Dysfunction Of Systemic Right Ventricle Research Articles

Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy

BackgroundSystemic right ventricle (RV) dysfunction is associated with lower transplant-free survival (TFS) in hypoplastic left heart syndrome (HLHS), but the likelihood of functional improvement and utility of heart failure (HF) medications is not understood. ObjectivesThe authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function. MethodsThe SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days. We compared TFS, HF medications, and surgical interventions in HLHS patients with and without functional normalization. ResultsOf 99 patients with HLHS and RV dysfunction, 52% had normalized function for ≥30 days. TFS at 2 years after dysfunction onset was lower in those without normalization (14% vs 78%, P < 0.001). Patients without normalization were less likely to reach target dosing (TD) of HF medications (27% vs 47% on 1 medication at TD, P < 0.001) and undergo Fontan completion (7% vs 53%, P < 0.001). Clinical factors associated with improved TFS were normalization of function for ≥30 days, onset of dysfunction after bidirectional Glenn, and exposure to ACE inhibition. ConclusionsOur cohort of HLHS patients with systemic RV dysfunction demonstrated a novel finding of improved TFS in those with functional normalization for ≥30 days. Achieving TD of HF medications was associated with improved outcomes. This may reflect patient stability and tolerance for HF medication more than its therapeutic effect, but it can help inform decisions to proceed with surgical palliation or list for transplant.

Comparative prognostic value of different echocardiographic parameters of the subpulmonary left ventricle in adults with a systemic right ventricle

Abstract Introduction Adults with complex congenital heart disease and a systemic right ventricle (SRV) are at increased risk of SRV dysfunction, heart failure, and premature death. In the systemic left ventricle (LV), impaired global longitudinal strain (GLS) is a strong predictor of adverse outcome. In the subpulmonary LV, however, echocardiographic assessment lacks standardisation and the relative prognostic significance of metrics of subpulmonary LV function remain unknown. Purpose To assess the correlation and relative prognostic power of quantitative metrics of subpulmonary LV function in adults with an SRV. Methods All adults with an SRV who underwent echocardiography in 2011-2019 at our specialist centre and had sufficient data for quantitative multiparametric subpulmonary LV assessment were included. LV systolic function was assessed by fractional area change (FAC), 4-chamber global longitudinal strain (GLS) and free wall longitudinal strain (FWLS). Linear regression was used to assess the correlation between echocardiographic variables of subpulmonary LV function. Cox regression analysis was used to assess the relation between echocardiographic predictors and the combined endpoint of death or transplantation. TimeROC analysis was performed to assess risk variable performance. Results In total, 120 patients (43.5±12.1 years, 60.0% male, 37.5% congenitally corrected transposition of the great arteries) were included. Most patients (94, 79.0%) were in NYHA functional class I/II and two-thirds (79, 66.4%) were receiving ≥1 heart failure therapy. One half of patients (63, 52.5%) had at least moderate systolic SRV dysfunction at baseline assessment with a median SRV FAC of 27[22-32]%. Subpulmonary LV function was visually preserved in most patients (98, 81.7%), with a LV FAC of 47.0[40.0-52.9]%. Median GLS was -15.2[-19.7–11.8]% and LV FWLS -19.9[-24.4–12.8]%. There was a weak correlation between LV FAC and GLS (r=0.25). During a median follow-up of 4.9[3.4-5.6] years, 24(20.0%) patients died and 3(2.5%) underwent heart or heart-lung transplantation. The 5-year transplant-free survival was 78.9%, (95%CI: 71.7-86.8%). On univariable Cox analysis, LV FAC (/5%, HR 0.55, 95%CI:0.47-0.65, p&amp;lt;0.0001) and GLS (/5%, HR 1.6, 95%CI:1.1-2.31, p=0.01), but not LV FWLS (/5%, HR 1.25, 95%CI:0.99-1.58, p=0.06) emerged as predictors of death or transplantation. LV GLS did not remain predictive in a bivariable model including LV FAC. LV FAC outperformed GLS on timeROC analysis (5-year AUC 0.87 vs. 0.64, p&amp;lt;0.0001; Figure). Conclusions Impaired systolic function of the subpulmonary LV strongly associated with mortality and heart or heart-lung transplantation in adults with an SRV. Both LV FAC and GLS are related to outcomes and should be a part of comprehensive echo assessment. LV FAC, a marker longitudinal and radial contraction, may better reflect pathophysiological mechanisms of subpulmonary LV failure and has greater prognostic significance in SRV patients.5-year ROC curves of LV FAC and GLS

Novel Bridge to Recovery: Right Ventricular Assist Device for Primary Graft Dysfunction in Pediatric Lung Transplantation

<h3>Introduction</h3> Primary graft dysfunction (PGD) is an early complication after lung transplantation (LTx). Extracorporeal membrane oxygenation (ECMO) is used for rescue of refractory hypoxemia due to severe PGD, although ventricular assist device support in PGD after LTx is less well documented. <h3>Case Report</h3> A 9 year old boy with Trisomy 21 underwent bilateral LTx for severe pulmonary arterial hypertension (PAH). Mean PA pressure was 73 mmHg, with a hypertrophied right ventricle (RV) and mild-moderately reduced RV function. He was transplanted with size matched lungs, blood type O- into O+. No HLA antibodies were identified. Marked vasoplegia was seen 8 hours post-operatively. Chest x-ray showed interstitial markings consistent with PGD. At 36 hours, P/F ratio had dropped to 59. Systemic inflammatory response (SIRS) and RV dysfunction resulted in acute kidney injury with Cr 256 µmol/L and hepatic dysfunction with ALT > 4,600. At 40 hours, right ventricular assist device (RVAD) with oxygenator was placed via central cannulation to the main PA and right atrium (RA). Peripheral sites were not available due to prior ECMO. Stenosis was seen of the right upper pulmonary vein. Oxygenation, lung aeration, and biochemical markers improved over 11 days after which he was weaned from RVAD and decannulated. At 8 months post-LTx, he is doing well. Graft function is excellent with most recent FEV₁ at 71% predicted, versus 37% pre-transplant. Acute kidney injury has resolved and liver function has returned to normal. <h3>Summary</h3> This case presents a novel successful approach in providing bridge to recovery for PGD after LTx and RV dysfunction in a pediatric patient with Down syndrome. This was a unique scenario with no peripheral cannulation access, RV systolic and diastolic dysfunction, and multi-system sequelae. Central cannulation (RA-PA) with RVAD/oxygenator allowed adequate support for the PGD to clear. To our knowledge, this is the first publication on RVAD/oxygenator use in pediatric lung PGD. This also documents the first patient with Down syndrome to receive a bilateral LTx in Canada. It is unclear if Down syndrome-related factors increased the risk for PGD; PV stenosis and SIRS may have played a role. Note that RA to aortic cannulation is not advised due to the need to maintain flow to the PAs, since there is no longer bronchial circulation supplying the lungs after transplant.

Systemic Right Ventricle Mechanical Support with Impella 5.5 as a Bridge to Cardiac Transplantation

<h3>Introduction</h3> A 39-year-old man with Senning atrial switch for dextro-transposition of the great arteries (d-TGA) presented with systemic right ventricle (RV) failure and cardiogenic shock. He had Impella 5.5 percutaneous mechanical support for his systemic RV and received cardiac transplantation 42 days later. To our knowledge, this has not been previously reported in the literature. <h3>Case Report</h3> A 39-year-old man with Senning atrial switch for d-TGA and long-standing systemic RV dysfunction on heart failure medical and resynchronization therapy was transferred for cardiogenic shock. Diuresis was unsuccessfully attempted with a furosemide drip, so inotropes and evaluation for advanced heart failure therapies were initiated. His condition initially improved, but on hospital day 8, he had a ventricular fibrillation cardiac arrest, requiring cardiopulmonary resuscitation and emergent femoral intra-aortic balloon pump (IABP) placement. Right heart catheterization showed cardiac index of 2.16 L/min/m^2 on milrinone, dobutamine, and IABP. He continued to clinically deteriorate. After multidisciplinary discussions, an axillary Impella 5.5 device was placed under fluoroscopic and TEE guidance. The device could not be rotated posteriorly away from the RV free wall due to the opposing posterior orientation of the atrioventricular valve and anterior course of the innominate artery (figure). However, there was significant hemodynamic improvement and no arrhythmias, so device was secured. He was listed for heart transplantation as UNOS Status 2. Forty-two days after Impella placement, a suitable donor heart became available. He underwent successful transplant with initial biopsy showing no rejection. He was discharged on post-operative day 12 and continues to do well as an outpatient. <h3>Summary</h3> Impella 5.5 device implantation in the systemic RV is a feasible strategy for patients with d-TGA and atrial switch awaiting durable VAD or transplant.

Vertical Ventricular Assist Device Placement to Systemic Right Ventricle for a Patient with Corrected Transposition of the Great Arteries and Mesocardia

Introduction Patients with physiologically repaired corrected transposition of the great arteries (cTGA) have morphological right ventricle (RV) as systemic ventricle. They may develop systemic RV failure in long-term and subsequently require heart transplantation with or without ventricular assist device (VAD) placement. However, VAD placement for systemic RV carries technical challenges, including trabeculated and diverse shapes of RV, close proximity to tricuspid valve and various apex positions. Case Report A case was 38-year-old female with cTGA and pulmonary stenosis. She had surgical history of physiological repair and redo pulmonary artery plasty at 10 and 19 years of age, respectively. She had history of heart failure symptoms with systemic RV dysfunction since 27 years of age. She was re-admitted for recurrent heart failure and listed for heart transplantation at 37 year of age. Her cardiac position was mesocardia and systemic RV was longitudinally elongated with severely dilated left atrium. The aorta was left anterior to the pulmonary trunk. Therefore, the standard VAD placement from cardiac apex may cause interference of sternal closure by pump body, inflow obstruction due to close proximity to the tricuspid valve, and difficult anastomosis to the short anterior ascending aorta. In contrast, the vertical VAD placement from diaphragmatic surface towards the aortic valve seemed to resolve these challenges. At the operation, a cardiopulmonary bypass was established through femoral cannulation. Under transesophageal echocardiogram guidance, the optimal inflow cannulation site was decided. After aortic cross-clamp, the systemic RV trabeculation was resected and the 3rd generation VAD pump was placed vertically from RV diaphragmatic surface. The outflow graft was laid along left side of the heart and anastomosed to left lateral ascending aorta. Postoperatively, the VAD flow had been stable with no evidence of sucking or thrombus formation. She was discharged and currently waiting for heart transplantation. Summary It is important to determine VAD placement technique with thorough understanding of unique characteristics of cTGA. Vertical VAD placement to systemic RV and anastomosis of outflow cannula to the left side of the aorta can be a good option for patients with systemic RV failure with mesocardia.

Abstract 16391: Systemic Ventricular Dysfunction and Long Term Outcomes in Patients With D-transposition of the Great Arteries (D-TGA), Atrial Switch, and Systemic Right Ventricle Physiology

Introduction: Patients with D-TGA palliated with atrial switch operations have the morphologic right ventricle in the systemic position. There is increased risk of atrial arrhythmias and systemic right ventricle (SRV) failure. We sought to analyze the long term outcomes of these patients. Methods: All patients with D-TGA and SRV followed in the Adult Congenital Heart Disease Clinic of a large tertiary care institution were reviewed. A comprehensive retrospective analysis of the medical record was performed, including consult notes, ECGs, echocardiograms and electrophysiology reports. Results: A total of 154 patients (63% male) aged 29±11 years were followed for a mean of 10±9 years (range 0-51). During follow-up, 3 patients underwent cardiac transplantation and 15 died; 5 had sudden death, 2 had cardiogenic shock, 5 had non-cardiac death (i.e., infective endocarditis, sepsis) and 3 had unknown causes of death. Heart failure symptoms were present in 53(34%) patients. Severe SRV systolic dysfunction occurred in 37(24%) patients, with a mean EF of 23±5.5%; moderate SRV dysfunction occurred in 67(44%) patients, mean EF 35±4%. Sinus node dysfunction was present in 75(49%) patients, complete AV block in 9(5%) patients, and a pacemaker placed in 60(39%) patients, with cardiac resynchronization therapy in 5. Atrial arrhythmias occurred in 94(61%) patients and ablations were performed in 47(31%) patients. An ICD was implanted in 37 patients; 5 patients had appropriate shocks, but 7 had inappropriate shocks due to atrial arrhythmias. Age (HR 1.07, p=001), heart failure symptoms (HR 4.9, p= 0.007), severe SRV enlargement (HR 3.7, p=0.03), severe systolic dysfunction (HR 5.4, p=0.003), severe systemic AV valve regurgitation (HR 5.2, p=0.002) and a QRS duration&gt; 122ms (HR 3.7, p=0.02) were significant predictors of mortality. The 15 year probability of sudden death was 3.2%(95% CI 0-6.9%). Conclusions: Atrial arrhythmias are common after atrial switch operations secondary to atriotomy scars. Further studies will need to determine whether restoration of sinus rhythm or cardiac synchrony may prevent further deterioration of the systemic right ventricle. Severe SRV dysfunction and prolonged QRS duration &gt;122ms were significantly correlated with mortality.

2401Cardiac resinchronization therapy for the systemic right ventricle: a single center experience

Abstract Introduction Cardiac resynchronization therapy (CRT) has become a treatment of choice in patients with chronic heart failure (HF). About 25% of patients with systemic right ventricle (SRV) progress to symptomatic HF, which may be refractory to drug therapy and is commonly associated with significant morbidity and mortality. For these reasons, CRT has been emerging as an effective treatment strategy for patients with SRV failure and electrocardiographic signs of ventricular dyssynchrony. Few studies have reported the acute and long - term effects of CRT in SRV subjects, with different findings. Our study aimed to describe the experience with CRT in SRV patients in a single tertiary centre. Purpose Assess the long term efficacy of CRT in patient with SRV Materials and methods All consecutive SRV patients who underwent CRT implantation and/or upgrading between 1994 and 2018 at our tertiary centre were included. Clinical and echocardiographic parameters before and after CRT implantation were collected and analyzed. Results A total of 21 patients (mean age 47.8±14.8 years, 13 M) were implanted with CRT-P (12, 57%) or CRT-D (9,43%) during the study period. 90% of patients showed an anatomy of congenitally corrected transposition of the great arteries (CCTGA), whereas 9.5% underwent Mustard procedure for transposition of the great arteries (TGA). Among CCTGAs, 11 (52.5%) subjects underwent previous surgical procedures, including implant of a conduit between the left ventricle (LV) and the pulmonary artery (PA) in 8 (38%) patients, tricuspid valve repair in 2 (9.5%) and surgical closure of atrial septal defect in 1 (5%). Before CRT implant/upgrading, 10 (48%) patients had a moderate to severe reduction in the SRV ejection fraction (EF) and 7 (33%) had a moderate to severe tricuspid regurgitation (TR). Overall, 15 (71%) patients referred a NYHA II or III. After a median follow up of 57 months (IQR 35–83), 43% of patients showed an improvement in their functional status, which was associated with an improvement of SRV EF and TR only in 22% and 33% of these patients. On the contrary, no patient reported a worsening in NYHA class, while SRV EF decreased in 28.5% and the grade of TR worsen in 23.8% of patients. Conclusions CRT is emerging as an effective treatment for SRV dysfunction. However, criteria for implantation are not well defined and the deterioration of SRV function related to subpulmonary univentricular pacing should be considered. Moreover, TR did not improve in this study, suggesting that concurrent tricuspid valve interventions may be necessary in patients with severe TR and may facilitate the improvement in RV function achieved with CRT. Proper planning, tertiary expertise and international collaborations are all paramount in this field.

P5685Abnormal anatomy of the coronary sinus in congenitally corrected transposition of the great arteries (double discordance): a pitfall for transvenous cardiac resynchronization therapy?

Abstract Background Congenitally corrected transposition of the great arteries (ccTGA) or double discordance is a rare congenital heart defect associating discordant atrioventricular and ventriculoarterial connections. Late prognosis depends on the progressive failure of the systemic right ventricle (RV). A possible cause for systemic RV dysfunction could be ventricular asynchrony. Cardiac resynchronization therapy (CRT) may thus be indicated in some cases. However, the cardiologists may experience some problems with the coronary sinus (CS) when implanting a 3-lead CRT transvenous system in these patients. Aim of the study To evaluate the anatomy of the CS and cardiac veins in specimens with ccTGA, in order to assess the feasibility of transvenous CRT. Material and methods Among the anatomic collection of the French Centre of Reference for complex CHD, 51 heart specimens had ccTGA with 2 ventricles. There were 33 post-natal and 18 fetal hearts. Hearts were reviewed with special attention paid to the course and drainage of the CS and cardiac veins. Segmental anatomy, location of the ventricular septal defect (VSD), status of the pulmonary outflow tract and anomalies of the atrioventricular valves were reviewed. Results Segmental anatomy was {S,L,L} in 46/51 hearts, {S,L,D} in 2 and {I,D,D} in 3. There was a VSD in 40 (outlet in 25, inlet in 11, both in 2, muscular in 2), pulmonary atresia in 13, subpulmonary stenosis in 6, abnormal tricuspid valve in 20/48 including Ebstein anomaly in 6, straddling in 9 (3 had replacement). The CS was always located behind the morphologically left atrium (LA). However, its anatomy was normal, with normal drainage into the morphologically right atrium, in only 25/51 (49% of cases). The CS was of reduced length with normal orifice in 17. Orifice was atretic with normal size CS in 2, and CS was completely absent in 6 with direct drainage of coronary veins into the LA. At least 1 available vein was found in all cases with patent CS orifice. Conclusion CS in ccTGA is always located behind the morphologically LA. However, its anatomy is abnormal in half of cases. The most frequent anomalies are reduced length (33%) and absent CS or atretic orifice (15.5%). The anatomy of CS should therefore be assessed by imaging techniques (multislice CT imaging or CS venography) before considering transvenous CRT in these patients.

Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle.

Knowledge gaps exist in the life expectancy and functional outcome of patients with congenitally corrected transposition (ccTGA) presenting early in life, which is relevant in the evaluation of early anatomic repair. In a single-center analysis, 91 patients with ccTGA were identified over 25 years, of which 31 presented with biventricular anatomy in the first year of life and formed the study cohort. End points for analysis included survival, moderate or worse tricuspid valve regurgitation, and systemic right ventricle (RV) dysfunction. Median follow-up was 4.9 years (range: 7 days to 20 years). Among 31 patients presenting in the first year of life, 9 (29%) never received cardiac surgery, while 22 (71%) underwent 36 cardiac operations. Overall freedom from moderate or severe systemic RV dysfunction was 75% at 10 years. Overall survival was 82% at 10 years. Surgical mortality was 5.6% (2/36). Among survivors with a systemic RV, 23 (100%) of 23 were Ross or NYHA class I or II at last follow-up. Congenitally corrected transposition presenting in the first year of life and maintaining a systemic RV can expect (1) long-term survival of more than 80% at 10 years, (2) low expected surgical mortality (overall 6%), and (3) 75% late freedom from major RV dysfunction at 10 years. Pending multi-institutional analyses, this experience with a systemic RV in ccTGA provides an initial benchmark for comparison when considering early elective anatomic correction.

Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management.

The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.

Mid-Term Outcomes in Patients with Congenitally Corrected Transposition of the Great Arteries: A Single Center Experience

Optimal management of patients with congenitally corrected transposition of the great arteries (ccTGA) is unclear. The goal of this study was to compare the outcomes in patients with ccTGA undergoing different management strategies. Patients with ccTGA believed suitable for biventricular circulation, treated between 1995 and 2016, were included. The cohort was divided into 4 groups: systemic right ventricle (RV) (patients without surgical intervention or with a classic repair), anatomic repair, Fontan palliation, and patients receiving only a pulmonary artery band (PAB) or a shunt. Transplant-free survival from presentation was calculated for each group. The cohort included 97 patients: 45 (46%) systemic RV, 26 (27%) anatomic repair, 9 (9%) Fontan, and 17 (18%) PAB/shunt. Median age at presentation was 2 months (range 0 days to 69 years) and median follow-up was 10 years (1 month to 28 years). At initial presentation, 10 (11%) patients had any RV dysfunction (8 mild, 2 severe), and 16 (18%) patients had moderate or severe tricuspid regurgitation (TR). During the study, 10 (10%) patients died, and 3 (3%) patients underwent transplantation. At last follow-up, 11 (11%) patients were in New York Heart Association class III/IV, 5 (5%) had moderate or severe systemic ventricle dysfunction, and 16 (16%) had moderate or severe systemic atrioventricular valve regurgitation. Transplant-free survivals at 10 years were 93%, 86%, 100%, and 79% for systemic RV, anatomic repair, Fontan palliation, and PAB/shunt, respectively (p= 0.33). On multivariate analysis, only systemic RV dysfunction was associated with a higher risk for death or transplant (p= 0.001). Transplant-free survival in ccTGA appears to be similar between patients with a systemic RV, anatomic repair, and Fontan procedure. Systemic RV dysfunction is a risk factor for death and transplant.

Management of Systemic Right Ventricular Failure in Patients With Congenitally Corrected Transposition of the Great Arteries.

In recent decades, significant progress has been made in the diagnosis and management of congenitally corrected transposition of the great arteries (ccTGA). Nevertheless, gradual dysfunction and failure of the right ventricle (RV) in the systemic circulation remain the main contributors to mortality and disability for patients with ccTGA, especially after adolescence. Anatomic repair of ccTGA effectively resolves the problem of failure of the systemic RV and has good early and midterm results. However, this strategy is applicable primarily in infants and children up to their teens and has associated risks and limitations, and new challenges can arise in the late postoperative period. Patients with ccTGA manifesting progressive systemic RV dysfunction beyond adolescence represent the major challenge. Several palliative options such as cardiac resynchronization therapy, tricuspid valve repair or replacement, pulmonary artery banding, and implantation of an assist device into the systemic RV can be used to improve functional status and to delay the progression of ventricular dysfunction in patients who are not suitable for anatomic correction of ccTGA. For adult patients with severe systemic RV failure, heart transplantation currently remains the only long-term lifesaving procedure, although donor organ availability remains one of the most limiting factors in this type of therapy. This review focuses on current surgical and medical strategies and interventional options for the prevention and management of systemic RV failure in adults and children with ccTGA.

Squeezing better function out of the systemic right ventricle by optimizing its pacing site

The morphologic right ventricle (RV) is the systemic pumping chamber predominantly in 2 types of biventricular congenital heart disease: congenitally corrected transposition of the great arteries and following repair of transposition of the great arteries (TGA) using a Senning or Mustard atrial switch procedure. In both conditions, the systemic RV is prone to fail. Indeed, after the atrial switch procedure for TGA, long-term systemic RV function is a major concern and profoundly affects patient outcomes. Roos-Hesselink et al 1 Roos-Hesselink J.W. Meijboom F.J. Spitaels S.E. et al. Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22–29 years). Eur Heart J. 2004; 25: 1264-1270 Crossref PubMed Scopus (198) Google Scholar found that while all patients had good RV function 14 years after undergoing an atrial switch procedure, almost two-thirds showed moderate-to-severe systemic RV dysfunction after 25 years. RV dysfunction was associated with QRS prolongation, a decline in exercise capacity and clinical status and presumably increased need for heart transplant. Reduced myocardial strain and other indices of RV performance form the basis for systemic RV failure in this setting. 2 Eyskens B. Weidemann F. Kowalski M. et al. Regional right and left ventricular function after the Senning operation: an ultrasonic study of strain rate and strain. Cardiol Young. 2004; 14: 255-264 Crossref PubMed Scopus (56) Google Scholar Conversely, RV dysfunction is not commonly seen in the subpulmonary RV when TGA is treated with an arterial switch operation. Therefore, it is the systemic position of the RV that predisposes it to failure. Acute hemodynamic benefits of biventricular and single-site systemic ventricular pacing in patients with a systemic right ventricleHeart RhythmVol. 10Issue 5PreviewPatients treated by atrial redirection surgery (Senning or Mustard procedure) for transposition of the great arteries (TGA) have an important risk for heart failure caused by dysfunction of the systemic right ventricle. Conventional nonsystemic ventricular pacing (non-systVP) may even further increase this risk. Full-Text PDF

Relationship between cardiac magnetic resonance imaging parameters and pregnancy outcomes in women post mustard repair: a multi-center study

Background Summary: To examine the relationship between cardiovascular magnetic resonance imaging (CMR) parameters and pregnancy outcomes post Mustard palliation. Background: Impaired systemic ventricular function and presence of a systemic right ventricle (RV) are associated with adverse events in pregnancy. Women with transposition of the great arteries post atrial switch (Mustard procedure) are at risk of worsening arrhythmia and heart failure antenatally. Contemporary guidelines suggest that women with “more moderate systemic RV dysfunction” should be advised against pregnancy; however a threshold RV ejection fraction (EF) has not been defined as all studies to date have examined RV function using echocardiography alone. The CMR characteristics of women post Mustard palliation undergoing pregnancy and the relationship between CMR parameters and pregnancy outcomes have not yet been described. Methods A total of 17 consecutive women post Mustard procedure seen at 2 tertiary care centers who had undergone CMR within 2 years of pregnancy were included. Parameters of ventricular function were assessed by a single experienced reader using steady-state free-precession cine CMR acquired in the short-axis orientation. Adverse cardiovascular events (sustained arrhythmia, heart failure, stroke, cardiac arrest and/or urgent cardiac intervention), obstetric complications (eclampsia, pre-term labour, hemorrhage) and fetal/neonatal events (stillbirth/death, prematurity, low birthweight, intensive care unit admission) were recorded. Results

Right Ventricle: Wrong Targets?

Pharmacotherapeutic algorithms developed to treat heart failure in adults with acquired heart disease have often provided the template for treatment of ventricular dysfunction or heart failure symptoms in patients with underlying congenital heart disease. Superficially at least, this is not completely irrational, because congenital patients often demonstrate similar levels of functional incapacity and a neurohomonal profile similar to that seen in acquired heart failure.1 Scratch beneath the surface, however, and the situation is much less clear. Systolic failure as the primary manifestation and driver of disease progression is rather rare, fundamentally the circulation in these patients is often quite different, and the rate of functional decline and adverse events tends to be substantially lower than that observed in the major studies of β-blockade and modifiers of the renin–angiotensin system in acquired heart failure. Consequently there is an ever-increasing catalog of, albeit small, randomized, controlled trials that have failed to show a significant clinical benefit of these therapies in congenital heart disease.2–6 The study by van der Bom et al,7 examining the role of valsartan in patients with a systemic right ventricle (RV) and published in this issue of Circulation , can now be added to that list. Article see p 322 In congenital heart disease with a biventricular physiology, there are 2 main situations where the circulation depends on the ability of the right ventricle (RV) to drive blood through the systemic vascular bed. These are transposition of the great arteries (TGA) after an atrial-level surgical correction and congenitally corrected transposition of the great arteries (ccTGA). In both settings, the RV is positioned beneath the aortic valve and is at high risk for long-term failure, the incidence of which increase with age. In each subgroup systemic RV dysfunction usually precedes the onset of clinical heart …

Sympathetic dysfunction of systemic right ventricle in congenitally corrected transposition of the great arteries

We evaluate the systemic right ventricle (SRV) failure of congenitally corrected transposition of the great arteries using the scintigraphic studies in a case with 32-year-old male who improved the heart failure by the use of beta-adrenergic blockers. The myocardial perfusion sympathetic nervous system activity mismatch with preserved coronary flow and wall thickness indicates a distinctive feature as SRV cardiomyopathy, suggesting the importance of recovery of SRV sympathetic dysfunction for improving the prognosis.

A pilot study on the effects of carvedilol on right ventricular remodelling and exercise tolerance in patients with systemic right ventricle

Patients with atrial repair for transposition of the great arteries and patients with congenitally corrected transposition have a right ventricle (RV) in the systemic position and they may develop RV dysfunction and exercise intolerance with advancing age. No data is available on the effect of carvedilol in patients with dysfunctional systemic RV. We studied with cardiovascular magnetic resonance (CMR), cardiopulmonary exercise testing, and standard 12-leads electrocardiogram, 8 adults (median age 26 years, range 18-31) with chronic stable heart failure and systemic RV dysfunction (6 patients with atrial repair and 2 patients with congenitally corrected transposition). Assessment was done before and after 12 months of carvedilol administration. The initial dose was 3.125 mg twice daily, and the target dose was 25 mg twice a day. Carvedilol administration was safe and the target dose was achieved in 5/8 (62%) patients. Right ventricular end-diastolic (119 ± 31 vs. 112 ± 28 ml/m², p=0.01) and end-systolic volumes decreased (79 ± 17 vs. 65 ± 14 ml/m², p=0.006), and RV ejection fraction improved (34 ± 6 vs. 42 ± 7%, p=0.004). Left ventricular ejection fraction increased (44 ± 8 vs. 49 ± 9%, p=0.01), suggesting a positive biventricular remodelling. Peak oxygen uptake did not change with carvedilol (26.8 ± 5.3 vs. 27.3 ± 5.7 ml O₂/Kg/min, p=0.58), whereas exercise duration increased (13.4 ± 2.6 vs. 17.3 ± 3.1 min, p=0.008). In this small cohort, carvedilol administration was safe and it was associated with positive RV remodelling as well as improved exercise duration.