Moderate Pulmonary Regurgitation Research Articles

Prognostic value of myocardial deformation parameters for outcome prediction in tetralogy of Fallot

The prognostic value of myocardial deformation parameters in adults with repaired tetralogy of Fallot (rTOF) has not been well-elucidated. We therefore aimed to explore myocardial deformation parameters for outcome prediction in adults with rTOF using cardiovascular magnetic resonance imaging (CMR). Adults with rTOF and at least moderate pulmonary regurgitation (PR) were identified from an institutional prospective CMR registry. Left (LV) and right ventricular (RV) global strain were recorded in longitudinal (GLS), circumferential (GCS) and radial (GRS) directions. Major adverse cardiovascular events (MACE) were defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia (>30seconds) or heart failure (hospital admission >24hours). In patients with pulmonary valve replacement (PVR), pre-and post-PVR CMR studies were analysed to assess for predictors of complete RV reverse remodelling, which we defined as RV end-diastolic volume indexed (RVEDVi) <110ml/m2. Logistic regression models were used to estimate the odds ratio (OR) per unit change in absolute strain value associated with clinical outcomes and receiver operator characteristic (ROC) curves were constructed with area under the curve (AUC) for select CMR variables. We included 307 patients (age 35±13 years, 59% male). During 6.1 years (3.3-8.8) of follow-up, pulmonary valve replacement (PVR) was performed in 142 (46%) and MACE occurred in 31 (10%). On univariate analysis, baseline biventricular ejection fraction (EF), mass and all strain parameters were associated with MACE. After adjustment for LVEF, only LVGLS remained independently predictive of MACE (OR 0.822 [0.693-0.976] p=0.025). Receiver operator curves identified an absolute LVGLS value less than 15 and LVEF <51% as thresholds for MACE prediction (AUC 0.759 [0.655-0.840] and 0.720 [0.608-0.810]). After adjusting for baseline RVEDVi, RVGCS (OR 1.323 [1.094-1.600] p=0.004), LVGCS (OR 1.276 [1.029-1.582] p=0.027) and LVGRS (OR 1.101 [1.0210-1.200], p=0.028) were independent predictors of complete remodelling post-PVR remodelling. Biventricular strain parameters predict clinical outcomes and post-PVR remodelling in rTOF. Further study will be necessary to establish the role of myocardial deformation parameters in clinical practice.

Quadricuspid pulmonary valve: A rare cause of pulmonary artery aneurysm.

A 17-year-old male presented to our hospital from an external center with complaints of chest pain and shortness of breath, referred with preliminary diagnoses of pulmonary stenosis and pulmonary artery dilation. Echocardiography revealed moderate pulmonary regurgitation and mild pulmonary stenosis. Coronary computed tomography (CT) angiography showed normal coronary arteries and no presence of a patent ductus arteriosus, but an aneurysmal dilation of up to 4 cm was observed in the pulmonary artery. The patient was found to have a quadricuspid (QPV) pulmonary valve, while the aortic valve appeared normal. No phenotypic anomalies or accompanying aortic or lung abnormalities were detected in this case. Our case represents the first in the pediatric age group diagnosed via CT scan. Our study contributes insights into the relationship between QPV anomaly and pulmonary artery aneurysm, aiding in the diagnosis and management of these rare conditions. Furthermore, our findings highlight the importance of evaluating the possibility of QPV in patients with pulmonary valve stenosis, regurgitation, or artery aneurysm, emphasizing the significance of coronary CT angiography in the diagnostic process.

Exercise Capacity and Reoperation Late After Transatrial Fallot Repair

The exercise capacity long after repair of tetralogy of Fallot, when performed exclusively with a transatrial repair, is unclear. It is also unknown whether echocardiography and cardiopulmonary exercise testing can predict the risk of reoperation in this patient group. We retrospectively reviewed the clinical records of 59 patients who underwent cardiopulmonary exercise testing after transatrial Fallot repair at a single centre. Patients underwent cardiopulmonary exercise testing at a mean age of 16.6±4.4 years, and at 15.3±4.1 years after Fallot repair. At testing, the volume of oxygen consumption at maximal exercise (VO2 max) was 71%±13% and the oxygen pulse was 80%±17% of predicted values. Seventeen (17) patients (29%) had a VO2 max superior to 80% of the predicted value. Thirty-two (32) patients (56%) had severe pulmonary regurgitation, three (5%) had moderate pulmonary regurgitation, and 12 (21%) had mild pulmonary regurgitation. After a mean of 7.8±3.9 years following cardiopulmonary exercise testing (23±5.3 years after the repair), 21 (40%) patients underwent reoperation. Right ventricular dilation and systolic function on echocardiography were both significantly associated with subsequent reoperation rates. Patients who had severe right ventricular dilation were eight times more likely to undergo subsequent reoperation (hazard ratio 8.67; 1.82-41.3; p=0.007). No cardiopulmonary exercise testing variable independently predicted reoperation. The exercise capacity at adolescence following transatrial repair of tetralogy of Fallot is maintained at around 70% of predicted values. Only the patients with normal right ventricular size and normal right ventricular function seemed to be protected from reoperation over the subsequent decade. We found no exercise variables which predicted reoperation.

Late Presentation of Pulmonary Hypertension Crisis Concurrent with Atrial Arrhythmia after Atrial Septal Defect Device Closure

Background: ASD occurs when there is a septal defect between the right and left atria, resulting in a left-to-right shunt that increases the volume of the right heart and pulmonary circulation. Increased pulmonary resistance can lead to pulmonary hypertension (PH), resulting in progressive deterioration of right ventricular function, leading to right heart failure and death. Prolonged elevation of atrial pressure induces progressive atrial dilatation and electrophysiological remodelling. Together with autonomic modulation, this leads to atrial arrhythmias (AAs). Patients with significant shunts leading to ventricular volume overload are considered for ASD closure. However, in some cases, PH occurs after ASD closure.  Case Presentation: We report a 21 yo man diagnosed with ASD Secundum Post Closure with Device (September 1th, 2023) and Pulmonary Hypertension Crisis. The left atria (LA), right atria (RA) and right ventricle (RV) were dilated. We also found moderate mitral regurgitation, severe tricuspid regurgitation, and mild to moderate pulmonary regurgitation. There was a decline in systolic function in the right ventricle, and grade III diastolic dysfunction in left ventricle. There was a well-seated device with no residual shunt on interatrial septal. The pulmonary arteries were confluence and dilated. From ECG we found atrial flutter with variable conduction. This patient was transferred to HCU. This patient treated with digoxin, furosemide, milrinon, ceftriaxone, miniaspi, sildenafil, electrophysiology, and 3D ablation.  Conclusion: Pulmonary hypertension can occur in cases of congenital heart defects, such as ASD. The operative management of ASD is closure of the ASD, but in some unique groups, this can lead to pulmonary hypertensive crisis after its closure.

Carcinoid syndrome with right-sided valve involvement - a case report and review of the literature.

The survival of patients with neuroendocrine tumors has substantially improved with modern treatment options. Although the associated carcinoid syndrome can be diagnosed early and controlled effectively, cardiologists still encounter patients with cardiac manifestations, particularly among individuals with persistently high levels of vasoactive mediators. Treatment options have been limited to surgical valve replacement in fully manifested disease. Since surgery is not always feasible, transcatheter valve implantation is becoming an interesting alternative. A case of a 50-year-old woman with carcinoid syndrome and right-sided valvular heart disease is presented. Moderate pulmonary valve stenosis and severe tricuspid valve regurgitation were diagnosed during the evaluation and treatment of neuroendocrine tumor. The possibility of rare valve involvement and the need for interdisciplinary cooperation in the diagnosis, monitoring and treatment of patients with neuroendocrine tumors producing vasoactive substances must be emphasized. The patient had a typically presenting carcinoid syndrome with a rare cardiac manifestation. Although monitoring and treatment were carried out in accordance with recommendations and appropriate to the clinical condition, rapid progression of the metastatic disease ultimately precluded invasive cardiac intervention.

The results of total repair for tetralogy of fallot with an abnormal coronary artery

The objective of this study evaluated the results of total repair for tetralogy of Fallot with an abnormal coronary artery.&#x0D; Methods: This was a review of seri Tetralogy of Fallot with an abnormal coronary artery cases who underwent total repair from 1/2018 to 6/2023.&#x0D; Results: There were 10 patients who underwent complete surgical repair, include 6 males and 4 females. The mean age was 6,8 months (range 4 months to 17 months)and the mean weight was 7,2 kilograms. 6 patients had Left Anterior Descending (LAD) artery originating from Right Coronary Artery (RCA), 3 patients with RCA originating from LAD and 1 patient with one major banche of RCA crossing the infundibulum. There were 7 patients with Pulmonary Valve Preserve (PVP) and 3 patients with Conduit Using (CU). The time of cardiopulmonary bypass, aortic clamping and ventilator were: 90 mins; 63 mins and 16 hours. There was not intrahospital mortality. Echocardiography post -operation: in PVP group all patient had a competence pulmonary valve and mean gradient was 12,5 mmHg; in CU group, there were not pulmonary valve regurgitation and mean gradient was 9 mmHg. During follow-up time 30 months after surgery, there was no late death; 2 patient in PVP group who had moderate pulmonary valve regurgitation and mean gradient was 22 mmHg; in CU group, there was a competence pulmonary valve with mean gradient 26 mmHg.&#x0D; Conclusion: There was a good result of total repair for tetralogy of Fallot with an abnormal coronary artery.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The studyis aretrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1years) than those with d-TGA (37.5 ± 8.3years) or complex ccTGA (40.8 ± 13.4years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Five-year follow-up after percutaneous pulmonary valve implantation using the Venus P-valve system for patients with pulmonary regurgitation and an enlarged native right ventricular outflow tract.

Percutaneous pulmonary valve implantation (PPVI) with the self-expandable Venus P-valve system is a promising treatment for patients with pulmonary regurgitation (PR) and a native right ventricular outflow tract (RVOT). However, limited data is available regarding its midterm outcomes. This study assessed the midterm clinical and echocardiographic outcomes following Venus P-valve implantation. From 2013 to 2018, 55 patients with moderate or severe PR after surgical RVOT repair with a transannular or RVOT patch were consecutively enrolled from six hospitals in China. Five-year clinical and echocardiographic outcomes were collected and evaluated. The primary endpoint was a freedom from all-cause mortality and reintervention. At 5 years, the primary endpoint was met for 96% of patients, corresponding to a freedom from all-cause mortality of 96% (95% confidence interval [CI]: 86%-99%) and freedom from reintervention of 98% (95% CI: 87%-100%). Endocarditis was reported in five patients (four patients within 1 year and one patient at 5 years) following PPVI. Transpulmonary gradient and stent orifice diameter remained stable compared to at discharge (p＞0.05). No paravalvular leak was reported while only 1 patient gradually increased to moderate PR during follow-up. Significant improvement of RV diameter and LVEF(p＜0.001) sustained over the 5-year follow-up, in consistent with remarked improved New York Heart Association(NYHA) functional class (p＜0.001). The 5-year results of the China VenusP Study demonstrated the midterm benefits of Venus P-valve implantation in the management of patients with severe PR with an enlarged native RVOT by providing sustained symptomatic and hemodynamic improvement.

Managing Challenges in a Symptomatic Patient with Moderate Pulmonary Stenosis: A Case Report

Introduction: Pulmonary stenosis is a common form of obstruction in the right ventricular outflow tract, characterized by the narrowing of the pulmonary valve, which results in obstructed blood flow. Case Presentation: A 17-year-old female presented with recurring shortness of breath, chest pain, and palpitations, especially during physical activity. On examination, a grade III/VI continuous murmur was heard at the upper left sternal border. Echocardiography confirmed moderate pulmonary valve stenosis, a left-to-right patent ductus arteriosus (PDA), and mild to moderate pulmonary regurgitation.

Adherence to clinical practice guidelines for pulmonary valve intervention after tetralogy of Fallot repair: A nationwide cohort study

ObjectivesTo determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. MethodsChildren and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (−) of PVR and presence (+) versus absence (−) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. ResultsIn total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication–). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR–/indication–). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). ConclusionsAlthough a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve

BackgroundVarious bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus on whether a particular valve is best for this application. Recently, the Inspiris Resilia valve (Edwards Lifesciences Inc) was approved for aortic valve replacement, and surgeons have begun using it for PVR. There is limited evidence on the performance of the Inspiris valve compared with other valves in the pulmonary position. MethodsThis study reviewed all patients who underwent PVR with a size 19- to 29-mm Inspiris valve or Mosaic valve (Medtronic Inc) from 2007 to 2022 at Lucile Packard Children's Hospital Stanford (Palo Alto, CA). Midterm outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient ≥36 mm Hg, and freedom from reintervention. ResultsA total of 225 consecutive patients who underwent PVR with a size 19- to 29-mm Mosaic (n = 163) or Inspiris (n = 62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On univariable and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient ≥36 mm Hg. ConclusionsEarly and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to that of other bioprosthetic valves used for PVR.

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.

1-Year Outcomes in a Pooled Cohort of Harmony Transcatheter Pulmonary Valve Clinical Trial Participants

BackgroundThe Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration–approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT). ObjectivesOne-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients. MethodsEligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately. ResultsIn the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately. ConclusionsThe Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability.

Use of the Inspiris valve in the native right ventricular outflow tract is associated with early prosthetic regurgitation

ObjectiveThe Inspiris Resilia prosthesis (Edwards Lifesciences) has been increasingly used in the pulmonic position with limited performance data. We sought to investigate its durability as a surgical pulmonary valve replacement (PVR). MethodsWe retrospectively reviewed patients who underwent PVR or conduit replacement with an Inspiris or non-Inspiris valve/conduit from 2018 to 2022. The primary end point was freedom from a composite of at least moderate pulmonary regurgitation, pulmonary stenosis, or valve/conduit reintervention. Secondary end points were individual components of the composite outcome. To account for baseline differences, propensity matching identified 70 patient pairs. ResultsA total of 227 patients (median age: 19.3 years [interquartile range, 11.8-34.4]) underwent PVR or conduit replacement (Inspiris: n = 120 [52.9%], non-Inspiris: n = 107 [47.1%]). Median follow-up was 26.6 months [interquartile range, 12.4-41.1]. Among matched patients, 2-year freedom from valve failure was lower in the Inspiris group (53.5 ± 9.3% vs 78.5 ± 5.9%, P = .03), as was freedom from at least moderate pulmonary regurgitation (54.2 ± 9.6% vs 86.4 ± 4.9%, P < .01). There was no difference in 2-year freedom from at least moderate pulmonary stenosis (P = .61) or reintervention (P = .92). Inspiris durability was poorer when implanted in the native right ventricular outflow tract compared with as a conduit, with 18-month freedom from valve failure of 59.0 ± 9.5% versus 85.9 ± 9.5% (P = .03). ConclusionsEarly durability of the Inspiris valve is poor when implanted in the native right ventricular outflow tract; its unique design may be incompatible with the compliant pulmonary root. Modified implantation techniques or alternative prostheses should be considered.

A mid- and long-team follow-up study of the application of single-valved bovine pericardium patches in right ventricular outflow tract reconstruction.

Pulmonary regurgitation following right ventricular outflow tract (RVOT) reconstruction may cause right heart dysfunction and even right heart failure. Installation of a single valve at this time point can effectively reduce pulmonary regurgitation, thereby protecting right heart function. Here, we analyzed the outcomes and mid- and long-term follow-up data of patients undergoing single-valved bovine pericardium patch (svBPP) placement for reconstruction and explored the effectiveness and gaps of svBPP in preventing right heart failure. A retrospective analysis was performed on patients undergoing RVOT reconstruction using BalMonocTM svBPP from October 2010 to August 2020. The follow up procedures included outpatient visits and collection of outcomes. The cardiac ultrasound-related indicators during the follow-up visits included ejection fraction (EF), right ventricular end-diastolic diameter (EDD), pulmonary regurgitation, and pulmonary artery stenosis. The survival rates and reoperation-free rate were analyzed by Kaplan-Meier method. Patients includes tetralogy of Fallot, pulmonary atresia and other complex congenital heart disease. A total of 5 patients (5.7%) died during the perioperative period. Early complications included pleural effusion, cardiac insufficiency, respiratory insufficiency, chylothorax, and atelectasis, all of which were cured. After discharge, 83 patients (94.3%) were effectively followed up. During follow-up, 1 patient died and 1 patient underwent reoperation. The 1-, 5-, and 10-year survival rates were 98.8%, 98.8%, and 98.8%, respectively, and the reintervention-free rates for the same intervals were 98.8%, 98.8%, and 98.8%, respectively. The last follow-up ultrasound revealed severe pulmonary stenosis in 0 cases, moderate stenosis in 2 cases, mild stenosis in 7 cases, and no stenosis in 73 cases. Pulmonary regurgitation was not found in 12 patients; however, there were 2 cases of severe pulmonary regurgitation, 20 cases of moderate pulmonary regurgitation, and 48 cases of mild pulmonary regurgitation. As shown in the mid- and long-term follow-up studies, BalMonocTM svBPP has good performance in RVOT reconstruction. It can effectively eliminate or reduce pulmonary valve regurgitation and protect right heart function. Both réparation à l'Etage ventriculaire (REV) and the modified Barbero-Marcial procedure can bring growth potential and reduce reoperation rate.

Congenital Pulmonic Valve Dysfunction Treated With SAPIEN 3 Transcatheter Heart Valve (from the COMPASSION S3 Trial)

Significant pulmonary regurgitation (PR) and pulmonary stenosis are common after surgical repair of some congenital heart defects. This prospective, single-arm, multicenter trial enrolled patients who underwent transcatheter heart valve (THV) implantation with a SAPIEN 3 valve to treat dysfunctional right ventricular outflow tract (RVOT) conduits or pulmonic surgical valves (≥ moderate PR and/or mean RVOT gradient ≥35mm Hg). The primary end point was a nonhierarchical composite of THV dysfunction at 1year comprising RVOT reintervention, ≥ moderate total PR, and mean RVOT gradient >40mm Hg. A performance goal of <25% of upper confidence interval (CI) was prespecified for the primary end point, using a 95% exact binomial CI. Patients (n=58) were enrolled between July 5, 2016 and July 17, 2018, with mean age of 32years. Prestenting was performed in 53.4%. At discharge, the device success was 98.1% (single valve without explant, < moderate PR, gradient <35mm Hg). At 30days, there were no major adjudicated adverse clinical events. At 1year, the primary end point composite was 4.3% (95% CI 0.5 to 14.5). The composite components were 0% (0 of 56) RVOT reintervention, 2.1% (1 of 47) ≥ moderatePR, and 2.1% (1 of 48) mean RVOT gradient >40mm Hg. No mortality, endocarditis, thrombosis, or stent fracture were reported at 1year. In conclusion, the SAPIEN 3 THV was safe and effective in patients with dysfunctional RVOT conduits or previously implanted valves in the pulmonic position to 1 year. Clinical trial registration: NCT02744677; https://clinicaltrials.gov/ct2/show/NCT02744677.

Left ventricle dysfunction in patients with critical neonatal pulmonary stenosis: echocardiographic predictors. A single-center retrospective study.

The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction (p < 0.05) in PVS and PAIVS patients.

Feasibility and Surgical Effect of Annulus Sparing in Consecutive Patients with Tetralogy of Fallot: A Retrospective Cohort Study

Objective: Annulus-sparing (AS) repair for tetralogy of Fallot (TOF) with a dysplastic pulmonary valve annulus (PVA) is a challenging procedure and is controversial. This study aimed to assess the feasibility and surgical effect of AS repair versus transannular patch enlargement (TAPE) repair, especially in individuals with dysplastic pulmonary valves. Methods: This retrospective cohort study included 375 pediatric patients with a primary diagnosis of TOF in the Center for Pediatric Cardiac Surgery of Fuwai hospital from January 2014 to June 2017. Among them, 60 consecutive and nonselective patients underwent 1-stage repair of TOF with aggressive PVA-preserving strategies performed by a single surgeon were enrolled in AS cohort. In AS cohort, patients were divided into AS, PVA z-score ≥−2 group (33 patients) and AS, PVA z-score &lt;−2 group (27 patients). During the same period, 315 patients underwent TAPE repair by other surgeons were enrolled as TAPE cohort, of these, 87 patients with PVA z-score ≥−2 were excluded. From the 228 patients in the TAPE group, 27 cases were selected as TAPE, PVA z-score &lt;−2 group according to the propensity score and 1:1 ratio with AS, PVA z-score &lt;−2 group. The primary outcome was a composite of reintervention, significant pulmonary regurgitation, and significant annular peak gradient (APG). Kaplan-Meier curve was plotted to show the survival rate of severe pulmonary regurgitation. Results: One death occurred after the TAPE operation in TAPE group, and 1 patient in the AS z ≥−2 group needed reintervention with a balloon. After a median follow-up of (30.3 ± 11.6) months, compared with AS z ≥−2 group, there was no difference in the technical performance score for severe pulmonary stenosis (APGs &gt; 20 mmHg) in the AS z &lt;−2 group. Compared with TAPE, AS repair was often accompanied by a postoperative APGs over 20 mmHg (P = 0.001). More patients underwent TAPE suffered from moderate or severe pulmonary regurgitation than those who received AS repair (20 (74.1%) vs. 7 (26.0%), P &lt; 0.001). AS repair was associated with a shorter duration of mechanical ventilation (20 vs. 29 hours, P = 0.039), faster discharge from the intensive care unit (2.0 vs. 4.0 days, P = 0.022) and shorter postoperative hospitalization (8.0 vs. 11.0 days, P = 0.008) compared with TAPE. Conclusions: APG demonstrated an upward trend in the TAPE group and a downtrend in the AS group after discharge from hospital. AS repair had an acceptable surgical effect in TOF patients, even in those with a dysplastic PVA. A higher APG remained upon hospital discharge in dysplastic patients with AS, but a downward trend was observed over time.

Left ventricular strain derived from cardiac magnetic resonance can predict outcomes of pulmonary valve replacement in patients with repaired tetralogy of Fallot.

PurposeSeveral adults with repaired tetralogy of Fallot (rToF) undergo pulmonary valve replacement (PVR) to reduce the right ventricular volume and retain right ventricular function. However, there is currently no consensus on the ideal time for PVR surgery in asymptomatic patients with rTOF with pulmonary regurgitation (PR). Clinical outcomes after PVR are also indeterminate. Recently, myocardial strain and strain rate derived from cardiac magnetic resonance (CMR) feature tracking were found to be more sensitive to right ventricular dysfunction than conventional parameters and therefore may add prognostic value in patients with rToF. We aimed to analyze whether pre-PVR left ventricular (LV) strain and strain rate detected by CMR feature tracking are associated with midterm outcomes after PVR in patients with rToF.MethodsSeventy-eight asymptomatic patients with rToF who required PVR due to moderate or severe PR were prospectively enrolled between January 2014 and June 2020. CMR cine sequences were obtained, and feature tracking parameters were measured preoperatively. Adverse events were documented during the follow-up. Receiver operating characteristic analysis was performed to determine the cutoff value. Kaplan–Meier curves were drawn with log-rank statistics; moreover, univariate and multivariate Cox proportional hazards regression analyses and Harrel C-indices were analyzed.ResultsDuring 3.6 ± 1.8 years of follow-up, 25 adverse events were recorded. Kaplan–Meier survival curves and univariate Cox analysis verified that patients with significantly reduced radial strain (RS), circumferential strain (CS), longitudinal strain (LS), RS rate at systole and diastole (RSRs and RSRe), and circumferential and LS rates at diastole (CSRe and LSRe) had worse event-free survival. After multivariate correction, only LS and LSRe remained significantly associated with adverse outcomes (hazard ratio = 1.243 [1.083–1.428] and 0.067 [0.017–0.258], respectively, all p < 0.05). The cutoff values of LS and LSRe were −12.30 (%) and 1.07 (s–1), respectively.ConclusionThe LV strain and strain rate prior to PVR are important prognostic factors for adverse events after PVR in rToF.

EP188: Novel HECW2 variant presenting with tachypnea and multisystemic congenital malformations

HECW2, otherwise known as NEDL2, encodes for a member of the HECT family of E3 ubiquitin ligases and stabilizes p73, a key mediator in neurodevelopment. HECW2 is primarily expressed in the brain, heart, and lungs. Heterozygous variants have been associated with developmental delays, intellectual disability, epilepsy, hypotonia, scoliosis and dysmorphic features. Systemic complications which have been reported include cardiomyopathy, skeletal abnormalities including pectus excavatum, and various brain abnormalities including white matter disease, cortical atrophy, ex-vacuo ventriculomegaly, and thinning of the corpus callosum. While many reported cases of HECW2-related disorder have been from de novo variants, there have been reports of familial inheritance with variable expressivity. To date, there have been no reported cases of HECW2-related disorder presenting with persistent tachypnea. A newborn male with a history of intrauterine growth restriction born at 39 2/7 weeks gestation to a 31 year old G4P0212 mother and a 35 year old father presented in the perinatal period with unexplained persistent tachypnea, difficulty feeding, persistent diaphoresis and failure to thrive. Pectus excavatum was noted at birth and initial evaluation for the persistent tachypnea showed multiple vertebral anomalies, congenital scoliosis and mild cardiomegaly on chest X-ray. Renal ultrasound demonstrated left central calyceal dilation. Echocardiogram and CT heart with contrast demonstrated multiple abnormalities including anomalous pulmonary venous return with right upper pulmonary vein draining into the superior vena cava (SVC), secundum atrial septal defect (ASD), dilated main pulmonary artery, right atrium and ventricle, mild right and left ventricular hypertrophy, mild tricuspid regurgitation and mild to moderate pulmonary regurgitation. CT imaging also demonstrated severe tracheobronchomalacia associated with left mainstem bronchus compression resulting in air trapping within the left lung. Cardiac catheterization was attempted for possible closure of the patient’s secundum ASD, but was unsuccessful due to inability to capture retroaortic rim. Diagnostic evaluation during catheterization demonstrated mild pulmonary hypertension, normal pulmonary vascular resistance (PVR) and a Qp:Qs of 1.9:1 consistent with pulmonary overcirculation. Tachypnea and significantly increased work of breathing persisted despite initiation of furosemide diuresis to manage pulmonary overcirculation. The patient ultimately required BiPap for respiratory support, though tachypnea persisted. A G-tube was placed to ensure safe formula administration. Additional physical exam findings included hypotonia, macrocephaly, infraorbital creases with almond-shaped eyes, ocular hypertelorism, broad nasal bridge and tip, mild retrognathia, arachnodactyly with larger hands and feet compared to growth centiles and Y-shaped gluteal fold. Family history was remarkable for White ancestry. A maternal half-sister was healthy. A 2 year old brother had speech delay. The patient’s father had a history of mild congenital scoliosis, heart murmur and intellectual disability. The patient’s mother had a history of anemia, heart murmur and learning delays. There was no history of genetic conditions, recurrent pregnancy loss, or infant death. Chromosomal microarray analysis (CMA) demonstrated no clinically significant findings (ISCN: arr[hg19](1-22x2,(X,Y)x1). Trio exome sequencing with mitochondrial analysis, performed from a peripheral blood sample, demonstrated a paternally inherited likely pathogenic variant in HECW2 (NM_020760.1:c.2581 C>T) in exon 11. While this variant has not been previously published as benign or pathogenic, in silico analysis demonstrated a potential deleterious effect of the arginine to tryptophan substitution on structure and/or function. Mitochondrial analysis was negative/normal. We present the first documented case of HECW2-related disorder presenting with perinatal tachypnea. The patient’s hypotonia, feeding difficulties, dysmorphic features, pectus excavatum, vertebral malformations, scoliosis, and cardiac involvement correlate with previously described manifestations of HECW2-related disorder.