Mustard Procedure Research Articles

Anticoagulation management in adult patients with congenital heart disease: a narrative review

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Stiff “Left Atrial” syndrome post-mustard procedure

Objectives: We describe the clinical course and management of two patients with post-capillary pulmonary hypertension due to diffuse pulmonary venous baffle calcification decades post-Mustard procedure. Background: From the late 1950s to the early 1990s, the definitive surgical repair for children with D-transposition of the great vessels (D-TGA) was an atrial switch procedure (either Senning or Mustard operation) which utilizes atrial-level baffles to shunt pulmonary venous blood to the morphologic right (systemic) ventricle and caval blood to the morphologic left (sub-pulmonary) ventricle. From a hemodynamic standpoint, baffle leaks and stenoses as well as precapillary pulmonary hypertension have all been described as both early and late complications [1]. Recently, delayed post-capillary pulmonary hypertension (in the absence of discrete baffle obstruction) decades post-atrial switch has also been described [2]. The underlying pathophysiology for this postcapillary pulmonary hypertension is unclear but is theorized to involve impaired diastology referable to the pulmonary venous baffle. Methods/Results: Using hemodynamic and imaging data, we describe two patients with extensive pulmonary venous baffle calcification and resultant pulmonary hypertension from the so-called “stiff left atrial (LA) syndrome.” This problem can be difficult to treat medically and is not amenable to catheter-based interventions. We hypothesize that this is an underlying mechanism for pulmonary hypertension in at least some post-Mustard and Senning patients. Conclusion: We describe the treatments and clinical course for each of these patients, and in particular describe how the surgical revision of the pulmonary venous baffle in one case led to the complete resolution of symptoms.

Advanced imaging improves detection of baffle leaks and stenoses after atrial switch compared with transthoracic echocardiography.

Current guidelines for adults with atrial switch repair recommend baseline cardiovascular magnetic resonance (CMR) for assessment of ventricular size and function, systemic and venous baffle obstruction and leaks, and valvular function. It also recommends transthoracic echocardiography (TTE) for outpatient follow up. Many such patients with implanted cardiac devices may need cardiac computed tomography (CCT) when CMR is not feasible. This study reviews and compares CMR, transesophageal echocardiography (TEE), CCT, cardiac catheterization with angiography and TTE in detection of baffle problems in patients after atrial switch operation. The medical records of patients who had at least one imaging study performed after atrial switch operation at our center from 2010 to 2020 were retrospectively reviewed. Results are reported as descriptive statistics for demographics and imaging findings. The principal outcome measure was detection of baffle leak and/or baffle stenosis. Fifty-seven patients had at least one cardiac imaging study after atrial switch operation (36 Senning and 21 Mustard operations) during the study period. Nearly 33% (19/57) had baffle complications of stenosis and/or baffle leaks identified. All 57 patients had TTE performed but baffle problems were noted by TTE in only 8 (14%) patients (7 baffle stenosis and 1 baffle leak). Of the 49 patients without known baffle problems by TTE, 24 had advanced imaging (TEE/CCT/CMR/angiography). Advanced imaging identified baffle problems in nearly half (11/24, 46%) of them (7 baffle leaks and 4 baffle stenosis). Baffle problems were present in (8/23) patients with transvenous cardiac devices. Baffle complications are common after atrial switch operations and in our study occur in 1/3rd of the patients. However, TTE is not sensitive enough to recognize these complications. Advanced imaging for detection of baffle complications should be considered in all patients after atrial switch operation.

Toward Meeting the Challenges of Improving Cardiovascular Health in Egypt.

Toward Meeting the Challenges of Improving Cardiovascular Health in Egypt.

HVAD Decommission in a Failing Mustard: Making Virtue out of Necessity

Introduction Ventricular assist device (VAD) for systemic right ventricular failure (SRVF) in transposition of the great arteries (TGA) patients has proven useful to reduce transpulmonary gradient (TPG) and bridge-to-transplantation. Tricuspid valve replacement (TVR) at the time of implant to address severe tricuspid regurgitation (TR) may be beneficial to achieve optimal unloading of the systemic right ventricle (SRV). We report successful decommissioning of a SRV VAD following device thrombosis in a patient who underwent TVR at VAD implant. Case Report A 37-year-old man who underwent a Mustard procedure for TGA at 10 months of age presented with acute heart failure, triggered by leg cellulitis and pulmonary embolism. He was found to have a severely impaired SRV, severe TR and a dilated and moderately impaired subpulmonic left ventricle (LV). Despite medical management and anticoagulation, he worsened requiring admission to the ICU for inotropic support. A right heart catheter (RHC) showed a mean pulmonary artery (PA) pressure of 48 mmHg and TPG of 15 mmHg, therefore ineligible for heart transplant. He underwent successful HeartWare-VAD implantation into his failing SRV with concomitant systemic TVR. His post-operative course was uneventful and he was discharged after 25 days. After six months, his TPG came down to 4 mmHg and he was listed for heart transplantation. He remained stable and free from VAD complications until he presented with thrombosis of the outflow graft after 27 months. Anticoagulation with bivalirudin was started without success and persistent reduced VAD flow of Summary VAD implantation is a feasible strategy for those with SRVF. Concomitant TVR to address severe TR may have additional benefit and in this particular case, likely to have contributed to successful VAD decommissioning following device thrombosis.

Clinical Course Long After Atrial Switch: A Novel Risk Score for Major Clinical Events.

BackgroundPatients with transposition of the great arteries corrected by an atrial switch operation experience major clinical events during adulthood, mainly heart failure (HF) and arrhythmias, but data on the emerging risks remain scarce. We assessed the risk for events during the clinical course in adulthood, and provided a novel risk score for event‐free survival.Methods and ResultsThis multicenter study observed 167 patients with transposition of the great arteries corrected by an atrial switch operation (61% Mustard procedure; age, 28 [interquartile range, 24–36] years) for 13 (interquartile range, 9–16) years, during which 16 (10%) patients died, 33 (20%) had HF events, defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF‐related death, and 15 (9%) had symptomatic ventricular arrhythmias. Five‐year risk of mortality, first HF event, and first ventricular arrhythmia increased from 1% each at age 25 years, to 6% (95% CI, 4%–9%), 23% (95% CI, 17%–28%), and 5% (95% CI, 2%–8%), respectively, at age 50 years. Predictors for event‐free survival were examined to construct a prediction model using bootstrapping techniques. A prediction model combining age >30 years, prior ventricular arrhythmia, age >1 year at repair, moderate or greater right ventricular dysfunction, severe tricuspid regurgitation, and mild or greater left ventricular dysfunction discriminated well between patients at low (<5%), intermediate (5%–20%), and high (>20%) 5‐year risk (optimism‐corrected C‐statistic, 0.86 [95% CI, 0.82–0.90]). Observed 5‐ and 10‐year event‐free survival rates in low‐risk patients were 100% and 97%, respectively, compared with only 31% and 8%, respectively, in high‐risk patients.ConclusionsThe clinical course of patients undergoing atrial switch increasingly consists of major clinical events, especially HF. A novel risk score stratifying patients as low, intermediate, and high risk for event‐free survival provides information on absolute individual risks, which may support decisions for pharmacological and interventional management.

A novel procedure of puncture to access the pulmonary venous atrium in complex congenital heart disease with surgical correction

BackgroundIn complex congenital heart disease, supraventricular arrhythmia develops long term after surgical repair. The arrhythmia could have pharmacological tolerance and sometimes be fatal. We report our experiences with puncturing tough or calcified surgical repair material to access the pulmonary venous atrium for the purpose of the management of arrhythmia in complex congenital heart disease with surgical correction. MethodsFrom June 2016, subsequent 9 patients underwent the procedure. Their age at the procedure ranged from 11 to 43 years old (median 26.4 years old). Surgical procedures were Mustard procedure (XenomedicaⓇ) in 6, lateral tunnel total cavo-pulmonary connection with autologous pericardium in 2, and extra-cardiac total cavo-pulmonary connection with expanded polytetrafluoroethylene (ePTFE) tube in 1. ResultsMulti-planar reconstruction imaging by cardiac computed tomography was done for making plans before the procedures. Under intracardiac echocardiography guidance, an 8 French steerable sheath was perpendicularly adjusted to target objects. The sharpened edge of 0.014 inch guide wire penetrated with gentle forward force. Subsequently, the puncture site was dilated from a small balloon to a large one step by step. In all patients, penetration was successfully completed. Catheter ablation was achieved in 8 patients and a pacemaker was implanted through the re-canalized superior caval vein in 1 patient. No complications were recorded. ConclusionThe trans-catheter puncture of the synthetic or calcified material is safe and feasible, although careful planning is required with imaging. A steerable sheath could easily be controlled to the make appropriate angle to the target object for preventing slippage and conveying effective force.

Mechanical Circulatory Support for the Failing Sub-Aortic Right Ventricle in Adults.

Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.

Biomarker sST2 in Adults with Transposition of the Great Arteries Palliated by Mustard Procedure

Background: Transposition of the great arteries (TGA) is a congenital heart defect (CHD) in which the aorta and pulmonary artery are transposed; it requires urgent surgical intervention. The Mustard procedure was an early surgery allowing survival into adulthood. However, this procedure is associated with long-term adverse effects including arrhythmias and heart failure (HF). A key factor impairing HF management in adults with CHD is lack of biomarkers to predict outcome. Soluble ST2 (sST2) is a protein secreted by myocytes in response to mechanical strain and fibrosis. No studies have focused specifically on sST2 in TGA individuals that underwent a Mustard procedure. In this study, we hypothesized adults with TGA palliated with a Mustard procedure would have higher levels of sST2 than healthy individuals, and this would correlate with functional class status. &#x0D; &#x0D; &#x0D; Methods: We screened the Pediatric Cardiology database for D-TGA subjects and Mustard operation. Healthy controls were recruited from clinic and the community. Patients were matched by age to a control group of 21 healthy individuals. Severity of symptoms was assessed by NYHA functional classification. sST2 levels were obtained using Critical Diagnostics Presage ST2 Assay kit. Additionally, cTnI, BNP, lipid panel, insulin, glucose, and EKG and echocardiography (TGA) were obtained at IUSM clinical laboratories. &#x0D; &#x0D; Results: We identified 45 patients with D-TGA and Mustard operation. 19 patients were included in analysis: 12 male and 7 female subjects aged 18 to 46, mean of 34.9 years. In the Mustard group, 9 subjects were assigned to NYHA class II, and 9 to class III. The control group was asymptomatic. sST2 levels in the Mustard group were elevated in 53%, while only 29% in the controls. Of the Mustard subjects with elevated sST2, 67% had elevated cTnI, 80% arrhythmias, 70% EKG T-wave abnormalities, and 80% took cardiac medications. 90% of these patients had low HDL, 40% high LDL, and 40% low insulin. &#x0D; &#x0D; Conclusion: This study demonstrates patients with Mustard operation averaged higher sST2 levels than healthy subjects. Importantly, of the patients with elevated levels, there was a significant association of sST2 with biomarker abnormalities and clinical heart failure signs. This suggests in patients with D-TGA palliated with Mustard procedure, sST2 may add predictive value to cardiac related morbidity and mortality.

Mechanical circulatory support as a bridge to candidacy in adults with transposition of the great arteries and a systemic right ventricle.

Clinical experience with continuous flow ventricular assist devices (VADs) in patients with transposition of the great arteries (TGA) including dextro-TGA and congenitally corrected TGA is rare, and indications as well as potential benefits or specific hurdles remain unclear. Therefore, our goal was to report on our experience regarding VAD therapy in adult patients with TGA as a bridge to candidacy. We performed a single-centre retrospective study of all adult patients with TGA with systemic right ventricular failure who had continuous flow VAD implants between 2010 and 2018. Study end points were all causes of death, major cardiac and cerebrovascular adverse events or pump thrombosis. Follow-up continued until the time of the heart transplant. A total of 6 patients (4 men) had a continuous flow VAD implanted in the context of a failing systemic right ventricle (dextro-TGA after the Mustard procedure: n = 3; congenitally corrected TGA: n = 3). Demographics: mean age 32 ± 5.7 years; median Interagency Registry for Mechanically Assisted Circulatory Support: level II (range 1-4), mean pulmonary artery 48 ± 13 mmHg, mean pulmonary vascular resistance 5.6 ± 3.5 Wood units. Postoperative data: intensive care unit stay: 16 ± 9.7 days; in-hospital survival: 100%; no early VAD-related complications occurred. Mean follow-up: 33 ± 18 months; persistent left-side paresis: n = 1; minor (non-disabling) stroke: n = 2. Post-VAD pulmonary artery: 19 ± 3.4 mmHg; P < 0.005; post-VAD pulmonary resistance: 2.2 ± 0.55 Wood units; P = 0.066. Four patients had heart transplants after a mean waiting time of 30 months after the VAD was implanted; 2 patients are still on the waiting list (waiting time: 52 and 24 months). Continuous flow VAD therapy is a feasible therapeutic option in adult patients with TGA and a failing systemic right ventricle as a bridge to candidacy and a bridge to a heart transplant.

Abstract 16879: Utility of Transthoracic Echocardiography in Adult Patients With D-Transposition of the Great Arteries and Atrial Switch Operation

Introduction: Per 2018 ACC AHA adult congenital heart disease guidelines, routine transthoracic echocardiography (TTE) is recommended for follow-up in patients with d-transposition of the great arteries (dTGA) after atrial switch operation at least annually. Little data are available to suggest utility in clinical management with frequent surveillance, either with or without symptoms. Hypothesis: We hypothesized that frequent TTEs would not lead to significant clinical changes in management and that TTEs performed for symptoms would lead to more detection of pathology and clinically relevant management decisions. Methods: We conducted a retrospective review of all echocardiograms performed for dTGA patients corrected with the atrial switch procedure at Texas Children’s Hospital from 2000-2009 to obtain baseline TTEs and 2010-2019 to analyze for changes in clinical management, associated with TTE performance. Results: We identified 50 patients with dTGA and atrial switch operation with 54% Mustard operation. A total of 333 echocardiograms were performed (6.7/person), 30% (100) with symptoms. The average age at time of all TTEs was 35 +/- 11 years. Most frequent symptoms were palpitations (n=39), dyspnea (n=30), and fatigue (n=27). Changes in management occurred after 9% of studies (10/100 of symptomatic, 21/233 asymptomatic patients, p = NS). Most of these were medication changes (n=18, 11 asymptomatic) and advanced imaging (n=12, 9 asymptomatic). Invasive management after TTE was rare (1 baffle stenting in asymptomatic patient) but after advanced imaging included 4 baffle stent implantations (3 asymptomatic). The interval between TTEs was 1.2 +/- 0.9 years, regardless of symptoms. Conclusions: In dTGA patients after atrial switch operation, routine screening TTEs and symptomatic TTEs had similar yield in detecting pathology leading to a clinical change in management. TTE and subsequent advanced imaging preceded invasive intervention (baffle stenting) in 10% of patients, mostly asymptomatic. Less frequent surveillance TTEs in asymptomatic and advanced imaging in symptomatic dTGA patients with atrial switch operation is cost-effective and should be considered .

Implantable cardioverter defibrillator insertion in a patient with Mustard procedure resulting in phrenic nerve stimulation.

Implantable cardioverter defibrillator insertion in a patient with Mustard procedure resulting in phrenic nerve stimulation.

Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures

ObjectiveTo assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch...

Extracorporeal Cardiopulmonary Resuscitation During Cesarean Delivery in a Patient With D-Transposition of the Great Vessels, Years Following Mustard Operation

Patients with surgically repaired complex congenital cardiac anomalies present unique characteristics that can make the implementation of extracorporeal membrane oxygenation (ECMO) support especially challenging. Very few series have reported the outcomes of ECMO support during pregnancy and peripartum. We report a case of successful extracorporeal cardiopulmonary resuscitation during cesarean delivery in a patient with surgically repaired d-transposition of the great arteries, and we discuss particular aspects that contributed to successful implementation of ECMO support and hospital discharge.

Systolic dysfunction of the subpulmonary left ventricle is associated with the severity of heart failure in patients with a systemic right ventricle

BackgroundThe study aimed to assess the relation between echocardiographic parameters of subpulmonary left ventricular (LV) size and function, and the severity of heart failure in patients with a systemic right ventricle (SRV). Methods and resultsA total of 157 patients (89 post Mustard/Senning operations, 68 with congenitally corrected transposition of great arteries [ccTGA]) were included. The size and function of the SRV and subpulmonary LV were assessed on the most recent echocardiographic exam. Clinical data were collected from the electronic records.The majority (133, 84.7%) were in NYHA functional class 1–2. Median BNP concentration was 79.5[38.3–173.3] ng/l, and 100 (63.7%) patients were receiving heart failure therapy. Both LV and SRV fractional area change (FAC) differed significantly between patients with NYHA class 1–2 vs 3–4 (48[41.5–52.8]% vs 34[28.6–38.6]%, p < 0.0001 and 29.5[23–35]% vs 22[20–27]%, p < 0.0001, respectively), but LV FAC had a higher discriminative power for functional class >2 than SRV FAC (AUC 0.90, p < 0.0001 vs 0.79; p < 0.0001, respectively). A LV FAC cut-off value <39.2% had the highest accuracy in identifying patients with NYHA class 3–4 (sensitivity 83% and specificity 88%). In multivariable logistic regression analysis, LV FAC and SRV FAC independently associated to NYHA class 3–4 (OR 0.80 [95%CI 0.72–0.88], p < 0.0001 and OR 0.85 [95%CI 0.76–0.96], p = 0.007, respectively). ConclusionsSubpulmonary LV systolic dysfunction is associated with NYHA functional class 3–4 in patients with ccTGA or after Mustard or Senning operation. Careful evaluation of the subpulmonary LV should be a part of the routine assessment of patients with a SRV.

Differential myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle after atrial switch operation for complete transposition of the great arteries.

This study aimed to assess diffuse myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle in patients after Senning or Mustard operation for complete transposition of the great artery (TGA) using cardiac magnetic resonance (CMR) T1 mapping. Thirty-one adult TGA patients after Senning (n=24) or Mustard (n=7) operation were studied at the age of 33.3±4.0years. Systemic right ventricular (RV) and subpulmonary left ventricular (LV) volumes, ejection fraction, and myocardial T1 values and extracellular volume fraction (ECV) were determined using CMR. The RV and LV ejection fractions were 47.0±10.9% and 61.3±7.4%, respectively. Compared to published normative values, patients had significantly greater RV and LV native T1 and ECV values (all p<0.001). For each of the basal, mid, and apical segments, the LV native T1 and ECV values were significantly greater in the left than the right ventricle (all p<0.05). There is a significant trend on progressive increase in ECV value from the basal towards the apical segments in both the right (p=0.002) and the left (p<0.001) ventricle. Modestly strong correlations were found between RV and LV native T1 (r=0.60, p<0.001) and ECV (r=0.49, p=0.005) values but not with ejection fractions of the respective ventricles. Differential myocardial fibrosis, with greater involvement of the subpulmonary left ventricle than the systemic right ventricle, is present in patients with TGA after atrial switch operation. Associations between the magnitude of RV and LV fibrosis suggests adverse ventricular-ventricular interaction at the cardiac extracellular matrix level.

Cardiac resynchronization therapy for the failing systemic right ventricle: A systematic review

Patients with a systemic right ventricle (SRV) are at high risk for development of heart failure early in life. An SRV is encountered in patients with congenitally corrected transposition of the great arteries (CCTGA) or dextro-transposition of the great arteries (DTGA) with previous atrial switch repair (Mustard or Senning procedure). Progressive heart failure is one of the leading cause of mortality in these patients. Therefore, cardiac resynchronization therapy (CRT) has gained increasing momentum for use in this challenging congenital heart disease (CHD) population. However, current guidelines differ in recommendations for CRT in patients with an SRV as evidence supporting CRT has thus far only been described in case reports and retrospectively in relatively small study populations. In fact, the European Society of Cardiology Guideline for the management of grown-up congenital heart disease consider CRT to be ‘experimental’ in this population. This systematic review critically summarizes current literature on CRT in SRV patients and provides future perspectives for further research in this challenging and growing CHD population.

Impella Placement in a Patient With d-Transposition of the Great Arteries After Mustard Procedure

Abstract Although durable ventricular assist devices (VAD) have previously been utilized for long-term ventricular support of the systemic ventricle in patients with a history of dextro-Transposition of the great arteries (d-TGA) after the Mustard procedure,1,2 the use of a temporary VAD has not been well-described as either bridge-to-decision or bridge-to-transplantation in these patients. Our case demonstrates the successful positioning and placement of an Impella® 5.0 device for refractory inotrope-dependent cardiogenic shock in this patient population, with simultaneous transesophageal echocardiographic (TEE) and fluoroscopic guidance, in a patient with significant barriers to heart transplantation. Unique considerations for placement of this device into the systemic ventricle post-Mustard include the crescent-shaped systemic ventricular geometry, proximity of the tricuspid valvular apparatus to device inlet placement, difficult visualization of the aortic valve due to its anterior position (analogous to the pulmonic valve in a non-congenital patient), and acoustic shadowing from the Impella® device itself. Here we demonstrate the critical role of multi-modality imaging in the visualization and positioning of this device in this patient with d-TGA post-Mustard anatomy.

VERY LONG-TERM COMPLICATIONS IN 132 PATIENTS WITH TRANSPOSITION OF THE GREAT ARTERIES WHO RECEIVED THE ATRIAL SWITCH OPERATION

Previous studies about long-term survivors of the atrial switch operation for d-transposition of the great arteries (TGA), known as the Mustard or Senning procedure, review complications predominantly in the first 3 decades after palliation. Little is published about very late complications after 30

SYSTEMIC VENOUS BAFFLE OBSTRUCTION AND LEAK INTERVENTION RESOLVES OXYGEN REQUIREMENT AND ASCITES IN TRANSPOSITION OF GREAT ARTERIES PALLIATED WITH MUSTARD PROCEDURE

Mustard procedure for the correction of transposition of the great arteries (TGA) is associated with a high rate of long-term complications including arrhythmia, systemic right ventricular dysfunction, and baffle obstruction. Desaturation is infrequent and ascites is rare. A 45 year old with TGA