Scimitar syndrome with vein stenosis in an infant.

Scimitar syndrome is characterised by right lung hypoplasia and abnormal pulmonary venous return, known as the 'scimitar vein'. We report the case of an infant girl with scimitar syndrome who developed a severe respiratory distress mimicking asthma. Pulmonary hypertension (PH) was diagnosed, attributed to scimitar vein stenosis and a left-to-right shunt. Scimitar vein stenosis, a rare complication of scimitar syndrome, can lead to severe PH, highlighting the importance of prompt management in specialised care centres.

Isolated Partial Anomalous Pulmonary Veins: A 10-Year Experience at a Single Center.

INTRODUCTION: Isolated partial anomalous pulmonary venous connection (PAPVC) is difficult to diagnose, and surgical indications remain controversial. We reviewed 10 y of isolated PAPVC cases. METHODS: The data of patients with isolated PAPVC admitted to the Anzhen Congenital Heart Disease Department from 2010 to 2019 were reviewed retrospectively. RESULTS: Thirty patients, aged between 4 mo and 32 y, were included in this study. Significant correlations were found between the right ventricle (RV), end-diastolic dimension Z-score (RVED-z) and age (r = 0.398, P = 0.03), and between estimated pulmonary pressure and age (r = 0.423, P = 0.02). However, no significant correlations were found between the RVED-z and the number of anomalous pulmonary veins (r = 0.347, P = 0.061), between estimated pulmonary pressure and the RVED-z (r = 0.218, P = 0.248), and between estimated pulmonary pressure and the number of anomalous veins (r = 0.225, P = 0.232). Transthoracic echocardiography (TTE) confirmed 90% of isolated PAPVC cases. Surgical repair was performed in 29 patients with RV enlargement, persistent low weight, pulmonary hypertension, or respiratory symptoms. Among the surgical patients, nine had elevated pulmonary pressure before surgery, which decreased postoperatively; no mortality or reintervention was observed. The mean duration of echocardiographic follow-up was 1.9 y. CONCLUSIONS: TTE is recommended for routine assessments, and further clarification can be obtained with computed tomography when TTE proves inconclusive for diagnosis. Transesophageal echocardiography and computed tomography are further recommended for adult patients if TTE fails to provide clear results. PAPVC should be considered as an underlying cause when unexplained RV enlargement is observed. Surgery is recommended for patients with RV enlargement, pulmonary hypertension, or respiratory symptoms.

Right S3 segmentectomy for lung cancer with partial anomalous pulmonary venous return in the right upper pulmonary vein: A case report.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital malformation where the pulmonary vein partially refluxes into the venous system. Here, we present the first robotic-assisted right S3 segmentectomy in a 70-year-old male with early-stage lung cancer and PAPVR in the right upper pulmonary vein. The patient, with suspected primary lung cancer (11 mm diameter, pure solid appearance in right S3 segment), exhibited clinical stage T1bN0M0 stage IA2. Preoperative computed tomography revealed severe lung emphysema, and right V1-3 returned directly to the superior vena cava. However, no signs of right-sided heart failure were observed, and echocardiogram was normal with a pulmonary-to-systemic blood flow ratio of 1.4. Successful robot-assisted right S3 segmentectomy with hilar nodal dissection was performed, and the patient was discharged on the sixth postoperative day without complications. One year postoperatively, there has been no recurrence of lung cancer or respiratory/right-sided heart failure symptoms.

Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

A rare variant of scimitar syndrome characterised by right pulmonary vein drainage to portal vein with eventeration of diaphragm.

While infradiaphragmatic total anomalous pulmonary venous drainage to portal vein is well described, hemianomalous drainage of right pulmonary veins to portal vein in Scimitar syndrome has not yet been reported.

STIC-HD live flow technology in the antenatal diagnosis of scimitar syndrome: A case report.

Scimitar syndrome (SS) is a rare entity with an incidence of approximately 1-3 in 200 000 people. It is typically characterized by complete or partial anomalous pulmonary venous drainage from the right lung into the systemic venous circulation, most commonly the inferior vena cava (IVC). For the first time, we report the diagnosis of SS in a fetus in utero using four-dimensional (4D) spatiotemporal image correlation combined with high-definition live flow rendering mode (STIC-HD live flow).

[Left Metastatic Lung Tumor for a Patient with Scimitar Syndrome:Report of a Case].

Scimitar syndrome is a subtype of partial anomalous pulmonary venous connection, a rare congenital disorder associated with hypoplasia of the right lung. In addition to the difficulty of isolated lung ventilation, resection of the left lung is associated with the risk of developing right heart failure due to increased right-to-left shunts. We report a case of a left lung metastasis of a patient with scimitar syndrome. The patient, a 58-year-old male, was diagnosed with scimitar syndrome at the age of 26 but had never experienced any symptoms. He underwent chemoradiotherapy for mid-pharynx carcinoma and achieved complete response. During follow-up, a nodule appeared in the lower lobe of the left lung. Since right heart catheterization revealed a pulmonary blood flow/systemic blood flow ratio (Qp/Qs) ratio of 2.6, intra-cardiac blood flow was diverted prior to pulmonary resection. Stanford type A acute aortic dissection occurred intra-operatively, and total aortic arch replacement was performed. Three months later, partial pulmonary resection was performed with extracorporeal membrane oxygenation (ECMO) on standby. As oxygenation was maintained by placing a blocker in the left lower lobe bronchus and ventilating the left upper lobe with high frequency jet ventilation, the operation was completed without using ECMO. The nodule was pathologically diagnosed as metastasis of mid-pharynx carcinoma. He did not develop heart failure and was discharged on post operated day 15.

Long-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum.

The natural history of an unrepaired isolated partial anomalous pulmonary venous connection(s) (PAPVC) and the absence of other congenital anomalies remains unclear. This study aimed to expand the understanding of the clinical outcomes in this population. Isolated PAPVC with an intact atrial septum is a relatively uncommon condition. There is the perception that patients with isolated PAPVC are usually asymptomatic, that the lesion generally has a limited hemodynamic impact, and that surgical repair is rarely justified. For this retrospective study, we reviewed our institutional database to identify patients with either 1 or 2 anomalous pulmonary veins that drain a portion of but not the complete ipsilateral lung. Patients with previous surgical cardiac repair, coexistence of other congenital cardiac anomalies that would result in either pretricuspid or post-tricuspid loading of the right ventricle (RV), or scimitar syndrome were excluded. We reviewed their clinical course over the follow-up period. We identified 53 patients; 41 with a single and 12 with 2 anomalous PAPVC. A total of 30 patients (57%) were men, with a mean age at the latest clinic visit of 47 ± 19 years (18 to 84 years). Turner syndrome (6 of 53, 11.3%), bicuspid aortic valve (6 of 53, 11.3%), and coarctation of the aorta (5 of 53, 9.4%) were commonly associated anomalies. A single anomalous left upper lobe vein was the most commonly identified variation. More than half of the patients were asymptomatic. Cardiopulmonary exercise test demonstrated a maximal oxygen consumption of 73 ± 20% expected (36 to 120). Transthoracic echocardiography demonstrated a mean RV basal diameter of 4.4 ± 0.8 cm, RV systolic pressure of 38 ± 13 (16 to 84) mm Hg. A total of 8 patients (14.8%) had ≥moderate tricuspid regurgitation. Cardiac magnetic resonance in 42 patients demonstrated a mean RV end-diastolic volume index of 122 ±3 0 ml/m2 (66 to 188 ml/m2), of which in 8 (14.8%), it was >150 ml/m2. Magnetic resonance imaging-based Qp:Qs was 1.6 ± 0.3. A total of 5 patients (9.3%) had established pulmonary hypertension (mean pulmonary artery pressure ≥25 mm Hg). In conclusion, isolated single or dual anomalous pulmonary venous connection is not necessarily a benign congenital anomaly because a proportion of patients develop pulmonary hypertension and/or RV dilation. Regular follow-up and on-going patient surveillance with cardiac imaging is advised.

Novel operative technique for Scimitar syndrome: Posterior flap technique.

Various surgical techniques have been reported for Scimitar syndrome, because of the heterogenous anatomy of the disease. We developed a novel surgical method to repair Scimitar syndrome, in which, a new pulmonary venous route is constructed behind the inferior vena cava using autologous flaps of the inferior vena cava and the interatrial septum. An adult case of Scimitar syndrome was repaired by this method with good results.

[Integrated management during the perinatal period for total anomalous pulmonary venous connection]. / å®Œå ¨æ€§è‚ºé™è„‰å¼‚ä½è¿žæŽ¥çš„å›´ç”ŸæœŸä¸€ä½“åŒ–ç®¡ç†.

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.

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection is a rare CHD with a wide variation in pulmonary venous anatomy and drainage. We present a ten-day-old newborn with a rare bizarre subtype of mixed total anomalous pulmonary venous connection.

Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.

Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.