Abstract
Background
Transcatheter mitral valve-in-valve (ViV) has emerged as a less invasive alternative to redo open surgical mitral valve replacement in patients at high surgical risk and is usually performed using a transseptal route by femoral venous access. Rarely, this approach may not be feasible owing to venous occlusions or congenital inferior vena cava (IVC) interruption.
Case Summary
A patient with agenesis of the femoral vein required a mitral ViV procedure, which was successfully done using transhepatic access.
Discussion
Agenesis of the femoral vein is a rare malformation. The transhepatic route can be an alternative for structural procedures in these patients, albeit with technical challenges and additional risks.
Take-Home Messages
Transhepatic access is a feasible alternative for transcatheter, transseptal procedures in patients with IVC interruption; however, special care must be taken to guarantee proper access for hemostasis. Hemorrhagic complications remain a concern in patients who undergo mitral ViV procedures owing to the need for anticoagulation therapy.
Key words: agenesis of femoral vein, inferior vena cava interruption, mitral valve-in-valve, transhepatic, transseptal
Visual Summary
History of Presentation
A 72-year-old woman with a cardiovascular history of rheumatic heart disease and 2 previous surgical mitral valve replacements (2001 and 2008) presented to our emergency department with NYHA functional class III dyspnea and fever.
Take-Home Messages
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Agenesis of the IVC is a rare malformation that is mostly incidentally discovered. With the increased number of transcatheter structural heart procedures being performed using transfemoral venous access, clinicians should be aware of venous anomalies.
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Transhepatic access is feasible for a variety of therapeutic cardiovascular procedures, but special care must be taken to guarantee prompt hemostasis using vascular plugs, coils, or absorbable gelatin sponge and protamine administration.
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Hemorrhagic complications remain a concern in patients who undergo mitral ViV procedures owing to the need for anticoagulation therapy.
Past Medical History
The patient was a former smoker and had the following comorbidities: hypertension, dyslipidemia, diabetes mellitus, acromegaly, and atrial flutter. Permanent pacemaker implantation was required in 2012. During the last open-heart operation for mitral replacement, a Biocor (St Jude) 31-mm bioprosthesis was implanted.
Investigations
Blood cultures were positive for Streptococcus infantarius, and a transesophageal echocardiogram (TEE) revealed preserved left ventricular function (ejection fraction 68%), mild right ventricular dysfunction, and severe bioprosthetic mitral regurgitation secondary to leaflet tear. The left atrium (LA) was enlarged (80 mL/m2), and no vegetations were detected (Video 1).
Management
A presumptive diagnosis of endocarditis was made, and antibiotics were initiated. After discussion with the heart team, the patient was deemed not eligible for surgery because of 2 previous surgical mitral valve replacements and a very high surgical risk based on the Society of Thoracic Surgeons score (16.75%) and EuroSCORE II score (17.95%). Following extended antibiotic therapy (12 weeks), she was referred for a mitral valve-in-valve (ViV) procedure. Computed tomography (CT) scan confirmed the bioprosthetic valve size with a predicted neo-left ventricular outflow tract (LVOT) area of 262 mm2, ensuring a low risk for LVOT obstruction (Figure 1).
Figure 1.
Computed Tomography Scan Predicted Neo-Left Ventricular Outflow Track of 262 mm2
3mensio (3mensio Medical Imaging) imaging tool was used for image analysis.
The first procedure was attempted through the right femoral vein, but we were unable to reach the right atrium with the transseptal sheath. Angiography revealed a prominent hemiazygos vein with drainage into the superior vena cava and right atrium (Video 2). The procedure was terminated and rescheduled after further discussions about planned strategies.
CT images were reviewed. The absence of the inferior vena cava (IVC) was confirmed, and a prominent hepatic vein (Figure 2) was noted. It was decided to perform the procedure through a percutaneous transhepatic access.
Figure 2.
Computed Tomography Scan Demonstrating the Azygos Vein and a Prominent Hepatic Vein
The second procedure attempt was performed under general anesthesia with TEE and fluoroscopic guidance. Ultrasound-guided access of the right internal jugular was obtained with placement of a 6-F vascular sheath. A multipurpose catheter was oriented to the hepatic vein; venous angiography was performed to guide entrance of an 18G Chiba needle, placed below the right costal margin between the midclavicular and the anterior axillary line (Figure 3). The trajectory of the needle through the liver parenchyma into the hepatic vein was assessed by subcostal ultrasound (Video 3). Intermittent needle aspiration and contrast injection confirmed the correct position in the vein; a 0.014-inch coronary guidewire (Balance Heavyweight; Abbott Vascular) was then positioned in the right atrium, and the Chiba needle was exchanged for a long 7-F vascular sheath.
Figure 3.
Illustration of the Ideal Puncture Site for the Transhepatic Access
Ideal puncture site for the transhepatic access at the second last intercostal space below the right costal margin, between the midclavicular and right anterior axillary line, aimed to the left acromion with an angulation of 20 to 30°Image adapted from ZygoteBody Professional.8
Transseptal puncture using traditional devices, a braided transseptal sheath (SL-1; Abbott Vascular) and a BRK needle (Abbott Vascular), was not feasible owing to lack of adequate septal contact. Using a steerable sheath (Agilis NXT; Abbott Vascular) with an Amplatz Right II 6-F catheter (Medtronic) inside, interatrial septum tenting was possible at a good spot (inferior and posterior), and a 0.014-inch coronary guidewire (Balance Heavyweight) was used to cross the fossa ovalis using diathermy (electrocautery) at 30 W. Heparin at a dose of 100 IU/kg was administered to maintain an activated coagulation time longer than 250 seconds. Next, septostomy was sequentially performed with a 4.0 × 20 mm coronary balloon (Abbott Vascular) and a 14 × 40 mm noncompliant vascular balloon (Atlas Gold; BARD) (Video 4).
With coaxiality provided by the steerable sheath, the surgical valve was crossed with a 6-F pigtail catheter, followed by careful positioning of an extra-stiff guidewire (Confida; Medtronic) into the left ventricular apex. Afterward, the steerable sheath was exchanged for a large-bore, 21-F introducer (Certitude transapical system; Edwards Lifesciences) (Video 5). A Sapien 3 Ultra 29-mm (Edwards Lifesciences) transcatheter heart valve (THV) was successfully implanted under rapid (150 beats/min) wire pacing (Figure 4, Video 6).
Figure 4.
Sapien 3 Ultra 29-mm Transcatheter Heart Valve (Edwards Lifesciences) Successfully Implanted
After valve implantation, TEE revealed a mean transprosthetic gradient of 2.5 mm Hg and absence of paravalvular leaks. The large-bore sheath was retracted until its distal tip was situated outside the hepatic vein but within the hepatic tissue tract. Then, an 18 × 14 mm Amplatzer vascular plug II (Abbott Vascular) was inserted and released into the parenchymal tract, between the hepatic vein and hepatic capsule, aiming for hemostasis (Video 7). Thrombus formation in the LA was detected uneventfully by TEE; at this point activated coagulation time was 280 s, so a shared decision was made to not administer protamine, which is usually required to further enhance hemostasis at the transhepatic access. Soon thereafter, the patient developed hemodynamic instability (heart rate 110 beats/min, blood pressure 70/50 mm Hg) and desaturation (90%). Chest ultrasonography revealed a right hemothorax, and a closed thoracocentesis was promptly performed with removal of 1,400 mL of blood. Transfusion of 2 U of red blood cells was needed, and manual compression was maintained at the entry site for 30 minutes. The patient’s hemodynamics and saturation level improved. Final angiography exhibited absence of extraluminal contrast extravasation from the hepatic vein (Video 7).
Follow-Up
The patient was transferred to the intensive care unit and extubated in the following day. Enoxaparin 1 mg/kg twice daily was started. Five days later, TEE revealed leaflet thrombosis, and acetylsalicylic acid 100 mg/d was added. The patient improved clinically. However, 2 weeks after the procedure, she experienced an acute hemorrhagic stroke (Figure 5) and died a few days later.
Figure 5.
Head Computed Tomography Scan of the Patient Showing Hemorrhagic Stroke
Discussion
Agenesis of the femoral vein is a rare malformation described in 0.005% to 1% of individuals;1 it is the most common variant of IVC interruption. In this anomaly, the segment of the IVC between the hepatic and renal veins is absent. Accordingly, blood from the lower extremities drains into the right atrium through the azygous system and collaterals; moreover, hepatic veins drain directly into this chamber. Although some patients may present with deep venous thrombosis,2 this anomaly is mostly asymptomatic and can be incidentally discovered during transfemoral venous access obtained for diagnostic and therapeutic cardiovascular procedures. Owing to the growth of transcatheter structural heart treatments that require large-bore femoral vein access (such as tricuspid and mitral valve interventions), clinicians should be aware of IVC interruption. In such situations, alternative routes using the superior vena cava or a surgical transatrial approach might be considered, albeit with technical challenges and additional risks. The transhepatic approach is a feasible alternative for transseptal access and has been previous described as a route for congenital,3 electrophysiological,4 and structural heart procedures, such as left atrial appendage closure,5 mitral balloon valvuloplasty,6 and transcatheter mitral edge-to-edge repair.7 To our knowledge, this is the first report of a transhepatic, transseptal mitral ViV procedure.
This case taught us some lessons. Before the procedure, electrocardiogram-gated CT angiography was performed to confirm surgical bioprosthetic dimensions and to assess the appearance of the interatrial septum and the risk of LVOT obstruction. However, particular attention should be also given to vascular access (as it is routinely performed for TAVI); the identification of IVC interruption or other abnormalities can anticipate difficulties and prevent complications. Subcostal ultrasound guidance was useful for orienting the trajectory of the needle for accessing the hepatic vein, minimizing the number of puncture attempts. As the distance from the hepatic vein to the LA is shorter than the distance from the femoral vein to the LA, the selection of the Edwards Sapien transapical system (with a reduced sheath and delivery catheter compared with the transfemoral system) precludes the maneuver of aligning the balloon and the THV inside the body; the tip of the transapical sheath was positioned in the LA, which also facilitated THV positioning. Special care should be taken to promote secure hemostasis when performing transhepatic access. In this case, the sudden resolution of severe mitral regurgitation in a patient with a large LA and atrial flutter probably favored thrombus formation, so a decision was made to not administer protamine; a delayed hemostasis resulted in a right hemothorax. As the final angiogram showed no extravasation of contrast material and the patient's clinical condition improved, only 1 vascular plug was implanted. Notwithstanding, another vascular plug and the application of coils or absorbable gelatin sponge are also described techniques that could lead to prompt hemostasis after a transhepatic route.
Conclusions
This case report highlights the feasibility of a percutaneous, transhepatic, transseptal mitral ViV procedure in a patient with an interrupted IVC. With the increased number of transcatheter structural heart procedures, we must be aware of the possibility of venous access abnormalities and the need for alternative access.
Visual Summary.
Images Illustrating the First and Second Procedures
(A-C) First procedure. (A) Transesophageal echocardiogram revealed a bioprosthetic mitral valve with leaflet tear and severe mitral regurgitation. (B) Angiogram showed agenesis of the inferior vena cava with a prominent azygos system. (C) Review of computed tomography images revealed a prominent hepatic vein and azygos system. (D-G) Second procedure. (D) Puncture of hepatic vein with 18G Chiba needle was performed under ultrasound and angiographic guidance. (E) After transseptal puncture, septostomy with a coronary balloon was required. (F) Sapien 3 Ultra 29-mm (Edwards Lifesciences) transcatheter heart valve was successfully implanted. (G) Amplatzer Vascular Plug II (Abbott Vascular) was released into the parenchymal tract, between the hepatic vein and hepatic capsule, aiming for hemostasis.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
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 Author Center.
Appendix
Equipment List.
Imaging
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Access
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Transseptal Puncture
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Mitral Valve-in-Valve Implant
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Appendix
TEE Revealing a Bioprosthetic Mitral Regurgitation Caused by a Leaflet Tear
No vegetations were detected.
Angiography Demonstrating a Prominent Hemiazygos Vein With Circulatory Drainage Into the Superior Vena Cava and Right Atrium
These anomalies are encountered in patients with agenesis of the inferior vena cava.
Transhepatic Puncture With a 18G Chiba Needle Guided by Ultrasonography and Angiography
Initial Septostomy Using a Steerable Sheath, 0.014-Inch Coronary Guidewire, and 4.0 × 20 mm Noncompliant Balloon
Insertion of a 21-F Sheath Over an Extra-Stiff Guidewire Positioned at the Left Ventricular Apex
A 29-mm Transcatheter Heart Valve Implanted Under Rapid Left Ventricular Wire Pacing
An 18 × 14 mm Vascular Plug Released Into the Hepatic Vein / Hepatic Tract for Hemostasis With No Extravasation of Contrast Dye
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
TEE Revealing a Bioprosthetic Mitral Regurgitation Caused by a Leaflet Tear
No vegetations were detected.
Angiography Demonstrating a Prominent Hemiazygos Vein With Circulatory Drainage Into the Superior Vena Cava and Right Atrium
These anomalies are encountered in patients with agenesis of the inferior vena cava.
Transhepatic Puncture With a 18G Chiba Needle Guided by Ultrasonography and Angiography
Initial Septostomy Using a Steerable Sheath, 0.014-Inch Coronary Guidewire, and 4.0 × 20 mm Noncompliant Balloon
Insertion of a 21-F Sheath Over an Extra-Stiff Guidewire Positioned at the Left Ventricular Apex
A 29-mm Transcatheter Heart Valve Implanted Under Rapid Left Ventricular Wire Pacing
An 18 × 14 mm Vascular Plug Released Into the Hepatic Vein / Hepatic Tract for Hemostasis With No Extravasation of Contrast Dye