Mechanical thrombectomy of COVID-19 DVT with congenital heart disease leading to phlegmasia cerulea dolens: a case report

Background COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both. Case presentation A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated. Conclusions This report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity.

Management Considerations of Hodgkin Lymphoma for Patients with Fontan Physiology

While treatment protocols for Hodgkin Lymphoma (HL) are well established, there is no literature available to guide therapy or estimate prognosis for patients with Fontan physiology who develop HL. The physiology of a Fontan procedure can result in the inability to tolerate chemotherapy toxicities, supportive care and infection. We present a series of 3 patients with Fontan physiology who were treated for HL and discuss their clinical course and treatment.

Successive perioperative management of laparoscopic liver resection in the reverse Trendelenburg position for a patient with Fontan physiology: a case report

Background Laparoscopic surgery for a patient with Fontan physiology is challenging because pneumoperitoneum and positive pressure ventilation could decrease venous return and the accumulated partial pressure of arterial carbon dioxide (PaCO2) could increase pulmonary vascular resistance, which might lead to disruption of the hemodynamics. Case presentation A 25-year-old man with Fontan physiology was scheduled to undergo laparoscopic liver resection for Fontan-associated liver disease (FALD) with noninvasive monitoring of cardiac output (CO) by transpulmonary thermodilution in addition to transesophageal echocardiography. The abdominal air pressure was maintained low, and we planned to switch to open abdominal surgery promptly if hemodynamic instability became apparent because of the accumulated PaCO2 or postural change. Consequently, the pneumoperitoneum had limited influence on circulatory dynamics, but central venous pressure significantly decreased with postural change to the reverse Trendelenburg position. Laparoscopic liver resection for FALD was performed successfully with no significant changes in CO and central venous saturation. Conclusions With strict circulation management, laparoscopic surgery for a patient with Fontan physiology can be performed safely. Comprehensive hemodynamic assessment by noninvasive transpulmonary thermodilution can provide valuable information to determine the time for shift to open abdominal surgery.

A Decision Rule for Determining the Optimal Transplant Listing Window for Patients With a Fontan Physiology

The Fontan is a complex surgical procedure used as a palliative treatment for children with univentricular hearts. In the past, the mortality rate was high and the associated comorbidities as a result of the Fontan circulation were many. However, as research into the condition developed, better understanding has led to a massive reduction in early mortality and a rapidly increasing population of such patients surviving well into adulthood. This has led to a large surge in patients with congenital heart disease being referred for cardiac transplant assessment. According to research, listing these patients at the optimal time is the key to improving transplant outcomes. However, determining that optimal time is unclear and controversial. In this article, I address this issue by developing an optimal timing rule that accounts for the factors faced by specialist cardiologists in determining when transplant ought to be considered for this cohort of patients.

Carotid approach to anterior circulation thromboembolectomy in an adult with failing fontan physiology: a case report

Background Anesthetic management of an adult with failing Fontan physiology is complicated given inherent anatomical and physiological alterations. Neurosurgical interventions including thromboembolectomy may be particularly challenging given importance of blood pressure control and cerebral perfusion. Case Presentation We describe a 29 year old patient born with double outlet right ventricle (DORV) with mitral valve atresia who after multi-staged surgeries earlier in life, presented with failing Fontan physiology. She was admitted to the hospital almost 29 years after her initial surgeries to undergo workup for a dual heart and liver transplant in the context of a failing Fontan with elevated end diastolic pressures, NYHA III heart failure symptoms, and liver cirrhosis from congestive hepatopathy. During the workup in the context of holding anticoagulation for invasive procedures, she developed a middle cerebral artery (MCA) stroke requiring a thromboembolectomy via left carotid artery approach. Discussion and Conclusions This case posed many challenges to the anesthesiologist including airway control, hemodynamic and cardiopulmonary monitoring, evaluation of perfusion, vascular access, and management of anticoagulation in an adult patient in heart and liver failure with Fontan physiology undergoing thromboembolectomy for MCA embolic stroke.