Scimitar Syndrome: An Unexpected Etiology of Right-Sided Heart Failure and Cardiac Cirrhosis in an Elderly Patient

Pivato, Erica; Arbune, Amit; Rovner, Aleksandr; Murad, Khalil

doi:10.1016/s0735-1097(18)33043-2

Cited by 2 publications

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“…It is classified into three groups according to age and severity of symptoms 4 . Group I, called "isolated adult," constitutes the form in adults, they are typically acyanotic, asymptomatic; the diagnosis is generally made as an incidental finding or by the development of symptoms years or decades after birth in association with heart failure or cirrhosis 20 ; they may present with a small atrial septal defect and do not develop pulmonary arterial hypertension 12,14,21 . Group II is characterized by its association with complex CHD, within which atrial septal defect and patent ductus arteriosus are excluded, it is known as "childhood CHD"; it is diagnosed in the neonatal period and presents with respiratory failure; it is commonly associated with PHT and due to its severity it frequently requires surgical treatment 22,23 .…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

Scimitar syndrome with late presentation

Henao¹,

Sánchez-Duque²,

Erazo-Criollo³

2022

RCCAR

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Introduction: Scimitar syndrome is a congenital malformation with an incidence of 1-3 per 100,000 live births, consisting of abnormal drainage of the right pulmonary veins. The age of diagnosis varies according to the severity of the pathology, which depends on the degree of cardiac and pulmonary malformation. The case of a patient with a late diagnosis of the syndrome due to a silent course is reported despite severe anatomical alterations. Clinical Case: A 76-year-old female was admitted due to a 6-month clinical picture of progressive dyspnea to minimal efforts. A transthoracic echocardiogram was performed showing severe pulmonary hypertension of 96 mmHg with abnormal communication between the right pulmonary veins and the inferior vena cava, confirmed by CT angiography. Discussion: The late clinical course in relation to severe anatomical malformations has not been described in the scientific literature due to its high mortality in childhood.

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Section: Discussion and Review Of The Literaturementioning

confidence: 99%

Scimitar syndrome with late presentation

Henao¹,

Sánchez-Duque²,

Erazo-Criollo³

2022

RCCAR

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“…El síndrome de la cimitarra puede permanecer sin diagnosticar hasta la edad adulta tardía y presentarse con hipertensión pulmonar e insuficiencia del VD. La evaluación cuidadosa de las estructuras cardiopulmonares y la presencia de un cortocircuito de izquierda a derecha debe ser parte de la evaluación de todos los pacientes adultos diagnosticados de HAP y falla del VD 11 .…”

Section: Discussionunclassified

Síndrome de Cimitarra: reporte de caso

Chinchilla-Chinchilla

Vallecillo-Torres

Mendoza-Solís

2021

Rev Hisp Cienc Salud

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El presente caso clínico es sobre un masculino de 5 meses de edad, nacido a término, con peso y talla adecuados para edad gestacional, APGAR 9/9, no sufrimiento, no reanimación, sin antecedente patológicos ni quirúrgicos conocidos. Consulta el servicio de emergencias del Hospital Nacional de Niños tras una semana de sintomatología respiratoria, con francos datos clínicos de dificultad respiratoria: retracciones intercostales, taquipnea, aleteo nasal. Requirió al ingreso uso de cánula de alto flujo de oxígeno para estabilizar la dificultad respiratoria; además de respuesta negativa a pruebas terapéuticas con salbutamol. La radiografía de tórax, entre otros hallazgos se documenta un leve borramiento de la silueta cardiaca y dextrocardia; en el ultrasonido se logra documentar la presencia de derrame pleural, además de datos de atelectasia derecha observados en 2 broncograma; como parte del plan inicial de manejo se decide internar al paciente para manejo hospitalario. Durante internamiento se confirma la dextrocardia por medio de ecocardiograma, además de hipoplasia de vasos pulmonares derechos, se realiza tomografía axial computarizada de vasos cardiacos la describe como resultado: hipoplasia pulmonar derecha, drenaje venoso anómalo de pulmón derecho a la vena cava inferior, secuestro pulmonar intralobar derecho, hipoplasia de la arteria pulmonar derecha, permitiendo así documentar la presencia de un Síndrome de Chimitarra. Se da manejo por parte del servicio de cardiología, donde se confirman los hallazgos y se reorganiza la lista de problemas, de esta forma se establece como problema uno al diagnóstico de Síndrome de Chimitarra. El caso es presentado en sesión clínica para definir manejo quirúrgico del mismo.

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Scimitar Syndrome: An Unexpected Etiology of Right-Sided Heart Failure and Cardiac Cirrhosis in an Elderly Patient

Cited by 2 publications

References 0 publications

Scimitar syndrome with late presentation

Scimitar syndrome with late presentation

Síndrome de Cimitarra: reporte de caso

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