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With the advent of improved neonatal care, increasingly vulnerable higher-risk patients with complex congenital heart anomalies are presenting for intervention. This group of patients will always have a higher risk of an adverse event during a procedure but by recognizing this risk and with the introduction of risk scoring systems and thus the development of novel lower risk procedures, the rate of adverse events can be reduced. This article reviews risk scoring systems for congenital catheterization and demonstrates how they can be used to reduce the rate of adverse events. Then, novel low risk strategies are discussed for low-weight infants e.g. patent ductus arteriosus (PDA) stent insertion; premature infants e.g. PDA device closure; and transcatheter pulmonary valve replacement. Finally, how risk is assessed and managed within the inherent bias of an institution is discussed. There has been a remarkable improvement in the rate of adverse events in congenital cardiac interventions, but now, as the benchmark of mortality rate is switched to morbidity and quality of life, continued innovation into lower risk strategies and understanding the inherent bias when assessing risk will be key to continuing this improvement. [ABSTRACT FROM AUTHOR]

Right ventricular parameters change in many cardiovascular diseases; so, the presence of normal right ventricular parameters is necessary to diagnose these diseases. Ten clinically healthy adult domestic short-haired cats including six males and four females in the range of 2.70 to 4.80 kg were studied using echocardiography without sedation. The speed and pressure of blood flow through the tricuspid and pulmonary valves, speed of the movement of tricuspid valve and tricuspid annular plane systolic excursion (TAPSE) were recorded using conventional pulsed-wave (PW) Doppler, tissue Doppler imaging (TDI) and M-mode, respectively. No significant statistical differences were observed between the measured values and sex, heart rate and body weight. A positive correlation was observed between the maximum velocity of the right ventricular outflow tract and heart rate and also between TAPSE slope and body weight. With determining the normal values of PW-TDI of the right ventricle in apparently healthy domestic short-haired cats, it is expected that with providing normal reference values, the diagnosis of heart diseases especially asymptomatic heart disease be achieved as soon as possible to provide the best appropriate therapeutic management and monitoring decision about them. [ABSTRACT FROM AUTHOR]

Background: For a long time, trans-femoral venous pressure (FVP) measurement was considered a simple alternative for estimating intra-abdominal pressure (IAP). Since intravesical [IVP] and intragastric [IGP] pressure measurements are sometimes contraindicated for anatomical and pathophysiological reasons, FVP raised hopes, especially among pediatricians. Pediatric FVP validation studies have never been published; recent results from adult studies cast doubt on their interchangeability. Therefore, we compared for the first time the measurement agreement between FVP and IVP and IGP in children. Material and methods: We prospectively compared FVP with IVP and IGP, according to the Abdominal Compartment Society validation criteria. Additionally, we analyzed the agreement as a function of IAP or right heart valve regurgitation and pulmonary hypertension. Results: In a real-life PICU study design, n = 39 children were included (median age 4.8 y, LOS-PICU 23 days, PRISM III score 11). In n = 660 FVP–IGP measurement pairs, the median IAP was 7 (range 1 to 23) mmHg; in n = 459 FVP–IVP measurement pairs, the median IAP was 6 (range 1to 16) mmHg. The measurement agreement was extremely low with both established methods (FVP–IGP: r2 0.13, mean bias −0.8 ± 4.4 mmHg, limits of agreement (LOA) −9.6/+8.0, percentage error (PE) 55%; FVP–IVP: r2 0.14, bias +0.5 ± 4.2 mmHg, limit of agreement (LOA) −7.9/+8.9, percentage error (PE) 51%). No effect of the a priori defined influencing factors on the measurement agreement could be demonstrated. Conclusions: In a study cohort with a high proportion of critically ill children suffering from IAH, FVP did not agree reliably with either IVP or IGP. Its clinical use in critically ill children must therefore be strongly discouraged. [ABSTRACT FROM AUTHOR]

Tricuspid regurgitation in patients with repaired tetralogy of Fallot is an important finding with a wide spectrum of primary and secondary etiologies. Moderate or severe tricuspid regurgitation is associated with a greater incidence of atrial tachyarrhythmias in these patients. It remains uncertain which patients with repaired tetralogy of Fallot may benefit from a tricuspid valve intervention at the time of pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Right ventricle to pulmonary artery anatomic discontinuity is common in complex congenital heart malformations. Handsewn conduits are a practised method of repair. In a proof-of-concept study, we evaluated pulmonary valve replacement with a handsewn pericardial valved pulmonary conduit in vitro and in vivo. METHODS: A pulsatile flow-loop model (in vitro) and an acute 60-kg porcine model (in vivo) were used. With echocardiography and pressure catheters, baseline geometry and fluid dynamics were measured. The pulmonary valve was replaced with a handsewn glutaraldehyde-treated pericardial valved pulmonary conduit corresponding to a 21-mm prosthetic valve, after which geometric measurements and fluid dynamics were reassessed. RESULTS: In vitro, 15 pulmonary trunks at 4 l/min and 13 trunks at 7 l/min, and in vivo, 11 animals were investigated. The valved pulmonary conduit was straightforward to produce at the operating table and easy to suture in place. All valves were clinically sufficient in vitro and in vivo. The mean transvalvular pressure gradient in the native valve and the conduit was 8 mmHg [standard deviation (SD): 2] and 7 mmHg (SD: 2) at 4 l/min in vitro, 19 mmHg (SD: 3) and 17 mmHg (SD: 4) at 7 l/min in vitro and 3 mmHg (SD: 2) and 6 mmHg (SD: 3) in vivo. CONCLUSIONS: Our proof-of-concept demonstrates no early evidence of structural damage to the conduit, and the fluid dynamic data were acceptable. The handsewn conduit can be produced at the operating table. Conduits to restore the right ventricle to pulmonary artery (RV–PA) anatomic discontinuity are used routinely in complex congenital malformations that involve the right ventricular outflow tract. [ABSTRACT FROM AUTHOR]

Data on the frequency and outcome of surgical interventions as a result of adverse events (AE) encountered in the pediatric and congenital cardiac catheterization laboratory are limited. This study analyzes the outcomes of specific types of AE that are most likely to require immediate surgical intervention. Data from the C3PO registry were analyzed to identify specific types of significant vascular/cardiac trauma or technical adverse events (stent/device/coil embolization/migration). The relationship between these AE and an "adverse outcome" (defined as either surgery, ECMO, or death) were analyzed. Between 01/2014 and 12/2017, 25,731 cases were entered into the C3PO registry. Vascular or cardiac trauma were observed in 92 cases (0.36% cases in C3PO), and technical adverse events were observed in 176 cases (0.68% cases in C3PO). The two highest procedure type risk categories (PREDIC3T) accounted for 61% of the cases in the cardiac/vascular trauma cohort, and 34% in the technical AE cohort. For vascular/cardiac trauma, 24 (26%) had an adverse outcome, with ECMO in 8 (9%), surgery in 19 (20%), and death in 9 (10%). For technical AE 25 (14%) had an adverse outcome, with ECMO in 3 (2%), surgery in 23 (13%), and death in 3 (2%). Survival after cardiac surgery secondary to an AE was 68% for cardiac/vascular trauma, and 96% for technical adverse events. RF perforation of the pulmonary valve was the procedure most likely to result in cardiac/vascular trauma (10%), with 57% of those having an adverse outcome. Atrial septal interventions accounted for 29% of all adverse outcomes in the cardiac/vascular trauma cohort. Non-elective or emergent cases were associated with a significantly higher incidence of an adverse outcome for both, cardiac/vascular trauma (OR 7.1) and technical adverse events (OR 2.7). Surgery within the last 30 days was associated with a significantly higher incidence of an adverse outcome for cardiac/vascular trauma only (OR 4.2). Significant cardiac/vascular trauma or stent/device/coil embolization/migration are rare, but high consequence AE. With appropriate surgical and ECMO backup, a high survival can be achieved. The potential need for and impact of immediate surgical backup seems to be higher for cardiac/vascular trauma (in particular after specific case types), than for device/coil migration/embolization, and as such case specific backup arrangements are required. [ABSTRACT FROM AUTHOR]

Introduction: Cardiac perforation is a rare life-threatening complication of cardiac catheterization. There is very little published literature detailing risk factors for cardiac perforation and outcomes from this complication in children. Materials and Methods: This was a retrospective study analyzing the cardiac catheterization case registry of the Congenital Cardiovascular Interventional Study Consortium. Children aged < 18 years were included during the study period of 9 years (January 2009–December 2017). The primary outcome measures were incidence of cardiac perforation, risk factors for and outcomes of patients who experience cardiac perforation during cardiac catheterization. Results: Cardiac perforation occurred in 50 patients from a total of 36,986 (0.14%). Cardiac perforation was more likely to occur in younger, smaller patients undergoing urgent/emergent and interventional procedures (p < 0.01). Cardiac peroration risk was significantly different across diagnostic and procedure categories (p < 0.01). Higher CRISP score (Area Under Curve [AUC] = 0.87), lower age, and procedure category (radiofrequency perforation of pulmonary valve, AUC = 0.84) were independent predictors of cardiac perforation. Cardiac perforation was associated with a significantly higher rate of mortality (14%), further emergency procedure (42%), ECMO (14%), and cardiac arrest (6%), p < 0.01. Conclusions: Cardiac perforation during cardiac catheterization is a life-threatening complication with a range of associated secondary complications. Higher CRISP score, lower age, and radiofrequency perforation of pulmonary valve are independent predictors. [ABSTRACT FROM AUTHOR]

Objective: This study sought to assess the prenatal features and clinical outcomes of cases with a fetal diagnosis of tetralogy of Fallot (TOF) with an absent pulmonary valve (APV) at our maternal-fetal medicine unit. Methods: Twelve cases of TOF and APV prenatally diagnosed at Kanuni Sultan Süleyman Training and Research Hospital between 2015 and 2020 were retrospectively reviewed. Prenatal characteristics, additional cardiac and extracardiac anomalies, and postnatal outcomes of the cases were examined. Results: The median gestational age at diagnosis was 22 weeks (range: 18-24 weeks). The absence of ductus arteriosus was found in all cases (100%). Karyotype analysis was performed in 5 cases. A chromosomal abnormality was detected in 3 of these cases (60%); 2 cases with 22q11 microdeletion, and 1 case with trisomy 21. Parents opted for termination of pregnancy in two of these cases; 1 case with 22q11 microdeletion, and 1 case with trisomy 21. Two patients experienced spontaneous intrauterine fetal demise. Finally, 8 live-born fetuses underwent total correction surgery during the postnatal period. Four (33.3%) out of 12 cases survived at the end of the 4-years follow-up period. Conclusion: While TOF with APV cases were predominantly associated with poor prognoses in the past, more promising results have been obtained in recent years in parallel with the developments in surgery and postnatal care. For this reason, we think that the prognosis will be even better in the coming years as the developments in surgical technique and care continue. [ABSTRACT FROM AUTHOR]

Metabolic programming may be induced by reduction or enhancement of litter size, which lead to neonatal over or undernutrition, respectively. Changes in neonatal nutrition can challenge some regulatory processes in adulthood, such as the hypophagic effect of cholecystokinin (CCK). In order to investigate the effects of nutritional programming on the anorexigenic function of CCK in adulthood, pups were raised in small (SL, 3 pups per dam), normal (NL, 10 pups per dam), or large litters (LL, 16 pups per dam), and on postnatal day 60, male rats were treated with vehicle or CCK (10 µg/Kg) for the evaluation of food intake and c-Fos expression in the area postrema (AP), nucleus of solitary tract (NTS), and paraventricular (PVN), arcuate (ARC), ventromedial (VMH), and dorsomedial (DMH) nuclei of the hypothalamus. Overnourished rats showed increased body weight gain that was inversely correlated with neuronal activation of PaPo, VMH, and DMH neurons, whereas undernourished rats had lower body weight gain, inversely correlated with increased neuronal activation of PaPo only. SL rats showed no anorexigenic response and lower neuron activation in the NTS and PVN induced by CCK. LL exhibited preserved hypophagia and neuron activation in the AP, NTS, and PVN in response to CCK. CCK showed no effect in c-Fos immunoreactivity in the ARC, VMH, and DMH in any litter. These results indicate that anorexigenic actions, associated with neuron activation in the NTS and PVN, induced by CCK were impaired by neonatal overnutrition. However, these responses were not disrupted by neonatal undernutrition. Thus, data suggest that an excess or poor supply of nutrients during lactation display divergent effects on programming CCK satiation signaling in male adult rats. • Body weight is inversely correlated to neuron activity in hypothalamic nuclei. • Opposite neonatal nutrition supply has divergent effects on CCK actions in adulthood. • CCK-induced anorexigenic effect is abolished in overnourished rats. • Neonatal undernourished rats have preserved CCK satiation signaling. • SL rats have lower CCK-induced NTS and PVN neuron activation. [ABSTRACT FROM AUTHOR]

Carcinoid heart disease (CHD) is the main complication of carcinoid syndrome (CS) associated with metastatic small intestine neuroendocrine tumours (NETs). The pathophysiology of CHD is partly understood but vasoactive hormones secreted by NETs, especially serotonin, play a major role, leading to the formation of fibrous plaques. These plaque‐like deposits involve the right side of the heart in >90% of cases, particularly the tricuspid and pulmonary valves, which become thickened, retracted and immobile, resulting in regurgitation or stenosis. CHD represents a major diagnostic and therapeutic challenge for patients with NET and CS and is associated with increased risk of morbidity and mortality. CHD often occurs 2–5 years after the diagnosis of metastatic NET, but diagnosis of CHD can be delayed as patients are often asymptomatic for a long time despite severe heart valve involvement. Circulating biomarkers (5HIAA, NT‐proBNP) are relevant tools but transthoracic echocardiography is the key examination for diagnosis and follow‐up of CHD. However, there is no consensus on the optimal indications and frequency of TTE and biomarker dosing regarding screening and diagnosis. Treatment of CHD is complex and requires a multidisciplinary approach. It relies on antitumour treatment, control of CS and surgical valve replacement in cases of severe CHD. However, cardiac surgery is associated with a high risk of mortality, notably due to perioperative carcinoid crisis and right ventricular dysfunction. Timing of surgery is the most crucial point of CHD management and relies on the case‐by‐case determination of the optimal compromise between tumour progression, cardiac symptoms and CS control. [ABSTRACT FROM AUTHOR]

Background: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma. Methods: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively. Results: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively. Conclusions: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence. [ABSTRACT FROM AUTHOR]

Background: There are few case reports in which non-cardiac surgery under regional anesthesia has been described in a patient with a pulmonary artery aneurysm. We report a case in which we managed a 74-year-old lady with a hip fracture for fixation under spinal anesthesia after thorough evaluation and meticulous planning. The patient was discharged home after 5 days without any perioperative events. Case presentation: A 74-year-old, hypertensive lady with an incidentally detected pulmonary artery aneurysm was posted for a dynamic hip screw. Two-dimensional echocardiography revealed normal biventricular function with an ejection fraction of 60%, no regional wall motion abnormalities, concentric right ventricular hypertrophy (tricuspid annular plane systolic excursion or TAPSE of 19 mm), severe calcific pulmonary stenosis (PS) with pressures 90/61 mm Hg, pulmonary valve annulus of 12 mm, and pulmonary artery systolic pressure of 35 mm Hg. The left and right pulmonary artery diameters were 17 mm and 20 mm, respectively. She was clinically asymptomatic with an oxygen saturation of 98% on room air. A sequential lumbar epidural anesthesia was planned for this patient given her age, overall frail condition, and to avoid airway instrumentation. As epidural was difficult due to fused spaces, a sub-arachnoid block was administered with continuous monitoring. Surgery was uneventful and the patient was discharged on the 5th postoperative day. Conclusions: With this case report, we suggest that a central neuraxial blockade could be administered in presence of severe PS with meticulous monitoring and careful planning. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to evaluate whether fetal echocardiographic parameters were predictive of the postnatal surgical treatment required for fetuses with Tetralogy of Fallot (TOF). Methods: The fetal echocardiographic and postnatal clinical data of all cases of prenatal TOF at Xinhua Hospital from 2016 to 2020 were reviewed. Patients were categorized based on the operation type, and cardiac parameters were compared between groups. Results: Of the 37 fetuses assessed, the development of the pulmonary valve annulus (PVA) was significantly poorer in the transannular patch group. Patients with a prenatal PVA z‐score (Schneider's method) ≥ –2.645, a PVA z‐score (Lee's method) ≥ –2.805, a PVA/aortic valve annulus diameter ratio ≥.697, and a pulmonary annulus index ≥.823 were more likely to undergo pulmonary valve‐sparing surgery. There was a strong correlation between prenatal and postnatal PVA z‐scores. The PVA growth potential was greater in the pulmonary valve‐sparing surgery group. Conclusions: PVA‐related parameters evaluated by fetal echocardiography can predict the type of surgical intervention required and are valuable in improving prenatal counseling in fetal cases of TOF. [ABSTRACT FROM AUTHOR]

This article discusses the challenges of supporting a successful pregnancy in a woman with multiple prosthetic heart valves and a complicated cardiac history, from both the patient and provider perspective. The patient is a 29-year-old female with truncus arteriosus type I with initial neonatal VSD closure and right ventricular to pulmonary artery conduit. At the age of 13, she subsequently required truncal and pulmonary valve replacements with mechanical prostheses. Standardizing an approach to anticoagulation in pregnancy in women with prosthetic heart valves is not always possible. Her story demonstrates the importance of an innovative approach to unique cases; by extrapolating what is known about pregnancy and prosthetic heart valves, cardiologists can provide the best outcomes. Simultaneously, non-directive counseling is essential throughout this period to engage the patient in shared decision-making when balancing risks and benefits of each approach to anticoagulation. [ABSTRACT FROM AUTHOR]

The purpose of this work is to present a patient‐specific (PS) modeling approach for simulating percutaneous transluminal angioplasty (PTA) endovascular treatment and assessing the balloon sizing influence on short‐term outcomes in peripheral arteries, i.e. without stent implantation. Two 3D PS stenosed femoral artery models, one with a dominant calcified atherosclerosis while the other with a lipidic plaque, were generated from pre‐operative computed tomography angiography images. Elastoplastic constitutive laws were implemented within the plaque and artery models. Implicit finite element method (FEM) was used to simulate the balloon inflation and deflation for different sizings. Besides vessel strains, results were mainly evaluated in terms of the elastic recoil ratio (ERR) and lumen gain ratio (LGR) attained immediately after PTA. Higher LGR values were shown within the stenosed region of the lipidic patient. Simulated results also showed a direct and quantified correlation between balloon sizing and LGR and ERR for both patients after PTA, with a more significant influence on the lumen gain. The max principal strain values in the outer arterial wall increased at higher balloon sizes during inflation as well, with higher rates of increase when the plaque was calcified. Results show that our model could serve in finding a compromise for each stenosis type: maximizing the achieved lumen gain after PTA, but at the same time without damaging the arterial tissue. The proposed methodology can serve as a step toward a clinical decision support system to improve angioplasty balloon sizing selection prior to the surgery. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Our goal was to evaluate the risk-adjusted effects of operative and non-operative repair on long-term outcomes in patients with congenitally corrected transposition of the great arteries and left ventricular outflow tract obstruction (CCTGA/LVOTO). METHODS We retrospectively reviewed 391 patients with CCTGA/LVOTO from 2001 to 2020 (operative group, 282; non-operative group, 109) in 3 centres in China. The operative group included 73 patients who underwent anatomical repair and 209 patients who underwent non-anatomical repair. The median follow-up time was 8.5 years. The inverse probability of treatment weighted-adjusted Cox regression and a Kaplan–Meier analysis were used to evaluate long-term outcomes. RESULTS Operative repair did not reduce the hazard ratio (HR) of death, tricuspid regurgitation or New York Heart Association functional class III/IV, but a significantly increased HR was observed for pulmonary valve regurgitation [HR, 2.84; 95% confidence interval (CI), 1.10–7.33; P  = 0.031]. Compared with that in the non-operative group, anatomical repair resulted in significantly increased HRs for death (HR, 2.94; 95% CI, 1.10–7.87; P  = 0.032) and pulmonary valve regurgitation (HR, 9.71; 95% CI, 3.66–25.77; P  < 0.001). Subgroup analysis showed that in patients with CCTGA/LVOTO with moderate or worse tricuspid regurgitation (≥ moderate), anatomical repair significantly reduced the HR of death. An inverse probability of treatment weighting-adjusted Kaplan–Meier analysis showed that the survival rates at 5 and 10 days postoperatively were 88.2 ± 4.0% and 79.0 ± 7.9%, respectively, in the anatomical repair group; these rates were significantly lower than the rates in the non-operative group (95.4 ± 2.0% and 91.8 ± 2.8%; P  = 0.032). CONCLUSIONS For patients with CCTGA/LVOTO, operative repair does not provide superior long-term outcomes, and anatomical repair results in a higher incidence of death. However, in patients with CCTGA/LVOTO with tricuspid regurgitation ≥ moderate, anatomical repair can reduce the risk of death in the long term. [ABSTRACT FROM AUTHOR]

Background: The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). Methods: A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. Results: All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed underexpanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3-8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8-94.7) and 83.5% (95%CI 73.8-93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. Conclusion: TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture. [ABSTRACT FROM AUTHOR]

We report a three-year-old male child who presented with congenital valvular defects, right ventricular malformation, and initial developmental delay. Genome sequencing showed rare deleterious biallelic missense variants in PLD1. In his parents' second pregnancy, echocardiogram at 13 weeks gestation revealed right-sided cardiac malformations resembling the clinical presentation of the family's first child. Targeted DNA analysis showed that the fetus carried the same biallelic PLD1 variants as their older sibling. This case helps to further delineate the clinical spectrum of PLD1-related defects and highlights the value of both genome sequencing in congenital heart disease and early fetal echocardiography to establish phenotype. [ABSTRACT FROM AUTHOR]

Background: Living well is as important as living longer. The objective of this study is to assess quality of life (QoL) in congenital heart disease (CHD) according to current AHA/ACC anatomical and physiological classifi- cation. Methods: Cross-sectional study examining the World Health Organization QoL Bref questionnaire (WHOQoL-Bref) in consecutive outpatient CHD patients from a single unit. Results: 191 CHD patients were studied. Median age was 28 ± 13 years and 59% were male. 44 (23%), 115 (60%) and 33 (17%) CHD patients showed mild, moderate and great anatomical defects respectively while 69 (36%) patients were in physiological Stage A, 27 (14%) in Stage B, 84 (44%) in Stage C and 11 (6%) in Stage D. No significant differences were seen in relation the anatomical classification and the different sections of the WHOQoL-Bref questionnaire. CHD patients in Stages C and D had significant lower physical domain scores than patients in the Stage A (p < 0.05). However, no significant differences were seen in the psychological, social relationships and environmental domains. The binary logistic regression analysis showed that having a higher educational level was a protective factor [OR 0.32 (95% CI, 0.12-0.87), p = 0.026] while being married or cohabit was a risk factor [OR 3.46 (95% CI, 1.13-10.63), p = 0.030] for having a worse rated QoL. Meanwhile, having a worse functional class (NYHA ≥2) [OR 3.44 (95% CI, 1.20-9.81), p = 0.021] was associated with dissatisfaction with health. Conclusion: Patients with advanced physiological stages scored lower on the physiological domain. No statistical significance was seen, according to the anatomical and physiological classification, in the psychological, social relationship and environmental domains. [ABSTRACT FROM AUTHOR]

Background: The severity of pectus excavatum is classified by the Haller Index (HI) and/or Correction Index (CI). These indices measure only the depth of the defect and, therefore, impede a precise estimation of the actual cardiopulmonary impairment. We aimed to evaluate the MRI-derived cardiac lateralization to improve the estimation of cardiopulmonary impairment in Pectus excavatum in connection with the Haller and Correction Indices. Methods: This retrospective cohort study included a total of 113 patients (mean age = 19.03 ± 7.8) with pectus excavatum, whose diagnosis was verified on cross-sectional MRI images using the HI and CI. For the development of an improved HI and CI index, the patients underwent cardiopulmonary exercise testing to assess the influence of the right ventricle's position on cardiopulmonary impairment. The indexed lateral position of the pulmonary valve was utilized as a surrogate parameter for right ventricle localization. Results: In patients with PE, the heart's lateralization significantly correlated with the severity of pectus excavatum (p ≤ 0.001). When modifying HI and CI for the individual's pulmonary valve position, those indices are present with greater sensitivity and specificity regarding the maximum oxygen-pulse as a pathophysiological correlate of reduced cardiac function (χ2 10.986 and 15.862, respectively). Conclusion: The indexed lateral deviation of the pulmonary valve seems to be a valuable cofactor for HI and CI, allowing for an improved description of cardiopulmonary impairment in PE patients. [ABSTRACT FROM AUTHOR]

Aortic valve replacement early in life may be inevitable. Ross operation, until present day, remains the favorite surgical option in pediatrics with irreparable aortic valve disease. Nonetheless, the necessity for re-operation was always its principal limitation due to aortic valve failure or homograft degeneration. We present our 25 years of experience in the pediatric population. From August 1994 until June 2018, 157 children below 18 years underwent the Ross operation. This retrospective review aims at assessing the long-term outcomes, as well as the risk factors for re-operation after Ross procedure. Median age was 10.9 years, of which seven patients were infants, 79 children, and 71 adolescents. The median follow-up time was 14 years. Hospital mortality was 0.6%. Freedom from autograft re-operation for children was 96.7% and 94.1% at 10 and 20 years, respectively; whereas for adolescents, it was 92.6% and 74.9% at 10 and 20 years. For children, freedom from homograft re-operation was 92.5%, 83.5%, and 56.2% at 10, 15, and 20 years; while for adolescents, it was 96.8%, 91.8%, and 86.7% at 10, 15, and 20 years. Homograft size (p = 0.008) and childhood (p = 0.05) were risk factors for homograft re-operation. Pulmonary valve diameter > 24 mm (p = 0.044) and adolescence (p = 0.032) were risk factors for autograft re-operation. Our experience demonstrated excellent early and late survival. While children have preferential outcomes concerning autograft re-operation, those who received a smaller homograft had a higher right-sided re-intervention incidence than adolescents. Pulmonary diameter > 24 mm at surgery was an indicator of future autograft failure. [ABSTRACT FROM AUTHOR]

Objective: This review aims to make a brief overview of blood biomarkers' clinical decision limits, possibly aiding in outcome prediction in all-aged Tetralogy of Fallot patients. Potentially, these biomarkers could also assist in necessity and timing of pulmonary valve replacement. Methods: Studies with all-aged patients with Tetralogy or Pentalogy of Fallot and blood biomarkers, BNP, NT-proBNP and hs-cTn, usage in clinical outcome prediction were included. Additionally, pulmonary valve replacement indications were considered. Other congenital heart diseases, biomarkers irrelevant to clinical outcome and associated pathologies or physiological status were the exclusion criteria. Keywords, Tetralogy and Pentalogy of Fallot, pulmonary valve replacement, blood biomarkers, yielded 69 suitable studies from Google Scholar, PubMed and Web-of-Science. 30 studies were selected. Results: Blood biomarkers were increased in TOF patients in comparison to controls; the higher the values, the worse adverse outcomes. Blood biomarkers combined with other biomarkers, imagistic methods or parameters showed promising results in outcome prediction. Conclusions: Blood biomarkers are validated as follow-up predictors in congenital heart disease paediatric patients. Further research is required to establish age-appropriate clinical decision limits. Pulmonary valve replacement timing remains controversial. [ABSTRACT FROM AUTHOR]

The inclusion technique was developed to reinforce the pulmonary autograft to prevent dilation after the Ross procedure. Anticommissural plication (ACP), a modification technique, can reduce graft size and create neosinuses. The objective was to evaluate pulmonary valve biomechanics using the inclusion technique in the Ross procedure with and without ACP. Seven porcine and 5 human pulmonary autografts were harvested from hearts obtained from a meat abattoir and from heart transplant recipients and donors, respectively. Five additional porcine autografts without reinforcement were used as controls. The Ross procedure was performed using the inclusion technique with a straight polyethylene terephthalate graft. The same specimens were tested both with and without ACP. Hemodynamic parameter data, echocardiography, and high-speed videography were collected via the ex vivo heart simulator. Porcine autograft regurgitation was significantly lower after the use of inclusion technique compared with controls (P <.01). ACP compared with non-ACP in both porcine and human pulmonary autografts was associated with lower leaflet rapid opening velocity (3.9 ± 2.4 cm/sec vs 5.9 ± 2.4 cm/sec; P =.03; 3.5 ± 0.9 cm/sec vs 4.4 ± 1.0 cm/sec; P =.01), rapid closing velocity (1.9 ± 1.6 cm/sec vs 3.1 ± 2.0 cm/sec; P =.01; 1.8 ± 0.7 cm/sec vs 2.2 ± 0.3 cm/sec; P =.13), relative rapid opening force (4.6 ± 3.0 vs 7.7 ± 5.2; P =.03; 3.0 ± 0.6 vs 4.0 ± 2.1; P =.30), and relative rapid closing force (2.5 ± 3.4 vs 5.9 ± 2.3; P =.17; 1.4 ± 1.3 vs 2.3 ± 0.6; P =.25). The Ross procedure using the inclusion technique demonstrated excellent hemodynamic parameter results. The ACP technique was associated with more favorable leaflet biomechanics. In vivo validation should be performed to allow direct translation to clinical practice. [Display omitted] [ABSTRACT FROM AUTHOR]

• IE is a feared complication of TPVI that affects valve durability and outcomes. • IE following TPVI in CHD exhibits several distinctive features. • Several risk factors are associated with IE. • Patient and parent education on IE prevention should be provided. The introduction of transcatheter pulmonary valve implantation (TPVI) has greatly benefited the management of right ventricular outflow tract dysfunction. Infective endocarditis (IE) is a feared complication of TPVI that affects valve durability and patient outcomes. Current recommendations provide only limited guidance on the management of IE after TPVI (TPVI-IE). This article, by a group of experts in congenital heart disease in children and adults, interventional cardiology, infectious diseases including IE, and microbiology, provides a comprehensive review of the current evidence on TPVI-IE, including its incidence, risk factors, causative organisms, diagnosis, and treatment. The incidence of TPVI-IE varies from 13–91/1000 person-years for Melody valves to 8–17/1000 person-years for SAPIEN valves. Risk factors include history of IE, DiGeorge syndrome, immunosuppression, male sex, high residual transpulmonary gradient and portal of bacteria entry. Staphylococci and streptococci are the most common culprits, whereas Staphylococcus aureus is associated with the most severe disease. In addition to the modified Duke criteria, a high residual gradient warrants a strong suspicion. Imaging studies are helpful for the diagnosis. Intravenous antibiotics guided by blood culture results are the mainstay of treatment. Invasive re-intervention may be required. TPVI-IE in patients with congenital heart disease exhibits several distinctive features. Whether specific valve types are associated with a higher risk of TPVI-IE requires further investigation. Patient and parent education regarding IE prevention may have a role to play and should be offered to all patients. [ABSTRACT FROM AUTHOR]

This study hypothesized that imaging provides information indicating the right ventricular (RV) involvement after anterior or inferior ST-elevation myocardial infarction (STEMI), beyond standard electrocardiogram (ECG) due to the increasing interest in RV function and assessment techniques. This study aimed to compare RV function between anterior and inferior MI without RV involvement using different echocardiographic modalities. This study included 100 patients with anterior (50 patients) and inferior (50 patients) STEMI, who underwent primary percutaneous coronary intervention (PPCI) and two-dimensional echocardiographic imaging within 24 h after PPCI with RV function analysis by left ventricular (LV) infarct size, LV filling pressure, and RV strain rate. Our primary endpoint was the subclinical RV dysfunction in anterior or inferior MI using tissue Doppler and speckle tracking (STE). The study population included 80 (80%) males and 20 (20%) females. Patients with the anterior STEMI had higher mean creatine kinase-MB (CKMB) and troponin than those with inferior STEMI. This study revealed worse RV dysfunction in patients with anterior than those with inferior STEMI, as reflected by significantly lower RV systolic function, tricuspid annular plane systolic excursion (p ≤ 0.0001), tissue Doppler-derived velocity (p ≤ 0.0001), and STE-derived strain magnitude and rate (p ≤ 0.0001). RV dysfunction occurs in patients without ECG evidence of RV STEMI. RV dysfunction is worse in anterior than inferior MI. Moreover, RV systolic functions were affected by declined LV ejection fraction irrespective of the infarction site, which clinically implies prognostic, treatment, survival rate, and outcome improvement between both conditions. (Trial registration ZU-IRB#:4142/26–12-2017 Registered 26 December 2017, email: IRB_123@medicine.zu.edu.eg). [ABSTRACT FROM AUTHOR]

Dextrocardia with situs inversus totalis is a rare congenital condition. We report herein a first experience of video-assisted minimally invasive mitral and pulmonary valve replacement through right anterior mini-thoracotomy as reoperation in patient with this complex anomaly. The good clinical and cosmetic results demonstrate that this innovative technique can be safely performed even in difficult anatomical conditions.

Agenesis of the aortic and pulmonary valves is a very rare congenital malformation of the semilunar valves. The literature describes no more than thirty cases of such anomaly in combination with congenital heart disease. Most descriptions include aplasia of either the aortic or pulmonic valve. The combination of such defect in both valves has been described in a much smaller number of scientific papers.In this article, we present a clinical case of the treatment of a patient with agenesis of aortic valve and severely hypoplastic pulmonary valve. As a result circulatory arrest occurred immediately after birth, which required the implementation of cardiopulmonary resuscitation. The child was resuscitated and transferred to the intensive care unit for further examination and treatment.

The pulmonary valve is the most frequently replaced cardiac valve in congenital heart diseases. Whether the valve alone or part of the right ventricular outflow tract have to be repaired or replaced depends on the specific pathological anatomy of the malformation. Once the decision to replace the pulmonary valve has been made, two options are available: the isolated transcatheter pulmonary valve replacement and the surgical implantation of a prosthetic valve either isolated or in combination with a procedure on the right ventricular outflow tract. In this paper, we will focus on the different past and present surgical options and present a new concept called “endogenous tissue restoration,” a promising alternative to the hitherto existing implants. From a general point of view, neither the transcatheter nor the surgical valvular implants are magic bullets in the arsenal for the management of valvular diseases. Smaller valves have to be frequently replaced because of outgrowth of the patients, larger tissue valves may present late structural valve deterioration, while xenograft and homograft conduits may calcify and therefore become narrowed within unpredictable incidence and interval following implantation. Based on long-term research efforts combining the knowledge of supramolecular chemistry, electrospinning, and regenerative medicine, endogenous tissue restoration has emerged most recently as a promising option to create long-term functioning implants. This technology is appealing because following resorption of the polymer scaffold and timely replacement through autologous tissue, no foreign material remain at all in the cardiovascular system. Proof-of-concept studies as well as small first-in-man series have been completed and have demonstrated favorable anatomic and hemodynamic results, comparable to currently available implants in the short term. Based on the initial experience, important modifications to improve the pulmonary valve function have been initiated.

Risk-benefit assessment Pre-emptive catheter ablation for repaired TOF before native RVOT transcatheter PVR is reasonable in the context of conventional risk/benefit analysis. Catheter ablation of monomorphic ventricular tachycardia for repaired Tetralogy of Fallot Catheter ablation of monomorphic VT for repaired TOF is unlike other forms of congenital heart disease. [Extracted from the article]

Pulmonary valve (PV) fibroelastomas are a rare pathology, with limited anecdotal literature surrounding them. Consequently, the natural history is unclear; however, two features have remained salient; they are asymptomatic and found incidentally. Here, we describe a 52-year-old female, presenting with symptoms suggestive pulmonary embolism (PE). Pulmonary angiography revealed a filling deficit in the pulmonary trunk (PT), adjacent to the PV. Subsequent investigation found a large PV fibroelastoma. The presence of symptoms is likely secondary to right ventricular outflow tract obstruction from the lesions large size. We describe our investigation and management of the lesion. The reporting of this case challenges the existing knowledge of PV fibroelastomas. [ABSTRACT FROM AUTHOR]

Clinical data: Infant, 11-month-old, male, diagnosis of Tetralogy of Fallot with retrotracheoesophageal course of the brachiocephalic vein. Usual findings of Tetralogy of Fallot on physical examination. Technical description: Chest radiography showed slightly reduced pulmonary vascular markings and no cardiomegaly. Normal preoperative electrocardiogram with postoperative right bundle branch block. Usual findings of Tetralogy of Fallot on echocardiogram. Postoperative computed tomography angiography confirmed left brachiocephalic vein with anomalous retrotracheoesophageal course, configuring a U-shaped garland vein, in addition to postoperative findings of total correction of Tetralogy of Fallot. Operation: Complete surgical repair was performed with pulmonary valve commissurotomy and placement of bovine pericardial patch to solve right ventricular outflow tract obstruction, pulmonary trunk enlargement, and ventricular septal defect closure. Comments: Systemic venous drainage may show variations in patients with Tetralogy of Fallot. These abnormalities are usually of little clinical relevance, as they are asymptomatic. We presented a rare case of retrotracheoesophageal course of an anomalous left brachiocephalic vein with intraoperative diagnosis, confirmed by imaging during postoperative follow-up, without compromising clinical management or surgical approach. [ABSTRACT FROM AUTHOR]

Abstract Background Cardiac papillary fibroelastoma (PFE) is a rare tumor, and especially rare when found on the pulmonary valve. Case presentation We report the case of a 70-year-old woman patient with a pulmonary valve PFE diagnosed incidentally during a follow-up of aortic regurgitation. Computed tomography and magnetic resonance imaging showed no suggestive signs of malignant tumors, and thrombus or myxoma was initially suspected. However, an initial transthoracic and transesophageal echocardiogram did not exclude the possibility of a malignant tumor attached to the wall of the pulmonary artery. Considering the embolization risk, we opted to perform tumorectomy, in which additional surgical procedures could then be conducted if intraoperative diagnosis showed a malignant tumor. Indeed, intraoperative findings showed the tumoral mass attached on the left semilunar cusp of the pulmonary valve, and intraoperative diagnosis of the tumor showed no malignancy. Planned tumorectomy was performed concomitantly with AVR. The pathologic examination of the removed tumor confirmed the diagnosis of PFE. Her postoperative course was uneventful without any sign of recurrence. Conclusion This case highlights the difficulty of accurate diagnostic imaging and provides valuable insight into a successful surgical treatment of pulmonary valve PFE without any complications.

The article discusses the use of point-of-care ultrasound (POCUS) in horses with acute gastrointestinal disease. The CRASH protocol, which is a time-sensitive and goal-oriented assessment of the cardiorespiratory system, is described. The protocol consists of seven windows that can be used to identify cardiovascular and respiratory abnormalities. The article presents case studies where the CRASH protocol was used to assess and monitor horses with acute gastrointestinal disease, and it concludes that the protocol can assist in triage and identify cardiorespiratory abnormalities. However, it also notes that a normal CRASH examination does not exclude the need for further evaluation. [Extracted from the article]

Rheumatic heart disease (RHD) is a widespread illness in developing countries. RHD causes 99% of mitral stenoses in adults and 25% of aortic regurgitation. However, it only causes 10% of stenoses of the tricuspid valve, and is almost always associated with left-side valvular lesions. Isolated right-side valves are rarely affected, but may result in severe rheumatic pulmonary regurgitation. Herein, we present a case of rheumatic right-sided valve disease with severe pulmonary valve contracture and regurgitation in a symptomatic patient, successfully managed by surgical valvular reconstruction with a tailored bileaflet bovine pericardial patch. The options for surgical approach are also discussed. To our knowledge, the presented rheumatic right-sided valve disease with severe pulmonary regurgitation is the first to be reported in the literature.

Background: Our purpose was to evaluate the correlations between right ventricular (RV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) in patients with tetralogy of Fallot (ToF) scheduled for pulmonary valve replacement (PVR) and post-PVR functional data. Methods: We retrospectively reviewed ToF patients scheduled for PVR who underwent two CMR examinations at our institution, one before the procedure (CMR-0), including contrast-enhanced sequences, and one after the procedure (CMR-1). Functional left and RV data were obtained by segmenting short-axis stacks on both CMR examinations, and normalised variations were calculated by dividing differences between CMR-1 and CMR-0 by the intercurring time interval, whereas the RV scar burden was assessed on CMR-0 LGE sequences both semiquantitatively and quantitatively. Data were reported as median and interquartile range, differences were appraised with the Mann–Whitney U test, while correlations were assessed with Spearman's ρ. Results: Fifteen patients with a median age of 25 years (16–29), including 9 (60%) males, with a median time interval between CMR-0 and CMR-1 of 17 months (12–23), were retrospectively reviewed. The semiquantitative LGE score at CMR-0 was 7 (6–9), and LGE volume was 4.49 mL (3.70–5.78), covering 5.63% (4.92–7.00) of the RV. RV LGE score showed a moderate positive correlation with the normalised variation of RV stroke volume (ρ = 0.662, p = 0.007) and a borderline moderate positive correlation with the normalised variation of RV end-diastolic indexed volume (ρ = 0.513, p = 0.050). Conclusions: The assessment of RV LGE before PVR may provide insights on post-PVR functional data, potentially facilitating a patient-tailored treatment pathway. [ABSTRACT FROM AUTHOR]

Right ventricle outflow tract obstruction (RVOTO) is a congenital pathological condition that contributes to about 15% of congenital heart diseases. In most cases, the replacement of the right ventricle outflow in pediatric age requires subsequent pulmonary valve replacement in adulthood. The aim of this study was to investigate the extracellular matrix scaffold obtained by decellularization of the porcine pulmonary valve using a new detergent (Tergitol) instead of Triton X-100. The decellularized scaffold was evaluated for the integrity of its extracellular matrix (ECM) structure by testing for its biochemical and mechanical properties, and the cytotoxicity/cytocompatibility of decellularized tissue was assessed using bone marrow-derived mesenchymal stem cells. We concluded that Tergitol could remove the nuclear material efficiently while preserving the structural proteins of the matrix, but without an efficient removal of the alpha-gal antigenic epitope. Therefore, Tergitol can be used as an alternative detergent to replace the Triton X-100. [ABSTRACT FROM AUTHOR]

Current indications for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) rely on cardiovascular magnetic resonance (CMR) image-based indices but are inconsistently applied, lead to mixed outcomes, and remain debated. This study aimed to test the hypothesis that specific markers of biventricular shape may discriminate differences between rTOF patients who did and did not require subsequent PVR better than standard imaging indices. In this cross-sectional retrospective study, biventricular shape models were customized to CMR images from 84 rTOF patients. A statistical atlas of end-diastolic shape was constructed using principal component analysis. Multivariate regression was used to quantify shape mode and imaging index associations with subsequent intervention status (PVR, n = 48 vs. No-PVR, n = 36), while accounting for confounders. Clustering analysis was used to test the ability of the most significant shape modes and imaging indices to discriminate PVR status as evaluated by a Matthews correlation coefficient (MCC). Geometric strain analysis was also conducted to assess shape mode associations with systolic function. PVR status correlated significantly with shape modes associated with right ventricular (RV) apical dilation and left ventricular (LV) dilation (p < 0.01), RV basal bulging and LV conicity (p < 0.05), and pulmonary valve dilation (p < 0.01). PVR status also correlated significantly with RV ejection fraction (p < 0.05) and correlated marginally with LV end-systolic volume index (p < 0.07). Shape modes discriminated subsequent PVR better than standard imaging indices (MCC = 0.49 and MCC = 0.28, respectively) and were significantly associated with RV and LV radial systolic strain. Biventricular shape modes discriminated differences between patients who did and did not require subsequent PVR better than standard imaging indices in current use. These regional features of cardiac morphology may provide insight into adaptive vs. maladaptive types of structural remodeling and point toward an improved quantitative, patient-specific assessment tool for clinical use. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977–1991), intermediate (1992–2006) and late (2007–2021). RESULTS The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7–30.5). There was a temporal trend towards less shunt palliation (–0.3% per year, 95% CI –0.05 to –0.1). Median age at intracardiac repair was 2.9 years (1.8–5.0), 0.8 years (0.5–1.3) and 0.5 years (0.4–0.7) (P  < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (–0.7% per year, 95% CI –0.5 to –1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5–1.0). Survival at 10 years was 79% (64–76), 90% (87–93) and 95% (92–98) (P  < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1–6), 12% (8–16) and 21% (13–29) (P  < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair. [ABSTRACT FROM AUTHOR]

Aims. Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by catheter-based interventions and complemented by various surgical procedures. We aim to determine a long-term treatment strategy to enable patients to be surgery free, depending solely on percutaneous interventions. Methods and Results. We selected five patients from among a cohort of patients with PA/IVS treated at birth with radiofrequency perforation and dilatation of the pulmonary valve. Patients had reached a pulmonary valve annulus of 20 mm or larger on their biannual echocardiographic follow-up, with right ventricular dilatation. The findings, together with the right ventricular outflow tract and pulmonary arterial tree, were confirmed by multislice computerised tomography. Based on the angiographic size of the pulmonary valve annulus, all patients were successfully implanted with either Melody® or Edwards® pulmonary valves percutaneously, regardless of their small weights and ages. No complications were encountered. Conclusion. We managed to stretch the age and weight limitations for performing percutaneous pulmonary valve implantation (PPVI): interventions were attempted whenever a pulmonary annulus size of >20 mm was reached, which was rationalised by the prevention of progressive right ventricular outflow tract dilatation and accommodating valves between 24 and 26 mm, which is enough to sustain a normal pulmonary flow in adulthood. [ABSTRACT FROM AUTHOR]

Clinical observation indicates that exercise capacity, an important determinant of survival in patients with congenital heart disease (CHD), is most decreased in children with reduced pulmonary blood flow (RPF). However, the underlying mechanism remains unclear. Here, we obtained human RPF lung samples from children with tetralogy of Fallot as well as piglet and rat RPF lung samples from animals with pulmonary artery banding surgery. We observed impaired alveolarization and vascularization, the main characteristics of pulmonary dysplasia, in the lungs of RPF infants, piglets, and rats. RPF caused smaller lungs, cyanosis, and body weight loss in neonatal rats and reduced the number of alveolar type 2 cells. RNA sequencing demonstrated that RPF induced the downregulation of metabolism and migration, a key biological process of late alveolar development, and the upregulation of immune response, which was confirmed by flow cytometry and cytokine detection. In addition, the immunosuppressant cyclosporine A rescued pulmonary dysplasia and increased the expression of the Wnt signaling pathway, which is the driver of postnatal lung development. We concluded that RPF results in pulmonary dysplasia, which may account for the reduced exercise capacity of patients with CHD with RPF. The underlying mechanism is associated with immune response activation, and immunosuppressants have a therapeutic effect in CHD-associated pulmonary dysplasia. [ABSTRACT FROM AUTHOR]

Patient size is a limiting factor for transesophageal echocardiography (TEE) in small dogs. The objective of this study was to describe imaging capabilities of two probes for TEE in small dogs. Prospective study in 10 client-owned dogs weighing <4 kg with congenital heart disease (CHD). Dogs had TEE performed with a microprobe (10T-D, GE Medical) and intracardiac echocardiography (ICE) catheter-based probe (AcuNav™, Biosense Webster) in the esophagus in alternating order. Ease of placement, ability to acquire images of the CHD, image quality, and probe limitations were recorded. Median weight was 2.4 kg (range, 1.0–3.2). Congenital abnormalities included patent ductus arteriosus (n = 9) and pulmonary valve stenosis (n = 1). The 10T-D microprobe was easy to place (n = 8) or achievable with gentle manipulation (n = 2), and image quality of the CHD was optimal (n = 8), adequate (n = 1), or poor (n = 1). The ICE probe was difficult to place in the esophagus even with an external support system (n = 9) or could not be placed (n = 1), and image quality of the CHD was optimal (n = 2), adequate (n = 3), or poor (n = 5). Both probes provided images in a 1.0-kg dog. Probe limitations included lack of lateral motion (microprobe), the need for an external support system (ICE probe) and inability to consistently maintain contact with the esophagus (ICE probe). The 10T-D microprobe provided high-quality TEE images more consistently than the ICE probe in the majority of dogs in this study; however, the lack of lateral motion can diminish its utility in some dogs. [ABSTRACT FROM AUTHOR]

This paper presents the results of experimental performance of an innovative left ventricular assist device working on a specially designed test stand. The introduced invention is mainly applied for supporting the heart in patients with end-stage heart failure accompanied by mitral valve regurgitation and pulmonary hypertension. Moreover, the developed device has the form of a flexible balloon forming an intracardiac inert gas-filled working chamber, which is divided into two smaller balloons (valve and ventricular) inflated with gas during use. In addition to supporting the heart, replacing the work of the left ventricle in a permanent manner is also possible. Furthermore, the designed test stand makes it possible to carry out realistic experiments in an environment resembling a human heart. Exemplary pressure measurements for hypotension and hypertension conditions are presented. [ABSTRACT FROM AUTHOR]

Background: Mucopolysaccharidosis Type II (MPS II) is a rare, progressive and ultimately fatal X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene. This report conducted a retrospective analysis to investigate the clinical characteristics, genotypes and management strategies in a large cohort of Chinese patients with MPS II. Methods: In this study, we explored 130 Chinese patients with MPS II between September 2008 and April 2022. Clinical manifestations, auxiliary examination, IDS pathogenic gene variants and IDS enzyme activity, surgical history were analysed in the study. Results: A total of 130 patients were enrolled and the mean age at diagnosis was 5 years old. This study found the most common symptoms in our patients were clawlike hands, followed by coarse facial features, birthmarks (Mongolian spot), delayed development, inguinal or umbilical hernia. The most commonly cardiac manifestations were valve abnormalities, which were mitral/tricuspid valve regurgitation (71.9%) and aortic/pulmonary valve regurgitation (36.8%). We had found 43 different IDS pathogenic gene variants in 55 patients, included 16 novel variants. The variants were concentrated in exon 9 (20% = 11/55), exon 3 (20% = 11/ 55) and exon 8 (15% = 8/55). A total of 50 patients (38.5%) underwent surgical treatment, receiving a total of 63 surgeries. The average age of first surgery was 2.6 years, and the majority of surgery (85.7%, 54/63) was operated before 4 years old. The most common and earliest surgery was hernia repair. Three patients were died of respiratory failure. Conclusion: This study provided additional information on the clinical, cardiac ultrasound and surgical procedure in MPS II patients. Our study expanded the genotype spectrum of MPS II. Based on these data, characterization of MPS II patients group could be used to early diagnosis and treatment of the disease. [ABSTRACT FROM AUTHOR]

Background: Our aim is to present the precautions and applications that can be taken to perform a laparoscopic operation safely in LVAD patients through a case study. Case: A forty-year-old 72 kg male patient was evaluated for laparoscopic cholecystectomy for acute cholecystitis. In preoperative echocardiographic evaluation, ejection fraction is 10%, right atrium and ventricle are slightly enlarged, right ventricular functions are severely depressed, 1-2 degrees tricuspid insufficiency, 1-2 degrees mitral insufficiency, aortic valve opens every 6-7 beats, mild and continuous aortic insufficiency, Small thrombus on the aortic noncoronary valve, pulmonary valve opening with each beat, interventricular septum in midline, inlet and outlet cannulas were reported as normal. Before induction, left radial artery cannulation was performed under ultrasound guidance. 2 mg midazolam, 80 mg lidocaine, 100 mg ketamine, 50 mcg fentanyl, 20 mg propofol, 30 mg rocuronium were administered intravenously to the patient who underwent preoxygenation for two minutes. Two minutes later, she was intubated in one go with a size 8 cuffed tube. Ephedrine was administered to the patient who became hypotensive with carbon dioxide insufflation, and when the response was insufficient, norepinephrine infusion was started at a dose of 0.1 mcg/kg/min. Intra-abdominal pressure was reduced to a level that would allow surgery. At the end of the operation, subcutaneous emphysema was noticed, and due to the risk of hypercarbia and hypoxia, he was transferred to the cardiovascular surgery intensive care unit to be intubated in order to make extubation more controlled. The patient was extubated on the same day and discharged on the 4th postoperative day. Conclusion: In direct proportion to the increasing number of patients with mechanical assist devices, the number of patients with LVAD implanted in anesthesia practice is also increasing. In the surgical plans of these patients, it is necessary to master the effects of anesthesia and surgery on LVAD physiology. When these patients are encountered, a safe surgery and anesthesia plan can be provided with adequate preliminary preparation, equipment and planning. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is effective for treating right ventricle outflow tract (RVOT) dysfunction. Factors associated with long-term valve durability remain to be investigated. Consecutive patients successfully treated by TPVI with Melody valves (n = 32) and SAPIEN valves (n = 182) between 2008 and 2020 at a single tertiary centre were included prospectively and monitored. The 214 patients had a median age of 28 years (range, 10–81). The RVOT was a patched native pulmonary artery in 96 (44.8%) patients. Median follow-up was 2.8 years (range, 3 months–11.4 years). Secondary pulmonary valve replacement (sPVR) was performed in 23 cases (10.7%), due to stenosis (n = 22, 95.7%) or severe regurgitation (n = 1, 4.3%), yielding an incidence of 7.6/100 patient-years with melody valves and 1.3/100 patient-years with SAPIEN valves (P = 0.06). The 5- and 10-year sPVR-freedom rates were 78.1% and 50.4% with Melody vs. 94.3% and 82.2% with SAPIEN, respectively (P = 0.06). The incidence of infective endocarditis (IE) was 5.5/100 patient-years with Melody and 0.2/100 patient-years with SAPIEN (P < 0.0001). Factors associated with sPVR by univariate analysis were RV obstruction before TPVI (P = 0.04), transpulmonary maximal velocity > 2.7 m/s after TPVI (p = 0.0005), valve diameter ≤ 22 mm (P < 0.003), IE (P < 0.0001), and age < 25 years at TPVI (P = 0.04). By multivariate analysis adjusted for IE occurrence, transpulmonary maximal velocity remained associated with sPVR. TPVI is effective for treating RVOT dysfunction. Incidence of sPVR is higher in patients with residual RV obstruction or IE. IE add a substantial risk of TPVI graft failure and is mainly linked to the Melody valve. Transcatheter pulmonary valve implantation is effective for treating right ventricular outflow tract dysfunction in patients with congenital heart diseases. Incidence of secondary valve replacement is higher in patients with residual obstruction or infective endocarditis. [Display omitted] [ABSTRACT FROM AUTHOR]

Objectives: Amoxicillin is the drug of choice in the management of streptococcal and enterococcal infective endocarditis (IE) but little is known regarding amoxicillin diffusion into infected heart valves. Herein, we assessed amoxicillin valvular distribution and related pharmacokinetic/pharmacodynamic (PK/PD) target attainment in IE patients undergoing heart valve surgery.Patients and Methods: In this 2-year prospective study, patients with IE treated by continuous infusion of amoxicillin and undergoing a surgical valve replacement were included. Both amoxicillin plasma and tissue concentrations were measured the day of surgery. Amoxicillin concentration in plasma and crushed heart valves were measured by a validated liquid chromatography method coupled with ultra-violet and tandem mass spectrometry, respectively. MIC and MBC of amoxicillin were determined for all available isolates. The rate of achievement of PK/PD efficacy parameters were assessed.Results: Twenty-two heart valves were removed from 20 patients. Bacterial aetiology was streptococcal (n = 17) and enterococcal (n = 3). Amoxicillin mean daily dose was 12 ± 3 g/24 h, mean plasma concentration was 29 ± 21 mg/L (n = 15), mean tissue concentration was 23 ± 15 mg/L (n = 22). Median diffusion rate was 62%. Patients reached a plasma concentration target >4XCMI (n = 13). Tissue concentrations were bactericidal for all streptococcal IE but not for enterococcal IE.Conclusions: Amoxicillin intravalvular measurements in IE treated patients showed significant penetration into the infectious site. These data are reassuring that in situ bactericidal concentrations can be largely achieved in the management of streptococcal IE and support the need for combination antibiotic therapy for enterococcal IE. [ABSTRACT FROM AUTHOR]