Clinical Presentation and Therapy of d-Transposition of the Great Arteries
d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3
- PDF / 232,653 Bytes
- 5 Pages / 439.37 x 666.14 pts Page_size
- 42 Downloads / 159 Views
34
David J. Driscoll
Contents 34.1 Introduction ................................................................................................................... 34.2 Pathologic Physiology................................................................................................... 34.3 Clinical Presentation ..................................................................................................... 34.4 Physical Examination.................................................................................................... 34.5 Echocardiographic and Cardiac Catheterization Issues ................................................ 34.6 Treatment: Management of d-TGA with Intact Ventricular Septum ............................ 34.6.1 Arterial Switch (Jatene) Procedure 34.6.2 Atrial Switch (Senning or Mustard) Procedure 34.7 Outcome ........................................................................................................................ 34.7.1 Arterial Switch (Jatene) Procedure 34.7.2 Atrial Switch (Senning or Mustard) Procedure
34.1
433 434 435 435 435 435 435 436 436 436 437
Introduction
d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3.8 % of all congenital cardiac defects. Forty percent of patients with d-TGA have an associated ventricular septal defect. Among patients with d-TGA, 6 % of those with intact ventricular septum and 31 % of those with ventricular septal defect have associated pulmonary stenosis.
D.J. Driscoll Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic College of Medicine, Rochester, MN, USA e-mail: [email protected] © Springer-Verlag Wien 2016 S. Rickert-Sperling et al. (eds.), Congenital Heart Diseases: The Broken Heart: Clinical Features, Human Genetics and Molecular Pathways, DOI 10.1007/978-3-7091-1883-2_34
433
D.J. Driscoll
434
34.2
Pathologic Physiology
In d-TGA systemic venous return (blood with low oxygen content) returns to the right ventricle and then is pumped to the body via the aorta without passing through the lungs for gas exchange (Fig. 34.1). Pulmonary venous return (oxygenated blood) returns to the left ventricle and then is pumped back to the lungs (Fig. 34.1). The effective pulmonary blood flow (the volume of deoxygenated blood that participates in gas exchange in the lungs) is low, albeit total pulmonary blood flow is increased. This is incompatible with life unless a communication exists between the two circuits to allow mixture of the oxygenated and deoxygenated blood. This mixture occurs at the patent foramen ovale (or atrial septal defect), the ductus arteriosus (if patent), and the ventricular septal defect (if present). This is a tenuous situation for a patient with an intact ventricular septum and no true atrial septal defect because mixing of the two circuits will decrease as the patent ductus ar
Data Loading...
