Pierścień naczyniowy
Epidemiologia

Pierścień naczyniowy to rzadka wrodzona wada sercowo-naczyniowa, występująca u 0,04-0,5% żywych urodzeń, częściej u chłopców (1,4-2 razy). Najczęstsze typy to podwójny łuk aorty (25-45%) oraz prawy łuk aorty z lewym więzadłem tętniczym (30-65%), które łącznie stanowią 85-95% przypadków. W około 10-25% współistnieją inne wady serca, a pętla tętnicy płucnej (około 10%) często łączy się z wadami wewnątrzsercowymi i anomaliami tchawiczo-oskrzelowymi. Diagnostyka opiera się na obrazowaniu: RTG klatki piersiowej, ezofagogramie, echokardiografii, a przede wszystkim na tomografii komputerowej wielorzędowej (MDCT) i rezonansie magnetycznym (MRI). Prenatalne wykrywanie pierścieni naczyniowych dzięki ultrasonografii płodowej i sekwencyjnemu skanowaniu przekrojowemu (SCS) poprawia rokowanie i planowanie leczenia.

Pierścień naczyniowy: Epidemiologia

Pierścień naczyniowy (ang. vascular ring) to rzadka wrodzona anomalia układu sercowo-naczyniowego, w której nieprawidłowo ukształtowane naczynia krwionośne otaczają i uciskają tchawicę i/lub przełyk. Stanowi zaledwie 1-3% wszystkich wrodzonych wad serca, co przekłada się na około 0,04-0,5% żywych urodzeń.12 Częstość występowania pierścieni naczyniowych szacuje się na około 1 na 2500 do 1 na 10 000 żywych urodzeń.34

Dane epidemiologiczne wskazują, że u chłopców wada ta występuje około 1,4-2 razy częściej niż u dziewczynek.56 Jednakże niektóre ośrodki donoszą, że uchyłek Kommerella (Kommerell diverticulum, KD) może występować częściej u kobiet.7 Nie wykazano predyspozycji etnicznych ani geograficznych.89

Występowanie poszczególnych typów pierścieni naczyniowych

Wśród pierścieni naczyniowych najczęściej występują:1011

  • Podwójny łuk aorty (double aortic arch, DAA) – stanowi 25-45% wszystkich przypadków
  • Prawy łuk aorty z aberrantną lewą tętnicą podobojczykową i lewostronnym przewodem tętniczym – stanowi 30-65% przypadków
  • Inne, rzadsze formy (m.in. ucisk przez tętnicę bezimienną, aberrantna lewa tętnica podobojczykowa, pętla tętnicy płucnej) łącznie stanowią około 6-32% przypadków

12

Dwa najczęstsze typy kompletnych pierścieni naczyniowych – podwójny łuk aorty oraz prawy łuk aorty z lewym więzadłem tętniczym – stanowią łącznie 85-95% wszystkich przypadków.1314

Szczególnym rodzajem anomalii naczyniowej jest pętla tętnicy płucnej (pulmonary artery sling), która stanowi około 10% przypadków, chociaż dokładna częstość jest trudna do ustalenia ze względu na niewielką liczbę opisanych przypadków.15

Współistniejące wady serca i czynniki ryzyka

Około 10-25% przypadków pierścieni naczyniowych współistnieje z innymi wrodzonymi wadami serca.16 Szczególnie pętla tętnicy płucnej jest związana z wadami wewnątrzsercowymi w 10-15% przypadków.17 Obecność złożonej wady serca znacząco wpływa na śmiertelność pacjentów z pierścieniem naczyniowym.18

Anomalie tchawiczo-oskrzelowe rzadko współwystępują z pierścieniami naczyniowymi, ale są powszechne w przypadku pętli tętnicy płucnej.19 Warto zauważyć, że u dzieci poczętych techniką zapłodnienia in vitro występuje zwiększone ryzyko wystąpienia tej wady.20

Wykazano również związek między mikrodeletami chromosomu 22q11 a różnymi anomaliami łuku aorty, choć większość przypadków występuje sporadycznie.21

Diagnostyka i nadzór

Aktualne metody diagnostyczne

Diagnostyka pierścieni naczyniowych ewoluowała w ostatnich dekadach dzięki postępowi w technikach obrazowania. Obecnie stosowane są następujące metody:2223

  • Zdjęcie rentgenowskie klatki piersiowej – zazwyczaj pierwsze badanie, ale o ograniczonej wartości diagnostycznej
  • Ezofagogram z barytem – tradycyjnie używany do identyfikacji ucisków przełyku
  • Echokardiografia – zalecana, aby wykluczyć towarzyszące wewnątrzsercowe wady, choć sama w sobie może nie wystarczyć do pełnego zobrazowania anatomii pierścienia naczyniowego
  • Tomografia komputerowa wielorzędowa (MDCT) – obecnie preferowana metoda diagnostyczna, zapewniająca szczegółową ocenę anatomiczną
  • Rezonans magnetyczny (MRI) – alternatywa dla CT, szczególnie cenna w planowaniu operacji
  • Bronchoskopia – w przypadku podejrzenia anomalii tchawicy

2425

Szczególnie tomografia komputerowa stała się metodą z wyboru w diagnostyce i charakterystyce pierścieni naczyniowych, zarówno w ocenie przedoperacyjnej, jak i pooperacyjnej.2627

Diagnostyka prenatalna

W ostatnich latach nastąpił wzrost wykrywania pierścieni naczyniowych w okresie prenatalnym dzięki udoskonaleniom w badaniach przesiewowych płodu.28 Pierścienie naczyniowe mogą być dokładnie diagnozowane za pomocą ultrasonografii płodowej, co poprawia rokowanie i umożliwia właściwe zaplanowanie badań postnatalnych i ewentualnej operacji.2930

Badanie sekwencyjnego skanowania przekrojowego (SCS) pozwala na dokładne rozpoznanie pierścieni naczyniowych przed porodem, ocenę kształtu i rozmiaru pierścieni oraz monitorowanie prenatalne dzieci do momentu urodzenia.31 Szczególnie istotna jest ocena relacji między pierścieniem naczyniowym a drogami oddechowymi, gdyż ma to kluczowe znaczenie dla rokowania.

Dzięki zwiększonej częstości wykrywania prenatalnego uświadomiono sobie, że częstość występowania tej rzadkiej wady jest dwukrotnie większa niż tetralogii Fallota.32 Jednakże częstość przeoczonych lub błędnych diagnoz pozostaje wysoka, szczególnie w przypadku izolowanych pierścieni naczyniowych – prenatalne dane statystyczne wskazują na zaledwie 1 na 1000.33

Wyzwania diagnostyczne

Diagnoza pierścieni naczyniowych stanowi wyzwanie i wymaga wysokiego indeksu podejrzenia ze względu na stosunkowo niską częstość występowania tych anomalii w porównaniu z innymi schorzeniami powodującymi zaburzenia oddechowe u dzieci.3435

U wielu pacjentów diagnoza jest opóźniona, ponieważ objawy przypisuje się innym, częstszym schorzeniom, takim jak laryngomalacja, zapalenie oskrzelików, astma czy refluks żołądkowo-przełykowy.36 Większość pacjentów jest diagnozowana przed ukończeniem 12 miesiąca życia, po licznych ocenach objawów ze strony układu oddechowego.37

Rokowanie i wyniki leczenia

Ogólne rokowanie w przypadku pierścienia naczyniowego jest stosunkowo dobre.38 Chirurgiczna naprawa pierścienia naczyniowego wiąże się z niskim ryzykiem operacyjnym przy braku złożonej choroby serca.39

Wyniki leczenia chirurgicznego pierścieni naczyniowych są znakomite – w niektórych ośrodkach o dużym doświadczeniu nie odnotowano śmiertelności operacyjnej w ciągu ostatnich 30 lat, a ponad 90% pacjentów obserwuje ustąpienie objawów.4041

Czynniki ryzyka związane ze śmiertelnością obejmują:42

  • Obecność złożonej choroby serca (główny czynnik wpływający na śmiertelność)
  • Zaburzenia oddechowe w momencie zgłoszenia
  • Podstawowe uwarunkowania genetyczne
  • Współistniejące wrodzone zwężenie tchawicy lub ciężka bronchomalacja
  • Tetralogia Fallota z nieobecnością zastawki płucnej
  • Złożona sinicza choroba serca
  • Konieczność wczesnego wsparcia oddechowego

Chorobowość wewnątrzszpitalna po operacji jest akceptowalna (około 18%) i obejmuje głównie wysięki opłucnowe i niedodmę płuc.43 Chociaż około 24% pacjentów pozostaje objawowych w długoterminowej obserwacji, połowa z nich ma objawy związane z astmą, które są kontrolowane farmakologicznie, co daje prawie 90% pacjentów wolnych od objawów w średnioterminowej obserwacji.44

Kontrowersje w postępowaniu

Ze wzrostem częstości diagnozowania prenatalnego pierścieni naczyniowych pojawiła się kontrowersja dotycząca optymalnego postępowania u bezobjawowych lub skąpoobjawowych noworodków i niemowląt.4546

W niedawnym brytyjskim badaniu ankietowym 20% respondentów zalecało naprawę chirurgiczną u bezobjawowych pacjentów.47 Szczegółowy algorytm chirurgiczny dla bezobjawowych pacjentów z rozpoznaniem pierścienia naczyniowego został niedawno zaproponowany przez grupę z Chicago, jeden z wiodących ośrodków w tej dziedzinie.48

Zabieg chirurgiczny u pacjentów bezobjawowych skutkuje minimalną chorobowością szpitalną, co potwierdza obecne zalecenie wczesnej naprawy chirurgicznej po dokładnej przedoperacyjnej ocenie towarzyszących anomalii tchawiczo-oskrzelowych.49 Jednakże sama obecność pierścienia naczyniowego nie jest wskazaniem do interwencji chirurgicznej – warto wiedzieć, że objawy u małych dzieci mogą ustąpić wraz ze wzrostem dziecka i klatki piersiowej.50

Niedawno Said i Biermann potwierdzili w dwóch różnych publikacjach wskazanie do leczenia chirurgicznego wszystkich kompletnych pierścieni naczyniowych (DAA, RAA z LLA, LAA z RLA) i pętli tętnicy płucnej (LPA), pozostawiając operację dla objawowych niekompletnych pierścieni naczyniowych lub tych bezobjawowych ze współistniejącymi wadami serca.51

Wnioski i perspektywy

Pierścienie naczyniowe, choć rzadkie, stanowią istotny problem kliniczny wymagający wczesnej diagnostyki i odpowiedniego postępowania. Obecne metody diagnostyczne, w szczególności tomografia komputerowa i rezonans magnetyczny, znacząco poprawiły możliwości rozpoznawania tych anomalii, zarówno prenatalnie, jak i postnatalnie.52

Wczesna diagnoza i skierowanie do specjalistycznych ośrodków może zapobiec długoterminowym powikłaniom i poprawić wyniki oddechowe u tych pacjentów.53 Szczególnie ważne jest, aby lekarze pamiętali o możliwości występowania pierścienia naczyniowego u dzieci z nawracającymi objawami ze strony układu oddechowego, które słabo reagują na leczenie farmakologiczne.54

Podejście multidyscyplinarne do diagnostyki i leczenia jest kluczowe dla osiągnięcia optymalnych wyników.55 Kompleksowa opieka nad tymi pacjentami wymaga skoordynowanych wysiłków dużego zespołu specjalistów.

Badania w przyszłości powinny skupić się na poprawie dokładności diagnostyki prenatalnej, wyjaśnieniu potencjalnych objawów, optymalnych strategiach diagnostycznych oraz wskazaniach do leczenia chirurgicznego, szczególnie w przypadku pacjentów bezobjawowych.56

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Vascular Rings and Pulmonary Artery Sling | Abdominal Key
    https://abdominalkey.com/vascular-rings-and-pulmonary-artery-sling/
    Vascular rings and pulmonary artery sling (PAS) represent 1 to 3 percent of all congenital cardiac malformations. The most frequent type of vascular ring is aberrant left subclavian artery, whereas the most rare condition is the PAS. […] Because some patients with vascular rings fail to manifest symptoms, the exact prevalence of this anomaly is not truly known. In one comprehensive series, the estimate ranged from 0.04 to 0.5 percent of live births or approximately 1 to 3 percent of surgical congenital cardiovascular anomalies. […] In a comprehensive cardiovascular screening program using echocardiography in school-age children, Chen and colleagues reported the following incidence of vascular rings in 186,213 screened children: PA sling (n= 11, 0.006 percent), anatomically complete vascular ring (DAA, right aortic arch with left ligamentum, n = 96, 0.05 percent).
  • #2 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    Vascular rings and pulmonary slings are congenital anomalies of the aortic arch and pulmonary artery. They are very important but rare causes for common respiratory symptoms, especially in infants. Their etiology is related to abnormal development of the embryonic aortic arches. When the abnormal blood vessels form a ring around the trachea and esophagus, it is termed vascular ring. These rings may be complete or incomplete. The severity of symptoms depends on the degree of compression of the trachea and esophagus. […] Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings.
  • #3 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital
    https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/
    Vascular rings occur in approximately 1 in 2,500 births, with a higher prevalence in boys. […] Vascular rings can be diagnosed based on symptoms or findings on a chest X-ray or echocardiogram. […] If a vascular ring is suspected in utero, you may meet with our Fetal Cardiology team to discuss possible impacts on pregnancy and delivery.
  • #4 Vascular Ring Surgical Repair: Re-Implantation of the Left Subclavian Artery into the Left Carotid Artery in a Pediatric Patient
    https://www.mdpi.com/2075-4418/14/16/1736
    Vascular rings have an estimated prevalence of 1 in 10,000 live births. […] These rare congenital anomalies of the aortic arch encircle and compress the trachea and/or esophagus, presenting as nonspecific symptoms that vary in severity depending on the affected structure and the degree of compression. […] Early surgical intervention is crucial in symptomatic vascular rings. […] Surgical repair focuses on relieving tracheal and/or esophageal compression by dividing the vascular ring, thereby providing symptomatic relief and preventing serious complications, such as sudden death or residual tracheobronchial damage. […] However, determining symptoms in infants and pediatric patients can be challenging due to symptom overlap with common conditions in these age groups. […] Traditionally, the initial evaluation of vascular rings involves chest X-ray, barium swallow, and echocardiography.
  • #5 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560919/
    Incidence of aortic arch anomalies: Approximately 1 to 3 percent of all congenital heart disease. […] Compared to females, males appear to have a 1.4 to 2 times greater risk of having a vascular ring than females. […] Among the aortic arch anomalies that can lead to a vascular ring, the following with the first two listed below comprising more than 90% of all vascular rings seen: Double aortic arch (DAA), RAA and aberrant LSCA and left PDA.
  • #6 Vascular Rings | Concise Medical Knowledge
    https://www.lecturio.com/concepts/vascular-rings/
    1%3% of all congenital cardiac malformations […] Male:female ratio, 2:1 […] Incidence of various forms: […] Right aortic arch with aberrant left subclavian artery and left-sided ductus arteriosus: 3065% of cases […] Double aortic arch: 2545% of cases […] All other forms (e.g., innominate artery compression, aberrant left subclavian artery, pulmonary artery sling) collectively: ~632% of cases […] 10%25% of cases are associated with underlying cardiac anomaly […] Increased risk in infants conceived by in vitro fertilization.
  • #7
    https://link.springer.com/article/10.1007/s11886-022-01764-8
    Vascular rings account for less than 1% of all congenital cardiovascular anomalies. […] Although vascular rings do not have gender predilection, some groups report a higher prevalence of Kommerell diverticulum (KD) in women. […] The diagnostic work-up of patients with vascular rings has evolved dramatically in the last few decades owing to the advancement in imaging modalities, applicable not only after birth but also during pregnancy. […] The presence of KD in adults with previous diagnosis of vascular ring increases the risk of aortic aneurysm and aortic dissection. […] Although being only a partial ring, the LPA sling represents a more complex spectrum of vascular rings.
  • #8 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    Vascular rings are uncommon anomalies and make up fewer than 1% of all congenital cardiac defects. They occur with about equal frequency in both sexes. No geographical or racial predominance exists. Some vascular rings are associated with other congenital heart defects; others may be isolated deficits. […] The two most common types of complete vascular rings are double aortic arch and right aortic arch with left ligamentum arteriosum. These make up 85-95% of the cases. […] Two other complete vascular rings that are extremely rare (1%) are (1) right aortic arch with mirror-image branching and (2) left ligamentum arteriosum and left aortic arch with retroesophageal right subclavian artery, right-side descending aorta, and right ligamentum arteriosum. […] Other named anomalies that produce symptoms but do not form a complete anatomic vascular ring make up the remainder and include the abnormally placed or anomalous innominate artery and the retroesophageal right subclavian artery with left-side aorta and left ligamentum arteriosum.
  • #9 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/vascular-rings-and-slings?embed_domain=hackmd.io%2F%40yIPUAFeCSL2JsU8smR5nJQ%2Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.ico&lang=us
    Vascular rings are rare, occurring in 1% of patients. No gender or ethnic predispositions have been identified. […] Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels.
  • #10 Vascular Aortic Arch Ring – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560919/
    Incidence of aortic arch anomalies: Approximately 1 to 3 percent of all congenital heart disease. […] Compared to females, males appear to have a 1.4 to 2 times greater risk of having a vascular ring than females. […] Among the aortic arch anomalies that can lead to a vascular ring, the following with the first two listed below comprising more than 90% of all vascular rings seen: Double aortic arch (DAA), RAA and aberrant LSCA and left PDA.
  • #11 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    Vascular rings are uncommon anomalies and make up fewer than 1% of all congenital cardiac defects. They occur with about equal frequency in both sexes. No geographical or racial predominance exists. Some vascular rings are associated with other congenital heart defects; others may be isolated deficits. […] The two most common types of complete vascular rings are double aortic arch and right aortic arch with left ligamentum arteriosum. These make up 85-95% of the cases. […] Two other complete vascular rings that are extremely rare (1%) are (1) right aortic arch with mirror-image branching and (2) left ligamentum arteriosum and left aortic arch with retroesophageal right subclavian artery, right-side descending aorta, and right ligamentum arteriosum. […] Other named anomalies that produce symptoms but do not form a complete anatomic vascular ring make up the remainder and include the abnormally placed or anomalous innominate artery and the retroesophageal right subclavian artery with left-side aorta and left ligamentum arteriosum.
  • #12 Vascular Rings | Concise Medical Knowledge
    https://www.lecturio.com/concepts/vascular-rings/
    1%3% of all congenital cardiac malformations […] Male:female ratio, 2:1 […] Incidence of various forms: […] Right aortic arch with aberrant left subclavian artery and left-sided ductus arteriosus: 3065% of cases […] Double aortic arch: 2545% of cases […] All other forms (e.g., innominate artery compression, aberrant left subclavian artery, pulmonary artery sling) collectively: ~632% of cases […] 10%25% of cases are associated with underlying cardiac anomaly […] Increased risk in infants conceived by in vitro fertilization.
  • #13 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    Vascular rings are uncommon anomalies and make up fewer than 1% of all congenital cardiac defects. They occur with about equal frequency in both sexes. No geographical or racial predominance exists. Some vascular rings are associated with other congenital heart defects; others may be isolated deficits. […] The two most common types of complete vascular rings are double aortic arch and right aortic arch with left ligamentum arteriosum. These make up 85-95% of the cases. […] Two other complete vascular rings that are extremely rare (1%) are (1) right aortic arch with mirror-image branching and (2) left ligamentum arteriosum and left aortic arch with retroesophageal right subclavian artery, right-side descending aorta, and right ligamentum arteriosum. […] Other named anomalies that produce symptoms but do not form a complete anatomic vascular ring make up the remainder and include the abnormally placed or anomalous innominate artery and the retroesophageal right subclavian artery with left-side aorta and left ligamentum arteriosum.
  • #14 G151 A retrospective study of all patients diagnosed with vascular rings between 2001–2015 at a tertiary paediatric hospital | Archives of Disease in Childhood
    https://adc.bmj.com/content/101/Suppl_1/A78.2
    Vascular rings are congenital anomalies occurring early in the development of the aortic arch and great vessels. The two most common types of complete vascular rings are double aortic arch and right aortic arch with left ligamentum arteriosum, making up 85-90% of cases. Vascular rings make up less than 1% of congenital cardiac defects. Primary symptoms associated with vascular rings relate to structures encircled by the ring such as the trachea and oesophagus. Traditionally identified by barium swallow, cross-sectional modalities (CT/MRI) are the chosen modality; diagnosis can be missed by echocardiography alone. […] A retrospective study identifying all patients with vascular rings using the cardiac database presenting between 2001-2015. 69 patients were diagnosed with a vascular ring. There was a male preponderance (45/69). The most common diagnosis was double aortic arch (40 patients), followed by right aortic arch with aberrant left subclavian (26 patients). Stridor was the commonest symptom (34 patients). […] Vascular rings can present in a variety of different ways. The majority of cases require surgery. Cross sectional studies should be undertaken to prevent missed diagnosis.
  • #15 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    The anomalous left pulmonary artery or pulmonary artery sling makes up about 10% of cases, though the exact number is difficult to determine because there are so few case reports. Although the pulmonary artery sling is not associated with the aortic arch or its branches, it arises from an abnormality of the sixth branchial arch and produces a complete ring. This anomaly is associated with intracardiac defects in 10-15% of cases.
  • #16 Vascular Rings | Concise Medical Knowledge
    https://www.lecturio.com/concepts/vascular-rings/
    1%3% of all congenital cardiac malformations […] Male:female ratio, 2:1 […] Incidence of various forms: […] Right aortic arch with aberrant left subclavian artery and left-sided ductus arteriosus: 3065% of cases […] Double aortic arch: 2545% of cases […] All other forms (e.g., innominate artery compression, aberrant left subclavian artery, pulmonary artery sling) collectively: ~632% of cases […] 10%25% of cases are associated with underlying cardiac anomaly […] Increased risk in infants conceived by in vitro fertilization.
  • #17 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    The anomalous left pulmonary artery or pulmonary artery sling makes up about 10% of cases, though the exact number is difficult to determine because there are so few case reports. Although the pulmonary artery sling is not associated with the aortic arch or its branches, it arises from an abnormality of the sixth branchial arch and produces a complete ring. This anomaly is associated with intracardiac defects in 10-15% of cases.
  • #18 Clinical Course of Vascular Rings and Risk Factors Associated With Mortality
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3341422/
    Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. […] The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. […] There have not been any studies evaluating the risk factors for mortality from vascular rings, until now. This study was performed to analyze the clinical characteristics and treatments of patients with various vascular rings and evaluate associated risk factors for mortality. […] The presence of a complex heart disease significantly influenced mortality (p=0.002). […] The presence of a complex heart disease was significantly associated with mortality. […] The factors associated with mortality are shown in Table 4. We found that only the presence of complex heart disease significantly influenced mortality.
  • #19 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    Vascular rings and pulmonary slings are congenital anomalies of the aortic arch and pulmonary artery. They are very important but rare causes for common respiratory symptoms, especially in infants. Their etiology is related to abnormal development of the embryonic aortic arches. When the abnormal blood vessels form a ring around the trachea and esophagus, it is termed vascular ring. These rings may be complete or incomplete. The severity of symptoms depends on the degree of compression of the trachea and esophagus. […] Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings.
  • #20 Vascular Rings | Concise Medical Knowledge
    https://www.lecturio.com/concepts/vascular-rings/
    1%3% of all congenital cardiac malformations […] Male:female ratio, 2:1 […] Incidence of various forms: […] Right aortic arch with aberrant left subclavian artery and left-sided ductus arteriosus: 3065% of cases […] Double aortic arch: 2545% of cases […] All other forms (e.g., innominate artery compression, aberrant left subclavian artery, pulmonary artery sling) collectively: ~632% of cases […] 10%25% of cases are associated with underlying cardiac anomaly […] Increased risk in infants conceived by in vitro fertilization.
  • #21 Vascular Rings and Slings | Radiology Key
    https://radiologykey.com/vascular-rings-and-slings/
    Vascular rings and slings represent approximately 1% of congenital cardiovascular anomalies, although this incidence may be underestimated because some lesions are asymptomatic. […] Most cases are sporadic, but there may be a genetic inheritance in some arch anomalies. Microdeletions of chromosome 22q11, in particular, have been associated with various arch anomalies. […] Imaging is performed to confirm the morphology of the ring or sling and the effect on adjacent structures. Chest radiography is usually the initial imaging examination. If the chest radiograph is abnormal or if there is high clinical concern of a vascular anomaly but a normal chest radiograph, CT or MRI is performed to confirm the diagnosis and provide detailed anatomy of the anomaly and its effect on adjacent structures.
  • #22 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text
    https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3
    Vascular rings are congenital abnormalities of the aortic arch-derived vascular and ligamentous structures, which encircle the trachea and oesophagus to varying degrees, resulting in respiratory or feeding difficulties in children. […] Imaging plays an important role in the evaluation and management of vascular rings. […] Vascular rings can be identified by several imaging examinations and occasionally multiple imaging tests may be required to make a diagnosis. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Vascular rings are complex and diagnosis is often challenging because of variable and non-specific clinical presentations. CT angiography plays an important role in the identification and definition of the anatomy of these complex anomalies, thus providing a roadmap to surgeons.
  • #23 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    Evaluation of suspected vascular rings can include a chest x-ray, esophagram, echocardiogram and a CT or MRI. There is much debate on the most optimal imaging evaluation for vascular rings due to the advancement in radiographic studies. […] Echocardiography is always indicated to exclude associated intracardiac defects. It is possible but often difficult to completely delineate the anatomy of the vascular ring by echocardiography alone. […] Both CT and MRI can accurately define the anatomy and three-dimensional relationships of vascular rings and are mandatory for surgical planning. The choice of imaging modality is typically individualized depending on the facility and considering patient safety factors. […] Vascular rings are surgically corrected if the patient is symptomatic. If the patient is asymptomatic or has mild symptoms, he/she can be monitored and treated conservatively. Many mild symptoms can resolve with growth. It is however always indicated to surgically correct patients with pulmonary slings, double aortic arch and right arch with a left ligamentum arteriosum upon diagnosis since these patients will progressively become more symptomatic over time.
  • #24 Vascular Ring Surgical Repair: Re-Implantation of the Left Subclavian Artery into the Left Carotid Artery in a Pediatric Patient
    https://www.mdpi.com/2075-4418/14/16/1736
    Vascular rings have an estimated prevalence of 1 in 10,000 live births. […] These rare congenital anomalies of the aortic arch encircle and compress the trachea and/or esophagus, presenting as nonspecific symptoms that vary in severity depending on the affected structure and the degree of compression. […] Early surgical intervention is crucial in symptomatic vascular rings. […] Surgical repair focuses on relieving tracheal and/or esophageal compression by dividing the vascular ring, thereby providing symptomatic relief and preventing serious complications, such as sudden death or residual tracheobronchial damage. […] However, determining symptoms in infants and pediatric patients can be challenging due to symptom overlap with common conditions in these age groups. […] Traditionally, the initial evaluation of vascular rings involves chest X-ray, barium swallow, and echocardiography.
  • #25 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    Evaluation of suspected vascular rings can include a chest x-ray, esophagram, echocardiogram and a CT or MRI. There is much debate on the most optimal imaging evaluation for vascular rings due to the advancement in radiographic studies. […] Echocardiography is always indicated to exclude associated intracardiac defects. It is possible but often difficult to completely delineate the anatomy of the vascular ring by echocardiography alone. […] Both CT and MRI can accurately define the anatomy and three-dimensional relationships of vascular rings and are mandatory for surgical planning. The choice of imaging modality is typically individualized depending on the facility and considering patient safety factors. […] Vascular rings are surgically corrected if the patient is symptomatic. If the patient is asymptomatic or has mild symptoms, he/she can be monitored and treated conservatively. Many mild symptoms can resolve with growth. It is however always indicated to surgically correct patients with pulmonary slings, double aortic arch and right arch with a left ligamentum arteriosum upon diagnosis since these patients will progressively become more symptomatic over time.
  • #26
    https://step2.medbullets.com/evidence/31676999
    A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. […] With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. […] Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.
  • #27 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text
    https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3
    Vascular rings are congenital abnormalities of the aortic arch-derived vascular and ligamentous structures, which encircle the trachea and oesophagus to varying degrees, resulting in respiratory or feeding difficulties in children. […] Imaging plays an important role in the evaluation and management of vascular rings. […] Vascular rings can be identified by several imaging examinations and occasionally multiple imaging tests may be required to make a diagnosis. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Vascular rings are complex and diagnosis is often challenging because of variable and non-specific clinical presentations. CT angiography plays an important role in the identification and definition of the anatomy of these complex anomalies, thus providing a roadmap to surgeons.
  • #28 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    Vascular rings represent 1% of congenital cardiovascular abnormalities. […] While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. […] Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair. […] With the increased rate of fetal diagnosis of vascular ring, the optimal management in asymptomatic or mildly symptomatic neonates and infants is controversial. […] In a recent UK survey, 20% of the responders advised surgical repair in asymptomatic patients.
  • #29 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    VR can be accurately diagnosed by fetal ultrasound improving prognosis in these patients, as it enables proper timing of additional postnatal investigation and surgery. […] Unfortunately, there are no guidelines on the better diagnostic work up for children suspected of VR. However, its reasonable that children with history of recurrent respiratory symptoms poor response to medical treatment, symptoms and physical examination of airway obstruction, dysphagia, and failure to thrive should undergo to flexible laryngo-tracheo-bronchoscopy to assess for laryngomalacia, vocal fold mobility, tracheal stenosis or malacia. […] Morbidity and mortality in patients after surgical correction have been reported to a greater extent, in those with respiratory distress at presentation, underlying genetic condition, associated congenital tracheal stenosis or severe bronchomalacia, tetralogy of Fallot with absent pulmonary valve syndrome, underlying complex cyanotic heart disease and need of early respiratory support.
  • #30 Prenatal ultrasound scans showing a vascular ring | Great Ormond Street Hospital
    https://www.gosh.nhs.uk/conditions-and-treatments/procedures-and-treatments/prenatal-ultrasound-scans-showing-vascular-ring/
    This information sheet from Great Ormond Street Hospital (GOSH) explains about vascular rings detected during a prenatal ultrasound scan and what this might mean for your child. […] We are still learning about the possible impact on the circulation and the babys health after birth as we have not been able to detect potential vascular rings in pregnancy until recently. […] Doctors think that vascular rings occur early in pregnancy when the baby is developing in the womb. […] Vascular rings develop when some of these arches continue to develop abnormally. […] If you would prefer not to have an amniocentesis, the babys chromosomes could be checked with a blood test after birth. […] Your baby will have an ultrasound scan in the first few weeks after birth, so that the vascular ring can be assessed. […] The doctor will monitor the vascular ring, particularly when your baby starts to have solid food. […] Some babies may have to have an operation to release and rearrange the vessels so that the trachea and oesophagus are no longer compressed.
  • #31 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text
    https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y
    A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The different types of vascular rings have different incidences, clinical manifestations and severities. Symptoms frequently encountered include noisy breathing and a barky cough, recurrent upper respiratory infections, wheezing, exertional dyspnea, and dysphagia. Delaying treatment may result in sudden death or residual tracheobronchial damage. The rate of missed diagnoses and misdiagnoses remains high, in particular, for isolated vascular rings, prenatal data statistics are only 1 in 1000. The purpose of this study was to investigate the value of prenatal ultrasound in the integrated management of vascular ring. SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth. Early diagnosis of congenital vascular rings is intended to improve the symptoms of compression in patients with airway compression. With the increase of prenatal detection rate of vascular rings, the incidence of rare diseases has been realized to be twice that of tetralogy of Fallot, and the evaluation of the relationship between vascular rings and airways is more important. However, there are few articles on the prenatal diagnosis of vascular ring diseases and with qualitative or quantitative assessments of airway prognosis in PubMed. Based on this single-center sample, we believe that the shape of the vascular ring, the route of the branching vessels, and the distance from the airway are critical to the prognosis of these diseases. Dynamic sequential cross-sectional scanning can observe these aspects very well and intuitively, has more advantages than the long axis, and can carry out prenatal monitoring and management of the fetus until birth.
  • #32 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text
    https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y
    A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The different types of vascular rings have different incidences, clinical manifestations and severities. Symptoms frequently encountered include noisy breathing and a barky cough, recurrent upper respiratory infections, wheezing, exertional dyspnea, and dysphagia. Delaying treatment may result in sudden death or residual tracheobronchial damage. The rate of missed diagnoses and misdiagnoses remains high, in particular, for isolated vascular rings, prenatal data statistics are only 1 in 1000. The purpose of this study was to investigate the value of prenatal ultrasound in the integrated management of vascular ring. SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth. Early diagnosis of congenital vascular rings is intended to improve the symptoms of compression in patients with airway compression. With the increase of prenatal detection rate of vascular rings, the incidence of rare diseases has been realized to be twice that of tetralogy of Fallot, and the evaluation of the relationship between vascular rings and airways is more important. However, there are few articles on the prenatal diagnosis of vascular ring diseases and with qualitative or quantitative assessments of airway prognosis in PubMed. Based on this single-center sample, we believe that the shape of the vascular ring, the route of the branching vessels, and the distance from the airway are critical to the prognosis of these diseases. Dynamic sequential cross-sectional scanning can observe these aspects very well and intuitively, has more advantages than the long axis, and can carry out prenatal monitoring and management of the fetus until birth.
  • #33 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text
    https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y
    A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The different types of vascular rings have different incidences, clinical manifestations and severities. Symptoms frequently encountered include noisy breathing and a barky cough, recurrent upper respiratory infections, wheezing, exertional dyspnea, and dysphagia. Delaying treatment may result in sudden death or residual tracheobronchial damage. The rate of missed diagnoses and misdiagnoses remains high, in particular, for isolated vascular rings, prenatal data statistics are only 1 in 1000. The purpose of this study was to investigate the value of prenatal ultrasound in the integrated management of vascular ring. SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth. Early diagnosis of congenital vascular rings is intended to improve the symptoms of compression in patients with airway compression. With the increase of prenatal detection rate of vascular rings, the incidence of rare diseases has been realized to be twice that of tetralogy of Fallot, and the evaluation of the relationship between vascular rings and airways is more important. However, there are few articles on the prenatal diagnosis of vascular ring diseases and with qualitative or quantitative assessments of airway prognosis in PubMed. Based on this single-center sample, we believe that the shape of the vascular ring, the route of the branching vessels, and the distance from the airway are critical to the prognosis of these diseases. Dynamic sequential cross-sectional scanning can observe these aspects very well and intuitively, has more advantages than the long axis, and can carry out prenatal monitoring and management of the fetus until birth.
  • #34 32: Vascular Rings and Slings | Veterian Key
    https://veteriankey.com/32-vascular-rings-and-slings/
    A vascular ring is an anomaly of aortic arch development that results in complete encirclement of the trachea and the esophagus by vascular structures. […] Because some vascular rings may not cause symptoms, their true prevalence is difficult to ascertain. They are clearly a rare anomaly and most evidence indicates that they represent approximately 13% of congenital cardiovascular anomalies. […] The diagnosis of vascular rings requires a high index of suspicion because of the relative infrequency of this entity compared with other conditions that cause respiratory distress in children, such as asthma, respiratory infection, and gastroesophageal reflux.
  • #35 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    In the group of vascular rings and slings as a whole, respiratory symptoms predominate in the initial presentation. The severity of compression determines the severity of symptoms. About 70% to 90% of patients will have respiratory symptoms. Stridor is present in almost all cases. Stridor may be more pronounced during feeding or activity. Wheezing, air trapping and hyperinflation are also common. Infants may have recurrent pneumonias. […] The diagnosis of vascular rings is challenging. A high index of suspicion is necessary because of the relatively low incidence of these anomalies in contrast to the common symptoms the patients exhibit. Many of the patients will have a delay in diagnosis due to attribution of symptoms to other more common etiologies and the difficulty of establishing this diagnosis without an advanced imaging study such as CT, MRI, echocardiography, or esophagram. Most patients are diagnosed by age 12 months after numerous evaluations for respiratory symptoms.
  • #36 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    Very recently, Said and Biermann confirmed in two different papers the indication to treat surgically all complete VRs (DAA, RAA with LLA, LAA with RLA) and LPA sling, reserving surgery to symptomatic incomplete VRs or those without symptoms and associated cardiac defects. […] Most of the patients with complete VRs and LPA sling have respiratory symptoms that often occur early in life (1-6 months), becoming progressive with the growth of the intrathoracic structures. […] Symptoms vary from apnoea and cyanosis to stridor, barky cough, wheezing, shortness of breath and dysphagia especially for solid food. […] Because the described clinical picture is often misinterpreted as laryngomalacia, bronchiolitis, asthma, and gastroesophageal reflux, clinician should keep in mind the possibility of a VR in children with recurrent respiratory symptoms with or without digestive symptoms and poor response to medical treatment.
  • #37 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    In the group of vascular rings and slings as a whole, respiratory symptoms predominate in the initial presentation. The severity of compression determines the severity of symptoms. About 70% to 90% of patients will have respiratory symptoms. Stridor is present in almost all cases. Stridor may be more pronounced during feeding or activity. Wheezing, air trapping and hyperinflation are also common. Infants may have recurrent pneumonias. […] The diagnosis of vascular rings is challenging. A high index of suspicion is necessary because of the relatively low incidence of these anomalies in contrast to the common symptoms the patients exhibit. Many of the patients will have a delay in diagnosis due to attribution of symptoms to other more common etiologies and the difficulty of establishing this diagnosis without an advanced imaging study such as CT, MRI, echocardiography, or esophagram. Most patients are diagnosed by age 12 months after numerous evaluations for respiratory symptoms.
  • #38 Clinical Course of Vascular Rings and Risk Factors Associated With Mortality
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3341422/
    Overall prognosis for a vascular ring was relatively good in this study, though vascular rings combined with complex heart disease showed a poor prognosis. […] In conclusion, vascular rings include several types of anomalies, each with different symptoms and prognosis. The presence of complex heart disease was significantly associated with mortality. Surgical repair for a vascular ring has a relatively good prognosis and the operative risk in the absence of complex heart disease is low. Therefore, early diagnosis and timely surgery in symptomatic patients are paramount.
  • #39 Clinical Course of Vascular Rings and Risk Factors Associated With Mortality
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3341422/
    Overall prognosis for a vascular ring was relatively good in this study, though vascular rings combined with complex heart disease showed a poor prognosis. […] In conclusion, vascular rings include several types of anomalies, each with different symptoms and prognosis. The presence of complex heart disease was significantly associated with mortality. Surgical repair for a vascular ring has a relatively good prognosis and the operative risk in the absence of complex heart disease is low. Therefore, early diagnosis and timely surgery in symptomatic patients are paramount.
  • #40 Vascular Rings Program | Lurie Children’s
    https://www.luriechildrens.org/en/specialties-conditions/vascular-rings-program/
    Lurie Children’s Vascular Ring Program treats children with severe vascular rings, using innovative and proven surgical methods to relieve pressure on the trachea and esophagus. Our center has one of the largest published surgical experiences with vascular ring; currently, we have operated on more than 500 patients. […] The results of vascular ring surgery are excellent we have not had an operative mortality rate in the past 30 years and more than 90% of our patients see their symptoms resolve.
  • #41 Expertise in the nuances of diagnosis, management and surgery for vascular rings – Mayo Clinic
    https://www.mayoclinic.org/medical-professionals/pediatrics/news/expertise-in-the-nuances-of-diagnosis-management-and-surgery-for-vascular-rings/mac-20530282
    Mayo Clinic pediatric cardiac surgeons have conducted retrospective studies on pediatric and adult patients who have undergone surgical correction of vascular rings. „We have found that, in general, surgical repairs have excellent outcomes and minimize complications related to chronic airway and esophageal compression,” says Dr. Stephens.
  • #42 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    VR can be accurately diagnosed by fetal ultrasound improving prognosis in these patients, as it enables proper timing of additional postnatal investigation and surgery. […] Unfortunately, there are no guidelines on the better diagnostic work up for children suspected of VR. However, its reasonable that children with history of recurrent respiratory symptoms poor response to medical treatment, symptoms and physical examination of airway obstruction, dysphagia, and failure to thrive should undergo to flexible laryngo-tracheo-bronchoscopy to assess for laryngomalacia, vocal fold mobility, tracheal stenosis or malacia. […] Morbidity and mortality in patients after surgical correction have been reported to a greater extent, in those with respiratory distress at presentation, underlying genetic condition, associated congenital tracheal stenosis or severe bronchomalacia, tetralogy of Fallot with absent pulmonary valve syndrome, underlying complex cyanotic heart disease and need of early respiratory support.
  • #43 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    A detailed surgical algorithm for asymptomatic patients with a diagnosis of vascular ring was recently proposed by the Chicago group, one of the leading center on the subject. […] Despite surgical repair, 40% of those patients remained symptomatic at follow-up. […] In our cohort, patients with the most severe forms of vascular rings were not detected before birth. […] Asymptomatic or mildly symptomatic patients are a peculiar subset of patients for whom there is an ongoing debate on the optimal management: Should surgical repair be performed or not, and if surgery is advised, which type of ring repair particularly regarding the KD? […] Overall in-hospital morbidity was acceptable (18%) and consisted mostly in pleural effusions and pulmonary atelectasis. […] While 24% of patients remained symptomatic at long-term follow-up, half of those were asthma-related and medically controlled, resulting in nearly 90% of patients symptoms-free at mid-term follow-up. […] Finally, surgery in asymptomatic patients resulted in minimal hospital morbidity, supporting the current recommendation of early surgical repair after meticulous preoperative evaluation of associated trachea-bronchial anomalies.
  • #44 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    A detailed surgical algorithm for asymptomatic patients with a diagnosis of vascular ring was recently proposed by the Chicago group, one of the leading center on the subject. […] Despite surgical repair, 40% of those patients remained symptomatic at follow-up. […] In our cohort, patients with the most severe forms of vascular rings were not detected before birth. […] Asymptomatic or mildly symptomatic patients are a peculiar subset of patients for whom there is an ongoing debate on the optimal management: Should surgical repair be performed or not, and if surgery is advised, which type of ring repair particularly regarding the KD? […] Overall in-hospital morbidity was acceptable (18%) and consisted mostly in pleural effusions and pulmonary atelectasis. […] While 24% of patients remained symptomatic at long-term follow-up, half of those were asthma-related and medically controlled, resulting in nearly 90% of patients symptoms-free at mid-term follow-up. […] Finally, surgery in asymptomatic patients resulted in minimal hospital morbidity, supporting the current recommendation of early surgical repair after meticulous preoperative evaluation of associated trachea-bronchial anomalies.
  • #45 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    Vascular rings represent 1% of congenital cardiovascular abnormalities. […] While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. […] Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair. […] With the increased rate of fetal diagnosis of vascular ring, the optimal management in asymptomatic or mildly symptomatic neonates and infants is controversial. […] In a recent UK survey, 20% of the responders advised surgical repair in asymptomatic patients.
  • #46 Management of prenatally detected vascular rings: a United Kingdom national survey | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/management-of-prenatally-detected-vascular-rings-a-united-kingdom-national-survey/B443DB896A4EABF160432F25F9A7884B
    To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA). […] Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies. […] There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention. […] This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.
  • #47 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    Vascular rings represent 1% of congenital cardiovascular abnormalities. […] While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. […] Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair. […] With the increased rate of fetal diagnosis of vascular ring, the optimal management in asymptomatic or mildly symptomatic neonates and infants is controversial. […] In a recent UK survey, 20% of the responders advised surgical repair in asymptomatic patients.
  • #48 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    A detailed surgical algorithm for asymptomatic patients with a diagnosis of vascular ring was recently proposed by the Chicago group, one of the leading center on the subject. […] Despite surgical repair, 40% of those patients remained symptomatic at follow-up. […] In our cohort, patients with the most severe forms of vascular rings were not detected before birth. […] Asymptomatic or mildly symptomatic patients are a peculiar subset of patients for whom there is an ongoing debate on the optimal management: Should surgical repair be performed or not, and if surgery is advised, which type of ring repair particularly regarding the KD? […] Overall in-hospital morbidity was acceptable (18%) and consisted mostly in pleural effusions and pulmonary atelectasis. […] While 24% of patients remained symptomatic at long-term follow-up, half of those were asthma-related and medically controlled, resulting in nearly 90% of patients symptoms-free at mid-term follow-up. […] Finally, surgery in asymptomatic patients resulted in minimal hospital morbidity, supporting the current recommendation of early surgical repair after meticulous preoperative evaluation of associated trachea-bronchial anomalies.
  • #49 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    A detailed surgical algorithm for asymptomatic patients with a diagnosis of vascular ring was recently proposed by the Chicago group, one of the leading center on the subject. […] Despite surgical repair, 40% of those patients remained symptomatic at follow-up. […] In our cohort, patients with the most severe forms of vascular rings were not detected before birth. […] Asymptomatic or mildly symptomatic patients are a peculiar subset of patients for whom there is an ongoing debate on the optimal management: Should surgical repair be performed or not, and if surgery is advised, which type of ring repair particularly regarding the KD? […] Overall in-hospital morbidity was acceptable (18%) and consisted mostly in pleural effusions and pulmonary atelectasis. […] While 24% of patients remained symptomatic at long-term follow-up, half of those were asthma-related and medically controlled, resulting in nearly 90% of patients symptoms-free at mid-term follow-up. […] Finally, surgery in asymptomatic patients resulted in minimal hospital morbidity, supporting the current recommendation of early surgical repair after meticulous preoperative evaluation of associated trachea-bronchial anomalies.
  • #50 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. […] Vascular rings (VRs) account for 1% of congenital cardiac defects. Their real incidence is difficult to establish as the clinical presentations are non-specific and range from early onset of symptoms to asymptomatic clinical setting into adulthood. […] Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients. […] The mere presence of a vascular ring is not an indication in itself for surgical intervention. It is important to know that symptoms in small children may disappear as the child and chest cage grow. It is generally accepted, however, that surgical intervention should be undertaken if the patient is symptomatic, and or in presence of significant compromise to the airways.
  • #51 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    Very recently, Said and Biermann confirmed in two different papers the indication to treat surgically all complete VRs (DAA, RAA with LLA, LAA with RLA) and LPA sling, reserving surgery to symptomatic incomplete VRs or those without symptoms and associated cardiac defects. […] Most of the patients with complete VRs and LPA sling have respiratory symptoms that often occur early in life (1-6 months), becoming progressive with the growth of the intrathoracic structures. […] Symptoms vary from apnoea and cyanosis to stridor, barky cough, wheezing, shortness of breath and dysphagia especially for solid food. […] Because the described clinical picture is often misinterpreted as laryngomalacia, bronchiolitis, asthma, and gastroesophageal reflux, clinician should keep in mind the possibility of a VR in children with recurrent respiratory symptoms with or without digestive symptoms and poor response to medical treatment.
  • #52 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text
    https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3
    Vascular rings are congenital abnormalities of the aortic arch-derived vascular and ligamentous structures, which encircle the trachea and oesophagus to varying degrees, resulting in respiratory or feeding difficulties in children. […] Imaging plays an important role in the evaluation and management of vascular rings. […] Vascular rings can be identified by several imaging examinations and occasionally multiple imaging tests may be required to make a diagnosis. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] Vascular rings are complex and diagnosis is often challenging because of variable and non-specific clinical presentations. CT angiography plays an important role in the identification and definition of the anatomy of these complex anomalies, thus providing a roadmap to surgeons.
  • #53 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. […] Vascular rings (VRs) account for 1% of congenital cardiac defects. Their real incidence is difficult to establish as the clinical presentations are non-specific and range from early onset of symptoms to asymptomatic clinical setting into adulthood. […] Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients. […] The mere presence of a vascular ring is not an indication in itself for surgical intervention. It is important to know that symptoms in small children may disappear as the child and chest cage grow. It is generally accepted, however, that surgical intervention should be undertaken if the patient is symptomatic, and or in presence of significant compromise to the airways.
  • #54 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x
    Very recently, Said and Biermann confirmed in two different papers the indication to treat surgically all complete VRs (DAA, RAA with LLA, LAA with RLA) and LPA sling, reserving surgery to symptomatic incomplete VRs or those without symptoms and associated cardiac defects. […] Most of the patients with complete VRs and LPA sling have respiratory symptoms that often occur early in life (1-6 months), becoming progressive with the growth of the intrathoracic structures. […] Symptoms vary from apnoea and cyanosis to stridor, barky cough, wheezing, shortness of breath and dysphagia especially for solid food. […] Because the described clinical picture is often misinterpreted as laryngomalacia, bronchiolitis, asthma, and gastroesophageal reflux, clinician should keep in mind the possibility of a VR in children with recurrent respiratory symptoms with or without digestive symptoms and poor response to medical treatment.
  • #55 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics
    https://tp.amegroups.org/article/view/114864/html
    Vascular rings and vascular-related aerodigestive compression syndromes encompass a variety of lesions that involve abnormal development of the aortic arch with resultant vascular compression of either the trachea, the esophagus, or both. True, or complete, vascular rings include double aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum and have a prevalence of approximately 11.3 per 10,000 live births. […] Despite the substantial variability in etiologies and presentations, a multidisciplinary approach to diagnostic evaluation has been standardized at Boston Childrens Hospital. […] The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result. […] The principle mechanisms of failure can be broadly categorized into ongoing vascular compression, development of constricting scar tissue reforming the vascular ring, tracheobronchomalacia, or a combination. […] Ongoing vascular compression may be related to an aberrant subclavian artery if the prior operation was merely division of the ligamentum arteriosum to relieve the vascular ring.
  • #56 Management of prenatally detected vascular rings: a United Kingdom national survey | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/management-of-prenatally-detected-vascular-rings-a-united-kingdom-national-survey/B443DB896A4EABF160432F25F9A7884B
    To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA). […] Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies. […] There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention. […] This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.