Taussig Bing anomaly (named after the authors that first described it- Helen B. Taussig and Richard J. Bing) is a cyanotic congenital heart disease characterized by the dual presence of a subpulmonic ventricular septal defect (VSD) along with a double outlet right ventricle (DORV).
Presentation
Taussig Bing anomaly was initially described in 1949 in a 5.5-year-old girl who died after injection of contrast during angiocardiography. An autopsy revealed a peculiar structural abnormality of the heart- both the aorta and the pulmonary artery originated from the right ventricle. At the same time, a subpulmonic ventricular septal defect (VSD) was observed and the right ventricle was hypertrophic [1] [2].
Hemodynamic changes seen in the Taussig Bing anomaly include:
- blood flow from the left ventricle (LV) through the VSD to the pulmonary artery preferentially
- blood flow from the right ventricle (RV) through the aorta preferentially
Another important characteristic of this disease is the absence of continuity of the mitral and semilunar valves [3] [4] [5]. This feature differentiates this condition from transposition with VSD and an overriding pulmonary artery.
As a consequence of this malformation, very small quantities of oxygenated blood enter the systemic circulation. This results in low oxygen saturation and cyanosis, which may be present at birth. Dyspnea at rest and developmental delay are the other typical features seen. Compensatory erythrocytosis may also be observed.
Death in the first described patient of the Taussig Bing anomaly, occurring after rapid injection of contrast material in the superior vena cava, was due to the elevation in pressure of the right side of the heart. This decreased the left-to-right shunt, leading to extremely low volumes of oxygenated blood being pumped towards the systemic circulation [6].
Entire Body System
- Surgical Procedure
Procedures / methods*, mortality From MEDLINE®/PubMed®, a database of the U.S. [biomedsearch.com]
KEYWORDS: Bing RJ; Taussig HB; cardiac surgical procedures/history/methods; cardiology/history; coronary vessel anomalies/surgery; double-outlet right ventricle/complications/surgery; heart catheterization/history; heart defects, congenital/history/surgery [ncbi.nlm.nih.gov]
Keywords: Multivariate Analysis, Hospital Mortality, Morbidity, Transposition of Great Vessels, Confidence Intervals, Double Outlet Right Ventricle, Cardiac Surgical Procedures, New York, Heart Ventricles, Heart Septal Defects < Back to Listings [acc.org]
View in: PubMed subject areas Aortic Coarctation Cardiac Surgical Procedures Child Child, Preschool Double Outlet Right Ventricle Female Heart Septal Defects, Ventricular Humans Infant Infant, Newborn Male Pulmonary Artery [profiles.uchicago.edu]
- Pulmonary Valve Stenosis
The presence of pulmonary valve stenosis also affects the condition. [ufhealth.org]
Another factor in determining the type of DORV is whether or not the condition includes pulmonary valve stenosis and how extensive the stenosis is. In addition, an infant can have DORV along with other types of congenital heart defects. [my.clevelandclinic.org]
- Congestive Heart Failure
Chest X-ray showed congestive heart failure. We decided to perform arterial switch operation (ASO) and VSD closure. Operation was performed via a re-median sternotomy. [hero.epa.gov]
All three had congestive heart failure. This could be expected since this disorder adds an additional left-to-right shunt to that already existent, through the septal defect and thus adds to the work of the right heart. [kundoc.com]
heart failure, ventricular hypertrophy, and pulmonary vascular disease. 7, 11 To our knowledge, we have identified one of the largest reported cohorts of consecutive patients with Taussig–Bing anomaly to undergo single-stage repair with primary ASO. [circ.ahajournals.org]
Fourteen patients were in congestive heart failure controlled with diuretics and four patients were moderately cyanosed (Sat n O 2 ≪85%). Amongst all patients, 14 had emergency balloon atrial septostomy (BAS) at time of admission. [academic.oup.com]
- Cyanotic Congenital Heart Disease
Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease. [ncbi.nlm.nih.gov]
Surgery indicated for relief of cyanotic congenital heart disease and PGE-1-dependent systemic blood flow. [ctsnet.org]
Surgery indicated for relief of cyanotic congenital heart disease and PGE-1-dependent systemic blood flow. The procedure was as follows: ventricular septal defect baffle closure, ascending aorta and arch reconstruction, and arterial switch operation. [ctsnet.figshare.com]
Engle M (1976) Cyanotic congenital heart disease, The American Journal of Cardiology, 10.1016/0002-9149(76)90324-6, 37:2, (283-308), Online publication date: 1-Feb-1976. [ahajournals.org]
Bing) is a cyanotic congenital heart disease characterized by the dual presence of a subpulmonic ventricular septal defect (VSD) along with a double outlet right ventricle (DORV). [symptoma.com]
Respiratoric
- Dyspnea
A 22-year-old male was admitted to our hospital due to persistent lower leg edema and dyspnea. He had been diagnosed with TBA. On physical examination, his peripheral oxygen saturation (Spo2) was 73% in room air. [hero.epa.gov]
Abstract The patient was a 30-day-old female infant with symptoms of severe dyspnea and cyanosis, when she was admitted to the ER. The echocardiography revealed DORV with subpulmonary VSD, and the diagnosis of Taussig-Bing anomaly was made. [koreascience.kr]
Dyspnea at rest and developmental delay are the other typical features seen. Compensatory erythrocytosis may also be observed. [symptoma.com]
A 23-year-old primigravida at term, presented with grade III dyspnea (New York Heart Association grading). [aeronline.org]
- Tachypnea
On examination, the child had tachycardia and tachypnea with oxygen saturation of 77% on room air. The precordium was hyper dynamic and a systolic murmur was auscultated. The chest radiogram revealed cardiomegaly and pulmonary plethora. [annalspc.com]
Difficulty breathing, such as tachypnea (really fast breathing) or shortness of breath. Difficulty eating or gaining weight. Heart murmur (extra sound in the heartbeat, which a healthcare provider can hear with a stethoscope). [my.clevelandclinic.org]
Case report Nine month male baby was admitted with fever and tachypnea. She was product of non-consanguineous marriage and was delivered normally. She weighed 3.9 kg while at birth 2.2 kg. [journals.viamedica.pl]
Cardiovascular
- Heart Disease
Bichell D (2019) Double-Outlet Right Ventricle Critical Heart Disease in Infants and Children, 10.1016/B978-1-4557-0760-7.00058-9, (694-704.e3),. [ahajournals.org]
Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease. [ncbi.nlm.nih.gov]
Conference Proceedings - Journal of Cholesterol and Heart Disease (2020) Volume 0, Issue 0 ? Taussig-Bing heart is one of the conotruncal malformation. [alliedacademies.org]
References SHOWING 1-9 OF 9 REFERENCES Detorsion defects in congenital cardiac anomalies: Report of three cases, with analysis of mechanisms of their formation Arch Path 1930 Congenital Heart Disease. London Congenital Heart Disease. [semanticscholar.org]
Diagnosis and Management of Adult Congenital Heart Disease, by Drs. Gatzoulis, Webb, and Daubeney, is a practical, one-stop resource designed to help you manage the unique challenges of treating long-term adult survivors of congenital heart disease. [books.google.com]
- Heart Failure
Chest X-ray showed congestive heart failure. We decided to perform arterial switch operation (ASO) and VSD closure. Operation was performed via a re-median sternotomy. [hero.epa.gov]
Failure Treatment study.CONCLUSIONS: Increased UACR is common in patients with heart failure, including non-diabetics. [read.qxmd.com]
After initially successful arterial switch, a high-grade stenosis of the singular coronary artery leads to a severe biventricular heart failure 5 weeks after the procedure. [ncbi.nlm.nih.gov]
Material and methods: One-month old patient, with heart failure. Diagnosed by echocardiography as Taussig-Bing heart. [medigraphic.com]
- Cyanosis
Abstract The patient was a 30-day-old female infant with symptoms of severe dyspnea and cyanosis, when she was admitted to the ER. The echocardiography revealed DORV with subpulmonary VSD, and the diagnosis of Taussig-Bing anomaly was made. [koreascience.kr]
We report a 6 years old male child, presented with difficulty in swallowing, crying and smiling from early infancy and recurrent episodes of cyanosis on exertion for about 2 years. [ncbi.nlm.nih.gov]
In Taussig-Bing anomaly, as the flow is from RV to aorta and LV blood preferentially enters the lung, cyanosis dates since birth. Consequently, neonates typically present with cyanosis, systolic murmur and heart failure. [journals.viamedica.pl]
A difference in the degree of hypoxemia/cyanosis in different extremities as a result of the site of a right to left shunt. [cachnet.org]
This results in low oxygen saturation and cyanosis, which may be present at birth. Dyspnea at rest and developmental delay are the other typical features seen. Compensatory erythrocytosis may also be observed. [symptoma.com]
- Heart Murmur
Electrocardiogram Endocardial cushion defect Heart murmurs and other sounds Hypoplastic left heart syndrome Left heart catheterization Patent ductus arteriosus Patent foramen ovale Pediatric heart surgery - discharge Pulmonary atresia Pulmonary valve [icdlist.com]
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]
Heart murmur Rapid breathing Rapid heartbeat Symptoms of DORV may include: Poor 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 [arh.adam.com]
Heart murmur Rapid breathing Rapid heartbeat Tests to diagnose DORV include: Chest x-rays Ultrasound exam of the heart ( echocardiogram ) Passing a thin, flexible tube into the heart to measure blood pressure and inject dye for special pictures of the [ufhealth.org]
- Systolic Murmur
Other features include underdevelopment, cardiomegaly, systolic murmur in left third intercostal space, loud second pulmonic sound. If survival continues for a few years, polycythaemia and clubbing of fingers and toes become evident. [whonamedit.com]
Consequently, neonates typically present with cyanosis, systolic murmur and heart failure. As the pulmonary vascular resistance increases, PBF curtails and cyanosis deepens but longevity prolongs. [journals.viamedica.pl]
The precordium was hyper dynamic and a systolic murmur was auscultated. The chest radiogram revealed cardiomegaly and pulmonary plethora. [annalspc.com]
He loves music and has composed more than 250 musical pieces.11 Description of Taussig-Bing Anomaly In 1949, Taussig and Bing described the case of a 5.5-year-old girl who had cyanosis since birth, developmental delay, systolic murmur, and dyspnea at [ncbi.nlm.nih.gov]
Neurologic
- Confusion
The aortic bodies should not be confused with the PARA-AORTIC BODIES in the abdomen (which are sometimes also called aortic bodies). [bioportfolio.com]
Because of this confusion in classification ... [obgyn.mhmedical.com]
Thanks, I will post on that board as well, I was confused as to where I should post last night. Thanks for uour kind words and support x [netmums.com]
This type of DOLV is also referred to as the tetralogy type of DOLV because it is often confused clinically and angiographically with tetralogy of Fallot as a result of the associated pulmonary stenosis. 28 The remaining six patients with a right anterior [radiologykey.com]
The heart, as described by Taussig and Bing is quite dissimilar from the D-TGA hearts with pulmonary overriding, a frequent source of confusion in literature. [ispub.com]
Workup
Echocardiography is the most common method used for the diagnosis of Taussig Bing anomaly. The primary four criteria that support the diagnosis include:
- Both pulmonary artery and aorta emerge from the right ventricle
- Blood from the left ventricle flows only through the ventricular septal defect
- The aorta is situated to the right or anteriorly to the pulmonary artery
- The absence of pulmonary–mitral continuity [7] [8].
This congenital heart anomaly can also be diagnosed prenatally via real-time 2-dimensional echocardiography. Real-time 3-dimensional echocardiography offers no special benefits over the 2-dimensional mode [9].
A chest X-ray in these patients may show non-specific changes that include cardiomegaly, increased pulmonary vascular markings and a prominent pulmonary artery (PA) segment. However, normal cardiac anatomy may be observed in patients who have pulmonary stenosis in addition to the primary defects.
Other imaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI), especially 3-dimensional MRI, can be used for the precise evaluation of the cardiac and blood vessel anatomy.
Physical examination may reveal findings suggestive of a large septal defect, such as a holosystolic murmur and a thrill at the base of the heart. No pathological blood pressure gradients between the upper and lower limbs have been observed [10].
Resting electrocardiogram (ECG) may show a right axis deviation, hypertrophy of the right ventricle, intraventricular conduction defects and negative T waves in leads I, avL and V5-V6. Holter monitoring may show evidence of arrhythmias such as a wide complex tachycardia and ventricular/ supraventricular premature beats. Stress testing may reveal a reduction in exercise capacity and a decreased consumption of maximal oxygen.
Axis
- Right Axis Deviation
Resting electrocardiogram (ECG) may show a right axis deviation, hypertrophy of the right ventricle, intraventricular conduction defects and negative T waves in leads I, avL and V5-V6. [symptoma.com]
Resting ECG showed right axis deviation, low atrial rhythm with a heart rate of 79 beats/minute, intraventricular conduction disturbances, right ventricular hypertrophy, negative T waves in I, avL, V5-V6. [crcd.eu]
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]
Electrocardiogram showed right axis deviation, clockwise loop, right ventricular hypertrophy and well preserved R waves in V5–V6 reflecting LV volume overload. Echocardiogram The visceroatrial situs was solitus with levocardia (Fig. 1A). [journals.viamedica.pl]
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]
Blocks
- Right Bundle Branch Block
Electrocardiography findings were sinus rhythm with first degree heart block, right bundle branch block and right ventricular hypertrophy. [aeronline.org]
Resulting from surgery, he experienced right bundle branch block (RBBB ) -- an "electrical" issue with the heart. There were complications getting him off of the heart-lung by-pass machine. Jonathan really struggled that first night. [mdjunction.com]
Eleven of 13 patients had electrocardiographic evidence of right ventricular hypertrophy or right bundle branch block. Thirteen of the 14 had x-ray or clinical evidence for cardiac enlargement. [kundoc.com]
Treatment
CONCLUSIONS: The arterial switch operation remains our preferred choice of treatment for children with Taussig-Bing anomaly. The position of the great arteries has no effect on postoperative morbidity and mortality. [biomedsearch.com]
Taussig-Bing anomaly and interrupted aortic arch-type A Source: Multimedia Manual of CT Surgery Published on 2016-07-14 Scimitar Syndrome Repair in Adults: Intermediate-Term Results Using an Extracardiac Conduit Extracorporeal life support can be a first-line treatment [ct-assist.com]
details of surgical treatment, potential complications and literature references. [books.google.com]
We present a video illustrating our preferred surgical option in the treatment of Taussig-Bing anomaly, in this case, with an associated type A interrupted aortic arch. [ncbi.nlm.nih.gov]
Prognosis
The coexistence of the Taussig-Bing anomaly and coarctation of the aorta is a highly complex situation carrying a dismal prognosis. [ncbi.nlm.nih.gov]
JOURNAL ARTICLE The coexistence of the Taussig-Bing anomaly and coarctation of the aorta is a highly complex situation carrying a dismal prognosis. [read.qxmd.com]
Prognosis is poor. Bibliography H. B. Taussig, R. J. Bing: Complete transposition of the aorta and levoposition of the pulmonary artery: clinical, physiological, and pathological findings. American Heart Journal, St. Louis, 1949, 37: 551-559. [whonamedit.com]
Prognosis for infants born with DORV and transposition of the great arteries is generally good, although it is dependent on specific anatomy. [emedicine.medscape.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 child's overall condition Outlook (Prognosis) How well the baby does depends on [arh.adam.com]
Etiology
Embryology, Etiology, Basic Principles of Analysis, and Fetal Circulation 2. Methods of Diagnosis 3. Special Diagnostic Procedures: Angiocardiography, Aortography, and Cardiac Catheterization 4. Cyanosis 5. [hup.harvard.edu]
Despite a cohort with no late mortality, [ 8 ] others have reported late mortality mostly with an unexplained etiology.[ 6, 7, 9 ] In our patient cohort, sudden nature of the late mortality primarily indicates arrhythmia, and an underlying coronary problem [tgkdc.dergisi.org]
The leaflets are showing the features of rheumatic etiology as in Figures 10-14 and severely regurgitant as shown in Figures 15-18. [m.scirp.org]
0.8% of congenital heart disease.1, 2, 3, 4, 5 The first case was reported by Neil Stenonis, who described a child with an open sternal line and protruding heart, liver and spleen.2 In 1706, Haller and Martínez separately reported cases of EC.6, 7 The etiology [revespcardiol.org]
The etiology was low cardiac output ( n = 4); sudden unexplained death ( n = 2); pneumonia ( n = 1); respiratory failure ( n = 1); bleeding ( n = 1); and rejection following orthotopic heart transplantation ( n = 1). [ejcts.oxfordjournals.org]
Epidemiology
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]
The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis. Working Party of the Italian Society of Pediatric Cardiology. Cardiol Young. 1999 May. 9(3):291-9. [Medline]. [emedicine.medscape.com]
& Wolfe, R., 1 Jan 2019, In : Epidemiology (Cambridge, Mass.). 30, 1, p. 38-47 10 p. [monash.edu]
[…] children) study A Saxena, S Ramakrishnan, A Roy, S Seth, A Krishnan, P Misra, M Kalaivani, B Bhargava, M Flather, P Poole-Wilson All India Institute of Medical Sciences, New Delhi, India and Royal Brompton Hospital and Imperial College, London Background: Epidemiological [annalspc.com]
Pathophysiology
The Congenital Heart Defects are presented with each chapter devoted to a single malformation, with incidence, morphology, associated anomalies, pathophysiology, diagnosis (including clinical pattern, electrocardiogram, chest X-ray, echocardiogram, cardiac [books.google.com]
The cardiovascular pathophysiology is determined predominantly by the degree of PS, which generally increases over time in early infancy. [thoracickey.com]
Such patients have complex pathophysiology, which can lead to unstable haemodynamics, arrhythmias and cardiac arrest under anesthesia. [aeronline.org]
Prevention
Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation. [utmb.influuent.utsystem.edu]
Occasionally, the ventricular septal defect needs to be enlarged to prevent late development of left ventricular outflow tract obstruction. [slideplayer.com]
Prevention Can I prevent DORV? There is no way to prevent DORV. Scientists are still trying to understand its cause. Outlook / Prognosis What is the outlook for a baby with DORV? Without surgery, a baby with DORV will eventually develop: Cyanosis. [my.clevelandclinic.org]
References
- Taussig H, Bing R. Complete transposition of the aorta and a levoposition of the pulmonary artery. Am Heart J. 1949;37:551-9.
- Stellin G, Zuberbuhler J, Anderson R, Siewers R. The surgical anatomy of the Taussig—Bing malformation. J Thorac Cardiovasc Surg. 1987;93:560-9.
- Roger H. Clinical researches on the congenital communication of the two sides of the heart by failure of occlusion of the interventricular septum. Bull de l' Acad de Med. 1879;8:1074.
- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. Sep 1958;2(5099):755-62.
- Heath D, Edwards J. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. Oct 1958 ;4.
- Van Praagh R. What is the Taussig-Bing malformation? Circulation. 1968;38(3):445-9.
- Comas J, Mignosa C, Cochrane A, Wilkinson J, Karl T. Taussig—Bing anomaly and arterial switch operation: aortic arch obstruction does not influence outcome. Eur J Cardiothorac Surg. 1996;10:1114-9.
- Parr G, Waldhausen J, Bharati S, Lev M, Fripp R, Whitman V. Coarctation in Taussig—Bing malformation of the heart. Surgical significance. J Thorac Cardiovasc Surg. 1983;86:280-7.
- Rodefeld M, Ruzmetov M, Vijay P, Fiore A, Turrentine M, Brown J. Surgical results of arterial switch operation for Taussig-Bing anomaly: is position of the great arteries a risk factor? Ann Thorac Surg. Apr 2207;83(4):1451-7.
- Yacoub M, Radley-Smith R. Anatomic correction of the Taussig—Bing anomaly. J Thorac Cardiovasc Surg. 1984;88:380-8.