Symptoma

Taussig Bing Anomaly

Taussig Bing Complex

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).

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]

    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]

    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]

  • 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]

  • Heart Disease

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

    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]

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

    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]

  • Cyanosis

    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]

    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]

    There was grade II clubbing and cyanosis. Her room air SpO 2 was 80–85% and increased to 92% with oxygen supplementation. There was no pedal edema. [aeronline.org]

  • Heart Murmur

    On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. [readbyqxmd.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 defect corrective surgeries Congenital heart disease Coronary artery fistula Cyanotic heart disease Dextrocardia Double aortic arch Double inlet left ventricle Ebstein's anomaly Eisenmenger syndrome Electrocardiogram Endocardial cushion defect Heart [icdlist.com]

  • Systolic Murmur

    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]

    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]

    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]

  • Palpitations

    Patient had a history of palpitations not associated with chest pain or any syncopal attack. At 15 years of age patient had breathlessness for which she was prescribed tablet furosemide 40 mg overdose (OD) by her primary care physician. [aeronline.org]

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]

    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]

    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]

    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]

  • Right Ventricular Hypertrophy

    Electrocardiography findings were sinus rhythm with first degree heart block, right bundle branch block and right ventricular hypertrophy. [aeronline.org]

    Cardiomegaly and right ventricular hypertrophy are usually present. Permanent and progressive cyanosis usually begins at birth. [whonamedit.com]

    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]

    Preoperatively, echocardiography of the patient was suggestive of double-outlet right ventricle (DORV) with large sub-pulmonic ventricular septal defect (VSD), right ventricular hypertrophy, bidirectional shunt and severe pulmonary artery hypertension [readbyqxmd.com]

    Her electrocardiogram showed sinus rhythm, high P waves in the second lead, and signs of right ventricular hypertrophy. [ncbi.nlm.nih.gov]

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]

and which forms of treatment currently in use provide the best outcomes to date [books.google.com]

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]

Although associated with an uncertain late prognosis, arterial level repairs are the most physiologic, and their results to date are encouraging. [link.springer.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]

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]

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]

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]

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]

1 Jan 2019, In : Epidemiology (Cambridge, Mass.). 30, 1, p. 38-47 10 p. [monash.edu]

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]

[…] 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 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]

Pathophysiology The pathophysiology of DORV varies, irrespective of the great arterial relationship (ie, side-by-side, d-transposition of the great arteries, l-transposition of the great arteries, normally related). [emedicine.medscape.com]

After obtaining approval from our Institutional Review Boards and waiving the need to obtain patient consent for this study, a retrospective review of medical records was performed with regard to initial clinical features, pathophysiologic findings, surgical [mafiadoc.com]

Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation. [ncbi.nlm.nih.gov]

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

In this cohort, a low mortality rate prevented identification of significant risk factors. [ahajournals.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.