単心室症

The Kawashima procedure is a variant of the bidirectional Glenn used in patients with interrupted inferior vena cava (IVC) and azygos continuation, though optimal timing and the role of fenestration remain debated. We report a 5-month-old boy with functional single ventricle physiology, transposition of the great arteries, pulmonary valve atresia with ventricular septal defect, interrupted IVC with azygos continuation, and bilateral superior vena cavae, who presented with cyanosis and failure to thrive. Imaging confirmed complex venous anomalies with hepatic venous drainage directly into the right atrium. The patient underwent a Kawashima procedure without fenestration, with stable postoperative hemodynamics, improved oxygen saturation, and an uncomplicated recovery. This case demonstrates that early Kawashima without fenestration is feasible in carefully selected infants, although exclusion of hepatic venous flow may predispose to pulmonary arteriovenous malformations, necessitating eventual Fontan completion. Favorable outcomes can be achieved in resource-limited settings with appropriate patient selection and multidisciplinary care.

BACKGROUND: Myocardial perfusion imagining (MPI) is a nuclear medicine technique used in the assessment of coronary artery disease. Differences in the perfusion of the myocardium at rest and stress and regions of perfusion defects can be indicators of various cardiac disease states. Respiratory motion (RM) can result in the degradation of image quality and the appearance of artificial regions of perfusion deficit. While RM correction is possible, many methods involve time-consuming calculation or additional equipment for motion tracking. This has limited the clinical uptake of such methods. This work presents a deep learning model for data-driven RM correction on dedicated cardiac SPECT scanners. PURPOSE: An AI-based method for estimating RM from gated projection data for MPI SPECT using a dedicated pinhole scanner was developed. Accuracy of these estimates and impact on image characteristics were assessed. METHODS: RM motion parameters were calculated for rest and stress MPI scans using a minimized root mean squared (RMS) alignment of reconstructed respiratory gates for 90 patients supplemented with 33 NCAT simulated patients. Using these parameters, an AI network was trained to estimate motion based on gated projection data prior to reconstruction. Accuracy of AI predictions relative to RMS data were assessed for clinical scans and down-sampled data to simulate noisy acquisitions. Scans were reconstructed with AI motion correction (MC), RMS MC, and no MC. Anterior myocardial wall thickness and its contrast relative to the interior of the ventricle were calculated in each case. RESULTS: AI motion predictions were accurate to within 1.86 ± 0.05 mm at clinical noise levels, increasing to 2.07 ± 0.07 mm at 1/8 signal. MC with AI was shown to provide a statistically significant increase anterior wall contrast and decrease measured wall thickness relative to no MC, with comparable results to RMS MC. CONCLUSION: Direct AI-based MC parameter estimation using gated projection data was shown to be feasible in clinical settings. Corrections based on AI estimates showed improved contrast and reduced myocardial wall thickness when incorporated into image reconstruction.

BACKGROUND: A prospective randomized controlled trial of exclusive human milk nutrition (EHM) in infants with single-ventricle physiology (SVP) was previously published. This study evaluates growth and health outcomes at 3-6 months. METHODS: Demographics, anthropometrics, and clinical outcomes were collected prospectively. Anthropometric z-scores were calculated using WHO standards. RESULTS: One hundred seven infants were randomized to EHM or control diet, with 23 infants meeting predefined exclusion criteria, 8 infants withdrawn/died, leaving 76 infants at discharge. At 3-6 months, 74 infants completed follow-up. Infants with hypoplastic left heart syndrome (HLHS) were similar (control 73%, EHM 86.5%; p = 0.24). Growth, z-scores, and hospitalization did not differ between groups up to 6 months. Median growth velocity was 25 (IQR: 20.24-30.99) and 22 (IQR: 18.76-23.94) g/day from birth to 3-6 months. Overall, 50-60% of infants achieved 100% PO intake by 3-6 months of age. CONCLUSIONS: Neonates with SVP receiving an EHM diet from birth to 30 days post-surgery had similar growth at 3-6 months of age compared to the control diet. Infants with SVP benefit from a protocolized nutritional approach, as evidenced by normal growth velocities in infancy. Centers may consider delaying gastrostomy tube placement until after the Glenn operation. TRIAL REGISTRATION: This trial is registered with ClinicalTrials.gov ( www. CLINICALTRIALS: gov , Trial ID: NCT02860702). IRB APPROVAL: This trial was approved by the Institutional Review Board of the University of Texas Health Sciences Center at San Antonio (ID: HSC20150779H). IMPACT: This multicenter RCT demonstrates the long-term benefits of exclusive human milk nutrition in infants with SVP, with improved growth and reduced necrotizing enterocolitis. A multidisciplinary approach and a well-thought-out nutritional algorithm can optimize growth in infants with complex congenital heart disease, similar to healthy term infants up to 6 months of age. The majority of infants with SVP requiring an operation within the first month of life are able to achieve 100% of their intake via PO by 6 months of age; therefore, delaying gastrostomy tube placement until after the Glenn operation may be considered.

AimThe present study was done to compare the hemodynamic effects of 3 drugs-milrinone, levosimendan, and dobutamine in patients undergoing bidirectional cavopulmonary connection (BCPC) surgery.Materials and MethodsIn this prospective observational study, a total of 75 patients between 3 months and 2 years of age who underwent BCPC were included. They were divided into milrinone (M), levosimendan (L), and dobutamine (D) groups. The primary aim was to compare the hemodynamic effects of 3 drugs in this subset of patients.ResultsThe mean age at surgery was 6.4 ± 2.6 months, with no significant differences in gender or weight among the groups. Perioperatively, heart rate and femoral venous pressure were significantly higher in the dobutamine group. Significant differences were noted in SpO2, Glenn pressures, Glenn pressure to mean arterial pressure (MAP) ratios, and near infrared spectroscopy values, with milrinone showing the most favorable outcomes, followed by levosimendan and dobutamine. Postoperative E/e' values were significantly lower in all groups (P < .001), with milrinone (4.7 ± 0.5) performing better than levosimendan (4.9 ± 0.9) and dobutamine (6.5 ± 1.2). Vasoactive inotropic scores decreased postoperatively in all groups but were significantly higher in the levosimendan group. No significant differences were observed in the duration of mechanical ventilation or intensive care unit stay. Notably, there were no mortalities during the study period.ConclusionsMilrinone offers superior hemodynamic benefits over levosimendan and dobutamine in patients undergoing BCPC using cardiopulmonary bypass. Its ability to significantly reduce Glenn pressure, lower the Glenn pressure to MAP ratio, and improve both systolic and diastolic functions makes it the preferred inotropic agent in this subset of patients.

BACKGROUND: Retained intravascular or intracardiac foreign bodies are rare but well-recognized in the surgical literature. However, delayed septic pulmonary embolism resulting from a retained transcardiac foreign body has not been previously reported. CASE DESCRIPTION: This case report describes a 57-year-old woman who presented with fever and cough for one month and worsening dyspnea over three days. Two years prior, she had been struck by a metallic object from a lawnmower but did not seek medical attention. Chest radiography demonstrated a linear metallic foreign body in the right lower lung field. Transthoracic echocardiography showed a rod-shaped structure confined to the right atrium, with vegetations surrounding the foreign body and extending into the right ventricle. CT angiography was subsequently performed, which identified a metallic foreign body traversing the diaphragm from the liver into the right heart, as well as emboli in the distal branches of the right pulmonary artery. Surgical exploration via median sternotomy confirmed a metallic rod measuring 10 cm in length and 0.5 cm in diameter penetrating the liver, diaphragm, and right atrium. The rod was successfully removed together with the pulmonary emboli. Pathological analysis demonstrated Aspergillus infection. The patient was discharged in stable condition and completed postoperative oral voriconazole therapy. CONCLUSIONS: Although penetrating cardiac foreign bodies may initially be nonfatal, the present case illustrates their potential for late-onset fungal colonization and embolic complications, highlighting the importance of timely surgical removal and vigilant follow-up.