A double outlet right ventricle with in tact ventricular septum is extremely rare. Dorv is a congenital heart defect, which means a baby who has it is born with it. Double outlet ventricles right or left have been estimated to account for approximately 1 % of congenital heart disease, and double outlet right ventricle dorv accounts for betwe en 3 and 9100,000 births obler 2008. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. In people with doubleoutlet right ventricle, there is also a hole between the lower heart chambers ventricles, called a ventricular septal defect vsd, which can be located in several places in the wall between the ventricles. Diagnosis and treatment of double outlet right ventricle.
Summary double outlet right ventricle is a cardiac malformation in which both the aorta and pulmonary artery arise from the right ventricle. In this situation, the left ventricle lv has no direct outlet to either great artery and ejects through an interventricular communication, usually referred. Modified rastelli procedure for double outlet right ventricle. Dorv is defined as a type of ventriculoarterial connection in which both great arteries arise either entirely or predominantly from the right ventricle 1, 2.
Double outlet right ventricle defines a heterogeneous group of cardiac malformations unified by an abnormal. Jun 11, 2016 parasternal longaxis scan in a patient with double outlet right ventricle, with the aorta anterior and right of the pulmonary artery with a subpulmonary ventricular septal defect vsd. Essential modifiers of double outlet right ventricle. Modified rastelli procedure for double outlet right. Bilateral infundibula and ventricular septal defects are highly debated criteria. Double outlet right ventricle is a rare congenital cardiac malformation that appears to result from lack of conotruncal inversion, failure of leftward conoventricular shift and persistance of a subaortic conus 1, 2. In double outlet right ventricle dorv, a congenital heart disease, something goes wrong during the formation of the heart in the womb and both great arteries are attached to the right ventricle. Two of the vsds appeared small and obstructive, while the remainder were of the large variety. Dorv shows extremely variable morphologic features and clinical presentations. Double outlet right ventricle dorv is a relatively rare congenital cardiovascular anomaly that is now receiving more clinical attention because it is surgically correctable. In contrast to doubleoutlet right ventricle, dolv is a rarity. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.
Double outlet right ventricle european journal of cardio. Double outlet right ventricle with intact ventricular septum. The term double outlet right ventricle refers to any cardiac anomaly in which both the aorta and pulmonary trunk originate, predominantly or entirely, from the right ventricle rv 1. In dorv, the pulmonary artery and the aorta the hearts two major arteries both connect to the right ventricle. We analysed reported cases in the medical literature to address these. This causes oxygenrich blood to flow from the left ventricle to the right ventricle and mix with oxygenpoor blood. May 16, 2018 double outlet right ventricle dorv is a congenital heart disease in which the aorta and pulmonary artery rise from the right ventricle. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Mahle, richard martinez, norman silverman, meryl s. The aorta connects to the right ventricle rv, the chamber of the heart that pumps oxygenpoor blood to the lungs, instead of to the left ventricle lv, the chamber that normally pumps oxygenrich blood to the body. Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect vsd by vsd rerouting to the pulmonary artery and arterial switch eur j cardiothorac surg 2002.
Together, the pulmonary artery and the aorta are known as the great arteries. Between 1993 and 2011, 36 consecutive patients presenting with doubleoutlet right ventricle or. Among the 31 cases the vsd was located in one of four locations. Chop has its own delivery unit, on the same floor as cardiac operating rooms and cardiac patient care units. In double outlet right ventricle dorv the two great arteries aorta and pulmonary artery both originate from the right ventricle and blood from the left ventricle passes across a vsd into the rv to reach the great arteries. Double outlet right ventricle is a very complex congenital heart defect. Pdf double outlet right ventricle dorv, a clinically significant congenital heart defect.
Pdf download for surgical treatment for double outlet right. Double outlet right ventricle dorv ist ein angeborener herzfehler mit einem gro. Anatomy, echocardiography, and surgical approach to double. Double outlet right ventricle dorv is a heart defect where the aorta connects to the heart in the wrong place. Double outlet right ventricle with giant pulmonary artery. In the normal heart, the pulmonary artery pa arises from the right ventricle and carries deoxygenated blue blood to the. A case of doubleoutlet right ventricle, unbalanced complete atrioventricular canal, hypoplastic aortic arch, coarctation of the aorta, and atrioventricular valve regurgitation was treated with a staged approach. Doubleoutlet right ventricle with complete atrioventricular. Double outlet right ventricle dorv is a particular mode of ventriculoarterial connection. Double outlet right ventricle the pediatric cardiac. Double outlet right ventricle dorv is among a group of complex cardiovascular pathologies that share the common feature fig. The management of doubleoutlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge.
Is anatomical repair advantageous seminars in thoracic and cardiovascular surgery, vol. Doubleoutlet right and left ventricles thoracic key. Bilateral infundibula and ventricular septal defects are highly. Parasternal longaxis scan in a patient with doubleoutlet right ventricle, with the aorta anterior and right of the pulmonary artery with a subpulmonary ventricular septal defect vsd. Introduction 1793 john abernethy 1st described the condition. Double outlet right ventricle dorv is a congenital heart disease in which the aorta and pulmonary artery rise from the right ventricle. Double outlet right ventricle dorv is a complex cardiac malformation with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily from the right ventricle rv, typically associated with discontinuity between the aorta and mitral valve. Surgical treatment for double outlet right ventricle with pulmonary. In double outlet right ventricle, the aorta and pulmonary artery connect partially or completely to the right ventricle. The fetal heart program at childrens hospital can monitor both baby and mother throughout the pregnancy and create a plan for labor and care after the birth. There is a relatively small amount of conus tissue arrow separating the pulmonary valve pv from the mitral valve mv. Double outlet right ventricle dorv is a type of ventriculoarterial connection in which both great arteries arise either entirely or predominantly from the right ventricle. A case of double outlet right ventricle, unbalanced complete atrioventricular canal, hypoplastic aortic arch, coarctation of the aorta, and atrioventricular valve regurgitation was treated with a staged approach. Essential modifiers of double outlet right ventricle circulation.
Double outlet right ventricle cardiology in the young. Download pdf pdf download for double outlet right ventricle with intact. If one defines double outlet right ventricle as the absence of aorticmitral valve fibrous continuity, then. In a normal heart, the pulmonary artery sends oxygenpoor blood from the right ventricle into the lungs, and the aorta sends oxygenrich blood from the left ventricle to the rest of the body. After some dispute, its existence was finally verified intraoperatively and in autopsy series.
Double outlet from the right ventricle is the normal situation in the early embryo, and a lack of transfer of the aorta into the left ventricle redefines the arrangement as a malformation 6,7. Anatomy, echocardiography, and surgical approach to double outlet right ventricle volume 18 supplement william t. Modified rastelli procedure is an optimal method for surgically treating double outlet right ventricle with leftmalposition of the great arteries. Double outlet right ventricle an overview sciencedirect. Doubleoutlet right ventricle is a form of ventriculoarterial connection. A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with double outlet right ventricle. Double outlet right ventricle childrens hospital of. Generally, an ebook can be downloaded in five minutes or less. Anderson, double outlet right ventricle, european journal of cardiothoracic surgery, volume 22, issue 5, november 2002. In a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the. Doubleoutlet right ventricle is a complex lesion that accounts for many anatomic variants leading to different physiologic presentations and a varied number of surgical approaches. Doubleoutlet right ventricle dorv is a relatively rare congenital cardiovascular anomaly that is now receiving more clinical attention because it is surgically correctable.
The definition formulated by the international society for nomenclature of paediatric and congenital heart disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. The body is not able to get enough oxygen, causing the heart to work harder to try to bring more oxygenrich blood to the body. Double outlet right ventricle dorv is a rare congenital disorder that has a yet to be identified cause and or predictive factor. Double outlet right ventricle dorv boston childrens. Double outlet right ventricle dorv is a heart disease that is present from birth congenital. Double outlet right ventricle is a form of ventriculoarterial connection. In segmental approach to congenital heart diseases, there are 3 facets to define. Dec 12, 2014 for a layman double outlet right ventricle is a congenital heart defect.
Pdf double outlet right ventricle deborah kozik, jai. Thirtytwo patients with double outlet right ventricle dorv were studied between 1960 and 1976. Doubleoutlet right ventricle revisited the journal of. Dorv may also occur with atrioventricular septal defect that often extends toward one or both outflow tracts figure 5. Jan 10, 2018 double outlet right ventricle dorv is a heterogeneous group of congenital heart diseases that require individualized surgical approach based on precise understanding of the complex cardiovascular anatomy. Double outlet right ventricle texas heart institute. Biventricular repair of doubleoutlet right ventricle with. For a layman double outlet right ventricle is a congenital heart defect.
The necropsy specimens of 33 examples of double outlet right ventricle were studied. Double outlet right ventricle dorv has long been a contentious topic in congenital heart pathology and surgery. The defect involves having both the pulmonary artery and the aorta come off the right ventricle. For the anesthesiologist caring for patients with this diagnosis, many of these anatomic factors will impact the patients clinical presentation, the physiologic. Cardiovascular system double outlet right ventricle.
Doubleoutlet right ventricle with noncommitted ventricular septal defect and 2 adequate ventricles. Doubleoutlet right ventricle is not hypoplastic left. In some cases, double outlet right ventricle is diagnosed prenatally. In a normal heart, as shown on the left, the pulmonary artery connects to the right ventricle and the aorta connects to the left ventricle. No arteries, or only a part of the aorta, are attached to the left ventricle. Associated congenital defects frequently compounded the difficulty of clinical diagnosis. It exhibits a wide spectrum of anatomic and physiologic variations based on the relationship of the ventricular septal defect vsd with the great vessels and the presence or absence of right ventricular outflow tract rvot obstruction. Patients require a variety of surgical interventions, and the long.
Double inlet right ventricle versus other types of double or common inlet ventricle. It is associated with trisomy and 18 but only rarely with chromosome 22q11 deletions. Doubleoutlet right ventricle dorv is a particular mode of ventriculoarterial connection. Cardiac catheterisation was performed in 27 patients, and the ventriculograms. At 4 days of life the patient underwent aortic arch repair, atrial septectomy, common atrioventricular valve regurgitation repair, and pulmonary artery banding. Pdf double outlet right ventricle is a rare congenital cardiac malformation that appears to result from lack of conotruncal inversion, failure of. When a baby is still in the womb, something goes wrong during the formation of the heart and both great arteries are attached to the right ventricle. The necropsy specimens of 33 examples of doubleoutlet right ventricle were studied. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling. Aug 15, 2006 double outlet right ventricle with noncommitted ventricular septal defect and 2 adequate ventricles.
A hole also exists between the two ventricles ventricular septal defect. In contrast to double outlet right ventricle, dolv is a rarity. Double outlet right ventricle dorv is a rare form of congenital heart disease. Doubleoutlet left ventricle with ventricular septal defect and pulmonary stenosis. This configuration allows oxygenpoor blood, to be carried throughout the body. Request pdf double outlet right ventricle double outlet right ventricle is a type of anomalous ventriculoarterial connection that represents 0. Doubleoutlet right ventricle is not hypoplastic left heart. A ninedayold boy born at 38 weeks with a birth weight of 3200 g was referred due to cyanosis and murmur. A clear understanding of the anatomy is critical to understanding the physiologic consequences of the specific type of dorv. In contrast to other major congenital heart defects, there are no systematic or comprehensive data regarding associations, aetiologies, and pathogenesis of dorv. Oblique sagittal 2dimensional mip image a and 3dimensional volume rendered left lateral oblique projection b demonstrate double outlet right ventricle, hypoplastic aortic arch open arrow, aortic.
Kiser jc, ongley pa, kirklin jw, clarkson pm, mcgoon dc. Double outlet right ventricle dorv is a conotruncal anomaly that. Double outlet right ventricle dorv for parents nemours. Doubleoutlet right ventricle is defined as an abnormal va connection in which more than half of both the aorta and the pulmonary artery arise from the morphologic rv. In 31 of the 33 cases, a ventricular septal defect vsd was present, and in two the ventricular septum was intact. Double outlet right ventricle genetic and rare diseases.
According to the literature, the frequency of this disease is about 1. Double outlet right ventricle dorv is a rare congenital heart defect, meaning its a condition a baby is born with. Double outlet right ventricle is defined as an abnormal va connection in which more than half of both the aorta and the pulmonary artery arise from the morphologic rv. Doubleoutlet right ventricle is not hypoplastic left heart syndrome we write to express our concerns that the report the complex genetics of hypoplastic left heart syndrome by liu et al. The lung circulation is often exposed to very high pressure and increased blood flow as with a large vsd. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Double outlet right ventricle dorv is a form of congenital heart disease where both of the great arteries connect in whole or in part to the right ventricle rv. Surgical treatment of dextrocardia with inversion of. The diagnosis of doubleoutlet right ventricle dorv characterizes a complex heterogeneous group of congenital cardiac malformations for which multiple classification schemes have been used. The surgical management of patients with doubleoutlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a.