High-Output Heart Failure PDF

High-output heart failure
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Author Wilson S Colucci, MD Amir Haghighat, MD Section Editor Stephen S Gottlieb, MD Deputy Editor Susan B Yeon, MD, JD, FACC
Last literature review for version 16.1: January 31, 2008 | This topic last updated: May 18, 2006 INTRODUCTION Most patients with heart failure (HF) have systolic dysfunction with a low cardiac output and elevated systemic vascular resistance or diastolic dysfunction in which an increase in ventricular stiffness impairs ventricular filling during diastole. In rare circumstances, the cardiac output is elevated and calculated systemic vascular resistance is very low. High-output HF is characterized by an elevated resting cardiac index beyond the normal range of 2.5 to 4.0 L/min per m2. Ineffective blood volume and pressure, chronic activation of the sympathetic nervous system and renin-angiotensin-aldosterone axis, increased serum vasopressin (antidiuretic hormone) concentrations, and chronic volume overload gradually cause ventricular enlargement, remodeling, and HF. As will be described in this topic review, a number of conditions lead to an obligatory increase in cardiac output, which can be associated with HF in some patients. However, these conditions are rarely the sole cause of HF; in most such patients, the high cardiac output provokes HF in the setting of reduced ventricular reserve from some underlying cardiac problem. Thus, the presence of high-output HF should prompt a search for another underlying cardiac problem. PHYSICAL FINDINGS Several characteristic findings are usually seen on physical examination in patients with high-output HF. The heart rate is typically between 85 and 105 beats per minute, but it may be higher with some causes, eg, thyrotoxicosis. Examination of the systemic veins may reveal a cervical venous hum, heard best over the deep internal jugular veins, particularly on the right side. Less often, a venous hum may be appreciated over the femoral veins. Examination of the arteries may display signs related to increased left ventricular stroke volume. The pulse is usually bounding with a quick upstroke, and the pulse pressure is typically wide. Pistol-shot sounds may be auscultated over the femoral arteries, and a systolic bruit may be heard over the carotid arteries. Although these findings may be seen in other cardiac conditions, such as aortic regurgitation or patent ductus arteriosus, in the absence of these conditions, these signs are highly suggestive of elevated left ventricular stroke volume due to a hyperdynamic state. Cardiac examination may reveal an enlarged heart with a midsystolic murmur in the second and third left intercostal spaces, and a third heart sound, which is due to the increased rate of ventricular filling. Patients with chronic high output also may develop the signs and symptoms classically associated with the more common "low-output" HF; specifically, they may develop pulmonary and/or systemic congestion, while maintaining the above normal cardiac output. PHYSIOLOGIC CAUSES FOR HIGH CARDIAC OUTPUT There are a number of physiologic circumstances that can substantially increase cardiac output:
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Excitement Exercise (see "Exercise physiology") Pregnancy Fever Pregnancy is associated with both systemic vasodilation and increased preload due to a rise in blood volume [1] . (See "Renal and urinary tract physiology in pregnant women" ). Fever increases metabolic demand and produces vasodilation, especially in the skin. Resolution of the fever is accompanied by a reduction in cardiac output to normal. A hot, and especially humid, environment also increases cardiac output through mechanisms similar to fever [ 2] . CAUSES OF HIGH-OUTPUT FAILURE The disorders contributing to high-output heart failure include: Systemic arteriovenous fistulas Hyperthyroidism Anemia, including the anemia of renal failure Beriberi (vitamin B1 or thiamine deficiency) Dermatologic disorders (eg, psoriasis) Renal disease Hepatic disease Skeletal disorders (eg, Paget's disease, multiple myeloma) Hyperkinetic heart syndrome Other potential causes of high-output HF include obesity [ 3] , polycythemia vera [4] , and carcinoid syndrome [5] . (See "Clinical features of the carcinoid syndrome"). Systemic arteriovenous fistulas Arteriovenous fistulas may be congenital or acquired; acquired cases may be iatrogenic or traumatic. As a result of such malformations, blood from a high-pressure artery is shunted to a low-pressure vein, thereby decreasing systemic vascular resistance. A compensatory increase in the heart rate and stroke volume ensues, and total plasma volume is increased. The elevation in cardiac output associated with these fistulas depends upon the size of the communication and the magnitude of the resultant reduction in systemic vascular resistance. The blood flowing through the fistula bypasses the capillary circulation; thus, for capillary perfusion to be normal, the total cardiac output has to be increased by the quantity of blood flowing through the fistula. Physical findings in patients with high-output failure from arteriovenous fistulas depend to some extent upon the underlying disease and the location and size of the shunt. Patients with sizable arteriovenous shunts have a wide pulse pressure with brisk arterial pulsations; the heart rate is usually slightly increased. There may be swelling, warmth, and redness, and a continuous "machinery murmur" with a thrill over fistulas located near the surface. Transient maximal occlusion of the fistula usually decreases heart rate, raises arterial pressure, and lowers venous pressure; this has been termed "Branham's sign" [6] . In the presence of an acquired fistula from trauma, the overlying skin is warmer and the increase in blood flow to the affected limb may result in an increase in size compared to the opposite limb. The chronically elevated venous pressure may lead to cellulitis, venostasis, edema, and a chronic dermatitis with pigmentation.
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Congenital fistulas Congenital arteriovenous fistulas result from arrest of the normal embryonic development of the vascular system. In fact, these fistulas are structurally similar to embryonic capillary networks. Congenital fistulas range from cutaneous hemangiomas to large channel communications that can distort a limb [7] . Some patients have multiple fistulas and the clinical presentation can be varied [8] . One congenital abnormality, hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), is characterized by hemorrhagic telangiectasias involving the mucous membranes, gastrointestinal tract, and skin of the lips, nose, and finger tips. In about 15 percent of afflicted individuals, arteriovenous malformations occur in the lung and may be associated with massive hemoptysis [ 9,10] . (See "Pulmonary AVMs, including hereditary hemorrhagic telangiectasia: Etiology and clinical features" ). Although the liver is less frequently involved than the lung, those patients with both lung and hepatic involvement may develop clinically significant arteriovenous shunts and high-output failure [ 10-13] . In some patients, high-output HF is the first manifestation of the disease [ 11] . Historical clues to the diagnosis include epistaxis, recurrent gastrointestinal bleeding, unexplained hematuria, hemoptysis, or family history of bleeding. Treatment of congenital fistulas The treatment of high output failure in patients with hereditary hemorrhagic telangiectasia poses a clinical challenge and is of limited benefit. In one study a patient with hepatic arteriovenous microfistulas and high output failure was successfully treated by selective hepatic artery embolization, leading to prompt symptomatic improvement, progressive decline in ascites and hepatomegaly, and normalization of cardiac output [13] . Balloon embolization has been used to treat intestinal or pulmonary lesions [ 12] . Limited surgical excision is an option in symptomatic patients with pulmonary fistulae, but only minimal tissue should be removed, since fistulas may continue to form and require further treatment [ 10] . If there is diffuse disease involvement, surgery may not be an option [ 12] . Giant cutaneous hemangiomas Giant cutaneous hemangiomas can also promote the development of high-output failure. Hemangiomas are the most common tumors of infancy, and seldom cause more than a cosmetic problem [14,15] . Fifty percent of cutaneous lesions are present at birth; the remainder usually surface by two months of age. In rare cases, high flow arteriovenous shunting in giant cutaneous hemangiomas can lead to the development of high-output failure [ 8,16] . (See "Epidemiology; pathogenesis; clinical features; and complications of infantile hemangiomas" ). There are several therapeutic approaches to the patient with giant cutaneous hemangiomas and HF. Arteriography with selective embolization has been shown to reverse the failure in select cases [ 14,15] . Administration of corticosteroids has been shown to be effective in mild cases, but less effective in severe cases [16,17] . Other options include surgical excision and radiation therapy to susceptible sites [ 14] . (See "Management of infantile hemangiomas" ). Hepatic hemangiomas In rare cases, involuting hemangiomas involve the liver and produce arteriovenous shunts and high-output failure in a condition called hepatic hemangiomatosis [ 18] . One review of 27 patients with hepatic hemangiomatosis observed that 23 had concurrent cutaneous hemangiomas and 25 had HF [18] . The diagnosis was suggested by the clinical triad of multiple enlarging cutaneous hemangiomas, hepatomegaly, and HF. Almost every one of the infants studied died of the disease or its complications. In one patient, hepatic artery ligation was performed after arteriography, and effectively cured the patient. Nonetheless, this approach remains untested in adult patients, since it may cause fatal hepatic necrosis. Acquired fistulas Acquired fistulas are usually posttraumatic or iatrogenic. Traumatic causes
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including bullet or knife wounds, especially in the thigh [ 19] . Iatrogenic fistulas are surgically constructed arteriovenous fistulas used for access to the circulation in patients undergoing chronic hemodialysis [20,21] . In one report of 15 patients, temporarily closing the fistula lowered the cardiac output by 0.3 to 11 L/min [20] . Anemia (see below) and volume expansion due to salt and water retention may contribute to the high-output state. ( See "Nonthrombotic complications of chronic hemodialysis arteriovenous vascular access"). Left ventricular hypertrophy due to hypertension and premature atherosclerosis are common in patients with end-stage renal disease. This substrate can increase the susceptibility to the stress imposed by an elevated cardiac output. (See "Myocardial dysfunction in end-stage renal disease" and see "Coronary heart disease in end-stage renal disease (dialysis)" ). Arteriovenous fistulas with high-output HF have also been described in patients with Wilms' tumor [ 22] , renal cell carcinoma [23] , and aortocaval fistula [24,25] . Arterial puncture for cardiac catheterization is an increasingly common iatrogenic cause of arteriovenous fistula. However, high-output failure is rare, with a reported incidence is 0.01 to 0.02 percent of all catheterizations and a clinical presentation ranging from two days to several months postcatheterization [26] . Most of the patients in whom this fistula develops have undergone separate ipsilateral arterial and venous punctures. Surgical repair of the fistula is associated with resolution of the cardiac failure. ( See "Arteriovenous fistulas of the lower extremity"). Other cases of iatrogenic arteriovenous fistulas associated with high-output failure include an ilioiliac arteriovenous fistula after lumbar disk surgery [27] and a patient with high-output failure following transjugular intrahepatic portal-systemic shunting [ 28] . Treatment Surgical therapy is an effective approach to close off or ablate arteriovenous fistulas when this is desired and feasible. In the series of patients with high-output dialysis fistulas described above, surgical correction of the fistula resulted in improvement in HF symptoms in 13 of 14 patients [29] . Transcatheter embolization has been successfully used in some cases of multiple site congenital malformations [30] . Hyperthyroidism Thyroid hormone is a potent regulator of whole body metabolism; an increased circulating level of thyroxine produces an increase in metabolism accompanied by a reduction in systemic vascular resistance and an obligatory increase in cardiac output [ 31-35] . (See "Cardiovascular effects of hyperthyroidism"). Thyroid hormone also increases the intrinsic contractility of heart muscle and myocardial oxygen demands and consumption [ 36] . The heart rate is increased and pulse pressure is widened. These changes can precipitate HF in patients with underlying heart disease which may be caused in part by a hyperthyroid cardiomyopathy [37] . An additional cause of HF in patients with hyperthyroidism is atrial fibrillation with an excessively rapid ventricular response. Atrial fibrillation, which is often paroxysmal, occurs in about 10 percent of hyperthyroid patients. ( See "Causes of atrial fibrillation" ). Hyperthyroidism, even in the absence of high-output failure, is associated with palpitations, due to both tachycardia and more forceful cardiac contraction, and exertional dyspnea, which is due more to respiratory muscle weakness than cardiac failure. (See "Overview of the clinical manifestations of hyperthyroidism in adults"). The physical findings in patients with hyperthyroidism are similar to those in other causes of high-output HF including tachycardia, which is present at rest, during sleep, and during exercise; a hyperdynamic precordium, indicative of the increase in cardiac contractility; systolic hypertension with a widened pulse pressure; brisk carotid and peripheral arterial pulsations; a loud first heart sound; atrial fibrillation; and
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occasionally a "scratchy" midsystolic murmur (Means-Lerman scratch) heard along the left sternal border, resulting from the rubbing together of the normal pleural and pericardial surfaces. ( See "Auscultation of cardiac murmurs" and see "Auscultation of heart sounds"). Other findings on physical examination are associated with hyperthyroidism and include exophthalmos, stare, fine tremor of the outstretched hands, and a firm thyroid gland with or without nodule formation. The skin may be moist and warm as a result of cutaneous vasodilation; blood flow to the skin in hyperthyroid patients can be increased three times or greater than that in euthyroid controls [ 33] . (See "Overview of the clinical manifestations of hyperthyroidism in adults" ). It is important to be aware of apathetic hyperthyroidism, a condition in the elderly population, in which many of the usual manifestations of thyrotoxicosis may be absent [ 38] . Such patients may present with unexplained HF or unexplained atrial fibrillation. Clues to thyrotoxicosis in the elderly include unusual alacrity in the presence of a widened pulse pressure and either atrial fibrillation or unexplained sinus tachycardia. Hyperthyroidism is more likely to lead to HF in elderly patients who often have underlying cardiac disease [39] . Mechanism Enhanced sympathoadrenal activation may be one of the mechanisms by which hyperthyroidism creates a hyperdynamic circulatory state [ 32,40] . In support of this is the finding that the metabolic and circulatory changes in hyperthyroid individuals are similar to those in normal volunteers during epinephrine infusion [ 31] . Furthermore, administration of sympatholytic agents such as reserpine, guanethidine, or beta blockers to hyperthyroid patients can decrease the heart rate, cardiac output, and pulse pressure toward normal [ 32] . However, sympatholytic agents alone do not completely normalize cardiac parameters in hyperthyroid patients. Thyroid hormone possesses strong, positive chronotropic and inotropic effects even in the absence of autonomic innervation. Thus, there may be an independent role for direct thyroid hormone activity. One study demonstrated the positive chronotropic effects of thyroid hormone on the sinoatrial cells in isolated rabbit atria; the rate of diastolic depolarization was increased and the duration of action potentials was decreased [41] . Other animal studies have demonstrated that thyroid hormone also has a direct positive inotropic effect; the enhancement of myocardial contractility in hyperthyroidism may be due to increased accumulation and release of calcium by the sarcoplasmic reticulum during excitation-contraction coupling or by the stimulation of myosin with a higher ATPase activity [ 42] . (See "Excitation-contraction coupling in myocardium" ). Chronic volume overload, hypercontractility, and tachycardia enhance the development of cardiac hypertrophy in hyperthyroid patients [32,39,43] . Direct activation of protein-synthesizing processes by thyroid hormone may also contribute to myocardial hypertrophy. Studies using radionuclide ventriculography have found that hyperthyroid patients have an abnormally low or absent increase in left ventricular ejection fraction response to exercise [37,44] . It is possible that this decrease in cardiac reserve could ultimately progress to impairment of ventricular function and the development of HF even at rest. Treatment The appropriate treatment for HF due to hyperthyroidism is to return the patient to a euthyroid state, usually with an antithyroid drug or radioactive iodine. The restoration of euthyroidism typically leads to normalization of the heart rate, arterial pulse pressure, cardiac output, and left ventricular ejection fraction, and a reduction in left ventricular diameter [ 43] . (See "Treatment of Graves' hyperthyroidism"). Beta blockers may be beneficial by slowing the heart rate, but they should be administered cautiously since there may be a further reduction in myocardial contractility. Beta blockers are particularly helpful in patients with sinus tachycardia or atrial fibrillation due to hyperthyroidism.
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Anemia Anemia, even when severe, rarely causes HF, and, when it does, it is likely that the high-output failure is superimposed upon some cardiac abnormality such as valvular heart disease or underlying left ventricular dysfunction. The loss of hemoglobin is partly compensated by the increase in cardiac output and widening of the arteriovenous O2 difference [ 45] . As an example, in a study of the hemodynamics in anemic patients, right heart catheterization was performed and found that normal cardiac hemodynamics were maintained in patients with hemoglobin values as low as 7 g/dL [46] . Furthermore, when the hemoglobin was less than 7 g/dL, cardiac output increased and HF did not occur. Only in cases of severe anemia, ie, hemoglobin less than 5 g/dL, does HF develop in the absence of underlying heart disease. Chronic anemia may be an important contributor to the development of HG in patients with end-stage renal disease. Although unproven, the correction or prevention of anemia in this setting may help prevent myocardial dysfunction. (See "Myocardial dysfunction in end-stage renal disease" ). Presentation The presentation of patients with anemia depends upon the time course of its development. Slow development of chronic anemia is well tolerated and frequently overlooked for some time [29] . Iron deficiency and the anemia of chronic disease are the most common causes, but a specific etiology must be identified and corrected where possible. ( See "Causes and diagnosis of anemia due to iron deficiency" and see "Anemia of chronic disease (anemia of chronic inflammation)"). Mechanism The mechanism of HF in anemia is not completely understood. Decreased left ventricular afterload secondary to reduced serum viscosity may play a role [ 47] . Additionally, severe anemia can result in left ventricular volume overload and increased stroke volume that can alter left ventricular function [ 48] . As an example, one study obtained serial echocardiograms in 124 patients with sickle cell anemia and found progressive chamber enlargement and increased left ventricular mass [ 49] . Moreover, the left ventricular systolic time interval and the left ventricular pre-ejection period were higher in the sickle cell group compared with same-aged controls, suggesting a decline in left ventricular function with time. Physical examination On physical examination, the skin, mucous membranes, and conjunctival membranes are pale. Arterial pulses are bounding and the pulse pressure is widened. A mild systolic flow murmur is frequently present along the left sternal border and heart sounds, particularly the pulmonic component of the second heart sound, may be prominent. (See "Examination of the arterial pulse"). Treatment The management of HF associated with anemia depends upon the type and severity of the anemia. Diagnostic tests for determining the type of anemia should be carried out immediately. (See "Approach to the adult patient with anemia" ). The treatment for HF due to chronic anemia should be specific for the cause of the anemia, eg, iron, folate, vitamin B12, or erythropoietin in patients with renal failure. If the anemia is more acute or the patient is sicker, more rapid therapeutic measures may be required while the work-up for the cause of the anemia is proceeding. Bedrest and oxygen by nasal cannula are appropriate. Transfusion of packed red blood cells must be done cautiously and slowly to avoid further volume overload and worsening HF. One-half unit should be given over three or four hours, and the patient should be monitored for dyspnea and signs of pulmonary edema. Diuretic therapy, beginning with an intravenous loop diuretic (eg, 20 to 40 mg furosemide) can be used to treat volume overload. Vasodilator therapy has no role because of the marked reduction in systemic vascular resistance. Chronic anemia and HF due to other causes Chronic anemia may play a contributory role in the development of HF. In patients with HF due to other common causes, there is evidence that chronic
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anemia is associated with the progression of HF, but anemia is also associated with a number of common comorbidities. There is conflicting evidence regarding whether anemia is an independent cause of HF progression and worse outcomes, or if it is simply associated with overall disease severity. ( See "Impact of anemia in patients with heart failure" ). Beriberi Beriberi heart disease is due to severe thiamine (vitamin B1) deficiency and is most common in Asia where the diet consists of a high intake of polished rice which is deficient in thiamine. In the United States, thiamine-enriched bread has virtually abolished the disease except in malnourished individuals (eg, alcoholics) or those on fad diets. Beriberi can also develop in patients on prolonged diuretic regimens [50] or in patients receiving enteral nutrition [ 51] . (See "Overview of water-soluble vitamins"). Beriberi disease is classically divided into two types [ 52] : A "dry" form, with neurologic manifestations, including peripheral neuropathies with both sensory and motor components A "wet" form, with cardiovascular involvement Seven diagnostic criteria for classic beriberi heart disease have been proposed [ 53] : Three or more months of thiamine-deficient diet Enlarged heart with normal sinus rhythm Dependent edema Signs of neuritis, pellagra or both Minor electrocardiographic changes such as non-specific ST-T changes No other identifiable cause for heart disease Response to thiamine therapy or autopsy evidence Laboratory diagnosis of thiamine deficiency can be made on the basis of an increase in thiamine pyrophosphate effect (TPPE) or a decrease in blood thiamine concentration, or a decrease in erythrocyte transketolase activity. Mechanism The mechanism for high-output failure in beriberi is multi-factorial. Thiamine deficiency initially presents as a high-output state secondary to vasodilation and an increase in blood volume [54] ; this is followed by eventual depression of myocardial function and the development of a low output state [55] . The high cardiac output is due to reduced systemic vascular resistance and augmented venous return. It is not totally clear what causes the marked reduction in systemic vascular resistance, but it may reflect direct vasomotor depression [54] . It is possible that the development of HF is due to the superimposition of impaired myocardial function. Postmortem gross examination of the heart shows dilation without other changes. Histopathologic study shows nonspecific changes, including interstitial myocardial edema, granulation of cell plasma, and hydropic or fatty degeneration [56] . A biochemical lesion due to thiamine deficiency may contribute to the cardiac failure in beriberi [ 54] . Pyruvate and lactate are important substrates for oxidation and energy production in heart muscle, but thiamine deficiency blocks their utilization; specifically, thiamine pyrophosphate is needed for the decarboxylation of pyruvate and its subsequent oxidation in the citric acid cycle. As a result of thiamine deficiency, pyruvate and its precursor lactate build up in the blood; increased blood lactate level has been
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used in the diagnosis of beriberi, but is nonspecific. Thiamine deficiency also inhibits the function of the hexose monophosphate shunt, contributing to insufficient oxygenation of the tissues. ( See "Overview of water-soluble vitamins"). Symptoms and physical examination Patients in Asia generally present with fatigue, malaise, and edema (wet beriberi) [54] . In the United States, severe malnutrition, peripheral neuropathy, and evidence of a high cardiac output are more common (dry beriberi). Anemia may be present, a result of iron and folate deficiency, and there may be hyperkeratinized skin lesions and painful glossitis. In addition to the characteristic clinical picture, laboratory studies show a reduced thiamine level [57] and increased serum pyruvate and lactate together with a low red cell transketolase [ 58] . Treatment With appropriate treatment, eg, up to 100 mg of thiamine intravenously and 25 mg/day for two weeks, the cardiac index and heart rate are reduced and there is an increase in systemic vascular resistance [59] . Treatment of concomitant nutritional anemia may also be required. In the United States, the association of thiamine deficiency with chronic alcoholism may result in the concomitant presence of alcoholic cardiomyopathy and a depression in left ventricular systolic function. Such patients do not respond well to digoxin, diuretics, or vasodilators; a diagnosis of thiamine deficiency must therefore be considered so that specific therapy with thiamine can be given. The initial loading doses of thiamine are in the range of 100 to 500 mg intravenously, followed by 25 to 100 mg per day orally for at least two weeks. (See "Alcoholic cardiomyopathy"). Patients with furosemide-associated thiamine depletion maintain their ability to absorb thiamine and respond to thiamine repletion with improved left ventricular function and effective diuresis [ 60] . Magnesium may have to be coadministered with thiamine, particularly in magnesium-depleted patients [52,61] . In animal models, magnesium depletion alone leads to a blunted response to thiamine supplementation and loss of thiamine from tissues [ 62] . The mechanism for this relationship is unknown, although it has been proposed that magnesium depletion may interfere with the activation of transketolase [61] . Dermatologic disorders A large increase in cardiac output may be associated with some dermatologic disorders such as active widespread psoriasis, exfoliative dermatitis, or Kaposi's sarcoma [63] . (See "Epidemiology, clinical manifestations, and diagnosis of psoriasis" ). The elevated cardiac output results from substantial cutaneous dilatation and an increase in blood flow to the skin since the widespread lesions are warm and red. As in the other causes of high-output HF, the increase in cardiac output itself rarely causes heart failure; it is more often a contributing factor to an already impaired cardiac reserve resulting from some underlying heart disease. Treatment The treatment is that of the primary skin disorder. As the lesions heal and warmth disappears, there is a concomitant reduction in the elevated cardiac output. Renal disease As noted above, high-output heart failure in patients with chronic renal failure most often results from an arteriovenous dialysis fistula and anemia (which is now less common due to the widespread use of erythropoietin) superimposed on underlying heart disease. (See "Erythropoietin for the anemia of chronic kidney disease among predialysis and peritoneal dialysis patients" and see "Erythropoietin for the anemia of chronic kidney disease in hemodialysis patients" ). Volume expansion due to sodium and water retention also may contribute to the high-output state. In acute postinfectious glomerulonephritis, for example, edema (including pulmonary edema) and hypertension can occur [64,65] . These changes are due primarily to volume expansion as the
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renin-angiotensin system is suppressed and the serum atrial natriuretic peptide concentration markedly elevated [65] . These changes are reversed with fluid removal, eg, with diuretics. Cirrhosis Cirrhosis of increasing severity is progressively associated with systemic vasodilation [66,67] , a high cardiac output [68] , a low blood pressure, and signs of reduced tissue perfusion (such as low urinary sodium excretion and increased plasma levels of the "hypovolemic" hormones renin, norepinephrine, and antidiuretic hormone) [62,68] . (See "Hyponatremia in cirrhosis", section on Vasodilation and hyperdynamic circulation). The increased cardiac output is primarily due to splanchnic vasodilation and the development of intrahepatic or mesenteric arteriovenous shunts. Intrapulmonary arteriovenous shunting is also observed in some patients [69] . The development of cardiac failure is insidious in these patients, but most die from hepatic failure prior to symptomatic heart disease. Treatment of the liver disease, which is often best achieved by hepatic transplantation, may normalize the cardiac output. In one study, the cardiac index decreased by a mean of 35 percent after transplantation [70] . Acromegaly Heart failure is not uncommon in newly diagnosed acromegaly. ( See "Clinical manifestations of acromegaly"). In a review of 102 such patients, 10 had overt heart failure at the time of diagnosis [71] . Compared to those without heart failure, these patients had an increase in left ventricular mass index that was largely due to chamber dilation, a reduction in left ventricular ejection fraction (42 versus 66 percent), and a significant elevation in cardiac index (4.3 versus 3.5 L/min per m2 versus 3.1 L/min per m2 in controls). Skeletal disorders A high cardiac output has been noted in patients with polyostotic fibrous dysplasia (McCune-Albright syndrome), osteitis deformans (Paget's disease) [ 72] , and multiple myeloma [73-75] . (See "Clinical manifestations and diagnosis of Paget's disease of bone" ). It is presumed that there are multiple minute arteriovenous fistulas in the bony lesions [ 74] . Extensive bone involvement (more than 20 percent of the skeleton) is required to increase the cardiac output to the point at which it may contribute to high-output problems [ 73,74] . An additional factor for the increase in cardiac output in Paget's disease may be increased cutaneous blood flow resulting from local heat production by the increased metabolic activity of affected bone. There appears to be a linear relationship between the amount of skeletal involvement and cardiac index in patients with this disease [76] . In one study, a statistically significant increase in cardiac index and heart size was noted in patients with greater than 15 percent skeletal involvement [ 77] . The high-output state is usually well tolerated for years. As with most causes of high-output HF, clinical manifestations are not typically seen in the absence of underlying heart disease. In most patients, treatment of the Paget's disease with a bisphosphonate or calcitonin leads to normalization of the cardiac index within six months [ 76] . Hyperkinetic heart syndrome The hyperkinetic heart syndrome is a poorly defined entity. It has been described in young patients and is associated with sinus tachycardia and elevated systemic blood pressure in the absence of hyperthyroidism or pheochromocytoma [78,79] . The cardiac output is considerably increased and is accompanied by systolic hypertension and a systolic flow murmur [ 78] . This may be a prelude to sustained hypertension later in life [ 79,80] . Symptoms include palpitations, mild hypertension, and chest discomfort; heart failure is not part of the syndrome. The syndrome is probably mediated through excess catecholamines or increased receptor sensitivity to beta agonists since it is responsive to beta blockers. Pregnancy Between 20 and 24 weeks of pregnancy, the resting cardiac output is increased to about 6 liters per minute. Increased blood volume, the presence of the placenta acting as an arteriovenous shunt,
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and increased metabolic demands may contribute to the elevated cardiac output in pregnancy. ( See "Renal and urinary tract physiology in pregnant women" ). Women with an underlying cause for a high cardiac output, such as an arteriovenous fistula, hereditary telangiectasia, or anemia, are more likely to develop high-output heart failure during pregnancy [ 81-86] . In addition, multiple pregnancies in a patient with underlying fistulas may progressively exacerbate vascular malformations and lead to the development of heart failure despite a history of previous uncomplicated pregnancies [ 82] . The treatment of high-output failure in pregnancy depends on the severity of the failure and its etiology. In mild cases, bed rest and a loop diuretic may be effective but often no specific therapy is necessary; the cardiac output generally normalizes between 2 days to 2 weeks post-partum [ 86] . (See "Management of heart failure in pregnancy"). In some cases of arteriovenous malformations, surgical resection of the fistula has been performed during pregnancy with resolution of the failure symptoms [ 81] . In more severe cases, emergent termination of the pregnancy has been required [85] . Miscellaneous causes Other causes of high-output heart failure include polycythemia vera, morbid obesity, cor pulmonale, carcinoid syndrome, and anagrelide used for the treatment of chronic myeloproliferative disorders [87] . Symptomatic therapy for the heart failure and treatment of the underlying disease is indicated. RECOMMENDATIONS Although high-output states are uncommon as a sole cause of heart failure, they may contribute to heart failure in patients with underlying cardiac disease and reduced ventricular reserve. As a result, the clinician must carefully consider the possibility of an associated cause in patients with physical findings that suggest an increase in cardiac output, including warm extremities, wide pulse pressure, bounding pulses, a hyperkinetic heart to palpation, and a systolic flow murmur. These findings may be less striking in patients with overt heart failure. Appropriate laboratory and diagnostic tests help to define the particular cause of the high cardiac output. Therapy is aimed at correcting the cause of the high-output and can sometimes dramatically reverse the heart failure. This approach should be more useful than standard therapy employed in typical low output heart failure (eg, angiotensin converting enzyme inhibitors, beta blockers, diuretics, and digoxin). (See "Overview of the therapy of heart failure due to systolic dysfunction" ). Vasodilators will be of little benefit in a patient who already has an extremely low systemic vascular resistance, while digoxin is of little benefit if the ejection fraction is near normal and the patient is in sinus rhythm; diuretics have a limited role in selected patients.
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REFERENCES
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