A 38-year-old Hispanic woman with paradoxical embolism and Libman-Sacks endocarditis involving the tricuspid valve.

Libman-Sacks endocarditis is a well-described clinical entity in patients with systemic lupus erythematosus. Transesophageal echocardiography is the definitive imaging modality used in assessing cardiac valvular involvement in this disease process. Herein we describe a young Hispanic woman with systemic lupus erythematosus and multiple tricuspid valvular vegetations who developed splenic and cerebral infarctions while on optimal anticoagulation therapy with warfarin in the setting of a newly diagnosed patent foramen ovale. A review of the literature on Libman-Sacks endocarditis and patent foramen ovale closure is presented.

The bitter end: the ubiquitin-proteasome system and cardiac dysfunction.

Many elements contribute to congestive heart failure: changes in perfusion, hemodynamic stresses, alterations in calcium metabolism, and dysregulation of cell signaling pathways. Intervention in these processes forms the basis for current heart failure therapies. Nevertheless, heart failure is primarily a disease of wear and tear; despite everything we know about cardiac physiology and the clinical manifestations of heart failure, only in rare instances does therapy for heart failure normalize cardiac function. Proteins are especially prone to the forces of wear and tear in the heart because they are the primary mechanisms for stress sensing and force generation. Recent evidence supports a role for protein damage and impaired clearance of damaged proteins in the pathophysiology of human heart failure syndromes. The process of monitoring and protecting cardiac cells from accumulation of damaged proteins is known as protein quality control, and the molecular chaperone and ubiquitin-proteasome systems are the primary effectors of this process. Insights from protein quality-control strategies may lead to new concepts about prevention and treatment of human heart failure. This review provides a general overview of these pathways and their known and postulated roles in human heart failure syndromes, with a focus on providing a clinically oriented understanding of these fundamental mechanisms.

Clinical predictors and characteristics of patients with chronic liver disease and intrapulmonary shunts.

BACKGROUND: Intrapulmonary shunting (IPS) is a well-described phenomenon in chronic liver disease but its significance is not known. HYPOTHESIS: The study was undertaken to enhance our understanding of the characteristics and prevalence of IPS. METHODS: We retrospectively studied 204 consecutive patients with end-stage chronic liver disease who underwent a dobutamine stress echocardiogram, along with a saline contrast bubble study, as part of their pretransplant evaluation. RESULTS: Intrapulmonary shunting of any degree was present in 56.4% of patients. Patients with IPS were more likely to report alcohol use and less likely to have diabetes mellitus. Patients in Child-Pugh classification C were more likely to have IPS than those with classification A or B. The resting room air PaO2 levels were significantly lower in patients with grade 3-4 IPS than in those with grade 1-2. Elevated estimated pulmonary systolic pressure on echocardiography was more prevalent in patients with than in those without IPS. CONCLUSIONS: A majority of patients with end-stage chronic liver disease undergoing transplant evaluation have IPS. There are important baseline differences between patients with and without IPS. The presence of IPS is associated with increased severity of chronic liver disease. Further study is required to define the prognostic significance of IPS and its impact on future liver transplantation.

Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension.

OBJECTIVES: The aim of this study was to evaluate the relationships between echocardiographic findings and clinical outcomes in patients with severe primary pulmonary hypertension (PPH). BACKGROUND: Primary pulmonary hypertension is associated with abnormalities of right heart structure and function that contribute to the poor prognosis of the disease. Echocardiographic abnormalities associated with PPH have been described, but the prognostic significance of these findings remains poorly characterized. METHODS: Echocardiographic studies, invasive hemodynamic measurements and 6-min walk tests were performed and outcomes prospectively followed in 81 patients with severe PPH. Subjects were participants in a 12-week randomized trial examining the effects of prostacyclin plus conventional therapy compared with conventional therapy alone. RESULTS: During the mean follow-up period of 36.9 +/- 15.4 months, 20 patients died and 21 patients underwent transplantation. Pericardial effusion (p = 0.003) and indexed right atrial area (p = 0.005) were predictors of mortality. Pericardial effusion (p = 0.017), indexed right atrial area (p = 0.012) and the degree of septal shift in diastole (p = 0.004) were predictors of a composite end point of death or transplantation. In multivariable analyses incorporating clinical, hemodynamic and echocardiographic variables, pericardial effusion and an enlarged right atrium remained predictors of adverse outcomes. Six-minute walk results, mixed venous oxygen saturation and initial treatment randomization were also independently associated with a poor prognosis. CONCLUSIONS: Pericardial effusion, right atrial enlargement and septal displacement are echocardiographic abnormalities that reflect the severity of right heart failure and predict adverse outcomes in patients with severe PPH. These characteristics may help identify patients appropriate for more intensive medical therapy or earlier transplantation.