Systemic Ventricular Dysfunction Research Articles

Two Cases of Wearing an Implantable Ventricular Assist Device in the Late Postoperative Period after the Fontan Operation Two Cases of Wearing an Implantable Ventricular Assist Device in the Late Postoperative Period after the Fontan Operation

Introduction Advancements in perioperative management and operation methods have yielded major improvements in the mid-term postoperative results of Fontan operations. This has progressively revealed various problems such as decreased ventricular function and exercise tolerance capacity over time, along with hindrances to multiple organs throughout the body. We herein report the two cases of wearing an implantable ventricular assist device in the late postoperative period after the Fontan operation. Case Report [Case 1] A 13 year-old female. Her ventricular function gradually decreased following TCPC when she was four years old. She had been hospitalized multiple times due to aggravation of her cardiac insufficiency and she was fitted with a Jarvik2000 following approval of her suitability for a transplant. Her systemic venous pressure prior to the operation was 18 mmHg, CI 1.2. With a heart catheter three years after the operation, it was confirmed that she had recovered to a comparatively satisfactory Fontan circulation, with a systemic venous pressure of 8 mmHg, CI 1.8. [Case 2] An 11 year-old girl. Although she had undergone TCPC at two years of age, it was already indicated at that point that her ventricular function had decreased. A viral infection at the age of eleven further decreased her cardiac function, which required an extracorporeal LVAD-ECMO. After the operation, her physical status has recovered satisfactorily and an HVAD was implanted following approval of her suitability for a transplant. Her systemic venous pressure before the operation was 17 mmHg, CI 1.5. However, with a heart catheter three months after the operation, it was confirmed that she had recovered to a comparatively satisfactory Fontan circulation, with a systemic venous pressure of 10 mmHg, CI 2.4. Summary Failed Fontan circulation is primarily caused by systemic ventricular dysfunction, increases in pulmonary vascular resistance, or by a combination of the two. For cases similar to what we experienced at our facility, we can expect that cardiac assistance can improve Fontan circulation for cases in which pulmonary vascular resistance before failure of the hemodynamics is low and in which increases in pulmonary vascular resistance is reversible.

249 Labor length and adverse cardiac outcomes among women with congenital heart disease

Women with congenital heart disease (CHD) are at high risk for peripartum morbidity, yet data is limited on the impact of labor on morbidity. Our objective was to evaluate whether exposure to labor and duration of labor were associated with rates of adverse maternal cardiac outcomes among women with CHD. This was a secondary analysis of a retrospective cohort study of women ≥18 years with CHD who received prenatal care and delivered at the Hospital of the University of Pennsylvania between 1998-2014 with a singleton gestation. Only the first pregnancy was counted for women who delivered more than once. Pregnancies that ended in termination or loss <24 weeks gestation were excluded. A composite adverse maternal cardiac outcome included any cardiac event during pregnancy or <6 months post-partum (specific outcomes listed in Figure), compared among women who did and did not labor. 168 women were included; 34 (20.2%) underwent planned cesarean delivery (CD) without labor and 134 (79.8%) labored. The overall rate of adverse cardiac outcome was 20.2%; 23.5% in the planned CD group and 19.4% in those that labored, regardless of mode of delivery (p=0.6). The most common adverse events were heart failure (13.7%) and arrhythmia (7.7%). As shown in the Figure, women who delivered in <24 hours (regardless of mode of delivery) had a lower rate of adverse cardiac outcome than those that delivered after ≥24 hours of labor, though not statistically significant (p=0.06). When adjusting for confounders including NYHA class, systemic ventricular dysfunction, and mode of delivery, women with labor length ≥15 hours had a 3.7-fold higher odds of adverse cardiac outcome (aOR 3.7 [1.1-12.9]). In this retrospective cohort of women with CHD, exposure to labor was not associated with an increased risk of adverse cardiac outcome. However, among those who labored, longer labor was associated with an increased risk, regardless of mode of delivery. Attention should be paid to the duration of labor in women with CHD, as achieving a vaginal delivery does not mitigate the risks of prolonged labor.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Cardiac resynchronization therapy in paediatric patients with congenital heart disease: single centre with 10 years of experience.

In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD. Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically. The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2-18 years) and the median weight was 39 kg (10-81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6-117 months). When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Multimodality Imaging of Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. D-TGA manifests during the neonatal period and can be surgically managed by atrial switch operation (AtrSO), arterial switch operation (ASO), Rastelli procedure, or Nikaidoh procedure. Arrhythmia, systemic ventricular dysfunction, baffle stenosis, and baffle leak are the common complications of AtrSO, whereas supravalvular pulmonary or branch PA stenosis, neoaortic dilatation, and coronary artery narrowing are the common complications of ASO. CCTGA may manifest late in life, even in adulthood. Surgeries for associated lesions such as tricuspid regurgitation, subpulmonic stenosis, and ventricular septal defect may be performed. A double-switch operation that includes both the atrial and arterial switch operations constitutes anatomic correction for CCTGA. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for presurgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. The authors review the imaging appearances of TGA, with a focus on pre- and postsurgical imaging. Online supplemental material is available for this article. ©RSNA, 2021.

Emerging Treatment Options for the Systemic Right Ventricle: A Single Centre Experience of Sacubitril/Valsartan in Transposition of Great Arteries

Over the last few decades, we are appreciating the need of tailored treatment options for adults with congenital heart disease. Patients with D-transposition of great arteries (D-TGA) with atrial switch operations or L-TGA often develop systemic right ventricular (sRV) dysfunction as a long-term complication. Sacubitril/valsartan has become guideline-based therapy for patients with symptomatic left ventricular systolic dysfunction, however it is unclear if these results can be extrapolated to patients with a sRV.

Impact of electrophysiological features acquired after anatomical repair of congenital corrected transposition of the great arteries on late mortality and ventricular dysfunction.

In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries. Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Fragmented QRS was defined as ≥1 discontinuous deflections in narrow QRS complexes, and ≥2 in wide QRS complexes, in 2 contiguous electrocardiogram leads. The primary end point was a composite of all-cause mortality and systemic ventricular dysfunction. A total of 74 patients were included. Among them, 30, 15 and 29 underwent the Senning arterial switch, the Senning Rastelli and the hemi-Mustard/bidirectional Glenn/Rastelli procedures, respectively. The primary end point occurred in 9 (12.2%) patients and included 7 late deaths and 2 cases of late-onset systemic ventricular dysfunction. Fragmented QRS and QRS prolongation were noted in 19 (25.7%) and 21 (28.4%) patients, respectively. In patients with the primary end point, QRS fragmentation (6/9 vs 10/65; P < 0.001) and QRS prolongation (6/9 vs 15/65; P = 0.013) were noted more frequently than in patients without the primary end point. No statistical differences in these electrocardiogram findings were found among patients treated with 3 surgical strategies. Appearance of QRS fragmentation or QRS prolongation is associated with death or ventricular dysfunction in anatomically repaired corrected transposition of the great arteries. Although there is a trend that QRS fragmentation and QRS prolongation appear more frequently in patients who had the Senning-arterial switch operation, there is no statistically significant difference associated with these electrocardiogram features among varied procedures.

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.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood

The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

LONG-TERM SURVIVAL AFTER HEART FAILURE IN CHILDREN WITH HYPOPLASTIC LEFT HEART SYNDROME

Hypoplastic left heart syndrome (HLHS) is one of the more common indications for staged surgical palliation. These patients can develop systemic ventricular dysfunction at different stages of the surgical palliation, but its effect on outcome remains unclear. We sought to describe HLHS patients developing persistent ventricular dysfunction and to compare transplant (tx) free survival of patients developing ventricular dysfunction before and after each surgical stage.

Utility of biomarkers in adult Fontan patients with decompensated heart failure

The incidence of heart failure is increasing within the Fontan population. The use of serological markers, including B-type natriuretic peptide, has been limited in this patient population. This was a single-centre retrospective study of Fontan patients in acute decompensated heart failure. Fontan patients underwent a 1:2 match with non-Fontan patients for each heart failure hospitalisation for comparative analysis. A univariate logistic regression model was used to assess associations between laboratory and echocardiographic markers and a prolonged length of stay of 7 days or greater. B-type natriuretic peptide levels were significantly lower in Fontan patients admitted for heart failure than that in non-Fontan patients [390.9 (±378.7) pg/ml versus 1245.6 (±1160.7) pg/ml, respectively, p < 0.0001] and were higher in Fontan patients with systemic ventricular systolic or diastolic dysfunction than that in Fontan patients with normal systemic ventricular function [833.6 (±1547.2) pg/ml versus 138.6 (±134.0) pg/ml, p = 0.017]. The change from the last known outpatient value was smaller in Fontan patients in comparison with non-Fontan patients [65.7 (±185.7) pg/ml versus 1638.0 (±1444.7) pg/ml, respectively, p < 0.0001]. Low haemoglobin and high blood urea nitrogen levels were associated with a prolonged length of stay. B-type natriuretic peptide levels do not accurately reflect decompensated heart failure in Fontan patients when compared to non-Fontan heart failure patients and should, therefore, be used with caution in this patient population.

NADPH oxidases and HIF1 promote cardiac dysfunction and pulmonary hypertension in response to glucocorticoid excess

Cardiovascular side effects are frequent problems accompanying systemic glucocorticoid therapy, although the underlying mechanisms are not fully resolved. Reactive oxygen species (ROS) have been shown to promote various cardiovascular diseases although the link between glucocorticoid and ROS signaling has been controversial. As the family of NADPH oxidases has been identified as important source of ROS in the cardiovascular system we investigated the role of NADPH oxidases in response to the synthetic glucocorticoid dexamethasone in the cardiovascular system in vitro and in vivo in mice lacking functional NADPH oxidases due to a mutation in the gene coding for the essential NADPH oxidase subunit p22phox. We show that dexamethasone induced NADPH oxidase-dependent ROS generation, leading to vascular proliferation and angiogenesis due to activation of the transcription factor hypoxia-inducible factor-1 (HIF1). Chronic treatment of mice with low doses of dexamethasone resulted in the development of systemic hypertension, cardiac hypertrophy and left ventricular dysfunction, as well as in pulmonary hypertension and pulmonary vascular remodeling. In contrast, mice deficient in p22phox-dependent NADPH oxidases were protected against these cardiovascular side effects. Mechanistically, dexamethasone failed to upregulate HIF1α levels in these mice, while vascular HIF1α deficiency prevented pulmonary vascular remodeling. Thus, p22phox-dependent NADPH oxidases and activation of the HIF pathway are critical elements in dexamethasone-induced cardiovascular pathologies and might provide interesting targets to limit cardiovascular side effects in patients on chronic glucocorticoid therapy.

Initial Experience in Adults with Complex Congenital Heart Disease Treated with Sacubitril/Valsartan for Heart Failure

Adults with congenital heart disease (ACHD) are an emerging population. Heart failure (HF) is a major cause of morbidity and mortality for ACHD. Sacubitril/valsartan has been shown to reduce the risk of hospitalizations and death from HF in acquired cardiovascular disease but there is little information available on its effect on HF in ACHD. We retrospectively studied patients with complex congenital heart disease (CHD) who were prescribed sacubitril/valsartan to treat HF. Data collected includes New York Heart Association (NYHA) classification, laboratory data, vital signs, and imaging by echocardiogram. Eight ACHD patients with HF with a median age of 40.6 [IQR 33.3-52.8] years were treated with sacubitril/valsartan for a median duration of 269 [IQR 102-473] days. Five (62.5%) had a systemic right ventricle and 3 (37.5%) had single ventricle anatomy. Five (62.5%) had severe systemic systolic ventricular dysfunction. At time of last follow-up, 3 (37.5%) patients at NYHA III had no change in functional class, 4 (50%) patients at NYHA IV improved to NYHA II. One patient has not yet returned for follow-up to evaluate functional status. No patients were noted to have a substantial change in renal function or potassium levels. Median brain natriuretic peptide values pre and post- sacubitril/valsartan were 2517 pg/mL and 1040 pg/mL, respectively. Median mean arterial pressures pre and post-sacubitril/valsartan were 83 mmHg and 77 mmHg, respectively. One (12.5%) patient required dose reduction due to hypotension. At time of last follow-up, 2 (25%) achieved 97 mg/100 mg dose of sacubitril/valsartan. No patients experienced symptoms related to angioedema. In a small cohort of adults with complex CHD and HF, sacubitril/valsartan was well-tolerated, with some patients experiencing improvement in NYHA class. More studies and a registry of outcomes of sacubitril/valsartan use in ACHD are needed.

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

Abnormal Coronary Anatomy in Patients with Transposition of the Great Arteries and Atrial Switch: A Predictor of Serious Cardiac Adverse Events?

Sudden cardiac death and heart failure are well known long-term complications after atrial switch for D-transposition of the great arteries (D-TGA). Right systemic ventricular dysfunction is common and myocardial ischemia has been implicated as a putative mechanism for sudden death, with coronary anomalies prevalent in 30% of cases. We sought to assess an association between adverse events and coronary anomalies in patients with D-TGA and atrial switch surgery. An observational study was conducted in 3 tertiary centers (Montreal Heart Institute, Canada, Nationwide Children’s hospital, Chicago, USA and Hopital cardiologique Louis Pradel de Lyon, France). Adults with D-TGA and atrial switch surgery qualified for inclusion if they had a major adverse cardiovascular event (MACE), i.e., ventricular arrhythmia, sudden cardiac death, heart failure, cardiac transplantation, or cardiovascular death. The prevalence of coronary anomalies was compared to historical controls. Forty-five patients were included. Twenty-one (46.7%) patients experienced a ventricular arrhythmia and 35 (77.8%) suffered from symptomatic heart failure and/or severe right ventricular dysfunction. Twelve patients (26.7%) had congenitally abnormal coronary arteries. There was no difference in the prevalence of coronary anomalies between the cohort with a MACE and a pooled population of 647 historical controls with D-TGA (28.7%, p = 0.89). In conclusion, the prevalence of congenital coronary anomalies is not higher in patients with D-TGA and atrial switch surgery who had adverse cardiovascular events. It could be hypothesized that ischemic complications in this patient population are more likely to be related to a supply-demand mismatch of the distal microvasculature rather than proximal coronary anomalies.

CardioMEMS Implantation in Patient With a Systemic Right Ventricle

A 42-year-old woman with repaired, complex cyanotic congenital heart disease complicated by systemic right ventricular dysfunction presented for worsening heart failure. She successfully underwent CardioMEMS implantation and has since remained out of the hospital with improved functional class. (Level of Difficulty: Beginner.)

The impact of pulmonary artery banding and cardiac resynchronization therapy for the adult patient with congenitally corrected transposition of the great arteries

It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Cardiopulmonary exercise testing showed that the predictive value of anaerobic threshold and peak oxygen uptake improved from 65 to 99% and 59 to 92%, respectively. In conclusion, pulmonary artery banding and cardiac resynchronization therapy can be the first choice of surgery for severe tricuspid regurgitation with severely impaired systemic right ventricular function. After improving systemic right ventricular dysfunction tricuspid surgery could be the next choice as a surgical intervention in the future.

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potentialunder-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs. The inclusion criteria included age ≥18 years, systemic RVs, and at least 3 months of treatment with ACE inhibitor, ARB, beta blocker, or aldosterone antagonist. The outcomes included RV end-diastolic and end-systolic dimensions, RV ejection fraction, functional class, and exercise capacity. EMBASE, PubMed, and Cochrane databases were searched. The selected data were pooled and analyzed with the DerSimonian-Laird random-effects meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. A Bayesian meta-analysis model was also used in the event that heterogeneity was low. Bias assessment was performed with the Newcastle-Ottawa Scale and Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. Six studies met the inclusion criteria, contributing a total of 187 patients; treatment with beta blocker was the intervention that could not be analyzed because of the small number of patients and diversity of outcomes reported. After at least 3 months of treatment with ACE inhibitors, ARBs, or aldosterone antagonists, there was no statistically significant change in mean ejection fraction, ventricular dimensions, or peak ventilatory equivalent of oxygen. The methodological quality of the majority of included studies was low, mainly because of a lack of a randomized and controlled design, small sample size, and incomplete follow-up. In conclusion, pooled results across the limited available studies did not provide conclusive evidence with regard to a beneficial effect of medical therapy in adults with systemic RV dysfunction. Randomized controlled trials or comparative-effectiveness studies that are sufficiently powered to demonstrate effect are needed to elucidate the efficacy of ACE inhibitors, ARBs, beta blockers, and aldosterone antagonists in patients with systemic RVs.

Sudden death in transposition of the great arteries with atrial switch surgery: Autopsy evidence of acute myocardial ischemia despite normal coronary arteries

BackgroundSudden death is the leading cause of mortality in patients with transposition of the great arteries (TGA) and atrial switch surgery. Understanding underlying mechanisms could contribute to identifying high-risk patients and preventing such catastrophic deaths. MethodsA total of 144 adults (≥18 years) with TGA and atrial switch surgery were followed at our adult congenital center since 1989. Four patients were excluded: two with double-outlet right ventricles and two with subsequent arterial switch surgery in childhood. ResultsOf the remaining 140 patients, age 37.6 ± 7.8 years, 37.1% female, 8 (6%) had a cardiac arrest of presumed arrhythmic etiology of whom 3 were resuscitated. The arrests occurred in 3 women and 5 men at age 30.5 ± 8.6 (range 22 to 50) years. None had established coronary artery disease, sustained ventricular arrhythmias, or syncope. Four (50%) had atrial arrhythmias and 6 (75%) had at least moderate systemic right ventricular dysfunction. For 5 patients in whom circumstances surrounding the arrests were documented, 3 occurred on exertion, 1 after consuming recreational methamphetamine, and 1 in the context of an atrial tachyarrhythmia. Autopsies were performed in 2 of 5 patients. Both revealed acute massive myocardial infarction of the hypertrophied systemic right ventricle with normal coronary arteries and chronic subendocardial ischemic lesions. ConclusionThis is the first report to provide histopathological evidence in support of a myocardial ischemia hypothesis as a cause of sudden death in this patient population, despite the absence of coronary atherosclerosis.

Accuracy of risk prediction scores in pregnant women with congenital heart disease

To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone. Single-center retrospective study. Tertiary care academic hospital. Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman. The primary outcome was defined by ≥1 of the following: arrhythmia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myocardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum. Of 178 women, the most common CHD lesions were congenital aortic stenosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and tetralogy of Fallot (12.9%). Thirty-five women (19.7%) sustained 39 cardiac events. Observed vs expected event rates were 9.9% vs 5% (P=.02) for CARPREG I score 0 and 26.1% vs 7.5% (P<.001) for ZAHARA scores 0.51-1.5. ZAHARA outperformed CARPREG I at predicting adverse cardiovascular outcomes (AUC 0.80 vs 0.72, P=.03) but was not significantly better than modified WHO. Clinical predictors of adverse cardiac event were symptoms (P=.002), systemic ventricular dysfunction (P<.001), and subpulmonary ventricular dysfunction (P=.03) with an AUC 0.83 comparable to ZAHARA (P=.66). CARPREG I and ZAHARA scores underestimate cardiac risk for lower risk pregnancies in these women. Of the three risk schemes, CARPREG I performed least well in predictive capacity. Clinical factors specific to the population studied are comparable to stratification schemes.