Uncovering the Hidden Cause of Recurrent Chest Infections in a Child: A Case Report.

Recurrent chest infections can present diagnostic challenges, especially when the underlying cause remains elusive despite initial evaluations and treatments. This case report details the clinical journey of a patient experiencing recurrent chest infections over several months, during which conventional diagnostic approaches initially failed to provide lasting relief. Here, we present the case of a 16-month-old female child who had been experiencing recurrent chest infections since the age of 10 months, ultimately diagnosed as a case of Partial Anomalous Pulmonary Venous Return (PAPVR).

Intraoperative Acute Cardiac Tamponade as a Result of Intracardiac Perforation Requiring Emergency Continuous Pericardiocentesis and Open Sternotomy: A Case Report and Literature Review of a Rare but Fatal Complication.

Intraoperative acute cardiac tamponade associated with iatrogenic intracardiac perforation from percutaneous interventional cardiac procedures is a rare but potentially catastrophic complication. We report a case of intraoperative acute hemopericardium caused by a left atrial (LA) perforation resulting in cardiac tamponade in a patient undergoing a baffling procedure for the correction of two anomalous pulmonary veins draining into her superior vena cava (SVC) that required continuous pericardiocentesis with autologous blood transfusion via the femoral vein and an emergency intraoperative transfer from the interventional cardiology cath lab to the cardiac operating room for an open sternotomy and primary repair. An 86-year-old female with known right-ventricular (RV) failure with preserved ejection fraction (left ventricular ejection fraction (LVEF): 50-55% on transesophageal echocardiography (TEE) one week prior) and atrial fibrillation was admitted for her third heat failure exacerbation in two months despite being adherent to her aggressive diuresis medication regimen. Upon her readmission and due to her symptomatic and seemingly refractory heart failure, the patient underwent a cardiac computer tomography (CT) with 3D reconstruction that showed previously undiagnosed partial anomalous pulmonary venous return (PAPVR) of two of her four pulmonary veins aberrantly draining into the SVC. This anatomic pathology was deemed to be the likely etiology of her repeated episodes of recurring heart failure exacerbations, shortness of breath, peripheral edema, and fatigue. The patient was counseled and consented to a percutaneous baffle of the two anomalous veins to redirect more of the returning pulmonary venous blood away from the SVC and to the LA. While under general endotracheal anesthesia (GETA) with a TEE in place during the procedure, the patient suddenly developed acute hypotension, tachycardia, and a reduction in expired carbon dioxide (EtCO2) was noted quickly followed by evidence of a rapidly accumulating hemopericardium on TEE. Cardiothoracic surgery was urgently consulted to the interventional cardiology cath lab while the patient underwent an emergency pericardiocentesis that momentarily alleviated her hemodynamic instability, cardiac tamponade physiology, and deteriorating overall clinical picture. While performing continuous pericardiocentesis with autologous return of the aspirated blood via femoral venous access the patient was urgently transported to the cardiac operating room and prepped for emergency sternotomy for primary repair of the LA. Following primary repair via sternotomy, multiple drains were placed and the thoracic cavity was closed with wires. The patient was immediately transported to the surgical intensive care unit (SICU) intubated, mechanically ventilated, and sedated. During this time, the patient progressively required additional vasoactive and inotropic agents to support her mean arterial pressure (MAP), and following a multidisciplinary discussion with the patient's family regarding her goals of care, the decision was made to withdraw further resuscitation efforts and the patient expired four hours later.

Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of "scimitar lung".

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular condition in which some of the pulmonary veins drain into the systemic circulation. We report on the cadaveric dissection of a 71-year-old Caucasian male donor who died of chronic obstructive pulmonary disease with hypertension. We noted a faint incisional scar on the thorax extending from the parasternal region at the 4th intercostal level to the midaxillary line. Since the straight-line incision followed the ribs and the scar was quite faint, surgery probably occurred when the donor was young. We also observed numerous surgical interventions of the heart, lungs, and vasculature to correct various defects. The morphology of the heart was normal, but was shifted more to the right side. An atrial septal defect (ASD) was closed with sutures. The right superior pulmonary vein that drained into the superior vena cava (SVC) was ligated close to the SVC and the right inferior, left superior, and inferior pulmonary veins all drained directly into the left atrium. We noticed a dilated coronary sinus entering the right atrium adjacent to the ASD; the ostium of the coronary sinus noticeably lacked the normal valve-like structure. We initially thought the right lung was a "horseshoe" lung, but realised that it was a "hypogenetic" lung with PAPVR and an accessory diaphragm. Compared to the left, the right secondary bronchi were much narrower and branched uncharacteristically, as seen in hypogenetic lung syndrome. The inferior lobe was highly disorganised, severely hypoplastic, and exhibited uncharacteristic morphology. The superior bronchopulmonary segment was markedly hypoplastic. The posterior and medial basal segments were not only hypoplastic and slender, but also extended like a tail to the left pulmonary cavity behind the heart/pericardium and in front of the oesophagus and aorta. The right lung, though hypoplastic, demonstrated patent bronchi and the lobes were inflatable. Based on the hypogenetic lung and PAPVR, we conclude that the donor exhibited 'scimitar' lung.

Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Scimitar syndrome (SS) is a rare congenital condition which includes partial anomalous pulmonary venous return (PAPVR) and a variable degree of pulmonary hypoplasia. We describe the clinical features, therapeutic approach and outcomes of patients who underwent cardiac catheterization and/or surgical repair of the scimitar vein at a single institution in the United States. This retrospective cohort study included all patients with SS who underwent scimitar vein surgical repair or cardiac catheterization from October 1989 through August 2021 in a tertiary care center. A total of 84 patients with SS were included and median follow-up time was 74 months. Patients diagnosed with SS under the age of one year had a significantly greater incidence of congenital heart defects (CHD) (p < 0.001), non-cardiac anomalies (p = 0.02), pulmonary hypertension (p = 0.02), and mortality (p = 0.04) compared to those diagnosed over the age of 1 year. Twenty-eight patients underwent surgical repair of the scimitar vein. Overall, eight (10%) patients died. Compared to surviving patients, deceased patients had a significantly higher incidence of pulmonary hypertension (PH), neonatal SS diagnosis, and extracorporeal membrane oxygenation (ECMO) support. Median scimitar vein pressure (20 mmHg) of deceased patients was significantly higher compared to pressures in surviving patients (11 mmHg; p = 0.02). PH, CHD, neonatal SS diagnosis, ECMO support, and markedly elevated scimitar vein pressure are associated with mortality. Scimitar vein surgical repair during infancy is commonly associated with PH and restenosis that requires re-intervention.

Partial Anomalous Pulmonary Venous Return in Adults.

Partial anomalous pulmonary venous return (PAPVR) is a spectrum of congenital cardiovascular abnormalities. It is most commonly found as an incidental finding. However, it can lead to severe pulmonary hypertension depending on the magnitude of the shunt involved. We report a case of a 60-year-old female patient with PAPVR detected incidentally on imaging. We aim to highlight the incidence of PAPVR in adults and to elaborate on its unique association with a duplicated superior vena cava.

Staged approach for correction of anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return into the azygous vein is a rare pathological finding. We describe the case of a 28-year-old girl who had a successful staged approach to treat this rare congenital heart disease. To avoid potential connection of a systemic venous return to the left atrium, the proximal part of the azygous vein was occluded with a percutaneous approach, then the azygous vein flow was redirected into the left atrium with a surgical procedure.

Case Report: An Infant With Kabuki Syndrome, Alobar Holoprosencephaly and Truncus Arteriosus: A Case for Whole Exome Sequencing in Neonates With Congenital Anomalies.

Kabuki syndrome is a rare multiple anomalies syndrome associated with mutations in KMT2D or KDM6A. It is characterized by infantile hypotonia, developmental delay and/or intellectual disability, long palpebral fissures with everted lateral third of the lower eyelids and typical facial features. Intracranial anomalies occur infrequently in patients with KS and holoprosencephaly has only been recently described. Additionally, though congenital heart diseases are common in patients with KS, to our knowledge truncus arteriosus has never been reported in a patient with KS. We present an unusual case of KS in an infant with holoprosencephaly and truncus arteriosus with partial anomalous pulmonary venous return. Duo whole exome sequencing in our patient identified a pathogenic nonsense variant in exon 10 of KMT2D (c.2782C > T; p. Gln928*) establishing the diagnosis. This report further expands the phenotypic spectrum of patients with Kabuki syndrome and emphasizes the utility of performing large scale sequencing in neonates with multiple congenital anomalies.

A unique case of unilateral pulmonary edema from partial anomalous pulmonary venous return in conjunction with superior vena cava stenosis.

While PAPVR is most commonly an incidental finding on chest CT, a unique case is presented where PAPVR in conjunction with SVC stenosis resulted in chronic symptomatic asymmetric pulmonary edema. The case reflects an unusual anatomic cause of unilateral edema, as a combination of both congenital and subsequently acquired anatomic anomalies.

Cardiac CT and Transesophageal Echocardiogram Evaluation of a Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return and a Persistent Left Superior Vena Cava.

The sinus venosus (SV) plays a significant role in the embryological heart as the initial structure where the cardinal, umbilical, and vitelline veins drain before remodeling into the caval veins. As the human heart develops, the SV incorporates into the posterior wall of the right atrium. Sinus venosus atrial septal defects (SVASDs) result from a defect in the wall present among the right pulmonary veins, the superior vena cava (SVC), and the right atrium. Persistent left superior vena cava (PLSVC) occurs when the Marshall ligament does not regress, and in most cases, the PLSVC enters the coronary sinus before draining into the right atrium. Pulmonary hypertension from chronic left to right shunting makes recognizing this condition clinically significant. In this case report, both cardiac CT and transesophageal echocardiogram were used to further evaluate an SVASD with partial anomalous pulmonary venous return (PAPVR) of the right superior pulmonary vein, in addition to a PLSVC. The incidence of the co-occurrence of SVASD and PLSVC, as well as the association between the two, were discussed in this case report. Future research should focus on the potential genetic causes of this co-occurrence. It should also focus on patient treatment and outcomes at different stages of presentation to optimize patient management and improve mortality.

Case Report: Constrictive Pericarditis in a Patient With Isolated Anomalous Right Upper Pulmonary Venous Return.

Thirty-eight-year-old male presented for evaluation of abdominal swelling, lower extremity edema and dyspnea on exertion. Extensive work-up in search of the culprit etiology revealed the presence of an Anomalous Right Upper Pulmonary Venous Return (ARUPVR) into the Superior Vena Cava (SVC). During the attempted repair, the pericardium was found to be thickened and constrictive. Only one other case of co-existent partial anomalous pulmonary venous return and constrictive pericarditis (CP) has been reported. The patient underwent a warden procedure with pericardial stripping with good outcomes at 45 days post-operatively. Thus, the presence of severe heart failure symptoms in the setting of ARUPVR should prompt further investigations. Also, further cases are needed to help guide management in these patients.

4-Dimensional Flow by Cardiac Magnetic Resonance Informs Surgical Planning in Partial Anomalous Pulmonary Venous Return.

Four-dimensional flow cardiac magnetic resonance enhances the visualization of blood flow in a 3-dimensional volume throughout the cardiac cycle, thus dramatically improving visualization of pulmonary venous anatomy by cardiac magnetic resonance. We demonstrate the impact of 4-dimensional flow on diagnosis and surgical planning for partial anomalous pulmonary venous return. (Level of Difficulty: Beginner.).

Anesthetic Management of Right Single-lung Ventilation in a Patient with Anomalous Left Superior Pulmonary Venous Return for Left Pulmonary Lobectomy.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are connected to the right atrium or to the systemic venous system. One lung ventilation (OLV) is required for a number of thoracic procedures. When switching to OLV, right-to-left shunt fraction increases, oxygenation is impaired, and hypoxemia may occur. Hypoxemia during OLV may affect the safety of the patient and is a challenge for the anesthesiologist and the surgeon. This case details the intraoperative anesthetic management of an elderly patient with a PAPVR who underwent single-lung ventilation for lung resection surgery.

Partial anomalous pulmonary venous return: A case series with management approach.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly that results in a left-to-right shunt. Based on the shunt fraction, PAPVR has a wide spectrum of presentations. If a significant left-to-right shunt is left unrepaired, pulmonary vascular remodeling can occur resulting in the development of pulmonary arterial hypertension (PAH). Furthermore, if the condition is associated with an atrial septal defect (ASD), the patient can develop shunt reversal and Eisenmenger's syndrome in setting of severe PAH. Management plans include close observation, surgical repair, and treatment with pulmonary artery vasodilator therapies. Here, we present multiple cases of PAPVR to highlight the wide spectrum of presentations and the individualized treatment for each case.

A case report: a 22-year-old septic patient with central venous pO2 of 198 mmHg.

BACKGROUND: Central venous saturation and central venous pressure can be determined with central venous catheters. Therefore, the tip of the catheter should be located in the superior vena cava. The location can be monitored by electrocardiography or X-ray. The central venous pressure curve is displayed on the monitor. The reference value of central venous saturation is >70%. Venous pO2 is normally 35-45 mmHg and central venous pressure 1-9 mmHg. CASE SUMMARY: We treated a 22-year-old patient with septic shock. Central venous saturation was 100% with a pO2 of 198 mmHg. The arterial blood gas analysis was comparatively low with saturation of 98% and pO2 of 111 mmHg. On chest X-ray, the central venous catheter tip appeared on the left side of the heart. On echocardiography, aortic positioning was not evident. On the monitor, a 'venous pressure-like' curve was seen, that did not stand in exact correlation to the electrocardiogram curve. The computed tomography (CT) image showed placement of the catheter in the upper left pulmonary vein. The patient had a partial anomalous pulmonary venous return. DISCUSSION: The C-wave of the central venous pressure curve normally occurs after the R-wave of the electrocardiogram. If C-waves appeared before R-waves, the central venous catheter placement is not central venous and must be checked. In our case, the apparent 'venous' pO2 in blood gas examination was higher than arterial pO2. The catheter position had to be in an oxygenated vessel proximal to the left ventricle. A vascular anomaly was a possible diagnosis and was confirmed on CT imaging.

Surgical Management of Concurrent Partial Anomalous Pulmonary Venous Return and Lung Cancer of the Same Lobe.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital left-to-right shunt where pulmonary veins drain into systemic circulation. This is a presentation of the case of a patient with concurrent PAPVR and adenocarcinoma of the same lobe. The pulmonary veins of right upper lobe drained into superior vena cava (SVC), whereas the middle and lower lobes veins drained into the left atrium as two distinct vessels. Surgeons should always perform a diligent search for anomalous vascular structure using computed tomographic (CT) angiography prior to surgery. In our case, surgical approach was "safe" because both pathologies developed in the same lobe.

Partial anomalous pulmonary venous return in Turner syndrome.

PURPOSE: The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome. METHODS AND RESULTS: All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients. CONCLUSION: When studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients.

Endovascular management of a vein of Galen aneurysmal malformation in an infant with challenging femoral arterial access.

A 5-month-old infant was to be treated with elective transarterial embolization for a vein of Galen aneurysmal malformation (VGAM). A team of endovascular surgical neuroradiologists, pediatric interventional radiologists, and pediatric cardiologists attempted conventional femoral arterial access, which was unsuccessful given the small caliber of the femoral arteries and superimposed severe vasospasm. Thereafter, eventual arterial access was achieved by navigating from the venous to the arterial system across the patent foramen ovale following a right femoral venous access. Embolization was then successfully performed. At a later date, the child underwent successful transvenous balloon-assisted embolization and eventual arterial embolization with cure of the VGAM.

Scimitar Syndrome and H-type Tracheo-esophageal Fistula in a Newborn Infant.

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava (IVC) creating a tubular opacity paralleling the right cardiac border on chest radiography which resembles a curved Turkish sword or scimitar. Associated pulmonary and vascular anomalies have been reported in cases of Scimitar syndrome, most commonly hypoplasia of right lung, dextroposition of the heart, hypoplasia of the right pulmonary artery, and aberrant arterial supply from the descending aorta to the affected lobe of the right lung. To the best of our knowledge, this is the first case of Scimitar syndrome with an H-type tracheoesophageal fistula that has ever been reported.

Right lung cancer with partial anomalous pulmonary venous return and absent right upper lobe.

When a vascular shunt is present, major lung resection may cause acute right heart failure. We report a case of right lung cancer with ipsilateral partial anomalous pulmonary venous return (PAPVR) and absent right upper lobe managed by pneumonectomy. A 48-year-old former smoker was diagnosed as right lung cancer; chest computed tomography (CT) revealed an anomalous right pulmonary vein draining into inferior vena cava. Bronchus of right upper lobe was not found in fiberoptic bronchoscopy. Right pneumonectomy was performed. Pathological examination revealed a T2aN2M0 squamous lung cancer. The patient tolerated well without notable descending of arterial oxygen pressure.