Echocardiographic assessment in AdultPatients with Congenital Heart Diseases

Athanasios KoutsakisCardiologist, Cl. Research Fellow

1st Cardiology Department, Aristotle University of Thessaloniki

George GiannakoulasAss. Professor in Cardiology

Ιπποκράτειες ημέρες καρδιολογίαςThessaloniki, 09 March 2018

Declaration of interest

• None

Epidemiology of CHD

• Total CHD birth prevalence: 9 per 1,000 live births

• 1.35 million newborns with CHD every year

• A major global health burden

1980 –

1970 –

1960 –

1940 –

0 10 20 30 40

Percent Survival to 18 Years Old

DecadeBorn with

CHD

50 60 70 80 90 100

20

Warnes CA, et al. J Am Coll Cardiol. 2001;37(5):1170-1175.

1990

Moons P et al. Circulation 2010.122:2264-2272

40

80

75

90

Survival to 18 years of age with CHD

% Survival

Incidence of CHD

Expanding Population ofAdolescents & Adults with CHD

Early CompleteRepairImproved Surgical

TechniquesAdvances inNICU Care

Increased EarlySurvivalLower Perioperative

Mortality

Increased Mid TermSurvival

Fetal Diagnosis

1970s: Introduction of echocardiography in clinical practice

Echo in ACHD: Role

• Establish or confirm anatomic diagnosis

• Assess new or residual lesions

• Assess valvular, atrial and ventricular function

• Guide transcatheter interventions

• Monitor intra & postoperative status

• Risk stratify for pregnancy

Echo in ACHD: Hints

• Understanding of CHD

• Original diagnosis and surgical procedures

• Examine the patient

• ECG and CXR

• Know & accept limitations of Echocardiography

• Combine with other imaging modalities

General Approach

• Start with standard views to get a “lay of the land”

• Follow blood flow through the heart as best possible

• Use clues, like pacemaker leads

• Evaluate residual stenoses, regurgitation, septal defects

The segmental approach• Atria & venous connections• Atrioventricular junction

– Concordance vs discordance– Double inlet ventricle

• Atrioventricular valves – Distinct valves, – common valve

• Ventricles• Ventriculoarterial junction

– Concordance vs discordance– Double outlet

• Semilunar valves• Great arteries

Specific Lesions

• Shunt lesions

• Obstructive lesions

• Complex CHD

Shunt lesions

ASDs

The 2nd most common congenital heart disease in adults (incidence of 1 in 1,500 live births).

ASD types include:

• Secundum ASD (80%)

• Primum ASD (15%)

• Sinus venosus defect SVC (5%)

• Sinus venosus defect IVC (1%)

• Unroofed coronary sinus (1%)

• 26 yo, male

• Asymptomatic

• Auscultation: mid-systolic murmur,

• Referred for echo (pre-participation athletic screening)

• Male, 60 yo

• NYHA II, palpitations since 9 months

• Known “hole” in the heart since 30 years ago

• ECG: SR, incomplete RBBB, right axis deviation

Qp:Qs 2

Echo in transcatheter closure of ASD

• Male, 78 yo

• Permanent AF known since 2010

• NYHA III

• Lower limb swelling

• sO2 94% at rest

• NT-proBNP 1081 pg/ml

• TTE: Secundum ASD, RV volume overload, RVSP: 87 mmHg

• PVR: 2W, PVRi 3,15W/m2, Qp:Qs 3,6

• 56 yo, male

• NYHA III

• Leg swelling, ascites

RVSP 80 mmHg

VSDs

The most common congenital malformation (30% of all)

• Isolated or associated with other conditions

• VSD types:

– Perimembranous 80%

– Muscular 15-20%

– Subarterial 5%

• 36 yo, female,

• Asymptomatic,

• Loud holosystolic murmur left parasternal border

Perimembranous VSD

Perimembranous VSD

VSD Eisenmenger

• 30 yo, male,

• NYHA III,

• Cyanosis, clubbing

• sO₂ 70%,

• Ht 62,3%

Muscular VSD

Muscular VSD

Repaired VSD

AVSDs

• Prevalence 2,9-7,4%– 30% in Down syndrome

– Common ovoid shaped AV junction

– Five leaflet common AV valve

• Types– Complete AVSD

– Partial (primum AVSDs): Atrial shunting only• Fused superior and inferior leaflet

attached to the crest of the VS

Complete AVSD

• 32 yo, female,

• Down syndrome,

• NYHA III,

• Cyanosis,

• sO₂ 70 % at rest,

• Ht 59%

Complete AVSD

PDA• 2,4% of CHDs

– Vessel distal to the left subclavian artery

– Shunting from the aorta to the pulmonary circulation

– Small PDAs generally asymptomatic

– Large PDAs → LA, LV overload → PH → Eisenmenger syndrome

PDA Eisenmenger

• 20 year-old, male

• Refugee from Iraq

• Suspected PH– Shortness of breath on mild exertion (last 9 months)- WHO III

• Free past Medical History

Obstructive lesions

LVOT obstruction

• BAV

– prevalence 1-2% in the population

• Subaortic Stenosis

• Supravalvular aortic stenosis

• 58 yo, female• NYHA III• Loud systolic

murmur• ECG: LV strain

Continuity equation

Aortic coarctation

• Narrowing of the aorta distal to the left subclavian artery

• 5-8% of all congenital heart defects

• Associated with other lesions of the aorta (BAV), Turner and William syndrome, congenital rubella, Takayasu disease, neurofibromatosis

• 55 yo, male

• Resistant hypertension under 4 medications

• BP gradient between upper and lower extremities (>20 mmHg),

• Radiofemoral pulse delay

• Systolic murmur over the thoracic spine

• ECG: LV strain

RVOT obstruction

RVOT stenosis

• Supravalvular

– Commonly associated with ToF, Noonan syndrome, Williams-Beuren syndrome

• Valvular (80-90%) 9% of all CHD

• Subvalvular

– Associated with other conditions (ToF, Double chambered RV)

• 41 yo, male

• Valvuloplasty due to PS (1996)

• Systolic murmur

Complex CHD

Tetralogy of Fallot (ToF)

• Most common cyanotic CHD

• 6,8% of all CHD

• Can be associated with:

– ASD (PoF)

– Right aortic arch

– Anomalous coronary arteries

– Di George syndrome

Uncorrected ToF

• Female, 17 yo

• Known heart murmur and a “hole” in the heart

• Multiple hospitalizations due to respiratory tract infections

• NYHA III

• Cyanosis, clubbing

• sO₂ 83%

Uncorrected ToF

Repaired ToF

• Echocardiographic assessment:

– PR severity

• Diastolic flow reversal in the branch pulmonary arteries, jet width, PRindex,

– Residual PS stenosis

• Severe: PG 64 mmHg, TRV > 3,5 m/sec

– RV function and dilation

– Residual VSD

Repaired ToF

• 45 yo, male

• NYHA II

• Ankle swelling

• ToF repair at the age of 10

ToF repaired

ToF repaired

d-TGA

• Venticuloarterial discordance

• Atrioventricular concordance

d-TGA

AoPA

Normal Circulation dTGAPA Ao

SVC/IVCPA PV

PA Ao

SVC/IVC

Surgical repair

• Arterial switch

• Atrial switch

– Mustard operation

– Senning operation

• Rastelli Procedure

TGA- Arterial switch

LV the systemic ventricle• Operation:

– Transection of the great vessels above the valve sinus

– Switch of the arteries• Translocation of the bifurcation of the PA anteriorly

to the Aorta

– Re-implantation of the coronary arteries

• Echocardiographic assessment– AV reg presence and severity– PA narrowing at the anastomotic site or in the

branches

Patient after arterial switch

• 17 yo, male

• NYHA II since 9 months,

• Diastolic heart murmur

• Arterial switch operation and VSD closure (2 months of age)

TGA- Atrial switch

RV the systemic ventricle

• Mustard/Senning operation: Redirect the venous blood on atrial level

– Systemic venous blood → LV

– Pulmonary venous blood → RV

TGA- Atrial switch

• Echocardiographic assessment:

– Narrowing or obstruction of the systemic venous channels → decision for stenting in symptomatic pts

– Baffle leaks

– RV size and function

– TV regurgitation → decision for surgery in symptomatic pts

– Parallel position of the great arteries

Patient after atrial switch

• 45yo, male

• Mustard operation (3 years old) (1976)

• NYHA II

• sO₂ 94%

ccTGA• 1% of CHDs

• Double discordance: Atrioventricular & Ventriculoarterial

• Great arteries run parallel

• RV supports the systemic circulation

• 90% of pts have associated cardiac defects– VSD

– Pulmonic stenosis

– Ebstein abnormality

• 26 yo, female

• Asymptomatic

• Routine control

• ECGs: P wave alternans

• 31 yo, female

• ccTGA

• TV replacement + VSD closure + PPM implantation (2001)

Ebstein’s anomaly• Rotation and apical displacement of the tricuspid

orifice into the RV

• Atrialized RV, small functional RV

• Abnormal leaflets

• Echocardiographic assessment: – Atrialized RV size

– TR severity (Systolic flow reversal in SVC and IVC) → Decision for repair

– RVOTO by the anterior leaflet

– Atrial shunt lesions

Univentricular heart

• One dominant functional pumping chamber

• Wide spectrum of conditions– Double inlet LV (most common)– Double inlet RV– Tricuspid atresia– Hypoplastic left & right heart syndrome

• Multiple associated malformations– ASD, VSD, PDA– LVOTO, RVOTO, Aortic coarctation– Discordant ventriculoarterial connections

Fontan type operations• Divert the systemic venous return to the PA without the

interposition of a subpulmonary ventricle• Echocardiographic assessment

– Dominant ventricle function and morphology– AV valve regurgitation degree– Arterial valves regurgitation or stenosis degree– Patency of pulmonary pathways, – Size of fenestrations

Conclusion

• Adult congenital heart diseases are increasingly common

• Echocardiography is a mainstay of evaluation

• Knowing what to expect is optimal

• Referral to an ACHD center is recommended

Thank You