Atrial thrombus after total anomalous pulmonary venous connection repair.

A 6-year-old boy had previously undergone total anomalous pulmonary venous connection repair and postoperative pulmonary vein stenosis release. Magnetic resonance imaging revealed blood stasis caused by a collision between the inflow from the pulmonary veins and the outflow from the left atrial appendage. A surgical specimen revealed evidence of advanced thrombus attachment.

De Novo and Progressive Pulmonary Vein Stenosis Following Pediatric Heart Transplantation: A Multicenter Retrospective Study.

Background: Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation leads to venous and arterial hypertension. Little is known about PVS after heart transplant (HTx) in children. We sought to describe the characteristics and outcomes of children who develop PVS after HTx.

Catheter ablation for patients with anomalous pulmonary venous return and atrial fibrillation: a case report and literature review.

Background: Anomalous pulmonary venous return involves the partial or complete absence of a connection between the pulmonary veins and the left atrium. The pulmonary vein potential plays a vital role in atrial fibrillation, and catheter ablation to isolate the pulmonary vein is crucial for treating this condition. However, when anomalous pulmonary venous return is present, it makes ablation more challenging and increases the risk of atrial fibrillation recurrence after the procedure.

Advancing prenatal diagnosis: Echocardiographic detection of Scimitar syndrome in China - A case series.

Objective: 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.

Methods: 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.

Scimitar Syndrome in Adulthood: Challenges in Management and Individualized Approaches.

Scimitar syndrome is a congenital disorder characterized by partial anomalous pulmonary venous return to the inferior vena cava (IVC). Clinical manifestation in adulthood is infrequent. The management approach has not been universally accepted and may be challenging.

Diagnostic value of reversed differential cyanosis in (supra)cardiac total anomalous pulmonary venous return.

Background: To investigate the occurrence of reversed differential cyanosis (RDC) in case of (supra)cardiac total anomalous pulmonary venous return (TAPVR), we explored the hemodynamic changes and oxygen saturation levels during the fetal-to-neonatal transition in (supra)cardiac TAPVR, thereby revealing determinant factors of RDC.

Methods: A computational model was used to simulate the cardiovascular fetal-to-neonatal transition up to 24 h after birth. Abnormalities associated with TAPVR, like patent ductus arteriosus (PDA) and persistent pulmonary hypertension of the neonate (PPHN), were imposed on the model.

Scimitar syndrome in a four-month-old infant.

Unlabelled: The Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable congenital anomaly of cardiopulmonary development characterised by an abnormal right-sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. These serious anomalies notwithstanding, their presentation is varied, ranging from asymptomatic to severe symptoms, particularly in the neonatal and early infantile period. Some symptomatic cases are misdiagnosed as recurrent lower respiratory tract infections or as heart failure, missing the underlying congenital anomaly.

Double Connection of Left-Sided Partial Anomalous Pulmonary Vein Return in a Young Man.

Double connection of partial anomalous pulmonary venous return is a very rare congenital anomaly where at least one pulmonary vein, but not all, drains into the left atrium and systemic venous circulation with subsequent left to right shunt.

Open-heart surgery in preterm infants: A single-center experience.

Background: Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants.

Materials Methods And Results: This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery.

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period.

Sinus Venosus Atrial Septal Defect as an Overlooked Source of Shortness of Breath Among Patients With Pulmonary Arterial Hypertension.

Sinus venosus atrial septal defects (SVASD) associated with partial anomalous pulmonary venous return (PAPVR) can be overlooked as a source of dyspnea in adult patients with pulmonary hypertension. We present the case of a 61-year-old male with exertional dyspnea initially attributed to pulmonary hypertension, who was subsequently diagnosed with SVASD and right superior PAPVR. This case underscores the critical importance of maintaining high clinical awareness and utilizing multimodal imaging techniques in cardiology to accurately diagnose and manage pulmonary hypertension secondary to congenital heart disease.

Complex ADHD Challenging Case: Managing Co-Occurring Attention-Deficit Hyperactivity Disorder and Congenital Heart Disease with a Limited Medication Formulary: A Case from Mexico.

DL is an 8-year-old Mexican boy with a posterior atrial septal defect and partial anomalous pulmonary venous return of the right lower pulmonary vein with resultant right heart dilation with normal right ventricular systolic and diastolic function and no arrhythmias. Surgical repair was deferred, and DL's condition was being medically managed with furosemide 0.5 mg/kg BID and spironolactone 0.

TAPVR: Molecular Pathways and Animal Models.

The venous pole of the heart where the pulmonary veins will develop encompasses the sinus venosus and the atrium. In the fourth week of development, the sinus venosus consists of a left and a right part receiving blood from the common cardinal vein, the omphalomesenteric and umbilical veins. Asymmetrical expansion of the common atrium corresponds with a rightward shift of the connection of the sinus to the atrium.

Total Anomalous Pulmonary Venous Connections, Human Genetics.

Partial anomalous pulmonary venous connections (PAVC) have been found after abnormal gene expressions involving several syndromes. Total anomalous pulmonary venous connection (TAPVC) is found in conjunction with heterotaxia syndrome as well as several other syndromes. It has been reported with an autosomal dominance with variable expression and incomplete penetrance.

Clinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return.

Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig.

A Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot.

Total anomalous pulmonary venous return (TAPVR) represents a group of anomalies consisting of a lack of connection between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible with a right-to-left atrial shunt.

Prenatal Ultrasound Markers of Isolated Total Anomalous Pulmonary Venous Return and a Sequential Approach to Reach Diagnosis.

This article comprehensively reviews the literature concerning prenatal ultrasound findings of isolated total anomalous pulmonary venous return (TAPVR) and the application of specific sonographic markers to differentiate among the TAPVR types. These markers can be categorized as direct and indirect, based on either morphological features or hemodynamic changes in TAPVR. Indirect markers include the ventricular disproportion, an increased distance between the left atrium (LA) and the descending aorta, as well as the dilatation of superior vena cava or coronary sinus for supracardiac or cardiac TAPVR, along with abnormal pulmonary venous spectral Doppler patterns.

Adult Partial Anomalous Pulmonary Venous Connection: Case Reports (Including a Case of Scimitar Syndrome).

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.