Superior vena cava stenosis presenting with bendopnea

Abstract

Bendopnea, or dyspnoea with bending forward, is a recently described symptom of heart failure that is associated with elevated ventricular filling pressures. Here, we describe a case of superior vena cava (SVC) stenosis that presented with bendopnea and resolved with SVC recanalisation. We suggest that SVC stenosis be considered in the differential diagnosis of patients who experience bendopnea.

Background

Bendopnea, or shortness of breath when bending forward at the waist such as when tying one’s shoes, is associated with elevated ventricular filling pressures.¹ Bendopnea is considered present if the dyspnoea occurs within 30 s of bending while not holding one’s breath. While first described in patients with advanced heart failure (HF), it has subsequently been described in patients evaluated in primary care or HF clinics, referred for cardiopulmonary exercise testing, admitted with decompensated HF or treated for pulmonary arterial hypertension. The reported prevalence of bendopnea in these populations ranged from 18% to 49%.^2–7

Superior vena cava (SVC) stenosis results from any condition that leads to obstruction of blood flow through the SVC. Previously, most cases were ascribed to malignancy either by direct invasion of a tumour into the SVC or by external compression of the SVC by an adjacent mediastinal process. However, benign aetiologies are increasingly recognised, a consequence of the increasing use of intravascular devices such as indwelling catheters.⁸ SVC stenosis classically presents with facial swelling or head fullness, arm swelling, dyspnoea or neurological manifestations. We present a patient who had SVC stenosis and developed progressive symptoms, including shortness of breath, elicited by bending, leading to his referral to an advanced HF specialist (JTT) to further evaluate the aetiology of his bendopnea.

Case presentation

A 57-year-old man had a history of non-ischaemic cardiomyopathy with complete left ventricular reverse remodelling, as well as known SVC stenosis diagnosed approximately 20 years previously as a complication from a longstanding ventriculoatrial shunt. He had progressive symptoms, including shortness of breath with bending, over the preceding 15 years. Evaluations by pulmonologists demonstrated normal pulmonary function tests. A new pulmonologist assumed his care, was concerned that the patient’s bendopnea was a manifestation of his cardiomyopathy, and referred him to the advanced HF clinic for further evaluation.

The patient was extremely limited in any kind of activity that required him to bend forward, including putting on shoes, doing yardwork or bending to pick up items. However, he could walk an unlimited amount on a flat surface, go up one flight of stairs without difficulty and was able to sleep supine without paroxysmal nocturnal dyspnoea. He described his symptoms with bending as dyspnoea, abdominal thumping, facial flushing, and feeling pressure build-up in his face and eyes. He reported that these symptoms had been progressive over the last 15 years.

Investigation

On physical examination, the patient had no jugular venous distension, hepatojugular reflux or peripheral oedema. When tested in the clinic, he developed his version of bendopnea within 12 s of bending. His N-terminal pro B-type natriuretic peptide was 27 pg/mL, and his recent transthoracic echocardiogram showed normal biventricular function with a left ventricular ejection fraction of 52%, no significant valvular abnormality, no evidence of diastolic dysfunction, and a normally-sized inferior vena cava that collapsed appropriately with inspiration consistent with a normal central venous pressure.

Given the patient’s normal pulmonary function tests, lack of evidence of elevated ventricular filling pressures, and coexisting symptoms of abdominal thumping, facial flushing and pressure with bending, it was felt that the SVC stenosis may be the aetiology of his symptoms. A CT scan of his chest revealed severe and chronic occlusion of the SVC (figure 1).

Figure 1.

CT of the chest demonstrating multiple areas of severe and chronic occlusion of the superior vena cava.

Treatment

The patient was referred to interventional radiology who performed a venogram and stenting of the SVC with two overlapping 16 mm×6 cm stents (figure 2), with improvement in venous flow (figure 3).

Figure 2.

Still frame of superior vena cava angioplasty.

Figure 3.

Still frame after superior vena cava angioplasty demonstrating increased venous flow.

Almost immediately, the patient reported a significant improvement in his symptoms such that when he bent forward, he no longer experienced dyspnoea, head fullness or pressure, or facial flushing. However, 12 days after this initial procedure, he developed pain along the right side of his neck and a recurrence of his previous symptoms. He underwent repeat imaging with a CT scan which demonstrated that the distal end of the stent abutted the wall of the left brachiocephalic vein (figure 4). He thus re-presented to interventional radiology who placed an additional stent, extending the distal aspect of the stent into the left brachiocephalic vein. The post-procedure venogram showed the prior and newly placed stents were patent with improved venous flow.

Figure 4.

CT scan demonstrating the superior vena cava stent abutting the wall of the brachiocephalic vein.

Outcome and follow-up

Following SVC recanalisation, the patient felt complete resolution of his symptoms with bending and felt that his ‘life had been restored’. His symptoms have not returned after 2 years of follow-up and the patient expressed extreme gratitude for the improvement in his quality of life.

Discussion

Since its first description in 2014, bendopnea has been described in several other patient populations with HF.^2–6 Bendopnea has also been observed in some patients without HF, including those with pulmonary artery hypertension,⁷ allergic bronchopulmonary aspergillosis⁹ and severe aortic stenosis.¹⁰ To our knowledge, this is the first case where bendopnea was among the presenting symptoms of SVC stenosis.

The typical symptoms of SVC stenosis are facial or neck swelling, upper extremity swelling, dyspnoea when not bending, cough and dilated chest vein collaterals.¹¹ Our patient differed because his symptoms, including dyspnoea, head fullness and facial flushing, manifested only with bending. The resolution of his bendopnea and associated symptoms with the initial stenting of the SVC, and then recurrence when the stenting was no longer patent, strongly suggest that his bendopnea was a consequence of SVC stenosis. It is likely that bendopnea in this case was not related to elevation of intracardiac pressures, but perhaps from pressure of the collateral vessels against the airway or phrenic nerve,^{12 13} though we can only speculate at this juncture.

This case has clinical implications given that relief of SVC stenosis led to an improvement and even complete resolution of symptoms; thus, it is important to identify this cause of bendopnea. Venography allowed the timely diagnosis, and recanalisation of the SVC has been advocated as the first-line treatment of symptomatic SVC stenosis.^{14 15}

In conclusion, this case report highlights the importance of including SVC stenosis in the differential diagnosis of bendopnea, particularly in patients who have risk factors for SVC stenosis, do not have evidence of elevated ventricular filling pressures, and have accompanying symptoms such as head fullness and facial flushing.

Patient’s perspective.

This event to me… is life changing. When a patient walks to you and complains about extreme shortness of breath when bending over, you shouldn’t just ignore it and simply think it’s a heart problem. I was like a couch potato for decades. After they opened up my neck vein… I was back to ‘normal’, working out, lifting heavy things like I used to do.

Learning points.

• Bendopnea is defined as shortness of breath within 30 s of bending without holding one’s breath.

• Superior vena cava (SVC) stenosis should be considered in the differential diagnosis of bendopnea.

• When evaluating patients with bendopnea, clinicians should assess for risk factors for SVC stenosis (eg, history of an indwelling central venous catheter) and inquire about other symptoms associated with SVC stenosis (head fullness and facial flushing), particularly if there is no evidence on examination of elevated ventricular filling pressures (eg, no jugular venous distention).