Abstract
Inferior vena cava (IVC) syndrome is commonly caused by deep vein thrombosis or a mass effect. We present an unusual case of IVC kinking and obstruction detected by echocardiography and confirmed by cardiac magnetic resonance and angiography. Management of marked tortuosity of the IVC secondary to a paralyzed hemidiaphragm is discussed. (Level of Difficulty: Beginner.)
Key Words: angiogram, echocardiogram, inferior vena cava, magnetic resonance imaging
Abbreviations and Acronyms: IVC, inferior vena cava
Graphical abstract
Inferior vena cava (IVC) syndrome is commonly caused by deep vein thrombosis or a mass effect. We present an unusual case of IVC kinking and…
A 74-year-old woman presented to the hospital with reports of worsening dyspnea and lower extremity swelling. She had chronic swelling in her lower extremities, but over the past few weeks the swelling significantly worsened. Physical examination revealed bilateral 3+ lower extremity pitting edema, abdominal distention, and decreased breath sounds on the right lower lung field. Vital signs included blood pressure of 125/63 mm Hg, heart rate of 87 beats/min, and blood oxygen saturation at 83% on room air.
At 30 years of age, she had a sky diving accident, secondary to failure of parachute deployment, which resulted in an L1 vertebral fracture that caused paraplegia. Her medical history also included chronic lymphedema, paroxysmal atrial fibrillation, right hemidiaphragm elevation, hypertrophic cardiomyopathy, and pulmonary hypertension.
An electrocardiogram demonstrated normal sinus rhythm. Her troponin level was not elevated, and the N-terminal pro–B-type natriuretic peptide level was slightly elevated at 298 pg/ml (reference range: <126 pg/ml). Chest radiography and chest computed tomography showed a markedly elevated, paralyzed, right hemidiaphragm (Figure 1A, Supplemental Figure 1). Echocardiography revealed a stenotic inferior vena cava (IVC) with an elevated velocity (2.0 m/s) at the cavoatrial junction (Figures 1B and 1C). The patient’s pulmonary artery systolic pressure was 50 mm Hg. Further imaging was pursued, with cardiac magnetic resonance confirming hemidiaphragm elevation causing significant tortuosity of the IVC at the cavoatrial junction, which measured approximately 8 mm (Figure 1D). Several venous collateral vessels, related to the IVC stenosis and that drained into the subclavian veins, were seen in the anterior abdominal wall and chest wall (Figure 1E). No evidence of thrombus formation or mass burden was seen. We postulated that the patient’s markedly elevated hemidiaphragm had, over time, resulted in the elongation, kinking, and severe stenosis of the IVC that led to her current presentation. Selective angiography showed a tortuous IVC that possibly could be amenable to percutaneous stenting (Figure 1F, Video 1). Right-sided heart catheterization demonstrated a mean gradient of 10 mm Hg between the IVC and the right atrium (mean right atrial pressure, 12 mm Hg; mean IVC pressure, 22 mm Hg).
Figure 1.
Kinked IVC
(A) Chest radiograph shows an elevated right diaphragm (arrows) (Supplemental Figure 1). (B) Subcostal echocardiography reveals a dilated inferior vena cava (IVC) with severe narrowing at the cavoatrial junction (arrow) accompanied by turbulent flow on color Doppler imaging. (C) Continuous-wave Doppler imaging directed at the turbulent flow at the cavoatrial junction reveals high velocity (2.0 m/s) indicative of severe obstruction. (D) Cardiac magnetic resonance demonstrates hemidiaphragm elevation causing significant tortuosity of the IVC at the cavoatrial junction, which measured approximately 8 mm (arrow). (E) Magnetic resonance imaging demonstrates several venous collateral vessels in the anterior abdominal and chest wall. (F) Selective angiography demonstrates marked dilation, elongation, and kinking of the IVC (arrow) (Video 1). RA = right atrium.
Online Video 1.
IVC Angiography
Selective angiogram demonstrates marked dilation, elongation, and kinking of the inferior vena cava resulting in severe stenosis at the junction of the inferior vena cava (IVC) and the right atrium.
Medical therapy was initiated with torsemide and spironolactone. The patient spent a total of 2 weeks in the hospital, with diuresis of 22.3 l, before discharge. Her symptoms improved, and she was discharged on the foregoing regimen.
This report, to our knowledge, is the first to describe marked kinking or tortuosity of the IVC at the cavoatrial junction secondary to elevation of the right hemidiaphragm, as demonstrated with multimodality imaging.
Acknowledgments
The authors are grateful to Rahul Sawlani, MD, of the Aurora St. Luke’s Medical Center Radiology Department for his assistance with the MRI images (Figure 1D and 1E); Jennifer Pfaff and Susan Nord of Aurora Cardiovascular and Thoracic Services for editorial preparation of the manuscript; and Brian Miller and Brian Schurrer of Advocate Aurora Research for assistance with the figures.
Footnotes
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Case Reportsauthor instructions page.
For a supplemental figure and video, please see the online version of this paper.
Appendix
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.