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Ann Thorac Surg 2006;81:222-223
© 2006 The Society of Thoracic Surgeons
Pediatric Cardiac Surgery, Denver Children's Hospital, University of Colorado, 1056 East 19th Ave, Denver, CO 80218
(Email: lacour-gayet.francois{at}tchden.org).
Truncus arteriosus associated with interrupted aortic arch (TA-IAA) is a rare disease. This Congenital Heart Surgeons Society (CHSS) multi-institutional retrospective cohort of 50 patients undergoing surgical intervention for TA-IAA is the largest series ever published [1]. Previous single institution studies have been done on less than 10 patients. The high mortality reported (61% mortality at 1 year) contrasts with the excellent results obtained when the truncus arteriosus is isolated. Furthermore, several centers [2–4] have reported that TA associated with IAA and TA associated with truncal valve regurgitation can be safely managed.
The reasons for the negative results reported by this CHSS study are multifactorial. The surgical outcome of TA-IAA is highly dependant on the preoperative status of the patient. The truncal valve regurgitation can be particularly severe in TA-IAA. If there is no data to suspect that the truncal valve is more malformed when associated with an IAA, any truncal regurgitation will be worsened by the premature closure of the ductus arteriosus, which induces an increase of the pulmonary flow and therefore of the truncal valve flow. Any delay in the institution of PGE1 may induce myocardial ischemia, NEC, and ultimately multiple organ failure. These complications are prevented by early PGE1 infusion, minimal FiO2, normal to elevated PCO2 (similar to HLHS management) and also very early repair in first days of life and fetal diagnosis. This multicentric series reports a median age at surgery of 9 days, which is rather late.
The risk of the TA-IAA repair is increased by noncardiac factors. The presence of either a prematurity or DiGeorge syndrome or an extracardiac anomaly is associated in this study with 100% mortality. All the severe risk factors are currently defined in the comprehensive Aristotle score as shown by a recent study on TA-IAA from Miyamoto and colleagues [5].
The questionable surgical technique applied to many of the patients in this series is a cause of failure as acknowledged by the authors. The optimal technique of TA-IAA repair was simultaneously published in the mid 1990s by Hanley and colleagues [4], Jahangiri and colleagues [3], Bove and colleagues [6], and us [7]. This technique includes one-stage repair in the first days of life, mobilization of the descending aorta, direct aortic anastomosis, transection of the common trunk, harvesting of a large pulmonary artery branches cuff, patch enlargement of the ascending aorta, and valved conduit reconstruction of the RVOT. An ascending aorta patch enlargement is essential [7] to prevent ascending aorta stenosis due to the mismatch between the truncal root and the small diameter of the ascending aorta, particularly in the presence of type B with aberrant right subclavian artery.
The authors are following the 1949 classification from Collet and Edwards [8]. In our opinion, the 1987 modified classification [9] from Van Praagh that excluded the Collet-Edwards type III is far more realistic. The pulmonary artery (PA) branches arise usually in a one and a half motion and do not raise a real technical challenge because we divide the truncus root and harvest a large PA branch cuff. The real difficulty is coming from the PA branches arising from the descending aorta or from a ductus-like vessel (type A3 of Van Praagh, not mentioned in this series).
This study proposed a heart transplant for the most critical forms associated with severe aortic regurgitation. With a 4-month average delay to obtain an infant donor heart, this option seems very risky. Repairing or replacing the truncal valve is a safer approach as shown by Jahangiri and colleagues [3].
All together this large series of TA-IAA repair shows poor short-term and long-term results. It is noticeable that the timeframe of the study closes in 1997, a time when the optimal surgical technique was just introduced.
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  F. Lacour-Gayet and S. Goldberg Surgical repair of truncus arteriosus associated with interrupted aortic arch MMCTS, March 28, 2008; 2008(0328): 2451. [Abstract] [Full Text] [PDF]
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