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Double Outlet Right Ventricle

Double outlet right ventricle is a congenital heart disease in which the pulmonary trunk and the aorta both arise from the right ventricle, with several anatomical variations and additional cardiac malformations. Hypoxia and other accompanying manifestations may be life-threatening. Echocardiography and other imaging studies of the heart and major vessels are used to confirm double outlet right ventricle.


Presentation

In double outlet right ventricle (DORV), the pulmonary trunk and a significant portion (> 50%) of the aorta (or sometimes the entire vessel) stem from the right ventricle, resulting in the transfer of primarily deoxygenated blood into systemic circulation [1] [2] [3] [4]. The severity of manifestations significantly depends on the presence of various accompanying defects, most notable being atrial septal defect (ASD), stenosis of the pulmonary valve, hypoplasia (or hypertrophy) of the left ventricle, mitral atresia, mitral stenosis, and coarctation of the aorta [5] [6]. In virtually all cases, however, a ventricular septal defect (VSD), arising as a compensatory mechanism of the heart, almost universally develops, but its location (subaortic, subpulmonary, non-committed or doubly-committed) may determine the amount of oxygenated blood that reaches the right ventricle from the left heart [2] [3]. Moreover, rare cases with an intact ventricular septum have been described [3]. Regardless, one of the most important clinical presentations of this rare congenital heart disease (CHD) is the onset of cyanosis during the first few months of life, often characterized by hypoxic spells, whereas tachycardia, tachypnea, sweating, deprivation of growth and inability to gain weight due to poor feeding and lethargy are additional features [3] [5] [6] [7]. Infants are often unresponsive and extremely tired after minor tasks, which are followed by marked increases in heart and respiration rate [7]. Arrhythmias, pulmonary hypertension and the formation of aneurysms are known complications that can be life-threatening [5].

Fatigue
  • Abnormal heartbeat, a heart murmur, sweating and fatigue are other possible symptoms. What are double outlet right ventricle care options? In most cases, double outlet right ventricle can be repaired with one or more surgical procedures.[nicklauschildrens.org]
  • […] babies include: rapid breathing rapid heartbeat sweating disinterest in feeding or tiring while feeding poor weight gain blue color of the skin, lips and nail beds ( cyanosis ) heart murmur (detected by doctor) In older children, symptoms may include: fatigue[childrenshospital.org]
  • […] include: A heart murmur (an extra, unusual heart sound) Breathing problems, including difficulty breathing, shortness of breath, or rapid breathing Difficulty eating and/or gaining weight Blueness of the lips, nails, and/or skin (cyanosis) Sweating Fatigue[my.clevelandclinic.org]
  • Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms.[icdlist.com]
  • Other sypmtoms include fatigue, difficulty breathing, and cyanosis. These symptoms are not specific for RVOT.[wikidoc.org]
Rigor
  • Much of the most rigorous genetic and developmental study of normal cardiac development and of the genesis of DORV has come from recent animal studies. 56 There are, however, two important limitations of the animal studies that must be considered in the[doi.org]
Aspiration
  • In this report, we are presenting a 2-week-old preterm girl presented with frequent aspiration pneumonitis, cyanosis, and heart murmur. Mechanical ventilation was extremely difficult because of endotracheal tube air leak and excessive secretions.[ncbi.nlm.nih.gov]
Failure to Thrive
  • These infants may also have difficulties breathing as well as poor feeding and a general failure to thrive. In severe cases, infants may have congestive heart failure, which requires immediate and aggressive therapy.[news-medical.net]
  • Your child may have symptoms like these: Bluish skin, lips or nailbeds (cyanosis) or pale skin Trouble feeding Failure to thrive Fast breathing or working hard to breathe Sweating more than normal Diagnosing Double Outlet Right Ventricle To diagnose this[seattlechildrens.org]
  • Symptoms include feeding difficulties and as a consequence poor weight gain, failure to thrive. Other sypmtoms include fatigue, difficulty breathing, and cyanosis. These symptoms are not specific for RVOT.[wikidoc.org]
  • Feeding tolerance, weight gain, breathing problems, and a general failure to thrive should be elucidated.[emedicine.medscape.com]
Heart Disease
  • Double outlet right ventricle (DORV) is an uncommon congenital heart disease with a poor prognosis.[ncbi.nlm.nih.gov]
Cyanosis
  • Herein, a newborn with cyanosis and murmur diagnosed as double outlet right ventricle with intact ventricular septum is reported.[ncbi.nlm.nih.gov]
  • Physical examination can reveal tachycardia, a heart murmur, tachypnea and signs of central cyanosis, after which echocardiography, the gold standard in detecting CHD, should be employed.[symptoma.com]
Back Pain
  • A 20-year-old male patient with palpitations, tachypnea, and tiredness with weak exercise capacity and back pain was diagnosed with heart murmur in different degrees; slight cyanosis, cachexia, and developmental retardation were found clinically.[ncbi.nlm.nih.gov]
Lethargy
  • […] congenital heart disease (CHD) is the onset of cyanosis during the first few months of life, often characterized by hypoxic spells, whereas tachycardia, tachypnea, sweating, deprivation of growth and inability to gain weight due to poor feeding and lethargy[symptoma.com]

Workup

The appearance of cyanosis during the neonatal period or infancy in the absence of an obvious cause must raise clinical suspicion to a CHD. This illustrates the importance of a detailed patient history and a proper physical examination. Firstly, the onset of symptoms and their course must be determined with the parents, as patients are mainly too young to provide adequate information. Family history is also an important component of the workup, as preexisting congenital malformations (but also other congenital diseases) within the family seem to increase the risk for many CHDs, including DORV. In addition, maternal use of antidepressants, such as selective serotonin reuptake inhibitors (SSRIs) during pregnancy, was also confirmed as a risk factor [7]. Physical examination can reveal tachycardia, a heart murmur, tachypnea and signs of central cyanosis, after which echocardiography, the gold standard in detecting CHD, should be employed [2] [8]. Echocardiography is highly useful due to the fact that it can detect CHD both prenatally and postnatally. Supplementary to traditional transthoracic 2D cross-sectional cardiac ultrasonography, limited by the experience of the physician and a very narrow field of observation, the introduction of 3D echocardiography has greatly improved the diagnostic accuracy, and is performed whenever possible and feasible [1] [8]. However, digital subtraction angiography (DSA), an invasive procedure carrying potential for catheter-related complications, and electrocardiographically (ECG)-gated dual-source computed tomography (DSCT), used in the pediatric population without arrhythmias, have been postulated as most reliable methods when it comes to detection of DORV [2].

Right Axis Deviation
  • ECG revealed gross right axis deviation and right ventricular hypertrophy. Echocardiogram showed features of double outlet right ventricle.[ncbi.nlm.nih.gov]
  • As a result of pressure and volume overload, there are signs of RV hypertrophy and there is a right axis deviation. There are a lot of ECG variations depending on the variety of DORV the presence of associated defects.[wikidoc.org]
  • Electrocardiogram • NSR • PR Prolongation • Atrial electrical abnormality • Counter clock loop • Clockwise QRS • RVH • Right axis deviation 20. Clinical classification • The More Common Types • A.[slideshare.net]
  • The electrocardiogram is notable for right axis deviation and a right ventricular hypertrophy pattern, with rR′, qR, or rsR′ pattern; these findings are not, however, sufficiently specific to be diagnostic.[thoracickey.com]
  • ECG The RV will be hypertrophied with right axis deviation and possible RAE. CXR Mild or no pulmonary stenosis will cause increase pulmonary blood flow resulting in cardiomegaly with increase pulmonary vascular markings.[pedcard.rush.edu]

Treatment

  • We report our experience with surgical treatment for DORV (as defined by the "90% rule") with pulmonary outflow tract obstruction (POTO).[ncbi.nlm.nih.gov]
  • Surgery is generally the treatment of choice, but there are several complications that may be associated with DORV.[news-medical.net]
  • We feel that biventricular repair of DORV-AVSD is the preferred treatment option, although further studies are needed to define longterm outcomes.[experts.umich.edu]

Prognosis

  • Double outlet right ventricle (DORV) is an uncommon congenital heart disease with a poor prognosis.[ncbi.nlm.nih.gov]
  • Prognosis and follow-up Not withstanding the potential complications that may arise, enormous strides in our understanding, diagnosis, and treatment of the condition make it possible for most people born with DORV to lead relatively normal and productive[news-medical.net]
  • Prognosis for infants born with DORV and transposition of the great arteries is generally good, although it is dependent on specific anatomy.[emedicine.com]
  • Factors that determine the type and number of operations the baby needs include: The type of DORV The severity of the defect The presence of other problems in the heart The childs overall condition Outlook (Prognosis) How well the baby does depends on[indiatoday.intoday.in]

Etiology

  • Pathophysiology & Etiology The pathophysiology of the DORV is quite variable [6].[wikidoc.org]
  • Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease. [4] Etiology No specific causal agent or predictive event has been identified.[emedicine.medscape.com]
  • Etiology DORV is thought to be the result of a malformation in the outlet portion of the embryonic ventricular loop at 3-4 weeks' gestation. Although mostly sporadic, familial cases have been reported.[emedicine.com]

Epidemiology

  • We queried the PubMed database using key words "double outlet right ventricle" and "DORV" for case reports, epidemiologic analyses and animal studies with this cardiac anomaly.[ncbi.nlm.nih.gov]
  • Both epidemiologic analyses of congenital heart disease and case reports were used.[doi.org]
  • Search term: Taussig-Bing malformation Taussig-Bing malformation International Classification of Diseases The International Classification of Diseases (ICD) World Health Organization's classification used worldwide as the standard diagnostic tool for epidemiology[embryology.med.unsw.edu.au]
  • 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 arch—particularly at the transposition end of the spectrum, right[slideshare.net]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of double-outlet right ventricle and its anesthetic implications for tracheoesophageal fistula repair are discussed.[ncbi.nlm.nih.gov]
  • (Click picture to show/hide bloodflows) Pathophysiology The hemodynamics involved with double outlet right ventricle are dependent on the anatomy of the great vessels and associated defects.[vhlab.umn.edu]
  • The cardiovascular pathophysiology is determined predominantly by the degree of PS, which generally increases over time in early infancy.[thoracickey.com]
  • Pathophysiologic description and classification is accomplished by relating the location of the VSD to the arrangement of the great vessels. Each combination results in a physiologic behavior similar to that of other CHDs.[emedicine.com]
  • The pathophysiology depends on the degree pulmonary stenosis. With pulmonary stenosis, the pulmonary blood flow is decreased with variable degrees of cyanosis (TOF type).[emedicine.medscape.com]

Prevention

  • Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age.[nemours.org]
  • This prevents infections around the heart. Antibiotics may also be needed after surgery. Call your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips.[nlm.nih.gov]
  • This prevents infections around the heart. Antibiotics may also be needed after surgery. When to Contact a Medical Professional Call your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips.[indiatoday.intoday.in]

References

Article

  1. Zidere V, Pushparajah K, Allan LD, Simpson JM. Three-dimensional fetal echocardiography for prediction of postnatal surgical approach in double outlet right ventricle: a pilot study. Ultrasound Obstet Gynecol. 2013;42(4):421-425.
  2. Shi K, Yang Z, Chen J, Zhang G, Xu H, Guo Y. Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography. Dettman RW, ed. PLoS ONE. 2015;10(6):e0130987.
  3. Li S, Ma K, Hu S, et al. Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg. 2014;148(3):817-824.
  4. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
  5. Lin CH, Huddleston C, Balzer DT. Transcatheter Ventricular Septal Defect (VSD) Creation for Restrictive VSD in Double-Outlet Right Ventricle. Pediatr Cardiol. 2013;34(3):743-747.
  6. Özgür S, Ceylan Ö, Doğan V, Örün UA. Double outlet right ventricle with intact ventricular septum. Turk Kardiyol Dern Ars. 2014;42(2):190-193.
  7. Baig SA, Saeed Z, Fatima A, Chagani H, Aziz S. Double outlet right ventricle, atrioventricular canal defect with severe pulmonary artery stenosis: a rare cyanotic congenital heart disease. J Pak Med Assoc. 2014;64(8):980-982.
  8. Gelehrter S, Owens ST, Russell MW, van der Velde ME, Gomez-Fifer C. Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. 2007;2(1):32-37.

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Last updated: 2019-07-11 20:13