Presentation
Median age at presentation was 2 days (birth--5.5 years). [ncbi.nlm.nih.gov]
[…] if the condition was present at the time of inpatient admission. [icd.codes]
Median age at presentation was 2 days (birth—5.5 years). [scipers.com]
Its pre-natal diagnosis warrants utmost importance provided its non-apparent presentation. This is the first case of this magnitude being reported and presented in Pakistan. [apicareonline.com]
Entire Body System
- Poor Feeding
These infants may also show signs of being easily fatigued with difficulties in breathing and gaining weight as well as poor feeding and a general failure in their ability to thrive. [news-medical.net]
feeding from becoming tired easily Bluish color of the skin and lips Clubbing (thickening of the nail beds) of toes and fingers (late sign) Failure to gain weight and grow Pale coloring Sweating Swollen legs or abdomen Trouble breathing Treatment requires [medlineplus.gov]
feeding from becoming tired easily Bluish color of the skin and lips Clubbing (thickening of the nail beds) of toes and fingers (late sign) Failure to gain weight and grow Pale coloring Sweating Swollen legs or abdomen Trouble breathing Exams and Tests [ufhealth.org]
- Congestive Heart Failure
The two major symptoms are congestive heart failure and cyanosis. In some cases, cyanosis can become severe because of reduced pulmonary blood flow. The goal of the surgical treatment of DORV is a complete anatomic repair. [pediatriccardiacinquest.mb.ca]
On the other hand, congestive heart failure is seen in patients who have an excessive amount of blood under high pressure being pumped into the lungs. Management of DORV and DILV In both cases, surgery is the only option. [news-medical.net]
Decreased arterial oxygen saturation is almost always present, but as a result of wide variation in anatomy, patients may or may not appear cyanotic and/or develop congestive heart failure. Patients may even be asymptomatic altogether. [vhlab.umn.edu]
Increased pulmonary blood flow results in volume overload of the left ventricle, congestive heart failure, and poor growth and development. [clinicalgate.com]
- Weight Gain
Common symptoms of double outlet right ventricle (DORV) in 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 [childrenshospital.org]
Babies who have DORV may show these symptoms: Tiring easily, especially when feeding Bluish color of the skin and lips Clubbing (thickening of the nail beds) of the fingers and toes Poor growth or poor weight gain Pale skin Sweating Difficulty breathing [fetaltonewborn.org]
In most cases, children will have symptoms in the first days or weeks of life, including: Blue or purple tint to lips, skin and nails (cyanosis) Poor eating and poor weight gain Rapid breathing or shortness of breath Profuse sweating, especially with [chop.edu]
- Swelling
Failure to thrive and gain weight Pallor of the skin Swelling or edema of the legs and the abdomen Sweating Breathing problems due to lack of oxygen or excessive pulmonary blood flow, such as breathlessness References https://medlineplus.gov/ency/article [news-medical.net]
Respiratoric
- Tachypnea
Clinical description DOLV usually manifests during the neonatal period with cyanosis, tachypnea, exertional dyspnea, failure to thrive, sweating (especially during feeding), unresponsiveness and/or fatigue. [orpha.net]
Gastrointestinal
- Failure to Thrive
Clinical description DOLV usually manifests during the neonatal period with cyanosis, tachypnea, exertional dyspnea, failure to thrive, sweating (especially during feeding), unresponsiveness and/or fatigue. [orpha.net]
Other complications such as difficulty breathing, poor feeding, and generally a failure to thrive are not uncommon. A common accompanying structural cardiac pathology with DORV is ventricular septal defects (VSD). [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]
- Regurgitation
[…] and tricuspid stenosis Tricuspid regurgitation, non-Ebstein's related Tricuspid valve, Other Truncal valve insufficiency Vascular ring VSD, Multiple VSD, NOS VSD, Single Category 2 Aneurysm, Ventricular, Left Aortic aneurysm (including pseudoaneurysm [resources.pmidcalc.org]
Most patients will continue to present with residual cardiac anomalies, such as aortic or mitral valve regurgitation, arrhythmias or hypertension. The documents contained in this web site are presented for information purposes only. [orpha.net]
[…] auscultatory signs are analogous to Eisenmenger’s syndrome with an isolated nonrestrictive ventricular septal defect: namely, a pulmonary ejection sound, a loud single second heart sound, and a Graham Steell murmur of pulmonary hypertensive pulmonary regurgitation [clinicalgate.com]
Cardiovascular
- Heart Failure
Postoperative right heart failure in both patients is explained by the impaired function of the right ventricle being exposed to gross pulmonary incompetence or by the persistence of elevated right ventricular pressure. [ncbi.nlm.nih.gov]
[…] disease; cardiac rhythm disturbances; heart failure and transplantation; disease of the thoracic aorta; tetralogy of Fallot; anesthesia; and postoperative care. [books.google.com]
There were three (8%) hospital deaths, due to right heart failure, and seven (21%) patients experienced 10 perioperative complications. [journals.lww.com]
Prognosis DOLV is associated with high mortality, generally due to heart failure, myocardial infarction or aortic thrombosis. With surgery, the five-year survival rate is estimated at 70-75%. [orpha.net]
- Heart Murmur
Cyanotic heart disease (Medical Encyclopedia) Dextrocardia (Medical Encyclopedia) Echocardiogram -- children (Medical Encyclopedia) Heart murmurs and other sounds (Medical Encyclopedia) Patent ductus arteriosus (Medical Encyclopedia) Ventricular septal [icdlist.com]
While examining your baby, your doctor will first listen for a heart murmur with a stethoscope. If your doctor hears a heart murmur or if your baby is showing symptoms of DORV, tests may be done to confirm the diagnosis. [fetaltonewborn.org]
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]
Common symptoms of double outlet right ventricle (DORV) in 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 [childrenshospital.org]
Symptoms may 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 [my.clevelandclinic.org]
Skin
- Sweating
Clinical description DOLV usually manifests during the neonatal period with cyanosis, tachypnea, exertional dyspnea, failure to thrive, sweating (especially during feeding), unresponsiveness and/or fatigue. [orpha.net]
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]
Common symptoms of double outlet right ventricle (DORV) in 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 [childrenshospital.org]
Symptoms may 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 [my.clevelandclinic.org]
Babies who have DORV may show these symptoms: Tiring easily, especially when feeding Bluish color of the skin and lips Clubbing (thickening of the nail beds) of the fingers and toes Poor growth or poor weight gain Pale skin Sweating Difficulty breathing [fetaltonewborn.org]
Workup
Workup There are several approaches that may be used to diagnose DORV, which in some cases can be done even before the baby is born, prenatally. [news-medical.net]
Treatment
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. [icdlist.com]
Diagnosis and Treatment of Double Outlet Right Ventricle. News-Medical, viewed 02 November 2022, https://www.news-medical.net/health/Diagnosis-and-Treatment-of-Double-Outlet-Right-Ventricle.aspx. [news-medical.net]
To our knowledge, this represents the second instance of surgical treatment of double-outlet left ventricle [I,D,D] reported in the literature. [ncbi.nlm.nih.gov]
Management and treatment Biventricular repair is the usual treatment for DOLV and is usually accomplished by an intraventricular baffle procedure, by the Rastelli procedure or by a variant of the Lecompte operation. [orpha.net]
Prognosis
The prognosis after this operation for single ventricle patients is better than for other treatments, and improvements of surgical technique continue to be made. [chla.congenital.org]
Prognosis DOLV is associated with high mortality, generally due to heart failure, myocardial infarction or aortic thrombosis. With surgery, the five-year survival rate is estimated at 70-75%. [orpha.net]
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 child's overall condition Outlook (Prognosis) How well the baby does depends on [ufhealth.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Etiology The etiopathology is not clearly understood. [orpha.net]
Double inlet left ventricle (DILV) is a rare condition and its exact etiology is currently unknown. It occurs in 5 to 10 out of 100 000 births. [news-medical.net]
Epidemiology
Summary Epidemiology Prevalence is unknown. The birth prevalence is estimated at less than 1/ 200,000 live births. [orpha.net]
Epidemiology [ edit ] DORV affects between 1% and 3% of people born with congenital heart defects. [1] Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature. [1] References [ edit ] ^ a b Obler D, Juraszek AL [en.wikipedia.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]
Pathophysiology
Anatomy and pathophysiology [ edit ] DORV occurs in multiple forms, with variability of great artery position and size, as well as of ventricular septal defect (VSD) location. It can occur with or without transposition of the great arteries. [en.wikipedia.org]
(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]
Prevention
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. [medlineplus.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. [ufhealth.org]
Table 19-1 Double Outlet Right Ventricle: Clinical Classification The aorta is either to the right of and anterior to the pulmonary trunk or side-by-side with the aorta to the right. 9 Each great artery arises above a conus that prevents fibrous continuity [clinicalgate.com]