A 46-year-old woman with a history of complex cong - highsky

[ArchivalUser]

A 46-year-old woman with a history of complex congenital heart disease and cyanosis is evaluated for fatigue and dyspnea on exertion. The patient has had previous palliative surgical intervention but remains cyanotic. She has been managed conservatively for many years, with recent regular phlebotomies.

Physical examination demonstrates central cyanosis and digital clubbing. The apical impulse is laterally displaced. There is a parasternal impulse and brief systolic and diastolic murmurs are noted at the left sternal border. The lungs are clear.

Laboratory testing demonstrates a hemoglobin of 17.5 g/dL (175 g/L) and a hematocrit of 60%. The platelet count is 150,000/μL (150 × 109/L) and the leukocyte count is normal. A blood smear shows a hypochromic, microcytic anemia. The electrocardiogram demonstrates right ventricular hypertrophy with strain, unchanged from the last evaluation. The chest radiograph demonstrates cardiomegaly primarily affecting the right side of the heart and reduced pulmonary vascularity.

What is the most appropriate management of this patient at this point?

A Phlebotomy
B Short-course iron therapy
C Institution of vasodilator therapy
D Heart transplant evaluation

[ArchivalUser]

is it TOF?
Hb is 17.5 and Hct is 60%,and anemia microcytic ?
it can be BB ??

[ArchivalUser]

maybe DD

[ArchivalUser]

a?

[ArchivalUser]

Correct Answer = B)


* Iron deficiency is a common cause for dyspnea and fatigue in patients with cyanotic heart disease.
* The most common cause of iron deficiency in patients with cyanotic heart disease is recurrent phlebotomy.

This patient has features of relative iron deficiency. Her hemoglobin and hematocrit are low for a patient with palliated cyanotic congenital heart disease. A normal hemoglobin for a cyanotic patient is 18“21 g/dL (180“210 g/L), and a normal hematocrit is 61% to 65%. The blood smear also demonstrates a hypochromic, microcytic picture. Further evaluation of the cause for iron deficiency is reasonable, but in this case, the likely cause is recurrent phlebotomy. Short-course iron therapy is indicated while evaluation proceeds. Iron 325 mg daily should be administered for 7 to 10 days and stopped when the hemoglobin and hematocrit begin to increase. Prolonged iron therapy may cause erythrocytosis and increased viscosity.

Cyanotic patients require higher hemoglobin and hematocrit than noncyanotic patients to allow adequate oxygenation of the tissues. Cyanotic patients should avoid iron deficiency, which can cause functional deterioration and is associated with an increased risk of stroke.

Therapeutic phlebotomy may be required for some cyanotic patients but should be reserved for patients with a hemoglobin >22 g/dL (220 g/L) and a hematocrit >65% with symptoms of hyperviscosity (mental slowing, reduced concentration, headaches) and no evidence of iron deficiency or dehydration. Phlebotomy is not indicated for asymptomatic patients. The most common cause of reduced hemoglobin and hematocrit in a cyanotic patient is excessive phlebotomy. The only indication for prophylactic phlebotomy is in the preoperative patient when the hematocrit is higher than 65% to reduce the chances of perioperative bleeding. For symptomatic patients, phlebotomy should be performed with volume replacement. This patient has a relatively low hemoglobin level, and phlebotomy would be contraindicated in this case.

Although vasodilator therapy for patients with severe pulmonary hypertension may be helpful, conservative measures should be considered first. Similarly, transplantation may be considered in the future for this patient if functional decline continues, but hematologic adjustment would be indicated initially.

[ArchivalUser]

bbb

[ArchivalUser]

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