Right ventricular outflow tract obstruction by cardiac hemangioma in asymptomatic patient.

Ventricular hemangiomas are rare benign tumors, pose diagnostic and therapeutic complexities. We report a case of a 52-year-old female with essential hypertension who developed a systolic ejection murmur during a hypertension clinic visit. The echocardiogram revealed a hyperechoic mass obstructing the right ventricular outflow tract, causing enlargement of the right atrium and ventricle, with a reduction in the right ventricular ejection fraction. Due to the risk of death, the patient underwent an emergency surgical resection along with tricuspid valve replacement. Postoperative recovery was uneventful, and subsequent cardiac magnetic resonance imaging showed an improvement in ejection fraction without residual tumor. This case highlights the diagnosis and therapeutic complexities of ventricular hemangiomas. With this report, we aim to provide a comprehensive review of ventricular hemangiomas and to enhance understanding of this condition for improved patient care.

Decreased clinical performance in TGA-ASO patients after RVOT interventions; a multicenter European collaboration.

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.

Neonatal hemodynamics of recipient twins after fetoscopic selective laser coagulation for twin-to-twin transfusion syndrome: An unicist classification.

To characterize the neonatal hemodynamic profiles in recipients born after twin-to-twin transfusion syndrome (TTTS) treated with fetoscopic selective laser coagulation (FSLC). Retrospective analysis during the first month of life of recipient twins. Of the 480 newborns born during an 11-year period, 138 recipient twins with prenatal FSLC were classified into four groups: no hemodynamic impairment (NoHI, n = 102, 74%), isolated high blood pressure (HighBP, n = 18, 13%), right ventricular outflow tract obstruction (RVOTO, n = 10, 7%), and cardiac failure (CF, n = 8, 6%). The time (median (IQR)) between FSLC and birth was significantly shorter in the HighBP (36 days (23-54)) and CF (44 days (18-54)) groups than in the RVOTO (91 days (68-112)) and NoHi (82 days (62-104)) groups (p < 0.001).     Conclusion: Four distinct and well-characterized groups of recipients were identified based on their hemodynamics. High blood pressure and heart failure occurred in approximately 20% of the infants and were associated with a time between laser coagulation and birth of less than 2 months. What is Known: • Twin-to-twin transfusion syndrome (TTTS) is characterized by a hemodynamic imbalance that leads to high fetal and neonatal mortality if left untreated. One-third of recipient twins born without prenatal fetoscopic laser coagulation (FSLC) develop a life-threatening cardiac failure. What is New: • Four distinct groups of recipient twins with prenatal FSLC have been identified based on their hemodynamics. High blood pressure and cardiac failure occurred in 20% of the infants and were associated with an interval between FSLC and birth of less than 2 months.

Systematic Analysis of PTFE Monocusp Leaflet Design in a Patient-Based 3D in-Vitro Model of Tetralogy of Fallot.

PURPOSE: Pulmonary valve (PV) monocusp reconstruction in transannular patch (TAP) right ventricular outflow tract (RVOT) repair for Tetralogy of Fallot has variable clinical outcomes across different surgical approaches. The study purpose was to systematically evaluate how monocusp leaflet design parameters affect valve function in-vitro. METHODS: A 3D-printed, disease-specific RVOT model was tested under three infant physiological conditions. Monocusps were sewn into models with the native main pulmonary artery (MPA) forming backwalls that constituted 40% and 50% of the reconstructed circumference for z-score zero PV annulus and MPA diameters (native PV z-score - 3.52 and - 2.99 for BSA 0.32m2). Various leaflet free edge lengths (FEL) (relative to backwall), positions (relative to PV STJ), and scallop depths were investigated across both models. Pressure gradient, regurgitation, and coaptation were analyzed with descriptive statistics and regression models. RESULTS: Increasing FEL beyond 100% of the MPA backwall decreased gradient but mildly increased regurgitation to a peak of 25%. Positioning the free edge 2 mm past the STJ mildly increased gradient for each FEL without significantly changing regurgitation compared to STJ placement. Scalloping leaflets trivially affected performance. Pre-folding leaflets improved mobility and slightly reduced gradient. CONCLUSIONS: Balancing gradient, regurgitation, and oversizing for growth, a set of leaflet designs have been selected for pre-clinical evaluation. Designs with leaflet widths 140-160% in the 40% backwall model (110-120% in the 50% backwall), positioned at or 2 mm past the STJ, demonstrated the best results. The next stage of ex-vivo testing will additionally consider native RVOT distensibility, native leaflet interactions, and TAP characteristics.

The Gasul Phenomenon Still Alive in the Developing World: A Case Report.

Background: In child, ventricular septal defect is the most prevalent congenital cardiac disease. Some ventricular septal defects have the potential for spontaneous closure. In poor nations closure based on indications may not be feasible. The patient's natural course may therefore be observed. The Gasul phenomenon, a right ventricular outflow obstruction, is one of the complications. Case Presentation: A 7-year-old child who had recurrent pneumonia, poor weight gain, and excessive sweating eventually had these symptoms go away. A large peri membranous ventricular septal defect and a small patent ductus arteriosus was detected on echocardiography during infancy. Later, the patient acquired a muscular ridge across the right ventricular outflow tract. Muscular ridge excision and closure of patent ductus arteriosus and ventricular septal defect were done. Patient was discharged in stable condition. Conclusion: Right ventricular outflow tract blockage can be avoided by performing early surgical closure of a ventricular septal defect.

Subpulmonary membrane: A rare cause of right ventricular outflow tract obstruction.

Subpulmonary membrane is a rare cause of right ventricular outflow tract (RVOT) obstruction, and only a few case reports exist with or without associated ventricular septal defect. We report a series of three cases with subpulmonary membrane causing RVOT obstruction. Two of these have been operated (the first case operated after unsuccessful attempt at balloon dilatation), and the third case is on follow-up at present.

Long-term follow-up after simultaneous arterial switch operation and aortic arch repair.

OBJECTIVES: Taussig-Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. METHODS: At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5-9] days, weight 3.38 (IQR 2.9-3.8) kg and follow-up 9.3 (IQR 3.1-14.5) years. The AA reconstruction was performed without patch material in 49 cases. RESULTS: There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. CONCLUSIONS: The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate.

Case report: Right ventricular outflow tract obstruction caused by multicomponent mesenchymal tumor.

Right ventricular outflow tract obstruction (RVOTO) is a cause of hemodynamic instability that can lead to right ventricular dysfunction. Cardiac tumors located in the right ventricle or surrounding structures can cause RVOTO. Herein, we present a rare case of a 21-year-old male with palpitations due to RVOTO caused by a cardiac multicomponent mesenchymal tumor. The tumor was localized in the right ventricular outflow tract, resulting in right side heart enlargement, tricuspid regurgitation, and RVOTO. Hence, tumor resection was performed. The patient was in a stable condition and discharged home on the 6th post-operative day. However, histopathological examination of the tumor specimen suggested a three-component mesenchymal tumor containing mucinous components, formed blood vessels, and fibrous tissue, which is like an atypical capillary hemangioma. After seven years of follow-up, the patient had no right heart enlargement, tricuspid regurgitation, and tumor recurrence. We believe surgical treatment is effective, and this case will provide a reference for clinicians to treat and evaluate the prognosis of similar three-component mesenchymal cardiac tumor cases in the future.

Outcomes and risk analysis after tricuspid valve surgery for non-Ebstein 2-ventricle congenital tricuspid valve diseases.

Objectives: Tricuspid valve (TV) surgery remains understudied and little data exist describing the surgical indications, outcomes, and prognostic factors for pediatric patients with non-Ebstein 2-ventricle congenital TV lesions. This study aims to describe early and late outcomes of pediatric patients with non-Ebstein congenital TV lesions undergoing isolated TV procedures at a single institution. Methods: All patients who underwent TV surgery for non-Ebstein congenital TV disease between 2006 and 2018 were included. Patients who had missing preoperative data, patients with single-ventricle physiology, congenitally corrected transposition of the great arteries, and patients undergoing TV intervention as part of repair of an atrioventricular canal defect were excluded. The primary end point was the occurrence of TV reintervention or TV regurgitation (TR) ≥ moderate. Results: A total of 85 patients were included. The tricuspid lesion was isolated TR in 80 (94.1%), isolated tricuspid stenosis in 3 (3.5%) and mixed disease in 2 (2.4%) patients. Median age at surgery was 33 years (interquartile range, 12-53 years). TV repair and TV replacement were performed in 66 (77.6%) and 19 (22.4%) patients, respectively. One (1.2%) patient underwent TV reoperation during the same admission. There was no in-hospital mortality. Median follow-up was 3.3 years (interquartile range, 0.1-4.7 years). The overall cumulative incidence of TV reintervention or TR deemed moderate or greater at 1, 3, and 5 years was 3% ± 2%, 11% ± 4%, and 20% ± 8%. In multivariable analysis, age younger than 12 years (P = .04) and mitral valve regurgitation deemed moderate or greater (P = .01) were independent risk factors for TV reintervention or recurrent TR deemed to be moderate or greater at last follow-up. Conclusions: TV surgery in patients with non-Ebstein congenital TV disease can be performed with good outcomes. TV reintervention or TR deemed moderate or greater occurred in 20% of patients on midterm follow-up. Patients younger than age 12 years are at higher risk for recurrent TR or TV reintervention, whereas preoperative MR deemed moderate or greater increases this risk, especially in patients older than age 12 years. There was no difference in outcomes between TV replacement and repair.

Acquired cyanosis secondary to RVOT obstruction due to pacemaker lead prolapse and vegetation in a child with ASD.

Pacemaker implantation in the paediatric population is associated with significant perioperative complications. We report a child with atrial septal defect who developed cyanosis following pacemaker implantation. An 8-year-old male child presented to us with fever of 2 months, along with cyanosis and clubbing. He underwent endocardial transvenous pacemaker implantation for congenital complete heart block. His echocardiography revealed right atrial vegetation (1 × 1 cm), moderate tricuspid regurgitation, and a prolapsed lead loop extending into the right ventricular outflow tract and pulmonary valve causing severe obstruction of pulmonary valve. He was started on empirical treatment followed by sensitive antibiotics based on culture report; however due to recurrent ventricular tachycardia he was taken for emergency surgery. He underwent closure of atrial septal defect, removal of pulse generator, removal of endocardial leads/vegetation, and pulmonary valve repair. Postoperative echocardiography revealed mild tricuspid regurgitation and resolution of pulmonic stenosis. This case presents a rare complication of severe right ventricular outflow tract obstruction following pacemaker implantation. .

A case of hypertrophic cardiomyopathy with right ventricular outflow tract and left midventricular obstruction.

We describe a case of a 59-year-old woman with hypertrophic cardiomyopathy who remained with right ventricular outflow tract obstruction after the pressure gradient in the left midventricle was resolved by a drug with a negative inotropic effect. The patient was diagnosed with hypertrophic cardiomyopathy 30 years previously and was only on low-dose beta-blocker therapy. She presented at our hospital with suspected exacerbation of heart failure because of the development and exacerbation of dyspnea and chest tightness. Transthoracic echocardiography showed an accelerated blood flow of 3 m/s in the middle of the left ventricle; thus, she was started on cibenzoline, a drug with a negative inotropic effect. After admission, intracardiac pressure measurement showed no pressure gradient in the left chamber. However, there was a pressure gradient of 18 mmHg between the apex of the right ventricle and the right ventricular outflow tract, and right ventricular outflow tract obstruction was confirmed on cardiac magnetic resonance imaging. We decided to reinforce the negative inotropic effect by adding bisoprolol, and the subjective symptoms and auscultatory systolic murmur were eliminated 2 months later. Learning objective: Hypertrophy of the right ventricular myocardium can occur in patients with hypertrophic cardiomyopathy (HCM). However, right ventricular outflow tract obstruction remains a rare finding in patients with HCM, despite the presence of morphological abnormalities such as right ventricular hypertrophy. In patients with HCM, obstruction of the right ventricle should be considered if the symptoms and auscultatory findings do not match the left ventricular imaging findings.

Results of the arterial switch operation for Taussig-Bing variants in the setting of a lower-middle income country: A single-institution experience.

BACKGROUND: This study was conducted to evaluate the surgical results of the arterial switch operation for Taussig-Bing variants, at a single institution in a lower-middle income country. METHODS: Between June 2010 and December 2018, all consecutive patients diagnosed with Taussig-Bing variants who underwent the arterial switch operation and ventricular septal defect closure were included in the study. RESULTS: A total of 72 patients of Taussig-Bing variants underwent arterial switch operation and ventricular septal defect closure. There were 10 early deaths (13.9%) and 2 late deaths (2.8%). Intraoperative ventricular septal defect enlargement (hazard ratio [HR] 7.23, 95% confidence interval [CI] 3.1294-16.7167; p < .001), secondary aortic cross-clamping (HR 28.38, 95% CI 4.8427-166.3484; p < .001), postoperative pneumonia (HR 5.64, 95% CI 1.2724-24.9917; p = .023), and postoperative sepsis (HR 5.28, 95% CI 1.3512-20.6553; p = .017) were risk factors for overall mortality by competing risk analysis. Sixty patients (83.3%) required septoparietal trabeculation division/resection during the arterial switch operation in an attempt to avoid right ventricular outflow tract obstruction. The reoperation rate for right ventricular outflow tract obstruction at last follow up was 6% (three patients). The estimated freedom from reoperation for right ventricular outflow tract obstruction at 1, 5, and 9 years was 98.3%, 91.9%, and 91.9%, respectively. CONCLUSIONS: The results of arterial switch operation for Taussig-Bing variants were satisfactory in the operative setting of a lower-middle income country, and performing extensive septoparietal trabeculation division might reduce the reintervention rate for right ventricular outflow tract obstruction in these patients.

Membranous septal aneurysm causing severe right ventricular outflow obstruction in an adult with trisomy 18.

Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.

Congenital, acquired, or both? The only two congenitally based, acquired heart diseases.

Discrete subaortic stenosis (DSS) is a type of left ventricular outflow tract obstruction whereas double-chambered right ventricle is a form of right ventricular outflow tract obstruction. Both of these cardiac malformations share lots of similar characteristics which classify them as acquired developmental heart diseases despite their congenital anatomical substrate. Both of them are frequently associated to ventricular septal defects. The initial stimulus in their pathogenetic process is anatomical abnormalities or variations. Subsequently, a hemodynamic process is triggered finally leading to an abnormal subaortic fibroproliferative process with regard to DSS or to hypertrophy of ectopic muscles as far as double-chambered right ventricle is concerned. In many cases, these pathologies are developed secondarily to surgical management of other congenital or acquired heart defects. Moreover, high recurrence rates after initial successful surgical therapy, particularly regarding DSS, have been described. Finally, an interesting coexistence of DSS and double-chambered aortic ventricle has also been reported in some cases.

Tetralogy of Fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery.

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.

Identification of Rare Variants in Right Ventricular Outflow Tract Obstruction Congenital Heart Disease by Whole-Exome Sequencing.

BACKGROUND: Pulmonary atresia (PA) is a kind of congenital heart disease characterized by right ventricular outflow tract obstruction. It is divided into PA with intact ventricular septum (PA/IVS) whose favorable form is pulmonary valvular stenosis (PS), and PA with ventricular septal defect (PA/VSD) whose favorable form is tetralogy of Fallot (TOF). Due to limitations in genetics etiology, whole-exome sequencing (WES) was utilized to identify new variants associated with the diseases. METHODS: The data from PS-PA/IVS (n = 74), TOF-PA/VSD (n = 100), and 100 controls were obtained. The common sites between PS and PA/IVS, PA/VSD and TOF, were compared. The novel rare damage variants, and candidate genes were identified by gene-based burden analysis. Finally, the enrichment analysis of differential genes was conducted between case and control groups. RESULTS: Seventeen rare damage variants located in seven genes were predicted to be associated with the PS through burden analysis. Enrichment analysis identified that the Wnt and cadherin signaling pathways were relevant to PS-PA/IVS. CONCLUSION: This study put forth seven candidate genes (APC, PPP1R12A, PCK2, SOS2, TNR, MED13, and TIAM1), resulting in PS-PA/IVS. The Wnt and cadherin signaling pathways were identified to be related to PS-PA/IVS by enrichment analysis. This study provides new evidence for exploring the genetic mechanism of PS-PA/IVS.

Right ventricular outflow tract obstruction caused by sinus of Valsalva aneurysm.

Right ventricular outflow tract (RVOT) obstruction caused by sinus of Valsalva aneurysm is a rare observation. We describe a successful case of valve-sparing root replacement using reimplantation technique for RVOT obstruction by a large right coronary sinus of Valsalva aneurysm in a 76-year-old man. In the pathological examination, the elastic fibers of the medial layer were defective not only in the wall of the aneurysmal Valsalva sinus but also in the remaining two sinus walls. Our experience illustrated that valve-sparing root replacement can be an effective procedure in such a case.