Eisenmenger’s syndrome surgery

Eisenmenger’s syndrome Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Cafer Zorkun, M.D., Ph.D. [3]

Overview

Congenital heart defects should undergo surgical repair before progression to Eisenmenger's syndrome. Once the condition develops into Eisenmenger's syndrome, no surgical cure is available except for heart and lung transplantation. However, palliative interventions may prolong the lifespan and improve the quality of life.

Surgery

• Congenital heart defects should undergo surgical repair before progression to Eisenmenger's syndrome.
• Once the condition develops into Eisenmenger's syndrome, no surgical cure is available except for heart and lung transplantation.

• However, palliative interventions may prolong the lifespan and improve the quality of life.
• For example, creation of an artificial ASD can decompress the high systolic right ventricular pressure in patients with suprasystemic pulmonary artery pressures and impending right ventricular failure.
• Surgical correction of the causative heart defect in adult patients is generally contraindicated. In patients who have developed PAH as a consequence of unrepaired CHD, the defect itself may be acting as a protective measure, preventing worsening of the pulmonary vascular resistance in the face of increasing right ventricular pressure. In children, development of pulmonary vascular disease (greater than 6 Woods units/m2) with poor vasodilator response are likely to face post-surgical complications, including right heart failure, increased pulmonary hypertension, and hypertensive crises.   Classically, it is thought to be of little benefit to undergo such risky procedures with a plethora of potential consequences. Additionally, even with clinical response to vasodilator therapies for the pulmonary arterial hypertension component, there are only a select few case reports that show benefit for the closure of the anatomical defect. However, there is promising evidence that cardiac surgery, along with close peri- and post-operational management of pulmonary vascular resistance with advanced PAH therapies, may improve safety and mitigate downstream problems