Hepatopulmonary Syndrome (HPS) demonstrated on Echocardiogram Bubble Study Intra-cardiac shunt: Bubbles appear in

Hepatopulmonary Syndrome on Echocardiogram (A4C) Hepatopulmonary Syndrome: Intrapulmonary shunting is most commonly demonstrated by contrast TTE when bubbles from agitated saline are visualized in the left atrium within 3–6 beats after being noted in the right side of the heart. Any sooner is consistent with intracardiac shunt. Dr. Andy Tau, MD @DrBloodandGuts #Hepatopulmonary #Syndrome #Echocardiogram #A4C #bubble #shunting #hepatology #clinical

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Hepatopulmonary Syndrome - Echocardiogram (A4c) with bubble study showing delayed right-to-left shunt consistent with intrapulmonary shunt 68M with HCV induced cirrhosis with hepatopulmonary syndrome (clubbing and spider angiomata, 82% on RA when walking). Dr. Jeffrey Kanne @JeffreyKanneMD #Hepatopulmonary #Syndrome #Echocardiogram #bubble #shunting #intrapulmonary #cardiology #clinical #pocus #hepatology #a4c

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Hepatopulmonary Syndrome - Illness Script HPS is a pulmonary disorder of chronic liver disease characterized by a triad of: 1) chronic liver disease 2) pulmonary vascular dilatation & 3) gas exchange abnormalities PATHOPHYSIOLOGY: • Intrapulmonary capillary vasodilation leads to impaired arterial oxygenation through V/Q mismatch • Mismatch? Dilated vessels + absent tone = increased flow & longer distance for O2 to cross // overperfusion + decreased RBC transit time = increased Aa gradient • Intrapulmonary arteriovenous shunting causes mixed blood to pass through pleural and pulmonary a/v communications // a higher presence in the lower lung zones may be behind the mechanism of orthodeoxia CLINICAL FEATURES: • Progressive dyspnea • Platypnea - worsening dyspnea when moving from supiine to upright • Orthodeoxia - decrease in PaO ≥ 5% (or 4 mmHg) when moving from supine to upright • Stigmata of cirrhosis: jaundice, ascites, palmar erythema, spider angiomas, digital clubbing DIAGNOSIS - TRIAD: • Liver disease &/or portal HTN • Aa gradient ≥15 mmHg (room air) - ABG • Pulmonary vascular dilatations - contrast-enhanced TTE with agitated saline - Microbubbles elude capture and reach the left heart TREATMENT • No correlation between the presence or severity of HPS and the severity of liver disease (CPT. PELD. MELD) • In one large study, HPS mortality rate of ~12% • No effective medical therapies other than supplemental oxygen • Liver transplant is the only definitive treatment. Marked improvement or resolution of syndrome in 85% post-transplant By @Alert_Oriented3 #HPS #Hepatopulmonary #Syndrome #cirrhosis #diagnosis #hepatology

Hepatopulmonary Syndrome - Diagnosis and Management Summary A defect in arterial oxygenation due to a gas exchange disorder occurring in the setting of liver disease and in the absence of intrinsic lung disease Caused by intrapulmonary vascular dilation (IVPD) either due to a diffusion-perfusion defect (Type 1) or anatomic shunt (Type 2) Clinical Presentation: • Dyspnea - Common but not specific to HPS alone • Platypnea - Dyspnea worse when sitting upright compared to lying supine • Orthopnea - PaO2 ↓ by ≥ 5% or ≥ 4mmHg from supine to upright position • Hypoxemia - V/Q mismatch and restricted diffusion from IPVDs Diagnostic Testing: • Pulse oximetry - Non-invasive initial screening tool using cutoff SpO2 < 96% detects severe HPS with 100% sens, 88% spec • Contrast-enhanced echocardiography - Assess for IPVD using peripherally injected agitated saline during echocardiography - Microbubbles appear in left heart 3-6 cardiac cycles after right atrial passage • Arterial blood gas (ABC) analysis - Quantifies degree of hypoxemia. Should be performed on room air in seated position after 10 minutes of rest HPS Diagnostic Criteria: • Underlying liver disease - Portal hypertension with or without cirrhosis • Intra-pulmonary vascular dilation - Positive findings on contrast-enhanced TTE. Can also be seen on radioactive lung perfusion scanning or pulmonary angiography (in select patients) • Oxygenation defect - PaO2 < 80mmHg or A-a gradient ≥ 15mmHg (≥ 20mmHg in patients aged 65 and older) on room air Treatment: • There is no established medical therapy for HPS. Supportive care with supplemental oxygen if PaO2 < 60mmHg, goal SpO2 > 88% • Management of underlying portal hypertension • Liver transplantation: results in almost uniform resolution of HPS features - Complete resolution of symptoms may take months after transplant - Lizzie Aby, MD @LizzieAbyMD #hepatopulmonary #syndrome #diagnosis #management #treatment #hepatology

Large atrial septal defect on cardiac POCUS with an oddly satisfying bubble study. Eric Abrams, MD @eabramsMD #Bubble #Study #ASD #Atrial #SeptalDefect #Clinical #Cardiology #POCUS #Echocardiogram #A4C #Large

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Double Shunt on Echocardiogram What do you see on this saline contrast study, in a patient with end-stage liver disease and pulmonary hypertension? 1. Early transit of bubbles = PFO 2. Late appearance with replenishment via pulm veins = intrapulmonary. #2 c/w hepatopulmonary syndrome #1: Pulm HTN (portopulmonary or other) causing ⬆️ RA pressure and R➡️L shunt as RAP>LAP as atrial septum shifts left In addition to dilated and probably dysfunctional RV, R-L shunt in two phases, first likely due to stretched PFO or ASD, and later (after ~5 seconds) appears to be coming from PVeins (extra-cardiac shunt in cirrhosis). by David H. Wiener, MD @DavidWienerMD #DoubleShunt #Echocardiogram #clinical #pocus #a4c #intrapulmonary #cardiology #saline #contrast

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Negative Contrast Effect in Right Atrium - Differential Diagnosis 1. Small secundum ASD or PFO with left to right shunt 2. Flow from IVC hampering RA opacification by agitated saline 3. Inadequate injection causing heterogenous opacification 4. Prominent Chiari network altering flow in RA Dr. Akram Neyaz @DrAkramNeyaz #Negative #Contrast #Effect #Right #Atrium #RA #Differential #Diagnosis #cardiology #clinical #echocardiogram #s4c #pocus #bubble

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McConnell's Sign on POCUS Echocardiogram - PSAX Positive McConnell's Sign (hypokinetic RV with apical sparing) indicates acute right heart strain. This is highly specific for pulmonary embolism in the right setting, but can also be seen in RV infarct & acute chest syndrome. Dr. Meghan Herbst @EUSmkh #McConnells #Sign #POCUS #Echocardiogram #PSAX #clinical #cardiology #labeled

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Rheumatic Heart Disease - Mitral Stenosis on Echocardiogram (1) Thickened and fibrosed MV leaflets - rheumatic etiology - with severe MS. (2) Lvef 25-30%. (3) RV dysfunction also present. (4) All 4 cardiac chambers dilated. (5) Layered clot seen in LA by the LAA appears clean. (6) spontaneous echo contrast. (7) patient in atrial fibrillation :)) this patient should also be having a significant TR with severe PAH and clinically be in CHF @echocardiography_tips #Rheumatic #Mitral #Stenosis #Echocardiogram #clinical #cardiology #a4c #thrombus

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Atypical Takotsubo Syndrome - Basal Subtype Atypical Takotsubo Syndrome in patient requiring ECMO 1. 6% of MI have non-obstructive coronaries 2. Basal (apical sparing) TTS is rare (2.2%) 3. Pheochromotyoma in 30% of basal TTS Dr. Omid Kiamanesh @OKiamanesh #Takotsubo #Syndrome #Cardiomyopathy #Basal #Subtype #clinical #cardiology #pocus #echocardiogram

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