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J Thorac Cardiovasc Surg 2003;126:1643-1644
© 2003 The American Association for Thoracic Surgery
Brief communications |
a Department of Cardiology, Taoyuan, Taiwan
b Department of Cardiac Surgery,, Taoyuan, Taiwan Chang Gung Children's Hospital
c Department of Pediatrics, Taoyuan, Taiwan
d Department of Surgery, Chang Gung University, Taoyuan, Taiwan
Received for publication April 4, 2003; accepted for publication June 18, 2003.
* Address for reprints: Wen-Jen Su, MD, Department of Cardiology, Chang Gung Children's Hospital, 5-7 Fu-Shin Street, Kweishan, Taoyuan 333, Taiwan
hwaien2003{at}yahoo.com.tw
Persistent fifth aortic arch (PFAA) is thought to be an extremely rare congenital heart disease (CHD). All previously reported cases were associated with major cardiac anomalies and diagnosed coincidentally.1 Moreover, its presence was usually beneficial to the associated cardiac anomalies. We report here a unique case of isolated PFAA (without associated cardiac anomaly) with systemic-to-pulmonary arterial connection, which caused severe congestive heart failure (CHF) in a 2-month-old male infant.
Clinical summary
A 2-month-old male infant weighing 3940 g was referred to our hospital for evaluation of a suspected atypical huge patent ductus arteriosus (PDA). He was the product of a 39-week gestation with a birth weight of 3480 g. He presented with severe CHF and failure to thrive. Chest radiographs revealed marked cardiomegaly, increased pulmonary vascular markings, pulmonary edema, and a convex pulmonary artery segment. The electrocardiogram demonstrated biventricular hypertrophy. A repeat echocardiogram done at our institution showed the segmental combination of solitus normal heart {S,D,S} (S = visceroatrial situs solitus, D = D-ventricular loop, S = conotruncus solitus); dilated left atrium, left ventricle, and pulmonary arteries; normal left-sided aortic arch; and preserved ventricular performance. From the parasternal short-axis view, a continuous, turbulent flow was detected at the bifurcation of the pulmonary trunk, which was not typical for PDA flow. From the modified suprasternal long-axis view, we detected a huge vessel originating from the left anterior aspect of distal ascending aorta (AsAo) but proximal to the transverse aortic arch (Figure 1) and connecting to the superior margin of the bifurcation of the pulmonary trunk. Isolated persistent fifth aortic arch with systemic-to-pulmonary arterial connection was suspected. Contrast computed tomographic 3-dimensional reconstruction of the heart and great vessels and ascending aortogram confirmed the diagnosis. Hemodynamic data revealed large left-to-right shunt (pulmonary/systemic blood flow ratio > 4) and severe pulmonary hypertension (pulmonary/systemic systolic blood pressure ratio = 1). The patient then underwent successful surgical ligation of this abnormal vessel through median sternotomy.
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Persistent fifth aortic arch is thought to be an extremely rare CHD because the fifth aortic arch almost always involutes during normal embryologic development. However, if it persists, an abnormal vessel originating from the distal AsAo proximal to the innominate artery will be formed,2 and it can manifest itself in many different congenital vascular malformations according to the intrinsic different embryologic maldevelopment. These include double-lumen aortic arch, subclavian artery as the first branch of the aortic arch, systemic-to-pulmonary arterial connection, systemic-to-systemic arterial connection.3 Therefore, it is probably not so rare if we include the asymptomatic, isolated, not yet diagnosed double-lumen aortic arch and subclavian artery as the first branch of the aortic arch.
All previously reported cases were associated with major CHD, such as pulmonary atresia, tricuspid atresia, coarctation of aorta, interrupted aortic arch, ventricular septal defect, or PDA, etc.1 The PFAA is usually hemodynamically beneficial or not harmful to the associated CHD and is diagnosed coincidentally.1 For example, double-lumen aortic arch is beneficial to the associated coarctation of aorta or interrupted aortic arch, and systemic-to-pulmonary arterial connection is beneficial to the associated pulmonary atresia or tricuspid atresia.1 Isolated double-lumen aortic arch or subclavian artery as the first branch of the aortic arch is not harmful to humans.4,5 However, our unique case with systemic-to-pulmonary arterial connection did not have any associated cardiovascular anomaly, and this isolated PFAA resulted in large left-to-right shunt, severe pulmonary hypertension, and CHF. Its clinical presentation was quite similar to huge PDA and thus might be misdiagnosed due to its rarity.
References
This article has been cited by other articles:
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  R. Subramanyan, A. Sahayaraj, P. Sekar, and K. M. Cherian Persistent fifth aortic arch J. Thorac. Cardiovasc. Surg., June 1, 2010; 139(6): e117 - e118. [Full Text] [PDF]
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