Subject(s)
Angioedemas, Hereditary/physiopathology , Pregnancy Complications, Cardiovascular/physiopathology , Pulmonary Embolism/physiopathology , Venous Thrombosis/physiopathology , Adult , Angioedemas, Hereditary/complications , Cesarean Section , Female , Humans , Pregnancy , Pregnancy Complications, Cardiovascular/genetics , Pulmonary Embolism/congenital , Risk Factors , Venous Thrombosis/congenitalABSTRACT
Pulmonary embolism is a complication of deep vein thrombosis. It occurs in the population with a normal clotting mechanism, but it may also occur in patients with congenital bleeding conditions. Here, we report on all cases of pulmonary embolism in congenital hemorrhagic disorders. All reported cases of pulmonary embolism in congenital coagulation disorders have been gathered by a time-unlimited PubMed search. Cross-checking of the references listed at the end of the single papers was carried out to avoid omissions. Seventy-two patients had an objectively demonstrated pulmonary embolism. The event occurred in patients with fibrinogen, factor V, factor VIII (FVII), FVIII, FIX, and FXI deficiency, and in those with von Willebrand's disease. No embolism was reported in FII, factor X, and FXIII deficiency. Thirty were women and 28 were men, whereas in the remaining 14 cases, sex was not reported. Age varied from 6 to 81 years (mean age 34.3 years). The management varied from only supportive to the administration of unfractionated heparin, low-molecular-weight heparin, and anti-vitamin K medications, accompanied by adequate replacement therapy. Evolution was fair or good in the majority of cases, but there were 10 fatalities. Risk factors were present in 61 patients. The most frequent of these were replacement therapy (35 cases), surgery (34), and old age (13). Some patients had more than one risk factor. Eleven patients had no risk factors. There are discrepancies in the prevalence of pulmonary embolism among different clotting disorders. The conditions most frequently affected are FVII deficiency and fibrinogen defects. The significance of the findings is discussed.
Subject(s)
Coagulation Protein Disorders/blood , Coagulation Protein Disorders/drug therapy , Fibrinolytic Agents/therapeutic use , Pulmonary Embolism/blood , Pulmonary Embolism/drug therapy , Adolescent , Adult , Aged , Aged, 80 and over , Blood Coagulation Factors/therapeutic use , Child , Coagulation Protein Disorders/complications , Coagulation Protein Disorders/congenital , Female , Heparin, Low-Molecular-Weight/therapeutic use , Humans , Male , Middle Aged , Pulmonary Embolism/complications , Pulmonary Embolism/congenitalSubject(s)
Catheterization, Swan-Ganz/methods , Clinical Protocols , Pulmonary Embolism/congenital , Pulmonary Embolism/surgery , Adult , Aged , Cardiac Catheterization/methods , Computed Tomography Angiography , Echocardiography, Transesophageal , Female , Humans , Male , Pulmonary Embolism/diagnostic imagingABSTRACT
Intrauterine pulmonary infarction is extremely rare. In a series of 775 stillborn and newborn infants, only a few cases of acute pulmonary infarction were reported. The authors present the findings from a full-term infant who died at 9 hours of age of a chronic pulmonary infarction. Several pathogenic mechanisms, including the entry of placental thromboplastin into the fetal circulation, are discussed.
Subject(s)
Pulmonary Embolism/congenital , Pulmonary Embolism/pathology , Fatal Outcome , Humans , Infant, Newborn , Male , Placenta/pathology , Pulmonary Embolism/diagnosisABSTRACT
We report an infant with a bronchiolitis-like illness and rapid deterioration who developed a cor pulmonale-like picture with a dilated right ventricle. Urinary organic acid assays established a probable diagnosis of Cbl-C-type methylmalonic aciduria, later confirmed by complementation studies. Despite medical intervention and cyanocobalamin treatment the patient died on his tenth hospital day. Postmortem examination showed the presence of thromboemboli in the pulmonary circulation. We hypothesize that acute cor pulmonale developed in this infant secondary to thromboembolism of his pulmonary circulation. A review of the literature shows that thromboembolism may be a part of this disease process.
Subject(s)
Homocystinuria/complications , Malonates/blood , Methylmalonic Acid/blood , Pulmonary Heart Disease/complications , Heart Ventricles/pathology , Homocystinuria/pathology , Humans , Infant , Male , Postmortem Changes , Pulmonary Embolism/complications , Pulmonary Embolism/congenital , Pulmonary Embolism/pathology , Pulmonary Heart Disease/congenitalABSTRACT
Authors report a case of newborn with thrombosis of the pulmonary artery, probably caused by a maternal deficit of antithrombin III. Deficit of antithrombin III (AT III) as the possible cause of thrombosis is discussed.