Critical Care Medicine-Neurologic Disorders>>>>>Congenital Heart Disease in Adults
Question 4#

A patient with known pulmonary arterial hypertension (PAH) from congenital heart disease has recently been diagnosed with ES.

What is the most appropriate treatment that should be initiated in this patient?

a. Digoxin
b. Beta blocker therapy
c. Calcium channel blocker therapy
d. Bosentan

Correct Answer is D

Comment:

Correct Answer: D

Patients with congenital heart disease are at increased risk and incidence of development of PAH. The mortality remains high among these patients if left untreated. More recently, safe and effective drug therapy (disease targeted therapy) has become available for the treatment of PAH, which includes endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids. Disease-targeted therapies such as sildenafil and bosentan have been associated with improved survival at 10 years as shown in the BREATH-5 Trial. It has been shown that disease-targeted therapies for PAH results in better survival when comparted to traditionally used drugs such as digoxin, beta blockers, and calcium channel blockers. And disease-targeted therapies are the mainstay of treating PAH. Patients with PAH due to ES has a better survival than patient’s with primary PAH. This may be due to the fact that right ventricular function in patients with ES, and resultant PAH is better than those with idiopathic PAH.

References:

  1. Gali N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with eisenmenger’ syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114:48-54.
  2. Beghetti M, Galie N. Eisenmenger’s syndrome. J Am Coll Cardiol. 2009;53(9):733-740. doi:10.1016/j.jacc.2008.11.025.
  3. Diller G-P, Körten MA, Bauer UM, et al. Current therapy and outcome of eisenmenger syndrome: data of the German National Register for Congenital Heart Defects. Eur Heart J. 2016;37(18):1449-1455.