A 52 year old lady with unrepaired congenital heart disease and chronic cyanosis presents with new onset fatigue and exertional dyspnea. She has a ventricular septal defect with Eisenmenger syndrome. The remainder of her history is significant for menorrhagia.
On examination, her BP is 121/68, HR 72 bpm and regular, JVP has prominent a wave, nondisplaced PMI, with a +2 parasternal pulse. She has an accentuated pulmonic S2; no gallops/canters are appreciated. There is a soft murmur at the LSB and an ejection click a the 2nd LIS. There is no diastolic murmur. Her carotid exam is normal. Cyanosis and digital clubbing is present.
Her lab results are significant for a hematocrit of 48%, hemoglobin 13.5 g/dL, sCr 1.3 mg/dL, and a normal fasting plasma glucose.
Her EKG demonstrates right axis deviation and RVH. The CXR reveals prominent central pulmonary arteries and decreased vascularity in the lung fields.
Which treatment is most appropriate at this time?
A) Low-dose iron therapyB) Observation and follow-up
C) Blood transfusion
D) Pulmonary vasodilator therapy
C: Blood transfusion
I will go with D. Pulmonary vasodilator therapy.
if I had an option of Heart-lung transplant I would choose that.
D:Pulmonary vasodilation. Left to right shunting, increase volume, leading to pulmonary hypertension...
D.
D. Pulmonary vasodilation will decrease the pulm art. resistance and increase the amount of blood blood going through the lungs and gets oxygenated while decreasing the amount of blood going through the right to left shunt.
i would also order a ferritin level to make sure the pt had adequate iron stores because an iron deficient anemia (with hx of menorrhagia) would worsen symptoms of cyanosis. and if low i would add low dose iron therapy.
also i like alifeya's lung/heart transplant but lets also add on a kidney and brain transplant just for fun.
C. This pt has a ventricular septal defect and eisenmenger's with CHRONIC cyanosis. The reason why she has an acute episode of fatigue and exertional dyspnea is due to the new onset of menorrhagia. Her baseline hemoglobin is likely higher due to the chronic cyanosis and a Hgb of 13.5 is low for this pt. The most appropriate treatment at THIS time is blood transfusion. Also, find the etiology of the menorrhagia. Down the road consider surgery to correct the ventricular septal defect.
Pt has already developed Eissenmenger's physiology, its too late to correct VSD now.