This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Leo A Bockeria Osman Makhachev Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bockeria, L. A Articles by Khiriev, T. Search for Related Content PubMed PubMed Citation Articles by Bockeria, L. A Articles by Khiriev, T. Related Collections Related Article

eComment: Congenital absence of the left pulmonary artery: the feasibility of an ‘ideal’ correction

Bakoulev Center for Cardiovascular Surgery, Moscow 121552, Russia

Absent left pulmonary artery and double aortic arch in tetralogy of Fallot: reconstruction using homograft saphenous vein or iliac artery

This is the first presentation of a successful experience with surgical correction of congenitally absent left pulmonary artery (ALPA) associated with (tetralogy of Fallot) TOF and double aortic arch (DAA) in English medical literature. Obviously, in the presence of the left lung's hilar artery an ‘ideal’correction should include the reversal of intracardiac anomalies as well as surgical reconstruction of physiological blood flow to ipsilateral lung. Just such approach has been presented by the authors of this publication [1].

We have found 64 cases of ALPA in TOF described in the literature. Complete repair aimed at only at the reversal of intracardiac anomalies (‘unipulmonary’ correction), has been performed in 39 patients with hospital mortality of 7.6% (3/39). In Bakoulev Scientific Center for Cardiovascular Surgery 18 patients have been operated on for TOF with ALPA, with 1 in-hospital death (5.6%).

Surgical reconstruction of the continuity between the pulmonary trunk (PT) and the hilar artery of ipsilateral lung, as we can judge from the available literature, was performed only in 11 patients aged 7 days–8 years (median 1.1 year). In 10 cases the anomaly was isolated: the RPA was absent in 8 and the LPA – in two patients. The first description of surgical connection of the PT with the hilar artery of ipsilateral lung in a patient with isolated absent PA was done by Kieffer et al. in 1965 [2]. A saphenous vein autograft was successfully implanted between the hilar LPA and the PT in an 8-year-old boy with isolated ALPA. We found only one paper dealing with the reconstruction of the blood flow to the right (ipsilateral) lung simultaneously with the VSD closure in an 18-months-old girl with absent RPA [3]. It is worth noting that the feasibility of reconstruction of the left lung supply in these cases was determined by the presence of the hilar LPA, which persisted due to collateral arterial blood flow. We agree with the authors' opinion on the importance of CT angiography for the identification of the hilar artery. The association of TOF with DAA is also a rare combination of CHD. Thus, according to the literature the rate of DAA in patients with TOF varies from 0.6 to 1.2%.

Sometimes, as it happened in the presented cases, DAA is not accompanied by the symptoms of tracheo-esophageal compression. Among 30 patients with DAA [isolated (n=17), associated with other CHD (n=13)], operated in our center 2 (6.7%) were free of symptoms of tracheal and esophageal compression. Two other of our patients with TOF and DAA underwent successful two-staged correction. Due to pulmonary arterial hypoplasia the first stage consisted in palliative correction: vascular ring was resected and the sectioned left small aortic arch was anastomosed with the LPA. Bahnson and Blalock in 1950 were the first to describe such an operation [4]. The feasibility of one-stage complete repair in cases of TOF with DAA and ALPA depends on the development of contralateral pulmonary artery and the left ventricle.

	References

Top References

 

1. Talwar S, Gupta A, Choudhary SK, Airan B. Absent left pulmonary artery and double aortic arch in tetralogy of Fallot: reconstruction using homograft saphenous vein or iliac artery. Interact CardioVasc Thorac Surg 2009;8:277–279.[Abstract/Free Full Text]
2. Kieffer SA, Amplatz K, Anderson RC, Lillehei CW. Proximal interruption of a pulmonary artery. Am J Roentgenol 1965;95:592–597.[Free Full Text]
3. Apostolopoulou SC, Kelekis NL, Brountzos EN, Rammos S, Kelekis DA. ‘Absent’ pulmonary artery in one adult and five pediatric patients: imaging, embryology, and therapeutic implications. Am J Roentgenol 2002;179:1253–1260.[Abstract/Free Full Text]
4. Bahnson HT, Blalock A. Aortic vascular rings encountered in the surgical treatment of congenital pulmonic stenosis. Ann Surg 1950;131:356–362.[Medline]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Leo A Bockeria Osman Makhachev Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bockeria, L. A Articles by Khiriev, T. Search for Related Content PubMed PubMed Citation Articles by Bockeria, L. A Articles by Khiriev, T. Related Collections Related Article