Congenital Heart Disease Adult CHD is not

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Congenital Heart Disease Congenital Heart Disease

Adult CHD is not rare ! 7 1000 Adult Survival 85  Adult CHD is not rare ! 7 1000 Adult Survival 85 %

Adult Congenital Heart Disease Newly diagnosis Previous diagnosed but not repaired Clinically insignificant lesion Eisenmenger syndromeAdult Congenital Heart Disease Newly diagnosis Previous diagnosed but not repaired Clinically insignificant lesion Eisenmenger syndrome Performed operation Cure: VSD, PDA New problems: TOF, Fontan….

Congenital Heart Disease (CHD) Simple congenital HD Atrial septal defect: ASD Ventricular septal defect: VSD PatentCongenital Heart Disease (CHD) Simple congenital HD Atrial septal defect: ASD Ventricular septal defect: VSD Patent ductus arteriosus: PDA Pulmonary stenosis: PS) Ebstein’s anomaly Aortic stenosis: AS Complex congenital HD) Tetralogy of Fallot: TOF Transposition of great arteries: TG

Clinical Problems in CHD Heart failure Infective endocarditis Pulmonary hypertension (PHT) Clinical Problems in CHD Heart failure Infective endocarditis Pulmonary hypertension (PHT)

Echo in congenital disease Diagnosis Severity Prognosis Decision of treatment  - OP Combined anomaly EvaluaionEcho in congenital disease Diagnosis Severity Prognosis Decision of treatment — OP Combined anomaly Evaluaion of cardiac function

ASD 80 80 80 99 99 998 1 0 40/ 0 100/ 0 ASD 80 80 80 99 99 998 1 0 40/ 0 100/

Diagnosis of ASD Clinical features Dyspnea, Cardiomegaly, Atrial fibrillation Direct findings Identification of shunt: 2 DDiagnosis of ASD Clinical features Dyspnea, Cardiomegaly, Atrial fibrillation Direct findings Identification of shunt: 2 D echo Confirm of shunt: Doppler Indirect findings Volume overloading of Rt heart — Enlargement of RV, RA, PV Pulmonary hypertension

Echo in ASD When we suspect ASD?  How we diagnose ASD?  What is typeEcho in ASD When we suspect ASD? How we diagnose ASD? What is type of ASD? How severe ASD? • Enlargement of RV, RA, PV • Pulmonary hypertension: TR jet velocity • Qp/Qs Combined congenital anomaly ASD size

ASD: Diagnosis 1. Identification of septal defect ( cf.  echo drop-out) 2. Confirm by DopplerASD: Diagnosis 1. Identification of septal defect ( cf. echo drop-out) 2. Confirm by Doppler ( cf. venous flow)

ASD diagnosis: Echo drop-out Normal ASD If difficult : Using color Doppler, RV enlargement ASD diagnosis: Echo drop-out Normal ASD If difficult : Using color Doppler, RV enlargement

ASD: Type S PV V Ostium secundum Most common Ostium primum Sinus venosus type ASD: Type S PV V Ostium secundum Most common Ostium primum Sinus venosus type

ASD: Secundum vs primum Secundum Primum ASD: Secundum vs primum Secundum Primum

ASD: Sinus Venosus Defect ASD: Sinus Venosus Defect

ASD: RV enlargement ASD Normal ASD: RV enlargement ASD Normal

ASD Normal. ASD: RV enlargement ASD Normal. ASD: RV enlargement

ASD Normal. ASD: RV enlargement ASD Normal. ASD: RV enlargement

ASD: Paradoxical Septal Motion Systole Diastole ASD: Paradoxical Septal Motion Systole Diastole

ASD: Pulmonary hypertension ASD: Pulmonary hypertension

ASD: Pulmonary hypertension P = 4 V 2 2 Simplified Bernoulli equation V 1 V 2ASD: Pulmonary hypertension P = 4 V 2 2 Simplified Bernoulli equation V 1 V

ASD: Pulmonary hypertension PAP systolic  = 4 V TR 2 + RAP ASD: Pulmonary hypertension PAP systolic = 4 V TR 2 + RAP

ASD: Pulmonary hypertension ASD: Pulmonary hypertension

ASD: Qp/Qs ASD: Qp/Qs

ASD: Combined anomaly Secundum ASD  mitral valve prolapse Primum ASD cleft mitral valve Sinus venosusASD: Combined anomaly Secundum ASD mitral valve prolapse Primum ASD cleft mitral valve Sinus venosus defect anomalous pulmonary venous return

MV prolapse MV prolapse

Cleft mitral valve Cleft mitral valve

ASD: Treatment Medical treatment Endocarditis prophylaxis: not recommended Surgical treatment Indication: Qp/Qs  2. 0 ASD: Treatment Medical treatment Endocarditis prophylaxis: not recommended Surgical treatment Indication: Qp/Qs 2. 0 Device closure: secundum ASD Contraindication Severe pulmonary hypertension

Device Size ( 4 - 38 mm ) ASD: Device Closure Device Size ( 4 — 38 mm ) ASD: Device Closure

Case: 45/Male, Dyspnea Case: 45/Male, Dyspnea

RV enlargement: “ASD” RV enlargement: “ASD”

Echo in ASD Unknown origin RV & RA enlargement  TEE in sinus venosus defect Echo in ASD Unknown origin RV & RA enlargement TEE in sinus venosus defect

VSD 62 80 80 99 99 998 1 0 40/0 100/ 0 VSD 62 80 80 99 99 998 1 0 40/0 100/

Echo diagnosis of VSD Clinical features Pansystolic murmur Direct findings Identify septal defect Confirm Lt toEcho diagnosis of VSD Clinical features Pansystolic murmur Direct findings Identify septal defect Confirm Lt to Rt shunt Indirect findings Rt heart volume overloadng — Enlargement of RV Pulmonary hypertension

VSD: Pansystolic Murmur VSD: Pansystolic Murmur

Echo in VSD When we suspect VSD?  Diagnosis of VSD?  What type of VSD?Echo in VSD When we suspect VSD? Diagnosis of VSD? What type of VSD? How severe VSD? RV size & function Pulmonary hypertension: TR jet vs VSD jet Qp/Qs Combined anomaly: AV prolapse, AR

VSD: Type Perimembranous Subarterial (infundibular) Muscular VSD: Type Perimembranous Subarterial (infundibular) Muscular

VSD RV side LV side VSD RV side LV side

VSD: Type VSD: Type

VSD: Perimembranous VSD: Perimembranous

VSD: Subarterial VSD: Subarterial

VSD: Type perimembranous subarterial VSD: Type perimembranous subarterial

VSD: Muscular VSD: Muscular

VSD with Septal Aneurysm VSD with Septal Aneurysm

VSD with Spontaneous Closure VSD with Spontaneous Closure

VSD: AR d/t AV prolapse VSD: AR d/t AV prolapse

VSD with pulmonary HTN VSD with pulmonary HTN

 P : 27 mm. Hg PASP: 37 mm. Hg P : 103 mm. Hg (BP: P : 27 mm. Hg PASP: 37 mm. Hg P : 103 mm. Hg (BP: 140/90) PASP: 37 mm. Hg. TR jet VSD jet. VSD with pulmonary HTN

VSD: Treatment Medical treatment  Endocarditis prophylaxis: not recommended Surgical treatment Indication: Qp/Qs  1. 5VSD: Treatment Medical treatment Endocarditis prophylaxis: not recommended Surgical treatment Indication: Qp/Qs 1. 5 ~ 2. 0 Device closure Contraindication Severe pulmonary hypertension

Patent Ductus Arterosus(PDA) Patent Ductus Arterosus(PDA)

PDA P

PDA: Physical Findings  Continuous M Wide pulse pressure Differential cyanosis Rt to Lt shunt PDA: Physical Findings Continuous M Wide pulse pressure Differential cyanosis Rt to Lt shunt

Ebstein anomaly Ebstein anomaly

Ebstein Anomaly Apical displacement of septal,  posterior leaflet of TV: 8 mm/m 22 Ebstein Anomaly Apical displacement of septal, posterior leaflet of TV: 8 mm/m

Ebstein Anomaly Apical displacement of septal,  posterior leaflet of TV: 8 mm/m 22 Ebstein Anomaly Apical displacement of septal, posterior leaflet of TV: 8 mm/m

Tetralogy of Fallot (TOF) Tetralogy of Fallot (TOF)

TOF Ventricular septal defect Pulmonic stenosis Rt ventricular hypertrophy Overriding of aorta TOF Ventricular septal defect Pulmonic stenosis Rt ventricular hypertrophy Overriding of aorta

TOF TO

TOF TO

TOF: Surgery TOF: Surgery

Clinical Problems in CHD Heart failure Infective endocarditis Pulmonary hypertension (PHT) Eisenmenger syndrome Clinical Problems in CHD Heart failure Infective endocarditis Pulmonary hypertension (PHT) Eisenmenger syndrome

Eisenmenger Syndrome 1897: Vicktor Eisenmenger  32 yo woman with dyspnea,  cyanosis Hemoptysis Autopsy: LargeEisenmenger Syndrome 1897: Vicktor Eisenmenger 32 yo woman with dyspnea, cyanosis Hemoptysis Autopsy: Large VSD 1958: Paul Wood “ Eisenmenger Syndrome”

Eisenmenger Syndrome Elevated pulmonary artery resistance and severe pulmonary HT secondary to  large L REisenmenger Syndrome Elevated pulmonary artery resistance and severe pulmonary HT secondary to large L R shunt Reversal of shunt

Eisenmenger Synd: Survival Mean age of death: 45 yr Eisenmenger Synd: Survival Mean age of death: 45 yr

Pulmonary  HT: Reversibility PA Pressure Pulmonary Blood Flow Pulmonary Vascular Resistance. X L to RPulmonary HT: Reversibility PA Pressure Pulmonary Blood Flow Pulmonary Vascular Resistance. X L to R shunt

Reversibility of Pulmonary HT 27 yo lady with dyspnea TR jet: 85 mm. Hg Reversibility of Pulmonary HT 27 yo lady with dyspnea TR jet: 85 mm. Hg

Reversibility of Pulmonary HT Post. Op 1 yr f/u Reversibility of Pulmonary HT Post. Op 1 yr f/u

2004. 12 60 mm. Hg 2005. 2 40 mm. Hg 2006. 4 25 mm. Hg 2004. 12 60 mm. Hg 2005. 2 40 mm. Hg 2006. 4 25 mm. Hg