PMC6120336_01
PMC6120336_02
Electrocardiography and the chest X-ray. (a) Electrocardiogram shows normal sinus rhythm at 75 beats/min with a prolonged PR interval; negative T in leads II, III, and aVF; and ST depression in V1 and V4-6. (b) Chest radiograph reveals a cardiothoracic ratio of 66%, right-sided aortic arch, and dilated pulmonary arteries.
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fig1
A chest radiograph revealed a cardiothoracic ratio of 66%, right-sided aortic arch, and prominently dilated PA without pulmonary congestion (Figure 1(a)). The electrocardiogram showed sinus rhythm with a prolonged PR interval and ST-T segment abnormalities (Figure 1(b)).
Transthoracic echocardiogram and cardiac magnetic resonance imaging (CMRI). (a) Parasternal long-axis view shows the ventricular septal defect (VSD) (white arrow) and overriding of the aorta. (b) Color Doppler image in the long-axis view shows two shunts through the VSD (white arrow). (c) Apical four-chamber view shows the hypertrophic right ventricle. (d) There is an RV outflow obstruction with a peak velocity of 4.9 m/s. (e) Analysis of CMRI reveals a normal RV end-diastolic volume (76 ml), reduced RV ejection fraction of 28%, and preserved left ventricular ejection fraction of 60%. (f) CMRI scan shows subvalvular pulmonary stenosis (yellow arrow).
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The echocardiogram showed a large ventricular septal defect with a bidirectional, mainly left-to-right shunt, overriding the aorta, right ventricular (RV) hypertrophy, and a stenotic RV outflow tract with a peak velocity of 4.9 m/s (Figure 2). The cardiac magnetic resonance imaging scan showed reduced RV ejection fraction (EF) of 28% and preserved LVEF of 60% with a LV end-diastolic volume index of 103 ml/m2 (Figures 2(e) and 2(f)).
Catheter angiography. (a) Right ventriculogram shows subvalvular pulmonary stenosis (black arrow). (b) Aortogram shows a right-sided aortic arch and patent Blalock-Taussig shunt. There is stenosis at the ostium of the left pulmonary artery (yellow arrow) and both pulmonary artery aneurysms. Ao: Aorta; BT shunt; Blalock-Taussig shunt; lt.PA: left pulmonary artery; rt.PA: right pulmonary artery; RV: right ventricle.
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fig3
The catheter angiogram revealed a patent BTS and severe stenosis at the ostium of the left PA (Figure 3).