Cardiac myxomas are primary cardiac tumors that presents with symptoms of hemodynamic derangement from obstruction of flow within the cardiac chambers or deformation of a cardiac valve and symptoms associated with embolization.

In this case report, we are presented with a 45-year-old female, diabetic with paroxysmal atrial fibrillation, fair functional capacity, ambulatory, with chief complaint of dyspnea. Admitted as a case of Left atrial myxoma with Pulmonary embolism (proximal right lower lobe, distal left main pulmonary artery division, and superior segmental division of the left lower lobe).

Left atrial myxoma was the first diagnostic consideration, followed by other primary cardiac tumors, and thrombus. Patient was then prepared for surgery and underwent excision of LA myxoma with CardioCel Bioscaffold patch on the inter-atrial septum, mitral ring annuloplasty with plication of the A2-A3 segment, thrombectomy of right inferior pulmonary vein, utilizing cardiopulmonary bypass. Upon pathological examination, the mass was found to be sarcoma with noted myxoid features. The objective of this report is to describe a case of this rare disease entity, and to discuss its presentation, pathological findings and management.

Human ; Female ; Middle Aged: 45-64 Yrs Old ; Pulmonary Embolism ; Pulmonary Artery ; Pulmonary Veins ; Atrial Fibrillation ; Atrial Septum ; Cardiopulmonary Bypass ; Hemodynamics ; Thrombectomy

OBJECTIVE: To explore the diagnosis, treatment and genetic characteristics of a neonate with severe pulmonary hypertension and respiratory failure. METHODS: Perinatal history, clinical manifestations, laboratory finding and diagnosis and treatment data of the child were collected. Whole exome sequencing was carried out for the child, and Sanger sequencing was used to verify the candidate variants. RESULTS: The female neonate has developed progressive respiratory failure and refractory pulmonary hypertension shortly after birth. Conventional treatment such as mechanical ventilation, vasoactive drugs, and inhaled nitric oxide were ineffective. She has developed sustained pulmonary hypertension after weaning from extracorporeal membrane oxygenation therapy, and had died after the treatment had ceased. Whole exome sequencing revealed that she has harbored a heterozygous de novo variant of c.682_683insGCGGCGGC (p.G234Rfs*148) of the FOXF1 gene, which was predicted as pathogenic based on guidelines from the American College of Medical Genetics and Genomics (ACMG), with evidence items of PVS1_Strong+PM2_Supporting+PS2. Based on her clinical manifestations and result of genetic testing, the child was diagnosed with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). CONCLUSION Discovery of the c.682_683insGCGGCGGC (p.G234 Rfs*148) variant of the FOXF1 gene has expanded the mutational spectrum of the FOXF1 gene, which has facilitated implementation of specific treatment and provided a basis for clinical diagnosis and genetic counseling.
Female ; Humans ; Child ; Infant, Newborn ; Pregnancy ; Persistent Fetal Circulation Syndrome/therapy* ; Hypertension, Pulmonary ; Pulmonary Veins ; Forkhead Transcription Factors/genetics*

OBJECTIVES: To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. METHODS: Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. RESULTS: Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). CONCLUSIONS TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.
Female ; Humans ; Pregnancy ; Echocardiography ; Heart Defects, Congenital/surgery* ; Pulmonary Veins/surgery* ; Retrospective Studies ; Scimitar Syndrome/surgery* ; Infant, Newborn

Atrial Fibrillation/surgery* ; Catheter Ablation ; Humans ; Pulmonary Veins

Atrial Fibrillation/complications* ; Humans ; Pulmonary Disease, Chronic Obstructive/complications* ; Pulmonary Veins

Pulsed electric field(PEF) provides high-energy instantaneous pulse and release energy to myocardial cell membrane, resulting in irreversible electroporation and causes myocardial cell contents leakage, destruction of intracellular homeostasis, cell death, and slight inflammatory response. PEF as non-thermal energy promotes the design and application of arrhythmia ablation catheter to enter a new stage. There are currently limited clinical studies that have proved the safety and effectieness of Farawave PEF catheter, PVAC GOLD PEF catheter, Lattice-tip Sphere-9 PEF and radiofrequency (RF) catheter used for atrial fibrillation ablation, but still need further discussion. The research of atrial fibrillation ablation with PEF is under study in China. In this paper, the design and application of PEF ablation for tachyarrhythmia are reviewed.
Atrial Fibrillation/surgery* ; Catheter Ablation ; Catheters ; Humans ; Pulmonary Veins/surgery* ; Tachycardia

Objective: To investigate the influencing factors of in-stent restenosis (ISR) following successful stent implantation in patients with ablation-associated severe pulmonary vein stenosis (PVS) who undergo atrial fibrillation ablation. Methods: Data of patients who underwent pulmonary vein angiography to confirm PVS after radiofrequency ablation for atrial fibrillation (AF) and received pulmonary vein stenting at Shanghai Chest Hospital from March 2010 to December 2017 were retrospectively analysed. All patients were followed up for a long period of time (pulmonary vein contract-enhanced CT within 6 to 12 months after operation was performed, and pulmonary angiography was performed if CT indicated stenosis>50%). The incidence of ISR was recorded. According to angiography, the patients were divided into ISR group and non-ISR group. The clinical and intraoperative imaging characteristics and interventional data were compared between the two groups. Logistic regression was used to analyse the influencing factors of ISR. Results: A total of 47 patients ((47.1±12.2) years old) were enrolled in this study, including 28 males(59.6%). There were 19 cases in ISR group and 28 cases in non-ISR group. Compared with the non-ISR group, the ISR group received more pulmonary vein isolation ((2.8±0.9) vs. (1.8±1.3), P=0.02), and the interval between last ablation and stenting was longer ((19.4±9.6) vs. (13.0±12.4), P=0.03). The incidence of ISR in patients with stent diameter≤8 mm was significantly higher than those with stent diameter>8 mm (33.3%(20/60) vs. 8.1%(3/37), P<0.01). Logistic regression analysis found that the number of radiofrequency ablation>1 (OR=2.1, 95%CI 1.3-3.9, P=0.02), and the time from the last ablation to stent placement>12 months (OR=1.5, 95%CI 1.1-2.5, P=0.03), reference diameter of stenosed distal vessel (OR=0.7, 95%CI 0.5-0.9, P=0.04), post procedural minimal luminal diameter (OR=0.4, 95%CI 0.2-0.8, P=0.02) and stent diameter (OR=0.6, 95%CI 0.3-0.9, P=0.03) were independent factors of ISR. Conclusions: The greater number of radiofrequency ablations and the longer time from the last ablation to stent placement increase the risk of ISR. The larger reference diameter of the stenosed distal vessel, stent diameter and post procedural minimal luminal diameter are the protective factors of ISR.
Adult ; Atrial Fibrillation ; Catheter Ablation ; China ; Constriction, Pathologic ; Coronary Restenosis ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Veins ; Retrospective Studies ; Stenosis, Pulmonary Vein ; Stents ; Treatment Outcome

Atrial Fibrillation/surgery* ; Catheter Ablation ; Electrophysiologic Techniques, Cardiac ; Humans ; Pulmonary Veins/surgery* ; Recurrence ; Treatment Outcome

The advent of catheter ablation technology has changed the treatment strategy for atrial fibrillation, and the efficacy of catheter ablation is accurate with small surgical trauma. Catheter ablation treatment of atrial fibrillation is significantly better than pharmacologic therapy of anti-arrhythmia and rate control. However, the clinic data of catheter ablation of atrial fibrillation show that the recurrence rate is high. The risk factors for recurrence after catheter ablation include age, sex, body mass index, related primary disease, left atrial volume, pulmonary vein volume, gene, atrial fibrillation types, surgery and so on. Regulation of the above factors is crucial in improving the clinical efficacy and prognosis of catheter ablation of atrial fibrillation.
Anti-Arrhythmia Agents ; Atrial Fibrillation ; Catheter Ablation ; Humans ; Pulmonary Veins ; Recurrence ; Risk Factors ; Treatment Outcome

PURPOSE: Details on the hemodynamic differences among Fontan operations remain unclear according to respiratory and cardiac cycles. This study was undertaken to investigate hemodynamic characteristics in different types of Fontan circulation by quantification of blood flow with the combined influence of cardiac and respiratory cycles. MATERIALS AND METHODS: Thirty-five patients [10 atriopulmonary connections (APC), 13 lateral tunnels (LT), and 12 extracardiac conduits (ECC)] were evaluated, and parameters were measured in the superior vena cava, inferior vena cava (IVC), hepatic vein (HV), baffles, conduits, and left and right pulmonary artery. Pulsatility index (PIx), respiratory variability index (RVI), net antegrade flow integral (NAFI), and inspiratory/expiratory blood flow (IQ/EQ) were measured by intravascular Doppler echocardiography. RESULTS: The PIx between APC and total cavopulmonary connection (TCPC; LT and ECC) showed significant differences at all interrogation points regardless of respiratory cycles. The PIxs of HVs and IVCs in APC significantly increased, compared with that in LT and ECC, and the RVI between APC and TCPC showed significant differences at all interrogation points (p < 0.05). The NAFI and IQ/EQ between APC and TCPC showed significant differences at some interrogation points (p < 0.05). CONCLUSION: Patients with different types of Fontan circulation show different hemodynamic characteristics in various areas of the Fontan tract, which may lead to different risks causing long-term complications. We believe the novel parameters developed in this study may be used to determine flow characteristics and may serve as a clinical basis of management in patients after Fontan operations.
Circulatory and Respiratory Physiological Phenomena ; Echocardiography, Doppler ; Fontan Procedure ; Hemodynamics ; Hepatic Veins ; Humans ; Pulmonary Artery ; Vena Cava, Inferior ; Vena Cava, Superior