DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood. One patient underwent liver and kidney transplant at three yr of age for hereditary polycystic disease, and the other had liver transplant at three months of age due to uncontrollable high-output failure secondary to a large hepatic hemangioma. Both patients developed mild dynamic LV outflow tract obstruction and systemic hypertension soon after transplant, both of which resolved by the time of discharge. Transient LV hypertrophy was observed in both cases. Corticosteroids, cyclosporine A, and tacrolimus were used as initial immunosuppressants. Both patients developed DSS long after the regression of the initial dynamic LV outflow tract obstruction. Herein, we report DSS as an atypical late cardiovascular complication after solid organ transplant and postulate that transient post-transplant hyperdynamic LV outflow tract obstruction and chronic immunosuppressive treatment may lead to subendocardial remodeling resulting in a fibromuscular membranous ridge in susceptible subjects.
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http://dx.doi.org/10.1111/petr.12270 | DOI Listing |
Pulmonary artery (PA) flow analysis is crucial for understanding the progression of pulmonary hypertension (PH). We hypothesized that PA flow characteristics vary according to PH etiology. In this study, we used 4D flow cardiovascular magnetic resonance imaging (CMR) to compare PA flow velocity and wall shear stress (WSS) between patients with pulmonary arterial hypertension (PAH) and those with heart failure with preserved ejection fraction and pulmonary hypertension (PH-HFpEF).
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