Cirugía congénitos

Pediatr Cardiol. 2025 Sep 26. doi: 10.1007/s00246-025-04020-2. Online ahead of print.

ABSTRACT

Postoperative patients with complex congenital heart disease (CHD), such as those who have undergone Fontan procedures or Tetralogy of Fallot (TOF) repairs, often experience complications, including arrhythmias and heart failure. Heart rate variability (HRV) analysis assesses autonomic function and predicts cardiac outcomes; however, conventional linear methods have limitations, particularly in pediatric populations with variable heart rates. Nonlinear HRV analysis offers a robust alternative. This study evaluated the utility of nonlinear HRV indices to identify early autonomic dysfunction in postoperative patients with CHD. A total of 41 postoperative patients with CHD (22 Fontan, 19 TOF) and 34 age-matched healthy controls underwent 24-h electrocardiogram recordings during sleep. Frequency- and time-domain HRV indices were calculated, alongside nonlinear measures such as Poincaré plot analysis and Higuchi fractal dimension (HFD). Statistical analyses compared the groups and assessed correlations between HRV indices and clinical parameters. Patients who underwent TOF repairs exhibited significantly lower low frequency, high frequency, standard deviation of all normal R-R intervals, and the root mean square of successive differences between R-R intervals values than those in the control group. Nonlinear indices, particularly HFD, were unaffected by heart rate, enhancing reliability in pediatric populations. Age-related declines in fractal indices in postoperative patients with TOF repairs suggest progressive autonomic dysfunction. Nonlinear HRV analysis offers valuable insights into autonomic dysregulation in postoperative patients with CHD, surpassing conventional methods in reliability. These approaches may enable early detection of autonomic deterioration, improving risk stratification and guiding management. Further validation in larger cohorts is warranted.

PMID:41003713 | DOI:10.1007/s00246-025-04020-2

J Pers Med. 2025 Aug 25;15(9):397. doi: 10.3390/jpm15090397.

ABSTRACT

Background/Objectives: The application of additive manufacturing in medicine, and specifically in personalised medicine, has achieved notable development. This article aims to present the results and benefits of applying a comprehensive methodology to simulate, plan, and manufacture customised three-dimensional medical prosthetic devices for use in surgery to restore bone structures with congenital and acquired malformations. Methods: To digitally reconstruct a bone structure in three dimensions from a medical image, a segmentation process is developed to correlate the anatomical model. Then, this model is filtered using a post-processing step to generate stereolithography (STL) files, which are rendered using specialised software. The segmentation of tomographic images is achieved by the specific intensity selection, facilitating the analysis of compact and soft tissues within the anatomical region of interest. With the help of a thresholding algorithm, a three-dimensional digital model of the anatomical structure is obtained, ready for printing the required structure. Results: The described cases demonstrate that the use of anatomical test models, cutting guides, and customised prostheses reduces surgical time and hospital stay, and achieves better aesthetic and functional results. Using materials such as polylactic acid (PLA) for presurgical models, appropriate resins for cutting guides, and biocompatible materials such as polyether ether ketone (PEEK) or polymethylmethacrylate (PMMA) for prostheses, the described improvements are achieved. Conclusions: The achievements attained demonstrate the feasibility of applying these techniques, their advantages and their accessibility in Ecuador. They also reinforce the ideas of personalised medicine in the search for medical treatments and procedures tailored to the needs of each patient.

PMID:41003100 | PMC:PMC12471141 | DOI:10.3390/jpm15090397

J Cardiovasc Dev Dis. 2025 Sep 12;12(9):350. doi: 10.3390/jcdd12090350.

ABSTRACT

(1) Background: Myocardial bridging (MB) is a congenital coronary anomaly with potential clinical significance. Artificial intelligence (AI) applied to cardiac computed tomography angiography (CCTA), particularly through CT-derived fractional flow reserve (CT-FFR), offers a novel, non-invasive approach for assessing MB. (2) Methods: We conducted a systematic review of the literature focusing on studies investigating AI-enhanced CCTA in the evaluation of MB. (3) Results: Ten studies were included. AI-based models, including radiomics, demonstrated moderate to high accuracy in predicting proximal plaque formation, and motion correction algorithms improved image quality and diagnostic confidence. Other findings were limited by the types of studies included and conflicting findings across studies. (4) Conclusions: AI-enhanced CCTA shows promise for the non-invasive functional assessment of MB and its risk stratification. Further prospective studies and validation are required to establish standardized protocols and confirm clinical utility.

PMID:41002629 | PMC:PMC12470274 | DOI:10.3390/jcdd12090350

J Cardiovasc Dev Dis. 2025 Aug 28;12(9):330. doi: 10.3390/jcdd12090330.

ABSTRACT

Pentacuspid aortic valve is an exceptionally rare congenital anomaly that is often associated with functional deterioration and aortopathy. We report a case of a 39-year-old male presenting with severe aortic regurgitation and an ascending aortic aneurysm in the setting of a pentacuspid aortic valve. The patient underwent a successful Bentall and hemiarch replacement using a composite mechanical valved conduit. This case emphasizes the potential association between rare aortic valve morphologies and ascending aortic pathology and includes a brief review of the existing literature on the pentacuspid aortic valve.

PMID:41002609 | PMC:PMC12471185 | DOI:10.3390/jcdd12090330

J Surg Case Rep. 2025 Sep 24;2025(9):rjaf750. doi: 10.1093/jscr/rjaf750. eCollection 2025 Sep.

ABSTRACT

Congenital diaphragmatic hernia (CDH), a rare but potentially life-threatening anomaly resulting from incomplete diaphragm closure during fetal development, typically presents neonatally but can rarely manifest later in infancy as tension gastrothorax-a critical condition of intrathoracic gastric herniation and distension causing severe respiratory distress and mediastinal shift; we describe a 2-month-old male infant who developed sudden dyspnea and mediastinal shift due to a left-sided Bochdalek hernia with a distended stomach occupying the thoracic cavity, requiring emergent laparotomy with gastric decompression and defect repair using non-absorbable sutures, leading to full lung re-expansion and recovery; this case highlights the diagnostic and therapeutic challenges of late-presenting CDH complicated by tension gastrothorax, the importance of distinguishing it from other causes of respiratory distress, and emphasizes that prompt recognition, timely decompression, and surgical repair are vital for favorable outcomes, urging clinicians to maintain high suspicion when imaging reveals an air-filled hemithorax in distressed infants.

PMID:41000549 | PMC:PMC12459967 | DOI:10.1093/jscr/rjaf750

Front Cardiovasc Med. 2025 Sep 10;12:1577847. doi: 10.3389/fcvm.2025.1577847. eCollection 2025.

ABSTRACT

BACKGROUND: Levosimendan is a calcium-sensitizing inotrope with vasodilatory properties, shown to improve cardiac output and reduce mortality in adults with advanced heart failure. However, data on its safety and efficacy in neonatal cardiac surgery are limited.

OBJECTIVE: To evaluate the intraoperative use of levosimendan in neonates with complex congenital heart defects (CHDs) undergoing open-heart surgery.

METHODS: We conducted a retrospective observational study of 59 neonates aged 2-30 days who underwent surgical correction of complex CHDs with cardiopulmonary bypass. Levosimendan was administered intracoronarily as part of the blood cardioplegia protocol in doses of 25-45 mcg/kg.

RESULTS: Compared to historical controls, the levosimendan group demonstrated a significant reduction in postoperative catecholamine requirements, including adrenaline and norepinephrine. In 12% of cases, surgery was completed without the use of any catecholamines. No rhythm disturbances were observed. The positive inotropic effect lasted up to 72 h without systemic hypotension. Median adrenaline doses were significantly lower (p < 0.05), and norepinephrine use was reduced from 12% to 5%.

CONCLUSIONS: Intracoronary administration of levosimendan during neonatal cardiac surgery appears to reduce catecholamine dependence and support myocardial recovery without causing rhythm disturbances or hypotension. Further randomized controlled trials are needed to validate these findings.

PMID:41000533 | PMC:PMC12457408 | DOI:10.3389/fcvm.2025.1577847

BMJ Case Rep. 2025 Sep 25;18(9):e266133. doi: 10.1136/bcr-2025-266133.

ABSTRACT

Congenital heart block is a rare disorder of neonates. It is generally diagnosed in the antenatal period or at birth and is commonly associated with transplacental passage of anti-Ro and/or anti-La antibodies to the fetus. It has a high mortality rate if left untreated. A pacemaker is considered as the treatment of choice, but the expertise for pacemaker implantation is available at only a few centres in India. We present a case of a late preterm neonate with antenatally diagnosed heart block due to anti-Ro antibody in the mother. The baby required delivery room resuscitation and mechanical ventilation. Postnatal ECG was also suggestive of third degree heart block and the baby landed into shock soon after birth. The baby was managed initially with ionotropic agents and an epicardial pacemaker was implanted within 6 hours of life. The baby was discharged successfully with stable vitals.

PMID:40998533 | DOI:10.1136/bcr-2025-266133

Int J Surg Case Rep. 2025 Sep 22;136:111977. doi: 10.1016/j.ijscr.2025.111977. Online ahead of print.

ABSTRACT

INTRODUCTION: Scoliosis is a complex spinal deformity with diverse etiologies, requiring tailored management strategies based on its classification as congenital, neuromuscular, or idiopathic.

PRESENTATION OF CASE: We present the case of a 14-year-old girl with severe sagittal imbalance and gait impairment following spinal fusion surgery for adolescent idiopathic scoliosis (AIS). Postoperative investigations revealed an undiagnosed core myopathy characterized by cervical flexor weakness, confirmed through electromyography and muscle biopsy, which contributed to her sagittal imbalance by necessitating compensatory cervical hyperextension and thoracic flexion.

DISCUSSION: This misdiagnosis of her scoliosis as idiopathic rather than neuromuscular underscores the importance of comprehensive preoperative evaluations, including neuromuscular assessments, in patients with atypical presentations. Avoiding further surgical intervention, the patient was managed conservatively with occupational therapy, resulting in partial functional improvement.

CONCLUSION: This case emphasizes the need for multidisciplinary approaches and vigilance in recognizing underlying conditions that may complicate scoliosis treatment.

LEVEL OF EVIDENCE: Level IV (Case Report).

PMID:40997629 | DOI:10.1016/j.ijscr.2025.111977

JACC Adv. 2025 Sep 24;4(11 Pt 1):102144. doi: 10.1016/j.jacadv.2025.102144. Online ahead of print.

ABSTRACT

BACKGROUND: Cardiac surgery is associated with postsurgical inflammatory conditions, including postoperative pericarditis, that confer morbidity and are understudied in the adult congenital heart disease (ACHD) population.

OBJECTIVES: The purpose of this study was to evaluate the incidence and risk factors for developing postoperative pericarditis in ACHD surgical operations.

METHODS: Retrospective study of ACHD patients who underwent cardiac surgery between 2015 and 2023 at a major New York medical center. Pericarditis diagnosis required: 1) no prior history of pericarditis; 2) at least 2/4 criteria for acute pericarditis (chest pain, friction rub, nonlocalizing ST-segment elevations or PR depressions, new/worsening pericardial effusion); and 3) treatment of pericarditis (nonsteroidal anti-inflammatory drugs, colchicine, steroids, interleukin 1 inhibition). Surgical data and patient characteristics were collected. Logistic regression was used to determine baseline variables associated with pericarditis after atrial septal defect (ASD) repair.

RESULTS: Among 214 ACHD patients undergoing cardiac surgery (median age 36 years [Q1-Q3: 26-53 years], 48.5% male), 47 patients (22.0%) developed postoperative pericarditis and were more commonly Asian or Black. The majority (37/47, 78.7%) of cases occurred within the first 7 days, with clinical resolution within 2 to 4 weeks, except for 4 patients (8.5%) who developed recurrent pericarditis. None developed tamponade or required pericardial drainage. Pericarditis occurred most frequently with shunt repair operations (27/47, 57.4%), in particular after ASD repair (24/29, 82.7%) using autologous pericardium (15/33, 45.4%) and aortic valve replacements (10/29, 34.4%). Logistic regression analysis of ASD repairs identified younger age, male sex, and Asian race as independent risk factors for pericarditis.

CONCLUSIONS: Roughly one in five ACHD cardiac surgical cases develop postoperative pericarditis, most frequently after ASD repairs using autologous pericardium or aortic valve replacement, yet only rarely had long-lasting complications.

PMID:40997554 | DOI:10.1016/j.jacadv.2025.102144

Bol Med Hosp Infant Mex. 2025;82(5):304-313. doi: 10.24875/BMHIM.24000099.

ABSTRACT

BACKGROUND: Invasive medical procedures in children with congenital heart disease (CHD) increase parental anxiety and stress with a negative impact on the disease and children's coping. Parental psychoeducational programs promote their mental health and positive impact on pediatric health. The objective was to evaluate the efficacy of an online psychoeducational program to promote the mental health of parents of children with CHD who are about to undergo an invasive medical procedure.

METHODS: Forty-three mothers and fathers of children with CHD about to undergo catheterization or cardiac surgery belonging to the Live Longer Mexico Foundation participated, divided alternately into two groups: 1) online psychoeducational program (n = 22), and 2) control (n = 21). Mental health was evaluated based on three indicators: parental stress, state/trait anxiety and worry before the procedure. The PSI-SF Parental Stress Index, the Parental Worry about Surgery Questionnaire and the Trait-State Anxiety Inventory were used.

RESULTS: The group with the psychoeducational program showed a statistically significant decrease in state anxiety and worry about the medical procedure. Additionally, state anxiety and worry about the medical procedure are significantly lower in the group that received the psychoeducational program compared to the control. Parental stress had no significant intra- and inter-group changes.

CONCLUSIONS: The online psychoeducational program is effective in favoring parental mental health, specifically on state/trait anxiety and worry about surgery in parents of children with CC nearing catheterization or cardiac surgery.

PMID:40997336 | DOI:10.24875/BMHIM.24000099

J Am Heart Assoc. 2025 Sep 25:e043042. doi: 10.1161/JAHA.125.043042. Online ahead of print.

ABSTRACT

BACKGROUND: Creation of a partial cavopulmonary anasthomosis in patients with isolated right-sided heart failure or patients with cyanosis and intracardial right-to-left shunt and reduced blood flow in the pulmonary circulation may provide a significant improvement in exercise capacity and also facilitate the performance of activities of daily living in patients with a deteriorated clinical condition. However, surgical partial cavopulmonary connection creation may be a high-risk procedure in this patient group. An interventional partial cavopulmonary connection may provide a less invasive treatment modality for these patients. The aim of the study was a retrospective evaluation of patients who underwent the creation of an interventional partial cavopulmonary connection.

METHODS: Each patient who was qualified for treatment underwent a preinterventional computed tomography scan with 3-dimensional reconstruction for improved procedure planning. As a preparation preceding the interventional partial cavopulmonary connection, a prestent (bare metal stent) was implanted into the superior vena cava during cardiac catheterization in most cases. The perforation was performed with the use of needle punctures as a straightforward method in all cases. Thereafter, a covered stent was implanted, connecting the superior vena cava and the right pulmonary artery.

RESULTS: Between July 2019 and July 2024, 14 patients (male n=6, 43%) underwent an interventional partial cavopulmonary connection. The indication for treatment was deteriorated clinical condition (n=12, 86%) or significant cyanosis (n=2, 14%). The procedure was performed successfully in all cases. The median follow-up time was 19.8 months (minimum 2.7; maximum 65.6).

CONCLUSIONS: Our study shows that transcatheter creation of partial cavopulmonary connection provides a promising treatment modality for selected adult patients with right-sided heart failure and may lead to clinical improvement in this patient group.

PMID:40996081 | DOI:10.1161/JAHA.125.043042

Saudi J Anaesth. 2025 Oct-Dec;19(4):628-630. doi: 10.4103/sja.sja_12_25. Epub 2025 Sep 3.

ABSTRACT

Noonan syndrome (NS) is an autosomal dominant multisystem disorder characterized by congenital cardiac defects, craniofacial anomalies, and hematological abnormalities. With an incidence of 1 in 1000 to 2500 live births, NS is one of the most common syndromes associated with congenital heart disease, second only to trisomy 21. Pregnant patients with NS present unique anesthetic challenges due to the syndrome's diverse manifestations and the physiological changes of pregnancy. This report describes a 28-year-old woman with NS who underwent elective cesarean delivery at 36 weeks of gestation. Her medical history included repaired congenital heart defects, pulmonary stenosis, severe scoliosis, and asthma. Spinal anesthesia was successfully employed using a low-dose combination of local anesthetics and opioids to achieve effective sensory block while maintaining hemodynamic stability. The patient's normal coagulation profile allowed neuraxial anesthesia to be a feasible option despite potential technical difficulties associated with spinal abnormalities. This case underscores the importance of a multidisciplinary approach to pregnant patients with NS, including comprehensive preoperative evaluation and individualized anesthetic planning. Neuraxial anesthesia can be a safe and effective option when tailored to the patient's specific physiology, ensuring optimal maternal and fetal outcomes while minimizing risks.

PMID:40994481 | PMC:PMC12456655 | DOI:10.4103/sja.sja_12_25

Br J Hosp Med (Lond). 2025 Sep 25;86(9):1-14. doi: 10.12968/hmed.2025.0284. Epub 2025 Sep 15.

ABSTRACT

Congenital pulmonary airway malformation (CPAM) is a rare cystic lung anomaly in newborns. It can range from asymptomatic to causing life-threatening respiratory distress. However, severe respiratory failure occurring in the immediate neonatal period is uncommon. We report the case of a neonate with CPAM who developed respiratory failure requiring urgent surgical management. The case aims to highlight the importance of early diagnosis and surgical intervention in neonates presenting with severe CPAM to prevent fatal outcomes and support complete recovery. A 29-hour-old male neonate was admitted with cyanosis and severe respiratory distress at birth. His oxygen saturation was only 50% on supplemental oxygen. Chest X-ray and computed tomography (CT) scan revealed a massive multicystic lesion in the left lung consistent with CPAM, with mediastinal shift and compression of the right lung. The baby was intubated and placed on mechanical ventilation, but his respiratory status remained critical. Emergent surgical resection of the left upper lobe was performed. Histopathological examination confirmed a mixed Type I and Type II CPAM. Following early surgical lobectomy, the neonate's respiratory status improved rapidly. He was weaned from the ventilator without difficulty, and mediastinal structures returned to midline. Post-operative course was uneventful, and the infant was discharged in good condition within two weeks. Follow-up at four years showed normal respiratory function, normal pulmonary development on imaging, and no evidence of recurrent pulmonary disease. Early diagnosis and intervention of neonatal CPAM are crucial in the presence of severe respiratory compromise. This case illustrates that urgent surgical resection was effective in correcting respiratory failure caused by a large CPAM, and complete recovery is achievable. Clinicians should consider congenital lung malformations when assessing newborns with unexplained respiratory distress.

PMID:40994381 | DOI:10.12968/hmed.2025.0284

Cardiol Young. 2025 Sep 25:1-6. doi: 10.1017/S1047951125109529. Online ahead of print.

ABSTRACT

OBJECTIVE: To determine the feasibility of developing and implementing a multidisciplinary bootcamp for early-career Paediatric Cardiac Critical Care practitioners.

SETTING: A one-day pre-conference bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022.

SUBJECTS: Physicians, fellows, nurses, advanced practice registered nurses, and respiratory therapists who work primarily in paediatric cardiac critical care units.

METHODS: A modified Delphi needs assessment with interprofessional content experts for the development of a mixed didactic and simulation-based bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022, with pre- and post-testing to evaluate knowledge gain and additional surveys to assess perceived value.

RESULTS: Eighty-three course participants came from a variety of professions and represented institutions nationally and internationally. Most participants (77%) had two or more years of professional experience, aligning with the bootcamp's focus on advanced learners. The bootcamp received strong participant evaluations: 84.1% (37/44) strongly agreed that the bootcamp improved their clinical knowledge. Ninety-seven percent (43/44) reported increased confidence in the ability to care for paediatric cardiac critical care patients. The bootcamp demonstrated a significant improvement in participant knowledge, with pre-test scores averaging 54.9% (95% CI: 49.9-59.9) compared to post-test scores of 64.5% (95% CI: 59.7-69.2), achieving statistical significance (p < 0.05).

CONCLUSIONS: Our pilot bootcamp has shown an improvement in immediate knowledge retention with valuable insights gathered to enhance future bootcamps. The results of this advanced interprofessional bootcamp evaluation will inform future iterations for providers in paediatric cardiac critical care.

PMID:40994242 | DOI:10.1017/S1047951125109529

Cardiol Young. 2025 Sep 25:1-7. doi: 10.1017/S1047951125109359. Online ahead of print.

ABSTRACT

BACKGROUND: Right ventricular outflow tract stenting is a palliative treatment option in symptomatic infants with tetralogy of Fallot or with pulmonary atresia with ventricular septal defect. Predominantly bare metal stents are used for this procedure. The authors sought to assess the efficacy and safety of using the covered coronary stent grafts for the right ventricular outflow tract stenting.

METHODS: Between November 2017 and July 2021, the covered coronary stent graft was used to widen the right ventricular outflow tract in 20 symptomatic patients (pulmonary atresia with ventricular septal defect n = 5, tetralogy of Fallot n = 15).

RESULTS: All stent grafts were implanted successfully. The median time of palliation was 156 (43-1578) days. Eleven patients required stent redilation. Fifteen patients required additional stent implantation to relieve a proximal obstruction in the right ventricular outflow tract. There were three complications observed: right ventricular outflow tract perforation (n = 1), stent embolisation (n = 1), and main pulmonary aneurysm (n = 1). Oxygen saturation improved immediately after the procedure. During the follow-up time, all stents were patent, and we observed a significant increase in the diameters of the pulmonary arteries. Sixteen patients had corrective surgery performed with complete and easy removal of the implanted stents.

CONCLUSIONS: Stenting of the right ventricular outflow tract with stent grafts was safe and effective and provided a durable method of palliation. Utilisation of the covered coronary stent graft facilitated surgical removal of the implanted stent during the surgical correction.

PMID:40994230 | DOI:10.1017/S1047951125109359

Pediatr Res. 2025 Sep 24. doi: 10.1038/s41390-025-04437-8. Online ahead of print.

ABSTRACT

BACKGROUND: Children with complex congenital heart disease (CCHD) are at high risk for early neurodevelopmental delays across all domains. Neuromotor delay often emerges first and may impact broader development. Identifying early biomarkers of motor function could capture a critical window for intervention. We assessed the prognostic value of neuron-specific enolase (NSE) and S100B in predicting 4-month motor outcomes in newborns undergoing cardiac surgery with cardiopulmonary bypass (CPB).

METHODS: Between December 2021 and October 2024, we conducted a prospective, single-centre study including term neonates with (CCHD) who required cardiac surgery within the first two months of life. NSE and S100B levels were measured at five perioperative time points. Blinded Alberta Infant Motor Scale (AIMS) assessment at four months evaluated motor outcomes.

RESULTS: Of 35 newborns, 27 completed follow-up. Preoperative NSE levels were significantly higher in infants with AIMS scores below the 10th percentile (32.7 vs. 20.9 ng/mL, p = 0.044) and negatively correlated with AIMS percentiles (ρ = -0.617, p = 0.006. There was no significant association between motor outcomes, MRI findings or S100B levels.

CONCLUSIONS: Higher preoperative NSE levels predict poor early motor outcomes in CCHD and may be a marker for early risk stratification and intervention.

IMPACT: Neuron-specific enolase (NSE) may serve as an early biomarker of neuromotor development in newborns with complex congenital heart disease (CCHD). Elevated preoperative NSE levels were associated with poorer motor outcomes at four months. NSE may serve as an additional biomarker within a multimodal risk stratification strategy, complementing clinical, imaging, and electrophysiological assessments to refine prognostic evaluation. These findings highlight the prognostic value of perioperative biomarkers for predicting early motor outcomes and support earlier identification of at-risk newborns, enabling targeted neurodevelopmental interventions. This work adds new evidence to limited literature on biological predictors of motor development after neonatal cardiac surgery.

PMID:40993357 | DOI:10.1038/s41390-025-04437-8

Int J Surg Case Rep. 2025 Sep 22;136:111963. doi: 10.1016/j.ijscr.2025.111963. Online ahead of print.

ABSTRACT

INTRODUCTION: Laubry-Pezzi syndrome is a rare congenital heart disease characterized by the association of a ventricular septal defect (VSD) and aortic regurgitation (AR) due to valvular prolapse. The natural course of this condition may lead to severe valvular damage and aortic root dilatation, particularly in cases of delayed diagnosis.

PRESENTATION OF CASE: We report two adult cases of Laubry-Pezzi syndrome managed surgically in Benin. The first patient, a 49-year-old man, presented with severe AR, a large subaortic VSD, and an ascending aortic aneurysm. He underwent VSD closure and a mechanical Bentall procedure. Postoperatively, he developed complete atrioventricular block requiring pacemaker implantation, with no further complications over 24 months. The second patient, a 19-year-old man, had severe AR, a 16 mm VSD, and dilated sinus of Valsalva. He underwent VSD closure, mechanical aortic valve replacement, and tricuspid annuloplasty. Recovery was uneventful, with favorable outcomes at the four-month follow-up, and ongoing surveillance is planned.

DISCUSSION: These two cases demonstrate the clinical progression of untreated Laubry-Pezzi syndrome in adults, highlighting challenges related to delayed diagnosis. Despite complex anatomical findings, surgical repair including Bentall and valve replacement was successfully performed in a resource-limited setting. Findings align with global literature in terms of pathology and outcomes, supporting the feasibility of advanced cardiac surgery in sub-Saharan Africa.

CONCLUSION: Early detection and intervention are critical to prevent severe valve and aortic complications in Laubry-Pezzi syndrome. This case report illustrates that, even in low-resource contexts, successful surgical outcomes are achievable through adapted techniques and interdisciplinary collaboration.

PMID:40992295 | DOI:10.1016/j.ijscr.2025.111963

Interdiscip Cardiovasc Thorac Surg. 2025 Sep 24:ivaf214. doi: 10.1093/icvts/ivaf214. Online ahead of print.

ABSTRACT

OBJECTIVES: Many models of bioprosthesis are available for pulmonary valve replacement in adults with congenital heart disease but there is a lack of randomized evidence to guide practice. We surveyed congenital cardiac surgeons to establish current practice and willingness to change within a clinical trial.

METHODS: An online survey was sent to all consultant congenital cardiac surgeons in adult congenital centres in UK and Ireland. Information was sought on preferred prostheses, factors influencing decision-making, implant technique, postoperative anticoagulation, practice variations in adolescents, and willingness to randomise patients to different prostheses within a trial.

RESULTS: Responses were obtained from 27 (69%) surgeons. 19 (70%) preferred an Edwards bovine pericardial valve, most commonly the Inspiris Resilia (7, 26%). Only 2 (7%) favoured the Hancock II valve, the remaining 6 (22%) preferred pulmonary homografts. Data regarding long-term freedom from reintervention (23, 85%) was the most important factor influencing prosthesis choice. 22 (81%) surgeons were willing to randomise adult patients to either a bovine pericardial valve or porcine xenograft in a clinical trial, with Perimount Magna Ease and Hancock II the most acceptable, respectively. Willingness to randomise dropped to 11 (41%) surgeons for adolescent patients.

CONCLUSIONS: This survey demonstrates heterogeneity in the choice of pulmonary valve prosthesis. Combined with a lack of evidence from clinical trials, our findings support the presence of clinical equipoise. Most surgeons are willing to change practice suggesting that a pragmatic, multicentre, randomized controlled trial comparing bovine pericardial versus porcine xenograft for pulmonary valve replacement in adults is feasible.

PMID:40991337 | DOI:10.1093/icvts/ivaf214

Chirurgie (Heidelb). 2025 Sep 24. doi: 10.1007/s00104-025-02371-3. Online ahead of print.

ABSTRACT

The thoracic outlet syndrome (TOS) refers to a group of clinical conditions caused by compression of nerves and blood vessels in congenital or acquired anatomical narrowing of the upper thoracic aperture. This includes the anatomical structures, such as the scalene muscles, the first rib, a possibly present cervical rib, the costoclavicular joint or the pectoralis minor muscle. Isolated venous compression is also known as thoracic inlet syndrome (TIS). The symptoms are diverse and the path to a correct diagnosis is often prolonged. In the absence of anatomical anomalies, conservative treatment is indicated, such as physical therapy and adequate pain management. Especially in young patients, TOS should be considered when embolic events in the upper extremities occur without other risk factors. Appropriate multimodal diagnostics and targeted therapy are challenging and require treatment in specialized departments. Surgical treatment is complex and over the years various procedures with a favorable outcome for the affected patients have been established.

PMID:40991022 | DOI:10.1007/s00104-025-02371-3

Pediatr Cardiol. 2025 Sep 24. doi: 10.1007/s00246-025-04021-1. Online ahead of print.

ABSTRACT

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, frequently diagnosed prenatally. To enhance understanding, we reviewed 18 years of our institutional outcomes for patients with classic ToF. There were 410 patients of whom 233/410 (56.8%) were diagnosed prenatally. A genetic abnormality was diagnosed in 38/410 (9.3%), most commonly 22q11.2 microdeletion (43.6%). There were 10/410 (2.4%) deaths in the neonatal period prior to any cardiac intervention. Prior to complete ToF repair, 66/400 (16.5%) of patients required cardiac procedures, in 15 in the first week after birth. Babies with an antenatal diagnosis were more likely to require at least one procedure before complete ToF repair than those with a postnatal diagnosis (p = 0.039). There was a trend to shorter median length of stay in patients with RVOT stent (median 5.4 days) or duct stenting (median 4.2 days) versus BTT shunt (median 8 days). Complete ToF repair was undertaken in 396/410 at a median age of 6 months (IQR: 4.0-8.0 months), with a 30-day postoperative survival rate of 99.5%. The actuarial survival for the whole group (n = 410) of 410 patients was 98.5% at 30 days, 96.2% at 1 year, and 95.2% at 3 years of age. Following complete surgical repair, reintervention was required in 25% of cases: 3.3% at 30 days, 10.6% at 1 year, and 19.2% at 5 years. The freedom from reintervention was 89.4% after 1 year and 80.8% after 5 years. There were no deaths beyond 2.5 years post-repair. In conclusion, ToF can be repaired with a low procedural mortality rate and promising long-term survival outcomes, but there may be pre-procedural deaths and there is a likelihood of requiring reinterventions during follow-up.

PMID:40991001 | DOI:10.1007/s00246-025-04021-1