Jonathan Boyd, MD
Clinical Fellow, Pediatric Critical Care Medicine
Monroe Carell Jr Children's Hospital at Vanderbilt
Nashville, Tennessee
Disclosure information not submitted.
Dustin Hipp, MD, MBA
Assistant Professor of Pediatrics
Vanderbilt University Medical Center, United States
Disclosure information not submitted.
Julie Stark, MD
Assistant Professor of Pediatrics
Vanderbilt University Medical Center, United States
Disclosure information not submitted.
Bevan Londergan, MD
Assistant Professor of Anesthesia; Chief, Pediatric Cardiac Anesthesiology
Vanderbilt University Medical Center, United States
Disclosure information not submitted.
Nhue Do, MD
Assistant Professor, Division of Pediatric Cardiac Surgery
Vanderbilt University Medical Center, United States
Disclosure information not submitted.
Title: Delayed Presentation of Obstructed Total Anomalous Pulmonary Venous Connection with SARS-CoV-2
Case Report Body: INTRODUCTION: Total anomalous pulmonary venous connection (TAPVC) constitutes 1-3% of all congenital heart disease annually. Infradiaphragmatic TAPVC often presents with pulmonary venous obstruction, requiring emergent intervention. SARS-CoV-2 is associated with coagulopathy, which could impact patients with congenital heart disease.
Description: A three-month old presented with increased work of breathing. In the emergency department, temperature was 38.4 Celsius, heart rate was 210 beats per minute, oxygen saturation was 54%, respiratory rate was 58 breaths per minute, and blood pressure was 97/78 mm Hg. The initial chest radiograph showed diffuse pulmonary edema. An echocardiogram showed obstructed infradiaphragmatic TAPVC with a 14 mm Hg gradient, a moderate secundum atrial septal defect, and markedly depressed right ventricular wall motion. A SARS-Co-V-2 PCR was positive.
The patient underwent emergent repair. Tranexamic acid administration was avoided due to thrombotic concerns for COVID positive patients. Preoperative antithrombin III activity was 112%. The heparin loading dose for cardiopulmonary bypass (CPB) was calculated using the Medtronic Hepcon HMS Plus. The patient was cooled to 28 degrees Celsius. During CPB, 46 mL/kg of fresh frozen plasma (FFP) was administered. In the post-bypass period 34 mL/kg of platelets and 7.7 mL/kg of cryoprecipitate were given empirically. Coagulation studies after initial transfusions were prothrombin time (PT) 19 seconds (s), partial thromboplastin time (PTT) 52.1 s, fibrinogen 388 mg/dL, and platelet count 178 *103 /mcL. An additional 23 mL/kg of platelets and 10 ml/kg of FFP were given for high chest tube output. PT and PTT peaked at 20.1 and 53.7 s; these improved to 15.4 and 34 s on post operative (POD) 2. Delayed sternal closure occurred on POD 4. The patient was discharged on POD 13 with no gradient across the pulmonary vein confluence by echocardiogram. Biventricular function was normal at hospital follow up.
Discussion: This patient did not have prolonged length of stay or thrombotic complications despite presenting in extremis with critical congenital heart disease and SARS-Co-V-2. This patient highlights potential differences in coagulation between children and adults with SARS-CoV-2 and the ability to safely perform CPB in active infection when needed.