Zwężenie cieśni aorty
Diagnostyka i diagnoza
Zwężenie cieśni aorty (Coarctation of the aorta) to wrodzona wada serca, charakteryzująca się zwężeniem aorty najczęściej tuż za odejściem lewej tętnicy podobojczykowej, stanowiąca 5-8% wszystkich wrodzonych wad serca. Diagnostyka opiera się na badaniu przedmiotowym (różnica ciśnienia tętniczego między kończynami górnymi i dolnymi, słabe tętno w kończynach dolnych, szmer skurczowy), echokardiografii z oceną gradientu ciśnień (szczytowy >20 mmHg wskazuje na istotne zwężenie), a także na obrazowaniu metodami MRI, CT oraz cewnikowaniu serca, które jest złotym standardem oceny hemodynamicznej. W diagnostyce prenatalnej czułość wynosi 50-72%, a istotnym parametrem jest indeks DA ≥1,28, sugerujący konieczność interwencji chirurgicznej z czułością 85% i swoistością 94%. Wczesne rozpoznanie jest kluczowe, gdyż nieleczone zwężenie prowadzi do poważnych powikłań, takich jak nadciśnienie, przerost lewej komory, rozwarstwienie aorty czy krwotok wewnątrzczaszkowy.
- Diagnostyka zwężenia cieśni aorty
- Badania diagnostyczne
- Echokardiografia
- Elektrokardiogram
- RTG klatki piersiowej
- Rezonans magnetyczny serca
- Tomografia komputerowa
- Cewnikowanie serca
- Diagnostyka różnicowa
- Szczególne aspekty diagnostyczne
- Indeksy diagnostyczne
- Znaczenie wczesnej diagnozy
- Podsumowanie diagnostyki zwężenia cieśni aorty
Diagnostyka zwężenia cieśni aorty
Zwężenie cieśni aorty (Coarctation of the aorta) to wrodzona wada serca polegająca na zwężeniu aorty, najczęściej w miejscu tuż za odejściem lewej tętnicy podobojczykowej. Stanowi około 5-8% wszystkich wrodzonych wad serca i wymaga dokładnej diagnostyki w celu określenia stopnia zwężenia i zaplanowania odpowiedniego leczenia12. Diagnoza zwężenia cieśni aorty może zależeć od stopnia nasilenia wady. Ciężkie zwężenie cieśni aorty jest zazwyczaj diagnozowane wkrótce po urodzeniu, natomiast łagodne formy mogą pozostać niewykryte aż do późniejszego okresu życia3.
Diagnostyka prenatalna
Zwężenie cieśni aorty może być wykryte jeszcze przed urodzeniem podczas rutynowych badań ultrasonograficznych w ciąży3. Chociaż diagnostyka prenatalna stanowi wyzwanie, specjalistyczne badanie echokardiograficzne płodu (fetalne echo) może ujawnić obecność zwężenia lub ryzyka jego wystąpienia45. Czułość diagnozy prenatalnej waha się od 50% do 72%, a opóźniona diagnoza zwężenia cieśni aorty jest nadal powszechna6.
Prenatalna diagnoza zwężenia cieśni aorty jest często oparta na wtórnych objawach, takich jak dysproporcja między wielkością komór serca i wielkich tętnic7. Większy stosunek (1,4) średnicy przewodu tętniczego do średnicy cieśni aorty może sugerować obecność zwężenia7. Trójwymiarowa echokardiografia może dostarczyć dodatkowych informacji przestrzennych, pomagając lekarzom postawić dokładną diagnozę7.
Warto zaznaczyć, że w niektórych przypadkach podczas życia płodowego zwężenie może być mniej widoczne, gdy przewód tętniczy (ductus arteriosus) pozostaje otwarty. Po jego zamknięciu po urodzeniu, zwężenie aorty staje się bardziej widoczne8.
Diagnostyka po urodzeniu
Badanie przedmiotowe stanowi kluczowy element wstępnej diagnostyki zwężenia cieśni aorty. Lekarz podczas badania fizycznego może stwierdzić następujące objawy93:
- Różnica ciśnienia tętniczego między górnymi i dolnymi kończynami (wyższe ciśnienie w ramionach i górnej części ciała, a niższe w nogach i dolnej części ciała)
- Słabe lub opóźnione tętno w kończynach dolnych
- Charakterystyczny szorstki szmer serca (szmer skurczowy najlepiej słyszalny na plecach)
Diagnoza kliniczna zwężenia cieśni aorty opiera się na konstelacji wspierających danych klinicznych, w tym obecności anatomicznego zwężenia wykazanego w badaniach obrazowych, gradientach opartych na badaniu dopplerowskim w echokardiografii, danych z cewnikowania serca oraz objawach w badaniu przedmiotowym, takich jak zróżnicowane ciśnienie krwi w kończynach górnych i dolnych10.
Badania diagnostyczne
W celu potwierdzenia diagnozy zwężenia cieśni aorty wykonuje się szereg badań diagnostycznych:
Echokardiografia
Echokardiografia (echo) jest badaniem pierwszego wyboru w diagnostyce zwężenia cieśni aorty. Wykorzystuje fale dźwiękowe do utworzenia obrazów bijącego serca i pokazuje przepływ krwi przez serce. Badanie to może często określić, która część i w jakim stopniu aorta jest zwężona. Echokardiogram pomaga również lekarzom w planowaniu leczenia zwężenia cieśni aorty311. Większość przypadków zwężenia cieśni aorty jest diagnozowana właśnie za pomocą echokardiografii12.
Podczas badania echokardiograficznego ocenia się anatomię aorty i poszukuje innych współistniejących anomalii serca, które mogą być obecne, a także uzyskuje się ważne informacje o funkcji serca5. Badanie dopplerowskie podczas echokardiografii pozwala na ocenę przepływu krwi przez zwężenie oraz oszacowanie gradientu ciśnień za pomocą zmodyfikowanego równania Bernoulliego13.
Elektrokardiogram
Elektrokardiogram (EKG) jest szybkim i bezbolesnym badaniem sprawdzającym pracę serca. Czujniki zwane elektrodami przykleja się do klatki piersiowej, a czasami do ramion lub nóg. Przewody łączą czujniki z urządzeniem, które wyświetla lub drukuje wyniki. Jeśli aorta jest bardzo zwężona, EKG może wykazać przerost ścian dolnych komór serca3. Jest to częsty objaw wtórny do zwiększonego obciążenia lewej komory, która musi pokonać opór zwężonej aorty14.
RTG klatki piersiowej
Zdjęcie rentgenowskie klatki piersiowej pokazuje stan serca i płuc. RTG klatki piersiowej może ukazać zwężenie aorty w miejscu koarktacji, a także powiększoną część aorty lub serce315. Charakterystycznym objawem na zdjęciu RTG może być tzw. „znak 3” w górnym lewym cieniu śródpiersia16. U pacjentów z długotrwałym zwężeniem cieśni aorty można zaobserwować wcięcia żeber spowodowane rozwojem krążenia obocznego17.
Rezonans magnetyczny serca
Rezonans magnetyczny serca (MRI) wykorzystuje pola magnetyczne i fale radiowe do tworzenia szczegółowych obrazów serca i naczyń krwionośnych. Może pokazać, która część i w jakim stopniu aorta jest zwężona. Lekarz może również wykorzystać wyniki MRI do kierowania leczeniem3. MRI serca jest obecnie uważany za złoty standard w ocenie zwężenia cieśni aorty i może być stosowany zarówno w diagnostyce, jak i monitorowaniu patologii18.
Badanie MRI/MRA (angiografia rezonansu magnetycznego) dostarcza szczegółowych informacji o anatomii łuku aorty i kolaterali, co jest szczególnie przydatne w przypadku starszych pacjentów, u których obrazowanie echokardiograficzne może być utrudnione1013.
Tomografia komputerowa
Tomografia komputerowa (CT) wykorzystuje serię zdjęć rentgenowskich do tworzenia szczegółowych przekrojowych obrazów ciała3. CT angiografia (CT z kontrastem) pozwala na dokładną ocenę struktur serca, lokalizacji i stopnia zwężenia cieśni aorty oraz wykrycie ewentualnych innych nieprawidłowości naczyniowych15.
Cewnikowanie serca
Cewnikowanie serca z angiografią wykorzystuje promienie rentgenowskie do badania naczyń krwionośnych serca, zwanych tętnicami wieńcowymi. Jest zwykle wykonywane w celu sprawdzenia, czy naczynie krwionośne jest zwężone lub zablokowane. Podczas cewnikowania serca, cienka, elastyczna rurka zwana cewnikiem jest umieszczana w naczyniu krwionośnym, zwykle w pachwinie lub nadgarstku, i kierowana do serca. Barwnik przepływa przez cewnik do tętnic w sercu. Barwnik ułatwia widoczność tętnic na obrazach rentgenowskich i filmach. Cewnikowanie serca może pomóc określić, jak duża część aorty jest zwężona3.
Cewnikowanie serca jest złotym standardem oceny hemodynamicznej zwężenia cieśni aorty i pozwala na wysokiej rozdzielczości wizualizację anatomii łuku aorty oraz ocenę krążenia obocznego. Gradient szczyt-szczyt >20 mmHg podczas cewnikowania jest uważany za dowód istotnego zwężenia cieśni aorty w międzynarodowych wytycznych219. Najsilniejszym zaleceniem do interwencji jest obecność nadciśnienia u pacjentów ze zwiększonym nieinwazyjnym gradientem między górnymi i dolnymi kończynami, potwierdzonym pomiarem inwazyjnym (szczyt-szczyt >20 mmHg)2.
Diagnostyka różnicowa
Zwężenie cieśni aorty może stanowić wyzwanie diagnostyczne ze względu na zmienne objawy kliniczne, które często nakładają się na inne schorzenia, szczególnie te powodujące nadciśnienie wtórne11. Biorąc pod uwagę znaczące nakładanie się objawów, szczególnie nadciśnienia, zwężenie cieśni aorty powinno być priorytetem w diagnostyce różnicowej, zwłaszcza u nastolatków z wtórnym nadciśnieniem11.
W diagnostyce różnicowej należy uwzględnić20:
- Pseudozwężenie cieśni aorty: wydłużenie, zwężenie lub zagięcie bez gradientu ciśnienia lub tworzenia krążenia obocznego, bez wcięć żeber
- Inne przyczyny nadciśnienia wtórnego
- Inne wrodzone wady serca powodujące zaburzenia przepływu krwi
Szczególne aspekty diagnostyczne
Diagnostyka u niemowląt
U około 25% przypadków izolowanego zwężenia cieśni (bez innych powiązanych wad serca), zwężenie jest na tyle poważne, że powoduje objawy w pierwszych dniach życia. Gdy przewód tętniczy się zamyka, lewa komora musi nagle pompować krew pod znacznie większym oporem przepływu w miejscu zwężenia. Może to prowadzić do niewydolności serca i wstrząsu. Ponieważ te noworodki czują się dobrze do momentu zamknięcia przewodu tętniczego, objawy pojawiają się szybko i są często ciężkie5.
U noworodków z podejrzeniem zwężenia cieśni aorty ocena diagnostyczna powinna obejmować8:
- Częste badania przedmiotowe (monitorowanie tętna i ciśnienia krwi w dolnej części ciała)
- Echokardiografię
- Pulse oksymetrię (monitorowanie zawartości tlenu we krwi za pomocą sondy świetlnej umieszczonej na dłoni lub stopie dziecka)
Diagnostyka u starszych dzieci i dorosłych
U pacjentów, którzy nie rozwijają niewydolności serca jako noworodki, zwężenie cieśni aorty może nie zostać wykryte, dopóki dziecko nie będzie miało kilku lat. U tych starszych pacjentów zwężenie cieśni aorty jest często najpierw podejrzewane z powodu szmeru serca lub nadciśnienia5.
U starszych pacjentów zwężenie cieśni aorty może być trudne do zdiagnozowania za pomocą echokardiografii powierzchniowej. Dla tych pacjentów, obrazowanie metodą rezonansu magnetycznego (MRI), przezprzełykowa echokardiografia (TEE) lub cewnikowanie serca z angiografią mogą być konieczne do postawienia diagnozy13.
U dorosłych diagnoza zwężenia cieśni aorty opiera się na wywiadzie medycznym, badaniu przedmiotowym i badaniach obrazowych. Bardzo ważne są badanie przedmiotowe i badania obrazowe u dorosłych z nadciśnieniem, kardiomegalią, niewydolnością serca i niewydolnością nerek22.
Rola obrazowania wielomodalnego
Obrazowanie wielomodalne odgrywa kluczową rolę w diagnostyce i monitorowaniu zwężenia cieśni aorty. Dane z echokardiografii, obrazowania serca metodą rezonansu magnetycznego (CMR) i tomografii komputerowej serca (CT) są integrowane dla poszczególnych pacjentów, aby wspomóc podejmowanie decyzji dotyczących interwencji i reinterwencji przez całe życie102.
Rola diagnostycznego cewnikowania serca jest coraz częściej zastępowana przez obrazowanie przekrojowe w monitorowaniu pacjentów po operacji naprawczej; jednak zachowuje ona znaczenie w przypadkach, gdy rozważana jest interwencja oparta na cewniku z powodu ponownego zwężenia, czy to angioplastyka balonowa, czy stentowanie19.
Indeksy diagnostyczne
W diagnostyce zwężenia cieśni aorty wykorzystuje się również specjalne parametry i indeksy. Jednym z nich jest indeks DA (Distal Aortic arch), który poprawia wskaźnik diagnostyczny płodowego zwężenia cieśni aorty. Wartość indeksu DA ≥1,28 wskazuje na przypadki zwężenia cieśni aorty wymagające interwencji chirurgicznej, z czułością 85% i swoistością 94%23.
Podczas cewnikowania serca gradient ciśnienia szczyt-szczyt wynoszący 20 mmHg jest uznawany za dowód istotnego zwężenia cieśni aorty w wytycznych międzynarodowych1924. Również w badaniu echokardiograficznym gradient szczytowy >20 mmHg sugeruje istotne zwężenie cieśni24.
Znaczenie wczesnej diagnozy
Wczesna diagnoza i interwencja są kluczowe, ponieważ nieleczone zwężenie cieśni aorty może prowadzić do poważnych powikłań, takich jak nadciśnienie, przerost lewej komory, rozwarstwienie aorty i krwotok wewnątrzczaszkowy, do trzeciej lub czwartej dekady życia11.
Szacuje się, że około 60-80% noworodków ze zwężeniem cieśni aorty nie jest diagnozowanych przed wypisem ze szpitala. Jednak jest to patologia, którą można łatwo rozpoznać w praktyce klinicznej poprzez dokładne badanie przedmiotowe, w tym pomiar ciśnienia tętniczego w kończynach górnych i dolnych18.
Wczesne rozpoznanie zwężenia cieśni aorty zmniejsza zachorowalność i śmiertelność. Wdrożenie odpowiedniej oceny może zapobiec wstrząsowi krążeniowemu u noworodków23.
Podsumowanie diagnostyki zwężenia cieśni aorty
Zwężenie cieśni aorty to wada wrodzona wymagająca dokładnej diagnostyki i dalszej obserwacji przez całe życie. Diagnoza może opierać się na badaniu przedmiotowym, badaniach obrazowych (echokardiografia, MRI, CT) oraz inwazyjnych procedurach (cewnikowanie serca). Szczególnie ważne jest wykrycie różnicy ciśnienia między górnymi i dolnymi kończynami, co jest charakterystycznym objawem tej wady2526.
Pacjenci ze zwężeniem cieśni aorty wymagają dożywotniego monitorowania przez specjalistę chorób wrodzonych serca, ponieważ mogą rozwijać ponowne zwężenie i powikłania w miejscu naprawy oraz pozostają w grupie zwiększonego ryzyka sercowo-naczyniowego przez całe życie1. Nawet po pozornie udanej naprawie w okresie niemowlęcym istnieje wysoki wskaźnik ponownych operacji, szacowany na około 50% do piątej dekady życia2.
Postępy w technikach obrazowania i chirurgicznych znacznie poprawiły rokowanie dla osób ze zwężeniem cieśni aorty, czyniąc ją punktem centralnym bieżących badań i praktyki klinicznej11.
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Materiały źródłowe
- #1 Coarctation of the Aorta: Diagnosis and Managementhttps://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. […] 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. […] The clinical diagnosis of CoA, therefore, relies on a constellation of supportive clinical data points including the presence of anatomical obstruction as demonstrated on cross-sectioning imaging.
- #2 Coarctation of the Aorta: Diagnosis and Managementhttps://www.mdpi.com/2075-4418/13/13/2189?type=check_update&version=2
Coarctation of the aorta (CoA) accounts for approximately 5â8% 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. […] 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. […] Multi-modality imaging plays a central role in the diagnosis and follow-up of CoA, with echocardiography, cardiac magnetic resonance (CMR) imaging and cardiac computed tomography (CT) data being integrated for individual patients to aid decision-making for intervention and reintervention throughout their lifetime.
- #2 Coarctation of the Aorta: Diagnosis and Managementhttps://www.mdpi.com/2075-4418/13/13/2189?type=check_update&version=2
Cardiac catheterisation is the gold standard for haemodynamic assessment of CoA and allows high-resolution visualisation of the anatomy of the aortic arch, as well as assessment of any collaterals. A peak-to-peak gradient of >20 mmHg on catheterisation is considered to be evidence of significant CoA in international guidelines. The strongest recommendation for intervention is for hypertensive patients with an increased non-invasive gradient between upper and lower limbs, confirmed with invasive measurement (peak-to-peak > 20 mmHg) with a preference for catheter treatment when technically feasible. […] 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.
- #3 Coarctation of the aorta | UM Health-Sparrowhttps://www.uofmhealthsparrow.org/departments-conditions/conditions/coarctation-aorta
Diagnosis of aortic coarctation may depend on how severe the heart condition is. Severe aortic coarctation is usually diagnosed soon after birth. The condition may be seen on ultrasound images taken during pregnancy. […] If the condition is mild, it may not be found until later in life. […] To diagnose aortic coarctation, a healthcare professional checks blood pressure in the arms and legs. Depending on which part of the aorta is affected, blood pressure may be high in the arms and low in the legs and ankles. The pulse may be weak or delayed in the legs. […] A whooshing sound called a heart murmur may be heard when listening to the heart. […] Tests are done to diagnose coarctation of the aorta. […] Echocardiogram. Sound waves are used to create images of the beating heart. An echocardiogram shows how blood flows through the heart. The test can often tell which part and how much of the aorta is narrowed. An echocardiogram also helps healthcare professionals plan treatment for coarctation of the aorta.
- #3 Coarctation of the aorta | UM Health-Sparrowhttps://www.uofmhealthsparrow.org/departments-conditions/conditions/coarctation-aorta
Electrocardiogram (ECG or EKG). This quick and painless test checks the heartbeat. Sensors called electrodes stick to the chest and sometimes to the arms or legs. Wires connect the sensors to a machine, which displays or prints results. If the aorta is very narrowed, an ECG may show of thickening of the walls of the lower heart chambers. […] Chest X-ray. A chest X-ray shows the condition of the heart and lungs. A chest X-ray might show a narrowing in the aorta at the site of the coarctation. […] Cardiac magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to create detailed images of the heart and blood vessels. It can show which part and how much of the aorta is narrowed. A healthcare professional also may use MRI results to guide treatment. […] Computerized tomography (CT) scan. A CT scan uses a series of X-rays to create detailed cross-sectional images of the body.
- #3 Coarctation of the aorta | UM Health-Sparrowhttps://www.uofmhealthsparrow.org/departments-conditions/conditions/coarctation-aorta
Coronary angiogram with cardiac catheterization. A coronary angiogram uses X-rays to look at the heart’s blood vessels, called the coronary arteries. It’s usually done to see if a blood vessel is narrowed or blocked. A coronary angiogram is part of a general group of heart tests and treatments called cardiac catheterization. […] During cardiac catheterization, a thin flexible tube called a catheter is placed in a blood vessel, usually in the groin or wrist, and guided to the heart. Dye flows through the catheter to arteries in the heart. The dye makes the arteries easier to see on X-ray images and video. Cardiac catheterization can help determine how much of the aorta is narrowed. […] CT angiogram. This test looks at the arteries that supply blood to the heart. It uses a powerful X-ray machine to make images of the heart and its blood vessels. A CT angiogram uses a dye and special X-rays to show how blood flows through the veins and arteries. The test can show the location and severity of coarctation of the aorta. It also can tell whether other blood vessels are affected. A CT angiogram also can be used to guide treatment.
- #4 Coarctation of the Aortahttps://med.uth.edu/heartandvascular/coarctation-of-the-aorta/
Diagnosis of Coarctation of the aorta is typically made through a combination of medical history review, physical examination, and diagnostic tests, including: […] Blood pressure measurements: Discrepancies in blood pressure between the arms and legs can be indicative of CoA. […] Echocardiography (echo): This ultrasound test provides detailed images of the heart and aorta and is the primary tool for diagnosing CoA. […] Magnetic resonance angiography (MRA) or computed tomography angiography (CTA): These imaging tests may be used to visualize the aortic narrowing more clearly. […] Chest X-ray: An X-ray can help assess the size and shape of the heart and identify CoA-associated abnormalities.
- #5 Coarctation of the Aorta | Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/c/coarctation
The diagnosis of coarctation is confirmed with echocardiography. This can look at the anatomy of the aorta. It can also check for other cardiac anomalies that may be present and give important information about the function of the heart. Sometimes other tests, such as a cardiac MRI or CT scan, may be used to look at the aorta and coarctation in more detail prior to intervention. […] Coarctation may also be diagnosed on fetal echocardiograms. It is one of the cardiac defects that may be found on screening ultrasounds. Early diagnosis allows for prompt intervention at the time of birth. However, while the ductus arteriosus is open in utero it can be unclear in some patients if there is a risk of a coarctation forming. Some infants may need to be observed closely after birth as the ductus arteriosus closes to detect if there is coarctation.
- #5 Coarctation of the Aorta | Diagnosis & Treatmenthttps://www.cincinnatichildrens.org/health/c/coarctation
Coarctation of the aorta is typically present from birth or shortly after birth. The age at which coarctation is found depends on the severity of the narrowing. […] In about 25% of cases of isolated coarctation (without other associated cardiac defects), the narrowing is severe enough to cause symptoms in the first days of life. When the ductus arteriosus closes, the left ventricle must suddenly pump against much higher resistance to blood flow at the area of the coarctation. This can lead to heart failure and shock. Because these newborns are well until the ductus arteriosus closes, symptoms appear quickly. They are often severe. […] In patients who do not develop heart failure as newborns, coarctation may not be found until the child is several years old. In these older patients, coarctation is often first thought of because of a heart murmur or high blood pressure.
- #6 Coarctation of the Aorta | Pediatric Echocardiographyhttps://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 can be present with an isolated narrowing of the aortic isthmus or with tubular hypoplasia of the aortic arch (transverse arch and isthmus). 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.
- #7 Prenatal diagnosis of coarctation of the aorta with a long and angled isthmus by two- and three-dimensional echocardiography: a case report | BMC Cardiovascular Disorders | Full Texthttps://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-021-01987-7
Fetal diagnosis of CoA is generally inferred from secondary findings of ventricular and great artery disproportion. […] A larger ratio (1.4) of ductus/aortic isthmus in diameter could also suggest the presence of CoA. […] Though we discuss above the needed echo parameters, capabilities, and some criteria to evaluate fetal CoA, it is really not easy to make an accurate prediction or diagnosis in utero. […] We present a fetal case of CoA diagnosed by 2DE and 3DE-STIC technology. The traditional 2DE has some limitations in showing the angled and hypoplastic aortic isthmus, while the 3D rendered image may provide additional spatial information to show great arteries in detail, help to find tiny vessels, and thus benefit the examiners to make an accurate diagnosis.
- #8 Coarctation of the Aorta | Texas Children’shttps://www.texaschildrens.org/content/conditions/coarctation-aorta-0
Coarctation of the aorta can be suspected during a routine prenatal ultrasound or after a fetal echocardiogram (focused ultrasound of the fetal heart performed by a fetal cardiologist). A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and cardiac expertise your baby will need at birth, optimizing outcomes. […] Coarctation of the aorta is among the most challenging diagnoses to make because in fetal life the aorta can appear less narrow while the ductus arteriosus (a normal vessel connecting the pulmonary artery to the aorta) is open. Once this vessel closes after birth, the narrowing of the aorta becomes more evident. Therefore, in some cases, a coarctation may not be diagnosed until after birth. […] The timing of diagnosis after birth will depend upon the severity of the narrowing and associated defects.
- #8 Coarctation of the Aorta | Texas Children’shttps://www.texaschildrens.org/content/conditions/coarctation-aorta-0
If there is prenatal suspicion, newborns will undergo observation and testing in the hospital after birth. This includes frequent physical exams (monitoring of pulses and blood pressures in lower body) and echocardiography. […] Testing to confirm the diagnosis typically includes an echocardiogram, a chest x-ray, an electrocardiogram and in some cases a CT scan, MRI or cardiac catheterization.
- #9 Coarctation of the Aorta (CoA)https://my.clevelandclinic.org/health/diseases/16876-aortic-coarctation
Coarctation of the aorta (CoA) is a heart defect some babies are born with. CoA means your child’s aorta, a large artery, is pinched or narrowed in one spot. This interferes with normal blood flow in your child’s body and may overtax their heart. Your child needs surgery or catheterization to repair the problem and prevent complications. […] Pediatric cardiologists usually diagnose coarctation of the aorta during infancy or early childhood. The timing depends on the severity of symptoms. Infants with moderate or severe symptoms are typically diagnosed soon after birth. Infants with mild or no symptoms may not be diagnosed until later in childhood when they begin to have high blood pressure. Aortic coarctation is rarely diagnosed in adulthood. […] Most babies and children receive a CoA diagnosis when a physical exam reveals certain red flags. Signs that a baby or child may have coarctation of the aorta include high blood pressure in their arms and upper body, but low blood pressure in their legs and lower body. Differences in pulse when measured at their neck versus their groin. A distinctive, harsh heart murmur that their provider hears when placing a stethoscope on their back.
- #10 Coarctation of the Aorta: Diagnosis and Managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
This, together with echocardiography-based Doppler gradients, cardiac catheter data, as well as physical examination findings such as discrepant upper and lower limb blood pressures, help to define the presence of significant CoA or recurrent CoA. […] CoA represents a lifelong condition. […] Multi-modality imaging plays a central role in the diagnosis and follow-up of CoA, with echocardiography, cardiac magnetic resonance (CMR) imaging and cardiac computed tomography (CT) data being integrated for individual patients to aid decision-making for intervention and reintervention throughout their lifetime. […] Cardiac catheterisation is the gold standard for haemodynamic assessment of CoA and allows high-resolution visualisation of the anatomy of the aortic arch, as well as assessment of any collaterals.
- #11 Coarctation of the Aorta – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430913/
Echocardiography is typically the first-line imaging modality used to diagnose coarctation of the aorta. […] While not definitive, certain radiographic features can strongly suggest the presence of coarctation of the aorta, guiding further diagnostic workup. […] Following a comprehensive clinical assessment in a patient with a suspected coarctation of the aorta, the evaluation to confirm the diagnosis and determine the severity of the condition may include: Blood pressure measurement, Electrocardiogram, Echocardiography, Chest radiography, Advanced imaging, Invasive testing, Additional studies. […] Coarctation of the aorta can present diagnostic challenges due to its variable clinical manifestations, which often overlap with other conditions, particularly those causing secondary hypertension.
- #11 Coarctation of the Aorta – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430913/
Given the significant overlap of symptoms, particularly hypertension, aortic coarctation should be considered a priority in the differential diagnosis, especially in adolescents presenting with secondary hypertension. […] When suspected, coarctation of the aorta is best evaluated with imaging studies such as echocardiography, which can identify the characteristic narrowing of the aorta and assess for associated cardiac anomalies.
- #11 Coarctation of the Aorta – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK430913/
Coarctation of the aorta is a congenital condition characterized by the narrowing of the aortic arch, typically located just beyond the left subclavian artery. […] The condition is diagnosed through clinical evaluation, advanced imaging techniques like echocardiography, computed tomography, and magnetic resonance imaging and occasionally invasive procedures like cardiac catheterization. […] Early diagnosis and intervention are crucial, as untreated coarctation can lead to serious complications, including hypertension, left ventricular hypertrophy, aortic dissection, and intracranial hemorrhage by the third or fourth decade of life. […] Advances in imaging modalities and surgical techniques have significantly improved the prognosis for individuals with coarctation, making it a focal point of ongoing research and clinical practice.
- #12 Coarctation of the Aorta – Pediatric Heart Specialistshttps://pediatricheartspecialists.com/heart-education/15-congenital-heart-defects/169-coarctation-of-the-aorta
The diagnosis of coarctation is usually made with an echocardiogram, which can define the anatomy of the aorta and evaluate for other cardiac anomalies. […] Occasionally in older children the region of coarctation is difficult to image well by echocardiography. In such cases, MRI or CT scan may be used. Cardiac catheterization is occasionally necessary for diagnosis.
- #13 Aortic Coarctation Workup: Approach Considerations, Imaging Studieshttps://emedicine.medscape.com/article/150369-workup
The diagnosis of coarctation of the aorta generally can be made on clinical evaluation, particularly the physical examination. No specific laboratory tests are necessary. […] Diagnosis of coarctation of the aorta is made by two-dimensional echocardiography, pulsed-wave Doppler ultrasonography, and color flow mapping. Classic findings are narrowing of the isthmus and posterior indentation or shelf. Blood flow velocities proximal and distal to obstruction, measured by Doppler tracings, can be used to estimate pressure gradient across the coarctation by a modified Bernoulli equation. Color flow mapping shows changes in color at the site of obstruction due to increases in blood velocity and turbulence. […] In older patients, coarctation may be difficult to diagnose by surface echocardiography. For these patients, magnetic resonance imaging (MRI), transesophageal echocardiography (TEE), or cardiac catheterization with angiography may be necessary to make the diagnosis.
- #14 Coarctation of the Aorta – Children’s Health Issues – Merck Manual Consumer Versionhttps://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/coarctation-of-the-aorta
Coarctation of the aorta is a narrowing of part of the aorta, the main blood vessel bringing red oxygenated blood from the heart to the body. […] The diagnosis is suspected based on symptoms and findings during a doctor’s examination and is confirmed with echocardiography. […] Coarctation is usually suspected when a doctor notices a heart murmur or differences in pulses or blood pressures between the arms and legs when doing a physical examination. A heart murmur is a sound created by turbulent blood flow through narrowed or leaking heart valves or through abnormal heart structures. In addition, the blood pressure measured in the arms may be quite high. […] Echocardiography (ultrasonography of the heart) and, sometimes, CT or MRI confirms the diagnosis. […] Electrocardiography (ECG) and chest x-rays are typically done. They may show an enlarged heart.
- #15 Coarctation of the Aorta | UH Harrington Heart & Vascular | University Hospitalshttps://www.uhhospitals.org/services/heart-and-vascular-services/conditions-and-treatments/aortic-disease/conditions-and-treatments/coarctation-of-the-aorta
Severe aortic coarctation is usually diagnosed soon after birth. […] The condition can also be revealed via ultrasounds done during pregnancy. […] A doctor will check for the following if coarctation of the aorta is suspected: High blood pressure in the arms, Lower blood pressure in the legs than in the arms, Weak or delayed pulse in the legs, A whooshing sound made by blood flowing through the narrowed aorta (heart murmur). […] Tests to confirm a diagnosis of coarctation of the aorta may include: Chest X-ray: Can show a narrowing in the aorta at the site of the coarctation or an enlarged portion of the aorta or both. […] Echocardiogram (Echo): Uses ultrasonic waves to assess your hearts structure and function. An echocardiogram often shows the location and severity of aortic coarctation.
- #15 Coarctation of the Aorta | UH Harrington Heart & Vascular | University Hospitalshttps://www.uhhospitals.org/services/heart-and-vascular-services/conditions-and-treatments/aortic-disease/conditions-and-treatments/coarctation-of-the-aorta
Electrocardiogram (ECG): Records the electrical activity of your heart over a period of time. If the aortic coarctation is severe, an ECG may reveal thickening of the walls of the lower heart chambers (ventricular hypertrophy). […] Computerized tomography (CT) scan: A CT scan uses X-rays to produces detailed cross-sectional images of the body. […] CT angiogram: In this test, special X-rays show how blood flows through the arteries and veins. A CT angiogram can reveal the location and severity of aortic coarctation and determine whether the condition is affecting other blood vessels in your body. […] Cardiac magnetic resonance imaging (MRI): A cardiac MRI uses magnetic fields and radio waves to create detailed images of the heart and blood vessels. This test can show the location and severity of the aortic coarctation.
- #16 Coarctation of the Aorta – Pediatrics – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/coarctation-of-the-aorta
Coarctation of the aorta is a localized narrowing of the aortic lumen that results in upper-extremity hypertension, left ventricular hypertrophy, and, if severe, malperfusion of the abdominal organs and lower extremities. […] Diagnosis is by echocardiography or by CT or MR angiography. […] Diagnosis is suggested by clinical examination (including BP measurement in all 4 extremities and comparing amplitude of brachial and femoral pulses), supported by chest x-ray and ECG, and established by 2-dimensional echocardiography with color flow and Doppler studies or, in older patients with a suboptimal echocardiographic window, with CT or MR angiography. […] Chest x-ray shows coarctation as a 3 sign in the upper left mediastinal shadow. […] ECG usually shows left ventricular hypertrophy but findings may be normal.
- #17 Coarctation of the aorta – Wikipediahttps://en.wikipedia.org/wiki/Coarctation_of_the_aorta
With imaging, resorption of the lower part of the ribs may be seen, due to increased blood flow over the neurovascular bundle that runs there. Prestenotic dilatation of the aortic arch and left subclavian artery, as well as indentation at the site of coarctation results in a classic 'figure 3 sign’ on x-ray.
- #18 SciELO Brazil – The importance of the early diagnosis of aorta coarctation The importance of the early diagnosis of aorta coarctationhttps://www.scielo.br/j/ramb/a/TgbSFrhPMBgthnxtgCmtrSf/
A general and thorough physical examination with the measurement of arterial pressure can reveal a discrepancy in limb arterial blood pressures (differential SBP popliteal/brachial greater than or equal to 20 mmHg). There is, in these cases, arterial hypertension in the upper limbs, which is related to the obstruction of the coarcted area and the activation of the renin-angiotensin system in response to the decrease in the perfusion of the renal artery below the obstruction. […] Some complementary examinations, such as x-ray, electrocardiogram, transthoracic and transesophageal echocardiography, angiography, computed tomography, and magnetic resonance imaging may assist in the diagnosis. […] Cardiac magnetic resonance is currently the gold standard test for evaluating aortic coarctation, and it can be used diagnosing and monitoring the pathology. […] The ideal treatment for correcting aortic coarctation remains under debate due to the lack of long-term clinical and imaging data to compare the different techniques used for correction.
- #18 SciELO Brazil – The importance of the early diagnosis of aorta coarctation The importance of the early diagnosis of aorta coarctationhttps://www.scielo.br/j/ramb/a/TgbSFrhPMBgthnxtgCmtrSf/
Coarctation of the aorta is a congenital heart disease characterized by a narrowing that occurs in the aortic artery. This constriction can occur anywhere along its entire length; however, it is more common between the origin of the left subclavian artery and the ductus arteriosus. Its incidence corresponds to 3 cases per 10,000 births. Thus, it is a common cardiopathy, but with high mortality and morbidity rates, which are related to a failure in the early diagnosis. […] In clinical practice, it is observed that many patients are undiagnosed due to the lack of a detailed clinical examination, since measuring the pulse of upper and lower limbs is not routine. It is estimated that approximately 60% to 80% of newborns with aortic coarctation are not diagnosed before hospital discharge. However, this is a pathology that can be easily recognized in clinical practice.
- #19 Coarctation of the Aorta: Diagnosis and Managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC10340190/
A peak-to-peak gradient of 20 mmHg on catheterisation is considered to be evidence of significant CoA in international guidelines. […] The strongest recommendation for intervention is for hypertensive patients with an increased non-invasive gradient between upper and lower limbs, confirmed with invasive measurement (peak-to-peak 20 mmHg) with a preference for catheter treatment when technically feasible. […] The role of diagnostic catheterisation is increasingly being superseded by cross-sectional imaging in the surveillance of patients following surgical repair; however, it retains importance where catheter-based intervention for re-stenosis is being considered, whether this is balloon angioplasty or stenting.
- #20 Coarctation of the aorta | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/coarctation-of-the-aorta?lang=us
Coarctation of the aorta (CoA) refers to a narrowing of the aortic lumen. […] Coarctations account for between 5-8% of all congenital heart defects. […] Patients may be asymptomatic in a setting of non-severe stenosis. […] Children and adults can present with angina pectoris and leg claudication. […] On clinical examination, diminished femoral pulses and differential blood pressure between upper and lower extremities may be noted. […] The urgency of treatment depends on the presence of congestive cardiac failure. […] Treatment can be either primary surgical repair with excision of the coarctation and end-to-end anastomosis, or balloon angioplasty. […] All modalities are capable of delineating the coarctation as well as collateral vessels, most common collateral pathway being subclavian artery to internal mammary artery to intercostal arteries (resulting in inferior rib notching) to post-coarctation part of descending thoracic aorta. […] Imaging differential considerations include: pseudo-coarctation of the aorta: elongation, narrowing or kinking with no pressure gradient or collateral formation, no rib notching.
- #21 Coarctation of the Aorta | Children’s Mercy Kansas Cityhttps://www.childrensmercy.org/departments-and-clinics/heart-center/understanding-coarctation-of-the-aorta/
Pulse oximetry: a way to monitor the oxygen content of the blood via a light probe placed on your baby’s hand or foot. […] Electrocardiogram (ECG): a visual representation of the heart’s electrical activity captured via monitors placed on the skin. […] Echocardiogram (echo): an ultrasound of the heart that evaluates the structure and the function of the heart by using sound waves. […] Chest X-ray: a test that uses a small amount of radiation to create an image (or picture) within the chest to include the heart, lungs, blood vessels and bones. […] A cardiac catheterization procedure is another option for repair of the coarctation in some individuals. […] The procedures may include a balloon dilatation (angioplasty) and/or device stent placement at the site of the narrowing. […] Your child will be seen by a cardiology provider to monitor for cardiac findings to include hypertension (high blood pressure) and the possible recurrence or the development of narrowing at the site of the surgery or cardiac catheterization treatment.
- #22 Late diagnosis of coarctation of the aorta in a 44-year-old male: a case report | BMC Cardiovascular Disorders | Full Texthttps://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-020-01753-1
Coarctation of the aorta is a rare congenital disease. In adults, the main manifestations include hypertension, weak or absent femoral pulses, heart failure, and left ventricular hypertrophy. […] This case shows that coarctation of the aorta can be cured and that hypertension caused by the condition can be controlled to some extent with medication. Based on our findings, we recommend a detailed physical examination for all patients suspected of having coarctation of the aorta; the examination should include blood pressure measurements of both the upper and lower extremities. […] In adults, diagnosis of coarctation of the aorta is based on medical history, physical examination, and imaging studies. It is very important of physical examination and imaging studies for adult hypertension with cardiomegaly and heart failure and renal insufficiency.
- #23 Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta | Scientific Reportshttps://www.nature.com/articles/s41598-023-31749-8
Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. The measurements that showed significant differences were aortic isthmus diameter Z-score (p0.001), ductus arteriosus diameter/aortic isthmus diameter (p0.001), and distal aortic arch (DA) index (p0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Measurement of the DA index improved the diagnostic rate of fetal CoA and a DA index 1.28 indicated fetal CoA cases requiring surgical intervention.
- #23 Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta | Scientific Reportshttps://www.nature.com/articles/s41598-023-31749-8
Prenatal detection of CoA remains a challenge despite its importance, which is indicated by the significantly higher mortality and morbidity in newborns without prenatal detection. […] Our results showed that the DA index is useful for screening neonatal patients to determine whether surgical intervention is needed. A DA index 1.28 was useful for detecting CoA in terms of both sensitivity and specificity compared to previous reports. […] To prevent severe postnatal respiratory and circulatory failure, detailed scanning of the fetal heart is critical when a cardiac disproportion is noted, or when cardiac structures are not visualized on routine echocardiographic scanning. The early recognition of CoA reduces morbidity and mortality. […] In conclusion, the prenatal detection rate of CoA may be improved when the DA index is used. The implementation of this assessment may prevent neonates from experiencing circulatory shock.
- #24 Coarctation of the Aorta – Cardiothoracic Surgeryhttps://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/coarctation-of-the-aorta/
Coarctation of the aorta is a narrowing that usually occurs near the site of insertion of the ductus arteriosus. It frequently occurs in association with other heart defects, including bicuspid aortic valve in 50% of patients and VSD in 30-60%. […] Diagnosis is confirmed by echocardiography, which will also show associated defects. A peak gradient of > 20 mmHg by echo suggests significant coarctation.
- #25 Aortic coarctation – Symptoms, diagnosis and treatment | BMJ Best Practicehttps://bestpractice.bmj.com/topics/en-gb/698
Aortic coarctation is characterised by a blood pressure differential between upper and lower extremities (upper lower). […] Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiography. […] Key diagnostic factors include hypertension presenting at a young age or resistant to treatment, diminished lower extremity pulses, and differential upper and lower extremity blood pressure (BP). […] 1st investigations to order include electrocardiogram (ECG), chest x-ray (CXR), and echocardiogram. […] Investigations to consider include computed tomography (CT) angiography, magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA), and cardiac catheterisation.
- #26 Aortic coarctation – Symptoms, diagnosis and treatment | BMJ Best Practice UShttps://bestpractice.bmj.com/topics/en-us/698
Aortic coarctation is characterized by a blood pressure differential between upper and lower extremities (upper lower). […] Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiography. […] Key diagnostic factors include hypertension presenting at a young age or resistant to treatment, diminished lower extremity pulses, and differential upper and lower extremity blood pressure (BP). […] 1st tests to order include electrocardiogram (ECG), chest x-ray (CXR), and echocardiogram. […] Tests to consider include computed tomography (CT) angiography, magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA), and cardiac catheterization.