七院聯合病例討論會

1. 七院聯合病例討論會 三軍總醫院 R2 李宗翰 / Vs. 喩永生主任

2. History • This 12-year-old boy was suffered from fever, cough, and abdominal pain for 3 days. • He is a victim of acute rheumatic fever, including arthritisand carditis and received regular Penicillin-G treatment monthly for 1 year.

3. History • He got an insert bite in his right lower leg with a small infected wound; the wound was treated at home and improved.

4. History • However, about 1 week later, the symptoms of low-grade fever, cough and abdominal pain with fullness sensation were noted. • 3 days later, progressive dyspnea during exertion was noted and then he was sent to our hospital for help.

5. Physical examination • The physical examination showed respiratory distress, bilateral coarse breathing sounds, grade Ⅰ-Ⅱ/Ⅳ systolic murmurs over LSB, distended abdomen, dilated jugular and superficial veins, hepatosplenomegaly and pitting edema of bilateral lower legs. • A small healed insert bite wound over right lower leg.

6. Image • The CXR showed minimal right side pleural effusion with normal heart size.

7. Laboratory data • WBC:14100 • Neutrophil:67% (40-74) • Lymphocyte:20% (19-48) • Monocyte:11% (3.4-9) • CRP:8.4 mg/dl (0-0.5) • Albumin:3.0 g/dl (3.4-4.8) • BUN:8 mg/dl (7-20) Creatinine:0.5 (0.5-1) • AST:126 U/L (-37) ALT:107 U/L (-41) • Total bilirubin: 1.2 mg/dl • Na:135 mmol/L (136-145) K:3.4 mmol/L (3.5-5.1) Cl:98 mmol/L

8. Admission course • Echocardiography: • Tricuspid valve regurgitation • Mitral valve regurgitation • Pulmonary valve regurgitation

9. Admission course • Abdominal sonography • Hepatosplenomegaly • Patent hepatic artery and portal vein • Mild to moderate ascites

10. Subjective findings • Fever • Cough • Abdominal pain with fullness • Exertional dyspnea • Insert bite

11. Objective findings • Coarse breathing sounds • Systolic heart murmurs over LLSB • Pitting edema over bilateral lower legs. • Hepatosplenomegaly • Wound of insert bite

12. Objective findings • Liver function impairment • Hypoalbuminemia • Hyponatremia • Elevated CRP level • Elevated WBC count • Pleural effusion • MR, TR, PR.

13. Problems • R/O Heart failure • Fever • Insert bite • Hepatosplenomegaly • Impaired liver functions • Pleural effusion • Ascites

14. Question • Patient traveling history • EKG finding (arrhythmia, ST or T wave change) • Echocardiography findings (compared with previous) • Arterial blood gas (hypoxia, hypercapnea) • Bacterial culture (blood,pleural effusion) • Virus isolation and identification • Pleural effusion study (transudate or exudate, AFB stain) • Heart rate, blood pressure (arrhythmia, hypertension) • Anemia or thrombocytopenia (hematologic abnormalities) • Urine routine (Proteinuria ,hematuria) • Indirect fluorescent antibody assay serology

15. Causes of splenomegaly • Infection • Bacterial: Typhoid fever, endocarditis, septicemia, abscess • Viral:E-B virus, CMV, and others • Protozoal: Malaria, toxoplasmosis • Hematologic processes • Hemolytic anemia: Congenital, acquired • Extramedullary hematopoiesis: thalassemia, osteopetrosis, myelofibrosis • Neoplasms • Malignant: Leukemia, lymphoma, histiocytoses, metastatic tumors • Benign: Hemagioma, hamartoma • Metabolic diseases • Lipidosis: Niemann-Pick, Gaucher disease • Mucopolysaccharidosis infiltration: Histiocytosis • Congestion • Cirrhosis • Cysts • Miscellaneous

16. Causes of ascites • Hepatic (cirrhosis, fibrosis, obstruction) • Renal (nephrotic syndrome, obstrutive, PD) • Cardiac(heart failure, constrictive pericarditis) • Infectious (abscess,TB, Chlamydia, schistosomia) • Gastraointestinal (infarcted bowel perforation) • Neoplastic (lymphoma, neuroblastoma) • Pancreatic (pancreatitis, ruptured pancreatic duct) • GYN (ovarian tumor, torsion, rupture) • Miscellaneous (SLE, VP shunt, chylous, hypothyroidism)

17. Heart failure • Symptoms • Fatigue, effort intolerance, anorexia, abdominalpain, dyspnea,cough • Sign • Engorged jugular vein, hepatomegaly, basilar rales, edema, cardiomegaly, gallop rhythm, holosystolic murmur of mitral or tricuspid valve regurgitation may be heard when ventricular dilatation is advanced.

18. Heart failure • Sign • Pitting edema occurred usually in the legs. • Pitting edema of the arms and face occurs rarely and then only in the late course of HF. • Ascites occurred most frequently in patients with tricuspid valve disease or constrictive pericarditis

19. Heart failure • Chest X ray • Exaggeration of the pulmonary arterial vessels to the periphery of the lung fields. (Patients with cardiomyopathymay have a relatively normal pulmonary vascular bed early in the course of disease) • Cardiomegalyis invariable noted. • Pleural effusion was more frequentin the right side than in the left side.

20. Heart failure • EKG • Left or right ventricular ischemic changes may correlate well with clinical and other non invasive parameters of ventricular function. • Low voltage QRS morphologic characteristic with ST-T wave abnormaluties may also suggest myocardial inflammatory disease but can be seen with pericarditis • Rhythm disease is also a potential cause of heart failure.

21. Diagnosis of congestive heart failure(Framingham criteria) • Major criteria (at least 1) • Paroxysmal nocturnal dyspnea • Neck vein distention • Rales • Cardiomegaly • Acute pulmonary edema • S3 gallop • Increased venous pressure (>16 cm H2O) • Positive hepatojugular reflux • Minor criteria (at least 2) • Extremity edema • Night cough • Dyspnea on exertion • Hepatomegaly • Pleural effusion • Vital capacity reduced by 1/3 from normal • Tachycardia (>120 bpm)

22. Acute versus Chronic • Acute: Sudden development of myocardial infection or rupture of a cardiac valve, no cardiomegaly, no peripheral edema. • Chronic: Dilated cardiomyopathy, multiple valvular heart disease, cardiomegaly, peripheral edema.

23. Right sided versus left sided • Left: Dyspnea or orthorpnea as the result of pulmonary congestion. • Right:Edema, congestive hepatomegaly, systemic venous distention.

24. Carditis in rheumatic fever • Rheumatic carditis is characterized by pancarditis, with active inflammation of myocardium, pericardium, and endocardium. • Endocarditis, which is manifest by one or more cardiac murmurs, is a universal finding in rheumatic carditis. • Most case consist of mitral valvular disease or combined aortic and mitral valvular disease. • Isolated aortic or right side valvular involvement is uncommon.

25. Rheumatic heart disease • Mitral valve: MR will result in increased atrium pressure and pulmoary congestion and symptoms of left side heart failure. Spontaneous improvement usually occurs with time even in sever cases. In chronic MR, pulmonary arterial pressure elevated and right ventricle and atrium become enlarged and right side heart failure develops. • MS will result in increased pressure and enlargement and hypertrophy of the left atrium, pulmonary venous hypertension, increased vascular resistance and pulmonary hypertension. • Aortic valve: AR will lead to volume overload with dilatation of LV. • Right-sided heart manifestations are rare. • Tricuspid valve: Primary tricuspid involvement is rare after rheumatic fever. TR is more common secondary to right ventricular dilatation resulting from unrepaired left side lesion. • Pulmonary valve: PR usually occurs on a functional basis secondary to pulmonary hypertension and is a late finding with severe mitral valve stenosis.

26. Pulmonary regurgitation • Isolated congenital pulmonary regurgitation is rare. • Pulmoary valvular insufficiency most often accompanies other cardiovascular disease or may be secondary to severe pulmonary hypertension.

27. Tricuspid regurgitation • Tricuspid regurgitation usually functional. • Secondary to marked RV dilatation of any cause and often associated with pulmonary hypertension. • When volume overload or intrinsic myocardial disease -> dilated right ventricle. • Tricuspid regurgitation often accompanies with right ventricular dysfunction.

28. Pulmonary regurgitation & Tricuspid regurgitation • Pulmonary hypertension • Cardiovascular disease -> RV dysfunction

29. Causes of pulmonary hypertension Conditions directly affecting pulmonary arteriesPrimary pulmonary hypertensionToxin-induced (ie, anorexic agents)VasculitisGranulomatoses, collagen-vascular disorders, arteritisHepatic cirrhosis/portal diseaseCongenital heart disease (Eisenmenger syndrome [ASD, VSD, PDA])InfectionHuman immunodeficiency virus

30. Causes of pulmonary hypertension Conditions affecting pulmonary parenchymaChronic obstructive lung diseaseInfiltrative/granulomatous diseasesSarcoidosis, pneumoconiosis, radiation, fibrosis, neoplasm, pneumonia, collagen-vascular diseasesCystic fibrosisUpper airway obstructionHigh-altitude diseaseArteriovenous fistulas within the lungRestrictive lung diseases

31. Causes of pulmonary hypertension Conditions affecting the thoracic cage and neuromuscular systemObesity-hypoventilation/sleep apneaPharyngeal-tracheal obstructionKyphoscoliosisPleural fibrosisNeuromuscular disordersMyasthenia gravis, poliomyelitis, central respiratory disorders

32. Causes of pulmonary hypertension • Conditions causing left atrial/or pulmonary venous hypertensionElevated left ventricular diastolic pressureSystolic failure, diastolic dysfunction, constrictive pericarditisAortic valve diseaseMitral valve diseaseCor triatriatumLeft atrial massesMyxoma or neoplasm, thrombusFibrosing mediastinitisCongenital pulmonary vein stenosisAnomalous pulmonary venous connectionPulmonary venoocclusive disease

33. Causes of pulmonary hypertension • Nonvasculitis conditions that result in pulmonary artery obstructionAcute and chronic thromboembolismHemoglobinopathies (eg, sickle cell disease)Primary or metastatic malignanciesPeripheral pulmonic stenosis • Congenital pulmonary hypoplasia

34. Vasculitis syndrome • Goodpasture disease • Pulmonary hemorrhage and glomerulonephritis. • Symptoms & signs • Hemoptysis, hematuria, proteinuria and hypertension.

35. Vasculitis syndrome • Wegener Granulomatosis • Fever, myalgia, cough, nasal discharge, hematuria proteinuria. • Hemoptysis and dyspnea from lung lesion. • Uveitis, conjuctivitis. • Peripheral granuloma. • Palpalbe purpuric nodules and ulcers

36. Vasculitis syndrome • Polyarteritis Nodosa • Associated with infection of group A streptococcus, hepatitis B, CMV, parvovirus , TB, E-B virus. • Symptoms & signs. • Fever • Abdominal pain (mesenteric arterial inflammation) • Hypertension, hematuria, proteinuria (renal vessels) • Purpura, edema, painful nodule (skin involvement) • Heart failure (myocarditis, myocardial ischemia) • Elevated liver function (hepatitis B infection more common in adults) • Elevated ESR, anemia and leukocytosis

37. An algorithm for the workup of a patient with unexplained pulmonaryhypertension

38. Pulmonary embolism • Precipitating factor • CVP insertion, immobility, heart disease, oral contraceptives, ventriculoatrial shunt, trauma, infection, dehydration, collagen vascular disease, shock , obesity, hematologic disorder. • Symptoms • Chest pain, dyspnea, cough, fever, hemoptysis. • Signs • Rales, heart murmurs, tachycardia, fever, diaphoresis, phlebitis, wheezing.

39. Pulmonary embolism • Lab • ABG: Respiratory alkalosis, arterial hypoxemia • Elevated WBC count, D-dimer, fibrinogen, protein C, protein S. • Chest:Often normal in patient with PE. • EKG: Nonspecific ST segment change and signs of cor pulmonale • Ventilation-perfusion image: Regional blood flow and ventilation defects . But normal V-P scan cant exclude a PE and are interpreted as high probability, intermediate probability, low probability, very low probability and normal. Lung disease can limit the utility of this study. • Pulmonary angiography is the gold standard diagnostic test for pulmonary embolism.

40. Cause of heart failure in child • Rheumatic fever • Acute hypertension • Thyrotoxicosis • Hemochromatosis-hemosiderosis • Cancer therapy (radiation, doxorubincin) • Sickle cell anemia • Endocarditis • Cardiomyopathy (Viral myocarditis, nonviral) • Myocarditis, hypertrophic, dilated.) • Cor pulmoale (cystic fibrosis)

41. Pulmonary regurgitation & Tricuspid regurgitation • Pulmonary hypertension • Infection • Pulmonary embolism • Cardiovascular disease -> RV dysfunction • Rheumatic fever • Endocarditis • Cardiomyopathy (carditis, dilated, hypertrophic )

42. Acute rheumatic fever • Major: (least 2 major or 1+2 minor) • Carditis, polyarthritis, erythema marginatum, subcutaneous nodules • Minor: • Fever, arthralgia, elevated ESR or CRP, prolonged PR interval • Supported evidence of GAS infection: • Positive throat culture or streptococcal antigen test • Elevated or increased streptococcal antibody titer

43. Infectious endocarditis • Etiology: Viridans-type streptococci and staphylococcus aureus are the leading causative agents responsible for endocarditis in pediatric patients. • Infective endocarditis is often a complication of congenital or rheumatic heart disease • Prolonged fever without other manifestation. • Low grade fever, fatigue, myalgia, arthralgia, headache, and at times, chills, nausea, and vomiting. • Splenomegaly and petechiae are relative common. • Osler nodes (tender, pea-sized intradermal nodules in the pads of the fingers and toes), Janeway lesion (painless small erythematous or hemorrhagic lesion on the palms and soles), splinter hemorrhages (linear lesions beneath the nails) • Embolic strokes, cerebral abscess, mycotic aneurysm, and hemorrhage in staphylococcus disease.

44. Infectious endocarditis • History • Congenital or rheumatic heart disease • Preceding dental, urinary tract or intestinal procedure • Intravenous drugs abuse • Central venous catheter • Prosthetic heart valve • Symptoms • Fever, chills, chest and abdominal pain, arthralgia, myalgia, dyspnea, malaise, night sweats, weight loss, CNS manifestations

45. Infectious endocarditis • Signs • Elevated temperature, tachycardia, embolic phenomena (Roth spots, petechia, splinter nail bed hemorrahge, Osler nodes, CNS or ocular lesions), Janeway lesions, New or changing murmur, splenomegaly, arthritis, heart failure, arrythmias, Metastatic infection (arthritis, meningitis, mycotic arterial aneurysm, pericarditis, abscesses, septic pulmonary emboli), clubbing • Labs • Blood culture (+), ESR ↑, CRP↑, anemia, leukocytosis, immune complexes, hypergammaglobulinemia, hypocomplementemia, rheumatoid factor, hematuria, renal failure • Chest X ray: bilateral infiltration, nodules, pleural effusions • Echocardiography: valve vegetations, prosthetic valve dysfunction or leak, myocardial abscess, new-onset valve insufficiency.

46. Infectious endocarditis • Duke criteria • Major: 1.Blood culture (+) 2.Evidence of endocarditis on echocardiography (intracardiac mass on a valve or other site, regurgitation flow near a prosthesis, abscess, partial dehiscence of prosthetic valves or new valve regurgitation flow) • Minor:fever, embolic-vascular signs, immune complex phenomena (GN, arthritis, rheumatoid factor, Osler nodes, Roth spots), • 2 major + 1 minor • 1 major + 3 minors • 5 minors

47. Infectious endocarditis • Etiology • Common: Native valve or other cardiac lesions • Viridans group streptococcus (dental procedure) • Staphylococcus aureus (drugs abuse) • Group D streptococcus (GU or GI tract procedures) • Uncommon: Native valve or other cardiac lesions • Streptococcus pneumoniae • Haemophilus influenzae • Coagulase-negative staphylococci • Coxiella burnetii (tick borne) • Neisseria gonorrhoeae (in human, sexual , intimate contact) • Brucella (zoonotic disease, contact with infected animals) • Chlamydia psittacli, Chlamydia trachomatis, Chlamydia pneumoniae (human, sexual,respira) • Legionella (fresh water or aerosols containing bacteria) • Bartonella (cat scratch) • HACEK group (H: respiratory;A:trauma, aspiration to lung, ) • Streptobacillus moniliformis • Pasteurella multocida (chesse, milk ) • Campylobacter fetus • Culture negative (6% of cases)

48. Myocarditis • Symptoms: • Fever, severe heart failure, respiratory distress. • Incubation time:1-7 days of the onset of symptoms. • Signs: • Cyanosis, distant heart sounds, weak pulses, tachycardia, Gallop rhythm, acidosis, and shock. • Evidence of hepatitis, aseptic meningitis, associated rash may be present.

49. Myocarditis • Lab: • Elevated CK, LDH • EKG: Sinus tachycardia, reduced QRS voltage, ST-segment and T-wave abnormalities, arrhythmia. • Echo: poor ventricular function, pericardial effusion, mitral valve regurgitation, absence of congenital heart lesion or coronary artery

50. Etiology of myocardial disease • Familial-Hereditary (myopathy, cardiomyopathy) • Infection (Virus, Richettsiae, Bacteria,parasite,fungus) • Metabolic, nutritional, endocarine • Connective tissue-Granulomatous disease-infiltrative (SLE, vasculitis, Scleroderma, sarcoidosis, dermatomyositis, leukemia) • Drugs-Toxins (chemo, alcohol,irradiation.) • Coronary arteries (Kawasaki disease, meidal necrosis, anomalous left coronary artery) • Other (anemia, ischemia)