Signs and symptoms of irreversible pulmonary arterial hypertension secondary to an initial congenital heart disease with left-right shunt (VSD, ASD, ductus arteriosus, atrioventricular canal, aortopulmonary window) that has not been corrected: the significant and prolonged increase in pulmonary blood flow leads to pulmonary arterial vasculopathy with irreversible thickening and rigidity of the vascular walls, and therefore increased lung resistance (> 10 UWood). This irreversible arterial hypertension appears more quickly in case of trisomy 21 (associated pulmonary capillary abnormalities) or genetic predisposition to pulmonary arterial hypertension.

Clinical signs: fatigue, cyanosis, chest pain, digital clubbing; hemoptysis and right heart failure (edema, hepatomegaly) are late. Risk of paradoxical cerebral embolism, endocarditis and thrombosis of pulmonary vessels.

Auscultation: strong B2 heart sound sometimes accompanied by a click, sometimes murmur of pulmonary regurgitation

Paraclinical examinations: polycythemia, RVH on ECG, atrial and/or ventricular arrhythmias

Associated pathologies: polycythemia, iron deficiency, hyperuricemia, coagulation disorders (hyperviscosity), stroke, brain abscess

Medical treatment of pulmonary arterial hypertension: bosentan, sildenafil, iloprost and/or epoprostenol.

Curative treatment: heart-lung transplant.

Anesthetic implications:

high-risk anesthesia; preoperative echocardiography;check the preoperative values of the patient's usual systemic systolic blood pressure because there is a risk of ischemia of the RV  in case of systemic hypotension;  keep peripheral resistances high to minimize the importance of the right-left shunt. Pulmonary resistance is theoretically fixed and irreversible but it seems that general anesthesia, the use of NO and especially  anincrease in FiO2, and decrease in PaCO2 with controlled ventilation, could lead to a moderate improvement in oxygenation.  Prevention of endocarditis. Thromboprophylaxis.

References : 

• Lyons B, Motherway C, Casey W, Doherty P. 
  The anaesthetic management of the child with Eisenmenger’s syndrome. 
  Can J Anaesth 1995; 42:904-9.

• Beghetti M, Galiè N. 
  Eisenmenger syndrome: a clinical perspective in a new therapeutic era for pulmonary arterial hypertension. 
  J Am Coll Cardiol 2009; 53: 733-40 
• Seal R.
  Adult congenital heart disease.
  Pediatr Anesth 2011; 21:615-22
• Lilot M, Tafer N, Mauriat P.
  Cardiopathies congénitales opérées : quelles spécificités anesthésiques ?
  Congrès SFAR 2015
• Saito K, Toyama H, Ejima Y, Yamauchi M.
  Preoperative assessment of the impact of positive pressure ventilation with noninvasive positive pressure ventilation in a patient with Eisenmenger syndrome : a case study.
  A&A Case Reports 2016 ; 7 : 193-5.

Updated: January 2023