Nadciśnienie płucne
Rokowania, prognozy i postęp choroby

Nadciśnienie płucne (PH) jest progresywną chorobą o złym rokowaniu, definiowaną przez średnie ciśnienie w tętnicy płucnej (mPAP) ≥20 mmHg podczas cewnikowania prawej komory serca. Kluczowa jest wczesna diagnoza i spersonalizowane leczenie, z celem terapeutycznym osiągnięcia niskiego profilu ryzyka, co znacząco wpływa na zmniejszenie śmiertelności. Stratyfikacja ryzyka według wytycznych ESC/ERS 2022 obejmuje ocenę dystansu w 6-minutowym teście marszu (>380-440 m), poziomu NT-proBNP oraz klasy czynnościowej WHO, które mają istotne znaczenie prognostyczne. Wskaźniki przeżycia dla pacjentów z klasą I/II wynoszą odpowiednio 93%, 86% i 78% po 1, 2 i 3 latach, przy śmiertelności rocznej na poziomie 7%. W PH-LHD istotne prognostycznie są TPG i PVR, natomiast DPG nie koreluje ze śmiertelnością. Podatność tętnicy płucnej (PAC) i efektywna elastyczność prawej komory (Ea) stanowią bardziej wiarygodne wskaźniki całkowitego obciążenia prawej komory i rokowania.

Prognoza nadciśnienia płucnego (Pulmonary hypertension Prognosis)

Nadciśnienie płucne (pulmonary hypertension, PH) jest postępującym, zagrażającym życiu schorzeniem, które charakteryzuje się podwyższonym ciśnieniem w tętnicy płucnej. Jest to choroba o złym rokowaniu, szczególnie gdy nie zostanie wcześnie zdiagnozowana i leczona.12 Pomimo postępów w leczeniu, śmiertelność pacjentów z nadciśnieniem płucnym pozostaje wysoka, a sama choroba często ma charakter nieodwracalny.3 Wczesna diagnoza i wdrożenie odpowiedniego leczenia mają kluczowe znaczenie dla zmniejszenia śmiertelności i ograniczenia niekorzystnych zdarzeń.4

Znaczenie stratyfikacji ryzyka

Stratyfikacja ryzyka w tętniczym nadciśnieniu płucnym (PAH) jest kluczowa dla oceny rokowania pacjenta. Najnowsze wytyczne Europejskiego Towarzystwa Kardiologicznego (ESC) oraz Europejskiego Towarzystwa Oddechowego (ERS) z 2022 roku podkreślają znaczenie stratyfikacji ryzyka nie tylko na początku diagnozy, ale również podczas dalszej obserwacji pacjentów.5 Osiągnięcie statusu niskiego ryzyka stało się celem terapeutycznym, co podkreśla znaczenie spersonalizowanego leczenia. Zastosowanie tych wytycznych jest również istotne przy ustalaniu czasu skierowania na przeszczep płuc.6

Wytyczne ESC/ERS z 2022 r. podkreślają znaczenie osiągnięcia statusu niskiego lub pośredniego-niskiego ryzyka podczas obserwacji. W konsekwencji, dla pacjentów z profilem pośredniego-wysokiego ryzyka podczas obserwacji wymagana jest modyfikacja leczenia. Osiągnięcie lub utrzymanie niskiego profilu ryzyka podczas obserwacji powinno stanowić cel terapeutyczny dla zdecydowanej większości pacjentów z PAH.7

Kluczowe wskaźniki prognostyczne

Dystans w teście 6-minutowego marszu (6MWD) stanowi zmienną o dużej wartości prognostycznej. Osiągnięcie dystansu większego niż 380-440 m wydaje się przewidywać lepsze wyniki. Konieczna jest jednak ostrożna interpretacja wartości 6MWD, ponieważ jego czułość w przewidywaniu rokowania może być zmieniona w zależności od charakterystyki pacjenta (wiek, płeć) i chorób współistniejących.8

N-końcowy propeptyd natriuretyczny typu B (NT-proBNP) jest niezależnym predyktorem przeżycia. Niedawny systematyczny przegląd i metaanaliza dotycząca pacjentów z PAH wykazały, że wysokie poziomy NT-proBNP są skorelowane ze znacznie zwiększonym ryzykiem śmiertelności lub przeszczepu płuc w PAH.9 Zmienność poziomów NT-proBNP podczas obserwacji pacjentów z idiopatycznym PAH (iPAH) poddanych leczeniu jest silniejszym wskaźnikiem przeżycia wolnego od przeszczepu (TFS) niż jego wartość przy rozpoznaniu.10

Klasa czynnościowa według WHO ma istotne znaczenie prognostyczne. Pomimo niższego ryzyka zgonu w porównaniu z pacjentami w klasie czynnościowej III lub IV, pacjenci z klasą czynnościową I lub II PAH nie są grupą niskiego ryzyka zgodnie z wytycznymi leczenia PAH, ze wskaźnikiem śmiertelności 7% w ciągu jednego roku.11 To ryzyko śmiertelności znacznie wzrasta do 22% w ciągu trzech lat.12 Wskaźniki przeżycia dla pacjentów w klasie czynnościowej I/II po jednym, dwóch i trzech latach wynosiły odpowiednio 93% (95% CI: 91%-95%), 86% (95% CI: 82%-89%) i 78% (95% CI: 73%-83%).13

Rola parametrów hemodynamicznych

Średnie ciśnienie w tętnicy płucnej (mPAP) jest jednym z kluczowych wskaźników używanych do diagnozy, klasyfikacji ciężkości, przewidywania rokowania i wskazań do operacji w przewlekłym zakrzepowo-zatorowym nadciśnieniu płucnym (CTEPH).14 Nadciśnienie płucne definiuje się jako średnie ciśnienie w tętnicy płucnej (mPAP) wynoszące 20 mmHg podczas cewnikowania prawej części serca (RHC).15

Interesujące jest, że średnica tętnicy płucnej (PA) nie jest związana z rokowaniem w tętniczym nadciśnieniu płucnym (PAH) i przewlekłym zakrzepowo-zatorowym nadciśnieniu płucnym (CTEPH). Rozszerzenie tętnicy płucnej jest często obserwowane w nadciśnieniu płucnym i jest uważane za długoterminowy skutek podwyższonego ciśnienia, jednak badania wskazują, że średnica PA przy rozpoznaniu nie jest czynnikiem predykcyjnym śmiertelności u pacjentów z PAH i CTEPH.16

W przypadku nadciśnienia płucnego związanego z chorobą lewego serca (PH-LHD), podczas gdy zarówno podwyższony gradient przezpłucny (TPG), jak i opór naczyń płucnych (PVR) przewidywały śmiertelność, gradient rozkurczowy tętnicy płucnej (DPG) nie spełniał takiej roli.17 W rzeczywistości wysoki DPG w warunkach podwyższonego PVR miał odwrotną relację ze śmiertelnością, najprawdopodobniej identyfikując chorą populację z wyższym ciśnieniem zaklinowania w tętnicy płucnej (PCWP) i cięższą niewydolnością lewego serca.18

Podatność tętnicy płucnej (PAC, definiowana jako objętość wyrzutowa podzielona przez [skurczowe PAP (sPAP) minus dPAP]) jest bardziej wiarygodnym miernikiem całkowitego obciążenia prawej komory, odzwierciedlającym zarówno komponenty oporu, jak i tętna. W kilku niedawnych badaniach pacjentów z niewydolnością lewego serca, PAC korelowała odwrotnie ze śmiertelnością, nawet u pacjentów z normalnym PVR.19

Efektywna elastyczność tętnicza prawej komory (Ea), definiowana u pacjentów z PH jako sPAP podzielone przez objętość wyrzutową, jest kolejnym parametrem całkowitego obciążenia prawej komory i może mieć wartość prognostyczną.20

Oczekiwana długość życia i czynniki wpływające

Oczekiwana długość życia będzie zależeć od typu nadciśnienia płucnego i jego przyczyny.21 Mediana czasu przeżycia po rozpoznaniu dla osób z grupą 1 PAH wynosi ponad 5 lat. Pozostałe grupy mają zróżnicowane wskaźniki śmiertelności.22 Według badania z 2022 roku dotyczącego trendów w Stanach Zjednoczonych, wskaźniki śmiertelności dla grupy 2 PH i grupy 3 PH są wyższe niż dla grupy 1 PH.23

W porównaniu z innymi grupami PH, wskaźniki śmiertelności dla osób z grupą 2 PH i grupą 3 PH odpowiednio podwoiły się i potroiły w ciągu lat.24 Nadciśnienie płucne jest prawie niezmiennie związane ze zwiększonym ryzykiem śmiertelności u pacjentów z chorobą lewego serca (LHD).25 Pacjenci z PH-LHD mają bardziej nasilone objawy, gorszą tolerancję wysiłku, doświadczają wyższych wskaźników hospitalizacji i częściej otrzymują wskazanie dotyczące potrzeby przeszczepu serca, co ma poważne implikacje dla jakości życia pacjentów i kosztów opieki zdrowotnej.26

Nadciśnienie płucne u noworodków i niemowląt

Nadciśnienie płucne (PH) u noworodków i niemowląt jest złożonym stanem związanym z kilkoma chorobami płuc, serca i układowymi, przyczyniającymi się do zachorowalności i śmiertelności.27 Prognoza PH jest związana z ciężkością choroby w momencie diagnozy; opóźnione leczenie zmniejsza szansę na przeżycie.28 Ciężkość odgrywa kluczową rolę w odpowiedniej strategii leczenia PH.29

Ludzka ocena PH przy użyciu echokardiografii jest zarówno czasochłonna, jak i wymagająca ekspertyzy, co może opóźnić opiekę do bardziej zaawansowanego stadium choroby, potencjalnie zmniejszając szansę na przeżycie. Dlatego istnieje wyraźna potrzeba automatycznej i usprawnionej metody, która pomoże klinicystom w ocenie PH u noworodków.30

Prognostyczne znaczenie leczenia

Gdy maksymalne terapie medyczne nie poprawiają statusu oceny ryzyka, należy rozważyć przeszczep płuc, który pozostaje ostateczną opcją terapeutyczną w najcięższych przypadkach PAH. Dlatego kluczowe jest określenie odpowiedniego momentu skierowania i umieszczenia na liście do przeszczepu płuc, aby zmniejszyć śmiertelność i zachorowalność.31

Zaobserwowany znaczny wskaźnik śmiertelności u pacjentów z klasą czynnościową I lub II oraz korzystna odpowiedź na terapie medyczne wspierają obecne wytyczne leczenia, zgodnie z którymi nawet pacjenci z klasą czynnościową I lub II wymagają ścisłego nadzoru i optymalizacji terapii PAH.32

Osoby, które otrzymują opiekę na wczesnym etapie choroby, mogą prowadzić normalne życie, zarówno pod względem jakości, jak i długości, przy ciągłym kierownictwie i zarządzaniu przez pracownika opieki zdrowotnej i zespół, z którym współpracują.33

Postępy w metodach prognostycznych

Rozwijane są modele predykcyjne dla mPAP u pacjentów z CTEPH o wyższej dokładności poprzez wykonywanie minimalnie inwazyjnych testów. Wydajność predykcyjna modeli jest poprawiana poprzez wybór optymalnej metody uczenia maszynowego i kombinacji zmiennych objaśniających. Takie modele predykcyjne mogą być przydatne do przewidywania mPAP u pacjentów z CTEPH.34

Metody automatycznej oceny mają potencjał znaczącej poprawy dokładności, niezawodności i spójności oszacowania PH u noworodków, zmniejszając liczbę pominiętych lub opóźnionych diagnoz ciężkiego PH. Ponieważ ciężkość PH determinuje pilność leczenia, może to z kolei poprawić rokowanie pacjentów z PH, umożliwiając wcześniejsze leczenie.35

Wyzwania w przewidywaniu rokowania

Problem przewidywania ciężkości PH jest trudny nie tylko ze względu na złożoność zadania, ale także z powodu nierównowagi danych.36 Pomimo ograniczonej wartości prognostycznej gradientu rozkurczowego tętnicy płucnej (DPG), nie wyklucza to możliwości, że może on być diagnostycznie użyteczny do identyfikacji pacjentów z prawdziwą chorobą naczyń płucnych.37

Rokowanie wydaje się nie poprawiać w ciągu ostatnich 5 lat, pomimo nowych opcji leczenia.38 Wyzwania związane z przewidywaniem rokowania podkreślają potrzebę dalszych badań nad markerami prognostycznymi i strategiami leczenia nadciśnienia płucnego.

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Frontiers | Emerging Molecules in Pulmonary Hypertension: Diagnosis, Risk Prediction, Treatment and Prognosis
    https://www.frontiersin.org/research-topics/60695/emerging-molecules-in-pulmonary-hypertension-diagnosis-risk-prediction-treatment-and-prognosisundefined
    Pulmonary arterial hypertension (PAH) is a severe arteriopathy characterized by progressive pulmonary vascular remodeling and increased pulmonary vascular resistance leading to right heart failure and often resulting in death. […] It remains a life-threatening disease with a high mortality rate. […] Its early diagnosis and treatment are of great significance to reduce mortality and limit adverse events. […] Despite these treatments, the mortality rate remains high as the condition often proves non-reversible. […] Treatment strategies and prognosis of patients with different subtypes of PAH.
  • #2 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Pulmonary hypertension (PH) is a progressive, life threatening type of high blood pressure. […] At the moment, theres no readily available cure for PH. […] Its a progressive disease, which means it can advance over time, sometimes much faster for some people than others. […] For many people, PH can lead to significant heart failure, and their overall health can be in great danger. […] Life expectancy will depend on the type of PH you have and the cause of your PH. […] The median survival time after diagnosis for people with group 1 PAH is more than 5 years. […] The other groups all have varied mortality rates. […] According to a 2022 study on trends in the United States, mortality rates for group 2 PH and group 3 PH are higher than those for group 1 PH. […] One 2019 study examined the outlooks of people in a referral center in France.
  • #3 Frontiers | Emerging Molecules in Pulmonary Hypertension: Diagnosis, Risk Prediction, Treatment and Prognosis
    https://www.frontiersin.org/research-topics/60695/emerging-molecules-in-pulmonary-hypertension-diagnosis-risk-prediction-treatment-and-prognosisundefined
    Pulmonary arterial hypertension (PAH) is a severe arteriopathy characterized by progressive pulmonary vascular remodeling and increased pulmonary vascular resistance leading to right heart failure and often resulting in death. […] It remains a life-threatening disease with a high mortality rate. […] Its early diagnosis and treatment are of great significance to reduce mortality and limit adverse events. […] Despite these treatments, the mortality rate remains high as the condition often proves non-reversible. […] Treatment strategies and prognosis of patients with different subtypes of PAH.
  • #4 Frontiers | Emerging Molecules in Pulmonary Hypertension: Diagnosis, Risk Prediction, Treatment and Prognosis
    https://www.frontiersin.org/research-topics/60695/emerging-molecules-in-pulmonary-hypertension-diagnosis-risk-prediction-treatment-and-prognosisundefined
    Pulmonary arterial hypertension (PAH) is a severe arteriopathy characterized by progressive pulmonary vascular remodeling and increased pulmonary vascular resistance leading to right heart failure and often resulting in death. […] It remains a life-threatening disease with a high mortality rate. […] Its early diagnosis and treatment are of great significance to reduce mortality and limit adverse events. […] Despite these treatments, the mortality rate remains high as the condition often proves non-reversible. […] Treatment strategies and prognosis of patients with different subtypes of PAH.
  • #5 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. […] The recently published 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines underline the importance of risk stratification not only at baseline but also during follow-up. […] Achieving a low-risk status has now become the therapeutic goal, emphasising the importance of personalised therapy. […] The application of these guidelines is also important in determining the timing for lung transplantation referral. […] In this review, we summarise the most relevant prognostic factors of PAH as well as the parameters used in PAH risk scores and their evolution in the guidelines over the last decade. […] The aim of this manuscript is to review the clinical and paraclinical biomarkers that influence PAH prognosis.
  • #6 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. […] The recently published 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines underline the importance of risk stratification not only at baseline but also during follow-up. […] Achieving a low-risk status has now become the therapeutic goal, emphasising the importance of personalised therapy. […] The application of these guidelines is also important in determining the timing for lung transplantation referral. […] In this review, we summarise the most relevant prognostic factors of PAH as well as the parameters used in PAH risk scores and their evolution in the guidelines over the last decade. […] The aim of this manuscript is to review the clinical and paraclinical biomarkers that influence PAH prognosis.
  • #7 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    Variation in NT-proBNP levels during idiopathic PAH (iPAH) patient follow-up under treatment is a stronger indicator of transplant-free survival (TFS) than its value at diagnosis. […] The 2022 ESC/ERS guidelines highlight the importance of achieving a low or intermediate-low-risk group during follow-up. […] Consequently, for patients with an intermediate-high-risk profile during follow-up, a modification of treatment is required. […] Achieving or maintaining a low-risk profile during follow-up should represent the therapeutic goal for the vast majority of patients with PAH. […] When maximal medical therapies fail to improve the risk score status, lung transplantation (LT) should be considered as it remains the ultimate therapeutic option in the most severe PAH cases. […] Therefore, it is crucial to define the right moment for LT referral and listing in order to reduce mortality and morbidity.
  • #8 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    We also focus on the utility of the main risk stratification tools used in everyday clinical assessment and how they affect PAH patient management, including therapy modification and lung transplantation (LT) referral or listing. […] The 6-min walk distance (6MWD) represents a variable of great prognostic value. […] Achievement of a 6MWD greater than 380-440 m appears to predict better outcomes. […] A careful interpretation of the 6MWD values is necessary, since its sensitivity for prognosis prediction may be altered depending on a patient’s characteristics (age, gender) and comorbidities. […] N-terminal pro-B-type natriuretic peptide (NT-proBNP) is demonstrated to be an independent predictor of survival. […] A recent systematic review and meta-analysis on PAH patients demonstrated that high levels of NT-proBNP are correlated with a significantly increased risk of mortality or LT in PAH.
  • #9 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    We also focus on the utility of the main risk stratification tools used in everyday clinical assessment and how they affect PAH patient management, including therapy modification and lung transplantation (LT) referral or listing. […] The 6-min walk distance (6MWD) represents a variable of great prognostic value. […] Achievement of a 6MWD greater than 380-440 m appears to predict better outcomes. […] A careful interpretation of the 6MWD values is necessary, since its sensitivity for prognosis prediction may be altered depending on a patient’s characteristics (age, gender) and comorbidities. […] N-terminal pro-B-type natriuretic peptide (NT-proBNP) is demonstrated to be an independent predictor of survival. […] A recent systematic review and meta-analysis on PAH patients demonstrated that high levels of NT-proBNP are correlated with a significantly increased risk of mortality or LT in PAH.
  • #10 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    Variation in NT-proBNP levels during idiopathic PAH (iPAH) patient follow-up under treatment is a stronger indicator of transplant-free survival (TFS) than its value at diagnosis. […] The 2022 ESC/ERS guidelines highlight the importance of achieving a low or intermediate-low-risk group during follow-up. […] Consequently, for patients with an intermediate-high-risk profile during follow-up, a modification of treatment is required. […] Achieving or maintaining a low-risk profile during follow-up should represent the therapeutic goal for the vast majority of patients with PAH. […] When maximal medical therapies fail to improve the risk score status, lung transplantation (LT) should be considered as it remains the ultimate therapeutic option in the most severe PAH cases. […] Therefore, it is crucial to define the right moment for LT referral and listing in order to reduce mortality and morbidity.
  • #11
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7691927/
    Limited data about the long-term prognosis and response to therapy in pulmonary arterial hypertension patients with World Health Organization functional class I/II symptoms are available. […] Survival rates for functional class I/II patients at one, two, and three years were 93% (95% confidence interval (CI): 91%95%), 86% (95% CI: 82%89%), and 78% (95% CI: 73%83%), respectively. […] The calculated risk of death of 22% within three years for functional class I/II patients underlines the need for careful assessment and optimal treatment of patients with functional class I/II disease. […] Despite recent advances in medical therapy for patients with PAH, mortality remains high and continues to be associated with FC. […] The observational registry meta-analysis revealed that, despite a lower risk of death compared with patients in FC III or IV, those with FC I or II PAH were not low risk as defined by PAH treatment guidelines with a 7% one-year morality rate.
  • #12
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7691927/
    This mortality risk increased substantially to 22% at three years. […] Together, these two analyses establish that some patients with FC I or II PAH are at a higher risk of death or worsening of PAH and medical therapy for PAH may offer considerable clinical benefit in such patients. […] The observed considerable mortality rate in FC I or II patients and a favorable response to medical therapies support the current treatment guidelines that even FC I or II patients require close surveillance and optimization of PAH therapy.
  • #13
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7691927/
    Limited data about the long-term prognosis and response to therapy in pulmonary arterial hypertension patients with World Health Organization functional class I/II symptoms are available. […] Survival rates for functional class I/II patients at one, two, and three years were 93% (95% confidence interval (CI): 91%95%), 86% (95% CI: 82%89%), and 78% (95% CI: 73%83%), respectively. […] The calculated risk of death of 22% within three years for functional class I/II patients underlines the need for careful assessment and optimal treatment of patients with functional class I/II disease. […] Despite recent advances in medical therapy for patients with PAH, mortality remains high and continues to be associated with FC. […] The observational registry meta-analysis revealed that, despite a lower risk of death compared with patients in FC III or IV, those with FC I or II PAH were not low risk as defined by PAH treatment guidelines with a 7% one-year morality rate.
  • #14 Development and web deployment of prediction model for pulmonary arterial pressure in chronic thromboembolic pulmonary hypertension using machine learning | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0300716
    Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with a poor prognosis caused by stenosis and occlusion of the pulmonary artery due to an organizing thrombus in the pulmonary artery that ultimately leads to pulmonary hypertension (PH) [1, 2]. PH is defined as a mean pulmonary artery pressure (mPAP) of 20 mmHg on right heart catheterization (RHC). […] The mPAP is one of the key indices used for the diagnosis, severity classification, prognosis prediction, and indications for surgery in CTEPH [3, 4]. […] We constructed a prediction model for mPAP in patients with CTEPH with higher accuracy by performing minimally invasive tests. The prediction performance of our model was improved by selecting the optimal machine learning method and combination of explanatory variables. Our prediction model may be useful for predicting the mPAP in patients with CTEPH.
  • #15 Development and web deployment of prediction model for pulmonary arterial pressure in chronic thromboembolic pulmonary hypertension using machine learning | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0300716
    Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with a poor prognosis caused by stenosis and occlusion of the pulmonary artery due to an organizing thrombus in the pulmonary artery that ultimately leads to pulmonary hypertension (PH) [1, 2]. PH is defined as a mean pulmonary artery pressure (mPAP) of 20 mmHg on right heart catheterization (RHC). […] The mPAP is one of the key indices used for the diagnosis, severity classification, prognosis prediction, and indications for surgery in CTEPH [3, 4]. […] We constructed a prediction model for mPAP in patients with CTEPH with higher accuracy by performing minimally invasive tests. The prediction performance of our model was improved by selecting the optimal machine learning method and combination of explanatory variables. Our prediction model may be useful for predicting the mPAP in patients with CTEPH.
  • #16
    https://link.springer.com/article/10.1007/s12471-020-01467-1
    Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. […] Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. […] The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients. […] Pulmonary artery diameter is not associated with prognosis in pulmonary arterial hypertension (PAH) and in chronic thrombo-embolic pulmonary hypertension. […] The prognosis seems not to have improved in the last 5 years, despite new treatment options. […] Our findings suggest that PA diameter at diagnosis is not predictive for mortality in PAH and CTEPH patients. This study therefore suggests that PA diameter cannot be used for the prognosis of these patients.
  • #17 Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure
    https://www.acc.org/latest-in-cardiology/articles/2015/04/14/10/07/diastolic-pulmonary-gradient-does-not-predict-survival-in-pulmonary-hypertension-due-to-left-heart-failure
    Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure […] Although both an elevated TPG and pulmonary vasculature resistance (PVR) predicted mortality, DPG failed to do so. […] In fact, high DPG in the setting of an elevated PVR had an inverse relation with mortality, most likely identifying a sicker population with higher PCWP and more severe left heart failure. […] Although DPG has limited prognostic value, this does not necessarily preclude the possibility that it may be diagnostically useful to identify patients with true pulmonary vascular disease. […] PVR which includes flow assessment, is a better predictor of mortality in individuals with PH-LHD, but may also incompletely describe total RV load. […] Pulmonary artery compliance (PAC, defined as stroke volume divided by [systolic PAP (sPAP) minus dPAP]) is a more robust measure of total RV load, reflecting both resistive and pulsatile components. It has inversely correlated with mortality in several recent studies of left heart failure patients, even in those patients with normal PVR. […] RV effective arterial elastance (Ea), defined in PH patients as sPAP divided by stroke volume is another lumped parameter of total RV load and may have prognostic value.
  • #18 Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure
    https://www.acc.org/latest-in-cardiology/articles/2015/04/14/10/07/diastolic-pulmonary-gradient-does-not-predict-survival-in-pulmonary-hypertension-due-to-left-heart-failure
    Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure […] Although both an elevated TPG and pulmonary vasculature resistance (PVR) predicted mortality, DPG failed to do so. […] In fact, high DPG in the setting of an elevated PVR had an inverse relation with mortality, most likely identifying a sicker population with higher PCWP and more severe left heart failure. […] Although DPG has limited prognostic value, this does not necessarily preclude the possibility that it may be diagnostically useful to identify patients with true pulmonary vascular disease. […] PVR which includes flow assessment, is a better predictor of mortality in individuals with PH-LHD, but may also incompletely describe total RV load. […] Pulmonary artery compliance (PAC, defined as stroke volume divided by [systolic PAP (sPAP) minus dPAP]) is a more robust measure of total RV load, reflecting both resistive and pulsatile components. It has inversely correlated with mortality in several recent studies of left heart failure patients, even in those patients with normal PVR. […] RV effective arterial elastance (Ea), defined in PH patients as sPAP divided by stroke volume is another lumped parameter of total RV load and may have prognostic value.
  • #19 Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure
    https://www.acc.org/latest-in-cardiology/articles/2015/04/14/10/07/diastolic-pulmonary-gradient-does-not-predict-survival-in-pulmonary-hypertension-due-to-left-heart-failure
    Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure […] Although both an elevated TPG and pulmonary vasculature resistance (PVR) predicted mortality, DPG failed to do so. […] In fact, high DPG in the setting of an elevated PVR had an inverse relation with mortality, most likely identifying a sicker population with higher PCWP and more severe left heart failure. […] Although DPG has limited prognostic value, this does not necessarily preclude the possibility that it may be diagnostically useful to identify patients with true pulmonary vascular disease. […] PVR which includes flow assessment, is a better predictor of mortality in individuals with PH-LHD, but may also incompletely describe total RV load. […] Pulmonary artery compliance (PAC, defined as stroke volume divided by [systolic PAP (sPAP) minus dPAP]) is a more robust measure of total RV load, reflecting both resistive and pulsatile components. It has inversely correlated with mortality in several recent studies of left heart failure patients, even in those patients with normal PVR. […] RV effective arterial elastance (Ea), defined in PH patients as sPAP divided by stroke volume is another lumped parameter of total RV load and may have prognostic value.
  • #20 Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure
    https://www.acc.org/latest-in-cardiology/articles/2015/04/14/10/07/diastolic-pulmonary-gradient-does-not-predict-survival-in-pulmonary-hypertension-due-to-left-heart-failure
    Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure […] Although both an elevated TPG and pulmonary vasculature resistance (PVR) predicted mortality, DPG failed to do so. […] In fact, high DPG in the setting of an elevated PVR had an inverse relation with mortality, most likely identifying a sicker population with higher PCWP and more severe left heart failure. […] Although DPG has limited prognostic value, this does not necessarily preclude the possibility that it may be diagnostically useful to identify patients with true pulmonary vascular disease. […] PVR which includes flow assessment, is a better predictor of mortality in individuals with PH-LHD, but may also incompletely describe total RV load. […] Pulmonary artery compliance (PAC, defined as stroke volume divided by [systolic PAP (sPAP) minus dPAP]) is a more robust measure of total RV load, reflecting both resistive and pulsatile components. It has inversely correlated with mortality in several recent studies of left heart failure patients, even in those patients with normal PVR. […] RV effective arterial elastance (Ea), defined in PH patients as sPAP divided by stroke volume is another lumped parameter of total RV load and may have prognostic value.
  • #21 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Pulmonary hypertension (PH) is a progressive, life threatening type of high blood pressure. […] At the moment, theres no readily available cure for PH. […] Its a progressive disease, which means it can advance over time, sometimes much faster for some people than others. […] For many people, PH can lead to significant heart failure, and their overall health can be in great danger. […] Life expectancy will depend on the type of PH you have and the cause of your PH. […] The median survival time after diagnosis for people with group 1 PAH is more than 5 years. […] The other groups all have varied mortality rates. […] According to a 2022 study on trends in the United States, mortality rates for group 2 PH and group 3 PH are higher than those for group 1 PH. […] One 2019 study examined the outlooks of people in a referral center in France.
  • #22 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Pulmonary hypertension (PH) is a progressive, life threatening type of high blood pressure. […] At the moment, theres no readily available cure for PH. […] Its a progressive disease, which means it can advance over time, sometimes much faster for some people than others. […] For many people, PH can lead to significant heart failure, and their overall health can be in great danger. […] Life expectancy will depend on the type of PH you have and the cause of your PH. […] The median survival time after diagnosis for people with group 1 PAH is more than 5 years. […] The other groups all have varied mortality rates. […] According to a 2022 study on trends in the United States, mortality rates for group 2 PH and group 3 PH are higher than those for group 1 PH. […] One 2019 study examined the outlooks of people in a referral center in France.
  • #23 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Pulmonary hypertension (PH) is a progressive, life threatening type of high blood pressure. […] At the moment, theres no readily available cure for PH. […] Its a progressive disease, which means it can advance over time, sometimes much faster for some people than others. […] For many people, PH can lead to significant heart failure, and their overall health can be in great danger. […] Life expectancy will depend on the type of PH you have and the cause of your PH. […] The median survival time after diagnosis for people with group 1 PAH is more than 5 years. […] The other groups all have varied mortality rates. […] According to a 2022 study on trends in the United States, mortality rates for group 2 PH and group 3 PH are higher than those for group 1 PH. […] One 2019 study examined the outlooks of people in a referral center in France.
  • #24 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Over the years, in comparison with the other groups of PH, the mortality rates for people with group 2 PH and group 3 PH doubled and tripled, respectively. […] People who get care early in the disease process can go on to live normal lives, in both quality and quantity, with the continued guidance and management of a healthcare professional and the team they work with.
  • #25 Predictors of hospitalisations for heart failure and mortality in patients with pulmonary hypertension associated with left heart disease: a systematic review | BMJ Open
    https://bmjopen.bmj.com/content/4/7/e004843
    PH is almost invariably associated with increased mortality risk in patients with LHD. […] However, effects on hospitalisation risk are yet to be fully characterised; while available evidence on the adverse effects of PH have been derived essentially from Caucasians. […] Patients with PH-LHD have more severe symptoms, worse tolerance to effort, experience higher hospitalisation rates and are more likely to receive an indication of the need for cardiac transplant with major implications for the quality of life of patients and healthcare costs. […] Several studies have reported PH-LHD to be associated with increased mortality, both in patients with systolic dysfunction and those with preserved left ventricular ejection fraction (LVEF). […] The majority of studies included in this review showed that PH is an independent predictor of mortality in patients with LHD, with the more consistent evidence being in those with HF and MR.
  • #26 Predictors of hospitalisations for heart failure and mortality in patients with pulmonary hypertension associated with left heart disease: a systematic review | BMJ Open
    https://bmjopen.bmj.com/content/4/7/e004843
    PH is almost invariably associated with increased mortality risk in patients with LHD. […] However, effects on hospitalisation risk are yet to be fully characterised; while available evidence on the adverse effects of PH have been derived essentially from Caucasians. […] Patients with PH-LHD have more severe symptoms, worse tolerance to effort, experience higher hospitalisation rates and are more likely to receive an indication of the need for cardiac transplant with major implications for the quality of life of patients and healthcare costs. […] Several studies have reported PH-LHD to be associated with increased mortality, both in patients with systolic dysfunction and those with preserved left ventricular ejection fraction (LVEF). […] The majority of studies included in this review showed that PH is an independent predictor of mortality in patients with LHD, with the more consistent evidence being in those with HF and MR.
  • #27
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    Pulmonary hypertension (PH) in newborns and infants is a complex condition associated with several pulmonary, cardiac, and systemic diseases contributing to morbidity and mortality. […] The PH prognosis is associated with the severity of the disease at diagnosis; thus, delayed treatment decreases the chance of survival. […] Human assessment of PH using echocardiography with the procedures mentioned above is both time-consuming and expertise-demanding, which may delay care to a more advanced stage of illness, potentially decreasing the chance of survival. […] Thus, there is a clear need for an automatic and streamlined method to assist clinicians in assessing PH in newborns. […] The severity plays a key role in the appropriate PH treatment strategy. […] The majority voting is not only helpful to enhance the performance, but it is also useful in case a single view has an unsatisfactory quality for a given subject, a common scenario in many real-world applications.
  • #28
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    Pulmonary hypertension (PH) in newborns and infants is a complex condition associated with several pulmonary, cardiac, and systemic diseases contributing to morbidity and mortality. […] The PH prognosis is associated with the severity of the disease at diagnosis; thus, delayed treatment decreases the chance of survival. […] Human assessment of PH using echocardiography with the procedures mentioned above is both time-consuming and expertise-demanding, which may delay care to a more advanced stage of illness, potentially decreasing the chance of survival. […] Thus, there is a clear need for an automatic and streamlined method to assist clinicians in assessing PH in newborns. […] The severity plays a key role in the appropriate PH treatment strategy. […] The majority voting is not only helpful to enhance the performance, but it is also useful in case a single view has an unsatisfactory quality for a given subject, a common scenario in many real-world applications.
  • #29
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    Pulmonary hypertension (PH) in newborns and infants is a complex condition associated with several pulmonary, cardiac, and systemic diseases contributing to morbidity and mortality. […] The PH prognosis is associated with the severity of the disease at diagnosis; thus, delayed treatment decreases the chance of survival. […] Human assessment of PH using echocardiography with the procedures mentioned above is both time-consuming and expertise-demanding, which may delay care to a more advanced stage of illness, potentially decreasing the chance of survival. […] Thus, there is a clear need for an automatic and streamlined method to assist clinicians in assessing PH in newborns. […] The severity plays a key role in the appropriate PH treatment strategy. […] The majority voting is not only helpful to enhance the performance, but it is also useful in case a single view has an unsatisfactory quality for a given subject, a common scenario in many real-world applications.
  • #30
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    Pulmonary hypertension (PH) in newborns and infants is a complex condition associated with several pulmonary, cardiac, and systemic diseases contributing to morbidity and mortality. […] The PH prognosis is associated with the severity of the disease at diagnosis; thus, delayed treatment decreases the chance of survival. […] Human assessment of PH using echocardiography with the procedures mentioned above is both time-consuming and expertise-demanding, which may delay care to a more advanced stage of illness, potentially decreasing the chance of survival. […] Thus, there is a clear need for an automatic and streamlined method to assist clinicians in assessing PH in newborns. […] The severity plays a key role in the appropriate PH treatment strategy. […] The majority voting is not only helpful to enhance the performance, but it is also useful in case a single view has an unsatisfactory quality for a given subject, a common scenario in many real-world applications.
  • #31 Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10342910/
    Variation in NT-proBNP levels during idiopathic PAH (iPAH) patient follow-up under treatment is a stronger indicator of transplant-free survival (TFS) than its value at diagnosis. […] The 2022 ESC/ERS guidelines highlight the importance of achieving a low or intermediate-low-risk group during follow-up. […] Consequently, for patients with an intermediate-high-risk profile during follow-up, a modification of treatment is required. […] Achieving or maintaining a low-risk profile during follow-up should represent the therapeutic goal for the vast majority of patients with PAH. […] When maximal medical therapies fail to improve the risk score status, lung transplantation (LT) should be considered as it remains the ultimate therapeutic option in the most severe PAH cases. […] Therefore, it is crucial to define the right moment for LT referral and listing in order to reduce mortality and morbidity.
  • #32
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7691927/
    This mortality risk increased substantially to 22% at three years. […] Together, these two analyses establish that some patients with FC I or II PAH are at a higher risk of death or worsening of PAH and medical therapy for PAH may offer considerable clinical benefit in such patients. […] The observed considerable mortality rate in FC I or II patients and a favorable response to medical therapies support the current treatment guidelines that even FC I or II patients require close surveillance and optimization of PAH therapy.
  • #33 Pulmonary Hypertension Life Expectancy, Outlook, Treatment
    https://www.healthline.com/health/pulmonary-hypertension-prognosis
    Over the years, in comparison with the other groups of PH, the mortality rates for people with group 2 PH and group 3 PH doubled and tripled, respectively. […] People who get care early in the disease process can go on to live normal lives, in both quality and quantity, with the continued guidance and management of a healthcare professional and the team they work with.
  • #34 Development and web deployment of prediction model for pulmonary arterial pressure in chronic thromboembolic pulmonary hypertension using machine learning | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0300716
    Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with a poor prognosis caused by stenosis and occlusion of the pulmonary artery due to an organizing thrombus in the pulmonary artery that ultimately leads to pulmonary hypertension (PH) [1, 2]. PH is defined as a mean pulmonary artery pressure (mPAP) of 20 mmHg on right heart catheterization (RHC). […] The mPAP is one of the key indices used for the diagnosis, severity classification, prognosis prediction, and indications for surgery in CTEPH [3, 4]. […] We constructed a prediction model for mPAP in patients with CTEPH with higher accuracy by performing minimally invasive tests. The prediction performance of our model was improved by selecting the optimal machine learning method and combination of explanatory variables. Our prediction model may be useful for predicting the mPAP in patients with CTEPH.
  • #35
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    The PH severity prediction problem is challenging, not only due to the complex task at hand but also because of the data imbalance. […] Our method has the potential to significantly improve the accuracy, reliability, and consistency of PH estimation in newborns, thereby reducing the number of missed or delayed diagnoses of severe PH. […] As the severity of PH determines the urgency of treatment, this, in turn, could improve the prognosis of PH patients by allowing for earlier treatment.
  • #36
    https://link.springer.com/article/10.1007/s11263-024-01996-x
    The PH severity prediction problem is challenging, not only due to the complex task at hand but also because of the data imbalance. […] Our method has the potential to significantly improve the accuracy, reliability, and consistency of PH estimation in newborns, thereby reducing the number of missed or delayed diagnoses of severe PH. […] As the severity of PH determines the urgency of treatment, this, in turn, could improve the prognosis of PH patients by allowing for earlier treatment.
  • #37 Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure
    https://www.acc.org/latest-in-cardiology/articles/2015/04/14/10/07/diastolic-pulmonary-gradient-does-not-predict-survival-in-pulmonary-hypertension-due-to-left-heart-failure
    Diastolic Pulmonary Gradient does not Predict Survival in Pulmonary Hypertension due to Left Heart Failure […] Although both an elevated TPG and pulmonary vasculature resistance (PVR) predicted mortality, DPG failed to do so. […] In fact, high DPG in the setting of an elevated PVR had an inverse relation with mortality, most likely identifying a sicker population with higher PCWP and more severe left heart failure. […] Although DPG has limited prognostic value, this does not necessarily preclude the possibility that it may be diagnostically useful to identify patients with true pulmonary vascular disease. […] PVR which includes flow assessment, is a better predictor of mortality in individuals with PH-LHD, but may also incompletely describe total RV load. […] Pulmonary artery compliance (PAC, defined as stroke volume divided by [systolic PAP (sPAP) minus dPAP]) is a more robust measure of total RV load, reflecting both resistive and pulsatile components. It has inversely correlated with mortality in several recent studies of left heart failure patients, even in those patients with normal PVR. […] RV effective arterial elastance (Ea), defined in PH patients as sPAP divided by stroke volume is another lumped parameter of total RV load and may have prognostic value.
  • #38
    https://link.springer.com/article/10.1007/s12471-020-01467-1
    Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. […] Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. […] The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients. […] Pulmonary artery diameter is not associated with prognosis in pulmonary arterial hypertension (PAH) and in chronic thrombo-embolic pulmonary hypertension. […] The prognosis seems not to have improved in the last 5 years, despite new treatment options. […] Our findings suggest that PA diameter at diagnosis is not predictive for mortality in PAH and CTEPH patients. This study therefore suggests that PA diameter cannot be used for the prognosis of these patients.