Zwężenie cieśni aorty
Epidemiologia

Zwężenie cieśni aorty (CoA) jest wrodzoną wadą serca stanowiącą 5-8% wszystkich wrodzonych wad, z częstością występowania około 4 na 10 000 żywych urodzeń, częściej u mężczyzn (stosunek płci około 2:1). Wada ta wykazuje silny komponent genetyczny, szczególnie w zespole Turnera (12-35% przypadków CoA), a także w innych zespołach genetycznych, takich jak Williamsa czy Noonana. CoA często współistnieje z innymi wadami serca, zwłaszcza z dwupłatkową zastawką aortalną (>70% pacjentów) oraz ubytkiem przegrody międzykomorowej. Diagnostyka prenatalna jest trudna, z czułością echokardiografii płodowej na poziomie 50-72%, a pulsoksymetria noworodkowa ma czułość około 80%, co wymaga wzmożonej czujności klinicznej i regularnych badań kontrolnych u noworodków z podejrzeniem CoA.

Epidemiologia zwężenia cieśni aorty

Zwężenie cieśni aorty (CoA) jest stosunkowo częstą wrodzoną wadą serca, stanowiącą około 5-8% wszystkich wrodzonych wad serca123. Według różnych źródeł, występuje z częstością około 4 przypadków na 10 000 żywych urodzeń456. W niektórych źródłach podaje się jednak nieco odmienne dane, wskazując na występowanie wady u 1 na 2500 urodzeń78.

Różnice płciowe i rasowe

Zwężenie cieśni aorty występuje częściej u mężczyzn niż u kobiet, ze stosunkiem płci męskiej do żeńskiej wynoszącym około 2:191011. Niektóre badania wskazują na nieco niższy stosunek, wahający się między 1,27:1 a 1,74:1121314. Warto zaznaczyć, że zwężenie cieśni aorty w lokalizacji brzusznej występuje głównie u kobiet, co stanowi wyjątek od ogólnej tendencji15.

Obserwuje się również różnice w występowaniu CoA w zależności od rasy. Wada ta jest około siedem razy częstsza u osób rasy kaukaskiej niż u osób pochodzenia azjatyckiego1617. Ogólnie, częstość występowania zwężenia cieśni aorty jest niższa (około 2%) w krajach azjatyckich w porównaniu do krajów europejskich i północnoamerykańskich1819.

Uwarunkowania genetyczne

Epidemiologia zwężenia cieśni aorty ujawnia istotny komponent genetyczny. Zespół Turnera (kariotyp 45,X0) jest silnie związany z tą wadą – u 12-35% pacjentek z zespołem Turnera występuje zwężenie cieśni aorty202122. U dziewcząt ze zdiagnozowanym zwężeniem cieśni aorty zaleca się badania przesiewowe kariotypu23.

Krewni pierwszego stopnia osób z obstrukcyjnymi lewostronymi wadami serca mają 10-krotnie zwiększone ryzyko rozwoju zwężenia cieśni aorty i innych wrodzonych wad serca2425. Badania epidemiologiczne wykazały, że w przypadku wad drogi odpływu lewej komory istnieje większe prawdopodobieństwo zgodnej diagnozy u wielu członków rodziny26.

Poza zespołem Turnera, zwiększoną częstość występowania zwężenia cieśni aorty zaobserwowano również w innych zespołach genetycznych, takich jak zespół Williamsa, zespół Noonana, zespół Rubenstein-Taybi, zespół Alagille’a, neurofibromatoza, zespół Kabuki oraz zespół PHACES27.

Współwystępowanie z innymi wadami

Zwężenie cieśni aorty często współwystępuje z innymi wrodzonymi wadami serca. Najczęstszą towarzyszącą anomalią jest dwupłatkowa zastawka aortalna, występująca u ponad 70% pacjentów z CoA28. Inne współwystępujące wady to ubytek przegrody międzykomorowej, małe struktury lewostronne (znane również jako wariant/kompleks Shone’a), a także warianty anatomii pojedynczej komory29.

Warto podkreślić, że obecność hipoplazji łuku aorty ma istotne znaczenie dla długoterminowego ryzyka rozwoju nadciśnienia tętniczego, co zwiększa ryzyko rozwoju innych chorób współistniejących i wymaga dożywotniej opieki medycznej30.

Diagnostyka prenatalna i wczesne wykrywanie

Prenatalna diagnostyka zwężenia cieśni aorty jest niezwykle trudna, z wysokim odsetkiem wyników fałszywie dodatnich w badaniach przesiewowych opartych na pulsoksymetrii i echokardiografii płodowej3132. Większość rzeczywistych przypadków CoA jest pomijana w diagnostyce prenatalnej, co skłania klinicystów do zwiększonej czujności i wzmożonego nadzoru u noworodków z podejrzeniem tej wady3334.

Badania analizujące skuteczność echokardiografii płodowej w prenatalnej diagnostyce CoA wykazały, że modele wieloparametryczne mają lepszą skuteczność diagnostyczną niż jakikolwiek pojedynczy ilościowy parametr echokardiograficzny35. W zależności od wieku ciążowego w momencie początkowego podejrzenia, badania echokardiograficzne płodu mogą być wykonywane co 4-6 tygodni, z ostatnią oceną po 30 tygodniu ciąży36.

Czułość diagnostyki płodowej CoA waha się od 50% do 72%, a opóźniona diagnoza zwężenia cieśni aorty jest nadal powszechna37. Niemowlęta, które umykają diagnostyce prenatalnej lub wczesnej diagnostyce noworodkowej, mogą prezentować wstrząs kardiogenny, który może być związany z głęboką dysfunkcją komorową38.

Badania przesiewowe po urodzeniu

Najlepszym sposobem badania przesiewowego pacjentów pod kątem wrodzonych wad serca jest pulsoksymetria wykonywana 24-48 godzin po urodzeniu39. Należy jednak pamiętać, że czułość pulsoksymetrii jako metody przesiewowej wynosi 80%, a fałszywie ujemne wyniki pulsoksymetrii nie są rzadkie40.

Zwężenie cieśni aorty jest często diagnozowane przy pierwszym badaniu noworodka lub podczas rutynowych badań niemowlęcia. Badanie tętna u niemowlęcia jest ważnym elementem badania, ponieważ mogą nie występować żadne inne objawy, dopóki dziecko nie będzie starsze41.

Nadzór i monitorowanie pacjentów

Zwężenie cieśni aorty stanowi schorzenie wymagające dożywotniego nadzoru medycznego. Nawet po pozornie udanej naprawie w okresie niemowlęcym istnieje wysokie ryzyko ponownej operacji, szacowane na około 50% do piątej dekady życia4243. Długoterminowe dane z obserwacji pacjentów z naprawionym CoA wykazują skrócenie oczekiwanej długości życia w porównaniu z populacją ogólną, z wysokim wskaźnikiem niekorzystnych zdarzeń sercowo-naczyniowych4445.

Algorytmy obserwacji i nadzoru

American College of Cardiology (ACC) opracowało algorytm praktyki klinicznej dla pacjentów z naprawionym zwężeniem cieśni aorty. Jest to narzędzie wspomagające decyzje oparte na wieku, które może być stosowane w obserwacji i nadzorze pacjentów z izolowanym naprawionym CoA przez całe życie46.

Każda ścieżka algorytmu zawiera sugerowany przewodnik dotyczący częstotliwości wizyt klinicznych i rodzaju badań, przy czym młodszy wiek i bardziej-niż-łagodne zmiany resztkowe wymagają ściślejszej obserwacji47. Starsze dzieci z naprawionym CoA z łagodnymi lub bez zmian resztkowych mogą być obserwowane rzadziej (nawet co 2 lata) z zastosowaniem echokardiografii48.

Tomografia komputerowa serca i rezonans magnetyczny serca są przydatnymi uzupełnieniami echokardiografii w seryjnej ocenie starszych dzieci i dorosłych z naprawionym CoA49. Według aktualnych wytycznych, każdy pacjent z CoA, naprawionym lub nie, powinien mieć co najmniej jedno badanie sercowo-naczyniowe MRI lub CT w celu określenia lokalizacji i ciężkości zwężenia, a także oceny przepływu obocznego50.

Monitorowanie nadciśnienia tętniczego

Po naprawie CoA pacjenci powinni być monitorowani pod kątem nadciśnienia tętniczego systemowego i maskowanego51. Pacjenci z naprawionym CoA mogą nadal mieć lub rozwijać nowe nadciśnienie tętnicze nawet po wczesnej naprawie i przy optymalnych wynikach chirurgicznych52.

Nawet po całkowitym usunięciu zwężenia, CoA jest związane z dysfunkcją rozkurczową niezależnie od obecności choroby wieńcowej5354. Nadciśnienie pozostaje kluczowym czynnikiem ryzyka niekorzystnych wyników sercowo-naczyniowych55.

Powikłania i rokowanie długoterminowe

Pomimo postępów w zakresie wczesnego wykrywania i leczenia, zwężenie cieśni aorty pozostaje istotnym czynnikiem przyczyniającym się do zachorowalności i śmiertelności sercowo-naczyniowej, szczególnie gdy współwystępuje z innymi wadami wrodzonymi56.

Długoterminowe powikłania obejmują ponowne zwężenie (rekoarktację) i tworzenie się tętniaków, z ryzykiem życiowym wynoszącym od 13 do 8% w zależności od pierwotnego typu naprawy57. Rekoarktacja występuje u jednej trzeciej pacjentów i zwraca uwagę na potrzebę dożywotniego nadzoru za pomocą echokardiografii5859.

Bez leczenia większość osób umiera przed 40 rokiem życia60. Średnia przeżywalność nieleczonych dorosłych ze zwężeniem cieśni aorty wynosi 35 lat, a śmiertelność wzrasta do 75% do 46 roku życia6162.

Ryzyko naczyń mózgowych

Tętniaki wewnątrzczaszkowe (ICA) występują u pacjentów ze zwężeniem cieśni aorty, narażając ich na ryzyko udaru mózgu63. Pacjenci ci cierpią zarówno na udar niedokrwienny, jak i krwotoczny w znacznie młodszym wieku w porównaniu do populacji ogólnej64.

Mogą istnieć modyfikowalne czynniki ryzyka, takie jak długotrwałe nadciśnienie tętnicze, które mogą zwiększać ryzyko rozwoju tętniaków wewnątrzczaszkowych u pacjentów z CoA6566. Z drugiej strony, dzieci z CoA, które są leczone wcześnie w życiu, nie wydają się mieć wyższej częstości występowania tętniaków wewnątrzczaszkowych67.

Zmiana podejścia do zwężenia cieśni aorty

Coraz więcej dowodów wskazuje, że zwężenie cieśni aorty stanowi uogólnioną arteriopatię, prowadzącą do zwiększonego ryzyka sercowo-naczyniowego przez całe życie pacjenta oraz konieczności dożywotniego nadzoru przez specjalistę chorób wrodzonych serca dorosłych6869.

Kiedyś uważano, że zwężenie cieśni aorty jest prostą wadą, jednak obecnie zdajemy sobie sprawę, że jest to znacznie więcej niż tylko zwężenie aorty70. Gromadzące się dowody dotyczące reaktywności naczyń i funkcji śródbłonka u pacjentów z CoA sugerują, że CoA nie jest prostą mechaniczną przeszkodą, którą można wyleczyć chirurgicznie71.

Zwężenie cieśni aorty prowadzi do przeciążenia ciśnieniowego lewej komory i niekorzystnej przebudowy lewej komory. Histologicznie charakteryzuje się to przerostem miocytów, zaburzeniem ich układu i włóknieniem72. Nawet po całkowitym usunięciu zwężenia, CoA jest związane z dysfunkcją rozkurczową niezależnie od obecności choroby wieńcowej73.

Te cechy nie są zapobiegane przez korekcję chirurgiczną, co potwierdza, że zwężenie cieśni aorty jest uogólnioną waskulopatią daleko wykraczającą poza zwężenie łuku aorty74.

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  1. 09.04.2026
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Materiały źródłowe

  • #1 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #2 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    Coarctation of the aorta (CoA) accounts for approximately 5–8% of all congenital heart defects. […] Developments in diagnosis and management have improved outcomes in this patient population. Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime. […] Coarctation of the aorta (CoA) is one of the most common congenital heart defects (CHD), accounting for approximately 5–8% of CHD, with an incidence of 4 per 10,000 live births. […] There have been significant advances in the diagnosis and management of CoA with the advent of multi-modality imaging, rapid advances in interventional techniques and the accumulation of long-term data for patients who have been operated on in childhood.
  • #3 Aortic Coarctation: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/150369-overview
    Coarctation of aorta represents 5%-8% of all congenital heart diseases, with the isolated form comprising 4%-6% of all congenital heart diseases. The prevalence of isolated forms is about 3-4 per 10,000 live births, and males are affected more frequently than females. […] Aortic coarctation is seven times more common in white persons than Asian persons. It has a lower incidence among Native Americans than other population groups in Minnesota. […] The male-to-female predominance is 1.3-2:1 in most series. […] The age at detection of coarctation of the aorta is dependent on the severity of the obstruction and the coexistence of other lesions.
  • #4 Clinical manifestations and diagnosis of coarctation of the aorta – UpToDate
    https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-coarctation-of-the-aorta
    CoA accounts for 4 to 6 percent of all congenital heart defects with a reported prevalence of approximately 4 per 10,000 live births. It occurs more commonly in males than in females (59 versus 41 percent). Most cases are sporadic. […] Although CoA can rarely be acquired, the vast majority of CoA cases are congenital.
  • #5 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    Coarctation of the aorta (CoA) accounts for approximately 5–8% of all congenital heart defects. […] Developments in diagnosis and management have improved outcomes in this patient population. Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime. […] Coarctation of the aorta (CoA) is one of the most common congenital heart defects (CHD), accounting for approximately 5–8% of CHD, with an incidence of 4 per 10,000 live births. […] There have been significant advances in the diagnosis and management of CoA with the advent of multi-modality imaging, rapid advances in interventional techniques and the accumulation of long-term data for patients who have been operated on in childhood.
  • #6 Coarctation of the Aorta | Concise Medical Knowledge
    https://www.lecturio.com/concepts/coarctation-of-the-aorta/
    Coarctation of the aorta is typically congenital and the clinical presentation depends on the age of the patient. […] Incidence in the United States: 4 per 10,000 live births. […] Accounts for approximately 6% of all congenital heart disease. […] More common in boys (2:1 men:women ratio). […] Up to 15% of girls with coarctation of the aorta have Turner syndrome. […] Accompanied by bicuspid aortic valve in over 70% of cases. […] Close follow-up is required as the risk of hypertension and re-coarctation remain.
  • #7 Coarctation of the Aorta (CoA) | Doctor
    https://patient.info/doctor/coarctation-of-the-aorta-pro
    There are no reliable figures for population incidence, estimated at 1 in 2,500 births. […] It is possibly more common in Caucasians and males. […] Affected parents have a higher risk of the abnormality affecting their child. […] Age at detection is determined by severity and whether there are other associated abnormalities.
  • #8 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Coarctation of the aorta (CoA) is the fifth most common congenital heart defect, accounting for 68% of live births with congenital heart disease, with an estimated incidence of 1 in 2500 births. […] It affects more male babies than female, with a reported ratio in males of between 1.27:1 and 1.74:1. […] The presence of associated arch hypoplasia is relevant to longer term risk for the development of hypertension so in addition to re-coarctation, these patients are at increased risk for developing other comorbidities and should have lifelong follow up care. […] One genetic condition noted to be associated with an increased risk of having coarctation of the aorta (12-35%) is Turner syndrome. […] Lastly, the etiology of CoA is not well understood and thought to be affected by various factors including a genetic component, environmental factors, and arteriopathy.
  • #9 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #10 Coarctation of the Aorta: Background, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/895502-overview
    Coarctation of the aorta is a common defect and occurs in 6-8% of patients with congenital heart disease. […] The prevalence of coarctation of the aorta appears to be lower (2%) in Asian countries than in European and North American countries. […] No definitive racial differences have been documented in coarctation of the aorta, although some authors have suggested that coarctation of the aorta is less common in Asians. […] The male-to-female ratio is 2:1, although this ratio is not valid in abdominal coarctation of the aorta, in which this rare lesion predominantly affects females. […] Generally, patients with coarctation of the aorta present early in life with CHF or later in life with hypertension. Studies continue to document that coarctation of the aorta is often missed in the first year of life, and the median age of referral to a pediatric cardiologist in one study was 5 years.
  • #11 Coarctation of the aorta | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/coarctation-of-the-aorta?lang=us
    Coarctations account for between 5-8% of all congenital heart defects. They are more frequent in males, M:F ratio of ~2-3:1. […] As with many congenital abnormalities, coarctation of the aorta is associated with other congenital anomalies.
  • #12 Management of adults with coarctation of aorta
    https://www.wjgnet.com/1949-8462/full/v12/i5/167.htm
    Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. […] CoA accounts for 4%-6% of all congenital heart defects, with an incidence is of 3-4 cases per 10000 live births. […] Males are commonly affected than females with a ratio between 1.27:1 and 1.74:1, respectively. […] Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. […] Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which remains the greatest cause of long-term mortality. […] Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.
  • #13 Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus | The Journal of Physiological Sciences | Full Text
    https://jps.biomedcentral.com/articles/10.1007/s12576-016-0512-x
    Coarctation of the aorta is the fifth or sixth most common congenital heart defect, accounting for 58% of neonates with congenital heart diseases. The incidence of CoA has been reported to one per 2500 births and the ratio of males to females with the condition to be between 1.27:1 and 1.74:1. In these studies, CoA was considered to be hemodynamically dominant in complex congenital heart defects with a left-to-right shunt. […] Early diagnosis and appropriate management of coarctation of the aorta (CoA) have resulted in a low mortality rate associated with the repair of CoA. It has been suggested that patients with CoA are at increased risk of myocardial infarction because systemic hypertension and premature atherosclerosis tend to develop early in life even after successful surgical treatment. The increased cardiovascular risk in this patient group is the result of both frequent relapses of hypertension and proatherogenic abnormalities that are present even in the absence of arterial hypertension. […] Accumulating evidence of vasoreactivity and endothelial function in patients with CoA suggests that CoA is not a simple mechanical obstruction that can be cured surgically.
  • #14 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Coarctation of the aorta (CoA) is the fifth most common congenital heart defect, accounting for 68% of live births with congenital heart disease, with an estimated incidence of 1 in 2500 births. […] It affects more male babies than female, with a reported ratio in males of between 1.27:1 and 1.74:1. […] The presence of associated arch hypoplasia is relevant to longer term risk for the development of hypertension so in addition to re-coarctation, these patients are at increased risk for developing other comorbidities and should have lifelong follow up care. […] One genetic condition noted to be associated with an increased risk of having coarctation of the aorta (12-35%) is Turner syndrome. […] Lastly, the etiology of CoA is not well understood and thought to be affected by various factors including a genetic component, environmental factors, and arteriopathy.
  • #15 Coarctation of the Aorta: Background, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/895502-overview
    Coarctation of the aorta is a common defect and occurs in 6-8% of patients with congenital heart disease. […] The prevalence of coarctation of the aorta appears to be lower (2%) in Asian countries than in European and North American countries. […] No definitive racial differences have been documented in coarctation of the aorta, although some authors have suggested that coarctation of the aorta is less common in Asians. […] The male-to-female ratio is 2:1, although this ratio is not valid in abdominal coarctation of the aorta, in which this rare lesion predominantly affects females. […] Generally, patients with coarctation of the aorta present early in life with CHF or later in life with hypertension. Studies continue to document that coarctation of the aorta is often missed in the first year of life, and the median age of referral to a pediatric cardiologist in one study was 5 years.
  • #16 Aortic Coarctation: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/150369-overview
    Coarctation of aorta represents 5%-8% of all congenital heart diseases, with the isolated form comprising 4%-6% of all congenital heart diseases. The prevalence of isolated forms is about 3-4 per 10,000 live births, and males are affected more frequently than females. […] Aortic coarctation is seven times more common in white persons than Asian persons. It has a lower incidence among Native Americans than other population groups in Minnesota. […] The male-to-female predominance is 1.3-2:1 in most series. […] The age at detection of coarctation of the aorta is dependent on the severity of the obstruction and the coexistence of other lesions.
  • #17 Aortic coarctation epidemiology and demographics – wikidoc
    https://www.wikidoc.org/index.php/Aortic_coarctation_epidemiology_and_demographics
    Coarctation of the aorta is a common congenital malformation. It occurs in about 7% of patients with congenital heart defects. It is more common in males than females with a ratio of 2:1. Up to 25% of patients with Turner syndrome have coarctation of the aorta. It is 7 times more common among caucasians than asians. […] Aortic coarctation is a common heart defect. It forms approximately 6-10% of all congenital heart disease cases. In live births, it accounts for approximately 5-7% of congenital heart disease in severely ill infants. […] The prevalence is lower in Asian countries compared to American and European countries.
  • #18 Coarctation of the Aorta: Background, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/895502-overview
    Coarctation of the aorta is a common defect and occurs in 6-8% of patients with congenital heart disease. […] The prevalence of coarctation of the aorta appears to be lower (2%) in Asian countries than in European and North American countries. […] No definitive racial differences have been documented in coarctation of the aorta, although some authors have suggested that coarctation of the aorta is less common in Asians. […] The male-to-female ratio is 2:1, although this ratio is not valid in abdominal coarctation of the aorta, in which this rare lesion predominantly affects females. […] Generally, patients with coarctation of the aorta present early in life with CHF or later in life with hypertension. Studies continue to document that coarctation of the aorta is often missed in the first year of life, and the median age of referral to a pediatric cardiologist in one study was 5 years.
  • #19 Aortic coarctation epidemiology and demographics – wikidoc
    https://www.wikidoc.org/index.php/Aortic_coarctation_epidemiology_and_demographics
    Coarctation of the aorta is a common congenital malformation. It occurs in about 7% of patients with congenital heart defects. It is more common in males than females with a ratio of 2:1. Up to 25% of patients with Turner syndrome have coarctation of the aorta. It is 7 times more common among caucasians than asians. […] Aortic coarctation is a common heart defect. It forms approximately 6-10% of all congenital heart disease cases. In live births, it accounts for approximately 5-7% of congenital heart disease in severely ill infants. […] The prevalence is lower in Asian countries compared to American and European countries.
  • #20 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #21 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Coarctation of the aorta (CoA) is the fifth most common congenital heart defect, accounting for 68% of live births with congenital heart disease, with an estimated incidence of 1 in 2500 births. […] It affects more male babies than female, with a reported ratio in males of between 1.27:1 and 1.74:1. […] The presence of associated arch hypoplasia is relevant to longer term risk for the development of hypertension so in addition to re-coarctation, these patients are at increased risk for developing other comorbidities and should have lifelong follow up care. […] One genetic condition noted to be associated with an increased risk of having coarctation of the aorta (12-35%) is Turner syndrome. […] Lastly, the etiology of CoA is not well understood and thought to be affected by various factors including a genetic component, environmental factors, and arteriopathy.
  • #22 Coarctation of the Aorta | Concise Medical Knowledge
    https://www.lecturio.com/concepts/coarctation-of-the-aorta/
    Coarctation of the aorta is typically congenital and the clinical presentation depends on the age of the patient. […] Incidence in the United States: 4 per 10,000 live births. […] Accounts for approximately 6% of all congenital heart disease. […] More common in boys (2:1 men:women ratio). […] Up to 15% of girls with coarctation of the aorta have Turner syndrome. […] Accompanied by bicuspid aortic valve in over 70% of cases. […] Close follow-up is required as the risk of hypertension and re-coarctation remain.
  • #23 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #24 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #25 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/chd/coarctation-aorta
    Coarctation is found in 6% to 8% of patients with congenital heart disease. Associated anomalies include bicuspid aortic valve, ventricular septal defect, small left sided structures (also known as Shones variant/complex) as well as variants of single ventricle anatomy. Coarctation of the aorta also accounts for a small percentage of children diagnosed later in life who present with systemic hypertension. […] There is an increased prevalence of coarctation in various genetic syndromes such as Turners syndrome (XO), Williams Syndrome, Noonan Syndrome, Rubenstein-Taybi syndrome, Alagille Syndrome, Neurofibromatosis, Kabuki Syndrome and PHACES syndrome. There is a slight male predominance. Offspring and other first-degree relatives diagnosed with an obstructive left-sided cardiac lesion are at ten times the risk of coarctation and other cardiac lesions.
  • #26 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Epidemiological studies have found that for left ventricular outflow tract lesions, there is a higher chance of concordant diagnosis in multiple family members. […] All patients with coarctation (repaired or not) should be monitored with lifelong congenital cardiology follow-up and imaging because long-term survival is reduced compared with normative populations and there is potential need for reintervention. […] The unoperated mean survival rate of adults with coarctation of the aorta is 35 years of age, with a mortality rate of 75% by 46 years of age. […] In general, the patients with CoA who are repaired at a later age are more likely to remain hypertensive. […] The 2015 scientific statement of the AHA/ACC provides competitive athletic participation guidelines for patients with congenital heart disease (CHD), including coarctation.
  • #27 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/chd/coarctation-aorta
    Coarctation is found in 6% to 8% of patients with congenital heart disease. Associated anomalies include bicuspid aortic valve, ventricular septal defect, small left sided structures (also known as Shones variant/complex) as well as variants of single ventricle anatomy. Coarctation of the aorta also accounts for a small percentage of children diagnosed later in life who present with systemic hypertension. […] There is an increased prevalence of coarctation in various genetic syndromes such as Turners syndrome (XO), Williams Syndrome, Noonan Syndrome, Rubenstein-Taybi syndrome, Alagille Syndrome, Neurofibromatosis, Kabuki Syndrome and PHACES syndrome. There is a slight male predominance. Offspring and other first-degree relatives diagnosed with an obstructive left-sided cardiac lesion are at ten times the risk of coarctation and other cardiac lesions.
  • #28 Coarctation of the Aorta | Concise Medical Knowledge
    https://www.lecturio.com/concepts/coarctation-of-the-aorta/
    Coarctation of the aorta is typically congenital and the clinical presentation depends on the age of the patient. […] Incidence in the United States: 4 per 10,000 live births. […] Accounts for approximately 6% of all congenital heart disease. […] More common in boys (2:1 men:women ratio). […] Up to 15% of girls with coarctation of the aorta have Turner syndrome. […] Accompanied by bicuspid aortic valve in over 70% of cases. […] Close follow-up is required as the risk of hypertension and re-coarctation remain.
  • #29 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/chd/coarctation-aorta
    Coarctation is found in 6% to 8% of patients with congenital heart disease. Associated anomalies include bicuspid aortic valve, ventricular septal defect, small left sided structures (also known as Shones variant/complex) as well as variants of single ventricle anatomy. Coarctation of the aorta also accounts for a small percentage of children diagnosed later in life who present with systemic hypertension. […] There is an increased prevalence of coarctation in various genetic syndromes such as Turners syndrome (XO), Williams Syndrome, Noonan Syndrome, Rubenstein-Taybi syndrome, Alagille Syndrome, Neurofibromatosis, Kabuki Syndrome and PHACES syndrome. There is a slight male predominance. Offspring and other first-degree relatives diagnosed with an obstructive left-sided cardiac lesion are at ten times the risk of coarctation and other cardiac lesions.
  • #30 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Coarctation of the aorta (CoA) is the fifth most common congenital heart defect, accounting for 68% of live births with congenital heart disease, with an estimated incidence of 1 in 2500 births. […] It affects more male babies than female, with a reported ratio in males of between 1.27:1 and 1.74:1. […] The presence of associated arch hypoplasia is relevant to longer term risk for the development of hypertension so in addition to re-coarctation, these patients are at increased risk for developing other comorbidities and should have lifelong follow up care. […] One genetic condition noted to be associated with an increased risk of having coarctation of the aorta (12-35%) is Turner syndrome. […] Lastly, the etiology of CoA is not well understood and thought to be affected by various factors including a genetic component, environmental factors, and arteriopathy.
  • #31 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. […] A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings. […] The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis.
  • #32 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patient’s lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist. […] CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings.
  • #33 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis. […] Studies analysing the performance of fetal echocardiography in the prenatal diagnosis of CoA have found that multiparametric models have a superior diagnostic performance to any one quantitative echocardiographic measure. […] CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. Histologically, this is characterised by myocyte hypertrophy, disarray and fibrosis. Ensuing alteration in myocardial mechanical properties manifests in the form of diastolic and systolic dysfunction, with LV failure being a significant cause of mortality. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease.
  • #34 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189?type=check_update&version=2
    The development of coordinated care for patients with adult congenital heart disease in the developed world has also led to improvements in outcomes. […] There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patient’s lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist. […] CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings.
  • #35 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis. […] Studies analysing the performance of fetal echocardiography in the prenatal diagnosis of CoA have found that multiparametric models have a superior diagnostic performance to any one quantitative echocardiographic measure. […] CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. Histologically, this is characterised by myocyte hypertrophy, disarray and fibrosis. Ensuing alteration in myocardial mechanical properties manifests in the form of diastolic and systolic dysfunction, with LV failure being a significant cause of mortality. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease.
  • #36 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/fetal/fetal-coa
    Prevalence of ~4 per 10 000 live births. […] Depending on the gestational age at the time of initial suspicion, fetal echocardiograms may be performed every 46 weeks, with the last evaluation obtained after 30 weeks. At the very least, one follow-up evaluation in the third trimester is reasonable to assess for progression of abnormalities and to detect those that may not have been evident on the initial scan. An important echocardiographic predictor for CoA is progression in the degree of hypoplasia of the LV and aortic dimensions during serial evaluations. […] Surveillance should include assessment of left heart growth (LV, mitral valve, aortic valve, aortic arch) with careful attention paid to detailed quantification of aortic dimensions and Doppler assessment of the left heart and aortic arch. This will assist the clinician to more reasonably predict postnatal expectations and whether or not PGE would be required postnatally.
  • #37 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/fetal/fetal-coa
    Coarctation of the aorta (CoA) is a narrowing of the aortic arch. It most commonly occurs in the region of the aortic isthmus (between the left subclavian artery and ductus arteriosus). Patients with a severe coarctation of the aorta are dependent on a patent ductus arteriosus (PDA) to preserve systemic blood flow and perfusion. CoA has a variable spectrum of severity, ranging from unequivocal ductal-dependent systemic blood flow, to patients with borderline small arch dimensions who also have a PDA. […] The sensitivity of fetal diagnosis of CoA varies from 50% to 72%, and delayed diagnosis of CoA is still common. Infants who elude prenatal or early neonatal diagnosis may present in cardiogenic shock that may be associated with profound ventricular dysfunction. […] 4%6% of all CHD in the United States
  • #38 Coarctation of the Aorta | Pediatric Echocardiography
    https://pedecho.org/library/fetal/fetal-coa
    Coarctation of the aorta (CoA) is a narrowing of the aortic arch. It most commonly occurs in the region of the aortic isthmus (between the left subclavian artery and ductus arteriosus). Patients with a severe coarctation of the aorta are dependent on a patent ductus arteriosus (PDA) to preserve systemic blood flow and perfusion. CoA has a variable spectrum of severity, ranging from unequivocal ductal-dependent systemic blood flow, to patients with borderline small arch dimensions who also have a PDA. […] The sensitivity of fetal diagnosis of CoA varies from 50% to 72%, and delayed diagnosis of CoA is still common. Infants who elude prenatal or early neonatal diagnosis may present in cardiogenic shock that may be associated with profound ventricular dysfunction. […] 4%6% of all CHD in the United States
  • #39 Prevalence of aortic coarctation in Bogotá-Colombia from 2001 to 2018. The diagnostic needs to improve
    https://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1405-99402022000200196&lng=en&nrm=iso&tlng=en
    The best way to screen patients for congenital heart disease is pulse oximetry 24-48 h after birth. […] It is important to mention that using pulse oximetry as a screening method has a sensitivity of 80% and that false negative oximeter results are not rare. […] The current study found a higher prevalence for aortic coarctation in Bogot in comparison with the study conducted by Tassinari et al. that worked with the same surveillance program: Surveillance and Follow-up Program for Children with Congenital Anomalies of the Health Secretary of Bogot. […] However, the prevalence found in Bogot is still lower compared to prevalence worldwide and in other continents. Furthermore, the prevalence found in Latinamerica was significantly lower compared to prevalence in Asia, Europe, and The United States. […] As a result of the information previously mentioned, we emphasize the importance of standardizing screening techniques in a local scale, as well as in a continental scale, so that every hospital can apply them, and no newborn is discharged without a diagnosis and a proper notification.
  • #40 Prevalence of aortic coarctation in Bogotá-Colombia from 2001 to 2018. The diagnostic needs to improve
    https://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1405-99402022000200196&lng=en&nrm=iso&tlng=en
    The best way to screen patients for congenital heart disease is pulse oximetry 24-48 h after birth. […] It is important to mention that using pulse oximetry as a screening method has a sensitivity of 80% and that false negative oximeter results are not rare. […] The current study found a higher prevalence for aortic coarctation in Bogot in comparison with the study conducted by Tassinari et al. that worked with the same surveillance program: Surveillance and Follow-up Program for Children with Congenital Anomalies of the Health Secretary of Bogot. […] However, the prevalence found in Bogot is still lower compared to prevalence worldwide and in other continents. Furthermore, the prevalence found in Latinamerica was significantly lower compared to prevalence in Asia, Europe, and The United States. […] As a result of the information previously mentioned, we emphasize the importance of standardizing screening techniques in a local scale, as well as in a continental scale, so that every hospital can apply them, and no newborn is discharged without a diagnosis and a proper notification.
  • #41 Coarctation of the aorta: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/000191.htm
    Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart defects). This abnormality accounts for about 5% of all congenital heart defects. It is most often diagnosed in children or adults under age 40. […] Coarctation is often discovered during a newborn’s first exam or a well-baby exam. Taking the pulse in an infant is an important part of the exam, because there may not be any other symptoms until the child is older. […] Without treatment, most people die before age 40. For this reason, doctors most often recommend that the person has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy. […] Lifelong follow-up with a cardiologist is recommended.
  • #42 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. […] A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings. […] The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis.
  • #43 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patient’s lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist. […] CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings.
  • #44 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. […] A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings. […] The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis.
  • #45 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patient’s lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist. […] CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings.
  • #46 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Older children with repaired coarctation of the aorta (CoA) with mild or no residual lesions may be followed less frequently (up to every 2 years) with echocardiography. […] Cardiac computed tomography and cardiac magnetic resonance imaging are useful adjuncts to echocardiography in the serial evaluation of older children and adults with repaired CoA. […] After CoA repair, patients should be monitored for systemic and masked hypertension. […] However, evidence-based guidelines for the care of these patients are lacking and significant practice variation exists. […] The goal of the congenital heart disease (CHD) Clinical Practice Algorithm for Repaired CoA is to provide an age-based decision-support tool that can be used in the follow-up and surveillance of patients with isolated repaired CoA throughout their lifespans.
  • #47 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Each pathway provides a suggested guide to the frequency of clinic visits and type of testing, with younger age and greater-than-mild residual lesions meriting closer follow-up. […] Patients with repaired CoA can continue to have or develop new hypertension even after early repair and with optimal surgical results. […] The Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section incorporated assessment and counseling for CV risk factors into the clinical algorithm based on current evidence for risk of vascular dysfunction in CoA. […] By using this tool, a standard approach for frequency of follow-up and testing, including echocardiography and cross-sectional imaging, for patients with isolated repaired CoA from birth to adulthood may be achieved.
  • #48 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Older children with repaired coarctation of the aorta (CoA) with mild or no residual lesions may be followed less frequently (up to every 2 years) with echocardiography. […] Cardiac computed tomography and cardiac magnetic resonance imaging are useful adjuncts to echocardiography in the serial evaluation of older children and adults with repaired CoA. […] After CoA repair, patients should be monitored for systemic and masked hypertension. […] However, evidence-based guidelines for the care of these patients are lacking and significant practice variation exists. […] The goal of the congenital heart disease (CHD) Clinical Practice Algorithm for Repaired CoA is to provide an age-based decision-support tool that can be used in the follow-up and surveillance of patients with isolated repaired CoA throughout their lifespans.
  • #49 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Older children with repaired coarctation of the aorta (CoA) with mild or no residual lesions may be followed less frequently (up to every 2 years) with echocardiography. […] Cardiac computed tomography and cardiac magnetic resonance imaging are useful adjuncts to echocardiography in the serial evaluation of older children and adults with repaired CoA. […] After CoA repair, patients should be monitored for systemic and masked hypertension. […] However, evidence-based guidelines for the care of these patients are lacking and significant practice variation exists. […] The goal of the congenital heart disease (CHD) Clinical Practice Algorithm for Repaired CoA is to provide an age-based decision-support tool that can be used in the follow-up and surveillance of patients with isolated repaired CoA throughout their lifespans.
  • #50 Aortic coarctation in a 49-year-old female | British Columbia Medical Journal
    https://bcmj.org/articles/aortic-coarctation-49-year-old-female
    Undiagnosed adults are commonly hypertensive but otherwise asymptomatic, unless significant hypertension exists, in which case, headache, epistaxis, heart failure, aortic dissection, or lower extremity claudication can be present. […] Current guidelines recommend that every patient with AC, repaired or not, should have at least one cardiovascular MRI or CT scan to determine the location and severity of coarctation as well as to evaluate collateral flow. […] A recent Cochrane review performed by Padua and colleagues evaluated several studies comparing endovascular stenting and surgical repair in the treatment of aortic coarctation. They concluded that there is insufficient evidence to determine the best treatment for coarctation of the thoracic aorta and emphasized the need for a randomized controlled clinical trial with high-quality samples and long-term follow-up. […] Although uncommon in adults, aortic coarctation can have significant mortality and should be included in the differential diagnosis for difficult-to-control hypertension.
  • #51 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Older children with repaired coarctation of the aorta (CoA) with mild or no residual lesions may be followed less frequently (up to every 2 years) with echocardiography. […] Cardiac computed tomography and cardiac magnetic resonance imaging are useful adjuncts to echocardiography in the serial evaluation of older children and adults with repaired CoA. […] After CoA repair, patients should be monitored for systemic and masked hypertension. […] However, evidence-based guidelines for the care of these patients are lacking and significant practice variation exists. […] The goal of the congenital heart disease (CHD) Clinical Practice Algorithm for Repaired CoA is to provide an age-based decision-support tool that can be used in the follow-up and surveillance of patients with isolated repaired CoA throughout their lifespans.
  • #52 Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2023/02/16/17/09/Clinical-Practice-Algorithm-For-the-Follow-Up-of-Repaired-Coarctation-of-the-Aorta
    Each pathway provides a suggested guide to the frequency of clinic visits and type of testing, with younger age and greater-than-mild residual lesions meriting closer follow-up. […] Patients with repaired CoA can continue to have or develop new hypertension even after early repair and with optimal surgical results. […] The Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section incorporated assessment and counseling for CV risk factors into the clinical algorithm based on current evidence for risk of vascular dysfunction in CoA. […] By using this tool, a standard approach for frequency of follow-up and testing, including echocardiography and cross-sectional imaging, for patients with isolated repaired CoA from birth to adulthood may be achieved.
  • #53 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease. […] Hypertension remains a key risk factor for adverse cardiovascular outcomes.
  • #54 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis. […] Studies analysing the performance of fetal echocardiography in the prenatal diagnosis of CoA have found that multiparametric models have a superior diagnostic performance to any one quantitative echocardiographic measure. […] CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. Histologically, this is characterised by myocyte hypertrophy, disarray and fibrosis. Ensuing alteration in myocardial mechanical properties manifests in the form of diastolic and systolic dysfunction, with LV failure being a significant cause of mortality. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease.
  • #55 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease. […] Hypertension remains a key risk factor for adverse cardiovascular outcomes.
  • #56 Coarctation of the Aorta – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430913/
    Coarctation of the aorta is a relatively common congenital heart defect, accounting for approximately 5% to 7% of all congenital heart diseases and occurring more frequently in males, with a male-to-female ratio of about 2:1. […] The epidemiology of aortic coarctation also reveals a significant genetic component. Turner syndrome is strongly associated with coarctation of the aorta, leading to an increased risk of left-sided obstructive heart lesions; karyotype screening is recommended for females diagnosed with aortic coarctation. Additionally, first-degree relatives of individuals with obstructive left-sided heart lesions have a 10-fold increased risk of developing aortic coarctation and other congenital heart defects. […] Despite advancements in early detection and treatment, coarctation of the aorta remains a significant contributor to cardiovascular morbidity and mortality, particularly when associated with other congenital anomalies.
  • #57 Case series: surgery for complications following aortic coarctation repair – Olayiwola – Journal of Visualized Surgery
    https://jovs.amegroups.org/article/view/119140/html
    Coarctation of the aorta (CoA) accounts for 57% of congenital heart defects with an incidence of 0.3/1,000 births. […] Long-term complications include re-stenosis and aneurysm formation, with a life-time risk between 138% depending on the primary type of repair. […] Lifelong surveillance with adequate imaging intervals may allow early identification of complications and planning for elective surgery. […] Referral to a specialised aortic centre for surveillance and expedited treatment is paramount for success. […] We suggest a more frequent follow-up protocol with agreed imaging interval for these patients. […] A more frequent imaging interval than the currently suggested by the guidelines is advised for those repaired in the adult life to be able to identify these cases before devastating consequences. […] Given the high rate and life-threatening nature of late complications, the need for life-long follow-up cannot be over-emphasized; however, this is not always available or adequate.
  • #58 SciELO Brazil – Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital
    https://www.scielo.br/j/ijcs/a/ZTDr6Rj4z7xPDzqrFW8fFmw/?lang=en
    Coarctation of the aorta is a congenital segmental narrowing of the aortic arch with severe hemodynamic repercussions and increased cardiovascular mortality. […] However, this goal has been challenged by high rates of underdiagnosis, which delay surgical correction, and by recoarctation in up to one third of operated patients. […] Recoarctation occurs in one-third of patients and draws attention for the need of lifelong surveillance by echocardiography. […] Late correction frequently occurs as a result of an underdiagnosis rate of over 62%, placing CoA as the most frequently misdiagnosed critical congenital heart disease. […] This complication demands a life-long surveillance by regular echocardiography in repaired patients. […] Our study found a high prevalence of LV hypertrophy at baseline, which markedly decreased after surgical correction.
  • #59 SciELO Brazil – Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital
    https://www.scielo.br/j/ijcs/a/ZTDr6Rj4z7xPDzqrFW8fFmw/?lang=en
    Importantly, these features are not prevented by surgical correction, reinforcing that coarctation is a generalized vasculopathy far beyond the narrowing of the aortic arch. […] In our study, recoarctation had a negative impact on the benefit of surgery in one-third of patients, which reinforces the need for lifelong surveillance by echocardiography.
  • #60 Coarctation of the aorta: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/000191.htm
    Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart defects). This abnormality accounts for about 5% of all congenital heart defects. It is most often diagnosed in children or adults under age 40. […] Coarctation is often discovered during a newborn’s first exam or a well-baby exam. Taking the pulse in an infant is an important part of the exam, because there may not be any other symptoms until the child is older. […] Without treatment, most people die before age 40. For this reason, doctors most often recommend that the person has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy. […] Lifelong follow-up with a cardiologist is recommended.
  • #61 Aortic coarctation in a 49-year-old female | British Columbia Medical Journal
    https://bcmj.org/articles/aortic-coarctation-49-year-old-female
    Aortic coarctation is characterized by localized narrowing of the thoracic aorta. While it is frequently diagnosed in infancy and childhood, first-time presentations in adults are rare. Unless significant hypertension exists, adult patients are usually asymptomatic. When aortic coarctation is not detected and repaired, survival averages 35 years, with mortality increasing to 75% by age 46. […] Although rarely diagnosed in adults, narrowing of the thoracic artery should be considered when patients present with difficult-to-control hypertension. […] Most cases are congenital and occur in about 4 of 10 000 live births. Diagnosis is most often made during infancy and childhood, and only rarely in adulthood. […] When aortic coarctation is not detected and repaired, survival averages 35 years, with mortality increasing to 75% by age 46. In addition to systemic hypertension, common complications of untreated AC include expedited coronary artery disease, stroke, aortic dissection, and heart failure.
  • #62 The Adult with Coarctation of the Aorta | IntechOpen
    https://www.intechopen.com/chapters/64223
    Epidemiological studies have found that for left ventricular outflow tract lesions, there is a higher chance of concordant diagnosis in multiple family members. […] All patients with coarctation (repaired or not) should be monitored with lifelong congenital cardiology follow-up and imaging because long-term survival is reduced compared with normative populations and there is potential need for reintervention. […] The unoperated mean survival rate of adults with coarctation of the aorta is 35 years of age, with a mortality rate of 75% by 46 years of age. […] In general, the patients with CoA who are repaired at a later age are more likely to remain hypertensive. […] The 2015 scientific statement of the AHA/ACC provides competitive athletic participation guidelines for patients with congenital heart disease (CHD), including coarctation.
  • #63 The Polls Are In: Head Imaging in Patients with Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2020/11/23/14/54/The-Polls-Are-In-Head-Imaging-in-Patients-with-Coarctation-of-the-Aorta
    Intracranial aneurysms (ICAs) occur in patients with Coarctation of the Aorta (CoA), putting them at risk for stroke. […] Recommendations to screen for the presence of ICAs are variable. […] There may be some modifiable risk factors, such as long-standing hypertension, that may increase the risk for the development of ICAs in patients with CoA. […] The 2018 ACC/AHA guidelines for the care of the adult with congenital heart disease (CHD) suggest that screening for ICAs by magnetic resonance angiography (MRA) or computed tomography angiography (CTA) may be reasonable in adults with CoA (COR IIb, LOE B-NR). […] In contrast, routine imaging to assess for ICAs in asymptomatic patients with CoA was not recommended in the 2020 ESC Guidelines. […] In the pediatric age group, screening practices are likewise variable and inconsistent.
  • #64 The Polls Are In: Head Imaging in Patients with Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2020/11/23/14/54/The-Polls-Are-In-Head-Imaging-in-Patients-with-Coarctation-of-the-Aorta
    This poll evaluated the customary approach to imaging surveillance for ICAs in patients with CoA. […] ICAs have a higher incidence in older patients with CoA. […] Compared to the general population, these adult patients suffer from both ischemic and hemorrhagic stroke at significantly younger ages. […] On the other hand, children with CoA who are treated early in life appear to not have a higher incidence of ICAs. […] This may question the previously held belief of a developmental relationship between CoA and ICAs, as well as the value of screening for cerebral vascular anomalies in infants and young children with isolated disease. […] This may also suggest that there are perhaps some modifiable risk factors, such as long-standing hypertension, that may be responsible for the development of ICAs in patients with CoA who are diagnosed and treated in later adulthood.
  • #65 The Polls Are In: Head Imaging in Patients with Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2020/11/23/14/54/The-Polls-Are-In-Head-Imaging-in-Patients-with-Coarctation-of-the-Aorta
    Intracranial aneurysms (ICAs) occur in patients with Coarctation of the Aorta (CoA), putting them at risk for stroke. […] Recommendations to screen for the presence of ICAs are variable. […] There may be some modifiable risk factors, such as long-standing hypertension, that may increase the risk for the development of ICAs in patients with CoA. […] The 2018 ACC/AHA guidelines for the care of the adult with congenital heart disease (CHD) suggest that screening for ICAs by magnetic resonance angiography (MRA) or computed tomography angiography (CTA) may be reasonable in adults with CoA (COR IIb, LOE B-NR). […] In contrast, routine imaging to assess for ICAs in asymptomatic patients with CoA was not recommended in the 2020 ESC Guidelines. […] In the pediatric age group, screening practices are likewise variable and inconsistent.
  • #66 The Polls Are In: Head Imaging in Patients with Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2020/11/23/14/54/The-Polls-Are-In-Head-Imaging-in-Patients-with-Coarctation-of-the-Aorta
    This poll evaluated the customary approach to imaging surveillance for ICAs in patients with CoA. […] ICAs have a higher incidence in older patients with CoA. […] Compared to the general population, these adult patients suffer from both ischemic and hemorrhagic stroke at significantly younger ages. […] On the other hand, children with CoA who are treated early in life appear to not have a higher incidence of ICAs. […] This may question the previously held belief of a developmental relationship between CoA and ICAs, as well as the value of screening for cerebral vascular anomalies in infants and young children with isolated disease. […] This may also suggest that there are perhaps some modifiable risk factors, such as long-standing hypertension, that may be responsible for the development of ICAs in patients with CoA who are diagnosed and treated in later adulthood.
  • #67 The Polls Are In: Head Imaging in Patients with Coarctation of the Aorta
    https://www.acc.org/Latest-in-Cardiology/Articles/2020/11/23/14/54/The-Polls-Are-In-Head-Imaging-in-Patients-with-Coarctation-of-the-Aorta
    This poll evaluated the customary approach to imaging surveillance for ICAs in patients with CoA. […] ICAs have a higher incidence in older patients with CoA. […] Compared to the general population, these adult patients suffer from both ischemic and hemorrhagic stroke at significantly younger ages. […] On the other hand, children with CoA who are treated early in life appear to not have a higher incidence of ICAs. […] This may question the previously held belief of a developmental relationship between CoA and ICAs, as well as the value of screening for cerebral vascular anomalies in infants and young children with isolated disease. […] This may also suggest that there are perhaps some modifiable risk factors, such as long-standing hypertension, that may be responsible for the development of ICAs in patients with CoA who are diagnosed and treated in later adulthood.
  • #68 Coarctation of the Aorta: Diagnosis and Management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
    Coarctation of the aorta (CoA) accounts for approximately 58% of all congenital heart defects. […] Developments in diagnosis and management have improved outcomes in this patient population. […] Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime. […] Coarctation of the aorta (CoA) is one of the most common congenital heart defects (CHD), accounting for approximately 58% of CHD, with an incidence of 4 per 10,000 live births. […] There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patients lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist.
  • #69 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189
    There is increasing evidence that CoA represents a generalised arteriopathy, leading to an enhanced cardiovascular risk across the patient’s lifetime and the need for lifelong follow-up with an adult congenital heart disease specialist. […] CoA represents a lifelong condition. Even after an apparently successful repair in infancy, there is a high rate of reoperation, reported to be around 50% by the fifth decade. A decreased life expectancy has been shown in long-term follow-up data of repaired CoA patients when compared to the general population, with a high rate of adverse cardiovascular events. […] Prenatal diagnosis of CoA is notoriously challenging, with a high rate of false positives on pulse oximetry and fetal echocardiography-based screening, leading to considerable anxiety for families and allocation of limited paediatric and neonatal resources to the admission and surveillance of neonates with equivocal findings.
  • #70 Coarctation of the Aorta – ACHA
    https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/coarctation-of-the-aorta/
    Most women with repaired coarctation can have successful pregnancies. […] About 15-20% of women with coarctation have high blood pressure during pregnancy. […] We used to think that coarctation was a simple defect. But we now realize that it is much more than a narrowed aorta. […] Coarctation of the aorta is a lifelong disorder. But the good news is that most people with coarctation continue to do well.
  • #71 Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus | The Journal of Physiological Sciences | Full Text
    https://jps.biomedcentral.com/articles/10.1007/s12576-016-0512-x
    Coarctation of the aorta is the fifth or sixth most common congenital heart defect, accounting for 58% of neonates with congenital heart diseases. The incidence of CoA has been reported to one per 2500 births and the ratio of males to females with the condition to be between 1.27:1 and 1.74:1. In these studies, CoA was considered to be hemodynamically dominant in complex congenital heart defects with a left-to-right shunt. […] Early diagnosis and appropriate management of coarctation of the aorta (CoA) have resulted in a low mortality rate associated with the repair of CoA. It has been suggested that patients with CoA are at increased risk of myocardial infarction because systemic hypertension and premature atherosclerosis tend to develop early in life even after successful surgical treatment. The increased cardiovascular risk in this patient group is the result of both frequent relapses of hypertension and proatherogenic abnormalities that are present even in the absence of arterial hypertension. […] Accumulating evidence of vasoreactivity and endothelial function in patients with CoA suggests that CoA is not a simple mechanical obstruction that can be cured surgically.
  • #72 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189?type=check_update&version=2
    The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis. […] Studies analysing the performance of fetal echocardiography in the prenatal diagnosis of CoA have found that multiparametric models have a superior diagnostic performance to any one quantitative echocardiographic measure. […] CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. Histologically, this is characterised by myocyte hypertrophy, disarray and fibrosis. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease. […] CoA remains a diagnostic and therapeutic challenge due to the difficulties of prenatal diagnosis, wide-ranging anatomical variation, long-term adverse cardiovascular sequelae of abnormal haemodynamics and impaired systemic vascular function, which are still not fully understood. Even where there is timely repair, these patients remain at enhanced cardiovascular risk throughout their lifetime.
  • #73 Coarctation of the Aorta: Diagnosis and Management
    https://www.mdpi.com/2075-4418/13/13/2189?type=check_update&version=2
    The majority of true CoA cases are missed prenatally, and therefore clinicians rightly have a low threshold for enhancing surveillance of those with a suspicion of this diagnosis. […] Studies analysing the performance of fetal echocardiography in the prenatal diagnosis of CoA have found that multiparametric models have a superior diagnostic performance to any one quantitative echocardiographic measure. […] CoA leads to pressure overload of the left ventricle and adverse left ventricular remodelling. Histologically, this is characterised by myocyte hypertrophy, disarray and fibrosis. […] Even after complete relief of obstruction, CoA is associated with diastolic dysfunction independent of the presence of coronary artery disease. […] CoA remains a diagnostic and therapeutic challenge due to the difficulties of prenatal diagnosis, wide-ranging anatomical variation, long-term adverse cardiovascular sequelae of abnormal haemodynamics and impaired systemic vascular function, which are still not fully understood. Even where there is timely repair, these patients remain at enhanced cardiovascular risk throughout their lifetime.
  • #74 SciELO Brazil – Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital
    https://www.scielo.br/j/ijcs/a/ZTDr6Rj4z7xPDzqrFW8fFmw/?lang=en
    Importantly, these features are not prevented by surgical correction, reinforcing that coarctation is a generalized vasculopathy far beyond the narrowing of the aortic arch. […] In our study, recoarctation had a negative impact on the benefit of surgery in one-third of patients, which reinforces the need for lifelong surveillance by echocardiography.