68. Case Report: WPW and HCM Phenotype VCU

Published: Oct. 9, 2020, 7:08 p.m.

CardioNerds\xa0(Amit Goyal\xa0&\xa0Daniel Ambinder) join Virginia Commonwealth University (VCU) cardiology fellows (Ajay Pillai, Amar Doshi, and Anna\xa0Tomdio) for a delicious skillet breakfast and amazing day in Richmond, VA! They discuss a fascinating case of a patient with Wolff-Parkinson-White\xa0(WPW) and hypertrophic cardiomyopathy (HCM). Dr. Keyur Shah provides the E-CPR and program director Dr. Gautham\xa0Kalahasty\xa0provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.\n\n\n\nJump to: Patient summary - Case media - Case teaching - References \n\n\n\nEpisode graphic by Dr. Carine Hamo\n\n\n\n\n\n\n\nThe\xa0CardioNerds Cardiology Case Reports\xa0series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an\xa0\u201cExpert CardioNerd Perspectives & Review\u201d (E-CPR)\xa0for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.\n\n\n\nWe are teaming up with the\xa0ACC FIT Section\xa0to use the\xa0#CNCR episodes\xa0to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an\xa0E-CPR\xa0segment and a message from the program director.\n\n\n\nCardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza\n\n\n\n\n\n\n\n\n\n\n\nPatient Summary\n\n\n\nA man in his mid-60s presented to the ED after an episode of unwitnessed\xa0syncope while drinking. Patient had\xa0suddenly passed out from a seated position with no prodrome or post-ictal state. He had episodes like this in the past, which were thought to be seizures, but otherwise PMHx only notable for alcohol use disorder. He denied any FH of SCD or syncope. In the ED, exam was unremarkable. Labs notable for mild thrombocytopenia, mild hyponatremia with AKI, 2:1 AST/ALT ratio, elevated NT-proBNP, and a very high lactate that rapidly corrected with fluids. EKG was notable for sinus tachycardia, short PR interval, wide QRS, and delta waves consistent with Wolff-Parkinson-White (WPW) pattern. Echo showed\xa0preserved\xa0LVEF, thickened LV septum (1.6 cm) and posterior wall (1.3 cm) concerning for hypertrophic cardiomyopathy (HCM). No outflow tract gradient was noted at rest or with stress, and the strain pattern demonstrated\xa0apical sparing. Evaluation\xa0for\xa0cardiac amyloid, including plasma cell dyscrasia and PYP scan, was negative. Cardiac MRI confirmed\xa0severely\xa0thickened LV inferior and inferolateral walls at 1.7 cm with no LVOT obstruction. 25% of the myocardium demonstrated\xa0patchy LGE.\xa0\xa0\n\n\n\nDue to concern for WPW syndrome, the patient underwent an EP study. This revealed\xa0a malignant septal accessory pathway that was successfully ablated with resolution of the WPW EKG features. Given large LGE burden in setting of HCM, patient underwent placement of primary prevention ICD. Genetic testing for PRKAG2 mutation is pending given comorbid WPW and HCM.\xa0\n\n\n\n\n\n\n\nCase Media\n\n\n\n\nAECDBFClick to Enlarge\n\n\n\nA. CXR: Slightly increased interstitial markings in the lung bases, an elevated right hemidiaphragm. No acute airspace disease or pulmonary edemaB. ECG: Sinus tachycardia rate 120bpm, PR interval 80ms, QRS 130ms, WPW pattern.\xa0 Arruda algorithm localizes to posterior septum.C. CMR:\xa0 Myocardium nulls before blood pool.D. CMR:\xa0 Delayed gadolinium enhancementE. Follow up ECG: NSR 78, repolarization abnormalities.\xa0 T wave memory inferior leads.F.