Asian Cardiovasc Thorac Ann 2008;16:e18-e20
© 2008 Asia Publishing EXchange Ltd
Double Outlet Right Ventricle with Anomalous Left Pulmonary Artery
Leo A Bockeria, MD,
Osman A Makhachev, MD,
Andrey V Sobolev, MD,
Viktoria V Plakhova, MD,
Sergey V Gorbachevsky, MD,
Sergey B Zaets, MD
Bakoulev Scientific Center for Cardiovascular Surgery, Moscow, Russia
For reprint information contact: Sergey B Zaets, MD, Tel: 1 201 346 8357, Fax: 1 609 580 8717, Email: zaets001{at}yahoo.com, 1 Wall Street, Apt. 5B, Fort Lee, NJ 07024, USA.
 |
ABSTRACT
|
|---|
We describe a rare case of anomalous origin of the left pulmonary artery from the ascending aorta with concomitant double-outlet right ventricle in a 2-year-old boy. He underwent successful 2-stage surgical treatment with transluminal balloon pulmonary valvuloplasty, followed by complete repair. A follow-up examination at 4 years after the operation showed good results.
|
INTRODUCTION
|
|---|
Anomalous origin of a pulmonary artery from the ascending aorta is a rare anomaly, usually reported in small series. Anomalous origin of the left pulmonary artery (LPA) from the ascending aorta occurs 4–8 times less frequently than anomalous right pulmonary artery (RPA). Anomalous LPA in a patient with tetralogy of Fallot was first described by Sikl.1 Apart from tetralogy of Fallot, the most frequent concomitant lesions in patients with anomalous RPA or LPA are patent ductus arteriosus, aortopulmonary window and aortic arch anomalies. Usually, anomalous RPA or LPA is associated with situs solitus with concordant atrioventricular and ventriculoarterial connection. The combination of anomalous pulmonary arteries with anomalous connection of cardiac segments is extremely rare. We found only one case each of anomalous RPA with tricuspid atresia, and anomalous RPA or LPA with double-outlet right ventricle (DORV).2–4 We describe a rare combination of anomalous LPA arising from the ascending aorta with DORV, which was successfully treated by staged surgical interventions.
|
CASE REPORT
|
|---|
A boy aged 2 years and 3 months was referred to our center with major complaints of respiratory distress, cyanosis and cyanotic spells noted at 3 and 15 months of age. Chest radiography showed asymmetric pulmonary vascularity and cardiomegaly. Two-dimensional echocardiography revealed concordant atrioventricular connection and DORV with a subaortic ventricular septal defect, valvular and subvalvular pulmonary stenosis, and anomalous LPA arising from the ascending aorta. Cardiac catheterization indicated severe hypertension in the LPA (Table 1
). Angiocardiography confirmed the diagnosis of DORV with anomalous LPA (Figure 1). Kinking of the left common carotid artery and an anomalous right subclavian artery were also evident. Angiometry demonstrated proximal stenosis of the LPA. Transluminal balloon pulmonary valvuloplasty of the pulmonary valvular stenosis was performed as first-stage surgical treatment, which resulted in increased pulmonary trunk and RPA pressure, up to 37/6 mm Hg (mean, 20 mm Hg), with increased pulmonary-to-systemic blood flow ratio to 0.79 and O2 saturation to 82%. Right lung edema was conservatively treated successfully during the first 24 hours. Three months after the first procedure, complete repair of the lesion was undertaken under cardiopulmonary bypass (211 min), hypothermia (minimal rectal temperature 23°C), and aortic cross clamping (153 min). Partial clamping of the LPA prior to the intervention showed no contraindications to repair. During the operation, the LPA was dissected from the aorta. The defect in the ascending aorta was closed with a Gore-Tex patch. After resection of the right ventricular outflow tract stenosis, a tunnel was created from the left ventricle to the aorta using a xenopericardial patch. Continuity between the right ventricle and the LPA was achieved using a xenopericardial conduit with a no. 21 mechanical valve (MIKS; Russia). Atrial fenestration (4 mm in diameter) was performed for right ventricular decompression. In spite of good immediate postoperative hemodynamics (right ventricle/aorta pressure ratio of 50/90 mm Hg), sternal closure was delayed. In the postoperative period, heart failure required cardiotonic support, sternal closure on the 3rd day, and prolonged mechanical ventilation with NO inhalation (8 days). Atrial septal fenestration provided a "pop-off" in the first postoperative days, which resulted in a temporary decrease in O2 saturation to 88%–90%. The patient was discharged on sildenafil and warfarin 30 days after surgery. At follow-up 4 years after complete repair, he was in NYHA functional class I–II on warfarin, with no cyanosis, heart murmur or hepatomegaly. Radiography revealed normal pulmonary vascularity and moderately enlarged right cardiac chambers. Echocardiography showed normal function of the atrioventricular valves. Peak and mean pressure gradients across the mechanical valve of the conduit reached 23 and 11 mm Hg, respectively, and his left ventricular ejection fraction was 63%.
View larger version (93K):
[in this window]
[in a new window]
|
Figure 1. Preoperative angiocardiograms; (A) Aortogram showing the left pulmonary artery arising from the ascending aorta, kinking of the left common carotid artery, and anomalous right subclavian artery; (B) Right ventriculogram showing both great arteries arising from an enlarged right ventricle, subpulmonary and pulmonary stenosis; the only continuation of the pulmonary trunk is the right pulmonary artery.
|
|
|
DISCUSSION
|
|---|
Successful complete repair of tetralogy of Fallot with anomalous LPA was first performed by Kirklin and colleagues.5 World experience of surgery in anomalous LPA does not exceed 40 cases. Wang and colleagues6 described a case of 2-stage palliative surgical treatment of DORV with concomitant anomalous LPA, which included banding of the pulmonary trunk, re-implantation of the LPA to the pulmonary trunk, ligation of the patent ductus arteriosus and delayed modified Blalock-Taussig shunt. To our knowledge, our patient is the first to undergo complete repair of DORV with concomitant anomalous LPA arising from the ascending aorta. At the palliative stage, pulmonary valvuloplasty was performed to increase blood oxygen saturation. For complete repair, a xenopericardial conduit with a mechanical valve was chosen to achieve good valvular function in a patient with severe pulmonary hypertension in the left lung, despite concerns regarding the need for anticoagulants. The risk associated with complete repair of cardiac lesions in patients with anomalous LPA is mainly caused by morphological changes in the vascular bed of the hypertensive lung; therefore, surgical correction is recommended during the first year of life. In our patient over 2-years old, proximal stenosis of the LPA probably served as a protective factor against severe irreversible vascular changes in the left lung. Heart failure in the early postoperative period might be explained by the pre-existing pulmonary hypertension.
We support the opinion that partial clamping of the anomalous pulmonary artery is an appropriate test prior to complete repair to evaluate the severity of vascular changes.7 Lung biopsy should also be considered. Other surgical strategies in patients with anomalous LPA arising from the ascending aorta and ipsilateral vascular changes are: correction of the concomitant cardiac defect combined with ligation or embolization of the anomalous pulmonary artery; complete repair with anastomosis between the LPA and the pulmonary trunk or right ventricle; and a right-sided systemic-to-pulmonary shunt. The described method of total repair, using a xenopericardial conduit with a mechanical valve to connect the right ventricle and LPA, might be considered as an alternative technique that may provide good early and midterm results.
|
REFERENCES
|
|---|
- Sikl H. Unusual malformation of the ductus arteriosus; departure of a principal branch of the pulmonary artery from the aorta. Cas Lek Cesk 1952;91:1366–9.[Medline]
- Menzer LL, Takahashi M. Tricuspid atresia and aortic origin of right pulmonary artery in a nine-year-old girl. West J Med 1974;121:147–9.[Medline]
- Gybels Y, Grapow MT, Todorov A, Wagner G, Zerkowski HR. Aberrant right pulmonary artery and double outlet ventricle: one-stage repair. Ann Thorac Surg 2000;69:630–2.[Abstract/Free Full Text]
- Wong JA, Cheng W. Preoperative management of right ventricular dysfunction in aortic origin of the right pulmonary artery. Pediatr Cardiol 2000;21:388.[Medline]
- Kirklin JW, Wallace RB, McGoon DC, DuShane JW. Early and late results after intracardiac repair of tetralogy of Fallot. 5-year review of 337 patients. Ann Surg 1965;162:578–89.[Medline]
- Wang JN, Wu JM, Yang YJ. Double-lumen aortic arch with anomalous left pulmonary artery origin from the main pulmonary artery-bilateral persistent fifth aortic arch-a case report. Int J Cardiol 1999;69:105–8.[Medline]
- Py A, Lazarus A, Spaulding C, Toussaint M, Planche C, Duboc D, et al. Left pulmonary artery originating from the ascending aorta in tetralogy of Fallot. Therapeutic strategy. Arch Mal Coeur Vaiss 1993;86:1069–72.[Medline]
TOP
ABSTRACT
INTRODUCTION
