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Tetralogy of Fallot.

Bailliard F, Anderson RH - Orphanet J Rare Dis (2009)

Bottom Line: Follow-up in patients born 30 years ago shows a rate of survival greater than 85%.Chronic issues that now face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias.As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved.

View Article: PubMed Central - HTML - PubMed

Affiliation: North Carolina Children's Heart Center, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA. frederique_bailliard@med.unc.edu

ABSTRACT
Tetralogy of Fallot is a congenital cardiac malformation that consists of an interventricular communication, also known as a ventricular septal defect, obstruction of the right ventricular outflow tract, override of the ventricular septum by the aortic root, and right ventricular hypertrophy. This combination of lesions occurs in 3 of every 10,000 live births, and accounts for 7-10% of all congenital cardiac malformations. Patients nowadays usually present as neonates, with cyanosis of varying intensity based on the degree of obstruction to flow of blood to the lungs. The aetiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria, and intake of retinoic acid. Associated chromosomal anomalies can include trisomies 21, 18, and 13, but recent experience points to the much more frequent association of microdeletions of chromosome 22. The risk of recurrence in families is 3%. Useful diagnostic tests are the chest radiograph, electrocardiogram, and echocardiogram. The echocardiogram establishes the definitive diagnosis, and usually provides sufficient information for planning of treatment, which is surgical. Approximately half of patients are now diagnosed antenatally. Differential diagnosis includes primary pulmonary causes of cyanosis, along with other cyanotic heart lesions, such as critical pulmonary stenosis and transposed arterial trunks. Neonates who present with ductal-dependent flow to the lungs will receive prostaglandins to maintain ductal patency until surgical intervention is performed. Initial intervention may be palliative, such as surgical creation of a systemic-to-pulmonary arterial shunt, but the trend in centres of excellence is increasingly towards neonatal complete repair. Centres that undertake neonatal palliation will perform the complete repair at the age of 4 to 6 months. Follow-up in patients born 30 years ago shows a rate of survival greater than 85%. Chronic issues that now face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias. As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved.

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Cardiac magnetic resonance imaging in an adult patient with tetralogy of Fallot repaired in childhood with an infundibular muscle resection and placement of a conduit from the right ventricle to the pulmonary arteries (a) has results in dilation and hypertrophy of the right ventricle. Additional images (b) show the stenotic and tortuous homograft conduit responsible for the dilated and hypertrophied right ventricle.
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Figure 9: Cardiac magnetic resonance imaging in an adult patient with tetralogy of Fallot repaired in childhood with an infundibular muscle resection and placement of a conduit from the right ventricle to the pulmonary arteries (a) has results in dilation and hypertrophy of the right ventricle. Additional images (b) show the stenotic and tortuous homograft conduit responsible for the dilated and hypertrophied right ventricle.

Mentions: Questions that remain regarding the management of tetralogy of Fallot pertain to the preference of surgical timing, as discussed above, and to the residual lesions of the disease. The management of young adults with pulmonary regurgitation or residual pulmonary stenosis is complex. A balance must be achieved between limiting the haemodynamic burden on the right ventricle, and minimizing the life-time number of surgical interventions required for a given patient. Cardiac magnetic resonance imaging has progressed significantly in the past ten years (Figures 9a and 9b). This provides reliable objective data, in addition to echocardiography and exercise testing, that physicians can use to determine the optimal timing of reintervention [41-43]. Recent advances in interventional cardiac catheterisation now provide safe options for treatment other than cardiac surgery. Devices such as the percutaneously placed pulmonary valve have shown promising results in the management of selected patients with pulmonary regurgitation and stenosis [44,45]. Ongoing advances in diagnostics and minimally invasive procedures will continue to help improve the care of these adult patients with tetralogy of Fallot.


Tetralogy of Fallot.

Bailliard F, Anderson RH - Orphanet J Rare Dis (2009)

Cardiac magnetic resonance imaging in an adult patient with tetralogy of Fallot repaired in childhood with an infundibular muscle resection and placement of a conduit from the right ventricle to the pulmonary arteries (a) has results in dilation and hypertrophy of the right ventricle. Additional images (b) show the stenotic and tortuous homograft conduit responsible for the dilated and hypertrophied right ventricle.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2651859&req=5

Figure 9: Cardiac magnetic resonance imaging in an adult patient with tetralogy of Fallot repaired in childhood with an infundibular muscle resection and placement of a conduit from the right ventricle to the pulmonary arteries (a) has results in dilation and hypertrophy of the right ventricle. Additional images (b) show the stenotic and tortuous homograft conduit responsible for the dilated and hypertrophied right ventricle.
Mentions: Questions that remain regarding the management of tetralogy of Fallot pertain to the preference of surgical timing, as discussed above, and to the residual lesions of the disease. The management of young adults with pulmonary regurgitation or residual pulmonary stenosis is complex. A balance must be achieved between limiting the haemodynamic burden on the right ventricle, and minimizing the life-time number of surgical interventions required for a given patient. Cardiac magnetic resonance imaging has progressed significantly in the past ten years (Figures 9a and 9b). This provides reliable objective data, in addition to echocardiography and exercise testing, that physicians can use to determine the optimal timing of reintervention [41-43]. Recent advances in interventional cardiac catheterisation now provide safe options for treatment other than cardiac surgery. Devices such as the percutaneously placed pulmonary valve have shown promising results in the management of selected patients with pulmonary regurgitation and stenosis [44,45]. Ongoing advances in diagnostics and minimally invasive procedures will continue to help improve the care of these adult patients with tetralogy of Fallot.

Bottom Line: Follow-up in patients born 30 years ago shows a rate of survival greater than 85%.Chronic issues that now face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias.As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved.

View Article: PubMed Central - HTML - PubMed

Affiliation: North Carolina Children's Heart Center, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA. frederique_bailliard@med.unc.edu

ABSTRACT
Tetralogy of Fallot is a congenital cardiac malformation that consists of an interventricular communication, also known as a ventricular septal defect, obstruction of the right ventricular outflow tract, override of the ventricular septum by the aortic root, and right ventricular hypertrophy. This combination of lesions occurs in 3 of every 10,000 live births, and accounts for 7-10% of all congenital cardiac malformations. Patients nowadays usually present as neonates, with cyanosis of varying intensity based on the degree of obstruction to flow of blood to the lungs. The aetiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria, and intake of retinoic acid. Associated chromosomal anomalies can include trisomies 21, 18, and 13, but recent experience points to the much more frequent association of microdeletions of chromosome 22. The risk of recurrence in families is 3%. Useful diagnostic tests are the chest radiograph, electrocardiogram, and echocardiogram. The echocardiogram establishes the definitive diagnosis, and usually provides sufficient information for planning of treatment, which is surgical. Approximately half of patients are now diagnosed antenatally. Differential diagnosis includes primary pulmonary causes of cyanosis, along with other cyanotic heart lesions, such as critical pulmonary stenosis and transposed arterial trunks. Neonates who present with ductal-dependent flow to the lungs will receive prostaglandins to maintain ductal patency until surgical intervention is performed. Initial intervention may be palliative, such as surgical creation of a systemic-to-pulmonary arterial shunt, but the trend in centres of excellence is increasingly towards neonatal complete repair. Centres that undertake neonatal palliation will perform the complete repair at the age of 4 to 6 months. Follow-up in patients born 30 years ago shows a rate of survival greater than 85%. Chronic issues that now face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias. As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved.

Show MeSH
Related in: MedlinePlus