CardioNerds (Amit Goyal & Daniel Ambinder) join Brigham and Women’s Hospital cardiology fellows (Mounica Yanamandala, Simin Lee and Maria Pabon Porras) for some fun times at the Charles River Esplanade! They discuss a complicated case of prosthetic valve endocarditis with aortic regurgitation. Dr. Dale Adler provides the E-CPR and program director Dr. Donna Polk provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Bibin Varghese with mentorship from University of Maryland cardiology fellow Karan Desai.
The CardioNerds Cardiology Case Reports series 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 “Expert CardioNerd Perspectives & Review” (E-CPR) for 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.
We are teaming up with the ACC FIT Section to use the #CNCR episodes to 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 E-CPR segment and a message from the program director.
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A male in his mid-40s with history of HIV on ART (undetectable VL, CD4 320) and idiopathic thoracic ascending aortic aneurysm (TAAA) with AR s/p bioprosthetic valve replacement 10 years prior presented with acute onset lightheadedness and pre-syncope. He was diagnosed with an idiopathic TAAA at age 30 after he was noted to have an incidental murmur. Over the next few years, his aortic root increased to over 7 cm with severe AR, LV dilation, and reduced LVEF of 45%. He underwent bioprosthetic aortic valve replacement and root repair with a Medtronic freestyle porcine aortic root with subsequent recovery of his LVEF to 50% and improved LV dilation. Thereafter, he was doing well until he reported a flu like illness 3 weeks prior to presentation with reported fever up to 101.3 F and associated myalgias. He denied any sick contacts or recent travel and was adherent to his HIV regiment. On the day of presentation, he was walking his dogs when he developed acute onset lightheadedness with presyncope. On presentation, he had a low grade fever, tachycardia, tachypnea, and hypoxia. On exam, cardiac exam was notable for loud blowing diastolic murmur, non-distended JVP, decreased breath sounds, warm extremities with bounding pulses and without edema. There were no stigmata of endocarditis. Labs revealed elevated cardiac and inflammatory biomarkers. Blood cultures were initially NGTD. CXR corroborated the exam with bilateral interstitial and airspace opacification with effusions. TTE showed LVEF 35% with global hypokinesis, dilated LV with LVEDD 7.5 cm, mild RV systolic dysfunction, severe AR with holo-diastolic flow reversal in the abdominal aorta, no prosthetic stenosis, and aortic root 31 mm. TEE showed a well-seated AVR with leaflet thickening and several echodensities. CT surgery deemed patient to be high risk for the OR. After a few days, patient required intubation for increased work of breathing and acute decompensation requiring vasoactive infusions. After multidisciplinary discussions, the patient ultimately underwent ViV TAVR with successful placement of a 29 mm Edwards Sapien 3 valve. That day, he was weaned off pressors, and subsequently extubated. Infectious workup remained negative thereby resulting in a final diagnosis of prosthetic aortic valve culture-negative endocarditis vs sterile prosthetic valve deterioration. He was discharged on intravenous empiric antibiotics and was doing well in clinic 2 months later.
A. CXR: Bilateral airspace and interstitial opacification. Small bilateral effusions. No pneumothorax.
C. CW Doppler across the aortic valve
D. PW Doppler showing holodiastolic flow reversal in the descending thoracic aorta
E. Pre-TAVR LV and aortic pressure tracings
F. Post-TAVR LV and aortic pressure tracings
Episode Schematics & Teaching
The CardioNerds 5! – 5 major takeaways from the #CNCR case
1. The patient had an idiopathic ascending aortic aneurysm. How do you typically approach the etiology for a thoracic aortic aneurysm?
- Let us first differentiate the terminology for the aorta. The ascending aorta includes the aortic root (which contains the sinuses of Valsalva), the aortic arch which includes the great vessels. The descending aorta begins beyond the left subclavian artery. The isthmus is the point where the aortic arch and the descending aorta join (also the site of the ductus arteriosus which turns into the ligamentum arteriosum). The abdominal aorta begins distal to the diaphragm.
- Thoracic Aortic Aneurysms (TAA) include aneurysms that occur from the aortic root to the crus of the diaphragm. There are various etiologies for TAAs, but the most common cause is medial necrosis. This is a non-specific pathologic finding that may be found in a number of conditions. The underlying histologic findings include loss of elastic fibers and vascular smooth muscle in the media, with accumulation of a basophilic ground substance leading to a cystic appearance.
- There are several ways to approach the etiology of TAAs. The causes may be classified into degenerative, genetic, infectious, inflammatory, and other causes. Genetic causes include but are not limited to Marfan’s Syndrome, Loeys-Dietz Syndrome, Ehlers-Danlos Type IV, Familial TAA, and Turner syndrome. Inflammatory conditions include giant cell arteritis, Takayasu arteritis, and HLA-B27 associated spondyloarthropathies. An important infectious cause includes syphilis as we have previously discussed on our CNCR Series: Ep #47 – Syphilitic Aortitis!
- Generally, aortopathies occurring proximal to the ligamentum arteriosum are more likely to be genetic while those occurring distally are more likely to be degenerative (related to atherosclerotic risk factors).
- Remember, in terms of management, TAAs tend to grow more rapidly as they become larger and depending on the etiology, there may be a lower cut-off for elective procedures as the guidelines suggest earlier intervention for specific conditions. For instance, patients with Loeys-Dietz (amongst other genetic syndromes), can be considered for elective TAA at aneurysms less than 50 mm given the high occurrence of dissection after 50 mm. The approach should be individualized to the patient’s underlying condition, body size and sex (as there may be smaller absolute aortic root size and diameter in women).
2. The patient had well controlled HIV and developed a TAA. What are the known cardiovascular risks associated with HIV?
- Cardiovascular disease, including myocardial, pericardial, and vascular (including coronary) disease, has become a major cause of morbidity and mortality in patients with HIV
- Vascular disease likely has several causes including traditional risk factors (e.g., diabetes, hypertension) and chronic inflammation which leads to accelerated atherosclerosis. Recall from the CardioNerds Prevention Series that HIV is a key “risk-enhancing factor”!
- HIV is an important cause of heart failure, including HIV-associated dilated cardiomyopathy. Some data has suggested that the HIV-1 virus may infect myocardial cells directly in a patchy distribution leading to progressive damage and fibrosis. Furthermore , underlying ischemia, co-infection with other pathogens (e.g., EBV, CMV, coxsackievirus B), cardiac autoimmunity, and drug toxicities can contribute to the development of HF. Patients with HIV are additionally at higher risk of developing HFpEF. The mechanism remains to be elucidated, though the extent of chronic inflammation, T-cell activation, and loss of adaptive immunity likely all play a role.
- Pericardial disease and specifically pericarditis was the most common manifestation of HIV-associated cardiac disease prior to the advent of potent ART.
- Other manifestations include pulmonary arterial hypertension (WHO Group 1), increased risk of venous thromboembolism, and possibly sudden cardiac death. Infective endocarditis rates are similar to patients without HIV, once corrected for other exposures (e.g., IV drug use).
3. The patient developed a new
loud decrescendo blowing diastolic murmur which was concerning for aortic regurgitation. What are the other physical exam findings associated with aortic regurgitation?
- As we reviewed in previous episodes (Ep #47 – Syphilitic Aortitis), the physical exam of patients with chronic AR is full of classic findings and eponyms! The classic murmur of AR is a diastolic, blowing, decrescendo murmur heard in the LUSB. The severity of AR correlates with the duration of the murmur more than the intensity. The increased blood flow across the AV can lead to a systolic flow murmur in the RUSB, and the regurgitant blood flow can limit opening of the MV causing a diastolic murmur best heard at the apex called the Austin-Flint murmur.
- Note, in chronic AR there is a rapid increase in systole BP and fall in diastole, leading to a widened pulse pressure and bounding pulses. Some physical exam findings (not an all-inclusive list!) associated with hyperdynamic pulse are included below. Note, some of these findings are not necessarily specific to chronic AR alone, but can also be seen in other states of hyperdynamic circulation.
- Water hammer pulse: a bounding pulse with rapid systolic rise followed by rapid diastolic collapse that can be best appreciated at the radial or brachial artery. It is accentuated by slightly lifting up the patient’s arm.
- Corrigan’s pulse: bounding carotid pulse with rapid rise and collapse
- Traube sign: “pistol shot” sounds heard over femoral arteries in both systole and diastole
- Duroziez sign: diastolic murmur heard over the femoral artery after compressing distally or a systolic murmur when compressed proximally
- Quincke’s pulse: capillary pulsations in the nail beds
- Muller’s sign: pulsations of the uvula
- de Musset sign: head bobbing synchronous with heartbeat
- Becker’s sign: visible pulsations of the retinal arteries on ophthalmoscopic examination
- Hill sign: positive when the popliteal cuff systolic pressure exceeds the brachial cuff systolic pressure by > 60 mmHg
- The etiology of the acute onset aortic regurgitation was thought to be from infective endocarditis. Returning to basics, what are the criteria for infective endocarditis?
- The Duke Criteria are the most sensitive and specific diagnostic criteria for diagnosing native valve infective endocarditis. However, for prosthetic valve endocarditis (PVE), the Duke criteria have decreased sensitivity and specificity. For possible endocarditis or “rejected” endocarditis based on Duke Criteria where there is a persistent suspicion of PVE, further advanced imaging should be considered including TEE, 18F-FDG PET/CT, and/or 4D cardiac CTA.
- There are pathologic and clinical Duke Criteria. The clinical criteria are included below from the Chambers and Bayer NEJM article
- A “definite” clinical diagnosis of infective endocarditis requires two major criteria, or one major and three minor or five minor criteria. “Possible” clinical diagnosis of IE includes one major criteria and one minor criteria or three minor criteria. A “rejected” clinical diagnosis of IE requires a clear alternative diagnosis for the patient’s clinical manifestations suggestive of IE or resolution of those symptoms with antibiotics for 4 days or less. The absence of pathologic evidence of IE on autopsy or surgery after 4 days or less of antibiotics can also reject the diagnosis of IE.
5. When is valvular intervention considered in prosthetic valve endocarditis?
- Prosthetic valve endocarditis (PVE) commonly requires a combined medical-surgical approach.. Early surgery in PVE, per the 2015 AHA scientific statement, is recommended for patients with one or more of the following clinical circumstances: (1) signs or symptoms of heart failure as a result of valve dehiscence, severe prosthetic valve dysfunction, or intracardiac fistula; (2) IE complicated by complete heart block or an aortic/annular abscess; (3) PVE caused by a fungal organism or a highly resistant organism; (4) persistent bacteremia despite antibiotic therapy ~ 7 days.
- These recommendations are based on limited observational evidence. In the largest study to date early surgery for conditions other than valve dysfunction and perivalvular abscess was not associated with improved hospital or one-year survival. Other indications to consider potential surgery after weighing the risk and benefits are (1) aortic or mitral PVE with persistent vegetations >10 mm after one or more embolic episode despite appropriate antibiotic therapy; (2) aortic or mitral PVE with isolated very large vegetations (>30 mm); and (3) patients with relapsing PVE.
- Feinstein, M. J. et al. Characteristics, Prevention, and Management of Cardiovascular Disease in People Living With HIV: A Scientific Statement From the American Heart Association. Circulation 140, e98–e124 (2019).
- Chambers, H. F. & Bayer, A. S. Native-Valve Infective Endocarditis. N. Engl. J. Med. 383, 567–576 (2020).
- UpToDate Article