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Introduction As survival rates of patients born with univentricular heart defects and palliated by the Fontan operation increase, Fontan failure is more frequently encountered in the adult years. The evidence for use of serological markers, including the brain natriuretic peptide (BNP), at the time of acute Fontan failure exacerbations has been limited in this patient population. Hypothesis We hypothesize that among patients with a Fontan circulation admitted for decompensation, serological markers, particularly BNP, will have a different profile when compared to the general heart failure population. Methods This was a single center retrospective study of Fontan patients who were admitted for acute decompensated Fontan failure. The patients underwent a 1:2 propensity match for age, gender and renal function with non-Fontan patients for each hospital encounter. Admission laboratory values and echocardiographic findings were collected and verified. Student's t-test and chi-square test were used for comparison between the two patient groups. A logistic regression model assessed association of patients’ variables with a length of stay greater than 3 days. Results Our study cohort consisted of 54 encounters for 20 Fontan patients with median age of 34 years and 108 encounters for 108 non-Fontan patients with a median age of 35 years. BNP levels were significantly lower in Fontan patients admitted for decompensation (p Conclusion Compared to the general heart failure population with structurally normal hearts, the BNP values in Fontan patients admitted due to decompensation are lower on admission with a smaller change from the last known outpatient value. Alternative laboratory studies profile should likely be used for risk stratification in this patient population.

Pulmonary hypertension, defined as mean pulmonary artery pressure ≥25 mmHg at rest as assessed by right heart catheterization, may be the end result of primary pathology of the pulmonary vasculature or of various other, systemic processes that affect the pulmonary vessels in either directly or indirectly. Pulmonary hypertension causes diverse effects on many other organs, the most important being the heart and specifically its right-sided chambers. Non-invasive and invasive methods to assess the severity of disease and the magnitude of cardiac effects have been extensively studied and many are now applied in clinical practice. This chapter will review in detail the evaluation of patients with pulmonary hypertension, including the methods used in the physical examination, echocardiogram and right heart catheterization, as well as guidance in interpretation of these results. Methods to evaluate the effects of the disease on the heart, to distinguish pulmonary hypertension caused by pulmonary vascular pathology from that caused by left heart disease, provocative maneuvers used to unveil pulmonary hypertension caused by left heart disease and pulmonary vasoreactivity testing are also discussed. Finally, we present the challenges of the diagnosis and treatment of special populations with pulmonary hypertension, such as adults with congenital heart disease.

Hemodynamic assessment of the patient with pulmonary hypertension begins with a careful physical exam and is followed by several noninvasive tests and ultimately right heart catheterization. Proper interpretation of the considerable data that is generated by this process requires a firm working knowledge of the pulmonary circulation and right ventricle along with major factors that can affect pulmonary vascular tone and cardiac output. This chapter reviews normal cardiopulmonary physiology and provides an in depth approach to assessing pulmonary hemodynamics in patients with pulmonary vascular disease. A detailed discussion of the physical exam and techniques used for transthoracic echocardiogram and right heart catheterization are provided along with a guide to interpreting their results. The skills and diagnostic approaches presented are necessary for the proper diagnosis of pulmonary vascular disease and should be used to distinguish patients with pulmonary arterial hypertension from patients with pulmonary hypertension associated with left heart failure or chronic thromboembolic pulmonary hypertension.

Background: Cryptogenic stroke is a dreadful outcome for patients with patent foramen ovales (PFO's). Higher incidences of cryptogenic strokes are reported in patients with PFO's and inherited thrombophilias (ITs) such as the Prothrombin and Factor V Leiden mutations. Since carriers of IT's are

Heart failure with preserved ejection fraction can lead to pulmonary hypertension. The aim of the current study was to evaluate the diagnostic role of exercise right heart catheterization in the unmasking of diastolic dysfunction. Between 2004 and 2012 164 symptomatic patients with exertional