They tell us about clinical symptoms of right failure, with edema and ascites. Therefore, we rule out left heart failure: EAo, dilated with DSVI and a MHO. Of the two we are left with, in constrictive pericarditis we have increased filling pressures in right chambers, which cause retrograde pressure increase with hepatic congestion, ascites, edema, etc. On the contrary, in pulmonary hypertension, the tricuspid valve is responsible (a priori) for "containing" this overpressure.

In a patient with clinical signs of heart failure who presents with ascites disproportionately elevated in relation to peripheral edema, the most likely etiology would be:

Dilated cardiomyopathy with significant left ventricular dysfunction.

[Contribution of echocardiography to the diagnosis of patients with chronic heart failure].

The echocardiographic examination is generally performed in patients with heart failure and it often gives a significant contribution to the differential diagnosis. Firstly, the evaluation of left ventricular pump function by measuring the ejection fraction (EF) can distinguish patients with heart failure into two different groups, with depressed or preserved EF. The most frequent causes of heart failure and depressed EF are coronary artery disease, idiopathic dilated cardiomyopathy (DCM) and hypertensive heart disease. Although the echocardiographic features of coronary artery disease versus idiopathic DCM may be similar, the demonstration of inducible ischemia at dobutamine echocardiographic test suggests the presence of significant coronary artery disease and may be useful in the selection of cases for coronary arteriography. The association of left ventricular hypertrophy, hypokinesis and, sometimes, significant dilation is compatible with hypertensive heart disease or end-stage hypertrophic cardiomyopathy. No useful echocardiographic findings can identify the patients with genetic DCM or affected by myocarditis from other cases with idiopathic DCM. Some advanced cases of right ventricular dysplasia/cardiomyopathy may show a biventricular involvement and mimic DCM; these patients are usually characterized at echo by predominant right ventricular dilation and multiple a-dyskinetic bulges in the absence of pulmonary hypertension. Very difficult to manage are the patients with significant left ventricular dysfunction and severe valvular heart disease (such as aortic stenosis or mitral regurgitation). According to the literature, the left ventricular systolic function is relatively preserved (EF > 40%) in 30-40% of patients with heart failure. In these cases a diastolic dysfunction may be hypothesized. Echo-Doppler evaluation can be helpful in the recognition of signs of increased left ventricular stiffness ("restrictive filling pattern") and of increased filling pressures. In the differential diagnosis one must first consider the most frequent heart disorders that may present with this clinical syndrome, coronary artery disease and hypertensive heart disease. Furthermore, other less common diseases characterized by heart failure due to predominant diastolic dysfunction are the following: hypertrophic and restrictive cardiomyopathies, infiltrative heart diseases, such as amyloidosis, and constrictive pericarditis. Restrictive cardiomyopathy is characterized by heart failure and preserved left ventricular EF in the absence of significant ventricular dilation and hypertrophy; typical, although not pathognomonic, echocardiographic features are atrial enlargement ad restrictive filling pattern. In distinguishing constrictive pericarditis from restrictive cardiomyopathy useful Doppler signs are the wide respiratory variability in flow velocities at mitral and tricuspid levels, due to increased ventricular interdependence caused by the presence of an abnormally rigid pericardium.

Restrictive cardiomyopathy

It should not be confused with constrictive pericarditis, a disease which presents similarly but is very different in treatment and prognosis. Signs and symptoms Untreated hearts with RCM often develop the following characteristics: M or W configuration in an invasive hemodynamic pressure tracing of the RA Square root sign of part of the invasive hemodynamic pressure tracing Of The LV Biatrial enlargement Thickened LV walls (with normal chamber size) Thickened RV free wall (with normal chamber size) Elevated right atrial pressure (>12mmHg), Moderate pulmonary hypertension, Normal systolic function, Poor diastolic function, typically Grade III - IV Diastolic heart failure. Those afflicted with RCM will experience decreased exercise tolerance, fatigue, jugular venous distention, peripheral edema, and ascites. Arrhythmias and conduction blocks are common.

Primary diastolic heart failure.

Diastolic heart failure is defined clinically when signs and symptoms of heart failure are present in the presence of preserved left ventricular systolic function (ejection fraction >45%). The incidence and prevalence of primary diastolic heart failure increases with age and it may be as high as 50% in the elderly. Age, female gender, hypertension, coronary artery disease, diabetes, and increased body mass index are risk factors for diastolic heart failure. Hemodynamic consequences such as increased pulmonary venous pressure, post-capillary pulmonary hypertension, and secondary right heart failure as well as decreased cardiac output are similar to those of systolic left ventricular failure, although the nature of primary left ventricular dysfunction is different. Diagnosis of primary diastolic heart failure depends on the presence of preserved left ventricular ejection fraction. Assessment of diastolic dysfunction is preferable but not mandatory. It is to be noted that increased levels of B-type natriuretic peptide does not distinguish between diastolic and systolic heart failure. Echocardiographic studies are recommended to exclude hypertrophic cardiomyopathy, infiltrative heart disease, primary valvular heart disease, and constrictive pericarditis. Myocardial stress imaging is frequently required to exclude ischemic heart disease. The prognosis of diastolic heart failure is variable; it is related to age, severity of heart failure, and associated comorbid diseases such as coronary artery disease. The prognosis of severe diastolic heart failure is similar to that of systolic heart failure. However, cautious use of diuretics and/or nitrates may cause hypotension and low output state. Heart rate control is essential to improving ventricular filling. Pharmacologic agents such as angiotensin receptor blockers, angiotensin-converting enzyme inhibitors, and calcium channel blockers are used in selected patients to decrease left ventricular hypertrophy. To decrease myocardial fibrosis, aldosterone antagonists have a potential therapeutic role. However, prospective controlled studies will be required to establish their efficacy in primary diastolic heart failure.

Right ventricular failure in left heart disease: from pathophysiology to clinical manifestations and prognosis.

Right heart failure (RHF) is a clinical syndrome in which symptoms and signs are caused by dysfunction and/or overload of the right heart structures, predominantly the right ventricle (RV), resulting in systemic venous hypertension, peripheral oedema and finally, the impaired ability of the right heart to provide tissue perfusion. Pathogenesis of RHF includes the incompetence of the right heart to maintain systemic venous pressure sufficiently low to guarantee an optimal venous return and to preserve renal function. Virtually, all myocardial diseases involving the left heart may be responsible for RHF. This may result from coronary artery disease, hypertension, valvular heart disease, cardiomyopathies and myocarditis. The most prominent clinical signs of RHF comprise swelling of the neck veins with an elevation of jugular venous pressure and ankle oedema. As the situation worsens, fluid accumulation becomes generalised with extensive oedema of the legs, congestive hepatomegaly and eventually ascites. Diagnosis of RHF requires the presence of signs of elevated right atrial and venous pressures, including dilation of neck veins, with at least one of the following criteria: (1) compromised RV function; (2) pulmonary hypertension; (3) peripheral oedema and congestive hepatomegaly. Early recognition of RHF and identifying the underlying aetiology as well as triggering factors are crucial to treating patients and possibly reversing the clinical manifestations effectively and improving prognosis.

Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management.

This is the case of a patient who presented with severe right-sided heart failure due to diastolic dysfunction that caused a dilemma of differential diagnosis between restrictive cardiomyopathy and constrictive pericarditis. Restrictive cardiomyopathy was diagnosed based on noninvasive and invasive hemodynamic testing. However, the patient did not respond to therapy and succumbed to worsening heart failure and multiple comorbidities. Clinical features of right heart failure with edema, ascites, jugular venous distention, and tender hepatomegaly are commonly seen in clinical practice. When systolic function is determined to be normal, diastolic causes of heart failure must be ruled out. These include myocardial disorders with a broad range of pathologies leading to restrictive physiology, of which amyloidosis is a prototype. Pericardial disorders leading to diastolic heart failure are usually in the form of constrictive physiology, when pericardial tamponade is ruled out. Differentiation between restrictive and constrictive pathologies is often difficult and requires careful attention to hemodynamic and Doppler echocardiographic features. We report a case of severe right heart failure illustrating some of the complexities in decision-making and the importance of meticulous hemodynamic and ancillary testing in the diagnosis and treatment of this often fatal condition.

Heart Failure With Preserved Ejection Fraction (HFpEF) -- Etiology -- Etiologies of Secondary Heart Failure With Preserved Ejection Fraction

Secondary HFpEF occurs in the presence of a clinical condition that directly causes the heart to fail. These conditions are sometimes referred to as "HFpEF mimics." [16] These mimics include but are not limited to restrictive and hypertrophic cardiomyopathies of any etiology, constrictive pericarditis, and valvular heart disease. [16] [7] In the United States, left-sided valvular disease is the most commonly encountered HFpEF mimic, and aortic stenosis should be suspected in all patients with signs, symptoms, or suspicion of HFpEF. [21] [16]

Heart failure

"Cardiac asthma" or wheezing may occur.Compromise of left ventricular forward function may result in symptoms of poor systemic circulation such as dizziness, confusion, and cool extremities at rest. Right-sided failure Right-sided heart failure is often caused by pulmonary heart disease (cor pulmonale), which is typically caused by difficulties of the pulmonary circulation, such as pulmonary hypertension or pulmonic stenosis. Physical examination may reveal pitting peripheral edema, ascites, liver enlargement, and spleen enlargement. Jugular venous pressure is frequently assessed as a marker of fluid status, which can be accentuated by eliciting hepatojugular reflux. If the right ventricular pressure is increased, a parasternal heave may be present, signifying the compensatory increase in contraction strength.

Exercise intolerance

Hazards Certain conditions exist where exercise may be contraindicated or should be performed under the direction of an experienced and licensed medical professional acting within his or her scope of practice. These conditions include: Decompensated heart failure Recent myocardial infarction Hypertrophic cardiomyopathy or cardiomyopathy from recent myocarditis Active or suspected myocarditis or pericarditis Low left-ventricular ejection fraction (LVEF) Severe aortic stenosis Unstable ischemia Unstable arrythmia Irregular or resting pulse greater than 100 bpm Resting systolic blood pressure >200 mm Hg or resting diastolic blood pressure >110 mm Hg Severe pulmonary hypertension Chronic fatigue syndrome Suspected or known dissecting aortic aneurysm Recent systemic or pulmonary embolus Pneumothorax and haemoptysis Thrombophlebitis

Heart Failure With Preserved Ejection Fraction (HFpEF) -- Differential Diagnosis

The differential diagnosis of exertional dyspnea with or without generalized fatigue is incredibly broad and includes cardiac and noncardiac etiologies. Additionally, because the clinical syndrome of heart failure is the common endpoint for many disease processes, the overarching differential diagnosis for heart failure is similarly broad. However, in patients with exertional dyspnea and an underlying risk factor for HFpEF or comorbidity strongly associated with the development of HFpEF, the following diagnoses should be considered: [7] [16] Coronary artery disease Hypertension Valvular disease, particularly left-sided valvular disease Infiltrative cardiac diseases such as amyloidosis, hemochromatosis, and sarcoidosis Hypertrophic cardiomyopathy Constrictive pericarditis.

List of causes of shortness of breath

Obstruction of the airway Cancer of the larynx or pharynx Empty nose syndrome Pulmonary aspiration Epiglottitis Laryngeal edema Vocal cord dysfunction Immobilization of the diaphragm Lesion of the phrenic nerve Polycystic liver disease Tumor in the diaphragm Restriction of the chest volume Ankylosing spondylitis Broken ribs Kyphosis of the spine Obesity Costochondritis Pectus excavatum Scoliosis Disorders of the cardiovascular system Aortic dissection Cardiomyopathy Congenital heart disease CREST syndrome Heart failure Ischaemic heart disease Malignant hypertension Pericardium disorders, including: Cardiac tamponade Constrictive pericarditis Pericardial effusion Pulmonary edema Pulmonary embolism Pulmonary hypertension Valvular heart disease Disorders of the blood and metabolism Anemia Hypothyroidism Adrenal insufficiency Metabolic acidosis Sepsis Leukemia Holocarboxylase synthetase deficiency Disorders affecting breathing nerves and muscles

Hypertrophic cardiomyopathy

Clinically, cats with hypertrophic cardiomyopathy commonly have a systolic anterior motion (SAM) of the mitral valve (see graphic). Cats with severe HCM often develop left heart failure (pulmonary edema; pleural effusion) because of severe diastolic dysfunction of the left ventricle. They may also develop a left atrial thrombus that embolizes, most commonly, to the terminal aorta creating acute pain and rear limb paralysis (see below). Sudden death can also occur but appears to be uncommon. Ultrasound of the heart (echocardiography) is necessary to diagnose HCM in cats. Measurement of circulating cardiac biomarkers, like N‐terminal‐proBNP (NT‐proBNP) and troponin I (TnI) may be used in cats to strengthen the suspicion of cardiac disease. There is a Point-of-care test for feline NT-proBNP available which can be used at the veterinary clinic when echocardiography is not possible to perform.

InternalMed_Harrison

The least common of the physiologic triad of cardiomyopathies is restrictive cardiomyopathy, which is dominated by abnormal diastolic function, often with mildly decreased contractility and ejection fraction (usually >30–50%). Both atria are enlarged, sometimes massively. Modest left ventricular dilation can be present, usually with an end-diastolic dimension <6 cm. End-diastolic pressures are elevated in both ventricles, with preservation of cardiac output until late in the disease. Subtle exercise intolerance is usually the first symptom but is often not recognized until after clinical presentation with congestive symptoms. The restrictive diseases often present with relatively more right-sided symptoms, such as edema, abdominal discomfort, and ascites, although filling pressures are elevated in both ventricles. The cardiac impulse is less displaced than in dilated cardiomyopathy and less dynamic than in hypertrophic cardiomyopathy. A fourth heart sound is more common than a third

InternalMed_Harrison

Diagnosis Due to the unstable condition of these patients, supportive therapy must be initiated simultaneously with diagnostic evaluation (Fig. 326-2). A focused history and physical examination should be performed, blood specimens sent to the laboratory, and an electrocardiogram (ECG) and chest x-ray obtained. Etiologies of Cardiogenic Shock or Pulmonary Edema Acute myocardial infarction/ischemia LV failure Ventricular septal rupture Papillary muscle/chordal rupture–severe MR Ventricular free wall rupture with subacute tamponade Other conditions complicating large MIs Post-cardiac arrest Post-cardiotomy Refractory sustained tachyarrhythmias Acute fulminant myocarditis End-stage cardiomyopathy LV apical ballooning Takotsubo’s cardiomyopathy Hypertrophic cardiomyopathy with severe outflow obstruction Aortic dissection with aortic insufficiency or tamponade Severe valvular heart disease Other Etiologies of Cardiogenic Shockb RV failure due to:

Constrictive Pericarditis -- History and Physical

On physical examination, the jugular venous pressure (JVP) is usually elevated, however, may be normal in early constrictive pericarditis. JVP does not decrease with inspiration, and this is known as Kussmaul's sign. Kussmaul's sign is also present in tricuspid valve disease, and right-sided heart failure.Pulsus paradoxus (more than 10 mm Hg drop in systolic blood pressure during inspiration) can be seen however this is more common in patients with cardiac tamponade. An accentuated heart sound heard earlier than third heart sound called pericardial knock can be heard in almost half of the patients. Abdominal examination may reveal ascites or hepatomegaly. Other signs of chronic illness like muscle wasting may be present based upon the etiology. Peripheral edema may be present as well.

Echocardiogram -- Clinical Significance

Transthoracic Echocardiography (TTE): When a patient presents clinically with dyspnea or chest pain, and there is a high pre-test probability that the shortness of breath is of cardiac origin, TTE is recommended. It is useful in identifying the cause, location, and severity of myocardial ischemia, atrial sizes, ejections fraction (EF), left ventricular hypertrophy, and presence of valvular disease. [22] An EF is a common piece of information that is available from a TTE, especially in those with systolic congestive heart failure (CHF), because it dictates the course of treatment. An important assessment of cardiac structure by TTE is the size and thickness of the cardiac structures. Left and right atrial size will help aid in the diagnosis of diastolic dysfunction, and right and left ventricular hypertrophy will aid in the diagnosis of heart failure. This is especially important when the diagnosis of hypertrophic cardiomyopathy (HCM) is suspected. Asymmetric hypertrophy of left and right ventricles is the bases of the diagnosis for HCM. Patients who are found to have preserved ejection fraction (>50%) CHF are treated mainly with diuretics (furosemide, bumetanide) for symptom management. [23] On the contrary, those who are found to have reduced EF (<50%) CHF may be treated with diuretics, with the addition of beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and aldosterone antagonist which have shown to have a mortality benefit. This benefit is not seen in those with preserved EF heart failure. The evaluation of the mitral, tricuspid, aortic, and pulmonary valves is another important indication of TTE. Patients who have a new-onset heart murmur, clinical suspicion for infective endocarditis, dyspnea, and near-syncope may have structural valve pathology including tricuspid, mitral, aortic, and pulmonary stenosis and regurgitation. TEE is more sensitive for the diagnosis of valvular pathology, although TTE is generally performed first, as it is less invasive. [22] Another aspect of TTE is assessing heart chamber pressures, and the most useful of these is right pulmonary artery pressure, which aids in the diagnosis of pulmonary artery hypertension. In the acute setting, bedside TTE can assess pericardial effusions, and aid in the diagnosis of cardiac tamponade, this may require immediate interventions, including pericardiocentesis.

Valvular heart disease

On auscultation of a patient with mitral stenosis, typically the most prominent sign is a loud S1. Another finding is an opening snap followed by a low-pitched diastolic rumble with presystolic accentuation. The opening snap follows closer to the S2 heart tone with worsening stenosis. The murmur is heard best with the bell of the stethoscope lying on the left side and its duration increases with worsening disease. Advanced disease may present with signs of right-sided heart failure such as parasternal heave, jugular venous distension, hepatomegaly, ascites and/or pulmonary hypertension (presenting with a loud P2). Signs increase with exercise and pregnancy. Mitral regurgitation Patients with mitral regurgitation may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, or pulmonary edema.

MYL4

Clinical significance MYL4 expression in ventricular myocardium has shown to abnormally persist in neonates up through adulthood in patients with the congenital heart disease, tetralogy of Fallot. Altered ALC-1 expression is also altered in other congenital heart diseases, Double outlet right ventricle and infundibular pulmonary stenosis. Moreover, in patients with aortic stenosis or aortic insufficiency, ALC-1 expression in left ventricles was elevated, and following valve replacement decreased to lower levels; ALC-1 expression also correlated with left ventricular systolic pressure. Additionally, in patients with ischemic cardiomyopathy, dilated cardiomyopathy and hypertrophic cardiomyopathy, ALC-1 protein expression is shown to be reactivated, and ALC-1 expression correlates with calcium sensitivity of myofilament proteins in skinned fiber preparations, as well as ventricular dP/dtmax and ejection fraction. Interactions ALC-1 interacts with: ACTC1 MYH7 References

[Evolution of dilated cardiomyopathy from hypertrophic obstructive cardiomyopathy: a case report].

A 20-year-old woman whose echocardiograms showed a rare evolution from hypertrophic to dilated cardiomyopathy during a nine year observation period is described. This patient was initially diagnosed as having hypertrophic obstructive cardiomyopathy (HOCM) at the age of 12 years. Her echocardiogram showed marked thickening of the interventricular septum (IVS) and left ventricular posterior wall (LVPW), asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM). Chest radiography revealed a prominent left ventricular border and a cardiothoracic ratio of 0.52. At 18 years of age she experienced onset of palpitation during the 16th week of pregnancy. Her echocardiogram recorded in June 1980 revealed a thickened IVS and LVPW with resolution of the SAM and of the narrow cavity. At 20 years of age she became pregnant again and was admitted to our hospital for the third time at 24 weeks gestation. On admission her blood pressure was 122/60 and her pulse was 56, and moist rales were audible over both lung fields. Peripheral edema was noted. Chest radiography revealed moderate cardiomegaly, a cardiothoracic ratio of 0.66, and congestion of the pulmonary vasculature. An echocardiogram showed thinning of the IVS and LVPW with hypokinesis and dilatation of the cavity compatible with dilated cardiomyopathy (DCM). She delivered a boy on July 15th 1982 at 32 weeks gestation following which she developed marked congestive heart failure. She expired one month later. A chest radiograph made one day before death revealed marked cardiomegaly, a cardiothoracic ratio of 0.76, prominent pulmonary vasculature and a pleural effusion. Autopsy was refused; therefore the terminal DCM-like features were not clarified pathologically. However, the slow progression from HOCM to HCM during eight years and then rapid progression from HCM to DCM during four months was most impressive.

List of MeSH codes (C14)

– heart failure, congestive – cardiomyopathy, dilated – dyspnea, paroxysmal – edema, cardiac – heart neoplasms – heart rupture – heart rupture, post-infarction – ventricular septal rupture – heart valve diseases – aortic valve insufficiency – aortic valve stenosis – aortic stenosis, supravalvular – williams syndrome – aortic stenosis, subvalvular – cardiomyopathy, hypertrophic – discrete subaortic stenosis – heart murmurs – heart valve prolapse – aortic valve prolapse – mitral valve prolapse – tricuspid valve prolapse – mitral valve insufficiency – mitral valve stenosis – pulmonary atresia – pulmonary valve insufficiency – pulmonary valve stenosis – leopard syndrome – pulmonary subvalvular stenosis – tricuspid atresia – tricuspid valve insufficiency – tricuspid valve stenosis

Importance of Clinical and Laboratory Findings in the Diagnosis and Surgical Prognosis of Patients with Constrictive Pericarditis.

International studies have reported the value of the clinical profile and laboratory findings in the diagnosis of constrictive pericarditis. However, Brazilian population data are scarce. To assess the clinical characteristics, sensitivity of imaging tests and factors related to the death of patients with constrictive pericarditis undergoing pericardiectomy. Patients with constrictive pericarditis surgically confirmed were retrospectively assessed regarding their clinical and laboratory variables. Two methods were used: transthoracic echocardiography and cardiac magnetic resonance imaging. Mortality predictors were determined by use of univariate analysis with Cox proportional hazards model and hazard ratio. All tests were two-tailed, and an alpha error ≤ 5% was considered statically significant. We studied 84 patients (mean age, 44 ± 17.9 years; 67% male). Signs and symptoms of predominantly right heart failure were present with jugular venous distention, edema and ascites in 89%, 89% and 62% of the cases, respectively. Idiopathic etiology was present in 69.1%, followed by tuberculosis (21%). Despite the advanced heart failure degree, low BNP levels (median, 157 pg/mL) were found. The diagnostic sensitivities for constriction of echocardiography and magnetic resonance imaging were 53.6% and 95.9%, respectively. There were 9 deaths (10.7%), and the risk factors were: anemia, BNP and C reactive protein levels, pulmonary hypertension >55 mm Hg, and atrial fibrillation. Magnetic resonance imaging had better diagnostic sensitivity. Clinical, laboratory and imaging markers were associated with death.

Heart Failure and Ejection Fraction -- History and Physical

A thorough history and physical exam should be obtained and performed in all patients with suspected HF, as the entire basis of the diagnosis is on clinical symptoms and signs. It also should include assessment of risk factors and possible etiologies of the HF. Symptoms of HF are similar regardless of the EF. Symptoms are more severe with exertion and either secondary to fluid accumulation (dyspnea, orthopnea, edema, and abdominal discomfort from hepatic congestion and ascites in the setting of right heart failure) or due to decreased cardiac output (fatigue, anorexia, and weakness). Other less typical symptoms include nocturnal cough, loss of appetite, wheezing, palpitations, depression, syncope, bendopnea (shortness of breath while bending forward), and dizziness. In advanced HF, patients may have resting sinus tachycardia, diaphoresis, narrow pulse pressure (less than 25 mmHg due to decreased cardiac output), and peripheral vasoconstriction (cool and pale extremities due to decreased perfusion). Volume overload manifests as peripheral edema (extremities edema, ascites, scrotal edema, and hepatosplenomegaly), elevated jugular venous pressure (JVP), and pulmonary congestion (rales on the exam and pleural effusions). Displaced apical impulse (laterally past the midclavicular line, which is a sign of LV enlargement), parasternal lift (right ventricular enlargement), and an S3 gallop. At each clinic visit, symptoms and signs of HF require assessment to monitor response to therapy and stability over time. It is also important to check vital signs and assess volume status during each clinic visit.

Congestive heart failure in adults. Six considerations in systematic diagnosis.

There are two key clinical questions to be answered in any patient with documented congestive heart failure. First, what is the underlying etiology? Second, what exacerbating factors are present? The ECG, the echocardiogram, and chest x-ray films may provide important diagnostic clues. The mode of presentation (acute vs chronic) is also of major importance. Attention should be paid to treatable or reversible factors, including infective endocarditis, occult aortic stenosis, recurrent pulmonary emboli, and chronic constrictive pericarditis. Finally, the possibility of unrecognized congenital heart disease (eg, atrial septal defect) should not be overlooked.

Dilated cardiomyopathy

Signs and symptoms Dilated cardiomyopathy develops insidiously, and may not initially cause symptoms significant enough to impact on quality of life. Nevertheless, many people experience significant symptoms. These might include: Shortness of breath Syncope (fainting) Angina, but only in the presence of ischemic heart disease A person suffering from dilated cardiomyopathy may have an enlarged heart, with pulmonary edema and an elevated jugular venous pressure and a low pulse pressure. Signs of mitral and tricuspid regurgitation may be present.

Aortic Valve Disease -- Differential Diagnosis

The differential diagnosis for patients presenting with aortic valve disease symptoms includes but is not limited to: Hypertrophic obstructive cardiomyopathy Restrictive cardiomyopathy Constrictive cardiomyopathy Congestive heart failure with reduced ejection fraction (HFrEF) Coronary artery disease Atrial fibrillation Atrial flutter Ischemic heart disease Pericardial effusion Pulmonary hypertension Chronic obstructive pulmonary disease Restrictive lung diseases Symptomatic anemia

Kussmaul's sign

Causes The differential diagnoses of Kussmaul's sign includes constrictive pericarditis, restrictive cardiomyopathy, pericardial effusion, and severe right-sided heart failure. With cardiac tamponade, jugular veins are distended and typically show a prominent x descent and an absent y descent as opposed to patients with constrictive pericarditis (prominent x and y descent), see Beck's triad. Other possible causes of Kussmaul's sign include: Right ventricular infarction - low ventricular compliance Right heart failure Cardiac tumours Tricuspid stenosis Restrictive cardiomyopathy Pulmonary embolism Constrictive pericarditis History Kussmaul's sign is named after the German doctor who first described it, Adolph Kussmaul (1822-1902). He is also credited with describing Kussmaul breathing. See also Pulsus paradoxus References Symptoms and signs: Vascular

[Clinical features of heart failure induced by pericarditis and myocarditis].

The clinical condition of heart failure induced by pericarditis is expressed as cardiac tamponade or constrictive pericarditis. In both conditions, cardiac filling is impaired either by pericardial fluid or nonelastic pericardial sac under increased diastolic pressure and cardiac output is reduced. Patients show signs of right side heart failure. Immediate pericardiocentesis or pericardiotomy is required for cardiac tamponade, and pericardiectomy is essential for constrictive pericarditis. The clinical expression of myocarditis ranges from asymptomatic state to fulminant fatal congestive heart failure. Recently, identification of viral infection, using polymerase chain reaction (PCR), has contributed to the diagnosis of myocarditis, involving cases of idiopathic congestive cardiomyopathy.

Diabetic cardiomyopathy

One particularity of diabetic cardiomyopathy is the long latent phase, during which the disease progresses but is completely asymptomatic. In most cases, diabetic cardiomyopathy is detected with concomitant hypertension or coronary artery disease. One of the earliest signs is mild left ventricular diastolic dysfunction with little effect on ventricular filling. Also, the diabetic patient may show subtle signs of diabetic cardiomyopathy related to decreased left ventricular compliance or left ventricular hypertrophy or a combination of both. A prominent “a” wave can also be noted in the jugular venous pulse, and the cardiac apical impulse may be overactive or sustained throughout systole. After the development of systolic dysfunction, left ventricular dilation and symptomatic heart failure, the jugular venous pressure may become elevated, the apical impulse would be displaced downward and to the left. Systolic mitral murmur is not uncommon in these cases. These changes are accompanied

InternalMed_Harrison

Suspected severe valve disease in symptomatic patients—dyspnea, angina, heart failure, syncope Infective endocarditis with need for cardiac surgery Asymptomatic patients with aortic regurgitation and cardiac enlargement or Prior to cardiac surgery in patients with suspected coronary artery disease New onset with angina or suspected undiagnosed coronary artery disease New-onset cardiomyopathy of uncertain cause or suspected to be due to coronary artery disease Prior to surgical correction, when symptoms or noninvasive testing suggests coronary disease Symptomatic patients with suspected cardiac tamponade or constrictive pericarditis Hypertrophic cardiomyopathy with angina Diseases of the aorta when knowledge of coronary artery involvement is necessary for management

Peripartum cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting typically between the last month of pregnancy and up to six months postpartum. As with other forms of dilated cardiomyopathy, PPCM involves systolic dysfunction of the heart with a decrease of the left ventricular ejection fraction (EF) with associated congestive heart failure and an increased risk of atrial and ventricular arrhythmias, thromboembolism (blockage of a blood vessel by a blood clot), and even sudden cardiac death. In essence, the heart muscle cannot contract forcefully enough to pump adequate amounts of blood for the needs of the body's vital organs. PPCM is a diagnosis of exclusion, wherein patients have no prior history of heart disease and there are no other known possible causes of heart failure. Echocardiogram is used to both diagnose and monitor the effectiveness of treatment for PPCM.

Pulmonary hypertension

WHO Group II – Pulmonary hypertension secondary to left heart disease Left ventricular systolic dysfunction Left ventricular diastolic dysfunction Valvular heart disease Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathy Congenital/acquired pulmonary venous stenosis WHO Group III – Pulmonary hypertension due to lung disease, chronic hypoxia Chronic obstructive pulmonary disease (COPD) Interstitial lung disease Mixed restrictive and obstructive pattern pulmonary diseases Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental abnormalities WHO Group IV – chronic arterial obstruction Chronic thromboembolic pulmonary hypertension (CTEPH) Other pulmonary artery obstructions Angiosarcoma or other tumor within the blood vessels Arteritis Congenital pulmonary artery stenosis Parasitic infection (hydatidosis)

Congestive Heart Failure -- History and Physical -- Minor Criteria

Ankle edema Dyspnea on exertion Hepatomegaly Nocturnal cough Pleural effusion Tachycardia (heart rate greater than 120 beats per minute)

Restrictive cardiomyopathy

The most common cause of restrictive cardiomyopathy is amyloidosis. Mechanism Rhythmicity and contractility of the heart may be normal, but the stiff walls of the heart chambers (atria and ventricles) keep them from adequately filling, reducing preload and end-diastolic volume. Thus, blood flow is reduced, and blood volume that would normally enter the heart is backed up in the circulatory system. In time, restrictive cardiomyopathy patients develop diastolic dysfunction and eventually heart failure. Diagnosis Diagnosis is typically made via echocardiography. Patients will demonstrate normal systolic function, diastolic dysfunction, and a restrictive filling pattern. 2-dimensional and Doppler studies are necessary to distinguish RCM from constrictive pericarditis. Cardiac MRI and transvenous endomyocardial biopsy may also be necessary in some cases. Reduced QRS voltage on EKG may be an indicator of amyloidosis-induced restrictive cardiomyopathy.


Very easy question in which we are being presented with a patient with Stauffer's syndrome, a paraneoplastic syndrome consisting of liver dysfunction secondary to toxic products secreted by a number of tumors (most frequently, renal cell carcinoma, like the one in this case). As in this case, cholestasis (elevation of alkaline phosphatase), mobilization of hepatic enzymes and lengthening of the prothrombin time indicate liver dysfunction. Metastases are ruled out (there are no defects of hepatic infiltration), intrahepatic tumor thrombosis (it would give another symptomatology and this would be more acute), and acute hepatitis and hemochromatosis (they are not in context nor is it what the author of the question wants us to think).

A 54-year-old man is diagnosed with a left renal tumor suggestive of renal cell carcinoma. His preoperative workup shows elevated levels of GPT, alkaline phosphatase and alpha-2-globulin and prolonged prothrombin time. The liver is diffusely enlarged, but without defects of hepatic infiltration. The most likely rationale for these findings is due to:

Presence of hepatotoxic substances produced by the tumor.

Stauffer's syndrome variant with cholestatic jaundice: a case report.

Cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer's syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, alpha-2-globulin, and gamma-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. Jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer's syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.

Stauffer's syndrome in renal cell carcinoma evidence for intravascular coagulation.

In 40 patients with non-metastasising (n = 31) and metastasising (n = 9) renal cell carcinoma, evidence of Stauffer's syndrome (increase in alkaline serum phosphatase and prolongation of prothrombin time) was found in 18 patients. Prolongation of prothrombin time was not due to depletion of vitamin K-dependent coagulation factors or manifest fibrinolysis, but due to the presence of circulating fibrinogen fibrinmonomer-FDP complexes. Ethanol gelation test was found to be positive in 28/40 subjects and soluble fibrin monomer complexes were increased in 38/40 patients. The resulting disturbance of fibrinogen-fibrin conversion was reflected by an increase in thrombin coagulase time and reptilase time. These findings suggests a state of latent compensated intravascular coagulation (presumably triggered within the vascular tumor). For diagnostic purposes the most sensitive indicator is thrombin coagulase time. Thrombin coagulase time normalised after tumor resection and was positive in patients with recurrent metastases. The increase in alkaline serum phosphatase was due to an increase in the hepatic isoenzyme. Such an increase was much more common than the elevation of total alkaline serum phosphatase. Regan's isoenzyme was only found in 1 subject. In parallel, gamma-GT was elevated in 24 patients. The study shows that Stauffer's syndrome occurs more frequently than commonly assumed when thrombin coagulase time, gamma-GT and the hepatic isoenzyme of alkaline serum phosphatase are determined in patients with renal cell carcinoma. DIC and low grade fibrinolysis may account for the coagulation abnormalities of the syndrome.

InternalMed_Harrison

Type of Disorder Bilirubin Aminotransferases Alkaline Phosphatase Albumin Prothrombin Time Acute hepatocellular necrosis (viral and drug hepatitis, hepatotoxins, acute heart failure) Alcoholic hepatitis, cirrhosis Infiltrative diseases (tumor, granulomata); partial bile duct obstruction Normal to 86 μmol/L (5 mg/dL) 85% due to indirect fractions Elevated, often >500 IU, ALT > AST Elevated, but usually <300 IU AST:ALT >2 suggests alcoholic hepatitis or cirrhosis Normal to moderate elevation Normal to slight elevation Normal to <3× normal elevation Normal to <3× normal elevation Normal to <3× normal elevation Elevated, often >4× normal elevation Elevated, often >4× normal elevation Fractionate, or confirm liver origin with 5’nucleotidase or γ glutamyl transpeptidase Normal Usually normal. If >5× above control and not corrected by parenteral vitamin K, suggests poor prognosis Fails to correct with parenteral vitamin K Fails to correct with parenteral vitamin K

[Liver disease associated with hypernephroma. A case report (author's transl)].

The observation of a non-metastatic reactive hepatopathy associated with a hypernephroma in a 39-year-old man who had had fever for 4 months led to a review of the literature and an analysis of basically three aspects of the disorder: a) The various manifestations of carcinoma of the kidney, which include a large number of paraneoplastic clinical symptoms (polycythemia, anemia, prolonged fever, hypercalcemia, hypertension, nefropathy, loss of salt, peripheral neuropathy, and amyloidosis); b) an alteracion of hepatic function known since 1961 which is characterized by an abnormal retention of sulfobromophthalein, increase of alkaline phosphatase, prothrombin decrease, dysproteinemia with hypoalbuminemia, and alpha2-globulin increase. It may or may not be accompanied by enlargement of the liver. c) Criteria of operability of the primary tumor.

Stauffer syndrome

Stauffer syndrome is a constellation of signs and symptoms of liver dysfunction that arises due to presence of renal cell carcinoma, and, more rarely, in connection with other malignant neoplasms, though the specific pathogenesis is currently unknown. It is named for Dr. Maurice Stauffer, a gastroenterologist at the Mayo Clinic in Rochester, MN. The hepatic abnormalities are not due to tumor infiltration of the liver or intrinsic liver disease; they instead reflect the presence of a paraneoplastic syndrome. Stauffer syndrome causes abnormal liver function tests, especially those that reflect the presence of cholestasis, i.e. abnormal bile flow. Hepatosplenomegaly may also be observed. The symptoms and signs resolve if the renal cell carcinoma (or another associated tumor) is successfully ablated. It is due to release of IL-6 from cancerous cell.

Hepatocellular carcinoma after renal transplantation in the absence of cirrhosis or viral hepatitis: a case series.

The occurrence of hepatocellular carcinoma (HCC) in renal transplant recipients has typically been associated with hepatitis B or C infection. We encountered two cases of HCC in renal transplant recipients with negative hepatitis B and C markers and no underlying liver pathology, in whom immunosuppression therapy consisted of prednisone and azathioprine (AZA). Patient no. 1 is a 66-year-old man with diabetes who underwent cadaveric renal transplantation 13 years before presentation. An ultrasound obtained for evaluation of a prolonged prothrombin time and decreased serum albumin level showed a suspicious nodular lesion in the left lobe of the liver. A computed tomographic (CT) scan confirmed a 4- x 5- x 5-cm mass that, on biopsy, was determined to be well-differentiated HCC. There was no evidence of metastasis, and the results of random biopsies of the surrounding parenchyma were normal. The patient underwent a left lateral segmentectomy, did well, and an initial alpha-fetoprotein (AFP) level of 85995 ng/mL decreased to 9 ng/mL. Approximately 20 months postoperatively, however, a surveillance CT scan showed three hypervascular lesions in the right lobe of the liver and the AFP level increased to 28,370 ng/mL. Subsequent percutaneous alcohol injections yielded good results, and the patient is alive and well 13 months later. Patient no. 2 is a 57-year-old man who underwent cadaveric renal transplantation 24 years earlier. A CT scan of the abdomen obtained for evaluation of lower abdominal pain showed a 4- x 4- x 6.5-cm mass in the right lobe of the liver that, on biopsy, was found to be poorly differentiated HCC. Multiple biopsies of adjacent liver parenchyma showed no evidence of cirrhosis, AFP level was normal, and imaging studies showed no evidence of tumor spread. The patient underwent a right hepatic lobectomy and is doing well without evidence of recurrence 27 months postoperatively. Our two patients had no evidence of viral hepatitis, cirrhosis, or metabolic liver disease, yet both developed HCC. The use of AZA may have had a role in the development of HCC. In renal transplant recipients on long-term immunosuppression therapy, particularly AZA, it is prudent to maintain a high index of suspicion for HCC when liver enzyme level or function abnormalities are encountered.

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In addition to enzyme tests, all jaundiced patients should have additional blood tests—specifically, an albumin level and a prothrombin time—to assess liver function. A low albumin level suggests a chronic process such as cirrhosis or cancer. A normal albumin level is suggestive of a more acute process such as viral hepatitis or choledocholithiasis. An elevated prothrombin time indicates either vitamin K deficiency due to prolonged jaundice and malabsorption of vitamin K or significant hepatocellular dysfunction. The failure of the prothrombin time to correct with parenteral administration of vitamin K indicates severe hepatocellular injury. The results of the bilirubin, enzyme, albumin, and prothrombin time tests will usually indicate whether a jaundiced patient has a hepatocellular or a cholestatic disease and offer some indication of the duration and severity of the disease. The causes and evaluations of hepatocellular and cholestatic diseases are quite different.

[Stauffer syndrome, paraneoplastic hepatic dysfunction syndrome associated with renal cell carcinoma (author's transl)].

In two patients with renal cell carcinoma, the following biochemical changes were found independently of hepatic metastases: increased alkaline phosphatase activity, rise in bromsulfothalein retention, hypoalbuminemia, increase in alpha2-globulin, and prolonged prothrombin time (Stauffer syndrome). In both cases the syndrome was found to be the first sign of the renal cell carcinoma. In one patient liver function returned to normal after removal of the neoplasm, in correlation with the good recovery. In the other case the abnormal laboratory findings persisted after total renal surgery. Clinically, diffuse pulmonary metastases occurred. Both case histories show the high significance in knowing Stauffer syndrome and its value for early diagnosis and operative success.

Metastatic liver disease

A liver metastasis is a malignant tumor in the liver that has spread from another organ affected by cancer. The liver is a common site for metastatic disease because of its rich, dual blood supply (the liver receives blood via the hepatic artery and portal vein). Metastatic tumors in the liver are 20 times more common than primary tumors. In 50% of all cases the primary tumor is of the gastrointestinal tract, other common sites include the breast, ovaries, bronchus and kidney. Tumor emboli entering the sinusoids through the liver blood supply appear to be physically obstructed by the Kupffer cells, but if tumor emboli are larger, they tend to become lodged in the portal venous branches. Presentation Hepatomegaly - with a nodular free edge of liver Tenderness Cachexic Ascites Jaundice Pyrexia - up to 10% of patients Alkaline Phosphatase (ALP) and gamma glutamyl transpeptidase (GGT) elevated Ultrasound scan and CT scan - multiple filling defects.

Liver tumor

Because hepatocellular adenomas are so rare, there are no clear guidelines for the best course of treatment. The complications, which include malignant transformation, spontaneous hemorrhage, and rupture, are considered when determining the treatment approach Estimates indicate approximately 20-40% of hepatocellular adenomas will undergo spontaneous hemorrhage. The evidence is not well elucidated, but the best available data suggests that the risk of hepatocellular adenoma becoming hepatocellular carcinoma, which is malignant liver tumor, is 4.2% of all cases. Transformation to hepatocellular carcinoma is more common in men. Currently, if the hepatic adenoma is >5 cm, increasing in size, symptomatic lesions, has molecular markers associated with HCC transformation, rising level of liver tumor markers such as alpha fetoprotein, the patient is a male, or has a glycogen storage disorder, the adenoma is recommended to be surgically removed. Like most liver tumors, the anatomy and location

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Neutropenia and lymphopenia are transient and are followed by a relative lymphocytosis. Atypical lymphocytes (varying between 2 and 20%) are common during the acute phase. Measurement of the prothrombin time (PT) is important in patients with acute viral hepatitis, because a prolonged value may reflect a severe hepatic synthetic defect, signify extensive hepatocellular necrosis, and indicate a worse prognosis. Occasionally, a prolonged PT may occur with only mild increases in the serum bilirubin and aminotransferase levels. Prolonged nausea and vomiting, inadequate carbohydrate intake, and poor hepatic glycogen reserves may contribute to hypoglycemia noted occasionally in patients with severe viral hepatitis. Serum alkaline phosphatase may be normal or only mildly elevated, whereas a fall in serum albumin is uncommon in uncomplicated acute viral hepatitis. In some patients, mild and transient steatorrhea has been noted, as well as slight microscopic hematuria and minimal proteinuria.

Liver tumor

Available evidence suggests that bleeding occurs in approximately 63% of patients with liver cell adenomatosis. Liver cell adenomatosis is also associated with becoming hepatocellular carcinoma. Like hepatic adenomas, they are diagnosed with imaging and biopsies as needed. Treatment of liver cell adenomatosis is difficult due to the multiple, widespread lesions. Liver imaging should be reviewed to see if it is possible to surgically remove the tumors. Liver transplantation is a treatment option for some patients.

Acute liver failure due to diffuse intrasinusoidal metastases of urothelial carcinoma.

We report one case of urothelial carcinoma with diffuse intrasinusoidal metastasis to the liver and clinical presentation mimicking fulminant hepatic failure. The patient was a 69-year-old man admitted to the hospital for upper gastrointestinal hemorrhage. Two years previously he had undergone a right nephrectomy for urothelial carcinoma (T2, GII). After five days of hospital admission, he developed progressive jaundice, ascites, deteriorating mental status with high serum enzyme activities (AST, ALT, LDH, alkaline phosphatase) and death 20 days after hospitalization. No grossly detectable hepatic metastatic nodules were demonstrated. A percutaneous postmortem liver biopsy revealed a diffuse infiltration of tumor cells into the hepatic sinusoids and venous invasion.

Nimesulide-induced fatal acute liver failure in an elderly woman with metastatic biliary adenocarcinoma. A case report.

Nimesulide is a selective inhibitor of the enzyme cyclooxygenase 2. Although considered to be a safe drug, cases of acute hepatitis and fulminant liver failure have been reported in Europe, the United States and South America, especially among elderly female patients. Until now, there had not been any reports in the literature relating to Brazilian subjects. An 81-year old female who had been using nimesulide therapy for six days presented hematemesis and epistaxis two days before hospitalization. Clinical examination showed an extensive coagulation disorder, diffuse hematomas, hypotension and tachypnea. Laboratory tests revealed abnormalities in coagulation tests; leukocytosis; reduced platelet, hemoglobin and red blood cell counts; and elevated direct bilirubin, serum aspartate transaminase (AST), gamma-glutamyl transpeptidase (GGT), alkaline phosphatase and renal function biomarkers. Hepatitis B and C tests were not reactive. Carcinoembryonic antigen (CEA), CA-19-9 and CA-125 levels were increased by, respectively, 1,000, 10,000 and 13 fold, whereas the alpha-fetoprotein level was normal, thus indicating a malignant tumor in the bile duct that did not originate from the liver. Thirty-six hours after hospitalization, the patient's condition worsened, leading to death. The necropsy findings included acute hepatitis with hepatocellular collapse, as well as metastasis of a carcinoma, probably from the bile duct. Despite the carcinoma presented by the patient, nimesulide use may have contributed towards the fatal acute liver failure. Until this issue has been clarified, caution is required in prescribing nimesulide for liver disease patients.

Fibrolamellar hepatocellular carcinoma

Diagnosis Due to lack of symptoms, until the tumor is sizable, this form of cancer is often advanced when diagnosed. Symptoms include vague abdominal pain, nausea, abdominal fullness, malaise and weight loss. They may also include a palpable liver mass. Other presentations include jaundice, ascites, fulminant liver failure, encephalopathy, gynecomastia (males only), thrombophlebitis of the lower limbs, recurrent deep vein thrombosis, anemia and hypoglycemia. The usual markers for liver disease – aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase – are often normal or only slightly elevated. FHCC often does not produce alpha fetoprotein (AFP), a widely used marker for conventional hepatocellular carcinoma. It is associated with elevated neurotensin levels. Diagnosis is normally made by imaging (ultrasound, CT or MRI) and biopsy

Metastasectomy

Preoperative evaluation involves imaging of the liver and its metastases, for example with ultrasound, computed tomography or magnetic resonance imaging. Positron emission tomography can be useful to check the entire body for metastases, although the test can be falsely normal with small lesions or preoperative chemotherapy. Baseline blood tests typically include liver function tests and tumour markers. During surgery, intraoperative ultrasound can aid the surgeon to find additional metastases. A clinical risk score first proposed by Fong et al. is often used to assess the risk of recurrence after hepatic resection. The score assigns one point to each of the following: CEA level >200 ng/ml Disease-free interval from primary to metastasis <12 months Node-positive primary More than one liver metastasis Largest hepatic tumor >5 cm The median survival for each score is:

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has been reported to be safe and useful in the control of major branch portal or hepatic vein invasion (thrombosis) by tumors. The studies have all been small. Vitamin K has been assessed in clinical trials at high dosage for its HCC-inhibitory actions. This idea is based on the characteristic biochemical defect in HCC of elevated plasma levels of immature prothrombin (DCP or PIVKA-2), due to a defect in the activity of prothrombin carboxylase, a vitamin K–dependent enzyme. Two vitamin K randomized controlled trials from Japan show decreased tumor occurrence, but a major phase III trial aimed at limiting postresection recurrence was not successful.

Hepatocellular carcinoma

Important features that guide treatment include: size spread (stage) involvement of liver vessels presence of a tumor capsule presence of extrahepatic metastases presence of daughter nodules vascularity of the tumor MRI is the best imaging method to detect the presence of a tumor capsule. The most common sites of metastasis are the lung, abdominal lymph nodes, and bone. Prevention Since hepatitis B and C are some of the main causes of hepatocellular carcinoma, prevention of infection is key to then prevent HCC. Thus, childhood vaccination against hepatitis B may reduce the risk of liver cancer in the future. In the case of patients with cirrhosis, alcohol consumption is to be avoided. Also, screening for hemochromatosis may be beneficial for some patients. Whether screening those with chronic liver disease for HCC improves outcomes is unclear.

Stauffer syndrome, clinical implications and knowledge gaps, does size matter? Case report.

Stauffer Syndrome (SS) is a paraneoplastic disorder associated with renal cell carcinoma (RCC). First described by Herbert Maurice Stauffer in 1961, it is characterized by hepatic dysfunction in the absence of metastasis, and elevated alkaline phosphatase, aminotransferases and prolonged prothrombin time. Initial reports did not include jaundice as an inclusion criterion. We aim to report the rare SS jaundice variant in a patient with a small renal mass and review the literature to determine if the size of the renal mass could influence the development of the SS. The aim of this article is to present the case of a 53-year-old male with Stauffer syndrome jaundice variant secondary to a 2.5 cm renal mass, treated with laparoscopic radical nephrectomy with complete resolution of the syndrome. This syndrome is yet to be fully understood, and as far as the evidence shows, size does not matter. This entity should always be in mind when encountered with a patient with liver dysfunction and jaundice with a suspicion or confirmed diagnosis of a renal mass. To date, and to our knowledge, there have been eleven reported cases of paraneoplastic cholestatic jaundice syndrome including the current case. Six cases presented in patients with small renal mases (< 4 cm), curiously there were not a laterality tendency.

Renal cell carcinoma

In cases where the tumor has spread into the renal vein, inferior vena cava, and possibly the right atrium, this portion of the tumor can be surgically removed, as well. When the tumor involved the inferior vena cava, it is important to classify which parts of the vena cava are involved and to plan accordingly, as sometimes complete resection will involve an incision into the chest with increased morbidity. For this reason, Dr. Gaetano Ciancio, adapted liver mobilization techniques from liver transplant to address retrohepatic or even suprahepatic inferior vena caval thrombus associated with renal tumors. With this technique, the whole abdominal inferior vena cava is able to be mobilized. This facilitates milking of the tumor down below the major hepatic veins by the surgeon’s fingers, bypassing the need for a thoracoabdominal incision or cardiopulmonary bypass. In cases of known metastases, surgical resection of the kidney ("cytoreductive nephrectomy") may improve survival, as well

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The prothrombin time may be elevated in hepatitis and cirrhosis as well as in disorders that lead to vitamin K deficiency such as obstructive jaundice or fat malabsorption of any kind. Marked prolongation of the prothrombin time, >5 s above control and not corrected by parenteral vitamin K administration, is a poor prognostic sign in acute viral hepatitis and other acute and chronic liver diseases. The INR, along with the total serum bilirubin and creatinine, are components of the MELD score, which is used as a measure of hepatic decompensation and to allocate organs for liver transplantation. Although tests may direct the physician to a category of liver disease, additional radiologic testing and procedures are often necessary to make the proper diagnosis, as shown in Fig. 358-1. The most commonly used ancillary tests are reviewed here, as are the noninvasive tests available for assessing hepatic fibrosis.

Anabolic steroid

Hepatic: elevated liver function tests (, , bilirubin, , ), hepatotoxicity, jaundice, hepatic steatosis, hepatocellular adenoma, hepatocellular carcinoma, cholestasis, peliosis hepatis; all mostly or exclusively with 17α-alkylated AAS. Renal: renal hypertrophy, nephropathy, acute renal failure (secondary to rhabdomyolysis), focal segmental glomerulosclerosis, renal cell carcinoma. Others: glucose intolerance, insulin resistance, immune dysfunction.

[Pitfalls in the workup of common hepatobiliary problems].

Hepatobiliary problems are not uncommon in general practice. Many cases can be solved on the basis of a thorough history, clinical examination, blood tests and abdominal ultrasound. The focus of this tutorial paper lies on a number of possible pitfalls in the workup of such problems, based on brief case histories. Atypical presentations of lithogenic biliary obstruction, with or without cholangitis, are discussed as a relatively frequent cause of management difficulties. Furthermore, certain caveats in the interpretation of tumor marker CA19-9, prothrombin time/INR, and ferritin are highlighted.

CKLF-like MARVEL transmembrane domain-containing 5

cancer, non-small-cell lung carcinoma, renal cell carcinoma, and breast cancer. The forced over expression of CMTM5-v1 in Huh7 human hepatic cells also inhibited the ability of these cells to grow in a mouse model of cancer. Finally, various cancer human cell lines including those of the liver, breast, prostate, colon, stomach, nasopharynx, laryngopharynx, esophagus, lung, and cervix express low levels of, or no, CMTM5-v1 and concurrently have highly methylated CpG sites near to the CMTM5 gene. These findings suggest that the CMTM5 gene may act as a tumor suppressor gene, i.e. a normal gene whose product(s) inhibit the development and/or progression of various cancers. The findings also support further studies to confirm and expand these relationships and determine if the expression of CMTM5 isoforms can be used as tumor markers for these cancers severities/prognoses and/or targets as for treating them.

Stauffer Syndrome: A Comprehensive Review of the Icteric Variant of the Syndrome.

Stauffer syndrome is a rare paraneoplastic syndrome classically associated with renal cell carcinoma (RCC). This association gave it the historical name of nonmetastatic nephrogenic hepatic dysfunction syndrome without jaundice. It is a syndrome of unclear pathophysiology characterized by a reversible anicteric elevation of liver enzymes, alkaline phosphatase, erythrocyte sedimentation rate (ESR), thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly in the absence of direct hepatobiliary obstruction or jaundice. A rare atypical variant of this syndrome with jaundice has been recently described in the literature. Thus, it is important to consider both these variants of Stauffer syndrome in the differential diagnosis of unexplained cholestasis in the absence of hepatic metastasis. This may allow early recognition and treatment of an occult malignancy. Herein, we present a comprehensive review of the literature available on the icteric variant of the Stauffer syndrome, outlining its association with various malignancies and the diagnostic challenges it poses. The objective of this review is to help clinicians in its early diagnosis and management.

Bland embolization

Patient with hepatic metastatic disease from neuroendocrine tumors, gastrointestinal stromal tumors other sarcomas, ocular melanoma, and a variety of “hypervascular” metastases (eg from breast cancer or renal cell cancer) may also be candidates for bland embolization, assuming the liver is the only site of disease, or when the procedure is being performed for palliation of symptoms. Since the purpose of hepatic embolization in these cases is to either treat symptoms or extend survival, patient who are asymptomatic from their secondary hepatic disease and who have disease elsewhere should probably not be considered candidates. Intra-arterial therapy based on ischemia induced by terminal vessel blockade should not be expected to be efficacious in patients with hypovascular tumors and has no proven role in the treatment of typical metastatic adenocarcinoma from most gastrointestinal malignancies.

Liver tumor

Hepatocellular adenomas are most often asymptomatic and often found incidentally on imaging. However, if signs and symptoms are present they include non-specific abdominal pain, yellowing of skin, and higher than normal gamma glutamyl transferase and alkaline phosphatase laboratory values. They are, in most cases, located in the right hepatic lobe and are frequently seen as a single lesion. Their size ranges from 1 to 30 cm. They can be difficult to diagnosis with imaging studies alone, because it can be hard to tell the difference between hepatocellular adenoma, focal nodular hyperplasia, and hepatocellular carcinoma. Molecular categorization via biopsy and pathological analysis aids in both diagnosis and understanding prognosis, particularly because hepatocellular adenomas have the potential to become malignant. It is important to note, percutaneous biopsy should be avoided, because this method can lead to bleeding or rupture of the adenoma. The best way to biopsy suspected hepatic

Elevated alpha-fetoprotein

Other tumor Rare AFP-secreting tumor types include carcinoma in a mixed Müllerian tumor. The Sertoli-Leydig cell tumor, which itself is rare, rarely secretes AFP. In Wilms tumor AFP is rarely elevated, but when it is elevated it may serve as a marker of disease progression or recurrence. Other Increased serum levels in adults are also seen in acute hepatitis, colitis and ataxia telangiectasia. Increased serum levels of alpha-fetoprotein are sometimes found in citrullinemia and argininosuccinate synthetase deficiency. Amniotic fluid AFP in amniotic fluid has one or two sources. The fetus normally excretes AFP into its urine, hence into the amniotic fluid. A fetus with one of three broad categories of defects also releases AFP by other means. These categories are open neural tube defect, open abdominal wall defect, and skin disease or other failure of the interior or exterior body surface.

The prognosis in cases of hepatocellular carcinoma after hepatectomy: young patients versus older patients.

Hepatocellular carcinoma (HCC) is uncommon in young adults and the prognosis of these patients is still unclear. In this retrospective study, we compared the clinicopathological characteristics and outcomes of young patients with HCC with those of older patients with HCC. We retrospectively reviewed the clinicopathological characteristics of a total of 1,124 patients with HCC who underwent hepatectomy at our institution between 2006 and 2010. Patients ≤40 years of age at the time of HCC diagnosis were classified in the younger group. One hundred and three patients (9.2%) were classified in the younger group. whereas, 1021 patients were classified in the older group. The incidences of hepatitis B virus infection, alpha-fetoprotein (AFP) levels, and indocyanine green retention test were all higher in younger patients than in older patients (p<0.05). Disease-free survival and overall survival were longer in older patients than in younger patients, without statistical significance. In younger patients, increased levels of protein induced by vitamin K antagonist-II (PIVKA-II) and alkaline phosphatase, portal vein tumor thrombosis, and intrahepatic metastasis were all predisposing factors for tumor recurrence after hepatectomy. Although the AFP levels were higher in younger patients with HCC than in older patients with HCC, disease-free survival and overall survival after liver resection were not significantly different between the two groups.

5'-nucleotidase

Medical relevance 5' Nucleotidase blood test The concentration of 5’nucleotidase protein in the blood is often used as a liver function test in individuals that show signs of liver problems. The combined assays of serum 5'nucleotisase and alkaline phosphatase (AP) activities are extremely helpful in differential diagnosis since serum 5'nucleotidase activity is increased in obstructive hepatobiliary disorders, but not in osseous disorders, whereas serum AP activity is generally increased in both categories of diseases. In other words, the test is used to determine if elevated protein levels are due to skeletal damage or liver damage. Normal levels of 5’nucleotidase are 2-17 units per liter. Elevated levels may indicate cholestasis, destruction of liver cells, hepatitis (liver inflammation), liver ischemia, a liver tumor, or use of liver-damaging drugs. Pregnancy and certain medications (acetaminophen, halothane, isoniazid, methyldopa, nitrofurantoin) may interfere with the test.

[A clinical study of renal tumors].

Seventy one patients with renal tumors treated at our clinic during the 11 years from 1970 to 1980 were clinically examined. The results are summarized as follows. The frequency of patients with renal tumors was 0.22% of the outpatients and 1.72% of the inpatients. Of the 71 renal tumors, 41 were renal adenocarcinoma, and 26 were renal pelvic tumors of which 23 were transitional cell tumors, 2 were squamous cell tumors, and 1 was adenocarcinoma. The other tumors were 1 adenoma, 1 hemangioma, 1 hematoma, and 1 foreign body granuloma. The right and left kidneys were affected at equal frequencies. Male patients were more commonly affected, the sex ratio being 39 to 32. The youngest case was a 29-year-old female, and the eldest was a 84-year-old male. As the initial symptoms and chief complaints, gross hematuria was most frequent (52 cases, 73.2%), followed abdominal tumor mass (32 cases, 45.1%), and fever (26 cases, 36.6%). Only 2 cases showed the classic triad, while 1 case had none of them. The period between onset of symptoms and admission, was within 1 year for all patients except for 2 cases. Metastasis was found in 52 cases. The lung was the most frequent site of metastasis (12 cases, 23.1%), followed by lymphnodes, bones, and liver. The clinical examinations performed and diagnostic techniques used were, renal function (BUN, Serum Cr), Hb, WBC, liver function (T. Bil, GOT, GPT), serum protein fraction, serum LDH, serum Ca, ESR, tumor marker (AFP, CEA), urine cytological examination, blood pressure, IVP (or RP), angiography. As the therapeutic method, nephrectomy was performed in 25 cases (35.2%), combined nephrectomy and irradiation therapy in 12 cases (16.9%), combined nephrectomy and chemotherapy in 11 cases (15.5%), combined nephrectomy and other therapy in 15 cases (21.1%), and conservative therapy in 8 cases (11.3%). For the entire traced series of renal tumors, the 1-, 3-and 5-year survival rates were 72.3, 49.8, and 49.8% respectively. For renal parenchymal tumors (renal adenocarcinoma), the 1-, 3-and 5-year survival rates were 77.8, 53.0, and 53.0%. The most important factor of prognosis was the stage of tumor. Patients with elevated erythrocyte sedimentation rate, and dysproteinemia also had distinctly unfavorable prognosis. In this study of therapy, the highest survival rate was seen for the patients treated by combined nephrectomy and irradiation therapy of both renal parenchymal and pelvic tumors.

Outcome of right hepatectomies in patients older than 70 years.

The increasing number of elderly patients undergoing liver resections mandates updating of clinical outcomes on this specific population. Case series. A tertiary care teaching hospital. Twenty-three patients older than 70 years who underwent right hepatectomies (including 7 extended right hepatectomies) between January 1, 1995, and October 31, 2001 (group 1) and 99 patients younger than 70 years who underwent 64 right hepatectomies and 35 extended right hepatectomies during the same period (group 2) were included for a total sample population of 122. Preoperative clinicopathological features, intraoperative factors, in-hospital mortality, postoperative complications, intensive care unit requirement, hospital stay, and course of main biochemical liver function test results of groups 1 and 2 were analyzed and compared. The 2 groups were similar for indications for surgery and the presence of underlying liver disease. Group 1 had a higher incidence of associated pulmonary diseases (21.7% vs 5%, P =.02) and patients with an American Society of Anesthesiologists score of III (ie, a patient with severe systemic disease limiting activity, but not incapacitating) (56.5% vs 26.3% of cases, P =.01). There were no differences in intraoperative requirement of packed red blood cells and in operation time. There were no in-hospital deaths in group 1; there were 2 deaths (2%) in group 2. Nine patients (39.1%) in group 1 and 32 patients (32.3%) in group 2 experienced postoperative complications (P =.53), of whom, respectively, 5 (21.7%) and 17 (17.2%) developed transient liver dysfunction (P =.56), and 4 (17.4%) and 5 (5.1%) required a supplementary intesive care unit stay (P =.06). The postoperative stay (mean [SD], 16 [14] days vs 13 [9] days, P =.88) and peak values of the aminotransferase level, total serum bilirubin level, and prothrombin time were similar in the 2 groups. The timing of the peak value of the total serum bilirubin level (mean [SD], 4.1 [4.8] days vs 2.5 [2.5] days, P =.28) and its period of normalization (mean [SD], 9.4 [10.8] days vs 6.7 [5.1] days, P =.67) were also similar for both groups. For patients with malignancies, the 3-year survival rate was 64.2% in group 1 and 53.9% in group 2 (P =.53). Being older than 70 years should not be a contraindication for major hepatectomies, provided that liver cirrhosis and severe associated medical conditions are ruled out during the preoperative evaluation.