Drenaje venoso anómalo. Vena cava superior izquierda persistente que drena en la aurícula derecha y vena cava superior derecha que drena en la aurícula izquierda: reporte de un caso / Anomalous Venous Drainage. Persistent Left Superior Vena Cava Draining into the Right Atrium and Right Superior Vena Cava Draining into the Left Atrium: Case Report

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.

Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.

Isquemia intestinal, una manifestación inusual de la embolia paradójica. Un reporte de caso / Intestinal ischaemia as a rare manifestation of a paradoxical embolism. A case report

Introducción. La embolia paradójica es un evento trombótico originado en la circulación venosa, que se manifiesta como embolismo arterial por medio de un defecto anatómico a nivel cardíaco o pulmonar. Se asocia principalmente a eventos cerebrovasculares, aunque se han encontrado casos de infarto agudo de miocardio, infarto renal y otros eventos isquémicos. Caso clínico. Paciente de 47 años, quien consultó por cuadro de dolor abdominal, que requirió manejo quirúrgico de urgencia, donde identificaron isquemia intestinal importante. Los estudios adicionales hallaron déficit de proteína S y persistencia de foramen oval permeable. Resultados. La presencia de trombosis arterial se conoce como trombosis de sitios inusuales y requiere de estudios para descartar trombofilias asociadas u otros estados protrombóticos. El déficit de proteína S es una trombofilia infrecuente, la cual se asocia en la vida adulta a eventos trombóticos de origen venoso. En presencia de defectos anatómicos, como un foramen oval permeable, puede progresar a embolia arterial, configurando un cuadro de embolismo paradójico. La estratificación de estos pacientes requiere imágenes que demuestran el defecto mencionado, así como el posible origen de los émbolos. El manejo se basa en anticoagulación plena, manejo de soporte, resolver las manifestaciones trombóticas existentes y un cierre temprano del defecto anatómico. Conclusiones. El embolismo paradójico debe sospecharse en caso de trombosis de sitios inusuales. Requiere de un estudio exhaustivo con imágenes y su manejo debe basarse en anticoagulación y cierre del defecto.

Introduction. Paradoxical embolism is a thrombotic event originating in the venous circulation, which manifests as arterial embolism through an anatomical cardiac or pulmonary defect. It is mainly associated with stroke, also presenting as acute myocardial infarction, renal infarction, and other ischemic events. Clinical case. A 47-year-old patient was admitted due to abdominal pain, which required emergency surgical management, finding significant intestinal ischemia. Additional studies found protein S deficiency and evidence of a patent foramen ovale. Discussion. Arterial thrombosis is known as unusual thrombosis; this situation requires to rule out associated thrombophilia or other prothrombotic diseases. Protein S deficiency is a rare thrombophilia, which in adults causes venous thrombosis. In the presence of anatomical defects, such as a patent foramen ovale, it can progress to arterial embolism, presenting a picture of paradoxical embolism. The study work of these patients requires imaging that demonstrates the aforementioned defect, as well as the possible origin of the emboli. Management is based on full anticoagulation, treatment of existing thrombotic manifestations, and management of the anatomical defect. Conclusions. Paradoxical embolism should be suspected in case of unusual thrombosis. It requires exhaustive studies based on imaging, and management should consist of anticoagulation and closure of the defect.

Cardiogenic shock due to pulmonary thromboembolism: the role of surgical thrombectomy.

A 53-year-old male patient with history of repaired hernia, who is admitted for 5 days of progressive dyspnea with diagnosis of abdominal sepsis, a computed tomography angiography was made, revealing pulmonary embolism in bilateral main pulmonary artery, and cardiac thrombectomy was performed.

Paciente masculino de 53 años de edad con historial de reparación de hernia, quien fue ingresado por presentar 5 días de disnea progresiva con diagnóstico de sepsis abdominal, se le realizó una angiografía por tomografía computada, revelando embolismo bilateral arteria pulmonar principal, se realizó una trombectomía cardíaca.

Stroke in Young Military Men With Heterozygous for MTHFR Gene Mutation or Factor V Leiden Gene Mutation Associated With Patent Foramen Ovale: Report of Two Cases and Therapeutic Strategy.

We report two cases of Brazilian patients (a 22-year-old male and a 48-year-old male) with ischemic stroke, whose arterial vascular study and echocardiographic investigation did not reveal any steno-occlusive arterial disease or typical cardioembolic finding, such as atrial fibrillation or myocardial dysfunction. A transcranial Doppler ultrasound and a transesophageal echocardiogram showed a patent foramen ovale (PFO), and the laboratory screening for coagulation abnormalities showed heterozygosity for MTHFR C677T and A1298C in one of the patients and heterozygosity for factor V Leiden gene mutations in the other patient. The significance of the association of PFO with Methylenetetrahydrofolate (MTHFR) C677T and A1298C variants or factor V Leiden mutation is discussed as a possible cause of ischemic stroke through paradoxical embolism from a venous source. There is a high prevalence of these two mentioned conditions in the general population, so we discuss two cases in which indication for anticoagulant therapy or percutaneous closure of PFO prevails.

Interatrial septal aneurysm associated with patent foramen ovale in a dog - case report / Aneurisma de septo interatrial associado a forame oval patente em cão - relato de caso

Atrial septal aneurysm (ASA) is a rare congenital deformity of variable clinical relevance, often reported in association with patent foramen ovale (PFO). Transthoracic Doppler echocardiography (TTE) is the first choice for diagnosis, but complementary techniques, such as microbubble contrast, should be used. Despite its importance, in Veterinary Medicine, there is little information related to the subject, and the present study is the second report of this condition in dogs. The objective was to report a case of ASA type IV LR associated with PFO in a Shar Pei bitch, diagnosed by a combined approach of transthoracic echocardiography backed by agitated saline solution (microbubbles). During clinical care, the owners reported episodes of seizures as their main complaint. Echocardiographic examination revealed a type 4 LR atrial septal aneurysm and left ventricular systolic dysfunction. The use of microbubble contrast showed right-to-left shunt, confirming PFO. The recommended treatment was medication. It was concluded that the rarity of ASA in dogs may be due to incomplete diagnoses and the transthoracic echocardiographic examination allows its analysis and classification. Furthermore, ASA can be found in animals with or without evidence of heart disease and it should be investigated in patients with neurological alterations.

O aneurisma do septo atrial (ASA) é uma rara deformidade congênita, de relevância clínica variável, frequentemente relatado em associação a forame oval patente (PFO). O ecodopplercardiograma transtorácico (TTE) é a primeira escolha para o diagnóstico, mas técnicas complementares, como o contraste por microbolhas, devem ser utilizadas. Apesar da importância, em medicina veterinária existe uma carência de informações relacionadas ao tema, sendo esse o segundo relato dessa condição em cão. Objetivou-se relatar um caso de ASA tipo IV LR, associado a PFO, em uma cadela Shar Pei, diagnosticado por uma abordagem combinada por ecodopplercardiograma transtorácico e costrastado com solução salina agitada (microbolhas). Ao atendimento clínico, os proprietários relataram, como queixa principal, episódios de crises convulsivas. O exame ecocardiográfico revelou aneurisma de septo atrial tipo 4 LR e disfunção sistólica em ventriculo esquerdo. A utilização de contraste por microbolhas constatou shunt direita-esquerda, confirmando PFO. O tratamento recomendado foi medicamentoso. Concluiu-se que a raridade do ASA em cães pode ser devido a diagnósticos incompletos, e o exame ecocardiográfico transtorácico permite sua análise e classificação. Ainda, ASA pode ser encontrado em animais com ou sem evidências de cardiopatia e sua investigação deve ser feita em pacientes com alterações neurológicas.

Transient ischemic attack + acute myocardial infarction + pulmonary embolism - three comcomitant embolic events in a patient with patent foramen ovale

INTRODUCTION: Patent foramen ovale (PFO) is the persistence of the opening between the atrial septum primum and atrial septum secundum at the location of the fossa ovalis. It is present in about 25% of adults and is a casual finding without hemodynamic repercussions. However, studies prove an association between PFO and potentially severe clinical conditions, such as ischemic stroke, pulmonary embolism (PE), and, more rarely, acute myocardial infarction (MI). Despite welldocumented consequences of paradoxical embolism through the PFO, the passage of venous clot from a patent foramen has been rarely described in the literature. We report a case of a patient with PFO and manifestation of three concomitant embolic events: venous thromboembolism, transient ischemic attack, and MI. CASE DESCRIPTION: A 49-year-old woman with a history of idiopathic pulmonary arterial hypertension, asthma, PFO, and two previous episodes of PE sought emergency care with dyspnea on mild exertion for 2 weeks and oxygen desaturation on room air. She was admitted for compensation and clinical investigation. Chest CT angiography revealed dilatation of the pulmonary trunk and filling defects in the segmental and subsegmental arteries bilaterally. Full anticoagulation was started with enoxaparin. On the fourth day of hospitalization, the patient presented sudden burning chest pain, frontal headache, decreased consciousness, and tachypnea. ECG showed ST-segment elevation inferior MI and complete heart block, which spontaneously reverted to sinus rhythm. Patient also developed sudden left-sided hemiparesis and ipsilateral hemineglect. Non-contrast brain CT ruled out hemorrhage and aortic angiotomography excluded dissection. Due to the reported PFO, an embolic etiology was hypothesized and it was decided not to perform a coronary cineangiography and also to suspend antiplatelet drugs. The patient had complete reversal of the deficits within hours and no changes were noted in a subsequent brain CT. Cardiac magnetic resonance imaging confirmed inferior acute MI, and echocardiogram revealed PFO with small right-to-left shunt. After 18 days, the patient was discharged under oral anticoagulation and outpatient follow-up revealed no further complications. CONCLUSION: We report a case of paradoxical embolism manifested in distinct sites in a young patient with PFO, with good outcomes with anticoagulant therapy.

Mechanisms of platypnea-orthodeoxia syndrome. / Mecanismos del síndrome de platipnea-ortodesoxia

Platypnea orthodeoxia syndrome (POS) is a clinical entity described in the middle of the last century. It is characterized by dyspnea and hypoxemia triggered by standing and relieved with recumbency. The diagnosis is predominately clinical. The degree of hypoxemia is variable; however, the diagnostic criteria include the decrease in arterial oxygen pressure more than 4 mmHg or oxygen saturation more than 5%. Even though many diseases cause this syndrome, there are only two responsible mechanisms, intracardiac, and intrapulmonary shunts. The coexistence of diverse structural and physiological abnormalities joined to gravitational forces that induce blood shunt after standing is crucial in each mechanism. The intracardiac mechanism is characterized by right to left blood shunt through atrial septal communications and, the right atrium pressure could be normal or increased. In addition, some patients have one or more coexistent aortic, spinal, or intracardiac alterations. The intrapulmonary mechanism is less frequent and is caused by parenchymal or vascular pathologies. Transthoracic echocardiogram is the first diagnostic modality; however, understanding the pathophysiology is the key for a rational diagnostic approach and subsequent diagnostic studies. Treatment is possible and effective in the majority of intracardiac mechanisms and some intrapulmonary. This review focuses on the pathophysiologic mechanisms of POS and their diagnostic workup.

El síndrome de platipnea ortodesoxia es una entidad clínica descrita a mediados del siglo pasado. Se caracteriza por disnea e hipoxemia que se desencadenan con la bipedestación y se alivia con el decúbito. El diagnóstico es predominantemente clínico. El grado de hipoxemia es variable; sin embargo, los criterios de diagnóstico incluyen disminución de la presión arterial de oxígeno de más de 4 mmHg o saturación de oxígeno de más de 5%. A pesar de que este síndrome es causado por gran cantidad de enfermedades, solo hay dos mecanismos responsables: los cortocircuitos intracardiacos e intrapulmonares. En cada mecanismo es crucial la coexistencia de diversas anomalías estructurales y fisiológicas que, unidas a las fuerzas gravitacionales, inducen un cortocircuito sanguíneo después de la bipedestación. En el mecanismo intracardiaco hay un cortocircuito sanguíneo de derecha a izquierda a través del del tabique interauricular y la presión auricular derecha puede ser normal o aumentada; además, algunos pacientes tienen una o más alteraciones aórticas, espinales, o intracardiacas coexistentes. El mecanismo intrapulmonar es menos frecuente y es causado por patologías parenquimatosas o vasculares. El ecocardiograma transtorácico es la primera modalidad de diagnóstico, sin embargo, comprender la fisiopatología es la clave para un enfoque de diagnóstico racional y estudios diagnósticos subsecuentes. El tratamiento es posible y eficaz en la gran mayoría de los mecanismos intracardiacos y en algunos intrapulmonares. Esta revisión se centra en los mecanismos fisiopatológicos del síndrome de platipnea ortodesoxia y su diagnóstico.

[Persistent foramen ovale as a cause of persistent hypoxemia after a pneumonia caused with COVID-19. Report of one case]. / Foramen oval persistente como causa de hipoxemia persistente en un paciente con neumonía grave COVID-19. Reporte de un caso.

In patients with severe COVID-19, it has been proposed as mechanism of respiratory failure, intra and extrapulmonary shunt. However, there are no reported or documented cases of this mechanism. Also, there are studies showing this mechanism is not relevant. In this report, we present the case of a patient with severe COVID-19 pneumonia, who after two months, during his rehabilitation period, presented persistent hypoxemia with orthodeoxia. Persistent oval foramen with shunt from right to left was diagnosed. The patient underwent a percutaneous closure of the defect and at 48 hours oxygen therapy could be discontinued and the patient discharged.

Comparison of right-to-left shunt characteristics in cryptogenic embolic ischemic stroke and non-cardioembolic ischemic stroke.

BACKGROUND: Patent foramen ovale (PFO) has been considered a potential mechanism of embolic stroke of undetermined origin. OBJECTIVE: The aim of the present study was to identify the features of the right-to-left shunt (RLS) in patients with undetermined embolic ischemic stroke and compare them with those of patients with non-cardioembolic ischemic stroke. METHODS: A retrospective study was conducted with 168 patients with stroke and RLS separated into the following two groups: the undetermined embolic stroke group (UES group) and non-cardioembolic stroke group (NCES group). All patients were assessed by transcranial Doppler to evaluate the presence and quantification of microembolic signals (MES) at rest and under Valsalva maneuver. RESULTS: Of all patients evaluated in the current study, 96 were included in the UES group and 72 in the NCES group. In the UES group, 65 patients had RLS with ≥10 MES (67.7%), which was higher than that observed in the NCES group (51.4%, p=0.038). According to the moment of the cardiac cycle, 75 patients (78.1%) in the UES group had a positive test at rest compared to 42 (58.3%) in the NCES group (p=0.007). CONCLUSIONS: The current study demonstrated that almost 70% of patients with undetermined embolic stroke and PFO presented a large RLS and more than 75% had RLS at rest. These findings suggest that the size of the shunt should be taken into account when evaluating whether PFO could be a possible mechanism underlying cryptogenic stroke.

Comparison of right-to-left shunt characteristics in cryptogenic embolic ischemic stroke and non-cardioembolic ischemic stroke / Comparação das características do shunt direita-esquerda em pacientes com AVC isquêmico criptogênio e AVC não cardioembólico

Abstract Background: Patent foramen ovale (PFO) has been considered a potential mechanism of embolic stroke of undetermined origin. Objective: The aim of the present study was to identify the features of the right-to-left shunt (RLS) in patients with undetermined embolic ischemic stroke and compare them with those of patients with non-cardioembolic ischemic stroke. Methods: A retrospective study was conducted with 168 patients with stroke and RLS separated into the following two groups: the undetermined embolic stroke group (UES group) and non-cardioembolic stroke group (NCES group). All patients were assessed by transcranial Doppler to evaluate the presence and quantification of microembolic signals (MES) at rest and under Valsalva maneuver. Results: Of all patients evaluated in the current study, 96 were included in the UES group and 72 in the NCES group. In the UES group, 65 patients had RLS with ≥10 MES (67.7%), which was higher than that observed in the NCES group (51.4%, p=0.038). According to the moment of the cardiac cycle, 75 patients (78.1%) in the UES group had a positive test at rest compared to 42 (58.3%) in the NCES group (p=0.007). Conclusions: The current study demonstrated that almost 70% of patients with undetermined embolic stroke and PFO presented a large RLS and more than 75% had RLS at rest. These findings suggest that the size of the shunt should be taken into account when evaluating whether PFO could be a possible mechanism underlying cryptogenic stroke.

RESUMO Antecedentes: Uma das potenciais fontes embólicas no acidente vascular cerebral (AVC) de origem indeterminada é o forame oval patente (FOP). Objetivo: O objetivo do presente estudo foi identificar as características do shunt direita-esquerda em paciente com AVC de etiologia indeterminada, presumidamente embólica, e comparar tais características com pacientes apresentando AVC por outras causas não embólicas. Métodos: Trata-se de um estudo retrospectivo com 168 pacientes com AVC e forame oval patente, separados em dois grupos: AVC embólico de etiologia indeterminada e AVC por outras causas não embólicas. Todos os pacientes foram submetidos a Doppler transcraniano, para avaliar a presença de shunt direita-esquerda por meio do teste de embolia paradoxal. Além da quantificação de microbolhas, também foi avaliada a presença de shunt em repouso e sob manobra de Valsalva. Resultado: Do total, 96 pacientes foram incluídos no primeiro grupo (AVC indeterminado) e 72, no segundo grupo (AVC não embólico). No primeiro grupo, 65 pacientes exibiram shunt com passagem de mais de 10 microbolhas (67,5%), enquanto no segundo grupo isso aconteceu em 51,4% (p=0,038) dos casos. Além disso, 75 pacientes (78,1%) do primeiro grupo tiveram teste positivo ao repouso, comparados com 42 pacientes (58,3%) no segundo grupo (p=0,007). Conclusão: O presente estudo demonstrou que até 70% dos pacientes com AVC de etiologia indeterminada e forame oval apresentaram shunts maiores; em mais de 75%, houve passagem de microbolhas ao repouso. Esses achados sugerem que as características do shunt, como quantidade de microbolhas e passagem ao repouso, devem ser levadas em consideração na avaliação do FOP como possível mecanismo subjacente ao AVC.

Central retinal artery occlusion associated with patent foramen ovale: a case report and literature review.

Patent foramen ovale might cause cryptogenic strokes, including retinal artery occlusion. Herein, we describe a previously healthy young man who presented with central retinal artery occlusion in the setting of patent foramen ovale and explore the need for transesophageal echocardiogram for its diagnosis. Cardiovascular workup and neuroimaging were unremarkable. Transthoracic echocardiogram bubble study revealed a right to left atrial shunt and subsequent transesophageal echocardiogram disclosed patent foramen ovale. This congenital cardiac anomaly was the likely conduit for a thrombo-embolic central retinal artery occlusion. We identified seven patients with patent foramen ovale associated with central retinal artery occlusion in the literature. Transthoracic echocardiogram was diagnostic in only one patient (14.3%), whereas transesophageal echocardiogram was required to reveal patent foramen ovale in the remaining six (85.7%). Our case and the previous reports support the link between central retinal artery occlusion and patent foramen ovale. Therefore, providers should consider the more sensitive transesophageal echocardiogram during the initial evaluation of young patients without immediately identifiable causes of retinal artery occlusion.