Update on the clinical utility of sildenafil in the treatment of pulmonary arterial hypertension

Sildenafil is an orally administered phosphodiesterase type 5 inhibitor that is approved for the treatment of pulmonary arterial hypertension (PAH). The hemodynamic effects of sildenafil are mitigated primarily via potentiating the effects of endogenous nitric oxide, leading to smooth muscle cell relaxation and reductions in pulmonary arterial pressures and pulmonary vascular resistance. When added to standard background therapy in patients with idiopathic or associated PAH from congenital heart disease, anorexigen use, or connective tissue disease, sildenafil treatment results in improved exercise capacity as measured by 6 minute walk distance, improved hemodynamics, and favorable changes in quality of life. Sildenafil use is contraindicated with concomitant nitrate administration, and caution should be exercised when used in combination with antihypertensive agents due to risks of precipitating hypotension. Side effects are generally mild, and include flushing, headaches, and epistaxis. The combination of sildenafil with intravenous epoprostenol is safe and well tolerated, and further improves exercise capacity. Sildenafil is approved only for treatment of PAH, and although emerging data suggest a potential role in treating other types of pulmonary hypertension, larger trials are required to confirm these findings

Anomalous left-sided superior vena cava with cephalad flow

The case of a 24-year-old male with complaints of migraine headaches was referred for echocardiography. The rest of medical history was unremarkable. Agitated saline contrast bubble study showed evidence of a right to left intracardiac shunt, probably secondary to a patent foramen ovale. Results of a transesophageal echocardiogram suggested the possibility of an anomalous venous circulation and eventually identified as anomalous left-sided superior vena cava with cardiac magnetic resonance imaging

Aortic Root Abscess with Aorta to Right Atrium Fistula due to Vancomycin-Intermediate Staphylococcus aureus (VISA)

We present a case of root abscess with aorta to right atrium fistula due to vancomycin-intermediate Staphylococcus aureus (VISA) after limb amputation and cardiac surgery. Patient underwent redo aortic valve replacement, patch repair of aorta to right atrial fistula, and tricuspid valve repair with a ring. Fistula formation is a rare complication of prosthetic valve endocarditis (PVE). This is the first case to discuss aortocavitary fistula (ACF) formation due to VISA. Transesophageal echocardiogram (TEE) is the preferred imaging modality to diagnose ACF

Management of Pulmonary Artery Aneurysms in Pulmonary Hypertension: A Single-Center Review of 3 Cases

Pulmonary artery aneurysms (PAAs) are defined as having pulmonary artery diameter of greater than 40 mm. PAAs are rare and can occur in various pulmonary diseases. There are no clear-cut guidelines regarding the management of PAAs, and recommendations for management are made based on expert consensus opinion, case reports, and institutional experience. This series highlights three patients with pulmonary hypertension (PH) and PAA. The clinical course and diagnostic findings and the decision-making involved in the treatment are reviewed. An overview of three distinct management strategies including medical management, heart/lung transplant, and surgical aneurysm repair is presented

Diagnosis and management of chronic thromboembolic pulmonary hypertension (CTEPH) in sickle cell disease: A review

Abstract Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease