The patient, a 20-day-old male, was admitted due to respiratory distress that had persisted for 20 days after birth. The main clinical manifestations included gradually worsening respiratory distress and edema. The patient received treatment including mechanical ventilation and diuretics. Echocardiography indicated cardiomegaly, pulmonary hypertension, and heart failure. A comprehensive systemic examination revealed a significant blowing vascular murmur upon auscultation over the anterior fontanelle and bilateral temporal regions. Further imaging studies including cranial magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance venography showed marked dilation of the superior sagittal sinus, transverse sinus, and sigmoid sinus, leading to a definitive diagnosis of dural arteriovenous fistula. After a multidisciplinary consultation, the patient underwent cerebral angiography and partial embolization of the left parietal arteriovenous fistula. Postoperatively, the patient was treated with positive inotropes, diuretics, and fluid restriction. Ultimately, the patient was weaned off the ventilator and discharged in improved condition. This article reports a case of neonatal dural arteriovenous fistula presenting with respiratory distress and discusses the multidisciplinary approach to managing this condition, which aids in early disease recognition and guides clinical decision-making.
Humans ; Male ; Infant, Newborn ; Central Nervous System Vascular Malformations/diagnosis* ; Respiratory Distress Syndrome, Newborn/etiology* ; Embolization, Therapeutic

Vascular malformations are aberrant proliferations of blood vessels that may present at birth or develop subsequently. Capillary malformations (CMs) represent the most common category of vascular malformations. They generally present as localized discoloration, including port-wine stains (PWSs) and telangiectasia. Certain PWSs, especially those affecting the V2 dermatome, may give rise to pyogenic granulomas or other epithelial or mesenchymal hamartomas.

We present a case of a 46-year-old male with a 5-year history of a solitary, progressively enlarging firm papule on an erythematous patch or “birthmark” on his left cheek. Despite infrequent manipulation, there were no reports of bleeding or any associated symptoms, including pain, pruritus, and thrills. The patient sought consultation for cosmetic reasons and wanted to have the lesion removed. Hence, an excision biopsy revealed a well-defined proliferation of mature thick and thin-walled vessels, lined by a single layer of endothelial cells, surrounded by dysmorphic and irregularly arranged vessels consistent with arteriovenous malformation with capillary malformation.

Capillary malformation (CM) is the most common form of vascular malformation. Fifty percent of port-wine stains (PWSs), which is the most common (CM) in children, are in the face area innervated by the second branch of the trigeminal nerve. Arteriovenous malformations manifest in the advanced stages of PWSs and, alongside pyogenic granuloma, represent underreported histological changes inside mature PWSs, especially in the face area innervated by the second branch of the trigeminal nerve (V2).

Congenital heart disease is the most common birth defect, affecting 1%–1.2% of live born infants. Pulmonary atresia with intact ventricular septum (PA-IVS) accounts for <1% of all total heart defects. The cause of PA-IVS has been unclear. Thus, experience for prenatal diagnosis of PA-IVS is limited in any single institution. This is the case of a 28-year-old gravida 1 para 0 who came in at 34 + 5 weeks of gestational age. Fetal two-dimensional (2D) echocardiography revealed Type II PA-IVS, higher risk for univentricular circulation postnatally. She gave birth at term by vaginal delivery, with confirmed findings through a 2D echocardiography. Prenatal diagnosis of PA-IVS allows options for the termination of pregnancy, fetal cardiac interventional therapy, early postnatal initiation of prostaglandin E1, and planned early neonatal interventional surgeries for palliation and repair. Early assessment of fetal cardiac features is useful for a better outcome.

Humans ; Pulmonary Atresia/therapy* ; Pregnancy ; Female ; China ; Consensus ; Fetal Heart ; Ultrasonography, Prenatal ; Heart Defects, Congenital/therapy* ; Ventricular Septum ; Cardiac Surgical Procedures/methods*

OBJECTIVES: Digital subtraction angiography (DSA) is the current gold standard for diagnosing spinal dural arteriovenous fistulas (SDAVF). However, DSA is invasive and associated with risks such as ionizing radiation and iodine contrast allergy. Contrast-enhanced magnetic resonance angiography (CE-MRA) with high temporal/high spatial resolution allows dynamic multiphase contrast-enhanced imaging with excellent detail. This study aims to evaluate the diagnostic value of spinal CE-MRA with high temporal/high spatial resolution for SDAVF. METHODS: Clinical data were retrospectively collected from patients who underwent both conventional spinal MRI and high temporal/high spatial resolution CE-MRA at Xiangya Hospital between January 1, 2021, and January 1, 2024, and who subsequently underwent DSA or surgery within 90 days. Two experienced radiologists independently reviewed all conventional MRI and CE-MRA images. The sensitivity and specificity of conventional MRI and CE-MRA for diagnosing SDAVF were calculated against the gold standard DSA findings. Kappa statistics were used to evaluate the consistency of MRI and CE-MRA compared to DSA. The diagnostic value was further assessed by calculating the area under curve (AUC) of the receiver operating characteristic (ROC). RESULTS: A total of 60 patients were included, of whom 47 were diagnosed with SDAVF and 13 were not. Conventional MRI had 3 false negatives and 1 false positive; CE-MRA had 2 false positives and 0 false negative. The sensitivity and specificity of conventional MRI were 93.62% and 92.31%, respectively. CE-MRA demonstrated 100% sensitivity and 84.62% specificity. The main cause of false positives was the misidentification of posterior spinal arteries as feeding arteries. CE-MRA clearly displayed most feeding arteries, and the accuracy of fistula localization was 74.47% (35/47). Kappa values for conventional MRI and CE-MRA were 0.814 and 0.896, respectively (both P<0.001), indicating good agreement, with CE-MRA outperforming conventional MRI. The AUCs for diagnosing SDAVF were 0.930 for conventional MRI and 0.923 for CE-MRA (both P<0.05). CONCLUSIONS Spinal CE-MRA with high temporal/high spatial resolution is a reliable, non-invasive imaging technique with high sensitivity for diagnosing SDAVF. It can clearly visualize feeding arteries and provides valuable preoperative diagnostic and localization information to support DSA or surgical planning.
Humans ; Central Nervous System Vascular Malformations/diagnosis* ; Male ; Female ; Retrospective Studies ; Middle Aged ; Magnetic Resonance Angiography/methods* ; Angiography, Digital Subtraction/methods* ; Aged ; Adult ; Sensitivity and Specificity ; Contrast Media ; Magnetic Resonance Imaging/methods*

Humans ; May-Thurner Syndrome ; Coronary Restenosis ; Constriction, Pathologic/surgery* ; Stents/adverse effects* ; Iliac Vein ; Treatment Outcome ; Retrospective Studies

Objective: To explore the efficacy and safety of transcatheter pulmonary valve perforation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The clinical data on surgical treatment and follow-up in 16 patients with PA-IVS who underwent transcatheter pulmonary valve perforation in Women and Children's Hospital, Qingdao University from October 2018 to October 2021 were analyzed retrospectively. The right ventricular systolic pressure and percutaneous oxygen saturation (SpO₂) were compared before and after operation. In addition, the SpO₂ and echocardiographic data at preoperative and the last follow-up were compared. Comparisons between groups were performed using paired-samples t test. Results: Among the 16 patients (10 males and 6 females) with the age at operation of 19 (14, 26) days, 12 cases underwent transcatheter pulmonary valve perforation successfully, 2 cases were transferred to surgery department for open-heart pulmonary valvulotomy, and the remaining 2 cases were transmitted to surgery department for transthoracic pulmonary valve perforation. The age at operation of the 12 patients who underwent transcatheter pulmonary valve perforation was 18 (14, 27) days, and the weight was (3.6±0.4) kg. The immediate postoperative right ventricular systolic pressure decreased significantly ((57±16) vs. (95±19) mmHg (1 mmHg=0.133 kPa), t=7.49, P<0.001), and the postoperative SpO₂ was improved effectively (0.90±0.48 vs.0.75±0.09, t=-5.61, P<0.001). The follow-up time was 22 (7, 33) months for 12 patients who underwent transcatheter pulmonary valve perforation successfully. At the last follow-up, the ratio of right to left ventricular transverse diameter was significantly higher than that before operative (0.55±0.05 vs. 0.45±0.05, t=-3.27,P=0.007). Furthermore, the Z-scores of pulmonary valvular diameter (-0.78±0.23 vs. -1.73±0.56, t=-8.52, P<0.001) and the tricuspid valvular diameter (-0.52±0.12 vs. -1.46±0.38, t=-10.40, P<0.001) were all significantly higher than preoperative data. At last, all the patients achieved biventricular circulation without death or major complications. Conclusion: Transcatheter pulmonary valve perforation is a safe and effective therapy for neonatal PA-IVS, and its curative effect has been confirmed by the medium follow-up data.
Child ; Male ; Infant, Newborn ; Humans ; Female ; Pulmonary Valve/surgery* ; Retrospective Studies ; Pulmonary Atresia/surgery* ; Heart Defects, Congenital

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.
Child ; Infant, Newborn ; Humans ; Infant ; Pulmonary Atresia/surgery* ; Ductus Arteriosus ; Retrospective Studies ; China ; Heart Defects, Congenital ; Ductus Arteriosus, Patent/surgery* ; Heart Septal Defects, Ventricular ; Stents

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.
Child ; Female ; Male ; Humans ; Pulmonary Atresia/surgery* ; Follow-Up Studies ; Retrospective Studies ; Heart Defects, Congenital/surgery*

Central venous lesion is a difficult problem in the vascular access complications of hemodialysis, which can cause serious clinical symptoms and affect the quality of hemodialysis and life of patients. We established arteriovenous fistula of the contralateral graft blood vessel with the used vein on the diseased side of the central vein of the patient. The arteriovenous fistula of the graft blood vessel was successfully punctured and hemodialysis was performed 2 weeks later. In this way, we not only solved the problem of venous hypertension and subsequent vascular access in the patient, but also reserved more vascular resources.
Humans ; Arteriovenous Shunt, Surgical/adverse effects* ; Blood Vessel Prosthesis Implantation ; Treatment Outcome ; Renal Dialysis ; Arteriovenous Fistula