Lobectomy with ECMO Support in an Infant Who Developed Pulmonary Interstitial Emphysema Following Repair of Hypoplastic Aortic Arch.

Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Tetralogia de Fallot em cão - relato de caso / Tetralogy of Fallot in a dog - case report / Tetralogía de Fallot en perro - relato de caso

La tetralogía de Fallot es una enfermedad congénita rara, caracterizada por estenosis de la válvula pulmonar, hipertrofia ventricular derecha, comunicación interventricular y dextroposición de la arteria aorta. La fisiopatología y las características clínicas con que se presenta dependen principalmente del grado de obstrucción de la arteria pulmonar. Desde el punto de vista clínico, los animales presentan cianosis, resistencia al ejercicio, disnea y síncopes. El diagnóstico definitivo y el pronóstico se obtienen mediante ecocardiografía (Doppler). Si bien la cirugía es el único método efectivo de tratamiento, en casos menos graves, existen medicamentos que actúan de manera paliativa. Este trabajo relata el caso de un perro mestizo de once meses con diagnóstico de tetralogía de Fallot, haciendo énfasis en la ecocardiografia (Doppler) como método de diagnóstico definitivo.

Tetralogy of Fallot is a rare congenital disease consisting of pulmonary valve stenosis, right ventricular hypertrophy, ventricular septal defect and aortic dextroposition. Pathophysiological consequences and clinical presentation depend mainly on the degree of pulmonary artery obstruction. Symptoms include cyanosis, exercise intolerance, dyspnea, and syncope. Definitive diagnosis and prognosis are obtained by Doppler echocardiography. Surgery is the only effective method of treatment, however, pharmacotherapy may be indicated as palliative treatment in mild cases of the disease. This work reports on the case of an eleven-month-old mongrel, diagnosed with tetralogy of Fallot and highlights the importance of Doppler echocardiography as the definitive diagnostic tool for this disease.

A tetralogia de Fallot é urna rara doença congênita, definida por estenose da valva pulmonar, hipertrofia ventricular direita, defeito do septo interventricular e dextroposição da aorta. Suas consequências fisiopatológicas, assim como a apresentação clínica, dependem principalmente do grau de obstrução da artéria pulmonar. Clinicamente, os animais apresentam cianose, intolerância ao exercício, dispneia e síncopes. O diagnóstico definitivo e o prognóstico são obtidos por meio da ecodopplercardiografia. A cirurgia é o único método efetivo para o tratamento; no entanto, em casos brandos, a terapia medicamentosa pode ser indicada como forma paliativa. O presente trabalho relata o caso de um cão de onze meses de idade, sem raça definida, diagnosticado com tetralogia de Fallot, enfatizando a ecoclopplercardiografia como método diagnóstico definitivo da doença.

Tetralogia de Fallot em cão - relato de caso / Tetralogy of Fallot in a dog - case report / Tetralogía de Fallot en perro - relato de caso

La tetralogía de Fallot es una enfermedad congénita rara, caracterizada por estenosis de la válvula pulmonar, hipertrofia ventricular derecha, comunicación interventricular y dextroposición de la arteria aorta. La fisiopatología y las características clínicas con que se presenta dependen principalmente del grado de obstrucción de la arteria pulmonar. Desde el punto de vista clínico, los animales presentan cianosis, resistencia al ejercicio, disnea y síncopes. El diagnóstico definitivo y el pronóstico se obtienen mediante ecocardiografía (Doppler). Si bien la cirugía es el único método efectivo de tratamiento, en casos menos graves, existen medicamentos que actúan de manera paliativa. Este trabajo relata el caso de un perro mestizo de once meses con diagnóstico de tetralogía de Fallot, haciendo énfasis en la ecocardiografia (Doppler) como método de diagnóstico definitivo.(AU)

Tetralogy of Fallot is a rare congenital disease consisting of pulmonary valve stenosis, right ventricular hypertrophy, ventricular septal defect and aortic dextroposition. Pathophysiological consequences and clinical presentation depend mainly on the degree of pulmonary artery obstruction. Symptoms include cyanosis, exercise intolerance, dyspnea, and syncope. Definitive diagnosis and prognosis are obtained by Doppler echocardiography. Surgery is the only effective method of treatment, however, pharmacotherapy may be indicated as palliative treatment in mild cases of the disease. This work reports on the case of an eleven-month-old mongrel, diagnosed with tetralogy of Fallot and highlights the importance of Doppler echocardiography as the definitive diagnostic tool for this disease.(AU)

A tetralogia de Fallot é urna rara doença congênita, definida por estenose da valva pulmonar, hipertrofia ventricular direita, defeito do septo interventricular e dextroposição da aorta. Suas consequências fisiopatológicas, assim como a apresentação clínica, dependem principalmente do grau de obstrução da artéria pulmonar. Clinicamente, os animais apresentam cianose, intolerância ao exercício, dispneia e síncopes. O diagnóstico definitivo e o prognóstico são obtidos por meio da ecodopplercardiografia. A cirurgia é o único método efetivo para o tratamento; no entanto, em casos brandos, a terapia medicamentosa pode ser indicada como forma paliativa. O presente trabalho relata o caso de um cão de onze meses de idade, sem raça definida, diagnosticado com tetralogia de Fallot, enfatizando a ecoclopplercardiografia como método diagnóstico definitivo da doença.(AU)

Covered-stent implantation in coarctation of the aorta: indications,materials, techniques and outcomes

Treatment strategies for coarctation of the aorta (CoA) include surgical repair, balloon angioplasty andstent implantation. Balloon angioplasty may be associated with complications such as recoarctation,dissection or aneurysm formation. Bare metal stent implantation prevents elastic recoil of the aorta andmay provide better and more predictable results than balloon angioplasty, and over the last decade, thishas often been the primary intervention for treating CoA. However, this effective option has not completelysolved the problem, since complications of aortic wall injury, either acutely or during follow-up, have beenencountered. Recently, covered-stent implantation has gained popularity as an alternative for patientswith native or recurrent CoA. Furthermore, these stents have played an important role in treating patientswith complications due to aortic wall injuries after surgical or transcatheter repair. Indications for coveredstentimplantation, a description of materials available, preferred implantation techniques and acute andlong-term outcomes are described in this article.

Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography.

State-of-the-art multidetector computed tomographic (CT) technology has replaced invasive angiography for evaluation of patients suspected to have aortic disease. Although most aortic disease is associated with atherosclerosis (ie, aneurysms and dissection), the spectrum of aortic disease is vast and includes various congenital and acquired entities. Radiologists should also be familiar with uncommon aortic diseases, which are divided into those that are congenital in origin and acquired disorders, and with their findings at multidetector CT. The first group includes patent ductus arteriosus, aortic hypoplasia, aortic coarctation, interrupted aortic arch, aortopulmonary window, common arterial trunk, supravalvular aortic stenosis, and vascular rings. The acquired disorders include aortic dissection due to extension of a coronary artery dissection, Marfan syndrome, large-vessel vasculitis such as Takayasu arteritis, and mycotic aneurysms. Finally, specific conditions associated with therapeutic maneuvers--such as recoarctation, stent-graft rupture, and endoleaks--can also be assessed with multidetector CT. Multidetector CT is an alternative tool helpful in establishing the primary diagnosis, defining anatomic landmarks and their relationships, and identifying associated cardiovascular anomalies. It is also an adjunct in the evaluation of complications during follow-up.

[Left aortic arch without a circumflex segment and a right descending aorta: a hypothetical case and a real example]. / Arco aórtico izquierdo sin segmento circunflejo y aorta descendente derecha: de un caso hipotético a un ejemplo real.

We studied a case of a patent left ductus arteriosus, with a left aortic arch connected to a right descending aorta without an aortic circumflex (retroesophageal) segment. We believe this is the first world-published example of this--so far--hypothetical anomaly, included in Kirkling and Edwards classification of vascular rings. The case was a 27-year-old deaf-mute female patient with a patent left ductus arteriosus, with important left to right shunt and moderate pulmonary arterial hypertension who underwent a successful closing surgical procedure. In the aortographic study a left (normal) aortic arch was seen, which after giving off a large left ductus arteriosus, crossed to the right and connected to a right descending aorta. In the upper part of the thorax the esophagus and trachea were displaced to the right by the left aortic arch. The esophagus in the middle and the upper inferior parts of the thorax was also displaced to the right by the descending right aortic segment. Complete vascular ring was not evident. The case--although without clinical importance--reinforces the pathogenic concept of the Rathke diagram of a complete double aortic primitive arch originating the pulmonary and supraaortic vascular structures which are formed by the obliteration or disappearance of particular segments of the structure. This knowledge explains the embryogenesis of these complex anomalies. Our case is believed to be formed by the very early disappearance of a frontal portion of the primitive left dorsal aorta.