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VALVULAR HEART DISEASE Drawing of the Heart and its Blood Vessels, from the Anatomical Notebooks, Leonardo da Vinci, Bibliotecha Ambrosiana, Milan, Italy, Late Fifteenth Century. VALVULAR HEART DISEASE INDEX 1. Aortic Incompetence. 2. Aortic Stenosis. 3. Mitral Incompetence. 4. Mitral Stenosis. 5. Mitral Valve Prolapse 6. Pulmonary Incompetence. 7. Pulmonary Stenosis. 8. Tricuspid Incompetence. 9. Tricuspid Stenosis. Appendix 1: Grading of the loudness of murmurs. Appendix 2: Auscultatory regions of the heart. Appendix 3: The JVP wave form. VALVULAR HEART DISEASE AORTIC INCOMPETENCE These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Aortic incompetence (AI) may be the consequence of diverse aetiologies, the distribution of which has changed over time. The most frequent causes of AI are now those related to aortic root disease and bicuspid aortic valve. Natural History Patients with acute AI have a poor prognosis without surgical intervention. There is little information in the literature on the progression from mild to severe AI. Patients with severe AI and symptoms have a poor prognosis. The natural history of aortic root aneurysm has been mainly studied in patients with Marfan’s syndrome. The strongest predictors of complication are the diameter of the aortic root at the level of the sinuses of Valsalva and the presence of a family history of cardiovascular events. Causes 1. 2. Valvular causes: ● Infective endocarditis, (acute onset) ● Rheumatic fever, (chronic onset) Aortic root disruptions: ● Marfans ● Hypertension ● Stanford type A dissecting aortic aneurysm. ● Congential aortic root aneurysms. ● Aortitis, (tertiary arthropathies) syphilis, rheumatoid arthritis, sero-negative Complications 1. Infective endocarditis. ● 2. Left heart failure: ● 3. Note that aortic incompetence may also be caused by endocarditis, as well as predisposing to it. Left ventricular hypertrophy and eventually failure, the heart needs to work against a volume load. In relative terms this is not as great as working against a pressure load, (as for aortic stenosis). Uncommonly angina pectoris, (possibly due to low diastolic pressures). Clinical Features In non acute cases: Symptoms 1. Initially a patient may be asymptomatic for years. 2. Exertional dyspnea is generally the first symptom. 3. Occasionally angina pectoris. 4. Palpitations, related to a hyperdynamic circulation. Signs 1. 2. 3. Pulse: ● Waterhammer, or “collapsing”. ● Prominent carotid pulsations, (“Corrigan’s sign”) Blood pressure: ● Systolic pressure mildly increased ● Diastolic pressure significantly reduced. ● Increased pulse pressure. Palpation: Apex beat: ● Hyperkinetic. ● Somewhat displaced. Thrill: ● 4. Heart sounds: ● 5. Occasionally detected at the left sternal edge, on expiration. Soft 2nd heart sound (A2) Murmur: ● Early decrescendo diastolic murmur. ● Maximal at the left sternal edge with valvular lesions, (right sternal edge with root lesions) ● Accentuated by sitting up and leaning forward with the breath held in expiration. ● May have an Austin Flint murmur, a diastolic murmur at the apex (sounds like M.S but no opening snap and S1 is soft. It is due to the regurgitant stream interfering with the anterior leaflet of the mitral valve during diastole). Clinical Indicators of Severity SIGN SEVERITY INDICATOR Pulse Collapsing nature and reflected in the blood pressure as a wide pulse pressure. Heart sounds S3 (left ventricle) and soft A2 Murmur Length of the murmur / presence of an Austin Flint murmur. Heart failure Left ventricular failure (late) Investigations CXR Check for: ● Cardiomegaly ● Signs of pulmonary congestion. ECG ● Check for signs of LHV and strain. Echocardiography Indications of severe AI on echocardiography include: 1 ● A central jet with ≥ 65% of the LV outflow tract. ● Moderate to severe LV enlargement in the absence of other aetiologies of LV enlargement. ● Regurgitant volume > 60 mls per beat. ● Regurgitant fraction ≥ 50% ● Effective regurgitant orifice area ≥ 0.3 cm2 Coronary angiography: Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical Therapy: ● In patients with chronic severe AI and heart failure, ACE-inhibitors are the treatment of choice when surgery is contraindicated or in cases with persistent postoperative LV dysfunction. ● 3. Surgical Therapy: ● 4. In patients with Marfan’s syndrome, beta-blockers slow the progression of the aortic dilatation and should also be given after operation. In patients with severe AI, the use of beta-blockers should be very cautious because the lengthening of diastole increases the regurgitant volume. However, they can be used in patients with severe LV dysfunction. The approach to the need for surgery can be summarized by the flow chart below. Screening: ● In patients with Marfan’s syndrome or in young patients with aortic root aneurysm, the family needs to be screened to detect asymptomatic cases. VALVULAR HEART DISEASE AORTIC STENOSIS These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Aortic stenosis (AS) has become the most frequent type of valvular heart disease in Europe and North America. It primarily presents as calcific AS in adults of advanced age. The second most frequent etiology, which dominates in the younger age group, is congenital, whereas rheumatic AS has now become rare. Natural History Calcific AS is a chronic progressive disease. Patients typically remain asymptomatic during long latent periods. Sudden cardiac death is a frequent cause of death in symptomatic patients but appears to be rare in the asymptomatic As soon as symptoms occur, the prognosis is dismal and mortality has been reported to be quite significant even within months of symptom onset which is often not promptly reported by patients. Causes 1. Degenerative calcific aortic valve, (most common cause in adults). 2. Calcified congenital bicuspid valve. 3. Rheumatic fever, (early adulthood) Complications 1. Excertional syncope. 2. Angina pectoris. 3. Infective endocarditis. 4. LVF, (late). 5 Sudden death. Clinical Features Symptoms 1. Initially a patient may be asymptomatic for years as the cardiac output is maintained by left ventricular hypertrophy. Once symptoms do occur, average life expectancy will be less than 5 years if untreated. 2. Exertional dyspnea is generally the first symptom. 3. Next symptoms to appear will be: 4. ● Angina pectoris, (this is not related to coronary artery disease) ● Exertional syncope ● Sudden death. Eventually signs of congestive cardiac failure, (this will indicate advanced disease with average survival time of less than 2 years) Signs 1. Pulse (carotid) ● 2. 3. Slow rising, sustained (or “plateau”) and small volume. Blood pressure: ● Reduced systolic pressure ● Reduced pulse pressure Palpation: Apex beat: ● Hyperdynamic, maybe slightly displaced. Thrill: ● 4. At the base of the heart, in the aortic area), if severe. Heart sounds: ● S4 5. ● Splitting of the second heart sound, (ie paradoxical splitting on expiration), if severe, (there is delayed left ventricular ejection and aortic valve closure) ● Soft or absent S2. ● Occasionally an early systolic ejection click, (just after the first heart sound). This will mean that the valve is mobile. Murmur: ● Harsh mid-systolic ejection murmur. ● Maximal in the 2nd right intercostal space and radiates to the carotids arteries. ● Accentuated by sitting up and leaning forward with the breath held in expiration. Clinical Indicators of Severity SIGN SEVERITY INDICATOR Pulse Plateau, also reflected in the BP, Reduced systolic and pulse pressures. Heart sounds S4, and paradoxical split of S2 Murmur Long and late peaking, +/- an associated thrill. Heart failure Left ventricular failure is a very late sign. Investigations CXR Check for: ● Cardiomegaly ● Signs of pulmonary congestion. ● Aortic valve calcification may be seen. ECG ● Check for signs of LHV and strain. Echocardiography Indications of the severity of AS on echocardiography include: 1 ● Severe: Valve area of < 1cm2 or < 0.6 cm2/ m2 BSA. ● Moderate: Valve area of 1.0-1.5 cm2 (0.6 cm2/m2 to 0.9 cm2/m2 BSA) or mean aortic gradient 30–50 mmHg in the presence of normal flow conditions. ● The combination of a markedly calcified valve (particularly without movement) with a rapid increase in peak aortic velocity of 0.3 m/s per year. ● AS with low flow gradients (<40 mmHg) and LV dysfunction with or without contractile reserve. Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical therapy: ● The progression of degenerative AS is an active process sharing a number of similarities with atherosclerosis. Thus, modification of atherosclerotic risk factors must be strongly recommended following the guidelines of secondary prevention in atherosclerosis. ● Symptomatic patients require early surgery, as no medical therapy for AS is able to delay the inevitability of surgery. ● However, patients who are unsuitable candidates for surgery may be treated with digitalis, diuretics, ACE-inhibitors, or angiotensin receptor blockers if they are experiencing heart failure. ● Beta-blockers should be avoided in AS. ● 3. Co-existing hypertension should be treated; however, treatment should be carefully titrated to avoid hypotension and patients more frequently evaluated. Surgical Therapy: ● Aortic valve replacement is the definitive therapy for severe AS. VALVULAR HEART DISEASE MITRAL INCOMPETENCE These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Mitral incompetence (MI) is now the second most frequent valve disease after AS. The treatment has been re-orientated as a result of the good results of valve repair. Causes can be grouped into organic, ischemic and functional and within these groups acute or chronic disease may occur. Organic MI covers all aetiologies in which leaflet abnormality is the primary cause of the disease, in opposition to ischaemic and functional MR, in which MR is the secondary consequence of LV disease. Natural History Organic: Acute MI is poorly tolerated and carries a poor prognosis in the absence of intervention. In asymptomatic MI, the estimated 5 year rates of death from any cause, death from cardiac causes, and cardiac events (death from cardiac causes, heart failure, or new AF) with medical management are approximately 22%, 14%, and 33%, respectively. Ischaemic: Acute MI, secondary to papillary muscle rupture, has a dismal short-term prognosis and requires urgent treatment. Patients with chronic ischaemic MI have a poor prognosis. Although coronary artery disease and LV dysfunction have prognostic importance, the presence and severity of MI are independently associated with increased mortality. Functional: The data on the natural history and results of surgery are limited. A precise analysis is difficult because of the limited number of series including small numbers of patients and mixing patients with or without revascularization. Several observational studies have shown the high prevalence of significant MI in chronic heart failure, as well as its independent association with a poor prognosis. However, its true prevalence and its pathogenic contribution to prognosis remain uncertain. Causes 1. Organic: Organic MI covers all aetiologies in which leaflet abnormality is the primary cause of the disease. These include: 2. ● Degenerative, (most common). ● Rheumatic fever. ● Infective endocarditis. ● Progression of mitral valve prolapse. ● Connective tissue diseases, (including Marfans) Ischaemic: Ischaemic MI is a frequent entity, which is, however, frequently overlooked in the setting of acute or chronic coronary disease. 3. ● Chronic ischaemic MI may result from an ACS leading to papillary muscle rupture or dysfunction. ● Chronic ischaemic MI is the consequence of a restriction in leaflet motion, which is due to tethering by the subvalvular apparatus in patients who have LV enlargement and/or dysfunction, in particular of the posterolateral wall. Functional: In this group, mitral valves are also structurally normal and MI is secondary to the changes in LV geometry resulting from impaired LV function. It includes MI seen in: ● Cardiomyopathies ● Ischaemic disease with severe LV dysfunction. Complications 1. Infective endocarditis. ● Note that mitral incompetence may also be caused by endocarditis, as well as predisposing to it. 2. Left ventricular failure. 3. Longer term, pulmonary hypertension with right ventricular failure. Clinical Features Symptoms In acute lesions there will be rapid onset of pulmonary edema. In the chronic setting there will be progressive dyspnea on exertion and features of LVF in general Signs 1. Pulse: ● 2. Blood pressure: ● 3. 4. May be reduced in severe cases. May be reduced in severe cases Apex beat: ● Displaced ● Diffuse ● Hyperdynamic. Thrill: ● May be detected in severe cases. There may be a left parasternal heave if RVH/ pulmonary hypertension is present. 5. Heart sounds: ● Soft S1 if severe. 6. Murmur: ● Harsh pan systolic murmur best heard at the apex with radiation to the axilla. Clinical Indicators of Severity SIGN SEVERITY INDICATOR Pulse Small pulse volume. Heart sounds S3 and early diastolic flow rumble. Murmur Enlarged LV Heart failure Signs of pulmonary hypertension. Investigations CXR Check for: ● Cardiomegaly ● Signs of pulmonary congestion. ECG ● Check for signs of LHV and strain. Echocardiography Indications of severe MI on echocardiography include: 1 ● Patients with severe LV dysfunction, LVEF< 30%, and/ or End systolic dimension > 55 mm ● Regurgitant volume > 60 mls per beat. ● Regurgitant fraction > 50% ● Effective regurgitant orifice area ≥ 0.4 cm2 Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical therapy (organic MI): 3. ● In acute MI, reduction of filling pressures can be obtained with nitrates and diuretics. ● Nitroprusside reduces afterload and regurgitant fraction. Inotropic agents should be added in case of hypotension. ● Anticoagulant therapy, with a target international normalized ratio (INR) range between 2 and 3, should be given in patients with MR and permanent or paroxysmal AF or whenever there is a history of systemic embolism or evidence of left atrial thrombus and during the first 3 months following mitral valve repair. ● In severe MI, maintenance of sinus rhythm after cardioversion is unlikely unless the MI is treated surgically. ● If AF occurs, heart rate should be controlled. ● There is no evidence to support the use of vasodilators, including ACEinhibitors, in chronic MI without heart failure and therefore they are not recommended in this group of patients. ● On the other hand, when heart failure has developed, ACE-inhibitors have a benefit and may be used in patients with advanced MR and severe symptoms who are not suitable for surgery or when there are still residual symptoms following the operation, usually as a result of impaired LV function. ● Beta-blockers and spironolactone should also be considered as appropriate. Surgical therapy: The general approach to surgery in patients with severe organic MI is summarized in the flow diagram below: VALVULAR HEART DISEASE MITRAL STENOSIS These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Although the prevalence of rheumatic fever has greatly decreased in industrialized countries, mitral stenosis (MS) still results in significant morbidity and mortality worldwide. Since its development 20 years ago, percutaneous mitral (balloon) commissurotomy (PMC) has impacted significantly upon the management of MS. Natural History Studies on natural history are old and non-controlled. In asymptomatic patients, survival was good up to 10 years, progression being highly variable with sudden deterioration, precipitated by complications, such as atrial fibrillation or embolism, in half of the patients. Symptomatic patients have a poor prognosis. Causes 1. Rheumatic heart disease, (nearly always the cause) Complications 1. Infective endocarditis. 2. Atrial fibrillation is common with the attendant risk of thrombo-embolic disease. 3. Pulmonary edema. 4. Pulmonary hypertension with consequent right ventricular failure. 5. Recurrent chest infections. Clinical Features Symptoms 1. Initially none, symptoms develop slowly. 2. Essentially those of left heart failure, (although the left ventricle does not fail, rather it is secondary to the increased left atrial pressures. Therefore: 3. 4. ● Exertional dyspnea. ● Orthopnea. ● Paroxysmal nocturnal dyspnea. Chest: ● Recurrent chest infections ● Occasionally hemoptysis (ruptured bronchial veins) Symptoms related to systemic emboli Signs 1. 2. Pulse ● Small volume ● AF is common. Blood pressure ● 3. May be reduced. Palpation: Apex beat ● Tapping, (reflects a loud and palpable S1) Note, the term “tapping” applies to MS, virtually by definition. Thrill ● 4. Heart sounds: ● 5. Diastolic thrill at the apex, (very rare) Loud S1 Murmur: ● A low pitched mid-diastolic “rumble”, best heard with the bell lightly held at the apex, with the patient leaning to the left side and breath held in expiration. May be accentuated by exercise. ● Opening snap may precede the murmur (a sudden tensing of an incompetently opened valve) ● The murmur may continue on with a “pre-systolic accentuation”, due to forceful left atrial systole, provided the patient is not in AF) Therefore: H1 → H2 → Opening snap → rumbling mid-diastolic murmur → pre-systolic accentuation → H1 6. Pulmonary hypertension, (late) ● Left parasternal heave ● Loud P2 ● Increased normal splitting of S2, (on inspiration) ● Increased “a” wave of the JVP (if not in AF) ● Pulmonary systolic murmur if there is associated PI, (rare) Clinical Indicators of Severity SIGN SEVERITY INDICATOR Pulse Small pulse pressure Heart sounds Soft S1,( indicates immobile cusps, S1 is usually loud in MS) Early opening snap, (due to increased left atrial pressure) Murmur Long diastolic murmur, (persists as long as there is a gradient) Heart failure Signs of pulmonary hypertension. Investigations CXR Look for: 1. Signs of pulmonary venous congestion. 2. Signs of left atrial enlargement: ● Double shadow behind the heart. ● Widened carina (> 70 degrees) ● Straightening of the left heart border. ● On the lateral, posterior displacement of the esophagus. ● On the lateral, a calcified valve, (which lies below the line joining the carina to the xiphisternum) ECG Look for: ● AF is common. ● P mitrale, if in sinus rhythm. ● Right ventricular hypertrophy / strain. Echocardiography Indications of the severity of MS on echocardiography include: 1 ● Valve area <1.5 cm2 or <1.7–1.8 cm2 in particular in cases of unusually large patients. Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medial therapy: 3. ● Diuretics or long-acting nitrates transiently ameliorate dyspnoea. ● Beta-blockers or heart-rate regulating calcium channel blockers are useful to slow the heart rate in cases of rapid AF and can greatly improve exercise tolerance by prolonging diastole and hence the time available for LV filling via the stenosed valve. ● Anticoagulant therapy with a target INR in the upper half of the range 2–3 is indicated in patients with either permanent or paroxysmal AF. ● In patients with sinus rhythm, anticoagulation is mandatory when there has been prior embolism or a thrombus is present in the left atrium, and recommended when TEE shows dense spontaneous echo contrast or in patients who have an enlarged left atrium (diameter >50 mm) ● Cardioversion is not indicated before intervention in patients with severe MS, as it does not usually restore sinus rhythm in the medium or long term. If atrial fibrillation is of recent onset and the left atrium only moderately enlarged, cardioversion should be performed soon after successful intervention. Sinus rhythm can be maintained with the use of class IC or III anti-arrhythmic drugs. Surgical Therapy: Decisions about surgical intervention are complex and will be determined by: ● The severity of the MS ● The severity of the patient’s symptoms ● The age and co-morbidities of the patient ● Echocardiographic hemodynamic parameters and predictors of PMC success. ● The expertise in a given procedure that is locally available. Options include: ● Percutaneous mitral (balloon) commissurotomy (PMC) ● Open heart mitral commissurotomy. ● Open heart surgery with mitral valve replacement. General principles of the decision making process are represented in the flow chart below: VALVULAR HEART DISEASE MITRAL VALVE PROLAPSE Introduction Despite years of research, the symptomatology and significance of mitral valve prolapse (MVP) remain controversial. Once termed the “disease of the 1980s”, the great significance attached to it has since been called into question. Initial studies that reported associated symptoms of MVP as chest pain, dyspnea, anxiety, and panic, were probably flawed by recruitment bias. Current thinking suggests that MVP could in most instances, simply reflect a normal variant rather than a single disease process. Recent studies further imply that the previously quoted incidence of MVP was overestimated by inaccurate echocardiographic diagnostic criteria and that associated symptoms, other than palpitations, are uncommon. 1 MVP is also commonly known as Barlow’s syndrome or Floppy valve syndrome. Natural History In general, primary MVP is a benign disorder with normal survival. In a small number of cases secondary complications may occur as listed below. Causes Can be primary or secondary. 1. Primary ● MVP can be identified by echocardiography in 2-4% of the general population and in most cases this finding simply reflects a normal congenital variant rather than a specific disease process. The MVP can be an inherited trait. 2. Secondary causes: In association with congenital conditions: ● It may be seen in association with other congenital cardiac abnormalities., (Ebstein anomaly/ Atrial septal defect) ● Connective tissue disorders, (Marfan syndrome and others) ● Muscle disorders, (duchenne muscular dystrophy/ myotonic dystrophy) Acquired: ● MVP may be associated with IHD ● Trauma ● Rheumatic heart disease Complications Those with associated structural abnormalities (ie, thickened, deformed, or redundant mitral valve leaflets) are more likely to suffer complications which may include: ● Endocarditis. ● Stroke. ● Progressive Mitral regurgitation. ● Syncope/ Sudden death have been described. Clinical Features Primary MVP is often detected as an incidental finding. Symptoms The following have been described: ● Atypical chest pain. ● Palpitations. ● Syncope. Sudden death due presumed arrhythmia as also been reported however this is extremely rare and in individual cases may not be related to the MVP at all. Signs 1. Pulse: ● 2. There are no specific characteristics. Blood pressure: ● 3. Palpation ● 5. There are no specific characteristics. Heart sounds: ● 6. There are no specific direct effects on the blood pressure. There may be a mid to late systolic click (possibly due to a sudden tensing of loose chordae tendinae as the leaflet prolapses) It has the characteristic of a prosthetic valve rather than the softer quality of a normal heart sound. Murmur: ● There is a late systolic murmur best heard with the patient leaning to the left with the breath held in expiration. Notes: ● Note that there may be a click only, a murmur only or a click and a murmur. ● The clicks and murmurs may vary in intensity and hence may not be heard all the time. ● Manoeuvres which reduce LV volume (sudden standing and valsalva) cause the click to come sooner (ie closer to the S1) and the murmur to become louder and longer. ● Manoeuvres which increase LV volume (squatting, passive leg raising, isometric handgrip) prevent prolapse till late in systole and the click and murmur move away from S1 toward S2 Investigations CXR: ● There are no specific features of note on CXR. ECG: ● There are no specific features of note on ECG Echocardiography: ● This will make the definitive diagnosis. Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical therapy: 3. ● Beta blockers have been advocated for troublesome symptoms due to benign arrhythmias. These are best initiated by and after the assessment of a cardiologist. ● Aspirin or even warfarin may be required in some cases where there has been a stroke or TIA or the patient develops AF. In most cases however this will not be necessary. Surgical therapy: ● This will not be necessary in the majority of primary cases, unless significant mitral incompetence develops. Careful observation by a cardiologist over time will be all that is necessary. ● In cases where the MVP is secondary to other cardiac disease the need for surgical intervention will be more likely. VALVULAR HEART DISEASE PULMONARY INCOMPETENCE Introduction Pulmonary incompetence is uncommon. It is usually remarkably well tolerated when it does occur. Natural History Pulmonary incompetence of itself is often well tolerated and the overall natural history and progress will more correlate with the underlying causative pathology. Causes 1. Chronic pulmonary hypertension from any cause. These may include: ● Primary pulmonary hypertension. ● Cor pulmonale ● Recurrent pulmonary embolism Other causes are rare: 2. Endocarditis. 3. Rheumatic heart disease. 4. Congenital lesions. Complications 1. Infective endocarditis. ● 2. Note that pulmonary incompetence may also be caused by endocarditis, as well as predisposing to it. But overall pulmonary valve endocarditis is rare. Right heart failure: Clinical Features The signs and symptoms will depend largely on the underlying pathology and its extent. Symptoms The symptoms of severe pulmonary incompetence/ pulmonary hypertension will include: ● Dyspnea ● Fatigue ● Syncope Signs 1. Pulse: ● 2. Blood pressure: ● 3. May be reduced in severe disease. May be reduced in severe disease. Palpation: Apex beat: ● Not typically displaced. ● There may be a right ventricular parasternal heave in cases of significant right ventricular strain. Thrill: ● 4. Heart sounds: ● 5. Not typically detected. An S4 may be heard, (right ventricular strain) Murmur: ● High pitched blowing diastolic murmur best heard at the second left intercostal space. ● There may be an associated mid-systolic ejection flow murmur. Investigations CXR Check for: ● Cardiomegaly ● Calcified pulmonary valve. ECG ● Check for signs of RHV and strain. Echocardiography ● This will confirm the diagnosis/ assess severity/ help determine the presence pulmonary hypertension. Coronary angiography: Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical Therapy: ● 3. Diuretics may alleviate the symptoms of right heart failure. Surgical Therapy: ● Isolated pulmonary incompetence is well tolerated and surgical intervention will only be necessary in severe cases. VALVULAR HEART DISEASE PULMONARY STENOSIS Introduction Pulmonary stenosis (PS) is a rare condition. It is usually seen in pediatric age groups as a congenital problem. Natural History Except for critical stenosis in neonates, survival is the rule in congenital PS. The long-term course of patients with mild PS is usually benign. It does not tend to progress in severity. However, untreated severe PS may result in outflow obstruction that does progresses over a period of years despite body growth, and will require intervention within 10 years of diagnosis. Causes ● An isolated congenital condition. ● As part of more complex congenital cardiac problem, such as Tetralogy of Fallot. ● Subvalvular PS may also occur as a narrowing of the infundibular or subinfundibular region, often with a normal pulmonic valve. This condition can be associated with a ventricular septal defect. Complications 1. Infective endocarditis. 2. Right heart failure Clinical Features Symptoms Those with moderately to severe PS may experience exertional dyspnea and fatigue Signs 1. Pulse: ● 2. May be reduced in severe cases. Blood pressure: ● 3. 4. Palpation ● Right ventricular heave where there is RV hypertrophy ● Thrill may be detected over the pulmonary valve in severe cases Heart sounds: ● 5. May be reduced in severe cases. Murmur mat be preceded by an ejection click Murmur: ● Harsh systolic ejection murmur maximal at 2nd left intercostal space. ● Murmur is increased by inspiration. Investigations CXR Check for: ● Cardiomegaly. ● Calcified pulmonary valve. ECG ● Check for signs of RHV and strain. ● RAD Echocardiography This will confirm the diagnosis and assess severity. The pulmonary valve area of a healthy adult is 2.0 cm2/m2 of body surface area. ● Mild valvular PS is defined by a valve area larger than 1 cm2 and a peak transvalvular gradient of less than 50 mm Hg. ● Moderately severe PS occurs if the valve area is 0.5-1.0 cm2, with a peak transvalvular gradient between 50 and 75 mm Hg. ● Severe PS is defined by a valve area smaller than 0.5 cm2 and a peak transvalvular gradient of greater than 75 mm Hg. Coronary angiography: Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical Therapy: ● 3. Diuretics may alleviate the symptoms of right heart failure. Surgical Therapy: ● Balloon valvuloplasty is the preferred option provided the valve is relatively compliant and mobile. ● Those with severe valvular fibrocalcific thickening are more likely to require a surgical approach. VALVULAR HEART DISEASE TRICUSPID INCOMPETENCE These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Minor degrees of tricuspid incompetence (TI) are commonly detected echocardiography and may be termed normal “physiological” TI. on Like MI the pathological causes of TI can grouped into organic, ischemic and functional. TI may also be associated with congenital heart disease. Natural History This will largely depend on the underlying pathology and associated pathologies. Even severe TI may be well tolerated for a long period of time. Functional TR may diminish or disappear as right ventricular failure improves following the treatment of its cause. However, TR may persist even after successful correction of left-sided lesions. Causes 1. Physiological: ● 2. 3. Organic: ● Bacterial endocarditis. This is nearly always seen in IV injecting drug addicts. ● Rheumatic fever. This is now rare in industrialized countries and is usually seen in association with left sided valvular disease is seen, or mixed (ie tricuspid stenosis and incompetence) disease. Ischaemic: ● 4. Minor degrees, very common echocardiographic finding. Papillary muscle infarction, with dysfunction or rupture in the setting of right ventricular infarction. This is much less common than ischaemic induced MI. Functional: Functional TI is due to annular dilatation and is secondary to right ventricular pressure and/or volume overload. Pressure overload may be due to: ● Pulmonary hypertension resulting from left sided heart disease. ● Cor pulmonale, due to chronic lung disease. ● Idiopathic pulmonary artery hypertension. ● Dilated cardiomyopathies in general. Volume overload may be due to: 5. ● Atrial septal defects ● Intrinsic disease of the right ventricle. Congenital ● Ebstein’s anomaly. Complications 1. Infective endocarditis ● Note that tricuspid incompetence may also be caused by endocarditis, as well as predisposing to it. ● Infective endocarditis of the tricuspid valve is especially seen with intravenous drug users. 2. Right ventricular failure 3. Cardiac cirrhosis (congestive hepatopathy) Clinical Features The signs and symptoms will predominantly relate to right sided heart failure. Symptoms These will primarily relate to cardiac failure. Signs 1. JVP ● 2. Palpation: ● 3. Elevated, often greatly so, with giant V waves, synchronous with the carotid pulse. Right ventricular heave on palpation. Murmur: A soft pan systolic murmur. 4. 5. ● This is maximal at the left lower sternal edge. ● It will increase on inspiration ● Note that whilst this murmur is often difficult to hear the diagnosis of TI can often be made on the other peripheral signs alone. Abdomen: ● Pulsatile hepatomegaly ● Possibly some associated signs of hepatic congestion, such as ascites and splenomegaly ● Hepatojugular reflux, pressure over the liver may be seen to result in an elevation of the JVP. Legs: ● Peripheral edema is common, and can be gross in chronic cases. ● Signs of chronic venous insufficiency in long standing cases, (hemosiderin deposition, varicosities, edema, venous eczema and ulceration) Clinical Indicators of Severity ● There are no well defined clinical parameters that correlate with severity. Investigations CXR ● Right atrial enlargement may be seen. ECG ● P pulmonale ● RVH and strain Echocardiography Indications of severe TI on echocardiography include: 1 ● Systolic reversal in the hepatic veins ● IVC dilation and respiratory diameter variation < 50% ● RA and RV dilation Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical therapy ● 3. Diuretics may improve signs of congestion, but attention to the underlying condition is more important. Surgical therapy ● The timing of surgical intervention and the appropriate technique remain controversial mostly due to the limited data available and their heterogeneous nature. ● As a general principle, and if technically possible, conservative surgery is preferable to valve replacement, and surgery should be carried out early enough to avoid irreversible right ventricular dysfunction. ● The possible need for correction of TI is usually considered at the time of surgical correction of left-sided valvular lesions. VALVULAR HEART DISEASE TRICUSID STENOSIS These guidelines are primarily based on the recommendations of The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology, 2007. 1 Introduction Tricuspid stenosis (TS) is almost exclusively of rheumatic origin. It is rarely observed in developed countries, although it is still seen in developing countries. Detection requires careful evaluation, as it is almost always associated with left-sided valve lesions that dominate the presentation. Natural History There is no good information on the natural history of isolated TS as the clinical picture is usually dominated by the associated left sided lesions. Causes The cause is almost exclusively rheumatic fever. Complications 1. Infective endocarditis. 2. Right heart failure. Clinical Features Symptoms Will relate to those of right heart failure. However the clinical picture is often overshadowed by features of associated left sided heart valve lesions. Signs 1. Pulse: ● 2. May be reduced in severe cases. Blood pressure: ● 3. Elevated JVP: ● 4. 5. Giant a wave may be seen with a slow y descent. Palpation: ● Apex beat is not displaced. ● Thrill is not an associated feature Heart sounds: ● 6. May be reduced in severe cases There are no particular features of note. Murmur: ● A diastolic murmur audible at the left sternal edge, accentuated by inspiration. ● It is similar to the murmur of MS, except for the site of maximal intensity and the effect of respiration, (louder on inspiration) 7. The lesion is not associated with pulmonary hypertension. 8. Abdomen: ● Presystolic pulsation of the liver caused by forceful atrial systole may be seen. Clinical Indicators of Severity These will relate to the signs of right sided heart failure. Investigations CXR ● Right atrial enlargement ● Calcified tricuspid valve. ECG ● Look for evidence of P pulmonale. Echocardiography ● No generally accepted grading of TS severity exists. ● A mean gradient of > 5 mm Hg is considered indicative of clinically significant TS. Coronary angiography: ● Coronary angiography is indicated in selected cases to detect associated coronary artery disease when surgery is planned. Knowledge of coronary anatomy improves risk-stratification and determines whether coronary revascularization is indicated in association with valvular surgery. Management 1. Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic guidelines) 2. Medical therapy: ● 3. In the presence of heart failure, diuretics are useful but of limited efficacy. Surgical therapy: ● Percutaneous balloon tricuspid dilatation has been performed in a limited number of cases, either alone or alongside PMC, (for the mitral valve) but frequently induces significant regurgitation. Data on evaluation of longterm results are lacking. ● Intervention on the tricuspid valve is usually carried out at the time of intervention on the other valves in patients who are symptomatic despite medical therapy. ● Conservative surgery or valve replacement, according to anatomy and surgical expertise in valve repair, is preferred to balloon commissurotomy, which can only be considered as a first approach in the rare cases of isolated TS Appendix 1 Grading of the loudness of murmurs: Grade 1 Very soft, requires an experienced listener. Grade 2 Soft. Grade 3 Moderate and without a thrill Grade 4 Loud with thrill just palpable. Grade 5 Very loud and thrill easily palpable. Grade 6 Very loud, may be heard without the aid of a stethoscope. Appendix 2 Auscultatory regions of the heart: Note, these regions show the optimal areas for listening to the heart valve indicated, they do not exactly correlate with surface anatomy of the anatomical location of the valve. Appendix 3 The JVP wave form: Components of the jugular venous pressure wave with relationships to the first and second heart sounds. References 1. Guidelines on the Management of Valvular Heart Disease. The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. European Heart Journal January 2007 28: 230-268. 2. Talley N.J, Clinical Examination 3rd ed 1996. Dr. J.Hayes 4 October 2007