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Ann Thorac Surg 2001;72:1748-1750
© 2001 The Society of Thoracic Surgeons

Case report

Exsanguinating hemoptysis revealing the absence of left pulmonary artery in an adult

Pascal Thomas, MD, FECTS*a,e, Martine Reynaud-Gaubert, MD, PhDa,e, Jean-Michel Bartoli, MDb, Annie Augé, MDc, Louise Garbe, MDd, Roger Giudicelli, MDa, Pierre Fuentes, MDa

a Department of Thoracic Surgery and Anesthesiology, University Méditerranée (Aix-Marseille II), School of Medicine, Marseille, France
b Department of Intensive Care Medicine, University Méditerranée (Aix-Marseille II), School of Medicine, Marseille, France
c Department of Radiology, La Timone Hospital, Marseille, France
d Department of Pathology, St. Marguerite Hospital, Marseille, France
e UPRES EA 2201, IFR Jean Roche, Marseille, France

Accepted for publication January 19, 2001.

* Address reprint requests to Dr Thomas, Department of Thoracic Surgery and Lung Transplantation, Ste Marguerite Hospital-CHU Sud, 270 Bd Ste Marguerite, 13274 Marseille Cedex 9, France
e-mail: pathomas{at}mail.ap-hm.fr


   Abstract
Top
 Abstract
Introduction
 Comment
 Acknowledgments
 References
 
Isolated absence of a pulmonary artery is an exceptional cause of massive hemoptysis. We report a 35-year-old woman with agenesis of the left pulmonary artery who presented with exsanguinating hemoptysis that prompted angiography with the aim to embolize the bleeding vessels selectively. The procedure could not be completed because of the presence of an anterior spinal artery branching from the aberrant systemic-to-pulmonary circulation. The patient successfully underwent an emergent pneumonectomy.


   Introduction
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 Abstract
 Introduction
Comment
 Acknowledgments
 References
 
Massive hemoptysis is an unusual, and life-threatening disease. Unilateral absence of a pulmonary artery diagnosed in adulthood is rather anecdotal. The conjunction of both conditions is an extremely rare and challenging event.

A 35-year-old woman was transferred to our institution for massive and recurrent hemoptysis. Her history included three episodes of more than 500/mL of blood expectorated over 48 hours. Besides, two episodes of blood-tinged sputum that resolved spontaneously first occurred 4 years earlier. No other symptoms could be retrieved, although the patient reported a lack of endurance, especially on the occasion of mountain trekking, and a history of recurrent mild respiratory infections since childhood.

Physical examination revealed an anxious, pale and moist patient, with mild dyspnea. The blood pressure was 100/60 mm Hg, the pulse was 120 beats/min, and the respiratory frequency 26 cycles/min. Room air pulse oxymetry was 93% necessitating 31/min of supplemental oxygen. The hemogram confirmed a substantial blood loss (red cells count: 3.090 Tera/L, hemoglobin: 5.6 mmol/L).

The chest x-ray on admission showed a reticular-lacy pattern in the left lung, and a confluent alveolar filling in the right lower lobe as the result of blood aspiration. No active bleeding nor any intrinsic abnormality of the bronchial tree was seen at bronchoscopy. Chest computed tomographic (CT)-scan revealed the absence of the left pulmonary artery, but the presence of a multitude of small vessels at the hilum. No other cardiovascular abnormality was noted, except a right descending aorta (Fig 1).



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  		Fig 1. Computed tomographic (CT) scan showing a smaller left lung than the right one, a right descending aorta, the absence of left pulmonary artery, and the presence of a multitude of small vessels (one of them labeled with an asterisk). (PA = pulmonary artery.)

 
The same day, another new 500 mL hemorrhage occurred, that prompted emergent angiography with the aim to embolize the bleeding vessels selectively. Global and selective opacifications showed the extensive development of systemic-to-pulmonary collateral vessels originating from the bronchial arteries (Fig 2A) and the left subclavian artery (Fig 2B), with a normal venous drainage (Fig 2C). Selective embolization was not attempted because of the presence of an anterior spinal artery arising from one of these aberrant branches. The patient was thus taken directly to the operating room.



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  		Fig 2. Selective arteriograms of anomalous systemic-to-pulmonary collateral vessels supplying the left lung. Selective arteriography of an enlarged and tortuous left bronchial trunk (A). Selective arteriography of the left subclavian artery with aberrant hypertrophic branches to the lung demonstrating a medullary artery (B). Normal venous return (C).

 
At operation, numerous prominent feeding vessels converging towards the lung were noted, originating from the parietal arteries. The hilum and the inferior pulmonary ligament also convoyed additional aberrant arteries issued from enlarged mediastinal and bronchial vessels. The vessel originating from the subclavian artery was identified entering the upper lobe. One vessel originating from a subdiaphragmatic artery was also identified crossing the diaphragmatic dome and penetrating the lower lobe. The main pulmonary artery was a tiny cord without lumen. In contrast, pulmonary venous drainage was anatomically normal. The aberrant systemic-to-pulmonary vasculature was electively ligated step-by-step, and pneumonectomy was otherwise performed in a routine fashion.

Postoperatively, the patient experienced aspiration of blood and clots in the remaining lung that necessitated fiberoptic bronchial toilet. However, she was discharged on day 12, and had an uneventful course in 18 months of follow-up.


   Comment
Top
 Abstract
 Introduction
 Comment
Acknowledgments
 References
 
Unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly that results principally from the failure of development of the ventral bud of the ipsilateral 6th aortic arch. More frequently, UAPA is combined with severe congenital cardiovascular malformations, hence explaining why the majority of individuals born with this condition die in the neonatal period or during infancy. In the absence of an associated cardiovascular shunt, few patients with UAPA will develop pulmonary hypertension [1]. Thus, isolated UAPA may be undetected until an incidental finding on routine chest x-ray, or the appearance of complications such as hemoptysis or recurrent pulmonary infections [2]. Undiagnosed smokers with this condition may live long enough to develop a bronchogenic carcinoma [3]. Some patients with uncomplicated UAPA complain from dyspnea on exertion, the mechanism of which remains unclear. As a matter of fact, pulmonary function test profiles including arterial blood gas at rest and after exercise, when available, may be within normal limits [4].

If diagnosed in a newborn, this anomaly may be accessible to a corrective surgery because the basic anomaly found at that age is a pulmonary artery discontinuity with a truncal stump and relatively normal distal branches [5]. In older individuals, intrapulmonary arteries consist of a severely narrowed or completely obstructed fibrous network. In those cases, the affected lung receives its blood-supply through numerous expanded bronchial arteries and additional aberrant vascularization originating from the persistence of embryonic channels and the progressive enlargement of the collaterality from the normal remaining post-aortic arch vessels.

As a consequence of receiving a systemic blood-flow, the capillary turgidity may result in endobronchial and intra-alveolar hemorrhages. Indeed, about 10% of the patients with UAPA develop inconsequential hemoptysis. In those cases, the clinical and radiologic features may mimic a pulmonary embolism with a suggestive ventilation-perfusion scan, and lead to inappropriate anticoagulation or thrombolytic therapy [6]. In contrast, massive hemorrhage is very uncommon. Bronchial artery embolization has been attempted successfully to control bleeding in some previously reported patients with UAPA. However, when long-term follow-up is available, it appears that the extensive development of the alternate vasculature exposes to the risk of partial embolization, or recruitment of other systemic collaterals, and finally failure of the procedure [7]. As typified by our case, the arterial anatomy should be evaluated thoroughly in order to detect spinal arteries arising from the aberrant bronchial circulation, and prevent spinal cord injury.

Finally, emergent pneumonectomy in the context of an exsanguinating hemoptysis carries the paradox to be a definitive and life-saving treatment, as well as a high-risk procedure. Successful pneumonectomy has been sporadically reported in the literature since 1970 [8]. However, uncontrollable bleeding during surgery has been described as well [2]. As experienced by our patient, aspiration of blood and clots in the remaining lung may jeopardize the early outcome. In that way, selective embolization if feasible, would certainly permit to postpone the operation after the acute phase and to prepare the patient in less dramatic conditions.


   Acknowledgments
Top
 Abstract
 Introduction
 Comment
 Acknowledgments
References
 
Supported by Assistance Publique, Hôpitaux de Marseille, France.


   References
Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 

  1. Pool P., Vogel J., Blount G. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 1962;10:706-732.[Medline]
  2. Bahler R., Carson P., Traks E., et al. Absent right pulmonary artery. Problems in diagnosis and management. Am J Med 1969;46:64-71.[Medline]
  3. Roman J., Jones S. Case report: congenital absence of the left pulmonary artery accompanied by ipsilateral emphysema and adenocarcinoma. Am J Med Sci 1995;309:188-190.[Medline]
  4. Bouros D., Pare P., Panagou P., et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108:670-676.[Abstract/Free Full Text]
  5. Moreno-Cabral R.J., McNamara J.J., Reddy V.J., Caldwell P. Unilateral absence of pulmonary artery: surgical repair with a new technique. J Thorac Cardiovasc 1991;102:463-465.[Medline]
  6. Vohra N., Alvarez M., Abramson A., Lockwood C. Hypoplastic pulmonary artery: an unusual entity mimicking pulmonary embolism during pregnancy. Obstet Gynecol 1992;80:483-485.[Medline]
  7. Mehta A., Livingston D., Kawalek W., et al. Pulmonary artery agenesis presenting as massive hemoptysis. A case report. Angiology 1987;38:67-71.
  8. Byrne R., Bloom D. Absence of the right pulmonary artery as a cause of hemoptysis. J Thorac Cardiovasc Surg 1970;592:264-268.



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