Cardiac care in trisomy 18: A path to improved outcomes (case report).

Background: Trisomy 18 (also known as Edwards syndrome) is a chromosomal disorder characterized by severe developmental anomalies and cognitive deficits. Cardiac complications are a leading cause of mortality in these patients, and the role of cardiac interventions remains controversial. Case Presentation: We report a case of a full-term baby girl with trisomy 18, born via elective cesarean section. The neonate presented with pulmonary atresia and a series of other cardiac abnormalities, necessitating immediate intervention. Despite the initial challenges, including a brief episode of desaturation post-intervention, the patient responded positively to a balloon pulmonary valvuloplasty and emergency patent ductus arteriosus stent insertion, illustrating the potential benefits of cardiac interventions in patients with trisomy 18. Discussion: This case highlights the successful application of cardiac interventions in a patient with trisomy 18, challenging the notion of universally denying such treatments to this population. Our findings suggest that selective interventions can improve quality of life and stabilize the condition, supporting the need for further research to establish clear guidelines for treatment in this demographic. Conclusion: This case adds to the growing evidence supporting the feasibility and potential benefits of cardiac interventions in patients with trisomy 18, advocating for a more individualized approach to treatment.

Mid-Term Outcome of Left Ventricle Reverse Remodeling After Atrial Septal Defect Closure: A Comparison Between Surgical and Device Groups.

Right ventricular (RV) volume overload occurs in patients with significant atrial septal defect (ASD II) shunts, which can impair left ventricle (LV) filling, thus decreasing LV indices. However, after ASD II closure, the left ventricle regains near normal dimensions. The purpose of this study was to compare the long-term outcomes of LV reverse remodeling between device and surgical closure. We retrospectively reviewed the echocardiographic data of 222 patients with isolated ASD II who underwent surgical (95 patients) or device closure (127 patients) between January 2012 and December 2017. The ASD II was significantly larger in the surgical closure group (p < 0.001.) leading to a higher degree of paradoxical interventricular septal (IVS) movement. In contrast, the LV volume was larger in the device closure group (p < 0.001). After a median follow-up period of 19.5 months, the maximum LV systolic and diastolic volumes were reached at 1 year in the device closure group and 2 years in the surgical closure group. IVS motion normalized in 91% of the device closure group compared to 57% of the surgical closure group (p = < 0.001). There was significant improvement in left ventricular indices and IVS motion after ASD II closure in both groups but more favorable in patients after device closure.

Transcatheter Mitral Valve-in-Valve Implantation in Pediatric Patients.

BACKGROUND: Transcatheter implantation of the Edwards Sapien 3 valve (Edwards Lifesciences, Irvine CA) within the bioprosthetic mitral valve (MV) is an established method of treatment in adults. However, it has not been well studied in the pediatric age group. METHODS: Transcatheter mitral valve-in-valve implantation was attempted in 4 symptomatic pediatric patients with a dysfunctional MV bioprosthesis implanted at an earlier stage due to severe MV stenosis or regurgitation. We reviewed our experience with MV implantation in this cohort. RESULTS: The mean age and weight of the patients at the time of the procedure were 11.4 years (range: 10-14 years) and 36 kg (range: 31-44 kg), respectively. The transmitral mean gradient dropped from a mean of 19.75 mm Hg (range: 15-22 mm Hg) to a mean of 1 mm Hg (range: 0-3 mm Hg) after the procedure. The mean fluoroscopy time was 55.25 minutes (range: 40-72 minutes), and the mean hospital length of stay was 4 days (range: 3-7 days). The patients' functional class improved from New York Heart Association class IV to class I during the follow-up period. CONCLUSIONS: Transcatheter mitral valve-in-valve implantation can be performed safely for dysfunctional bioprosthetic MVs in the pediatric age group with favorable early and midterm outcomes. This procedure offers a viable alternative in patients who have high surgical risk or are deemed unfit for conventional surgery. However, we still recommend a long-term study of this approach in a large cohort, multicentre study.

INTRODUCTION: L'implantation de la prothèse valvulaire Edwards Sapien 3 (Edwards Lifesciences, Irvine, CA) par cathéter dans la bioprothèse valvulaire mitrale (VM) est une méthode de traitement établie chez les adultes. Toutefois, cette méthode n'a pas fait l'objet d'études approfondies auprès d'enfants. MÉTHODES: Une tentative d'implantation valvulaire mitrale de type valve-in-valve par cathéter a été réalisée chez quatre enfants symptomatiques qui avaient une bioprothèse VM dysfonctionnelle implantée antérieurement en raison d'une sténose VM ou d'une régurgitation grave. Nous avons passé en revue notre expérience d'implantation VM auprès de cette cohorte. RÉSULTATS: L'âge et le poids moyens des patients au moment de l'intervention étaient respectivement de 11,4 ans (étendue : 10-14 ans) et de 36 kg (étendue : 31-44 kg). La moyenne du gradient moyen transmitral a baissé. Elle est passée de 19,75 mmHg (étendue : 15-22 mmHg) à 1 mmHg (étendue : 0-3 mmHg) après l'intervention. La durée moyenne de la fluoroscopie était de 55,25 minutes (étendue : 40-72 minutes), et la durée moyenne du séjour à l'hôpital était de quatre jours (fourchette : 3-7 jours). La classification fonctionnelle des patients selon la New York Heart Association a montré une baisse. Les patients sont passés de la classe IV à la classe I durant la période de suivi. CONCLUSIONS: L'implantation valvulaire mitrale de type valve-in-valve par cathéter peut être pratiquée de façon sûre chez les enfants porteurs d'une bioprothèse VM dysfonctionnelle dont les issues à court ou à moyen terme sont favorables. Cette intervention est une alternative viable pour ces patients dont le risque lié à l'intervention chirurgicale est élevé ou considérés inaptes à subir une intervention chirurgicale traditionnelle. Toutefois, nous recommandons encore une étude à long terme sur cette approche, voire une vaste étude multicentrique de cohorte.

Peripheral Pulmonary Artery Stenosis Is Not a Contraindication to a Single-ventricle Pathway.

BACKGROUND: The presence of peripheral pulmonary artery stenosis (PPAS) involving lobar and/or segmental pulmonary artery (PA) branches might preclude proceeding to a single-ventricle pathway. We adopted a strict strategy for PA rehabilitation and surgical reconstruction in patients who are unable to progress to a single-ventricle pathway. METHODS: We conducted a retrospective review of 22 patients with single-ventricle physiology who underwent surgical reconstruction for PPAS from April 2008 to March 2020. Our surgical approach was single stage in 15 patients (68.2%) and 2 stage in 7 patients (31.8%) depending on the presence or absence of PA hypoplasia distal to the PPAS. RESULTS: The PPAS was type 3 (lobar) in 19 patients (86.4%) and type 4 (segmental) in 3 patients (13.6%). The mean number of PA angioplasties performed was 8 ± 5.6 per patient. There was no mortality in this series with a median of 52 months (range, 8-143) of follow-up. Twenty patients (90.9%) were able to progress in the single-ventricle pathway with 12 patients (54.5%) undergoing a Fontan operation and 8 patients (36.4%) achieving bidirectional Glenn shunt and awaiting a Fontan operation. Two patients (9.1%) were not suitable to progress to the single-ventricle pathway because of elevated PA pressure. All patients who achieved cavopulmonary connection were alive and asymptomatic with no signs of elevation of PA pressure. No patient required further reinterventions for the PA. CONCLUSIONS: PPAS is not a contraindication for single-ventricle palliation. With careful planning and aggressive early surgical reconstruction, most of these patients can successfully progress to cavopulmonary connections.

Late Outcomes After Pulmonary Arterial Reconstruction in Patients With Arterial Tortuosity Syndrome.

BACKGROUND: Surgical pulmonary artery reconstruction in patients with arterial tortuosity syndrome has excellent outcomes. In this study, we report our late outcomes after more than a decade of experience with such complex interventions. METHODS: We conducted a retrospective review of 33 arterial tortuosity syndrome patients who underwent pulmonary artery reconstruction. The mean preoperative right ventricular to left ventricular pressure ratio was 1.19 ± 0.2. Our surgical approach included either a single-stage complete repair through a median sternotomy (17 patients) or a two-stage repair through sternotomy/left thoracotomy (16 patients), depending on the degree of distal involvement in the left pulmonary artery. RESULTS: Median age was 36 months. All patients had distal segmental peripheral pulmonary artery stenosis. Thirty patients (90.1%) were symptomatic before surgery. There was one hospital death due to viral pneumonia 78 days after the surgery (in-hospital mortality 3%). The mean right ventricular to left ventricular pressure ratio decreased to 0.31 ± 0.07 early postoperatively (P < 0.001), representing a 74% reduction compared with preoperative values. Follow-up was 100% complete for all hospital survivors (32 of 33) with a mean follow-up of 70.42 ± 43.32 months (range, 2 to 143). There was no late mortality or need for reintervention (surgical or catheter based) after hospital discharge. In late postoperative catheterization, the mean right ventricular to left ventricular pressure ratio was 0.27 ± 0.05 (P = .003 compared with early postoperative value). All patients were asymptomatic on their most recent follow-up. CONCLUSIONS: A strategy of complete surgical reconstruction of all stenotic pulmonary artery segments in patients with arterial tortuosity syndrome is recommended for sustainable successful outcomes more than a decade later.

Late outcomes of surgical reconstruction of peripheral pulmonary arteries.

OBJECTIVE: The ideal management of peripheral pulmonary artery stenosis is still controversial. We adopted a primary surgical approach to this complex lesion with excellent early outcomes. In this study, we analyzed our late outcomes. METHODS: We performed a retrospective review of 91 patients with biventricular anatomy who underwent peripheral pulmonary artery reconstruction from March 2008 to July 2020. Our surgical approach included either a single-stage complete repair through median sternotomy or a 2-stage repair through sternotomy/left thoracotomy, depending on the degree of distal involvement of the left pulmonary artery branches. RESULTS: Median age was 26 months. Syndromic etiology was established in 54 patients (59.3%) versus nonsyndromic etiology in 37 patients (40.7%). Single-stage repair was achieved in 68 patients (74.7%). There were 2 (2.2%) in-hospital mortalities. The mean right ventricular to aortic systolic pressure ratio decreased from 1.07 ± 0.20 preoperatively to 0.32 ± 0.07 immediately postoperatively (P < .001), representing a 70.1% reduction. At 1-year postoperative catheterization, the mean right ventricular to aortic systolic pressure ratio was 0.28 ± 0.05 (P < .001 compared with immediately postoperative value). With a median follow-up of 68 months (IQR, 39-117.5 months), there was no late mortality after discharge. All patients were active and asymptomatic on the most recent follow-up. There were no early or late reinterventions on pulmonary arteries. CONCLUSIONS: Late outcomes of surgical reconstruction of peripheral pulmonary arteries are excellent and durable in various pathologies (syndromic and nonsyndromic) with a significant reduction in right ventricular to aortic systolic pressure ratio, low mortality, and no reintervention.

Unusual presentation of COVID-19 in a child complicated by massive acute pulmonary embolism and lung infarction.

The Novel Coronavirus 2019 (SARSCoV- 2), which was first reported on in Wuhan, China, in late December 2019, causes a respiratory illness called COVID- 19 Disease. COVID-19 is most likely causing a hypercoagulable state, however the prevalence of acute venothromboembolism is still unknown. Limited data suggest pulmonary microvascular thrombosis may play a role in progressive respiratory failure. Here, we report a case of a child with an unusual presentation of COVID-19 presented initially by dry cough without fever and complicated by massive acute pulmonary embolism and lung infarction and treated successfully by hydroxychloroquine and azithromycin, in addition to anticoagulant therapy.

Transcatheter mitral valve-in-valve implantation in a pediatric patient.

An 11-year-old girl, with 25 mm mosaic tissue valve presented with clinical and echocardiographic appearance of stenotic mitral valve prosthesis. Her condition was treated via a transcatheter valve-in-valve implantation using a 23 mm Edwards Sapien 3, with satisfactory outcome.