Thank you to Alayn for presenting the case of a middle aged man with Hennekam syndrome c/b by protein losing enteropathy, immunodeficiency, anasarca and recurrent pericardial effusions. The patient has had a prolonged course with recurrent infections and an untappable pericardial effusion. We discussed clinical and radiographical evidence of tamponade vs constrictive pericardial disease and focused on the management of his complex effusion w/ the possibility of a window vs pericardial stripping.
- In any patient presenting with ACS (UA, NSTEMI or STEMI) the standard of care is DAPT for 1 year regardless of management (medical vs interventional) AND regardless of type of stent placed.
- We learned that Hennekam syndrome is rare autosomal recessive intestinal lymphagiectasia-lymphedema syndrome associated with mental delay, immune deficiency and recurrent effusions. See this article for more info!
- Despite radiographic evidence suggestive of tamponade in this case, the diagnosis should be made clinically. Beck’s triad of 1) elevated JVP 2) tachycardia/hypotension and 3) distant heart sounds are components with a pulses paradox > 10 mmHg is suggestive tamponade physiology.
- A patient with tamponade will have a normal BNP (as the myocardium is not stretched).
- Make sure to measure a pulses correct! It is the systolic BP drop with inspiration and is positive when > 10 mmHg. Here is the link on YouTube how to measure this correctly.
More on Tamponade
- In patient with concern for tamponade a positive pulses > 10 mmHg is 98% sensitive and 84% specific (Circulation 64: 663-640).
- Physiology includes increased intrapericardial pressures that then impair diastolic filling and result in a drop in cardiac output.
- Other causes of pulses paradox include the following:
- Constrictive Pericarditis
- Asthma/COPD Exacerbations
- Large PE
- Tension PTX
- Large Pleural Effusions
Thank you to Lev for presenting the sad case of a middle aged woman with NASH cirrhosis and decompensation transferred from LTU given she is not a transplant candidate 2/2 to a BMI > 40. She remains on pressors with c/f rarer cause of refractory shock including AI or thiamine deficiency.
- In any patient with cirrhosis remember the pneumonic VIBES to help with relevant history taking = Volume (ascites), Infection (SBP), Bleeding, Esophageal (Varices), Screening (HCC).
- The original Model for End Stage Liver Disease score is made up of a patient’s serum bilirubin, INR and Cr. This score is a validated predictor of survival among patients with advanced liver disease and is used to determine allocation of organs for transplant.
- The MELD-Na score has since replaced the original MELD scoring system given improved predictive accuracy, especially for those with ascites. There is an increase in mortality of ~5% for each millimole decrease in serum sodium between 125 and 140 (Clinical Liver Disease, April 2015).
- A BMI > 40 is often a contraindication for transplantation given prior studies in the 1990s showing worse outcomes. These studies are felt to be flawed given they did not account for fluid overload which may have been driving these results.
- The BMI > 40 cut off is still debated and practice may be changing with newer data supporting expanding this cut-off – a 2015 meta-analysis in Liver International looked at 13 studies spanning a 13 year period and found no difference in mortality b/t obese patients and controls (Saab S, et al. The impact of obesity on patient survival in liver transplant recipients: A meta-analysis. Liver International. 2015;35:164.