Mol Genet Genomic Med
November 2024
: Pulmonary arterial hypertension (PAH) is a devastating, life-threatening disease with poor prognosis when left untreated. The long-term prognosis is definitely influenced by the natural progression of PAH but late disease-specific complications may also contribute. : We present a patient with a long-standing idiopathic PAH in whom progressive dilatation of pulmonary trunk and pulmonary arteries leads to compression of the left main coronary artery and the left atrium with hemodynamic compromise.
View Article and Find Full Text PDFBackground: Aortic root dilation, dissection and rupture are major clinical problems in Marfan syndrome (MFS). Although β-blockers remain the standard of preventive treatment, preliminary results from animal studies and a selected group of severely affected MFS children show significant benefit from treatment with losartan, an angiotensin II receptor blocker with TGF-β inhibiting potential. Large-scale human trials are now needed to confirm these results.
View Article and Find Full Text PDFAn 18-year-old male patient, known with familial hypertrophic obstructive cardiomyopathy underwent a septal myectomy 10 years ago for significant left ventricular outflow tract (LVOT) obstruction. During follow-up a progressive increase in LVOT gradient was noted in association with severe mitral valve regurgitation. The patient underwent percutaneous alcohol septal ablation to induce regression of left ventricular hypertrophy.
View Article and Find Full Text PDFObjectives: This study was designed to evaluate the value of 64-slice computed tomography (CT) to visualize the cardiac veins and evaluate the relation between variations in venous anatomy and history of infarction.
Background: Cardiac resynchronization therapy (CRT) is an attractive treatment for selected heart failure patients. Knowledge of venous anatomy may help in identifying candidates for successful left ventricular lead implantation.
Purpose: The prevalence of most minor cardiovascular manifestations in Marfan syndrome (MFS) is unknown. We assessed the prevalence of minor cardiovascular manifestations in MFS to evaluate their usefulness in a diagnostic setting.
Methods: Seventy-seven patients with MFS (aged 4 months to 55 years) underwent echocardiography to assess the presence of mitral valve prolapse and the diameter of the main pulmonary artery.
Aims: Knowledge of the complex three-dimensional anatomy of the right atrium is mandatory for the electrophysiologist and interventional cardiologist, but its understanding remains difficult. We hypothesized that the left hand, loosely clenched, is a good three-dimensional model to understand the position of the different anatomical and electrical regions in the right atrium. For validation, we compared the hand with an endocast that had been prepared from an adult human right atrium and with a three-dimensional electro-anatomical CT image of the right atrium.
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