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 < 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 Vicktor Eisenmenger : 32 yr women with dyspnea, cyanosis, hemoptysis  Autopsy : large VSD 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 )

1. Pulmonary arterial hypertension (PAH) 1.1 Idiopathic (IPAH) 1.2 Familial (FPAH) 1.3 Associated with (APAH): Collagen vascular disease Congenital systemic-to-pulmonary shunts Portal hypertension HIV infection Drugs and toxins 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 Pulmonary veno-occlusive disease (PVOD) 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 Etiology of pulmonary HTN

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  PVR ECHO = 10 X (TRV/TVI RVOT )  TRV/TVI RVOT ) >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 BosentanSitaxsentan Ambrisentan  Phosphodiesterase inhibitor SildenafilTadalafil

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 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

Yoshida K. Circulation 1990;81:879 Site of Acceleration Flow and Direction of MR Jet Lateral Medial A1A3 A2 P1P3P2

 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 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