Infra-diaphragmatic TAPVR with Congenital Diaphragmatic Hernia: The Gut or The Heart: A Surgical Conundrum
Presenter
Aamisha Gupta, M.D., Rady Children's Hospital, San Diego, CA
Aamisha Gupta, M.D.1, Kanishka Ratnayaka, MD2, Justin Ryan, Ph.D.1, Timothy Fairbanks, M.D.1, Sanjeet Hegde, M.D., Ph.D.3, John Nigro, M.D.1, John W. Moore, M.D.1 and Howaida El-Said, M.D., Ph.D., FSCAI4, (1)Rady Children's Hospital, San Diego, CA, (2)-, San Diego, CA, (3)UC San Diego Health, San Diego, CA, (4)The University of California, San Diego, San Diego, CA
Title:
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nfra-diaphragmatic TAPVR with Congenital Diaphragmatic Hernia: The Gut or The Heart: A Surgical Conundrum Introduction:
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ongenital heart disease (CHD) occurs in ~10-15% of patients with congenital diaphragmatic hernia (DH), with ~30% decrease in survival. Total anomalous pulmonary venous return(TAPVR) with concomitant DH is 0.27-0.46%, with a mortality of 80%. We present unconventional management of left DH and infra-diaphragmatic TAPVR with transcatheter stent implantations in the ductus venosus (DV) and a horizontal segment (anomalous pulmonary vein egress) between the umbilical vein and portal vein, followed by staged surgical repair. Clinical Case:
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full-term infant with known DH and postnatal diagnosis of infra-diaphragmatic TAPVR went for cardiac catheterization where an 8 French sheath was placed in the umbilical vein (UV). A stiff 0.014” guidewire (Ironman, Abbott Vascular, Chicago,Ill) was positioned through the DV into the left innominate vein. A second guidewire (BMW, Abbott Vascular, Chicago, Ill) and microcatheter/catheter (Renegade, Boston Scientific, Marlborough, MA and Glide, Terumo Medical, Phoenix, AZ) was also positioned through the same 8F-sheath protecting the vertical vein against kinking during DH reduction. DH repair (temporary closure) was performed with Integra-BioMesh (SurgiMend Princeton,NJ). Simultaneous kissing stent (Formula 418, Cook Medical, Bloomington IN) implantation protecting the DV and pulmonary venous egress horizontal vein was performed. Over the following two weeks, stepwise abdominal closure in 5 day intervals was followed by final closure at day of life 15. After DH repair, the patient was extubated and fed. Subsequently, TAPVR repair was performed on day of life 27. 6 month follow-up contrast enhanced CT scan showed significant decrease in portal venous system dilation and unobstructed pulmonary veins. Discussion:
Infra-diaphragmatic TAPVR is notorious for requiring urgent cardiac surgical intervention within the first few days of life due to obstruction of the vertical vein. The concomitant finding of DH in this patient lead to a complex scenario of how to best tackle a situation with systems that were interconnected above and below the diaphragm. As stated above, a rare finding of TAPVR with DH has significant mortality. Therefore after thoughtful discussion, with the use of 3D modeling and imaging, and a collaboration between cardiac, interventional, and general surgical specialties, a staged approach was engineered for this patient. The role of temporarily relieving cardiac obstruction with interventional catheterization and stent placement allowed for subsequent correction of the DH and ultimately relief for intrathoracic lung space. As optimized cardiopulmonary hemodynamics are essential for congenital cardiac surgery, this allowed for a successful full infra-diaphragmatic TAPVR repair. More studies and cases are needed to ultimately follow long term outcomes. However, given the success of our case, our unconventional management highlights a possibility for future difficult scenarios.