Ventricular assist device implantation in patients with a failing systemic right ventricle: a call to expand current practice
Ventricular assist device (VAD) implantation is an established treatment modality for patients with end-stage heart failure, and improves symptoms and survival. In the Netherlands, it is not yet routinely considered in patients with congenital heart disease and failing systemic right ventricle (SRV). Recently, a VAD was implanted in 2 SRV patients, one who underwent a Mustard procedure during infancy for transposition of the great arteries (male, 47 years old) and one with a congenitally corrected transposition of the great arteries (male, 54 years old). The first patient is doing well >1 year after implantation; the second patient will be discharged home soon. These examples and other reports demonstrate the feasibility of adopting VAD implantation into routine care for SRV failure. In conclusion, patients with SRV failure may be suitable candidates for VAD implantation: they are relatively young, usually have a preserved subpulmonary left ventricular function, and their specific anatomical and physiological characteristics often make them unsuitable for cardiac transplantation. Therefore it is important to recognise the possibility of VAD implantation early in the process of SRV failure, and to timely refer these patients to a heart failure clinic with experience in VAD implantation in this group of patients for optimisation, screening, and implantation.
KeywordsCardiac assist devices Heart failure Heart defects Congenital
Current use of ventricular assist device therapy and gap for patients with failing systemic right ventricle
Left ventricular assist device (LVAD) implantation as destination therapy is an established treatment for patients with end-stage heart failure who are not eligible for cardiac transplantation. It improves both symptoms and prognosis . However, in the Netherlands, it has until recently not been used as a treatment option for congenital heart disease (CHD) patients with failing systemic right ventricle (SRV). This group includes patients late after Mustard or Senning procedure for transposition of the great arteries (TGA) or patients with congenitally corrected TGA (CCTGA). Current survival of Mustard/Senning patients is 82% at 40 years postoperatively . For CCTGA patients, freedom from death or cardiac transplantation was 84% at 40 years of follow-up . SRV failure is likely to be a major and substantial problem in the upcoming years [2, 3]; in our centre alone, 61 SRV patients are currently under follow-up. SRV patients have a complex anatomy, adhesions due to (sometimes multiple) prior sternotomies, and pulmonary hypertension, and are consequently likely to be rejected for cardiac transplantation due to current shortage of donor organs. The European Society of Cardiology (ESC) guideline for adult CHD does not yet contain an advice regarding VAD implantation but mentions long-term mechanical circulatory support as an important area of research . Recently, we implanted a VAD in 2 SRV patients. In this paper we aim to illustrate the feasibility of this procedure, to stress the clinical necessity to expand current indications for VAD therapy to this group, and especially to consider it as destination therapy.
Cases of VAD implantation in SRV: clinical and surgical considerations
The second patient is a 54-year-old man with CCTGA, who underwent tricuspid valve replacement with a bioprosthesis and mitral valve annuloplasty 2 years before VAD implantation, the latter of which was complicated by partial ring dehiscence. The defect was closed percutaneously with 2 vascular plugs. In 2017, he received an ICD for primary prevention because of a poor SRV function. Recently, his clinical condition deteriorated rapidly and he was admitted because of congestion. He was rejected for cardiac transplantation because of renal dysfunction and, consequently, screened and accepted for VAD implantation. Transthoracic echocardiography (TTE) confirmed the poor SRV function (GLS −2.0%, FAC 7.3%). Pre-operative admission was prolonged due to biliary pancreatitis, which was treated with laparoscopic cholecystectomy. At the time of surgery, the patient was in NYHA class IV, INTERMACS level 2. Because of a decline in subpulmonary left ventricular function, treatment with levosimendan (Orion Corporation) was initiated. The VAD was implanted through a left-sided anterolateral thoracotomy combined with upper hemisternotomy because of a relatively dorsolateral position of the SRV and favourable anatomy for this approach. Again, multiple trabeculations were resected in the SRV cavum before the VAD (HVAD, Medtronic, USA) was implanted. Early after surgery, VAD flow dropped due to a deviation of the inflow cannula towards the septum resulting in obstruction of the inflow cannula. The cannula was subsequently repositioned. Seven days after VAD implantation, the patient was transferred to the coronary care unit. The remaining post-operative period was uneventful and patient is about to be discharged home.
Peri-operative challenges in the first case included the lack of space between the SRV and the sternum, and the trabeculations in the SRV. The former resulted in mid-basal insertion of the VAD instead of the more apical position that is common for VAD implantation in the left ventricle (Figs. 1 and 2). The latter necessitated resection of multiple trabeculations to prevent obstruction of the inflow cannula. The need for resection of trabeculations was expected in both cases, as pre-operative imaging clearly showed a heavily trabeculated SRV in both patients. This approach has been described previously . In our second case, in addition to resection of trabeculations, a different surgical approach was used because of a relatively dorsolateral position of the SRV. In both patients, the challenging positioning of the inflow cannula could be partially explained by the presence of a tricuspid valve prosthesis, making TEE-guided localisation of optimal inflow cannula position less evident.
In general, SRV patients may have complex cardiac and thoracic anatomy, for example dextrocardia or situs inversus. As these cases demonstrate, anatomical variations in SRV patients require a patient-tailored surgical approach for VAD implantation (median (re)sternotomy versus lateral thoracotomy and upper hemisternotomy). An alternative device position should be considered when lack of space prevents apical implantation of the VAD, and inflow cannula orientation is of paramount importance for unobstructed VAD inflow. Pre-, intra- and post-operative imaging (for example with computed tomography angiography, epicardial/transoesophageal echocardiography, and transthoracic echocardiography, respectively) is crucial to plan and evaluate the operative approach .
Patients with SRV failure are potentially good VAD candidates
Medical eligibility criteria and contraindications for VAD implantation as destination therapy in patients with SRV, according to our dedicated team
Major criteria for VAD eligibility (all should apply)
VAD contraindicated if one/more of the following
– End-stage SRV failure (NYHA IIIb–IV, INTERMACS II–IV)
– INTERMACS I
a. Despite optimal medical therapy
– Severe non-cardiac comorbidity with life expectancy <1 year
b. Despite optimal treatment of tricuspid valve regurgitation
– Poor subpulmonary LV function
c. Despite CRT if indicated
– Non-reversible severe kidney dysfunction (eGFR <30 ml/min/1.73m2)
d. Despite effort to sustain sinus rhythm
– Active systemic infection
– Ineligible for cardiac transplantation
– Unacceptably high operative risk
In conclusion, VAD implantation as destination therapy should be considered in patients with severe SRV failure. Despite the risk of complications, VAD therapy is a reasonable option in patients with failing SRV but requires a dedicated and experienced team.
The design of Fig. 1, by Ron Slagter, was greatly appreciated.
This work was supported by general research grants received by the department of Cardiology of the Leiden University Medical Center, by Medtronic, Biotronik, Boston Scientific, and Edwards Lifesciences.
Conflict of interest
T.E. Zandstra, M.G. Hazekamp, B. Meyns, S.L.M.A. Beeres, E.R. Holman, P. Kiès, M.R.M. Jongbloed, H.W. Vliegen, A.D. Egorova and M.J. Schalij declare that they have no competing interests. M. Palmen is a proctor for Medtronic. Proctoring fees are paid to the department research fund. L.F. Tops has received a speaker fee from Medtronic.
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