Cirugía congénitos

Kyobu Geka. 2025 Jun;78(6):444-447.

ABSTRACT

Pulmonary enteric adenocarcinoma is a rare subtype of adenocarcinoma that was introduced in the 2015 World Health Organization( WHO) Histological Classification and Lung Cancer Treatment Protocol (8th Edition). Distinguishing it from lung metastases of colorectal cancer poses a significant clinical challenge. We report three cases of pulmonary enteric adenocarcinoma with distinct computed tomography(CT) findings. Pulmonary enteric adenocarcinoma typically presents as a large mass, often characterized as substantial and lacking a significant ground glass shadow. However, in the first case, a partially filled nodular shadow was observed;in the second case, a well-difined cystic-like mass shadow was identified;and in the third case, a nodular shadow with an internal cavity was noted.

PMID:40589021

Neoreviews. 2025 Jul 1;26(7):e477-e489. doi: 10.1542/neo.26-7-034.

ABSTRACT

Double outlet right ventricle (DORV) is a complex congenital heart disease with a wide spectrum of anatomical variations and clinical presentations. In this conotruncal anomaly, greater than 50% of the semilunar valve diameter of both the aorta and pulmonary artery arises from the morphologic right ventricle. Additionally, patients with DORV have a ventricular septal defect that allows blood to egress from the left ventricle and may obstruct either the pulmonary or systemic circulations. Physiological characteristics differ among patients based on the orientation of the great arteries, the presence of additional intracardiac shunts, and cardiac abnormalities. A thorough understanding of an individual's clinical features based on their DORV phenotype is essential for clinicians caring for these vulnerable patients. Knowledge of the expected postnatal hemodynamic derangements as transitional physiology evolves allows for tailored therapeutic approaches at the bedside. In this review, we will discuss the main DORV subtypes, delineate the physiology of each subtype, and provide clinical considerations and medical management strategies to guide the care for patients with this complex cardiac lesion.

PMID:40588272 | DOI:10.1542/neo.26-7-034

J Neurosurg Case Lessons. 2025 Jun 30;9(26):CASE25253. doi: 10.3171/CASE25253. Print 2025 Jun 30.

ABSTRACT

BACKGROUND: Neurenteric cysts are rare congenital lesions of the CNS, typically found in the cervical or thoracic spine and presenting in early life. Only 5% of spinal neurenteric cysts are intramedullary. The authors report the case of an intradural intramedullary neurenteric cyst in a 68-year-old woman.

OBSERVATIONS: The patient presented with 4 months of progressive left extremity numbness, weakness, and imbalance consistent with cervical myelopathy. MRI revealed a 1-cm cystic lesion in the intradural intramedullary cervical spine. Multilevel posterior cervical laminectomy for cyst resection was performed with gross-total resection. Histopathological analysis revealed squamous, columnar, and pseudostratified epithelium positive for epithelial membrane antigen and cytokeratin and negative for glial fibrillary acidic protein and S100 protein, consistent with a neurenteric cyst. Her symptoms and examination significantly improved after surgery.

LESSONS: Neurenteric cysts comprise 1% of spinal lesions, and 5% are intradural intramedullary. Symptoms progress gradually but may fluctuate with cyst size changes. MRI is the preferred imaging, with histopathological analysis required for diagnosis. Operative intervention for gross-total resection is the recommended management. This case emphasizes that neurenteric cysts should be considered in older adults with cervical myelopathy and a cystic intramedullary mass and demonstrates the benefit of complete surgical removal. https://thejns.org/doi/10.3171/CASE25253.

PMID:40587885 | PMC:PMC12210062 | DOI:10.3171/CASE25253

Am J Case Rep. 2025 Jun 30;26:e947068. doi: 10.12659/AJCR.947068.

ABSTRACT

BACKGROUND Renal ectopia (RE) is a congenital malformation in which one or both kidneys fail to reach their normal anatomical position. Most cases are asymptomatic, but they can occasionally lead to complications. This case report presents the incidental finding of a pelvic ectopic kidney during a pre-transcatheter aortic valve replacement (TAVR) evaluation, showing the importance of recognizing anatomical variations in surgical planning for vascular access. CASE REPORT A 68-year-old man undergoing evaluation for severe symptomatic aortic stenosis was incidentally found to have a left pelvic kidney on computed tomography (CT) angiography. The CT scan revealed a dual arterial supply, with one artery entering the kidney medially and an accessory artery wrapping laterally around the kidney, which may have contributed to the failure of its ascent during embryological development. He had no urinary symptoms, and renal function was normal, so no further intervention was required. The identification of this vascular anomaly emphasizes the need for proper preoperative imaging to avoid complications. CONCLUSIONS Recognizing anatomical variations like renal ectopia can help optimize complex vascular procedure planning and prevent complications. Additionally, this case highlights the association between multiple renal arteries and renal ectopia, suggesting further research is needed to understand these correlations.

PMID:40587412 | DOI:10.12659/AJCR.947068

Ann Afr Med. 2025 Jun 30. doi: 10.4103/aam.aam_244_24. Online ahead of print.

ABSTRACT

The thoracic duct is susceptible to damage from a variety of surgical operations, including esophagectomy, lung resection, and mediastinal or aortic surgery, because of its anatomical placement. Lymphatic leaks are known as chylothorax, which is a common complication of coronary artery bypass grafting. However, In congenital cardiac surgeries, the incidence of chylothorax ranges from 2.5% to 4.7%, while in esophageal procedures, it is 0.4%-4%. There have been a few reports of a chylous lymphatic leak following coronary bypass surgery. In this case report, we would like to present the anesthetic management of a chyle leak that developed a couple of days following triple coronary arterial bypass surgery, which was successfully managed with suprainguinal fascia iliaca compartment block for the embolization of the lymphatic duct.

PMID:40587160 | DOI:10.4103/aam.aam_244_24

Multimed Man Cardiothorac Surg. 2025 Jun 30;2025. doi: 10.1510/mmcts.2025.060.

ABSTRACT

Coronary artery aneurysms are exceedingly rare and are most often discovered incidentally. Rupture of such aneurysms is even more uncommon. In this case report, we describe an 82-year-old patient who presented with sudden-onset chest pain and was diagnosed with a fissurating aneurysm of the right coronary artery. The aneurysm was associated with a congenital abnormality of the coronary venous sinus return. The patient underwent emergency surgery. Given her advanced age, the surgical strategy focused solely on addressing the imminent risk of rupture. A 10-mm Gore-Tex graft was implanted because direct suture repair or vein grafting was deemed unsuitable. The patient recovered well postoperatively and was given dual antiplatelet therapy. At nine months of follow-up, she remains asymptomatic, with no recurrence of chest pain.

PMID:40586769 | DOI:10.1510/mmcts.2025.060

Oxf Med Case Reports. 2025 Jun 27;2025(6):omaf074. doi: 10.1093/omcr/omaf074. eCollection 2025 Jun.

ABSTRACT

Cystic hygroma is a rare congenital anomaly typically observed in newborns, with cases in adults being exceptionally rare. In adults, these lesions often remain asymptomatic and present unique challenges owing to the absence of established treatment protocols. This report describes a 23-year-old male with a large pulmonary cystic hygroma manifesting as progressive chest discomfort, breathing difficulties, and systemic symptoms. Diagnostic imaging revealed an extensive cystic structure within the mediastinal-pulmonary region, which was managed surgically via cystotomy. Histopathological analysis confirmed the diagnosis, and the patient experienced a complete recovery with no recurrence during follow-up. This case highlights the need for standardized guidelines in the management of pulmonary cystic hygromas and emphasizes the importance of including cystic hygroma in the differential diagnosis of pulmonary masses in adults. Further studies are essential to optimize therapeutic approaches and enhance outcomes for adult patients with this rare condition.

PMID:40585459 | PMC:PMC12202292 | DOI:10.1093/omcr/omaf074

Phys Med Rehabil Clin N Am. 2025 Aug;36(3):647-662. doi: 10.1016/j.pmr.2025.03.008. Epub 2025 May 24.

ABSTRACT

Hospitalized pediatric heart transplant (PHT) recipients face unique and multifaceted challenges that impact their functional outcomes, including motor skills, activities of daily living, feeding, and communication. Perioperative complications, lengthy hospitalizations, physical deconditioning, and comorbidities associated with complex congenital heart disease are important considerations as they can have a profound impact on their functional abilities and progress of individuals toward achieving independence. This article explores the role of rehabilitation providers in addressing these functional challenges through performance-based outcome measures and aims to support the development of tailored rehabilitation programs to achieve improved quality of life and long-term independence for PHT recipients.

PMID:40581444 | DOI:10.1016/j.pmr.2025.03.008

J Pediatr Surg. 2025 Jun 26:162427. doi: 10.1016/j.jpedsurg.2025.162427. Online ahead of print.

ABSTRACT

AIM: To describe the collective experience of six international tertiary paediatric surgery centres performing thoracoscopic salvage surgery for oesophageal atresia.

METHODS: Retrospective review of all neonates undergoing thoracoscopic repair of oesophageal atresia following a previous failed repair via thoracotomy, from September 2018 - May 2024, at 6 international tertiary paediatric surgery centres. Data collected included demographics, co-morbidities, operative details and post-operative clinical course. Results are presented as median with range.

RESULTS: 24 babies were included. Median gestational age was 34 weeks (26-40), birthweight was 1858g (780-3300). 19 were Gross type C (79%), 4 type B (17%), with 1 type A (4%). 7/24 (29%) had thoracoscopic traction sutures, and 2/24 (8%) had a cervical oesophagostomy formed prior to thoracoscopic repair. Definitive thoracoscopic repair was undertaken at 23 weeks (7-116) after initial thoracotomy, at 6 months of age (1-26) and weight of 5340g (1050-1100). Operative time was 245 minutes (120-585). 23/24 (96%) were completed thoracoscopically. Follow-up was 36 months (7-130). 17/24 (71%) developed an oesophageal stricture requiring a median of 5 dilatations (1-45). 2/24 (8%) developed a fistula to the airway. 2/24 (8%) developed significant gastro-oesophageal reflux disease requiring a fundoplication. One patient required an aortopexy and re-do aortopexy for management of tracheomalacia. There was 1 death at 11 months of age (2 months post definitive repair) in a patient with complex congenital cardiac disease. 22/23 (96%) patients are feeding exclusively orally.

CONCLUSION: thoracoscopic salvage surgery in oesophageal atresia when undertaken by experienced surgeons is feasible, safe and with good clinical outcomes.

PMID:40581151 | DOI:10.1016/j.jpedsurg.2025.162427

Eur J Cardiothorac Surg. 2025 Jun 19:ezaf205. doi: 10.1093/ejcts/ezaf205. Online ahead of print.

ABSTRACT

OBJECTIVES: To evaluate gender authorship in two cardio-thoracic surgical journals.

METHODS: We performed a bibliometric analysis of all articles published from 2017 to 2022 in the European Journal of Cardio-Thoracic Surgery and the Interdisciplinary Cardiovascular and Thoracic surgery. For each article, the gender and academic rank of the first, senior and corresponding author was verified by Internet search, email contact or use of the application Genderize.io. Articles were categorized based on topic, type and country of origin. The Cochran-Armitage test was used to evaluate gender authorship trend over time.

RESULTS: 5243 articles were included in the analysis. Women represented 18% of first authors, 7% of senior authors and 13% of corresponding authors and no trend was seen over time. Women represented 16% of first authors and 7% of senior authors in adult cardiac surgery, 23% of first authors and 9% of senior authors in congenital cardiac surgery, and 19% of first authors and 8% of senior authors in thoracic surgery. Male first authors were more frequently full professor (17% vs 5%) and associate professor (16% vs 8%) and male senior authors were more frequently full professor (48% vs 31%) and associate professor (16% vs 8%) compared to female.

CONCLUSIONS: The proportion of female authors is significantly lower than that of male authors in highest-impact European cardio-thoracic surgery journals and no significant increase in female authorship has been demonstrated in recent years. Increasing awareness of gender disparities is essential to facilitate equal career opportunities and academic advancement for women in cardio-thoracic surgery.

PMID:40581079 | DOI:10.1093/ejcts/ezaf205

J Cardiothorac Vasc Anesth. 2025 May 30:S1053-0770(25)00451-3. doi: 10.1053/j.jvca.2025.05.059. Online ahead of print.

ABSTRACT

OBJECTIVES: To evaluate the prevalence of chronic postsurgical pain (CPSP) in children after cardiac surgery.

DESIGN: Cohort study.

SETTING: Quaternary academic pediatric hospital.

PARTICIPANTS: Patients age 6 to 18 years at the time of their surgery and a surgery date ≥6 months prior to the study period (June 1, 2014, to November 1, 2021).

INTERVENTIONS: Patients completed 3 validated surveys measuring chronic pain, the impact of pain on quality of life (QoL), and neuropathic pain symptoms. Respondents' medical records were then reviewed for perioperative management.

MEASUREMENTS AND MAIN RESULTS: Pain severity, pain trajectory, and analgesic consumption are described for each comparative group of interest (time period, 2014-2019 vs 2020-2021), intraoperative opioid selection (remifentanil vs sufentanil), surgical approach (sternotomy vs thoracotomy), and intraoperative pain management technique (presence/absence of fascial plane block). The response rate was 10%. Reference tables from validated scoring systems were used when available. At the time of survey completion or in the preceding week, 34% reported pain, with none to mild interference in QoL. Twenty percent reported symptoms consistent with neuropathic pain, with no difference between recipients of sternotomy and recipients of thoracotomy. Patients who received intraoperative remifentantil had higher pain scores. Single-shot fascial plane blocks reduced postoperative acute pain scores.

CONCLUSIONS: The validated surveys showed a high prevalence of CPSP, including a sizeable proportion of neuropathic pain. The prevalence of CPSP is within the reported prevalence in adults despite a mean study population age of 10 years. This finding encourages improvements in acute pain management, earlier identification of patients with CPSP for management, and further investigation of CPSP after pediatric cardiac surgery.

PMID:40579245 | DOI:10.1053/j.jvca.2025.05.059

Heart. 2025 Jun 27:heartjnl-2025-325811. doi: 10.1136/heartjnl-2025-325811. Online ahead of print.

NO ABSTRACT

PMID:40579215 | DOI:10.1136/heartjnl-2025-325811

Heart. 2025 Jun 27:heartjnl-2024-325296. doi: 10.1136/heartjnl-2024-325296. Online ahead of print.

ABSTRACT

BACKGROUND: The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries.

METHODS: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease-International Study II (APPROACH-IS II) represents an international cross-sectional investigation of PROs in 8415 patients from 53 centres across 32 countries. Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8, PHQ-8); anxiety (Generalized Anxiety Disorder Scale-7) and QoL (Linear Analog Scale). Gross National Income per capita in US dollars was used for stratifying countries according to income levels.

RESULTS: Large intercountry disparities in PROs were observed. Switzerland demonstrated the highest mean scores for physical functioning, self-rated health and QoL, while Senegal had the lowest scores. Patients from Malta demonstrated the highest mean scores for mental health, and Senegal had the lowest scores. With regard to depressive symptoms and anxiety, Pakistan had the lowest mean scores, while Turkey had the highest scores. Patients from high-income nations reported significantly better physical functioning, mental functioning and QoL.

CONCLUSION: Large intercountry disparities in PROs were observed. APPROACH-IS II is a pioneering international endeavour that comprehensively evaluated PROs among adults with CHD, drawing participants from nations with different income levels.

TRIAL REGISTRATION NUMBER: NCT04902768.

PMID:40579213 | DOI:10.1136/heartjnl-2024-325296

BMJ Case Rep. 2025 Jun 27;18(6):e264831. doi: 10.1136/bcr-2025-264831.

ABSTRACT

Tricuspid atresia is a complex congenital cardiac anomaly with single ventricle physiology characterised by intracardiac mixing of oxygenated and deoxygenated blood. Managing such parturient demands an in-depth understanding of the cardiac defect, the stage of surgical palliation, functional status, and the cardiovascular impact of pregnancy and labour. This report details a female in her late twenties with tricuspid atresia and hypoplastic right ventricle, previously treated with a bidirectional Glenn shunt, who underwent a successful emergency caesarean section at 34 weeks using neuraxial anaesthesia. The case underscores the anaesthetic challenges and perioperative strategies involved in managing patients with such complex cardiac physiology.

PMID:40579205 | DOI:10.1136/bcr-2025-264831

JACC Case Rep. 2025 Jun 25;30(16):104200. doi: 10.1016/j.jaccas.2025.104200.

ABSTRACT

BACKGROUND: Cor triatriatum sinister is a rare congenital heart disease that can mimic left atrial obstructive lesions. Symptoms vary based on the severity of obstruction.

CASE SUMMARY: We report a case of a 14-year-old girl with severe exercise intolerance. Echocardiography and computed tomography scan revealed a fibromuscular membrane causing left atrial obstruction and an anomalous venous drainage with a persistent left superior vena cava to the coronary sinus. Surgical intervention is definitive, typically via sternotomy.

DISCUSSION: Given the complexity and lack of expertise in minimal invasive thoracotomy for such lesions, we used 3-dimensional (3D) heart printing for virtual surgical planning. This approach facilitated a safe and precise repair. To our knowledge, this is the first reported case using 3D printing to enable a minimally invasive thoracotomy for cor triatriatum sinister correction.

TAKE-HOME MESSAGES: Minimally invasive approaches should not be dismissed for complex congenital heart defects. Multimodal imaging can enhance surgical feasibility.

PMID:40579109 | DOI:10.1016/j.jaccas.2025.104200

JACC Case Rep. 2025 Jun 25;30(16):104341. doi: 10.1016/j.jaccas.2025.104341.

ABSTRACT

BACKGROUND: Right ventricle-pulmonary artery (RV-PA) conduit stenosis is a relatively uncommon complication after a childhood Ross-Konno procedure.

CASE SUMMARY: A man in his 20s presented with progressive dyspnea for 1 year. He had previously undergone modified Konno and Ross-Konno procedures for congenital subaortic stenosis followed by pulmonic balloon valvuloplasty for RV-PA stenosis. Echocardiography demonstrated reduced left ventricular ejection fraction, severe pulmonary hypertension, and right ventricular outflow tract obstruction. Cardiac computed tomography revealed right ventricular outflow/conduit stenosis with calcification. Right heart catheterization showed elevated right ventricular outflow/conduit pressure gradient, confirming severe conduit stenosis. He was referred for surgical pulmonic valve and conduit repair.

DISCUSSION: Surgical conduit replacement remains the most definitive treatment strategy in small, calcified conduits. Multimodal imaging strategies aid in comprehensive assessment of anatomy and function of RV-PA conduit and valves to guide intervention strategies in adults with complex congenital heart disease.

PMID:40579096 | DOI:10.1016/j.jaccas.2025.104341

JACC Case Rep. 2025 Jun 25;30(16):103884. doi: 10.1016/j.jaccas.2025.103884.

ABSTRACT

BACKGROUND: Determining the optimal surgical approach for complex congenital heart disease (CHD) can be challenging using conventional 2-dimensional transthoracic echocardiography (TTE) and cross-sectional imaging, including cardiac magnetic resonance (CMR) and computed tomography.

CASE SUMMARY: An 11-month-old female patient presented with heterotaxy syndrome, dextrocardia, double-outlet right ventricle, and complex pulmonary valve stenosis. Fetal echocardiography and postnatal TTE established the complexity of the cardiac anatomy and led to a multidisciplinary discussion to plan a complex staged cardiac repair.

DISCUSSION: Three-dimensional (3D) printed and virtual heart models, along with CMR angiography, provided detailed anatomical visualization and spatial conceptualization, which aided in assessing the feasibility of a staged biventricular repair. Postoperative CMR guided subsequent surgical procedures, evaluated newly created structures such as baffles, and identified complications.

TAKE-HOME MESSAGE: This case highlights the critical role of multimodality imaging, including advanced imaging with virtual and 3D printed modeling, in surgical planning in a patient with complex CHD.

PMID:40579094 | DOI:10.1016/j.jaccas.2025.103884

JACC Case Rep. 2025 Jun 25;30(16):104014. doi: 10.1016/j.jaccas.2025.104014.

ABSTRACT

BACKGROUND: Congenital coronary artery abnormalities represent an uncommon class of congenital heart disease with an associated risk of sudden cardiac death. There is a paucity of data for the evaluation and management of affected patients with an intraseptal course.

CASE SUMMARY: A 27-year-old woman was found to have a single coronary trunk arising from the right sinus with a 2.7 cm intraseptal course of the left main coronary artery. She underwent stress cardiac magnetic resonance and catheterization, to assess for inducible ischemia before surgical repair with transconal unroofing. Her recovery was uneventful, and she remains free of symptoms.

DISCUSSION: Management of congenital coronary artery anomalies requires an exhaustive evaluation. Further studies are warranted to define the ideal work-up, indication, and timing of intervention and the efficacy of medical management in improving symptoms.

TAKE-HOME MESSAGES: Congenital coronary anomalies represent a rare but consequential cause of exertional chest pain. This diagnosis requires multimodal imaging to inform decision making.

PMID:40579080 | DOI:10.1016/j.jaccas.2025.104014

JACC Case Rep. 2025 Jun 25;30(16):104249. doi: 10.1016/j.jaccas.2025.104249.

ABSTRACT

BACKGROUND: A 21-year-old male with complex congenital heart disease (pulmonary atresia with intact ventricular septum and major aortopulmonary collateral arteries [MAPCAs]) was seen in our outpatient clinic. Diagnosed shortly after birth, the patient underwent Rashkind atrial septostomy and was deemed amenable to surgical correction/partial unifocalization or palliative intervention.

CASE SUMMARY: On examination, the patient was cyanotic with no signs of heart failure. Multimodality imaging showed MAPCAs from the aorta to the pulmonary circulation with a variable degree of stenosis suggestive of segmental pulmonary hypertension (PH). The decision of the multidisciplinary meeting was for a conservative approach and close monitoring with the potential of palliative interventions if there is a deterioration in patients' functional status.

DISCUSSION: Segmental PH presents a complex scenario, necessitating multimodality imaging for therapeutic considerations.

TAKE-HOME MESSAGE: In cyanotic congenital heart disease, the presence of MAPCAs and segmental PH presents a complex scenario, necessitating multimodality imaging.

PMID:40579077 | DOI:10.1016/j.jaccas.2025.104249

Thorac Cardiovasc Surg. 2025 Jan;73(S 03):e39-e45. doi: 10.1055/a-2635-3320. Epub 2025 Jun 27.

ABSTRACT

Central airway stenosis following congenital heart malformation surgery is a rare but significant cause of postoperative weaning failure. In selected cases, bronchoscopic interventions are effective treatment options for managing these kind of airway obstructions and achieving successful weaning.The data of six pediatric patients who were unable to be weaned from mechanical ventilation due to central airway obstruction following congenital heart malformation surgery were retrospectively analyzed. Rigid and flexible bronchoscopies were performed under general anesthesia for six patients.Six patients (4 males and 2 females; age range: 4 months to 6 years) with an airway obstruction after surgery due to congenital heart malformations included the study. Three patients had an obstruction of the left main bronchus, two of the right main bronchus, and one of bilateral main bronchus. Balloon dilatation was applied to one patient, mechanical dilatation was applied to three patients, and airway stent was applied to two patients. Two of six patients died from nonprocedural causes (acute respiratory distress syndrome due to pneumonia and cardiac arrest due to severe heart failure) and four patients were weaned successfully from mechanical ventilation and they were still alive during the follow-up period. No procedural-related mortality was seen in the study population. In one patient, stent placement could not be performed due to desaturation and hemodynamic instability during the procedure, and in another patient, granulation tissue developed due to a covered metallic stent, and the metallic stent was removed and replaced with a biodegradable stent.In selected cases, bronchoscopic interventions offer efficient approach to managing airway obstructions due to congenital heart malformation surgery.

PMID:40578812 | DOI:10.1055/a-2635-3320