Materiały źródłowe

• #1 Double outlet right ventricle

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10560996/

  This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle. […] Double outlet right ventricle (DORV) was first described pathologically in 1957. […] The consensus definition by the International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD) ultimately settled on the hearts with both arterial trunks supported predominantly by underlying morphologic RV where both great arteries arise entirely or predominantly from the morphological RV. […] In essence, DORV is a purely descriptive term that is not used to name a discrete congenital cardiac malformation but rather to corral a broad range of phenotypes. […] The most frequently used classification of DORV is based on the location of the VSD and the position of great arteries, the relationship of great arteries to each other, and the presence or absence of outflow tract obstruction.

• #2 Double-outlet right ventricle – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537

  In double-outlet right ventricle, the lower right heart chamber, called the right ventricle, has two openings for blood to exit instead of one. Some blood goes to the body instead of just the lungs. There also is a hole between the two lower heart chambers. The hole is called a ventricular septal defect. 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. […] Babies with double-outlet right ventricle also have a hole between the lower heart chambers. The lower heart chambers are called the ventricles. The hole is called a ventricular septal defect. The hole causes oxygen-rich blood to mix with oxygen-poor blood. Babies with this hole may not get enough oxygen in the bloodstream. Their skin may appear gray or blue.

• #3 Orphanet: Double outlet right ventricle

  https://www.orpha.net/en/disease/detail/3426

  A rare cono-truncal anomaly in which both the aorta and pulmonary artery originate, either entirely or predominantly, from the morphologic right ventricle. […] Double outlet right ventricle (DORV) accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000. […] Diagnosis is based on 3D echocardiography showing both great arteries arising from the right ventricle. In complex forms, cardiac catheterization-angiography, magnetic resonance imaging (MRI) and /or computed tomography (CT) scan are required. Recently, 3D printed models have been very useful to plane the surgery. […] DORV can be diagnosed by fetal echocardiography with a good degree of accuracy when the two vessels arise entirely from the right ventricle.

• #4 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 problem—and 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 baby’s 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: […] Chest X-ray, which takes pictures of the your child’s heart, lungs, and blood vessels, and can show if the heart is enlarged

• #5 Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center

  https://www.degruyter.com/document/doi/10.1515/jpm-2018-0316/html?lang=en

  To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with double outlet right ventricle (DORV). […] A correct prenatal diagnosis of DORV was made in 96.3% of the cases. […] DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. […] DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to terminations or declined postnatal therapy. […] Without additional anomalies, the prognosis is good, although approximately 60% of children will have single ventricle palliation. […] The aim of this study was to assess the spectrum of associated cardiac, extracardiac and chromosomal anomalies, the intrauterine course and postnatal outcome of fetuses with DORV.

• #6 Double Outlet Right Ventricle (DORV): Overview, Symptoms and Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/double-outlet-right-ventricle

  Double outlet right ventricle, or DORV, is a heart condition present at birth. The bodys main artery and the lung artery do not connect to the usual areas in the heart. […] In DORV, both the pulmonary artery and the aorta (the outflow vessels) come from the right ventricle. […] DORV frequently will have additional heart defects and is very unique to each child. […] When you are pregnant, there are many tests that can tell the doctor if your baby has a heart (cardiac) problem. […] The following are some prenatal tests that may be done: […] Complex heart defects are usually found during a pregnancy (prenatal) ultrasound. […] A fetal echocardiogram is a special ultrasound used by a pediatric heart doctor (cardiologist). […] The cardiologist will assess your baby after birth with an echocardiogram to confirm the diagnosis.

• #7 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 doesn’t form properly during fetal development. In most cases, the cause is unknown. […] DORV differs in each baby based on the location of the VSD, the positioning of the aorta and pulmonary artery (which are normally side-by-side), and other cardiac defects involved. The impact these variables have on the normal blood flow through the heart will determine the baby’s prognosis and the intervention required. […] 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 baby’s heart structure and blood flow.

• #8 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  Clinical laboratory studies (eg, hematologic analysis, urinalysis) are not likely to be of diagnostic help; late findings may include polycythemia, but this and other findings of chronic cyanosis are nonspecific. […] Echocardiography is used to evaluate anatomy, hemodynamics, and function of the heart both prior to and after surgical repair or palliation, and it is the most important means of establishing diagnosis of double outlet right ventricle (DORV) with transposition of the great arteries. […] In experienced centers, the accuracy of the prenatal echocardiographic diagnosis (and prognosis) of fetuses with conotruncal anomalies in general is good (ie, correct diagnosis in 77% of cases in a major center). […] However, defining the exact spatial relationship of the great arteries can be problematic in some fetuses (ie, 7 of 17 fetuses with DORV anatomy, of which 6 were thought to have a subpulmonary VSD, had incorrect prenatal assessment of the great artery relationships).

• #9 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. […] A fetal echocardiogram will be performed to confirm the diagnosis and give information about what treatment will be needed. […] Delivery will usually occur at a centre where pediatric cardiologists and cardiac surgeons are available nearby. […] Most cases of DORV are sporadic and not inherited. […] For isolated DORV, the risk of recurrence is about 1.5 to 2 in 100.

• #10 Double Outlet Right Ventricle (DORV) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/dorv

  Double outlet right ventricle (DORV) is a rare congenital heart defect, meaning its a condition a baby is born with. […] If your newborn baby has a bluish tint to the skin or your young child has symptoms of a heart condition, your pediatrician will refer you to a pediatric cardiologist (heart doctor) for a full examination. […] The doctor may also order one or more of the following tests to diagnose DORV and any related heart conditions: Electrocardiogram (ECG or EKG), Echocardiogram (cardiac ultrasound), Chest X-ray, Cardiac catheterization. […] Children with double outlet right ventricle (DORV) will need surgery to close the hole in the heart and connect the aorta to the left ventricle and the pulmonary artery to the right ventricle. […] The most common surgical procedure for DORV with a ventricular septal defect (VSD) is called intraventricular tunnel repair. […] If your child has other heart conditions associated with DORV, he or she may also need other types of surgery.

• #11 Double Outlet Right Ventricle (DORV) (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/dorv.html

  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. […] 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 team’s preparations. […] If the diagnosis is made after the birth, it’s the baby’s skin color and breathing problems that indicate a problem. Doctors listening to the baby’s heartbeat may hear an abnormal sound (a murmur) and use these tests to look at the baby’s heart: pulse oximetry, chest X-ray, electrocardiogram (ECG or EKG), echocardiogram, cardiac MRI or CT scan. […] Babies with DORV often have more than one heart problem. So while doctors check to see how the outlet vessels and VSD are arranged, they will also look for other problems.

• #12 Double-outlet right ventricle – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537

  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. […] 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. […] If more details about the heart are needed, other tests may be done. Tests to check the heart may include: Heart CT scan. This also is called a cardiac CT. This test uses X-rays to create cross-sectional images of specific parts of the body. Heart MRI scan. This test uses magnetic fields and radio waves to create detailed pictures of the heart. Cardiac catheterization. A doctor places a long, thin flexible tube called a catheter into a blood vessel, usually in the groin or wrist. It’s guided to the heart. Dye flows through the catheter to the heart arteries. The dye helps the arteries show up more clearly on images taken during the test. During this test, the healthcare professional also can measure pressure and oxygen levels in the chambers of the heart and in the blood vessels.

• #13 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. […] If not, a provider usually diagnoses double outlet right ventricle in the days or weeks after birth because of a babys symptoms. […] Your babys healthcare provider may order one or more tests, like: Noninvasive imaging tests: Computed tomography (CT), magnetic resonance imaging (MRI) and X-ray take pictures from outside your babys body. […] Almost all babies with DORV need open-heart surgery within their first year of life. […] Most babies have good outcomes from surgery for DORV. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis. […] With surgery, most babies who have double outlet right ventricle live to be adults. […] People who had a surgical repair for DORV can carry a pregnancy. […] About 90% of people survive at least 10 years after surgery.

• #14 Double Outlet Right Ventricle | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/double-outlet-right-ventricle

  Double outlet right ventricle (DORV) is a form of a congenital heart disease in which both great arteries are connected to the right ventricle instead of one great artery connected to each ventricle. […] In some cases, double outlet right ventricle is diagnosed while a child is still in the womb. […] Several different tests are used to diagnose DORV after a baby is born. These include: Electrocardiogram (EKG or ECG), which is a record of the electrical activity of the heart; Echocardiogram (also called echo or ultrasound), which is when sound waves create an image of the heart; Chest X-ray; Cardiac MRI, which is a 3D image that shows the heart’s abnormalities. […] Sometimes, cardiac catheterization will be required. A thin, flexible tube (catheter) is inserted through a vein or artery in the leg and into the heart to provide detailed information about the structure and function of the heart and lungs.

• #15 Double Outlet Right Ventricle – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/double-outlet-right-ventricle

  In double outlet right ventricle (DORV), both the aorta and pulmonary valve connect to the right ventricle. […] Diagnosis is by ECG, imaging, and cardiac catheterization. […] Diagnosis is with chest x-ray, MRI, CT, ECG, echocardiography, and cardiac catheterization. […] The ECG consistently shows right ventricular hypertrophy with right axis. […] Echocardiography demonstrates the anatomy well, showing the size and location of the VSD, arrangement of the aortic and pulmonary arteries, and the presence or degree of pulmonary stenosis, either below or at the valve level. […] MRI or CT is valuable in planning surgical intervention by providing details of the spatial relationships of the VSD and great vessels. […] Cardiac catheterization may have a role in defining the hemodynamics, including any gradient across the VSD, pulmonary artery pressure, and vascular resistance.

• #16

  https://journals.lww.com/jiae/fulltext/2020/04030/double_outlet_ventricle__echocardiographic.11.aspx

  Double outlet ventricle is a type of conoventricular anomaly. The great arteries arise predominantly (more than 50%) from one of the ventricle. […] Double outlet right ventricle (DORV) is the most common anomaly in this group of abnormalities. The correct anatomical diagnosis requires a detail segmental approach in echocardiography. The echocardiographic features of DORV reviewed in detail followed by a brief discussion on DOLV. […] DORV is a type of ventriculo-arterial connection where more than 50% of both great arteries arise from the morphological right ventricle. DORV constitutes approximately 1% of all congenital heart disease with a reported incidence of 0.06 cases per 1000 live births. […] DORV is a type of conotruncal defect in which a variable amount of conal tissue is present under each semilunar valve.

• #17

  https://journals.lww.com/jiae/fulltext/2020/04030/double_outlet_ventricle__echocardiographic.11.aspx

  The important determinants that influence the clinical features include (i) anatomical location of ventricular septal defect (VSD) in relation to great arteries, (ii) size of the VSD, (iii) relationship of the great arteries and (iv) presence of outflow obstruction. […] Echocardiogram remains the mainstay in diagnosis of DORV for identifying the location of the VSD, its relation to the great arteries, relationship of great arteries, outflow obstructions, ventricular hypoplasia, atrioventricular valve anomalies, and other associated anomalies. […] Surgery for DORV depends on the anatomy, physiology, age of the patient. If the VSD is conotruncal, repair is often possible by routing the VSD into the nearest semilunar valve annulus. […] Echocardiogram remains to be the basic tool in evaluation for double outlet right ventricle.

• #18 Double Outlet Right Ventricle – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/double-outlet-right-ventricle

  In double outlet right ventricle (DORV), both the aorta and pulmonary valve connect to the right ventricle. […] Diagnosis is by ECG, imaging, and cardiac catheterization. […] Diagnosis is with chest x-ray, MRI, CT, ECG, echocardiography, and cardiac catheterization. […] The ECG consistently shows right ventricular hypertrophy with right axis. […] Echocardiography demonstrates the anatomy well, showing the size and location of the VSD, arrangement of the aortic and pulmonary arteries, and the presence or degree of pulmonary stenosis, either below or at the valve level. […] MRI or CT is valuable in planning surgical intervention by providing details of the spatial relationships of the VSD and great vessels. […] Cardiac catheterization may have a role in defining the hemodynamics, including any gradient across the VSD, pulmonary artery pressure, and vascular resistance.

• #19 Double Outlet Right Ventricle Surgery Workup: Approach Considerations, Imaging Studies

  https://emedicine.medscape.com/article/904397-workup

  Electrocardiographic findings are rarely diagnostic for double outlet right ventricle (DORV). Common findings in a child with double outlet right ventricle include right ventricular hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular hypertrophy. […] Echocardiography generally provides enough information for accurate and adequate diagnosis, and provides the needed information to plan the surgical approach in neonates and young infants. […] Cardiac catheterization, once the criterion standard for confirming double outlet right ventricle, is now rarely required in the evaluation or preoperative planning of this cardiac disorder. […] MRI has been used in the diagnosis of double outlet right ventricle, but it is not yet a routine or well-established diagnostic modality for this condition.

• #20 Double Outlet Right Ventricle Surgery Workup: Approach Considerations, Imaging Studies

  https://emedicine.medscape.com/article/904397-workup

  Regardless of the end of the clinical spectrum (tetralogy of Fallot [TOF] or transposition of the great arteries [TGA]) at which double outlet right ventricle occurs, findings on anteroposterior and lateral chest radiography depend on the degree of pulmonary (or subpulmonary) stenosis. […] Preoperative CT scanning is potentially useful for identifying coronary artery anatomy in children with TOF or Fallot type of double outlet right ventricle.

• #21 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Diagnosis-and-Treatment-of-Double-Outlet-Right-Ventricle.aspx

  The imaging technique most often used to diagnose DORV is echocardiography. This technique uses sound waves to create an image of the heart, which shows both great arteries predominantly committed to the right ventricle. This technique can also be used to determine the VSD in relation to the great vessels as well as the spatial relationship between the aorta and the pulmonary artery. Moreover, the presence of associated cardiac anomalies may also be identified. Prenatal fetal echocardiograms can be done to diagnose DORV. […] Chest x-rays can also be done and typically tend to correlate with the clinical picture. However, they are not able to distinguish between DORV and other congenital heart defects. If there are any residual ambiguities after conducting an echocardiogram, then MRI may be used. However, MRI is particularly challenging due to the need for possible sedation and a more prolonged diagnostic process. Electrocardiograms are useful in that they produce results that indicate ventricular hypertrophy and atrial enlargement.

• #22 Double-outlet right ventricle – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537

  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. […] 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. […] If more details about the heart are needed, other tests may be done. Tests to check the heart may include: Heart CT scan. This also is called a cardiac CT. This test uses X-rays to create cross-sectional images of specific parts of the body. Heart MRI scan. This test uses magnetic fields and radio waves to create detailed pictures of the heart. Cardiac catheterization. A doctor places a long, thin flexible tube called a catheter into a blood vessel, usually in the groin or wrist. It’s guided to the heart. Dye flows through the catheter to the heart arteries. The dye helps the arteries show up more clearly on images taken during the test. During this test, the healthcare professional also can measure pressure and oxygen levels in the chambers of the heart and in the blood vessels.

• #23 Double Outlet Right Ventricle | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/double-outlet-right-ventricle

  Cardiac magnetic resonance imaging (MRI), which uses safe, powerful magnets and radio waves to create pictures of your child’s heart […] Echocardiogram (echo or ultrasound), which uses sound waves to create pictures of the heart chambers, valves, walls, and blood vessels a safe, noninvasive procedure that uses ultrasound waves to create an image of the size, shape, and movement of your child’s heart, valves, and chambers along with how blood flows (also called an echo or cardiac ultrasound) […] Electrocardiogram (EKG or ECG), which is a noninvasive procedure that shows electrical activity by placing stickers on your child’s chest. […] We may need to perform a cardiac catheterization, a test that involves inserting a thin tube into the baby’s blood vessels, usually in the groin to reach the heart. This test allows us to measure blood pressure and oxygen levels and get detailed pictures of the heart and vessels.

• #24 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  MRI may serve as an adjunct tool to echocardiography for determination of visceral and atrial situs as vasculo-vascular and vasculo-visceral relationships. […] In some patients with DORV with remote VSD, MRI may aid in defining the spatial relationship between VSD and the semilunar valves. […] A new modality in MRI is the 3-dimensional MRI, which is increasingly used as an adjuvant to echocardiography and angiography for such purposes. […] Angiography may add anatomic and physiologic details to information found by echocardiography. […] ECG in patients with DORV with transposition of the great arteries reveals no specific findings indicative of the diagnosis. […] Usually, normal sinus rhythm and possible prolonged PR interval are present. […] Right axis deviation and right ventricular hypertrophy (RVH) are present most often.

• #25 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  Some authors have described real-time 3-dimensional echocardiography as a way to improve cardiac imaging and diagnosis of complex congenital heart disease (CHD) through a clear display of cardiac morphology using volumetric views combined with sequential segmental approach. […] However, others have recently concluded that information provided by real-time 2-dimensional echocardiography in fetuses with and without CHD were consistent with that provided by real-time 3-dimensional echocardiography. […] Therefore, no clear advantage of real-time 3-dimensional echocardiography over real-time 2-dimensional echocardiography has been documented. […] Sometimes chest radiography may provide valuable clues for the diagnosis of DORV with transposition of the great arteries. […] CT has been described as an effective diagnostic modality, especially in identifying coronary artery anomalies prior to cardiac surgery to aid in adequate procedure planning.

• #26 Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130987

  To evaluate the feasibility and diagnostic accuracy of retrospective electrocardiographically (ECG)-gated dual-source computed tomography (DSCT) for the assessment of double outlet right ventricle (DORV) and associated multiple malformations in pediatric patients. […] Retrospective ECG-gated DSCT is a better diagnostic tool than TTE for pediatric patients with complex congenital heart disease such as DORV. Combined with TTE, it may reduce or even obviate the use of invasive cardiac catheterization, and thus expose the patients to a much lower radiation dose. […] The purpose of this study was to evaluate the feasibility and diagnostic accuracy of retrospective ECG-gated DSCT for DORV and associated multiple malformations in pediatric patients. […] DSCT appears to be slightly inferior to TTE for the detection of intracardiac anomalies (sensitivity: DSCT, 91.30%; TTE, 100%). […] Our study suggested that retrospective ECG-gated DSCT has satisfactory diagnostic accuracy in the preoperative assessment of DORV and associated multiple malformations in pediatric patients and can provide excellent diagnostic image quality without the disadvantage of increasing the radiation dose.

• #27 Orphanet: Double outlet right ventricle

  https://www.orpha.net/en/disease/detail/3426

  A rare cono-truncal anomaly in which both the aorta and pulmonary artery originate, either entirely or predominantly, from the morphologic right ventricle. […] Double outlet right ventricle (DORV) accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000. […] Diagnosis is based on 3D echocardiography showing both great arteries arising from the right ventricle. In complex forms, cardiac catheterization-angiography, magnetic resonance imaging (MRI) and /or computed tomography (CT) scan are required. Recently, 3D printed models have been very useful to plane the surgery. […] DORV can be diagnosed by fetal echocardiography with a good degree of accuracy when the two vessels arise entirely from the right ventricle.

• #28 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The individualized surgical approach in individuals with both arterial trunks arising from the morphologically right ventricle is dictated by the extreme morphological variability encountered in this setting, with each patient being unique. […] An individualized surgical approach has been designed to take account of the morphological variations, identifying the anatomy with the preoperative three-dimensional CT scan reconstruction. […] The key point identified for this review topic is that the choice of the optimal approach depends on two variables, namely the position of the interventricular communication, and the relationship of the arterial outlets. […] The optimal surgical approach, of course, is to connect the left ventricle in unobstructed fashion to the aorta, leaving the right ventricle in free communication with the pulmonary trunk, without needing to interpose a prosthetic conduit.

• #29 Double Outlet Right Ventricle Surgery Workup: Approach Considerations, Imaging Studies

  https://emedicine.medscape.com/article/904397-workup

  Electrocardiographic findings are rarely diagnostic for double outlet right ventricle (DORV). Common findings in a child with double outlet right ventricle include right ventricular hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular hypertrophy. […] Echocardiography generally provides enough information for accurate and adequate diagnosis, and provides the needed information to plan the surgical approach in neonates and young infants. […] Cardiac catheterization, once the criterion standard for confirming double outlet right ventricle, is now rarely required in the evaluation or preoperative planning of this cardiac disorder. […] MRI has been used in the diagnosis of double outlet right ventricle, but it is not yet a routine or well-established diagnostic modality for this condition.

• #30 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  Left axis deviation is present in patients with atrioventricular (AV) septal defect. […] Some ECG variations may be noted, depending on the type of DORV with transposition of the great arteries. […] Echocardiography has mostly eliminated the need to perform cardiac catheterization in these patients; however, catheterization may still be necessary in certain circumstances. […] Catheterization may be required for the following reasons: Need for further definition of coronary artery anatomy, Need to determine coexistent conditions that cannot be elucidated by echocardiography, Need to confirm restrictive VSD by measuring ventricular pressures, Need to determine pulmonary vascular resistance (and reactivity) in patients suspected of having increased resistance.

• #31 Double-outlet right ventricle – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537

  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. […] 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. […] If more details about the heart are needed, other tests may be done. Tests to check the heart may include: Heart CT scan. This also is called a cardiac CT. This test uses X-rays to create cross-sectional images of specific parts of the body. Heart MRI scan. This test uses magnetic fields and radio waves to create detailed pictures of the heart. Cardiac catheterization. A doctor places a long, thin flexible tube called a catheter into a blood vessel, usually in the groin or wrist. It’s guided to the heart. Dye flows through the catheter to the heart arteries. The dye helps the arteries show up more clearly on images taken during the test. During this test, the healthcare professional also can measure pressure and oxygen levels in the chambers of the heart and in the blood vessels.

• #32 Double outlet right ventricle (DORV) | Children’s Health Queensland

  https://www.childrens.health.qld.gov.au/health-a-to-z/cardiac/double-outlet-right-ventricle

  Double outlet right ventricle (DORV) is a condition where the right chamber (ventricle) of the heart has two outlets the pulmonary artery and the aorta, which causes the mixing of oxygen-rich red blood and oxygen-poor blue blood. […] Many cases of DORV are diagnosed via ultrasound when a baby is still growing in the womb. It may also be detected if a doctor hears a heart murmur when examining your child. […] Tests to diagnose DORV include: Electrocardiogram (ECG) measures the electrical activity of the heart. Echocardiogram (Echo) uses sounds waves to produce a moving picture of the heart. Cardiac catheterisation A thin flexible tube (called a catheter) is inserted into a blood vessel in the groin and fed up into the heart to measure pressures and oxygen levels, and visualise heart structures using X-ray equipment. The procedure is performed under a general anaesthetic.

• #33 Double outlet right ventricle

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10560996/

  This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle. […] Double outlet right ventricle (DORV) was first described pathologically in 1957. […] The consensus definition by the International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD) ultimately settled on the hearts with both arterial trunks supported predominantly by underlying morphologic RV where both great arteries arise entirely or predominantly from the morphological RV. […] In essence, DORV is a purely descriptive term that is not used to name a discrete congenital cardiac malformation but rather to corral a broad range of phenotypes. […] The most frequently used classification of DORV is based on the location of the VSD and the position of great arteries, the relationship of great arteries to each other, and the presence or absence of outflow tract obstruction.

• #34

  https://journals.lww.com/jiae/fulltext/2020/04030/double_outlet_ventricle__echocardiographic.11.aspx

  The important determinants that influence the clinical features include (i) anatomical location of ventricular septal defect (VSD) in relation to great arteries, (ii) size of the VSD, (iii) relationship of the great arteries and (iv) presence of outflow obstruction. […] Echocardiogram remains the mainstay in diagnosis of DORV for identifying the location of the VSD, its relation to the great arteries, relationship of great arteries, outflow obstructions, ventricular hypoplasia, atrioventricular valve anomalies, and other associated anomalies. […] Surgery for DORV depends on the anatomy, physiology, age of the patient. If the VSD is conotruncal, repair is often possible by routing the VSD into the nearest semilunar valve annulus. […] Echocardiogram remains to be the basic tool in evaluation for double outlet right ventricle.

• #35 Double outlet right ventricle

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10560996/

  This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle. […] Double outlet right ventricle (DORV) was first described pathologically in 1957. […] The consensus definition by the International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD) ultimately settled on the hearts with both arterial trunks supported predominantly by underlying morphologic RV where both great arteries arise entirely or predominantly from the morphological RV. […] In essence, DORV is a purely descriptive term that is not used to name a discrete congenital cardiac malformation but rather to corral a broad range of phenotypes. […] The most frequently used classification of DORV is based on the location of the VSD and the position of great arteries, the relationship of great arteries to each other, and the presence or absence of outflow tract obstruction.

• #36 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The critical point is to understand whether the right ventricle remains in free communication with the pulmonary trunk subsequent to construction of the interventricular tunnel. […] When both outflow tracts arise from the right ventricle, as long as the pulmonary root is distant from the orifices of the atrioventricular valves, the distance between the atrioventricular valves and the aortic valvar orifice can increase without changing the relationship between the right and left outflow tracts. […] Should the pulmonary root not possess a well-developed infundibulum, however, and is very close to, or even in continuity with, the atrioventricular valves, it may become impossible to maintain the normal relationships between the right and left ventricular outflow tracts. […] In this setting, the creation of an unrestricted tunnel from the left ventricle to the aorta can compromise the remaining connection between the tricuspid and pulmonary valves.

• #37 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  When taking account of these features, it becomes possible to separate hearts with both arterial trunks arising from the right ventricle into two categories. […] In the first group, the arrangement of the arterial roots is such that the distance between the tricuspid and pulmonary valves is at least equal to the diameter of the aortic root. […] When this criterion is satisfied, it is usually possible to create the required unrestrictive tunnel to the aortic root without causing any obstruction to the residual connection between right ventricle and pulmonary valve. […] In the second group, the distance between tricuspid and pulmonary valves is less than the diameter of the aortic root. […] Recognition of this feature means that simple creation of a tunnel from the interventricular communication to the aortic root will not be feasible, and another surgical option will be required.

• #38 Double-Outlet Right Ventricle | Radiology Key

  https://radiologykey.com/double-outlet-right-ventricle/

  Double-outlet right ventricle is a rare congenital heart disease in which both vessels originate mainly from the right ventricle. Its diagnosis on routine ultrasound (US) examination is challenging because the four-chamber view is usually normal. The prognosis in cases detected prenatally is generally poor, due to a high association with chromosomal, extracardiac, and cardiac anomalies. […] Prenatal diagnosis with ultrasound (US) is challenging. The four-chamber view appears normal, unless there is a left axis deviation or a large VSD that extends posteriorly, which is then visible on the four-chamber view. […] On the five-chamber view or long-axis view, there is usually a perimembranous VSD with an overriding vessel. To be considered as DORV, the semilunar valve should override the VSD by at least 50%.

• #39 Double-Outlet Right Ventricle | Thoracic Key

  https://thoracickey.com/double-outlet-right-ventricle-8/

  Double-outlet right ventricle (DORV) is what Vierordt called partial transposition of the great arteries (TGA) in 1898, meaning that only the aorta was transposed from the left ventricle (LV) to the right ventricle (RV). […] In 1957, A. Calhoun Witham entitled his paper Double Outlet Right Ventricle, A Partial Transposition Complex. This is how DORV was introduced. […] My dear wife, Dr. Stella Van Praagh (who died in 2006), having studied DORV for years, used to say, DORV is all of congenital heart disease. Each case must be diagnosed individually and completely, so that one can make the wisest decisions concerning management. […] DORV is present when both great arteries are aligned entirely or predominantly with the morphologically RV. […] As far as differential diagnosis is concerned, we make the diagnosis of tetralogy of Fallot (TOF), for example, if there is aortic valvemitral valve direct fibrous continuity. […] Diagnoses should be specific and mutually exclusive. For example, the diagnosis should be TOF, or DORV, not TOF and DORV. Similarly, the diagnosis should be DORV or TGA, not DORV with (and) TGA. This is a basic principle of classification.

• #40 Double-outlet right ventricle – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/double-outlet-right-ventricle/cdc-20389537

  In double-outlet right ventricle, the lower right heart chamber, called the right ventricle, has two openings for blood to exit instead of one. Some blood goes to the body instead of just the lungs. There also is a hole between the two lower heart chambers. The hole is called a ventricular septal defect. 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. […] Babies with double-outlet right ventricle also have a hole between the lower heart chambers. The lower heart chambers are called the ventricles. The hole is called a ventricular septal defect. The hole causes oxygen-rich blood to mix with oxygen-poor blood. Babies with this hole may not get enough oxygen in the bloodstream. Their skin may appear gray or blue.

• #41 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. […] If not, a provider usually diagnoses double outlet right ventricle in the days or weeks after birth because of a babys symptoms. […] Your babys healthcare provider may order one or more tests, like: Noninvasive imaging tests: Computed tomography (CT), magnetic resonance imaging (MRI) and X-ray take pictures from outside your babys body. […] Almost all babies with DORV need open-heart surgery within their first year of life. […] Most babies have good outcomes from surgery for DORV. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis. […] With surgery, most babies who have double outlet right ventricle live to be adults. […] People who had a surgical repair for DORV can carry a pregnancy. […] About 90% of people survive at least 10 years after surgery.

• #42 Double-Outlet Right Ventricle | Thoracic Key

  https://thoracickey.com/double-outlet-right-ventricle-8/

  Double-outlet right ventricle (DORV) is what Vierordt called partial transposition of the great arteries (TGA) in 1898, meaning that only the aorta was transposed from the left ventricle (LV) to the right ventricle (RV). […] In 1957, A. Calhoun Witham entitled his paper Double Outlet Right Ventricle, A Partial Transposition Complex. This is how DORV was introduced. […] My dear wife, Dr. Stella Van Praagh (who died in 2006), having studied DORV for years, used to say, DORV is all of congenital heart disease. Each case must be diagnosed individually and completely, so that one can make the wisest decisions concerning management. […] DORV is present when both great arteries are aligned entirely or predominantly with the morphologically RV. […] As far as differential diagnosis is concerned, we make the diagnosis of tetralogy of Fallot (TOF), for example, if there is aortic valvemitral valve direct fibrous continuity. […] Diagnoses should be specific and mutually exclusive. For example, the diagnosis should be TOF, or DORV, not TOF and DORV. Similarly, the diagnosis should be DORV or TGA, not DORV with (and) TGA. This is a basic principle of classification.

• #43 Anatomy and Diagnosis of Double-outlet Right Ventricle | Japanese Society of Pediatric Cardiology and Cardiac Surgery

  https://jspccs.jp/english/publication/journals/backnumber/e2402/e2402093/

  Any heart in which both great vessels arise entirely or predominantly from the right ventricle is considered to have double-outlet right ventricle (DORV). […] If more than half of the aorta is connected to the right ventricle in a patient with tetralogy of Fallot (ToF), the diagnosis of ToF with DORV can be made. […] The morphology of DORV is encompassed by a careful description of the ventricular septal defect with its relationship to the semilunar valves, the great arteries relationships to each other, coronary artery anatomy, the presence of pulmonary/aortic outflow tract obstruction, and other associated cardiac lesions. […] In this report the most common forms of DORV, those occurring with atrioventricular concordance, will be considered.

• #44 Double Outlet Right Ventricle | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/double-outlet-right-ventricle/

  Double outlet right ventricle (DORV) is a rare cardiac birth defect where both great arteries (the pulmonary artery and the aorta) arise from the right ventricle. No great arteries are connected to the left ventricle (the chamber that normally pumps blood to the body). […] There are different types of double outlet ventricles based on the location of the ventricular septal defect and the position of the great arteries. Prenatal diagnosis is important as babies with DORV may have additional organ or chromosomal abnormalities, such as 22q11 Deletion Syndrome. […] Depending on their stability, babies with DORV require heart surgery in the first few days to months of life to correct the heart defect. […] A double outlet right ventricle (DORV) (1) forms when the aorta (2) and the pulmonary artery (3) both arise from the right ventricle (4). This creates a ventral septal defect (VSD) (5), which is a hole in the heart wall (septum) that separates the right and left ventricles. This opening (5) allows deoxygenated blood from the body and oxygenated blood from the lungs to mix. As a result, the other organs in the body do not receive enough oxygen.

• #45 Double Outlet Right Ventricle (DORV): Overview, Symptoms and Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/double-outlet-right-ventricle

  An echocardiogram is a special ultrasound used by a pediatric heart doctor (cardiologist) to look closely at your babys heart and surrounding blood vessels. […] This is a test to measure pressures in the heart. […] Cardiac MRI can provide detailed information on the type and severity of heart disease. […] The doctors may suggest genetic testing to find out more about this. […] All forms of DORV will need some type of heart surgery to fix the blood flow. […] This means that the blood flow is back to normal and the hole in the bottom chambers (VSD) is closed. […] When surgery happens depends on your childs unique DORV. […] The medical team may order tests like an echocardiogram or genetic studies based on your babys condition. […] If your babys condition is severe, they will need to be transferred to Nationwide Childrens Hospital Cardiothoracic Intensive Care Unit (CTICU) shortly after birth by the Nationwide Childrens Mobile Intensive Care team.

• #46 Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies? | Egyptian Journal of Radiology and Nuclear Medicine | Full Text

  https://ejrnm.springeropen.com/articles/10.1186/s43055-021-00677-w

  The term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. […] The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning. […] Our study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV.

• #47 Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies? | Egyptian Journal of Radiology and Nuclear Medicine | Full Text

  https://ejrnm.springeropen.com/articles/10.1186/s43055-021-00677-w

  It is superior to echocardiography in providing a comprehensive mapping of the anatomy of complex anomalies especially that of extra-cardiac structures such as aorta, systemic veins, pulmonary veins, and pulmonary arteries. […] However, it can miss tiny intracardiac malformations. […] So combining the results of MDCT and echocardiography would be beneficial to guarantee the accuracy of diagnosis of DORV.

• #48 Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130987

  To evaluate the feasibility and diagnostic accuracy of retrospective electrocardiographically (ECG)-gated dual-source computed tomography (DSCT) for the assessment of double outlet right ventricle (DORV) and associated multiple malformations in pediatric patients. […] Retrospective ECG-gated DSCT is a better diagnostic tool than TTE for pediatric patients with complex congenital heart disease such as DORV. Combined with TTE, it may reduce or even obviate the use of invasive cardiac catheterization, and thus expose the patients to a much lower radiation dose. […] The purpose of this study was to evaluate the feasibility and diagnostic accuracy of retrospective ECG-gated DSCT for DORV and associated multiple malformations in pediatric patients. […] DSCT appears to be slightly inferior to TTE for the detection of intracardiac anomalies (sensitivity: DSCT, 91.30%; TTE, 100%). […] Our study suggested that retrospective ECG-gated DSCT has satisfactory diagnostic accuracy in the preoperative assessment of DORV and associated multiple malformations in pediatric patients and can provide excellent diagnostic image quality without the disadvantage of increasing the radiation dose.

• #49 Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies? | Egyptian Journal of Radiology and Nuclear Medicine | Full Text

  https://ejrnm.springeropen.com/articles/10.1186/s43055-021-00677-w

  The term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. […] The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning. […] Our study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV.

• #50 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  Some authors have described real-time 3-dimensional echocardiography as a way to improve cardiac imaging and diagnosis of complex congenital heart disease (CHD) through a clear display of cardiac morphology using volumetric views combined with sequential segmental approach. […] However, others have recently concluded that information provided by real-time 2-dimensional echocardiography in fetuses with and without CHD were consistent with that provided by real-time 3-dimensional echocardiography. […] Therefore, no clear advantage of real-time 3-dimensional echocardiography over real-time 2-dimensional echocardiography has been documented. […] Sometimes chest radiography may provide valuable clues for the diagnosis of DORV with transposition of the great arteries. […] CT has been described as an effective diagnostic modality, especially in identifying coronary artery anomalies prior to cardiac surgery to aid in adequate procedure planning.

• #51 Double Outlet Right Ventricle With Transposition Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/896082-workup

  Some authors have described real-time 3-dimensional echocardiography as a way to improve cardiac imaging and diagnosis of complex congenital heart disease (CHD) through a clear display of cardiac morphology using volumetric views combined with sequential segmental approach. […] However, others have recently concluded that information provided by real-time 2-dimensional echocardiography in fetuses with and without CHD were consistent with that provided by real-time 3-dimensional echocardiography. […] Therefore, no clear advantage of real-time 3-dimensional echocardiography over real-time 2-dimensional echocardiography has been documented. […] Sometimes chest radiography may provide valuable clues for the diagnosis of DORV with transposition of the great arteries. […] CT has been described as an effective diagnostic modality, especially in identifying coronary artery anomalies prior to cardiac surgery to aid in adequate procedure planning.

• #52 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  Systemic and pulmonary afterloads have to be normal. […] Given the difficulties of diagnosis, and the multiple therapeutic indications, very close collaboration between cardiologists and surgeons is indispensable if further progress is to emerge in the understanding and management of this complex congenital cardiac lesion.

• #53 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  Systemic and pulmonary afterloads have to be normal. […] Given the difficulties of diagnosis, and the multiple therapeutic indications, very close collaboration between cardiologists and surgeons is indispensable if further progress is to emerge in the understanding and management of this complex congenital cardiac lesion.

• #54 Double Outlet Right Ventricle (DORV): Overview, Symptoms and Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/double-outlet-right-ventricle

  An echocardiogram is a special ultrasound used by a pediatric heart doctor (cardiologist) to look closely at your babys heart and surrounding blood vessels. […] This is a test to measure pressures in the heart. […] Cardiac MRI can provide detailed information on the type and severity of heart disease. […] The doctors may suggest genetic testing to find out more about this. […] All forms of DORV will need some type of heart surgery to fix the blood flow. […] This means that the blood flow is back to normal and the hole in the bottom chambers (VSD) is closed. […] When surgery happens depends on your childs unique DORV. […] The medical team may order tests like an echocardiogram or genetic studies based on your babys condition. […] If your babys condition is severe, they will need to be transferred to Nationwide Childrens Hospital Cardiothoracic Intensive Care Unit (CTICU) shortly after birth by the Nationwide Childrens Mobile Intensive Care team.

• #55 Double Outlet Right Ventricle (DORV): Overview, Symptoms and Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/double-outlet-right-ventricle

  These tests include an echocardiogram and an EKG. […] Your baby may need help with breathing, extra oxygen, or may need to be started on a medicine called prostaglandin E (PGE, or „prostins”). […] After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). […] Your cardiologist will follow up with your babys heart needs even after they are discharged from the hospital. […] Most of the time, congenital heart defects dont happen again with future pregnancies.

• #56 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. […] If not, a provider usually diagnoses double outlet right ventricle in the days or weeks after birth because of a babys symptoms. […] Your babys healthcare provider may order one or more tests, like: Noninvasive imaging tests: Computed tomography (CT), magnetic resonance imaging (MRI) and X-ray take pictures from outside your babys body. […] Almost all babies with DORV need open-heart surgery within their first year of life. […] Most babies have good outcomes from surgery for DORV. […] Without surgery, a baby with double outlet right ventricle will eventually develop: Cyanosis. […] With surgery, most babies who have double outlet right ventricle live to be adults. […] People who had a surgical repair for DORV can carry a pregnancy. […] About 90% of people survive at least 10 years after surgery.

• #57 Double Outlet Right Ventricle | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/double-outlet-right-ventricle/

  Double outlet right ventricle (DORV) is a rare cardiac birth defect where both great arteries (the pulmonary artery and the aorta) arise from the right ventricle. No great arteries are connected to the left ventricle (the chamber that normally pumps blood to the body). […] There are different types of double outlet ventricles based on the location of the ventricular septal defect and the position of the great arteries. Prenatal diagnosis is important as babies with DORV may have additional organ or chromosomal abnormalities, such as 22q11 Deletion Syndrome. […] Depending on their stability, babies with DORV require heart surgery in the first few days to months of life to correct the heart defect. […] A double outlet right ventricle (DORV) (1) forms when the aorta (2) and the pulmonary artery (3) both arise from the right ventricle (4). This creates a ventral septal defect (VSD) (5), which is a hole in the heart wall (septum) that separates the right and left ventricles. This opening (5) allows deoxygenated blood from the body and oxygenated blood from the lungs to mix. As a result, the other organs in the body do not receive enough oxygen.

• #58 Double Outlet Right Ventricle in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=double-outlet-right-ventricle-in-children-160-113

  A diagnosis after birth starts with a health history and physical exam. A cardiologist will need to do tests to make the diagnosis as well. The most important of these is an echocardiogram. This test shows the structure of the heart and the blood flow through the heart. […] 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. […] Most children with DORV go on to lead normal and active lives. But they will always need special follow-up care with cardiologists. […] DORV can lead to a number of complications. […] Children with heart abnormalities like DORV are also at higher risk for infection of the heart valves. […] Double outlet right ventricle is a type of heart malformation. It is present from birth. […] This is a serious condition often treated early in life with surgery. […] DORV can cause serious complications. These include heart failure, high blood pressure in the lungs, and death.

• #59 Double-Outlet Right Ventricle – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/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. […] If your developing baby is diagnosed with double-outlet right ventricle before birth, Seattle Childrens Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.

• #60 Double Outlet Right Ventricle in Children | UMass Memorial Health

  https://www.ummhealth.org/health-library/double-outlet-right-ventricle-in-children

  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. […] Most children with DORV go on to lead normal and active lives. But they will always need special follow-up care with cardiologists. […] 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. […] Double outlet right ventricle is a type of heart malformation. It is present from birth. […] This is a serious condition often treated early in life with surgery. […] DORV can cause serious complications. These include heart failure, high blood pressure in the lungs, and death.

• #61

• #62 Double Outlet Right Ventricle Treatment: Symptoms & Causes Explained – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/double-outlet-right-ventricle-treatment-symptoms-causes-explained/

  Double Outlet Right Ventricle (DORV) is a rare but serious congenital heart defect that disrupts the normal flow of blood through the heart and body. […] Understanding DORV is crucial for early diagnosis and effective management. […] DORV is typically diagnosed in infancy or early childhood. […] Advances in prenatal imaging, such as fetal echocardiography, have made it possible to diagnose DORV before birth in some cases, allowing for early planning and intervention. […] A heart murmur is detected in nearly 100% of DORV cases during a physical exam. […] Surgical repair addresses structural abnormalities in the heart to restore normal blood flow. […] Surgical repair offers the best chance for a normal or near-normal life, with most children experiencing significant improvement after recovery. […] Early intervention is crucial to preventing complications and achieving the best outcomes.

• #63 Double Outlet Right Ventricle Treatment: Symptoms & Causes Explained – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/double-outlet-right-ventricle-treatment-symptoms-causes-explained/

  Double Outlet Right Ventricle (DORV) is a rare but serious congenital heart defect that disrupts the normal flow of blood through the heart and body. […] Understanding DORV is crucial for early diagnosis and effective management. […] DORV is typically diagnosed in infancy or early childhood. […] Advances in prenatal imaging, such as fetal echocardiography, have made it possible to diagnose DORV before birth in some cases, allowing for early planning and intervention. […] A heart murmur is detected in nearly 100% of DORV cases during a physical exam. […] Surgical repair addresses structural abnormalities in the heart to restore normal blood flow. […] Surgical repair offers the best chance for a normal or near-normal life, with most children experiencing significant improvement after recovery. […] Early intervention is crucial to preventing complications and achieving the best outcomes.

• #64 Double Outlet Right Ventricle – Sheikh Shakhbout Medical City

  https://ssmc.ae/doctors-specialities/double-outlet-right-ventricle/

  Double Outlet Right Ventricle (DORV) is a congenital heart defect characterized by both the aorta and pulmonary artery arising predominantly from the right ventricle. […] Diagnosing DORV typically involves several imaging techniques: […] Echocardiography: This is the primary tool used to visualize heart structure and function. […] Double Outlet Right Ventricle is a complex congenital heart defect that requires careful diagnosis and management. Early intervention through surgical repair can significantly improve outcomes for affected individuals.

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