Search Results (3,418)

Background: In high-risk and frail patients with multivessel coronary artery disease (MV CAD), guidelines indicated complete revascularization with or without the use of cardiopulmonary bypass (CPB) bears a high morbidity and mortality risk. In cases where catheter interventions were deemed unsuitable and conventional coronary artery bypass grafting (CABG) posed an unacceptable perioperative risk, patients were scheduled for minimally invasive direct coronary artery bypass (MIDCAB) grafting or minimally invasive multivessel coronary artery bypass grafting (MICS-CABG). We called this approach “palliative revascularization.” This study assesses the safety and impact of palliative revascularization on clinical outcomes and overall survival. Methods: A consecutive series of 57 patients undergoing MIDCAB or MICS-CABG as a palliative surgery between 2008 and 2018 was included. The decision for palliative surgery was met in heart team after carefully assessing each case. The patients underwent single or double-vessel revascularization using the left internal thoracic artery and rarely radial artery/saphenous vein segments, both endoscopically harvested. Inpatient data could be completed for all 57 patients. The mean follow-up interval was 4.2 ± 3.7 years, with a follow-up rate of 91.2%. Results: Mean patient age was 79.7 ± 7.4 years. Overall, 46 patients (80.7%) were male, 26 (45.6%) had a history of atrial fibrillation and 25 (43.9%) of chronic kidney disease. In total, 13 patients exhibited a moderate EuroSCORE II, while 27 were classified as high risk, with a EuroSCORE II exceeding 5%. Additionally, 40 patients (70.2%) presented with three-vessel disease, 17 (29.8%) suffered an acute myocardial infarction within three weeks prior to surgery and 50.9% presented an impaired ejection fraction. There were 48 MIDCAB and nine MICS CABG with no conversions either to sternotomy or to CPB. Eight cases were planned as hybrid procedures and only 15 patients (26.3%) were completely revascularized. During the first 30 days, four patients (7%) died. A myocardial infarction occurred in only one case, no patient necessitated immediate reoperation. The one-, three- and five-year survival rates were 83%, 67% and 61%, respectively. Conclusions: MIDCAB and MICS CABG can be successfully conducted as less invasive palliative surgery in high-risk multimorbid patients with MV CAD. The early and mid-term results were better than predicted. A higher rate of hybrid procedures could improve long-term outcome in selected cases. Full article

Background: Optimal timing for surgery following acute rotator cuff tears remains unclear. This study examines how the timing of arthroscopic rotator cuff repair (RCR) affects retear rates and functional outcomes. Methods: This PROSPERO-registered review (CRD42024528249) followed PRISMA guidelines and included randomized trials, and cohort, studies on adults with imaging-confirmed full-thickness rotator cuff tears. Studies lacking timing data or key outcomes were excluded. Risk of bias was assessed using ROBINS-I. Meta-analysis of retear rates was performed comparing surgical timing. Qualitative analysis was conducted classifying results as early-beneficial, delayed-detrimental, or neutral. Results: Our review included 13 studies and 871 patients with an average age of 57.9. Meta-analysis of eight studies comparing retear rates between early and delayed RCR demonstrated a significant benefit associated with early intervention risk ratio 0.60 (95% CI: 0.38–0.96). Functional outcomes also favored early intervention with four studies demonstrating significantly greater postoperative functional improvements in the early intervention group. Conclusions: Early arthroscopic RCR decreased the rate of retear and improved functional outcomes. No study found early intervention to be detrimental or delayed intervention to be superior. These findings support consideration of early repair when clinically appropriate. Future studies should determine more finite timing guidelines. Full article

Background and Objectives: Effective myocardial protection is essential for successful cardiac surgery outcomes, especially in complex and prolonged procedures. To this end, Del Nido (DN) and histidine-tryptophan-ketoglutarate (HTK) cardioplegia solutions are widely used; however, their comparative efficacy in adult surgeries with prolonged aortic cross-clamp (ACC) times remains unclear. This study aimed to compare the efficacy and safety of DN and HTK for myocardial protection during prolonged ACC times in adult cardiac surgery and to define clinically relevant thresholds. Materials and Methods: This retrospective study included a total of 320 adult patients who underwent cardiac surgery under cardiopulmonary bypass (CPB) with an aortic cross-clamp time ≥ 90 min. Data were collected from the medical records of elective adult cardiac surgery cases performed at a single center between 2019 and 2025. Patients were categorized into two groups based on the type of cardioplegia received: Del Nido (n = 160) and HTK (n = 160). The groups were compared using 1:1 propensity score matching. Clinical and biochemical outcomes—including troponin I (TnI), CK-MB, lactate levels, incidence of low cardiac output syndrome (LCOS), and need for mechanical circulatory support—were analyzed between the two cardioplegia groups. Subgroup analyses were performed according to ACC duration (90–120, 120–150, 150–180 and >180 min). The predictive threshold of ACC duration for each complication was determined by ROC analysis, followed by the analysis of independent predictors of each endpoint by multivariate logistic regression. Results: Intraoperative cardioplegia volume and transfusion requirements were lower in the DN group (p < 0.05). HTK was associated with lower TnI levels and less intra-aortic balloon pump (IABP) requirement at ACC times exceeding 180 min. Markers of myocardial injury were lower in patients with an ACC duration of 120–150 min in favor of HTK. The propensity for ventricular fibrillation after ACC was significantly lower in the DN group. Significantly lower postoperative sodium levels were observed in the HTK group. Prolonged ACC duration was an independent risk factor for LCOS (odds ratio [OR]: 1.023, p < 0.001), VIS > 15 (OR, 1.015; p < 0.001), IABP requirement (OR: 1.020, p = 0.002), and early mortality (OR: 1.016, p = 0.048). Postoperative ejection fraction (EF), troponin I, and CK-MB levels were associated with the development of LCOS and a VIS > 15. Furthermore, according to ROC analysis, HTK cardioplegia was able to tolerate ACC for up to a longer duration in terms of certain complications, suggesting a higher physiological tolerance to ischemia. Conclusions: ACC duration is a strong predictor of major adverse outcomes in adult cardiac surgeries. Although DN cardioplegia is effective and economically advantageous for shorter procedures, HTK may provide superior myocardial protection in operations with long ACC duration. This study supports the need to individualize cardioplegia choice according to ACC duration. Further prospective studies are needed to establish standard dosing protocols and to optimize cardioplegia selection according to surgical duration and complexity. Full article

Background: Hip fractures represent a major clinical challenge, particularly in elderly and frail patients, where postoperative pain control must balance effective analgesia with motor preservation to facilitate early mobilization. Various regional anesthesia techniques are used in this setting, including the pericapsular nerve group (PENG) block, fascia iliaca compartment block (FICB), femoral nerve block (FNB), and quadratus lumborum block (QLB), yet optimal strategies remain debated. Objectives: To systematically review the efficacy, safety, and clinical applicability of major regional anesthesia techniques for pain management in hip fractures, including considerations of fracture type, surgical approach, and functional outcomes. Methods: A systematic literature search was conducted following PRISMA 2020 guidelines in PubMed, Scopus, Web of Science, and the virtual library of the Hospital Central de la Defensa “Gómez Ulla” up to March 2025. Inclusion criteria were RCTs, systematic reviews, and meta-analyses evaluating regional anesthesia for hip surgery in adults. Risk of bias in RCTs was assessed using RoB 2.0, and certainty of evidence was evaluated using the GRADE approach. Results: Twenty-nine studies were included, comprising RCTs, systematic reviews, and meta-analyses. PENG block demonstrated superior motor preservation and reduced opioid consumption compared to FICB and FNB, particularly in intracapsular fractures and anterior surgical approaches. FICB and combination strategies (PENG+LFCN or sciatic block) may provide broader analgesic coverage in extracapsular fractures or posterior approaches. The overall risk of bias across RCTs was predominantly low, and certainty of evidence ranged from moderate to high for key outcomes. No significant safety concerns were identified across techniques, although reporting of adverse events was inconsistent. Conclusions: PENG block appears to offer a favorable balance of analgesia and motor preservation in hip fracture surgery, particularly for intracapsular fractures. For extracapsular fractures or posterior approaches, combination strategies may enhance analgesic coverage. Selection of block technique should be tailored to fracture type, surgical approach, and patient-specific functional goals. Full article

Neuromuscular hip dysplasia (NHD) is a common deformity in children with cerebral palsy (CP). Although some predictive factors of NHD are known, the prediction of NHD is in its infancy. We present a Clinical Decision Support System (CDSS) designed to calculate the probability of developing NHD in children with CP. The system utilizes an ensemble of three machine learning (ML) algorithms: Neural Network (NN), Support Vector Machine (SVM), and Logistic Regression (LR). The development and evaluation of the CDSS followed the DECIDE-AI guidelines for AI-driven clinical decision support tools. The ensemble was trained on a data series from 182 subjects. Inclusion criteria were age between 12 and 18 years and diagnosis of CP from two specialized units. Clinical and functional data were collected prospectively between 2005 and 2023, and then analyzed in a cross-sectional study. Accuracy and area under the receiver operating characteristic (AUROC) were calculated for each method. Best logistic regression scores highlighted history of previous orthopedic surgery (p = 0.001), poor motor function (p = 0.004), truncal tone disorder (p = 0.008), scoliosis (p = 0.031), number of affected limbs (p = 0.05), and epilepsy (p = 0.05) as predictors of NHD. Both accuracy and AUROC were highest for NN, 83.7% and 0.92, respectively. The novelty of this study lies in the development of an efficient Clinical Decision Support System (CDSS) prototype, specifically designed to predict future outcomes of neuromuscular hip dysplasia (NHD) in patients with cerebral palsy (CP) using clinical data. The proposed system, PredictMed-CDSS, demonstrated strong predictive performance for estimating the probability of NHD development in children with CP, with the highest accuracy achieved using neural networks (NN). PredictMed-CDSS has the potential to assist clinicians in anticipating the need for early interventions and preventive strategies in the management of NHD among CP patients. Full article

Anomalies of coronary artery origins are rare but significant conditions that can range from benign to life-threatening. Early detection through imaging is crucial in preventing adverse outcomes. The treatment strategy varies depending on the type and severity of the anomaly, ranging from pharmacological treatment to surgery. A 22-year-old male patient, after syncope, after excluding other causes, had an exercise drill test, which was clinically negative and ECG-positive. Angio-CT revealed an undeveloped left main coronary artery (LMCA), and the circulation was supplied through the right coronary artery (RCA). The RCA provides the left anterior descending artery (LAD), and the LAD retrogradely supplies the left circumflex artery (LCX). The myocardial perfusion scintigraphy showed a slight lack of perfusion in the anterior wall (6% of total perfusion). The patient was qualified for further observation. A 77-year-old female underwent cardiac CT due to stenocardia. CT showed a lack of LMCA. The initial segment of the RCA gave rise to the left coronary artery (LCA), which encircled the aortic bulb posteriorly and bifurcated into branches resembling the LCX and LAD. After the Heart Team consultation, the patient was deemed eligible for conservative treatment. Angio-CT is a valuable tool for detecting coronary artery anomalies. Full article

The management of resectable pancreatic ductal adenocarcinoma (R-PDAC) and borderline resectable pancreatic ductal adenocarcinoma (BR-PDAC) remains a topic of active debate. Although neoadjuvant therapy (NAT) has shown clinical benefits in BR-PDAC, especially in increasing resectability and achieving higher rates of margin-negative (R0) resections, its role in R-PDAC is less clearly defined. Additionally, the role of immunotherapy in PDAC is still being explored, with ongoing trials investigating new combinations to overcome the tumor’s immune-resistant microenvironment. This article provides a comprehensive narrative review of the current evidence comparing NAT with upfront surgery in pancreatic cancer management, focusing on randomized controlled trials and meta-analyses that assess outcomes in R-PDAC and BR-PDAC. The review aims to determine whether NAT offers a significant survival advantage over traditional post-operative strategies and to clarify which clinical scenarios may benefit most from NAT. The literature was identified through a systematic search of PubMed, Scopus, and Google Scholar databases up to March 2025. Article selection adhered to the PRISMA guidelines. Our review of existing evidence supports NAT as the standard of care for BR-PDAC. Meanwhile, management of R-PDAC should be tailored individually, guided by risk stratification that considers both clinical parameters and molecular features. Immunotherapy and targeted therapies are still in early research phases, and their further integration as NAT remains controversial. Full article

Background: Fear of falling (FOF) is a significant concern among older adults, especially after total knee arthroplasty (TKA). FOF can limit daily activities, reduce quality of life, and hinder recovery. This study aimed to investigate the prevalence, severity, and impacts of FOF in patients undergoing TKA and identify factors contributing to increased FOF. Methods: A prospective observational study was conducted at King Abdulaziz Medical City in Riyadh, Saudi Arabia, from April 2024 to December 2024. This study included 52 participants aged 20 to 75 years who had undergone primary TKA. Data were collected at two time points: after TKA and at three months post-surgery. The Short Falls Efficacy Scale-International (SFES-I) was used to assess the severity of FOF, and the Short Form 36 (SF-36) was used to measure the quality of life. Descriptive statistics, t-tests, and logistic regression were used for analysis. Results: This study included 52 participants (mean age: 63.77 ± 6.65 years; 82.7% female). Post-TKA, all participants exhibited high FOF (mean SFES-I score: 56.75 ± 8.30). After three months, the mean SFES-I score decreased significantly to 49.04 ± 12.45 (t = 4.408, p < 0.05). Post-TKA, SF-36 showed significant improvements in the physical function, role of physical limitations, bodily pain, vitality, social function, role of emotional limitations, and mental health subdomains. Bilateral total knee arthroplasty, body mass index, and some SF-36 subcomponents—such as general health, vitality, and role of emotional limitations—were identified as factors leading to increased FOF. Conclusions: FOF remains prevalent and severe in TKA patients, even at three months post-surgery, affecting rehabilitation outcomes. Early identification and tailored interventions for FOF should be considered essential components of comprehensive TKA recovery programs. Full article

This study evaluates the relationship between preoperative cartilage quality, measured by T2 mapping, and patient-reported outcomes following labral tear treatment. We retrospectively reviewed patients aged 14–50 who underwent primary hip arthroscopy with either labral repair or reconstruction. Preoperative T2 values of femoral, acetabular, and labral tissue were assessed from MRI by blinded reviewers. International Hip Outcome Tool (iHOT-12) scores were collected preoperatively and up to two years postoperatively. Associations between T2 values and iHOT-12 scores were analyzed using univariate mixed linear models. Twenty-nine patients were included (mean age of 32.5 years, BMI 24 kg/m², 48.3% female, and 22 repairs). Across all patients, higher T2 values were associated with higher iHOT-12 scores at baseline and early postoperative timepoints (three months for cartilage and six months for labrum; p < 0.05). Lower T2 values were associated with higher 12- and 24-month iHOT-12 scores across all structures (p < 0.001). Similar trends were observed within the repair and reconstruction subgroups, with delayed negative associations correlating with worse tissue quality. T2 mapping showed time-dependent correlations with iHOT-12 scores, indicating that worse cartilage or labral quality predicts poorer long-term outcomes. These findings support the utility of T2 mapping as a preoperative tool for prognosis in hip preservation surgery. Full article

Background/Objectives: Breast cancer was the leading cause of malignant tumors among women in 2022. About two-thirds of breast cancer cases are hormone-receptor-positive. In these patients, aromatase inhibitors are a mainstay of treatment, but associated musculoskeletal symptoms can negatively affect patient compliance. Aromatase-inhibitor-induced carpal tunnel syndrome represents one of the main causes of aromatase inhibitor discontinuation, with a non-compliance rate of up to 67%, potentially leading to increased cancer mortality. This study investigates estrogen receptor expression in aromatase-inhibitor-induced carpal tunnel syndrome tissues, in order to better define its etiopathogenesis and derive preventive or therapeutic measures that can improve aromatase inhibitor patient compliance. To our knowledge, there is no study on this subject in the literature. Methods: Between 2023 and 2024, we recruited 14 patients at the Jewish Hospital of Rome, including seven patients with aromatase-inhibitor-induced carpal tunnel syndrome (study group) and seven with postmenopausal idiopathic carpal tunnel syndrome (control group). Each patient was evaluated based on a clinical visit, a questionnaire, instrumental exams, and serum hormone dosages and were treated with open carpal tunnel release surgery, during which transverse carpal ligament and flexor tenosynovium samples were collected. For immunohistochemical experiments, sections were treated with anti-estrogen receptor α and anti-estrogen receptor β antibodies. Results: The immunohistochemical features in the study and control groups were similar, demonstrating that tissues affected by aromatase-inhibitor-induced carpal tunnel syndrome are targets of direct estrogen action and that estrogen deprivation is correlated with disease etiogenesis. Surgery was effective in patient treatment. Conclusions: Aromatase-inhibitor-induced carpal tunnel syndrome represents a newly defined form of the disease. This syndrome represents one of the main causes of aromatase inhibitor discontinuation, due to its negative impact on the patient’s quality of life. The identification by clinicians of aromatase inhibitor use as a possible risk factor for carpal tunnel syndrome development is of essential importance, as early diagnosis and prompt management can improve patient compliance and overall breast cancer treatment outcomes. Full article

Background/Objectives: Iatrogenic abductor muscle injury following intramedullary nailing for proximal hip fractures can negatively impact postoperative rehabilitation and clinical outcomes. To quantify iatrogenic abductor muscle injury after intramedullary nailing and detect the degree of degenerative change in muscle around the entry point of trochanteric fractures. Methods: This cross-sectional study used data from a single center database from May to December 2023. This study utilized ultrasound examinations performed by a single expert orthopedic surgeon. This study included 61 patients who underwent intramedullary nailing surgery for adult hip fractures. All surgeries were performed by a single experienced hip surgeon. Patients who declined sonographic evaluation or did not undergo ultrasound during their admission were excluded. For more accurate comparison, sonography was also conducted on the healthy, non-operative limb. Descriptive statistics were used to summarize patient and ultrasound findings. A subgroup analysis using Fisher’s exact test was performed to assess the association between implant type and the incidence of iatrogenic gluteus medius tendon injury. Results: Of the 61 patients, tendon tears were identified in 35 cases (57%) on the affected side, with 20 cases (33%) involving gluteus medius tendon tears without fractures on the ipsilateral facet. Gluteus minimus tendon tears were observed in 13 cases (21%), while gluteus medius tendon tears were noted in 31 cases (51%). In the unaffected limbs, tendon degeneration was detected in the form of tendinosis and calcification. Overall, 39 patients (64%) exhibited abductor tendon tendinosis, and 30 patients (49%) were diagnosed with calcification. Conclusions: Gluteus medius and Gluteus minimus are important abductors for hip disease rehabilitation. Iatrogenic gluteus medius tendon injury during the intramedullary nailing showed 33%. Abductor degeneration also showed 92% of the unaffected limbs. This study suggests that abductor degeneration can be a risk factor of falling among the elderly population and an iatrogenic abductor injury can be an obstacle for the early recovery of ambulation in the hip fracture patients. Prevention of abductor degeneration and iatrogenic abductor injury might be important for the hip fracture prevention and rehabilitation. Full article

Tuberculosis (TB) is an ancient and re-emerging granulomatous infectious disease that continues to challenge public health. Early diagnosis and prompt effective treatment are crucial for preventing disease progression and reducing both morbidity and mortality. These steps play a vital role in infection control and in lowering death rates at both individual and population levels. Although diagnostic methods have improved sufficiently in recent decades, TB can still present with ambiguous laboratory and imaging features. This ambiguity can lead to diagnostic pitfalls and potentially disastrous outcomes due to delayed diagnosis. In this article, we present a case of TB that was difficult to diagnose. The disease had invaded the mediastinum, right atrium, right coronary artery, and inferior vena cava (IVC), resulting in Budd–Chiari syndrome. This rare presentation created clinical, laboratory, and radiological confusion, resulting in a diagnostic dilemma that ultimately led to open cardiac surgery. The patient initially presented with progressive shortness of breath on exertion and fatigue, which suggested possible heart disease. This suspicion was reinforced by computed tomography (CT) imaging, which showed infiltrative mass lesions predominantly in the right side of the heart, invading the right coronary artery and IVC, with imaging features mimicking angiosarcoma. Although laboratory findings revealed an exudative effusion with lymphocyte predominance and elevated adenosine deaminase (ADA), the Gram stain was negative for bacteria, and an acid-fast bacilli (AFB) smear was also negative. These findings contributed to diagnostic uncertainty and delayed the confirmation of TB. Open surgery with excisional biopsy and histopathological analysis ultimately confirmed TB. We conclude that TB should not be ruled out solely based on negative Mycobacterium bacteria in pericardial effusion or AFB smear. TB can mimic aggressive tumors such as angiosarcoma or lymphoma with invasion of the surrounding tissues and blood vessels. Awareness of the clinical presentation, imaging findings, and potential diagnostic pitfalls of TB is essential, especially in endemic regions. Full article

Background/Objectives: Gallbladder cancer (GBC) is a lethal malignancy curable only by surgical resection in early stages (Tis, T1, T2). Significant controversy exists regarding the optimal extent of surgery. This review summarizes recent trends and evidence on surgical strategies for Tis, T1, and T2 GBC to guide practice and research. Methods: This narrative review synthesizes recent literature on surgical management of Tis, T1a, T1b, and T2 GBC based on American Joint Committee on Cancer (AJCC) 8th edition staging. It examines simple vs. extended cholecystectomy (simple cholecystectomy (SC) vs. extended/radical cholecystectomy (EC/RC)), the role of lymphadenectomy (LND) and hepatectomy, and minimally invasive surgery (MIS). Results: Simple cholecystectomy is curative for Tis/T1a GBC. For T1b, regional LND is essential for staging/potential benefit, especially examining ≥5–6 nodes. Tumor size is critical; SC alone may suffice for T1b < 1 cm (low lymph node metastasis (LNM) risk), while EC/RC with LND is indicated for ≥1 cm (higher LNM risk). Routine hepatectomy for T1b lacks survival support. For T2 GBC, mandatory regional LND (≥6 nodes) is required for both T2a and T2b substages due to high LNM rates; T2b has higher LNM than T2a. Routine hepatectomy for T2 is debated; evidence suggests no routine benefit for T2a beyond LND, with conflicting findings for T2b. R0 resection is paramount. MIS is feasible for early stages in experienced hands. Conclusions: Management of early GBC is moving towards risk stratification. SC is standard for Tis/T1a. Adequate regional LND is crucial for T1b (especially ≥1 cm) and mandatory for T2 GBC. Routine hepatectomy, particularly for T2b, remains controversial. Tailored surgery prioritizes R0 resection and comprehensive LND, necessitating further standardized research. Full article

Background/Objectives: Pituitary tumors account for over 90% of all sellar region masses. However, a spectrum of rare neoplastic, inflammatory, infectious, and vascular lesions—benign and malignant—can arise in the intra- and parasellar compartments and clinically and radiologically mimic PitNETs. We report a cohort of 47 such rare and cystic midline intracranial lesions, emphasizing their distinctive morphological, clinical, and imaging features and the personalized treatment strategies applied. Methods: In this retrospective single-center study, we reviewed all patients treated for suspected PitNETs via transsphenoidal approach between 2015 and 2024. Of 529 surgical cases, we excluded confirmed PitNETs, meningiomas, and classical intradural craniopharyngiomas. Collected data encompassed patient demographics, tumor characteristics, presenting symptoms, extent of resection or medical therapy, endocrine outcomes, and follow-up information. Results: Among all 529 patients who underwent surgical treatment for sellar lesions from 2015 to 2024, 47 cases (8.9%) were identified as rare or cystic masses. Forty-six underwent transsphenoidal resection; one patient with hypophysitis received corticosteroid therapy alone. Presenting symptoms included headache (n = 16), dizziness (n = 5), oculomotor disturbances (n = 2), and visual impairment (n = 17). Endocrine dysfunction was found in 30 patients, 27 of whom required hydrocortisone replacement. Histopathological diagnoses were led by colloid cysts (n = 14) and Rathke’s cleft cysts (n = 11). The remaining 22 cases comprised plasmacytoma, germinoma, lymphoma, pituicytoma, inverted papilloma, metastatic carcinoma, chordoma, nasopharyngeal carcinoma, chloroma, and other rare entities. Preoperative imaging diagnosis proved incorrect in 38% (18/47) of cases, with several lesions initially misidentified as PitNETs. Conclusions: Nearly 9% of presumed PitNETs were rare, often benign or inflammatory lesions requiring distinct management. Most could be safely resected and demonstrated excellent long-term outcomes. Yet, despite advanced imaging techniques, accurate preoperative differentiation remains challenging, with over one-third misdiagnosed. Clinical red flags—such as early hormone deficits, rapid progression or atypical imaging findings—should prompt early interdisciplinary evaluation and, when indicated, image-guided biopsy to avoid unnecessary surgery and ensure tailored therapy. Full article

The image represents the post-mortem heart of a 28-year-old female patient, diagnosed in childhood with complete common atrioventricular canal defect. At time of diagnosis, the family refused surgery, as did the patient during her adulthood. Despite being advised against pregnancy, she became pregnant. On presentation to hospital, she was cyanotic, with clubbed fingers, and hemodynamically unstable, in sinus rhythm, with Eisenmenger syndrome and respiratory failure partially responsive to oxygen. During pregnancy, owing to systemic vasodilatation, the right-to-left shunt is increased, with more severe cyanosis and low cardiac output. Echocardiography revealed the complete common atrioventricular canal defect, with a single atrioventricular valve with severe regurgitation, right ventricular hypertrophy, pulmonary artery dilatation, severe pulmonary hypertension and a hypoplastic left ventricle. The gestational age at delivery was 38 weeks. She gave birth to a healthy boy, with an Apgar score of 10. The vaginal delivery was chosen by an interdisciplinary team. The cesarean delivery and the anesthesia were considered too risky compared to vaginal delivery. Three days later, the patient died. The autopsy revealed hepatomegaly, a greatly hypertrophied right ventricle with a purplish clot ascending the dilated pulmonary arteries and a hypoplastic left ventricle with a narrowed chamber. A single valve was observed between the atria and ventricles, making all four heart chambers communicate, also insufficiently developed interventricular septum and its congenital absence in the cranial third. These morphological changes indicate the complete common atrioventricular canal defect, with right ventricular dominance, which is a rare and impressive malformation that requires mandatory treatment in early childhood in order for the condition to be solved. Full article