Pulmonary Regurgitation Research Articles

In vitro bench testing using patient-specific 3D models for percutaneous pulmonary valve implantation with Venus P-valve.

Due to the wide variety of morphology, size, and dynamics, selecting an optimal valve size and location poses great difficulty in percutaneous pulmonary valve implantation (PPVI). This study aimed to report our experience with in vitro bench testing using patient-specific three-dimensional (3D)-printed models for planning PPVI with the Venus P-valve. Patient-specific 3D soft models were generated using PolyJet printing with a compliant synthetic material in 15 patients scheduled to undergo PPVI between July 2018 and July 2020 in Central China Fuwai Hospital of Zhengzhou University. 3D model bench testing altered treatment strategy in all patients (100%). One patient was referred for surgery because testing revealed that even the largest Venus P-valve would not anchor properly. In the remaining 14 patients, valve size and/or implantation location was altered to avoid valve migration and/or compression coronary artery. In four patients, it was decided to change the point anchoring because of inverted cone-shaped right ventricular outflow tract (RVOT) ( n = 2) or risk of compression coronary artery ( n = 2). Concerning sizing, we found that an oversize of 2-5 mm suffices. Anchoring of the valve was dictated by the flaring of the in- and outflow portion in the pulmonary artery. PPVI was successful in all 14 patients (absence of valve migration, no coronary compression, and none-to-mild residual pulmonary regurgitation [PR]). The diameter of the Venus P-valve in the 3D simulation group was significantly smaller than that of the conventional planning group (36 [2] vs. 32 [4], Z = -3.77, P <0.001). In vitro testing indicated no need to oversize the Venus P-valve to the degree recommended by the balloon-sizing technique, as 2-5 mm sufficed.

Physician-Modified Endograft-Facilitated Transcatheter Pulmonary Valve Replacement in Large Right Ventricular Outflow Tract.

Transcatheter pulmonary valve replacement (TPVR) in patients with a congenital or acquired abnormality resulting in enlarged right ventricular outflow tract (RVOT) is challenging and may preclude treatment with dedicated devices. We describe a technique using a physician-modified endograft to facilitate TPVR. Six patients underwent physician-modified endograft-facilitated TPVR for severe symptomatic pulmonary insufficiency with enlarged RVOT. The fenestration was created in a commercially available endograft before implantation, which was then deployed from the dominant branch pulmonary artery into the RVOT, with the fenestration aligned with the ostium of the nondominant pulmonary artery. A covered stent was placed through the fenestration into the nondominant branch pulmonary artery, and a transcatheter heart valve was deployed within the endograft at the level of the original pulmonary valve. Four patients had tetralogy of Fallot, 1 had pulmonary atresia, and 1 had rheumatic valve disease. The RVOT/main pulmonary artery was severely enlarged (diameter, 44.2 [43.5-50.6] mm). All patients had reduced right ventricular (RV) function and dilated RVs (RV end-diastolic volume, 314 [235-316] mL). Successful endograft, covered stent, and transcatheter heart valve deployment were achieved in all cases without stent/valve embolization, vascular complications, or bleeding complications. At 30 days, 1 patient had mild pulmonary insufficiency, while others had none. The RV size measured by echocardiography was significantly reduced after TPVR (RV area, 34.4 [baseline] versus 29.0 [pre-discharge] versus 25.3 [30 days] cm2; P=0.03). During median follow-up of 221.5 (range, 29-652) days, there were no deaths or need for pulmonary valve reintervention. One patient developed severe tricuspid regurgitation due to entrapment of the anterior tricuspid leaflet by the endograft. The patient underwent successful tricuspid replacement and resection of the offending endograft with preservation of the pulmonary valve prosthesis. Simple fenestration of an off-the-shelf endograft and associated covered stent placement through the fenestration allows TPVR for patients with dysfunctional native or patch-repaired pulmonary valves and RVOT enlargement.

Some results of riamilovir (triazavirine) usage in medical staff for prevention and treatment of COVID-19

Riamilovir, a drug registered in Russia (chemical formula: methylthionitrooxodihydrotriazolotriazinide sodium, trade name: Triazavirin) is a synthetic analogue of guanine and a drug of direct antiviral action. Currently, there are conflicting data regarding usage of riamilovir as a preventive and therapeutic agent in coronavirus infections. The purpose of this study was to analyze some results of riamilovir usage, both for prevention of the SARS-CoV-2 infection and for the treatment of COVID-19 during the first wave of the new coronavirus pandemic.&#x0D; The analysis was based on a survey of 62 medical staff workers at a single medical institution in Ekaterinburg who was ill with COVID-19, being divided into 4 groups: (1) those who did not receive riamilovir (control), (2) persons who received riamilovir only as a disease prevention, (3) subjects who received the drug as a therapeutic agent, (4) those who received riamilovir before and during the disease.&#x0D; The data concerning usage of riamilovir for the prevention of infection with the SARS-CoV-2 virus have shown the following consequences: increased duration of hospitalization, an increased incidence of COVID-19 complications, i.e., fever, shortness of breath, pulmonary insufficiency, pneumonia, higher frequency of neurological disorders, which were not reported elsewhere. Severe clinical course of the disease was observed much more often in cases of prophylactic riamilovir administration, and the rehabilitation period was incomplete 2 months after the disease. Clinical symptoms of muscle and joint pain were documented at later terms in all persons who received riamilovir to prevent a new coronavirus infection. Usage of riamilovir for therapeutic purposes made it possible to avoid the development of pulmonary insufficiency, severe course of the infectious disease, and entirely restore the state of health.&#x0D; The study did not reveal the usefulness of riamilovir for prevention of COVID-19 complications when taking the drug before infection with the SARS-CoV-2 virus. However, the use of riamilovir for therapeutic purposes prevents development of severe clinical cases and is associated with 4-fold reduced risk of pain in muscles, joints, and spine among the COVID-19 patients.

Unmet Clinical Needs for Transcatheter Pulmonary Valves.

A common feature of congenital heart disease is the presence of right ventricular outflow tract (RVOT) obstruction that can range from mild to severe and can lead to atresia of the pulmonary valve, in extreme conditions. RVOT abnormalities can frequently be corrected surgically or via interventional means. However, most of these patients will ultimately develop pulmonary valve insufficiency and eventual right ventricular dilation, which will require a pulmonary valve replacement at some point in their life to mitigate the detrimental effects of pulmonary valve regurgitation (PVR) on the right ventricle (RV). The evolution from the studies done by Philip Bonhoeffer to implant a pulmonary valve via transcatheter means, have provided a bedrock for transcatheter pulmonary valve replacement (TPVR). Yet, several areas of unmet need for a demographic of patients still exist. Here, we discuss the clinical unmet needs in children under 20 Kg and expand the use of hybrid and other TPVR approaches along with the current indications and contraindications for pulmonary valve replacement. The constraints and limitations from commercially available pulmonary valves will be discussed from a clinical standpoint. Finally, we explore the use of hybrid and periventricular delivery of transcatheter pulmonary valves in younger patients.

Diagnostic value of two-dimensional echocardiographic measurements of the pulmonary artery diameter and the pulmonary artery distensibility index to detect pulmonary hypertension in horses

Introduction/objectivesEchocardiographic diagnosis of pulmonary hypertension (PH) in horses is usually based on Doppler interrogation of tricuspid (TR) or pulmonic regurgitation (PR). This study aimed at investigating two-dimensional echocardiographic (2DE) variables, including pulmonary artery diameter (PAD) and pulmonary artery distensibility index (PADI), to diagnose PH. Animals, materials and methods41 healthy horses; 82 horses with TR or PR and normal intracardiac pressure gradients; and 35 horses with TR or PR velocities indicating PH. The 2DE variables were compared between groups, cut-offs for the diagnosis of PH were determined, and conditional inference trees served to identify the variable best predicting PH in the absence of TR or PR. ResultsHorses with PH had larger end-diastolic cross-sectional (short-axis) PAD (PADed-sx) (5.8 ± 1.0 cm; mean ± SD) and smaller PADI[/PADps] (15.4 ± 7.7%) than healthy horses (5.0 ± 0.6 cm, P<0.0001; 20.5 ± 4.4%, P<0.001) and horses with TR or PR but no PH (5.3 ± 0.6 cm, P=0.003; 18.8 ± 4.2%, P=0.005). PADed-sx predicted PH with sensitivity (Se) = 36% and specificity (Sp) = 94% (cut-off 6.0 cm, AUC = 0.668, P=0.002), while PADI[/PADps] predicted PH with Se = 43% and Sp = 92% (cut-off 13.7%, AUC = 0.662, P=0.004). In the absence of TR, PADed-sx and the end-diastolic long-axis aortic diameter-to-PADed-sx ratio (AoDed-lx/PADed-sx) were most suitable to diagnose PH. ConclusionPulmonary artery crosssectional (short-axis) diameter at end-diastole measured in a right-parasternal long axis view of the left ventricular outflow tract and Pulmonary artery distensibility index were moderately specific but not very sensitive to diagnosing PH. Nonetheless, they may be used as complementary indices suggesting PH in the absence of TR or PR.

Echocardiographic parameters recommended for assessing the severity of tricuspid regurgitation: concordance and discordance.

Current guidelines recommend integrating several echocardiographic indices to evaluate the severity of tricuspid regurgitation (TR). Discordance of indices, including qualitative and quantitative methods, commonly exists in practice. The discordance among these parameters has not yet been fully elucidated. A total of 127 patients with recognizable TR jets without pulmonary regurgitation or intracardiac shunt were prospectively enrolled. We evaluated 8 parameters by 2-dimensional (2D) echocardiography: proximal iso-velocity surface area (PISA)-derived regurgitant volume (RVol), PISA-derived effective regurgitant orifice area (EROA), PISA radius, vena contracta width (VCW), color Doppler jet area, tricuspid valve annular diameter, inferior vena cava (IVC) diameter, and peak E wave. According to current guidelines, each echocardiographic parameter was determined to represent either severe or non-severe TR. A concordance score was calculated as the number of concordant parameters divided by 8, with a higher score reflecting better concordance. Data were further categorized into 3 subgroups: complete concordance (0 discordant parameters), high concordance (1-2 discordant parameters), and low concordance (3-4 discordant parameters). The mean concordance score was 81%±17% for the entire cohort. There were 48 (38%) patients with complete concordance, including 6 patients with severe TR. In contrast, the low concordance group (n=43, 34%) mostly comprised severe TR patients (36 patients). When considering only EROA, RVol, and VCW, concordance improved, with 98 patients (77%) in complete agreement. Concordance seems limited when using echocardiographic parameters to assess TR severity. Applying only EROA, RVol, and VCW results in better concordance, as recommended by the current guidelines.

Rationale and Design of the Multicenter Catheter Ablation of Ventricular Tachycardia Before Transcatheter Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Study

Patients with repaired tetralogy of Fallot are at elevated risk for ventricular arrhythmia and sudden cardiac death. Over the past decade, the pathogenesis and natural history of ventricular tachycardia has become increasingly understood, and catheter ablation has emerged as an effective treatment modality. Concurrently, there has been great progress in the development of a versatile array of transcatheter valves that can be placed in the native right ventricular outflow tract for the treatment of long-standing pulmonary regurgitation. Although such valve platforms may eliminate the need for repeat cardiac operations, they may also impede catheter access to the myocardial substrates responsible for sustained macro-reentrant ventricular tachycardia. This manuscript provides the rationale and design of a recently devised multicenter study that will examine the clinical outcomes of a uniform, preemptive strategy to eliminate ventricular tachycardia substrates before transcatheter pulmonary valve implantation in patients with tetralogy of Fallot.

Comparison and Validity of Fragmented QRS (fQRS) Against Cardiac Magnetic Resonance (CMR) in Predicting Right Heart Function in Tetralogy of Fallot Patients After Reconstructive Surgery

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Patients with repaired TOF (rTOF) expose to moderate to severe pulmonary regurgitation (PR), right ventricular (RV) dysfunction, and RV failure. Although cardiac magnetic resonance (CMR) provides details of RV function and structure, an electrocardiogram (ECG) is accessible and inexpensive. Objectives: We aimed to assess the correlation between clinical symptoms and ECG parameters with CMR findings. Methods: This cross-sectional study assessed the patients with rTOF who underwent CMR between May 2020 and September 2022 in Imam Reza Hospital. The signs and symptoms of rTOF were assessed, and a pediatric cardiologist interpreted ECG parameters. We considered CMR findings, clinical symptoms, and ECG parameters with CMR findings in rTOF. In addition, fragmented QRS (fQRS), the additional spikes within the QRS complex, was used to indicate conduction disturbance due to myocardial scar or fibrosis. Data were analyzed by SPSS version 18. Results: Among 42 patients with rTOF, 20 patients were male. Although the QT correction (QTc) had a significant correlation with CMR parameters, fQRS showed a positive correlation with right ventricular ejection fraction (RVEF) less than 47% (OR 2.06, 95% CI 1.45 - 2.93; P-value = 0.005), right ventricular end-diastolic volume (RVEDV) more than 150 mL/m2 (OR 4.4, 95% CI 2.03 - 9.5; P-value = 0), and right ventricular end-systolic volume (RVESV) more than 80 mL/m2 (OR 2.83, 95% CI 1.59 - 5.04; P-value = 0). We found a murmur in the clinical examination of 64.3% of patients. Our results showed that tachycardia and tachypnea were the most common signs in these patients. Surgery at a young age was significantly correlated to lower ascending aortic volume, right pulmonary artery size, left pulmonary artery size, right atrium and left atrium volume, and main pulmonary artery flow. Conclusions: Compared to other ECG parameters, fQRS could strongly predict RVESV, RVEDV, and RVEF as the right heart indexes. Besides, repair surgery at a younger age reduces the possibility of needing pulmonary valve replacement surgery at older ages. Therefore, clinicians should consider these issues in patients.

Long-Term Postnatal Follow-Up in Monochorionic TTTS Twin Pregnancies Treated with Fetoscopic Laser Surgery and Complicated by Right Ventricular Outflow Tract Anomalies.

Right ventricular outflow tract anomalies (RVOTAs), such as pulmonary stenosis (PS), pulmonary atresia (PA), and pulmonary insufficiency (PI), are typical cardiac anomalies in monochorionic twins, and they are complicated by twin-to-twin transfusion syndrome (TTTS). The aim of this study was to conduct a long-term postnatal cardiological evaluation of prenatal RVOTAs in monochorionic diamniotic twin pregnancies complicated by TTTS and treated with fetoscopic laser surgery (FLS) and to analyze possible prenatal predictors of congenital heart disease (CHD). Prenatal RVOTAs were retrospectively retrieved from all TTTS cases treated with FLS in our unit between 2009 and 2019. Twenty-eight prenatal cases of RVOTAs (16 PI, 10 PS, 2 PA) were observed out of 335 cases of TTTS. Four cases did not reach the postnatal period. CHD was present in 17 of the remaining 24 cases (70.8%), with 10 being severe (58.8%; 10/17); nine cases of PS required balloon valvuloplasty, and one case required biventricular non-compaction cardiomyopathy. The risk of major CHD increased with prenatal evidence of PS and decreased with the gestational age at the time of TTTS and with the prenatal normalization of blood flow across the pulmonary valve. Despite treatment with FLS, the majority of monochorionic diamniotic twin pregnancies complicated by TTTS with prenatal RVOTAs had CHD at long-term follow-up.

Artificial intelligence-based model to classify cardiac functions from chest radiographs: a multi-institutional, retrospective model development and validation study

Chest radiography is a common and widely available examination. Although cardiovascular structures-such as cardiac shadows and vessels-are visible on chest radiographs, the ability of these radiographs to estimate cardiac function and valvular disease is poorly understood. Using datasets from multiple institutions, we aimed to develop and validate a deep-learning model to simultaneously detect valvular disease and cardiac functions from chest radiographs. In this model development and validation study, we trained, validated, and externally tested a deep learning-based model to classify left ventricular ejection fraction, tricuspid regurgitant velocity, mitral regurgitation, aortic stenosis, aortic regurgitation, mitral stenosis, tricuspid regurgitation, pulmonary regurgitation, and inferior vena cava dilation from chest radiographs. The chest radiographs and associated echocardiograms were collected from four institutions between April 1, 2013, and Dec 31, 2021: we used data from three sites (Osaka Metropolitan University Hospital, Osaka, Japan; Habikino Medical Center, Habikino, Japan; and Morimoto Hospital, Osaka, Japan) for training, validation, and internal testing, and data from one site (Kashiwara Municipal Hospital, Kashiwara, Japan) for external testing. We evaluated the area under the receiver operating characteristic curve (AUC), sensitivity, specificity, and accuracy. We included 22 551 radiographs associated with 22 551 echocardiograms obtained from 16 946 patients. The external test dataset featured 3311 radiographs from 2617 patients with a mean age of 72 years [SD 15], of whom 49·8% were male and 50·2% were female. The AUCs, accuracy, sensitivity, and specificity for this dataset were 0·92 (95% CI 0·90-0·95), 86% (85-87), 82% (75-87), and 86% (85-88) for classifying the left ventricular ejection fraction at a 40% cutoff, 0·85 (0·83-0·87), 75% (73-76), 83% (80-87), and 73% (71-75) for classifying the tricuspid regurgitant velocity at a 2·8 m/s cutoff, 0·89 (0·86-0·92), 85% (84-86), 82% (76-87), and 85% (84-86) for classifying mitral regurgitation at the none-mild versus moderate-severe cutoff, 0·83 (0·78-0·88), 73% (71-74), 79% (69-87), and 72% (71-74) for classifying aortic stenosis, 0·83 (0·79-0·87), 68% (67-70), 88% (81-92), and 67% (66-69) for classifying aortic regurgitation, 0·86 (0·67-1·00), 90% (89-91), 83% (36-100), and 90% (89-91) for classifying mitral stenosis, 0·92 (0·89-0·94), 83% (82-85), 87% (83-91), and 83% (82-84) for classifying tricuspid regurgitation, 0·86 (0·82-0·90), 69% (68-71), 91% (84-95), and 68% (67-70) for classifying pulmonary regurgitation, and 0·85 (0·81-0·89), 86% (85-88), 73% (65-81), and 87% (86-88) for classifying inferior vena cava dilation. The deep learning-based model can accurately classify cardiac functions and valvular heart diseases using information from digital chest radiographs. This model can classify values typically obtained from echocardiography in a fraction of the time, with low system requirements and the potential to be continuously available in areas where echocardiography specialists are scarce or absent. None.

Comprehensive echocardiographic assessment of right ventricular function, pulmonary arterial elastic properties and ventricular-vascular coupling in adult patients with repaired tetralogy of fallot: clinical significance of 3D derived indices.

We aimed to comprehensively analyze by three-dimensional speckle-tracking echocardiography (3DSTE) and Doppler echocardiography right ventricular (RV) performance, pulmonary arterial (PA) elastic properties and right ventricular-pulmonary artery coupling (RVPAC) in patients with repaired tetralogy of Fallot (rTOF) and assess the feasibility and clinical utility of related echocardiographic indices. Twenty-four adult patients with rTOF and twenty-four controls were studied. RV end-diastolic volume(3D-RVEDV), RV end-systolic volume(3D-RVESV), RV ejection fraction(3D-RVEF), RV longitudinal strain(3D-RVLS) and RV area strain(3D-RVAS) were calculated by 3DSTE. RV end-systolic area (RVESA) was obtained by planimetry. Pulmonary regurgitation (PR) was assessed as trivial/mild or significant by cardiac magnetic resonance (CMR) and color-Doppler. Pulmonary artery (PA) elastic properties were determined using two-dimensional/Doppler echocardiography. RV systolic pressure (RVSP) was measured using standard Doppler methods. RVPAC was assessed using various 3DSTE-derived parameters (3DRVAS/RVSP, 3DRVLS/RVESA, 3DRVAS/RVESV). Overall, 3DRVEF and 3DRVAS were impaired in rTOF patients compared with controls. PA pulsatility and capacitance were reduced (p = 0.003) and PA elastance was higher (p = 0.0007) compared to controls. PA elastance had a positive correlation with 3DRVEDV (r = 0.64, p = 0.002) and 3DRVAS (r = 0.51, p = 0.02). By ROC (receiver operating characteristics) analysis, 3DRVAS/RVESV, 3DRVAS/RVSP and 3DRVLS/RVESA cutoff values of 0.31%/mmHg, 0.57%/mmHg and 0.86%/mmHg, respectively, had 91%, 88% and 88% sensitivity and 81%, 81% and 79% specificity in identifying exercise capacity impairment. In rTOF patients increased 3DSTE-derived RV volumes and impaired RV ejection fraction and strain are associated with reduced PA pulsatility and capacitance and increased PA elastance. 3DSTE-derived RVPAC parameters using different afterload-markers are accurate indices of exercise capacity.

The so-called "one-and-a-half" ventricular repair: where are we after 40 years?

The indications, timing, and results of the so-called "one-and-a-half ventricle repair", as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues. We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results. Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis. The so-called "one-and-a-half ventricular repair", which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.

Predictors of Early and Late Mortality after Transcatheter Aortic Valve Implantation: A Multicenter Retrospective Chinese Study

Abstract Background: Patients undergoing TAVR constitute a high-risk population given their comorbidities and out-of-hospital mortality rates remain high despite significant improvements in the overall procedural outcomes. Objectives: The objective of this study was to determine the early and late mortality rates following transfemoral transcatheter aortic valve replacement (TAVR) and identify the risk factors for poor outcomes. Methods: This study population examined patients extracted from 43 publicly funded hospitals in Hong Kong between 2010 and 2019. The study constitutes retrospective analysis of mortality outcomes for severe aortic stenosis patients undergoing TAVR. The primary end points include out-of-hospital 30-day, 1-year, 1–2-year, and 2–5-year mortality rates. Results: A total of 448 patients underwent TAVR and were included into the study. The rates of mortality following TAVR were 1.7%, 3.3%, 1.3%, and 0.22% at 30 days, 1, 1–2, and 2–5 years, respectively. Age and chronic renal failure (CRF) were concluded to be associated with postprocedural mortality. Further analysis of the baseline echocardiographic parameters revealed a higher prevalence of right atrial enlargement (RAE) and tricuspid and pulmonary regurgitation in the deceased subgroup. Conclusion: We report the 30-day, 1-, 1–2-, and 2–5-year all-cause mortality for TAVR of 1.7%, 3.3%, 1.3%, and 0.22% at 30 days. Factors associated with a higher prevalence of mortality include age, CRF, RAE, and tricuspid and pulmonary regurgitation.

Self-Expanding Pulmonary Valves in 53 Patients With Native Repaired Right Ventricular Outflow Tracts.

Self-expanding pulmonary valve grafts have been designed for percutaneous pulmonary valve implantation (PPVI) in patients with native repaired right ventricular (RV) outflow tracts (RVOTs). However, their efficacy, in terms of RV function and graft remodelling remain unclear. Patients with native RVOTs who received Venus P-valve (N=15) or Pulsta valve (N= 38) implants between 2017 and 2022 were enrolled. We collected data on patient characteristics and cardiac catheterization parameters as well as imaging and laboratory data before, immediately after, and 6 to 12 months after PPVI and identified risk factors for RV dysfunction. Valve implantation was successful in 98.1% of patients. The median duration of follow-up was 27.5 months. In the first 6 months after PPVI, all patients exhibited resolution of paradoxical septal motion and a significant reduction (P < 0.05) in RV volume, N-terminal pro-B-type natriuretic peptide levels, and valve eccentricity indices (-3.9%). Normalization of the RV ejection fraction (≥ 50%) was detected in only 9 patients (17.3%) and was independently associated with the RV end-diastolic volume index before PPVI (P= 0.03). Nine patients had residual or recurrent pulmonary regurgitation or paravalvular leak (graded as ≥ mild), which was associated with a larger eccentricity index (> 8%) and subsided by 12 months postimplantation. We identified the risk factors likely to be associated with RV dysfunction and pulmonary regurgitation following PPVI in patients with native repaired RVOTs. RV volume-based patient selection is recommended for PPVI of a self-expanding pulmonary valve, along with monitoring of graft geometry.

Repaired Tetralogy of Fallot Pressure Assessment: Insights from 4D-Flow Pressure Mapping

Tetralogy of Fallot (TOF) is the most prevalent cyanotic congenital heart defect (CHD) that alters normal blood flow through the heart and accounts for 10% of all CHD. Pulmonary stenosis and regurgitation are common in adults who have undergone TOF repair (rTOF) and can impact the load on the right ventricle, blood flow pressure, and pulmonary hemodynamics. Pressure mapping, obtained through 4D-flow magnetic resonance imaging (4D-flow MRI), has been applied to identify abnormal heart hemodynamics in CHD. Hence, the aim of this research was to compare pressure drop and relative pressures between patients with repaired TOF (rTOF) and healthy volunteers. An in vitro validation was performed, followed by an in vivo validation. We hypothesized that pressure drop is a more stable pressure mapping method than relative pressures to detect altered hemodynamics. A total of 36 subjects, 18 rTOF patients and 18 controls underwent cardiac MRI scans and 4D-flow MRI. Pressure drops and relative pressures in the MPA were higher in rTOF patients compared to the controls (p &lt; 0.05). Following the in vitro validation, pressure drops proved to be a more stable pressure mapping method than relative pressures, as the flow loses its laminarity and becomes more turbulent. In conclusion, this study demonstrated that flow hemodynamics in rTOF can exhibit altered pressure maps. Pressure mapping can help provide further insight into rTOF patients’ hemodynamics to improve patient care and clinical decisions.

Veno-venous extracorporeal membrane oxygenation support of pulmonary insufficiency using Avalon Elite® cannula in pediatric patients.

The "Avalon Elite®" cannula is a double-lumen cannula used to establish veno-venous extracorporeal membrane oxygenation support. The reported advantages are that extracorporeal circulation can be established by cannulating through the right internal jugular vein only, and there is less re-circulation than with a two-cannula technique. It is available in a wide range of cannula sizes and can be used in a variety of patients, from children to adults. We herein report three pediatric cases in which an Avalon Elite® cannula was useful. The first was a case of acute mitral regurgitation due to idiopathic chordal rupture for postoperative severe lung injury and atelectasis due to cardiogenic pulmonary edema. The second was a case of end-stage radiation pneumonitis for safe transfer to facility of lung transplantation. The third was a convalescent case of fulminant myocarditis with severe atelectasis due to cardiogenic pulmonary edema. In each case, veno-venous extracorporeal membrane oxygenation using an Avalon Elite® cannula was established, the expected sufficient support was secured, and a good clinical course was obtained without major complications associated with an Avalon Elite® cannula.

Acute Changes in Right Ventricular Function in Pediatric Patients with Pulmonary Valve Stenosis Undergoing Percutaneous Valvuloplasty: A Speckle-Tracking Study.

Pulmonary valve stenosis determines multiple effects on the right ventricular dimension and function. Percutaneous balloon valvuloplasty is the treatment of choice in severe pulmonary valve stenosis in patients of all ages. However, little is known regarding right ventricular function immediate changes after percutaneous balloon dilation. Pediatric patients with isolated pulmonary valve stenosis represent a pure clinical model of chronic RV pressure overload not affected by other confounders or comorbidities. This study seeks to explore right ventricle (RV) mechanics in pediatric patients early after percutaneous balloon pulmonary valvuloplasty (BPV) for valvar pulmonary stenosis (PS). Forty-three pediatric patients (19 males), mean age 3.2 ± 4.9 years old, with severe pulmonary valve stenosis and indication for percutaneous balloon valvuloplasty were recruited. All patients underwent standard transthoracic echocardiography (TTE), and speckle-tracking echocardiography (STE) with an analysis of right ventricle free-wall longitudinal strain (RVFWLS) one day before and one day after the procedure. For each patient, we collected invasive parameters during the interventional procedure before and after BPV. After the procedure, there was an immediate significant reduction in both peak-to-peak transpulmonary gradient (peak-to-peak PG) and ratio between the right ventricle and aortic systolic pressure (RV/AoP) with a drop of ∆29.3 ± 14.67 mmHg and ∆0.43 ± 0.03, respectively. Post-procedural echocardiography showed peak and mean transvalvar pressure gradient drop (∆50 ± 32.23 and ∆31 ± 17.97, respectively). The degree of pulmonary valve regurgitation was mild in 8% of patients before the procedure, affecting 29% of our patients post-BPV (p = 0.007). The analysis of right ventricular mechanics showed a significant improvement of fractional area change (FAC) immediately after BPV (40.11% vs. 44.42%, p = 0.01). On the other hand, right ventricular longitudinal systolic function parameters, TAPSE and global RVFWLS, did not improve significantly after intervention. The segmental analysis of the RVFWLS showed a significant regional increase in the myocardial deformation of the apical segments. Percutaneous BPV represents an efficient and safe procedure to relieve severe pulmonary valve stenosis. The analysis of the right ventricular function on echocardiography demonstrated an immediate global systolic function improvement, while longitudinal systolic function was persistently impaired 24 h after intervention, possibly due to the necessity of a longer recovery time.

Rightward imbalanced pulmonary perfusion predicts better exercise stroke volume in children after Fallot repair

BackgroundResidual lesions following Fallot repair are primarily pulmonary regurgitation and right ventricular outflow tract obstruction. These lesions may impact exercise tolerance, particularly because of a poor increase in left ventricular stroke volume. Pulmonary perfusion imbalance is also common, but its effect on cardiac adaptation to exercise is not known. AimTo assess the association between pulmonary perfusion asymmetry and peak indexed exercise stroke volume (pSVi) in young patients. MethodsWe retrospectively studied 82 consecutive patients with Fallot repair (mean age 15.2±3.8 years) who underwent echocardiography, four-dimensional flow magnetic resonance imaging and cardiopulmonary testing with pSVi measurement by thoracic bioimpedance. Normal pulmonary flow distribution was defined as right pulmonary artery perfusion between 43 and 61%. ResultsNormal, rightward and leftward flow distributions were found in 52 (63%), 26 (32%) and four (5%) patients, respectively. Independent predictors of pSVi were right pulmonary artery perfusion (β=0.368, 95% confidence interval [CI] 0.188 to 0.548; P=0.0003), right ventricular ejection fraction (β=0.205, 95% CI 0.026 to 0.383; P=0.049), pulmonary regurgitation fraction (β=–0.283, 95% CI –0.495 to –0.072; P=0.006) and Fallot variant with pulmonary atresia (β=–0.213, 95% CI –0.416 to –0.009; P=0.041). The pSVi prediction was similar when the categorical variable right pulmonary artery perfusion>61% was used (β=0.210, 95% CI 0.006 to 0.415; P=0.044). ConclusionIn addition to right ventricular ejection fraction, pulmonary regurgitation fraction and Fallot variant with pulmonary atresia, right pulmonary artery perfusion is a predictor of pSVi, in that rightward imbalanced pulmonary perfusion favours greater pSVi.

Electromechanical Dyssynchrony Is Associated With Right Ventricular Remodeling and Dysfunction Independently of Pulmonary Regurgitation Late After Tetralogy of Fallot Repair

The aim of this study was to investigate the relationship among right ventricular (RV) dilatation, dysfunction, and electromechanical dyssynchrony (EMD) in patients with repaired tetralogy of Fallot (rTOF). Data from a prospective rTOF registry of subjects with moderate or greater pulmonary regurgitation (PR) and contemporary imaging were analyzed. Electrocardiograms and echocardiograms were analyzed for EMD (prolonged QRS duration [QRSd], echocardiographic septal flash, and mechanical delay) and mechanical dispersion. The relationship among these, RV measurements on cardiac magnetic resonance, exercise capacity, and incident arrhythmia or death was analyzed with adjustment for PR. In total, 271 patients with rTOF (42% women; median age, 32years; interquartile range [IQR], 23-34 years) were included. Patients had moderate to severe PR (median PR fraction, 38%; IQR, 30%-47%), moderate to severe RV enlargement (median RV end-diastolic volume index, 161 mL/m2; IQR, 138-186 mL/m2) and mild RV systolic dysfunction (median RV ejection fraction [RVEF], 44%; IQR, 38%-48%). Eleven patients (4%) experienced ventricular arrhythmia or death. Presence of EMD was associated with larger RV size (RV end-diastolic volume index and RV end-systolic volume index, P=.006 and P<.001, respectively) and lower RVEF (P<.001). A sharp inflection in the relation among QRSd, RV size, and RVEF was observed when QRSd exceeded 150msec (3.1% decrease in RVEF for every 20-msec increase in QRSd between 160 and 200msec). Similar inflection points were observed for the mechanical delay between the RV basal-lateral and midseptal segments. The mechanical delay was higher in patients with vs without incident atrial arrhythmia (371 vs 276msec, P=.014). In adults with rTOF, EMD is independently associated with larger RV size, lower RVEF, and incident atrial arrhythmias.

Pulmonary valve reconstruction for acquired pulmonary regurgitation in patients with treated congenital heart disease.

Pulmonary valve regurgitation is a common problem after relief of right ventricular outflow tract (RVOT) obstruction with a transannular patch. Pulmonary valve replacement with a homograft or xenograft is the routine treatment. Longevity of biological valves and the availability of homografts are limited. Alternatives to restore RVOT competence are evaluated. The goal of this study was to present intermediate-term results for pulmonary valve reconstruction (PVr) in patients with severe regurgitation. PVr was performed in 24 patients (August 2006‒July 2018). We analysed perioperative data, pre- and postoperative cardiac magnetic resonance (CMR) imaging studies, freedom from valve replacement and risk factors for pulmonary valve dysfunction. The underlying diagnoses were tetralogy of Fallot (n = 18, 75%), pulmonary stenosis (n = 5, 20.8%) and the double outlet right ventricle post banding procedure (n = 1, 4.2%). The median age was 21.5 (14.8-23.7) years. Main (n = 9, 37.5%) and branch pulmonary artery procedures (n = 6, 25%) and surgery of the RVOT (n = 16, 30.2%) were often part of the reconstruction. The median follow-up after the operation was 8.0 (4.7-9.7) years. Freedom from valve failure was 96% at 2 and 90% at 5 years. The mean longevity of the reconstructive surgery was 9.9 years (95% confidence interval: 8.8-11.1 years). CMR before and 6 months after surgery showed a reduction in the regurgitation fraction [41% (33-55) vs 20% (18-27) P = 0.00] and of the indexed right ventricular end-diastolic volume [156 ml/m2 (149-175) vs 116 ml/m2 (100-143), P = 0.004]. Peak velocity across the pulmonary valve (determined by CMR) half a year after surgery was 2.0, unchanged. PVr can be achieved with acceptable intermediate-term results and may delay pulmonary valve replacement.