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Michalis Koullouros, Tarak A.M. Chouari, Andrew Stewart, Keith Kerr, Dana Dawson, Isolated cardiac desminopathy, European Heart Journal - Cardiovascular Imaging, Volume 18, Issue 7, July 2017, Page 822, https://doi.org/10.1093/ehjci/jex049
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Extract
A 32-year-old woman presented with an 8-week history of increasing dyspnoea and peripheral oedema. Electrocardiogram (ECG) showed junctional bradycardia (54 bpm) with left axis deviation, poor R-wave progression and T-wave inversion in precordial leads (Panel A). The NTpro-BNP was 1900 pg/mL. Echocardiography showed a normal size left ventricle (LV) with good systolic function, a flat interventricular septum, dilated right ventricle (RV) with severe tricuspid regurgitation (TR) and estimated pulmonary artery pressure of 25–30 mmHg, bi-atrial dilatation (PanelsB and C), with an increased transmitral E-wave velocity and steep deceleration time (PanelD). Bubble contrast detected no intra-cardiac shunt (PanelE).
Cardiac catheterization was unremarkable. A pacing wire placed in the right atrium (RA) confirmed electrically silent atria. Invasive studies revealed left and right ventricular end-diastolic pressures (LVEDP = 26 mmHg, RVEDP = 27 mmHg) equalization, with a typical dip-and-rise to plateau sign of the RV diastolic pressure seen in constrictive and restrictive physiology (Panel F). Echocardiography did not show any variation in the trans-mitral flows (Panel G) and cardiac magnetic resonance demonstrated a normal pericardium (PanelH). Indeed, pulmonary capillary wedge pressure (PCWP) was 24 mmHg and pulmonary vascular resistance was 2.8 WU, demonstrating predominantly pulmonary venous hypertension.
