UNASSIGNED: To investigate the clinical value of echocardiographic detection in the prenatal early diagnosis of Scimitar syndrome (SS) in fetuses, and to develop better and more accurate management strategies for improved prognosis.
UNASSIGNED: A retrospective analysis was conducted on medical records and fetal echocardiographic findings of all cases diagnosed as SS between April 1, 2016 and June 1, 2021. To summarize its echocardiographic features and distinguishing points, comprehensive clinical data and prognostic information were gathered.
UNASSIGNED: Six patients were diagnosed with SS during the study period. Major associated abnormalities included atrial septal defect (n = 3), right inferior pulmonary vein anomalies (n = 2), ventricular septal defect (n = 1), and right aortic arch (n = 1). Post-surgery, all patients exhibited unobstructed pulmonary vein flow and absence of pulmonary hypertension. The average follow-up duration was 24 months, during which five infants underwent surgical intervention for SS.
UNASSIGNED: Comprehensive prenatal screening, particularly combined coronal and sagittal views of the fetal thorax, enables accurate diagnosis of right SS. This approach not only aids in timely intervention but also provides crucial prognostic insights for the child\'s future well-being.

Partial anomalous pulmonary venous connection (PAPVC) is a congenital heart defect in which one or more pulmonary veins drain abnormally into the systemic venous circulation, leading to the development of pulmonary arterial hypertension. It can be supracardiac type, draining into the superior vena cava or right atrium (also called cardiac type) and infracardiac type with drainage into the inferior vena cava (IVC). We present two cases-supracardiac and infracardiac types of PAPVC in this case report.

Right atrial isomerism is a rare and severe isomerism. It is frequently associated with complex congenital heart disease and various extracardiac anomalies. Imaging diagnosis of right atrial isomerism is a challenge. Multisystem and complex anomalies in a 24-week-old fetus were diagnosed with prenatal ultrasound, postnatal computed tomography angiography (CTA), and autopsy. The ultrasound detected most major cardiovascular anomalies including right atrial isomerism and total anomalous pulmonary venous connection. The CTA further detected thoracic and abdominal malformations such as bilateral morphologically right bronchus, diaphragmatic hernia, asplenia, midline liver, and intestinal malrotation. The autopsy confirmed both ultrasound and CTA findings with additional findings, namely, bilateral trilobed lungs and bilateral morphological right auricles. Prenatal ultrasound and postnatal CTA can be complementary to each other in detecting multi-system complex anomalies. Their combined use can be useful for prenatal counseling and postpartum management.

暂无摘要。

Scimitar syndrome, a rare congenital cardiac anomaly, involves abnormal pulmonary vein drainage into systemic veins, leading to distinct imaging features resembling a curved-blade sword. This case report presents a unique instance of scimitar syndrome in Pakistan, emphasizing its clinical importance and the challenges of management. A 26-year-old female with a history of recurrent pulmonary infections and respiratory symptoms since childhood was diagnosed with scimitar syndrome. Radiological assessments, including chest X-rays, computed tomography pulmonary angiograms (CTPA), and transthoracic echocardiography, confirmed the presence of a curved vessel originating from the right hemidiaphragm and connecting with the inferior vena cava (IVC). The patient and her medical team opted for conservative management, involving multidisciplinary care, tailored treatment for infections, and regular monitoring. The rarity of Scimitar syndrome necessitates careful diagnosis and management decisions. While surgical intervention is often recommended, this case demonstrates the complexities of choosing conservative management based on patient preferences and the evolving clinical course. A literature review reveals varied outcomes of surgical and conservative approaches, emphasizing the need for personalized strategies. Radiological techniques, such as CTPA and MRI, play pivotal roles in diagnosis and monitoring. This case report underscores the clinical significance of scimitar syndrome, particularly in regions with limited reported cases, like Pakistan. The multidisciplinary management approach, the decision-making process regarding conservative treatment, and the unique radiological findings contribute to the medical community\'s understanding of this rare condition.

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.

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.

Scimitar syndrome is a rare congenital anomaly characterized by partial or total anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We report a case of a 67-year-old female who presented with cough and dyspnea and was diagnosed with scimitar syndrome and pulmonary arterial hypertension based on comprehensive imaging and hemodynamic evaluation. This case highlights the importance of considering scimitar syndrome as a cause of pulmonary hypertension even in adult patients.

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).

UNASSIGNED: Scimitar syndrome is a very rare congenital cardio-pulmonary disease with anomalous right pulmonary vein draining either partially or completely into the inferior vena cava. It is called Scimitar syndrome due to the classical appearance in the chest X-ray, which resembles the curved blade of Turkish sword \'Scimitar\'. It commonly associates with atrial septal defect (ASD), hypoplasia of the right lung, dextroposition of the heart, and pulmonary hypertension (PHT).
UNASSIGNED: A 67-year-old lady, diagnosed with atrial fibrillation and moderate PHT 3 years ago, presented with worsening bilateral ankle oedema and New York Heart Association class III shortness of breath. Chest X-ray showed the Scimitar appearance. The trans-thoracic and trans-oesophageal echocardiograms revealed a 46 mm ASD and a partial anomalous pulmonary venous drainage (PAPVD) of the right upper pulmonary vein (RUPV) into the right atrium at the junction of the atria. Three-dimensional reconstruction of the computed tomographic pulmonary angiogram confirmed Scimitar syndrome of the right lower pulmonary vein (RLPV). We managed her conservatively on her wish. After 13 months, she succumbed due to a massive stroke.
UNASSIGNED: We describe a very rare case of an elderly lady who has Scimitar syndrome with an ASD and evidence of PAPVD of the RUPV; thus, we intend to provide an antecedent for further cases, for prompt and accurate diagnosis and timely interventions in order to prevent life-threatening complications.