% AI

CT and MRI Pseudoaneurysm with

mural thrombus

6 x 5.8 x 5 cm

Bicuspid AO valve with calcification

Dr. Mudy 21 mm St Judes with 22 mm hemashield graft

53 yo white female

Followed by Dr Gornick since retirement of Dr Gobel Hx of Ragib syndrome (persistent left svc connected

to la along with asd. As child age 12 underwent repair by Dr Aldo

Castenada consisting of tunneling of left svc to RA and closure asd

Subsequent development sinus and av nodal dysfunction and atrial arrhythmias

Clinically did well over the years, annual to bi annual echoes including TEE at one point in time

Occasional cardioversion but for the most part stable on propafenone therapy

C.U. Hx continued(2)

Scheduled ct for structure and volumes but not done due to insurance issues 2013

At fu in 2014 noted to have new evidence of right sided venous distention on exam and gradual reduction in exercise tolerance confirmed by CPST 18 ml/kg/min

Ct scan for structure and function obtained Note complexity 1)hemodynamic problems

2)arrhythmia issues 3) pacemaker leads with chronic failure

C.U. Hx continued (3)

3 pronged plan Arrhythmia ablation by Dr Melby and Dr

Gornick. Typical atrial flutter and 2 other scar mediated flutters on right side. Note access to la via venosus defect

Surgery by Dr Flavin and Moga with venosusdefect closed and tricuspid ring placed, old pacing leads transected and left in svc

Dr Gornick removed transected leads and placed new dual chamber pacing system in post op period

AO

RV

c a r d i o l o g y g r a n d r o u n d s · obstruction of lv‐aortic pathway in the area of vsd...

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