The Impact of Dominant Ventricular Morphology on the Early Postoperative Course After the Glenn Procedure.

The dominant ventricular morphology affects both the early and late outcomes of the Fontan procedure, but its impact on the patients' status immediately following the Glenn procedure is unknown. This study aims to evaluate the effect of the infants' dominant ventricular morphology on the immediate course after undergoing the Glenn procedure. This single-center, retrospective study included all patients who underwent the Glenn procedure between October 2003 and May 2016. The patients were divided into two groups according to their dominant ventricular morphology. Their postoperative records were reviewed and compared. Out of the 89 patients who underwent the Glenn procedure during the study period, 40 (44.9%) had dominant right ventricular morphology and 49 (55.1%) had left ventricular morphology. There were no significant group differences in baseline characteristics or operative data. The maximal postoperative vasoactive-inotropic score was significantly higher and the extent of ventricular dysfunction was significantly more severe in the dominant right ventricle group (P < 0.05). The length of hospitalization was slightly but not significantly longer in the hypoplastic LV group. It is concluded that patients with a dominant LV morphology had a superior ventricular function and required less inotropic support compared to that of a dominant RV morphology in the immediate postoperative course following the Glenn procedure. Survival was not affected by these differences. Further study to determine the pathophysiologic basis for these differences is warranted.

Evaluating the response to cardiac resynchronization therapy performed with a new ventricular morphology-based strategy for congenital heart disease.

BACKGROUND: In cardiac resynchronization therapy (CRT) for patients with congenital heart disease (CHD) and a ventricular morphology other than a systemic left ventricle (LV), we previously proposed pacing sites that are different from those used for a systemic LV. The leads should be placed laterally on opposite sides of both ventricles in patients with short-axis dyssynchrony and a single ventricular physiology with two ventricles, whereas they should be placed at the farthest sites along the longitudinal direction in the right ventricle (RV) in patients with long-axis dyssynchrony of the RV. Moreover, in patients with interventricular dyssynchrony and a biventricular physiology with a systemic RV, they should be placed at sites that both ventricles can contract simultaneously. We retrospectively investigated 27 consecutive procedures in 24 patients with CHD who underwent CRT to evaluate the effectiveness of a new ventricular morphology-based CRT strategy. The responder rate was 63% (17/27). The reasons for a non-response to CRT in 10 cases were as follows: non-optimal lead positions during CRT, 4; no systemic ventricular conduction delay or heart failure symptoms before the CRT, 5; short follow-up periods after the CRT, 2; and an extremely dilated systemic RV, 1. The responder rate became 88% (14/16), after excluding the procedures without a ventricular conduction delay or heart failure symptoms and those with non-optimal lead positions. This new strategy for CRT can provide favorable results for CHD patients with a systemic ventricular conduction delay and heart failure.

Biventricular morphology in adults born with a ventricular septal defect.

Ventricular septal defects - large, surgically closed or small, untreated - have demonstrated lower peak exercise capacity compared with healthy controls. The mechanisms behind these findings are not yet fully understood. Therefore, we evaluated biventricular morphology in adults with a ventricular septal defect using MRI. Adults with either childhood surgically closed or small, untreated ventricular septal defects and healthy controls underwent cine MRI for the evaluation of biventricular volumes and quantitative flow scans for measurement of stroke index. Scans were analysed post hoc in a blinded manner. In total, 20 operated patients (22±2 years) and 20 healthy controls (23±2 years) were included, along with 32 patients with small, unrepaired ventricular septal defects (26±6 years) and 28 controls (27±5 years). Operated patients demonstrated larger right ventricular end-diastolic volume index (103±20 ml/m2) compared with their controls (88±16 ml/m2), p=0.01. Heart rate and right ventricular stroke index did not differ between operated patients and controls. Patients with unrepaired ventricular septal defects revealed larger right ventricular end-diastolic volume index (105±17 ml/m2) compared with their controls (88±13 ml/m2), p<0.01. Furthermore, right ventricular stroke index was higher in unrepaired ventricular septal defects (53±12 ml/minute/m2) compared with controls (46±8 ml/minute/m2), p=0.02, with similar heart rates. Both patient groups' right ventricles were visually characterised by abundant coarse trabeculation. Positive correlations were demonstrated between right ventricular end-diastolic volume indices and peak exercise capacity in patients. Left ventricle measurements displayed no differences between groups. In conclusion, altered right ventricular morphology was demonstrated in adults 20 years after surgical ventricular septal defect repair and in adults with small, untreated ventricular septal defects.

Cardiovascular magnetic resonance demonstration of the spectrum of morphological phenotypes and patterns of myocardial scarring in Anderson-Fabry disease.

BACKGROUND: Although it is known that Anderson-Fabry Disease (AFD) can mimic the morphologic manifestations of hypertrophic cardiomyopathy (HCM) on echocardiography, there is a lack of cardiovascular magnetic resonance (CMR) literature on this. There is limited information in the published literature on the distribution of myocardial fibrosis in patients with AFD, with scar reported principally in the basal inferolateral midwall. METHODS: All patients with confirmed AFD undergoing CMR at our center were included. Left ventricular (LV) volumes, wall thicknesses and scar were analyzed offline. Patients were categorized into 4 groups: (1) no wall thickening; (2) concentric hypertrophy; (3) asymmetric septal hypertrophy (ASH); and (4) apical hypertrophy. Charts were reviewed for clinical information. RESULTS: Thirty-nine patients were included (20 males [51%], median age 45.2 years [range 22.3-64.4]). Almost half (17/39) had concentric wall thickening. Almost half (17/39) had pathologic LV scar; three quarters of these (13/17) had typical inferolateral midwall scar. A quarter (9/39) had both concentric wall thickening and typical inferolateral scar. A subgroup with ASH and apical hypertrophy (n = 5) had greater maximum wall thickness, total LV scar, apical scar and mid-ventricular scar than those with concentric hypertrophy (n = 17, p < 0.05). Patients with elevated LVMI had more overall arrhythmia (p = 0.007) more ventricular arrhythmia (p = 0.007) and sustained ventricular tachycardia (p = 0.008). CONCLUSIONS: Concentric thickening and inferolateral mid-myocardial scar are the most common manifestations of AFD, but the spectrum includes cases morphologically identical to apical and ASH subtypes of HCM and these have more apical and mid-ventricular LV scar. Significant LVH is associated with ventricular arrhythmia.

Effect on left ventricular mass and geometry in patients with takotsubo cardiomyopathy.

OBJECTIVES: Takotsubo cardiomyopathy (TTC) is a condition of transient left ventricular (LV) dysfunction. The effects on LV mass (LVM) and geometry have not been studied enough in TTC. Retrospectively, we analyzed our TTC cohort both by transthoracic echocardiography (TTE) and magnetic resonance imaging (MRI), for comparative purposes. DESIGN: Thirteen women undergoing TTE and MRI, at onset and three months later, were included. LVM was estimated by MRI, and two TTE methods. Segmental wall thickness (SWT) was measured, while radial strain was assessed by TTE. Data analysis included Wilcoxon's test (between phases), Mann-Whitney U test and McNemar's test (between and within groups). Bland-Altman analyses were used for intertechnique coherence, while interactions regarding TTE were tested using Spearman's coefficient. RESULTS: LVM decreased during recovery (p < 0.05), by MRI and one of the TTE methods; truncated ellipsoid formula (TEF), which also showed relatively better coherence compared with MRI. SWT decreased in two of three sites, by both modalities, but with ambiguous coherence there between. The TEF data interacted partially with a demonstrated increase in radial strain. CONCLUSIONS: TTC associates with acute increase in LVM, which appears to be an apical effect, tending to follow the changes in concentric wall motion. MRI and TTE show adequate coherence; primarily for the TEF method regarding LVM.

Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.

BACKGROUND: Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. METHODS AND RESULTS: This nationwide cohort study included all children undergoing operation with single-ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow-up was 98.7% complete (10 patients emigrated). Mean follow-up was 11.3 years (maximum, 26.7 years). Long-term survival was significantly higher in patients with left ventricular compared with RV dominance (10-year survival: 91.0% [95% CI, 87.3%-93.6%] versus 71.1% [95% CI, 66.4%-75.2%]). RV dominance had a significant impact on outcomes after first-stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology. CONCLUSIONS: This study provides clinically relevant knowledge about the long-term prognosis in patients with different underlying cardiac anomalies undergoing single-ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.

Carbon monoxide affects early cardiac development in an avian model.

INTRODUCTION: Carbon monoxide (CO) is a toxic gas that can be lethal in large doses and may also cause physiological damage in lower doses. Epidemiological studies suggest that CO in lower doses over time may impact on embryo development, in particular cardiac development, however other studies have not observed this association. METHODS: Here, we exposed chick embryos in ovo to CO at three different concentrations (3, 9, 18 ppm) plus air control (4 protocols in total) for the first 9 days of development, at which point we assessed egg and embryo weight, ankle length, developmental stage, heart weight, ventricular wall thickness, ventricular-septal thickness and atrial wall thickness. RESULTS: We found that heart weight was reduced for the low and moderate exposures compared to air, that atrial wall and ventricular wall thickness was increased for the moderate and high exposures compared to air and that ventricular septal thickness was increased for low, moderate and high exposures compared to air. Ventricular wall thickness was also significantly positively correlated with absolute CO exposures across all protocols. CONCLUSIONS: This intervention study thus suggests that CO even at very low levels may have a significant impact on cardiac development.

Ventricular morphology of single-ventricle hearts has a significant impact on outcomes after Fontan palliation: a meta-analysis.

OBJECTIVES: A conclusive interpretation of the role of ventricular dominance in outcomes after Fontan palliation has not been formulated yet. We conducted a systematic review and meta-analysis of scientific literature to give an insight into the impact of ventricular morphology in single-ventricle palliation, focusing on its influence on survival, morbidities, ventricular performance and functional capacity. METHODS: A systematic review of PubMed, Web of Science and Scopus databases was performed. A random-effect meta-analysis was conducted, and survival data were reconstructed using the published Kaplan-Meier survival curves. RESULTS: Twenty-seven studies were selected, for a total of 4529 left-dominant versus 4844 right-dominant patients. Estimated survival at 1, 5, 10, 20 and 30 years of follow-up was 0.99 [95% confidence interval (CI) = 0.98-0.99], 0.95 [95% CI = 0.94-0.96], 0.92 [95% CI = 0.91-0.93], 0.86 [95% CI = 0.84-0.88] and 0.68 [95% CI = 0.65-0.83] for left-dominant patients and 0.94 [95% CI = 0.93-0.95], 0.89 [95% CI = 0.88-0.9], 0.85 [95% CI = 0.83-0.87], 0.69 [95% CI = 0.63-0.75] and 0.59 [95% CI = 0.5-0.69] for right-dominant patients, respectively. Survival was statistically lower for right-dominant patients (P < 0.001), with an hazard ratio for the mortality of 2.38 (2.03-2.80); also, they displayed significantly longer hospital stay, worse ventricular function, larger ventricular volumes and a higher incidence of moderate or severe atrioventricular valve regurgitation when compared to left-dominant patients. CONCLUSIONS: According to our meta-analysis, the morphology of the dominant ventricle has a significant impact on outcomes after Fontan palliation. Right-dominant patients experience an inferior long-term survival when the anatomical right ventricle is included in the systemic circulation.

Dominant Ventricular Morphology and Early Postoperative Course After the Fontan Procedure.

BACKGROUND: Single ventricle heart disease comprises a wide variety of critical heart defects that lead to the provision of systemic cardiac output by one dominant ventricle. It requires staged surgical palliation that culminates in Fontan circulation. Dominant ventricular morphology in single ventricle patients reportedly has an impact on postoperative morbidity and mortality with varying results. The objectives of this study were to examine the association between ventricular morphology and the early postoperative course after the Fontan procedure. METHODS: A retrospective cohort study in a tertiary referral pediatric medical center that included 98 consecutive patients who underwent Fontan procedure between October 2009 and May 2016. Postoperative outcomes were compared between patients with left ventricular morphology and those with right ventricular morphology (crude effect and regression analysis). RESULTS: Patients with right ventricular morphology had longer postoperative hospitalizations compared to patients with left ventricular morphology (26.5 days vs 18.2 days, respectively, P = .028), higher postoperative maximal vasoactive-inotropic scores (25.6 vs 12.4, P = .02), higher serum lactate levels (7.7 mmol/L vs 6.4 mmol/L, P = .03), higher proportions of ventilation throughout 24 h or more (16 patients [38%] vs 8 patients [14%], P = .009), higher proportions of ventricular dysfunction (12 patients [29%] vs 5 patients [9%], P = .0001), and lower blood oxygen saturation levels at discharge (87% vs 92%, P = .03). CONCLUSIONS: The Fontan procedure in patients with right ventricular morphology is associated with longer postoperative hospitalization and worse early postoperative characteristics (ventricular dysfunction and atrioventricular valve regurgitation) as well as higher rates of early, transient signs of sub-optimal postoperative hemodynamics compared to those with left ventricular morphology.

Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden.

Background Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation. Methods and Results This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic-mitral atresia, aortic atresia-mitral stenosis (AA-MS), and aortic-mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob-LV), miniaturized and slit-like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA-MS (35%) was associated with both mortality (all other subtypes versus AA-MS: interstage-I: hazard ratio [HR], 2.7; P=0.006; overall: HR, 2.2; P=0.005) and MAEs (HR, 2.4; P=0.0009). Glob-LV (57%), noticed in all patients with AA-MS, 61% of patients with aortic stenosis-mitral stenosis, and 19% of patients with aortic atresia-mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob-LV: interstage-I: HR, 4.5; P=0.004; overall: HR, 3.4; P=0.0007) and MAEs (HR, 2.7; P=0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (P>0.15). Patients with AA-MS (35%) or Glob-LV (38%) palliated with a Blalock-Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA-MS: HR, 2.6; P=0.04; Glob- LV: HR, 2.1; P=0.03). Conclusions Glob-LV and AA-MS are independent morphological risk factors for adverse short- and long- term outcome, especially if a Blalock-Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.

Quantitation of Poststress Change in Ventricular Morphology Improves Risk Stratification.

Shape index and eccentricity index are measures of left ventricular morphology. Although both measures can be quantified with any stress imaging modality, they are not routinely evaluated during clinical interpretation. We assessed their independent associations with major adverse cardiovascular events (MACE), including measures of poststress change in shape index and eccentricity index. Methods: Patients undergoing SPECT myocardial perfusion imaging between 2009 and 2014 from the Registry of Fast Myocardial Perfusion Imaging with Next-Generation SPECT (REFINE SPECT) were studied. Shape index (ratio between the maximum left ventricular diameter in short axis and ventricular length) and eccentricity index (calculated from orthogonal diameters in short axis and length) were calculated in end-diastole at stress and rest. Multivariable analysis was performed to assess independent associations with MACE (death, nonfatal myocardial infarction, unstable angina, or late revascularization). Results: In total, 14,016 patients with a mean age of 64.3 ± 12.2 y (8,469 [60.4%] male were included. MACE occurred in 2,120 patients during a median follow-up of 4.3 y (interquartile range, 3.4-5.7). Rest, stress, and poststress change in shape and eccentricity indices were associated with MACE in unadjusted analyses (all P < 0.001). However, in multivariable models, only poststress change in shape index (adjusted hazard ratio, 1.38; P < 0.001) and eccentricity index (adjusted hazard ratio, 0.80; P = 0.033) remained associated with MACE. Conclusion: Two novel measures, poststress change in shape index and eccentricity index, were independently associated with MACE and improved risk estimation. Changes in ventricular morphology have important prognostic utility and should be included in patient risk estimation after SPECT myocardial perfusion imaging.

The Impact of Multimorbidity Burden, Frailty Risk Scoring, and 3-Directional Morphological Indices vs. Testing for CSF Responsiveness in Normal Pressure Hydrocephalus.

Objective: Multimorbidity burden across disease cohorts and variations in clinico-radiographic presentations within normal pressure hydrocephalus (NPH) confound its diagnosis, and the assessment of its amenability to interventions. We hypothesized that novel imaging techniques such as 3-directional linear morphological indices could help in distinguishing between hydrocephalus vs. non-hydrocephalus and correlate with responsiveness to external lumbar drainage (CSF responsiveness) within NPH subtypes. Methodology: Twenty-one participants with NPH were recruited and age-matched to 21 patients with Alzheimer's Disease (AD) and 21 healthy controls (HC) selected from the Alzheimer's Disease Neuroimaging Initiative (ADNI) database. Patients with NPH underwent testing via the NPH programme with external lumbar drainage (ELD); pre- and post-ELD MRI scans were obtained. The modified Frailty Index (mFI-11) was used to stratify the NPH cohort, including Classic and Complex subtypes, by their comorbidity and frailty risks. The quantitative imaging network tool 3D Slicer was used to derive traditional 2-dimensional (2d) linear measures; Evans Index (EI), Bicaudate Index (BCI) and Callosal Angle (CA), along with novel 3-directional (3d) linear measures; z-Evans Index and Brain per Ventricle Ratio (BVR). 3-Dimensional (3D) ventricular volumetry was performed as an independent correlate of ventriculomegaly to CSF responsiveness. Results: Mean age for study participants was 71.14 ± 6.3 years (18, 85.7% males). The majority (15/21, 71.4%) of participants with NPH comprised the Complex subtype (overlay from vascular risk burden and AD); 12/21 (57.1%) were Non-Responders to ELD. Frailty alone was insufficient in distinguishing between NPH subtypes. By contrast, 3d linear measures distinguished NPH from both AD and HC cohorts, but also correlated to CSF responsiveness. The z-Evans Index was the most sensitive volumetric measure of CSF responsiveness (p = 0.012). Changes in 3d morphological indices across timepoints distinguished between Responders vs. Non-Responders to lumbar testing. There was a significant reduction of indices, only in Non-Responders and across multiple measures (z-Evans Index; p = 0.001, BVR at PC; p = 0.024). This was due to a significant decrease in ventricular measurement (p = 0.005) that correlated to independent 3D volumetry (p = 0.008). Conclusion. In the context of multimorbidity burden, frailty risks and overlay from neurodegenerative disease, 3d morphological indices demonstrated utility in distinguishing hydrocephalus vs. non-hydrocephalus and degree of CSF responsiveness. Further work may support the characterization of patients with Complex NPH who would best benefit from the risks of interventions.

The presence of an additional ventricular chamber does not change the outcome of Fontan circulation: a comparative study.

OBJECTIVES: The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS: We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS: A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4-20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively). CONCLUSIONS: A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle.

The anatomic substrates for outflow tract arrhythmias.

Much has been learned regarding the cellular and molecular mechanisms involved in producing idiopathic ventricular tachycardias originating from the ventricular outflow tract. Questions remain, however, regarding their genesis. In part, this reflects the uncertainty regarding the anatomic arrangement of their myocardial components. This is exacerbated by the lack of consensus regarding the appropriate terms to describe their components, as shown by the current use of "cusp," used interchangeably to describe either the valvar sinuses or the leaflets of the arterial valves. Such uncertainty can be resolved only by providing precise definitions of leaflets, sinuses, and the sinutubular junction. Further problems are created when the arterial roots are described in an attitudinally incorrect manner. Current techniques reveal the location of the cardiac components as they are positioned within the thorax. In this review, we discuss these potential difficulties, putting them into the context of the anatomy of the ventricular outflow tracts as revealed by virtual dissection of computed tomographic datasets. We provide a brief account of cardiac development, suggesting that such evidence can provide vital clues to the potential origins of arrhythmias originating from the myocardial components of the ventricular outflow tracts.

Changes in left ventricular shape and morphology in the presence of heart failure: a four-dimensional quantitative and qualitative analysis.

PURPOSE: The presence and progression of heart failure (HF) are associated with cardiac remodelling, defined as cellular, molecular and interstitial changes which occur after injury and manifest as changes in left ventricular (LV) size, mass, geometry and function. This research study was designed to investigate the changes to LV morphology and shape which occur in the presence of heart failure using three-dimensional (3D) modelling and analysis of cardiac-gated CT scans from both healthy individuals and patients classified with HF. METHODS: A number of quantitative and qualitative strategies were applied to cardiac CT scans of HF patients and healthy controls (n = 7) in order to analyse changes to LV size, shape and structure and to examine LV remodelling in the different classes of HF. Three-dimensional wireframe representations of endocardial and epicardial borders were created, three-dimensional computer stereolithography models of the inner LV cavity and myocardial wall segments were generated and three-dimensionally printed and a number of clinical LV dimension and shape indices were measured. All data were analysed using one-way ANOVA with post hoc Tukey method for multiple comparisons for significant variables. RESULTS: Results of most significance included abnormalities in LV mass and end-systolic dimensions and significantly increased septal wall thickness among mid-range ejection fraction cases. Also of importance were significant increases in both dimension-based and volumetric sphericity index measures in all HF cases. Three-dimensional printed models provided qualitative information as to changes in inner LV cavity and myocardial wall morphology across the cardiac cycle for healthy and HF cases and validated quantitative findings. CONCLUSION: Findings from this study can successfully be applied to motivate the research and development of new HF treatment strategies and devices as well as for the development of a realistic cardiac simulator system.

Influence of various therapeutic strategies on right ventricular morphology, function and hemodynamics in pulmonary arterial hypertension.

BACKGROUND: In idiopathic pulmonary arterial hypertension (IPAH) treatment goals include improving right ventricular (RV) function, hemodynamics and symptoms to move patients to a low-risk category for adverse clinical outcomes. No data are available on the effect of upfront combination therapy on RV improvement as compared with monotherapy. The aim of this study was to evaluate echocardiographic RV morphology and function in patients affected by IPAH and treated with different strategies. METHODS: Sixty-nine consecutive, treatment-naive IPAH patients treated with first-line upfront combination therapy at 10 centers were retrospectively evaluated and compared with 2 matched cohorts treated with monotherapy after short-term follow-up. Evaluation included clinical, hemodynamic and echocardiographic parameters. RESULTS: At 155 ± 65 days after baseline evaluation, patients in the oral+prostanoid group (Group 1) had the most clinical and hemodynamic improvement compared with the double oral group (Group 2), the oral monotherapy group (Group 3) and the prostanoid monotherapy group (Group 4). The more extensive reduction of pulmonary vascular resistance in Groups 1, 2 and 4 was associated with significant improvement in all RV echocardiographic parameters compared with Group 3. Considering the number of patients who reached the target goals suggested by established guidelines, 8 of 27 (29.6%) and 7 of 42 (16.7%) patients in Groups 1 and 2, respectively, achieved low-risk status, as compared with 2 of 69 (2.8%) and 6 of 27 (22.2%) in Groups 3 and 4, respectively. CONCLUSIONS: In advanced treatment-naive IPAH patients, an upfront combination therapy strategy seems to significantly improve hemodynamics and RV morphology and function compared with oral monotherapy. The most significant results seem to be achieved with prostanoids plus oral drug, whereas the use of the double oral combination and prostanoids as monotherapy seem to produce similar results.

Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology.

Objective: Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV). Methods: All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX. Results: Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6). Conclusion: SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.

Endoscopic third ventriculostomy and choroid plexus cauterization with a rigid neuroendoscope in infants with hydrocephalus.

OBJECT Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been shown to improve the success rate compared with ETV alone in infants (less than 24 months) with hydrocephalus who were treated in developing countries. The authors sought to evaluate the safety and efficacy of this procedure, using a rigid neuroendoscope, in a single North American center, and to assess whether the ETV success score (ETVSS), the CURE Children's Hospital of Uganda ETVSS (CCHU ETVSS), and other pre- and intraoperative variables could predict success. METHODS The authors performed a retrospective review of consecutive ETV/CPC procedures performed using a rigid neuroendoscope to treat infantile hydrocephalus. The infants underwent the procedure at Miami Children's Hospital between January 2007 and 2014, with at least one postoperative follow-up. Duration of follow-up or time to failure of ETV/CPC, the primary outcome measure, was documented. A repeat CSF diversion procedure or death was considered as a failure of ETV/CPC. The time to event was measured using a Kaplan-Meier analysis. The authors analyzed ETVSS, CCHU ETVSS, and pre- and intraoperative variables to determine their suitability to predict success. RESULTS Eighty-five patients (45 boys) with a mean age of 4.3 months (range 1 day to 20 months) underwent ETV/CPC. Etiology included intraventricular hemorrhage of prematurity in 44 patients (51.7%), myelomeningocele (MMC) in 7 (8.2%), congenital aqueductal stenosis in 12 (14.1%), congenital communicating hydrocephalus in 6 (7.1%), Dandy-Walker complex in 6 (7.1%), postinfectious hydrocephalus in 5 (5.8%), and other cause in 5 (5.8%). Six procedure-related complications occurred in 5 (5.8%) patients, including 2 hygromas, 1 CSF leak, and 3 infections. There were 3 mortalities in this cohort. ETV/CPC was successful in 42.1%, 37.7%, and 36.8% of patients at 6, 12, and 24 months follow-up, respectively. The median (95% CI) time to ETV/CPC failure was 4.0 months (0.9-7.1 months). In univariate analyses, both the ETVSS (hazard ratio [HR] 1.03; 95% CI 1.01-1.05; p = 0.004) and CCHU ETVSS (HR 1.48; 95% CI 1.04-2.09; p = .028) were predictive of outcome following ETV/CPC. In multivariate analysis, the presence of prepontine scarring was associated with ETV/CPC failure (HR 0.34; 95% CI 0.19-0.63; p < 0.001). Other variables, such as radiological criteria (prepontine interval, prepontine space, aqueductal stenosis, Third Ventricular Morphology Index) and intraoperative findings (ventriculostomy pulsations, extent of CPC), did not predict outcome. CONCLUSIONS ETV/CPC is a feasible alternative to ETV and ventriculoperitoneal shunt in infants with hydrocephalus. Both the ETVSS and CCHU ETVSS predicted success following ETV/CPC in this single-center North American cohort of patients.