Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Congestive Heart Failure by Hiba Tamim, MD

Patients with sickle syndromes most commonly develop heart failure from high output secondary to anemia. Symptoms of dyspnea on exertion, palpitations, and weakness and physical findings of murmurs, third heart sounds, and enlarged heart, which occur because of the anemia, may be mistaken for intrinsic cardiac disease. Older patients and those who receive frequent transfusion may develop cardiac disease which may lead to heart failure.

Present Illness. Note onset of symptoms, presence of edema, dyspnea on exertion, PND, orthopnea, chest pain, cough, fever, or palpitations.

Past Medical History. Document history of murmur, rheumatic fever, hypertension, myocardial infarction, cardiac evaluation, and transfusion history including the total number of units transfused.

Review of Symptoms. Determine present medications, allergies and do a general ROS.

• General. Pallor, degree of distress.
• Vital Signs. Respiratory and pulse rate, blood pressure, temperature.
• HEENT. Exophthalmos, retinal vascular changes.
• Neck. Bruits, thyroid enlargement, venous distension, venous waves, V waves, Kussmaul’s sign.
• Chest. Effusion or basilar rales.
• Cardiac. PMI, RV lift, ventricular filling wave, thrills, S1, S2 (P2 > A2), S3, S4, murmurs, or rubs.
• Abdomen. Hepatomegaly, pulsations, ascites.
• Extremities. Edema, peripheral pulses.

• Minimum Lab. CBC with reticulocyte count, Chest x-ray, ECG, Chemistry pannel, blood gasses.
• Additional lab. Cardiac ECHO, V/Q lung scan, iron studies, ferritin, B12, folate, pregnancy test, or thyroid studies.

• Anemia. Increasing severity of the anemia is the most common cause of CHF in the sickle cell patient. CBC will document the change in hemoglobin. Patients with stable but borderline hemoglobins may have cardiac decompensation with fever, infection, or pneumonia. Reticulocyte count will determine whether there is increased hemolysis or decreased production of erythrocytes.
• Cor Pulmonale. Pulmonary hypertension is seen in patients with sickle syndromes, usually secondary to recurrent obstruction of pulmonary arteries by sickled cells. Findings are of primarily right heart failure including increased neck veins, right ventricular lift, increase P2 and a right sided S3, hepatomegaly, edema, ECG changes of right atrial with or without right ventricular hypertrophy, and lack of rales.
• Myocardial Disease. As patients are living longer, atherosclerotic heart disease and other causes of myocardial dysfunction are more common. Occasionally young patients present with myocardial ischemia or infarction. Findings of diffuse cardiomegaly, ECG changes of damage, poor contractility or hypodynamic areas on ECHO, and thallium exercise scans may be positive.
• Pericardial Disease. Acute or chronic constriction may simulate right or left heart failure. Presence of rub, Kussmaul’s sign, paradox, and ECHO findings are helpful in diagnosis.
• Hypertensive Heart Disease. Although hypertension is rare in patients with sickle cell anemia, it occurs with normal frequency in patients with other sickle syndromes. Older patients present with heart failure secondary to myocardial disease and renal insufficiency from poorly controlled hypertension.
• Hyperthyroidism. Mild hyperthyroidism can cause decompensation because of the high output state from the anemia.
• Hemochromatosis. Transfusion of 500 mg Fe/kg packed red cells may cause myocardial dysfunction. Iron studies, ferritin, echo cardiogram, and liver biopsy with quantification iron establishes the diagnosis of iron overload
• Valvular Heart Disease. Flows murmurs from high cardiac output secondary to anemia are difficult to differentiate from murmurs of valvular heart disease. In addition, many patients carry the diagnosis of rheumatic heart disease because flow murmurs with symptoms from sickle cell disease are mistaken for rheumatic fever in childhood. Insufficiency murmurs are usually related to valvular disease. Some reports suggest that mitral valve prolapse may be more frequent, however, this observation may relate more tothe prevalence of this anomaly in the general population. ECHO may help determine the true etiology of the murmur.

The treatment of choice for heart failure in most hemoglobinopathy patients is transfusion not digitalization and diuretics. Echo cardiograms are very helpful in assessing left ventricular function in these patients.

• Anemia. Careful transfusion with simultaneous diuresis is the treatment of choice for heart failure in the presence of severe anemia. Occasionally it is necessary to do a partial exchange transfusion removing whole blood while simultaneously administering pack red cells. The cause of the increasing anemia (See Anemia). Admission is almost mandatory.
• Cor Pulmonale. Careful diuresis is the usual treatment. Consider chronic portable oxygen therapy for hypoxia and chronic transfusion for anemia.
• Cardiomyopathy Patients with both cardiac disease and anemia will require a higher hematocrit to remain asymptomatic. The patient frequently needs chronic transfusion to maintain a hemoglobin level in the 9 to 10 gm/dl. Some patients can avoid transfusions by treatment with hydroxyurea. Erythropoietin may be helpful is there is relative deficiency. In addition, treatment by digitalization, diuresis, pre-load, and after-load reduction may all be indicated in patients with documented myocardial dysfunction.
• Pericardial Disease. Patients should be admitted for pericardiocentesis to establish a diagnosis and initiate appropriate therapy.
• Hypertension. Treatment of the elevated blood pressure is the treatment of choice.
• Thyroid Disease. Treatment requires admission for careful administration of beta lockers, transfusion, and endocrinology evaluation. Because of the potential for severe aggravation of the sickle syndrome, aggressive treatment to control the hyperthyroid state is indicated. Radioiodine administration allows excellent control in most patients.
• Hemochromatosis. Treatment of heart failure from the cardiomyopathy by high dose intravenous desferoxamine is used acutely. Chronic deferoxamine therapy is used to control the iron overload. The removal of iron may be complicated by increased requirements for transfusion to control the symptoms of heart failure and anemia.(See iron overload and chelation chapter)
• Valvular Heart Disease. Patient with new onset CHF and valvular heart disease should be admitted for evaluation and initiation of therapy. Most patients require transfusion to maintain a higher hemoglobin. Patients should be treated for the valvular heart disease using standard indications for surgery or medical management.

Nursing Considerations:

Assess patients anxiety level. Reassure patient, explain diagnosis and treatment regimen

 Instruct patient about transfusion procedures as necessary

 Instruct patient of necessary lifestyle changes:

-smoking cessation

-decrease alcohol intake

-increased exercise/activity level as tolerated

-low fat/low sodium diet

Instruct patient to space activities as needed

 Instruct patients to have blood pressure checked regularly, stress the importance of compliance with treatment regimen. Encourage patients to take medications as ordered preferably in the morning to avoid sleep interruption with diuretics.

 Explain side-effects of medications. Instruct patients to report immediately any swelling of the ankles, increased weight (> 3-5 lb/week), decreased urine output, increased fatigue, dizziness, yellow or blurred vision, vomiting

 Provide Social Services consultation as needed for home oxygen therapy requirements

? Provide education on etiology of heart murmurs and prompt medical evaluation if symptoms occur.

Patients with heart murmurs probably should be treated with SBE prophylaxis using standard American Heart Association guidelines.

Hypertension should be treated aggressively in the patients with elevated blood pressure. Transfusions should be used very judiciously and for established indications. Iron administration is reserved for patients with documented deficiency. Patients receiving chronic transfusions require regular monitoring for iron overload and initiation of desferoxamine therapy when the liver iron is greater than 6 mg/gm dry weight on a liver biopsy. Patients may need reevaluation to determine the need for treatment with cardiovascular drugs and the true etiology of murmurs.

Once the nature of murmurs are determined it is important to inform the patients and patients of the etiology. If the murmur is flow related, it is important that they know that treatment is not necessary. Patients with pathologic murmurs should be informed about SBE prophylaxis. The importance of avoiding iron and unnecessary transfusions must also be outlined.

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Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000