Pulmonary Capillary Recruitment in Fontan Patients

Inclusion Criteria

• •Fontan failures will be defined as patients with single ventricle physiology and TCPC with clinical signs of dysfunction defined as any of the following:
• •1. Decreased functional class WHO/NYHA II or more and clinical signs of congestion (ascites, peripheral edema, increased JVP) and/or NT-proBNP \> 399 pg/ml based on \[17\].
• •2. Age \> 18 years
• •3. Known Fontan's increased pressures a. Fontan mean pressure of ≥15 mmHg and/or transpulmonary gradient ≥ 5 mmHg. b. Pulmonary vascular resistance ≥ 2 woods units. And/or supporting evidence of end-organ damage
• •1\. Clinical or paraclinical evidence of liver congestion and/or fibrosis
• •a. Evidence in ultrasound or other imaging techniques of liver congestion and/or changes related to cardiac cirrhosis.
• •b. Fibroscan with grade 2 or + 2. Paraclinical signs of portal hypertension defined as 2 or more:
• •1. Thrombocytopenia defined as platelet count \< 150.0000
• •2. Leukocytopenia defined as white blood cell less than 4.500
• •3. Esophageal varices on esophagoduodenoscopy.
• •4. Splenomegaly on imaging -