Spectrum of cardiac lesions associated with Isolated Cleft Mitral Valve and their impact on therapeutic choices

Abstract Background: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management. Methods: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014. Results: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation. Conclusion: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.

Estenose hipertrófica do piloro: caracterização clínica, radiológica e ecográfica

Estenose hipertrófica do piloro é uma condição comum em infantes com 2 - 12 semanas de idade e cuja causa permanece desconhecida. O diagnóstico clínico baseia-se na história de vômitos não-biliosos em jato, sinais de hiperperistalse gástrica e "tumor" pilórico palpável ao exame físico. Os autores ilustram os típicos achados desta alteração em seriografias do trato gastrointestinal superior e na ultra-sonografia abdominal. Critérios diagnósticos são descritos e as aplicações desses dois métodos são estabelecidos segundo a literatura vigente.

O uso de stents farmacológicos no tratamento da estenose das artérias vertebrais

OBJETIVO: Relatar a viabilidade e segurança da angioplastia transluminal percutânea com stents recobertos com paclitaxel para tratamento de estenose de artéria vertebral em 14 pacientes, após seguimento de dois anos. MATERIAIS E MÉTODOS: Catorze pacientes com idade média de 67,2 anos foram submetidos a tratamento endovascular mediante angioplastia percutânea e implante de stent farmacológico. O objetivo primário deste trabalho foi assegurar a segurança do procedimento. O desfecho secundário foi a eficácia clínica, definida como sintomas isquêmicos recorrentes e taxas de reestenose. RESULTADOS: O grau de estenose variou de 50% a 99% (média de 73,3% ± 10,9). A taxa de sucesso técnico da angioplastia foi de 100%. Não houve complicações diretamente relacionadas ao procedimento. Aos 24 meses de seguimento, nenhum paciente apresentou recorrência dos sintomas. A taxa de reestenose intra-stent foi de 7,1%, embora tenha sido assintomática na totalidade dos casos. CONCLUSÃO: Este estudo sugere que a angioplastia da artéria vertebral com o uso de stents recobertos com paclitaxel é uma técnica viável e promissora em termos de segurança e eficácia na prevenção da isquemia recorrente e reestenose.

Estenose arterial pós-transplante hepático: tratamento com angioplastia transluminal percutânea

Vascular complications after liver transplantation include oclusion or stenosis at the sites of anastomosis in the hepatic artery, portal vein, and vena cava. Balloon angioplasty of these stenosis carries little risk and is a useful procedure for the treatment of these problems. The purpose of this paper was to assess whether percutaneous transluminal angioplasty can help to prolong allograft survival and impruve allograft function in patient with hepatic artery stenosis after liver transplantation. We report a 43-year-old mate with stenosis of hepatic artery anastomosis after liver transplantation. An abrupt elevation of liver enzymes and serum bilirrubin levels was noted on the fifth postoperative month. The patient underwent percutaneous liver biopsy, which revealed important ductal depletion due to hypoperfusion, even though Doppler ultrasound examination demonstrated arterial flow. An angiogram confirmed severe stenosis of the arterial anastomosis with poor intraparenchymal arterial perfusion pattern. In an attempt to preserve the graft, a percutaneous transluminal angioplasty was performed using microballoons mounted on a hydrophylic micro guidewire. Intervention proceeded without complications. Liver enzimes and bilirrubin levels decreased within twenty-four hours of angioplasty. Normal levels were achieved after one week. Seven month after angioplasty, the patient is in a optimal clinical condition with no signs of graft impairment. We conclude that percutaneous transluminal angioplasty of hepatic artery stenosis after liver transplantation is relatively safe and may help decrease allograft loss.

Estenose esofagiana congênita por coristoma

Oesophageal choristomas are a very uncommon cause of congenital oesophageal stenosis. A high index of suspection is necessary for differential diagnosis with commoner causes of oesophageal suboclusion in childhood, mainly acquired oesophageal obstruction caused by gastroesophageal reflux. We present a case report and review the clinical, endoscopic and radiologic features of the disease and consider the need for padronization of surgical techniques to treat this condition, which is still controversial.