Download Atrial Septal Defect

ASD with Severe PHT
R4 권성진
 Congenital heart disease in adult
- Newly diagnosed
- Already diagnosed patients without undergoing OP
: Clinically insignificant lesion or Eisenmenger syndrome
- Patients who underwent operation
Cure : VSD, PDA
New problem : TOF…
 Clinical problems in CHD
- Heart failure
- Infective endocarditis
- Pulmonary hypertension
 ASD : 2nd m/c adult congenital heart disease (20%), Acyanotic
 Type
- Ostium Secundum : m/c(75%), fossa ovalis involve, mid septal location
- Ostium Primum : 15%, artrioventricular septal defect, adjacent to
aortic valve, associated with MV or TV cleft, small VSD
- Sinus venosus : near entry of SVC (or IVC), associated with anomalous
pulmonary venous return
- Coronary sinus : rare
 Pathophysiology
Left-to-right shunt  increasing pulmonary blood flow  increased pulmonary
overcirculation leads pulmonary vascular occlusive disease
- Pulmonary hypertension
- Right ventricular failure
- Atrial arrhythmias
 Symptoms and signs
- No symptoms & signs when Qp/Qs < 1.5
- Effort breathlessness and respiratory infections
 Auscultation : wide fixed split S2, Accentuated P2, middiastolic rumbling m
 CXR : RAE, RVE, cardiomegaly, prominent pulmonary artery
 ECG : Rt axis deviation, RVE, rsR’ pattern in V1
 Echocardiographic Finding
- Volume overload & enlargement of Rt heart
- Paradoxical septal movement
- Color doppler & PW : shunt flow (Qp/Qs)
- Pul HTN : 4x(TR velocity)2 + RA Pr
- TR, PR, MR
Key Issues to Evaluate and Monitor in Adults With ASD
ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease
 Medical Mx
- Prompt treatment of respiratory tract infections
- Antiarrhythmic for AF or supraventricular tachycardia
- Usual measures for hypertension, coronary disease, or HF
 Operative repair
- Usually with a patch of pericardium or of prosthetic material
- Perrcutaneous transcatheter device closure
- Ideally in children ages 3 ~ 6
- Significant left-to-right shunt flow : Qp/Qs > 2:1
- Contraindication : severe pulmonary hypertension !
 Eisenmenger Syndrome
- 1897 Vicktor Eisenmenger
: 32 yr women with dyspnea, cyanosis, hemoptysis  Autopsy : large VSD
- 1958 Paul Wood : “Eienmenger syndrome”
- Elevated pulmonary artery resistance and severe pulmonary HT secondary to
large Lt to Rt shunt
- Reversal of shunt
- Pulmonary artery pressure
= pulmonary blood flow x pulmonary vascular resistance
 Pul HTN definition
- Mean pulmonary arterial pressure >25 mmHg (resting), >30 mmHg (exercise)
- Pulmonary vascular resistance (resting) >3 Wood units (>240 dyn/sec/cm-5)
Etiology of pulmonary HTN
1. Pulmonary arterial hypertension (PAH)
1.1 Idiopathic (IPAH)
1.2 Familial (FPAH)
1.3 Associated with (APAH):
1.3.1 Collagen vascular disease
1.3.2 Congenital systemic-to-pulmonary shunts
1.3.3 Portal hypertension
1.3.4 HIV infection
1.3.5 Drugs and toxins
1.3.6 Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary
hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenect
omy)
1.4 Associated with significant venous or capillary involvement
1.4.1 Pulmonary veno-occlusive disease (PVOD)
1.4.2 Pulmonary capillary hemangiomatosis (PCH)
1.5 Persistent pulmonary hypertension of the newborn
2. Pulmonary hypertension with left heart disease
2.1 Left-sided atrial or ventricular heart disease
2.2 Left-sided valvular heart disease
3. Pulmonary hypertension associated with lung diseases and/or hypoxemia
3.1 Chronic obstructive pulmonary disease
3.2 Interstitial lung disease
3.3 Sleep-disordered breathing
3.4 Alveolar hypoventilation disorders
3.5 Chronic exposure to high altitude
3.6 Development abnormalities
4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease
4.1 Thromboembolic obstruction of proximal pulmonary arteries
4.2 Thromboembolic obstruction of distal pulmonary arteries
4.3 Non-thrombotic pulmonary embolism (tumor, parasites, foreign material)
5. Miscellaneous
Sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangiomatosis, compression
of pulmonary vessels by adenopathy, tumor, fibrosing mediastinitis, or other process
 Echocardiographic Assessment of PA pressure
- PAPs (systolic PA pressure)
= 4 X (TR peak velocity)2 + RA pressure
- PAPm (mean PA pressure)
= 4 X (peak PR velocity)2 = 79 – 0.45 X (RVOT AT)
- PAPd (end-diastolic PA pressure)
= 4 X (PR end-diastolic velocity)2 + RAP
 Echocardiographic RV Evaluation
 Tricuspid valve : E / E’ ratio > 6 → RAP greater than 10 mmHg
 PVRECHO = 10 X (TRV/TVIRVOT) + 0.16
 TRV/TVIRVOT) >0.2 = Elevated PVR (2 WU)
 RV Tei index (Myocardial performance index) : RA pr corrleation
 Tricuspid annular plane systolic excursion (TAPSE)
 RV dp/dt
 Therapy
- Supportive Mx
- Heart failure medication
- Antiarrhythmics and implantable defibrilltors
- Anticoagulation
 Disease-targeting PAH therapy
 Prostacyclins and analogues
Epoprostenol (iv) Treprostinil (sc/iv)
Beraprost
Iloprost (inhaled/iv)
 Endothelin receptor antagonists
Bosentan
Sitaxsentan
Ambrisentan
 Phosphodiesterase inhibitor
Sildenafil
Tadalafil
MVP with Severe MR
R4 권성진
 Causes of Mitral Regurgitation
- 65% MVP or floppy mitral valve
- 27% ischemic MR
- 5% endocarditis
- 1% rheumatic, 2% others
Walter, Clin Cardiology, 1994
 MVP
 Most common valve abnormality (prevalence : 5 ~ 10%)
 Systolic displacement of MV leaflets into LA by at least 2 mm (in parasternal
view)
 Valve components larger in relation to LV
 Primary MVP : autosomal dominant
 Secondary MVP : ventriculovalvular disproportion
 Clinical course of MVP
- Broad spectrum of severities, often benign course (asymptomatic)
- Complications : VT > Valve OP > Endocarditis > CVA > SCD > VF
- Important risk factors of natural history in asymptomatic patients
Primary : EF < 50%, MR > 2+
Secondary : LA > 40mm, atrial fibrillation, age > 50 yrs
Avierinos et al. Circulation 2002; 106:1355
 Role of Echo in MVP & MR
- Diagnosis
- Define mitral leaflet involvement and morphology
- Detection and quantification of regurgitation (VC width, ERO, RV, RF)
- Asssess LV function & LA, LV, RV size, PAP
Mitral Valve Apparatus
Anatomy of Mitral Valve
Monin JL. JACC 2005;46:302
Site of Acceleration Flow and Direction of MR Jet
A2
A3
Medial
A1
Lateral
P3
P2
P1
Yoshida K. Circulation 1990;81:879
 Clinical course of MVP
- Broad spectrum of severities, often benign course (asymptomatic)
- Complications : VT > Valve OP > Endocarditis > CVA > SCD > VF
- Important risk factors of natural history in asymptomatic patients
Primary : EF < 50%, MR > 2+
Secondary : LA > 40mm, atrial fibrillation, age > 50 yrsf
Avierinos et al. Circulation 2002; 106:1355
 Role of Echo in MVP & MR
- Diagnosis
- Define mitral leaflet involvement and morphology
- Detection and quantification of regurgitation (VC width, ERO, RV, RF)
- Asssess LV function & LA, LV, RV size, PAP
Classification of the Severity of Valve Disease in Adults
ACC/AHA VHD Guidelines: 2008 Focused Update
Management Strategy for Patients With Chronic Severe MR
ACC/AHA VHD Guidelines: 2008 Focused Update