Good morning everyone!
Thank you Carine and Tim for presenting the case of a middle aged man with a history of complete heart block (of unclear etiology) status post PM placement, who presented with evidence of L and R sided thromboembolism (PE and LV thrombus) as well as new cardiomyopathy, who was ultimately diagnosed with cardiac sarcoidosis!
- New onset heart block in a young person cannot be assumed to be native conduction disease. We must rule out secondary causes (toxic/metabolic, infiltrative, or ischemic processes).
- Malignancy is the most common cause of a hemorrhagic, pericardial effusion
- Cardiac sarcoidosis is rare, and has been described in 5% of patients with systemic sarcoid, but autopsy studies indicate that subclinical cardiac involvement is present in up to 70% of cases
Complete Heart Block in a Young Patient
- In >50% of cases, no specific, reversible causes are identified. However, when a young patient presents with CHB, the diagnosis idiopathic progressive conduction disease should NOT be assumed (always search for secondary, reversible causes).
- Congenital Causes of Complete Heart Block
- Autoimmune congenital CHB: usually due to transplacental passage of maternal autoantibodies to Ro/SSA and/or La/SSB that damage developing conduction tissue
- Structural heart abnormalities due to congenital heart disease (eg congenitally corrected transposition of great arteries, endocardial cushion defects)
- Idiopathic familial congenital CHB
- Acquired Causes of Complete Heart Block
- Iatrogenic: meds, post-cardiac surgery, post-catheter ablation, post-TAVR
- Pathologic : Ischemia : Infiltrative – sarcoidosis, amyloidosis, malignancies : Myocarditis (eg Lyme Disease) : Endocarditis with abscess formation : Electrolyte abnormalities : Endocrinopathy
Cardiac Sarcoidosis (Blast from the past of VA & Moffitt Pearls!)
- Clinical evidence of myocardial involvement have been described in 5% of patients with systemic sarcoid, but autopsy studies indicate that subclinical cardiac involvement is present in up to 70% of cases!
- Clinical Manifestations depend on location and extent of granulomatous inflammation.
- AV block or bundle-branch block: most common finding in patients with clinically evident cardiac sarcoid
- Sudden cardiac death
- Pericardial disease
- When to suspect cardiac sarcoidosis?
- Young adults (< 55 yoa) with unexplained 2nd or 3rd degree AV block
- Young adults (< 55 yoa) with new ECG abnormalities or symptoms in the absence of coronary artery disease or inherited CV disease
- Patients with sustained monomorphic VT
- Patients with clinical diagnosis of extracardiac sarcoidosis
- Diagnosis: Challenging and frequently missed/delayed
- There are various guidelines proposed by various different societies! Usually based on a combination of ECG, echo, MRI/PET, and endomyocardial biopsy
- ECHO: 1st line imaging. LV dilatation, septal thinning, segmental or global hypokinesia of the LV or RV, aneurism formation, valvular regurgitation, simulated LV hypertrophy from infiltration into the myocardium.
- Cardiac MRI: Technique of choice for the dx of cardiac sarcoid w/ the highest sensitivity and specificity. Late gadolinium enhancement (LGE) may have prognostic value in evaluating chronic sarcoid as you have scar formation and increased risk of death presumably from arrhythmia.
- Cardiac PET: Excellent imaging modality for active sarcoid, but less specific as can have false positives with other inflammatory myocardial diseases.
Evernote Link: https://www.evernote.com/shard/s338/sh/c0552d05-75f7-426f-a6db-b194b5c0b8a2/f66c945f72b4e02c809d99fef3a09c12
Picking up where we left off w/ last week’s intern report, I thought I’d add a brief synopsis of the data on thrombolysis in sub-massive PE, thanks to Esteban for bringing the case to report.
#MOPETT trial: This is the “clot burden” trial. Patients w/ PE dx on CT angio w/ involvement of at least 70% of lung areas received thrombolysis compared to just anti-coagulation. Only 121 patients but some significant findings. Followup was long, about 2.5 years, primary outcome was development of pulm htn. At various times of follow-up the estimated PA pressure on echo was about 10-15 mmHg higher in the anti-coag only group (mostly LMWH like enox) compared to the tPA group, which finished w/ a normal avg PA pressure. There were no differences in bleeding rates which is obviously different from the ot her big trial below, but remember small sample size, based on the other rates observed you would only expect about 0-1 major bleeding events in a n=60 group of tPA (they did use half dose tPA for what it’s worth). No mortality difference, but again, smaller sample size.
#PEITHO trial: This is the echo + troponin study for sub-massive PE, not clot burden. Much larger, about 1000 patients randomized to heparin alone vs tPA+heparin, shorter f/u, only 30 days. There was a significantly reduced incidence of shock but no mortality benefit. This study did have a higher incidence of major bleeding, including intracranial bleeds, (2% vs 0.2% treatment:heparin groups), in the tPA cohort, though no excess mortality.
#Cochrane review: in 2015 A cochrane review was published pooling 18 RCTs but overall the grading of the studies was low quality mostly due to heterogeneity in the definition of sub-massive PE, there was a trend towards mortality benefit in TPA but less than a ringing endorsement.
Paul presented a case of a fellow who got a BKA and then bilateral PE’s! We talked about Hampton’s Hump and Westmark’s sign.
Hampton’s hump is the appearance of a “hump” representing the wedge infarct of a lung segment.
“Originally described in 1940 by Hampton and Castleman, Hampton’s hump is a peripheral wedge-shaped opacification abutting the pleura, signifying pulmonary infarction distal to a pulmonary embolism. The majority of pulmonary emboli do not result in infarction of the distal lung, presumably because of vascular collateralization. Infarction is most likely to occur in patients with large pulmonary emboli or underlying lung disease.”
As pointed out by Arielle and LT, it’s an area of relatively increased opacity corresponding to oligemia from the PE. Our patient actually had this!
“A chest radiograph showed a Westermark sign (Panel A, arrow), with a focal area of oligemia in the right middle zone and cutoff of the pulmonary artery in the upper lobe of the right lung. Computed tomographic pulmonary angiography confirmed the presence of a thrombus in the right pulmonary artery (Panel B, arrow), with an occlusive thrombus in the pulmonary arteries of the right upper and middle lobes. Another thrombus could be seen in multiple branches of the left pulmonary artery.”
Happy day after 4/20. CVG got lost in the L and D unit at the ZSFG trying to get back from report which is literally my waking nightmare. Anyway, we talked about alc hep. Thanks for the case dave Anderson.
#STOPAH trial: This is one of those trials that literally changed practice during my time in residency. I remember my first wards month at ZSFG giving pentoxifylline to alc hep patients, then a couple years ago this head to head trial of prednisolone vs pentoxi vs pentoxi+pred vs placebo groups in each arm. Prednisolone showed a slight mortality benefit at 28 days compared to placebo (14% of cohort had died, OR=0.72), but at 90d and one year no difference between any groups. This confirms what Dr. Cello had known for years, which is that long-term survival has never really been shown to be impacted by prednisolone/steroids, and that the benefits seem to wash out the further away from the hospital and steroids you get. We do know that transplant has a proven mortality benefit, and according to Dr. Cello women seem to have better outcomes post-transplant than men.
#arterial vs venous ammonia in acute liver failure: The oft-maligned ammonia level does have prognostic value in acute liver failure with regards to cerebral herniation. Geoff Buckle did some agile googling and found the following reference, from a small study, that showed ARTERIAL ammonia levels above 200 portended an increased risk of cerebral herniation. This can be helpful in your triage, when deciding whether to take pts to the unit for hypertonic saline.
too da loo!
- We’ve had a billion posts (actually just 3) about Rhabdo, so I’m just gonna consolidate the links here (and not recreate the wheel!)
- So what about that AKI 2/2 Rhabdo?
- AKI risk is lower in those w/ CK levels @ admission < 15000-20000; BUT you can see AKI in people with CKs as low as 5000
- RFs for AKI – dehydration, sepsis, acidosis
- The most important mgmt point is to HYDRATE people and MAINTAIN UOP (3cc/kg/hr)
- Like LT was saying, much of the damage occurs 2/2 volume depletion —> renal ischemia, tubular obstruction 2/2 heme pigment casts, and tubular injury from free chelatable iron
- Watch for metabolic abnormalities! (hyperK, hypoCa, hyperPhos, hyperUricemia)
- And what about that Lymphocytopenia?
- In a study of lymphocyte count < 600 , the most common causes were:
- Bacterial or fungal sepsis (24%)
- Postop state (22%)
- Malignancy (17%)
- Use of glucocorticoids (15%)
- Cytotoxic hero +/- radiation (9%)
- Trauma or hemorrhage (8%)
- For a more exhaustive list:
Evernote Link: https://www.evernote.com/l/ADWmx_2yUXtLLreKREi4KXUiDoyQV3kuI6s
Thank you Chloe, for presenting the case of a young woman with a history of granulomatous hypophysitis who presented with fevers and cavitary lung lesions. We discussed various entities on the ddx, including ANCA-vasculitis and lymphomatoid granulomatosis! Best,
- Hypophysitis (or pituitary inflammation) is most frequently associated with ACTH and TSH deficiency.
- Check out the paper on cavitary lung nodules: PMID18400799
Approach to Hypophysitis (Inflammation of Pituitary)
- Hypophysitis is often classified by histologic findings: lymphocytic, granulomatous, plasmacytic, and xanthomatous
- Clinical manifestations : headache out of proportion to size of lesion, hypopituitarism : preferential hypofunction of ACTH and TSH-secreting cells have been described, but DI, hyperprolactinemia, GH excess, and autoimmune thyroiditis can also occur.
- Eventually, progressive pituitary atrophy can occur with fibrosis.
||Most common form; often occurs in late pregnancy or post-partum period
||Majority are idiopathic, but known causative entities include GPA and TB
|Plasmacytic (IgG4-associated) Hypophysitis
||Often associated with infiltration of other organs, such as the pancreas
||Most rare, characterized by foamy histiocytes
Approach to Cavitary Lung Lesions
- Common bacterial infections: septic pulmonary emboli, necrotizing pneumonias, lung abscess
- Uncommon bacterial infections: actino, nocardia
- Mycobacteria: TB and NTB
- Fungal: aspergillosis, zygomycosis, histo, blasto, cocci, paracocci, crypto, PCP
- Parasites: echinococcus, paragonimiasis
- Rheum: vasculitis
- Other: PE with infarct, bullae/cysts, pulmonary sequestration
Evernote Link: https://www.evernote.com/shard/s338/sh/811bec00-0472-4001-b377-312981438543/93397a1e311670c8e2720d5ced8fe093
Thanks to Albert and Geoff for presenting an extremely compelling case of a young woman with 3 weeks of dyspnea on exertion found to have a large anterior mediastinal mass compressing her great vessels and a large pericardial effusion.
The incredible Sarah Schaeffer wrote amazing pearls the anterior mediastinal mass ddx that I’ll re-post here
The differential depends on whether the mass is in the anterior, middle, or posterior mediastinum
Anterior Mediastinal Mass – the terrible Ts + others
- Thymus – thymoma, cyst, hyperplasia, carcinoma
- (Terrible) Lymphoma
- Germ Cell Tumor – Teratoma, seminoma, non-seminomatous GCT (choriocarcinoma, etc)
- Intrathoracic Thyroid – substernal goiter, ectopic thyroid
- Others: Parathyroid adenoma, hemangioma, lipoma, liposarcoma, fibroma, fibrosarcoma, Foramen of Morgagni (diaphgragm) hernia
Middle Mediastinal Mass
- Bronchogenic cyst
- Pericardial cyst
- Lymphadenopathy – lymphoma, sarcoid, metastatic lung CA, etc
- Enteric cyst
- Esophageal tumors
- Vascular masses and enlargement
Posterior Mediastinal Mass
- Neurogenic tumors – neurofibroma, neurolemona, neurosarcoma, ganglioneuroma, ganglioneuroblastoma, neuroblastoma, chemodectoma, pheochromocytoma (among the 10% extra-adrenal)
- Thoracic spine lesions
Some additional nuggets
- Most Anterior mediastinal masses are malignant – 60% in one case series. Thymoma is the most common.
- In women hodgkins disease incidence peaks in the third decade of life. Based on this patient’s symptoms
Tissue is the issue
- Oncologists have drilled into us that tissue is critical. In anterior mediastinal masses, distinguishing between thymoma and lymphoma can be tricky
- In general options for biopsy are percutaneous (CT guided), Endobronchial (if abutting the airway), and surgical (via mediastinoscopy or open resection).
- Geoff taught us that if a thymoma capsule ruptures during biopsy, it can seed the mediastinum or, more catastrophically, the pleura. Pleural seeing makes a previously curable disease potentially incurable.
- at times, if a mass looks very thymoma-like, surgeons will proceed directly to open resection. The big downside of this is that if the disease is lymphoma, resection is not the appropriate treatment.
What on earth is mediastinoscopy? A surgical procedure. See below