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D - transposition of the great vessels — Clinical Guidelines

Overview

D-transposition of the great vessels (d-TGV) is a congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle, leading to systemic and pulmonary circulations being parallel rather than sequential. Surgical interventions like the Mustard and Senning procedures aim to correct this anomaly, though complications such as arrhythmias, pulmonary vascular disease, and valve regurgitation are common 1 3 9 14.

Diagnosis

Echocardiography: Essential for initial diagnosis and postoperative evaluation of baffle function and valve status 5 14 15.

Electrophysiological Studies: Useful in identifying intraatrial reentrant tachycardias post-Mustard/Senning procedures 1.

Selective Coronary Arteriography: Venous techniques may be necessary in neonates for accurate coronary anatomy assessment 2.

Cardiopulmonary Exercise Testing: Evaluates exercise tolerance and cardiovascular responses post-surgery 3.

Pulmonary Function Assessment: Important for evaluating pulmonary vascular disease 7.

Management

Surgical Correction: Mustard or Senning procedures for anatomical correction 1 5 12 13.

Catheter Ablation: For recurrent intraatrial reentrant tachycardias targeting protected areas in the systemic venous atrium 1.

Pulmonary Artery Catheterization: Utilize percutaneous suprasternal puncture techniques when arterial access is challenging 16.

Management of Complications:

- Valve Regurgitation: Operative correction possible for deformities but not for Ebstein-type malformations 9. - Chylothorax: Surgical revision of the interatrial baffle and thoracic duct ligation may be required 6. - Subpulmonic Obstruction: Echocardiographic monitoring and potential surgical intervention 15.

Special Populations

Pediatrics: Focus on postoperative echocardiography and exercise tolerance assessments 3 5 14.

Pregnancy: No definitive evidence linking oral contraceptives to increased incidence of d-TGV 11.

Comorbidities: Dynamic left ventricular outflow tract obstruction requires careful preoperative assessment and management 4.

Key Recommendations

Perform echocardiography preoperatively and postoperatively to assess anatomical correction and baffle function 5 14. (Evidence: Strong)

Utilize electrophysiological studies in patients with recurrent arrhythmias post-Mustard/Senning procedures for targeted catheter ablation 1. (Evidence: Moderate)

Consider percutaneous suprasternal puncture for pulmonary artery catheterization in neonates with d-TGV when arterial access is limited 16. (Evidence: Moderate)

Monitor for and manage complications such as valve regurgitation and subpulmonic obstruction with appropriate interventions 9 15. (Evidence: Moderate)

Evaluate pulmonary vascular disease through hemodynamic and echocardiographic assessments in patients with d-TGV and intact ventricular septum 7. (Evidence: Moderate)

References

1 Kriebel T, Tebbenjohanns J, Janousek J, Windhagen-Mahnert B, Bertram H, Paul T. Intraatrial reentrant tachycardias in patients after atrial switch procedures for d-transposition of the great arteries Endocardial mapping and radiofrequency catheter ablation primarily targeting protected areas of atrial tissue within the systemic venous atrium. Zeitschrift fur Kardiologie 2002. link 2 Day RW, Isabel-Jones JB, Wetzel GT, Oku GS, Jarmakani JM. Description of a venous technique for selective coronary arteriography in newborns with d-transposition of the great arteries. Journal of the American College of Cardiology 1989. link90433-6) 3 Ensing GJ, Heise CT, Driscoll DJ. Cardiovascular response to exercise after the Mustard operation for simple and complex transposition of the great vessels. The American journal of cardiology 1988. link90666-2) 4 Stewart S, Harris PJ, Manning J. The midterm and long-term results of the Mustard operation in patients with transposition of the great vessels and dynamic left ventricular outflow tract obstruction. The Annals of thoracic surgery 1986. link62768-3) 5 Pasalodos J, González V, Suárez de Lezo J, Concha M, Vallés F. Contrast bidimensional echocardiography in the morphologic and functional postoperative evaluation of the Senning technique for complete transposition of the great vessels. American heart journal 1984. link90552-0) 6 Copeland JG, Shaut C. Bilateral chylothorax complicating Mustard repair of transposition of the great vessels. Archives of internal medicine 1982. link 7 Newfeld EA, Paul MH, Muster AJ, Idriss FS. Pulmonary vascular disease in transposition of the great vessels and intact ventricular septum. Circulation 1979. link 8 Powers WF, Swyer PR. Diminished limb blood flow in infants with transposition of the great vessels: An adaptation to chronic hypoxia?. Acta paediatrica Scandinavica 1977. link 9 Jaffe RB. Systemic atrioventricular valve regurgitation in corrected transpositon of the great vessels. Angiographic differentiation of operable and nonoperable valve deformities. The American journal of cardiology 1976. link90289-7) 10 Carter GA. Retrograde catheterization of the pulmonary veins following surgical correction of transposition of the great vessels. Angiology 1976. link 11 Yasuda M, Miller JR. Prenatal exposure to oral contraceptives and transposition of the great vessels in man. Teratology 1975. link 12 Stansel HC. A new operation for d-loop transposition of the great vessels. The Annals of thoracic surgery 1975. link64433-5) 13 Taguchi K, Matsumura H, Hirao M, Kato K, Itano M. A new approach to total repair of transposition of the great vessels: a technique for atrial autotransplantation. The Journal of thoracic and cardiovascular surgery 1975. link 14 Nanda ND, Stewart S, Gramiak R, Manning JA. Echocardiography of the intra-atrial baffle in dextro-transposition of the great vessels. Circulation 1975. link 15 Nanda NC, Gramiak R, Manning JA, Lipchik EO. Echocardiographic features of subpulmonic obstruction in dextro-transposition of the great vessels. Circulation 1975. link 16 Rahimtoola SH, Ongley PA, Swan HJ. Percutaneous suprasternal puncture (Radner technique) of the pulmonary artery in transposition of the great vessels. Circulation 1966. link

D - transposition of the great vessels