Cirugía congénitos

Am J Transl Res. 2025 May 15;17(5):3546-3553. doi: 10.62347/SMEE7102. eCollection 2025.

ABSTRACT

INTRODUCTION: Spinal meningoceles are congenital anomalies characterized by meningeal herniation through vertebral defects, most commonly occurring in the lumbosacral region. Intrathoracic meningoceles are rare and are typically associated with neurofibromatosis type 1 (NF-1). True anterior thoracic meningoceles unassociated with NF-1 represent exceptionally rare clinical entities.

CASE SUMMARY: This is a retrospective case report. We report a 15-year-old female with severe kyphoscoliosis and a non-NF-1-associated anterior thoracic meningocele who presented with progressive spastic paraparesis. Imaging examinations revealed thoracolumbar scoliosis with 100-degree kyphotic angulation, midline anterior spina bifida at T7, and a well-defined cystic structure exhibiting hypointense T1 and hyperintense T2 signals (measuring 5.7×4.5 cm) anterior to the T7-T8 vertebral bodies. Surgical intervention included posterior spinal laminar decompression, T7 vertebral osteotomy, microscopic dural sac reduction, and stabilization using a pedicle screw system with autologous bone grafting. The patient was discharged without any complications 12 days after surgery, and at the 12-month follow-up, the patient achieved ambulation with a walker and leg brace.

CONCLUSION: This case underscores the necessity of tailored surgical strategies for anterior thoracic meningoceles complicated by severe spinal deformities. The integration of microsurgical techniques and 3D-printed anatomical modeling may optimize procedural safety and functional outcomes.

PMID:40535678 | PMC:PMC12170365 | DOI:10.62347/SMEE7102

J Pediatr Surg Case Rep. 2025 May;116:102989. doi: 10.1016/j.epsc.2025.102989. Epub 2025 Mar 21.

ABSTRACT

INTRODUCTION: Lipomas are typically slow-growing tumors with the highest incidence in the fourth through sixth decades of life, less commonly occurring in pediatric patients, particularly extending across multiple body compartments. Excision is often reserved for cases that cause cosmetic or compressive symptoms and, due to the slow growth pattern, is less likely to be necessary in younger patients.

CASE PRESENTATION: A 2-year-old female with congenital albinism presented with a painless but visible 3 × 4 cm non-mobile left lateral neck mass that had been present for 2 months. MRI without contrast demonstrated a lobulated lesion in the left inferolateral supraclavicular region extending into the left thoracic inlet and thoracic apex. Due to concern for developing mass effect on carotid space structures, resection of the mass was performed. Complete excision was achieved through a single lower cervical incision, requiring dissection from the brachial plexus, carotid sheath, subclavian vessels, and extrapleural thoracic apex. Final pathology revealed an adipocytic neoplasm consistent with a lipoma with a total specimen size measuring 9 × 6.5 × 5 cm. The patient was discharged on postoperative day one and was healing well without complaint at the time of follow-up.

CONCLUSION: Despite their benign nature, cases such as the one presented here demonstrate the capability of lipomas to adhere to and involve surrounding critical structures, particularly when located in the cervicothoracic region and occurring in younger patients. However, with careful surgical planning involving cross-sectional imaging, such lesions can successfully be completely resected through a single cervical incision without morbidity.

PMID:40535308 | PMC:PMC12176381 | DOI:10.1016/j.epsc.2025.102989

J Med Case Rep. 2025 Jun 18;19(1):280. doi: 10.1186/s13256-025-05241-y.

ABSTRACT

BACKGROUND: Right-sided infective endocarditis is a rare clinical entity, with isolated pulmonary valve infective endocarditis being extremely uncommon. Infective endocarditis carries a high mortality rate and significant complications, making early identification and prompt management crucial in improving outcomes. This case highlights an unusual presentation of right-sided infective endocarditis isolated to the pulmonic valve in a pediatric patient with no apparent preexisting heart disease.

CASE PRESENTATION: A 7-year-old girl of Yoruba ethnicity presented with septicemic illness, congestive heart failure, and no evidence of congenital cardiac lesion, underlying valvular disease, or identifiable predisposing factors. She had underweight malnutrition, cachexia, and severe respiratory distress. Echocardiography, which was delayed due to resource limitations, ultimately revealed isolated myxomatous vegetation on the pulmonary valve, dilated right cardiac chambers, and pulmonary hypertension. Blood cultures grew Pseudomonas aeruginosa. The patient was managed with antimicrobial agents, an anticardiac failure regimen, antiplatelets, and supportive therapy. Management was complicated by financial constraints, which delayed optimal intervention.

CONCLUSION: Although isolated pulmonary valve infective endocarditis is rare in the pediatric population, particularly in the absence of identifiable heart disease, a high index of suspicion is essential. Early diagnosis via echocardiography and prompt, adequate treatment are crucial for favorable outcomes. Awareness of potential diagnostic delays and financial barriers can aid in optimizing timely intervention and improving prognosis.

PMID:40533870 | PMC:PMC12178004 | DOI:10.1186/s13256-025-05241-y

Pediatr Cardiol. 2025 Jun 18. doi: 10.1007/s00246-025-03920-7. Online ahead of print.

ABSTRACT

Extubation on the operating table is increasingly utilized to minimize ventilator-associated complications and promote early recovery in pediatric cardiac surgery. However, its safety across diverse congenital heart disease (CHD) populations remains insufficiently defined. To evaluate the feasibility, safety, and clinical outcomes of on-table extubation across a broad spectrum of corrective and palliative congenital heart surgeries in children. This retrospective multicenter study included 986 pediatric patients (aged 7 days to 16 years) who underwent on-table extubation after CHD surgery between 2019 and 2025. Patients were grouped as corrective (n = 632) or palliative (n = 354) cases. Primary outcomes were reintubation and mortality. Secondary outcomes included ICU and hospital stay durations, and incidence of ventilator-associated pneumonia (VAP). Overall reintubation and mortality rates were 5.78 and 1.22%, respectively. Corrective procedures demonstrated significantly lower reintubation (4.11%) and mortality (0.63%) compared to palliative surgeries (8.76 and 2.26%, respectively; p < 0.01 and p < 0.05). Highest complication rates were observed in HLHS (reintubation and mortality 40%) and aortopulmonary shunt (53.13 and 21.88%). In contrast, Glenn and Fontan procedures showed low reintubation (1.69, 2.91%) and minimal mortality. No cases of VAP were reported. Mean ICU and hospital stays were 3.69 and 9.7 days. Of the 57 reintubation events, 23 (40.4%) occurred within 6 h of extubation, suggesting extubation failure, while 34 (59.6%) occurred between 6 and 24 h, potentially due to secondary complications. Early reintubations (0-6 h) were more common in aortopulmonary shunt (17 cases) and coarctation/IAA repair (3 cases), whereas later reintubations (6-24 h) predominated in ToF (5 cases), truncus arteriosus (4 cases), and TGA (3 cases). A moderate correlation was found between reintubation and mortality (Spearman's r = 0.45, p < 0.01). On-table extubation is a safe and feasible strategy in pediatric cardiac surgery, particularly in corrective procedures and select single-ventricle palliation. However, caution is warranted in high-risk physiologies such as HLHS and shunt-dependent circulation. Careful perioperative evaluation remains essential for optimal outcomes.

PMID:40533645 | DOI:10.1007/s00246-025-03920-7

J Am Coll Cardiol. 2025 Jun 24;85(24):2386-2398. doi: 10.1016/j.jacc.2025.04.028.

ABSTRACT

BACKGROUND: Survival for hypoplastic left heart syndrome (HLHS) and variants has improved over the past 4 decades; however, survival remains low compared with other forms of congenital heart disease. There is a paucity of data concerning long-term outcomes.

OBJECTIVES: This study aims to: 1) examine long-term survival and the impact of patient factors on survival for newborns with HLHS; and 2) examine functional and health outcomes, including quality-of-life (QOL) in adulthood.

METHODS: The study cohort included patients with HLHS and variants undergoing the Norwood or hybrid procedure between January 1984 and December 2023. Data on patient characteristics and management were abstracted from medical records. Vital status was ascertained by direct subject and family contact, review of the medical record, and publicly available data. Functional outcomes and QOL in adults (≥18 years of age) were assessed by patient reports. The primary outcome was death or cardiac transplantation at last follow-up.

RESULTS: In the study period, 2,012 neonates underwent staged reconstructive surgery for HLHS (Norwood, n = 1,921 and hybrid, n = 91). Transplant-free survival was 31.0% at 35 years. Transplant-free survival improved over time but is not different across recent eras. Most responders reported good to excellent general health.

CONCLUSIONS: In this cohort of newborns undergoing staged reconstructive surgery for HLHS, fewer than one-third are alive without a transplant at 35 years of age. Survival has not improved in recent years. However, there is a group of survivors who report good to excellent outcomes and QOL, consistent with a "high-performing" Fontan phenotype.

PMID:40533128 | DOI:10.1016/j.jacc.2025.04.028

J Invasive Cardiol. 2025 Jun 5. doi: 10.25270/jic/25.00142. Online ahead of print.

NO ABSTRACT

PMID:40532221 | DOI:10.25270/jic/25.00142

Braz J Cardiovasc Surg. 2025 Jun 18;40(4):e20240442. doi: 10.21470/1678-9741-2024-0442.

ABSTRACT

INTRODUCTION: The Norwood operation has transformed the approach to hypoplastic left heart syndrome and its variants. Given the complexity of this procedure, postoperative residual injuries are prevalent.

OBJECTIVE: To evaluate the impact of significant residual injuries on clinical outcomes and mortality in Norwood procedure patients at a high-volume tertiary center in a developing nation using the technical performance score (TPS).

METHODS: This single-center, retrospective study included patients who underwent the Norwood procedure between December 2018 and February 2023. Data on demographics, echocardiograms, complications, intensive care unit stay, and mortality were collected. Logistic regression and linear analyses assessed the impact of TPS on outcomes.

RESULTS: Of 69 patients, nine (13%) were excluded due to incomplete echocardiographic data, leaving 60 (87%) for TPS classification. Among them, 28 (47%) were male. TPS classification was as follows: 40 (66%) in class 1 (excellent), five (8.3%) in class 2 (adequate), and 15 (25%) in class 3 (inadequate), indicating significant residual lesions or need for reintervention. The 30-day mortality rate was 21.6%, increasing to 41.6% before the next stage. In TPS class 3, 30-day mortality was 33% vs. 17% in classes 1 and 2 (P = 0.27). Interstage mortality was 60% in class 3 compared to 35% in other groups (P = 0.13). Major complications were significantly higher in TPS class 3 (93% vs. 55.5%, P = 0.04).

CONCLUSION: TPS effectively predicts major complications post-Norwood and serves as a valuable tool for improving patient outcomes.

PMID:40530992 | PMC:PMC12175616 | DOI:10.21470/1678-9741-2024-0442

J Thorac Dis. 2025 May 30;17(5):2980-2988. doi: 10.21037/jtd-2024-2185. Epub 2025 May 28.

ABSTRACT

BACKGROUND: Various surgical techniques have been reported for repairing Ebstein anomaly. Cone repair provides nearly anatomical tricuspid valve (TV) reconstruction with promising outcomes. We reviewed our experience with cone repair to evaluate biventricular remodeling and the outcomes of the annular support procedure.

METHODS: Between January 2008 and December 2021, cone repair was performed in 33 consecutive patients with Ebstein anomaly. Mean age was 32.0±16.8 years (range, 1.1-66.8 years). Previous TV repair had been performed in two patients with the Hetzer and the Carpentier techniques (6%). Severe tricuspid regurgitation (TR) was observed in 32 patients (97%). Twenty patients had preoperative magnetic resonance imaging (MRI) data; 15 patients had postoperative MRI data.

RESULTS: Modifications included the addition of an annuloplasty band (21 patients with pericardial strips, two patients with prosthetic rings) in 23 patients (69.7%) and papillary muscle repositioning in one patient (3%). Bidirectional cavopulmonary anastomosis was performed in two patients (6%), with one of them undergoing a Fontan operation in the third postoperative years. No mortality was observed. The mean follow-up duration was 7.5±4.6 years. Two patients (6%) required late TV re-repair in the first and sixth postoperative years. At follow-up, five patients (16.1%) reported no or trivial TR, 16 (51.6%) had mild TR, and 5 (16.1%) had mild to moderate TR. Freedom from late TV reoperation was 78.8%±13.4% at 5 years. The TV reoperation rate was significantly low in the patients who underwent tricuspid annuloplasty with a band (P=0.02). Preoperative and postoperative MRI data demonstrated a significant right ventricular (RV) volume decrease after cone repair [RV end-diastolic volume index (mL/m2): preoperative/postoperative =207.4±40.2/105.5±41.3, P=0.001]. Left ventricular ejection fraction (LVEF) remained unchanged after cone repair, while left ventricular stroke volume (LVSV) significantly increased [LVEF (%): preoperative/postoperative =60.8±5.3/61.2±5.4, P=0.10; LVSV (mL): preoperative/postoperative =64.0±1.8/71.4±12.7, P=0.041].

CONCLUSIONS: Cone repair for Ebstein anomaly has low mortality and morbidity rates. The addition of an annuloplasty band was associated with a low incidence of the TV reoperation. Moreover, cone repair might impact left ventricular function due to ventricular interdependency. Longer follow-up is essential to determine the late durability of cone repair and both ventricular functional changes.

PMID:40529772 | PMC:PMC12170008 | DOI:10.21037/jtd-2024-2185

Ann Thorac Surg Short Rep. 2024 Nov 6;3(2):368-372. doi: 10.1016/j.atssr.2024.10.016. eCollection 2025 Jun.

ABSTRACT

BACKGROUND: The objective of this study was to evaluate long-term outcomes for valve-sparing root replacement (VSRR) in patients with congenital aortopathy.

METHODS: In 107 patients undergoing VSRR in an elective setting with preoperative data from 1998 to 2022, information about operative strategy and postoperative course was collected. Patients were assigned to the congenital aortopathy group if a genetic mutation was confirmed or if a bicuspid aortic valve was present and compared with patients with tricuspid aortic valve. Long-term follow-up was obtained through outpatient visits or by telephone interviews.

RESULTS: Fifty-two percent (n = 56) were congenital aortopathy patients. Patients without congenital aortopathy were older (P < .001) and had higher rates of preoperative hypertension (P = .030) and hyperlipidemia (P = .001). Early survival was excellent with no in-hospital death. Postoperative echocardiography showed a good result, with aortic regurgitation being mild or less in 93% (n = 99). Nevertheless, aortic valve reoperation was needed in 7% of patients. Rates of transient ischemic attacks (2%), strokes (5%), and need for pacemaker implantation (4%) during follow-up were comparable between groups. There was no difference in long-term outcome or need for aortic valve reoperation between the groups.

CONCLUSIONS: VSRR in congenital aortopathy patients has good long-term results. During follow-up, the rate of thromboembolic events might have an impact on optimal medical therapy.

PMID:40525198 | PMC:PMC12167554 | DOI:10.1016/j.atssr.2024.10.016

Ann Thorac Surg Short Rep. 2024 Dec 16;3(2):469-472. doi: 10.1016/j.atssr.2024.12.001. eCollection 2025 Jun.

ABSTRACT

Automated implantable cardioverter defibrillator device implantation in young children is a rare procedure that typically necessitates intrapericardial lead placement. The extravascular implantable cardioverter defibrillator device can be implanted with the patient in a substernal position and with a straightforward and minimally invasive technique. The largest incision is made to accommodate the generator device. The alignment of the coils and device provides a good vector for defibrillation while permitting somatic growth. Here we describe implantation of an extravascular implantable cardioverter defibrillator in a 2-year-old child, among the youngest patients to receive this device to date.

PMID:40525144 | PMC:PMC12167553 | DOI:10.1016/j.atssr.2024.12.001

Int J Cardiol Congenit Heart Dis. 2025 Apr 3;20:100580. doi: 10.1016/j.ijcchd.2025.100580. eCollection 2025 Jun.

ABSTRACT

BACKGROUND: The diagnosis and management of atrioventricular (AV)-conduction disorders in patients with a Fontan circulation can be challenging. Little is known about the effects of various pacing strategies in single-ventricle patients. Here we report 1) the feasibility of transesophageal electrophysiological study (EPS) to assess AV-conduction in a patient with limited venous access and 2) the potential of echocardiography to guide epicardial systemic right ventricular (sRV) lead positioning and to evaluate the hemodynamic consequences of sRV pacing in order to mitigate long-term effects of single site ventricular pacing.

MATERIAL AND METHODS: A 21-year old male with hypoplastic left heart syndrome, palliated with Norwood and Glenn procedures, and ultimately extracardiac total cavopulmonary connection was seen for a regular check-up. He reported difficulty cycling against the wind. During exercise stress test, a 2:1 AV-block occurred at atrial frequencies >100 bpm with recovery of 1:1 AV-conduction at sinus rates of 80-100 bpm. In order to discriminate between a 2:1 conducted atrial tachycardia and an impaired anterograde AV-conduction during sinus rhythm in the setting of bilateral femoral vein and unilateral subclavian/jugular vein occlusion, EPS by transesophageal pacing was proposed.

RESULTS: Bipolar transesophageal pacing of the left atrium confirmed an anterograde AV-Wenckebach point at 103 bpm, confirming the indication for AV-sequential pacing. Epicardial leads were surgically placed on the atrium and sRV apex. During intraoperative sRV pacing, transesophageal echocardiography confirmed the ventricular contraction pattern to remain synchronous with stable estimated cardiac output. Transthoracic echocardiography was performed postoperatively to assess the effects of sRV pacing on ventricular (dys)synchrony, systolic function and estimated cardiac output. These parameters remained unchanged during sRV pacing, compared to intrinsic conduction, an important finding in light of preserving sRV function.

CONCLUSIONS: EPS to assess AV conduction could safely be performed by transesophageal pacing in this patient with Fontan circulation. Moreover, echocardiographic guidance of epicardial sRV pacemaker lead placement was feasible and may help to define the optimal pacing site in Fontan patients.

PMID:40524979 | PMC:PMC12167887 | DOI:10.1016/j.ijcchd.2025.100580

Respirol Case Rep. 2025 Jun 16;13(6):e70223. doi: 10.1002/rcr2.70223. eCollection 2025 Jun.

ABSTRACT

The association of two rare but important congenital conditions-congenital chylothorax (CCT) and congenital pulmonary airway malformation (CPAM)-can be challenging to manage, especially in the absence of well-established protocols. We report an association between CPAM and CCT in a newborn. After birth, CCT did not respond to conservative treatment, and at the time of CPAM resection, thoracic duct ligation and abrasive pleurodesis were also performed. Despite these interventions, the CCT persisted even with the subsequent administration of octreotide and propranolol. Finally, after 56 days, chemical pleurodesis with povidone-iodine was performed. Chest tube drainage ceased, and the thoracic drain was removed 4 days later. Conservative treatment remains the first-line approach for neonatal CCT. However, when CCT is associated with CPAM and fails to respond to conservative measures, thoracic duct ligation should be considered at the time of lung malformation resection. If these interventions remain ineffective, chemical pleurodesis is a viable therapeutic option.

PMID:40524891 | PMC:PMC12168237 | DOI:10.1002/rcr2.70223

Am Heart J. 2025 Jun 14:S0002-8703(25)00197-8. doi: 10.1016/j.ahj.2025.06.008. Online ahead of print.

ABSTRACT

BACKGROUND: The long-term effect of open fenestration in Fontan patients is unclear, leading to wide practice variation of fenestration creation and closure. We evaluated the long-term outcomes of the fenestration using data from the Fontan Outcome Registry using Cardiac magnetic resonance Examinations (FORCE) study.

METHODS: Patients were categorized by fenestration status determined by post-Fontan cardiac magnetic resonance imaging (CMR) as open fenestration, non-fenestrated Fontan, spontaneous closure, and device closure. The primary outcome was the time from the CMR to the earliest event of death, listing or receiving a heart transplant, plastic bronchitis, or protein-losing enteropathy. The association between fenestration status and the outcome measure was evaluated using Cox proportional hazard models, adjusted for patients' clinical and CMR characteristics.

RESULTS: The cohort consisted of 2,923 patients with a median age at CMR of 14.3 years. Patients with open fenestration were younger and less likely to have a systemic left ventricle. Non-fenestrated Fontan patients were more likely to have a systemic left ventricle and lower indexed single ventricle end-diastolic volume (SVEDVi). An open fenestration was associated with adverse outcomes adjusted for clinical variables (hazard ratio 1.70, 95% CI [1.09, 2.64], p=0.02). The association was no longer significant when adjusted for CMR variables, while every 10ml/m2 increase in SVEDVi was associated with a 5% increase in the hazard of clinical outcomes (p<0.0001).

CONCLUSIONS: Open fenestration is associated with adverse outcomes when adjusted for clinical characteristics. The association disappeared when additionally adjusting for CMR variables. The current practice of fenestration closure in selected patients leads to comparable outcomes with spontaneous closure and non-fenestrated Fontan.

PMID:40523442 | DOI:10.1016/j.ahj.2025.06.008

J Neurosurg Case Lessons. 2025 Jun 16;9(24):CASE24894. doi: 10.3171/CASE24894. Print 2025 Jun 16.

ABSTRACT

BACKGROUND: Neuroenteric cysts are exceedingly rare congenital anomalies. Although the exact pathogenesis is incompletely understood, neuroenteric cysts can arise when foregut duplication cysts extend into the spinal canal. This process is likely related to failure of endodermal cell separation from ectodermal counterparts during week 3 of development, and symptoms depend on location and degree of encroachment on surrounding structures. Complete resection remains the treatment of choice with the lowest incidence of recurrence, but the optimal surgical approach is debated. Associated congenital anomalies can add surgical complexity.

OBSERVATIONS: The authors present the case of a 7-week-old infant diagnosed with a large mediastinal extradural neuroenteric cyst, along with multiple other congenital anomalies. A combined thoracic and laparoscopic abdominal approach was required for complete resection.

LESSONS: This case illustrates the importance of multispecialty collaboration, advanced imaging for preoperative planning, and thoughtful timing of intervention. A combined thoracoscopic/thoracotomy and laparoscopic approach, while surgically demanding, was safe and provided excellent visibility for complete resection of the large neuroenteric cyst. https://thejns.org/doi/10.3171/CASE24894.

PMID:40523345 | PMC:PMC12171098 | DOI:10.3171/CASE24894

J Extra Corpor Technol. 2025 Jun;57(2):66-73. doi: 10.1051/ject/2024037. Epub 2025 Jun 16.

ABSTRACT

Medical procedural registries are uniquely positioned to support shared decision-making through risk prediction modeling, support quality assessment and improvement through performance benchmarking, and provide public reporting of evidence-based practices and outcomes. For example, the Centers for Disease Control and Prevention (CDC) consulted the Extracorporeal Life Support Organization (ELSO) registry to assess the severity of the swine flu outbreak in 2009-2010. The development and growth of The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) has positively contributed to the congenital heart surgery community by developing objective mortality STAT categories and complexity stratification for operations, a common nomenclature for classifying operations and reporting the costs associated with complications for nine benchmark operations. Within the setting of adult cardiac surgery, the Perfusion Down Under Collaborative has used its registry to develop quality improvement initiatives, including those related to the management of arterial outlet temperature, glucose, and arterial pCO2. The PERForm registry leverages data from nearly 50 US hospitals to support targeted quality improvement initiatives within the setting of adult cardiac surgery. The PERForm registry participants receive benchmark reports and participate in quarterly collaborative learning meetings noted for unblinding hospital performance data. In 2014, with no current congenital cardiopulmonary bypass (CPB) registries, various experts within the congenital perfusion community and leaders from the PERForm registry began working to develop a pediatric perfusion registry. From this work, the PediPERForm Learning Network (PLN) and its associated congenital perfusion registry became active and began collecting data in October 2021.

PMID:40523133 | PMC:PMC12169737 | DOI:10.1051/ject/2024037

Front Cardiovasc Med. 2025 May 30;12:1556289. doi: 10.3389/fcvm.2025.1556289. eCollection 2025.

ABSTRACT

BACKGROUND: Patients with congenital heart disease (CHD) who are operated on after birth are at risk for neurodevelopmental (ND) impairment. Before birth, altered fetal hemodynamics due to the CHD may lead to reduced cerebral perfusion and oxygen supply. The placenta as a critical organ may enhance this pathology.

METHODS: Neonates with operated complex CHD were included. We scored the placental pathology and analyzed structural and volumetric brain changes of perioperative brain MRI and ND outcome data using the Bayley III at 1 year of age.

RESULTS: A total of 45 (13 female) patients with D-transposition of the great arteries (n = 19, 42.2%), single ventricle CHD (n = 14, 31.1%), left ventricular outflow tract CHD (n = 7, 15.6%), and other (n = 5, 11.1%) were analyzed. Placental findings were abnormal in 21 of 45 patients (46.7%). Pre- and postoperative cMRI were analyzed in 26 (57.8%) and 36 (80%) patients, respectively, while 18 (40%) patients had both (pre-/postoperative) cMRI. Half of our patients had structural brain lesions before (50%) and after (52.8%) surgery, mild intracerebral hemorrhages (pre, 11.1%; post, 22.2%), small cerebral strokes (pre/post, 8.9%), white matter injury (pre/post, 0%/4.5%), and mild hypoxia (pre/post, 4.5%). Abnormal placental findings were not associated with more structural brain lesions but were associated with smaller total brain volumes, cortical gray matter, and cerebellar structures (all p < 0.05), but not with ND outcome at 1 year of age.

CONCLUSIONS: Abnormal placental findings in patients with complex CHD are associated with smaller brain volumes, underlining the impact of placental function on brain development as a cofactor in patients with CHD.

PMID:40520938 | PMC:PMC12162681 | DOI:10.3389/fcvm.2025.1556289

J Am Heart Assoc. 2025 Jun 17;14(12):e041384. doi: 10.1161/JAHA.125.041384. Epub 2025 Jun 16.

ABSTRACT

BACKGROUND: Literature reporting neurodevelopmental outcomes for patients who undergo ventricular assist device (VAD) therapy is limited to posttransplant cohorts. This study aims to determine the prevalence of optimal neurodevelopmental outcome and factors associated with nonoptimal outcome in patients implanted with a VAD at ≤15 months of age.

METHODS: Patients followed by the Complex Pediatric Therapies Follow-Up Program were included in a prospective-inception cohort study if born between January 2006 and December 2022 and implanted with a VAD at ≤15 months of age. A modified optimal neurodevelopmental outcome was defined as scores of ≥80 on the Bayley Scales of Infant and Toddler Development and on the Adaptive Behavior Assessment System, and in the absence of cerebral palsy, permanent hearing loss, visual impairment, or seizure disorder. Firth multiple regression analysis was used to determine independent factors associated with nonoptimal outcome.

RESULTS: A total of 56 patients underwent VAD implant at ≤15 months with neurodevelopmental assessments available for 39/40 patients who survived to 2 years. The mean age of VAD implant was 5.45 (SD 3.99) months, 69.2% were male, and 38.5% had congenital heart disease. Optimal neurodevelopmental outcome was seen in 25.6% of patients. Neurological insult (OR, 12.34 [95% CI, 1.29-1660.36], P=0.026) was the only independent factor identified associated with nonoptimal outcome.

CONCLUSIONS: Optimal outcome was demonstrated in one quarter of patients who had a VAD at ≤15 months of age and underwent neurodevelopmental testing at 2 years of age. A potentially modifiable factor of neurological insult was demonstrated as being independently associated with nonoptimal outcome.

PMID:40519203 | DOI:10.1161/JAHA.125.041384

J Cardiothorac Vasc Anesth. 2025 May 15:S1053-0770(25)00375-1. doi: 10.1053/j.jvca.2025.05.016. Online ahead of print.

NO ABSTRACT

PMID:40518335 | DOI:10.1053/j.jvca.2025.05.016

J Cardiothorac Vasc Anesth. 2025 May 24:S1053-0770(25)00431-8. doi: 10.1053/j.jvca.2025.05.041. Online ahead of print.

ABSTRACT

OBJECTIVES: To investigate the relationship between the pulmonary-to-systemic-blood-flow ratio (Qp/Qs) and the cross-sectional area (CSA) and diameter of the right internal jugular vein (RIJV) in pediatric patients with congenital heart disease using multivariate analysis.

DESIGN: Retrospective study.

SETTING: Operating room of a tertiary children's hospital.

PARTICIPANTS: Pediatric patients aged <10 years with congenital heart disease who underwent cardiovascular surgery under general anesthesia.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: Multiple linear regression analyses were performed, with the CSA of the RIJV as the primary outcome and the anteroposterior and transverse diameters of the RIJV as secondary outcomes. These were designated as dependent variables, while Qp/Qs served as the independent variable. The analyses were adjusted for the following factors: weight, sex, postbidirectional Glenn operation, preoperative diuretic use, number of previous median sternotomies, single ventricle, trisomy 21, superior vena cava pressure, systolic blood pressure, heart rate, hemoglobin concentration, partial pressure of carbon dioxide, pH of arterial blood, and cardiac index. The adjusted regression coefficient estimates for Qp/Qs were as follows: 0.88 (95% confidence interval: 0.84-0.94, p < 0.001), 0.94 (95% confidence interval: 0.92-0.97, p < 0.001), and 0.94 (95% confidence interval: 0.91-0.97, p < 0.001) in the CSA, anteroposterior diameter, and transverse diameter of the RIJV, respectively.

CONCLUSIONS: A higher Qp/Qs was associated with a smaller CSA, as well as reduced anteroposterior and transverse diameters of the RIJV. Specifically, for each unit increase in Qp/Qs, the CSA of the RIJV decreased by approximately 12%.

PMID:40518334 | DOI:10.1053/j.jvca.2025.05.041

Heart Lung Circ. 2025 Jun 13:S1443-9506(25)00135-0. doi: 10.1016/j.hlc.2025.01.010. Online ahead of print.

ABSTRACT

AIM: Atrial septal defects (ASD) are the most common congenital cardiac malformations. Over the preceding decades, a host of minimally invasive and interventional techniques for ASD closure have emerged. Minimally invasive ASD (MIASD) repair utilises thoracotomies, endoscopic, robotic, and even beating heart approaches to facilitate MIASD repair. We performed a systematic review to summarise the literature describing the outcomes of patients following MIASD repair.

METHOD: PubMed, Medline, and Scopus were searched by two authors for manuscripts describing outcomes following MIASD repair. Overall, 1,972 titles were screened, 1,945 were excluded, and 27 were included in the review. The primary outcome was mortality and secondary outcomes included postoperative morbidity.

RESULTS: Measures of mortality were largely limited to 30 days postoperatively, with 0% mortality reported for MIASD up to 30 days. Rates of postoperative morbidity were low in both sternotomy and MIASD groups, with no consistent differences identified. Rates of intensive care unit and hospital stay were consistently reported to be lower with MIASD than with the conventional approach.

CONCLUSIONS: MIASD repairs have continued to evolve and improve. Among the various approaches to MIASD repair, all have been associated with low rates of morbidity and mortality. Surgical ASD repair continues to be versatile, allowing for repair of all manner of ASDs, and allowing for the concomitant management of other cardiac conditions. Regular advancements in this field, including the optimisation and development of surgical techniques with regular evaluation of patient outcomes, will ensure appropriate patient selection and optimisation of outcomes for patients with ASD.

PMID:40517112 | DOI:10.1016/j.hlc.2025.01.010