Successful percutaneous intervention in a rare case of aberrant systemic artery to a normal lung presented with hemoptysis

Abstract

Aberrant systemic artery to the lung without evidence of sequestration is a rare congenital anomaly. Treatment of choice for this anomaly is controversial. A 27-year-old man with bicuspid aortic valve (AV) and a history of AV replacement was admitted to our center with hemoptysis. Aberrant systemic artery to the lung without sequestration was diagnosed. Endovascular closure of aberrant vessel with septal occluder was performed. After 2 years the previous device was embolized to the distal part and he came back with hemoptysis. The second endovascular procedure was performed with a larger device with a successful result. There are surgical and interventional treatment options for systemic arterialization to a normal lung without sequestration. In this case we used Amplatzer septal occluder (St. Jude Medical, Minneapolis, MN, USA) for occlusion of the aberrant vessel.

<Learning objective: Systemic arterialization of the lung is a rare congenital anomaly, the treatment of choice for this anomaly is controversial, using septal occluder may be a useful method for management of this anomaly. Here we report a patient with this rare anomaly who had an educational clinical course. The type of management performed is not reported in other articles with similar cases.>

Introduction

Systemic arterialization of the lung without sequestration is a rare congenital anomaly [1]. There are surgical and interventional treatment options for this anomaly. The treatment of choice in this anomaly is controversial. Here we report a unique case of aberrant systemic artery to the lung which was managed by percutaneous intervention.

Case report

A 27-year-old man was admitted to our center because of hemoptysis in November 2018. He had a history of bicuspid aortic valve (AV) and AV replacement 15 years previously. He had no fever, palpitation, or chest pain. He was a non-smoker and denied positive family history. His-vital signs were stable. There was no cyanosis, clubbing, and no signs of heart failure. Cardiovascular examination had no important points. He had no previous history of tuberculosis or recurrent pulmonary infections. The complete blood profile was normal. He was under treatment with warfarin and his coagulation tests were within therapeutic range. The electrocardiography, chest X-ray, and bronchoscopy had no abnormal findings. Transthoracic echocardiography showed mild left ventricular enlargement and systolic dysfunction, mechanical bileaflet AV prosthesis had acceptable leaflet motion and gradient. Contrast-enhanced chest computed tomography angiography (CT angiography) showed a dilated anomalous artery (12 mm in diameter) arising from lateral aspect of descending aorta supply to left basal segments and normal pulmonary artery branches supplying the affected area (Fig. 1A). No direct communications existed between the anomalous artery and the veins of the basal segments. The veins of the basal segments drained into the left atrium via inferior pulmonary vein. Final diagnosis was made as anomalous systemic arterial supply to the left lower lobe without pulmonary sequestration. CT angiography convinced us of acceptable pulmonary perfusion especially in left lower pulmonary lobe (Fig. 1B).

Fig. 1.

An axial computed tomography (CT) section from CT pulmonary angiography (CTPA) in mediastinal window shows an aberrant artery (arrow) (A). Coronal reformatted maximal intensity projection image from CTPA shows that the basal segments of the left lung receive their arterial supply from descending thoracic aorta via an aberrant artery (arrow) (B). First angiography before device employment, white arrow shows the aberrant vessel (C).

We scheduled the patient for endovascular intervention. Catheterization was done using 4 French (Fr.) femoral sheath and catheters. Abdominal aortogram revealed an aberrant vessel originating from the descending aorta (Fig. 1C). Pulmonary injection proved acceptable pulmonary perfusion. Due to malalignment with femoral approach, the procedure was performed via left brachial access. We worried about extension of the device to the aorta. By CT angiogram we found the smallest part of the aberrant artery was about 11 mm, then we thought we could try OCCLUTECH (Jena, Germany) muscular ventricular septal defect (VSD) size 12 mm as duct occluder, we deployed the device at the narrowest part of the aberrant vessel and after deployment we checked the stability by pushing and pulling maneuvers. Post procedural injection showed no residual flow to the distal part of the aberrant vessel. The patient's recovery was uneventful, and he was free from hemoptysis during follow-up.

In January 2020 after 2 years he presented again with hemoptysis. Contrast-enhanced chest CT angiography was performed. The previous device was embolized to the distal part of the aberrant vessel (Fig. 2). The patient was subjected to angiography for another intervention. Abdominal aortogram revealed the same aberrant vessel originating from the descending aorta and the previous device was embolized to the distal part of the aberrant artery without occlusion of the arterial blood flow. He was again a candidate for endovascular closure of the aberrant vessel.

Fig. 2.

An axial computed tomography (CT) section from CT pulmonary angiography in mediastinal window after second admission due to hemoptysis recurrence shows embolization of the previous device to the distal part of the aberrant vessel (arrow).

For the second attempt we decided to implant the device in the proximal part of the aberrant vessel and place the proximal disc of septal occluder in the aorta. In this way the device was less likely to shrink and embolize to the distal part as happened after the first attempt. First, we proved no major branch separation from aorta near the aberrant vessel origin. Again we chose left brachial artery approach for sending 8 Fr. sheath to the target artery and by pigtail from femoral course we checked the precise deployment of Amplatzer septal occluder 19 mm device (St. Jude Medical, Minneapolis, MN, USA) (Fig. 3A). We used this size of occluder as its aortic side was much higher than the waist part and it is impossible to emigrate to the distal part. The distal disc of this device was bigger enough than the tunnel side of the aberrant vessel and pushing and pulling maneuvers convinced us about fixing the device in position. At the end of the procedure we checked the proximal and distal part of the aorta (at the origin of aberrant vessel) for possible gradient so we ruled out aortic obstruction and compression by occluder d

Fig. 3.

Closure of the aberrant vessel using Amplatzer septal occluder 19 mm (black arrow) and the previous device embolized to distal part (white arrow) (A). An axial computed tomography (CT) section from CT pulmonary angiography in mediastinal window shows the second device used for aberrant vessel occlusion (black arrow) and the previous device embolized to distal part (white arrow) (B).

He recovered from hemoptysis and the device was in the proper position in further imaging done for follow up (Fig. 3B).

Discussion

Isolated systemic arterial supply to a normal lung without bronchial sequestration is a rare congenital abnormality [1]. However, this anomaly had normal bronchial communications and the involved lung segment was not sequestrated [2].

Focal pulmonary hypertension and high-output cardiac failure develops by systemic pressure transmission to the pulmonary vasculature [1]. The anomalous artery most commonly arises from the thoracic aorta and there is normal venous drainage by inferior pulmonary vein into the left atrium [3].

These patients are mostly asymptomatic, and sometimes present with hemoptysis especially in Asian patients, exertional dyspnea, and overload of left heart [1,4]. The main differential diagnosis of this abnormality is pulmonary sequestration. It is essential to confirm the presence of normal tracheobronchial connections [2].

CT angiography is an appropriate diagnostic method for evaluating the origin and course of aberrant artery.

It is recommended to treat all patients suffering from this anomaly with or without symptoms due to the potential risk of developing congestive heart failure, recurrent infections, and hemorrhagic complications [5].

The goal of treatment is to relieve the left ventricle volume overload and patient's symptoms. There are surgical and endovascular treatment options. When aberrant systemic artery is the only supply, surgical lobectomy is preferred. Surgical ligation or endovascular treatment (embolization) for the aberrant vessel occlusion is used when the involved segment has dual blood supply [1].

In reviewing the literature, surgical procedures were done in some studies. Currarino et al. used partial lobectomy and vessels ligation. They reported high incidence of mortality in their cases [6].

Jariwala et al. described a case of anomalous aberrant systemic artery to pulmonary venous fistula. There was normal pulmonary arterial and venous supply to both the lungs which was closed with embolization Amplatzer vascular plugs [2].

Geyik et al. described a case of systemic artery to pulmonary artery malformation where the systemic artery drained into the pulmonary artery. They occluded the artery with the help of coils, glue, and detachable balloon [7].

Kristensen et al. described endovascular treatment of an aneurysmal aberrant systemic artery supplying a pulmonary sequestrum by Amplatzer vascular plug II [8].

In reviewing the literature, trans-arterial embolization with metallic coil or Amplatzer vascular plug has been reported in anomalous systemic arterial supply and pulmonary sequestration [9,10]. The interventional embolization is more effective and minimally invasive than surgery.

In our patient for the first procedure we worried about extension of the device to the aorta so we tried OCCLUTECH muscular VSD size 12 mm as duct occluder for the patient with a good result and no complications, but after 2 years the device was embolized to the distal part.

We thought the reason of the first procedure failure was gradual shrinkage of device over time and that is why the device was distal embolized, so we decided to implant the device in the proximal part of the aberrant vessel and place the proximal disc of the septal occluder in the aorta after confirmation of no side branch separation near the origin of the aberrant vessel. In this manner, shrinkage and distal embolization of the device was less likely to happen.

After device deployment, we checked aorta gradient before and after the device for excluding obstruction and compression of the aorta by occluder. To the best of our knowledge, we are the first to use interventional closure of anomalous systemic arterial supply to the normal basal segments by septal occluder. In Iran occluder devices are covered by insurance.

Further follow up of the case will answer the long-term prognosis of this procedure.

Conclusion

Anomalous systemic artery to a normal lung without pulmonary sequestration is rare and few cases have been reported similar to our case. Here we report a patient who had educational clinical course. The type of management performed is not reported in other articles with similar cases. First we occluded the narrowest part of aberrant vessel using septal occluder without extension to aorta as we worried about aortic obstruction with the device's disc. The success of this procedure did not take more than 2 years and the patient's symptoms recurred due to device embolization.

To eliminate the possibility of device shrinkage and distal embolization we placed the proximal disc of the septal occluder in the aorta for the second attempt.

For patients with complicated conditions, percutaneous interventions could be a safer alternative. However, further follow up can determine the exact prognosis of this treatment option. The clinical course and type of device used for management of this rare congenital case have not been reported in similar cases.

Declaration of Competing Interest

No potential conflict of interest relevant to this article was reported.