Bicuspid aortic valve aortopathies: An hemodynamics characterization in dilated aortas.

Bicuspid aortic valve (BAV) aortopathy remains of difficult clinical management due to its heterogeneity and further assessment of related aortic hemodynamics is necessary. The aim of this study was to assess systolic hemodynamic indexes and wall stresses in patients with diverse BAV phenotypes and dilated ascending aortas. The aortic geometry was reconstructed from patient-specific images while the aortic valve was generated based on patient-specific measurements. Physiologic material properties and boundary conditions were applied and fully coupled fluid-structure interaction (FSI) analysis were conducted. Our dilated aortic models were characterized by the presence of abnormal hemodynamics with elevated degrees of flow skewness and eccentricity, regardless of BAV morphotype. Retrograde flow was also present. Both features, predicted by flow angle and flow reversal ratios, were consistently higher than those reported for non-dilated aortas. Right-handed helical flow was present, as well as elevated wall shear stress (WSS) on the outer ascending aortic wall. Our results suggest that the abnormal flow associated with BAV may play a role in aortic enlargement and progress it further on already dilated aortas.

Uhl's disease: An uncommon presentation of a rare disease.

Uhl's disease, also known as Uhl anomaly, is a rare disease secondary to selective but uncontrolled apoptosis of right ventricular myocytes during the perinatal period, after complete cardiac development, leading to the absence of right ventricular myocardium and the direct apposition of endocardium to epicardium without a myocardial layer in between, resulting in right ventricular failure. The present paper describes a case of Uhl's disease with an uncommon presentation. A 28-year-old man was admitted with dyspnea and cyanosis. Transthoracic echocardiography showed severe dilation of the right chambers, impaired right ventricular systolic function and a large ostium secundum atrial septal defect (ASD). Cardiac catheterization revealed pulmonary hypertension, with increased pulmonary capillary wedge pressure (mean 19mmHg) and Qp:QS 0.88:1. At this point, the authors considered that a main diagnosis of ASD could not explain the clinical features and hemodynamic data. A primary disease of the right ventricle was the most likely hypothesis and cardiac magnetic resonance imaging was performed, which demonstrated an extremely thin-walled right ventricle, with almost complete absence of right ventricular free wall myocardium, compatible with Uhl's disease.

Eisenmenger syndrome in the adult--experience with new drugs for the treatment of pulmonary hypertension.

BACKGROUND: The last few years have seen significant progress in the treatment of advanced pulmonary arterial hypertension (PAH). The efficacy of new drugs has been proved mainly in idiopathic PAH or PAH associated with connective tissue diseases. As the pathophysiologic patterns are similar, it is reasonable to use these drugs also in Eisenmenger syndrome or in other congenital heart defects with PAH related to initial high pulmonary flow. OBJECTIVES: To evaluate our early experience with new drugs for PAH in patients with Eisenmenger syndrome. METHODS: A retrospective study of five patients, aged 28 to 51 years (39.6 +/- 9.94), four female, with Eisenmenger syndrome due to atrial septal defect (n = 2), patent ductus arteriosus (n = 2) or ventricular septal defect (n = 1), who began therapy with iloprost (n = 4, later associated with sildenafil in one patient) and bosentan (n = 1), between April 2001 and May 2003. The existence of severe and fixed PAH, with predominant right-to-left shunt, was confirmed by hemodynamic study in all cases. The patients were evaluated by clinical examination, Doppler echocardiography and the six-minute walk test before treatment and throughout follow-up (9 to 34 months, 19.8 +/- 9.04). Before treatment two patients were in NYHA class III and three in class III with periods in class IV. By Doppler echocardiography the right ventricle-right atrium (RV-RA) gradient was 74 to 111 mmHg (90.6 +/- 15.73) and the Tei index was 0.53 to 2.05 (1.13 +/- 0.62). In the six-minute walk test the distance was 214 to 500 meters (296.8 +/- 115.27). RESULTS: All patients improved clinically, though three are still in class III. One patient is in class II and one patient died. At the latest evaluation the RV-RA gradient was 60 to 112 mmHg (84.8 +/- 19.11) and the Tei index was 0.5 to 1.33 (0.85 +/- 80.32). In the six-minute walk test a net increase in the distance covered was evident: 376 to 520 meters (420 +/- 57.89). The treatment was well tolerated in all cases, without serious adverse effects. CONCLUSIONS: Though the number of patients was small, our initial experience with the new specific drugs for PAH in Eisenmenger syndrome showed promising results, with clinical and functional improvement and without adverse effects.