CARCINOID HEART DISEASE

Dr. Kornfield presented a very interesting case of a patient referred to pulmonary clinic with shortness of breath, presumed to be related to undiagnosed COPD.

A 68 year-old veteran with a history of tobacco use and metastatic carcinoid presented with one year of progressively worsening dyspnea on exertion, such that he could only walk ~50 feet before resting. He denied any significant cough or sputum production and had no benefit from use of a bronchodilator. He noted lower extremity edema without orthopnea or paroxysmal nocturnal dyspnea. On exam, JVP was estimated at 12 cm H20 and a new, soft systolic murmur was noted. A TTE revealed thickened tricuspid valve leaflets, severe TR, RVSP upper limit of normal, and EF 60-65% EF. He was diagnosed with carcinoid heart disease (!) and referred for consideration of valve replacement surgery.

Carcinoid heart disease is a rare manifestation of advanced carcinoid tumors typically involving right heart valves (most commonly the tricuspid valve), and resulting in right heart failure.

There is a 20% prevalence of cardiac involvement in patients with carcinoid syndrome.

TAKE HOME POINTS

•DDx for dyspnea on exertion is always broad but should include RV failure.

•Suspect carcinoid heart disease in patients with carcinoid tumors and symptoms suggestive of right heart failure. Confirm with echocardiography.

•While multiple medical therapies are available to treat carcinoid, surgery is the effective intervention for valvular disease.

Adenovirus hemorrhagic cystitis

Dr. Coetzer presented a fascinating case of an immunocompromised man with history of renal transplantation who presented with fever, acute onset hematuria and flank pain.

We walked through an anatomical diagnostic schema for hematuria before we dived into our HPI questions

Picture courtesy of  UpToDate

Picture courtesy of UpToDate

His UA had >1000 RBC, 9 WBC, no protein, no casts, no dysmorphic RBC’s. The patient had a CT without contrast that was not suggestive of nephrolithiasis or masses and the allograft appeared normal. There were no significant occupational exposures or culprit medications.

Given his immunocompromised state, infectious workup included EBV, CMV, quantiferon TB , Brucella antibodies, coccioides antibodies, urine Histoplasma antibodies, ureoplasma, mycoplasma, blood and urine cultures, all of which were negative. Adenovirus viral load was found to be >590,000,000 by PCR, consistent with adenovirus hemorrhagic cystitis (AHC).


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References:

—deVries CR, Freiha FS. Hemorrhagic cystitis: a review. J Urol. 1990 Jan. 143(1):1-9.

—Dorairajan, L., Manikandan, R., & Kumar, S. (2010). Hemorrhagic cystitis: A challenge to the urologist. Indian Journal of Urology, 26(2), 159. doi: 10.4103/0970-1591.65380

—Hofland, C., Eron, L., & Washecka, R. (2004). Hemorrhagic adenovirus cystitis after renal transplantation. Transplantation Proceedings, 36(10), 3025–3027. doi: 10.1016/j.transproceed.2004.10.090

—Bradshaw, C. S. (2002). Characteristics of adenovirus associated urethritis. Sexually Transmitted Infections, 78(6), 445–447. doi: 10.1136/sti.78.6.445

—Akiyama, H., Kurosu, T., Sakashita, C., Inoue, T., Mori, S.-I., Ohashi, K., … Kitamura, T. (2001). Adenovirus Is a Key Pathogen in Hemorrhagic Cystitis Associated with Bone Marrow Transplantation. Clinical Infectious Diseases, 32(9), 1325–1330. doi: 10.1086/319992

—Parasuraman, R., Zhang, P. L., Samarapungavan, D., Rocher, L., & Koffron, A. (2013). Severe Necrotizing Adenovirus Tubulointerstitial Nephritis in a Kidney Transplant Recipient. Case Reports in Transplantation, 2013, 1–5. doi: 10.1155/2013/969186


SYNCOPE

We reviewed an NEJM clinical case of a 63 year old man evaluated for syncope. We used Dr. Harmon’s framework (see below) and practiced using clinical prediction tools (JAMA Rational Clinical Exam: Did this patient have cardiac syncope) and EGSYS (Clinical Predictors of Cardiac Syncope in Initial Evaluation of patients referred urgently to a general hospital) to help generate a differential and create a targeted approach to work-up.

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Dr. Bryn McGhee referenced The Cost Effective Evaluation of Syncope, summarized below.

Cost-effective Work-up for Syncope/Take-Home Points:

1) A meticulous history and physical examination

2) orthostatics

3) electrocardiogram

Routine blood tests, neuro-imaging with computed tomography scans, MRIs, carotid Doppler, echocardiography, and inpatient telemetry monitoring rarely contribute to the diagnosis but add substantial cost.

There are multiple risk stratification tools that help identify high-risk patients and guide management. Applying these tools can reduce syncope-related costs substantially without increasing risks to patients.

Diagnosis (Spoiler Alert)

The patient was found to have 2 masses compressing the tricuspid annulus at the juncture of the atrium and the right ventrilce, which were determined to be saphenous vein aneurysms. The syncopal episode was ultimately felt to be due to decreased preload (either due to reflex sympathy or orthostatic hypotension) compounded by impaired filling on the right side of the heart by saphenous-vein-graft aneurysm.

A systematic review of saphenous-vein–graft aneurysm showed that it arises a mean of 13 years after CABG, usually occurs during the sixth decade of life, and is found predominantly in men.

External compression of the right atrium or right ventricle occurs in nearly 20% of cases.

Chest pain or shortness of breath develops in more than half the cases of saphenous-vein– graft aneurysm, but in nearly one third of cases, the aneurysm is discovered incidentally.

Myocardial infarction is the presenting symptom in approximately 8% of cases, and heart failure and syncope each occur in approximately 2%.

Angiography of the coronary artery and graft may be of limited use if there is an intra-luminal thrombus or the aneurysm is thrombosed, because the aneurysm can be missed or its size underestimated.

Cardiac CT is the preferred test, with cardiac magnetic resonance imaging (MRI) as an alternative, to confirm the diagnosis and determine the effect on adjacent heart structures.

References

Albassam et al. Did This Patient Have Cardiac Syncope? The Rational Clinical Examination Systematic Review. JAMA, June 25 2019, Volume 321, Number 24 pages: 2448-2457: https://jamanetwork.com/journals/jama/fullarticle/2736568

Steven Angus, MD, The Cost-Effective Evaluation of Syncope. Medical Clinics of North America. volume 100, Issue 5, September 2016, Pages 1019-1032: https://www-sciencedirect-com.liboff.ohsu.edu/science/article/pii/S0025712516372741?via%3Dihub

Weiner et al. Case 25-2018: A 63-Year-Old Man with Syncope. New England Journal of Medicine. August 16 2018. 379:670-680: https://www-nejm-org.liboff.ohsu.edu/doi/pdf/10.1056/NEJMcpc1800340?articleTools=true















SCAD SCARE

Dr. Kholghi presented a classic case of spontaneous coronary artery dissection (SCAD) in a young woman with prior IVF pregnancies who was admitted with chest pain and this EKG:

Yikes! Residents quickly identified the ST elevations in the inferior & lateral leads with reciprocal ST depressions.  How is this different than the EKG changes seen in acute pericarditis? In pericarditis, the ST elevations are typically diffuse and concave with associated PR-segment depressions. Reciprocal ST depressions (as seen in this EKG), are not expected in pericarditis.

Yikes! Residents quickly identified the ST elevations in the inferior & lateral leads with reciprocal ST depressions.

How is this different than the EKG changes seen in acute pericarditis? In pericarditis, the ST elevations are typically diffuse and concave with associated PR-segment depressions. Reciprocal ST depressions (as seen in this EKG), are not expected in pericarditis.

Code STEMI was activated and the patient was taken for cath, which revealed a dissection of the OM1.

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Take Away Points

  • Consider a diagnosis of SCAD in a young women presenting with chest pain or ACS  

  • Risk factors include postpartum status, multiparity and exposure to hormonal therapy, so this is important historical information to gather

  • Conservative therapy (rather than revascularization), is the preferred treatment strategy

Below is a link for a related case from NEJM Case Records of the Massachusetts General Hospital (thanks for the recommendation, Dr. Burnett!)

Hyperkalemia Master Class

Dr. Levin presented a case of a patient who had “changes on tele” and ended up having this EKG:

Yikes! He had a normal EKG prior.   Let’s point out some of the scary changes here:   1. Where are the P waves? are they gone entirely or just very small?  2. Are those T waves peaked?  3. Why is the QRS suddenly so wide?

Yikes! He had a normal EKG prior.

Let’s point out some of the scary changes here:

1. Where are the P waves? are they gone entirely or just very small?

2. Are those T waves peaked?

3. Why is the QRS suddenly so wide?

Labs were obtained and he had a K of 7.6!

Here’s our approach to the evaluation of hyperkalemia:

(Content adapted from the excellent curbsider’s podcast: Hyperkalemia Master Class)

  1. Does it make sense for this patient to have hyperkalemia?

    • Is the sample hemolyzed?

    • If not, consider causes of pseudohyperkalemia: ie very high WBC count like in CLL, thrombocytosis (PLT >1,000,000), anything that increases the likelihood of the cells lysing in the phlebotomy tube

  2. Rule out urinary obstruction (if anyone can comment on the mechanism of this, please do in the comments section! For a prize of resident education …okay and some candy too)

  3. Evaluate for hyperglycemia (can ca

When do we treat?

  • In one study by Einhorn et al:

    • Potassium 5.5 OR for death

    • Potassium >6, OR for death 31

  • Consider treating (ie shifting etc) when K>6.0 + EKG changes or >6.5 regardless of EKG changes

What do we treat ACUTELY with?

  • Calcium for membrane stabilization (Calcium gluconate typically but Calcium chloride is also an option)

  • Insulin & glucose (Don’t forget to recheck glucose q30-60min after for up to 3-4 hours!)

  • Beta agonist (The dose of albuterol for shifting is 20mg. To give you an idea, the dose of a standard albuterol neb is 2.5mg)

  • Bicarb (typically reserved for when the cause of the hyperkalemia is acidosis. Keep in mind also that bicarb will shift calcium into cells— something you may not want in the setting of EKG changes)

But how do we get it out of the body?

  • If they can pee, urine is the best bet (lasix or lasix + IVF so minimize the adverse side effects of the diuretics)

  • Reserve kayexylate (or SPS) for those who cannot urinate or those who have chronic hyperkalemia. AVOID this in patients w/ CKD or ESRD, s/p renal transplantation or any kind of bowel obstruction/decreased bowel motility (refer to our recent blog post here

Thanks for reading! If you’re enthralled and need to know MORE about hyperkalemia, we highly recommend the hyperkalemia master class episode of the Curbisider’s Podcast here