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].

• 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]

  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]

  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]

  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]

• Dyspnea

  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]

  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]

• Dyspnea at Rest

  Dyspnea at rest and developmental delay are the other typical features seen. Compensatory erythrocytosis may also be observed. [symptoma.com]

• Tachypnea

  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]

  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]

• 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]

  Congenital Heart Disease. in: Staples Press, London1939: 193 Google Scholar Brown J.W. Congenital Heart Disease. in: Staples Press, London1950: 264 Google Scholar Lev M. Volk B.W. [amjmed.com]

  References Related articles: Pathology: Vascular: Congenital heart disease Promoted articles (advertising) [radiopaedia.org]

  Females with this complex heart disease, when pregnant, have to undergo significant cardiovascular challenges during pregnancy and delivery. [aeronline.org]

  Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease. [ncbi.nlm.nih.gov]

• Cyanosis

  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]

  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]

  There was grade II clubbing and cyanosis. Her room air SpO2 was 80–85% and increased to 92% with oxygen supplementation. There was no pedal edema. [aeronline.org]

  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

  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]

  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]

  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 [medlineplus.gov]

  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]

  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]

• Ulcer

  Royal College of Nursing leads national audit of the management of venous leg ulcers. J Tissue Viability 2006; 16(1):6–7. [PubMed] 9. Taussig HB, Crawford H, Pelargonio S, Zacharioudakis S. [ncbi.nlm.nih.gov]

  The second death occurred 4 years after arterial switch and 3.5 years after prosthetic (21-ram Medtronic-Hall) neoaortic valve replacement due to ex- sanguinating gastric ulcer with a therapeutic prothrom- bin time. [docslide.com.br]

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.

• Pneumoperitoneum

  Six months after an uncomplicated neonatal ASO with aortic arch reconstruction, the patient succumbed to severe pneumonia complicated by sepsis, pneumothoraces, and pneumoperitoneum. [ahajournals.org]

• 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 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]

  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]

• Erythrocytosis

  Compensatory erythrocytosis may also be observed. [symptoma.com]

  Although her cyanosis, clubbing, and erythrocytosis were consistent with an Eisenmenger complex, the cyanosis since birth made Taussig and Bing suspect “some totally different malformation.”1 Angiocardiography was performed. [ncbi.nlm.nih.gov]

details of surgical treatment, potential complications and literature references. [books.google.com]

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]

Such anatomical complexity made treatment difficult. This palliative procedure allows future biventricular repair. [ncbi.nlm.nih.gov]

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]

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 [ufhealth.org]

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]

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]

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]

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.medscape.com]

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. [QxMD MEDLINE Link]. [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]

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]

Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation. [mayoclinic.pure.elsevier.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]

We used titrated doses of epidural lignocaine 2% to prevent precipitous hypotension and did not require vasopressors in the intraoperative period. [aeronline.org]

1. Taussig H, Bing R. Complete transposition of the aorta and a levoposition of the pulmonary artery. Am Heart J. 1949;37:551-9.
2. Stellin G, Zuberbuhler J, Anderson R, Siewers R. The surgical anatomy of the Taussig—Bing malformation. J Thorac Cardiovasc Surg. 1987;93:560-9.
3. 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.
4. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. Sep 1958;2(5099):755-62.
5. 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.
6. Van Praagh R. What is the Taussig-Bing malformation? Circulation. 1968;38(3):445-9.
7. 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.
8. 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.
9. 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.
10. Yacoub M, Radley-Smith R. Anatomic correction of the Taussig—Bing anomaly. J Thorac Cardiovasc Surg. 1984;88:380-8.