138 Aortic valve disease Aortic stenosis (AS) Causes: Senile calcification is the commonest. Al so congenital bicuspid valve. Presentation: Angina, dyspnea, dizziness, syncope, systemic e mboli if infec- tive endocarditis, CCF, sudden death. Signs : Slow rising pulse with narrow pulse pressure (feel for diminished and delayed carotid upstroke \200\224 \200\234parvus e t tardus \200\235); heaving, undisplaced apex beat; LV heave; aortic thrill; ejection systo lic murmur (heard at the base, left sternal edge and the aortic area; radiates to the carot ids). As stenosis worsens, A 2 is increasingly delayed, giving first a single S 2 and then reversed splitting. But this sign is rare. More common is a quiet A 2 . In severe AS, A 2 m ay be inaudible (calcified valve). There may be an ejection click (pliable valve) or an aud ible S 4 (said to occur more commonly with bicuspid valves, but not in all populations). Te sts: ECG: P-mitrale, LVH with strain pattern; LAD (left anterior hemi- block); poor R wave progression; LBBB or complete AV block (calcified ring). CXR: LVH; calcified aortic valve; poststenotic dilatation of ascending aorta. Echo is diagnostic (p. 104). Doppler echo can e stimate the gradient across valves: Severe stenosis if gradient >50 mm Hg and valve area <0 .5 cm 2 . If the aortic jet velocity is >4 m/sec (or is ^ by >0.3 m/sec per year) risk of c omplications is increased. Cardiac catheter can assess valve gradient, LV function, coronar y artery disease, the aortic root. Differential diagnosis: HOCM. Management: Symptomatic pa tients have a poor prognosis: 2 \200\2233-yr survival if angina/syncope; 1 \200\2232-yr sur vival with cardiac failure. Prompt valve replace- ment (p. 140) is recommended. In asymptom atic patients with severe AS and a deteriorating ECG, valve replacement is also recommended . If the patient is not medically fit for surgery, percutaneous valvuloplasty may be attemp ted. Endocarditis prophylaxis (p. 143). Percutaneous valve replace- ment is still experimen tal but may be approved soon. Aortic sclerosis is senile degeneration of the valve. There i s an ejection systolic murmur, no carotid radiation, and a normal pulse and S 2 . Aortic re gurgitation (ar) Causes : Congenital valve disease : Rheumatic fever, infective endocarditi s, rheumatoid arthritis, SLE, pseudoxanthoma elasticum, appetite suppressants (e.g., fenflu ramine, phentermine). Aortic root disease: Hypertension, trauma, aortic dissection, seroneg ative spon- dyloarthritis (ankylosing spondylitis, reactive arthritis, psoriatic arthritis) , Marfan \200\231s syndrome, osteogenesis imperfecta, syphilitic aortitis. Symptoms: Dyspne a, palpitations, cardiac Signs: Collapsing (water-hammer) pulse, wide pulse pressure, displ aced, hyperdynamic apex beat, high-pitched early diastolic murmur (heard best in expiration , with patient sitting forward). Associated signs: Corrigan \200\231s sign (carotid pulsa- tion), Quincke \200\231s sign (capillary pulsations in nail beds), Duroziez \200\231s sign (femoral diastolic murmur as blood flows backwards in diastole), Traube \200\231s sign ( \200\234pistol shot \200\235 sound over femoral arteries). In severe AR, an Austin Flint mu rmur may be heard (p. 53). Investigations: ECG: LVH. CXR: Cardiomegaly, dilated ascending a orta, pul- monary edema. Echocardiography is diagnostic. Cardiac catheterization to assess severity of lesion, anatomy of aortic root, LV function, coronary artery disease, other val ve disease. Management: Indications for surgery: ^ Symptoms, enlarging heart on CXR/echo, E CG deterioration (T wave inversion in lateral leads), infective endocarditis refractory to medical therapy. Aim to replace the valve before significant LV dysfunction occurs. Endocar ditis prophylaxis (p. 143). e n i c i d e m r a l u c s a v o d r a C i 139 failure. Right-heart valve disease Tricuspid regurgitation Causes : Pulmonary hypertension, rheumati c fever, infective endocarditis (IV drug abusers), carcinoid syndrome, con- genital (e.g., ASD, AV canal, Ebstein \200\231s anomaly). Symptoms: Fatigue, hepatic pain on exertion, asc ites; edema. Signs: Giant v waves and prominent y descent in JVP (p. 49); RV heave; pansyst olic mur- mur, heard best at lower sternal edge in inspiration; pulsatile hepatomegaly; jau ndice; ascites. Management: Treat underlying cause. Drugs : Diuretics, digoxin, ACE-inhibit ors. Valve replacement (20% operative mortality). Tricuspid stenosis Causes: Rheumatic feve r; almost always occurs with mitral or aortic valve disease. Symptoms: Fatigue, ascites, ed ema. Signs: Giant a wave and slow y descent in JVP (p. 49); opening snap, early diastolic m ur- mur heard at the left sternal edge in inspiration. Diagnosis: Doppler echo. Treatment: Diuretics; surgical repair. Pulmonary stenosis Causes : Usually congenital (Turner \200\231 s syndrome, Noonan \200\231s syndrome, William \200\231s syndrome, Fallot \200\231s tetralo gy, rubella). Acquired causes : Rheumatic fever, carcinoid syndrome. Symptoms: Dyspnea, fat igue, edema, ascites. Signs: Dysmorphic facies (congenital causes); promi- nent a wave in J VP; RV heave. In mild stenosis, there is an ejection click, ejection systolic murmur (which radiates to the left shoulder); widely split S 2 . In severe stenosis, the murmur becomes longer and obscures A 2 . P 2 becomes softer and may be inaudible. Tests: ECG: RAD, P-pulmo nale, RVH, RBBB. CXR : Poststenotic dilatation of pulmonary artery; RV hypertrophy; right a trial hypertrophy. Cardiac catheterization is diagnostic. Treatment: Pulmonary valvuloplast y or valvotomy. Pulmonary regurgitation is caused by any cause of pulmonary hyper- tension (p. 195). A decrescendo murmur is heard in early diastole at the left sternal edge (the Gra ham Steell murmur). 140 Cardiac surgery Valvuloplasty can be used in mitral or pulmonary stenosis (pliable, non- ca lcified valve, no regurgitation). A balloon catheter is inserted across the valve and infla ted. Valvotomy Closed valvotomy is rarely performed now. Open valvotomy is performed under cardiopulmonary bypass through a median sternotomy. Valve replacements Mechanical valves ma y be of the ball-cage (Starr \200\223 Edwards), tilting disc (Bj rk \200\223Shiley), or double tilting disc (St. Jude) type. These valves are very durable but the risk of thromboe mbolism is high; patients require lifelong anticoagulation. Xenografts are made from por- c ine valves or pericardium. These valves are less durable and may require replacement at 8 \200\22310 yrs. Anticoagulation is not required unless there is AF. Homografts are cadaveri c valves. They are particularly useful in young patients and in the replacement of infected valves. Complications of pros- thetic valves : Systemic embolism, infective endocarditis, hemolysis, struc- tural valve failure, arrhythmias. CABG see Table 5.13. Cardiac transplant ation Consider this when cardiac disease is severely curtailing quality of life and surviva l is not expected beyond 6 \200\22312 months. Refer to a specialty center. Main contraindic ations: Malignancy, age >70, significant peripheral or cerebrovascular disease, DM with end -organ dys- function, noncompliance. e n i c i d e m Table 5.13 Coronary artery bypass grafts Indications for cabg: To improve survival \212 \212 Left main disease Triple vessel disease involving proximal part of the left anterior descen ding, especially with LV dysfunction r a l u c s a v o d r a C To relieve symptoms \212 \212 \212 Procedure: Surgery is planned based on the result of angiograms. Not all stenoses are bypassable. The heart is stopped and blood is oxygenated a nd pumped artificially by a machine outside the body (cardiac bypass). The patient \200\231 s own veins (saphenous) or arteries (internal mammary or radial)are used as the graft. Seve ral grafts may be placed. >50% of vein grafts close in 10 yrs (low-dose aspirin helps preve nt this). Arterial grafts last longer. After CABG: If angina persists or recurs (from poor run-off from the graft, distal disease, new atheroma, or graft occlusion), restart antiangi - nal drugs and consider angioplasty (repeat surgery has increased risk). Mood, sex, and in tellectual problems are common early. Cardiac reha- bilitation improves survival: \212 \212 \212 \212 \212 Angina unresponsive to drugs Unstable angina (sometimes) If angioplasty is unsuccessful i Exercise: Walk Drive at 4 \200\2238 wks: No need to tell DMV Get back to work (e.g., at 3 m onths) Address smoking cessation, BP, lipids Aspirin, statin for life \206\222 cycle \206\222 swim \206\222 jog 141 Infective endocarditis Fever + new murmur = endocarditis until proven otherwise . Classific ation \212 50% of all endocarditis occurs on and presents with acute heart failure. abnormal valves te nds to run a subacute course . Endocarditis on Predisposing cardiac lesions: Aortic or mitr al valve disease, tricuspid valves in IV drug users, coarctation, patent ductus arteriosus, VSD, prosthetic valves. Endocarditis on prosthetic valves may be \200\234early \200\235 ( acquired at the time of surgery, poor prognosis) or \200\234late \200\235 (acquired hemato genously). Causes Bacteria: Any cause of bacteremia exposes valves to the risk of bac- teri al colonization (e.g., dental work, UTI, urinary catheterization, cystos- copy, respiratory infection, endoscopy (controversial), colonic carcinoma, gall bladder disease, skin diseas e, IV cannulation, surgery, abortion, fractures). Quite often, no cause is found. Streptoco ccus viridans is the commonest (35 \200\22350%). Others: Enterococci, Staphylococcus aureus /epidermidis, diphthe- roids and microaerophilic streptococci. Rarely : HACEK group of Gram \200\223ve ( Haemophilus \200\223 Actinobacillus \200\223 Cardiobacterium \200\223 Eik enella \200\223 Kingella ), bacteria Coxiella burnetii , Chlamydia . Fungi: These include Candida, Aspergillus, and Histoplasma . Other causes : SLE (Libman \200\223Sacks endocardit is), malignancy. Clinical features The patient may present with any of the following: Signs of infection: Fever, rigors, night sweats, malaise, weight loss, anemia, sple- nomegaly, a nd clubbing. Cardiac lesions: Any new murmur or a change in the nature of a preexisting mur mur should raise the suspicion of endo- carditis. Vegetations may cause valve destruction a nd severe regurgitation normal valves . It follows an acute course \212 or valve obstruction. An aortic root abscess causes prolongation of the P \200\223R interva l and may lead to complete AV block. LVF is a common cause of death. Immune complex deposit ion: Vasculitis (p. 416) may affect any ves- sel. Microscopic hematuria is common; glomerul onephritis and acute renal failure may occur. Roth spots (boat-shaped retinal hemorrhage wi th pale center), splinter hemorrhages (on finger or toe nails), Osler \200\231s nodes (pain - ful pulp infarcts in fingers or toes) and Janeway lesions (painless palmar or plantar mac ules) are pathognomonic. Embolic phenomena: Emboli may cause abscesses in the relevant orga n, e.g., brain, heart, kidney, spleen, GI tract. In right-sided endocarditis, pulmonary abs cesses may occur. Diagnosis The Duke criteria for definitive diagnosis of endocarditis are given. Blood cultures: Take three sets at different times and from different sites at peak fever. 85 \200\22390% are diagnosed from the first two sets; 10% are culture-negative. Bloo d tests: Normochromic, normocytic anemia, neutrophil leukocytosis, high ESR/CRP. Also check electrolytes, Mg 2+ , LFTs. Urinalysis for microscopic hematuria. CXR (cardiomegaly) and E CG (prolonged P \200\223R interval) at regular intervals. Echocardiography TTE may show veg etations, but only if >2 mm. TEE is more sensitive, and better for visualizing mitral lesio ns and possible development of aortic root abscess. See Table 5.14. Management Consultation with Infectious Disease and Cardiology should be considered. \212 \212 Antibiotics (see Table 5.15) Consider surgery if: Heart failure, progressive heart block, v alvular obstruction; repeated emboli or cerebral emboli; fungal endocarditis; persistent ba cteremia; myocardial abscess; unstable infected prosthetic valve. 142 Prognosis 30% mortality with staphylococci; 14% with bowel organisms; 6% with sensitive str eptococci. Table 5.14 Duke criteria for infective endocarditis Major criteria: \212 Positive blood culture: \212 \212 Typical organism in two separate cultures or Persistently +ve blood cultures, e.g., three, >12 h apart (or majority if \211 4) \212 Endocardium involved: \212 +ve echocardiogram (vegetation, abscess, dehiscence of prosthetic valve) or New valvular re gurgitation (change in murmur not sufficient) \212 Minor criteria: \212 \212 \212 \212 \212 How to diagnose: Definite infective endocardi tis: Two major or one major and three minor or all five minor criteria (if no major criteri on is met). Predisposition (cardiac lesion; IV drug abuse) Fever >38 C Vascular/immunological signs +v e blood cultures that do not meet major criteria +ve echocardiogram that does not meet majo r criteria e n i c i d e m Table 5.15 Antibiotic therapy for infective endocarditis \212 Consult Infectious Disease for the appropriate regimen based on bac- terial sensitivities i n your hospital. The following are guidelines only: \212Empirical therapy: Nafcillin or ox acillin 2 g/4 h IV + gentamicin or tobramycin 1 mg/kg q12h IV. r a l u c s a v o d r a C \212Streptococci: Penicillin (PCN) G 20 \200\22330 million U/d IV for 4 \200\2236 wks; the n amoxicillin 1 g/8 h PO for 2 wks. Monitor minimum inhibitory concen- tration (MIC) and ad d gentamicin 1 mg/kg q8 \200\22312h IV for the first 2 wks. Monitor gentamicin levels. \212Enterococci: Ampicillin a 1 g/6 h IV + gentamicin 1 mg/kg q8 \200\22312h IV for 4 wks. Monitor gentamicin levels. i \212Staphylococci : Oxacillin or nafcillin 2 g/4 h IV + gentamicin 1 mg/kg q12h IV. Treat for 6 \200\2238 wks; stop gentamicin after 1 wk. \212Coxiella: Doxycycline 100 mg/12 h PO indefinitely + co-trimoxazole, rifampin, or ciprofloxacin. \212Fungi: Flucytosine 3 g/6 h IVI over 30 mins followed by fluconazole 50 mg/24 h PO (a h igher dose may be needed). Amphotericin if flucyto- sine resistance or Aspergillus . Micona zole if renal function is poor. a For penicillin allergy, use vancomycin 1 g/12 h IV Prevention of endocarditis Anyone with congenital heart disease, acquired valve disease, or prosthetic valves is at risk of infective endocarditis and should take prophylactic anti- biotics before procedures that may result in bacteremia. The recommenda- tions of the Ameri can Heart Association are: 143 Which conditions? Prophylaxis recommended \212 Prophylaxis not recommended \212 Prosthetic valve(s) Mitral prolapse (no regurgitation) Functional/innocent murmur \212 \212 \212 \212 \212 Which procedures? Prophylaxis recommended \212 \212 \212 \212 \212 Previous endocarditis Septal defects Mitral prolapse with regurgitation Acquired valve dise ase Surgical shunts \212 Prophylaxis not recommended \212 Flexible bronchoscopy \212 Diagnostic upper GI endoscopy \212 TEE ( \212 Cesarean or normal delivery Cardiac catheterization \212 high-risk pati ent) Dental procedures Upper respiratory tract surgery Esophageal dilatation Sclerotherapy of va rices Surgery/instrumentation of lower bowel, gall bladder, or GU tract p. 104) (unless Which regimen? \212Dental procedures \212Local or no anesthetic: Amoxicillin 2 g PO, 1 h before the procedure. Alternative (if penicillin allergy or >1 dose of penicillin in previous month): Clindamycin 600 mg PO or ce phalexin 2 g PO or azithromy- cin/clarithromycin 500 mg 1 h pre procedure. \212Intolerant of oral meds : Ampicillin 2 g IV 30 min preprocedure. \212Upper respiratory tract procedures As for dental procedures. \212 \212Gastrointestinal and genitourinary procedures \212High risk (prosthetic valve, prior endocarditis, surgical shunt): Ampicillin 2 g IM/IV + gentamicin 1.5 mg/kg IV/IM 30 min preprocedure. Vancomycin 1 g IV + gentamicin if penici llin allergy. \212Moderate risk : Amoxicillin 2 g PO 1 h preprocedure. Vancomycin 1 g IV if penicillin allergy. Diseases of heart muscle Acute myocarditis Causes : Inflamed myocardium from viruses (cox- sackie, enterovirus, adenovirus, HIV, and others); bacteria ( Clostridia , diph- theria, Me ningococcus, Mycoplasma , psittacosis); spirochetes ( Leptospirosis , syphilis, Lyme diseas e); protozoa (Chagas \200\231 disease); drugs; toxins; vasculitis (p. 416). Signs and sympt oms: Fatigue, dyspnea, chest pain, palpitations, tachycardia, soft S 1 , S 4 gallop. Tests: ECG: ST segment elevation/depression, T wave inversion, atrial arrhythmias, transient AV b lock. Serology is rarely helpful for acute management. Management: Treat the underlying cau se. Supportive measures. Patients may recover or develop intractable heart failure (p. 128) . Dilated cardiomyopathy A dilated, poorly contractile heart of unknown cause. Associations : Alcohol, BP ^ , hemochromatosis, viral infection, autoimmune, peri- or postpartum, thyrot oxicosis, congenital (X-linked). Prevalence: 0.2%. Presentation: Fatigue, dyspnea, pulmonar y edema, RVF, emboli, AF, VT. Signs: ^ Pulse, BP, ^ JVP, displaced, diffuse apex, S 3 ga llop, mitral or tricus- pid regurgitation (MR/TR), pleural effusion, edema, jaundice, hepat omegaly, ascites. Tests: CXR: Cardiomegaly, pulmonary edema. ECG : Tachycardia, nonspe- cif ic T wave changes, poor R wave progression. Echo: Globaly dilated hypokinetic heart with lo w ejection fraction. Look for MR, TR, LV mural thrombus. Management: Bed rest, diuretics, d igoxin, ACE-inhibitor, -blockers, anti coagulation. Consider cardiac transplantation. Mo rtality: Variable depending on severity of symptoms. Hypertrophic cardiomyopathy HOCM from LV outflow tract (LVOT) obstruction from asymmetric septal hypertrophy. Prevalence: 0.2%. A utosomal dominant inheritance, but 50% are sporadic. 70% have mutations in genes encoding -myosin, -tropomyosin, and troponin T. May present at any age. Ask about family histor y of sudden death. The patient: Angina, dyspnea, palpitation, syncope, sudden death (VF is amenable to implantable defibrillators). Pulse with rapid upstroke, a wave 144 in JVP, double apex beat, systolic thrill at lower left sternal edge, harsh ejection systol ic murmur. Tests: ECG: LVH, progressive T wave inversion, deep Q waves (inferior + lateral leads), AF, WPW syndrome (p. 121), ventricular ectopy, VT. Echo : Asymmetrical septal hyper trophy, small LV cavity with hypercontractile pos- terior wall, midsystolic closure of aort ic valve, systolic anterior movement of mitral valve. Cardiac catheterization may provoke V T. It helps assess severity of gradient, coronary artery disease or mitral regurgitation. E lectrophysiological studies may be needed if arrhythmias. Exercise test, Holter monitor, an d echo to risk stratify yearly for increased risk of sudden death. Management : \222 -bloc kers or verapamil for symptoms (p. 112). Amiodarone 100 \200\223200 mg/d for arrhythmias (A F, VT). Anticoagulate for paroxysmal AF or systemic emboli. Septal myectomy (surgical, or c hemical, with alcohol, to reduce LV outflow tract gradient) is reserved for those with seve re symptoms. Consider implantable defibrillator if patient has risk factors for sudden deat h (VT on Holter, family history of sudden death, syncope, drop in BP pressure with exercise , wall thickness >3 cm). Mortality: 5.9%/yr if <14 yrs; 2.5%/yr if >14 yrs. Poor prognostic factors: Age < 14yrs or syncope at presentation; family history of HOCM/sudden death. Rest rictive cardiomyopathy Causes: Amyloidosis, hemochromatosis, sarcoi- dosis, scleroderma, L ffler \200\231s eosinophilic endocarditis, endomyocardial fibrosis. Presentation is like constrictive pericarditis (p. 147). Features of RVF predomi- nate: ^ JVP, with prominent x and y descents, hepatomegaly, edema, ascites. Diagnosis: Echo, cardiac catheterization, +/ - endomyocardial biopsy, MRI. Cardiac myxoma Rare benign cardiac tumor. Prevalence \211 5 /10,000, \231\200 : \231\202 2:1. Usually sporadic, may be familial (autosomal dominan t). It may mimic infective endocarditis (fever, weight loss, clubbing, ^ ESR), or mitral st enosis (left atrial obstruction, systemic emboli, AF). A \200\234tumor plop \200\235 may b e heard, and signs may vary according to posture. Tests: Echocardiography, CT or MRI. Treat ment : Excision. e n i c i d e m r a l u c s a v o d r a C i 145 The heart in various, mostly rare, systemic diseases See Table 5.16. Table 5.16 The heart in various, mostly rare, systemic diseases This list reminds us to look at the heart and the whole patient, not just in exams (where t hose with odd syndromes congregate), but always. Acromegaly (p. 324): BP ^ , LVH, hypertrop hic cardiomyopathy, high-output cardiac failure, coronary artery disease Amyloidosis (p. 635): Restrictive cardiomyopathy Ankylosing spondylitis (p. 402): Conductio n defects, AV block, AR Beh et \200\231s disease (p. 416): AR, arterial venous thromb i Cushing \200\231s syndrome (p. 309): Hypertension Down \200\231s syndrome: ASD, VSD, mitr al regurgitation Ehlers \200\223Danlos syndrome: Mitral valve prolapse + hyperelastic skin aneurysms and GI bleeds. Joints are loose and hypermobile; mutations exist, e.g., in gen es for procollagen (COL3A1); there are six types. Friedreich \200\231s ataxia: Hypertrophic cardiomyopathy Hemochromatosis (p. 216): AF, card iomyopathy (Continued) Table 5.16 (Continued) Holt \200\223Oram syndrome: ASD or VSD with upper limb defects Human immunodeficiency virus (p. 566): Myocarditis, dilated car- diomyopathy, effusion, ventricular arrhythmias, SBE/IE , noninfective thrombotic (murantic) endocarditis, RVF (pulmonary hypertension), metastatic Kaposi \200\231s sarcoma Hypothyroidism (p. 301): Sinus bradycardia, low pulse pressure, pericar- dial effusion, coronary artery disease, low-voltage ECG Kawasaki disease: Coronary arteritis similar to PAN; more common than rheumatic fever as a cause of acquired heart disease. Klinefelter \200\231s syndrome: Male; ASD. Psychopathy, learning difficulties, libi- do , gynecomastia, sparse facial hair and small firm testes; XXY Marfan \200\231s syndrome: Mitral valve prolapse, AR, aortic dissection. Look for long fingers and a high-arched palate. Noonan \200\231s syndrome: ASD, pulmonary stenosis low-set ears PAN (p. 416): Small and medium vessel vasculitis + angina, MI, arrhyth- mias, CHF, pericarditis and conduction defects Rheumatoid nodules: Conduction defects, pericarditis, LV dysfunction, AR, coronary arteritis. Look for arthritis signs (p. 399). Sarcoidosis (p. 192): Infiltrating granulomas may cause complete AV block, ventricular or s upraventricular tachycardia, myocarditis, CHF, restrictive cardiomyopathy; ECG may show Q w aves. Syphilis (p. 587): Myocarditis, ascending aortic aneurysm Systemic lupus erythematosu s (p. 410): Pericarditis/effusion, myocarditis, Libman \200\223Sacks endocarditis, mitral v alve prolapse, coronary arteritis Systemic sclerosis (p. 410): Pericarditis, pericardial ef fusion, myocardial fibrosis, myocardial ischemia, conduction defects, cardiomyopathy Thyrot oxicosis (p. 298): Pulse ^ , AF emboli, wide pulse pressure, hyper- dynamic apex, loud h eart sounds, ejection systolic murmur, pleuro- pericardial rub, angina, high output cardiac failure 146 Turner \200\231s syndrome: Female; coarctation of aorta. Look for webbed neck; XO. William \200\231s syndrome: Supravalvular aortic stenosis (visuospatial IQ ) Pericardial diseases Acute pericarditis Inflammation of the pericardium that may be primary or secondary to systemic disease. Causes: \212 \212 \212 \212 \212 \212 \212 \212 \212 \212 \212 \212 \212 \212 Viruses (coxsackie, flu, Epstein \200\223Barr, mumps, varicella, HIV) Bacteria (pneumonia, rheumatic fever, TB) Fungi Myocardial infarct Dressler \200\231s syndrome Uremia Rheumatoid arthritis SLE Myxedema Trauma Surgery Malignancy Radiotherapy Procainamide; hydralazine Clinical features: Central chest pain worse on inspiration or lying flat relief by sitti ng forward. A pericardial friction rub may be heard. Look for evidence of a pericardial eff usion or cardiac tamponade (see below). Tests: ECG classically shows concave (saddle-shaped ) ST segment eleva- tion, but may be normal or nonspecific (10%). Can also see PR depressio n. Blood tests: CBC, ESR, electrolytes, cardiac enzymes, viral serology, blood cultures, an d, if indicated, autoantibodies, fungal cultures, thyroid function tests. Cardiomegaly on c xr may indicate a pericardial effusion. Echo to eval- uate cardiac function and evaluate fo r effusions. Treatment: Analgesia, e.g., ibuprofen 400 mg/8 h PO with food. Treat the cause . Consider colchicine before steroids/immunosuppressants if relapse or continuing symptoms occur. 15 \200\22340% DO recur. Pericardial effusion Accumulation of fluid in the pericardi al space. Causes : Any cause of pericarditis (see above). Clinical features: Dyspnea, ^ JVP (with prominent x descent [p. 49]), bron- chial breathing at left base (Ewart \200\231s si gn: Large effusion compressing left lower lobe). Look for signs of cardiac tamponade (see b elow). Diagnosis: CXR shows an enlarged, globular heart. ECG shows low-voltage QRS complexe s and alternating QRS morphologies (electrical alternans). Echocardiography shows an echo-f ree zone surrounding the heart. Management: Treat the cause. Pericardiocentesis may be diag nostic (sus- pected bacterial pericarditis) or therapeutic (cardiac tamponade). Send peri- cardial fluid for culture, TB culture, and cytology. Constrictive pericarditis The heart is encased in a rigid pericardium. Causes : Often unknown; also TB or after any pericarditis. Clinical features: These are mainly of right-heart failure with ^ JVP (with prominent x an d y descents [p. 49]); Kussmaul \200\231s sign (JVP rising paradoxi- cally with inspiration ); soft, diffuse apex beat; quiet heart sounds; S 3 ; dia- stolic pericardial knock, hepato splenomegaly, ascites, and edema. Tests: CXR: Small heart pericardial calcification (if none, CT/MRI helps distinguish from other cardiomyopathies). Echo : Cardiac catheterization . Management: Surgical excision. Cardiac tamponade Accumulation of pericardial fluid raises intrapericar- dial pressure, hence poor ventricular filling and fall in cardiac output. Ca uses : Any pericarditis (above), aortic dissection, hemodialysis, warfarin, trans-septal pu ncture at cardiac catheterization, post cardiac biopsy. Signs: Pulse ^ , BP , pulsus par adoxus, JVP ^ , Kussmaul \200\231s sign, muffled S 1 and S 2 . Diagnosis: Beck \200\231s tr iad: Falling BP; rising JVP; small, quiet heart. cxr : Big globular heart (if >250 mL fluid ). ecg : Low-voltage QRS electrical alter- nans. Echo is diagnostic : Echo-free zone (> 2 cm or >1 cm if acute) around the heart diastolic collapse of right atrium and right ve ntricle. Management: Seek expert help. The pericardial effusion needs urgent drain- age. Se nd fluid for culture, ZN stain/TB culture and cytology. e n i c i d e m r a l u c s a v o d r a C i 147 Figure 5.28 Pericarditis I III aVR aVL aVF II V1 V2 V3 V4 V5 V6 Congenital heart disease The spectrum of congenital heart disease in adults is considerably differ- ent from that in infants and children; adults are unlikely to have complex lesions . The commonest lesions, in descending order of frequency, are: Bicuspid aortic valve These function well at birth and go undetected. Most eventually develop AS (requiring valve repl acement) and/or AR (pre- disposing to IE/SBE)(p. 141). Atrial septal defect A hole connects the atria. Ostium secundum defects (high in the septum) are most common; ostium primum def ects (opposing the endocardial cushions) are associated with AV valve anomalies. Primum ASD s present early. Secundum ASDs are often asymptomatic until adult- hood, as the L R shun t depends on compliance of the right and left ventricles. The latter decreases with age (es p. if BP ^ ). This augments L R shunting, causing dyspnea and heart failure, usually by age 40 \200\22360. There may be pulmonary hypertension, cyanosis, arrhythmia, hemoptysis, a nd chest pain. Signs: AF; ^ JVP; wide, fixed split S 2 ; pulmonary ejection systolic murmur . Pulmonary hypertension may cause pulmonary or tricuspid regurgitation. Complications: Par adoxical embolism (rare). Reversal of left to right shunt ( Eisenmenger complex ). Eisenmen ger complex is a congenital heart defect at first associated with a left-to-right shunt, wh ich may lead to pul- monary hypertension and shunt reversal. If so, cyanosis develops ( h eart failure and respiratory infections) and Eisenmenger \200\231s syndrome is present. Tes ts: ECG: RBBB with LAD and prolonged P \200\223R interval (primum defect) or RAD (secundum defect). CXR : Small aortic knuckle, pulmonary plethora, progressive atrial enlargement. Ec hocardiography is diagnostic. Cardiac catheterization shows step up in O 2 saturation in th e right atrium. Treatment: In children, surgical closure is recommended before age 10 yrs. In adults, closure is recommended if symptomatic, or if asymptomatic but having pulmonary t o systemic blood flow ratios of \211 1.5:1. Ventricular septal defect A hole connecting t he two ventricles. Causes: Congenital (prevalence 2:1,000 births); acquired (post-MI). 148 Symptoms: May present with severe heart failure in infancy or remain asymptomatic and be de tected incidentally in later life. Signs: These depend upon the size and site of the VSD : Smaller holes, which are hemodynamically less significant, give louder murmurs. Classically , a harsh pansystolic murmur is heard at the left sternal edge, accompanied by a systolic t hrill, left parasternal heave. Larger holes are associated with signs of pulmonary hyper tension. Complications: AR, infundibular stenosis, infective endocarditis, pulmonary hypert ension, Eisenmenger complex. Tests: ECG: Normal (small VSD), LAD + LVH (moderate VSD) or LV H + RVH (large VSD). CXR : Normal heart size mild pulmonary plethora (small VSD) or card iomegaly, large pulmonary arteries and marked pul- monary plethora (large VSD). Cardiac cat heter : Step up in O 2 saturation in right ventricle. Treatment: This is medical, at first, as many VSDs close spontaneously. Indications for surgical closure are failed medical ther apy, symptomatic VSD, shunt >3:1, SBE/IE. Give SBE/IE prophylaxis for untreated defects (p. 143). Coarctation of the aorta Congenital narrowing of the descending aorta; usually occur s just distal to the origin of the left subclavian artery. More common in boys. Association s: Bicuspid aortic valve, Turner \200\231s syndrome. Signs: Radiofemoral delay, weak femora l pulse, BP ^ , scapular bruit, sys- tolic murmur (best heard over the left scapula). Compl ications: Heart failure, infective endocarditis. Tests: CXR shows rib notching. Treatment: Surgery. Pulmonary stenosis may occur alone or with other lesions (p. 140). e n i c i d e m r a l u c s a v o d r a C i 149 Driving and the heart The rules on driving with heart conditions vary from state to state, and you should become familiar with the laws in your state before discussing driving with y our patients. In general, it is the responsibility of the driver, but you should guide your patients appropriately. Pulmonary medicine L. Dwight Wooster , M.D., F.C.C.P. e n i c i d e m y r a n o m u P Introduction 150 Outline of pulmonary diseases 151 History and physical exam 151 Clinical s tudies in pulmonary medicine 155 Pneumonia 160 Specific pneumonias 164 Complications of pne umonia 165 Cystic fibrosis 166 Bronchiectasis 168 Lung abscess 169 Fungal infection of the lung 169 Neoplasms of the lung 173 Asthma 176 Asthmatic bronchitis/Refractory asthma 177 Ch ronic obstructive pulmonary disease 179 Interstitial lung disease 183 Acute respiratory dis tress syndrome 185 Respiratory failure 186 Pulmonary embolism 188 Pneumothorax 189 Pleural effusion 190 Sarcoidosis 192 Hypersensitivity pneumonitis/Extrinsic allergic alveolitis (EA A) 194 Hypersensitivity pneumonitis with eosinophilia 194 Pulmonary hypertension 195 Indust rial dust diseases 196 Cor pulmonale 198 Obstructive sleep apnea/hypopnea syndrome 200 Lung transplantation 201 l 6 150 Introduction The respiratory system consists of the central nervous system, the periph- era l nervous system, the nose and sinuses, the mouth and upper airways, the lungs, chest wall, and diaphragm and the pulmonary circulation. The functions of the respiratory system inclu de filtering and humidification of air, oxygenating of blood and elimination of carbon diox ide, and the pro- tection of each respiratory system component. Pulmonary disorders or thos e disorders affecting the lungs cause dysfunction of these respiratory functions, resulting in a variety of symptoms, the most common of which is dyspnea, difficulty in breathing. Ot her forms of breathlessness are ortho- pnea (shortness of breath when lying down), dyspnea on exertion, parox- ysmal dyspnea and nocturnal paroxysmal dyspnea (sudden paroxysms of dys pnea), and platypnea, or shortness of breath when standing up. Cough, chest tightness or di scomfort, wheezing, and hemoptysis are other frequent symptoms suggestive of respiratory sy stem disorders. Outline of pulmonary diseases Airway diseases-predominately obstructive \212 \212 \212 \212 \212 \212 Lung parenchyma disease-predominately restrictive \212 Pulmonary fibrosis \212 Sarcoidosis \212 Lung abscess \212 Pneumonia \212 Malignancy: Primary or metastat ic Chest wall diseases-restrictive \212 \212 \212 e n i c i d e m y r a n o m u P Acute bronchitis and upper respiratory tract infections Emphysema Chronic bronchitis/bronch iectasis Asthma Chronic obstructive pulmonary disease Bronchiolitis l Skeletal: Pectus deformities, kyphoscoliosis, ankylosing spondylitis Extraskeletal: Obesity Neurological: Multiple Guillain-Barr syndrome, paralyzed diaphragm sclerosis, amyotrophic lateral sclerosis, Vascular disease-neither obstructive nor restrictive \212 \212 Pulmonary emboli (PE) Pulmonary hypertension History and physical exam Refer to pp. 36 \200\22339; in addition to the clinical points in the chapter of Clinical Skills, a few other points; Smoking history Ask the patient: \200 \234Have you ever smoked? How long? Number of cigarettes per day? Filtered or unfiltered? G eneric versus brand-named cigarettes? Menthol or nonmenthol? \200\235 Did the patient \200 \231s par- ents smoke? If one has stopped smoking, how long? Ask about other tobacco produc ts; question about snuff or chew, fre- quency of use, history of previous dental or mouth e xaminations. Ask about other inhalants, such as THC/cocaine. Ask about occupational history ; nature of the patient \200\231s work; exposure to fumes, chemicals dyes; e.g., if taking a history from a fire-fighter, is there a history of smoke inhalation? Is there a modificat ion of symptoms when away from work environment compared to symptoms at work? Ask about con struction projects at work or at home. Question the patient about exposure to variations in tempera- ture or humidity. Physical exam of the chest In addition to examination of the ch est discussed in Clinical Skills (p. 36): \212 \212 151 Listen to chest sound during quiet breathing and with deep breathing. With the stethoscope over the upper and anterior portion of the chest, have the patient perform a forced expirat ion \200\224listen for wheezes or rhonchus. Chest radiographs The routine chest radiograph (p. 691) is the first radio- graphic step in diagnosing pulmonary disease. Although chest computed tomography (ct) scans have greater l ung and mediastinum resolution and are commonly ordered, the routine chest x-ray (cxr) is l ower in cost and has less radiation exposure. The noncontrast ct has a radiation exposure o f 7.00 millisieverts, whereas a routine cxr has an exposure of 0.02; concerning cost, nationa l data from commercial insurance and Medicare show a cost range of $620-$386 for chest ct; the cost range for a cxr is $77-$51. Detailed analysis of chest radiographs are reviewed in the Radiology chapter. The replications of chest radiographs in Figure 6.1 show characteri stic changes of infiltrates or lung lesions in all five lobes. Other chest imaging Chest CT is modality of choice for evaluating lung or thoracic abnormalities detected on a routine cxr; excellent for lung parenchyma, mediastinum, and chest wall abnormalities. High-resolut ion CT (HRCT) produces cross-section images of 1 \200\2232 mm, in contrast to usual ct cros s-sections of 7 \200\22310 mm; excellent for delineation of lung parenchyma and small airwa ys, especially in interstitial lung disease. Helical CT produces quicker ct scans with sing le-breath imaging, con- trast images with collection of continuous data, and may develop th ree-dimensional (3-D) images. In 2010, the National Cancer Institute (NCI) reported on a lu ng cancer screening study that included participants who were between 55 and 74 years of ag e and who were smokers of at least 30 pack-years. The participants were randomized to low-d ose helical ct or to screening routine chest radiographs. The findings indicated that scree n- ing with low-dose helical ct was beneficial in reducing mortality from lung cancer. None theless, the nci recommended further lung cancer screening studies and advocated cost-effec tive review of low-dose helical ct for lung cancer screening. Magnetic resonance imaging (M RI) is less sensitive than chest ct in the evaluation of lung parenchyma; effective for stu dy of chest wall, breast, and mediastinum. Nuclear medicine techniques Ventilation and perf usion nuclear scans were previously used to diagnose PE; ct angiography is now the radio- graphic tool of choice to evaluate for PE. Ventilation/perfusion (V ) scans /Q are helpf ul in preoperative analysis of lung resection candidates, for regional blood flow and venti lation functional analysis of the proposed lung to be removed, and in calculating its contr ibution or noncontribution to overall lung function in comparison to the uninvolved lung. P ositron emission tomography (PET) scans utilize a radiolabeled glu- cose compound, fluoro-2 -deoxyglucose, which is absorbed by metabolically active cells. 18-F decays and is detected by the PET camera. PET is useful for evaluation of solitary pulmonary nodules and for the inclusion or exclusion of metastatic lung disease. False negatives occur with neoplastic pr ocesses of low metabolic activity, such as carcinoid tumors and bronchioloalveolar carcinom a. False-positive results are detected in inflammatory processes having a high metabolic ac tivity, such as pneumonias and inflammatory, granulomatous disorders. Pulmonary angiography