Całkowite anomalousne powrotne żyły płucne
Zapobieganie i profilaktyka

Całkowite anomalousne powrotne żyły płucne (TAPVR) to wrodzona wada serca charakteryzująca się nieprawidłowym odpływem wszystkich czterech żył płucnych do układu żylnego systemowego lub prawego przedsionka. Pomimo braku jednoznacznej etiologii, profilaktyka obejmuje szczepienie przeciwko różyczce przed ciążą, badania genetyczne oraz kontrolę chorób przewlekłych, takich jak cukrzyca, w trakcie ciąży. Zalecane jest codzienne przyjmowanie 400 µg kwasu foliowego, unikanie alkoholu, narkotyków oraz ekspozycji na toksyczne substancje. Wczesna diagnostyka prenatalna i postnatalna jest kluczowa, gdyż opóźnienie może prowadzić do poważnych powikłań, w tym nadciśnienia płucnego i wstrząsu kardiogennego.

  1. Profilaktyka całkowitego anomalnego powrotu żył płucnych (TAPVR)
    1. Profilaktyka przedkoncepcyjna
    2. Profilaktyka w czasie ciąży
    3. Wczesne rozpoznanie i postępowanie
    4. Stabilizacja przedoperacyjna
    5. Zapobieganie powikłaniom pooperacyjnym
    6. Współpraca interdyscyplinarna
    7. Kolejne rozdziały

Profilaktyka całkowitego anomalnego powrotu żył płucnych (TAPVR)

Całkowite anomalousne powrotne żyły płucne (TAPVR) są wrodzoną wadą serca, w której wszystkie cztery żyły płucne nieprawidłowo uchodzą do układu żylnego systemowego lub do prawego przedsionka. Chociaż dokładna przyczyna większości wrodzonych wad serca, w tym TAPVR, pozostaje nieznana, istnieją pewne strategie profilaktyczne, które mogą zmniejszyć ogólne ryzyko występowania wad wrodzonych u dziecka12.

Profilaktyka przedkoncepcyjna

Kobiety planujące ciążę powinny zastosować następujące środki zapobiegawcze:3

  • Szczepienie przeciwko różyczce – Infekcja różyczką w czasie ciąży może wpłynąć na rozwój serca płodu. Kobiety powinny zostać zaszczepione przed próbą poczęcia dziecka4
  • Badania genetyczne – Niektóre czynniki dziedziczne mogą odgrywać rolę w rozwoju wrodzonych wad serca. Wiele członków rodziny może być dotkniętych podobnymi wadami. Warto skonsultować się z lekarzem w sprawie badań przesiewowych w kierunku chorób genetycznych przed zajściem w ciążę5

Profilaktyka w czasie ciąży

Kobiety w ciąży powinny stosować następujące zalecenia profilaktyczne:67

  • Regularne wizyty prenatalne – Dobra opieka prenatalna jest kluczowa dla monitorowania rozwoju dziecka i wczesnego wykrywania potencjalnych problemów8
  • Kontrola chorób przewlekłych – Kobiety z cukrzycą powinny dążyć do dobrej kontroli poziomu cukru we krwi, co może zmniejszyć ryzyko wad serca u płodu910
  • Unikanie szkodliwych substancji:
    • Alkohol i narkotyki – całkowita abstynencja w czasie ciąży11
    • Malowanie i czyszczenie produktami o silnym zapachu – te czynności powinny być wykonywane przez inne osoby12
    • Leki, zioła i suplementy diety – stosowanie wyłącznie po konsultacji z lekarzem1314
  • Suplementacja kwasu foliowego – Codzienne przyjmowanie 400 mikrogramów kwasu foliowego wykazało zmniejszenie wad wrodzonych mózgu i rdzenia kręgowego oraz może pomóc w zmniejszeniu ryzyka wad serca15
  • Badanie na odporność przeciwko różyczce – Przeprowadzenie badania krwi na początku ciąży w celu sprawdzenia odporności na różyczkę. W przypadku braku odporności, należy unikać potencjalnej ekspozycji na różyczkę i zaszczepić się bezpośrednio po porodzie16

Wczesne rozpoznanie i postępowanie

Chociaż według niektórych źródeł nie ma znanego sposobu zapobiegania TAPVR17, wczesna diagnoza i interwencja są kluczowe dla poprawy rokowania:18

  • Wczesna diagnostyka – Opóźnienie w diagnozie i skierowaniu może prowadzić do poważnych konsekwencji, takich jak ciężkie nadciśnienie płucne, wstrząs kardiogenny i śmiertelność19
  • Leczenie chirurgiczne – Korekcyjna operacja jest konieczna dla wszystkich pacjentów z tą wadą. Nie istnieje procedura paliatywna. Wszystkie niemowlęta z niedrożnością żył płucnych powinny być operowane wkrótce po diagnozie, w okresie noworodkowym20

Stabilizacja przedoperacyjna

Optymalna stabilizacja pacjenta przed operacją z punktu widzenia sercowo-naczyniowego i metabolicznego jest istotna:21

Zapobieganie powikłaniom pooperacyjnym

Po operacji TAPVR istotne jest zapobieganie powikłaniom, szczególnie nadciśnieniu płucnemu:2425

  • Zapobieganie nadciśnieniu płucnemu (PAH) – Jest to najskuteczniejsza strategia postępowania w przypadku nadciśnienia płucnego26
  • Odpowiednia sedacja i analgezja – Szczególnie podczas odsysania przez rurkę dotchawiczą27
  • Środki zwiotczające i głęboka sedacja – Jeśli hemodynamika jest stabilna28
  • Utrzymanie zasadowicy oddechowej – Utrzymanie pH 7,45-7,5 poprzez hiperwentylację w sytuacji ostrego przełomu nadciśnienia płucnego29
  • Farmakoterapia – Bosentan/Sildenafil w przypadku braku dostępności tlenku azotu do inhalacji30
  • Leki inotropowe – Stosowane w celu utrzymania rzutu serca31
  • ECMO – Pozaustrojowa oksygenacja membranowa stała się najszerzej stosowaną metodą mechanicznego wspomagania krążeniowo-oddechowego u dzieci po operacjach kardiochirurgicznych32

Współpraca interdyscyplinarna

Postępowanie z noworodkiem lub niemowlęciem z TAPVR wymaga ścisłej współpracy między lekarzem kierującym, kardiologiem dziecięcym, kardiochirurgiem dziecięcym oraz intensywistą33. Taka interdyscyplinarna opieka jest kluczowa dla uzyskania optymalnych wyników leczenia.

Wczesna korekcja chirurgiczna TAPVR pozwala dziecku na zasadniczo normalny wzrost i rozwój z dobrą jakością życia, dlatego szybka diagnoza i odpowiednia interwencja mają kluczowe znaczenie profilaktyczne34.

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 19.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #2
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #3 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #4 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #5 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #6 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #7 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #8 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #9 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #10 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #11 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #12 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #13 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #14 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #15 Total anomalous pulmonary venous return (TAPVR)
    https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Symptoms
    Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent these conditions. However, there are some things you can do that might reduce your child’s overall risk of birth defects and possibly heart defects, too, such as: […] Get a rubella (German measles) vaccine. A rubella infection during pregnancy may affect your baby’s heart development. Be sure to get vaccinated before you try to conceive. […] Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor. […] Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don’t take any drugs, herbs or dietary supplements without consulting your doctor first. Don’t smoke or drink alcohol during pregnancy. […] Take a multivitamin with folic acid. Daily consumption of 400 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord and may help reduce the risk of heart defects as well.
  • #16 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001115.htm
    Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease. […] Women who are pregnant should get good prenatal care: […] Avoid alcohol and illegal drugs during pregnancy. […] Tell your provider that you are pregnant before taking any new medicines. […] Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. […] Pregnant women who have diabetes should try to get good control over their blood sugar level. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
  • #17 FloridaHealthFinder | Total anomalous pulmonary venous return | Health Encyclopedia | FloridaHealthFinder
    https://quality.healthfinder.fl.gov/health-encyclopedia/HIE////1/001115
    There is no known way to prevent TAPVR.
  • #18
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #19
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #20
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #21
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #22
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.
  • #23
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    If the size of the interatrial communication appears small and immediate surgery is not indicated, balloon atrial septostomy or blade atrial septostomy may be performed to enlarge the communication. […] The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] The complication of pulmonary venous obstruction occurs later in 5-10% of patients and is usually evident in the first 6 months following surgery. […] The most effective strategy to manage PAH (Pulmonary artery hypertension) is prevention. […] Maintenance of adequate sedation and analgesia, particularly during ET suctioning.
  • #24
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    If the size of the interatrial communication appears small and immediate surgery is not indicated, balloon atrial septostomy or blade atrial septostomy may be performed to enlarge the communication. […] The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] The complication of pulmonary venous obstruction occurs later in 5-10% of patients and is usually evident in the first 6 months following surgery. […] The most effective strategy to manage PAH (Pulmonary artery hypertension) is prevention. […] Maintenance of adequate sedation and analgesia, particularly during ET suctioning.
  • #25
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #26
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    If the size of the interatrial communication appears small and immediate surgery is not indicated, balloon atrial septostomy or blade atrial septostomy may be performed to enlarge the communication. […] The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] The complication of pulmonary venous obstruction occurs later in 5-10% of patients and is usually evident in the first 6 months following surgery. […] The most effective strategy to manage PAH (Pulmonary artery hypertension) is prevention. […] Maintenance of adequate sedation and analgesia, particularly during ET suctioning.
  • #27
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    If the size of the interatrial communication appears small and immediate surgery is not indicated, balloon atrial septostomy or blade atrial septostomy may be performed to enlarge the communication. […] The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] The complication of pulmonary venous obstruction occurs later in 5-10% of patients and is usually evident in the first 6 months following surgery. […] The most effective strategy to manage PAH (Pulmonary artery hypertension) is prevention. […] Maintenance of adequate sedation and analgesia, particularly during ET suctioning.
  • #28
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #29
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #30
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #31
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #32
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #33
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Muscle relaxant and deep sedation if hemodynamics are stable. […] Maintaining a pH of 7.45 -7.5, i.e. respiratory alkalosis in acute pulmonary hypertensive crisis situation, by hyperventilation. […] Bosentan/Sildenfil in the absence of inhaled nitric oxide. […] Inotropes to maintain cardiac output. […] Extracorporeal membrane oxygenation (ECMO) has become the most widely used mode of mechanical cardiopulmonary support for children after cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist.
  • #34
    https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx
    Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. […] If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. […] Corrective surgery is necessary for all patients with this condition. No palliative procedure exists. All infants with pulmonary venous obstruction should be operated on soon after diagnosis, in the newborn period. […] Optimal stabilization the patient prior to surgery from a cardiovascular and metabolic standpoint is important. […] Intensive anti congestive measures with digitalis and diuretics should be provided for infants without pulmonary venous obstruction.

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