Ann Thorac Surg 1998;66:251-252
© 1998 The Society of Thoracic Surgeons
Case Reports
Unilateral absence of pulmonary artery and ventricular septal defect in an infant
Kazuhito Imanaka, MDa,
Susumu Shimizu, MDa,
Jun Matsumoto, MDa,
Kohei Hashizume, MDb,
Keiji Tsuchiya, MDc,
Tamiko Takemura, MDd
a Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, Tokyo, Japan
b Department of Pediatric Surgery, Japanese Red Cross Medical Center, Tokyo, Japan
c Department of Pediatrics, Japanese Red Cross Medical Center, Tokyo, Japan
d Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan
Accepted for publication January 30, 1998.
Address reprint requests to Dr Imanaka, Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo 113, Japan
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Abstract
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A 2-month-old boy was diagnosed as having a rare combination of absence of left pulmonary artery and ventricular septal defect. He underwent intracardiac repair at the age of 8 months. Reconstruction of the left pulmonary artery was impossible because it could not be visualized through a median sternotomy. Although his early postoperative course was uneventful, he needed ipsilateral pneumonectomy 3 months later because of unremitting hemoptysis. Hilar left pulmonary artery, which used to exist, was not found even by histologic examination.
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Introduction
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Unilateral absence of the pulmonary artery (PA) is a rare anomaly. It is thought to result from failure of the sixth aortic arch to connect with the pulmonary trunk [1]. Here we describe a patient with left unilateral absence of the PA and a ventricular septal defect.
A 2-month-old boy weighing 4.4 kg was noted to have systolic murmur, tachypnea, and retracted breathing. Chest radiography showed an emphysematous and hypervascular right lung, a small, hypovascular left lung, and leftward deviation of the enlarged cardiac silhouette. Cardiac catheterization revealed left unilateral absence of the PA, a ventricular septal defect, right aortic arch, pulmonary hypertension, and a ductus dimple in the left innominate artery. The PA pressure was 88/39 mm Hg. Pulmonary vascular resistance was 10.4 Wood units · m2, and the pulmonary-to-systemic flow ratio was 1.2. The origin of the left PA was absent. Pulmonary venous wedge injection showed that a vessel about 3 mm in diameter, which appeared to be the intrapulmonary left PA, was filled with contrast material to near the hilum (Fig 1).
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Fig 1. Angiography at 2 months of age. (A) Right ventriculography. The origin of the left pulmonary artery was entirely absent. (B) Wedge injection into the left pulmonary vein. A vessel that appeared to be parenchymal pulmonary artery (arrow) existed.
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Because the patient’s clinical symptoms were mild, observation with medication was continued. Episodes of respiratory infection recurred during follow-up, so he underwent repeat catheterization at 7 months of age when he weighed 5.8 kg. Pulmonary artery pressure had diminished to 48/21 mm Hg and pulmonary vascular resistance was 2.3 Wood units · m2. The pulmonary-to-systemic flow ratio increased to 2.0. Therefore, patch closure of the ventricular septal defect and reconstruction of the left PA through a median sternotomy were planned.
At operation, no tissue that suggested a remnant of the intrapericardial left PA was found. The left mediastinal pleura was then incised. However, the left PA was not visible because the left hilum was shifted and completely hidden behind the heart. Therefore, patch closure of the ventricular septal defect and left lung biopsy were performed.
The patient recovered uneventfully. Because histologic examination of the lung biopsy specimen showed hypoplasia of the bronchioles, alveoli, and vessels, PA reconstruction through another approach was not undertaken. However, hemoptysis began to occur 2 months after the operation, and episodes of massive hemoptysis recurred. Angiography at 11 months of age showed many collateral vessels from the innominate artery and descending aorta (Fig 2). The target vessels for embolization, however, could not be determined. He needed left pneumonectomy. The pulmonary vein was single, and the hilar PA was histologically not found. Some vessels beside the segmental and subsegmental bronchi were narrow and thickened. Some were thin walled. The alveolar development was normal in most parts. He recovered uneventfully and has since been doing well for 4 years.
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Fig 2. Digital subtraction angiography at 11 months of age. Many collateral vessels to the left lung from the brachiocephalic artery and descending aorta were observed. However, it was unclear where the origin of hemoptysis was or which vessel should be embolized. No infradiaphragmatic arterial connection to the left lung was observed.
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Comment
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Among several problems that should be considered for better management of patients with unilateral absence of the PA, indication and timing of PA reconstruction are important and controversial [2–4]. Unilateral absence of the PA can interfere with the patient’s growth, and unfavorable sequelae such as fatal pulmonary hypertension [3, 5], hemoptysis [6], and infection [7] can develop, which need pneumonectomy for salvage in some cases [6, 7]. Pulmonary artery reconstruction can be an effective prevention against such sequelae in patients who have reconstructable PA and reparable lung.
In the present case, the left hilar PA occluded between 2 months and 11 months of age. A lung with an occluded PA is of little use. Therefore, an operation should be undertaken as early as possible [4]. When observation is continued, the interval of catheterization should not be long and the patency of the parenchymal PA must be reconfirmed every time. Arteriovenous fistula of the PA [3] must be ruled out. In this case, the resected left lung itself appeared reparable, although lung hypoplasia was suggested formerly. Pulmonary artery reconstruction may, therefore, not have been given up only with a disappointing histologic examination of a single lung biopsy specimen.
The surgical strategy also needs due consideration. One-stage repair may be difficult, as in this case. Preoperative three-dimensional recognition of the best approach and the distance to the hilum by computed tomography or magnetic resonance imaging is essential. Such examination may reveal that staged repair is preferable in some cases. In patients with a right-to-left shunting anomaly, an ipsilateral systemic-to-pulmonary shunt is a good choice as the initial operation of the staged repair. In patients with left-to-right shunting anomaly, PA reconstruction increases shunt flow and should not be the initial operation, let alone systemic-to-pulmonary shunt. It is safer to perform intracardiac repair first, and then PA reconstruction next.
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References
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- Cucci C.E., Doyle E.F., Lewis E.W., Jr Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 1964;29:124-131.[Abstract/Free Full Text]
- Moreno-Cabral R.J., McNamara J.J., Reddy V.J., Caldwell P. Unilateral absent pulmonary artery: surgical repair with a new technique. J Thorac Cardiovasc Surg 1991;102:463-465.[Medline]
- Shakibi J.G., Rastan H., Nazarian I., Paydar M., Aryanpour I., Siassi B. Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair. Jpn Heart J 1978;19:439-451.[Medline]
- Presbitero P., Bull C., Harworth S.G., de Leval M.R. Absent or occult pulmonary artery. Br Heart J 1984;52:178-185.[Abstract/Free Full Text]
- Pool P.E., Vogel J.H.K., Blount S.G. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 1962;10:706-729.[Medline]
- Taguchi T., Ikeda K., Kume K., et al. Isolated unilateral absence of left pulmonary artery with peribronchial arteriovenous malformation showing recurrent hemoptysis. Pediatr Radiol 1987;17:316-318.[Medline]
- Canver C.C., Pigott J.D., Mentzer R.M., Jr Neonatal pneumonectomy for isolated unilateral pulmonary artery agenesis. Ann Thorac Surg 1991;52:294-295.[Abstract]
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Abstract
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