Nitric OxidePulmonary 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 -

Exclusion Criteria:

1. Presence of fenestration - the shunts include veno-veno collaterals
2. Significant anemia defined as Hgb < 120 mg/dl
3. Pregnancy at the moment of the procedure.
4. Taking angiotensin-converting enzyme inhibitors or angiotensin receptor blockers.
5. Usage of nitrates, phosphodiesterase inhibitors or calcium blockers within 3 months of the study.

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