An Unusual Case of Scimitar Syndrome with Drainage to the Right Atrium.
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ABSTRACT:
SUBMITTER: Kim SJ
PROVIDER: S-EPMC9314222 | biostudies-literature | 2022 Jul
REPOSITORIES: biostudies-literature
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An Unusual Case of Scimitar Syndrome with Drainage to the Right Atrium.
Kim Shin-Jae SJ Ann Soe Hee SH Park Sangwoo S
Journal of cardiovascular imaging 20220701 3
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Project description:Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC), in which all or part of the right lung is drained by right pulmonary veins that anomalously connect to the inferior vena cava (IVC). The affected lung and its associated airways are often hypoplastic. In addition, aortopulmonary collateral vessels may be involved on the affected side, causing sequestration of that side; such involvement is commonly associated with cardiac defects. We report a case of infantile scimitar syndrome that involved a typical association with the right lung, but with extremely unusual associations with congenital hydrocephalus and heart blockage. The presentation of this case and the role of different diagnostic approaches and management are discussed.
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Project description:Cardiovascular hydatid disease is caused by parasitic infection of Echinococcus granulosus, which could be asymptomatic or life-threatening depending on lesion site, granuloma size, and disease progression. Diagnosis and treatment of cardiac echinococcosis should be under comprehensive consideration. In this case, we reported a successful right atrium-inferior vena cava bypass surgery in a 31-year-old female with unresectable right atrial echinococcosis and inferior vena cava obstruction.
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Project description:Scimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel. A 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the 'scimitar sign'. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign. Although the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.
| S-EPMC6601148 | biostudies-literature
Project description:Scimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel. A 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the 'scimitar sign'. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign. Although the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.
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Project description:BackgroundPlatypnea orthodeoxia syndrome (POS) is a condition characterized by onset or worsening of dyspnoea and desaturation in upright position that is relieved by returning to a supine position. This case report illustrates a sudden onset of severe platypnea caused by compression of the right atrium (RA) due to aortic dilatation and unilateral diaphragmatic paralysis after a recent stroke.Case summaryA 71-year-male patient with a medical history of recent stroke of the left hemisphere was referred to emergency department with acute dyspnoea. During observation in the emergency department, desaturation was noted in upright position. A contrast computed tomography excluded pulmonary embolism but revealed a dilated aortic root and an elevated right hemidiaphragm. The RA was compressed between these two structures (sandwiched). Given the clinical suspicion of a POS, a transoesophageal echocardiography was performed which confirmed the presence of a persistent foramen ovale (PFO) in supine position. In upright position, there was a torrential increase in right-to-left shunting. The PFO was closed using an Occlutech™ device. Directly after the procedure, the patient was symptom free.DiscussionA rise in RA pressure or difference in flow pattern in the RA can make a PFO become symptomatic. Elevated RA pressure was ruled out. Most anatomical pathologies influencing the flow pattern develop slowly over time. This case shows a presentation of POS after a recent stroke possible due to change in anatomy because of right hemidiaphragm paralysis in combination with the aortic dilatation.
| S-EPMC9290560 | biostudies-literature
Project description:BackgroundScimitar syndrome (SS) comprises of an anomalously draining right pulmonary vein (APV), to the inferior vena cava (IVC), maldevelopment of the right pulmonary artery (RPA), and the right lung, with variable number of aorto-pulmonary collaterals (APC) to the right lung. It can cause pulmonary hypertension if left untreated. Surgical correction is the method of choice. We report a case of variant SS with dual drainage of the APV to the IVC and left atrium (LA) that was addressed with a transcatheter approach.Case summaryA 13-year-old child was evaluated for dyspnoea. Chest x-ray and transthoracic echo (TTE) were suggestive of SS with an additional central atrial septal defect (ASD). Cardiac computed tomography (CT) revealed dual drainage of the APV to the IVC and via a meandering vein to the LA and three APCs. The ASD was closed, and the APCs were coiled. The connection of the APV to the IVC was closed with a device, rerouting the pulmonary vein blood to the LA. The child is doing well on follow-up after 2 years.DiscussionVariant forms of SS are rare. Our case had ASD, multiple APCs, well-developed RPA and right lung and a dual drainage of the APV. This allowed for transcatheter management. Otherwise, surgery is the default choice. Multimodality imaging with TTE, CT, magnetic resonance imaging, and cardiac catheterization will help in diagnosis and anatomical delineation.
| S-EPMC9671289 | biostudies-literature
Project description:IntroductionTeratomas are neoplasms originating from embryonic tissues, characterized by a diverse composition of cells from all three germ layers in varying ratios. Rarely reported in the heart, we present here a rare case of a teratoma in the right atrium of a newborn.Case presentationA 20-h-old newborn was referred for a mild heart murmur. An echocardiogram revealed a 6 × 9 mm mass in the right atrium, attached to the interatrial septum. The mass caused mild tricuspid regurgitation without any significant pressure gradient. Surgery to remove the mass was successful. Pathological examination confirmed the mass as a teratoma. The child was discharged in excellent health and has had normal follow-up exams.DiscussionMature teratomas, a common type of germ cell tumor, are rare in the mediastinum, accounting for 10-15 % of mediastinal masses. These tumors arise from displaced primordial germ cells, which may become malignant. They can metastasize to the heart via various routes, causing symptoms like cough, dyspnea, and chest pain due to compression of mediastinal structures. Severe cases may lead to superior vena cava syndrome or hemoptysis. Tumor location influences clinical presentation, with some posing life-threatening risks.ConclusionEarly diagnosis and intervention were crucial in preventing severe complications. This case emphasizes the importance of vigilant clinical evaluation and timely surgical management for infants presenting with cardiac symptoms.
| S-EPMC11562404 | biostudies-literature
Project description:BackgroundScimitar syndrome is a rare congenital disease characterized by partial or total anomalous pulmonary venous return from the right lung into the systemic venous system, and accounts for 0.5-2% of all congenital heart disease. Severe forms of the disease are diagnosed in childhood. However, because of the benign form of the syndrome in adults, many are asymptomatic, or present only mild symptoms including exertional dyspnoea, arrhythmias, and respiratory infections. We report an atypical presentation with hepatomegaly.Case summaryA 24-year-old woman was evaluated for abdominal discomfort. Physical examination revealed a remarkable hepatomegaly. Chest X-ray revealed dextroversion, enlargement of the right cavities, and a curvilinear opacity known as 'scimitar sign'. A transthoracic echocardiography demonstrated right ventricular dilation and a venous collector draining into right suprahepatic vein, which was severely dilated, with large hepatomegaly. Scimitar syndrome was confirmed by magnetic resonance imaging (MRI). Therefore, the patient underwent surgery, redirecting the pulmonary venous return to left atrium. Three months later, the patient remained asymptomatic and both the hepatomegaly and the right chamber volumes normalized.DiscussionAbdominal discomfort, as in our clinical case, is a highly atypical presentation of scimitar syndrome. It is important for physicians to be aware that diagnostic suspicion can be established from a chest X-ray, on which the scimitar sign can be distinguished in many cases. The diagnosis must be confirmed with other imaging modalities, such as echocardiography, MRI, or computed tomography. Corrective surgery may relieve the symptoms related to liver congestion at follow-up.
| S-EPMC8374964 | biostudies-literature
Project description:Scimitar syndrome is a clinical triad of anomalous pulmonary venous drainage, lung hypoplasia, and anomalous aortic blood supply to the lung segment. When there is dual pulmonary venous drainage both to inferior vena cava and left atrium, it is called scimitar variant. A young child presenting with recurrent chest infections, dextroposition of the heart, and scimitar shadow on chest X-ray was identified to have scimitar variant after a detailed evaluation and managed successfully by catheter interventions. This report discusses the embryogenesis and angiographic findings of scimitar variants, indications for interventions, and issues in its management.
| S-EPMC7727905 | biostudies-literature