Zespół wątrobowo-płucny – Diagnostyka i diagnoza

Zespół wątrobowo-płucny
Diagnostyka i diagnoza

Zespół wątrobowo-płucny (ZWP) to powikłanie zaawansowanej choroby wątroby, charakteryzujące się triadą: chorobą wątroby i/lub nadciśnieniem wrotnym, wewnątrzpłucnym rozszerzeniem naczyń (IPVD) oraz hipoksemią. Diagnostyka opiera się na kryteriach gazometrycznych: PaO2 <80 mmHg lub gradient pęcherzykowo-tętniczy tlenu (A-aO2) ≥15 mmHg (≥20 mmHg u pacjentów >64 lat) podczas oddychania powietrzem atmosferycznym. Pulsoksymetria z saturacją <96% stanowi badanie przesiewowe, jednak jej czułość jest ograniczona. Ortodioksja (spadek PaO2 o ≥5% w pozycji pionowej) ma specyficzność 100% dla ZWP. Echokardiografia kontrastowa z agitated saline jest złotym standardem w wykrywaniu rozszerzenia naczyń płucnych, a scyntygrafia perfuzyjna z 99mTc-MAA pozwala na ilościową ocenę przecieku (frakcja przecieku mózgowego ≥6% jest istotna). Tomografia komputerowa i angiografia płucna mogą wspomagać diagnostykę, ale są mniej powszechne ze względu na inwazyjność i koszty. Ciężkość ZWP klasyfikuje się na podstawie PaO2: łagodny/umiarkowany ≥60 mmHg, ciężki 50-60 mmHg, bardzo ciężki <50 mmHg, co ma znaczenie prognostyczne i wpływa na kwalifikację do przeszczepu wątroby.

Wprowadzenie do zespołu wątrobowo-płucnego

Zespół wątrobowo-płucny (ZWP, ang. Hepatopulmonary syndrome, HPS) charakteryzuje się triadą objawów: nieprawidłową oksygenacją tętniczą wywołaną rozszerzeniem wewnątrzpłucnych naczyń krwionośnych występującą u pacjentów z chorobą wątroby, nadciśnieniem wrotnym lub wrodzonymi przetokami wrotno-systemowymi. Jest to poważne powikłanie zaawansowanej choroby wątroby, które istotnie wpływa na rokowanie i jakość życia pacjentów¹².

Częstość występowania ZWP wśród pacjentów z przewlekłą chorobą wątroby waha się od 4% do 47% (średnio około 25%), w zależności od przyjętych kryteriów diagnostycznych, zastosowanych metod i badanej populacji. W jednym z prospektywnych badań obejmujących 111 pacjentów z marskością wątroby, ZWP rozpoznano u 24% badanych przy użyciu echokardiografii kontrastowej, badania gazów krwi tętniczej i testów funkcji płuc¹².

Kryteria diagnostyczne ZWP

Diagnoza zespołu wątrobowo-płucnego opiera się na obecności trzech kluczowych elementów¹²³:

Choroba wątroby i/lub nadciśnienie wrotne
Wewnątrzpłucne rozszerzenie naczyń (IPVD – intrapulmonary vascular dilatation)
Zaburzenia wymiany gazowej prowadzące do hipoksemia/” title=”hipoksemia” class=”to-tag” data-termid=”27596″>hipoksemii

Kryteria diagnostyczne ZWP obejmują¹²³:

Ciśnienie parcjalne tlenu (PaO2) poniżej 80 mmHg podczas oddychania powietrzem atmosferycznym, LUB
Gradient pęcherzykowo-tętniczy tlenu (A-aO2) ≥15 mmHg podczas oddychania powietrzem atmosferycznym; u pacjentów powyżej 64 roku życia za diagnostyczny uznaje się gradient A-aO2 ≥20 mmHg (pomiar w pozycji siedzącej)

Badania przesiewowe w kierunku ZWP

Pulsoksymetria

Pulsoksymetria jest tanim, szybkim i bezbolesnym narzędziem do wstępnej oceny hipoksemii i badania przesiewowego w kierunku ZWP u pacjentów kwalifikowanych do przeszczepienia wątroby¹². Saturacja tlenu poniżej 96% świadczy o PaO2 poniżej 70 mmHg i jest uznawana za wynik dodatni w badaniu przesiewowym¹. U pacjentów z dodatnim wynikiem badania przesiewowego należy wykonać badanie gazometryczne krwi tętniczej, które pomaga określić PaO2 i gradient A-aO2².

Należy jednak pamiętać, że pulsoksymetria może nie wykryć wczesnych stadiów hipoksemii, a jej czułość w wykrywaniu ZWP u pacjentów ocenianych pod kątem przeszczepu wątroby jest ograniczona¹².

Ortodioksja

Ortodioksja, czyli zmniejszenie saturacji tlenowej po przyjęciu pozycji pionowej (lub ze zmiany pozycji leżącej do stojącej) ze spadkiem PaO2 o co najmniej 5%, może występować u pacjentów z ZWP. Specyficzność ortodioksji dla diagnozy ZWP wynosi 100%¹².

Diagnostyka zaburzeń wymiany gazowej

Badanie gazometryczne krwi tętniczej

Badanie gazów krwi tętniczej jest złotym standardem w potwierdzeniu ZWP i najczulszym testem w diagnostyce¹². Próbkę krwi pobiera się z tętnicy w celu pomiaru poziomu tlenu, dwutlenku węgla oraz równowagi pH¹.

Hipoksemia występuje w marskości wątroby, a różnica pęcherzykowo-tętnicza tlenu (A-aO2) ≥15 mmHg (lub ≥20 mmHg u osób w wieku 65 lat lub starszych) podczas oddychania powietrzem atmosferycznym może potwierdzić diagnozę¹.

Wpływ pozycji ciała na wyniki badań gazometrycznych

Pozycja pacjenta podczas pobierania próbek krwi do badania gazometrycznego ma istotny wpływ na wyniki. Częstość występowania ZWP różni się w zależności od zastosowanych kryteriów gazometrycznych i pozycji, w jakiej pobrano gazy¹².

W jednym z badań częstość występowania ZWP w pozycji leżącej i siedzącej wynosiła odpowiednio 27,8% i 23,2% (kryteria klasyczne), 34% i 25,3% (kryteria nowoczesne) oraz 22,2% i 19% (kryteria dostosowane do wieku)¹.

Uzyskiwanie pomiarów gazów krwi w pozycji leżącej i stosowanie nowoczesnych kryteriów jest bardziej czułe w diagnostyce ZWP. Jednakże badanie gazometryczne z pacjentem w pozycji siedzącej wykrywa większą liczbę przypadków ciężkich i bardzo ciężkich, co wspiera przeprowadzanie analizy gazów krwi tętniczej z pacjentem w pozycji siedzącej¹².

Diagnostyka wewnątrzpłucnego rozszerzenia naczyń

Echokardiografia kontrastowa

Echokardiografia kontrastowa z użyciem wzburzonego roztworu soli fizjologicznej (agitated saline) jest złotym standardem w diagnostyce rozszerzenia naczyń płucnych i najbardziej specyficznym testem dla ZWP¹²³.

W badaniu tym roztwór soli fizjologicznej jest wzburzany w celu wytworzenia mikropęcherzyków o średnicy 10 mikrometrów. Pojawienie się mikropęcherzyków w lewym przedsionku między 4. a 6. cyklem serca po ich pojawieniu się w prawym przedsionku wskazuje na rozszerzenie naczyń płucnych. Jeśli mikropęcherzyki pojawiają się po lewej stronie serca przed 3. cyklem serca, wskazuje to na przeciek wewnątrzsercowy¹².

Przezprzełykowe badanie echokardiograficzne (TEE) jest lepsze od przezklatkowego (TTE) w diagnozowaniu rozszerzenia naczyń płucnych i przecieków wewnątrzsercowych. Jednak to badanie jest inwazyjne i bardziej ryzykowne ze względu na możliwość wystąpienia żylaków przełyku u wielu pacjentów z marskością wątroby i nadciśnieniem wrotnym¹².

Scyntygrafia perfuzyjna płuc

Scyntygrafia perfuzyjna płuc z użyciem makroagregatu albuminy znakowanej technetem-99m (99mTc-MAA) jest kolejnym testem stosowanym do wykrywania rozszerzenia naczyń płucnych¹²³.

W normalnych warunkach cząsteczki 99mTc-MAA o średnicy 20-50 μm są zatrzymywane w kapilarach płucnych. Jednak w przypadku ZWP, ze względu na rozszerzenie naczyń płucnych, cząsteczki te mogą przechodzić przez płuca i być wykrywane w innych narządach, takich jak mózg, nerki czy wątroba¹².

Scyntygrafia pozwala na wykrycie wychwytu w narządach poza płucami, co umożliwia obliczenie frakcji przecieku. Frakcja przecieku mózgowego ≥6% jest uznawana za istotną¹. Badanie to nie jest jednak tak czułe jak echokardiografia kontrastowa¹².

99mTc-MAA jest bardziej specyficzne niż echokardiografia i może również ilościowo określić stopień przecieku¹. Dodatni wynik badania 99mTc-MAA z wychwytem znacznika w narządach pozapłucnych u pacjenta z marskością wątroby jest specyficzny dla ZWP¹.

Angiografia płucna

Angiografia płucna może diagnozować i rozróżniać między typem I i typem II ZWP¹². Jest to jednak badanie bardziej kosztowne i inwazyjne, dlatego nie jest preferowaną metodą diagnostyki¹².

W angiografii płucnej u pacjentów z ZWP można zaobserwować dyfuzyjnie drobną lub plamistą konfigurację naczyniową¹. Typ I ZWP charakteryzuje się obecnością przedwłośniczkowych rozszerzonych naczyń, które dobrze reagują na tlenoterapię, podczas gdy typ II charakteryzuje się małym rozszerzeniem naczyń i malformacjami tętniczo-żylnymi¹.

Dodatkowe badania obrazowe

Tomografia komputerowa

Tomografia komputerowa (TK) może bezpośrednio ukazać zmiany morfologiczne w naczyniach płucnych i ułatwić diagnozę ZWP na podstawie specyficznych objawów¹. Rekonstrukcje MIP (maximum intensity projection) lub grube przekroje lepiej obrazują nieprawidłowości naczyniowe niż HRCT (high-resolution computed tomography)¹.

Najlepszą wskazówką diagnostyczną w TK są rozszerzone tętnice obwodowe (2 razy większe niż sąsiadujące oskrzela). Stosunek ZWP wynosi typowo (2,0 ± 0,2)¹.

Zdjęcia rentgenowskie klatki piersiowej i TK często nie wykazują oznak ZWP, ale brak nieprawidłowości w tych badaniach nie wystarcza do wykluczenia tego zespołu¹.

Badania ultrasonograficzne i czynnościowe

Rutynowe badanie ultrasonograficzne serca (USG serca) może pośrednio diagnozować ZWP poprzez pomiar zmian w strukturze komór¹. Testy funkcji płuc (PFT) mogą wykazywać nieprawidłowości u pacjentów z ZWP, jednak brakuje im specyficzności¹².

Klasyfikacja ciężkości ZWP

Ciężkość ZWP jest definiowana na podstawie PaO2¹:

Stopień ciężkości	PaO2 (mmHg)
Łagodny do umiarkowanego	≥60
Ciężki	50-60
Bardzo ciężki	<50

Ciężkość ZWP jest ważnym czynnikiem prognostycznym determinującym przeżycie, optymalny moment i ryzyko związane z przeszczepieniem wątroby¹.

Diagnostyka różnicowa

W diagnostyce różnicowej ZWP należy uwzględnić¹²:

Nadciśnienie wrotno-płucne (PPH) – charakteryzuje się nadciśnieniem płucnym w kontekście nadciśnienia wrotnego
Dziedziczna telangiektazja krwotoczna (HHT) – znana również jako zespół Oslera-Webera-Rendu
Inne pierwotne choroby płuc, które mogą prowadzić do hipoksemii

Należy odróżnić ZWP od przecieku wewnątrzsercowego prawo-lewego¹. Kluczowym czynnikiem w diagnostyce ZWP jest wykluczenie innych przyczyn niż ZWP, które mogą być związane z marskością wątroby i hipoksemią¹.

Współistnienie ZWP z innymi chorobami układu oddechowego, które pogarszają wymianę gazową, może występować nawet w 30% przypadków. Jednak skojarzenie z chorobami śródmiąższowymi płuc (ILD) jest rzadko opisywane w literaturze¹.

Znaczenie wczesnej diagnostyki

Wczesna diagnoza ZWP ma kluczowe znaczenie dla zapobiegania wysokiej zachorowalności i śmiertelności związanej z tym stanem¹². Pacjenci z ZWP mają gorsze rokowanie niż osoby z przewlekłą chorobą wątroby bez ZWP¹.

Średni czas przeżycia po diagnozie ZWP wynosi 10,5 miesiąca w porównaniu z 40,8 miesiąca u osób z przewlekłą chorobą wątroby bez ZWP¹. Dla pacjentów z marskością wątroby i ZWP średni czas przeżycia wynosi dwa lata bez przeszczepu wątroby¹.

ZWP jest często późno lub błędnie diagnozowany. Od momentu wystąpienia objawów oddechowych do postawienia ostatecznej diagnozy upływa średnio 4,8 roku¹. Dlatego wszystkie ośrodki transplantacji wątroby powinny rutynowo badać pacjentów pod kątem ZWP¹.

Algorytm diagnostyczny

Zalecany algorytm diagnostyczny dla ZWP obejmuje¹²³:

Badanie przesiewowe za pomocą pulsoksymetrii u pacjentów z chorobą wątroby
Jeśli pulsoksymetria wykazuje saturację tlenu <96%, należy wykonać badanie gazometryczne krwi tętniczej
Ocena gradientu pęcherzykowo-tętniczego tlenu (A-aO2)
Wykonanie echokardiografii kontrastowej z użyciem wzburzonego roztworu soli fizjologicznej w celu wykrycia rozszerzenia naczyń płucnych
W razie potrzeby scyntygrafia perfuzyjna płuc z użyciem 99mTc-MAA w celu ilościowej oceny stopnia przecieku
Badania dodatkowe: TK klatki piersiowej, angiografia płucna, testy funkcji płuc

Ocena przedtransplantacyjna powinna obejmować badanie gazometryczne krwi tętniczej, echokardiografię przezklatkową z kontrastem, badanie gazometryczne krwi tętniczej po podaniu 100% tlenu oraz ocenę przecieku płucnego za pomocą scyntygrafii z makroagregatem albuminy¹.

Leczenie i rokowanie

Przeszczepienie wątroby jest jedyną znaną skuteczną terapią dla ZWP¹²³. Obecność ZWP jest wskazaniem do przeszczepu wątroby, ponieważ jest to jedyna terapia obecnie dostępna dla pacjentów ze znaczną hipoksemią¹.

Obecnie pacjenci z PaO2 ≤60 mmHg otrzymują wyższy priorytet do przeszczepu wątroby¹. Ustąpienie ZWP zazwyczaj następuje po przeszczepieniu wątroby, z 5-letnim przeżyciem 70% lub więcej¹.

Tlenoterapia jest jedynym dostępnym leczeniem objawowym dla ZWP, ale nie zmienia ona naturalnego przebiegu choroby¹. Spontaniczne ustąpienie ZWP bez leczenia jest mało prawdopodobne¹.

Podsumowanie

Zespół wątrobowo-płucny jest poważnym powikłaniem zaawansowanej choroby wątroby, charakteryzującym się nieprawidłową oksygenacją tętniczą wywołaną rozszerzeniem wewnątrzpłucnych naczyń krwionośnych. Wczesna diagnoza ma kluczowe znaczenie dla poprawy rokowania. Diagnostyka opiera się na wykryciu choroby wątroby, zaburzeń wymiany gazowej i rozszerzenia naczyń płucnych.

Podstawowe badania w diagnostyce ZWP obejmują badanie gazometryczne krwi tętniczej i echokardiografię kontrastową. Przeszczepienie wątroby pozostaje jedyną skuteczną metodą leczenia, z możliwością całkowitego ustąpienia objawów ZWP w ciągu roku po zabiegu.

Złożoność diagnostyki i leczenia pacjentów z ZWP wymaga silnego, interdyscyplinarnego podejścia zespołowego obejmującego hepatologów, pulmonologów, chirurgów transplantologów, specjalistów medycyny uzależnień, lekarzy podstawowej opieki zdrowotnej, farmaceutów, pracowników socjalnych i edukatorów pielęgniarskich.

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Materiały źródłowe

#1 Hepatopulmonary syndrome: What we know and what we would like to know
https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/
Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] The diagnosis requires taking an arterial blood gas sample of a seated patient with alveolar-arterial oxygen gradient (AaO2) 15 mm Hg, or 20 mm Hg in those over 64 years of age. […] The IPVD are identified through a transthoracic contrast echocardiography or a macroaggregated albumin lung perfusion scan (99mTc-MAA). […] The diagnostic criteria proposed for HPS are the presence of liver disease, an AaO2 15 mmHg or 20 mmHg in patients over 64 years old detected by arterial blood gas analysis in a seating position, and the demonstration of IPVD by means of a positive contrast-enhanced echocardiography.
#1 Hepatopulmonary syndrome in adults: Prevalence, causes, clinical manifestations, and diagnosis – UpToDate
https://www.uptodate.com/contents/hepatopulmonary-syndrome-in-adults-prevalence-causes-clinical-manifestations-and-diagnosis
Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVDs) in the setting of liver disease, portal hypertension, or congenital portosystemic shunts. […] The prevalence, causes, clinical manifestations, and diagnostic evaluation of HPS are reviewed here. […] Estimates of the prevalence of HPS among patients with chronic liver disease range from 4 to 47 percent (on average one quarter), depending upon the diagnostic criteria, methods used, and population studied. […] In one prospective study of 111 patients with cirrhosis, HPS was reported in 24 percent when transthoracic contrast echocardiography, blood gas analysis, and pulmonary function testing were used for its detection. […] In another prospective study of patients with liver cirrhosis, 26 percent had HPS by similar criteria. […] The diagnostic evaluation of HPS includes impaired oxygenation and intrapulmonary vascular dilatations, with shunt assessment performed through transthoracic contrast echocardiography and other methods.
#1 Hepatopulmonary Syndrome – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK562169/
Hepatopulmonary syndrome is hypoxemia due to dilated intrapulmonary vasculature in the presence of liver disease or portal hypertension. To avoid the high morbidity and mortality associated with this condition, it must be promptly diagnosed and treated. The diagnostic criteria for HPS include: Partial pressure of oxygen (PaO2): Less than 80 mm Hg while breathing room air, or alveolar-arterial oxygen gradient (A-aO2), 15 mm while breathing room air; in patients over 64, A-aO2 20 mm Hg is considered diagnostic (they should be resting in a seated position). Pulmonary vascular dilatation: As shown by positive contrast-enhanced echocardiography or by radioactive lung-perfusion scanning (showing brain shunt fraction 6%). Portal hypertension: With or without cirrhosis. The initial screening for HPS involves using a pulse oximeter to evaluate PaO2. An O2 saturation of less than 96% signifies PaO2 less than 70 mm Hg and is considered a positive screen. If the screen is positive, the patient should undergo arterial blood gas (ABG) analysis, which helps to determine PaO2 and A-aO2. Contrast-enhanced echocardiography with agitated saline is the gold standard for diagnosing pulmonary vascular dilatation. Normal saline is agitated to generate microbubbles 10 micrometers in diameter. The appearance of microbubbles in the left atria between the 4th and 6th cardiac cycle indicates pulmonary vasodilatation. If the microbubbles appear on the heart’s left side before the 3rd cardiac cycle, it shows intracardiac shunting. A transesophageal echocardiogram study is superior to transthoracic echocardiography in diagnosing pulmonary dilation and intracardiac shunting. However, this test is invasive and riskier due to esophageal varices in many patients with cirrhosis and portal hypertension. Radioactive lung perfusion scanning is another test to establish pulmonary vessel dilatation. However, it is not as sensitive as contrast-enhanced echocardiography. Scintigraphy can reveal uptake in other organs besides the lung, which allows the calculation of the shunt fraction. Brain shunt fraction 6% is considered significant. Pulmonary angiography can diagnose and distinguish between type I and type II HPS. However, it is a more expensive and invasive test, so it is not a preferred method of diagnosis. The management of patients with HPS requires a strong interprofessional team approach involving hepatologists, pulmonologists, transplant surgeons, addiction medicine specialists, primary clinicians, pharmacists, social workers, and nurse educators. Prompt diagnosis and early liver transplant are the only way to alter the course of this fatal disease.
#1 Hepatopulmonary syndrome: What we know and what we would like to know
https://www.wjgnet.com/1007-9327/full/v22/i25/5728.htm
A contrast echocardiography is a sensitive, qualitative and non-invasive method which allows the screening of IPVD, which are the main characteristic of HPS. […] The 99mTc-MAA is another technique which is capable of detecting the presence of IPVD. […] Pulse oximetry is a cheap, rapid and painless method of estimating arterial pO2. […] The available data on the symptoms and exploration findings in HPS patients is mainly based on those with cirrhosis and therefore should not be extrapolated to all HPS patients.
#1 Pulmonary Complications of Cirrhosis – Page 4
https://www.medscape.com/viewarticle/777530_4
Hypoxemia is common in patients with end-stage liver disease, with a prevalence that has been reported to be as high as 45% of carefully evaluated patients. When patients awaiting liver transplantation are assessed, 15%-30% have hepatopulmonary syndrome. […] The characteristics of hepatopulmonary syndrome include an oxygen saturation 80%, an increased alveolar-arterial gradient ( 15 mm Hg) while breathing room air, portal hypertension with or without cirrhosis, and dilation of intrapulmonary vessels. […] Pulse oximetry is a useful screening test, although it can underestimate disease in early hypoxemia. Orthodeoxia, an arterial desaturation that occurs upon assuming an upright posture (or from supine to standing) with a fall in PaO2 of 5%, can be demonstrated in these patients. Arterial blood gases are the test of choice for confirmation of hepatopulmonary syndrome. Hypoxemia occurs in cirrhosis, and evidence of an alveolar-arterial oxygen difference of 15 mm Hg (or 20 mm Hg in those 65 years of age or older) while breathing room air can establish the diagnosis.
#1 Hepatopulmonary syndrome
https://johnsonmemorial.org/jmh-health/disease-conditions/con-20373340
These tests can help find if you have hepatopulmonary syndrome: […] Arterial blood gas. For this test, blood is taken from an artery to measure levels of oxygen and carbon dioxide and the pH balance. […] Chest imaging. X-rays, CT scanning or echocardiogram imaging with a salt solution given through a vein, called a saline contrast study, can help rule out other heart or lung conditions. […] Pulse oximetry. In pulse oximetry, a sensor attached to the finger or ear uses light to see how much oxygen is in the blood.
#1 Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis?
http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017001200006
Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis? […] Different blood gas criteria have been used in the diagnosis of hepatopulmonary syndrome (HPS). […] The prevalence of HPS in the supine and seated position was 27.8% and 23.2% (classic), 34% and 25.3% (modern) and 22.2% and 19% (adjusted for age), respectively. […] Obtaining blood gas measurements in the supine position and the use of modern criteria are more sensitive for the diagnosis of HPS. […] Blood gas analysis with the patient seated detects a greater number of severe and very severe cases. […] The presence of HPS was not associated with an increase in mortality regardless of blood gas criterion used. […] A decreased blood oxygenation is demonstrated by performing an arterial blood gas analysis, although pulse oximetry is also used, especially in HPS screening.
#1 Evaluation of Hepatopulmonary Syndrome with 99mTc-Macroaggregated Albumin Scintigraphy | Journal of Nuclear Medicine Technology
https://tech.snmjournals.org/content/50/4/377
Hepatopulmonary syndrome is characterized by intrapulmonary vascular dilatation causing hypoxemia in patients with liver disease. 99mTc-macroaggregated albumin (99mTc-MAA) scintigraphy has diagnostic value in suspected hepatopulmonary syndrome by detecting a clinically significant right-to-left shunt. […] 99mTc-MAA scintigraphy also provides the added value of quantification of the shunt. […] An elevated alveolararterial gradient with a partial pressure of oxygen of less than 60, echocardiographic and scintigraphic evidence of intrapulmonary shunting, and no known chronic lung disease in the setting of cirrhosis were consistent with HPS in our patient. […] Hypoxemia can be distinguished from HPS and other etiologies through bubble echocardiography, with arrival of bubbles in the left heart at least 3 cardiac cycles after contrast appearance in the right heart, or through 99mTc-MAA scintigraphy showing uptake in the brain.
#1 SciELO Brazil – Hepatopulmonary syndrome: an update Hepatopulmonary syndrome: an update
https://www.scielo.br/j/spmj/a/VXYTNbsCXRD6VFCpVhFcccP/?lang=en
The anatomical substrate and main structural change stemming from HPS is pulmonary capillary dilatation. […] Based on the concept of the syndrome, diagnosis can be achieved through complementary examinations to prove the presence of IPVD and gas exchange abnormalities in patients with liver disease. […] The majority of studies suggest that arterial gas analysis is essential, beginning at the first consultation for cirrhotic patients who are candidates for liver transplantation. […] In the standardized diagnosis, two methods may be used to confirm the presence of IPVD: contrast echocardiography and lung perfusion scintigraphy using macroaggregated albumin labeled with technetium-99m (99mTcMAA). […] Contrast echocardiography is the preferred method for diagnosing IPVD because of its greater sensitivity, in comparison with 99mTcMAA, and its ability to rule out intra-heart communication.
#1 Evaluation of Hepatopulmonary Syndrome with 99mTc-Macroaggregated Albumin Scintigraphy | Journal of Nuclear Medicine Technology
https://tech.snmjournals.org/content/50/4/377
99mTc-MAA scintigraphy is more specific than echocardiography and also can quantify and measure the degree of the shunt. […] A positive 99mTc-MAA scan with tracer uptake in extrapulmonary organs in a cirrhotic patient is specific for HPS. 99mTc-MAA scintigraphy also quantifies the extent of a shunt.
#1 Hepatopulmonary syndrome – Wikipedia
https://en.wikipedia.org/wiki/Hepatopulmonary_syndrome
The hepatopulmonary syndrome is suspected in any patient with known liver disease who reports dyspnea (particularly platypnea). […] Patients with clinically significant symptoms should undergo pulse oximetry. […] Hepatopulmonary syndrome (HPS) consists of the triad of liver dysfunction, otherwise unexplained hypoxemia, and intrapulmonary vascular dilation (IPVD). […] A useful diagnostic test is contrast echocardiography. […] Pulmonary angiography may reveal diffusely fine or blotchy vascular configuration. […] The distinction has to be made with an intracardiac right-to-left shunt.
#1 Hepatopulmonary Syndrome: Oxidative Stress and Physical Exercise – European Medical Journal
https://www.emjreviews.com/hepatology/article/hepatopulmonary-syndrome-oxidative-stress-and-physical-exercise/
Hepatopulmonary syndrome (HPS) may be defined by hepatic disease, gas exchange abnormalities that may lead to hypoxaemia, and the presence of pulmonary vascular dilations. […] The diagnosis is made when the AaDO2 is 20 mmHg or when there is hypoxaemia, which is defined by a partial pressure of oxygen (PaO2) 70 mmHg. Intravascular lung dilations may be present, which facilitates confirmation of the diagnosis made by an echocardiogram. […] Liver disease can contribute to HPS, where oxidative stress is one of the main factors responsible for the reduction of gas exchange, and physical exercise can be performed as a way of modifying this pathophysiological state. […] HPS may be classified into two subtypes based on the PaO2 and angiograph findings. HPS type I is defined by the presence of precapillary diffuse dilations that respond well to oxygen therapy. HPS Type II is characterised by small vasodilatation and arteriovenous malformation. […] The clinical features of intrapulmonary vasodilation and gas exchange abnormalities are better understood when investigated in combination with the antioxidant system, inflammatory processes associated with bacterial translocation, and the presence of portosystemic and intrapulmonary shunts.
#1 Frontiers | Advances in Diagnostic Imaging of Hepatopulmonary Syndrome
https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.817758/full
Pulmonary angiography can directly show the changes in the pulmonary vessels with high sensitivity and specificity, but it is more traumatic, expensive, and limited in application. CT can directly show the morphological changes in the pulmonary vessels, and facilitate the diagnosis of HPS based on specific signs. […] Overall, the progress of research on the diagnostic imaging of HPS has been relatively slow. In the future, there is a need to increase the clinical and basic research on HPS to further deepen the understanding of its pathogenesis and investigate new examination techniques to improve diagnostic efficiency.
#1 Hepatopulmonary Syndrome | Radiology Key
https://radiologykey.com/hepatopulmonary-syndrome/
Hepatopulmonary Syndrome Jud W. Gurney, MD, FACR Key Facts Terminology […] Best imaging finding: Dilated peripheral arteries on CT […] MIP reconstructions or thick sections better depict vascular abnormality than HRCT […] No correlation between hypoxemia in HPS and severity of liver disease (Child classification) […] Dyspnea presenting symptom in 20% […] Best diagnostic clue: Dilated peripheral arteries (2x larger than adjacent bronchi) on CT […] HPS ratio typically (2.0 0.2) […] Peripheral arteries dilated (artery larger than accompanying bronchus) […] Macroaggregated albumin bypasses lungs and results in systemic activity in brain and kidneys.
#1 Hepatopulmonary Syndrome: Symptoms, Causes, Treatment, and Outlook
https://www.healthline.com/health/hepatopulmonary-syndrome
Chest X-rays and CT scans often dont show signs of hepatopulmonary syndrome, but a lack of findings from these tests isnt enough to rule it out. […] Doctors use a variety of noninvasive tests to diagnose hepatopulmonary syndrome. […] Hepatopulmonary syndrome is often a late or missed diagnosis. It takes people an average of 4.8 years from the time respiratory symptoms start to receive a definitive diagnosis. […] Liver transplant is the only known effective treatment for hepatopulmonary syndrome. […] Hepatopulmonary syndrome worsens the outlook for people with the underlying liver disease. […] The average survival time after hepatopulmonary syndrome diagnosis is 10.5 months compared with 40.8 months for people with chronic liver disease without hepatopulmonary syndrome. […] Hepatopulmonary syndrome only develops in people with liver disease. About 18% of people develop symptoms of hepatopulmonary syndrome before symptoms of liver disease.
#1 Frontiers | Advances in Diagnostic Imaging of Hepatopulmonary Syndrome
https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.817758/full
The diagnostic criteria for HPS: (1) presence of liver disease, (2) evidence of intrapulmonary vascular dilatation (IPVD), which is also the basic pathological change, and (3) a pulmonary bubble arterial oxygen differential pressure [P [A-a] O2 gradient] that is â¥15 mmHg (or >20 mmHg if age â¥65 years). […] The focus of clinical diagnosis is to identify evidence of PV in patients with chronic liver disease complicated with hypoxemia. Therefore, clinical assessment must be combined with the results of imaging examination. […] Routine cardiac B-ultrasound can indirectly diagnose HPS by measuring the changes in the ventricular structure. At present, contrast echocardiography and Tc-MAA dynamic lung perfusion imaging can indirectly indicate the existence of an intrapulmonary right to left shunt.
#1 Hepatopulmonary syndrome – Past to present | Annals of Hepatology
https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-hepatopulmonary-syndrome-past-present-S1665268119319180
While abnormal pulmonary function studies are frequently observed in HPS, these lack specificity. […] A pulmonary arteriography study in patients with HPS reveal two types of vascular patterns, type I or diffuse pattern and the type II or focal pattern. […] High-resolution chest computerized tomography (CT) and evaluation of pulmonary blood transit time are newer diagnostic modalities for assessing HPS.
#1 Hepatopulmonary syndrome differential diagnosis – wikidoc
https://www.wikidoc.org/index.php/Hepatopulmonary_syndrome_differential_diagnosis
Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). […] On the basis pulse oximetry, arterial blood gas (ABG) analysis, contrast enhanced echocardiography, 99mTc scan (lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin), chest CT scan, pulmonary angiography, and pulmonary function test, hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). […] Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). […] Severity of HPS is defined based on PaO2, while below 50 is extremely sever, 50-60 is sever, and more than 60 is defined as moderate to mild.
#1 SciELO Brazil – The hepatopulmonary syndrome The hepatopulmonary syndrome
https://www.scielo.br/j/abcd/a/GPzm8Rj8YvWQWpHJG7xzSyQ/
The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. […] Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (15 mmHg, or 20 mmHg in patients 64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. […] HPS diagnosis depends initially on the presence of liver disease or portal hypertension, an elevated age-adjusted alveolar-arterial oxygen gradient (AaPO2), and evidence of intrapulmonary vasodilatation. […] The arterial blood gas reveals an elevated age-adjusted AaPO2 with or without hypoxemia. […] The transthoracic microbubble contrast echocardiography is the preferred screening test for intrapulmonary vasodilatation. […] Liver transplantation is the only effective established therapy for HPS. […] The severity of HPS is an important predictor to determine survival, the better moment and the risks for liver transplantation.
#1 Hepatopulmonary syndrome – Past to present | Annals of Hepatology
https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-hepatopulmonary-syndrome-past-present-S1665268119319180
Hepatopulmonary syndrome (HPS) is the one of the complication of liver cirrhosis with portal hypertension, irrespective of etiology, age and sex. […] Contrast echocardiogram is the better screening tool to demonstrate intrapulmonary shunt. […] There is no specific clinical finding to diagnose but digital clubbing, cyanosis, dyspnoea, platypnoea, and spider naevi are more common among cirrhosis with HPS. […] A key factor in the diagnosis of HPS is the exclusion of causes other than HPS that may be involved in cirrhosis presenting with hypoxemia. […] The P (A-a) O2 seems to be an ideal parameter for the assessment of abnormalities of arterial oxygenation in HPS. […] Contrast enhanced echocardiography is the preferred screening test for HPS. […] In order to overcome the disadvantages of contrast echocardiography, 99mTechnetium macroaggregated albumin (Tc-99m MAA) lung perfusion scan is used to diagnose HPS.
#1 Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination | Archivos de BronconeumologÃa
https://www.archbronconeumol.org/en-hepatopulmonary-syndrome-diffuse-interstitial-lung-articulo-S1579212918303069
Hepatopulmonary syndrome (HPS) is characterized by a triad of impaired in arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease. The most common respiratory symptom is progressive dyspnea. Platypnea-orthodeoxia (increased dyspnea and reduced oxygen saturation in an orthostatic position) can occur in up to 25% of patients. Other signs suggestive of HPS are telangiectasia, nail clubbing, and peripheral cyanosis. Echocardiography with agitated saline contrast is the method most widely used for detecting and confirming IPVD. Liver transplantation (LT) is the current treatment of choice, since medical options have not demonstrated effectiveness. HPS has been reported to coexist with other respiratory diseases that worsen gas exchange in up to 30% of cases. However, the association with diffuse interstitial pulmonary disease (ILD) has been rarely reported in the literature. HPS in a patient with ILD complicates the diagnostic process and may limit the treatment options for both entities.
#1 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/24190-hepatopulmonary-syndrome
If your pulse oximetry test shows low oxygen saturation, they’ll follow up with a more specific test called an arterial blood gas (ABG). […] Contrast echocardiography is the radiology method of choice for diagnosing dilated pulmonary veins. […] Supplemental oxygen is the only available treatment for hepatopulmonary syndrome. […] Liver transplantation is the only known cure for hepatopulmonary syndrome. […] If you have hepatopulmonary syndrome, you probably already have chronic liver disease. […] For those with cirrhosis and hepatopulmonary syndrome, the average is two years without a liver transplant. […] Healthcare providers understand that hepatopulmonary syndrome diminishes your overall prognosis and quality of life but also that a liver transplant can cure it.
#1
https://www.xiahepublishing.com/2310-8819/JCTH-2015-00044
A severe and common pulmonary vascular complication of liver disease is hepatopulmonary syndrome (HPS). Prevalence varies according to various study groups from 4% to 47%. While the most common presenting symptom of HPS is dyspnea, it is usually asymptomatic, and thus all liver transplant candidates should be screened for its presence. Pulse oximetry is a useful screening method, but arterial blood gas examination is the gold standard. If there is an abnormal P (A-a)O2 gradient, microbubble transthoracic echocardiography should be done for diagnosis. HPS diagnosis consists of a triad: the presence of liver disease and/or portal hypertension, elevated room air alveolar-arterial oxygen gradient [P(A-a)O2 gradient] 15 mmHg or 20 mmHg when age 65 years], and evidence of intrapulmonary vascular dilation (IPVD), especially in the basal parts of the lungs. Microbubble transthoracic echocardiography (MTTE), nuclear lung scanning, and rarely pulmonary angiography and high-resolution computed tomography (CT) can be used for detection of IPVD. MTTE is considered for the diagnosis of HPS. HPS is usually asymptomatic, so all liver transplant centers should routinely screen patients for HPS. The only known effective therapy for HPS is LT, with an improvement in oxygenation at 1 year. Complete resolution of HPS after LT is usually seen within a year in most HPS patients.
#1 Pulmonary Complications of Cirrhosis – Page 4
https://www.medscape.com/viewarticle/777530_4
Contrast-enhanced echocardiography can provide a qualitative estimate of shunting following injection of microbubbles into the venous system. […] Ventilation-perfusion lung scanning using macroaggregated albumin can be a quantitative measure of right-to-left shunting because these macroaggregates are typically trapped within the lung vasculature and do not reach the systemic circulation. […] The presence of hepatopulmonary syndrome is an indication for liver transplantation because it is the only therapy currently available to patients with significant hypoxemia. The pretransplant evaluation should include arterial blood gases, contrast-enhanced transthoracic echocardiography, arterial blood gases after 100% oxygen administration, and estimation of pulmonary shunting using macroaggregated albumin scanning. […] Currently, patients with a PaO2 60 mm Hg are granted higher priority for liver transplantation. Resolution of hepatopulmonary syndrome generally follows liver transplantation, with a 5-year survival of 70% or more.
#1 Hepatopulmonary syndrome in adults: Natural history, treatment, and outcomes – UpToDate
https://www.uptodate.com/contents/hepatopulmonary-syndrome-in-adults-natural-history-treatment-and-outcomes
Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations in the setting of liver disease, portal hypertension, or congenital portosystemic shunts. […] The epidemiology, pathophysiology, clinical manifestations, and diagnostic evaluation are discussed separately. (See „Hepatopulmonary syndrome in adults: Prevalence, causes, clinical manifestations, and diagnosis”.) […] Spontaneous resolution of HPS without treatment is unlikely.
#2 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/24190-hepatopulmonary-syndrome
Hepatopulmonary syndrome occurs in a small percentage of people with liver disease and/or portal hypertension. It causes the capillaries in your lungs to expand, which affects their ability to transfer oxygen into your blood. This causes hypoxemia (low blood oxygen levels). […] Hepatopulmonary syndrome is a rare complication of liver disease that causes low oxygen levels in your blood (hypoxemia). […] Hepatopulmonary syndrome occurs in an estimated 25% of people with chronic liver disease. […] Healthcare providers recognize hepatopulmonary syndrome by a triad of features that appear together: Portal hypertension, usually in the setting of liver disease; Dilation of the veins entering your lungs (intrapulmonary vascular dilatations); Low blood oxygen (hypoxemia). […] Most of the time, you’ll already be diagnosed with liver disease and/or portal hypertension when you develop hepatopulmonary syndrome.
#2 Hepatopulmonary syndrome: What we know and what we would like to know
https://www.wjgnet.com/1007-9327/full/v22/i25/5728.htm
Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] The diagnosis requires taking an arterial blood gas sample of a seated patient with alveolar-arterial oxygen gradient (AaO2) 15 mm Hg, or 20 mm Hg in those over 64 years of age. […] The IPVD are identified through a transthoracic contrast echocardiography or a macroaggregated albumin lung perfusion scan (99mTc-MAA). […] The diagnostic criteria proposed for HPS are the presence of liver disease, an AaO2 15 mmHg or 20 mmHg in patients over 64 years old detected by arterial blood gas analysis in a seating position, and the demonstration of IPVD by means of a positive contrast-enhanced echocardiography.
#2 Hepatopulmonary syndrome: What we know and what we would like to know
https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/
Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. […] The diagnosis requires taking an arterial blood gas sample of a seated patient with alveolar-arterial oxygen gradient (AaO2) 15 mm Hg, or 20 mm Hg in those over 64 years of age. […] The IPVD are identified through a transthoracic contrast echocardiography or a macroaggregated albumin lung perfusion scan (99mTc-MAA). […] The diagnostic criteria proposed for HPS are the presence of liver disease, an AaO2 15 mmHg or 20 mmHg in patients over 64 years old detected by arterial blood gas analysis in a seating position, and the demonstration of IPVD by means of a positive contrast-enhanced echocardiography.
#2 Hepatopulmonary Syndrome – Pulmonary Disorders – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/hepatopulmonary-syndrome
Hepatopulmonary syndrome should be suspected in patients with known liver disease who report dyspnea (particularly platypnea). Patients with such symptoms should have pulse oximetry. […] If the symptoms are severe (eg, dyspnea at rest), arterial blood gases should be measured with the patient breathing room air and 100% oxygen to determine shunt fraction. […] A useful diagnostic test is contrast echocardiography. Intravenous microbubbles from agitated saline that are normally trapped in the pulmonary capillaries rapidly (ie, within 7 heartbeats) traverse the lung and appear in the left atrium. Similarly, IV technetium-99mlabeled albumin may traverse the lungs and appear in the kidneys and brain. […] If the diagnosis is suspected, do pulse oximetry and consider arterial blood gas measurement and imaging (eg, contrast echocardiography).
#2 Hepatopulmonary Syndrome: Symptoms, Causes, Treatment, and Outlook
https://www.healthline.com/health/hepatopulmonary-syndrome
Hepatopulmonary syndrome is a complication of liver disease that occurs when blood vessels in your lungs expand. This expansion of the blood vessels can interfere with your lungs ability to transfer oxygen to your red blood cells. […] Hepatopulmonary syndrome is usually considered a rare condition because it only affects people with liver disease, but its relatively common among people with chronic liver disease. […] Studies suggest that 5% to 32% of people with cirrhosis in liver transplantation centers have hepatopulmonary syndrome. […] Doctors may suspect that you have hepatopulmonary syndrome if you have unexplained oxygen saturation under 96%. Oxygen saturation is a measure of how much oxygen your red blood cells are carrying. […] An oxygen saturation under 96% is particularly suggestive of hepatopulmonary syndrome if you also have one of the following: platypnea, finger or toe clubbing, cyanosis.
#2 Advance in diagnosis and treatment of hepatopulmonary syndrome | Articles | New Medicine
https://yxxz.whuznhmedj.com/en/journal/6294.html
Hepatopulmonary syndrome (HPS) is a common pulmonary complication of advanced liver disease. The early diagnosis rate of HPS is low, and the treatment is limited, which seriously affects the survival rate and quality of life of patients. […] In this article, we summary pathogenesis, clinical manifestations, diagnosis and treatment progress of HPS, in order to improve the clinical understanding and diagnosis level of HPS. […] Pulse oximetry is insufficient for timely diagnosis of hepatopulmonary syndrome in children with liver cirrhosis. […] Pulse oximetry is insensitive for detection of hepatopulmonary syndrome in patients evaluated for liver transplantation. […] Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. […] Technetium-99m-labeled macroaggregated albumin lung perfusion scan for diagnosis of hepatopulmonary syndrome: a prospective study comparing brain uptake and whole-body uptake.
#2
https://www.tropicalgastro.com/articles/32/1/hepatopulmonary-syndrome-prevalence.html
Those presenting with intrapulmonary arterial dilation but no other two criteria (arterial blood hypoxemia) were defined as subclinical hepatopulmonary cases. […] The mean (orthodeoxia) DSaO2 in the HPS patients was 4.1 1.8 compared to non-HPS patients, who showed no change (p=0.001). […] PaO2 and Aa O2 were most sensitive in diagnosis of HPS. […] There is a relationship of HPS with the severity of cirrhosis by Childs grading and MELD score.
#2 Hepatopulmonary syndrome diagnostic study of choice – wikidoc
https://www.wikidoc.org/index.php/Hepatopulmonary_syndrome_diagnostic_study_of_choice
Among the patients who present with clinical signs of hepatopulmonary syndrome, the atrial blood gas analysis is the most sensitive test for diagnosis. […] The following findings are confirmatory for hepatopulmonary syndrome: Presence of bubbles on the left heart in contrast-enhanced transthoracic echocardiography with agitated saline, Presence of radioactivity on the left heart in Technetium 99m-labeled macroaggregated albumin scanning, Diffusion defect in atrial blood gas analysis particularly studying, Abnormal liver function studies, Abnormal pulmonary function test.
#2 Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis?
http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017001200006
Blood gas criteria with different cutoffs and assessment using both the PaO2 and A-a PO2 methods have been used for the diagnosis of HPS. […] The position of the patient when obtaining blood gas levels has also changed; it is often performed in both the supine and seated/upright position. […] The objectives of this study were: a) to analyze the effects of using different blood gas criteria and the position in which arterial blood gases are obtained (supine or seated/upright) in estimating HPS prevalence; and b) to evaluate the influence of the presence of HPS (with different criteria) in pre- and post-LT survival. […] The prevalence of HPS according to the blood gas criteria used for diagnosis and the position in which the gases are obtained are shown in table 1. […] The severity distribution of patients diagnosed with HPS was different depending on the position in which the blood gas analysis was obtained.
#2 Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis?
http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017001200006
The proportion of severe and very severe cases increased when arterial blood gas analysis was obtained when the patient was seated. […] This supports the implementation of arterial blood gases analysis with the patient seated, as more severe and very severe cases are detected in this way. […] In this study we did not observe a lower survival rate in HPS patients either on the active liver transplant waiting list (pre-LT) or post-LT, regardless of the criteria used and without adding exceptional MELD points in HPS patients. […] In conclusion, we found that the position at which arterial blood gas analysis and blood gas criteria are used for the diagnosis of HPS influences the prevalence and distribution of the severity.
#2 Review article: Update on current and emergent data on hepatopulmonary syndrome
https://www.wjgnet.com/1007-9327/full/v24/i12/1285.htm
Hepatopulmonary syndrome (HPS) is a frequent pulmonary complication of end-stage liver disease, characterized by impaired arterial oxygenation induced by intrapulmonary vascular dilatation. […] A classification of HPS according to the severity of hypoxemia has been suggested. Contrast-enhanced echocardiography represents the gold standard method for the detection of intrapulmonary vascular dilatations which is required, in combination with an elevated alveolar arterial gradient to set the diagnosis. […] The revised diagnostic criteria for HPS comprise the triad of chronic liver disease, pulmonary vascular dilatation and gas exchange abnormalities in the absence of other causes of impaired pulmonary function. […] The presence of intrapulmonary dilatations can be assessed by several methods, but contrast-enhanced echocardiography with agitated saline is considered the gold standard technique.
#2 What Is Hepatopulmonary Syndrome?
https://www.icliniq.com/articles/respiratory-health/hepatopulmonary-syndrome
2-D transthoracic agitated saline contrast echocardiography test is used to identify intrapulmonary vascular dilation. […] Macroaggregated Albumin Lung Perfusion Scan- A technetium -99 labeled macro aggregated albumin total body scan (MAA scan) is helpful to evaluate the extent to which hepatopulmonary syndrome is contributing to a patient’s oxygenation abnormalities. […] Computed Tomography (CT) of the Chest- This test is carried out to rule out the other abnormalities involved in causing abnormal oxygenation. […] The diagnosis includes:- Medical History and Physical Examination: History of liver disease or transplantation. Examination for fever, jaundice, and oxygen levels.- Imaging studies: Chest X-ray and CT scan to assess lung abnormalities and check pulmonary hypertension. […] Contrast-enhanced echocardiography with agitated saline is the gold standard for diagnosing pulmonary vascular dilation. […] Pulmonary angiography is also used to diagnose and distinguish type I and II hepatopulmonary syndromes.
#2 Hepatopulmonary Syndrome – MD Searchlight
https://mdsearchlight.com/gut-health/hepatopulmonary-syndrome/
Hepatopulmonary syndrome (HPS) is a condition related to serious liver disease or high blood pressure in the vein leading to the liver. It is characterized by enlarged blood vessels in the lungs, which reduces the levels of oxygen in the blood. […] To assess if a patient has Hepatopulmonary Syndrome or HPS, doctors use several evaluation methods. […] The types of tests that are needed for Hepatopulmonary Syndrome (HPS) include: 1. Pulse oximeter: This device measures the oxygen levels in the blood. 2. Contrast-enhanced echocardiography with agitated saline: This involves injecting bubbles into a vein and performing an ultrasound to assess abnormal blood flow. 3. Transesophageal echocardiogram study: This provides a detailed look at the heart and its blood flow, but is more invasive. 4. Radioactive lung perfusion scanning: This involves injecting radiolabeled particles into a vein to observe blood flow in the lungs. 5. Pulmonary angiography: This can differentiate between different types of HPS, but is invasive and costly. 6. Chest x-rays, computerized tomography (CT), and pulmonary function tests: These tests can also be useful for diagnosis and assessment of HPS.
#2 Hepatopulmonary syndrome: What we know and what we would like to know
https://pmc.ncbi.nlm.nih.gov/articles/PMC4932208/
A contrast echocardiography is a sensitive, qualitative and non-invasive method which allows the screening of IPVD, which are the main characteristic of HPS. […] The 99mTc-MAA is another technique which is capable of detecting the presence of IPVD. […] The available data on the symptoms and exploration findings in HPS patients is mainly based on those with cirrhosis and therefore should not be extrapolated to all HPS patients. […] In terms of mortality rates for those on the active transplantation list, in a prospective study, no differences were observed between patients who had HPS and those who did not. […] When evaluating a patient listed for LT, it is recommended that they are tested for the presence of HPS.
#2 Hepatopulmonary syndrome | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/hepatopulmonary-syndrome?lang=us
Hepatopulmonary syndrome (HPS) is a clinical syndrome defined by the presence of the following: […] abnormalities in oxygenation […] elevation in the alveolar-arterial (A-a) oxygen gradient with or without hypoxemia on room air. […] Contrast-enhanced transthoracic echocardiography using agitated saline is a sensitive means of diagnosis, and will demonstrate passage of microbubbles into the left atrium within 3-6 cardiac cycles from opacification of the right atrium. […] A Tc-99m MAA (micro-aggregated albumin) lung scan is a useful method to detect intrapulmonary vascular dilatation.
#2 Pulmonary Complications of Cirrhosis – Page 4
https://www.medscape.com/viewarticle/777530_4
Contrast-enhanced echocardiography can provide a qualitative estimate of shunting following injection of microbubbles into the venous system. […] Ventilation-perfusion lung scanning using macroaggregated albumin can be a quantitative measure of right-to-left shunting because these macroaggregates are typically trapped within the lung vasculature and do not reach the systemic circulation. […] The presence of hepatopulmonary syndrome is an indication for liver transplantation because it is the only therapy currently available to patients with significant hypoxemia. The pretransplant evaluation should include arterial blood gases, contrast-enhanced transthoracic echocardiography, arterial blood gases after 100% oxygen administration, and estimation of pulmonary shunting using macroaggregated albumin scanning. […] Currently, patients with a PaO2 60 mm Hg are granted higher priority for liver transplantation. Resolution of hepatopulmonary syndrome generally follows liver transplantation, with a 5-year survival of 70% or more.
#2 Frontiers | Advances in Diagnostic Imaging of Hepatopulmonary Syndrome
https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.817758/full
Pulmonary angiography can directly show the changes in the pulmonary vessels with high sensitivity and specificity, but it is more traumatic, expensive, and limited in application. CT can directly show the morphological changes in the pulmonary vessels, and facilitate the diagnosis of HPS based on specific signs. […] Overall, the progress of research on the diagnostic imaging of HPS has been relatively slow. In the future, there is a need to increase the clinical and basic research on HPS to further deepen the understanding of its pathogenesis and investigate new examination techniques to improve diagnostic efficiency.
#2 Hepatopulmonary Syndrome Diagnosis Guide – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group
https://www.acibademhealthpoint.com/hepatopulmonary-syndrome-diagnosis-guide/
Another is the arterial blood gas (ABG) test. It checks oxygen and carbon dioxide in the blood. If oxygen is low, it might mean HPS is causing breathing problems. […] PFTs are important too. They show how well the lungs work. If your PFTs are not good, this could mean you have HPS. […] Arterial blood gas (ABG) testing is key in diagnosing hepatopulmonary syndrome. It gives key info on oxygen levels in the blood. Healthcare workers use this info to check for the syndrome and its seriousness. […] Ultrasound imaging is key in spotting hepatopulmonary syndrome (HPS). Itâs a noninvasive way to show intrapulmonary vascular dilatations (IPVDs), a key sign of HPS. This makes it a must-have for healthcare pros to confirm a HPS diagnosis. […] Finding out if someone has hepatopulmonary syndrome (HPS) needs many medical tests. We use these tests together to understand if HPS is there and how bad it is.
#2
https://www.bjbms.org/ojs/index.php/bjbms/article/view/2020
Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet. Currently, the treatment of choice for HPS is orthotopic liver transplantation. Non-specific clinical criteria and the lack of standardized diagnostic criteria for determining HPS can lead to diagnostic errors. […] Portopulmonary hypertension and hereditary hemorrhagic telangiectasia, also known as OslerWeberRendu syndrome, are pulmonary complications of liver disease which should be differentially diagnosed from HPS.
#2
https://journals.lww.com/ajg/fulltext/2023/10001/s3882_hepatopulmonary_syndrome.4109.aspx
Hepatopulmonary syndrome (HPS) is an oxygenation defect caused by the development of intrapulmonary vascular dilation in the setting of advanced liver disease. […] To avoid high mortality and morbidity, diagnosing and treating promptly is critical. […] The diagnosis and treatment of HPS highlight the role of the interprofessional team approach in care for these complicated patients. HPS diagnosis requires oxygenation defect, intrapulmonary vascular dilatation, and liver disease (with or without portal hypertension). […] The only successful treatment for HPS is a liver transplant.
#2 Pulmonary Complications of Cirrhosis – Page 4
https://www.medscape.com/viewarticle/777530_4
Hypoxemia is common in patients with end-stage liver disease, with a prevalence that has been reported to be as high as 45% of carefully evaluated patients. When patients awaiting liver transplantation are assessed, 15%-30% have hepatopulmonary syndrome. […] The characteristics of hepatopulmonary syndrome include an oxygen saturation 80%, an increased alveolar-arterial gradient ( 15 mm Hg) while breathing room air, portal hypertension with or without cirrhosis, and dilation of intrapulmonary vessels. […] Pulse oximetry is a useful screening test, although it can underestimate disease in early hypoxemia. Orthodeoxia, an arterial desaturation that occurs upon assuming an upright posture (or from supine to standing) with a fall in PaO2 of 5%, can be demonstrated in these patients. Arterial blood gases are the test of choice for confirmation of hepatopulmonary syndrome. Hypoxemia occurs in cirrhosis, and evidence of an alveolar-arterial oxygen difference of 15 mm Hg (or 20 mm Hg in those 65 years of age or older) while breathing room air can establish the diagnosis.
#2 Hepatopulmonary Syndrome – OpenAnesthesia
https://www.openanesthesia.org/keywords/hepatopulmonary-syndrome/
Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations in the setting of portal hypertension, leading to right-to-left shunts and hypoxia. […] Diagnosis requires the presence of the following: portal hypertension with or without cirrhosis or end-stage liver disease; oxygenation defect; PaO2 80 mmHg; OR alveolar-arterial oxygen gradient 15 mmHg while breathing room air; pulmonary vascular dilatation. […] Workup typically includes: Pulse oximetry, Arterial blood gas, Contrast echocardiography. […] Liver transplantation is the only known cure for HPS.
#3 Hepatopulmonary Syndrome: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/24190-hepatopulmonary-syndrome
Hepatopulmonary syndrome occurs in a small percentage of people with liver disease and/or portal hypertension. It causes the capillaries in your lungs to expand, which affects their ability to transfer oxygen into your blood. This causes hypoxemia (low blood oxygen levels). […] Hepatopulmonary syndrome is a rare complication of liver disease that causes low oxygen levels in your blood (hypoxemia). […] Hepatopulmonary syndrome occurs in an estimated 25% of people with chronic liver disease. […] Healthcare providers recognize hepatopulmonary syndrome by a triad of features that appear together: Portal hypertension, usually in the setting of liver disease; Dilation of the veins entering your lungs (intrapulmonary vascular dilatations); Low blood oxygen (hypoxemia). […] Most of the time, you’ll already be diagnosed with liver disease and/or portal hypertension when you develop hepatopulmonary syndrome.
#3 Hepatopulmonary Syndrome – OpenAnesthesia
https://www.openanesthesia.org/keywords/hepatopulmonary-syndrome/
Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations in the setting of portal hypertension, leading to right-to-left shunts and hypoxia. […] Diagnosis requires the presence of the following: portal hypertension with or without cirrhosis or end-stage liver disease; oxygenation defect; PaO2 80 mmHg; OR alveolar-arterial oxygen gradient 15 mmHg while breathing room air; pulmonary vascular dilatation. […] Workup typically includes: Pulse oximetry, Arterial blood gas, Contrast echocardiography. […] Liver transplantation is the only known cure for HPS.
#3 Hepatopulmonary syndrome diagnostic study of choice – wikidoc
https://www.wikidoc.org/index.php/Hepatopulmonary_syndrome_diagnostic_study_of_choice
There is no single diagnostic study of choice for the diagnosis of hepatopulmonary syndrome, but hepatopulmonary syndrome can be diagnosed based on history of liver disease, atrial blood gas analysis (widened alveolar-arterial oxygen gradient measurement); and evidences of intra-pulmonary vascular dilation or arterio-venous communications that result in a right-to-left intrapulmonary shunt. […] There is no single diagnostic study of choice for the diagnosis of hepatopulmonary syndrome, but hepatopulmonary syndrome can be diagnosed based on history of liver disease, atrial blood gas analysis (widened alveolar-arterial oxygen gradient measurement); and evidences of intra-pulmonary vascular dilation or arterio-venous communications that result in a right-to-left intrapulmonary shunt. […] Among the patients who present with clinical signs of hepatopulmonary syndrome, the contrast-enhanced transthoracic echocardiography with agitated saline is the most specific test for the diagnosis.
#3 Evaluation of Hepatopulmonary Syndrome with 99mTc-Macroaggregated Albumin Scintigraphy | Journal of Nuclear Medicine Technology
https://tech.snmjournals.org/content/50/4/377
Hepatopulmonary syndrome is characterized by intrapulmonary vascular dilatation causing hypoxemia in patients with liver disease. 99mTc-macroaggregated albumin (99mTc-MAA) scintigraphy has diagnostic value in suspected hepatopulmonary syndrome by detecting a clinically significant right-to-left shunt. […] 99mTc-MAA scintigraphy also provides the added value of quantification of the shunt. […] An elevated alveolararterial gradient with a partial pressure of oxygen of less than 60, echocardiographic and scintigraphic evidence of intrapulmonary shunting, and no known chronic lung disease in the setting of cirrhosis were consistent with HPS in our patient. […] Hypoxemia can be distinguished from HPS and other etiologies through bubble echocardiography, with arrival of bubbles in the left heart at least 3 cardiac cycles after contrast appearance in the right heart, or through 99mTc-MAA scintigraphy showing uptake in the brain.
#3 Pulmonary Complications of Cirrhosis – Page 4
https://www.medscape.com/viewarticle/777530_4
Contrast-enhanced echocardiography can provide a qualitative estimate of shunting following injection of microbubbles into the venous system. […] Ventilation-perfusion lung scanning using macroaggregated albumin can be a quantitative measure of right-to-left shunting because these macroaggregates are typically trapped within the lung vasculature and do not reach the systemic circulation. […] The presence of hepatopulmonary syndrome is an indication for liver transplantation because it is the only therapy currently available to patients with significant hypoxemia. The pretransplant evaluation should include arterial blood gases, contrast-enhanced transthoracic echocardiography, arterial blood gases after 100% oxygen administration, and estimation of pulmonary shunting using macroaggregated albumin scanning. […] Currently, patients with a PaO2 60 mm Hg are granted higher priority for liver transplantation. Resolution of hepatopulmonary syndrome generally follows liver transplantation, with a 5-year survival of 70% or more.
#3
https://journals.lww.com/ajg/fulltext/2023/10001/s3882_hepatopulmonary_syndrome.4109.aspx
Hepatopulmonary syndrome (HPS) is an oxygenation defect caused by the development of intrapulmonary vascular dilation in the setting of advanced liver disease. […] To avoid high mortality and morbidity, diagnosing and treating promptly is critical. […] The diagnosis and treatment of HPS highlight the role of the interprofessional team approach in care for these complicated patients. HPS diagnosis requires oxygenation defect, intrapulmonary vascular dilatation, and liver disease (with or without portal hypertension). […] The only successful treatment for HPS is a liver transplant.