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Journal of Interventional Cardiac... Mar 2024
PubMed: 38480673
DOI: 10.1007/s10840-024-01784-0 -
ASAIO Journal (American Society For... Jun 2024Patients supported on ventricular assist devices (VADs) benefit from rehabilitation while awaiting heart transplantation to recover from surgery, prevent deconditioning,...
Patients supported on ventricular assist devices (VADs) benefit from rehabilitation while awaiting heart transplantation to recover from surgery, prevent deconditioning, and, in most cases, optimize transplant candidacy. With bleeding and neurological dysfunction as the most common VAD complications, the importance of rehabilitation dramatically increases when a patient on a VAD also suffers from a neurological injury. The rehabilitation needs for cardiac conditioning and neurological reeducation are not the same. Patients with severe neurological deficits require intense rehabilitation that often includes base-of-support challenges, usage of bolsters and balls, partial weight-bearing treadmill training, and assumption of various body positions in prone, kneeling, or quadruped for neuromotor reeducation. However, some devices are more conducive to rehabilitation than others. For children supported by the CentriMag in particular, rehabilitation is challenged by short cannula tubing, an external motor, a large interface, and an intensive care unit (ICU) admission. We report a safe and successful physical therapy course of a pediatric stroke patient with a diagnosis of Ebstein's anomaly supported by a CentriMag right VAD (RVAD) while awaiting heart transplant in the ICU.
PubMed: 38870474
DOI: 10.1097/MAT.0000000000002254 -
World Journal For Pediatric &... May 2024Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity,...
Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.
PubMed: 38693812
DOI: 10.1177/21501351241239305 -
Cardiology in the Young Apr 2024Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe...
Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.
Topics: Infant, Newborn; Humans; Ebstein Anomaly; Hepatoblastoma; Univentricular Heart; Liver Neoplasms
PubMed: 38247377
DOI: 10.1017/S1047951124000039 -
PCN Reports : Psychiatry and Clinical... Dec 2023Along with the improved prognosis of patients with congenital heart disease, the associated diverse complications are under scrutiny. Due to various medical restrictions...
BACKGROUND
Along with the improved prognosis of patients with congenital heart disease, the associated diverse complications are under scrutiny. Due to various medical restrictions on their upbringing, patients with congenital heart disease often have coexisting mental disorders. However, reports on patients with congenital heart disease and coexisting eating disorders are rare. Here, we report the case of a patient who developed anorexia nervosa (AN) following surgery for Ebstein's anomaly.
CASE PRESENTATION
A 21-year-old female with Ebstein's anomaly who underwent Fontan surgery was transferred to our institution with suspected AN after >2 years of intermittent stays at a medical hospital for decreased appetite. Initially, she did not desire to lose weight or fear obesity, and we suspected that she was suffering from appetite loss due to a physical condition associated with Fontan circulation. However, the eating disorder pathology gradually became more apparent.
CONCLUSION
Our experience suggests that patients with congenital heart disease are more likely to have a psychological background and physical problems that might contribute to eating disorders than the general population.
PubMed: 38868727
DOI: 10.1002/pcn5.154 -
Journal of Cardiovascular Magnetic... Dec 2023Due to the heterogeneity of anatomic anomalies in Ebstein's anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes,...
Effect of concomitant atrial septal defect on left ventricular function in adult patients with unrepaired Ebstein's anomaly: a cardiovascular magnetic resonance imaging study.
BACKGROUND
Due to the heterogeneity of anatomic anomalies in Ebstein's anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes, which ultimately cause left ventricular (LV) deterioration remain unclear. The current study aimed to investigate the effect of concomitant ASD on LV function using cardiovascular magnetic resonance (CMR) imaging in patients with EA.
METHODS
This study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls. Left/right ventricular (RV, the RV was defined as a summation of the functional RV and atrialized RV in EA patients) volumes and functional parameters, LV strain parameters, and echocardiogram indices were compared among the four groups. Associations between variables were evaluated via Spearman or Pearson correlation analyses. The association between risk factors and LV ejection fraction (EF) was determined via multivariate linear regression analysis.
RESULTS
Both EA patients and ASD patients had a higher RV/LV end-diastolic volume (RVEDV/LVEDV) as well as lower LV and RV ejection fractions (LVEF/RVEF) compared to healthy controls (all p < 0.05). Moreover, the EA patients with ASD had a significantly higher RVEDV/LVEDV and a lower LVEF and RVEF than those without ASD (all p < 0.05). Multivariate linear regression analysis revealed that the presence of ASD was independently associated with LVEF (β = - 0.337, p < 0.001). The RVEDV/LVEDV index was associated with LVEF (r = - 0.361, p < 0.001). Furthermore, the LV longitudinal peak diastolic strain rate (PDSR) was lower in EA patients with ASD than those without ASD, patients with simple ASD, and healthy controls (p < 0.05).
CONCLUSION
Concomitant ASD is an important risk factor of LV dysfunction in patients with EA, and diastolic dysfunction is likely the predominate mechanism related to LV dysfunction.
Topics: Adult; Humans; Ventricular Function, Left; Ebstein Anomaly; Predictive Value of Tests; Magnetic Resonance Imaging; Ventricular Dysfunction, Left; Stroke Volume; Heart Septal Defects, Atrial
PubMed: 38057914
DOI: 10.1186/s12968-023-00976-3 -
JTCVS Techniques Aug 2023
PubMed: 37555042
DOI: 10.1016/j.xjtc.2023.04.010 -
Pacing and Clinical Electrophysiology :... Dec 2023Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified...
INTRODUCTION
Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified surgical technique for Glenn anastomosis and a combined interventional and electrophysiologic approach, the transvenous pacemaker was successfully implanted.
FINDINGS AND CONCLUSIONS
We reported a novel technique of pacemaker implantation in a 27-year-old woman, underlying Ebstein anomaly of the tricuspid valve, who developed intermittent complete atrioventricular block at 5 years after surgical repair. The patient had a tricuspid valve replacement and a novel modified bidirectional Glenn anastomosis for one-and-a-half ventricle repair. The Glenn circuit was conducted by opening a window between the posterior wall of the superior vena cava (SVC) and the anterior wall of the right pulmonary artery (RPA), combined with putting a Goretex membrane in the SVC below the SVC-RPA window without disconnecting the SVC from the right atrium. The transvenous pacemaker was implanted by perforating the Goretex membrane, then passing the leads from the axillary vein through the perforated membrane and placing them in the coronary sinus and right atrium.
Topics: Female; Humans; Adult; Ebstein Anomaly; Tricuspid Valve; Vena Cava, Superior; Treatment Outcome; Pacemaker, Artificial; Polytetrafluoroethylene
PubMed: 37120827
DOI: 10.1111/pace.14705 -
Pediatric Cardiology Dec 2023Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the...
Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation. In this report, we present the results of a strategy to redirect patients utilizing the Da Silva Cone operation to achieve a 2 or 1.5 ventricle circulation. Single-center retrospective study including all consecutive cases of Da Silva Cone operation after Starnes procedure. Between 2019 and 2023, six conversions from Starnes procedure to Cone reconstruction were performed. All were critically ill before their Starnes procedure; four on extracorporeal membrane oxygenation. Two patients were successfully rerouted to a two-ventricle repair; the remainder to 1.5 ventricle circulation. RV pressure estimates showed no correlation with success. Post-Cone intensive care and hospital stays were brief, median 5 and 6 days, respectively. All are between 2.5 and 6 years old, without indications for SV palliation. There were no deaths, with follow up ranging 1 month-4 years. No repeat interventions were performed on the tricuspid valves. One subject had a surgical pulmonary valve replacement. Tricuspid regurgitation was mild in all. The Da Silva Cone operation offers successful redirection of EA patients from a SV pathway to a 1.5 or 2 ventricle pathway after Starnes procedure. The approach is feasible and durable in midterm follow-up. The decision to initially proceed with Starnes need not be an irrevocable decision to continue down a SV palliation pathway.
PubMed: 38038752
DOI: 10.1007/s00246-023-03336-1 -
Pediatric Cardiology Jun 2024This study aimed to analyze prenatal cardiac ultrasound markers of outcome in fetuses with Ebstein's anomaly (EA). From a retrospective database, 35 fetuses diagnosed...
This study aimed to analyze prenatal cardiac ultrasound markers of outcome in fetuses with Ebstein's anomaly (EA). From a retrospective database, 35 fetuses diagnosed with EA at fetal medicine centers in Brazil, Italy, and Poland were retrieved. The primary outcome was perinatal mortality. We analyzed prenatal cardiac ultrasound markers of outcomes and perinatal follow-up. Gestational age at diagnosis, extracardiac fetal anomalies, spontaneous fetal demise, and gestational age at each event were recorded. In postnatal survivors, data on cardiac surgery and short-term postoperative outcomes were collected. Our study included a cohort of 35 fetuses with EA (mean gestational age of 29.4 weeks), in which 6 fetuses were excluded due to termination of pregnancy (3), pregnancy still ongoing (2), and missed follow-up (1). Of the remaining 29 cases, severe tricuspid regurgitation and absence of anterograde pulmonary flow (pulmonary atresia) were observed in 88%. Significant cardiomegaly accounts for 58% of these data with a mean cardiothoracic ratio of 0.59. The cardiovascular profile (CVS) score ≤ 6 in six patients with one survival (4 fetal deaths, one stillbirth, and one survival). All fetuses with CVS score of 5 had intrauterine demise. Seventeen fetuses were born alive (53.1% of 29 cases). Of the remaining fetuses, one (1%) fetal was a stillbirth, six (20%) fetuses were neonatal deaths, and five (17%) fetuses were fetal deaths. Of the nineteen patients who underwent surgery to correct the cardiac defect, 17 survived after surgery. Among the survivors, biventricular cardiac repair was performed using the cone technique (da Silva's approach) in the majority of cases. We observed 2 abnormal karyotypes among in the remaining 29 fetuses. One of the patients with abnormal karyotype was a fetus with ascites and large for gestational age. The other patient with abnormal karyotype underwent cardiac surgery and progressed to neonatal death. Nine patients (25%) had extracardiac anomalies (genitourinary anomalies and single umbilical artery), being that 2 of them are alive and 4 died (2 had fetal and 2 neonatal death). Fetal EA is associated with high mortality. The most common prenatal marker associated with non-survival was CVP score ≤ 6. Fetuses that survived and underwent postnatal corrective surgery are significantly favorable outcomes.
PubMed: 38825661
DOI: 10.1007/s00246-024-03530-9