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ACQUIRED VITIUMS Diagnostic and medical therapy of vitiums JUDIT VEGH MD PhD VALVULAR HEART DISEASE CONGENITAL ACQUIRED – AORTIC STENOSIS – AORTIC REGURGITATION – – – MITRAL STENOSIS MITRAL REGURGITATION MITRAL PROLAPSE – TRICUSPID STENOSIS – TRICUSPID REGURGITATION – PULMONARY STENOSIS – PULMONARY REGURGITATION AORTIC STENOSIS Supravalvular (connective tissue membrane) Valvular (rheumatic, cong. bicuspid aortic valve) Subvalvular (connective tissue, muscular type) AORTIC STENOSIS Ethyology – 71 % calcificated (degenerativ) – 28 % bicuspid aortic valve – 1 % rheumatic PATHOPHYSIOLOGY Tight aortic orifice Increased demand of left ventricule (systolic pressure gradient between the left ventricule and aorta) Left ventricular hypertrophy relative myocardial ischaemia Symptoms – Mainly in male persons (80%) – 40-50 year-olds – Blood pressure is low – Low pulse amplitude (tardus et parvus) – Nervous system symptoms – losing consciousness (decreased brain perfusion) – insufficient coronary flow – Left ventricule insufficiency, angina Progression – mainly 7 mmHg/yr AORTIC STENOSIS PHYSICAL FINDINGS – Ejection type systolic murmur (2R2) (transmitted to the jugular notch, carotid arteries – NOT to the axillar region) – protosystolic rasping murmur (high pressure blood stream turnes over the aortic cuspids) doesn’t change with breathing – Erb point – Tardus et parvus (low and slight) AORTIC STENOSIS ECG: – Left ventricular hypertrophy, left ventricule strain signs Carotid pulse: delayed peak, with coarse systolic vibrations Echocardiography: Chest X-Ray: aortic configuration, left ventricule hypertrophy hardly increases heart size – Characterisation of severity of the stenosis: size of orifice- area of the aortic valve mild 1.5-4 cm2 gradient below 30 mmHg moderate 0.75-1.5 cm2 gradient 30-50 mmHg severe < 0.75 cm2 gradient more than 50 Hgmm CW- flow velocity P: 4xV2 - pressure gradient – highest actual gradient (Bernoulli-formula) – monitoring left ventricular function – when is an operation required? -> symptoms, decreased left ventricular function Haemodynamic investigation – condition of coronary arteries AORTIC STENOSIS AORTIC STENOSIS AORTIC STENOSIS DISEASE HISTORY - TREATMENT – asymptomatic for a long time – When to operate? - When left ventricular function is still sufficient - below 0,75 cm2 alatt – operation indication Arteficial valve: plastic: more permanent, anticoagulation biological: lasts for 10-12 years, when anticoagulation therapy is contraindicated Balloondilation: old patient, transient effect Medication: – In case of atrial fibrillation: digitalis glycosides – Cardioversion: with amiodarone – ACE inhibitors, nitrate, beta blocker, diuretic (carefully) AORTIC REGURGITATION AORTIC VALVE CLOSURE IS INSUFFICIENT PART OF THE BLOOD STREAM IS REGURGITATING BACK INTO THE LEFT VENTRICULE DIASTOLIC OVERLOAD OF LEFT VENTRICULE SYSTOLIC OVERLOAD OF THE LEFT VENTRICULE LEFT VENTRICULE DILATION, HYPERTROPHY CARDIAC HEART FAILURE CAUSES – CUSPIDS contract (2/3-rheumatic) – come off, perforate (bacterial endocarditis) – Aortic root is expanding (Marfan syndrome) – Lues, Bechterew AORTIC REGURGITATION ACUTE CAUSES: Infectiv endocarditis Trauma Aortic dissection No LV hypertrophy Significant increase in loading volume of left ventricule during a short time Therapy: operation CHRONIC CAUSES: Degenerativ Bicuspid aortic valve Rheumatic fever Infectiv endocarditis Chronic aortic dissection Marfan’s syndrome Permanent volume overload leads to dilatation, LV hypertrophy AORTIC REGURGITATION SYMPTOMS Physical examination: decrescendo type proto-/mesodiastolic murmur 3M-4L1 Austin –Flint murmur (functional mitral stenosis) Corrigan pulse (high pulse amplitude) Musset sign: inclination of head due to pulse Quincke symptome: pulsation when pressing nail-bed Duroziez murmur: systolic and diastolic murmur over the femoral arteries ECG: diastolic, then systolic Continous chest X-ray: enlarged Echocardiography: overload left ventricule pulses visible X-ray: enlarged heart – Grade of regurgitation I-IV (Color Doppler) – Left ventricular function – Left ventricular size Haemodynamic examinations: – Condition of coronary arteries AORTIC REGURGITATION Treatment of heart failure -digitalis glycosides, diuretics, ACE inhibitors, beta blockers Operation – – – Plastic valve is preferated because of longer lasting Exeption: contraindication of anticoagulation At Grade III-IV: operation is necessary already at NYHA II-III AORTIC STENOSIS AND REGURGITATION TOGETHER Both physical examination findings Is the aortic stenosis relativ or organic ? (organic if the carotid pulses with coarse systolic vibrations) Echocardiography: grade of regurgitation seems larger then it is MITRAL STENOSIS Cuspids become fibrotic, unite, contract, scar over Mitral orifice contracts Blood is collected to the left atrium Left atrium streches, dilates pressure symptoms -> disturbancies of rate and rhythm Increased pressure in pulmonary circulation, congestion (pulmonary edema, effort dyspnoe) Accelerates the development of right ventricular hypertrophy and failure Increased pressure in main circulation (edema, nycturia – patient must sleep on a high pillow) Permanent edema, liver congestion MITRAL STENOSIS female predominance rheumatic origin - rarely a development disorder (cor triatrum) SYMPTOMS – Depend on the stage (develop early) mitral rose – small vessel configuration on the face – Physical examination: loud snapping 1st sound opening snap – valve is turned over by the blood stream praesystolic increasing diastolic murmur (in case of atrial fibrillation there is a silent, hardly recognizable murmur) ECG: P mitrale - left atrial overload CHEST XRAY: mitral configuration-enlarged left atrium, heart border passes the sternum on the right side, impresses the esophagus, pulmonary veins are wide KERLEY LINES – interlobular fluid MITRAL STENOSIS ECHOCARDIOGRAPHY: – Continous Wave: estimating transvalvular (main and maximal) blood flow velocity and gradient Pulsatile Doppler: Diastolic pressure gradient (E, A wave) Area of mitral valve: GORLIN FORMULA: MVA = 220/PHT normally 2,5-4 CM2, mild stenosis: above 1,5 cm2 moderate stenosis: 1-1,5cm2, severe stenosis: 0,6-1 cm2 – Size: dilated LA, normal LV – Intracardial thrombus CATHETERIZATION – Exact gradient can only calculated by transseptal catheterization - dangerous MITRAL STENOSIS TREATMENT Medical treatment – – – – anticoagulation (wide atrium, atrial fibrillation) Digitalis glycosides, beta blockers (AF) diuretics ACE inhibitors Operation: comissurotomy valve replacement (NYHA III)-plastic MITRAL REGURGITATION Male predominance CAUSES – – – – – – – Rheumatic fever Endocarditis Papillar dysfunction (AMI, IHD) Mitral prolapse Functional enlargment of the mitral anulus (DCM) HOCM Congenital broken mitral cuspids MITRAL REGURGITATION closure of the cuspids is insuffitient LV LA overload Pulmonary pressure is increasing pulmonary congestion LA regurgitation LV diastolic overload LV hypertrophy MITRAL REGURGITATION SYMPTOMS – Depend of the stage, mainly dyspnoe Physical findings – holosystolic murmur , pm on the apex, transmitted to the axillar region ECG: – LV-, biventricular hypertrophy – Mitrale P CHEST X-ray – Mitral configuration, left ventricular enlargement, postcapillary pressure increasement ECHOCARDIOGRAPHY – Color Doppler: Grade I-IV – Monitoring left ventricular function – Size of the left atrium, thrombus CORONAROGRAPHY - MITRAL REGURGITATION TREATMENT When condition requires treatment operation is necessary ( NYHA II ) Operation: valve replacement Medical therapy: ACE inhibitors digitalis glycosides diuretics MITRAL PROLAPSE (special case of mitral regurgitation) Cause: congenital connective tissue-weakness of the mitral apparate Wide severity scale Symptoms: neurotic complains, palpitation Physical finding: mesosystolic click Diagnosis: echocardiography Therapy: beta blockers, sedative drugs, (valve replacement) Significance: makes susceptible to endocarditis DUPLICATE MITRAL VITIUM Both physical examination findings Differential diagnosis: tricuspidal regurgitation (gets louder at breathing in, while mitral vitium gets more silent!) atrial septal defect Diagnosis: ECHOCARDIOGRAPHY NOTA BENE! : stenosis is overestimated Important to know wheather the stenosis or the regurgitation dominates! – Stenosis: treatment of the mitral stenosis – Regurgitaiton: early operation, NYHA II TRICUSPID VALVULAR DISEASES rarely alone, usually develop together with other valvulopathies REGURGITATION usually relativ (wide RV) Isolated regurg.: endocarditis, carcinoid Special case: EBSTEIN anomaly Holosystolic murmur getting louder while breathing in (4L)(Rivero-Carvallo sign) Transmitted to the liver ECG: pulmonal P XRay: wide right side ECHOCARD.: Grade I-IV RV pressure can be measured Therapy: elimination of the cause results in disappearing -if organic and significant: valvular plastic surgery biological arteficial valve STENOSIS Proto-meso diastolic and praesystolic murmur ECG: pulmonal P X-ray: wide RA Therapy: if significant: valve replacement ARTEFICIAL VALVES biograft human pork aortic valve other pericardial tissue permanent high risk of thrombosis not so long lasting not strong enough plastic made of carbon anticoagulation (INR:2,5-3,5) low risk of thrombosis Danger: Endocarditis! Any surgical procedure must be done in antibiotical protection!