Galeano-Lovera, Santiago F.

Acquired angioedema due to C1-inhibitor deficiency (AAE-C1INH) is a rare condition characterized by the localized swelling of the deeper skin layers and mucous membranes, especially the face, lips, tongue, throat, and gastrointestinal tract. AAE-C1INH is strongly associated with lymphoproliferative disorders, although it can also be linked to autoimmune conditions, solid tumors, infections, or even occur without an identifiable cause. We present the case of a 45-year-old female patient with complaints of recurrent abdominal pain, bloating, and joint swelling. Laboratory testing showed decreased C1q and C4 complement levels, and C1 esterase inhibitor levels, indicative of AAE-C1INH. Further work up confirmed a diagnosis of extranodal marginal zone lymphoma with involvement of the bone marrow and spleen. Treatment with rituximab led to resolution of angioedema symptoms and almost complete remission of underlying lymphoma. This case underscores the importance of evaluating an underlying lymphoproliferative disorder in AAE-C1INH. Therefore, the early participation of a multidisciplinary team including specialists in immunology, hematology, and oncology is necessary for appropriate management.

This case report describes a woman in her early 60s who went into cardiac arrest immediately after administration of an echocardiogram contrast agent.

Introduction: Fontan pathway obstruction is a serious complication characterized by an anatomical or functional narrowing anywhere in the cavo-pulmonary pathways. Here we report the first case where an innovative Fontan conduit rehabilitation procedure with intravascular lithotripsy was used achieving a significant increase in the pathway size. Case: A 28-year-old male with tricuspid atresia status-post Fontan palliation (age 23 months) with bare metal stent implant to treat obstruction at age 15, presented with syncope, ascites and varicose veins. Imaging studies revealed a cirrhotic liver morphology and Fontan pathway obstruction with minimal diameter of 12 mm. Cardiac catheterization confirmed severe Fontan conduit obstruction in addition to LPA stenosis. Serial covered Cheatham-Platinum stents were implanted for conduit coverage followed by Palmaz stents. Non-compliant Atlas balloon dilation to 20 atm was performed without expansion of the Fontan pathway beyond 12 mm due to severe calcification. A 7.0 x 60 mm shock-wave lithotripsy balloon was added alongside the Atlas balloon to modulate the heavily calcified wall with subsequent expansion of the conduit to 21 mm after inflating the Atlas balloon to 14 atm. Discussion: Several studies demonstrated that transcatheter stenting of stenotic Fontan conduits can be safely performed. Intravascular lithotripsy has emerged as a novel therapy for the treatment of coronary vascular calcification. We report the first case of an innovative percutaneous Fontan conduit rehabilitation procedure using a lithotripsy shock wave balloon in an adult with severely calcified Fontan pathway obstruction and prior stents, achieving a 75% increase in the pathway size without significant complications. Further studies are needed to evaluate the long-term safety and efficacy of this novel approach, but this case presents an exciting option for future directions in the percutaneous treatment of Fontan pathway obstruction.