121. Case Report: Complex Shock in Shone Complex – University of Wisconsin-Madison

Cardionerds: A Cardiology Podcast

CardioNerds (Amit Goyal & Daniel Ambinder) join Dr. Rayan Jo Rachwan, Dr. Anupama Joseph, and Dr. Mohammed Merchant from the University of Wisconsin-Madison for a classic Madison dinner cruise! They discuss the following case: Mixed shock secondary to severe right ventricular outflow tract obstruction with Gemella Haemolysans prosthetic pulmonary valve endocarditis in a young patient with Shone Complex (syndrome). Dr. Ford Ballantyne III provides the E-CPR segment for this episode. Special introductory music composed by Dr. Rayan Jo Rachwan. We are excited to welcome University of Wisconsin- Madison to the CardioNerds Healy Honor Roll and Dr. Rayan Jo Rachwan as the CardioNerds Ambassador. Claim free CME just for enjoying this episode! Jump to: Patient summary - Case media - Case teaching - References CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Patient Summary A 26-year-old male with history of bicuspid aortic valve and Shone Complex (syndrome)—status post coarctation repair, subaortic resection and Ross-Konno operation—presenting with 3 months of constitutional and respiratory symptoms. Initial evaluation demonstrated that patient was in a state of mixed shock due to a large pulmonary Melody valve thrombus with superimposed Gemella Haemolysans prosthetic valve endocarditis. He required treatment with inotropes, pressors, followed by intubation and extracorporeal membrane oxygenation (ECMO). Patient was treated initially via right heart catheterization with balloon dilation and stent placement to his right ventricle-to-pulmonary artery conduit, which lead to significant improvement in his hemodynamics. Patient was then decannulated from ECMO, extubated, weaned off pressor support and later underwent a successful surgical resection of the infected pulmonary homograft and Melody valve/stents and replacement with pulmonary-valved conduit. He was also discharged on a prolonged course of antibiotics. Case Media - Shone Complex A B C D Click to Enlarge A. CXR, B. ECG, C. TV Doppler, D. PV Doppler CTA Chest •Melody pulmonic valve repair with large thrombus arising from the mid-distal valve extending into the main pulmonary artery and proximal left pulmonary artery. Evaluation for distal subsegmental pulmonary emboli is limited. •Tiny focus of air in the thrombus may be related to contrast injection. Infection is less likely. •Enlarged right heart chambers, may be in part chronic right heart enlargement and/or new right heart strain. No pulmonary infarct. CT chest abdomen and pelvis with contrast •Findings suggestive of acute hepatitis and acute pancreatitis. No pancreatic hypoenhancement or peripancreatic fluid collection. •No abscess within the abdomen or pelvis. •Small caliber of the infrarenal abdominal aorta and bilateral iliac arteries, probably congenital. TTE 1 TTE 2 TTE 3 TTE 4 TTE 5 TTE 6 RHC with balloon dilation of the RV-PA conduit and evidence of multiple levels of stent fracture. Pulmonary angiogram showing no evidence of distal embolization or significant pulmonary embolism and no evidence of perforation. There is evidence of moderate pulmonary insufficiency into a dilated right ventricle. Episode Teaching - Shone Complex Pearls 1. Patients with congenital heart disease are more predisposed to infective endocarditis (IE). Therefore, there should be a low-threshold for infectious workup in the case of unexplained fever or malaise without associated symptoms for >72 hours. Every routine visit should screen for symptoms and signs of IE. 2. Treatment of right ventricular (RV) outflow tract obstruction with balloon dilation +/- stenting can be considered as a bridge to valve replacement...