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1.
Rev. urug. cardiol ; 38(1): e203, 2023. tab
Article in Spanish | LILACS, UY-BNMED, BNUY | ID: biblio-1522877

ABSTRACT

La coartación de aorta (CoAo) es una cardiopatía congénita caracterizada por un estrechamiento de la aorta descendente distal al origen de la arteria subclavia izquierda, lo que determina una obstrucción al flujo sanguíneo. Es imperativo realizar un diagnóstico precoz y tratamiento oportuno para evitar complicaciones y la muerte en algunos casos. Se describen las características clínicas y anatómicas, así como el tratamiento y la evolución en niños menores de 15 años asistidos en un hospital pediátrico de referencia de Uruguay.


Coarctation of the aorta (CoAo) is a congenital heart disease characterized by a narrowing of the descending aorta distal to the origin of the left subclavian artery, which determines an obstruction to blood flow. It is imperative to make an early diagnosis and timely treatment to avoid complications and death in some cases. The clinical and anatomical characteristics are described, as well as the treatment and evolution in children under 15 years of age attended in a reference pediatric hospital in Uruguay.


A coarctação da aorta (CoAo) é uma doença cardíaca congênita caracterizada por estreitamento da aorta descendente distal à origem da artéria subclávia esquerda, o que determina uma obstrução ao fluxo sanguíneo. É imperativo fazer um diagnóstico precoce e tratamento oportuno para evitar complicações e morte em alguns casos. São descritas as características clínicas e anatômicas, bem como o tratamento e a evolução em crianças menores de 15 anos atendidas em um hospital pediátrico de referência no Uruguai.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Aortic Coarctation/diagnosis , Aortic Coarctation/surgery , Aortic Coarctation/mortality , Retrospective Studies , Age and Sex Distribution
2.
Arch. argent. pediatr ; 120(1): e25-e28, feb 2022. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1353751

ABSTRACT

La enfermedad de Tay-Sachs es una enfermedad metabólica hereditaria neurodegenerativa. Existen cuatro tipos según el inicio de los síntomas clínicos: infantil, infantil de inicio tardío, juvenil y adulto. El tipo infantil tiene el peor pronóstico. Recientemente, se describieron diferentes anomalías que acompañan a los trastornos metabólicos e influyen en el pronóstico. Presentamos el caso de un lactante con enfermedad de Tay-Sachs junto con coartación aórtica y reflujo vesicoureteral bilateral (RVU) de grado V. Se realizó el seguimiento del paciente en el consultorio externo de Cardiología Pediátrica. En la ecografía abdominal, se observó ectasia pielocalicial, y se detectó reflujo vesicoureteral bilateral de grado V en la cistouretrografía miccional. No se ha informado previamente la coexistencia de estas anomalías. Este caso pone de manifiesto que no se deben subestimar las anomalías del examen neurológico en los pacientes con una cirugía cardíaca reciente, porque podría perderse la oportunidad de diagnosticar enzimopatías congénitas.


Tay-Sachs disease is a neurodegenerative inherited metabolic disease. There are four forms classified by the time of first clinical symptoms: infantile, late infantile, juvenile and adult. Infantile form has the poorest prognosis. Lately, different abnormalities which accompany metabolic disorders and affect the prognosis have been described. We present an infant with Tay-Sachs disease accompanied by coarctation of the aorta and bilateral grade V vesicoureteral reflux (VUR). The patient was followed up in the outpatient clinic of Pediatric Cardiology. The abdominal ultrasonography showed pelvicalyceal ectasia; bilateral grade V VUR in voiding cystourethrography was found. This coexistence has not been previously reported. This case emphasizes that abnormalities in the neurological examination of cardiac postsurgical patients should not be underestimated because the opportunity to diagnose inborn errors of metabolism could be missed.


Subject(s)
Humans , Male , Infant , Aortic Coarctation/complications , Aortic Coarctation/diagnosis , Tay-Sachs Disease/diagnosis , Vesico-Ureteral Reflux/complications , Vesico-Ureteral Reflux/diagnosis
3.
J. vasc. bras ; 21: e20200175, 2022. graf
Article in Portuguese | LILACS | ID: biblio-1356456

ABSTRACT

Resumo Coarctação da aorta abdominal é uma causa rara de claudicação de membros inferiores e hipertensão refratária. O tratamento é complexo e exige conhecimento de diversas técnicas de reconstrução vascular. Apresentamos um caso de coarctação ao nível das artérias renais, seu tratamento e revisão da literatura. Paciente feminina, 65 anos, com hipertensão refratária desde os 35 anos, utilizando cinco medicações anti-hipertensivas em dose máxima. Pressão arterial média de 260/180mmHg e claudicação incapacitante (menos de 20 metros) bilateral. Angiotomografia computadorizada demonstrou coarctação de aorta justarrenal de 4 mm de maior diâmetro, calcificação circunferencial no local da estenose e tortuosidade da aorta infrarrenal. Foi submetida a tratamento híbrido, com ponte ilíaco-birrenal e implante de stent Advanta V12 no local da estenose. A paciente evoluiu satisfatoriamente e, 60 dias depois da cirurgia, apresentava-se com uma pressão arterial de 140/80mmHg, em uso de apenas duas medicações anti-hipertensivas e sem claudicação.


Abstract Coarctation of the abdominal aorta is a rare etiology of intermittent claudication and refractory hypertension. Treatment is complex and requires knowledge of several vascular reconstruction techniques. We report a case of aortic coarctation at the level of the renal arteries, describing its treatment and presenting a literature review. Female patient, 65 years old, with refractory hypertension since the age of 35, using five antihypertensive medications at maximum doses. Blood pressure was 260/180mmHg and she had disabling claudication (less than 20 meters). Computed tomography angiography showed a 4mm coarctation in the juxtarenal aorta, with circumferential calcification at the stenosis site, and tortuous infrarenal aorta. Hybrid repair was performed with an iliac-birenal bypass and implantation of an Advanta V12 stent at the stenosis site. The patient's postoperative course was satisfactory, she was free from claudication, and her blood pressure 60 days after surgery was 140/80mmHg, taking two antihypertensive medications.


Subject(s)
Humans , Female , Aged , Aortic Coarctation/surgery , Aortic Coarctation/complications , Aorta, Abdominal , Aortic Coarctation/diagnosis , Renal Artery , Stents , Angioplasty, Balloon , Hypertension, Renovascular/surgery , Hypertension, Renovascular/etiology , Intermittent Claudication/surgery , Intermittent Claudication/etiology
5.
Med. leg. Costa Rica ; 37(1): 87-92, ene.-mar. 2020.
Article in Spanish | LILACS | ID: biblio-1098375

ABSTRACT

Resumen La coartación de aorta es una cardiopatía congénita con altas tasas de morbilidad y mortalidad, que usualmente es subdiagnosticada a pesar de la disponibilidad de herramientas diagnósticas. El grado de severidad de las manifestaciones clínicas de la coartación de aorta va a depender del grado de obstrucción, así como de la presencia de defectos cardiacos y lesiones extracardíacas asociados. En la población pediátrica la modalidad terapéutica mayormente utilizada es la reparación quirúrgica; mientras que la angioplastia con balón y la colocación de una endoprótesis son menos utilizadas en esta población, ya que asocian mayor riesgo de reestenosis con la consecuente reintervención, estas técnicas son principalmente utilizadas en pacientes mayores. A pesar del éxito en la reparación de la coartación de aorta, los pacientes deben continuar un seguimiento estrecho a largo plazo, que incluye mediciones de la presión arterial de manera periódica, así como estudios por imagen de la estructura cardíaca, debido a la aparición tardía de complicaciones cardiovasculares asociadas.


Abstract Coarctation of the aorta is a congenital heart disease with high rates of morbidity and mortality, which is usually underdiagnosed despite the availability of diagnostic tests. The degree of severity of the clinical manifestations of coarctation of the aorta will depend on the level of obstruction, as well as the presence of cardiac defects and associated extracardiac lesions. In the pediatric population the most used therapeutic modality is surgical correction; while balloon angioplasty and stent placement are less used in this population, since they are associated with a greater risk of restenosis with the subsequent reintervention; these techniques are mostly used in older patients. Despite the success in the repair of coarctation of the aorta, patients should continue a close long-term follow-up, which includes periodic blood pressure measurements, as well as imaging studies that assess the cardiac structure, due to the late onset of associated cardiovascular complications.


Subject(s)
Aortic Coarctation/diagnosis , Heart Defects, Congenital/complications , Hypertension/complications
6.
Int. j. cardiovasc. sci. (Impr.) ; 33(1): 3-11, Jan.-Feb. 2020. tab, graf
Article in English | LILACS | ID: biblio-1090639

ABSTRACT

Abstract Background: Coarctation of the aorta is a congenital segmental narrowing of the aortic arch with severe hemodynamic repercussions and increased cardiovascular mortality. Early surgical correction and life-time echocardiographic follow-up must be performed to improve prognosis. However, this goal has been challenged by high rates of underdiagnosis, which delay surgical correction, and by recoarctation in up to one third of operated patients. Objectives: The objectives of this study were: (i) to register the frequency of common clinical signs at diagnosis of coarctation of the aorta; (ii) to describe the course of echocardiographic parameters before and during the follow-up of coartectomized subjects; (iii) to analyze the clinical prognosis of patients according to baseline characteristics, occurrence of recoarctation and associated malformations. Methods: Case-series of 72 patients coarctectomized between June 1996 and November 2016 in a tertiary care hospital. Clinical, echocardiographic and surgical variables were considered. All patients were submitted to coarctectomy by posterolateral thoracotomy and end-to-end anastomosis. Data were classified as parametric or non-parametric by Kolmogorov-Smirnov test. Parametric data were expressed as mean and standard deviation, and non-parametric data as median and interquartile range. Continuous variables were analyzed using paired t-tests, and categorical variables were compared by chi-square test. For all analysis, a p-value of less than 0.05 was considered statistically significant. Statistical analysis was performed using SPSS, version 20.0 (IBM, Chicago, IL, USA). Results: The mean follow-up time was 5.8 years (range: 0-20 years). At diagnosis, most patients had heart murmur (88%), non-palpable pulse in the lower limbs (50%), left ventricular hypertrophy (78%), and bicuspid aortic valve (33%), with a mean aortic peak gradient of 55 mmHg. After surgical correction, those without recoarctation were less symptomatic (60 vs 4.5%; p < 0.001), had lower aortic peak gradient (54 ± 3.8 vs 13 ± 0.8; p = 0.01) and left ventricle mass (95 ± 9.2 vs. 63 ± 11; p = 0.01), and the most common complications were late hypertension (39.2%), and recoarctation (27.6%). Recoarcted patients did not show improvement of neither clinical nor echocardiographic variables. Age at repair and bicuspid aortic valve groups had comparable results with controls. Surgical procedure was safe; mean time of hospitalization was 10 days and mean surgery time 2.3 hours. Conclusions: Coarctectomy improves cardiac symptoms and left ventricular hypertrophy, with a slight effect on the incidence of hypertension. Recoarctation occurs in one-third of patients and draws attention for the need of lifelong surveillance by echocardiography.


Subject(s)
Humans , Male , Female , Aortic Coarctation/surgery , Aortic Coarctation/diagnosis , Prognosis , Echocardiography/methods , Bicuspid Aortic Valve Disease , Hypertension
9.
Rev. Assoc. Med. Bras. (1992) ; 65(2): 240-245, Feb. 2019. graf
Article in English | LILACS | ID: biblio-990336

ABSTRACT

SUMMARY INTRODUCTION: Coarctation of the aorta is a congenital heart disease characterized by a narrowing that occurs in the aortic artery. This constriction can occur anywhere along its entire length; however, it is more common between the origin of the left subclavian artery and the ductus arteriosus. Its incidence corresponds to 3 cases per 10,000 births. Thus, it is a common cardiopathy, but with high mortality and morbidity rates, which are related to a failure in the early diagnosis. METHOD: In the research, articles of the national and international literature in Pubmed, Scielo and Lilacs databases were selected using the following descriptors: coarctation, aorta, diagnosis, heart diseases, congenital abnormalities. RESULTS: The pathophysiology of CoA and its systemic implications in the life of newborn and adults are well elucidated. However, due to the lack of habit to palpate pulses and to check the blood pressure in both upper and lower limbs during the physical examination, it is still a pathology little diagnosed in childhood. There are several techniques used in the repair of coarctation, each with their specifics, although, when not treated, aneurysms, heart failure, coronary diseases, and stroke are the main complications arising from the evolution of this pathology, which explains the low survival rate of these patients. CONCLUSION: Coarctation of the aorta is, therefore, a cardiac malformation of significant importance due to its incidence and its significant mortality risk. In this sense, the early diagnosis stands out as an essential piece for better prognosis of the patient.


RESUMO INTRODUÇÃO: A coarctação da aorta é uma cardiopatia congênita caracterizada por um estreitamento que ocorre na artéria aorta. Essa constrição pode ocorrer em qualquer local ao longo de toda a sua extensão, entretanto, é mais comum entre a origem da artéria subclávia esquerda e o ducto arterioso. Sua incidência corresponde a três casos a cada 10.000 nascimentos, sendo, desse modo, uma cardiopatia comum, porém com elevada taxa de mortalidade e morbidade, as quais estão relacionadas à falha no diagnóstico precoce. MÉTODOS: Este artigo trata-se de uma revisão bibliográfica narrativa da literatura. Na pesquisa foram selecionados artigos na literatura nacional e internacional nas bases de dados PubMed, SciELO e Lilacs, utilizando-se os seguintes descritores: coarctação, aorta, diagnóstico, cardiopatias, anormalidades congênitas. RESULTADOS: A fisiopatologia da coarctação da aorta e as implicações sistêmicas dessa cardiopatia na vida do recém-nascido e do adulto estão bem elucidadas. Entretanto, devido à falta de costume em palpar pulsos e aferir a pressão arterial nos membros superiores e inferiores durante o exame físico, ainda é uma patologia pouco diagnosticada na infância. Existem diversas técnicas utilizadas no reparo da coarctação, cada uma com suas especificidades, porém, quando não tratada, aneurismas, insuficiência cardíaca, coronariopatias e acidentes vasculares encefálicos são as principais complicações provenientes da evolução dessa patologia, o que explica uma baixa sobrevida desses pacientes. CONCLUSÃO: A coarctação da aorta é, portanto, uma malformação cardíaca de importância relevante devido a sua incidência e ao seu potencial risco de mortalidade. Nesse sentido, o diagnóstico precoce destaca-se como peça fundamental para um melhor prognóstico do paciente.


Subject(s)
Humans , Aortic Coarctation/diagnosis , Aortic Coarctation/surgery , Aortic Coarctation/physiopathology , Early Diagnosis
10.
An. bras. dermatol ; 93(3): 405-411, May-June 2018. tab, graf
Article in English | LILACS | ID: biblio-949868

ABSTRACT

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.


Subject(s)
Humans , Aortic Coarctation/diagnosis , Facial Neoplasms/diagnosis , Eye Abnormalities/diagnosis , Neurocutaneous Syndromes/diagnosis , Hemangioma/diagnosis , Aortic Coarctation/complications , Aortic Coarctation/diagnostic imaging , Propranolol/therapeutic use , Brain/abnormalities , Brain/diagnostic imaging , Facial Neoplasms/drug therapy , Magnetic Resonance Imaging , Eye Abnormalities/complications , Eye Abnormalities/diagnostic imaging , Stroke/etiology , Neurocutaneous Syndromes/complications , Neurocutaneous Syndromes/diagnostic imaging , Face/diagnostic imaging , Hemangioma/drug therapy , Infant
11.
Univ. med ; 59(1)20180000. tab
Article in Spanish | LILACS, COLNAL | ID: biblio-994870

ABSTRACT

Introducción: La hipertensión arterial es una de las enfermedades más prevalentes en atención primaria y el principal factor de riesgo para enfermedad cardivoascular. La hipertensión arterial secundaria es frecuente entre los pacientes con diagnóstico de hipertensión arterial, con una prevalencia del 10 % que puede incrementar hasta 20 % o 40 % en pacientes con hipertensión refractaria al tratamiento. Su identificación temprana se asocia con mejores desenlaces. Objetivo: Evaluar en la literatura las principales causas de hipertensión arterial secundaria e identificar el abordaje diagnóstico inicial de las patologías asociadas. Métodos: Selección y lectura de artículos de bases de datos Pubmed y Google Scholar y de revisiones de UpToDate que trataran el tema de hipertensión arterial secundaria. Conclusiones: Es importante reconocer aquellos pacientes que puedan estar cursando con hipertensión arterial de causa secundaria, ya que esto modifica el enfoque terapéutico, facilita el tratamiento y mejora los desenlaces; incluso puede llegar a la cura y resolución.


Hypertension is one of the most common diseases encountered in primary care settings and a major risk. factor for cardiovascular disease. Secondary hypertension is common in patients with hypertension diagnosis; its prevalence is about 10% and can be as high as 40% in patients whom are resistant to treatment. Its early recognition and treatment allows for better outcomes. Objective: To evalúate and identify the main causes for secondary' hypertension and to identify the diagnosis and evaluation of related conditions. Nfethods: Selection and review of articles from Pubmed and Google scholar and Iiterature reviews from Uptodate. Conclusions: It is important to identify secondary hypertension since this will modify treatment, outcomes and in some scenarios might be curable.


Subject(s)
Sleep Apnea, Obstructive/pathology , Hyperaldosteronism , Hypertension/diagnosis , Aortic Coarctation/diagnosis , Hypertension, Renal/diagnosis
12.
Rev. bras. hipertens ; 24(4): 173-177, 20171210.
Article in Portuguese | LILACS | ID: biblio-1380168

ABSTRACT

A Coarctação de Aorta (CoA) é uma doença congênita que causa estreitamento do vaso ao longo de seu trajeto. Corresponde aproximadamente 5% das doenças cardíacas congênitas. A CoA frequentemente ocorre no arco distal ou na região descendente. As manifestações clínicas são diversas, sendo a hipertensão arterial um marcador importante de gravidade e lesões em orgãos-alvo. Muitos apresentam cefaleia, tontura, pulsação da cabeça e pescoço, fraqueza, claudicação de membros inferiores. Os aneurismas cerebrais correspondem a 50% nos pacientes com CoA, descritos em 1871 por Eppinger. A fisiopatologia é incerta, contudo é de conhecimento o baixo risco de ruptura e que seu tamanho é menor que os demais aneurismas. Raramente os aneurismas são encontrados em pessoas acima de 50 anos sendo mais comum entre 20-30 anos. Os pacientes que sobrevivem após os dois anos sem intervenção evoluem sem complicações nas maioria dos casos. Relato de Caso: paciente, 46 anos, engenheiro e jogador de futebol de salão master federado desde os 15 anos de idade. Apresentou-se na emergência com quadro de cefaleia de forte intensidade, a pior da vida, com paraparesia de membros inferiores, náuseas e vômitos. Ao exame físico estava em mal estado geral, sopro sistólico contínuo em região interescapular e sopro em diamante em região de precordio, pressão arterial de 180 x100 mmHg, normocardio, força muscular grau 2 em membros inferiores, ausência de pulsos pediosos em membros inferiores e anisocoria. Realizado tomografia de crâneo que evidencia a hemorragia subaracnoidea fisher 2, posteriormente com a angiotomografia cerebral constatou dois aneurismas saculares. Realizado eletrocardiograma, ecocardiograma e fundo de olho sem lesões de órgãos-alvo. Devido à característica do sopro evidenciado no exame e ao Rx de tórax com Sinal de Rosler e 3 invertido solicitou-se uma angiotomografia de aorta que mostrou uma CoA da aorta descendente. Comentários: o caso apresentando apresenta um paciente de 46 anos, jogador de futebol profissional sem sintomas até o presente momento, em tratamento irregular para hipertensão arterial desde os 24 anos de idade. Na grande maioria dos casos de CoA os pacientes adultos jovens e idosos evoluem com lesões de órgãos-alvo e complicações decorrentes da hipertensão de longa data. O presente caso demonstra a necessidade de um exame físico completo, a investigação ativa de hipertensão secundária em pacientes jovens


Coarctation of the Aorta (CoA) is a congenital disease that causes narrowing of the vessel along its way. Measure up 5 % of congenital heart disease. CoA often occurs in the distal arch or descending aorta. Clinical manifestations are diverse, arterial hypertension being an important marker of severity and lesions in organs, many show with headache, dizziness, pulsation of the head and neck, weakness, claudication of the legs. Described in 1871 by Eppinger, Cerebral aneurysms correspond to 50% in patients with CoA. Rarely, aneurysms are found in people over 50 years of age and are more common in the 20-30 year age group. Patients who survive without the necessity of intervention, after two years, have being develop uncomplicated. Case Report: patient, 46 years old, engineer and federated master soccer player since 15 years old. Admited in the emergency department reporting the worst headache of his life, associated, paraparesis of lower limbs, nausea and vomiting. At physical exam: continuous systolic murmur in interscapular region and diamond shaped systolic murmur at precordium. Arterial blood pressure was 180 x100 mmHg, normocardium, muscle strength level 2 in lower limbs , absence of pedis's pulses in lower limbs and anisocoria. Cranial CT scan showed hemorrhage subarachnoid (Fisher 2) and the cerebral angiotomography revealed two saccular aneurysms. Electrocardiogram, echocardiogram and fundus of the eye without lesions. Owing to the characteristic of the murmur evidenced at physical exam and the Rosler's Signal, "3 Sign" seen and at chest X- Ray, an aortic angiotomography was performed which showed a Coa of the descending aorta. Conclusion: the case presents a patient of 46 years, professional soccer player with no symptoms until the present moment, in irregular treatment for hypertension since the 24 years of age. In majority of cases of CoA, young-adult and elderly patients develop with target organ damage and complications resulting from long-standing hypertension. Therefore, a complete physical examination, the active investigation of secondary hypertension in young patients, becomes essential.


Subject(s)
Humans , Male , Middle Aged , Aortic Coarctation/diagnosis , Aneurysm/diagnosis
14.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 25(3): 141-146, jul.-set. 2015. ilus
Article in Portuguese | LILACS, SES-SP | ID: lil-788855

ABSTRACT

Os avanços na tecnologia nas últimas décadas em relação ao tratamento intervencionista das cardiopatias congênitas têm permitindo excelentes resultados, em termos de morbimortalidade, a curto e longo prazo para este grupo especial de pacientes.Este artigo descreve os principais tratamentos disponíveis realizados com cateterismo intervencionista neste grupo de pacientes.


Technological advances in recent decades, in relation to interventionist treatment of congenital heart defects, have enabled excellent short- and long-term results for this group of patients, in terms of morbidity and mortality. This article describes the main treatmentsavailable involving interventionist catheterization in this group of patients.


Subject(s)
Humans , Male , Female , Child , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/therapy , Catheterization/methods , Indicators of Morbidity and Mortality , Stents , Ductus Arteriosus/surgery , Aortic Coarctation/diagnosis , Aortic Coarctation/therapy , Heart Septal Defects, Atrial/diagnosis , Heart Septal Defects, Atrial/therapy , Echocardiography, Transesophageal/methods , Pulmonary Valve Stenosis/diagnosis , Pulmonary Valve Stenosis/therapy , Prostheses and Implants
16.
Rev. chil. dermatol ; 31(2): 185-193, 2015. ilus, tab
Article in Spanish | LILACS | ID: biblio-836020

ABSTRACT

El síndrome de PHACE consiste en una entidad neurocutánea, cuyo nombre deriva del acrónimo en inglés para la constelación de alteraciones asociadas a algunos hemangiomas segmentarios de localización cefálica-cervical: Malformaciones de fosa Posterior, hemangiomas, anormalidades arteriales, coartación de la aorta y defectos cardíacos, anomalías oculares. Cuando se encuentran defectos de la línea media (Sternal defects) se denomina síndrome de PHACES. Su etiología es desconocida y se presenta con mayor frecuencia en mujeres. Requiere de un estudio exhaustivo de malformaciones asociadas, dado que pueden ser causa importante de morbimortalidad. Presentamos nuestra experiencia en dos casos clínicos, con excelente respuesta a tratamiento con propranolol, junto con una revisión de la literatura.


PHACE syndrome is a neurocutaneous entity, derived from an acronym that describes the constellation of alterations associated with some segmental hemangiomas in the cephaliccervical region: Posterior fossa malformations, hemangiomas, arterial anomalies, aorta coarctation, cardiac defects, and eye abnormalities. When midline anomalies are found (Sternal defects) the term PHACES syndrome is used. Its etiology is unknown, and is more frequent in females. It requires a thorough study of associated malformation, because is an important source or morbimortality. We present our experience with two cases, with excelent response to treatment with propranolol, along with a review of the literature.


Subject(s)
Humans , Male , Female , Infant, Newborn , Eye Abnormalities/diagnosis , Aortic Coarctation/diagnosis , Neurocutaneous Syndromes/diagnosis , Neurocutaneous Syndromes/drug therapy , Eye Abnormalities/drug therapy , Aortic Coarctation/drug therapy , Magnetic Resonance Imaging , Propranolol/therapeutic use
17.
Arq. bras. cardiol ; 100(2): e16-e20, fev. 2013. ilus
Article in Portuguese | LILACS | ID: lil-667964

ABSTRACT

MSM, homem, 69 anos, procurou atendimento médico por dor em dorso esquerdo e membro inferior direito. A radiografia de tórax revelou alargamento do mediastino. Estava em observação quando apresentou rebaixamento da consciência e choque. Foi observado enfisema subcutâneo em hemitórax esquerdo e abolição do murmúrio vesicular em base do mesmo pulmão. Foi feita a intubação orotraqueal e realizada drenagem de hemitórax esquerdo, com saída de líquido serossanguinolento. O ecocardiograma revelou ventrículo esquerdo (D/S): 44/29 mm; septo 12 mm; parede posterior 13 mm; discreta dilatação em raiz da aorta, presença de lâmina de dissecção e hematoma periaórtico. As valvas e pericárdio eram normais. O paciente foi transferido para o InCor. O exame físico (21 out 2004: 10h45) revelou paciente sedado, com intubação orotraqueal, com palidez cutânea, frequência cardíaca 90 bpm, pressão arterial 130 x 80 mmHg, drenagem torácica sanguinolenta do dreno tórax. Eletrocardiograma - frequência 90 bpm, ritmo sinusal, baixa voltagem no plano frontal e diminuição de voltagem em derivações esquerdas (fig. 1). A tomografia revelou enfisema subcutâneo bilateral, aorta torácica com contornos imprecisos na sua porção descendente (da artéria subclávia até porção média), colapso do pulmão esquerdo e grande coleção de características hemáticas em mesmo hemitórax e no mediastino médio e posterior. Pequeno pneumotórax à direita; pequeno derrame pleural à direita com alterações do parênquima subjacente. A análise do coração foi prejudicada pela presença do hemotórax. Durante a realização de tomografia apresentou ausência de pulsos, midríase, com assistolia, sem resposta às manobras de ressuscitação e faleceu (21 out 2011; 15h).


The patient, MSM, a 69-year-old man, sought medical care due to left dorsal and right lower limb pain. The chest x-ray showed mediastinal enlargement. He was undergoing examination when he lost consciousness and went into shock. Subcutaneous emphysema was observed in the left hemithorax, as well as abolition of breath sounds at auscultation. Tracheal intubation was performed with draining of blood-tinged fluid from the left hemithorax. Echocardiography showed left ventricle with 44/29 mm; septum, 12 mm; posterior wall, 13 mm; mild aortic root dilation, dissection of the lamina and periaortic hematoma. The valves and pericardium were normal. The patient was transferred to Instituto do Coraçao - InCor. Physical examination (21 Oct 2004: 10:45) showed that the patient was sedated with tracheal intubation, pale, heart rate at 90 bpm, blood pressure 130 x 80 mmHg, bloody drainage in the chest tube. Electrocardiogram - frequency 90 bpm, sinus rhythm, low voltage in the frontal plane and decreased voltage in left leads (Fig. 1). Computed tomography showed bilateral subcutaneous emphysema, thoracic aorta with inaccurate borders in its descending portion (from the subclavian artery to the middle portion), collapsed left lung and extensive collection of hematic characteristics in same hemithorax and middle and posterior mediastinum. Small right pneumothorax; small right pleural effusion with underlying parenchymal alterations. The analysis of the heart was impaired by the presence of hemothorax. While undergoing computed tomography, the patient showed no pulse, mydriasis, with asystole unresponsive to resuscitation and died (21 Oct 2011; 15:00 h).


Subject(s)
Aged , Humans , Male , Aortic Aneurysm, Thoracic/complications , Aortic Coarctation/complications , Shock/etiology , Aortic Aneurysm, Thoracic/pathology , Aortic Coarctation/diagnosis , Aortic Coarctation/pathology , Back Pain/etiology , Chest Pain/etiology , Fatal Outcome , Hemothorax/etiology , Hemothorax/pathology , Lower Extremity
19.
S. Afr. fam. pract. (2004, Online) ; 55(2): 168-169, 2013.
Article in English | AIM | ID: biblio-1270018

ABSTRACT

Coarctation of the aorta is a congenital condition generally; and may vary from mild to severe. Symptomatology depends on early or late presentation. Young patients may present within the first few weeks of life with poor feeding; tachypnea and lethargy. They usually progress to overt congestive heart failure and shock. Older children may present with a murmur or hypertension. Diagnosis is often made after hypertension is detected as an incidental finding during evaluation of other problems; such as trauma or more common illnesses. Coarctation of the aorta can be cured surgically


Subject(s)
Aortic Coarctation/diagnosis , Aortic Valve Stenosis , Hypertension/prevention & control , Plastic Surgery Procedures
20.
Article in English | IMSEAR | ID: sea-157463

ABSTRACT

Coarctation of the aorta is a congenital cardiac malformation that can go undiagnosed with only hypertension as a marker of its presence, because clinical signs can be subtle and overlooked if a complete physical examination is not performed. Here we present a case report of 20 year old young male patient with hypertension who was diagnosed to have Coarctation of Aorta and Bicuspid aortic Valve.Our patient was relatively asymptomatic until he presented with headache and exertional dyspnoea.


Subject(s)
Aortic Coarctation/complications , Aortic Coarctation/diagnosis , Aortic Coarctation/therapy , Aortic Valve/abnormalities , Aortic Valve/complications , Aortic Valve/diagnosis , Aortic Valve/therapy , Dyspnea/etiology , Hypertension/etiology , Heart Valve Diseases/complications , Heart Valve Diseases/diagnosis , Heart Valve Diseases/therapy , Humans , Male
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