Przepuklina mitralna – Rokowania, prognozy i postęp choroby

Przepuklina mitralna
Rokowania, prognozy i postęp choroby

Przepuklina zastawki mitralnej (MVP) dotyczy około 2-3% populacji i charakteryzuje się szerokim spektrum rokowania, z większością pacjentów pozostających bezobjawowymi i z prawie normalną długością życia. W badaniu długoterminowym (średnio 13,7 roku) u 53 pacjentów z MVP, 15% doświadczyło powikłań, takich jak postępująca niedomykalność mitralna (MR), migotanie komór, infekcyjne zapalenie wsierdzia, z czego 2 pacjentów zmarło z powodu MVP. Czynniki prognostyczne gorszego rokowania obejmują obecność niedomykalności mitralnej, dysfunkcję skurczową lewej komory, wiek >50 lat, powiększenie lewego przedsionka, wiotki płatek zastawki oraz migotanie przedsionków. Około 5-10% pacjentów rozwija ciężką MR, która znacząco pogarsza rokowanie, zwłaszcza u osób z obniżoną funkcją lewej komory, gdzie ryzyko zgonu wynosi 20% w ciągu roku i 50% w ciągu 5 lat bez interwencji chirurgicznej.

Przepuklina mitralna (Mitral valve prolapse) – Prognoza

Naturalna historia choroby
Czynniki ryzyka gorszego rokowania
Progresja do niedomykalności mitralnej
Wpływ leczenia na rokowanie
Predykcja niepowodzenia naprawy zastawki
Podsumowanie rokowania
Kolejne rozdziały

Przepuklina mitralna (Mitral valve prolapse) – Prognoza

Przepuklina zastawki mitralnej (MVP – Mitral valve prolapse) jest jedną z najczęstszych wad zastawkowych serca, występującą u około 2-3% populacji ogólnej. Rokowanie u pacjentów z przepukliną mitralną cechuje się szerokim spektrum możliwych wyników, choć większość chorych pozostaje bezobjawowa i ma niemal normalną przewidywaną długość życia.¹²

Naturalna historia choroby

W badaniu długoterminowego przebiegu przepukliny mitralnej u 53 pacjentów z udokumentowanym fonokardiograficznie śródskurczowym kliknięciem lub późnoskurczowym szmerem (lub obydwoma objawami) po średnio 13,7 latach obserwacji, stwierdzono, że 38 pacjentów żyło bez poważnych powikłań, a 7 zmarło z przyczyn niezwiązanych z wadą. U dwóch pacjentów przepuklina mitralna była powiązana z przyczyną zgonu. Inne powikłania obejmowały migotanie komór (1 pacjent) oraz infekcyjne zapalenie wsierdzia (3 pacjentów). Postępująca niedomykalność mitralna rozwinęła się u 5 pacjentów, wymagając wymiany zastawki u 2 z nich. Powyższe powikłania wystąpiły łącznie u 8 pacjentów (15%), i były istotnie statystycznie (P = 0,15) związane z późnoskurczowym szmerem, a nie z izolowanym śródskurczowym kliknięciem.³

Czynniki ryzyka gorszego rokowania

Badania wskazują, że pewne czynniki predysponują pacjentów z przepukliną mitralną do gorszego rokowania. W badaniu opartym na społeczności, obejmującym 833 osoby z bezobjawową przepukliną mitralną z długoterminową obserwacją w hrabstwie Olmsted w stanie Minnesota, najlepszymi predyktorami śmiertelności sercowej były obecność niedomykalności mitralnej (MR) oraz dysfunkcja skurczowa lewej komory w momencie rozpoznania.⁴

To samo badanie zidentyfikowało określone czynniki ryzyka chorobowości sercowej (definiowanej jako wystąpienie niewydolności serca, zdarzeń zakrzepowo-zatorowych, migotania przedsionków lub potrzeby operacji kardiochirurgicznej), w tym:⁵

Wiek powyżej 50 lat
Powiększenie lewego przedsionka
Stopień niedomykalności
Obecność wiotkiego płatka zastawki
Obecność migotania przedsionków w momencie wykonania wyjściowego badania echokardiograficznego

⁵

Progresja do niedomykalności mitralnej

Około 5-10% pacjentów z przepukliną zastawki mitralnej rozwija ciężką niedomykalność mitralną (MR), co stanowi istotne powikłanie wpływające na rokowanie.⁶ Generalnie młodzi pacjenci (poniżej 50 lat) z prawidłową funkcją lewej komory i bez objawów mają doskonałe przeżycie w przypadku leczenia zachowawczego, nawet ci z ciężką niedomykalnością mitralną.⁷

Pacjenci z wysokim ryzykiem (tj. z umiarkowaną do ciężkiej niedomykalnością mitralną i obniżoną funkcją skurczową lewej komory) mają zwiększoną chorobowość i śmiertelność sercową.⁸ Osoby z ciężką niedomykalnością, które nie przejdą naprawy zastawki, mają złe rokowanie – 20% ryzyko zgonu w ciągu jednego roku i 50% ryzyko zgonu w ciągu pięciu lat.⁹

Wpływ leczenia na rokowanie

Naprawa zastawki mitralnej stanowi idealną procedurę dla pacjentów z przepukliną mitralną, z uznanymi korzyściami nad wymianą zastawki. Wynik leczenia pacjenta zależy jednak od wielu czynników, takich jak przedoperacyjne dane kliniczne i echokardiograficzne, a także doświadczenie chirurgiczne.¹⁰

Naprawa zastawki mitralnej może pomóc pacjentom żyć tak długo jak osoby bez problemów zastawkowych. Jedno z badań wykazuje, że oczekiwana długość życia osób, które przeszły naprawę zastawki jest taka sama jak oczekiwana długość życia populacji ogólnej. Dotyczyło to osób w wieku od 40 do 89 lat.¹¹

Predykcja niepowodzenia naprawy zastawki

Badania z wykorzystaniem uczenia maszynowego (ML) wskazują, że możliwe jest przewidywanie niepowodzenia naprawy zastawki mitralnej i/lub nawrotu niedomykalności mitralnej. Algorytm XGboost wykazał najlepsze zdolności dyskryminacyjne dla przewidywania niepowodzenia naprawy MV i/lub nawrotu MR na 1-miesięcznej obserwacji kontrolnej (AUC: 0,75) oraz na 3-letniej obserwacji kontrolnej (AUC: 0,92).¹²

Zdolność przewidywania chirurgicznego lub pooperacyjnego niepowodzenia naprawy zastawki mitralnej może przełożyć się na lepszy dobór kandydatów do zabiegu i może skutkować lepszym informowaniem pacjentów o ich krótko- i długoterminowym rokowaniu.¹³

Podsumowanie rokowania

Przepuklina zastawki mitralnej nie powinna być postrzegana jako złowróżbny rozpoznanie, jednak pacjenci, u których diagnoza ta jest związana z późnoskurczowym szmerem, powinni być dokładnie obserwowani ze względu na wyższe ryzyko powikłań.¹⁴ Oczekiwana długość życia dla osób z przepukliną zastawki mitralnej zależy od wielu czynników, które lekarz powinien omówić z pacjentem, informując o indywidualnej sytuacji i rokowaniu.¹⁵

Mitral valve prolapse jest najczęściej łagodnym schorzeniem, ale wymaga regularnej oceny kardiologicznej, szczególnie przy obecności czynników ryzyka gorszego rokowania lub rozwoju znaczącej niedomykalności mitralnej.¹⁶¹⁷

Kolejne rozdziały

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Materiały źródłowe

#1 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#2 Mitral Valve Prolapse: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17241-mitral-valve-prolapse
Mitral valve prolapse (MVP) is a common form of mitral valve disease that affects 2% to 3% of the general population. MVP is often harmless. Many people have no symptoms and don’t even know they have it. […] If you have a mitral valve prolapse diagnosis, talk with your provider to learn about the level of severity and what you can expect. […] The life expectancy for people with mitral valve prolapse depends on many factors. These include: […] Mitral valve repair can help you live just as long as people without valve issues. One study shows that the life expectancy of people who had a repair is the same as the life expectancy of the general population. This was true for people ages 40 to 89. […] People who have severe regurgitation but don’t get a valve repair face poor outcomes. They have a 20% chance of dying within one year and a 50% chance of dying within five years. […] Your provider can tell you about your situation and prognosis.
#3 Long-term prognosis of mitral-valve prolapse – PubMed
https://pubmed.ncbi.nlm.nih.gov/865549/
We examined the natural history of mitral-valve prolapse in 53 patients who had had a midsystolic click or late systolic murmur (or both) documented phonocardiographically a mean of 13.7 years earlier. Thirty-eight patients were alive without serious complications, and seven had died of unrelated causes. In two patients prolapse was implicated in the cause of death. Other complications were ventricular fibrillation in one patient and bacterial endocarditis in three. Progressive mitral regurgitation developed in five patients, requiring valve replacement in two. These complications occurred in a total of eight patients (15 per cent), and were significantly (P = 0.15) associated with a late systolic murmur rather than an isolated midsystolic click. Thus it appears that the diagnosis of mitral-valve prolapse should not be regarded as ominous; however, patients in whom this diagnosis is associated with a late systolic murmur should be followed carefully.
#4 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#5 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
The study also identified certain risk factors for cardiac morbidity (defined as the occurrence of heart failure, thromboembolic events, atrial fibrillation, or need for cardiac surgery), including age older than 50 years, left atrial enlargement, the degree of regurgitation, the presence of a flail leaflet, and the presence of atrial fibrillation at the time of the baseline echocardiogram.
#6 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#7 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#8 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#9 Mitral Valve Prolapse: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17241-mitral-valve-prolapse
Mitral valve prolapse (MVP) is a common form of mitral valve disease that affects 2% to 3% of the general population. MVP is often harmless. Many people have no symptoms and don’t even know they have it. […] If you have a mitral valve prolapse diagnosis, talk with your provider to learn about the level of severity and what you can expect. […] The life expectancy for people with mitral valve prolapse depends on many factors. These include: […] Mitral valve repair can help you live just as long as people without valve issues. One study shows that the life expectancy of people who had a repair is the same as the life expectancy of the general population. This was true for people ages 40 to 89. […] People who have severe regurgitation but don’t get a valve repair face poor outcomes. They have a 20% chance of dying within one year and a 50% chance of dying within five years. […] Your provider can tell you about your situation and prognosis.
#10 Machine Learning Prediction Models for Mitral Valve Repairability and Mitral Regurgitation Recurrence in Patients Undergoing Surgical Mitral Valve Repair
https://www.mdpi.com/2306-5354/8/9/117
Mitral valve regurgitation (MR) is the most common valvular heart disease and current variables associated with MR recurrence are still controversial. […] Although mitral valve (MV) repair represents the ideal procedure for MVP, with recognized advantages over MV replacement, patient outcome depends on multiple factors, such as pre-operative clinical and echocardiographic data, as well as surgical experience. […] The ability of predicting surgical or post-surgical MV repair failure may translate into a better selection of candidates and may result in better information to patients about their short- and long-term prognosis. […] However, echocardiographic and surgical variables associated with MR recurrence are still controversial, suggesting that the underlying causes for MR recurrence need to be better understood.
#11 Mitral Valve Prolapse: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17241-mitral-valve-prolapse
Mitral valve prolapse (MVP) is a common form of mitral valve disease that affects 2% to 3% of the general population. MVP is often harmless. Many people have no symptoms and don’t even know they have it. […] If you have a mitral valve prolapse diagnosis, talk with your provider to learn about the level of severity and what you can expect. […] The life expectancy for people with mitral valve prolapse depends on many factors. These include: […] Mitral valve repair can help you live just as long as people without valve issues. One study shows that the life expectancy of people who had a repair is the same as the life expectancy of the general population. This was true for people ages 40 to 89. […] People who have severe regurgitation but don’t get a valve repair face poor outcomes. They have a 20% chance of dying within one year and a 50% chance of dying within five years. […] Your provider can tell you about your situation and prognosis.
#12 Machine Learning Prediction Models for Mitral Valve Repairability and Mitral Regurgitation Recurrence in Patients Undergoing Surgical Mitral Valve Repair
https://www.mdpi.com/2306-5354/8/9/117
We found that the XGboost presented the best discriminative abilities for the prediction of MV repair failure and/or MR recurrence at 1M-FU (AUC: 0.75) and at 3Y-FU (AUC: 0.92). […] Our findings demonstrated that a ML approach based on an XGboost algorithm can predict MV repair failure at 3Y with good discrimination and significant higher PPV than LR, whereas predicting early surgical outcomes of MV repair procedures is more challenging and further research is needed.
#13 Machine Learning Prediction Models for Mitral Valve Repairability and Mitral Regurgitation Recurrence in Patients Undergoing Surgical Mitral Valve Repair
https://www.mdpi.com/2306-5354/8/9/117
Mitral valve regurgitation (MR) is the most common valvular heart disease and current variables associated with MR recurrence are still controversial. […] Although mitral valve (MV) repair represents the ideal procedure for MVP, with recognized advantages over MV replacement, patient outcome depends on multiple factors, such as pre-operative clinical and echocardiographic data, as well as surgical experience. […] The ability of predicting surgical or post-surgical MV repair failure may translate into a better selection of candidates and may result in better information to patients about their short- and long-term prognosis. […] However, echocardiographic and surgical variables associated with MR recurrence are still controversial, suggesting that the underlying causes for MR recurrence need to be better understood.
#14 Long-term prognosis of mitral-valve prolapse – PubMed
https://pubmed.ncbi.nlm.nih.gov/865549/
We examined the natural history of mitral-valve prolapse in 53 patients who had had a midsystolic click or late systolic murmur (or both) documented phonocardiographically a mean of 13.7 years earlier. Thirty-eight patients were alive without serious complications, and seven had died of unrelated causes. In two patients prolapse was implicated in the cause of death. Other complications were ventricular fibrillation in one patient and bacterial endocarditis in three. Progressive mitral regurgitation developed in five patients, requiring valve replacement in two. These complications occurred in a total of eight patients (15 per cent), and were significantly (P = 0.15) associated with a late systolic murmur rather than an isolated midsystolic click. Thus it appears that the diagnosis of mitral-valve prolapse should not be regarded as ominous; however, patients in whom this diagnosis is associated with a late systolic murmur should be followed carefully.
#15 Mitral Valve Prolapse: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17241-mitral-valve-prolapse
Mitral valve prolapse (MVP) is a common form of mitral valve disease that affects 2% to 3% of the general population. MVP is often harmless. Many people have no symptoms and don’t even know they have it. […] If you have a mitral valve prolapse diagnosis, talk with your provider to learn about the level of severity and what you can expect. […] The life expectancy for people with mitral valve prolapse depends on many factors. These include: […] Mitral valve repair can help you live just as long as people without valve issues. One study shows that the life expectancy of people who had a repair is the same as the life expectancy of the general population. This was true for people ages 40 to 89. […] People who have severe regurgitation but don’t get a valve repair face poor outcomes. They have a 20% chance of dying within one year and a 50% chance of dying within five years. […] Your provider can tell you about your situation and prognosis.
#16 Mitral Valve Prolapse: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/155494-overview
Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR). […] Overall, young patients (age 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR. […] Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates. […] In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.
#17 Mitral Valve Prolapse: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17241-mitral-valve-prolapse
Mitral valve prolapse (MVP) is a common form of mitral valve disease that affects 2% to 3% of the general population. MVP is often harmless. Many people have no symptoms and don’t even know they have it. […] If you have a mitral valve prolapse diagnosis, talk with your provider to learn about the level of severity and what you can expect. […] The life expectancy for people with mitral valve prolapse depends on many factors. These include: […] Mitral valve repair can help you live just as long as people without valve issues. One study shows that the life expectancy of people who had a repair is the same as the life expectancy of the general population. This was true for people ages 40 to 89. […] People who have severe regurgitation but don’t get a valve repair face poor outcomes. They have a 20% chance of dying within one year and a 50% chance of dying within five years. […] Your provider can tell you about your situation and prognosis.