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Chapter 9 - Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

from Section 2 - Right-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

Similar to other variants of tetralogy of Fallot (TOF), TOF with absent pulmonary valve syndrome includes an anterior maligned ventricular septal defect, an aorta that “overrides” the septal defect, and a hypertrophied right ventricle. However, it is distinct in that there is an absent or rudimentary, incompetent pulmonary valve. The pulmonary valve, if present, may have some degree of stenosis but the dominant pathophysiology is pulmonary regurgitation. Patients usually have unobstructed flow to the pulmonary arteries and therefore do not have cyanosis or hypercyanotic spells associated with other tetralogy variants. The main and branch pulmonary arteries are generally dilated due to pulmonary regurgitation and excessive flow and can be large enough to cause a mass effect on surrounding structures, including the airways and lungs. Patients with unrepaired tetralogy with absent pulmonary valve syndrome have respiratory symptoms and cyanosis related to the degree of intracardiac shunting and airway compression. This chapter addresses the perioperative cardiorespiratory challenges of caring for an infant with this syndrome.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 52 - 56
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Cowan, J. R. and Ware, S. M. Genetics and genetic testing in congenital heart disease. Clin Perinatol 2015; 42: 373–93.CrossRefGoogle ScholarPubMed
Dorobantu, D. M., Stoicescu, C., Tulloh, R. M., et al. Surgical repair of tetralogy of Fallot with absent pulmonary valve: favorable long-term results. Semin Thorac Cardiovasc Surg 2019; 31: 847–9.CrossRefGoogle ScholarPubMed
Hew, C. C., Daebritz, S. H., Zurakowski, D., et al. Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome. Ann Thorac Surg 2002; 73: 1778–85.CrossRefGoogle ScholarPubMed
Rabinovitch, M., Rabinovitch, S., David, I., et al. Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves. Am J Cardiol 1982; 50: 804–13.CrossRefGoogle ScholarPubMed
Yeager, S. B., Van, D. V., Waters, B. L., et al. Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome. Echocardiography 2002; 19: 489493.CrossRefGoogle ScholarPubMed
Zach, M., Beitzke, A., Singer, H., et al. The syndrome of absent pulmonary valve and ventricular septal defect–anatomical features and embryological implications. Basic Res Cardiol 74: 5468.CrossRefGoogle Scholar

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