Ann Thorac Surg 2003;75:273-275
© 2003 The Society of Thoracic Surgeons
Case report
Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease
Tooru Araki, MDa*,
Masahiro Kamada, MDa,
Yoshio Okamoto, MDa,
Sadahiko Arai, MDb,
Osamu Oba, MDb
a Department of Pediatrics, Hiroshima City Hospital, Hiroshima, Japan
b Department of Cardiovascular Surgery, Hiroshima City Hospital, Hiroshima, Japan
Accepted for publication June 10, 2002.
* Address reprint requests to Dr Araki, Department of Pediatrics, Fukuyama National Hospital, 4-14-17 Okinogamichou, Fukuyama, Hiroshima 720-8520, Japan.
e-mail: arakit{at}fukuyama-hosp.go.jp
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Abstract
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We present the case of 52-day-old girl with a common atrioventricular canal. Severe liver dysfunction persisted following complete repair of the cardiac defect. A patent ductus venosus appeared to be the source of the hemodynamic disturbance responsible for hepatic dysfunction. Given her critical condition, coil embolization of the ductus venosus was performed, after which the patient improved rapidly. The ductus venosus should be tested for patency when liver dysfunction persists after the corrective cardiac surgery, and coil embolization is the treatment of choice in gravely ill children.
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Introduction
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The ductus venosus (DV)? involutes rapidly after birth in term neonates, usually before the 18th day [1]. We report the case of a 52-day-old girl with Down’s syndrome and severe liver dysfunction who underwent coil embolization of a patent DV following complete repair of common atrioventricular canal (CAVC).
The patient was delivered at 39 weeks of gestation by cesarean section to a 37-year-old mother. Hydrops fetalis and cardiomegaly, detected by sonography, were the indications for surgery. Her birth weight was 3,420 g, and multiple anomalies characteristic of Down’s syndrome were observed. A diagnostic echocardiogram demonstrated CAVC, type C, massive common atrioventricular valve regurgitation (CAVVR), patent ductus arteriosus (PDA), and a patent DV. Although intensive medical treatment was started, the patient’s condition worsened, and she went into shock on day 6. Her serum transaminase concentrations were markedly elevated: aspartate aminotransferase, 9,202 IU/mL; and alanine aminotransferase, 606 IU/mL. Repeat echocardiogram showed massive CAVVR, left-to-right shunting through the PDA, and a peak flow velocity in the ascending aorta of 0.4 m/sec with a short ejection time. These findings suggested low output syndrome. Therefore, PDA ligation and two-patch repair of the CAVC were performed on days 6 and 30, respectively. Cardiac output improved, as did the urinary output. However, liver dysfunction with hyperbilirubinemia (total serum bilirubin concentration greater than 15 mg/dL) and somnolence persisted, and the patient’s operative incision did not heal. We performed nine exchange transfusions, which did not significantly improve the patient’s clinical condition, although liver function and level of activity did improve transiently. Serologic evaluation ruled out hepatitis B, hepatitis C, and cytomegalovirus infection.
An echocardiogram showed a patent DV which appeared to contribute to the persistent liver dysfunction. Coil embolization of the DV was performed on the 52nd day of life. At the time of embolization, the patient weighed 3,289 g. A 5-French sheath was introduced into the right internal jugular vein, and a 5-French catheter was used for measuring the intravenous pressures and obtaining angiograms of the portal vein (PV) and the DV. The mean pressure in the PV was 13 mm Hg, and that in the right atrium was 12 mm Hg. Although most of the contrast medium ran off into the inferior vena cava through the DV, a portal venogram showed narrowing of the peripheral PV and hepatic veins (Fig 1).
The DV measured 3.5 mm in diameter without narrowing. We used an 8-cm long, 0.038-inch detachable coil with an 8 mm outer diameter (Detachable coil and delivery system for closure of PDA; William Cook Europe A/S, Bjaeverskov, Denmark). The coil was advanced into the DV under ultrasonic guidance and was anchored without projecting into the PV or inferior vena cava (Fig 2).
The wedged hepatic vein pressure was 18 mmHg, and ultrasound including color Doppler sonography demonstrated complete occlusion of the DV and almost normal-sized intrahepatic portal veins. The patient’s condition was satisfactory after the procedure, and there were no complications. Serum transaminase and total bilirubin concentrations gradually returned to normal levels, and the operative incision healed.
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Fig 1. Anteroposterior projection of portography before embolization. The ductus venosus is clearly patent (black arrow); the portal vein is well visualized (white arrows).
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Fig 2. Anteroposterior projection of an inferior vena cavagram after embolization of the ductus venosus. The final position of the coil in the ductus venosus (black arrow) occludes this structure but does not project into the inferior vena cava (white arrow).
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Comment
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Surgical management of a patent DV is required in some symptomatic patients [2, 3]. To our knowledge, only one patient has been treated by coil embolization [4]. We report a rare case of a patient with congenital heart disease whose DV was embolized with a coil.
Liver damage was originally caused by circulatory collapse which was attributed to a large PDA and massive CAVVR. Liver damage did not improve and actually deteriorated after the open heart surgery. Therefore, we considered that patent DV inhibited restoring the liver function, including protein synthesis and bilirubin conjugation. Coil embolization of DV restored sufficient portal flow which subsequently achieved hepatocellular recovery. The gravity of the patient’s illness led us to choose radiologic intervention over surgery to minimize iatrogenic stress. Prior to embolization, we could have tried the balloon occlusion test in the DV and compared the PV pressure prior to and during occlusion [2, 4]. However, we believed that the smooth flow of contrast medium from the PV to the hepatic veins excluded the likelihood of PV atresia and severe PV stenosis.
Our experience raises two important points: 1) the DV should be tested for patency when liver dysfunction persists after the corrective cardiac surgery, and 2) coil embolization of the DV rather than surgical ligation is the treatment of choice in gravely ill children.
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References
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- Fugelseth D., Lindemann R., Liestol K., et al. Ultrasonographic study of ductus venosus in healthy neonates. Arch Dis Child 1997;77:131-134.[Abstract/Free Full Text]
- Uchino T., Endo F., Ikeda S., et al. Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus. Gastroenterology 1996;110:1964-1968.[Medline]
- Barsky M.F., Rankin R.N., Wall W.J., et al. Patent ductus venosus, problems in assessment and management. Can J Surg 1989;32:271-275.[Medline]
- Schwartz Y.M., Berkowitz D., Lorber A. Transvenous coil embolization of a patent ductus venosus in a 2-month-old child. Pediatrics 1999;103:1045-1047.[Free Full Text]
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Abstract
Introduction
