PMC6543216_01
Transthoracic echocardiography showing the shunt flow across the atrial septal defect (ASD) and patent ductus arteriosus (PDA). Panel A shows a parasternal short-axis image of ASD flow (yellow arrow). Color M-mode echocardiogram shows the left-to-right shunt through the ASD. Panel B shows an image of the PDA (yellow arrow) from the supraclavicular view. Color M-mode echocardiogram clearly shows the left-to-right shunt through the PDA. LA: left atrium, RA: right atrium, Ao: aorta, PA: pulmonary artery
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A large PDA and secundum ASD with left-to-right shunts were clearly detected by color Doppler echocardiography (Fig. 1).
Transesophageal echocardiography showing the shunt flow across the atrial septal defect (ASD) and patent ductus arteriosus (PDA). Panel A is a mid-esophageal horizontal view showing two ASDs (yellow arrows). Color M-mode echocardiogram shows a left-to-right dominant bidirectional shunt through the ASD. Panel B is an upper-esophageal aortic arch short-axis view showing the connection (yellow arrow) between the aortic arch (Ao) and main pulmonary artery (PA). Color M-mode echocardiogram clearly demonstrates bidirectional shunt flow through the PDA. LA: left atrium, RA: right atrium
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On transesophageal echocardiography, however, both shunts of PDA and ASD were revealed to be left-to-right dominant with a bidirectional flow (Fig. 2), which was consistent with near-Eisenmenger pathophysiology.
Volume-rendered images of multidetector-row computed tomography are shown. Panel A shows a window-shaped patent ductus arteriosus (PDA) between the anterior surface of the aortic arch (Ao) and left pulmonary artery (PA). Panel B shows a large, oval-shaped secundum atrial septal defect (ASD) and another small secundum ASD. SVC: superior vena cava, IVC: inferior vena cava, RV: right ventricle, RVOT: right ventricular outflow tract
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ASD (30x18 mm) and another small secundum ASD were also confirmed (Fig. 3). Although the shunt flow of both PDA and ASD were bidirectional and the pulmonary artery pressure (PAP) was as high as 100/50 mmHg (mean 79 mmHg), the pulmonary vascular resistance index (PVRi) was borderline for surgical repair, with 6.3 Wood units m2 as measured by right heart catheterization (Table).
The clinical course after surgery is shown. The day after shunt repair, because pulmonary arterial hypertension (PAH) crisis occurred, the patient received PAH-specific drugs, inhaled nitric oxide (NO), and intravenous epoprostenol. After the titration of epoprostenol and oral diuretics, she was discharged on the 30th hospital day. PAH-specific drugs were gradually withdrawn based on the results of a serial right heart catheter examination. At 14 months' follow-up, the mean pulmonary arterial pressure (mPAP) had significantly improved. PVR: pulmonary vascular resistance, VO2: oxygen uptake
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PAH-specific drugs were then gradually withdrawn based on the results of a serial right heart catheter examination (Fig. 4).