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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).


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].

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]
Dyspnea at Rest
  • Dyspnea at rest and developmental delay are the other typical features seen. Compensatory erythrocytosis may also be observed.[symptoma.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]
  • 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]
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]
  • Every topic and chapter has been revised and updated to reflect the latest medical and surgical treatments for all congenital and acquired heart diseases. New surgical approaches, including hybrid procedures, have been updated.[books.google.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]
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]
  • 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]
  • […] particularly in the past decade as the method of choice in the treatment of Taussig-Bing anomaly.[ 6 , 7 ] Primary repair has the advantage of avoiding multiple surgeries and also, with primary palliation, patients are exposed to a longer period of cyanosis[tgkdc.dergisi.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]
Heart Murmur
  • […] 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]
  • 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]
  • 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 Pale[medlineplus.gov]
  • 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 heart[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]

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.

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]

Treatment

  • Such anatomical complexity made treatment difficult. This palliative procedure allows future biventricular repair.[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]
  • We report a patient with Taussig-Bing anomaly who had fungal endocarditis after several cardiac operations and was successfully treated with transeptal aortic valve replacement concomitant with other surgical and medical treatments.[ncbi.nlm.nih.gov]
  • , details of surgical treatment, potential complications and literature references.[books.google.com]
  • Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease.[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]
  • 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]

Etiology

  • 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[tgkdc.dergisi.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

  • Pagina 340 - The Framingham Study: an epidemiological investigation of cardiovascular disease. National Institutes of Health publication, 1974:74-599. ‎[books.google.it]
  • 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]
Sex distribution
Age distribution

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]
  • Get comprehensive coverage of cardiac anatomy, pathophysiology, natural history and disease management.[books.google.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, dextro-transposition of the great arteries, left-transposition of the great arteries, normally related).[emedicine.medscape.com]

Prevention

  • 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]
  • Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. ‎ Pagina 332 - Crane JP, LeFevre ML, Winborn RC, et al.[books.google.ro]
  • HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS.[readbyqxmd.com]

References

Article

  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.

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Last updated: 2018-06-22 02:24