Transjugular Ductus Venosus Stent Implantation in an Extremely Premature, 1 kg Neonate, With Infradiaphragmatic Total Anomalous Pulmonary Venous Connection
Julian E Cameron, MD, Children's Hospital Los Angeles, Los Angeles, CA
Julian E Cameron, MD1, Neil D Patel, M.D., FSCAI1, Patrick Morris Sullivan, M.D., FSCAI1, Cheryl M. Takao, M.D.1 and Sarah Badran, M.D., FSCAI2, (1)Children's Hospital Los Angeles, Los Angeles, CA, (2)Helen DeVos Children's Hospital, Ada, MI
Title:
Transjugular ductus venosus stent implantation in an extremely premature, 1 kg neonate, with infradiaphragmatic total anomalous pulmonary venous connection
Introduction
:
Infradiaphragmatic total anomalous pulmonary venous connection (TAPVC) typically requires prompt surgical intervention secondary to the natural obstruction in the ductus venosus (DV) postnatally. In select cases requiring pre-surgical stabilization, palliative stenting of the DV has been performed. We present a case of successful transjugular DV stent implantation in an extremely premature, low-birth weight neonate with postnatally diagnosed infradiaphragmatic TAPVC, with a particular emphasis on portal sinus anatomy.
Clinical Case
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The patient was born at 26 6/7 weeks gestation with a birth weight of 935 grams. Echocardiogram on day of life 9 demonstrated infradiaphragmatic TAPVC. Due to an increase in gradient across the DV by echocardiogram, progressive pulmonary edema, and hypotension, cardiac catheterization with DV stent implantation was performed on day of life 17 at a weight of 1 kg. Previous case studies have reported umbilical venous access for angiography and/or intervention, which was not possible in our case as the umbilical vein had already closed naturally. Therefore, the decision was made to have two venous access points, one from the femoral vein to access the pulmonary artery for angiography and another from the internal jugular vein to access the DV for intervention. Initial pulmonary artery angiography demonstrated a significantly dilated vertical vein draining to the right portal vein, with a severely constricted (<1mm), faintly visible DV. From the internal jugular vein sheath, the DV was crossed using a 1.8Fr FineCross MG microcatheter and a 0.014’’ Asahi Sion Blue wire. The wire was positioned in the left portal vein and angioplasty of the DV was performed using a 3mm x 8mm Takeru balloon followed by placement of a 4mm x 12mm Resolute Onyx drug-eluting stent. Follow-up angiography demonstrated that the stent crossed and jailed the rightward part of the portal sinus where the vertical vein was draining. Therefore, the FineCross MG microcatheter and 0.014’’ Asahi Scion Blue wire were utilized to cross the side cell of the existing stent and access the vertical vein via the right portal vein. Angioplasty of the side cell was performed using a 3mm x 8mm Takeru balloon and the 4mm balloon from the previously placed stent, which resulted in fracturing of the side cell. During catheter and sheath manipulation, it was noted that the existing stent became deformed and there was residual obstruction at the DV, so a second stent was placed. A 4mm x 16mm Formula 418 stent was implanted across the DV, extending into the right side of the portal sinus allowing for unobstructed return from the vertical vein. The patient required two subsequent catheterizations for balloon atrial septostomy and DV stent dilation. He underwent successful TAPVC repair at 37 weeks corrected gestational age.
Discussion
:
This case demonstrates that stenting of the DV can be performed in patients with infradiaphragmatic TAPVC who are not surgical candidates. It highlights the importance of understanding the portal venous anatomy as it relates to the DV and pulmonary venous drainage.