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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
 oraz 
Roxana Enache
Roxana Enache
   | 05 maj 2022
Romanian Journal of Cardiology's Cover Image
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Article Category: Case Presentation
Data publikacji: 05 maj 2022
Zakres stron: 903 - 910
DOI: https://doi.org/10.47803/rjc.2020.31.4.903
Słowa kluczowe
© 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.
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.
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.
(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).
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.
(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.
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.
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.
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.
eISSN:
2734-6382
Język:
Angielski
Częstotliwość wydawania:
4 razy w roku
Dziedziny czasopisma:
Medicine, Clinical Medicine, Internal Medicine, Cardiology, Pediatrics and Juvenile Medicine, Paediatric Cardiology, Surgery, Cardiac Surgery
Kanał RSS czasopisma