Podwójne ujście prawej komory

Podwójne ujście prawej komory
Epidemiologia

Podwójne ujście prawej komory (DORV) to rzadka wada wrodzona serca, stanowiąca 1-3% wszystkich wrodzonych wad serca, z częstością występowania szacowaną na 0,09-0,14 na 1000 żywych urodzeń (0,9-1,4 na 10 000 urodzeń). Charakteryzuje się całkowitym lub przeważającym odprowadzeniem aorty i tętnicy płucnej z prawej komory. Występuje w różnych wariantach anatomicznych, najczęściej z ubytkiem przegrody międzykomorowej podzastawkowym aortalnym (subaortic VSD) i zwężeniem podzastawkowym płucnym. DORV często współistnieje z innymi wadami serca (np. ubytek przegrody przedsionkowo-komorowej, koarktacja aorty) oraz anomaliami pozasercowymi, a w około 40% przypadków towarzyszą mu aberracje chromosomalne, takie jak trisomie 13, 18, 21 oraz delecja 22q11.2 (zespół DiGeorge’a). Diagnostyka prenatalna opiera się na ultrasonografii i echokardiografii płodowej, co umożliwia wczesne rozpoznanie i planowanie leczenia.

Epidemiologia podwójnego ujścia prawej komory (DORV)

Częstość występowania i rozpowszechnienie
Charakterystyka demograficzna
Czynniki genetyczne i związane z nimi anomalie
Diagnostyka prenatalna i nadzór
Anomalie współistniejące

Warianty anatomiczne DORV i ich częstość występowania

Układy anatomiczne

Implikacje kliniczne i nadzór medyczny

Postępowanie kliniczne
Nadzór i dalsze leczenie
Kolejne rozdziały

Epidemiologia podwójnego ujścia prawej komory (DORV)

Podwójne ujście prawej komory (ang. Double Outlet Right Ventricle, DORV) jest stosunkowo rzadką wrodzoną wadą serca, charakteryzującą się nieprawidłowym połączeniem naczyniowym, w którym zarówno aorta jak i tętnica płucna odchodzą całkowicie lub w przeważającej części z prawej komory serca. Stanowi to około 1-3% wszystkich wrodzonych wad serca.¹²³

Częstość występowania i rozpowszechnienie

Na podstawie danych epidemiologicznych, częstość występowania DORV szacuje się na 0,09-0,14 przypadku na 1000 żywych urodzeń.¹² W przeliczeniu na 10 000 urodzeń, daje to około 0,9-1,4 przypadku. Norweski kompleksowy, populacyjny, retrospektywny projekt badawczy wykazał roczną medianę prewalencji DORV na poziomie 1,18 na 10 000 urodzeń.³ Inne źródła podają, że roczna zapadalność odpowiada 1,07 przypadku na 100 000 żywych urodzeń.⁴

Alternatywne dane wskazują na częstość występowania od 1 przypadku na 6 000 do 1 przypadku na 10 000 żywych urodzeń.⁵⁶ Badania przeprowadzone w Stanach Zjednoczonych oszacowały częstość występowania DORV na poziomie 0,09 przypadku na 1000 żywych urodzeń.⁷ Natomiast dane z Teksasu sugerują częstość na poziomie 6-15 przypadków na 100 000 żywych urodzeń.⁸

Charakterystyka demograficzna

Większość źródeł nie wskazuje na predylekcję płciową w przypadku DORV.⁹¹⁰ Jednak niektóre dane sugerują, że DORV może występować częściej u chłopców niż u dziewczynek.¹¹ Nie zaobserwowano żadnej predylekcji rasowej dla tej wady.¹²

Objawy DORV zazwyczaj pojawiają się w okresie noworodkowym, jednakże w niektórych przypadkach (np. przy ubytku przegrody międzykomorowej podzastawkowym i łagodnym lub umiarkowanym zwężeniem zastawki płucnej) diagnoza może zostać postawiona później w okresie niemowlęcym.¹³

Czynniki genetyczne i związane z nimi anomalie

DORV może występować jako izolowana wada lub w skojarzeniu z innymi anomaliami sercowymi i pozasercowymi.¹⁴ Większość przypadków DORV występuje sporadycznie, bez znanej przyczyny, jednak częściej może pojawić się w powiązaniu z pewnymi zespołami genetycznymi.¹⁵¹⁶

Anomalie chromosomalne stwierdzono w około 40% przypadków DORV opisanych w literaturze medycznej.¹⁷ Wśród specyficznych aberracji chromosomalnych związanych z DORV wymienia się:¹⁸¹⁹²⁰

²¹²²

Anomalie chromosomalne występują u 10-20% płodów z DORV, przy czym trisomie 18 i 13 są najczęstsze.²³ Z tego powodu zalecane jest poradnictwo i badania genetyczne dla wszystkich kobiet w ciąży, u których płód ma zdiagnozowane DORV.²⁴

Diagnostyka prenatalna i nadzór

DORV można wykryć przed urodzeniem podczas rutynowego badania ultrasonograficznego płodu.²⁵²⁶ W przypadku podejrzenia wady, pacjentka jest kierowana do kardiologa dziecięcego, który przeprowadza płodową echokardiografię (ultrasonografię serca) w celu lepszej oceny struktury serca i przepływu krwi u płodu.²⁷

DORV stanowi 4-8% prenatalnie diagnozowanych wrodzonych wad serca. Wysoki związek DORV z wadami serca, anomaliami pozasercowymi i aberracjami chromosomalnymi odpowiada za niższą częstość występowania po urodzeniu (1-3%).²⁸

Anomalie współistniejące

Inne często współistniejące anomalie sercowe to:²⁹

Ubytek przegrody przedsionkowo-komorowej
Zespoły kardiosplenicznej
Koarktacja aorty
Atrezja zastawki mitralnej
Zastawki przedsionkowo-komorowe okrakowujące lub nakładające się z następczą hipoplazją leżącej poniżej komory

Anomalie pozasercowe są obecne w dwóch trzecich przypadków, szczególnie anomalie przewodu pokarmowego i ośrodkowego układu nerwowego.³⁰

Warianty anatomiczne DORV i ich częstość występowania

DORV to heterogeniczna grupa nieprawidłowych połączeń komorowo-tętniczych, gdzie obie wielkie tętnice (tętnica płucna i aorta) odchodzą głównie z morfologicznie prawej komory. Istnieje kilka wariantów anatomicznych DORV, różniących się położeniem ubytku przegrody międzykomorowej (VSD) oraz wzajemnym ułożeniem wielkich naczyń.¹

Układy anatomiczne

Najczęściej obserwowanym układem anatomicznym w DORV jest ten z prawidłowo ułożonymi wielkimi naczyniami, tj. z aortą położoną do tyłu i po prawej stronie, z ubytkiem przegrody międzykomorowej podzastawkowym aortalnym (subaortic VSD) i zwężeniem podzastawkowym płucnym. W kolejności od najwyższej do najniższej częstości występowania, następnie występuje:

Ubytek przegrody międzykomorowej podzastawkowy aortalny bez zwężenia płucnego
Ubytek przegrody międzykomorowej z podwójnym połączeniem (doubly-committed VSD) lub bez połączenia (non-committed VSD)
Niezwykle rzadki ubytek przegrody międzykomorowej podzastawkowy płucny (subpulmonary VSD)

W jednej z największych serii przypadków DORV opisanej przez Aoki i współpracowników, w ciągu 10 lat 73 pacjentów przeszło operację serca z powodu różnych typów DORV. U większości pacjentów aorta i pień płucny znajdowały się bok w bok, u 18 (25%) wykazano aortę tylną i prawostronną, u 12 (17%) wykazano aortę przednią i prawostronną, u 5 (7%) wykazano aortę przednią i lewostronną, a tylko u 2 (2,8%) wykazano aortę przednią.³

Anderson i współpracownicy zwrócili uwagę na rzadkość przedniej lewostronnej aorty i donieśli, że w tym typie układu, podzastawkowy płucny ubytek przegrody międzykomorowej (subpulmonary VSD) jest jeszcze rzadszy (najczęściej spotykany jest podzastawkowy aortalny VSD). W serii Aoki i współpracowników tylko 2 (2,8%) z 5 pacjentów z przednią lewostronną aortą miało podzastawkowy płucny VSD.⁴

Implikacje kliniczne i nadzór medyczny

DORV stanowi poważne wyzwanie dla kardiochirurgów ze względu na brak standardowego podejścia chirurgicznego.¹ Leczenie i postępowanie kliniczne w DORV zależy przede wszystkim od jego typu (tj. VSD, Fallot, przełożenie wielkich pni tętniczych (TGA), niepołączony (odległy) VSD), a także od obecności towarzyszących anomalii sercowych.²

Postępowanie kliniczne

Wszystkie dzieci urodzone z DORV wymagają dożywotniego nadzoru przez kardiologa specjalizującego się w leczeniu wrodzonych wad serca.³⁴ Większość dzieci z DORV potrzebuje operacji naprawczej w ciągu kilku miesięcy od urodzenia.⁵ Może być wykonany więcej niż jeden rodzaj operacji, a typ zabiegu zależy od specyficznej struktury serca.⁶

Bez operacji u dziecka z DORV ostatecznie rozwinie się:⁷

Sinica
Niewydolność serca
Nadciśnienie płucne (wysokie ciśnienie krwi w płucach)

Po operacji, większość dzieci z DORV dożywa wieku dorosłego. Każdy, kto przeszedł operację DORV, wymaga dożywotniej opieki ze strony kardiologa lub lekarza specjalizującego się w opiece nad sercem.⁸

Nadzór i dalsze leczenie

W Norwegii większość dzieci otrzymuje zindywidualizowane leczenie DORV z niskim odsetkiem powikłań, reoperacji chirurgicznych i śmiertelności. Jednak ubytek przegrody przedsionkowo-komorowej pozostaje potencjalnym czynnikiem determinującym śmierć pooperacyjną.⁹

Śmiertelność wśród leczonych dzieci wydaje się ustabilizować na niskim poziomie w ostatnich kilku dekadach, a poważne powikłania są rzadkie.¹⁰ Wyniki leczenia prawostronnych jednokomorowych wad serca są dobre, a przez ten czas obserwuje się poprawę skuteczności leczenia.¹¹

Po operacji pacjenci mogą doświadczyć różnych komplikacji, które wymagają nadzoru, takich jak:¹²

Zwężenie podzastawkowe aortalne
Zwężenie podzastawkowe płucne
Niedomykalność zastawki przedsionkowo-komorowej
Niewydolność przewodu (ze zwężeniem i/lub niedomykalnością)
Niedomykalność neoaortalna (po procedurach przełożenia tętniczego)
Koarktacja lub rekoarktacja aorty
Mała lub dysfunkcyjna prawa komora (może wiązać się ze złożoną naprawą wewnątrzsercową)
Problemy z rytmem serca, takie jak blok serca, arytmia przedsionkowa i arytmia komorowa
Nagła śmierć
Infekcyjne zapalenie wsierdzia
Zjawiska zakrzepowo-zatorowe

Wczesne rozpoznanie i leczenie podwójnego ujścia prawej komory może zapobiec pogorszeniu stanu dziecka.¹³ Chociaż dzieci mają dobry wskaźnik przeżycia po operacji, nadal będą miały tylko połowę sprawnego serca.¹⁴

Wiele dzieci z DORV może prowadzić pełne i aktywne życie, ale wymagają dożywotniego dalszego leczenia.¹⁵¹⁶

Kolejne rozdziały

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Materiały źródłowe

#1 Orphanet: Double outlet right ventricle
https://www.orpha.net/en/disease/detail/3426
Double outlet right ventricle (DORV) accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000. […] All patients that have received surgery for a DORV require life-long surveillance by a cardiologist.
#1 Double-Outlet Right Ventricle | Thoracic Key
https://thoracickey.com/double-outlet-right-ventricle-6/
DORV may exist as an isolated condition or in association with cardiac or extracardiac anomalies. The reported incidence ranges from 0.03 to 0.14 per 1000 live births. It occurs in about 1% of all congenital heart disease. […] Several chromosomal abnormalities have been associated with DORV, including trisomy 13, trisomy 18, and chromosome 22q11 deletion.
#1 SciELO Brazil – Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect
https://www.scielo.br/j/abc/a/CWx3S7yhgjDsNxd3CNtjZTJ/
Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. […] The most prevalent anatomic arrangement observed in DORV is that of normally committed great arteries, i.e., posterior and right-sided aorta, with subaortic VSD and subpulmonary stenosis. Then, from the highest to the lowest frequency, it is followed by subaortic VSD without pulmonary obstruction, doubly-committed or non-committed VSD, and the extremely rare subpulmonary VSD. […] Aoki et al reported one of the largest case series of DORV. In 10 years, 73 patients underwent heart surgery for correction of different types of DORV. A large majority of patients, the aorta and the pulmonary trunk were side by side, 18 (25%) showed a posterior and right-sided aorta, 12 (17%) showed an anterior and right-sided aorta, 5 (7%) showed an anterior and left-sided aorta, and only 2 (2.8%) showed an anterior aorta.
#1 Double-Outlet Right Ventricle | SpringerLink
https://link.springer.com/book/10.1007/978-3-031-49707-0
Double-outlet right ventricle (DORV) represents a great challenge for cardiac surgeons due to the absence of standard surgical approach and this book is the first complete manual detailing decision-making in all anatomical types of DORV: subaortic, subpulmonary, subarterial and noncommitted defects. […] It provides information regarding the molecular mechanisms and clinical consequences of the most important syndromes in patients with cyanotic congenital heart defects, arterial hypoxemia and myocardial remodeling. […] The book is therefore of importance to all cardiac surgeons and healthcare professionals working with this group of patients.
#2 Double outlet right ventricle – Wikipedia
https://en.wikipedia.org/wiki/Double_outlet_right_ventricle
DORV affects between 1% and 3% of people born with congenital heart defects. […] Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.
#2 Double outlet right ventricle | PPT
https://www.slideshare.net/slideshow/double-outlet-right-ventricle/42632465
Double outlet right ventricle (DORV) is a congenital heart defect where both the aorta and pulmonary artery arise completely or predominantly from the right ventricle. […] Epidemiology Isolated /With extracardiac anomalies Incidence:0.03 to 0.14 per 1000 live births 1% of all CHD Association: coarctation, aortic arch hypoplasia, or interrupted aortic archparticularly at the transposition end of the spectrum, right atrial isomerism Chromosomal abnormalities :Trisomy 13,trisomy 18, 22q11 deletion. […] Decision making for Rx Diagnosis is DORV Differentiate from TOF Location of the VSD Disposition of great arteries PS or no PS Size of VSD […] DORV with Transposition-like Physiology and Pulmonary Stenosis Rastelli repair REV procedure The REV procedure (rparation ltage ventriculaire) entails division of the main PA with extensive mobilization,translocation of the PA anterior to aorta (Lecompte maneuver),and direct connection of the PA to the RV, thus eliminating the use of prosthetic materials.
#2 SciELO Brazil – Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect
https://www.scielo.br/j/abc/a/CWx3S7yhgjDsNxd3CNtjZTJ/
Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. […] The most prevalent anatomic arrangement observed in DORV is that of normally committed great arteries, i.e., posterior and right-sided aorta, with subaortic VSD and subpulmonary stenosis. Then, from the highest to the lowest frequency, it is followed by subaortic VSD without pulmonary obstruction, doubly-committed or non-committed VSD, and the extremely rare subpulmonary VSD. […] Aoki et al reported one of the largest case series of DORV. In 10 years, 73 patients underwent heart surgery for correction of different types of DORV. A large majority of patients, the aorta and the pulmonary trunk were side by side, 18 (25%) showed a posterior and right-sided aorta, 12 (17%) showed an anterior and right-sided aorta, 5 (7%) showed an anterior and left-sided aorta, and only 2 (2.8%) showed an anterior aorta.
#2 Double Outlet Right Ventricle Surgery: Background, Problem, Relevant Anatomy
https://emedicine.medscape.com/article/904397-overview
In the United States, the incidence of double outlet right ventricle is an estimated 0.09 cases per 1000 live births. Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease. […] The clinical presentation and management of double outlet right ventricle (DORV) is primarily dependent on its type (ie, ventricular septal defect [VSD], Fallot, transposition of great arteries [TGA], noncommitted [remote] VSD), as well as the presence of associated cardiac anomalies. […] Recognition and identification of clinically significant cardiac anomalies might first be based on a complete history of the patient’s condition and its progression from parents. Elucidate feeding tolerance, weight gain, breathing problems, and a general failure to thrive.
#3 Double outlet right ventricle | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/double-outlet-right-ventricle?embed_domain=hackmd.io%25252F%252540yIPUAFeCSL2JsU8smR5nJQ%25252Fbnjhjgjghjghjgh
Double outlet right ventricle (DORV) is a congenital cardiac anomaly where both the aorta and pulmonary trunk arise from the morphologically right ventricle. It is reported to account for ~2% of congenital cardiac defects. The estimated incidence is at ~1:10,000 births. […] Most cases are thought to have a sporadic occurrence.
#3 Current outcomes of live-born children with double outlet right ventricle in Norway
https://pmc.ncbi.nlm.nih.gov/articles/PMC9762987/
This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). […] Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. […] In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. […] We found an annual incidence of DORV corresponding to 1.07 per 100.000 live births in Norway and found that the total mortality, including death following palliative care, was significant. However, most children with DORV can be offered tailored treatment encompassing primary BiV, staged BiV or UniV repair with favourable outcomes. The mortality rate among treated children seems to have stabilized at a low level in the last few decades, and severe complications are rare. The presence of AVSD remains an important determinant of increased risk of postoperative death.
#3 SciELO Brazil – Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect
https://www.scielo.br/j/abc/a/CWx3S7yhgjDsNxd3CNtjZTJ/
Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. […] The most prevalent anatomic arrangement observed in DORV is that of normally committed great arteries, i.e., posterior and right-sided aorta, with subaortic VSD and subpulmonary stenosis. Then, from the highest to the lowest frequency, it is followed by subaortic VSD without pulmonary obstruction, doubly-committed or non-committed VSD, and the extremely rare subpulmonary VSD. […] Aoki et al reported one of the largest case series of DORV. In 10 years, 73 patients underwent heart surgery for correction of different types of DORV. A large majority of patients, the aorta and the pulmonary trunk were side by side, 18 (25%) showed a posterior and right-sided aorta, 12 (17%) showed an anterior and right-sided aorta, 5 (7%) showed an anterior and left-sided aorta, and only 2 (2.8%) showed an anterior aorta.
#3 Orphanet: Double outlet right ventricle
https://www.orpha.net/en/disease/detail/3426
Double outlet right ventricle (DORV) accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000. […] All patients that have received surgery for a DORV require life-long surveillance by a cardiologist.
#4 Current outcomes of live-born children with double outlet right ventricle in Norway
https://pmc.ncbi.nlm.nih.gov/articles/PMC9762987/
This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). […] Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. […] In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. […] We found an annual incidence of DORV corresponding to 1.07 per 100.000 live births in Norway and found that the total mortality, including death following palliative care, was significant. However, most children with DORV can be offered tailored treatment encompassing primary BiV, staged BiV or UniV repair with favourable outcomes. The mortality rate among treated children seems to have stabilized at a low level in the last few decades, and severe complications are rare. The presence of AVSD remains an important determinant of increased risk of postoperative death.
#4 SciELO Brazil – Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect
https://www.scielo.br/j/abc/a/CWx3S7yhgjDsNxd3CNtjZTJ/
Anderson et al called attention to the rarity of the anterior left-sided aorta and reported that, in this type of arrangement, the subpulmonary VSD is even more uncommon (subaortic VSD is the most frequently encountered). In the series of Aoki et al only 2 (2.8%) out of the 5 patients with anterior left-sided aorta had subpulmonary VSD. […] This case report is important due to the rarity of this anatomical type of DORV. The cases of DORV with anterior and left-sided aorta and subpulmonary VSD described in the report of Aoki are the only two cases reported in the international medical literature about this condition. We believe that this is the first case reported in the national literature of this rare type of DORV.
#4 Double-Outlet Right Ventricle – Seattle Children’s Hospital
https://www.seattlechildrens.org/conditions/double-outlet-right-ventricle/
Double-outlet right ventricle is a birth defect of the heart. […] Doctors consider this a rare heart defect. About 1 in every 10,000 babies has double-outlet right ventricle. […] All children with double-outlet right ventricle will need lifelong follow-up with a cardiologist who specializes in congenital heart defects.
#5 Double Outlet Right Ventricle (DORV)
https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle
Double outlet right ventricle (DORV) describes a heart with two major arteries linking to its right ventricle (heart chamber). […] Surgery repairs the problem, but children born with DORV need lifelong follow-up care. […] DORV is rare. It happens just once for every 6,000 to 10,000 newborns. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis, Heart failure, Pulmonary hypertension (high blood pressure in the lungs). […] With surgery, most babies who have double outlet right ventricle live to be adults. Anyone whos had surgery for DORV needs lifelong care from a cardiologist or provider who specializes in taking care of your heart.
#5 Double-outlet right ventricle – Overview – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537
Double-outlet right ventricle is a heart condition present at birth. That means it’s a congenital heart defect. In this condition, the lower right heart chamber has two openings for blood to exit the heart instead of one. Some of the blood from the lower right heart chamber goes to the body instead of the lungs. […] Double-outlet right ventricle may occur with other heart conditions present at birth such as other holes in the heart and blood vessel or heart valve changes. […] Having a family history of congenital heart conditions may increase the risk of congenital heart defects such as double-outlet right ventricle. […] A test called an echocardiogram can diagnose double-outlet right ventricle. The test uses sound waves to create images of the beating heart. It can show blood flow through the heart and heart valves. […] Most babies with double-outlet right ventricle have heart repair surgery within a few months of age. More than one type of surgery may be done. The type of surgery depends on the specific heart structure.
#6 Double Outlet Right Ventricle (DORV) | Little Hearts Matter
https://www.lhm.org.uk/double-outlet-right-ventricle/
Double Outlet Right Ventricle (DORV) is a congenital heart condition (a problem that a baby is born with). […] Heart conditions that affect the blood flow to the lungs occur in 1 in 6,000 babies. […] As yet the causes of single ventricle heart conditions are unknown. […] Many families who have a child with congenital heart disease ask why their child was born with the condition. […] Some congenital heart conditions are linked to a genetic disorder such as Edwards syndrome or 22q Deletion. […] Congenital heart conditions occur in 1 in 130 pregnancies. […] Most but not all congenital heart centres around the country offer surgery for these conditions. […] The outcomes of treatment for right-sided single ventricle conditions are good. […] Over that time we have seen the success of treatment improve. […] Although children have a good survival rate following surgery they will still only have half a working heart.
#6 Double Outlet Right Ventricle | Cleveland Clinic Childrenâs
https://my.clevelandclinic.org/pediatrics/services/double-outlet-right-ventricle-treatment
It can be a big shock to learn your baby has a serious heart problem. […] Double outlet right ventricle (DORV), is a congenital (born with it) condition that can be repaired early with surgery usually within your childs first year. […] Finding and treating your babys double outlet right ventricle as early as possible helps your child thrive as they grow. […] To diagnose double outlet right ventricle, we start by listening to your childs heart with a stethoscope. […] DORV is different for each child. […] Most children with DORV need surgery. […] Getting an early diagnosis and treatment for double outlet right ventricle can prevent their condition from getting worse.
#7 Double Outlet Right Ventricle Surgery: Background, Problem, Relevant Anatomy
https://emedicine.medscape.com/article/904397-overview
In the United States, the incidence of double outlet right ventricle is an estimated 0.09 cases per 1000 live births. Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease. […] The clinical presentation and management of double outlet right ventricle (DORV) is primarily dependent on its type (ie, ventricular septal defect [VSD], Fallot, transposition of great arteries [TGA], noncommitted [remote] VSD), as well as the presence of associated cardiac anomalies. […] Recognition and identification of clinically significant cardiac anomalies might first be based on a complete history of the patient’s condition and its progression from parents. Elucidate feeding tolerance, weight gain, breathing problems, and a general failure to thrive.
#7 Double Outlet Right Ventricle (DORV)
https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle
Double outlet right ventricle (DORV) describes a heart with two major arteries linking to its right ventricle (heart chamber). […] Surgery repairs the problem, but children born with DORV need lifelong follow-up care. […] DORV is rare. It happens just once for every 6,000 to 10,000 newborns. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis, Heart failure, Pulmonary hypertension (high blood pressure in the lungs). […] With surgery, most babies who have double outlet right ventricle live to be adults. Anyone whos had surgery for DORV needs lifelong care from a cardiologist or provider who specializes in taking care of your heart.
#8 Double Outlet Right Ventricle (DORV) | Texas Children’s
https://www.texaschildrens.org/content/conditions/double-outlet-right-ventricle-dorv
Double outlet right ventricle (DORV) is a congenital heart defect, meaning it is present at birth. It occurs when the heart doesnât form properly during fetal development. In most cases, the cause is unknown. […] DORV occurs in an estimated 6 to 15 infants per 100,000 live births. It is more common in males. […] Genetic counseling and testing is recommended for all women pregnant with a baby with DORV. […] Double outlet right ventricle may be detected before birth during a routine prenatal ultrasound. Your OB/GYN will refer you to a pediatric cardiologist, who will perform a fetal echocardiogram (cardiac ultrasound) to better view and evaluate your babyâs heart structure and blood flow. […] Babies born with DORV will need to continue to see a heart specialist throughout their lives, transitioning at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects.
#8 Double Outlet Right Ventricle (DORV)
https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle
Double outlet right ventricle (DORV) describes a heart with two major arteries linking to its right ventricle (heart chamber). […] Surgery repairs the problem, but children born with DORV need lifelong follow-up care. […] DORV is rare. It happens just once for every 6,000 to 10,000 newborns. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis, Heart failure, Pulmonary hypertension (high blood pressure in the lungs). […] With surgery, most babies who have double outlet right ventricle live to be adults. Anyone whos had surgery for DORV needs lifelong care from a cardiologist or provider who specializes in taking care of your heart.
#9 Double Outlet Right Ventricle With Transposition: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/896082-overview
Double outlet right ventricle (DORV) is present in 0.5-1.5% of all patients with congenital heart disease (CHD). The estimated frequency of DORV is 1 case per 10,000 live births. […] A recent study showed a higher prevalence of DORV, tetralogy of Fallot, and truncus arteriosus, in addition to endocardial cushion defects and single ventricle, in certain regions of the country. […] No sex predilection is reported. […] The presentation is usually in the newborn period with this entity; however, in some circumstances (eg, subaortic VSD with mild-to-moderate PS), the diagnosis may not be made until later in infancy.
#9 Current outcomes of live-born children with double outlet right ventricle in Norway
https://pmc.ncbi.nlm.nih.gov/articles/PMC9762987/
This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). […] Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. […] In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. […] We found an annual incidence of DORV corresponding to 1.07 per 100.000 live births in Norway and found that the total mortality, including death following palliative care, was significant. However, most children with DORV can be offered tailored treatment encompassing primary BiV, staged BiV or UniV repair with favourable outcomes. The mortality rate among treated children seems to have stabilized at a low level in the last few decades, and severe complications are rare. The presence of AVSD remains an important determinant of increased risk of postoperative death.
#10 Double outlet right ventricle – wikidoc
https://www.wikidoc.org/index.php/Double_outlet_right_ventricle
Double Outlet of the Right Ventricle (DORV) accounts for 1-1.5% of all congenital heart diseases. […] It has an incidence of 1 in 10,000 live births. […] Generally, it is diagnosed during the first month of life, and there is no sex or race predilection.
#10 Current outcomes of live-born children with double outlet right ventricle in Norway
https://pmc.ncbi.nlm.nih.gov/articles/PMC9762987/
This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). […] Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. […] In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. […] We found an annual incidence of DORV corresponding to 1.07 per 100.000 live births in Norway and found that the total mortality, including death following palliative care, was significant. However, most children with DORV can be offered tailored treatment encompassing primary BiV, staged BiV or UniV repair with favourable outcomes. The mortality rate among treated children seems to have stabilized at a low level in the last few decades, and severe complications are rare. The presence of AVSD remains an important determinant of increased risk of postoperative death.
#11 Double Outlet Right Ventricle (DORV) | Texas Children’s
https://www.texaschildrens.org/content/conditions/double-outlet-right-ventricle-dorv
Double outlet right ventricle (DORV) is a congenital heart defect, meaning it is present at birth. It occurs when the heart doesnât form properly during fetal development. In most cases, the cause is unknown. […] DORV occurs in an estimated 6 to 15 infants per 100,000 live births. It is more common in males. […] Genetic counseling and testing is recommended for all women pregnant with a baby with DORV. […] Double outlet right ventricle may be detected before birth during a routine prenatal ultrasound. Your OB/GYN will refer you to a pediatric cardiologist, who will perform a fetal echocardiogram (cardiac ultrasound) to better view and evaluate your babyâs heart structure and blood flow. […] Babies born with DORV will need to continue to see a heart specialist throughout their lives, transitioning at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects.
#11 Double Outlet Right Ventricle (DORV) | Little Hearts Matter
https://www.lhm.org.uk/double-outlet-right-ventricle/
Double Outlet Right Ventricle (DORV) is a congenital heart condition (a problem that a baby is born with). […] Heart conditions that affect the blood flow to the lungs occur in 1 in 6,000 babies. […] As yet the causes of single ventricle heart conditions are unknown. […] Many families who have a child with congenital heart disease ask why their child was born with the condition. […] Some congenital heart conditions are linked to a genetic disorder such as Edwards syndrome or 22q Deletion. […] Congenital heart conditions occur in 1 in 130 pregnancies. […] Most but not all congenital heart centres around the country offer surgery for these conditions. […] The outcomes of treatment for right-sided single ventricle conditions are good. […] Over that time we have seen the success of treatment improve. […] Although children have a good survival rate following surgery they will still only have half a working heart.
#12 Double Outlet Right Ventricle | Abdominal Key
https://abdominalkey.com/double-outlet-right-ventricle/
Epidemiology […] DORV is a rare congenital cardiac anomaly. Its frequency is approximately 0.09 per 1000 births. There is no racial or gender predominance. […] […] Relatively uncommon congenital anomaly seen in 0.09 in 1000 births.
#12 Double outlet right ventricle | PPT
https://www.slideshare.net/slideshow/double-outlet-right-ventricle/42632465
After surgery Subaortic obstruction Subpulmonary obstruction AV valve regurgitation Conduit failure (with stenosis and or regurgitation) Neoaortic regurgitation (following arterial switch procedures) Coarctation or recoarctation of the aorta Small or dysfunctional RV (may relate to complex intracardiac repair) Rhythm problems such as heart block, atrial arrhythmia, and ventricular arrhythmia Sudden death Endocarditis Thromboembolic phenomena.
#13 Double Outlet Right Ventricle With Transposition: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/896082-overview
Double outlet right ventricle (DORV) is present in 0.5-1.5% of all patients with congenital heart disease (CHD). The estimated frequency of DORV is 1 case per 10,000 live births. […] A recent study showed a higher prevalence of DORV, tetralogy of Fallot, and truncus arteriosus, in addition to endocardial cushion defects and single ventricle, in certain regions of the country. […] No sex predilection is reported. […] The presentation is usually in the newborn period with this entity; however, in some circumstances (eg, subaortic VSD with mild-to-moderate PS), the diagnosis may not be made until later in infancy.
#13 Double Outlet Right Ventricle | Cleveland Clinic Childrenâs
https://my.clevelandclinic.org/pediatrics/services/double-outlet-right-ventricle-treatment
It can be a big shock to learn your baby has a serious heart problem. […] Double outlet right ventricle (DORV), is a congenital (born with it) condition that can be repaired early with surgery usually within your childs first year. […] Finding and treating your babys double outlet right ventricle as early as possible helps your child thrive as they grow. […] To diagnose double outlet right ventricle, we start by listening to your childs heart with a stethoscope. […] DORV is different for each child. […] Most children with DORV need surgery. […] Getting an early diagnosis and treatment for double outlet right ventricle can prevent their condition from getting worse.
#14 Double-Outlet Right Ventricle | Thoracic Key
https://thoracickey.com/double-outlet-right-ventricle-6/
DORV may exist as an isolated condition or in association with cardiac or extracardiac anomalies. The reported incidence ranges from 0.03 to 0.14 per 1000 live births. It occurs in about 1% of all congenital heart disease. […] Several chromosomal abnormalities have been associated with DORV, including trisomy 13, trisomy 18, and chromosome 22q11 deletion.
#14 Double Outlet Right Ventricle (DORV) | Little Hearts Matter
https://www.lhm.org.uk/double-outlet-right-ventricle/
Double Outlet Right Ventricle (DORV) is a congenital heart condition (a problem that a baby is born with). […] Heart conditions that affect the blood flow to the lungs occur in 1 in 6,000 babies. […] As yet the causes of single ventricle heart conditions are unknown. […] Many families who have a child with congenital heart disease ask why their child was born with the condition. […] Some congenital heart conditions are linked to a genetic disorder such as Edwards syndrome or 22q Deletion. […] Congenital heart conditions occur in 1 in 130 pregnancies. […] Most but not all congenital heart centres around the country offer surgery for these conditions. […] The outcomes of treatment for right-sided single ventricle conditions are good. […] Over that time we have seen the success of treatment improve. […] Although children have a good survival rate following surgery they will still only have half a working heart.
#15 Double Outlet Right Ventricle | Loma Linda University Children’s Health
https://lluch.org/conditions/double-outlet-right-ventricle
Double outlet right ventricle (DORV) is a type of congenital heart malformation. This means it is present from birth. […] Only a small number of newborns have heart malformations. Of these, DORV is relatively rare. […] Most cases of DORV happen without any known cause. But they may be more likely to happen with certain syndromes. […] DORV can be treated with surgery. Surgery can correct blood flow so that it moves the right way from the left ventricle to the aorta and from the right ventricle to the pulmonary artery. […] DORV can cause serious complications. These include heart failure, high blood pressure in the lungs, and death. […] Many children with DORV can lead full and active lives. But they need lifelong follow-up care.
#15 Double Outlet Right Ventricle in Children
https://healthlibrary.brighamandwomens.org/library/diseasesconditions/Pediatric/160,113
DORV can be treated with surgery. Surgery can correct blood flow so that it moves the right way from the left ventricle to the aorta and from the right ventricle to the pulmonary artery. Surgery is also needed to fix the defect in the ventricular wall. […] DORV can lead to a number of complications. The risk for complications varies according to the type of DORV, other heart conditions present, and the time of diagnosis. Early treatment can cut the chance of later problems. […] Many children with DORV can lead full and active lives. But they need lifelong follow-up care.
#16 Double-Outlet Right Ventricle – DORV
https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/double-outlet-right-ventricle-dorv.html
DORV is a rare cardiac abnormality, where both the pulmonary artery and aorta arise from the right ventricle of the heart. […] DORV is often an abnormality that arises without a clear cause. […] Most cases of DORV are sporadic and not inherited.
#16 Double Outlet Right Ventricle in Children
https://healthlibrary.osfhealthcare.org/Library/Encyclopedia/160,113
Double outlet right ventricle (DORV) is a type of congenital heart malformation. Only a small number of newborns have heart malformations. Of these, DORV is relatively rare. […] Most cases of DORV happen without any known cause. But they may be more likely to happen with certain syndromes. […] DORV can cause serious complications. These include heart failure, high blood pressure in the lungs, and death. […] Many children with DORV can lead full and active lives. But they need lifelong follow-up care.
#17 Double outlet right ventricle – Wikipedia
https://en.wikipedia.org/wiki/Double_outlet_right_ventricle
DORV affects between 1% and 3% of people born with congenital heart defects. […] Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.
#18 Double-Outlet Right Ventricle | Thoracic Key
https://thoracickey.com/double-outlet-right-ventricle-6/
DORV may exist as an isolated condition or in association with cardiac or extracardiac anomalies. The reported incidence ranges from 0.03 to 0.14 per 1000 live births. It occurs in about 1% of all congenital heart disease. […] Several chromosomal abnormalities have been associated with DORV, including trisomy 13, trisomy 18, and chromosome 22q11 deletion.
#19 Double outlet right ventricle | PPT
https://www.slideshare.net/slideshow/double-outlet-right-ventricle/42632465
Double outlet right ventricle (DORV) is a congenital heart defect where both the aorta and pulmonary artery arise completely or predominantly from the right ventricle. […] Epidemiology Isolated /With extracardiac anomalies Incidence:0.03 to 0.14 per 1000 live births 1% of all CHD Association: coarctation, aortic arch hypoplasia, or interrupted aortic archparticularly at the transposition end of the spectrum, right atrial isomerism Chromosomal abnormalities :Trisomy 13,trisomy 18, 22q11 deletion. […] Decision making for Rx Diagnosis is DORV Differentiate from TOF Location of the VSD Disposition of great arteries PS or no PS Size of VSD […] DORV with Transposition-like Physiology and Pulmonary Stenosis Rastelli repair REV procedure The REV procedure (rparation ltage ventriculaire) entails division of the main PA with extensive mobilization,translocation of the PA anterior to aorta (Lecompte maneuver),and direct connection of the PA to the RV, thus eliminating the use of prosthetic materials.
#20 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.
#21 Double Outlet Right Ventricle (DORV) | Rady Children’s Hospital
https://www.rchsd.org/health-article/double-outlet-right-ventricle-dorv/
Double outlet right ventricle (DORV) is a heart defect where the aorta connects to the heart in the wrong place. DORV is a congenital heart defect, which means a baby who has it is born with it. […] In most cases, DORV happens with no known cause. Babies with certain genetic conditions such as trisomy 13, trisomy 18, or DiGeorge syndrome (22q11.2 deletion) are more at risk for DORV. […] DORV sometimes is seen on ultrasound scans before birth. A fetal echocardiogram (a more detailed ultrasound scan) may provide more information and guide the delivery teams preparations. […] Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery.
#22 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.
#23 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.
#24 Double Outlet Right Ventricle (DORV) | Texas Children’s
https://www.texaschildrens.org/content/conditions/double-outlet-right-ventricle-dorv
Double outlet right ventricle (DORV) is a congenital heart defect, meaning it is present at birth. It occurs when the heart doesnât form properly during fetal development. In most cases, the cause is unknown. […] DORV occurs in an estimated 6 to 15 infants per 100,000 live births. It is more common in males. […] Genetic counseling and testing is recommended for all women pregnant with a baby with DORV. […] Double outlet right ventricle may be detected before birth during a routine prenatal ultrasound. Your OB/GYN will refer you to a pediatric cardiologist, who will perform a fetal echocardiogram (cardiac ultrasound) to better view and evaluate your babyâs heart structure and blood flow. […] Babies born with DORV will need to continue to see a heart specialist throughout their lives, transitioning at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects.
#25 Double Outlet Right Ventricle (DORV) | Texas Children’s
https://www.texaschildrens.org/content/conditions/double-outlet-right-ventricle-dorv
Double outlet right ventricle (DORV) is a congenital heart defect, meaning it is present at birth. It occurs when the heart doesnât form properly during fetal development. In most cases, the cause is unknown. […] DORV occurs in an estimated 6 to 15 infants per 100,000 live births. It is more common in males. […] Genetic counseling and testing is recommended for all women pregnant with a baby with DORV. […] Double outlet right ventricle may be detected before birth during a routine prenatal ultrasound. Your OB/GYN will refer you to a pediatric cardiologist, who will perform a fetal echocardiogram (cardiac ultrasound) to better view and evaluate your babyâs heart structure and blood flow. […] Babies born with DORV will need to continue to see a heart specialist throughout their lives, transitioning at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects.
#26 Double Outlet Right Ventricle (DORV) | Rady Children’s Hospital
https://www.rchsd.org/health-article/double-outlet-right-ventricle-dorv/
Double outlet right ventricle (DORV) is a heart defect where the aorta connects to the heart in the wrong place. DORV is a congenital heart defect, which means a baby who has it is born with it. […] In most cases, DORV happens with no known cause. Babies with certain genetic conditions such as trisomy 13, trisomy 18, or DiGeorge syndrome (22q11.2 deletion) are more at risk for DORV. […] DORV sometimes is seen on ultrasound scans before birth. A fetal echocardiogram (a more detailed ultrasound scan) may provide more information and guide the delivery teams preparations. […] Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery.
#27 Double Outlet Right Ventricle | Mount Sinai – New York
https://www.mountsinai.org/locations/childrens-heart/conditions/double-outlet-right-ventricle
If your child has a diagnosis of double outlet right ventricle (DORV), you will want to know as much as possible about the problemâand the solution. And you want to know as quickly as possible. […] Our Fetal Heart Program specialists can diagnose double outlet right ventricle before your baby is born. When we detect the condition during pregnancy, our doctors will monitor mother and baby throughout the pregnancy. Your dedicated team of specialists will explain how we can help you and will develop a plan for your babyâs birth and afterward. […] In other cases, we find the DORV after birth. We may perform certain diagnostic tests in your child to confirm the presence of DORV so we can plan treatment. Diagnosis may include: […] Patients with DORV require lifelong follow-up care, and the Childrenâs Heart Center is here for your child.
#28 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.
#29 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.
#30 Double-Outlet Right Ventricle | Radiology Key
https://radiologykey.com/double-outlet-right-ventricle/
Double-outlet right ventricle (DORV) refers to a group of complex heart anomalies in which both great arteries arise entirely, or to a great extent, from the right ventricle. […] DORV accounts for 4% to 8% of prenatal congenital heart diseases (CHDs). The high association of DORV with cardiac, extracardiac, and chromosomal anomalies is responsible for its lower postnatal incidence (1% to 3%). […] Associated anomalies are frequent in fetuses with DORV. Chromosomal abnormalities are found in 10% to 20% of cases; trisomies 18 and 13 are the most frequent, and trisomy 21 and 22q11 microdeletion are also found in DORV. Other commonly associated cardiac anomalies are atrioventricular septal defect, cardiosplenic syndromes, aortic coarctation, mitral atresia, and straddling or overriding of the atrioventricular valves with consequent hypoplasia of the underlying ventricle. Finally, extracardiac anomalies are present in two-thirds of cases, especially gastrointestinal and central nervous system anomalies.