PMC9280097_01
Chest X-ray showing mild cardiomegaly of left ventricular contour, right aortic arch, multiple end-on vessels in the medial two-thirds of the right lung, and lack of vascularity in the lateral one-third of the right lung along with oligemia of the left lung. Electrocardiogram showing right-axis deviation, right ventricular hypertrophy, and fails to demonstrate large left ventricular forces
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F1
Chest X-ray showed cardiomegaly with a left ventricular contour and a cardiothoracic ratio of 60%, right aortic arch, dilated main pulmonary artery segment, multiple end-on vessels in the medial two-thirds of the right lung without any plethora in the lateral one-third of the lung field along with relative oligemia of the left lung [Figure 1].
There is no evidence of left ventricular volume overload in apical four-chamber view (a) and subxiphoid short-axis view (b). The mean pulmonary artery pressure predicted from the peak of the pulmonary regurgitation jet on Doppler (c) was 56 mmHg. Long axial view of the aortic arch (d) showing a large right-sided arterial duct
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F2
The mean right pulmonary artery pressure predicted from the peak of a trivial Grade I pulmonary regurgitant jet on Doppler was 56 mmHg [Figure 2].
Suprasternal view (a) demonstrates a LPDA from RAA supplying the isolated LPA). A high parasternal short-axis view (b) with color flow imaging in systole (c) and diastole (d) showing pulmonary trunk (PA) continuing as RPA and a RPDA inserting into the RPA with systolic right-to-left flows and diastolic left-to-right flows. LPDA: Left-sided arterial duct, RAA: Right aortic arch, LPA: Left pulmonary artery, RPA: Right pulmonary artery, RPDA: Right-sided arterial duct
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F3
A small restrictive second arterial duct originating from the base of left innominate artery connected to an isolated left pulmonary artery with continuous flows and showed a peak gradient of 64 mmHg [Figure 3].
Simultaneous recording of both aortic and right pulmonary artery pressures (a) are almost similar with a 6 mmHg difference between the two vessels. After balloon occlusion of the right-sided arterial duct (b), an increase of aortic pressure and fall of right pulmonary artery pressure was accompanied by a relative bradycardia due to Branham effect
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F4
The right pulmonary artery pressures were nearly equal to aortic pressures [Figure 4].
Pulmonary angiogram (a) showing pulmonary trunk (MPA) continuing as a dilated RPA which is dilated in the medial zones and pruned in the lateral zones of right lung. The isolated LPA filled from a left-sided arterial duct (b) arising from the base of LIA. Aortogram (c) showing right aortic arch, a large right-sided tubular arterial duct (RPDA) connecting to the RPA. A complete balloon occlusion of the RPDA (d) is confirmed on an aortogram before assessing the pressures. LPA: Left pulmonary artery, RPA: Right pulmonary artery, LIA: Left innominate artery
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F5
Left arterial duct angiogram demonstrated a good-sized left hilar pulmonary artery [Figure 5].