Ethanol sclerotherapy for the treatment of low-flow vascular malformations can cause catastrophic cardiopulmonary complications, including pulmonary embolism and pulmonary hypertension, that can result in right heart failure and fatal arrhythmias, leading to death. We here report a case of abrupt cardiovascular collapse that developed immediately following ethanol sclerotherapy in 31-year-old f...

A microscopic study was made of the lungs of 18 patients with congenital malformations of the heart in which there was obstruction of pulmonary venous drainage. Alterations in various components of the pulmonary vascular bed and of the lung parenchyma were described and correlated with the clinical course and the type of malformation present. A quantitative evaluation of the pulmonary arterial ...

Pulmonary arteriovenous fistulas (PAVFs) are rare vascular malformations (PAVMs) of the lung that could lead to severe hypoxiemia due to right-to-left intrapulmonary shunts. They may occur as isolated entities or associated with Osler-Rendu-Weber syndrome or hereditary haemorrhagic telangiectasia (HHT). We study 3 patients with Rendu-OslerWeber disease and a large arteriovenous malformation inv...

OBJECTIVE To investigate changes over time in the prevalence at live birth of cardiovascular malformations and to compare "anatomical" and "physiological" diagnostic hierarchies within a population. DESIGN Retrospective and prospective ascertainment of all congenital cardiovascular malformations diagnosed in infancy. SETTING The resident population of one health region. PATIENTS All infan...

Background Knowledge of caval contribution to flow in the Right and Left Pulmonary Artery (RPA and LPA) of Fontan patients is of great interest, because uneven flow distribution to the lungs is associated with pulmonary arteriovenous malformations. However, in Fontan circulation more than 1 source of flow to the RPA and LPA is present, which makes difficult to quantify the flow distribution wit...

Pulmonary bulla in children represent interesting entities. Mostly congenital bronchopulmonary foregut malformations and acquired cysts like pneumatocoeles have also been described. We present a case of a 12 1/2 years old girl with acute onset respiratory distress symptoms harbouring a huge pulmonary cyst exhibiting mass effects, resulting in mediastinal deviation. Following initial workup, cys...

Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we pr...

Thoracic congenital malformations may be associated with long-term pulmonary morbidity. Certainly, this is the case for Esophageal Atresia (EA) and Congenital Diaphragmatic Hernia (CDH). These conditions have variable degrees of impaired development of both the airways and the lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications themselves...

DURING the past decade vascular malformations of the superior mediastinum have been the object of intense clinical interest. However, the occurrence of anomalies of the pulmonary artery as a cause of tracheobronchial obstruction has gone almost unrecognized. We previously reported 3 cases in which significant respiratory obstruction was produced by a left pulmonary artery coursing anomalously a...

BACKGROUND Surgical excision is useful only for localized and limited lesions. The purpose of this study was to evaluate the clinical curative effect of percutaneous sclerotherapy of massive venous malformations of the face and neck using fibrin glue combined with OK-432 and pingyangmycin. METHODS Eighteen patients with massive venous malformations were treated with an injection of fibrin glu...