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Romanian Journal of Cardiology
Volumen 31 (2021): Edición 4 (December 2021)
Acceso abierto
Neurofibromatosis 1-Noonan Syndrome Associated with Pulmonary Stenosis and Hypertrophic Cardiomyopathy
Daniela-Noela Radu
Daniela-Noela Radu
,
Monica Dobrovie
Monica Dobrovie
,
Razvan Capsa
Razvan Capsa
y
Roxana Enache
Roxana Enache
| 05 may 2022
Romanian Journal of Cardiology
Volumen 31 (2021): Edición 4 (December 2021)
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Article Category:
Case Presentation
Publicado en línea:
05 may 2022
Páginas:
903 - 910
DOI:
https://doi.org/10.47803/rjc.2020.31.4.903
Palabras clave
neurofibromatosis 1-Noonan syndrome
,
pulmonary stenosis
,
hypertrophic cardiomyopathy
© 2021 Daniela-Noela Radu et al., published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.
Figure 1
ECG: sinus rhythm, normal QRS axis, minor right bundle branch block and inverted T waves in DI, aVL, V2–V6.
Figure 2
Transthoracic echocardiography modified parasternal short axis view. (A) 2D and color Doppler examination: large colour-flow pulmonary regurgitation jet width suggestive for severe pulmonary regurgitation. (B) CW Doppler examination: dense, steep deceleration of pulmonary regurgitation jet CW signal, with a pressure half time of 98 ms, suggestive for severe pulmonary regurgitation; peak antegrade velocity 2.6 m/s, peak antegrade gradient 26 mmHg, suggestive for large residual pulmonary stenosis.
Figure 3
(A) Transthoracic echocardiography, apical RV-focused: normal size RV (B) Transthoracic 2D speckle-tracking echocardiography: slightly reduced RV longitudinal strain.
Figure 4
Transthoracic echocardiography, apical 2-chamber view, 2D examination: left ventricular apical hypertrophy in diastole (A) with systolic obstruction at this level (B).
Figure 5
(A) Transthoracic echocardiography, short-axis view of the apex, 2D examination: left ventricular apical hypertrophy. (B) Transthoracic 2D speckle-tracking echocardiography: paradoxical strain of the left ventricular apex suggestive of hypertrophic cardiomyopathy.
Figure 6
Cine end-diastolic images: Left: short axis at the level of the papillary muscles. Right: four chamber view. Apical left ventricular hypertrophy with a normal sized right ventricle can be observed.
Figure 7
Late gadolinium enhancement images show isolated left ventricular apical fibrosis (arrow). From left to right: three chamber view, four chamber view and two chamber view.
Figure 8
T1 mapping before contrast injection in short axis. Left: level of the papillary muscles. Right: apical level. The stars show the area for which the T1 time is measured: mid septal (non hypertrophied myocardium) 1004 ms with a calculated extracellular volume fraction of 26%, versus apical (hypertrophied myocardium) 1042 ms with a calculated extracellular fraction of 30% - suggestive of interstitial fibrosis in the hypertrophied apical segments.
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